Two pediatric Henoch-Schönlein purpura cases whose severe gastrointestinal hemorrhage treated by cyclophosphamide

Henoch-Schönlein purpura (HSP) is a vasculitis, that in­volves various organ systems and show different clinical picture. It can be presented with purpura-like skin rash, abdominal pain, arthritis and renal involvement. Rarely, severe skin, gastrointestinal (GIS) and renal involvement occur and can...

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Bibliographic Details
Published in:Dicle tıp dergisi 2013-06
Main Authors: Ece, Aydın, Yavuz, Sevgi, Tan, İlhan, Uluca, Ünal, Karabel, Duran
Format: Article
Language:Turkish
Subjects:
Henoch-Schönlein purpura, severe system involvement, cyclophosphamide, management
Henoch-Schönlein purpurası, ağır sistem tutulumu, siklofosfamid, tedavi
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Summary:Henoch-Schönlein purpura (HSP) is a vasculitis, that in­volves various organ systems and show different clinical picture. It can be presented with purpura-like skin rash, abdominal pain, arthritis and renal involvement. Rarely, severe skin, gastrointestinal (GIS) and renal involvement occur and can lead to early or late complications. In this paper, it was aimed to report, two HSP children with se­vere GIS involvement as recurrent intestinal hemorrhage, which did not respond to high dose intravenous steroids, but successfully treated by intravenous cyclophospha­mide. One of our patients had also severe necrotic skin lesions that necessitate skin grafting and the other had severe renal involvement as nephrotic syndrome. Intra­venous cyclophosphamide may be useful in children with HSP, when severe GIS involvement not respond to high dose steroids. Henoch-Schönlein purpurası (HSP) çeşitli sistemleri tu­tan ve farklı klinik seyirler gösteren bir vaskülittir. Purpura tarzında döküntü, karın ağrısı, artrit, böbrek tutulumu ile ortaya çıkabilmektedir. Nadiren, deri, gastrointestinal sis­tem (GİS) ve böbrek tutulumu ağır seyretmekte ve erken veya geç dönemde çeşitli komplikasyonlara yol açabil­mektedir. Bu yazıda yüksek doz intravenöz steroid teda­visine yanıt vermeyen tekrarlayan GİS kanaması intrave­nöz siklofosfamid ile başarılı bir şekilde tedavi edilen ağır GİS tutulumlu iki HSP\'li çocuk olgunun sunulması amaç­landı. Hastalarımızdan birinde GİS tutulumuna ilaveten deri grefti gerektirecek kadar ağır nekrotik cilt tutulumu, diğerinde ise nefrotik proteinüri ile seyreden böbrek tu­tulumu mevcuttu. HSP\'li çocuklarda yüksek doz steroide yanıt alınamayan ağır GİS tutulumunda intravenöz siklo­fosfamid yararlı olabilir.
ISSN:1308-9889
1300-2945