2014 - endo bridge · 2020. 11. 18. · 2014 5 23-26 october, 2014 regnum carya hotel, antalya -...

23-26 October, 2014Regnum Carya Hotel, Antalya - Turkey

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Bridging the World of Endocrinology

SELECTED CASES

2014

2014

5 23-26 October, 2014 Regnum Carya Hotel, Antalya - Turkey

SELECTED CASESDATE TIME SESSION HALL SUBJECT

24.10 11.20-12.50 Pituitary A O-02 / CUSHING'S SYNDROME DURING PREGNANCY

24.10 11.20-12.50 Pituitary A O-03 / AN ACTH DEPENDENT CUSHING’S SYNDROME WITH UNKNOWN ECTOPIC ORIGIN

24.10 11.20-12.50 Pituitary A O-04 / HYPOPHYSITIS IN A MALE PATIENT

24.10 11.20-12.50 Thyroid BO-09 / PROGRESSIVE SEVERE DYSPNEA AND HYPOXIA DUE TO THYROID LYMPHOMA: STEROID ADMINISTRATION MAY BE LIFE-SAVING

24.10 11.20-12.50 Obesity / Lipids CO-12 / GESTATIONAL DIABETES MELLITUS ACCOMPANIED WITH SEVERE NON-FAMILIAL HYPERTRIGLYCERIDEMIA, A CASE REPORT

24.10 11:20-12:50Male

reproductive endocrinology

D

O-13 / IDENTIFYING MALE HYPOGONADISM, RECOGNIZING THE ASSOCIATION OF HYPOGONADISM WITH COMORBIDITIES SUCH AS METABOLIC SYNDROME, DIABETES AND OBESITY

24.10 14.00-15.30 Pituitary A O-06 / ROLE OF STEREOTACTIC RADIOSURGERY FOR TREATMENT OF HYPOPHYSITIS

24.10 14.00-15.30 Pituitary AO-07 / RESTORATION OF FERTILITY IN A MAN WITH MACROPROLACTINOMA AND POSTOPERATIVE HYPOPITUITARISM

24.10 14.00-15.30 Pituitary A O-08 / SILENT CORTICOTROPH PITUITARY ADENOMA PROGRESSING TO CUSHING DISEASE

24.10 14.00-15.30 Thyroid B

O-10 / PAPILLARY MICROCARCINOMA IN A PATIENT WITH MULTIPLE FINE NEEDLE THYROID ASPIRATIONS RESULTING IN ATYPIA OF UNDETERMINED SIGNIFICANCE

24.10 14.00-15.30 Thyroid B O-11 / A RARE CONDITION: UNILATERAL GRAVES’ DISEASE

24.10 14:00-15:30Female


D O-14 / INFERTILITY DUE TO COMBINATION OF ENDOCRINE, AUTOIMMUNE AND GENETIC CAUSES

24.10 14:00-15:30Female


D O-15 / APPROACH TO CONGENITAL ADRENAL HYPERPLASIA IN PREGNANCY

24.10 14:00-15:30Female


D

O-16 / TESTOSTERONE AND CORTISOL SECRETING OVARIAN STEROID CELL TUMOR IN A POSTMENOPAUSAL WOMAN: AN UNUSUAL CAUSE OF HIRSUTISM

24.10 14:00-15:30Female


D O-19 / A YOUNG WOMAN WITH DIABETES & PRIMARY AMENORRHOEA

2014


DATE TIME SESSION HALL SUBJECT

25.10 11.20-12.50 Diabetes A O-17 / DO GUIDELINES ALWAYS SHOW THE DIRECTION?

25.10 11:20-12:50 Diabetes A O-18 / A GIRL WITH SEVERE INSULIN RESISTANCE AND TYPE 2 DM DIAGNOSED AS ALSTROM SYNDROME

25.10 11:20-12:50 Adrenal B O-23 / A RARE CAUSE OF ADRENOCORTICOTROPIC HORMONE INDEPENDENT CUSHING'S SYNDROME

25.10 11:20-12:50 Adrenal B O-24 / A RARE CASE:MALIGNANT PARAGANGLIOMA

25.10 11:20-12:50 Bone / Calcium C O-20 / A 43-YEAR OLD MAN WITH SEVERE OSTEOPOROSIS

25.10 11.20-12.50 Bone/Calcium C O-25 / CASE STUDY OF POSTMENOPAUSAL WOMAN

25.10 11:20-12:50 Bone/Calcium C O-26 / A RARE CAUSE OF PRIMARY HYPERPARATHYROIDISM: PARATHYROID CARCINOMA

25.10 11:20-12:50 Neuroendocrine tumors D O-29 / ECTOPIC ACTH SYNDROME

25.10 14:00-15:30 Adrenal B O-21 / PRIMARY ADRENAL LYMPHOMA AS A RARE CAUSE OF UNILATERAL ADRENAL MASS

25.10 14:00-15:30 Adrenal BO-22 / A RARE CAUSE OF HYPERTENSION, ALDOSTERONE SECRETING ADRENOCORTICAL NEOPLASIA ; A CASE REPORT

25.10 14.00-15.30 Bone/Calcium C O-27 / A VEILED FEMALE PATIENT WITH OSTEOMALACIA DIAGNOSED AS CELIAC DISEASE

25.10 14.00-15.30 Bone/Calcium C O-28 / THE USE OF CINACALCET IN PRIMARY HYPERPARATHYROIDISM: A PRACTICE REVIEW

25.10 14.00-15.30 Neuroendocrine tumors D

O-30 / AN INSULINOMA LOCALISED BY BOTH IMAGING STUDIES AND SELECTIVE ARTERIAL CALCIUM STIMULATION TEST

2014


ORAL PRESENTATIONS

2014


Introduction: Cushing’ syndrome (CS) is extremely rare in pregnancy with fewer than 150 cases in the world literature as individual cases and small case series. The clinical di-agnosis of CS may be missed because of the overlapping features of weight gain, hypertension, fatigue, hyperglyce-mia, and emotional changes that occur in pregnancy. The biochemical diagnosis may also be difficult to establish be-cause of the normal hypercortisolism of pregnancy. Here, we report a case of CS during pregnancy due to a pituitary adenoma presenting with severe hypertension and diabetes.

Clinical case: A 33-year-old pregnant woman (gravida 2 para 1) was admitted at 12 weeks of gestation with the com-plaints of headache, blurred vision, increased somnolence, and asthenia. On admission, she was found to have a blood pressure of 170/95 mmHg. Physical examination revealed mild moon face, facial acne, hirsutism, wide and purple stri-ae over the abdomen, and grade 3 pitting bipedal edema. Se-vere proximal muscle weakness was also noticed. Laboratory investigations showed hyperglycemia, severe hypokalemia, and proteinuria. Further investigations revealed high urinary and serum cortisol levels with loss of the normal circadian variation, accompanied by lack of supression of serum cor-tisol after both 2 and 8 mg of dexamethasone, supporting

the diagnosis of CS. She was started on anti-hypertensive treatment with alpha-methyldopa. Her blood pressure re-main elevated with maximum dose of alpha-methyldopa, so nifedipine was subsequently added. Diabetes was managed with basal-bolus insuline therapy. During follow-up, her blood pressure remained uncontrolled, the degree of ede-ma increased, her platelet level began to decrease, and her conciousness level began to disturb. After the consultation with obstetricians, termination of pregnancy was decided. A therapeutic abortion was performed at the 14 weeks of gestation. After abortion, pituitary MRI was obtained and re-vealed a 4-mm lesion on the right side of the pituitary. Infe-rior petrosal sinus sampling demonstrated a maximum cen-tral to peripheral ACTH gradient of 12.9, indicating CD. The patient underwent transsphenoidal surgery. Histopatholog-ical examination confirmed a pituitary adenoma with posi-tive immunohistochemical staining for ACTH. Postoperative seum ACTH and cortisol levels were suggestive of complete remission and hypertension, diabetes, and proteinuria re-solved very soon after the operation. Conclusion: If undi-agnosed and hence untreated, outcomes are poor for both mother and the fetus in CS during pregnancy. Best outcome is achieved by a multidisciplinary approach towards an indi-vidualised management.

O-02 Pituitary

CUSHING’S SYNDROME DURING PREGNANCYNAFIYE HELVACI, MIYASE BAYRAKTAR

HACETTEPE UNIVERSITY SCHOOL OF MEDICINE, DEPARTMENT OF INTERNAL MEDICINE, DIVISION OF ENDOCRINOLOGY AND METABOLISM,ANKARA, TURKEY

2014


ACTH-dependent Cushing’ syndrome (CS) is characterized by ACTH excess, arising either from tumors of the pituitary or ectopic tumors like small cell lung carcinomas, medul-lary thyroid carcinomas, pheochromocytomas, as well as thymic, pancreatic, and bronchial carcinoids which are often indolent. High dose dexamethasone suppression and CRH stimulation tests, magnetic resonance imaging (MRI) and in-ferior petrosal sinus sampling (IPSS) is the diagnostic tests for distinguishing between pituitary and ectopic ACTH-se-creting tumors. We are presenting here an ACTH-dependent Cushing’ syndrome case with unknown ectopic origin.

Clinical Case: A 18 year old women who applied with the symptoms of hypertension, weight gain, strias on the ab-domen. Basal ACTH and cortisol levels were 123 pg/ml and 36.19 mcg/dl, respectively. The diagnostic 2 mg and 8 mg dexamethasone suppression are resulted with serum cor-tisol levels 15.7 and 25.5 mcg/dl, respectively. On MRI scan, a hypo-intense 2.2x1.8 mm adenoma was found in the right lobe of hypophysis. Abdominal tomography displayed bilat-eral adrenal gland hyperplasia. DOTA 68 GA scintigraphy showed lymph nodes in the right hilus that were smaller

than 1 cm and not strongly visualized. Bronchoscopic biop-sy and mediastinoscopy weren €™t suitable for the lesions and bronhco-alveolar lavage was not diagnostic. IPSS was performed two times and it’ results supported ectopic ACTH syndrome (EAS). FDG PET was negative for localization of the ectopic ACTH secretion. Ketoconazole was given for blocking of adrenal corticosteroid synthesis before opera-tion. The patient underwent to bilateral adrenolectomy and the pathology was bilateral adrenal cortex hyperplasia. The prednisolone and fludrocortisone was started and the pa-tient has followed up for the origin of EAS.

Conclusion: Localization of ectopic ACTH-secreting tumors is often frustrating. Up to 50% of these neoplasms are not initially detectable, despite complete radiological screen-ing and venous ACTH sampling.Long-term follow-up is of-ten necessary, and the primary lesion is generally discov-ered within 2 - 5 years of follow-up. Particular attention is required to check the lung and thymus where epithelial or carcinoid tumors are the main causes of ectopic ACTH pro-duction

O-03 Pituitary

AN ACTH DEPENDENT CUSHING’ SYNDROME WITH UNKNOWN ECTOPIC ORIGIN

ISMAIL BEYPINAR1, DEMET SAHINER LAZZARI1, HUMEYRA BOZOGLAN2, RAMAZAN SARI2, HASAN ALTUNBAS2, MUSTAFA KEMAL BALCI2

1 DEPARTMENT OF INTERNAL MEDICINE 2 DEPARTMENT OF ENDOCRINOLOGY AND METABOLISM

2014


Introduction: Several forms of inflammation of the pituitary can be recognized histologically,including lymphocytic,gran-ulomatous and xanthomatous. Lymphocytic hypophysitis is the most common form.There is no specific biochemical or serological marker.It occurs mostly in women especially in the peripartum period and seen rarely in men.Here we re-port a male patient with hypophysitis.

Case Report: A 28-year-old man admitted with complaints of headache, naussea, fatigue and decreased libido for the past 9 months.He had no past medical history of autoim-mune disease. The first laboratory results were; glucose:

82 mg/dl (70-110) Na:136 mmol/L (135-146), K:3,87 mmol/L (3,5-5,1).His fT3:2,04 pg/ml (2.5-3,9) fT4: 0,05 ng/dl (0,61-1,2).TSH:0,46 µIU/ml (0,34-5,6),FSH:2,68 mIU/mL(1,27-19,26),LH:0,1 mIU/mL(1,24-8,62),total testosteron

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O-04 Pituitary

HYPOPHYSITIS IN A MALE PATIENTOZDEN OZDEMIR BASER1, EDA DEMIR ONAL1, NARIN NASIROGLU IMGA1, DILEK SENOZ BERKER1,

SERDAR GULER2

1 ANKARA NUMUNE EGITIM VE ARASTIRMA HASTANESI ENDOKRINOLOJI VE METABOLIZMA HASTALIKLARI UNITESI 2 CORUM HITIT UNIVERSITESI ENDOKRINOLOJI VE METABOLIZMA HASTALIKLARI UNITESI

2014


Background:There has been limited experience on the role of radiosurgery for hypophysitis.

Clinical Case: First case is a 42 years old female.4 years ago she was admitted for her complaints of headache, menstru-al irregularity and galactorrhea.Pituitary hormone levels at first diagnosis are presented in Table 1.MRI revealed wid-ening of the sella, with a 1 cm necrotic cystic lesion.She underwent biopsy by transsphenoidal surgery.The histo-pathological work-up confirmed diagnosis of xanthomatous hypophysitis.Whole body bone scintigraphy and FDG-PET did not reveal inflammation outside the hypophysis.Methyl-prednisolone (120 mg/day) was initiated and the dose was decreased over 3 months.However she had recurrences of headache, fever and nuchal rigidity at each attempt to de-crease the dose.The recurrences were of hypophysitis and improved after high dose steroid therapy.After a total of 4 re-currences azothiopurine(AZT) 50 mg/day was initiated how-ever due to hepatotoxicity the dose could not be increased further.Gamma-knife stereotactic radiosurgery(GKR) was performed after 2 years of diagnosis.Sella MRI obtained 3 months after GKR showed the lesion decreased in size.Addi-tionally headaches improved after GKR. About 8 months af-ter GKR she had pituitary abscess which improved after an-tibiotherapy.AZT was discontinued and steroid was reduced to low-replacement dose.After 2 years of GKN she has no recurrence of hypophysitis. Her headache improved, and in the latest sella MRI the lesion decreased further in size.After GKR it was possible to decrease the steroid dose. Even

she is on 7.5 mg/day of prednisolon for about 10 months, still she has not any recurrence.

The second case is a 29 years old female.3 years ago she was admitted for her complaints of headache, menstrual irregularity, galactorrhea polydipsia and polyuria. Pituitary hormone levels at first diagnosis are presented in Table 1.Water deprivation test confirmed central diabetes insipi-dus.MRI revealed paritial empty sella, pituitary height was 2 mm at midline and the infundibilum was thick with contrast enhancement.The MRI findings were compatible with lym-phocytic hypophysitis. Cabergoline and nasal desmopressin acetate were initiated.During follow-up headaches did not remit and menstrual irregularity did not improve.Also cen-tral hypothyroidism, growth hormone deficiency and hypo-cortisolism emerged. Hormone replacement therapies,50 mg/day AZT and 60 mg/day prednisolon were initiated.While she was on 32 mg/day prednisolon she had bilateral avas-cular necrosis of the knees and hips. Steroid treatment was changed to 15 mg/day of hydrocortisone, and Cyber-knife stereotactic radiosurgery(CKR) was applied for the hypoph-ysitis.2 months after CKR, findings on MRI improved slight-ly.14 months after CKR she has persistent hormone defi-ciencies and hyperprolactinemia however her headaches improved.

