J Korean Soc Pediatr Nephrol.  2007 Apr;11(1):106-111.

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

Affiliations
  • 1Department of Pediatrics, College of Medicine Korea University, Seoul, Korea. guroped@korea.ac.kr
  • 2Department of Pathology, College of Medicine Korea University, Seoul, Korea.

Abstract

Henoch-Schonlein 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.

Keyword

Purpura; Henoch-Schonlein; Nephritis; Intestinal perforation

MeSH Terms

Abdominal Pain
Arteries
Arthralgia
Azathioprine
Biopsy
Blood Pressure
Child*
Cyclosporine
Dipyridamole
Edema
Female
Furosemide
Hematuria
Humans
Hyperlipidemias
Hypoalbuminemia
Immunoglobulin A
Intestinal Perforation
Joints
Kidney
Methylprednisolone
Nephritis
Proteinuria
Purpura
Purpura, Schoenlein-Henoch*
Reference Values
Skin
Vasculitis
Azathioprine
Cyclosporine
Dipyridamole
Furosemide
Immunoglobulin A
Methylprednisolone
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