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Adrenocortical insufficiency in infants with congenital diaphragmatic hernia: a pilot study
1Department of Surgery, Children's Hospital and Regional Medical Center, University of Washington, Seattle 98105, USA.
Insights
Critically ill infants with congenital diaphragmatic hernia (CDH) may have a weakened adrenal stress response, with most showing subnormal cortisol levels. This suggests potential benefits from corticosteroid therapy in managing CDH patients.
Area of Science:
- Neonatalogy
- Pediatric Endocrinology
- Critical Care Medicine
Background:
- Premature infants typically exhibit abnormal serum cortisol levels, unlike full-term infants.
- The stress response is crucial for the recovery of critically ill infants.
- Congenital diaphragmatic hernia (CDH) is a serious condition in newborns.
Purpose of the Study:
- To investigate whether critically ill, full-term infants diagnosed with congenital diaphragmatic hernia (CDH) exhibit a subnormal adrenal stress response.
- To assess the adrenal function in neonates with CDH.
Main Methods:
- Serum cortisol levels were measured in 10 infants with CDH using fluorescent polarization immunoassay.
- Adrenal function was further evaluated using the Cosyntropin stimulation test to measure cortisol response to exogenous adrenocorticotropic hormone.
Main Results:
- A significant proportion of infants (79%) presented with subnormal cortisol levels (<7 microgm/dL).
- Six out of ten infants with CDH did not survive.
- While not statistically significant, survivors tended to have higher cortisol levels than non-survivors. The Cosyntropin test revealed inappropriately low cortisol in 2 of 4 fatally ill infants, but normal responses in the 2 survivors.
Conclusions:
- Infants with congenital diaphragmatic hernia may possess an impaired adrenal stress response, even with a life-threatening condition.
- Further large-scale prospective studies are recommended to validate the association between CDH and adrenal insufficiency.
- Corticosteroid therapy might prove beneficial for infants diagnosed with CDH and suspected adrenal dysfunction.
Background/Purpose:
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.
Methods:
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).
Results:
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.
Conclusions:
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.