Conclusion:Stereotactic radiosurgery may be beneficial for improvement of the symptoms in persistent hypophysitis

O-06 Pituitary

ROLE OF STEREOTACTIC RADIOSURGERY FOR TREATMENT OF HYPOPHYSITIS

ESRA HATIPOGLU, PINAR KADIOGLUDIVISION OF ENDOCRINOLOGY AND METABOLISM, DEPARTMENT OF INTERNAL MEDICINE, CERRAHPASA MEDICAL SCHOOL,

UNIVERSITY OF ISTANBUL, ISTANBUL, TURKEY

2014


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2014


Introduction: Testosterone has been known to increase tu-mor size and elevate serum prolactin levels in men with re-sistant prolactinomas. Herein, we present a man with mac-roprolactinoma and postoperative hypopituitarism, whose fertility was restored with high dose cabergoline (CAB), aro-matase inhibitor and human chorionic gonadotropin (HCG).

Clinical Case: A 25-year-old man was admitted with the story of a transcranial operation for pituitary macroadenoma per-formed at another center a year before. Headache and loss of libido were his complaints before that operation, his baseline serum prolactin was 470ng/ml (normal limits:4.6-21.4). Pre-operative Magnetic Resonance Imaging (MRI) reported a pi-tuitary mass of 45x26mm with compression of optic chiasm. Immunhistochemical examination of operation specimens demonstrated a prolactin (+) pituitary adenoma with Ki-67 3%. He had postoperative hypopituitarism and was on levothyrox-ine 100mcg/day, transdermal testosterone 50mg/day, pred-nisolone 5mg/day and CAB 1.5mg/week on admission.In our center, an adenoma fulfilling the pituitary fossa with erosion of sphenoidal bone was reported at MRI. Serum prolactin was measured following dilution and was 7205.2ng/ml. Diagnosis of a macroprolactinoma with postoperative hypopituitarism was

performed. Considering about mass enlarging effect of tes-tosterone on prolactinomas in men, transdermal testosterone was withdrawn. Cabergoline dose was titrated up to 3.5mg/week within a few weeks.At 24th week of high dose CAB, he still suffered from sexual dysfunction. His prolactin was mildly elevated, testosterone was undetectable. Pituitary MRI demon-strated significant tumor shrinkage. Anastrozole 1mg/day, and transdermal testosterone 50mg/day were added. On the first month of therapy, he reported significant improvement in sexual functions, though his testosterone was low, but detect-able, with stable prolactin.He again admitted for fertility, while he was using those mentioned above. Pituitary MRI demon-strated insignificant tumor change. Transdermal testosterone was stopped, HCG 5000IU twice/week was introduced. At third month of therapy, his total testosterone was within high nor-mal limits with elevated prolactin. Spermiogram demonstrated motile sperms (of B-C-D groups) in his semen. Due to the risk of tumor enlargement, sperm preservation was performed. His former exogenous testosterone was reintroduced.

Conclusion: We believe this case is worth reporting as he provides new therapeutic strategies for the treatment of re-sistant prolactinomas.

O-07 Pituitary

RESTORATION OF FERTILITY IN A MAN WITH MACROPROLACTINOMA AND POSTOPERATIVE

HYPOPITUITARISMMELEK EDA ERTORER, OKAN BAKINER, EMRE BOZKIRLI, FILIZ E. HAYDARDEDEOGLU,

AYSENUR I. TORUNBASKENT UNIVERSITY, FACULTY OF MEDICINE, DIVISION OF ENDOCRINOLOGY, ADANA

2014


2014


Introduction: Subclinical Cushing Syndrome has been firstly defined in patients with adrenal incidentaloma who lack clinical symptoms of hypercortisolism. Patients with incidental pitu-itary adenoma or adrenocortical carcinoma may also have this syndrome. We present a case of subclinical Cushing syndrome admitted with an incidental pituitary adenoma who developed clinical Cushing disease during follow-up.

Clinical case: A 49-yr-old woman was referred for a pituitary adenoma detected incidentally in cranial magnetic resonance imaging (MRI) performed for dizziness by a neurologist. The patient had no other complaint. She did not have diabetes mel-litus or hypertension. At physical examination, blood pressure was normal, her body mass index was 23 kg/m2. Facial pleth-ora, moon-face or purple striae were not present. At labora-tory examination, serum glucose, potassium and hormones of anterior hypophysis were detected at reference ranges. Bone mineral densitometry was normal. A 5x8 mm adenoma was present at pituitary MRI. Endogenous autonomous hypercorti-solemia was present with a morning ACTH level of 45 pg/ml re-vealing ACTH dependent Cushing. Pseudocushing was exclud-ed by low dose dexamethasone supression test (DST) combined with CRH. Inferior Petrosal Sinus Sampling test confirmed the central origin (Table). Regarding lack of clinical symptoms of Cushing syndrome, mutation analysis for a generalised gluco-corticoid resistence syndrome was performed, it was negative. Six months later, the patient did not have any complaints. Phys-ical examination was normal. The size of the adenoma was the same. After -one year from the last control visit, the patient ad-mitted to the clinic with a complaint of progressive weight gain. She had gained approximately 15 kg during the -six months be-fore admission. At physical examination, she had facial pleth-ora, moon-face, central obesity and supraclavicular fat pads. There was no supression by DST, 24 hour urinary cortisol was

high (690 mcg/day). ACTH was 83.6 pg/ml. A progression in the size of pituitary adenoma was detected (15x13 mm). Trans-sphenoidal pituitary surgery was suggested to the patient with the diagnosis of clinical Cushing disease.

Conclusion: Among non-functional corticotroph adenomas, approximately 6% are silent corticotroph adenomas causing subclinical Cushing syndrome. Transforming risk of subclini-cal Cushing to clinical Cushing syndrome is a matter of debate. Silent corticotroph adenomas have the potential of evolving to Cushing’s disease, but it is not yet possible to predict which pa-tient will have Cushing’s disease in future. The reason for lack of clinical hypercortisolism has been attributed to abnormal ACTH structure, diminished adrenal gland responsiveness or levels of ACTH expression. Underlying genetic mechanisms or predispositions triggered by radiation are thought to be like-ly causes. Silent corticotroph adenomas need long-term fol-low-up until better predictive markers are identified.

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O-08 Pituitary

SILENT CORTICOTROPH PITUITARY ADENOMA PROGRESSING TO CUSHING DISEASE

EKREM ALGUN1, KEREM ERSOY2, INAN ANAFOROGLU2

1 RTE UNIVERSITY, FACULTY OF MEDICINE, DEPARTMENT OF ENDOCRINOLOGY 2 TRABZON KANUNI EDUCATION AND RESEARCH HOSPITAL, DEPARTMENT OF ENDOCRINOLOGY AND METABOLISM

2014


Background: Thyroid nodules are common features with a slow growth pattern even if they are malign. When sudden rapid thyroid nodule growth occurs; hemorrhage into a be-nign or malign nodule, differentiating into anaplastic thy-roid carcinoma, metastatic cancer and thyroid lymphoma should be suspected. Primary thyroid lymphoma is an un-common malignancy and its incidence was found to be 2.06 x 10-6 cases per year. Primary thyroid lymphoma is a very uncommon malignancy, frequently presenting with rapidly enlarging neck mass, hoarseness, dyspnea and dysphagia. Dyspnea is seen in 55-65% of patient with primary thyroid lymphoma, but presenting with progressive respiratory dis-tress due to thyroid lymphoma is rare. Diagnosis is often es-tablished by fine needle aspiration (FNA) biopsy, open biopsy and/or surgical intervention. FNA’ role in the diagnosis of primary thyroid lymphoma is limited. Here we presented a patient with severe progressive dyspnea and hypoxia, who benefited from methylprednisolone atshort-term period and was treated with surgery and chemotherapy afterwards.

Case presentation: A 70-year-old woman applied to the emergency department with fatigue and severe progressive dyspnea. She defined goitre for 6 months, which enlarged rapidly for the last month. Progressive dyspnea and stridor were present for 4 days, which had worsened in the last 12 hours. Physical examination revealed an enlarged thyroid gland. The artery oxygen saturation was 59.2% and the ar-

terial PO2 was detected as 33.9 mmHg, despite an oxygen supplement of 2 lt/m. Ultrasound of the neck showed a mass of 7 cm in largest diameter in the left lobe of the thyroid. Hy-pothyroidism was detected on thyroid function tests. There was tracheal deviation with no signs of pulmonary oedema or pulmonary infiltrates on chest x ray. After admission her dyspnea worsened and oxygen and bronchodilator treat-ment gave no benefit. Intravenous methylprednisolone was administered, and the patient’ symptoms improved after treatment. Meanwhile levothyroxine treatment was started and FNA biopsy from the big mass was performed. The biop-sy specimens were compatible with lymphocytic thyroiditis. Computed tomography of the neck was performed, and a thyroid mass with compression of the trachea was detected. Total thyroidectomy was planned for acute airway obstruc-tion and the need for precise diagnosis. While thyroidecto-my, tracheostomy was also performed because the mass was invading to the sublottic area. The mass was identified as high grade diffuse large B-cell non-Hodgkin’ lymphoma. After thyroidectomy chemotherapy was given and she has been well till now.

Conclusion: Primary thyroid lymphoma may present with severe dyspnea, and patients with rapidly enlarging neck mass and dyspnea should be evaluated for primary thyroid lymphoma. Prompt administration of steroid therapy may be effective for improving respiratory symptoms.

O-09 Thyroid

PROGRESSIVE SEVERE DYSPNEA AND HYPOXIA DUE TO THYROID LYMPHOMA: STEROID ADMINISTRATION

MAY BE LIFE-SAVINGDERYA KOSEOGLU1, DILEK BERKER1, AYDIN ACAR2, SERDAR DENIZ3, SERDAR GULER4

1 ANKARA NUMUNE EDUCATION AND RESEARCH HOSPITAL, DEPARTMENT OF ENDOCRINOLOGY AND METABOLISM 2 ANKARA NUMUNE EDUCATION AND RESEARCH HOSPITAL, DEPARTMENT OF OTORHINOLARYNGOLOGY HEAD AND NECK

SURGERY DEPARTMENT 3 ANKARA NUMUNE EDUCATION AND RESEARCH HOSPITAL, DEPARTMENT OF INTERNAL MEDICINE

4 HITIT UNIVERSITY, FACULTY OF MEDICINE, ANKARA NUMUNE EDUCATION AND RESEARCH HOSPITAL, DEPARTMENT OF ENDOCRINOLOGY AND METABOLISM

2014


Introduction: Fine needle aspiration (FNA); which is a safe, accurate and cost-effective method, plays an important role in the evaluation of patients with a thyroid nodule. FNA re-duces unnecessary surgery for patients with benign nodules and allows timely surgical intervention for patients with ma-lignant nodules. However, the undetermined diagnostic cat-egory, which includes atypia of undetermined significance (AUS) causes a heterogeneous group of diagnostic scenarios and presents a major challenge for both pathologists and clinicians. In the literature, malignancy risk for AUS ranges from 6% to 48% for resected cases and 5% to 27% for all cases. Here, we report a case of papillary microcarcinoma who had recurrent FNA’s having different cytological results in different times such as benign or consistent with AUS.

Clinical Case:A-47-year-old woman who had a diagnosis of Hashimoto thyroiditis was admitted to the hospital for the evaluation of her thyroid function. She was followed annualy with thyroid ultrasonography, she had radiologically benign

thyroid nodules smaller than 1 cm and she had a FNA result consistent with Hashimoto thyroidits. In her seventh year of follow-up; it is shown that one of the nodules in the left lobe of the thyroid gland was enlarging (10x15x19mm), becoming iso-hyperechogenic and having peripheral vascularisation. The first FNA was reported as AUS. FNA was repeated 2 months later and cytology result was consistent with benign follicular epithelioid cells and colloid. There were two oth-er results of AUS in the following months with an interval of 6 months and thyroid scintigraphy showed a normoactive nodule in the left lobe. Because malignancy could not be ruled out, total thyroidectomy was performed. The patholog-ic findings indicated papillary microcarcinoma (0.4 cm) and chronic lymphocytic thyroiditis.

Conclusion: The result of AUS in FNA causes a diagnostic and therapeutic dilemma. Cytologic, clinical and radiologic findings should be evaluated together when deciding to per-form surgery or repeat FNA.

O-10 Thyroid

PAPILLARY MICROCARCINOMA IN A PATIENT WITH MULTIPLE FINE NEEDLE THYROID ASPIRATIONS

RESULTING IN ATYPIA OF UNDETERMINED SIGNIFICANCE

BASAK SAYDAM1, OKAN BULENT YILDIZ2

1 HACETTEPE UNIVERSITY FACULTY OF MEDICINE / DEPARTMENT OF INTERNAL MEDICINE 2 HACETTEPE UNIVERSITY FACULTY OF MEDICINE / DEPARTMENT OF ENDOCRINOLOGY AND METABOLISM

2014


Introduction: Graves €™ disease almost always presents as a diffuse hyperfunctioning goiter involving both lobes of the thyroid gland. There are only a few case reports of unilateral Graves €™ disease not caused by the absence of the con-tralateral thyroid lobe in the literature. Herein, we describe a case of unilateral Graves €™ disease involving the right thyroid lobe of a bilobar gland.

Case Report: A 25-year-old man presented with symptoms and signs of thyrotoxicosis; thyrotropin (TSH) was undetectable, while free thyroxine (fT4) and free triiodothyronine (fT3) were in-creased. Thyroid scintigraphy with Tc99m showed that uptake of the radioisotope was uniformly and unilaterally increased in the right lobe, with no uptake in the left lobe of thyroid gland. A color Doppler Ultrasonography of the thyroid gland was re-vealed a nonhomogeneous, enlarged and hypervascular right lobe which was consistent with unilaterally Graves €™ disease. Nevertheless, the left lobe of thyroid gland appeared homoge-neous and hypovascular on ultrasound imaging. Finally, thyroid autoantibodies were positive. Unilateral Graves €™ disease was diagnosed, and treated with methimazole and propronalol. In the sixth month, the complaints and laboratory (suppressed TSH with T3 toxicosis) of the patient were not improved despite adequate antithyroid therapy. He was referred for radioiodine ablation therapy with 15 mCi of I-131 sodium iodide. After two months of radioiodine ablation therapy, the patient had com-plete resolution of his hyperthyroid symptoms. However, his fT4 and fT3 levels decreased significantly, while TSH levels increased which was consistent with permanent hypothyroid condition. Subsequently, the patient was taken levothyroxine replacement therapy.

Conclusion: We report the unilateral Graves €™ disease in the right lobe of a bilobar thyroid gland as an extremely

rare variant of Graves €™ disease. This hyperthyroid patient has positive thyroid autoantibody, hypervascular and heter-ogeneously enlarged right thyroid lobe on ultrasonography (compared to the hypovascularand homogeny left thyroid lobe), and the uniformly distributed, increased Tc99m uptake in the right thyroid lobe pointing to existence of unilateral Graves €™ disease as the diagnosis. The pathophysiology of unilateral Graves €™ disease has not been clearly elucidat-ed, but clinicians should be aware that Graves €™ disease can present with unilateral involvement of the thyroid gland.

