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Improved medical-alert ID ownership and utilization in youth with congenital adrenal hyperplasia following a parent educational intervention.

ABSTRACT: BACKGROUND:Classical congenital adrenal hyperplasia (CAH) is a potentially life-threatening condition, and adrenal crisis is a major cause of morbidity and mortality in affected children. Medical-alert identification (ID) could prevent complications of adrenal crisis by identifying the need for time-sensitive, critical treatment. Our objectives were to evaluate usage of medical-alert IDs by CAH youth, ownership and awareness of IDs amongst their parents, and the effect of an in-clinic educational intervention on ID utilization. METHODS:Fifty families of youth with classical CAH secondary to 21-hydroxylase deficiency (11.2±5.0 years old, 58% female) were prospectively studied. An in-clinic needs assessment survey was administered at baseline to parents, paired with an educational intervention, and a follow-up needs assessment phone survey 1 month post-intervention. A quality improvement (QI) framework was utilized with plan-do-study-act (PDSA) process-improvement cycles. RESULTS:At baseline, 20/50 (40%) CAH families owned a medical-alert ID, of which only 10/20 (50%) of ID owners reported usage >3 days per week. Only 26/50 (52%) parents were aware of ID options. Post-intervention, ID ownership doubled to 39/50 (78%; p<0.05), usage amongst ID owners reached 100% (39/39), and awareness increased to 42/50 (84%; p<0.05). A surprising barrier reported by five Spanish-speaking families was the inability to order medical-alert IDs online. CONCLUSIONS:Only a small percentage of CAH youth frequently wear a medical-alert ID, but utilization can be effectively improved with an in-clinic educational intervention. Further study is merited to assess a potential reduction in morbidity and mortality of adrenal crisis with increased medical-alert ID utilization.

SUBMITTER: Vidmar AP

PROVIDER: S-EPMC7140978 | biostudies-literature | 2018 Jan

REPOSITORIES: biostudies-literature

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Improved medical-alert ID ownership and utilization in youth with congenital adrenal hyperplasia following a parent educational intervention.

Vidmar Alaina P AP Weber Jonathan F JF Monzavi Roshanak R Koppin Christina M CM Kim Mimi S MS

Journal of pediatric endocrinology & metabolism : JPEM 20180101 2

<h4>Background</h4>Classical congenital adrenal hyperplasia (CAH) is a potentially life-threatening condition, and adrenal crisis is a major cause of morbidity and mortality in affected children. Medical-alert identification (ID) could prevent complications of adrenal crisis by identifying the need for time-sensitive, critical treatment. Our objectives were to evaluate usage of medical-alert IDs by CAH youth, ownership and awareness of IDs amongst their parents, and the effect of an in-clinic ed ...[more]

PMID: 29315077

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Project description:IntroductionAdrenocortical hyperplasia and adrenal rest tumor (ART) formation are common in congenital adrenal hyperplasia (CAH). Although driven by excessive corticotropin, much is unknown regarding the morphology and transformation of these tissues. Our study objective was to characterize CAH-affected adrenals and ART and compare with control adrenal and gonadal tissues.Patients/methodsCAH adrenals, ART and control tissues were analyzed by histology, immunohistochemistry, and transcriptome sequencing. We investigated protein expression of the ACTH receptor (MC2R), steroidogenic (CYP11B2, CYP11B1, CYB5A) and immune (CD20, CD3, CD68) biomarkers, and delta-like 1 homolog (DLK1), a membrane bound protein broadly expressed in fetal and many endocrine cells. RNA was isolated and gene expression was analyzed by RNA sequencing (RNA-seq) followed by principle component, and unsupervised clustering analyses.ResultsBased on immunohistochemistry, CAH adrenals and ART demonstrated increased zona reticularis (ZR)-like CYB5A expression, compared to CYP11B1, and CYP11B2, markers of zona fasciculata and zona glomerulosa respectively. CYP11B2 was mostly absent in CAH adrenals and absent in ART. DLK1 was present in CAH adrenal, ART, and also control adrenal and testis, but was absent in control ovary. Increased expression of adrenocortical marker MC2R, was observed in CAH adrenals compared to control adrenal. Unlike control tissues, significant nodular lymphocytic infiltration was observed in CAH adrenals and ART, with CD20 (B-cell), CD3 (T-cell) and CD68 (macrophage/monocyte) markers of inflammation. RNA-seq data revealed co-expression of adrenal MC2R, and testis-specific INSL3, HSD17B3 in testicular ART indicating the presence of both gonadal and adrenal features, and high expression of DLK1 in ART, CAH adrenals and control adrenal. Principal component analysis indicated that the ART transcriptome was more similar to CAH adrenals and least similar to control testis tissue.ConclusionsCAH-affected adrenal glands and ART have similar expression profiles and morphology, demonstrating increased CYB5A with ZR characteristics and lymphocytic infiltration, suggesting a common origin that is similarly affected by the abnormal hormonal milieu. Immune system modulators may play a role in tumor formation of CAH.

Dataset Information

Improved medical-alert ID ownership and utilization in youth with congenital adrenal hyperplasia following a parent educational intervention.

Publications

Improved medical-alert ID ownership and utilization in youth with congenital adrenal hyperplasia following a parent educational intervention.

Similar Datasets

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