Health Status of Adults with Congenital Adrenal Hyperplasia: A Cohort Study of 203 Patients

Arlt, Wiebke; Willis, Debbie; Wild, Sarah H.; Krone, Nils; Doherty, Emma J.; Hahner, Stefanie; Han, Thang S.; Carroll, Paul; Conway, Gerard; Rees, Aled; Stimson, Roland H; Walker, Brian R.; Connell, John; Ross, Richard

doi:10.1210/jc.2010-0917

Cited by 444 publications

(599 citation statements)

References 39 publications

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“…Many studies have demonstrated elevated BMI (9,15,16,20,23) in adults with CAH. Measurement of body composition using DXA has previously demonstrated increased fat mass in young adults with CAH (15, 16).…”

Section: Discussionmentioning

confidence: 99%

“…Our patients had increased insulin release during the OGTT and older patients tended to have higher HbA1c than younger ones, suggesting an increased risk of T2DM in the older CAH males. Insulin resistance has been found in adult CAH by all investigators (6,8,9,12,13,18,(20)(21)(22)(23), but one (18) and has most commonly been expressed as homeostasis model assessment (HOMA) index. We did not calculate this index because fasting glucose was low in our patients, which may lead to a falsely low estimation of insulin resistance.…”

Section: Discussionmentioning

confidence: 99%

“…Previous reports on cardiovascular and metabolic profiles in CAH adults have mainly included young females, in spite of the fact that males in general have a higher cardiovascular risk, and the results have been contradictory (6,8,9,(11)(12)(13)(14)(15)(16)(17)(18)(19)(20)(21)(22). A very recent publication described however a cohort of 203 individuals with CAH including 62 males reporting adverse metabolic profiles (23).…”

Section: Introductionmentioning

confidence: 99%

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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: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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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

View full text Add to dashboard Cite

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“…19 Surgical interventions may sometimes be necessary to tackle elevated gonadal cancer risks or urinary issues. 16,36 These surgical interventions should not be contentious, but firm supporting data are lacking.…”

Section: Medicalisationmentioning

confidence: 99%

The human rights of intersex people: addressing harmful practices and rhetoric of change

Carpenter

2016

Reproductive Health Matters

108

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Intersex people and bodies have been considered incapable of integration into society. Medical interventions on often healthy bodies remain the norm, addressing perceived familial and cultural demands, despite concerns about necessity, outcomes, conduct and consent. A global and decentralised intersex movement pursues simple core goals: the rights to bodily autonomy and self-determination, and an end to stigmatisation. The international human rights system is responding with an array of new policy statements from human rights institutions and a handful of national governments recognising the rights of intersex people. However, major challenges remain to implement those statements. Human rights violations of intersex individuals persist, deeply embedded in a deliberate history of silencing. Rhetoric of change to clinical practices remain unsubstantiated. Policy disjunctions arise in a framing of intersex issues as matters of sexual orientation and gender identity, rather than innate sex characteristics; this has led to a rhetoric of inclusion that is not matched by the reality.This paper provides an overview of harmful practices on intersex bodies, human rights developments, and rhetorics of change and inclusion.

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“…The importance of ensuring transitional care is highlighted, especially for CAH -see the paper by Arlt in the adrenal Yearbook chapter [4] -as many adults are not under follow-up. There are many adverse health outcomes in CAH when good surveillance is lacking.…”

Section: Testicular Adrenal Rest Tumors In Boys Adolescents and Adulmentioning

confidence: 99%

Evidence-Based Medicine in Pediatric Endocrinology

Butler¹,

Williams²,

O’Riordan³

2011

Yearbook of Pediatric Endocrinology 2011

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c Leicester Royal Infirmary, Leicester University Hospitals Trust, Leicester, UK As this is an evidence-based medicine chapter, by definition we aim for the integration of best research evidence with clinical expertise and patient values [1] and strive to apply this evidence to all our decisions in clinical practice in pediatric endocrinology. As before, we have included a selection of the best evidence-based medicine papers across the breadth of this specialty with the primary aim of informing and supporting you -the clinicians -to provide the best clinical care for your patients. In addition to this evidence, we refer you to the consensus statements and guidelines that ESPE and other endocrine societies have published over the past year. Although we have included rigorous studies on the metabolic outcome of growth hormone (GH) treatment, as much of the information on GH safety currently comes from preliminary cohort analyses where the evidence of potential risks is not yet of sufficient power, we have not discussed these, but refer you to presentations at the ESPE meeting itself. Reliable evidence is likely to be produced in subsequent years. Group. jdrfapp@jaeb.org Diabetes Care 2010;33:17-22. Background: The authors aimed to determine whether continuous glucose monitoring (CGM) is effective in the management of type 1 diabetes when implemented in clinical practice. Methods: A 6-month randomized controlled trial (RCT) was undertaken to evaluate CGM in children, adolescents, and adults with type 1 diabetes. CGM was commenced in patients with an outpatient training session, two follow-up phone calls, and outpatient visits at 1, 4, 13, and 26 weeks. The control group had a less intensive training and follow-up. Those with starting HbA1c >/=7.0%, the main aim was change in HbA1c at 6 months. Mechanism of the yearResults: The frequency of use of CGM decreased from 7.0 days/week in the first month in the > or =25-year-old group, 6.3 days/week in the 15-to 24-year-olds, and 6.8 days/week in the 8-to 14-yearolds to 6.5, 3.3, and 3.7 days/week at the end of the study period, respectively (p < 0.001 for all age groups). Those with a starting HbA1c >/=7.0% demonstrated a reduction in HbA1c with CGM in all groups at 6 months (p = 0.02). Severe hypoglycemia decreased from 27.7 events per 100 person-years (in control group 6 months) to 15.0 events per 100 person-years in the 6-month follow-up CGM phase (p = 0.08). Conclusions: Frequent use of CGM in a clinical care setting improves HbA1c and also reduces hypoglycemia. Sustained frequent use of CGM is less likely in children and adolescents. 194Gary Butler/Carrie Williams/Stephen O'Riordan Quality-of-life measures in children and adults with type 1 diabetes: Juvenile Diabetes Research Foundation Continuous Glucose Monitoring randomized trialLawrence JM, Beck RW, Laffel L, Wysocki T, Xing D, Huang ES, Ives B, Kollman C, Lee J, Ruedy KJ, Tamborlane WV Juvenile Diabetes Research Foundation Continuous Glucose Monitoring Study Group. Diabetes Care 2010;33:2175-77. Back...

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Health Status of Adults with Congenital Adrenal Hyperplasia: A Cohort Study of 203 Patients

Cited by 444 publications

References 39 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

The human rights of intersex people: addressing harmful practices and rhetoric of change

Evidence-Based Medicine in Pediatric Endocrinology

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