1. Sakata S, Fuwa Y, Goto S, Fukui M, Yuasa H, Takuno H, Sarui H, Matsui I, Ogawa T, Sasano N 1993 Two cases of Graves €™disease with presentation of unilateral diffuse uptake of ra-dioisotopes. J Endocrinol Invest 16:903 - 907.

2. Gratz S, Barth P, Arnold R, Behr TM 2004 Graves €™ disease with unilateral radioisotope uptake. Nuklear-medizin 43: N66 - 68.

3. Chen LC, Green JB. First reported case of unilateral Graves’ disease in the left lobe of a bilobar thyroid gland. Thyroid. 2011 ;21(6):683-6.

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O-11 Thyroid

A RARE CONDITION: UNILATERAL GRAVES’ DISEASESEYID AHMET AY1, FERHAT DENIZ1, EBUZER KALENDER2, MUAMMER URHAN2, ARIF YONEM1

1 DEPARTMENT OF ENDOCRINOLOGY AND METABOLISM, GULHANE MILITARY MEDICAL FACULTY HAYDARPASA TRAINING HOSPITAL

2 DEPARTMENT OF NUCLEAR MEDICINE, GULHANE MILITARY MEDICAL FACULTY HAYDARPASA TRAINING HOSPITAL

2014


Introduction: It is well known that plasma lipid levels in-crease during pregnancy due to increased estrogen, proges-terone, and human placental lactogen. However, expected elevations for triglyceride (TG) and total cholesterol levels during a normal gestational period usually do not exceed 332 mg/dL and 337 mg/dL, respectively. Here, we present a case report of 32 year old woman with gestational diabetes and non-familial severe hypertriglyceridemia.

Case report: A 32 year old woman was admitted to our clin-ic at 27th week of her first pregnancy. She was diagnosed as gestational diabetes after a 75 gr glucose loading test and recommended of dietary restriction of glucose. She had complaint of mild headache and fatigue. Neither abdomi-nal pain, nor nause and vomiting was reported. No clinical signs, suggestive of hyperlipidemia, were detected. She had a sligtly increased level of triglyceride (240 mg/dl) and nor-mal cholesterol levels before pregnancy and she had no rel-evant family history. Serum lipid profile revealed following findings: TG: 15.600 mg/dl; total cholesterol: 459 mg/dl; HDL cholesterol:43 mg/dl. Her amylase and lipase levels were within normal limits. Abdominal ultrasound imaging was unremarkable with absense of cholelithiasis and all antena-tal obstetrical evaluation were normal. Fasting plasma glu-cose was 126 mg/dl, thyroid, liver and renal function tests were normal. Intensive insulin therapy and low molecular weight heparin (LMWH) were administered with medical

nutrition therapy (dietary restriction of carbonhidrate and fat(20 gr per day)). After five days of treatment despite high-er doses of insulin (80 IU/day), blood glucose levels were still above the normal limit and triglyceride level was 12.300 mg/dl. Metformin therapy was started to improve glucose sen-sitivity and lower triglyceride levels at 850 mg doses once a day and titrated to three times a day. After three days with metformin blood glucose was decreased within the target range while TG level was remained higher. A therapeutic plasmapheresis with human albumin infusion, targeting a TG level below 1.000 mg/dl, for preventing acute pancreatitis was performed. After one session of plasmapheresis which was very painfull for the patient TG levels fell from 7.300 mg/dl to 4460 mg/dl. The following day we suggested an addi-tional apheresis session but she did not approved. She did not tolerate omega -3 fatty acids. Her TG levels ranged from 750 mg/dl to 1100 mg/dl until delivery. She delivered at 38th week of pregnancy via elective cesarean section a healthy female baby with normal birth weight. Serum TG level de-creased immediately to 380 mg/dl after delivery.

Discussion: Pregnancy had been reported to induce severe hyperlipoproteinemia and these patients may carry risk of developing hyperlipoproteinemia in their future life. Met-formin therapy may be a good choice in management of hypertriglyceridemia in patients with gestational diabetes mellitus.

O-12 Lipids/Obesity

GESTATIONAL DIABETES MELLITUS ACCOMPANIED WITH SEVERE NON-FAMILIAL

HYPERTRIGLYCERIDEMIA, A CASE REPORTMAZHAR MUSLUM TUNA, FARUK KILINC, ZAFER PEKKOLAY, HIKMET SOYLU,

ALPASLAN KEMAL TUZCUDICLE UNIVERCITY SCHOOL OF MEDICINE ENDOCRINOLOGY AND METABOLISM DEPARTMENT

2014


Testosterone deficiency (TD) is an underdiagnosed and un-dertreated condition among Georgian men. Study of hypo-gonadism in males, revealed that 38.7% of men visiting a primary care practice and aged 45 and older had positive tests for hypogonadism, defined as a total testosterone (TT) level <300 ng/dL.We reporta case of 48-year-old man, who came to the clinicfor scheduled visit. Today, he complains for fatigue, weight gain, and decreased libido, although he denies erectile dysfunction. He states that his wife is con-cerned that he may be depressed, given his lack of interest in sex. He takes glibenklamide/metformin for type 2 diabe-tes mellitus (T2DM), which was diagnosed 4 years ago.Body mass index (BMI) was: 31.1 kg/m2, Bloodpressure (BP): 138/90 mmHg. He had HbA1c: 6.4%, serum TT level at 9:30 AMwas 290 ng/dL, which was confirmed by retest results.

Werecommended weight loss, Letrazolium and TRT. After 3 months, he returns for his scheduled follow-up evaluation. He has lost 7 lb, reducing his BMI slightly to 30.1 kg/m2. His blood pressure is 128/86 mm Hg, and his metabolic profile is improved. His fasting plasma glucose has decreased to 101 mg/dL, and his HbA1C is 6.0%. His complete blood count and liver function tests remain normal. he has experienced no side effects from his TRT. His TT has increased andreached target range (390 ng/dL). His mood has improved, and he reports an increase in the frequency of intimacy with his wife. Despite his controlled symptoms and improved overall health profile, he is still overweight. He should be counseled that if he continues to lose weight, he may further improve his blood pressure and glycemic control as well as sexual performance.

O-13 Male reproductive endocrinology

IDENTIFYING MALE HYPOGONADISM, RECOGNIZING THE ASSOCIATION OF HYPOGONADISM WITH

COMORBIDITIES SUCH AS METABOLIC SYNDROME, DIABETES AND OBESITY

SHOTA JANJGAVA, ELEN GIORGADZE, TAMAR ZEREKIDZE, MAKA KORINTELI, KETEVAN ASATIANI , NATIA CHKHEIDZE

NATIONAL INSTITUTE OF ENDOCRINOLOGY

2014


Introduction: Multiple gynaecological, endocrine, autoim-mune and genetic conditions may lead to secondary infer-tility in women. There are evidences that antiphospholipid syndrome, methylentetrahydrofolate reductase (MTHFR) gene mutations, autoimmune thyroiditis, infertility and in vi-tro fertilization (IVF) failure might be associated.

Case report: A 34 years old Caucasian female was consult-ed by endocrinologist due to secondary infertility. Her pu-bertal development was normal, menstrual cycle was regu-lar with ovulation every month. She has healthy 14 years old daughter born at 7 months of gestation. Secondary infertility was diagnosed in 2007. Since that time woman was inves-tigated and treated in Lithuania, Latvia, Russia, Greece and Turkey. She underwent 3 intrauterine insemination and 5 IVF procedures. In July 2012 after the third IVF procedure she conceived, but spontaneously aborted at 8th gestation week. In 2010 during laparoscopy small patches of endometriosis

and ovarian cyst were destroyed. She underwent cervical conisation due to cancer.

Since age of 4 she suffers from epilepsy and is taking gab-apentin for several years. In 2011 subclinical hypothyroidism and hyperprolactinaemia was diagnosed and treatment with levothyroxin 50 mkg and bromocriptin 3.75 mg was started.

Her father had stroke and mother had two heart attacks and deep veins thrombosis.

On examination secondary sexual features, weight, blood pressure was normal. Data of the last pelvic ultrasound, hysterosalpingogram and laparoscopy were normal. There were no sellar changes on MRI.

Hormone profile: TSH 3.92 mIU/l (0.4 - 4); FT4 16.35 pmol/l (12 - 23); FT3 5 pmol/l (3,5 - 6,5); ATPO 69,9 kIU/l (

O-14 Female reproductive endocrinology

INFERTILITY DUE TO COMBINATION OF ENDOCRINE, AUTOIMMUNE AND GENETIC CAUSES

LINA ZABULIENE1, ALGIRDAS UTKUS2, JURGITA URBONIENE3

1 CLINICS OF RHEUMATOLOGY, TRAUMATOLOGY-ORTHOPEDICS AND RECONSTRUCTIVE SURGERY, FACULTY OF MEDICINE, VILNIUS UNIVERSITY; VILNIUS, LITHUANIA; ANTAKALNIO OUT-PATIENT CLINIC, VILNIUS, LITHUANIA

2 DEPARTMENT OF HUMAN AND MEDICAL GENETICS, FACULTY OF MEDICINE, VILNIUS UNIVERSITY; VILNIUS, LITHUANIA 3INFECTIOUS DISEASES AND TUBERCULOSIS HOSPITAL, VILNIUS UNIVERSITY HOSPITAL SANTARISKIU KLINIKOS, VILNIUS,

LITHUANIA

2014


Introduction: Congenital adrenal hyperplasia is an autoso-mal recessive disease, which is caused by defects in enzymes involved in cortisol biosynthesis. In 95% of cases there is a defect in the 21-hydroxylase enzymes. The gene responsi-ble for the disease, CYP21A2, is located in the short arm of chromosome 6 (6p21.3). There are more than 10 different common mutations that cause the disease. Those mutations typically cause three different types of 21-hydroxylase de-ficiencies: salt wasting, simple virilizing, and non-classical congenital adrenal hyperplasia. Here we present a pregnant patient with non-classical type congenital adrenal hyperpla-sia.

Clinical case: A 31 year-old woman presented to our clinic with the intention of losing weight. She had hypothyroidism and has been taking L-thyroxine replacement therapy. Past medical history revealed two miscarriages, one at eleventh and the other at sixth weeks of gestation. She had a seven year-old son from consanguineous marriage.

She had hirsutismus and irregular menstrual cycles. Ferri-man Gallway score was 12 on physical examination.Laboratory evaluation revealed high 17(OH) progesterone level (12.85ng/ml), therefore, high dose ACTH stimulation test (250 mcg) was performed (Table-1). The increased levels of 17(OH) progester-one in response to ACTH, supported the diagnosis of congeni-

tal adrenal hyperplasia. Further analysis revealed two different mutations in CYP21A2 gene: Q318S and P453S. Moreover, her husband’ genetic analysis showed P453S mutation in CYP21A2 gene. Non-classical congenital adrenal hyperplasia was diag-nosed and the patient was started on dexamethasone therapy at 6th week of the pregnancy.

Gender analysis and genetic testing of the fetus has not con-cluded yet.

Conclusion: The aim of prenatal treatment in congenital adrenal hyperplasia is to suppress the fetal pituitary ACTH secretion by exogenous glucocorticoids for reducing or pre-venting virilization of the external genitalia of the affected female. For successful results, dexamethasone must be started before ninth week of the pregnancy. Besides, if the fetus is male or unaffected female, the treatment should be discontinued, to avoid possible side effects of unnecessary steroid use.

Resim 1


APPROACH TO CONGENITAL ADRENAL HYPERPLASIA IN PREGNANCY

ROVSHAN HASANOV, MIYASE BAYRAKTARDEPARTMENT OF INTERNAL MEDICINE, DIVISION OF ENDOCRINOLOGY AND METABOLISM, HACETTEPE UNIVERSITY SCHOOL OF

MEDICINE

2014


Introduction: Not otherwise specified (NOS) steroid cell tu-mors of the ovary are a group of sex-cord stromal tumors that can produce steroids andaccount for less than 0.1% of allovariantumors. They are usually associated with andro-genic changes in 56-77% andCushingsyndrome in 6-10%. It presents a real diagnostic challenge to both clinicians and pathologists. We hereby report a 65-year-old postmeno-pausal woman presenting with Cushing syndrome and hy-perandrogenemia due to a NOS steroid cell tumor of ovary.

Clinical case: The patient admitted with a complaint of marked and progressive hirsutism that has been present for 2 years. She had a history of diabetes mellitus for 3 months, hypertension and hperlipidemia for 10 years. Physical ex-amination revealed signs of virilization with hirsutism (Fer-riman-Gallwey scale, score 16), acnea and frontal balding. She had a round and plethoric face, central adiposity and supraclavicular fat pads. Laboratory work-up yielded hyper-cortisolemia, high levels of total testosterone, 149.5 ng/dL (reference:14-76 ng/dL), free testosterone, 10.6 pg/ml (ref-erence: 0.29-3.18), normal levels of DHEA-S, 160 µg/dL (ref-erence: 35-430 µg/dL). Confirmation of Cushing syndrome and exclusion of a possible pseudo-Cushing syndrome was accomplished by further work-up. Her plasma adrenocorti-cotropic hormone (ACTH) level was relatively low (9 and <5 pg/mL) compatible with ACTH independent Cushing syn-drome.Adrenal glands were completely normal, an ovarian

tumor was suspected. Neither computed tomography nor magnetic resonance imaging scans showed any abnormal-ity. PET/CT scan was also normal. A 20 mm, hyperechoic, solid lesion in the right ovary was detected by transvaginal ultrasonography. Ovarian neoplasm could not be excluded during sonographic follow-up of the patient. The decision for operation was made based on the persistence of small sized ovarian mass and hysterectomy and bilateral salpin-goophorectomy were performed. No intra or postoperative complications were seen. Macroscopically, the size of mass was found to be 1.9x1.7 cm. Upon microscopic examination, the ovary contained a well delineated tumor composed of clear and compact cells with scarce Reincke crystalloids. The tumor cells exhibited nuclear reactivity for steroidogen-ic factor (SF-1) and strong cytoplasmic positivity for inhibin. They were negative for ACTH, corticotropin releasing hor-mone (CRH) and Wilm’s tumor protein-1 (WT1). These his-topathological features were consistent with a not otherwise specified (NOS) steroid cell tumor of the ovary.

Conclusion: This case presents a rare co-existence of hy-perandrogenemia and Cushing syndrome in a patient pre-senting with virilization. Postmenopausal virilization may arise from adrenal or ovarian androgen-secreting tumors. After exclusion of adrenal masses, oophorectomy may be necessary to establish the definitive diagnosis for patients with hirsutism and elevated testosterone levels.


