Jaundice and bleeding from peripheral intravenous sites in a neonate. Neonatal hemochromatosis (idiopathic neonatal iron-storage disease).

Abstract

REPORT OF A CASE A 2.4-kg boy of 39 weeks' gestational age was born to a 27-year-old grava 3, para 1, abortus 1 white woman. The antenatal period was uncomplicated. At 1 hour of life, he developed bilateral pneumothoraces, which were treated with chest tubes. On the second day of life, he developed jaundice (Figure 1), edema, and oliguria. Bleeding from peripheral intravenous sites was noted. Neurologically, he was normal. Laboratory examination revealed the following values: ferritin, 560 μg/L (reference range, 11-12 μg/L); iron, 29 μmol/L (164 μg/dL) (reference range, 7-25 μmol/L [40-140 μg/dL]); conjugated bilirubin, 144 μmol/L (8.4 mg/dL); prothrombin time, greater than 80 seconds; and partial thromboplastin time, greater than 120 seconds. Hepatic transaminase levels and a complete blood cell count were within normal limits. Because of the patient's coagulopathy, a liver biopsy was contraindicated. A biopsy specimen was obtained from the patient's lower lip (Figure 2and