We report a case of 2.8 kg term female baby born of 3rd degree consanguineous marriage referred at 22 hrs of life with complaints of respiratory distress and poor feeding. Elder sibling was a female who died at 30 days of life with complaints of respiratory distress, poor feeding and shock. At admission baby was in shock. Baby was ventilated and started on inotropes. In view of persistent hypoxemia despite 100% Fio2, baby was started on inhaled nitric oxide therapy. Liver function test, CBC, Renal function test, Electrolytes were normal. Chest X ray was normal. Baby was euglycemic. There was a progressive fall in arterial PH from 7.352 at admission to 6.886 with base excess increasing from -5 to -20.1. Baby was given IV bicarbonate infusion. Despite this baby had 3 episodes of cardiac arrest within 15 hrs of admission and baby died at 38 hrs of life.
