idiopathic pulmonary hemosiderosis


Also found in: Acronyms.

id·i·o·path·ic pul·mo·nar·y he·mo·sid·er·o·sis

a rare, generally fatal disease of unknown cause, typically affecting small children; characterized by infiltration of pulmonary alveoli with hemosiderin-containing macrophages progressing to diffuse pulmonary fibrosis. Clinical features include recurrent pulmonary hemorrhages, anemia, dyspnea, and cardiorespiratory failure.

idiopathic pulmonary hemosiderosis

Clinical immunology A rare possibly autoimmune condition affecting children < age 10 characterized by hemosiderin deposition in lungs Clinical Recurrent hemoptysis, anemia, weakness, clubbing, hepatosplenomegaly Prognosis Poor, recurrent episodes; death often in 5 yrs due to cor pulmonale; it may be associated with celiac disease and improve with a gluten-free diet. See Hemosiderosis.

Ceelen,

Wilhelm, 1884-1964.
Ceelen-Gellerstedt syndrome - repeated sudden attacks of dyspnea and hemoptysis leading to diffuse pulmonary hemosiderosis. Synonym(s): idiopathic pulmonary hemosiderosis
References in periodicals archive ?
Long- term clinical course of patients with Idiopathic Pulmonary Hemosiderosis (1979-1994): prolonged survival with low dose corticosteroid therapy.
Inhaled corticosteroids in idiopathic pulmonary hemosiderosis: 2 cases.
Recurrence of Idiopathic pulmonary hemosiderosis in a young adult patient after bilateral single lung transplantation.
Eight Years Follow-up of a Case with Idiopathic Pulmonary Hemosiderosis After Corticosteroid Therapy.
He was the lead author on the article being discussed here, "Idiopathic Pulmonary Hemosiderosis Presenting as a Rare Cause of Iron Deficiency Anemia in a Toddler--A Diagnostic Challenge."
Idiopathic Pulmonary Hemosiderosis. J Pediatr Sci 2010; 6: 56.
Derbent M, Ozcay F, Saatci U, Ozbek N.Severe iron deficiency anemia in a child with idiopathic pulmonary hemosiderosis: a case report.

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