PW02-024-B - First report of AA amyloidosis in Blau syndrome
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چکیده
منابع مشابه
PW02-024-B - First report of AA amyloidosis in Blau syndrome
Introduction Systemic AA amyloidosis is a life-threatening complication of different chronic infectious and inflammatory diseases. The deposition of amyloid fibrils derived from the serum amyloid A (SAA) protein represents its pathological hallmark. A long lasting and increased serum level of SAA is a prerequisite to its development. The group of inherited autoinflammatory diseases includes dif...
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Case Report A Japanese girl presented with fever, annular erythematous plagues and elevation of hepatocellular enzyme at 2 months of age. She had deformed ears, a broad saddlelike nose and periorbital edema. At 16 years of age, she had lipodystrophy of the face and upper limbs, a protuberant abdomen, and severe fat deposition into the peritoneal and the pleural cavity. Painful nodular erythema,...
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Churg Strauss syndrome is a rare systemic and pulmonary vasculitis exceptionally associated with AA amyloidosis. We report the case of a 65-year old woman with past medical history of asthma. She developed polyarthralgia, headache and purpura. A laboratory workout found hypereosinophilia (1150/µL), positive p-ANCA, microscopic haematuria and proteinuria at 2g/day. A diagnosis of Churg-Strauss s...
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We report a 13-year-old Indian boy with nephrotic syndrome caused by renal AA amyloidosis. Workup of the AA amyloidosis revealed chronic hepatitis B. Laser microdissection of the Congo-red-positive glomeruli and vessels followed by liquid chromatography and tandem mass spectrometry confirmed the presence of serum amyloid A (SAA) protein and ruled out hereditary and familial amyloidosis. Further...
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Introduction Blau syndrome is a rare autoinflamattory granulomatous disease and inherited as autosomal dominant.The classical triad of Blau syndrome is granulomatous dermatitis, symmetric arthritis and recurrent uveitis. However, all of these findings may not be together in the patients. In the majority of patients, the disease is characterized by early onset that usually before 3-4 years of ag...
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ژورنال
عنوان ژورنال: Pediatric Rheumatology
سال: 2013
ISSN: 1546-0096
DOI: 10.1186/1546-0096-11-s1-a165