BILIRUBIN OXIDASE (BOX) FEEDINGS TO DELAY OR ELIMINATE THE NEED FOR EXCHANGE TRANSFUSION (EX) IN A FULL TERM G6PD DEFICIENT INFANT. 1299

In spite of intensive photoRx (irradiance ≈35 uW per cm²), serum bilirubin (SB) exceeded 20 mg/dl for 12 hours in a 2 day old G6PD deficient term infant with suspected sepsis and a 9.4% reticulocyte count. Parents requested a trial of BOX (IND) to delay EX until father's blood could be used. BOX (60 Amano units per kg/day) was started at age 75 hours with q3h formula feedings. Stools were collected for assay of bile pigments and BOX activity. Stool bilirubin and bilirubin mono and diglucuronide (B, BMG, BDG) decreased dramatically after 19 hours of BOX and remained low until 21 to 36 hours after stopping BOX, in parallel with enzyme activity levels in the stools (seegraph). Decreased enterohepatic circulation of bilirubin 2° to its oxidation in the gut by BOX may have contributed to the faster than expected fall in SB. No EX was done; no BOX side effects were noted. BAER was normal. We conclude that oral BOX is potentially safe, well tolerated and useful for Rx of neonatal jaundice. We suggest testing in a phase I clinical trial.

Figure 1