The high risk of chronic liver disease in multitransfused juvenile hemophiliac patients.

Eighty-seven asymptomatic children with either hemophilia A or B were treated before they were 21 years of age. Seventy-two received factor concentrates and 15 cryoprecipitate or fresh-frozen plasma only. Thirty-two of the 72 in the former group have persistently elevated alanine aminotransferase values compared to one of 15 in the latter group. In a subset of children treated with factor concentrates before five years of age, four of seven are chronically HBsAg positive. Liver biopsies were performed in 13 of the 32 asymptomatic patients with abnormal ALT values in the fraction group. It is recommended that until further data become available, children with mild hemophilia and all less than 5 years of age should receive only cryo or FFP.     

Urinary phosphate and cyclic AMP excretion following citrate-induced hypocalcemic stimulation of the neonatal parathyroid glands.

Sixteen neonates, ranging in gestational age from 27 to 41 weeks and in postnatal age from birth to 8 days, were evaluated for their renal response to an endogenous PTH stimulus in 22 separate experiments. The PTH stimulus was generated by the decreased serum ionized Ca that accompanies exchange transfusion with citrated blood. The neonates increased their serum PTH from 95.8 +/- 13.1 to 133.9 +/- 15.4 microliterEq/ml (mean +/- SEM) during the transfusion, while increasing their urinary cAMP from 0.77 +/- 0.11 to 1.45 +/- 0.22 nmol/ml, and their urinary P from 12.9 +/- 2.6 to 30.6 +/- 6.1 mg/dl in the four hours following the exchange transfusion. This response was not related to postnatal or gestational age. We speculate that lack of renal responsiveness to PTH does not play a major role in the pathogenesis of early neonatal hypocalcemia.     

Phenobarbital and diphenylhydantoin levels in neonates with seizures

Little information is available regarding appropriate plasma levels of anticonvulsant drugs in neonates. We determined the plasma levels of phenobarbital and diphenylhydantoin following initial administration and during maintenance therapy in 59 neonates with seizures. Following intravenous administration of 15 to 20 mg/kg, levels of 20.7 +/- 4.4 microgram/ml were achieved for phenobarbital and levels of 14.5 +/- 3 microgram/ml for DPH. Maintenance doses of 5 mg/kg of phenobarbital resulted in initial drug accumulation followed by more rapid elimination of the drug with increasing duration of exposure. Therapeutic plasma levels of DPH could not be achieved by oral administration in the neonate.