Substitution therapy in adult patients with congenital adrenal hyperplasia

Reisch, Nicole

doi:10.1016/j.beem.2014.11.002

Cited by 22 publications

(15 citation statements)

References 88 publications

(82 reference statements)

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“…Doses of fludrocortisone can be substantially reduced in adults, and may no longer be required in some patients, even those with salt-losing forms of CAH37). However, optimal dosage of fludrocortisone in adults has not been studied, and the adequacy of mineralocorticoid supplementation must be monitored by observing blood pressure and plasma renin activity2).…”

Section: Medical Treatment Of Adolescents and Adults With 21-hydroxylmentioning

confidence: 99%

“…In such cases, serum progesterone levels should be maintained below 0.6 ng/mL38). In the third trimester of pregnancy, glucocorticoid dose may be increased up to 50%37). During labor and delivery, stress doses of glucocorticoids should be administered; however, there are no controlled studies or guidelines regarding optimal doses of glucocorticoids at this point2).…”

Section: Management Of Women With 21-hydroxylase Deficiency During Prmentioning

confidence: 99%

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Management issues of congenital adrenal hyperplasia during the transition from pediatric to adult care

Choi

Yoo

2017

Korean J Pediatr

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Steroid 21-hydroxylase deficiency is the most prevalent form of congenital adrenal hyperplasia (CAH), accounting for approximately 95% of cases. With the advent of newborn screening and hormone replacement therapy, most children with CAH survive into adulthood. Adolescents and adults with CAH experience a number of complications, including short stature, obesity, infertility, tumor, osteoporosis, and reduced quality of life. Transition from pediatric to adult care and management of long-term complications are challenging for both patients and health-care providers. Psychosocial issues frequently affect adherence to glucocorticoid treatment. Therefore, the safe transition of adolescents to adult care requires regular follow-up of patients by a multidisciplinary team including pediatric and adult endocrinologists. The major goals for management of adults with 21-hydroxylase deficiency are to minimize the long-term complications of glucocorticoid therapy, reduce hyperandrogenism, prevent adrenal or testicular adrenal rest tumors, maintain fertility, and improve quality of life. Optimized medical or surgical treatment strategies should be developed through coordinated care, both during transition periods and throughout patients' lifetimes. This review will summarize current knowledge on the management of adults with CAH, and suggested appropriate approaches to the transition from pediatric to adult care.

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Section: Medical Treatment Of Adolescents and Adults With 21-hydroxylmentioning

confidence: 99%

Section: Management Of Women With 21-hydroxylase Deficiency During Prmentioning

confidence: 99%

Management issues of congenital adrenal hyperplasia during the transition from pediatric to adult care

Choi

Yoo

2017

Korean J Pediatr

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“…Hydrocortisone is regarded with a relative potency of 1 and dexametasone with 30 times higher. However, research studies and clinical experience showed that the androgen-suppressing effect of dexametasoneis even 70-100 times higher, and therefore the potential for over treatment remains high [16,17]. Authors suggested an incorrect dosage because of a wrong assumption of its potency as a main cause of the myth of 'growth toxic' glucocorticoid [18].…”

Section: Discussionmentioning

confidence: 99%

A follow-up history of young man with apparent cortisone reductase deficiency (ACRD) – several years after diagnosis

Zajkowska

Rydzewska

Wojtkielewicz

et al. 2017

Pediatr Endocrino Diabetes Metab

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“…Treatment in classic 21OHD is necessary to compensate for GC and MC deficiencies and also to correct adrenal androgen excess. ideally, the treatment should be monitored to avoid iatrogenic comorbidities and to enable a good quality of life (60). However, this goal is not reached up to now as increased comorbidities and mortality are reported in patients with CAH.…”

Section: Treatment Of Classic Cah In Adultsmentioning

confidence: 99%

MANAGEMENT OF ENDOCRINE DISEASE: Congenital adrenal hyperplasia due to 21-hydroxylase deficiency: update on the management of adult patients and prenatal treatment

Bachelot

Grouthier

Courtillot

et al. 2017

European Journal of Endocrinology

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Congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency is characterized by cortisol and in some cases aldosterone deficiency associated with androgen excess. Goals of treatment are to replace deficient hormones and control androgen excess, while avoiding the adverse effects of exogenous glucocorticoid. Over the last 5 years, cohorts of adults with CAH due to 21-hydroxylase deficiency from Europe and the United States have been described, allowing us to have a better knowledge of long-term complications of the disease and its treatment. Patients with CAH have increased mortality, morbidity and risk for infertility and metabolic disorders. These comorbidities are due in part to the drawbacks of the currently available glucocorticoid therapy. Consequently, novel therapies are being developed and studied in an attempt to improve patient outcomes. New management strategies in the care of pregnancies at risk for congenital adrenal hyperplasia using fetal sex determination and dexamethasone have also been described, but remain a subject of debate. We focused the present overview on the data published in the last 5 years, concentrating on studies dealing with cardiovascular risk, fertility, treatment and prenatal management in adults with classic CAH to provide the reader with an updated review on this rapidly evolving field of knowledge.

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Substitution therapy in adult patients with congenital adrenal hyperplasia

Cited by 22 publications

References 88 publications

Management issues of congenital adrenal hyperplasia during the transition from pediatric to adult care

Management issues of congenital adrenal hyperplasia during the transition from pediatric to adult care

A follow-up history of young man with apparent cortisone reductase deficiency (ACRD) – several years after diagnosis

MANAGEMENT OF ENDOCRINE DISEASE: Congenital adrenal hyperplasia due to 21-hydroxylase deficiency: update on the management of adult patients and prenatal treatment

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