Congenital adrenal hyperplasia in children – a survey on the current practice in the UK

Niranjan, Usha

doi:10.1515/jpem-2014-0362

Cited by 2 publications

(1 citation statement)

References 9 publications

(9 reference statements)

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“…A broad variation between different countries in the approach to GC replacement therapy was observed. This finding is not entirely unexpected considering previously reported variations in GC regimens for different centers within the United Kingdom (37). In some countries the recommended glucocorticoid dose range for children was exceeded in as many as 75% of patient visits, while other countries recorded doses below 10 mg/m 2 per day in up to 57% of cases.…”

Section: Discussionsupporting

confidence: 68%

International practice of corticosteroid replacement therapy in congenital adrenal hyperplasia: data from the I-CAH registry

Bacila

Freeman

Daniel

et al. 2021

European Journal of Endocrinology

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Objective: Despite published guidelines no unified approach to hormone replacement in congenital adrenal hyperplasia (CAH) exists. We aimed to explore geographical and temporal variations in the treatment with glucocorticoids and mineralocorticoids in CAH. Design: This retrospective multi-center study, including 31 centers (16 countries), analyzed data from the International-CAH Registry. Methods: Data was collected from 461 patients aged 0-18 years with classic 21-hydroxylase deficiency (54.9% females) under follow-up between 1982 – 2018. Type, dose and timing of glucocorticoid and mineralocorticoid replacement was analyzed from 4174 patient visits. Results: The most frequently used glucocorticoid was hydrocortisone (87.6%). Overall, there were significant differences between age groups with regards to daily hydrocortisone-equivalent dose for body surface, with the lowest dose (median with interquartile range) of 12.0 (10.0 – 14.5) mg/ m2/ day at age 1 - 8 years and the highest dose of 14.0 (11.6 - 17.4) mg/ m2/ day at age 12-18 years. Glucocorticoid doses decreased after 2010 in patients 0-8 years (p<0.001) and remained unchanged in patients aged 8-18 years. Fludrocortisone was used in 92% of patients, with relative doses decreasing with age. A wide variation was observed among countries with regards to all aspects of steroid hormone replacement. Conclusions: Data from the I-CAH Registry suggests international variations in hormone replacement therapy, with a tendency to treatment with high doses in children.

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Section: Discussionsupporting

confidence: 68%

International practice of corticosteroid replacement therapy in congenital adrenal hyperplasia: data from the I-CAH registry

Bacila

Freeman

Daniel

et al. 2021

European Journal of Endocrinology

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Real-World Estimates of Adrenal Insufficiency–Related Adverse Events in Children With Congenital Adrenal Hyperplasia

Ali

Bryce

Haghpanahan

et al. 2020

The Journal of Clinical Endocrinology &Amp; Metabolism

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Background Although congenital adrenal hyperplasia (CAH) is known to be associated with adrenal crises (AC), its association with patient or clinician reported sick day episodes (SDE) is less clear. Methods Data on children with classic 21-hydroxylase deficiency CAH from 34 centers in 18 countries, of which 7 were Low or Middle Income (LMIC) and 11 were High Income (HIC), were collected from the International CAH Registry and analysed to examine the clinical factors associated with SDE and AC. Results 518 children with a median of 11 children (range 1, 53) per center had 5,388 visits evaluated over a total of 2,300 patient years. The median number of AC and SDE per patient year per center was 0 (0, 3) and 0.4 (0.0, 13.3), respectively. Of the 1,544 SDE, an AC was reported in 62 (4%), with no fatalities. Infectious illness was the most frequent precipitating event, reported in 1,105 (72%) and 29 (47%) of SDE and AC, respectively. On comparing cases from LMIC and HIC, the median SDE per patient year was 0.75 (0, 13.3) vs 0.11 (0, 12.0) (p<0.001), respectively and the median AC per patient year was 0 (0, 2.2) vs 0 (0, 3.0) (p=0.43), respectively. Conclusions The real-world data that are collected within the I-CAH Registry show wide variability in the reported occurrence of adrenal insufficiency related adverse events. As these data become increasingly used as a clinical benchmark in CAH care, there is a need for further research to improve and standardise the definition of SDE.

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Congenital adrenal hyperplasia in children – a survey on the current practice in the UK

Abstract: Our survey highlights the diversity in managing children with CAH in the UK as compared with the recommendations of the Endocrine Society. It also demonstrates inconsistent involvement of essential specialist services, which are essential for optimal management of this condition.

Cited by 2 publications

References 9 publications

International practice of corticosteroid replacement therapy in congenital adrenal hyperplasia: data from the I-CAH registry

International practice of corticosteroid replacement therapy in congenital adrenal hyperplasia: data from the I-CAH registry

Real-World Estimates of Adrenal Insufficiency–Related Adverse Events in Children With Congenital Adrenal Hyperplasia

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