Adrenocortical insufficiency in infants with congenital diaphragmatic hernia: a pilot study

Prior reports have documented that premature infants do not have normal serum levels of cortisol. In contrast, full-term infants usually have adequate cortisol levels. The stress response in critically ill infants may be vital to their recovery. The purpose of this pilot study was to determine whether critically ill full-term infants with congenital diaphragmatic hernia (CDH) show a subnormal adrenal stress response.

Random serum cortisol levels in infants with CDH (n = 10) were measured using fluorescent polarization immunoassay. In addition, serum cortisol levels were measured after exogenous adrenocorticotropic hormone stimulation (Cosyntropin stimulation test).

Six of the 10 infants studied died. Most (79%) of the cortisol levels were subnormal (<7 microgm/dL). Although no significant differences in mean cortisol levels from terminally ill infants compared with surviving infants were detected, survivors tended to have higher cortisol levels. Cosyntropin stimulation resulted in inappropriately low cortisol levels in 2 of the 4 fatally ill patients tested (<30 microgm/dL) and normal responses in the 2 survivors tested.

Infants born with CDH may have an inadequate adrenal stress response despite a life-threatening anomaly. A large-scale prospective study may be warranted to confirm this apparent association. Corticosteroid therapy may be beneficial in this population of patients.