Afzal Noureen, Mushtaq Ammara, Rahman Arshalooz, Qureshi Sonia
Department of Paediatrics, Aga Khan Secondary Hospital, Karimabad.
J Pak Med Assoc. 2012 Aug;62(8):845-7.
Idiopathic pulmonary haemosiderosis is an uncommon disorder, which is characterized by iron deficiency, recurrent haemoptysis and diffuse parenchymal infiltration on chest radiograph. We report an 8 year old child who had past history of multiple blood transfusions with diagnosis of iron deficiency anaemia and recurrent chest infection since the age of 21/2 year. At the age of 8 years, the child presented with fever and severe respiratory distress requiring intubation and ventilation. On Chest X-ray, bilateral white out was found and CT scan lung showed diffuse alveolar involvement. Lung biopsy confirmed haemosiderin-laden macrophages. Child was put on steroids and despite severe anaemia (Hb 3.2 g/dl), he showed improvement and survived. To our knowledge, this is the first case of idiopathic pulmonary haemosiderosis reported from Pakistan.
特发性肺含铁血黄素沉着症是一种罕见的疾病，其特征为缺铁、反复咯血以及胸部X线片显示弥漫性实质浸润。我们报告一名8岁儿童，自2岁半起有多次输血史，诊断为缺铁性贫血并反复发生胸部感染。8岁时，该儿童出现发热和严重呼吸窘迫，需要插管和通气。胸部X线检查发现双侧肺野呈白色，肺部CT扫描显示弥漫性肺泡受累。肺活检证实有含铁血黄素巨噬细胞。该儿童接受了类固醇治疗，尽管贫血严重（血红蛋白3.2g/dl），但仍有改善并存活下来。据我们所知，这是巴基斯坦报道的首例特发性肺含铁血黄素沉着症病例。
