Reduction of steroid replacement therapy in patients with congenital adrenal hyperplasia in the transition period from child- to adulthood

2007 ◽  
Vol 115 (S 1) ◽  
Author(s):  
N Reisch ◽  
HP Schwarz ◽  
F Beuschlein ◽  
M Reincke
Keyword(s):  
Congenital Adrenal Hyperplasia ◽  
Replacement Therapy ◽  
Transition Period ◽  
Adrenal Hyperplasia

Diagnosis, treatment and preventive measures in congenital adrenal hyperplasia due to 21-hydroxylase deficiency in patients in adulthood

2016 ◽  
Vol 3 (1) ◽  
pp. 26-32
Author(s):  
Natalya V. Molashenko ◽  
A. I Sazonova ◽  
E. A Troshina
Keyword(s):  
Congenital Adrenal Hyperplasia ◽  
Replacement Therapy ◽  
Diagnostic Tests ◽  
Preventive Measures ◽  
Adrenal Hyperplasia ◽  
Hydroxylase Deficiency ◽  
21 Hydroxylase Deficiency

In the article there are presented main approaches to the diagnosis, treatment and follow-up of patients with classical and nonclassical forms of 21-hydroxylase deficiency. The techniques of diagnostic tests and parameters of hormonal indices for the assessment of the adequacy of replacement therapy with glucocorticoids and mineralocorticoids are considered.

scholarly journals Pecularities of congenital adrenal hyperplasia replacement therapy in children

2014 ◽  
Vol 0 (1(46)) ◽  
pp. 53-58
Author(s):  
Н. Б. Зелінська ◽  
Н. Л. Погадаєва ◽  
Є. В. Глоба ◽  
І. Ю. Шевченко
Keyword(s):  
Congenital Adrenal Hyperplasia ◽  
Replacement Therapy ◽  
Adrenal Hyperplasia

scholarly journals A Bloodspot Androstenedione Assay Suitable for Home-Monitoring of Steroid Replacement Therapy in Congenital Adrenal Hyperplasia

1989 ◽  
Vol 26 (3) ◽  
pp. 262-267 ◽  
Author(s):  
Sandra M Egan ◽  
P Betts ◽  
S Thomson ◽  
A M Wallace ◽  
P J Wood
Keyword(s):  
Detection Limit ◽  
Bovine Serum Albumin ◽  
Serum Albumin ◽  
Congenital Adrenal Hyperplasia ◽  
Replacement Therapy ◽  
Sex Difference ◽  
Bovine Serum ◽  
Home Monitoring ◽  
Adrenal Hyperplasia ◽  
Glucocorticoid Replacement Therapy

A bloodspot assay has been developed using an antiserum raised against androstenedione-3-carboxymethyloxime-bovine serum albumin (AD-3CMO-BSA) conjugate and 125I-AD-3CMO-histamine tracer. The method has a detection limit of 0·6 nmol/L blood and a working range from 0·6 to 40 nmol/L blood. The between-batch precision ranged from 8·4% to 23·3%. Bloodspot androstenedione (AD) concentrations were measured in 50 neonates (26 male, 24 female) and in 95 children (54 male, 41 female) aged 6 months to 18 years. No sex difference in concentrations was found in neonates, in pre-pubertal children up to 8 years of age, in pubertal children (males 8–16 years, females 8–14 years) or post-puberty. Bloodspot AD concentrations ranged from <0·6 to 2·7 nmol/L in neonates, <0·6 nmol/L in pre-pubertal children, 0·6–2·1 nmol/L in pubertal children and < 0·6–4·6 nmol/L post-puberty. Daytime bloodspot profiles in 10 children on replacement therapy for congenital adrenal hyperplasia generally showed good correlation between 17α-hydroxyprogesterone and AD concentrations ( r = 0·928, P < 0·001). Bloodspot AD profiles have advantages over 17α-hydroxyprogesterone profiles for the assessment of the adequacy of glucocorticoid replacement therapy.

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

2021 ◽  
Author(s):  
Irina Bacila ◽  
Nicole Freeman ◽  
Eleni Daniel ◽  
Marija Sandrk ◽  
Jillian Bryce ◽  
...  
Keyword(s):  
Congenital Adrenal Hyperplasia ◽  
Replacement Therapy ◽  
Hormone Replacement ◽  
Age Groups ◽  
Temporal Variations ◽  
Adrenal Hyperplasia ◽  
Hydroxylase Deficiency ◽  
High Doses ◽  
Multi Center Study ◽  
21 Hydroxylase Deficiency

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.

scholarly journals Would Cortisol Measurements Be a Better Gauge of Hydrocortisone Replacement Therapy? Congenital Adrenal Hyperplasia as an Exemplar

2020 ◽  
Vol 2020 ◽  
pp. 1-9
Author(s):  
Peter C Hindmarsh ◽  
John W Honour
Keyword(s):  
Quality Of Life ◽  
Congenital Adrenal Hyperplasia ◽  
Adrenal Insufficiency ◽  
Replacement Therapy ◽  
Plasma Cortisol ◽  
Poor Quality ◽  
Adrenal Hyperplasia ◽  
Mortality And Morbidity ◽  
Glucocorticoid Replacement Therapy

There is an increase in mortality and morbidity as well as poor quality of life in patients with congenital adrenal hyperplasia (CAH) and other causes of adrenal insufficiency. Glucocorticoid replacement therapy should aim to replace the missing cortisol as close as possible to the normal circadian rhythm using hydrocortisone. Dosing should be based on the individual’s absorption and clearance of the drug. Adequacy of dosing should be checked using 24-hour profiles of plasma cortisol with samples drawn preferably every hour or at least every 2 hours. Measurement of cortisol should be the preferred method of assessing replacement therapy as it is over- and undertreatment with hydrocortisone, both of which can occur over a 24-hour period, which leads to the problems observed in patients with CAH and adrenal insufficiency.

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