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Health status of adults with congenital adrenal hyperplasia: a cohort study of 203 patients.

Arlt W, Willis DS, Wild SH, Krone N, Doherty EJ, Hahner S, Han TS, Carroll PV, Conway GS, Rees DA, Stimson RH, Walker BR, Connell JM, Ross RJ, United Kingdom Congenital Adrenal Hyperplasia Adult Study Executive (CaHAS - J. Clin. Endocrinol. Metab. (2010)

Bottom Line: Anthropometric measurements were compared with Health Survey for England data, and psychometric data were compared with appropriate reference cohorts.Subjective health status was significantly impaired and fertility compromised.This was associated with an adverse metabolic profile and impaired fertility and quality of life.

View Article: PubMed Central - PubMed

Affiliation: Centre for Endocrinology, Diabetes, and Metabolism, School of Clinical and Experimental Medicine, University of Birmingham, Birmingham, United Kingdom.

ABSTRACT

Context: No consensus exists for management of adults with congenital adrenal hyperplasia (CAH) due to a paucity of data from cohorts of meaningful size.

Objective: Our objective was to establish the health status of adults with CAH.

Design and setting: We conducted a prospective cross-sectional study of adults with CAH attending specialized endocrine centers across the United Kingdom.

Patients: Participants included 203 CAH patients (199 with 21-hydroxylase deficiency): 138 women, 65 men, median age 34 (range 18-69) years.

Main outcome measures: Anthropometric, metabolic, and subjective health status was evaluated. Anthropometric measurements were compared with Health Survey for England data, and psychometric data were compared with appropriate reference cohorts.

Results: Glucocorticoid treatment consisted of hydrocortisone (26%), prednisolone (43%), dexamethasone (19%), or a combination (10%), with reverse circadian administration in 41% of patients. Control of androgens was highly variable with a normal serum androstenedione found in only 36% of patients, whereas 38% had suppressed levels suggesting glucocorticoid overtreatment. In comparison with Health Survey for England participants, CAH patients were significantly shorter and had a higher body mass index, and women with classic CAH had increased diastolic blood pressure. Metabolic abnormalities were common, including obesity (41%), hypercholesterolemia (46%), insulin resistance (29%), osteopenia (40%), and osteoporosis (7%). Subjective health status was significantly impaired and fertility compromised.

Conclusions: Currently, a minority of adult United Kingdom CAH patients appear to be under endocrine specialist care. In the patients studied, glucocorticoid replacement was generally nonphysiological, and androgen levels were poorly controlled. This was associated with an adverse metabolic profile and impaired fertility and quality of life. Improvements in the clinical management of adults with CAH are required.

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Related in: MedlinePlus

Subjective health in status in CAH according to SF-36 (A) and HADS (B) questionnaires and shown for male classic (n = 65), female classic (n = 103), and female nonclassic (n = 31) CAH patients with box plots representing median and interquartile ranges; whiskers represent the 5th and 95th percentiles. A z-score of 0 represents the median of the normal reference population; the lower the SF-36 z-scores and the higher the HADS z-scores, the worse is self-perceived wellbeing and mood. SF-36 dimensions: PF, physical functioning; RP, role limitations due to physical problems; BP, bodily pain; GH, general health; VT, vitality; SF, social functioning; RE, role limitations due to emotional problems; MH, mental health.
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Figure 3: Subjective health in status in CAH according to SF-36 (A) and HADS (B) questionnaires and shown for male classic (n = 65), female classic (n = 103), and female nonclassic (n = 31) CAH patients with box plots representing median and interquartile ranges; whiskers represent the 5th and 95th percentiles. A z-score of 0 represents the median of the normal reference population; the lower the SF-36 z-scores and the higher the HADS z-scores, the worse is self-perceived wellbeing and mood. SF-36 dimensions: PF, physical functioning; RP, role limitations due to physical problems; BP, bodily pain; GH, general health; VT, vitality; SF, social functioning; RE, role limitations due to emotional problems; MH, mental health.

Mentions: Subjective health status was significantly impaired across all eight SF-36 domains in the three CAH subgroups, with most prominent differences to age- and sex-matched controls for the domains general health, vitality, and role limitations due to emotional problems (Table 3 and Fig. 3A). The HADS questionnaire revealed increased anxiety scores for all three CAH subgroups and increased depression scores in patients with classic CAH (Table 4 and Fig. 3B).


