Bone Mineral Status in Children with Congenital Adrenal Hyperplasia

Fleischman, Amy; Ringelheim, Julie; Feldman, Henry A.; Gordon, Catherine M.

doi:10.1515/jpem.2007.20.2.227

Cited by 23 publications

(28 citation statements)

References 34 publications

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“…In children and young adults, bone mineral density (BMD) was reported to be increased (4), normal (5,6), or decreased (7). Other pediatric studies found decreased BMD in males (8), in long-term treated girls (9), and at puberty (10).…”

Section: Introductionmentioning

confidence: 99%

Bone mineral density, bone markers, and fractures in adult males with congenital adrenal hyperplasia

Falhammar

Nyström

Wedell

et al. 2013

European Journal of Endocrinology

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Objective: The aim of this study was to determine bone mineral density (BMD), markers of bone metabolism, fractures, and steroids reflecting hormonal control in adult males with congenital adrenal hyperplasia (CAH). Subjects, methods, and design: We compared CAH males with 21-hydroxylase deficiency (nZ30), 19-67 years old, with age-and sex-matched controls (nZ32). Subgroups of CYP21A2 genotypes, age, glucocorticoid preparation, poor control vs overtreatment, and early vs late (O36 months) diagnosis were studied. BMD measured by dual energy X-ray absorptiometry and markers of bone metabolism and androgens/17-hydroxyprogesterone levels were investigated. Results: All, including older (O30 years), CAH patients had lower BMD in all measured sites compared with control subjects. The null group demonstrated lower BMD in more locations than the other groups. Osteoporosis/osteopenia was present in 81% of CAH patients compared with 32% in controls (R30 years). Fracture frequency was similar, osteocalcin was lower, and fewer patients than controls had vitamin D insufficiency. IGF1 was elevated in the milder genotypes. In patients, total body BMD was positively correlated to weight, BMI, total lean body mass, and triglycerides, and negatively to prolactin. Patients on prednisolone had lower BMD and osteocalcin levels than those on hydrocortisone/cortisone acetate. Patients with poor control had higher femoral neck BMD. There were no differences in BMD between patients with an early vs late diagnosis. Conclusions: CAH males have low BMD and bone formation markers. BMD should be monitored, adequate prophylaxis and treatment established, and glucocorticoid doses optimized to minimize the risk of future fractures.

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Section: Introductionmentioning

confidence: 99%

Bone mineral density, bone markers, and fractures in adult males with congenital adrenal hyperplasia

Falhammar

Nyström

Wedell

et al. 2013

European Journal of Endocrinology

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“…It is known that osteoporosis risk is higher in adult patients with CAH compared to the normal population (6)(7)(8)(9). However, in younger CAH patients there are contradictory results in published studies and case reports that reported that BMD values were not changed or decreased (10)(11)(12)(13)(14)(15)(16)(17)(18). This situation may be related to smaller numbers of patients, extensive range of age distribution, and heterogeneous glucocorticoid dose among groups.…”

Section: Introductionmentioning

confidence: 99%

Bone mineral density and vitamin D status in children and adolescents with congenital adrenal hyperplasia

Demirel¹,

Kara²,

Tepe³

et al. 2014

Turk J Med Sci

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To determine the prevalence of and risk factors for decreased bone mineral density (BMD) and vitamin D deficiency in children and adolescents with congenital adrenal hyperplasia (CAH). Materials and methods:This study was conducted on 30 girls and 22 boys with CAH (age range = 5-20 years) with median age of 12.0 years. BMD values of lumbar vertebras (L1-L4), which were determined by dual-energy X-ray absorptiometry, were used to calculate z-scores according to chronological age. A serum 25-hydroxyvitamin D level of <15 ng/mL was considered as indicative of vitamin D deficiency.Results: Mean vitamin D level was 14.8 ng/mL in the whole group. Twenty-seven (51.9%) children had vitamin D deficiency and it was more prevalent during pubertal ages. Vitamin D levels were found to be significantly lower in pubertal females. BMD z-score was below -1 standard deviation in 40.1% of cases with significantly higher mean age and lower vitamin D levels. Conclusion:Decreased BMD z-score and vitamin D deficiency were common in these children with CAH. Vitamin D levels were significantly lower in girls and pubertal children. Decreased BMD z-score was related to older age and lower levels of vitamin D. Periodical controls of vitamin D status and vitamin D supplementation were recommended in these cases, whenever required.

