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   Recurrent Henoch-Schonlein Purpura in Familial Mediterranean Fever  
   
نویسنده Nickavar A ,Ehsanipour F
منبع Acta Medica Iranica - 2008 - دوره : 46 - شماره : 4 - صفحه:349 -352
چکیده    Familial mediterranean fever (fmf) is a relatively rare disorder, characterized by recurrentself-limited attacks of fever and polyserositis. diagnosis is made by clinical features, gene identificationon chromosome 16 and clinical response to specific treatment. different types of vasculitis have beenreported in fmf. henoch-schِnlein purpura (hsp) is one of them, usually with a benign clinicalcourse. repeated attacks of hsp have been rarely reported in fmf. this is the report of a 7-year-oldgirl who presented initially with recurrent fever and abdominal pain. after the primary diagnosis offmf and appropriate treatment, she experienced two documented repeated attacks of hsp with severerenal involvement (crescentic glomerulonephritis) and protracted abdominal pain in the second one.glomerulonephritis was controlled by methyl-prednisolone pulse therapy plus oral corticosteroid andazathioprine, but abdominal pain was resistant to steroids and revealed completely by intravenousimmunoglobulin (ivig) administration. in conclusion, it is suggested to consider the recurrence of hspin cases with fmf to prevent irreversible renal complications. ivig seems to be a good choice for themanagement of intractable abdominal pain of hsp.
کلیدواژه Familial Mediterranean Fever ,Henoch-SchِNlein Purpura ,Vasculitis ,Crescenticglomerulonephritis ,Intravenous Immunoglobulin
آدرس Iran University Of Medical Sciences, School Of Medicine,, Department Of Pediatrics,, ایران, Iran University Of Medical Sciences, School Of Medicine,, Department Of Pediatrics,, ایران
پست الکترونیکی anickavar@yahoo.com
 
     
   
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