Health status of adults with congenital adrenal hyperplasia: a cohort study of 203 patients.

Arlt W, Willis DS, Wild SH, Krone N, Doherty EJ, Hahner S, Han TS, Carroll PV, Conway GS, Rees DA, Stimson RH, Walker BR, Connell JM, Ross RJ, United Kingdom Congenital Adrenal Hyperplasia Adult Study Executive (CaHAS - J. Clin. Endocrinol. Metab. (2010)

Subjective health in status in CAH according to SF-36 (A) and HADS (B) questionnaires and shown for male classic (n = 65), female classic (n = 103), and female nonclassic (n = 31) CAH patients with box plots representing median and interquartile ranges; whiskers represent the 5th and 95th percentiles. A z-score of 0 represents the median of the normal reference population; the lower the SF-36 z-scores and the higher the HADS z-scores, the worse is self-perceived wellbeing and mood. SF-36 dimensions: PF, physical functioning; RP, role limitations due to physical problems; BP, bodily pain; GH, general health; VT, vitality; SF, social functioning; RE, role limitations due to emotional problems; MH, mental health.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3066446&req=5

Figure 3: Subjective health in status in CAH according to SF-36 (A) and HADS (B) questionnaires and shown for male classic (n = 65), female classic (n = 103), and female nonclassic (n = 31) CAH patients with box plots representing median and interquartile ranges; whiskers represent the 5th and 95th percentiles. A z-score of 0 represents the median of the normal reference population; the lower the SF-36 z-scores and the higher the HADS z-scores, the worse is self-perceived wellbeing and mood. SF-36 dimensions: PF, physical functioning; RP, role limitations due to physical problems; BP, bodily pain; GH, general health; VT, vitality; SF, social functioning; RE, role limitations due to emotional problems; MH, mental health.
Mentions: Subjective health status was significantly impaired across all eight SF-36 domains in the three CAH subgroups, with most prominent differences to age- and sex-matched controls for the domains general health, vitality, and role limitations due to emotional problems (Table 3 and Fig. 3A). The HADS questionnaire revealed increased anxiety scores for all three CAH subgroups and increased depression scores in patients with classic CAH (Table 4 and Fig. 3B).

Bottom Line: Anthropometric measurements were compared with Health Survey for England data, and psychometric data were compared with appropriate reference cohorts.Subjective health status was significantly impaired and fertility compromised.This was associated with an adverse metabolic profile and impaired fertility and quality of life.

View Article: PubMed Central - PubMed

Affiliation: Centre for Endocrinology, Diabetes, and Metabolism, School of Clinical and Experimental Medicine, University of Birmingham, Birmingham, United Kingdom.

ABSTRACT

Context: No consensus exists for management of adults with congenital adrenal hyperplasia (CAH) due to a paucity of data from cohorts of meaningful size.

Objective: Our objective was to establish the health status of adults with CAH.

Design and setting: We conducted a prospective cross-sectional study of adults with CAH attending specialized endocrine centers across the United Kingdom.

Patients: Participants included 203 CAH patients (199 with 21-hydroxylase deficiency): 138 women, 65 men, median age 34 (range 18-69) years.

Main outcome measures: Anthropometric, metabolic, and subjective health status was evaluated. Anthropometric measurements were compared with Health Survey for England data, and psychometric data were compared with appropriate reference cohorts.

Results: Glucocorticoid treatment consisted of hydrocortisone (26%), prednisolone (43%), dexamethasone (19%), or a combination (10%), with reverse circadian administration in 41% of patients. Control of androgens was highly variable with a normal serum androstenedione found in only 36% of patients, whereas 38% had suppressed levels suggesting glucocorticoid overtreatment. In comparison with Health Survey for England participants, CAH patients were significantly shorter and had a higher body mass index, and women with classic CAH had increased diastolic blood pressure. Metabolic abnormalities were common, including obesity (41%), hypercholesterolemia (46%), insulin resistance (29%), osteopenia (40%), and osteoporosis (7%). Subjective health status was significantly impaired and fertility compromised.

Conclusions: Currently, a minority of adult United Kingdom CAH patients appear to be under endocrine specialist care. In the patients studied, glucocorticoid replacement was generally nonphysiological, and androgen levels were poorly controlled. This was associated with an adverse metabolic profile and impaired fertility and quality of life. Improvements in the clinical management of adults with CAH are required.

Show MeSH
Related in: MedlinePlus