Normalization of height and excess body fat in children with salt-wasting 21-hydroxylase deficiency

Mendes‐dos‐Santos, Carolina T.; Lemos-Marini, Sofia Helena Valente de; Baptista, Mónica; Guerra, Gil; De-Mello, Maricilda P; Paulino, Maria Fernanda Vanti Macedo; Morcillo, André Moreno

doi:10.2223/jped.2095

Cited by 8 publications

(9 citation statements)

References 33 publications

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“…Body composition of children with CAH is an emerging area of focus, and recent data indicate that body mass index (BMI) and/or fat mass is elevated in children and adults with CAH . In normal growth, BMI increases rapidly in infancy and peaks at around the first year of life.…”

Section: Introductionmentioning

confidence: 99%

Obesity in children with congenital adrenal hyperplasia in the Minnesota cohort: importance of adjusting body mass index for height‐age

Sarafoglou

Forlenza

Addo

et al. 2017

Clinical Endocrinology

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Summary Objectives To evaluate obesity and overweight in children with congenital adrenal hyperplasia (CAH) and associations with glucocorticoids, fludrocortisone and disease control. Adjusting body mass index-for-height-age (BMIHA) percentile is proposed to correct misclassification of obese/overweight status in CAH children with advanced bone age and tall-for-age stature. Design Longitudinal. Patients One hundred and ninety-four children with CAH seen from 1970 to 2013: 124 salt wasting (SW); 70 simple virilizing (SV); 102 females. Measurements Body mass index (BMI) end-points were overweight (85–94 percentile) and obese (≥95 percentile). Results Approximately 50% of the children had at least one BMI measurement ≥95 percentile and about 70% had at least one ≥85 percentile. Using BMIHA percentiles, obesity incidence decreased slightly in SW children (47–43%) and markedly in SV children (50–33%); however, overweight status was not reduced. Only 6% of SW and 1% of SV children were persistently obese (≥3 clinic visits) when BMIHA was applied, whereas overweight status persisted in 35% of SW and 33% of SV children. Most obesity or overweight when using BMIHA occurred before age 10 and there was no association with hydrocortisone (HC) or fludrocortisone dosing. Adiposity rebound for SW children occurred by 3.3 years and in SV females by age 3.8 years, over a year earlier than the adiposity rebound for healthy children. Conclusion Children with CAH are at higher risk for early onset obesity and overweight with or without using BMIHA but rates of persistent obesity were lower than previously reported. Careful HC dosing during early childhood is needed to prevent increased weight gain and an early adiposity rebound.

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Section: Introductionmentioning

confidence: 99%

Obesity in children with congenital adrenal hyperplasia in the Minnesota cohort: importance of adjusting body mass index for height‐age

Sarafoglou

Forlenza

Addo

et al. 2017

Clinical Endocrinology

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“…al, 2011; Cetinkaya & Kara, 2011; Moreira et al, 2013). Medes-dos-Santos et al (2011) concluded in their cross sectional, retrospective study that height in these children was similar to unaffected children of the same age, yet children with CAH had higher body fat, which tended to be visceral. Similarly, Moreira et al (2013) found that children with CAH presented with a higher prevalence of obesity and metabolic syndrome, but child weight was not correlated with glucocorticoid treatment.…”

Section: Resultsmentioning

confidence: 94%

“…Of the ten, five articles focused on growth issues, both height and weight. Obesity was found to be a significant problem in children with CAH (Volkl, Simm, Beier, & Dorr, 2006; Mendes-dos-Santos et. al, 2011; Cetinkaya & Kara, 2011; Moreira et al, 2013).…”

Section: Resultsmentioning

confidence: 99%

Management of Childhood Congenital Adrenal Hyperplasia—An Integrative Review of the Literature

Fleming¹,

Riper²,

Knafl³

2017

Journal of Pediatric Health Care

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Congenital adrenal hyperplasia (CAH) is an endocrine condition that requires parents to administer steroids up to three times daily, supplementing maintenance doses with oral or injectable doses during times of illness. The purpose of this integrative review was to explore the management, care and associated health issues for children with CAH as well as the family response to the condition. Four literature indexes were searched with 39 articles included. Four themes emerged: 1) health and development related issues; 2) effects of excess androgens; 3) life experience of CAH; and 4) managing and averting adrenal crisis. Families having a child with CAH face complex concerns related their child’s growth, the fear of adrenal crisis events, and the consequences of atypical genitalia for affected girls. Future studies should focus on interventions that provide guidelines to increase parental preparedness in managing adrenal crises as well as creating support systems for affected girls.

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“…Therefore, the goal of therapy in SW-21OHD patients is to normalize the androgen levels and total-body sodium depletion to allow normal growth and development and avoid volume overload (16). In our study, the required glucocorticoid dose (adjusted per square meter) was stable during the first two years of life.…”

Section: Discussionmentioning

confidence: 99%

Mineralocorticoid replacement during infancy for salt wasting congenital adrenal hyperplasia due to 21-hydroxylase deficiency

Gomes

Madureira

Mendonca

et al. 2013

Clinics

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OBJECTIVE:The protocols for glucocorticoid replacement in children with salt wasting 21-hydroxylase deficiency are well established; however, the current recommendation for mineralocorticoid replacement is general and suggests individualized dose adjustments. This study aims to retrospectively review the 9-∝-fludrocortisone dose regimen in salt wasting 21-hydroxylase deficient children who have been adequately treated during infancy.METHODS:Twenty-three salt wasting 21-hydroxylase deficient patients with good anthropometric and hormonal control were followed in our center since diagnosis. The assessments of cortisone acetate and 9-∝-fludrocortisone doses, anthropometric parameters, and biochemical and hormonal levels were rigorously evaluated in pre-determined intervals from diagnosis to two years of age.RESULTS:The 9-∝-fludrocortisone doses decreased over time during the first and second years of life; the median fludrocortisone doses were 200 μg at 0-6 months, 150 μg at 7-18 months and 125 μg at 19-24 months. The cortisone acetate dose per square meter was stable during follow-up (median = 16.8 mg/m2/day). The serum sodium, potassium and plasma rennin activity levels during treatment were normal, except in the first month of life, when periodic 9-∝-fludrocortisone dose adjustments were made.CONCLUSIONS:The mineralocorticoid needs of salt wasting 21-hydroxylase deficient patients are greater during early infancy and progressively decrease during the first two years of life, which confirms that a partial aldosterone resistance exists during this time. Our study proposes a safety regiment for mineralocorticoid replacement during this critical developmental period.

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Normalization of height and excess body fat in children with salt-wasting 21-hydroxylase deficiency

Cited by 8 publications

References 33 publications

Obesity in children with congenital adrenal hyperplasia in the Minnesota cohort: importance of adjusting body mass index for height‐age

Obesity in children with congenital adrenal hyperplasia in the Minnesota cohort: importance of adjusting body mass index for height‐age

Management of Childhood Congenital Adrenal Hyperplasia—An Integrative Review of the Literature

Mineralocorticoid replacement during infancy for salt wasting congenital adrenal hyperplasia due to 21-hydroxylase deficiency

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