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The patient was the fourth of affected male siblings. Cortisol (1.3 micrograms per cent), cortisone (9.6), and corticosterone sulfate (0.1) concentrations were low in cord blood. The larger amount of cortisone may have originated from maternal cortisol. Aldosterone was undetectable in cord blood, indicating lack of fetal secretion or maternofetal transfer. Unexpectedly normal concentrations of 11-deoxycorticosterone (DOC) sulfate in cord serum could represent maternal transfer of DOC, with subsequent fetal sulfurylation. Low estrone and estradiol concentrations in maternal and cord serum were consistent with absence of the fetal adrenals. Despite the low levels of the steroids, the propositus had a normal lecithin-sphingomyelin ratio at 38 weeks' gestation. Circulatory insufficiency developed within half an hour after birth and responded to gluco- and mineralocorticoid therapy. The three untreated siblings died between 14 and 67 hours of age. It is evident that early recognition of this condition may be lifesaving.

The postnatal rate of rise of the indirect bilirubin concentration was compared with cord hematocrit and bilirubin values in 44 newborn infants with Rh isoimmune hemolytic disease. Cord blood values failed to predict the severity of hyperbilirubinemia with sufficient accuracy to warrant their use as therapeutic guidelines. A statistically significant positive correlation was found between the cord serum indirect bilirubin concentration and its subsequent rise in 19 infants who had received antenatal phenobarbital therapy, but this relationship was not observed in untreated infants. The phenobarbital-treated infants had a slower postnatal rise of indirect bilirubin than did nontreated controls. There was no reliable indicator of the severity of hyperbilirubinemia other than careful monitoring of the serum bilirubin concentration during the early hours of life.