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Journal of the Korean Society of Pediatric Nephrology 2007;11(1): 106-111. doi: https://doi.org/10.3339/jkspn.2007.11.1.106
신염-신증후군과 소장 천공을 동반한 $Henoch-Schddot{o}nlein$ 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 $Henoch-Schddot{o}nlein$ 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
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ABSTRACT
[ $Henoch-Schddot{o}nlein$ ] 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 | $Henoch-Schddot{o}nlein$ | Nephritis | Intestinal perforation
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