Absence of Exercise-Induced Leptin Suppression Associated with Insufficient Epinephrine Reserve in Patients with Classic Congenital Adrenal Hyperplasia Due to 21-Hydroxylase Deficiency

Riepe, Felix G.; Krone, Nils; Krüger, Schulamith; Sweep, Fred C.G.J.; Lenders, J. W. M.; Dötsch, Jörg; Mönig, H.; Sippell, W.G.; Partsch, C. J.

doi:10.1055/s-2005-865836

Cited by 25 publications

(17 citation statements)

References 29 publications

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“…Even a small increment of a few beats per minute within the normal range can increase the cardiovascular risk (28). Our younger patients had normal heart rates in accord with previous studies of young patients (29)(30)(31). The heart rate in CAH males was correlated with other cardiovascular risk factors.…”

Section: Discussionsupporting

confidence: 78%

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Cardiovascular risk, metabolic profile, and body composition in adult males with congenital adrenal hyperplasia due to 21-hydroxylase deficiency

Falhammar¹,

Nyström²,

Wedell³

et al. 2011

European Journal of Endocrinology

105

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Objective: Lifelong glucocorticoid therapy in patients with congenital adrenal hyperplasia (CAH) or the disease per se may result in increased cardiovascular risk. We therefore investigated cardiovascular and metabolic risk profiles in adult CAH males. Subjects and methods: We compared CAH males (nZ30), 19-67 years old, with age-and sex-matched controls (nZ32). Subgroups of different ages (!30 years or older) and CYP21A2 genotypes (null, I2splice, and I172N as the mildest mutation) were studied. Anthropometry, fat and lean mass measured by dual-energy X-ray absorptiometry, lipids, liver function tests, homocysteine, lipoprotein-(a), glucose and insulin during an oral glucose tolerance test (OGTT), urine albumin, adrenal hormones, and 24 h ambulatory blood pressure measurements were studied. Results: CAH males were shorter. Waist/hip ratio and fat mass were higher in older patients and the I172N group. Heart rate was faster in older patients, the I2splice, and I172N groups. Insulin levels were increased during OGTT in all patients and in the I172N group. g-glutamyl transpeptidase was increased in older patients and in the I172N group. Testosterone was lower in older patients. Homocysteine was lower in younger patients, which may be cardioprotective. The cardiovascular risk seemed higher with hydrocortisone/cortisone acetate than prednisolone. Urinary epinephrine was lower in all groups of patients except in I172N. Conclusions: Indications of increased risk were found in CAH males R30 years old and in the I172N group. In contrast, younger CAH males did not differ from age-matched controls. This is likely to reflect a better management in recent years.

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Section: Discussionsupporting

confidence: 78%

“…Epinephrine production has been shown to be impaired in adolescents and young adults with classic CAH (1,(29)(30)(31). This defect is certainly the result of insufficient prenatal cortisol secretion from the adrenal cortex, necessary for adrenomedullary organogenesis and epinephrine production (1).…”

Section: Discussionmentioning

confidence: 99%

Cardiovascular risk, metabolic profile, and body composition in adult males with congenital adrenal hyperplasia due to 21-hydroxylase deficiency

Falhammar¹,

Nyström²,

Wedell³

et al. 2011

European Journal of Endocrinology

105

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“…On the other hand, plasma epinephrine and urine MN levels were similar in the CAH subgroups. Compensatory increases in sympathetic nerve activity resulting in an increase in NE secretion have been reported in adrenalectomized patients [20] and patients with Addison’s disease [21], but not in patients with CAH [4,22,23]. Zuckerman-Levin et al [15] found that resting and stimulated NE levels in patients with isolated glucocorticoid deficiency tended to be higher compared with control subjects, but these did not reach statistical significance when compared with the control subjects.…”

Section: Discussionmentioning

confidence: 99%

Evaluation of Adrenomedullary Function in Patients with Congenital Adrenal Hyperplasia

et al. 2009

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Background/Aims: Congenital adrenal hyperplasia (CAH) is characterized by adrenal insufficiency with or without salt wasting. It is also accompanied by adrenomedullary hypofunction. The aim of the present study was to investigate adrenomedullary function in patients with CAH due to 21-hydroxylase and 11β-hydroxylase deficiencies and in age-matched normal subjects. Methods: We measured plasma catecholamines (epinephrine and norepinephrine) and urine metanephrine in 44 patients with CAH, 32 due to 21-hydroxylase deficiency (17 patients with the salt-wasting form and 15 patients with the simple virilizing form), and 12 due to 11β-hydroxylase deficiency, and in 25 healthy controls. Results: Plasma epinephrine and urine metanephrine levels were significantly higher in the controls than in patients with CAH (p = 0.02 and p < 0.001, respectively). Plasma norepinephrine levels were significantly lower in the controls than in patients with CAH (p < 0.001). Interestingly, patients with the salt-wasting form had lower norepinephrine levels in comparison to the other subgroups of CAH. Conclusion: Despite the fact that CAH patients have insufficient epinephrine secretion, these patients have the ability to increase compensatory norepinephrine. However, this increase is much lower in patients with the salt-wasting form. These findings need to be confirmed by other studies.

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“…In the last few years, several publications have described the negative impact that 21-hydroxylase deficiency has on adrenal medullary development [8], and it has been demonstrated that CAH patients have a compromised catecholamine reserve that results in a diminished catecholamine response to stress [41, 42]. To date, these studies have not led to any firm therapeutic consequences.…”

Section: Experimental Therapies In Cahmentioning

confidence: 99%

Adult Consequences of Congenital Adrenal Hyperplasia

Arlt

Krone

2007

Horm Res Paediatr

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Background: Congenital adrenal hyperplasia (CAH) caused by 21-hydroxylase deficiency accounts for 95% of all CAH cases and is one the most common inborn metabolic disorders. While consensus and guidelines on therapeutic management in infancy and childhood are available, data regarding the treatment of adults with CAH are scarce. This review highlights the issues that need to be addressed when caring for the adult CAH patient. Issues include glucocorticoid and mineralocorticoid replacement, adrenal crisis, female and male fertility, genetic counselling, prenatal dexamethasone treatment, pregnancy and the odds of long-term morbidity and mortality in these patients. Conclusions: Large-scale audit studies are urgently required to help optimise management and long-term outcome of these patients, as are optimisation of glucocorticoid replacement and biochemical monitoring tools. It is very important that the adult CAH patient receive regular monitoring by a multidisciplinary team at the secondary- or tertiary-care level.

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Absence of Exercise-Induced Leptin Suppression Associated with Insufficient Epinephrine Reserve in Patients with Classic Congenital Adrenal Hyperplasia Due to 21-Hydroxylase Deficiency

Cited by 25 publications

References 29 publications

Cardiovascular risk, metabolic profile, and body composition in adult males with congenital adrenal hyperplasia due to 21-hydroxylase deficiency

Cardiovascular risk, metabolic profile, and body composition in adult males with congenital adrenal hyperplasia due to 21-hydroxylase deficiency

Evaluation of Adrenomedullary Function in Patients with Congenital Adrenal Hyperplasia

Adult Consequences of Congenital Adrenal Hyperplasia

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