CASE REPORT

DR VESELINKA DJURISICINSTITUTE FOR CHILDREN’S DISEASES

MONTENEGRO

• Female infant, 8 month old;• Admitted to hospital due to:

– Diarrhoea;– Metabolic disbalance; – With sings of enteropathy.

History of Present Illness

• 15 min before admission to hospital present with sudden abdominal cramping, cyanosis of limbs, she was lethargic, with drooping head.

• Day before admision, she was sleepless, agitated, inconolably crying, with non bilious, non-projectile vomiting (4 times/day), and she had 10 regular stools.

• 9 days before she was addmited to hospital due to vomiting, diarrhoea and high fever.

Personal hystory:

• Third child from regular pragnancy and term delivery completed with caesarean section.

• Birth weight: 2830 g; Birth length: 53 cm; AS 9• Breastfeeded 3 mo, after that continued

adapted milk formula, 1 month later started mixed non-milk nutrition.

• No history of allergy, regularly vaccinated

Family history

• Older brother – convulsions trated with AET;• Father – epilepsy;• Mother – chronic enteropathy in childhood

suggested gluten free diet, but she refused;• Grandfather – COPD;

Clinical finding

• Weight 7 kg• Agitated, crying, groaning, dehydrated, afebrile

(36,7 C), hemodynamically stable;⁰• Vital sings: RR 36/min, CF 136/min, spO2 93%; • Skin: pale, marble, with limbs cyanosis. • Left torticollis, slight axial hypotonia.• Normal auscultatory findings of lungs and heart . • Abdominal examination: abdominal distension ,soft

and nontender, without tumefacts and organomegaly.

Laboratory findingsAcido Base Balance

pH 7,240

pCO2 3,65 kPa

pO2 8,51 kPa

HCO3 11,5 mmol/l

BE - 14,2

sO2 92,9%

Full Blood Count

ESR 3

WBC 31,4 10,1

RBC 6,21 5,20

HGB 125 108

MCV 64 65

MCH 20 20,7

HCT 40% 34,2%

PLT 835 376

Coagulation status

PT 16,7 s

INR 1,31

aPTT 21,2 s

D – dimer 0,64 mg/l

Fibrinogen 2,2 g/l

BiochemistryCRP 0,2Glucose 3,0Total protein 52Albumin 30Blood urea nitrogen 1,7

Creatinine 24Sodium 135Potassium 3,5Calcium 2,13AST 42ALT 32ALP 326CK 82LDH 327AFP 1,5

Normal urin dipstick and sediment findings

• Tissue transglutaminase antibody:– IgG 269;– IgA > 300;

• Anti – gliadin antibodies: – IgG 6 ;– IgA > 300;

Microbiology

• Stool culture, ova and parasite testing, Rotavirus and Adenovirus: NEGATIVE.

• Stool: positive for Candida sp.• Urin culture: negative.

Radiology findings

• X-ray plain film

air-fluid levels

Radiology findings

• Ultrasound revealed mass suspected to intussusception in right hemiabdomen: Target sign (also known as the doughnut sign)Pseudokidney sign

Radiology findings

• Abdominal CT scan reveals dilated and fluid-filled loops of small bowel with air-fluid levels the classic ying-yang sign of an intussusceptum inside an intussuscipiens in right hemiabdomen.

• Rectoscopy was performed: reveal normal. – The lining of the colon appears smooth and pink,

with numerous folds.– No abnormal growths, pouches, bleeding, or

inflammation is present.

COURSE

• Treated with antibiotics: – metronidazole – gentamicin

• Corticosteroids: – methylprednisolone

• H2 blockers: – ranitidine

• Transfusion of fresh frozen plasma, 3 times

• After exclusion of acute abdomen, cow protein free diet was introduced, but without any improvement.

• Spontaneus desinvagination. • After obtainig coeliac serology, gluten free diet

has started, occurs clinical improvement with metabolic stabilisation.

Conclusion

• Case of rare but serious clinical presentation of celiac crisis.

• It is important to recognize that CD may present in “crisis.”

• The possible precipitating factors in present patient are unrecognized coeliac disease, hypokalemia and previous infection.

Discussion

• Incidence of celiac disease is on rise in Montenegro. • Prevalence of CD is found to be........ dopuniti

ukoliko postoje podaci.....• Celiac crisis is a life-threatening complication of CD. • Clinically, it is characterized by severe diarrhea,

dehydration and metabolic disturbances like hypokalemia, hypomagnesemia, hypocalcemia, hypoproteinemia and metabolic acidosis.

Definition of celiac crisis

• Acute onset or rapid progression of gastrointestinal symptoms attributable to celiac disease requiring hospitalization and/or parenteral nutrition along with at least 2 of the following:

Signs of severe dehydration including: hemodynamic instability and/or orthostatic changesNeurologic dysfunction

Renal dysfunction: creatinine >2.0 g/dL

Metabolic acidosis: pH <7.35

Hypoproteinemia (Albumin < 3.0 g/dL)

Abnormal electrolytes including: hyper/hyponatremia, hypocalcemia, hypokalemia or hypomagnesemiaWeight loss > 10 lbs

Metabolic pathophysiology in celiac crisis

• Celiac crisis may not respond to a gluten-free diet alone. In severely ill children with celiac crisis, the use of corticosteroids may cause dramatic improvement. *

• Lloyd-Still described 3 cases of celiac crisis successfully treated with corticosteroids. **

• The role of steroids now is controversial as gluten free and good nutritional diet are considered good enough to tide over the crisis ***

* Mihailidi E, Paspalaki P, Katakis E, Evangeliou A. Celiac Disease: A Pediatric Perspective. International Pediatrics 2003;18:141-8.** Lloyd-Still JD, Grand RJ, Khaw KT, Shwachman H. The use of corticosteroids in celiac crisis. J Pediatr. 1972; 81: 1074-1081.*** Walia A, Thapa BR. Celiac crisis. Indian Pediatr. 2005; 42: 1169

Grazie per l'attenzione

Saluti da Montenegro