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Incidence of adrenal crisis in CAH differs between treating hospitals

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August 10, 2018

An analysis of Australian hospital data showed that the incidence of adrenal crisis in young children with congenital adrenal hyperplasia, or CAH, varied by treating hospital, with variation likely due to differences between clinicians, according to findings published in Clinical Endocrinology.

“Cortisol dose escalation is regarded as the cornerstone of [adrenal crisis] prevention in all forms of [adrenal insufficiency], including CAH,” Georgina L. Chrisp, a graduate student at the University of Notre Dame Australia School of Medicine, Darlinghurst, and colleagues wrote in the study background. “Parents and guardians are taught to increase their child’s dose of oral glucocorticoid during intercurrent illness or to administer an intramuscular injection when oral glucocorticoid therapy cannot be taken or absorbed. However, efforts by health professionals to educate [caregivers] and patients about the importance of stress dosing have not resulted in the elimination of [adrenal crises]. Indeed, recent studies have shown that [adrenal crisis] events continue to occur, even among well-educated parents.”

Chrisp and colleagues analyzed data from 74 children with CAH and prescribed glucocorticoid replacement therapy, presenting at one of three pediatric hospitals in New South Wales, Australia, between 2000 and 2015 with an acute medical illness (median age, 3 years; 79.9% with salt-wasting CAH). Of the three hospitals, two (hospitals A and B) have a practice guideline consisting of a protocol for acute adrenal insufficiency or adrenal crisis management that includes instructions on immediate administration of hydrocortisone, fluid resuscitation and specific treatment of hypoglycemia and abnormal electrolytes (66.7% of patients). The third hospital (hospital C) uses a specific management protocol for each patient developed by its regular pediatric endocrinologist (33.3% of patients). Researchers used stepwise logistic regression models to identify predictors of an adrenal crisis diagnosis, hospital admission and the use of IV hydrocortisone in the hospital.

Within the cohort, there were 321 presentations for treatment of an acute medical illness, for a median of three presentations per child, with 66.4% admitted. IV hydrocortisone was administered to 49.2% of patients and median length of stay was 2 days. Researchers observed 29 cases of adrenal crisis (9%) and stress dosing (defined as any administration before arrival at the hospital) was reported by 64.2% of caregivers, with 41.7% using oral glucocorticoid dose escalation only and 22.1% using intramuscular hydrocortisone with or without oral glucocorticoid.

Researchers noted that admission for acute illness and the use of stress dosing varied by hospital (P < .001 and P < .01, respectively). Hospital C admitted the most children (80.4%) and had the highest proportion of patients using stress doses (75.7%). Patients in hospital C were also much more likely to receive intramuscular hydrocortisone (45.8%) vs. patients in hospital A (7%).

In logistic regression analysis, researchers found that the treating hospital a patient presented to was associated with a diagnosis of adrenal crisis. The OR for adrenal crisis diagnosis in hospital B vs. hospital A was 4.42 (95% CI, 1.25-15.65), whereas the OR for the same diagnosis was 4.63 (95% CI, 1.65-12.94) in comparing hospital C vs. hospital A. The presence of reduced level of consciousness was also associated with an adrenal crisis diagnosis (OR = 11.27; 95% CI, 4.57-27.8), but not the number of adrenal crisis signs and symptoms, according to researchers. Admission was also associated with the treating hospital, the number of patient signs and symptoms and patient age.

Researchers also found that use of any stress management therapy (oral or intramuscular) before presenting at the hospital was associated with the treating hospital (OR = 2.5; 95% CI, 1.52-4.1), as well as a diagnosis of gastroenteritis (OR = 2.67; 95% CI, 1.17-6.09). Hospitals B and C had a lower likelihood of patients who received oral stress doses vs. hospital A, according to researchers.

The treating hospital was also associated with IV hydrocortisone use (OR = 2.47; 95% CI, 1.39-4.37 for hospital C vs. hospital A).

“Prevention of symptomatic [adrenal insufficiency/adrenal crisis] remains an issue in the management of patients with CAH,” the researchers wrote. “Importantly, the results of this investigation demonstrate that patient factors alone are not the sole determinants of health outcomes in CAH and that parental experience with the management of illness influences the use of stress dosing even among well-educated parents.”

The researchers added that the results demonstrate the value of inter-hospital comparison studies for chronic diseases, and the importance of consistent, guideline-based application of adrenal crisis prevention strategies. – by Regina Schaffer

Disclosures: The authors report no relevant financial disclosures.