SEMINAR ON:- DISEASES OF PARATHYROD MULTIPLE ENDOCRINE NEOPLASIAS APUDOMAS

SUBMITTED BY:- TANVI OHRI ROLL NO. 16941 BDS THIRD

YEAR.

increasing osteoclastic resorption of bone increasing intestinal absorption of

calcium increasing synthesis of 1,25-(OH)2D3 increasing renal tubular reabsorption of

calcium increasing excretion of phosphate.

A clinical condition chracterised by exessive secretion of parathyroid harmone

1. Primary hyperparathyroidism results from a hyper function of the parathyroid glands themselves. There is over secretion of PTH due to adenoma, hyperplasia or, rarely, carcinoma of the parathyroid 

2. Secondary hyperparathyroidism:-IT is the reaction of the parathyroid glands to a hypocalcemia caused by something other than a parathyroid pathology, e.g. chronic renal failure.

3. Tertiary hyperparathyroidism result from hyperplasia of the parathyroid glands and a loss of response to serum calcium levels. This disorder is most often seen in patients with chronic renal failure and is an autonomous activity

GENERAL:-weight loss,tiredness,lassitude GI TRACT:-nausea,

vomiting,anorexia,constipation,peptic ulceration

CNS:-drowsiness ,lack of concentration,depression,loss of memory,myopathy

RENAL:-polyurea,polydipsia,stones formation,nephrocalcinocis,may be precipitation of gout and uraemia

BONE:-bone pain,tenderness, fractures,deformities,osteitis fibrosa cystica

CARTILAGE:-chondrocalcinosis CADIOVASCULAR:-hypertension SKIN:-pruritus EYE:-corneal calcification SOFT TISSUE:-ectopic calcification of soft

tissue of hands and feet

FOR DIAGNOSIS:- BIOCHEMICAL:-1. SERUM CALCIUM:-raised2. SERUM PHOSPHATE:-decreased3. SERUM PTH:-raised4. SERUM ALKALINE PHOSPHATE:-raised RADIOLOGICAL:-1. X_RAY OF PHALANGES SHOW

SUBPERIOSTEAL EROSIONS2. LATERAL X-RAY OF SKULL SHOW “PEPPER

POT” APPEARANCE3. X-RAY OF KIDNEY SHOW SCATTERED

OPACITIES DUE TO NEPHROCALCINOSIS4. SOFT TISSUE CALCIFICATION

FOR LOCALISATION OF TUMOUR:-

Ultrasonography of pararhyroid Selective neck vein catheterisation and

pth measurements Ct scan Substraction imaging by radionuclide

studies

Asymptomatic old patients with serum calcium level less than 3.0mmol/L do not require treatment.

Surgical removal of adenoma. If there is hyperplasia of all the 4

parathyroid glands, then treatment is to remove them & transplant some of the parathyroid tissue in the fore arm

If hypercalcaemia retuns after transplantation, a part of tissue can be removed under local anaesthesia

Hypocalcaemia , after surgery is beter treated by calcium:-

-1-alpha hydroxicholecalciferol(alphacalcidol) 2.0microgm DAILY FOR 48hrs BEFORE SURGERY & CONTINUE FOR 1-2 WEEKS AFTER SURGERY

-Calcium gluconate (i.V.) AS 10ml 10% SOLUTION

3-4hrs DURING POSTOPERATIVE PERIOD -Oral calcium supplements used

Hypercalcaemia  is an elevated calcium level in the blood.[1] (Normal range: 9–10.5 mg/dL or 2.2–2.6 mmol/L)

Nousea Vomitting Pain in abdomen Dehydration Hypotension followed by different stages

of unconciousness

hypoparathyroidism is decreased function of the parathyroid glands, leading to decreased levels of parathyroid hormone

Tetany Cataract Psychosis Basal ganlia calcification Epilepsy Papilloedema Candidiasis of nails, skin & mucous

membrane

True hypoparathyroidism:-Deficient production of pth due to heritable or acquired causes

Pseudo hypoparathyroidism:-A Heritable or congenital disorder in which there is tissue resistance to the effects of pth.

1. POSTOPERATIVE:-due to complication of thyroid surgery , damage to parathyroid glands or their blood supply &surgery of graves disease

2. IDIOPATHIC HYPOPARATHYROID:-it is either due to autoimmune destruction of parathyroid glands or is associated with mucocutanious canadiasis

3. INFANTILE HYPOPARATHYROIDISM:-it may be transient or persistent. Transient occurs in infants born to mother who were suffering. Persistent is seen due to thymic aplasia

4. PSEUDOHYPOPARATHYROIDISM:-This is a congenital variety in which parathroidharmone production is normal, but there is resistence to its effect.in this mental retardation ,skeletal or osseous abnormalties i.e.short 4th &5th metacarpals & metatarsals with short stature.Serum PTH level elevated.

5. PSEUDO-PSEUDOHYPOPARATHYROIDISM:-ossious menifestations are present without any clinical symptoms & signs.

Parathroid harmone substitution threpy Ca OR vitd ANALOGUE(1ALPHA

CALCIDOL) In acute condition, calcium gluconate(i.V)

10ml 10% solution 3-4 hrs INTERVAL BY ALPHA CALCIDOL OR CALCITROL IN DOSES OF 1-3 microgm DAILY

It is chracterised by low levels of ionised calcium leading to increased neuromascular excitability. Ca LEVEL MAY REMAIN NORMAL.