TESTOSTERONE AND CORTISOL SECRETING OVARIAN STEROID CELL TUMOR IN A POSTMENOPAUSAL

WOMAN: AN UNUSUAL CAUSE OF HIRSUTISMINAN ANAFOROGLU1, ULKU METE URAL2, IBRAHIM SEHITOGLU3, OZGUR METE4, SLYVIA ASA4,

EKREM ALGUN5

1 TRABZON KANUNI EDUCATION AND RESEARCH HOSPITAL, DEPARTMENT OF ENDOCRINOLOGY AND METABOLISM, TRABZON 2 RTE UNIVERSITY, FACULTY OF MEDICINE, DEPARTMENT OF OBSTETRICS AND GYNECOLOGY,RIZE

3 RTE UNIVERSITY, FACULTY OF MEDICINE, DAPARTMENT OF PATHOLOGY, RIZE 4 UNIVERSITY OF TORONTO, DEPARTMENT OF LABORATORY MEDICINE AND PATHOBIOLOGY, TORONTO,

5 RTE UNIVERSITY, FACULTY OF MEDICINE, DEPARTMENT OF ENDOCRINOLOGY AND METABOLISM, RIZE

2014


Introduction: For vast majority of Georgian people the cost of healthcare is mostly paid by private insurance or services out-of-pocket. The costs associated with this pattern of payment vastly reduce the access to healthcare for people with chronic diseases.

Clinical Case: This is the case study of one of the ordinary Georgian patient.Mr. B is 64 years old man, who was diagnosed type 2 diabetes 15 years ago. He has medical history of myocardial infarction (MI) at the age 58 and arterial hypertension II degree from the age 51. His last visit to endocrinologist was 3 years ago. Since then he is taking metformin 2000 mg/day, glimepiride 2 mg/day and prescription from cardiologist: atorvastatin, ACE inhibitors and cardioaspirin.He came to the clinic with several complaints, such as: fatigue and excessive sweeting during nighttime. As the patient has low income we could not perform all necessary blood tests, but choose the ones with greater informational value. The lab answers revealed that patient’s HbA1c was 7.3%; he had elevated transaminase levels (≈1.5 fold from ULN) and microalbumin and glucose in the urinalysis. According to HbA1c levels glycaemia goal was achieved for this patient, but we wondered why he had excessive sweeting and glucose in urine if everything was normal?! We asked for the glucose profile, which showed

glycemic variability during the whole day and night. Glyce-mic variability explained the false normal HbA1c level. The next step was to choose the appropriate treatment that will individually fit our patient. What does the modern interna-tional guidelines recommend for this particular situation? Unfortunately nothing! This case is an example that every diabetes is different from each other and individual treat-ment is important in achieving goal for each patient. We pay attention on long duration of the disease, glucose variability, elevated risk factors of cardio-vascular disease, impaired liver function tests,mild diabetic nephropathy and low in-come. According to all this factors we choose low doses of basal insulin (that is paid by government) and SU with lower risks). After 2 weeks patient showed glucose profile without any significant improvement. After 1 month we prescribed DPP-4 inhibitors instead SU and the results were much more better after 2 weeks. Importantly patient did not feel any discomfort with this regimen and was happy with chosen treatment in spite of high price of this new antihyperglyce-mic agents.

Conclusion: Guidelines not always show the right treatment direction and individual based approach is very important for each patient with type 2 diabetes.

O-17 Diabetes

DO GUIDELINES ALWAYS SHOW THE DIRECTION?TAMAR ZEREKIDZE, ELEN GIORGADZE, SHOTA JANJGAVA


2014


Alstrom syndrome is an autosomal recessive disease devel-oping due to mutations in ALMS 1 gene. Alstrom syndrome is a multiorgan disease characterized by retinal degeneration, childhood obesity, insÃ¼lin resistance, type 2 DM, hypertri-glyceridemia, short stature, dilated cardiomyopathy, hepatic and renal involvement. In this case report, a girl diagnosed with type 2 DM at the age 7 and with Alstrom syndrome at the age 25 is presented.

A 25 year old female patient referred to our clinic due to high blood glucose. There was consanguine marriage between parents and no history of diabetes was present in her five brothers. Patient had nystagmus when she was 3 months old ,her vision decreased constantly and she was totally blind when she was 15 years old. DM was diagnosed when she was 7 years old and insÃ¼lin treatment was initiated. She had oligomenorrhea since menarche. In physical examination her height 140 cm, weight 57 kg and waist circumference 91 cm measured and body mass index was calculated as 29 kg/m2. Fundus examination revealed retinitis pigmentosa. Ferriman Galwey score was 8. Acanthosis nigricans, poly-dactly and syndactly were not seen. No pathologic finding was observed in systemic examination. In laboratuary analy-sis blood glucose was 184 mg/dl, HbA1c: % 9, C-peptid level 7,09 ng/ml (0,9-7,1), creatinin 0,9 mg/dl (0,6-1,3) ,24 hour urine protein excretion was 1400 mg/day. Serum triglyceride level was 575 mg/dl ,HDL-K 29 mg/dl, LDL-K 104 mg/dl, AST

96 U/L (5-34), ALT 166 U/L (0-55) and GGT 176 U/L (9-36). Albumin, bilrubin and INR level were normal, viral hepatitis markers, ANA, Anti LKM and ASMA were negative. Abdomi-na USG revealed hepatomegaly and grade 3 steatosis. Total testosteron and DHEA-S was 114 ng/dl (10-57) and 555 ng/dl (95-511) respectively. TSH and IGF-1 values were normal. Audimetric analysis showed mild type bilateral sensorineu-ral hearing loss. Echocardiography was normal. At admis-sion she was using insÃ¼lin 160 U/day. Low fat and low calo-ry diet was initiated. Ramipril 2,5 mg and metformin 500 mg/day was started. Metformin dose was gradually increased to 2000 mg/day. At follow up visits blood glucose measurment was over 200 mg/dl and sitagliptin 100 mg/day was added to treatment. By this treatment blood glucose levels was im-proved and daily insÃ¼lin need was decreased.

The diagnosis of Alstrom syndrome could be difficult as the disease is rare, and some symptoms arise in infancy while the others appear with the growth of the child and in adult-hood. Marshall et al. determined major criteria according to age groups in order to help diagnosis of Alstrom syndrome (1). In the patient presented in this case report 1 major (nys-tagmus,blindness) and 5 minor criteria (obesity,type 2 DM, hearing loss,hepatic disorder,short stature and hyperandro-genism) were present suggesting the diagnosis of Alstrom syndrome. We didn €™t perform ALSM 1 mutation analysis. In differential diagnosis due to lack of mental retardation.

O-18 Diabetes

A GIRL WITH SEVERE INSULIN RESISTANCE AND TYPE 2 DM DIAGNOSED AS ALSTROM SYNDROME

ALPER CAGRI KARCI, ZEYNEP CETIN, MUSTAFA UNAL, SERDAR GULERANKARA NUMUNE EDUCATIONAL AND RESEARCH HOSPITAL ENDOCRINOLOGY AND METABOLISM DEPARTMENT

2014


Introduction: Mayer–Rokitansky–Küster–Hauser (MRKH) syn-dromecombines congenital absence of the vagina with abnor-mal development of the uterus, ranging from a rudimentary bicornuate uterus that may not open into the vaginal canal to a virtually normal uterus. The incidence of the syndrome is one in 4,500 female newborns. MRKH syndrome patients have a correctly timed pubarche and thelarche with a normal female karyotype (46, XX). MRKH occurs as a purely genital malforma-tion (type 1), but may also occur with associated malformations (type 2 and MURCS association; Müllerian,, Renal, Cervicotho-racic Somite abnormalities).A failure of fusion of Müllerian duct derivatives during gestational weeks 4–12 results in mal-formation of the vagina and uterus.

Case Presentation: We describe a case of 19 years old Saudi woman with Type 1 diabetes for 6 years and primary hypo-thyroidism, having presented to the Endocrinology Clinic in July 2013 with primary amenorrhoea, associated with cycli-cal abdominal pain for 4 years. Her secondary sexual char-acteristics had appeared at the age of 14 years. There was no history of galactorrhea, hirsutism, visual disturbance or anosmia. The patient was born pre-term at 8 months of ges-tation and had mild cerebral palsy. The family history was unremarkable. Her younger sister, aged 17 years had nor-mal menstrual cycles. On examination the patient’s BMI was 18.41 kg/m² and she was hemodynamically stable. There were no dysmorphic features. The neurologic examination revealed a limping gait and atrophy of right hand and right lower-limb muscles. No syndactyly or scoliosis was ob-served. The rest of the systemic examination was unyielding.

The patient had normal secondary sexual characteristics (Tanner 4). She was seen by the gynecologist who reported a blind vagina. Subsequently, a progesterone challenge failed

to induce withdrawal bleeding.

Investigations revealed a normal CBC along with normal re-nal, hepatic, thyroid & bone profiles. Random blood glucose was 163 mg/dl and HbA1c was 9.7 %. Serum Estradiol, FSH, LH, Testosterone and DHEA levels were within normal lim-its. Urinalysis was normal. Karyotyping showed normal 46 XX female karyotype.

Radiology studies showed a normal chest film & no abnor-mality was detected in the skeletal survey. Echocardiogram was unremarkable. MRI of abdomen & pelvis (Fig 1) revealed an atretic uterine cervix without visualization of the vagina. Otherwise, the uterine anatomy, ovaries & kidneys were re-ported as normal.

The patient was on Glargine & Aspart insulin and 50µg of Thyroxine daily, besides 3 monthly depot Medroxy-proges-terone acetate(150 mg) injection for the control of her cycli-cal abdominal pain.

Our patient was finally diagnosed to have the rarest form of Mayer-Rokitansky-Kuster-Hauser syndrome (V5b, C0, U0, A0, M0) ,as she had isolated vaginal atresia with normal uterus. She was referred to a higher center for possible vag-inoplasty .

Conclusion: In MRKH syndrome there is an uncommon oc-currence in malformations of the distal genital system ,while the spectrum of abnormalities occur more commonly in the proximal part, with adnexal and extragenital malformations. Our patient is unique in the sense, that she had the rarest type of MRKH syndrome with vaginal atresia and normally functioning uterus along with Type 1 diabetes and hypothy-roidism.

O-19 Female Reproductive Endocrinology

A YOUNG WOMAN WITH DIABETES & PRIMARY AMENORRHOEA

MUHAMMAD SHOAIB ZAIDI, ASIM HASSAN, EBTELAH ALMOQBELUNIVERSITY DIABETES CENTER,KING ABDULAZIZ UNIVERSITY HOSPITAL.

2014


Resim 1

2014


Introduction: Osteoporosis is the most common metabolic bone disorder characterized by a loss of bone osteoid that reduces bone integrity, resulting in an increased risk of frac-tures. Osteoporosis is usually asymptomatic until fractures occur. The World Health Organization (WHO) has established diagnostic criteria for osteoporosis based on bone densitom-etry. Multiple risk factors including many chronic diseases and medications have been shown to be independently asso-ciated with low bone mass causing secondary osteoporosis.

Case report: A 43-year-old man was referred to our clinic after plain radiography for a solitary pulmonary nodule re-vealed compression fractures of T7 and L1 which were as-ymptomatic. The patient had no history of trauma, clinical fractures or chronic disease. He denied taking any medica-tions. He had pruritus that relieved by antihistaminics for 2 months. He had smoking history of 30 pack-years Physical examination revealed dermographism.

A dual-energy x-ray absorptiometry (DEXA) showed a T-score of −4.7 in the spine and of −1.5 in the femoral neck areas, respectively. Because of the unusually low bone min-eral density, further work-up was performed revealing nor-mal results for blood cell count, calcium, phosphorus, and 25-hydroxyvitamin D levels, and kidney and liver functions, thyroid function tests, testosterone, 24 hour urine free cor-tisol and 1 mg overnight dexamethasone suppression test.

Serum tryptase was 23.1 μg/L (reference range, <11.4). A bone marrow biopsy was performed because of suspicion of the presence of mastocytosis. Bone marrow biopsy con-firmed the diagnosis of mastocytosis.

Conclusion: Mastocytosis is a rare disease that required perseverance in the diagnostic evaluation of this patient. Once osteoporosis is identified with a suspicion of secondary cause, a through history, physical examination and a com-prehensive diagnostic work-up must be performed to deter-mine the underlying disorder.

O-20 Bone/Calcium

A 43-YEAR OLD MAN WITH SEVERE OSTEOPOROSISSELIN KUCUKYURT KAYA1, BULENT OKAN YILDIZ2

1 INTERNALMEDICINEDEPARTMENT, HACETTEPE UNIVERSITY SCHOOL OF MEDICINE 2 DIVISION OF ENDOCRINOLOGY AND METABOLISM DEPARTMENT OF INTERNAL MEDICINE HACETTEPE UNIVERSITY SCHOOL OF

MEDICINE

2014


Secondary adrenal involvement during the course of non-Hodgkin’ lymphoma is rarely seen. Primary adrenal lymphoma is even rarer, and bilateral adrenal involvement is seen in about 70% of the patients. Unilateral primary ad-renal lymphoma is a very rare entity and although chemo-therapy is the treatment of choice, patients presenting with unilateral disease can undergo unnecessary surgery with a presumptive diagnosis of adrenocortical cancer.

We herein present a 69-year-old man who was referred to our endocrinology clinic for evaluation of 8-cm right adrenal mass. The patient began to experience fatigue, weight loss, right back pain, and cough 6 months before his admission and computerized tomography of the thorax demonstrated a 3.5 cm non-adenomatous right adrenal mass lesion. En-docrinological evaluation revealed that the tumor was non-functioning and he was suggested unilateral adrenalectomy, but didn €™t accept to be operated. His complaints wors-ened for the last two months and a repeat computerized to-mography of the thorax demonstrated an 8 cm right adrenal mass lesion. Abdominal MRI revealed non-adenomatous right adrenal tumor and a positron-emission-tomography scan was performed only showing significant uptake in the right adrenal gland.

On admission his physical examination revealed hepato-splenomegaly. Laboratory examination showed that the pa-tient was anemic and had severe hypercalcemia, elevated lactate dehydrogenase, creatinine, uric acid, C-reactive pro-tein levels, and high erythrocytesedimentation rate. Needle biopsy was performed with a presumptive diagnosis of pri-mary adrenal lymphoma and showed CD5, CD20 and CD23 positive diffuse large B-cell lymphoma. The proliferation index Ki-67 was 95%. He received 6 course of R-CHOP che-motherapy which resulted in reduction of the tumor size, but remission could not be achieved. He is currently waiting for radiotherapy.

Radiological findings suggesting malignant adrenal tumor, patient history, constitutional symptoms, and laboratory findings like hypercalcemia, high sedimentation rate and elevated lactate dehydrogenase levels should alarm physi-cians that the tumor may not be an adrenocortical cancer and although unilateral primary adrenal lymphoma is very rare, further investigations should be made before referring the patient for adrenalectomy.