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“…Similar results were obtained using calcaneal quantitative ultrasonometry [Stikkelbroeck et al, 2003] and lumbar QCT [Elnecave et al, 2008]. Normal results have been found both in women with CAH [Guo et al, 1996;Mora et al, 1996;Stikkelbroeck et al, 2003] and in girls or female adolescents [Gussinyé et al, 1997;Fleischman et al, 2007;Elnecave et al, 2008, Sahakitrungruang et al, 2008. Conversely, markedly reduced bone mass measurements have been described in other studies [Cameron et al, 1995;Jääskeläinen and Voutilainen, 1996;Girgis and Winter, 1997;Hagenfeldt et al, 2000;Paganini et al, 2000;de Almeida Freire et al, 2003;King et al, 2006;Sciannamblo et al, 2006;Bachelot et al, 2007;Falhammar et al, 2007;Chakhtoura et al, 2008;Zimmermann et al, 2009].…”

Section: XX Dsd: Bone Health In Cahmentioning

confidence: 52%

“…Several studies demonstrated that bone mass measurements of the lumbar spine [Mora et al, 1996;Gussinyé et al, 1997;Stikkelbroeck et al, 2003;Fleischman et al, 2007;Elnecave et al, 2008, Sahakitrungruang et al, 2008, femoral neck [Stikkelbroeck et al, 2003;Fleischman et al, 2007] and the whole skeleton [Guo et al, 1996;Mora et al, 1996;Fleischman et al, 2007;Elnecave et al, 2008, Sahakitrungruang et al, 2008 did not differ significantly from those of healthy controls or reference values. Similar results were obtained using calcaneal quantitative ultrasonometry [Stikkelbroeck et al, 2003] and lumbar QCT [Elnecave et al, 2008].…”

Section: XX Dsd: Bone Health In Cahmentioning

confidence: 99%

“…Few studies investigated bone metabolism rate by measurements of specific biochemical markers of bone formation and bone resorption [Paganini et al, 2000;Sciannamblo et al, 2006;Bachelot et al, 2007;Falhammar et al, 2007;Fleischman et al, 2007;Zimmermann et al, 2009]. Young patients showed low bone formation rate, assessed by bone-specific alkaline phosphatase (BAP) measurements, and high bone resorption rate, assessed by urinary concentration of N-terminal telopeptide of type I collagen (NTx) [Fleischman et al, 2007].…”

Section: XX Dsd: Bone Health In Cahmentioning

confidence: 99%

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Bone Health in Disorders of Sex Differentiation

Bertelloni

Baroncelli

Mora³

2010

Sex Dev

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Sex steroids are main regulators of skeletal growth, maturation and mass in both men and women. People with disorders of sex development (DSD) may experience problems in developing normal bone growth, structure and mass, because abnormal sex steroid secretion or action may be operative. In complete androgen insensitivity syndrome several reports documented reduced bone mineral density (BMD). Reduced BMD is evident in patients with not removed or removed gonads, but it is poorer in the latter, mainly when compliance with estrogen replacement therapy is not guaranteed. Large impairment of BMD does not seem to be present in patients with partial androgen insensitivity syndrome or 5α-reductase-2 deficiency, providing that gonads are not removed or that substitutive therapy is optimized. In congenital adrenal hyperplasia, BMD may be impaired as a result of not optimal glucocorticoid administration. In Turner syndrome, impaired BMD may result from the combined actions of estrogen deficiency, low bone dimensions, altered bone geometry, deficient cortical bone, and trabecular bone loss. Optimal estrogen administration seems to be important in preserving bone mass and enhancing trabecular bone volume. On the whole, bone health represents a main clinical issue for the management of persons with disorders of sex differentiation, and well designed longitudinal studies should be developed to improve their bone health and well-being.

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Bone Mineral Status in Children with Congenital Adrenal Hyperplasia

Abstract: Congenital adrenal hyperplasia (CAH) is caused by a deficiency in an adrenal enzyme resulting in alterations in Cortisol and aldosterone production.

Cited by 23 publications

References 34 publications

Bone mineral density, bone markers, and fractures in adult males with congenital adrenal hyperplasia

Bone mineral density, bone markers, and fractures in adult males with congenital adrenal hyperplasia

Bone mineral density and vitamin D status in children and adolescents with congenital adrenal hyperplasia

Bone Health in Disorders of Sex Differentiation

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