Neuromuscular function, calcium, potassium & magnesium’s low levels

Hypocalcaemia:-1. Malabsorption2. Osteomalacia3. Hypoparathyroidism4. Chronic renal failure5. Acute pancreatitis6. Anticonvulsans (dilantin)

Hypomagnesaemia Alkalosis or hypokalaemia:-1. Repeated vomiting2. Excessive intake of alkalies3. Hysterical-hyperventilation leading to

alkolosis4. Primary hyperaldosteronism5. ACUTE ANION LOAD

(CITRATE,LACTATE ,hco3etc)

It may be latent or menifest. IN CHILDREN:-triad of carpopedal spasm,

stridor, convulsions may occurred in various combinations

IN ADULT:-tingling sensatins in peripheral parts of limbs or around the mouth. Less often,painfull carpopedal spasms may occur. Stridor & convulsions are rare.

Absence of signs Signs can be indused by provocative

tests:- TROUSSEAU’S SIGN:-raising of blood

pressure above systolic level by inflation of cuff produce chracterstic carpal spasm within 3-5 minutes

CHVOSTEK’S SIGN:-a tap at facial nerve at angle of jaw produses twitching of facial muscles

TREAMENT OF HYPOCALCAEMIA:-Injetion of 20ml of 10% calcium gluconate may be given to correct hypocalcaemia & relieve tatany. An intrammuscular injection of 10ml may be used for prolonged effect.

TREATMENT OF ALKALOSIS:-Isotonic solution (i.V.) For vomiting.

-Withdrawl of alkalies. If not relieved, ammonium chloride 2.0g ORALY AFTER EVERY 4hrs

-Hyperventilation:-inhalation of 5% co2 in oxygen may be prescrived or ask for rebreath the expired air from asuitable bag.

-Treated bypsycothrepy.

In which more than one endocrine glands become the site

of neoplasia.

It is an autosomal dominant disorder associated with mutation of MEN-1 tumor suppressor gene on chromosome 11.

In this group the parathyroid glands are most frequently involved(chief cell hyperplasia-90%) producing hyperparathyroidism which is often mild.

There is often multiple pancreaticoduodenal neuroendocrine tumors causing Zollinger-Ellison syndrome

The anterior pituitary is involved next (65%) causing acromegaly or cushings syndrome or there may be hyperplasia of adrenal cortex and carcinoid tumor.

Duodenal ulceration is a common feature of this syndrome.

It may be related to hyperparathyroidism as hypercalcaemia promotes the secretion of gastrin.

Prathyroidectomy often reduces the secretion of the acid and sometimes cures duodenal ulceration.

In fact operation for duodenal ulcer should be postponed untill all endocrine lisions have been treated.unless the patient has pancreatic gastrinoma , for which total gastrectomy should be performed.

Other associated disorders include thyroid neoplasms(adenoma or differentiated thyroid carcinoma) , adrenal neoplasms and lipomas.

In this case tere is medullary carcinoma of thyroid with pheochromocytoma and hyperparathyroidism.

It is further divided into 2a and 2b. In type 2a hyperparathyroidism is seen in

25% of patients whereas in 2b there are additional neurofibromas affecting lips,eyelids and face producing swellings in these regions, megacolon and ganglioneuromatosa.

Hyperparathyroidism is rarely seen with this syndrome.

APUDOMAS are endocrine tumors which arise from apud cells.

The ‘APUD’ cells are widely distributed in the body and have common cytochemical characteristics related to synthesis of polypeptides and amines.

The name APUD was first proposed by Pearse in 1968 and is derived from initial letters of main properties of these cells---

1. High content of amine.2. The capacity for amine precursor

uptake from the environment.3. Presence of amino acid decarboxylase

for conversion of amino acid into amines.

The average diameter of these apud cells is 100-200 micrometers and contain dense storage granules of their polypeptide products.

Majority of gastrointestinal apud cells possess long apical processes which reach the glandular lumen ending in tufts of microvilli.

Pearse suggested that neural crest cells should be considered as ancestors of these apud cells.It is well accepted fact that the apud cells, be it in the carotid body, the thyroid, anterior pituitary,elimentary tract and even parathyroid arise from neural crest or neuroectoderm.

APUDOMAS can be conveniently classified into the following groups:-

A. ORTHOENDOCRINE.1.Tumors secreting normal polypeptides of their

cells of origin eg. Insulinoma,gastrinoma, pancreatic glucogonoma, calcitoninoma.

2.Tumors secreting normal amines of their cells of origin eg. Phoechromocytoma, neuroblastoma and ganglioneuroma(all from adrenal medulla) and carcinoid tumor.

B. PARAENDOCRINE APUDOMA SYNDROME(PES)1.Tumors of endocrine glands secreting hormones

characteristic of other glands.2.Tumors of organs, not usually regarded as

endocrine in nature, secreting hormones eg.oat cell carcinoma of bronchus producing ectopic ACTH & WDHA syndrome which is non- beta-islet cell tumor of pancreas presenting with water diarrhoea, hypocalcaemia and hypo or achlorhydria.