O-21 Adrenal

PRIMARY ADRENAL LYMPHOMA AS A RARE CAUSE OF UNILATERAL ADRENAL MASS

NURDAN GUL1, AYSE KUBAT UZUM1, FERIHAN ARAL1, SEVGI KALAYOGLU-BESISIK2, FARUK ALAGOL1, REFIK TANAKOL1

1 ISTANBUL UNIVERSITY, ISTANBUL FACULTY OF MEDICINE, DEPARTMENT OF INTERNAL MEDICINE, DIVISION OF ENDOCRINOLOGY AND METABOLISM

2 ISTANBUL UNIVERSITY, ISTANBUL FACULTY OF MEDICINE, DEPARTMENT OF INTERNAL MEDICINE, DIVISION OF HEMATOLOGY

2014


Introduction: Primary hyperaldosteronism (PHA) could be the most common identitable and potentially curable form of secondary HTA. The hyperfunctioning aldosterone-secreting adrenocortical tissue is either an adenoma, hyperplasia, or carcinoma. We report a adrenocortical neoplasia case, which presented with HTA.

Case Presentation: In April 2014, a 31-year old man, ad-mitted to Emergency Department (ED)with loss of vision in the right eye. He didn’t have any past medical history and he wasn’t taking any medication prior to his initial presentation. He had no significant family history.

Acute occlusion of right retinal vein,hypertensive retinopathy and high blood pressure (BP: 170/100 mmHg ) were detect-ed. Other phyical examination findings were unremarkable. His initial laboratuary results were Na: 140 mg/dl K:3.54 mg/dl Cr: 0,8 mg/dlGFR>100 ml/min/1,73m2.

Mean of ambulatory blood pressure monitoring was found 155/96 mmHg. His renal CT anjiography was normal. Adre-nal CT revealed a 1,6 cm x 1,2cm, 0 HU dansity nodular le-sion at left adrenal corpus with radiological characteristics of benign adenoma,while right adrenal gland was normal. His cardiovascular evaluation was normal (ECG, Echocardi-ography). serumaldosterone level was elevated (506.55 pg/ml, normal:35-300 pg/mL ), while renin was low (0.87 pg/ml normal: 2.71-16.51 pg/mL). Other hormonal evaluation including TSH, 24-hour urinary free cortisol excretion, low dose dexamethasone supression test, DHEAS, urine and

serum catecholamine levels were normal.

We decided to by-pass adrenal venous sampling (AVS) be-cause of young patient age and well detected adenoma in CT scan. Surgical resection of the adrenal mass was performed at August 2014. Pathology revealed a 2,1x1,1x0,9 cm adre-nocortical neoplasia with invasion to periadrenal fat tissue and adrenal gland capsule. Ki67 index was %2, there was no large vessel involvement or atypical mitosis . After surgery patient’s blood pressure became normal.

Discussion: A recent study suggested a clinical prediction score to diagnose unilateral PHA. This prediction score contains; 3 points for typical Conn’s adenoma on imag-ing , 2 points for hypokalemia <3,5 mmol/lt , 2 points if eGFR (MDRD) >100 ml/min/1.73m2, 1 point if 80-100 ml/min/1.73m2 and 0 point if 2 . According to this study, a score of at least 5, predicts a lateralized AVS with a specificity of %100 (95% CI,91-100) and a sensivity of 53% (95% CI, 38-68). Another study supports the prediction score in only young population (age <40).

By using this score, AVS could be omitted in patients young-er than age 40 if a unilateral adrenal adenoma on CT scan is detected and there is coexisting hypokalemia (K<3,5 mg/dl) or GFR>100 ml/min/1,73m2.

In our case, we used this prediction score and surgical pro-cedure was made without AVS. And the pathology revealed adrenocortical neoplasia .

O-22 Adrenal

A RARE CAUSE OF HYPERTENSION, ALDOSTERONE SECRETING ADRENOCORTICAL NEOPLASIA; A CASE

REPORTHAKAN BABAOGLU1, ALPER GURLEK2, MERVE TOMBUL1

1 INTERNAL MEDICINE DEPARTMENT, HACETTEPE UNIVERSITY SCHOOL OF MEDICINE 2 DIVISION OF ENDOCRINOLOGY AND METABOLISM DEPARTMENT OF INTERNAL MEDICINE HACETTEPE UNIVERSITY SCHOOL OF

MEDICINE

2014


Background : Primary bilateral adrenal hyperplasias are rare reasons of primary adrenal Cushing’s syndrome (CS).Here we present a case with isolated primary pigmented nodular adrenocortical disease (PPNAD).

Clinical case : 26 year-old woman was admitted to our out-patient clinic at 2005, for having irregular menstrual periods, severe headache,increased facial hair and difficulty in walking.

When she was examined for muscle weekness a year ago, twelfth thoracal vertebrae compression fracture was detect-ed by magnetic resonance imaging (MRI). Electromyogram findings were normal. Bone mineral densitometry (BMD) re-vealed a total lomber vertebrae T score of -4.28 and Z score of -3.86 whereas femoral neck T and Z scores were -4.48 and -4.45, respectively. She was given NSAIDs and calcitonin ; which did not improve her symptoms.

On physical examination, she had moon face, hirsutism, cen-tral obesity with a BMI of 24.75 kg/m2, thin extremities, and muscle weekness that she could only walk with assistance. Violaceous striae were observed over upper thighs and up-per arms. Her blood pressure (BP) was 150/100 mmHg.

Serum glucose, kidney and liver function tests, and serum electrolytes were normal. 24 hour urinary free cortisol (UFC) was 135.40 mcg (21-85) and serum cortisol level did not sup-press with overnight low dose dexamethasone supression test (20.5 mcg/dl). Basal morning plasma ACTH and cortisol levels were <5 pg/ml and 17.5 mcg/dl, respectively. Midnight plasma cortisol was 19.5 mcg/dl. After overnight high dose dexamethasone, serum cortisol level was 22.4 mcg/dl.

Computed tomography (CT) detected normal adrenal glands. Adrenal venous sampling localised left adrenal gland as the source of hypercortisolism.

The patient underwent left adnelectomy. Histopathologic as-sessment confirmed micronodular hyperplasia of the adre-nal gland, with internodular cortical atrophy and brown-pig-mented cells (Picture 1).

Patient needed steroid replacement therapy for the follow-ing year. She also gave birth to her first child in 2009. She had no symptoms or signs of hypercortisolism ; until 2014.

In 2014, she was admitted for recurrency of symptoms . BP was 120/80 mmHg, mild increase in abdominal fat and mild hirsutism was detected but no striae were observed.

24 hour UFC was 30.83 mcg and 33.29 mcg in two subsequent measurements. Serum cortisol level was not supressed with overnight low dose dexamethasone (6.68 mcg/dl). Morning serum ACTH and cortisol were 9.47 mcg/dl and 18.9 pg/ml, respectively. After overnight high dose dexamethasone, se-rum cortisol level was 11.95 mcg/dl. Right adrenal gland was normal in CT. She was osteopenic.

No other signs of Carney complex were found. Relapsed CS is diagnosed and right adrenelectomy is planned.

Conclusion : We report a case of isolated PPNAD, which could not be detected by adrenal imaging, and presented as unilateral adrenal cortisol hypersecretion and cured with unilateral adrenelectomy; but relapsed after 7 years.

O-23 Adrenal

A RARE CAUSE OF ADRENOCORTICOTROPIC HORMONE INDEPENDENT CUSHING’S SYNDROME

SEDA OGUZ, MIYASE BAYRAKTAR, BULENT OKAN YILDIZDEPARTMENT OF INTERNAL MEDICINE, DIVISION OF ENDOCRINOLOGY AND METABOLISM, HACETTEPE UNIVERSITY SCHOOL OF

MEDICINE, ANKARA, TURKEY

2014


Abstract: Malignant paraganglioma is a rare type of cate-cholamine-secreting tumors.Here,we report a case of para-ganglioma with lymph node metastases.The tumor was in the abdomen,large(>5 cm)and after surgery we detect-ed lymph node metastases.She is going to be treated with 131I-MIBG. We emphasize that a careful follow-up after sur-gery of a paraganglioma is important for an adequate diag-nostic and therapeutic approach.

Introduction: Paragangliomas are tumors arising from catecholamine-producing chromaffin cells of extraadrenal tissues.They are 15-20% of all catecholamine-producing tu-mors. They are rare causes of hypertension.These tumors have cardiovasculer risks,enlarge with time and may cause mass-effect symptoms,some has malignant potential.Ma-lignancy prevalence varies between 10 and 17%.We present below a paraganglioma case with lymph node metastases.

Case Report: 57 year old female patient admitted to a hos-pital with abdominal pain,weight loss,flushing and uncon-trolled hypertension.Physical exam was normal,in the labo-ratory tests,she had anemia and primary hypothyroidism.A mass near pancreas was detected in USG.Abdominal CT was performed and it revealed a mass in the inferior pan-creatic tail;measuring 75x55x50 mm.The mass was adja-cent with abdominal tissues and its borders were deleted (invasion?).In PET-CT,the mass had high pathological activ-ity(SUVmax:19.8) and its border with adjacent tissues could not been seen(invasion?).Her chromogranin A level was 655 ng/ml(the upper limit:5cm)or have an extraadrenal loca-tion have a high risk for malignancy.The common sites of metastases are bones,lungs,liver,lymph nodes.Total tumor excision is palliative.Radiotherapy may be useful for bone metastases. 131I-MIBG therapy can be used if tumor has MIBG uptake.Chemotherapy may be used if there is no ra-dionuclide uptake.

O-24 Adrenal

A RARE CASE: MALIGNANT PARAGANGLIOMAZEYNEP CETIN, MUSTAFA UNAL, DILEK BERKER, SERDAR GULER

ANKARA NUMUNE RESEARCH AND TRAINING HOSPITAL,DEPARTMENT OF ENDOCRINOLOGY AND METABOLISM

2014


Introduction: Underdiagnosisof vertebral fractures is a worldwide problem.

Clinical Case:PostmenopausalGeorgian female, 65 years old. Age at menopause 46 years.Asymptomatic,never used HT,used calcium and vitamin D supplementsoccasionally.She is a non-smoker and consumesless than 3 units of al-cohol per week.Mother became slightly stooped at old age. No other risk factors andchronic conditionshave been iden-tified.Her activity levels are low to moderate.Medical histo-ry:She fractured forearm at age of 57 following the fall walk-ing down the street .DXA findings: L1-L4T-score -(-1.6),Hip T-score- (-1.9).Laboratory tests are all normal. As she had low bone mass and fragility fracture we consideredheroste-oporotic and decided to treat her as such. In order to reveal

the prevalence of vertebral fractures we decidedto perform VFA. We chose VFAfor identifyingvertebralfractures as they are not often recognised on routine X-ray.VFA showed frac-ture.

It is known that preexisting vertebral fractures predict fu-ture fractures independent of BMD. Presence of vertebral fractures are a strong indication of aggressive therapyof os-teoporosis. Treatment plan included discussing of lifestyle modification and dietary habits.She was prescribedbisphos-phonate, calcium1000mg and vitamin D 800IU per day.

Conclusion: Diagnosing osteoporosisby BMD alone misses many patients with prevalent vertebral fractures.

O-25 Bone/Calcium

CASE STUDY OF POSTMENOPAUSAL WOMANMARINA TSAGARELI, ELEN GIORGADZE, TEA SULIKASHVILI


2014


Parathyroid carcinoma is one of the rarest known malig-nancies. It accounts for approximately 1% of patients with primary hyperparathyroidism. The majority (90%) of para-thyroid cancer tumors are hormonally functional and hyper-secrete parathyroid hormone (PTH).We are presenting here a rare case of primary hyperparathyroidism with parathyroid carcinoma after third time repeated surgery achieved nor-mal Ca and PTH levels.Clinical Case: A 42 year-old wom-an presented with a history of hypercalcinemia without any symptoms during pregnancy and she underwent a parathy-roidectomy operation. Result of pathology report came back as a suspicious parathyorid adenoma or carcinoma without muscle or neurol invasion. After 1,5 years later her calci-um level was found 12 mg/ dl and PTH 249,5 pg/ml and she submitted to further investigation for new treatment options to our hospital. Pathologly slides from first operation were consultated with our hospital pathology department and result was parathyroid carsinoma. Technetium-99m ses-tamibi scan was negative but cervical ultrasound showed that the parathyroid mass on the inferior of right thyroid gland, so the patient underwent a second surgery with right lobe thyroidectomia and radical neck dissection. Post op-erative PTH level was 43 pg/ml but in a two weeks times PTH was detected 123 pg/ml, because of that calcimimetic drug (namely cinacalcet 30 mg per day) is given to the pa-tient. During therapy PTH levels continued high (168 pg/

ml), therefore Technetium-99m sestamibi scan along with cervical ultrasound was performed again and this time left inferior thyroid gland localization was found positive. Fur-thermore, PET-CT was perfomed and result came without any metastasis. For all these, the patient underwent third surgery with left lobe thyroidectomia and radical neck dis-section. After surgery PTH levels dropped to 10.19 pg/ml and cinacalcet was stopped. After that PTH and Ca levels of her remained stable.Conclusion:Parathyroid carcinoma is an extremely rare endocrine malignancy, which typically pres-ents with the clinical sequelae of extremely elevated serum calcium levels. The primary modality of treatment is surgi-cal en-block resection of the tumor and any involved sur-rounding structures. Persistent or recurrent disease occurs in more than 50% of patients with parathyroid carcinoma, reoperation is rarely curative and eventual relapse is likely.In inoperable cases, few palliative treatment options exist, although treatment with calcimimetics can effectively con-trol hypercalcemia in some patients.Parathyroid carcinoma is typically associated with a prolonged clinical course, with multiple recurrences. We are discussing here a rare case of primary hyperparathyroidism with parathyroid carcinoma afterthird time repaeted surgery achieved normal Ca and PTH levels. Due to limited value of adjuvant therapies, the recommended treatment for recurrences and metastases is also surgical resection, when technically feasible.

O-26 Bone/Calcium

A RARE CAUSE OF PRIMARY HYPERPARATHYROIDISM: PARATHYROID

CARCINOMADEMET SAHINER LAZZARI1, RAMAZAN SARI2, CUMHUR ARICI3, ELIF INANC GURER4,

HASAN ALTUNBAS2, MUSTAFA KEMAL BALCI2

1 DEPARTMENT OF INTERNAL MEDICINE, AKDENIZ UNIVERSITY FACULTY OF MEDICINE 2 DIVISION OF ENDOCRINOLOGY AND METABOLISM,AKDENIZ UNIVERSITY FACULTY OF MEDICINE

3 DEPARTMENT OF GENERAL SURGERY, AKDENIZ UNIVERSITY FACULTY OF MEDICINE 4 DEPARMENT OF PATHOLOGY, AKDENIZ UNIVERSITY FACULTY OF MEDICINE

2014


Introduction: Osteomalacia is a metabolic bone disorder, characterized by decreased mineralization of newly formed osteoid at sites of bone turnover (1).

Several different disorders cause osteomalacia. Severe vi-tamin D deficiency secondary to inadequate dietary intake, lack of sun exposure, gastric bypass or malabsorption, isthe most common cause of osteomalacia in adults (2,3).

Bone pain is a characteristic symptom of osteomalacia. The biochemical features are increased serum alkaline phos-phatase and PTH values, and low calcium, phosphorus, and 25(OH)D values in most cases (4).

