Kabra S K, Bhargava Sumit, Lodha Rakesh, Satyavani A, Walia M
Pediatric Pulmonology Division, Department of Pediatrics, All India Institute of Medical Sciences, New Delhi 110 029, India.
Indian Pediatr. 2007 May;44(5):333-8.
To describe the clinical details and follow up of children with idiopathic pulmonary hemosiderosis.
Retrospective case series.
Pediatric chest clinic of a tertiary care hospital.
Children diagnosed as suffering from idiopathic pulmonary hemosiderosis (IPH).
Charts of patients diagnosed as IPH were reviewed for clinical features and treatment regimen. Diagnosis was based on presence of iron deficiency anemia, chest radiography and demonstration of hemosiderin laden macrophages in bronchoalveolar lavage (BAL), gastric aspirate, or sputum. Treatment consisted of oral prednisolone, hydroxychloroquine (HCQ) and inhaled corticosteroids (ICS).
The common clinical features in 26 children with IPH (mean age 75 months) included: cough, breathlessness, fever, hemoptysis and wheezing in 26 (100%), 22 (85%), 19 (73%),15 (58%) and 14 (54%) children, respectively. Clubbing, hepatomegaly and splenomegaly was seen in 16 (62%), 15 (58%) and 10 (38%) children, respectively. Hemosiderin laden macrophages were documented in BAL and gastric aspirate in 92% and 30% patients, respectively. Symptoms did not recur in 17 patients who received prednisolone and HCQ initially. 5 patients had recurrence of symptoms and required short courses of oral prednisolone, 4 patients required frequent courses of prednisolone and were started on azathioprine. Older age, longer duration of illness, history of hemoptysis and jaundice were associated with poor response.
Treatment with prednisolone and hydroxychloroquine followed by inhaled corticosteroids may improve survival in children with IPH.
描述特发性肺含铁血黄素沉着症患儿的临床细节及随访情况。
回顾性病例系列研究。
一家三级护理医院的儿科胸部诊所。
诊断为患有特发性肺含铁血黄素沉着症（IPH）的患儿。
查阅诊断为IPH的患者病历，了解其临床特征和治疗方案。诊断基于缺铁性贫血、胸部X线检查以及支气管肺泡灌洗（BAL）、胃抽吸物或痰液中含铁血黄素巨噬细胞的显示。治疗包括口服泼尼松龙、羟氯喹（HCQ）和吸入性糖皮质激素（ICS）。
26例IPH患儿（平均年龄75个月）的常见临床特征包括：咳嗽，26例（100%）；呼吸急促，22例（85%）；发热，19例（73%）；咯血，15例（58%）；喘息，14例（54%）。分别有16例（62%）、15例（58%）和10例（38%）患儿出现杵状指、肝肿大和脾肿大。分别有92%和30%的患者在BAL和胃抽吸物中记录到含铁血黄素巨噬细胞。最初接受泼尼松龙和HCQ治疗的17例患者症状未复发。5例患者症状复发，需要短期口服泼尼松龙治疗，4例患者需要频繁使用泼尼松龙治疗，并开始使用硫唑嘌呤。年龄较大、病程较长、咯血和黄疸病史与反应不佳有关。
泼尼松龙和羟氯喹随后联合吸入性糖皮质激素治疗可能改善IPH患儿的生存率。
