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CASE REPORT

CASE REPORT. Dr Veselinka Djurisic Institute for Children ’s Disease s Montenegro. Female infant, 8 month old; Admitted to hospital due to: Diarrhoea ; Metabolic disbalance; With sings of enteropathy. . History of Present Illness.

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CASE REPORT

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  1. CASE REPORT Dr VeselinkaDjurisic Institute for Children’s Diseases Montenegro

  2. Female infant, 8 month old; • Admitted to hospital due to: • Diarrhoea; • Metabolic disbalance; • With sings of enteropathy.

  3. 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.

  4. 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

  5. Family history • Older brother – convulsions trated with AET; • Father – epilepsy; • Mother – chronic enteropathy in childhood suggested gluten free diet, but she refused; • Grandfather – COPD;

  6. Clinical finding • Weight 7 kg • Agitated, crying, groaning, dehydrated, afebrile (36,7⁰C), hemodynamically stable; • 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.

  7. Laboratory findings

  8. Normal urin dipstick and sediment findings

  9. Tissue transglutaminase antibody: • IgG 269; • IgA > 300; • Anti – gliadin antibodies: • IgG 6 ; • IgA > 300;

  10. Microbiology • Stool culture, ova and parasite testing, Rotavirus and Adenovirus: NEGATIVE. • Stool: positive for Candida sp. • Urin culture: negative.

  11. Radiology findings • X-ray plain film • air-fluid levels

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

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

  14. 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.

  15. COURSE • Treated with antibiotics: • metronidazole • gentamicin • Corticosteroids: • methylprednisolone • H2 blockers: • ranitidine • Transfusion of fresh frozen plasma, 3 times

  16. 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.

  17. 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.

  18. 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.

  19. 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:

  20. Metabolic pathophysiology in celiac crisis

  21. 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

  22. Grazie per l'attenzione Saluti da Montenegro

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