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[特发性肺含铁血黄素沉着症、乳糜泻与心肌病]

[Idiopathic pulmonary hemosiderosis, celiac disease and cardiomyopathy].

作者信息

Yacoub M, Mahjoub H, Abroug S, Bousnina M, Harbi A, Essoussi A S

机构信息

Services de pédiatrie A et B, CHU Farhat Hached, Sousse, Tunisie.

出版信息

Arch Pediatr. 1994 Jun;1(6):587-90.

PMID:7994352
Abstract

UNLABELLED

BACKGROUND--Idiopathic pulmonary hemosiderosis (IPH), a rare and possibly immune disease, is sometimes associated with coeliac disease and myocardiopathy.

CASE REPORTS

CASE NO 1--A 2 year-old boy with IPH was investigated because he suffered from frequent, soft stools. Small bowel biopsy showed partial villous atrophy. Circulating gliadin antibodies were present. The patient was placed on a gluten-free diet. CASE NO 2--An 8 year-old girl was admitted because she suffered from severe anemia (Hb: 4 g/100 ml). She was found to have IPH and myocardiopathy. She had no manifestation, but a systematic search for coeliac disease was positive (total villous atrophy; presence of circulating gliadin and alveolar basement membrane antibodies). The patient was placed on a gluten-free diet, prednisone and diuretics, but she died during a relapse 2 months later. CONCLUSION--It is worthwhile checking for coeliac disease in all patients with IPH. The presence of myocardiopathy is a negative prognosis.

摘要

未标注

背景——特发性肺含铁血黄素沉着症(IPH)是一种罕见的可能为免疫性的疾病,有时与乳糜泻和心肌病相关。

病例报告

病例1——一名患有IPH的2岁男孩因频繁排软便接受检查。小肠活检显示部分绒毛萎缩。存在循环麦醇溶蛋白抗体。该患者接受无麸质饮食。病例2——一名8岁女孩因严重贫血(血红蛋白:4g/100ml)入院。她被发现患有IPH和心肌病。她没有相关表现,但对乳糜泻进行的系统检查呈阳性(全绒毛萎缩;存在循环麦醇溶蛋白和肺泡基底膜抗体)。该患者接受无麸质饮食、泼尼松和利尿剂治疗,但2个月后在复发期间死亡。结论——对所有IPH患者检查是否患有乳糜泻是值得的。心肌病的存在预后不良。

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