Severe Nephritic-nephrotic Syndrome with Small Bowel Perforation in a Child with Henoch-Schönlein Purpura

Kim, Gun-Ha; Shin, Hye-Kyung; Yim, Hyung-Eun; Hong, Young-Sook; Lee, Joo-Won; Won, Nam-Hee; Yoo, Kee-Hwan

doi:2007.11.1.106

J Korean Soc Pediatr Nephrol > Volume 11(1); 2007 > Article

J Korean Soc Pediatr Nephrol 2007;11(1): 106-111. doi: https://doi.org/10.3339/jkspn.2007.11.1.106

신염-신증후군과 소장 천공을 동반한 Henoch-Schönlein 자반증 1례

김건하, 신혜경, 임형은, 홍영숙, 이주원, 원남희, 유기환

¹고려대학교 의과대학 소아과학교실
²고려대학교 의과대학 소아과학교실
³고려대학교 의과대학 소아과학교실
⁴고려대학교 의과대학 소아과학교실
⁵고려대학교 의과대학 소아과학교실
⁶고려대학교 의과대학 병리학교실
⁷고려대학교 의과대학 소아과학교실

Severe Nephritic-nephrotic Syndrome with Small Bowel Perforation in a Child with Henoch-Schönlein Purpura

Gun-Ha Kim, Hye-Kyung Shin, Hyung-Eun Yim, Young-Sook Hong, Joo-Won Lee, Nam-Hee Won, Kee-Hwan Yoo

¹Department of Pediatrics, College of Medicine Korea University
²Department of Pediatrics, College of Medicine Korea University
³Department of Pediatrics, College of Medicine Korea University
⁴Department of Pediatrics, College of Medicine Korea University
⁵Department of Pediatrics, College of Medicine Korea University
⁶Department of Pathology, College of Medicine Korea University
⁷Department of Pediatrics, College of Medicine Korea University

ABSTRACT

[ Henoch-Schönlein ] Purpura(HSP) is a form of vasculitis that typically affects small arteries in the skin, joints, intestinal tract and kidneys. It usually resolves spontaneously but sometimes can cause serious problems in the kidneys and intestinal tract. A 6-year-old girl with purpura, arthralgia and abdominal pain for 2 weeks was admitted. She also showed gross hematuria, generalized edema and decreased urine output. Blood pressure was in the upper normal range. Initial laboratory findings showed hypoalbuminemia, hyperlipidemia, microhematuria and nephrotic-range proteinuria(27.2 g/day). Initially, she was treated with pulse methylprednisolone, azathioprine, albumin and furosemide. Her renal biopsy revealed diffuse mesangial proliferation with strong IgA deposition. There were no crescents. On the third hospital day, she complained of severe abdominal pain and free peritoneal air was seen on abdominal X-ray. Primary repair of small bowel was performed and two pin-point sized holes were found. One week later, she still showed heavy proteinuria. Therefore, we added an ACE inhibitor and dipyridamole, and changed azathioprine to cyclosporine. One month later, the urine protein/creatinine ratio was decreased to 17.8 from 57, but heavy proteinuria has been still persisted. Here we report a rare case of a patient with HSP who had both severe nephrritc-nephrotic syndrome and small bowel perforation.

Key words: Purpura | Henoch-Schönlein | Nephritis | Intestinal perforation