Long‐term clinical course of patients with idiopathic pulmonary hemosiderosis (1979–1994): Prolonged survival with low‐dose corticosteroid therapy
Identifieur interne : 002657 ( Main/Exploration ); précédent : 002656; suivant : 002658Long‐term clinical course of patients with idiopathic pulmonary hemosiderosis (1979–1994): Prolonged survival with low‐dose corticosteroid therapy
Auteurs : Nural Kiper [Turquie] ; Ayhan Göçmen [Turquie] ; Uger Özçelik [Turquie] ; Embiya Dilber [Turquie] ; Deniz Anadol [Turquie]Source :
- Pediatric Pulmonology [ 8755-6863 ] ; 1999-03.
English descriptors
- Teeft :
- Alternate days, Alveolar basement membrane, Blood transfusions, Celiac disease, Chest diseases, Chloroquine therapy, Common findings, Consanguinity, Correct diagnosis, Corticosteroid, Corticosteroid treatment, Diffuse parenchymal infiltrates, Dyspnea, Etiology, Expiratory volume, Extrapulmonary disease, Gastric washings, Hemoptysis, Hemosiderosis, Idiopathic, Infiltrates, Insidious onset, Iron deficiency anemia, Laboratory findings, Macrophage, Moderate reticulocytosis, Multiple blood transfusions, Normal life, Pediatr, Pediatr pulmonol, Pediatric pulmonology, Physical examination, Pulmonary hemosiderosis, Pulmonary symptoms, Rare disease, Residual volume, Severe pallor, Side effects, Similar symptoms, Steroid, Steroid therapy, Steroid treatment, Symptom, Total lung capacity, Transfusion, Unknown etiology, Vital capacity.
Abstract
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.
Url:
DOI: 10.1002/(SICI)1099-0496(199903)27:3<180::AID-PPUL5>3.0.CO;2-8
Affiliations:
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transfusions</term><term>Normal life</term><term>Pediatr</term><term>Pediatr pulmonol</term><term>Pediatric pulmonology</term><term>Physical examination</term><term>Pulmonary hemosiderosis</term><term>Pulmonary symptoms</term><term>Rare disease</term><term>Residual volume</term><term>Severe pallor</term><term>Side effects</term><term>Similar symptoms</term><term>Steroid</term><term>Steroid therapy</term><term>Steroid treatment</term><term>Symptom</term><term>Total lung capacity</term><term>Transfusion</term><term>Unknown etiology</term><term>Vital capacity</term></keywords></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">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. 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