Clinical Case: A 29-year-old female with no medical history was admitted to the emergency department with the com-plaints of peri-oral numbness, paresthesias of the hands and feet, muscle cramps and carpopedal spasm. Also she had progressive myalgia for several months. On physical examination diffuse tenderness on muscles and carpopedal spasm were noted. Laboratory examination established hypocalcemia (5,98 mg/dl, ionized Ca:0,8 mMol/L), hypo-phosphatemia (P:2,43 mg/dl), hypoalbuminemia (3,06 g/dl), hypomagnesemia (0,79 mg/dl), elevated PTH levels (282,6 pg/ml), elevated ALP levels (181 U/L), low 25(OH)D levels (5 µg/L), and mild transaminase elevation ( ALT:49 U/L, AST:56 U/L). Intravenous calcium gluconate and magnesium were given and her symptoms recovered. She was prescribed cal-

citriol and calcium carbonate and referred to internal medi-cine outpatient clinic.

Lack of sun exposure due to being veiled was considered in the etiology but mild transaminase elevation, hypoalbu-minemia and previous history of periodic diarrhea led us to investigate celiac disease. Tissue transglutaminase IgA and anti endomysial antibody were both positive. Upper gastro-intestinal endoscopy for duodenal biopsy and bone mineral density have been scheduled.

Conclusion: Vitamin D deficiency due to lack of sun expo-sure is one of the major cause of osteomalacia but another underlying cause like celiac disease has to be considered in differential diagnosis.

References1. S. Christodoulou, T. Goula, A. Ververidis, and G. Dro-

sos. Vitamin D and Bone Disease.Biomed Res Int. 2013; 2013: 396541.

2. Evaluation, Treatment, and Prevention of Vitamin D De-ficiency, Endocrine Society Practice Guidelines JCEM, 2011.

3. Holick MF 2007 Vitamin D Deficiency. N Engl J Med 357:266 - 281

4. Tom D. Thacher, MD and Bart L. Clarke, MD, Vitamin D Insufficiency,Mayo Clin Proc. Jan 2011; 86(1): 50 - 60.

O-27 Bone/Calcium

A VEILED FEMALE PATIENT WITH OSTEOMALACIA DIAGNOSED AS CELIAC DISEASE

AYSE SENDUR1, ALPER GURLEK2

1 DEPARTMENT OF INTERNAL MEDICINE, HACETTEPE UNIVERSITY SCHOOL OF MEDICINE, ANKARA, TURKEY 2 DEPARTMENT OF INTERNAL MEDICINE, DIVISION OF ENDOCRINOLOGY AND METABOLISM, HACETTEPE UNIVERSITY SCHOOL OF

MEDICINE, ANKARA, TURKEY

2014


Introduction: Primary hyperparathyroidism is largely caused by a single parathyroid adenoma for which surgery is the treat-ment of choice, particularly if there is an end organ damage or if the serum calcium exceeds 2.8 mmol/L. However, surgery is not an appropriate option for all patients and medical alterna-tives are required. Cinacalcet is a calcimimetic used to treat hy-percalcaemia in patients unsuitable for surgery. We evaluated out practice of Cinacalcet in this cohort.

Methods: A retrospective review of all the patients diagnosed with primary hyperparathyroidism treated with Cinacal-cet between (2010 – 2013) was conducted. Serum calcium (Ca) and parathyroid hormone (PTH) levels were assessed prior to commencing Cinacalcet and periodically every 2-3 months up to a year after commencing. Cinacalcet was given as 30mg tablets, either once daily or twice daily.

Results: A total of 16 patients were reviewed (mean age 77 years, range 60-94). Of the 16 patients, 14 were female (88%) and 2 were male (12%). Patients were on either a daily (9) or a twice-daily dose (7). Indications for giving Cinacalcet included the following: patient refused surgery (56%), patient was unfit for surgery (38%) or an adenoma was not identified (6%). Pri-or to initiating Cinacalcet the mean PTH was 13.96 u/L (range 6.2-43.1) and the mean Ca was 2.88 u/L (range 2.68-3.18). The calcium levels reduced for all patients on Cinacalcet after a year (mean Ca 2.51, range 2.24-2.98). The 12.5% of the group who developed side-effects were using Cinacalcet twice daily regime. There was no significant difference in the amount by which the Ca level reduced between the once daily dose (mean = 2.52) and the twice-daily dose (mean= 2.51), (p < 0.01).

Conclusions: Cinacalcet is very effective at reducing serum calcium levels in patients with primary hyperparathyroid-ism. Normo-calcaemia was achieved in all patients using

Cinacalcet. A once daily dosing regime was found to be as effective as a twice-daily regime. We can therefore conclude that Cinacalcet is an effective alternative to surgery at a low-er dose than recommended.

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O-28 Bone/Calcium

THE USE OF CINACALCET IN PRIMARY HYPERPARATHYROIDISM: A PRACTICE REVIEW

WAIEL A. BASHARI, SONAL HALAI, A. M. MOHAMED, M. I. BUTT, S. O. OYIBOPETERBOROUGH CITY HOSPITAL, UNITED KINGDOM

2014


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2014


A very rare condition, called ectopic (outside location) ACTH (adrenocorticotropic hormone) syndrome, accounts for less than 1% of cases of hypercorticizm.

Clinical Case. Patient D, 16 years old boy, was admitted to our clinic at 20/06/2012 with following concerns: face puffi-ness with matronism and rash, headache, elevated arterial pressure up to 140-160/90-120 mmHg, bright crimson stri-ae on the thighs, abdomen and arms, pain and heaviness in lower limbs, periodic cough with scanty sputum, insomnia and depression.

20/04/2012 - 30/04/12 patient was hospitalized in Sochi hos-pital with left ureter stone diagnosis. He was treated and discharged with satisfactory condition.

On admission to our clinic Cortizol level was 08:00h- 235.8 ng/ml; 16:00h –354 ng/ml; 23:00h– 218.9 ng/ml; ACTH (08:00h) >150;

ACTH (23:00)-78.6 pg/ml;Cortisol in urea- 949 Ug/24h;Arte-rial pressure - 135/90 mmHg.

ECG - rhythm correct, sinus bradycardia 53’. Electrical axis of the heart was in normal position. No conduction interrup-tion. No changes in the heart muscle. Left ventricular over-load indications.

MRI of the brain with contrast showed no pathology. MRI of Adrenal Gland was also without any pathology.

Chest CT showed that in the lower lobe of right lung was visualized nodal formation 5 mm in size. In the right pleu-ral cavity was small amount of fluid. Left lung was without pathology. Heart were slightly enlarged, in pericardium was small amount of fluid.

A number of clinical studies conducted in our hospital al-lowed us to confirm Ectopic ACTH Syndrome diagnosis.

Patient was citizen of Russian and for financial reasons his family decided to take him in Russia for surgery treatment. Clinical examinations were retested and diagnosis of Ectopic ACTH Syndrome was changed by diagnosis - Central Hyper-coticism. Nodular formation of right lung 5 mm in size was considered as the fibrous tissue developed after pneumo-nia. It was made right adrenalectomy, but the range of ACTH was also high and was considered necessary to do inferior petrosal sinus sampling (ipss), which show normal range of ACTH. So they approved our diagnosis and right lung lo-bectomy was performed. In postoperative period ACTH and cortisol levels returned to normal range.

Today patient D is under our surveillance. At present pa-tients hormonal range is still normal.

O-29 Neuroendocrine tumors

ECTOPIC ACTH SYNDROMEMAKA KORINTELI, ELEN GIORGADZE, NATIA CHKHEIDZE, SHOTA JANJGAVA


2014


Introduction: Pancreatic endocrine tumors are rare lesions, with a reported incidence of four cases per 1 million pa-tient-year. Of these lesions, insulinomas are the most com-mon. However, it is a diagnostic dilemma for the clinicians. The classic diagnostic test for an insulinoma is the 72-hour fasting test. Selective arterial calcium stimulation (SACS) test is performed to distinguish a focal abnormality (insuli-noma) from a diffuse process. We report a patient who was diagnosed with insulinoma using Computerized Tomogra-phy (CT), SACS and consequently underwent surgery.

Case Report: A 24 year old man applied to the emergen-cy department because of lightheadedness, palpitations, weakness, sweating and dizziness. A capillary glucose level determined in the emergency department was 39 mg/dl. He was given %30 dextrose IV, and his symptoms resolved. The patient reported that he had similar symptoms intermittant-ly, which started 3 months ago.

He did not have any past medical history and he was not tak-ing any medication. There was no family history of hyper-parathyroidism, peptÄ±c ulcer disease or hypoglisemia.

The phyical examination was unremarkable. He was a well developed man. His abdomen was soft and nontender, with no palpable mass or organomegaly.

On outpatient evaluation, serum values after an overnignt fast were; Glucose:62 mg/dl (normal range, 70-100 mg/dl), Insu-lin:3.43 microIU/ml (1.9-2.3), C-peptide:1.79 ng/ml (0.9-7.1).

He was hospitalised to perform 72-hour fasting test; serum glucose, insulin and C-peptide concentrations during the test are shown in table 1. Ultrasonography of the abdomen revealed a hypoechoic lesion between pancreas and duodenum. CT scan

confirmed a 10 mm hypervascular lesion of the pancreatic head, which was competible with neuroendocrine tumor.

The patient underwent celiac axis arteriography and SACS test in which calcium gluconate was injected into the superior mes-enteric, gastroduodenal, main hepatic, distal splenic and prox-imal splenic arteries and blood was collected from the hepatic vein subsequently, for measurement of insulin levels. A positive result is defined as doubling or tripling of the basal hepatic vein insulin concentration at times 30, 60, 90 sec after arterial cal-cium injection (Table-2). There was a positive response when calcium was injected to superior mesenteric artery; which con-firmed a pancreatic head / neck lesion.

Our patient underwent successful enucleation of the insulo-noma. Resected tumor was 1.5 x 1.5 cm in size. There was no evidence of invasion, abnormal lymph nodes or liver me-tastases. Pathological evaluation revealed a well differenti-ated insulinoma with amyloid deposition.

Discussion: We report an insulinoma case that SACS test confirmed the localisation of the CT detected lesion.

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O-30 Neuroendocrine tumors

AN INSULINOMA LOCALISED BY BOTH IMAGING STUDIES AND SELECTIVE ARTERIAL CALCIUM

STIMULATION TESTMURAT TORGUTALP, MIYASE BAYRAKTAR2, ESRA FIRAT1

1 DEPARTMENT OF INTERNAL MEDICINE, DIVISION OF ENDOCRINOLOGY AND METABOLISM, HACETTEPE UNIVERSITY SCHOOL OF MEDICINE, ANKARA, TURKEY

2 HACETTEPE UNIVERSITY SCHOOL OF MEDICINE, DEPARTMENT OF INTERNAL MEDICINE, DIVISION OF ENDOCRINOLOGY AND METABOLISM, ANKARA, TURKEY

2014


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POSTERPRESENTATIONS

2014


Introduction: Parathyroidectomy is the curative treatment of primary hyperparathyroidism and is recommended for all patients with symptomatic patients and asymptomatic pa-tients that meet criteria of current guidelines for parathy-roidectomy. Postoperative complications of parathyroidecto-my include recurrent laryngeal nerve injury that may cause transient or permanent voice changes, hematoma, wound infection, persistent and transient hypocalcaemia. Also postoperative hyperthyroidism is a complication occurring after parathyroidectomy in primary hyperparathyroidism and is attributed to palpation thyroiditis. Palpation thyroid-itis is supposed to result from traumatic injury or rupture of isolated thyroid follicles caused by pressure on the gland. We presented a transient thyrotoxicosis case occured after parathyroid surgery.

Case: 29 year old woman was referred to our endocrinol-ogy outpatient clinic due to hypercalcaemia. Initial labora-tory results were as follow; Calcium 10,67 mg/dl (8,4-10,2), phosphorus 2,8 mg/dl (2,4-5,4), intact parathormone 271 pg/ml (10-65), albumin 4,5 g/dl (3,5-5,5), vitamin D 18,58 ng/ml (30-90), creatinine 0,5 mg/dl (0,2-1,3), TSH 3,38 uIU/ml (0,4-4,2) freeT4 1,27 ng/dl (0,8-2,7). Parathyroid ultrasound revealed a 5x9x20 mm parathyroid adenoma at inferior pos-terior aspect of the right thyroid lobe and thyroid gland was normal. Bone densitometry showed osteopenia and neph-rolitiasis was detected in renal ultrasonography. Surgery

was recommended to patient. Parathyroidectomy was per-formed to the patient by general surgeon. Pathologic result was parathyroid adenoma. Post-operative intact parathor-mone and calcium level were 5.38 pg/ml and 7.4 mg/dl re-spectively. Calcium replacement was started. 3 days after parathyroidectomy the patient suffered from palpitations and laboratory results revealed thyrotoxicosis (TSH 0.06 uIU/ml (0,4-4,2), freeT4 3,93 ng/dl (0,8-2,7), freeT3 8,86 pg/ml (1,8-5,2). Thyroid uptake and scintigraphy using 99mTc pertechnetate was performed and uptake was low (<%0.1). Propronalol was started for palpitation. 9 days after parathy-roidectomy freeT4 and TSH levels were 1,53 ng/dl (0,8-2,7) and 0,03 uIU/ml (0,4-4,2) respectively. Thyroid function tests were completely improved 15 days after parathyroidectomy.

Discussion: Thyrotoxicosis after parathyroidectomy may be seen due to manipulation of adjacent thyroid gland and release of thyroid hormones. In our patient low radioiodine uptake suggest that thyrotoxicosis was due to release of pre-formed thyroid hormones. A prospective study revealed that thyrotoxicosis is a common complication of parathyroidecto-my that affects %29 of patients and occur between 7-21 days after parathyroidectomy. Moreover, thyroid function tests may be performed in selected cases following parathyroid surgery when excessive manipulation of the thyroid gland happens intra operatively and in patients presenting postop-eratively with features suggestive of thyrotoxicosis.

P-01 Thyroid

TRANSIENT THYROTOXICOSIS AFTER PARATHYROIDECTOMY: CASE REPORT

MURAT SAHIN, AYTEN OGUZ, DILEK TUZUN, KAMILE GULDEPARTMENT OF ENDOCRINOLOGY AND METABOLISM, KAHRAMANMARAS SUTCU IMAM UNIVERSITY FACULTY OF MEDICINE,

KAHRAMANMARAS,TURKEY

2014


I consider the case showing that the multiple endocrine neoplasia type 2A (MEN 2) is rare autosomal dominant syn-drome. MEN 2A associates medullary thyroid cancer (MTC) with pheochromocytoma in 20 - 50% of cases and with pri-mary hyperparathyroidism (HPT) in 5 - 20% of cases.

Case under discussion: A 34-year-old woman visited an endocrinologist after her mother was diagnosed as having aggressive MTC. Her mother underwent the thyroidectomy about 10 years ago when she was 50 years old.

Our woman had discomfort in the neck. She had no weight loss, fever, or thyroid nodule. On physical examination of thy-roid gland and abdomen were normal.

