Case 6: Liver Failure and Rash in a 6-week-old Girl

Presentation

A 6-week-old girl is admitted to the NICU with liver dysfunction and jaundice. One week before presentation she had developed an erythematous macular rash on the chest and extremities, oral thrush, and a low-grade fever. Physical examination reveals jaundice, hepatosplenomegaly, and a desquamating macular rash on the left lower extremity. Her vital signs are normal for age. She was born to a 34-year-old G7P2321 woman via normal spontaneous vaginal delivery at 37 weeks' gestation. Birthweight was 3,060 g (35th percentile). The infant’s mother had routine prenatal care, and her course was complicated by intrahepatic cholestasis of pregnancy. Results of first-trimester maternal Treponema pallidum enzyme immunoassay were negative.

Laboratory evaluation reveals acute liver failure, coagulopathy, conjugated hyperbilirubinemia, lymphocyte-predominant leukocytosis, disseminated intravascular coagulation, anemia, and thrombocytopenia. Laboratory test results are notable for hemoglobin level, 7.2 g/dL (72 g/L); total leukocyte count, 25,000/μL (25.0 × 10⁹/L) with 68% lymphocytes and 18% neutrophils; platelet count, 20 × 10³/μL (20 × 10⁹/L); aspartate aminotransferase, 1,164 U/L (19.4 μkat/L); alanine aminotransferase, 536 U/L (8.9 μkat/L); albumin, 1.8 g/dL (18 g/L); international normalized ratio, 1.8; and partial thromboplastin time, 34.9 seconds. Her C-reactive protein level was elevated at 19.1 mg/L (181.9 nmol/L).