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CASE REPORT: IGA VASCULITIS WITH RESISTANT GASTROINTESTINAL FINDINGS.

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Introduction: IgA vasculitis is a systemic small vessel vasculitis accompanied by gastrointestinal tract (GIT) and renal involvement. In these patients, abdominal pain accompanied by purpura is a major presentation at the time of admission.

Case report: An 11-year-old male patient was admitted to the hospital because of severe abdominal pain and purpura involving the buttocks and lower extremities. He was diagnosed as IgA vasculitis with severe GIT involvement. In laboratory evaluation, he had normal levels of hemoglobin (13.4 gr/dL) and hematocrit (%38) red blood cell 5.11 106 /mm3, leukocyte count 6900 /mm3, thrombocyte count 213000 /mm3, and ESR level 9mm/h. CRP was elevated as 22.1 mg/L (N: 0-5 mg/L). He had normal levels of PT, aPTT and INR, and normal urinalysis. The patient with severe abdominal pain was admitted to the hospital. Pediatric surgery consultation with abdominal ultrasonography showed no invagination and surgical pathology was not considered. Oral prednisolone 1 mg/kg/day was administered; however, the response to the treatment was not received. Resistant GIT involvement was considered, IV pulse methylprednisolone of 30 mg/kg/day was given consecutively for 3 days. Further investigations were planned and endoscopic biopsy revealed Helicobacter pylori infection, which was thought to worsen the prognosis. Factor 13 level was normal. Regarding the association of resistant IgA vasculitis with FMF, MEFV analysis resulted in M694V/R202Q compound heterozygosity. H. pylori eradication therapy together with daily colchicine for FMF resulted in complete remission.

Conclusion: IgA vasculitis is often accompanied by severe gastrointestinal symptoms. One should emphasize the association of FMF as a monogenic autoinfammatory disease, and coexistent H. pylori infection as contributing factors of resistant severe gastrointestinal involvement.

Ceyhun Acari (1), Ozge Altug Gucenmez (1), Betul Aksoy (2), Sevket Erbil Unsal (1)

(1) Department of Pediatrics, Division of Pediatric Rheumatology, Dokuz Eylul University School of Medicine, Izmir, Turkey

(2) Department of Pediatrics, Division of Pediatric Gastroenterology and Hepatology, Dokuz Eylul University School of Medicine, Izmir, Turkey

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Title Annotation:Poster Presentation Abstracts
Author:Acari, Ceyhun; Gucenmez, Ozge Altug; Aksoy, Betul; Unsal, Sevket Erbil
Publication:Erciyes Medical Journal
Article Type:Case study
Date:Mar 1, 2017
Words:328
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