VARICELLA AND VARICELLA IMMUNITY IN PATIENTS WITH LYSINURIC PROTEIN INTOLERANCE

Two patients with lysinuric protein intolerance (LPI) had near-fatal g eneralized varicella infection with severe interstitial pneumonitis, h epatitis, decreased platelet count, bleeding and hypoalbuminaemia. Act ive haemolysis resulted in anaemia and massive haemoglobinuria. Serum lactate dehydrogenase activity and ferritin concentration, which in pa tients with LPI in normal circumstances exceed the upper reference val ues 3-fold to 10-fold, increased to > 10000 U/L and > 10000 mu g/L, re spectively. The patients were treated with fresh frozen plasma, red-ce ll transfusions and intravenous acyclovir for 14 days, and recovered c linically in a month. Retrospectively, 3 of the 32 other known Finnish patients with LPI had had varicella infection that had been more seve re than that in the other children in the family or in subjects in the neighbourhood and had led to hospital admission. Varicella antibodies were measured in 24 patients; 5 had no antibodies and 5 had very low antibody titres. Primary vaccination of three patients with living var icella vaccine increased antibody titres measurably in one patient. We suggest that patients with LPI who have no varicella tester antibodie s should be treated with acyclovir if exposed to varicella and should be (re)vaccinated against chickenpox.