Blood Pressure in a Large Cohort of Children and Adolescents With Classic Adrenal Hyperplasia (CAH) Due to 21-Hydroxylase Deficiency

BACKGROUND Data on blood pressure (BP) in children and adolescents with classic congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency are conflicting in the literature. PATIENTS AND METHODS BP data of n = 716 children and adolescents (aged 3–18 years) from a national CAH database wer...

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Published in:American journal of hypertension 2016-02, Vol.29 (2), p.266-272
Main Authors: Bonfig, Walter, Roehl, Friedrich-Wilhelm, Riedl, Stefan, Dörr, Helmut Günther, Bettendorf, Markus, Brämswig, Jürgen, Schönau, Eckhard, Riepe, Felix, Hauffa, Berthold, Holl, Reinhard W., Mohnike, Klaus
Format: Article
Language:English
Subjects:
Adolescent
Adrenal Hyperplasia, Congenital - physiopathology
Blood Pressure
Child
Child, Preschool
Cohort Studies
Female
Humans
Male
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Summary:BACKGROUND Data on blood pressure (BP) in children and adolescents with classic congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency are conflicting in the literature. PATIENTS AND METHODS BP data of n = 716 children and adolescents (aged 3–18 years) from a national CAH database were analyzed. BP data were adjusted for height and compared to contemporary national reference data. A systolic and diastolic BP above the 95th centile was defined as hypertensive. RESULTS Overall prevalence of hypertension was 12.5%. Prevalence of hypertension was higher in younger children than in adolescents (18.5% vs. 4.9%). Until 8 years of age, fludrocortisone dose/m2/day correlated significantly with BP in regression analysis (P < 0.0001). BP correlated significantly with body mass index standard deviation score (BMI-SDS) (P < 0.0001), but not with hydrocortisone dose. In patients with salt-wasting CAH, BMI-SDS and BP were significantly higher compared to patients with simple virilising CAH, P < 0.01. CONCLUSION Especially young CAH children seem to be at risk for—most likely transient—hypertension, since the prevalence of hypertension decreases with age. In children up to 8 years of age, the used fludrocortisone dose is a significant risk factor for hypertension. Therefore we recommend accurate measurement of BP and careful fludrocortisone dosing in children with CAH.
ISSN:0895-7061
1941-7225
DOI:10.1093/ajh/hpv087