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 Görüntüleme 20
 İndirme 1
AİLEVİ AKDENİZ ATEŞİ VE POLİARTERİTİS NODOZA BİRLİKTELİĞİ OLAN PEDİATRİK BİR OLGU SUNUMU
2008
Dergi:  
Gazi Medical Journal
Yazar:  
Özet:

Ailevi Akdeniz atesi (FMF), tekrarlayan ve sıklıkla kendini sınırlayan ates, se- röz yüzeylerde inflamasyon ve bu nedenle olusan karın, göğüs ve eklem ağrısı ile seyreden otozomal resesif geçis gösteren bir hastalıktır. FMF’li hastalarda, poliarteritis nodoza (PAN) ve Henoch- Schönlein purpurası (HSP) gibi vas- külitik hastalıkların sıklığının arttığı bilinmektedir. Patogenez açık olmamakla birlikte immünkompleks mekanizma ile iliskili olabilir. Bu yazıda karın ağrısı ve ates sikayetiyle basvuran ve FMF tanısı ile kolsisin tedavisi baslandıktan sonra, izlemde kontrol altına alınamayan semptomları ve yüksek akut faz yanıtı nedeni ile eslik eden vaskülitik hastalık düsünülerek yapılan angiografikin- celemede izole hepatik arter anevrizması tespit edilen 9 yasında erkek hasta sunulmaktadır. Bu hasta, FMF ve PAN’ın nadir olan birlikteliğinin yanı sıra, renal ve çölyak arterlerde tutulum olmaksızın izole hepatik arter tutulumunun olabileceği ve immun supresif tedaviye eklenen pulse siklofosfamid tedavisi ile alınan yanıt vurgulamak amacı ile sunulmaktadır. Anahtar Kelimeler: Ailevi Akdeniz Ateşi (FMF), Poliarteritis Nodoza, Hepa- tik Arter Anevrizması.   POLYARTERITIS NODOSA IN A CASE OF FAMILIAL MEDITERRA- NEAN FEVER ABSTRACT Familial Mediterranean fever (FMF) is an autosomal recessive disease, cha- racterized by recurrent and self-limited attacks of fever, usually accompanied by polyserositis. Several vasculitic diseases such as Henoch-Schönlein purpura and polyarteritis nodosa (PAN) have been reported with increased frequency in patients with FMF. Although the pathogenesis is not clearly defined, it has been hypothesized that immune complexes may play a role in the association of the vasculitides and FMF. In this report we describe a 9-year-old boy admitted to our hospital with abdominal pain and fever. FMF was detected and colchicine treatment was given. Despite the treatment, his constitutional symptoms persis- ted. Due to the increased likelihood for the development of a vasculitic process in patients with FMF, angiography was performed because of a suspicion of PAN and angiography showed hepatic artery aneurysms. He responded well to pulse methylprednisolone and pulse cyclophosphamide therapy in addition to colchicine. We emphasize the rare association of FMF and PAN and insulated hepatic artery aneurysmal angiographic signs of PAN. Key Words: Familial Mediterranean Fever (FMF), Polyarterities Nodosa (PAN), Hepatic Artery Aneurysm.

Anahtar Kelimeler:

A Pediatric Fact of Fire and Polyarteritis Nodose Association
2008
Yazar:  
Özet:

Family Mediterranean atheism (FMF) is a repeated and often self-restricted atheism, an inflammation in the se-rush surfaces and, therefore, the resulting stomach, chest and joint pain. In patients with FMF, the frequency of vascular diseases such as polyarthritis nodosis (PAN) and Henoch-Schonlein purple (HSP) is known to increase. Although pathogenesis is not clear, it can be associated with the immune complex mechanism. In this article, after the pain of the abdomen and the atheic scratch and the diagnosis of FMF and the treatment of colsys are printed, the symptoms that are uncontrolled in the monitoring and the cause of high acute phase response, the vascular disease is thought to be angiographic - in the cell, the male patient is presented in the 9th century that is detected with anevrism of the liver artery isolated. This patient is presented in order to emphasize the rare association of FMF and PAN, as well as the insulation of the insulation of the liver artery without adhesion in the renal and thyroid arteries and the response received by pulse cyclophosphamide therapy added to immunosuppressive therapy. Keywords: Family Mediterranean Fever (FMF), Polyarthritis Nodoza, Hepa-tick Arter aneurysm.   Polyarthritis NODOSA IN A CASE OF FAMILIAL MEDITERRA- NEAN FEVER ABSTRACT Family Mediterranean fever (FMF) is an autosomal recessive disease, cha-racterized by recurrent and self-limited attacks of fever, usually accompanied by polyserositis. Several vasculitic diseases such as Henoch-Schönlein purpura and polyarteritis nodosa (PAN) have been with increased frequency in patients with FMF. Although the pathogenesis is not clearly defined, it has been hypothesized that immune complexes may play a role in the association of the vasculitides and FMF. In this report we describe a 9-year-old boy admitted to our hospital with abdominal pain and fever. FMF was detected and colchicine treatment was given. Despite the treatment, his constitutional symptoms persis- ted. Due to the increased likelihood for the development of a vasculitic process in patients with FMF, angiography was performed because of a suspicion of PAN and angiography showed hepatic artery aneurysms. He responded well to pulse methylprednisolone and pulse cyclophosphamide therapy in addition to colchicine. We emphasize the rare association of FMF and PAN and insulated hepatic artery aneurysmal angiographic signs of PAN. Keywords: Familial Mediterranean Fever (FMF), Polyarterities Nodosa (PAN), Hepatic Artery Aneurysm.

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Gazi Medical Journal

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