Ultrasound examination of the thyroid gland showed nodules in left and right lobe (0,7Ñ…0,7Ñ…1,0cm, 0,5Ñ…0,5cm) and lymphadenopathy. It was recommended her to implement the ultrasound monitoring. After two years, the ultrasound showed that the nodules increased (1,2Ñ…0,7Ñ…1,0cm, 0,7Ñ…0,5Ñ…0,6cm) and more than five supraclavicular lymph nodes had increased with sign of degeneration. Results of the fine-needle aspiration biopsy of the thyroid showed the follicular thyroid cancer with metastases in these lymph nodes. The patient had normal serum calcium; increased levels of calcitonin, serum metanephrine, and normetanephrine.

On CT scan of the abdomen, the tumor of adrenal gland was found. However, the patient had not symptoms of pheochro-mocytoma.

Thereafter, our patient underwent a thyroidectomy. The results of pathological tests revealed a medullary thyroid carcinoma with multifocal growth pattern and capsule inva-sion. The amyloid test was positive. The lymph nodes were

replaced with the tumor.

Later, patient underwent an adrenalectomy. Results of the pathological tests confirmed pheochromocytoma.

It should be noted that all the family members had signed an informed consent providing the authors with an authori-zation to publish this information.

Discussion: MEN 2A makes up 70 - 80% of cases of MEN 2. As genetic testing for RET mutations has become available, it has become apparent that 70 - 95% of individuals with MEN 2A develop MTC, 50% develop pheochromocytoma, and 15 - 30% develop HPT.

Generally, the MTC is the first manifestation of MEN 2A. Up to 70% of such patients already have cervical lymph node metastases. Pheochromocytomas usually present after the MTC or concomitantly; however, they are the first symptom in 13 - 27% of individuals with pheochromocytomas and MEN 2A.

Our patient had two components of MEN 2A: the MTC and pheochromocytoma, which have been diagnosed at the same time.

Considering the high risk of metastasis, we recommend testing gene RET all the family members of the patient. Ide-ally, testing should be completed preoperatively.

Conclusions: The article presents a rare case of MEN 2A that was only diagnosed after family history MTC became known. Therefore, we urge physicians to pay more attention to the main signs of MEN 2A, since early diagnosis can pre-vent further complications of this fatal disease.

P-02 Thyroid

MEDULLARY THYROID CANCER AS A PART OF MULTIPLE ENDOCRINE NEOPLASIA TYPE 2A

YULIYA DOLINSKAYAINSTITUTE OF INTERNAL MEDICINE, SIBERIAN BRANCH OF RUSSIAN ACADEMY OF MEDICAL SCIENCES

2014


Background: Radioiodine uptake is rarely observed in nor-mal non-lactating breast tissue. Investigation of the in vivo regulation of iodide uptake in breast tissue may be useful for the induction of radioiodine uptake in breast cancer tissue for diagnostic and therapeutic purposes.

Case Report: We report a case of post-menopausal wom-en who underwent radioiodine therapy for papillary thyroid carcinoma andbreast uptake of radioiodine on post-therapy whole body scan (WBS) was observed. The scintigraphic and associated clinical characteristics of radioiodine breast up-take in non breastfeeding thyroid cancer patient undergoing routine radioiodine ablation treatment and whole-body ra-dioiodine scanning was investigated. In this patient, elevated serum prolactin levels (117 ng/mL) were documented at the

time when radioiodine uptake in the breast was observed. The hyperprolactinemia was due to pituitary adenoma which confirmed hypophysis MRI. Medical treatment with bro-mocriptine was given to this patient. When prolactin levels were normalized (by use of dopamine receptor agonist drug in this case), breast tissue uptake was no longer evident on WBS. Six months after she continued that use of dopamine receptor agonist drug , her serum prolactin level was nor-malized to 13.4 ng/mL and breast uptake of iodine was no longer present in a follow-up whole body scan.

Conclusion:This case provide direct evidence that endog-enous hyperprolactinemia can induce significant uptake of radioiodine by breast tissue, even in non-lactating mamma-ry gland.

P-03 Thyroid

RADIOIODINE UPTAKE IN NON-LACTATING MAMMARY GLANDS

UMUT ELBOGA1, MESUT OZKAYA2, HAKAN KORKMAZ2, AYTEN ERAYDIN2, Y. ZEKI CELEN1

1 GAZIANTEP UNIVERSITY ,DEPARTMENT OF NUCLEAR MEDICINE 2 GAZIANTEP UNIVERSITY, DEPARTMENT OF ENDOCRINOLOGY

2014


Introduction: The gastric emptying rate has a decisive role in the regulation of carbohydrate metabolism in diabetic pa-tients.

Objective: Here we describe the clinical case of autonom-ic dysfunction and the associated motility disorder leading to hypoglycemia. A 32 years old manwas admittedto theen-docrinology department with severe recurrent convulsions and transient unconsciousness. His diabetes developed 20 years before when the ill of epidemicparotitis. Although he did not have digestive symptoms, the 13C- octanoic breast test (13C-OBT) gastric emptying was done.The half-time of gastric emptying (HTE) was extremely long (HTE: 318,2 min, normal range: 40-75 min.). The all functional tests reflected a severe stage of gastroparesis, with a dominance of para-sympathetic dysfunction. The glucose in the blood during four time per day was measured on consecutive week.On all

of this time the postprandial glucose was much lower than the fasting value and on 2 of the 6 days it fell down to the hypoglycemic range.Parenteral, later oral metoclopramide, alpha lipoic acid and benfotiamine were administered. The insulin treatment and the diet were also adjusted. 3 months later the patient was symptom-free of hypoglycemia, the HTE was less longer (246.4 min.), did not reveal post-break-fast glucose lowering while the diabetic gastroparesis re-mained severe.

Conclusions:The measurement of the gastric emptying might (13C-OBT) be a part of the differential diagnostics of newly appearing severe hypoglycemia in diabetic patients with chronic complications of disease. The pathogenic and symptomatic treatment of diabetic gastroparesis and neu-ropathy results a stable glucose metabolism, an improve-ment in quality of life.

P-04 Diabetes

SEVERE HYPOGLYCEMIA AS A MANIFESTATION OF DIABETIC GASTROPARESIS

IRYNA KOSTITSKAIVANO-FRANKIVSK NATIONAL MEDICAL UNIVERSITY

2014


Introduction: Thyroid storm is not an uncommon compli-cation among patients with hyperthyroidism. The same is true with Diabetic Ketoacidosis among patients with poorly controlled Type 1 diabetes mellitus. The simultaneous oc-currence of these two endocrine emergencies is however infrequent.

Case Presentation: A 17 year-old female presented with a diffuse abdominal pain and vomiting for one day. She was diagnosed with Graves’ disease with Type 1 diabetes three months earlier. At the emergency room, she appeared drowsy, agitated, highly febrile with body temperature of 38.7 degree Celsius and tachycardic with pulse rate of 160 beats/min. A grade II diffuse goiter was palpated with bilat-eral exophthalmoses and dry skin were found.

Investigation and Management: Initial capillary blood glu-cose revealed hyperglycemia of 499 mg/dl and arterial blood gas noted compensated metabolic acidosis. She had urine

ketones of 50 mg/dl. Furthermore, her thyroid profile re-vealed a significantly low thyroid stimulating hormone and an elevated free T4. Burch and Wartofsky scoring showed a score of 65, highly suggestive of thyrotoxic crisis.

Patient was managed with high dose propylthiouracil, dexa-methasone and propranolol simultaneously with hydration, Insulin and Bicarbonate correction for the acidosis. Sub-sequently after meticulous management of the condition patient improved significantly and was discharged with full recovery.

Conclusion: The simultaneous presentation of two endo-crine emergencies such as Diabetic Ketoacidosis and Thy-roid Storm is possible. In the setting of Diabetes Mellitus, the concomitant occurrence of Hyperthyroidism may predis-pose patient to succumb to life-threatening complications like Diabetic Ketoacidosis and Thyroid Storm

P-05 Diabetes

FATAL COMBINATION: THYROID STORM AND SEVERE DIABETIC KETOACIDOSIS

JEROME REBOLLOS BARRERA, ADAMIER ABDULLAZAMBOANGA CITY MEDICAL CENTER

2014


Objective: to estimate the parameters of quality of life (QOL) in type adolescens with diabetes mellitus type 1 ( DM1T ).

Materials and methods: the study group was included 42 ad-olescents (15 ± 0,9 years), desease duration was 2-7 years (an average 4,6 ± 1,4 years). We conducted general exam-ination and psychological questionnaire ADDQoL.

Results:the mean level of QOL was 1,38 ± 1,0 points. The majority of patients (67%) rated their QOL as "very good" and "good €, 62% of the patients stated that if they did not have diabetes, QOL "would be better".

DM1T has a negative impact on all aspects of life (- 2,8 ± 0,8). The most negative effect was noted in (-4): professional life, travel, freedom of choice of food and drinks. To a lesser extent diabetes affects (-2) freedom of movement family life, outside, motivation, the reaction of others, confidence in the future, financial position, living conditions.

It was found that diabetes has a more pronounced effect on the QOL of boys than girls (p = 0,045).Boys often showed a

decrease in the scales: of travel (-5.9), freedom of choice of food (-5.8), of beverages (-5.8), professional life (-5), free time (-4, 8), confidence in the future (-4), physical condi-tion (-4), confidence (-3.9), social services (-3.5), wealth (-3.5), freedom of movement (- 3).While the girls are most reduced following indicators: professional life (-2.8), free-dom of choice of food (-2), leisure (-1.8), appearance (-1.8), the scope of travel (-1.7), social services (-1.5), freedom of movement (-1.4), physical condition (-1.3).

The study found relationship between the level of glycemia (p = 0,042), HbA1c (p = 0,02), duration of disease (p = 0,036) with the aspects of quality of life, according to a ADDQoL.

Conclusions: 1. DM1T has a negative impact on all aspects of quality of

life. 2. We found gender differences, providing a more pro-

nounced effect on the QOL of boys over girls. 3. The level of blood glucose, HbA1c, duration of disease affects the quality of life.

P-06 Diabetes

THE AUDIT OF DIABETS- DEPENDENT QUALITY OF LIFE IN ADOLESCENTS WITH TYPE 1 DIABETES

MARIIA MATVEEVA, JULIA SAMOYLOVASIBERIAN STATE MEDICAL UNIVERSITY

2014


Introduction: Diabetic foot ulcers are the leading cause of lower limb amputations in the diabetic population. For treat-ment, there are several different techniques one of which is local recombinant human derived epidermal growth factor (rhEGF) into the wound.

Case Report: A 42-year old male patient diagnosed with type 2 diabetes five years ago was referred to our clinic for the foul-smelling, leaky lesion on his first toe of the right foot for one month. He was admitted to the hospital with the diagnosis of uncontrolled diabetes and diabetic foot in-fection and intensive insulin and antibiotic therapies were started(Fig.1). The patient was a truck driver and the reason for the infection was found to be a neuropathic ulcer due to repetitive foot trauma. Tissue culture was taken and Pip-eracillin/Tazobactam 3x4.5gr IV antibiotherapy was started soon after. Enterococcus spp. was detected in the culture. The debridement of the lesion was done by plastic and re-constructive surgeons (Fig.2). After the succesful debride-ment and proper antibiotic therapy, the infection resolved. For the rapid wound healing, the diabetic foot infection com-mittee of our hospital decided to administer rhEGF locally. 75 mcg rhEGF was given three times a week intra-lesionally. The rapid wound healing was observed after three weeks of therapy (Fig. 3).

Discussion: Diabetic Foot Syndrome is defined as the clin-ical situation that includes diabetic neuropathy, peripheral vessel disease, Charcot neuroarthropathy, ulcer, osteomy-elitis and amputation. Local rhEGF administration is a novel method for the treatment of chronic diabetic foot ulcers. A double-blind study done by JosÃ© I FernÃ¡ndez-Montequin et al. showed that this is an efficacious method on wound healing. In our case, we also detected a marked healing and

rapid granulation tissue formation in a diabetic foot ulcer that could be resulted with amputation.

Intra-lesional human-derived epidermal growth factor administration is a new treatment option for the treatment of diabetic foot syndrome. New, large, randomized controlled clinical trials are needed to support the effectiveness of this meth-od.

Resim 1

Resim 2

P-07 Diabetes

INTRA-LESIONAL EPIDERMAL GROWTH FACTOR FOR THE TREATMENT OF NON-INFECTIVE DIABETIC

FOOT ULCEROZLEM HALILOGLU1, MUCAHIT OZYAZAR1, ABDULKADIR ERCALIŞKAN2, ERDEM KOLEMEN2

1 ISTANBUL UNIVERSITY CERRAHPASA MEDICAL FACULTY DEPARTMENT OF ENDOCRINOLOGY AND METABOLISM 2 ISTANBUL UNIVERSITY CERRAHPASA MEDICAL FACULTY DEPARTMENT OF INTERNAL MEDICINE

2014


Resim 3

2014


Introduction: Werner’ syndrome (WS) is an rare autosomal recessive, hereditary systemic disease with progeroid fea-tures, usually presenting in adolescence. Its clinical mani-festations are short stature, scleroderma-like skin changes, cataracts, premature aging of the face, gray hair and gen-ital hypoplasia. Also endocrinological abnormalities were showed such as osteoporosis, hyperglycemia and predispo-sition to malignancy could occur at the late stagees of the syndrome. Death of WS can be seen due to myocardial in-farction or malignancy. Cirrhosis is an extremely rare man-ifestation of WS. Here, we want to present a case with WS who had cirrhosis due to steatohepatitis during of follow-up period in our clinic.

Case Report: We previously reported two siblings with WS that we followed since our the initial diagnosis. They were at 20 and 16 years of ages at their first diagnosis. Elder sister was died after 13 years follow up due to sudden cardiac ar-rest at 33 years old. The living second case is now 31 years-old. He had been followed the last presention for liver failure were evalueted and cirrhosis was diagnosed due to steato-hepatitis by liver biopsy. In 1999 initial diagnosis; cataracts, diabetes mellitus, short stature, and genital hypoplasia (mi-cropenis and right cryptorchidism) were found. In 2002 was showed steatohepatitis by liver biopsy which was performed for the persisting high liver transaminases. In 2007 devel-oped gray hair, high-pitched voice, hypergonadotropic hypo-gonadism and osteopenia. He was a month ago had been hospitalised for nausea and vomiting with hematemesis. He was admitted to our hospital performed in the upper gas-trointestinally endoscopy by the department of gastroenter-ologia was found esophageal varices and to them was per-formed band ligation. Laboratory investigations performed in our clinic revealed the following results: Hb 9.4 g/dL, MCV

75 fL, WBC 4000 µL, Plt 103000 µL. Blood analysis showed glucose 201 mg/dL total protein 7.1 g/dL, albumin 2.8 g/dL, ferritin 5.4 ng/dL, HbA1c 7.0%, PT 14.6 second, AST 59 U/L (0 to 40), ALT 30 U/L (0 to 41), protein electrophoresis was showed as hyper gammaglobuline (Î³ 40.15%) with broad-based monoclonal spike. It was not found for other causes of chronic liver disease. Abdominal doppler ultrasonography was showed the portal vein diameter 10 mm, long axis of the spleen 130 mm. An abdominal CT showed splenomegaly, intra-abdominal minimal free fluid and liver was consistent with chronic liver disease. A liver biopsy was performed and revealed cirrhosis due to steatohepatitis (Figure 1). He is still following by the both endocrinology and gastroentero-logia departments.

