Patients with Classic Congenital Adrenal Hyperplasia Have Decreased Epinephrine Reserve and Defective Glycemic Control during Prolonged Moderate-Intensity Exercise

Green-Golan, Liza; Yates, Catherine; Drinkard, Bart; VanRyzin, Carol; Eisenhofer, Graeme; Weise, Martina; Merke, Deborah P.

doi:10.1210/jc.2007-0493

Cited by 44 publications

(28 citation statements)

References 25 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: 89%

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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: 89%

“…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

View full text Add to dashboard Cite

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“…A similar reduced glucose response to maximal exercise was seen in CAH patients, with only 20% of the normal increase (26,30,31,32). These results indicate that the altered glucose response relates to an impaired A response to stress rather than cortisol levels.…”

Section: European Journal Of Endocrinologysupporting

confidence: 55%

“…Levels of A in both infants and adults with CAH was significantly lower than in healthy subjects (22,23,24,25). The glucocorticoid therapy in CAH had little effect on A levels (10,26). Thus, it is conceivable that in acute and intensive stress, cortisol has a permissive role, whereas the capacity for A increase may be the major limiting factor for physical capacity.…”

Section: European Journal Of Endocrinologymentioning

confidence: 96%

Effect of a pre-exercise hydrocortisone dose on short-term physical performance in female patients with primary adrenal failure

Šimůnková

Jovanovic²,

Rostrup³

et al. 2016

European Journal of Endocrinology

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Objective: Many patients with primary adrenal insufficiency (Addison's disease) take extra doses of glucocorticoids during stressful events, but a benefit has not been demonstrated in controlled trials. Here, we investigated the effects of a pre-exercise hydrocortisone dose on cardiorespiratory, hormonal and metabolic parameters in response to short-term strenuous physical activity. Design: This was a randomized placebo-controlled, cross-over clinical trial. Participants: Ten women with Addison's disease and 10 age-matched healthy females participated in the study. Measurements: All women in the study underwent maximal incremental exercise testing. A stress dose of 10 mg hydrocortisone or placebo was given 1 h prior to exercise on two occasions. Blood samples were drawn before, and 0, 15 and 30 min post exercise. Oxygen uptake, maximal aerobic capacity, endocrine and metabolic responses to physical activity, as well as health status by questionnaires were evaluated. Results: Maximal aerobic capacity and duration of exercise were significantly lower in patients than in healthy subjects and did not improve with the treatment. After an extra hydrocortisone dose serum cortisol was significantly higher than in the healthy subjects (P!0.001). Post-exercise glucose and adrenaline levels were significantly lower and free fatty acids insignificantly higher in patients irrespective of stress dose. Stress dosing did not alter other metabolic or hormonal parameters or quality of life after the exercise. Conclusions: The patients did not benefit from an extra dose of hydrocortisone in short strenuous exercise. Stress dosing may not be justified in this setting. Whether stress dosing is beneficial in other types of physical activity will have to be examined further.

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“…Menurut Charmandari dkk, 14 glukokortikoid sebaiknya diberikan saat aksis hipotalamus-hipofisis-adrenal sedang aktif, yakni pukul 04.00-16.00, dengan dosis terbesar pada pagi hari. Dosis glukokortikoid juga harus disesuaikan saat pasien melakukan aktivitas fisis 15 dan saat pubertas, 16,17 karena kecukupan dosis glukokortikoid pada masa pubertas sangat menentukan DMT pada masa dewasa. 18 Sebagian besar subjek memiliki DMT normal.…”

Section: Pembahasanunclassified

Karakteristik Densitas Tulang Anak dengan Hiperplasia Adrenal Kongenital yang Mendapat Terapi Glukokortikoid

Widodo¹,

Batubara²,

Ifran³

et al. 2016

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Hasil. Tigapuluh dua subjek, 25 perempuan dan 7 lelaki, 18 dengan HAK tipe virilisasi sederhana dan 14 tipe salt-losing, diikutsertakan dalam penelitian, median usia 6 tahun. Diagnosis 24 subjek ditegakkan pada usia <1 tahun, tipe salt-losing terdiagnosis pada usia lebih muda. Semua subjek memiliki status gizi baik hingga obesitas, dan 29/32 subjek memiliki status pubertas sesuai usia. Semua pasien HAK mendapat terapi glukokortikoid teratur sejak saat diagnosis, dengan median dosis 17,7 mg/m 2 /hari atau 3,8 gram dalam 6 bulan terakhir, dan rerata lama pengobatan 7,7 tahun. Terapi mineralokortikoid pada subjek dengan median dosis 50 mcg/hari. Ditemukan DMT normal pada 24/32 subjek, 7 osteopenia, dan 1 osteoporosis. Delapan di antara pasien dengan DMT normal, memiliki Z-score >+1. Rerata Z-score DMT L1-L4 subjek +0,29 (SB 1,46). Terdapat korelasi lemah antara DMT dengan dosis kumulatif glukokortikoid enam bulan terakhir (r= -0,36; p=0,04), dan tidak ditemukan korelasi dengan dosis glukokortikoid/LPB/hari (r= -0,29; p=0,11) maupun dengan durasi terapi (r= -0,07; p=0,69). Kesimpulan. Sebagian besar anak HAK yang mendapat terapi substitusi glukokortikoid memiliki DMT normal. Terdapat korelasi lemah antara DMT dengan dosis kumulatif glukokortikoid enam bulan terakhir, sehingga diperlukan penelitian lebih lanjut dengan durasi dosis kumulatif yang berbeda-beda. Sari Pediatri 2011;12(5):307-14.Kata kunci: hiperplasia adrenal kongenital, glukokortikoid, densitas mineral tulang, anak, dual energy x-ray absorptiometry

show abstract

Patients with Classic Congenital Adrenal Hyperplasia Have Decreased Epinephrine Reserve and Defective Glycemic Control during Prolonged Moderate-Intensity Exercise

Abstract: CAH patients have defective glycemic control and altered metabolic and hormonal responses during prolonged moderate-intensity exercise comparable to brisk walking.

Cited by 44 publications

References 25 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

Effect of a pre-exercise hydrocortisone dose on short-term physical performance in female patients with primary adrenal failure

Karakteristik Densitas Tulang Anak dengan Hiperplasia Adrenal Kongenital yang Mendapat Terapi Glukokortikoid

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