Newborn Girl with Massive Hepatomegaly, Anemia, and Thrombocytopenia

CLINICAL PRESENTATION A 36-week-old newborn female was delivered via an emergency cesarean section secondary to severe maternal preeclampsia nd nonreassuring fetal heart tracings at a level II hospital center. The mother was 29 years old, with a history of a spontaneous bortion and an ectopic pregnancy. Routine prenatal screening for syphilis, hepatitis, and human immunodeficiency virus were egative. The -fetoprotein level at 16 weeks’ gestation and a level II fetal sonogram were normal. Her pregnancy was omplicated by a febrile illness 2 weeks before delivery and pregnancy-induced hypertension, for which she was treated with agnesium sulfate. At delivery, the infant had respiratory distress, and a distended abdomen was noted. The Apgar scores were 8 and 9 at 1 nd 5 minutes, respectively. The baby was admitted to the special care unit for respiratory distress. On initial evaluation after irth, the baby’s vital signs consisted of a temperature of 99.2°F, respiratory rate of 34 breaths per minute, heart rate of 141 beats er minute, and blood pressure of 61/24 mm Hg, with a mean arterial pressure of 36 mm Hg. The birth weight was 2555 g. he was breathing with mild subcostal retractions, and her breath sounds were coarse to auscultation. A II/VI systolic ejection urmur was noted. The abdomen was distended with a palpable firm mass which occupied the entire area. The remainder of er physical examination was otherwise unremarkable with no dysmorphic features, neurologic abnormalities, skin lesions, rash, r edema of the extremities. On admission, the baby required 35% O2 via a nasal canula at 1.5 L/min to maintain normal O2 saturations. Chest adiography revealed clear lung fields and normal heart size. The baby was started empirically on ampicillin and cefotaxime after blood culture was sent. She was maintained nil per os, receiving intravenous fluids only. Echocardiography demonstrated ormal heart function and a small patent ductus arteriosus. Abdominal radiography was abnormal only for hepatomegaly. Initial laboratory findings included a white blood cell count of 17,000/mm (5,000 to 21,000), with 19% neutrophils (32 o 62), 30% bands (11 to 18), and 43% lymphocytes (40 to 50). The baby was anemic, with a hematocrit of 30% (45 to 65) and hrombocytopenic with a platelet count of 55,000/mm (200,000 to 400,000). Serum electrolytes were normal, but the baby equired intravenous fluids containing 18% dextrose to maintain her glucose level of 56 mg/dL. The blood urea nitrogen was 2 mg/dL (4 to 15) and creatinine level was 1.4 mg/dL (0.3 to 0.6). Her liver function ests were as follows: total protein, 2.2 g/dL (6.3 to 8.2); albumin, 1.7 mg/dL (3.9 to 5), ST (SGOT), 102 U/L (11 to 77); and ALT (SGPT), 21 IU/L (9 to 52). Clotting tudies were prolonged with a prothrombin time (PT) of 150 seconds (10.9 to 13.4), artial thromboplastin time (PTT) of 240 seconds (23.8-32.2), and fibrinogen of 181 g/dL (148 to 376). By the second day of life, the baby was placed on mechanical ventilation for orsening respiratory distress and oxygen requirement. She was transfused with packed ed blood cells (PRBCs), platelets, and fresh-frozen plasma (FFP) for anemia and oagulopathy. Up to this point, the baby urinated only 7 mL in total. Multiple pediatric ubspecialists were consulted, including infectious disease, gastroenterology, and hemaology and oncology. Urine studies for vanillylmandelic acid and homovanillic acid were ecommended but never sent due to her extreme oliguria. The -fetoprotein level was ormal at 37,500 ng/mL. The diagnosis of an enteroviral infection was suspected in light f the maternal history of a febrile illness 2 weeks before delivery. A full sepsis workup was erformed.