scholarly journals Cardiovascular risk factors in children and adolescents with congenital adrenal hyperplasia due to 21-hydroxylase deficiency

2013 ◽  
Vol 80 (4) ◽  
pp. 471-477 ◽  
Author(s):  
Anbezhil Subbarayan ◽  
Mehul T. Dattani ◽  
Catherine J. Peters ◽  
Peter C. Hindmarsh
Keyword(s):  
Risk Factors ◽  
Cardiovascular Risk ◽  
Congenital Adrenal Hyperplasia ◽  
Children And Adolescents ◽  
Cardiovascular Risk Factors ◽  
Adrenal Hyperplasia ◽  
Hydroxylase Deficiency ◽  
21 Hydroxylase Deficiency

Metabolic profile, cardiovascular risk factors and health-related quality of life in children, adolescents and young adults with congenital adrenal hyperplasia

2019 ◽  
Vol 32 (8) ◽  
pp. 871-877 ◽  
Author(s):  
Roopa Vijayan ◽  
Nisha Bhavani ◽  
Praveen V. Pavithran ◽  
Vasantha Nair ◽  
Usha V. Menon ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Risk Factors ◽  
Cardiovascular Risk ◽  
Congenital Adrenal Hyperplasia ◽  
Cardiovascular Risk Factors ◽  
Metabolic Profile ◽  
Good Control ◽  
Adrenal Hyperplasia ◽  
Age And Sex

Abstract Background The present study was designed to evaluate the metabolic profile, cardiovascular risk factors and quality of life in children with congenital adrenal hyperplasia (CAH) and compare it with age- and sex-matched controls. Methods Fifty-two patients aged 3–21 years with classic CAH due to 21-hydroxylase deficiency were included in the study. Metabolic profiling was done for 36 cases and compared with 28 healthy age- and sex-matched controls. Quality of life was assessed in all 52 children and their parents using a validated Pediatric Quality of Life Inventory (PedsQL) questionnaire and was compared with normative data from the same population. Results The median age was 12 years with 14 (27%) males and 38 (73%) females. Out of the total 52 patients, 35 (67%) had salt wasting and 17 (33%) had simple virilising CAH. The median height standard deviation score (SDS) of cases was similar to that of controls (−0.72 vs. −0.64, p = 0.57) and 81% of females had normal pubertal status indicating a good control of the disease. Weight SDS, body mass index (BMI) SDS, mean diastolic blood pressure and insulin resistance were significantly higher in cases when compared to controls (0.31 vs. −0.3; 0.96 vs. 0.17; 67.8 ± 10.49 vs. 61 ± 8.49 and 2.1 vs. 0.95, respectively). The quality of life was significantly reduced in all domains as per parents’ perspective, whereas the children reported reduced quality of social and school functioning. There was no significant correlation between quality of life and metabolic parameters. Conclusions Children with CAH despite a reasonably good control of the disease have a higher cardiovascular risk and reduced quality of life when compared to healthy controls.

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

2011 ◽  
Vol 164 (2) ◽  
pp. 285-293 ◽  
Author(s):  
Henrik Falhammar ◽  
Helena Filipsson Nyström ◽  
Anna Wedell ◽  
Marja Thorén
Keyword(s):  
Cardiovascular Risk ◽  
Congenital Adrenal Hyperplasia ◽  
Older Patients ◽  
Liver Function Tests ◽  
Oral Glucose ◽  
Adult Males ◽  
Adrenal Hyperplasia ◽  
Hydroxylase Deficiency ◽  
Increased Risk ◽  
21 Hydroxylase Deficiency

ObjectiveLifelong 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 methodsWe compared CAH males (n=30), 19–67 years old, with age- and sex-matched controls (n=32). 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.ResultsCAH 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. γ-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.ConclusionsIndications of increased risk were found in CAH males ≥30 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.

Sign in / Sign up

Export Citation Format

Share Document