Quality of life is less impaired in adults with congenital adrenal hyperplasia because of 21-hydroxylase deficiency than in patients with primary adrenal insufficiency

Reisch, Nicole; Hahner, Stefanie; Bleicken, Benjamin; Flade, Linda; Gil, Francesco Pedrosa; Loeffler, Melanie; Ventz, Manfred; Hinz, Andreas; Beuschlein, Felix; Allolio, Bruno; Reincke, Martín; Quinkler, Marcus

doi:10.1111/j.1365-2265.2010.03920.x

Cited by 69 publications

(66 citation statements)

References 35 publications

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“…However, there are no studies on personality traits in patients with PAI. To date, studies in patients with PAI have demonstrated impaired QoL (9,10,11,12,13,14,15), and a higher risk for the development of affective disorders such as depression or bipolar disorder (16). The QoL impairments in patients with PAI have been attributed, at least in part, to intrinsic imperfections of hormone replacement therapy (12).…”

Section: Introductionmentioning

confidence: 99%

Psychological morbidity and impaired quality of life in patients with stable treatment for primary adrenal insufficiency: cross-sectional study and review of the literature

Tiemensma¹,

Andela²,

Kaptein³

et al. 2014

European Journal of Endocrinology

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Context: A high prevalence of psychological morbidity and maladaptive personality as well as impaired quality of life (QoL) is observed in patients with and without hydrocortisone dependency following (cured) Cushing's syndrome. However, it is currently unclear whether a similar pattern is present in patients with chronic glucocorticoid replacement for primary adrenal insufficiency (PAI). Objective: To evaluate psychological functioning, personality traits, and QoL in patients with PAI. Design and subjects: A cross-sectional study including 54 patients with stable treatment for PAI and 54 healthy matched controls. Both patients and controls completed questionnaires on psychological functioning (Apathy Scale, Irritability Scale, Mood and Anxiety Symptoms Questionnaire short form, and Hospital Anxiety and Depression Scale), personality traits (Dimensional Assessment of Personality Pathology short form), and QoL (Multidimensional Fatigue Inventory, Short Form 36, EuroQoL-5D, Nottingham Health Profile, and Physical Symptom Checklist). Results: Patients with PAI suffered from more psychological morbidity (i.e. irritability and somatic arousal) and QoL impairments compared with controls (all P!0.01). There were no differences regarding maladaptive personality traits between patients and controls. However, there was a strong and consistent positive association between the daily hydrocortisone dose and prevalence of maladaptive personality traits (i.e. identity problems, cognitive distortion, compulsivity, restricted expression, callousness, oppositionality, rejection, conduct problems, social avoidance, narcissism, and insecure attachment, all P!0.05). There was also a strong relation between the mean daily hydrocortisone dose and both psychological morbidity (i.e. depression, P!0.05) and QoL impairments (i.e. general health perception, several measures of physical functioning, and vitality, all P!0.05). Conclusion: Patients on stable glucocorticoid replacement therapy for PAI report psychological morbidity and impaired QoL. Psychological morbidity, impaired QoL, and maladaptive personality traits were all associated with higher dosages of hydrocortisone.

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Section: Introductionmentioning

confidence: 99%

Psychological morbidity and impaired quality of life in patients with stable treatment for primary adrenal insufficiency: cross-sectional study and review of the literature

Tiemensma¹,

Andela²,

Kaptein³

et al. 2014

European Journal of Endocrinology

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“…I undersøkelser av helserelatert livskvalitet er det ulike resultater, men de største studiene, fra Norge, Storbritannia og Tyskland, viser signifikant lavere livskvalitet enn i den øvrige befolkningen (3,12,18).…”

Section: Helserelatert Livskvalitetunclassified

Klassisk medfødt binyrebarkhyperplasi

Nermoen¹,

Husebye²,

Myhre³

et al. 2017

Tidsskriftet

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“…Current data on HRQoL in adult patients with 21-OHD shows conflicting results [11][12][13][14], however the most recent studies showed impaired HRQoL also in 21-OHD patients [15][16][17].…”

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confidence: 97%

“…For medical treatment of 21-OHD standard medications are hydrocortisone (HC), prednisolone (PR) and dexamethasone (DX) [16,18]. Since those glucocorticoids have a different biological strength, dosage for PR and DX were converted into milligrams of hydrocortisone equivalent (PR was converted 1 to 5 to HC, DX 1 to 70 to HC) [19,20].…”

Section: Subjectsmentioning

confidence: 99%

Improvement of health-related quality of life in adult women with 21-hydroxylase deficiency over a seven-year period

et al. 2012

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Mutations in the gene encoding 21-hydroxylase cause the most common form of congenital adrenal hyperplasia (CAH) [1,2]. Clinical management aims at replacement therapy with glucocorticoids and mineralocorticoids to correct hypocortisolism, hypoaldosteronism and to normalize hyperandrogenism, which often results in higher glucocorticoid doses than in primary adrenal insufficiency (PAI) [3]. Physicians face the problem to adjust to ideal dosage of glucocorticoids in order to avoid under-as well as overtreatment [4,5]. Undertreatment may result in higher risk of adrenal crisis, disturbed pubertal development, reduced final height, infertility, and androgen-driven insulin resistance, however overtreatment results in obesity, impaired glucose homeostasis, infertility and reduced bone mineral density (BMD) [1]. However, the best regimen and approach to Improvement of health-related quality of life in adult women with 21-hydroxylase deficiency over a seven-year period Adjustment for age and sex was performed by transformation of score values into age-and sex-adjusted Z-scores using data sets from respective normative groups. Data regarding glucocorticoid therapy, clinical and hormonal parameters were assessed. We found that two of eight scales of SF-12 showed a significant improvement and four of eight scales a positive trend to better scores. No significant changes were seen in scores for HADS or for steroid hormone levels. Daily hydrocortisone equivalent dose per body surface significantly decreased over the study period. No changes in BMI were observed over the study period. We conclude that improvement of HRQoL in adult female 21-OHD patients is possible. Several factors might be involved in this improvement including reduced daily hydrocortisone equivalent dose per body surface.

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Quality of life is less impaired in adults with congenital adrenal hyperplasia because of 21-hydroxylase deficiency than in patients with primary adrenal insufficiency

Abstract: HRQoL in CAH is only mildly impaired and significantly less than in PAI patients. Differences between PAI and CAH in HRQoL suggest relevant modulating factors of HRQoL other than hormone replacement therapy itself.

Cited by 69 publications

References 35 publications

Psychological morbidity and impaired quality of life in patients with stable treatment for primary adrenal insufficiency: cross-sectional study and review of the literature

Psychological morbidity and impaired quality of life in patients with stable treatment for primary adrenal insufficiency: cross-sectional study and review of the literature

Klassisk medfødt binyrebarkhyperplasi

Improvement of health-related quality of life in adult women with 21-hydroxylase deficiency over a seven-year period

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