Psychological adjustment in children and adults with congenital adrenal hyperplasia

Berenbaum, Sheri A.; Bryk, Kristina; Duck, Stephen C.; Resnick, Susan M.

doi:10.1016/j.jpeds.2004.03.037

Cited by 69 publications

(75 citation statements)

References 37 publications

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“…Gender atypical behaviour has been described in young girls as well as in women with CAH due to a CYP21A2 deficiency (7,9). The consequences for psychological functioning and well-being in adult life are not well known (8,(10)(11)(12)(13). To our knowledge, only two previous studies addressed subjective health status in men with CAH.…”

Section: Introductionmentioning

confidence: 96%

“…To our knowledge, only two previous studies addressed subjective health status in men with CAH. One study showed favourable subjective health status in 16 Finnish men aged 16 years or more (11), and the other showed unfavourable emotionality scores in adolescent and adult males with CAH (8).…”

Section: Introductionmentioning

confidence: 97%

“…The long-term outcome of the disease and its various treatment strategies have been addressed in a few studies, mostly in females (3,7,8). Gender atypical behaviour has been described in young girls as well as in women with CAH due to a CYP21A2 deficiency (7,9).…”

Section: Introductionmentioning

confidence: 99%

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Subjective health status in men and women with congenital adrenal hyperplasia: a population-based survey in Norway

Nermoen

Husebye

Svartberg

et al. 2010

European Journal of Endocrinology

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Background: Patients with classical congenital adrenal hyperplasia (CAH) require life-long corticosteroid therapy, with uncertain health outcome. Investigations of subjective health status in unselected populations of adult patients are needed. Objective: To identify all adult Norwegian patients with CAH and obtain population-based data on subjective and psychological health status, working ability and fertility. Patients, methods and design: Classical CAH patients were identified through search in electronic diagnosis registries at all the university hospitals in Norway. The diagnosis was verified by scrutiny of medical records. The patients were invited to a questionnaire survey including medical history, and the Short Form-36 (SF-36) and Quality of Life Scale questionnaires. The questionnaire responses and fertility data were compared with normative data. Results: We identified 104 adult patients (101 alive) with classical CAH (63% female), yielding overall incidence at 1/20 000 live births (1/16 000 in females). Seventy-two (72%) responded; median age 38 years (range 18-72). All the SF-36 scales were significantly impaired, most pronounced for general health and vitality perception. Working disability was reported by 19% of the patients, compared with 10% in the general population. The female patients were often single, and the CAH women had only 21% of the expected number of children compared with the general population. Conclusion: In this population-based survey of patients with classical CAH, we found that subjective health status and working ability were impaired, and that fertility was reduced in females. There is a need for improvement of the medical treatment and the general care of this patient group.

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Section: Introductionmentioning

confidence: 96%

Section: Introductionmentioning

confidence: 97%

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Subjective health status in men and women with congenital adrenal hyperplasia: a population-based survey in Norway

Nermoen

Husebye

Svartberg

et al. 2010

European Journal of Endocrinology

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“…The time which the initial diagnosis was suspected was too late for his parents to accept a new gender and he was lost to follow up. It has been shown that early diagnosis and treatment of CAH in a severally virilized female allows better psychological adjustment apart from preserving fertility (5). If the diagnosis is late, it is a dilemma whether to change the gender because of possible female fertility or to remove the female organs and continue to rear the child as a male.…”

Section: Discussionmentioning

confidence: 99%

A young patient with severe virilization, hypertension and bilateral adrenal hyperplasia

Abhayaratna

Somasundaram

2014

Sri Lanka J. Diabetes Endocrinol. Metab.

