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儿童特发性肺含铁血黄素沉着症

Idiopathic pulmonary haemosiderosis in childhood.

作者信息

Vaish Hans

机构信息

Pediatrics, Meher Hospital, Dehradun, Uttarakhand, India

出版信息

BMJ Case Rep. 2025 Jul 7;18(7):e265327. doi: 10.1136/bcr-2025-265327.

Abstract

This report describes a case involving a boy in his middle childhood with a history of recurrent iron deficiency anaemia, accompanied by respiratory distress, tachypnoea and cyanosis. The respiratory distress improved with pulse doses of methylprednisolone and respiratory support using high-flow oxygen via heated humidified nasal cannula. During each admission, respiratory distress was resolved after administering pulse methylprednisolone. The child was admitted five times with such episodes in the paediatric intensive care unit (PICU). X-rays and high-resolution CT of the chest performed during respiratory distress showed diffuse ground-glass opacities and multiple areas of consolidation. After excluding sepsis, autoimmune diseases and vasculitis, a bronchoscopic alveolar lavage was conducted, revealing haemosiderin-laden macrophages. Having ruled out other causes of diffuse alveolar haemorrhage, this case was identified as idiopathic pulmonary haemosiderosis (IPH). Following discharge from the PICU and resolution of respiratory distress, the child was started on maintenance immunosuppression with prednisolone at 15 mg/day.

摘要

本报告描述了一例涉及一名童年中期男孩的病例,该男孩有反复缺铁性贫血病史,伴有呼吸窘迫、呼吸急促和发绀。呼吸窘迫通过静脉注射甲基强的松龙以及使用经加热湿化鼻导管的高流量氧气进行呼吸支持后得到改善。每次入院期间,静脉注射甲基强的松龙后呼吸窘迫均得到缓解。该患儿因此类发作在儿科重症监护病房(PICU)住院五次。呼吸窘迫期间进行的胸部X线和高分辨率CT显示弥漫性磨玻璃影和多个实变区域。在排除败血症、自身免疫性疾病和血管炎后,进行了支气管肺泡灌洗,发现含铁血黄素巨噬细胞。在排除弥漫性肺泡出血的其他原因后,该病例被诊断为特发性肺含铁血黄素沉着症(IPH)。从PICU出院且呼吸窘迫缓解后,该患儿开始接受泼尼松龙15毫克/天的维持性免疫抑制治疗。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1581/12231081/7f765b1ef616/bcr-18-7-g001.jpg

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