P94 ‘element’ary, my dear watson

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A baby girl was born at 34+5 weeks gestation, weighing 2.5 kg, to non-consanguineous Caucasian parents. Antenatally her mother had prolonged rupture of membrane and Group B Streptococcus on vaginal swab. Her mother received antibiotics during labour. After delivery, the infant had a dose of vitamin K. Two hours later, she was commenced on benzylpenicillin and gentamicin for neonatal sepsis as she was found to be hypotensive. Femoral pulses were present. She received 10 ml/kg fluid bolus and was admitted to neonatal intensive care unit. Two hours later, she became jaundice. Bilirubin level was above the exchange transfusion threshold. Phototherapy was started. Direct Coombs test was negative. At 13 hours of age, she was hypotensive again, with temperature of 38°C. She was bleeding more than usual from her umbilical vessels when lines were inserted. Dopamine was commenced. Cranial ultrasound was unremarkable. Echocardiography showed a small patent ductus arteriosus. Clotting results were deranged, hence, she received further doses of vitamin K, fresh frozen plasma and cryoprecipitate. Albumin was 14 g/L, but liver enzyme levels were normal. She was hypoglycaemic and thrombocytopenic. Her urine output was poor. Creatinine was 169micromol/L and sodium 125 mmol/L. Aciclovir and fluconazole were added. Due to ongoing hypotension, adrenaline, noradrenaline and hydrocortisone were commenced. She was transferred to the liver centre paediatric intensive care unit. She was intubated and ventilated after platelet transfusion. Ultrasound abdomen showed slight heterogeneity of the liver parenchyma. Acute liver failure workup was sent. She was started on vasopressin for ongoing hypotension, 20% dextrose infusion, furosemide infusion, with ongoing need for clotting products. Antibiotics were rationalised to cefotaxime, metronidazole, aciclovir and ambisome. While she was about to have MRI abdomen, she developed pulmonary haemorrhage, requiring tranexemic acid and recombinant Factor VIIa. MRI abdomen was abandoned. Lip biopsy revealed deposition of stainable iron in the salivary gland epithelium, confirming the diagnosis of neonatal haemochromatosis. Liver transplant was considered but was clinically too unstable. She was not suitable for haemofiltration due to ongoing bleeding concerns. Exchange transfusion and intravenous immunoglobulins had minimal effect on her clinical condition. Intensive care was withdrawn and she passed away.

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