pulmonary alveolar proteinosis in children: a case series
نویسندگان
چکیده
pulmonary alveolar proteinosis, (pap) is a rare disease of unknown etiology, characterized by accumulation of intra-alveolar proteinaceous material which is rich in lipid and positive on periodic acid-schiff stain. two clinically different pediatric types have been defined as congenital pap which is fulminant and fatal, and a late-onset pap which is similar to the adult form and less severe. eight children with late-onset pap were hospitalized from 1998 to 2005 in mofid children hospital. characteristics of these patients and the methods of diagnosis and treatment are presented in this case series. keywords: pulmonary alveolar proteinosis, respiratory failure, respiratory distress, children. normal 0 false false false microsoftinternetexplorer4
منابع مشابه
Pulmonary alveolar proteinosis in children: a case series
Pulmonary alveolar proteinosis, (PAP) is a rare disease of unknown etiology, characterized by accumulation of intraalveolar proteinaceous material which is rich in lipid and positive on periodic acid-Schiff stain. Two clinically different pediatric types have been defined as congenital PAP which is fulminant and fatal, and a late-onset PAP which is similar to the adult form and less severe. Eig...
متن کامل[A case of pulmonary alveolar proteinosis].
概要 症例例,47才,男 性,教 員.主 訴 は,労 作時息切れ,咳 殿,喀 痰.28才 頃 より慢性気管支炎 と 言われていたが,最 近息切 れが増強 し受診 した.理 学 的所見では,胸 部で両下肺野後面に少数 の捻髪 音が聴 かれ,軽 度のばち状指趾が認め られた.胸 藻写真 で例,差 右両下肺野 に淡い微細粒状影 を,呼 吸機能検査 で例,拘 束性障害 と拡散能力の低 下を,動 脈血 ガス分析では,低 酸素血症 と過換気 の状態 を認 めた 。開胸肺生検に より組織学的に肺胞蛋 白症 と診断 された.L-シ ス チン内服で若 干の改善が認 め られ たが,Ramirez-Rら の方法 に準 じて病変の高度な左肺 の肺洗浄を施行 し,自 覚的 および 他覚的 症状 例著明に改筈 された.肺 洗浄液 の分析 で例,蛋 白 と燐脂質は正常 であ り,燐 脂質で例 レシチ ンと スフ...
متن کاملPulmonary Alveolar Proteinosis
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متن کاملPulmonary alveolar proteinosis.
Pulmonary alveolar proteinosis (PAP) is a rare pulmonary disease characterised by alveolar accumulation of surfactant. It may result from mutations in surfactant proteins or granulocyte macrophage-colony stimulating factor (GM-CSF) receptor genes, it may be secondary to toxic inhalation or haematological disorders, or it may be auto-immune, with anti-GM-CSF antibodies blocking activation of alv...
متن کاملPulmonary alveolar proteinosis.
Pulmonary alveolar proteinosis is a rare cause of respiratory distress in neonates. We present a 4 month old infant who presented with progressive respiratory distress since birth and failure to thrive. He was initially treated as a case of diffuse alveolar disease but on open lung biopsy was diagnosed as pulmonary alveolar proteinosis. The child expired at 7 months of age.
متن کاملPrimary pulmonary alveolar proteinosis.
INTRODUCTION Pulmonary alveolar proteinosis is an uncommon disease characterized by the accumulation of surfactant proteins and phospholipids within the alveolar spaces. Acquired disease can be idiopathic (primary) and secondary. The prevalence of acquired pulmonary alveolar proteinosis is about 0.37 per 100,000 persons. Common symptoms are dyspnea and cough. Chest X-ray shows bilateral perihil...
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عنوان ژورنال:
journal of research in medical sciencesجلد ۱۵، شماره ۲، صفحات ۱۲۰-۰
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