OUTCOME OF IDIOPATHIC PULMONARY HEMOSIDER OSIS IN PEDIATRIC-PATIENTS - A REVIEW OF 8 CASES

Idiopathic pulmonary hemosiderosis is characterized by alveolar hemorr hage with secondary pulmonary fibrosis, Outcomes in eight retrospectiv ely studied patients seen over a 12-year period In two hospital depart ments (pediatric pneumology and pediatrics) are reported. Tests for im munoallergy and an intestinal biopsy were done in survivors, Mean foll ow-up was six and a half years, Two patients died, two developed chron ic respiratory failure, and one developed a restrictive ventilatory de fect. Four patients had an extrapulmonary manifestation (one case each of vitiligo, cardiomyopathy, endocapillary glomerulonephritis, and ce liac disease). The unpredictable but often grim prognosis of pediatric idiopathic pulmonary hemosiderosis warrants extensive investigations for a cause.