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A young patient with severe virilization, hypertension and bilateral adrenal hyperplasia A young patient with severe virilization, hypertension and bilateral adrenal hyperplasia AbstractWe describe an unusual late presentation of 11β-hydroxylase deficiency in a severely virilized, 23 year old patient, who presented with intracerebral haemorrhage, hypertension and short stature. The patient was raised as a male but had hypospadias, absent testicles from birth and had precocious puberty during his childhood. Plasma testosterone level was elevated (16 ng/mL) with suppressed FSH and LH levels (< 1 U/L). Basal 17-hydoxyprogesterone was markedly elevated (>19.2 ng/mL-ref range: 0.5-2.1 ng/mL). CT scan abdomen showed bilateral marked adrenal hyperplasia with mullerian structures and karyotyping showed 46 XX with negative sex-determining region Y (SRY) test. A clinical diagnosis of 11β-hydroxylase deficiency was made in view of hypertension with severe virilization in a 46 XX individual. The patient was managed with antihypertensive drugs, monitoring of serum testosterone and 17-hydroxyprogesterone levels since she the patient decided to remain as a male.

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“…Congenital adrenal hyperplasia results in women with enhanced spatial performance [4], as does a lifelong deficiency in gonadotropin-releasing hormone (GnRH) in men [5].…”

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confidence: 99%

The effect of rapid and depot testosterone and estradiol on spatial performance in water maze

Hodosy¹,

Ostatníková

Riljak

et al. 2012

Open Life Sciences

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The effect of rapid and depot testosterone and estradiol on spatial performance in water maze of receptor types. So, particular brain regions can be targets for the actions of different classes of steroids. For example, estrogen receptors are thinly distributed in the neocortex of the rat, but are prevalent in preoptic and hypothalamic areas and the anterior pituitary. Also some neurons express receptors for more than one steroid.Authors reported testosterone modified spatial abilities resulting from animal research [3] and clinical studies of individuals exposed prenatally to unusual hormone environments. Congenital adrenal hyperplasia results in women with enhanced spatial performance [4], as does a lifelong deficiency in gonadotropin-releasing hormone (GnRH) in men [5].Cent. Eur. J. Biol. • 7(4) • 2012 • 596-602 DOI: 10.2478/s11535-012-0046-z IntroductionAlthough there is no major difference in performance in current standardized intelligence tests between females and males, there are described sex differences in particular subtypes of the tests [1]. These have been reported in certain cognitive tasks for many years, and it appears that testosterone (TST) and other androgens play a crucial role [2]. Spatial learning and memory have been one of the major topics of interest since the late seventies. Keywords: Water maze • Spatial performance • Testosterone • Estradiol • Hippocampus • RatsAbstract: Men and women differ in some cognitive functions including spatial abilities. These differences seem to be affected by sex steroids, but the results are controversial. The aim of this work is to describe the effects of rapid or depot testosterone and estradiol on spatial memory in rats. Thirty-two adult male Wistar rats were divided into 6 groups. Five groups were gonadectomized, and one group was left as control. Castrated groups received sterile oil, testosterone isobutyras, testosterone propionate, estradiol dipropionate or estradiol benzoate. We evaluated spatial performance (escape latency, overall improvement, and time in the quadrant after platform removal) of the rats in a spatial water maze. Animals receiving exogenous sex steroids showed higher plasma concentrations of the particular hormones. Experimental groups improved during the acquisition spatial trials in the water maze. No significant differences between the groups during probe trial were found. In overall improvement, the testosterone depot and estradiol depot groups showed less improvement in comparison to the control groups (P<0.05). No differences in respect to administered hormones were found in corresponding receptor gene expression in hippocampus. In conclusion, exogenous testosterone affects spatial memory of adult castrated males.

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Psychological adjustment in children and adults with congenital adrenal hyperplasia

Cited by 69 publications

References 37 publications

Subjective health status in men and women with congenital adrenal hyperplasia: a population-based survey in Norway

Subjective health status in men and women with congenital adrenal hyperplasia: a population-based survey in Norway

A young patient with severe virilization, hypertension and bilateral adrenal hyperplasia

The effect of rapid and depot testosterone and estradiol on spatial performance in water maze

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