Skip Navigation LinksHome > June 2004 - Volume 39 - Issue > P1186 PROTEIN LOSING ENTEROPATHY WITH AGAMMAGLOBULINAEMIA
Journal of Pediatric Gastroenterology & Nutrition:
ABSTRACTS: Poster Session Abstracts

P1186 PROTEIN LOSING ENTEROPATHY WITH AGAMMAGLOBULINAEMIA

Vass, N.1; Kovács, J.1; Bereczki, C.1; Várkonyi, Á.1; Megyeri, P.1

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1Department of Pediatrics, University of Szeged, Szeged, Hungary

Submitted by: vassnoemi@freemail.hu

Introduction: History:A 6 month-old well developing (Pw:50–75%, Ph:50%) infant had a scarlet fever in March 2002. She recovered after a 10 days of Penicillin therapy. She was admitted to the Intensive Care Unit in May 2002 because of an acute generalised oedema with the suspicion of nephrosis syndrome.

Methods: Examinations: Serumprotein: 30 g/L; albumin:16g/L. There were no measurable protein in the urine samples, and the protein excretion in 24-hr collected urine is 0.1 g/die. Microbiological examinations in both the urine and stool were negative. These results excluded the possibility of nephrosis syndrome. Neither right heart failure, nor pericarditis could be verified. The serious hypoproteinaemia of this well fed infant was suspicious for a protein losing entheropathy. Gastrointestinal passage, small bowell biopsy, chest and abdominal CT, and several microbiological investigations of stool samples were carried out. Intestinal lymphangiectasia of the small bowell was verified by the upper passage examination and a small bowel biopsy. A serious protein losing was apparent at the large intestine after 99mTc-marked albumin infusion. At the 25th day of her treatment, she had an acute diarrhoea, and Clostridium difficile infection was diagnosed. At this time the protein levels in her serum were: 27g/L, albumin:15 g/L, IgG: 0.08 g/L, IgA: 0.09 g/L, IgM:0.34 g/L.

Results: Therapy: Sustitution therapy with parenteral albumin (5ml/kg, 5% albumin, 5 times) and gammaglobulin (IVIG 400 mg/kg, 4 times) were given. Biosorbin MCT was used in her nutrition.

Conclusion: Outcome:After 6 weeks of treatment she left our department without any symptoms. One year later she was healthy with normal laboratory results. A Clostridium difficile infection was presumed in the background of her disease and the IVIG substitution prevented the secondary infections.

© 2004 Lippincott Williams & Wilkins, Inc.

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