Long-term clinical course of patients with idiopathic pulmonary hemosiderosis (1979-1994): Prolonged survival with low-dose corticosteroid therapy

Idiopathic pulmonary hemosiderosis (IPH) is a rare disease of unknown etiology characterized by recurrent episodes of pulmonary symptoms such as cough, hemoptysis, and dyspnea. Our study consisted of 23 patients: 12 males and 11 females with IPH. The diagnosis was based on history, presence of anemi...

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Published in:	Pediatric pulmonology Vol. 27; no. 3; pp. 180 - 184
Main Authors:	Kiper, Nural, Göçmen, Ayhan, Özçelik, Uger, Dilber, Embiya, Anadol, Deniz
Format:	Journal Article
Language:	English
Published:	New York John Wiley & Sons, Inc 01-03-1999 Wiley-Liss
Subjects:	Adolescent Biological and medical sciences Bones, joints and connective tissue. Antiinflammatory agents budesonide Child Child, Preschool children consanguinity corticosteroid Dose-Response Relationship, Drug Female Glucocorticoids - therapeutic use Hemosiderosis - diagnosis Hemosiderosis - drug therapy Hemosiderosis - mortality Humans Infant Long-Term Care Lung Diseases - diagnosis Lung Diseases - drug therapy Lung Diseases - mortality Male Medical sciences Pharmacology. Drug treatments Prednisolone - therapeutic use Prognosis pulmonary hemosiderosis Spirometry Survival Rate Treatment Outcome Human Corticosteroid Prognosis Radiodiagnosis Respiratory disease Low dose Lung Antiinflammatory agent Idiopathic Metabolic diseases Long term Survival Hemosiderosis Radiography Chemotherapy Treatment Pigments Child
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Summary:	Idiopathic pulmonary hemosiderosis (IPH) is a rare disease of unknown etiology characterized by recurrent episodes of pulmonary symptoms such as cough, hemoptysis, and dyspnea. Our study consisted of 23 patients: 12 males and 11 females with IPH. The diagnosis was based on history, presence of anemia, and characteristic chest X‐ray, and was confirmed by showing macrophages laden with hemosiderin in gastric washings or bronchoalveolar lavage and/or open lung biopsy. All but one patient were diagnosed in our department between 1979–1994. There was a history of multiple blood transfusions for anemia in 10 patients. Consanguinity between parents was noted in 11 patients. Severe pallor, cough, hemoptysis, and hepatomegaly were the most common findings on physical examination. All but 2 patients had hypochromic microcytic anemia of varying severity. In 12 children, moderate reticulocytosis was noted. Corticosteroids were administered with doses ranging from 5 mg every other day to 2 mg/kg/day depending on the severity of the episodes (duration of disease from 2–14 years). It is our impression that patients with IPH, benefit from long‐term steroid treatment which in turn results in a milder course. Long‐term low‐dose steroid treatment appeared to prevent crises and assured a prolonged survival. Pediatr Pulmonol. 1999; 27:180–184. © 1999 Wiley‐Liss, Inc.
Bibliography:	ArticleID:PPUL5 istex:08A5D6DB9B83F1930D56EFAC956B581A422E3074 This study was presented in poster form at the Second International Congress on Pediatric Pulmonology, June 3-6, 1996, in Nice, France ark:/67375/WNG-9FV6SC06-7 This study was presented in poster form at the Second International Congress on Pediatric Pulmonology, June 3–6, 1996, in Nice, France ObjectType-Article-2 SourceType-Scholarly Journals-1 ObjectType-Feature-1 content type line 23
ISSN:	8755-6863 1099-0496
DOI:	10.1002/(SICI)1099-0496(199903)27:3<180::AID-PPUL5>3.0.CO;2-8