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Horm Res Paediatr. 2010;74(1):41-9. doi: 10.1159/000313368. Epub 2010 Apr 15.

Alterations in lipid and carbohydrate metabolism in patients with classic congenital adrenal hyperplasia due to 21-hydroxylase deficiency.

Author information

1
1st Medical Clinic, Department of Endocrinology and Metabolic Diseases, Johannes Gutenberg University of Mainz, Mainz, Germany. zimmeran@uni-mainz.de

Abstract

BACKGROUND:

Classic 21-hydroxylase deficiency (21HD) presents some traits of the metabolic syndrome.

AIM:

To characterize discrete alterations of lipid and carbohydrate metabolism in children and young adults with classic 21HD, which could predict early atherogenesis.

PATIENTS AND METHODS:

Twenty-seven Caucasian patients with classic 21HD (4-31 years); 27 sex-, age- and BMI-matched controls. Clinical parameters, hormonal status and genotype were assessed in all patients. Lipid parameters, including relative (%) and absolute (mg/dl) small-dense low-density lipoproteins subfractions (sd-LDL) were measured in patients and controls. Oral glucose tolerance tests were performed in both groups.

RESULTS:

sd-LDL (%) was significantly higher in patients than controls (39.7 +/- 5.9 vs. 35.5 +/- 5.7%; p = 0.008). The same applies for absolute sd-LDL (mg/dl) (42.6 +/- 11.9 vs. 36.4 +/- 7.5; p = 0.029). HDL-cholesterol was lower in patients (p = 0.032). Fasting glucose and insulin were significantly higher in patients. Similar differences were noticed for HOMA-IR (p = 0.001), IRI (p = 0.001) and HOMA-B (p = 0.002). IRI correlated directly and significantly with the total hydrocortisone dose and the duration of treatment. Fasting glucose correlated with absolute sd-LDL. No obvious differences were seen between clinical forms or genotype groups.

CONCLUSIONS:

Substitution therapy should be adapted particularly at young ages to prevent early atherogenesis and cardiovascular risk in later life.

PMID:
20395657
DOI:
10.1159/000313368
[Indexed for MEDLINE]

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