Management of Childhood Congenital Adrenal Hyperplasia-An Integrative Review of the Literature.
Louise Fleming, Marcia Van Riper, Kathleen Knafl
PMID 28416079WHAT IT FOUND
Children with congenital adrenal hyperplasia remained at risk for low blood sugar during illness even when stress dosed, and caregivers who received written instructions plus a demonstration reported more confidence.
Key findings
01Caregivers who received both written instructions and a clinician demonstration on how to stress dose, orally and by injection, had higher confidence scores.
02During common childhood illnesses, children with CAH who were stress dosed appropriately were still at risk for hypoglycemia, and the authors suggested additional glucose supplementation.
03In a cohort of 51 children, lower glucocorticoid doses in infancy did not accelerate bone age or growth, and children reached height within the target range, but final height remained below genetic potential.
STILL TO COME
How it was doneWhat they foundWhat it means for RNs
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What it does not show
Most included studies were cross-sectional or descriptive and came from one institution or city, so they may not reflect the full range of treatment protocols used for children with congenital adrenal hyperplasia. Only one of the 39 studies involved an intervention, and it was not randomized, so this review cannot show which treatments work. Many studies used structured measures but did not report reliability and validity. Studies of stress dosing and adrenal crisis used very small samples, including samples under 10. Weight and obesity findings did not consistently account for parental body mass index or diet, so the effects of congenital adrenal hyperplasia and environment are hard to separate. Family and gender findings relied mostly on parent report, with few sibling, marital, or parenting covariates, and observers were not described. The review searched only English peer-reviewed articles from January 1, 2000 to June 1, 2015, so relevant non-English or outside-date work may be missing. Only seven studies addressed family life experience, and few examined how families manage social challenges, so conclusions about family response are limited. Cultural and economic differences affected findings, including medication cost in some countries and gender assignment practices in one study.
Declared interests
The supplied article text does not include an author conflict-of-interest statement. The publication types list non-U.S. government and NIH extramural research support.
The easy way to misread this
Do not read this review as proof that written instructions, stress dosing, growth hormone, or prenatal dexamethasone improve long-term outcomes. Most included studies were observational, the caregiver education study measured confidence rather than crisis prevention, and only one intervention study was nonrandomized.