In conclusion, reason of the our presentation in WS is very rare finding circcosis due to steaohepatitis.

Figure 1. Microscopically, liver is replaced by regenerative nodules surrounded by fibrous bands (Hematoxyline& Eosin (HE), x40)

P-08 Diabetes

A RARE CASE WITH WERNER’ SYNDROME WAS DEVELOPED CIRRHOSIS DUE TO STEATOHEPATITIS

DURING OF FOLLOW-UP PERIODMEHTAP EVRAN1, GAMZE AKKUS1, MURAT SERT1, TAMER TETIKER1, MAHMUT SANSAL2

1 CUKUROVA UNIVERSITY MEDICAL FACULTY DEP. OF INTERNAL MEDICINE DIV. OF ENDOCRINOLOGY 2 CUKUROVA UNIVERSITY MEDICAL FACULTY DEP. OF INTERNAL MEDICINE DIV. OF GASTROENTEROLOGY

2014


Background:Type 2 diabetes mellitus is not any more rare in young.

Method:A 20 year old obese female with a BMI of 38 kg/m²presented with polyuria, polydipsia and fatigue. She was given a treatment based on insulin as she was Type 1 dia-betic. She was autistic(IQ=57). Her grandmother had Type 2 diabetes mellitus and her cousin had Type 1 diabetes melli-tus. Her fasting plasma glucose and HbA1C were 155 mg/dl and 10.2% respectively at diagnosis. Fasting c-peptide was found normal (6,99) , anti-GAD (<5) andanti-insulin (<0.40) antibodieswere found negative. Her lipid and hormon pro-file including thyroid hormones were normal. Diabetic com-plications such asneuropathy, nephropathy and retinopa-

thy didn’t occur. Finally, her insulin treatment was stopped and metformin was started together with diet+exercise. 6 months later, herfasting plasma glucose and HbA1C were 79 mg/dl and 5,8%; 12 monthslater,herfasting plasma glucose and HbA1C were 85 mg/dl and 5,2% respectively.

Result:Our patient continued well with lifestyle changes (di-et+exercise) and metformin.

Conclusion:Type 2 diabetes mellitusis occuring as a new clinical problem in children and adolescents. Recent reports indicate an increasing prevalence oftype 2 diabetes melli-tusin young. Management of choice is lifestyle intervention followed by pharmacological treatment (e.g., metformin).

P-09 Diabetes

IS EVERY YOUNG DIABETIC TYPE 1?OMUR TABAK, BELGIN BORAN, AYSE FADILOGLU, EYYUP CAVDAR, MEHMET HURSITOGLU

ISTANBUL KANUNI SULTAN SULEYMAN EGITIM VE ARASTIRMA HASTANESI

2014


Introduction: Disorders of sexual differentiation (DSD) are in itself rare entities but in past 15 years cases of anomalies of male sexual differentiation are on the rise.Several research-ers have pointed out the role of several endocrine disruptors as a probable cause of these disorders. We report a case of 46XY DSD child whose in-utero development was probably interfered with the maternal intake of Lamotrigine.

Case Report: A case of 1 year old child born of non-consan-guineous marriage with father’ age being 29 & mother’ age being 25 at the time of conception has been presented to us with genital ambiguity noted since birth. The baby was born of full term normal delivery with birth weight of 3.6 kg pre-ceeded by an uneventful antenatal period excepting the fact that the mother was suffering from seizure disorder & was on lamotrigine 150 mg/day during pregnancy. There was no episode of seizure during the antenatal period. No historyof maternal virilization during pregnancy. The baby cried im-mediately after birth & had no history of prolonged jaundice, hypoglycemia or hypotension since birth. There was history suggestive of global developmental delay since birth. The genital examination of the child revealed under androge-nized male genitalia in the form of labio-scrotal folds with mild pigmentation & rugosity which contained palpable go-nads bilaterally, the volume being around 1 ml each. There was a phallus with a length of 2.5 cm & diameter of 1 cm and urethral orifice present at base of the phallus. Initial ante-rior pituitary hormonal evaluation revealed a normal thyroid profile and normal hypothalamo-pitutory-gonadal axis in the form of normal serum LH, FSH and normal basal testoster-one levels which was appropriate for a child aged 9 months.The serum DHEAS (Dehydroepiandrosterone-sulfate) and serum androstenedione also measured. hCG (human chori-onic gonadotrophin) stimulation test performed by injecting

5000 IU of hCG intramuscularly & collecting blood for stim-ulated hormone profiles after 72 hours.From the aforemen-tioned data the serum testosterone: androstenedione ratio was found to be 866.66 which disprove 17-Beta-Hydroxy steroid dehydrogenase deficiency. The ratio of testosterone to dihydrotestosterone (T:DHT) was calculated as 21.5. The definitive diagnosis of 5-Alpha Reductase type 2 deficiency in post pubertal male requires a ratio of 35 but it has been mentioned in medical literature that in the neonatal period any ratio greater than 8.5 may point to 5-alpha Reductase Deficiency & in infants the ratio typically exceeds 10.Thus it suggests diagnosis of 5-alpha reductase type 2 deficiency.

Conclusion: Our case probably for first time throws light on the possibility of lamotrigine as an endocrine disruptor causing ambiguous genitalia because of an inhibitory impact on the enzymes of androgen biosynthetic pathway, perhaps 5-Î± alpha reductase enzyme also.

Resim 3

P-10 Male reproductive endocrinology

A UNIQUE CASE OF LAMOTRIGINE INDUCED DISORDER OF SEXUAL DIFFERENTIATION

SATYAM CHAKRABORTY, ANIRBAN SINHA, ANIMESH MAITI, BIPLAB MANDAL, LAKSHMI DEY, SUBHODIP PRAMANIK, RAVINDRA SHUKLA

DEPARTMENT OF ENDOCRINOLOGY, MEDICAL COLLEGE AND HOSPITAL, KOLKATA

2014


Resim 4

Resim 5

2014


Introduction: Parathyroid carcinoma is a rare cause of hy-perparathyroidism. In most cases, hyperparathyroidism is caused by a single benign adenoma. The vast majority of the remaining cases are caused by parathyroid hyperplasia or multiple adenomas. We present here a case of a young Fil-ipina who presented with multiple fractures on all extremi-ties and bilateral parathyroid adenoma.

Case Presentation: A 19 year-old Filipina presented with bone pains for 7 months associated with fatigue, anorexia, weight loss and muscle weakness. Subsequently, she had multiple fractures on all extremities and fixed hard masses on the left humerus and tibia. Her past medical and family histories were unremarkable.

Investigations:Corrected calcium was elevated at 15.4mg/dL, Phosporus was decreased at - . Intact PTH (iPTH) was extremely high at 2001 pg/ml (8.5-72.5). X-ray of the extrem-ities showed osteopenia, endosteal resorptive changes and multiple pathologic fractures. Bone biopsy revealed brown cell tumor/multifocal polyostotic giant cell tumor and nega-tive for malignancy.). Ultrasonography of the neck revealed parathyroid adenoma inferiorl ofleft thyroid gland measuring

2.3 x1.1x1.0cm. Ultrasound-guided FNAB revealed findings consistent with parathyroid carcinoma. She was hydrated and was given diuretic to control the severe hypercalcemia. She underwent 3 ½ gland parathyroidectomy with en-bloc left thyroid lobectomy. Intraoperative findings showed a ___ left mass with non well-circumscribed borders invading the capsule and local tissues and a right mass _______ .Serum Calcium and iPTH immediately after OR decreased to 12.8 mg/dL and 211.8 pg/ml respectively. Further reduction was noted after 24 hr of surgery (iPTH, 48 pg/ml; Corrected Cal-cium: and 9.2 mg/dL).

Discussion/Conclusion: Parathyroid carcinoma is a rare malignancy of the parathyroid glands. These tumors usually secrete parathyroid hormone, thereby producing hyperpara-thyroidism, which is usually severe. Surgery with en-bloc re-section is the initial therapy, but when the tumor is no longer amenable to surgical intervention with intent to cure, treat-ment becomes focused on the control of hypercalcemia.

Key words: Parathyroid carcinoma, hypercalcemia, hyper-parathyroidism

P-11 Bone/Calcium

BROWN CELL TUMOR FROM PARATHYROID CARCINOMA

JEROME REBOLLOS BARRERA, CHRISTIAN CESAR ESPLANAZAMBOANGA CITY MEDICAL CENTER

2014


Introduction: Primary hyperparathyroidism, one of the most common causes of hypercalcemia, results most oftenfrom the occurrence of one or more adenomas in previously nor-mal parathyroid glands. Inherited forms of primary hyper-parathyroidism are uncommon.We present a case of primary hyperparathyroidism caused by parathyroid adenoma asso-ciated with hyperaldosteronism (Conn’s syndrome), which is rarely seen in clinical practice.

Case: A 38-year-old male patient was admitted to our poly-clinic for hypercalcemia detected by another center. In an-amnesis he took ramipril+hydrochlorothiazide for hyperten-sion 2 years ago.After starting hypopotassemia this medicine has replaced with amlodipine. Family history was normal.TA 150/90 mmHg, pulse 74/min. The other systemic and labo-ratory findings were normal. Serum Ca 10.9mg/dl (8.8-10.2), inorganic P 2.4mg/dl (2.7-4.5), Mg 2.3(1.6-2.6), albumin 3.9 gr/dl, PTH 200pg/ml (12-88), Na 138mmol/L, K+ 2.8mmol/L, 25-OH vitamin D3 22ng/ml (14-66). Calcium level in 24-hour urine 251mg/day, inorganic P 88mg/day, and K+ value in spot urine 66mmol/L. Aldosterone level was measured after nor-malization of his serum potassium level (result: 26.9ng/dl), plasma renin activity was <0.15ng/mL/hour. Since associat-ed primary hyperparathyroidism and adrenal adenoma were found, the patient was evaluated for MEN syndromes. Levels of catecholamine and its metabolites in 24-hour urine were normal. In the 1 mg dexamethasone suppression test, se-rum cortisol level was suppressed. Serum prolactin, TSH, ACTH, GH, IGF-1 and calcitonine values were normal. Thy-roid ultrasound revealed a 24x14x12.5 mm hypoechoic area at the upper posterior part of the right thyroid lobe, within the thyroid capsule (intrathyroidal parathyroid adenoma?). MIBI scintigraphy of parathyroid glands was consistent with the ultrasound finding. Abdominal MRI revealed 1.6cm ad-

renal gland lesion consistent with adenoma. Pituitary MRI was normal.

The patient was operation on parathyroid adenoma (Picture 1). At the after the operation serum Ca, inorganic P andPTH levels were foundnormal. The result of pathology confirmed the parathyroid adenoma. He had started spironolactone for hypertension, but the drug was discontinued as he suffers from erectile dysfunction. He was referred for laparoscopic operation for the lesion (1.6 cm) identified at the left surre-nal gland. Pathologic examination revealed surrenal adeno-ma (Picture 2). Since it was seen that his TA was normal and hypokalemia was corrected during the follow-up examina-tions, he was not prescribed any anti-hypertensive medica-tion. Genetic screening revealed that he is negative for the mutation of the MENIN gene.

Conclusions: In our case, diagnosis of MEN 1 syndrome could not be confirmed genetically. However, the main rea-son of the presentation is the fact that association of para-thyroid adenoma and Conn syndrome is rarely seen in clin-ical practice.

Picture 1. It was seen parathyroid adenoma at the operation

P-12 Bone/Calcium

A RARE CASE OF PRIMARY HYPERPARATHYROIDISM ASSOCIATED WITH PRIMARY ALDOSTERONISM

BARIS SARIAKCALI, MEHTAP EVRAN, MURAT SERT, TAMER TETIKERCUKUROVA UNIVERSITY MEDICAL FACULTY DEP. OF ENDOCRINOLOGY, ADANA, TURKEY

2014


Picture 2. Pathologic examination revealed surrenal adenoma

2014


Hypopituitarism results from the impaired production of one or more of the anterior pituitary trophic hormones. Reduced pituitary function can result from inherited disorders; more commonly, it is acquired and reflects the mass effects of tu-mours or can be the consequence of head trauma or radi-ation. Radiation damage occurs after cranial irradiation in cases where the hypothalamus and pituitary fall within the field of radiation. Hormone deficiency is dose dependant. The most vulnerable is the growth hormone axis, followed by gonadotropins and less vulnerable adenocorticotropic and TSH axis.

Case: We report on a 54 years old male who had maxillary sinus adenocarcinoma, which was resected surgically with a full dose radiation therapy (60Gy) following. 3 years later he was first admitted to an urologist because of ED and then to our clinic on account of low values of testosterone. He told us that he was not feeling well for the last 2 years and that it got worse in the last 6 months. He was tired, had no energy, felt cold, lost almost all hair on the extremities and chest, had no libido, lost 12kg, slept more than usual.

At clinical examination he was pale, had very fine, dry skin, weak pigmentation, almost no secondary sexual character-istic but there were no other pathological findings.

Laboratory findings: Normal total blood count, mild hypo-natriemia (134mmol/L) and hypokalemia (3.7mmol/L), all other biochemical findings were normal.

Hormone findings: TSH 6.2, FT3 3.29; FT4 5.2; Cortisol in 1ug Synacten test after 30 min 240; ACTH 12.9; IGF1 <25; testos-terone 1.7; LH 0.6; FSH 1.2; prolactin 64;

MR of the head: no changes in sellar space.

Discussion: With laboratory findings we confirmed the di-agnosis of hypopituitarism. The cause was radiation therapy of the maxillary sinus adenocarcinoma. The patientreceived hormone replacement therapy with hydrocortisone 30mg/d and levothyroxine 75mcg/d.

After 3 months he felt better, the urologist did not find any contraindications for testosterone therapy, therefore we added testosterone gel 50mg/d.

After 6 months the patient’s hormonal status was normal, he felt much better, gained some weight, shaved regularly, libido was normal. We are continuing with the hormone-re-placement therapy at regular check-up.

Conclusion: Hypopituitarism can be a life-threatning com-plication of cranial irradiation in cancer survivors. The clin-ical findings are usually gradual and nonspecific. Regular assessments of the anterior-pituitary function are important in such patients to achieve a timely diagnosis and enable the introduction of appropriate hormone-replacement therapy.

P-13 Pituitary

HYPOPITUITARISM AFTER FULL DOSE CRANIAL RADIATION THERAPY - CASE REPORT

EVA ZEMLJICUNIVERSITY MEDICAL CENTRE MARIBOR, SLOVENIA

w w w . e n d o b r i d g e . o r g

DMR Congress / Incentive / OrganizationBarbaros Blv. Akdoğan Str. No: 23/2

Beşiktaş, İstanbul - TurkeyPhone : +90 533 111 9 367 • Fax : +90 212 258 5 029

[email protected] • www.dmrturizm.com.tr

The Society of Endocrinology and Metabolism of TurkeyMeşrutiyet Cad. Ali Bey Apt. 29/12Kızılay, Ankara - TurkeyPhone : +90 312 425 20 72 • Fax : +90 312 425 20 [email protected] • www.turkendokrin.org

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