PMID: 375718Jun 1, 1979Paper

Familial idiopathic pulmonary hemosiderosis

American Journal of Diseases of Children
R C BeckermanJ L Pinnas

Abstract

Two brothers, aged 3 and 6 years, respectively, had their pulmonary conditions diagnosed as idiopathic pulmonary hemosiderosis (IPH). Both boys had severe iron-deficiency anemia, chronic cough, hemoptysis, and exertional dyspnea, and one had recurrent epistaxis. The results of light microscopic lung histopathologic studies in both patients showed numerous hemosiderinladen macrophages and chronic interstitial pneumonitis. No specific patterns of immunofluorescence of the alveolar capillary basement membranes were found. The results of electron microscopic examinations showed intact alveolar and capillary basement membranes and no evidence of electron-dense deposits. The lack of clinical or biochemical evidence for renal disease as well as the absence of serum antinuclear and antibasement membrane antibodies excluded associated autoimmune disorders. Evaluation for milk-protein allergy was negative and neither child demonstrated a clinical response to a milk-free diet. Sequential pulmonary function studies performed over four years showed episodes of acute obstructive airway disease that correlated with pulmonary hemorrhage and mild persistent restrictive lung disease. The results of this family study suggested that some cases of ...Continue Reading

Citations

Aug 2, 2012·Case Reports in Medicine·Argyris TzouvelekisDemosthenes Bouros
Oct 16, 2013·Orphanet Journal of Rare Diseases·Jessica TaytardUNKNOWN French RespiRare® group
Mar 10, 2010·Journal of Tropical Pediatrics·Xiaobo ZhangMingzhi Zhang
Apr 1, 1983·European Journal of Pediatrics·C ChryssanthopoulosC Panagiotidou
Sep 24, 1999·Chest·M M SaeedT G Keens
Oct 27, 2007·Journal of Forensic and Legal Medicine·Homeyra MasoumiHenry F Krous
May 1, 1983·The Journal of Pediatrics·C D CassimosC Panagiotidou
Feb 14, 2006·Paediatric Respiratory Reviews·Thomas G NuessleinChristian H L Rieger
Mar 10, 1999·Respiratory Medicine·N Milman, F M Pedersen
Jul 14, 2005·Revue de pneumologie clinique·S MaalejA Ben Kheder
Jun 10, 2003·Pediatric Critical Care Medicine : a Journal of the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies·M L Cannon, L A Bauman
Oct 4, 2018·The Journal of International Medical Research·Yajun ZhangYuhong Tao
Apr 6, 2020·Current Allergy and Asthma Reports·Mary Buckley, Heather Van Mater

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