Authors

Riepe FG. Krone N. Kruger SN. Sweep FC. Lenders JW. Dotsch J. Monig

H. Sippell WG. Partsch CJ.

Institution Division of Pediatric Endocrinology, Department of Pediatrics,

Christian-Albrechts-Universitat Kiel, Kiel, Germany.

friepe@pediatrics.uni-kiel.de

Title

Absence of exercise-induced leptin suppression associated with

insufficient epinephrine reserve in patients with classic congenital

adrenal hyperplasia due to 21-hydroxylase deficiency.

Source

Experimental & Clinical Endocrinology & Diabetes. 114(3):105-10, 2006

Mar.

Abstract

OBJECTIVE: Patients with congenital adrenal hyperplasia (CAH) due to

21-hydroxylase deficiency suffer from glucocorticoid and mineralocorticoid

deficiency. They have insufficient epinephrine reserves and increased

basal leptin levels and are often insulin resistant. In healthy subjects,

an inhibitory effect of acute catecholamine elevation on the leptin plasma

concentrations has been reported. However, it is not yet known how leptin

levels respond to exercise in CAH patients. METHODS: We performed a cycle

ergometer test in six CAH patients to measure the response of plasma

leptin, glucose and the catecholamines, epinephrine (E) and norepinephrine

(N), as well as their respective metabolites, metanephrine (M) and

normetanephrine (NM), to intense exercise. RESULTS: Baseline leptin

concentrations in CAH patients were not different from those of controls.

Leptin levels decreased significantly with exercise in healthy controls,

whereas they remained unchanged in CAH patients. In contrast to controls,

CAH patients showed no rise of plasma glucose. Basal and stimulated E and

M levels were significantly lower in CAH patients compared to controls.

Baseline and stimulated N and NM levels were comparable, showing a

significant rise after exercise. Peak systolic blood pressure and peak

heart rate in both groups were comparable. CONCLUSION: CAH patients do not

manifest exercise-induced leptin suppression. The most probable reason for

this is their severely impaired epinephrine stress response. In addition,

epinephrine deficiency is leading to secondary changes in various

catecholamine dependent metabolic pathways, e. g., energy balance.

Although obvious clinical sequelae are so far unknown, the

catecholamine-deficient state and the resulting hyperleptinemia might

contribute to the severity of the disease in CAH.