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Long-term Growth in Congenital Adrenal Hyperplasia
Ankita Maheshwari1, Vaman Khadilkar1, Priyanka Gangodkar1
1Growth and Pediatric Endocrine Unit, Hirabai Cowasji Jehangir Medical Research Institute, Jehangir Hospital, 32 Sassoon Road, Pune, 411001, India.
Insights
Growth in children with congenital adrenal hyperplasia (CAH) is significantly impacted by puberty. Careful monitoring of growth and pubertal development in pediatric CAH patients is crucial for achieving optimal final height outcomes.
Area of Science:
- Pediatric endocrinology
- Genetics and inherited conditions
Background:
- Congenital adrenal hyperplasia (CAH) is a group of rare genetic disorders affecting the adrenal glands.
- Classic CAH presents with significant hormonal imbalances, impacting growth and development.
- Early diagnosis and management are essential for improving patient outcomes.
Purpose of the Study:
- To retrospectively evaluate the growth patterns of children diagnosed with classic congenital adrenal hyperplasia (CAH).
- To specifically assess the influence of puberty on growth and final height in pediatric CAH patients.
- To analyze longitudinal growth data and final achieved height in a cohort of children with CAH.
Main Methods:
- Retrospective analysis of 30 children (14 boys) with classic CAH.
- Data collected included height Z scores, target height Z scores, height velocities, and relevant laboratory parameters.
- Study period spanned December 2002 to December 2016, with a mean follow-up of 9.9 years.
Main Results:
- Children received hydrocortisone treatment; mean 17-hydroxyprogesterone levels were analyzed.
- Central precocious puberty developed in 15 children, with 13 receiving GnRH analogue treatment.
- Eighteen children reached final height, with mean final heights below target height, and attenuated growth velocity during puberty.
Conclusions:
- Final height achieved by children with CAH was not significantly different from their target height Z scores.
- Growth velocity was observed to be attenuated during pubertal years in this cohort.
- Continuous monitoring of growth and pubertal progression is vital for optimizing final height in pediatric CAH patients.
Objectives:
To retrospectively assess growth of children with congenital adrenal hyperplasia (CAH) with special reference to puberty and to assess longitudinal growth and final height of subset of children with CAH.
Methods:
A retrospective analysis of 30 children (14 boys) with classic CAH (11 salt wasters, 19 simple virilisers) followed up for a mean duration of 9.9 ± 2.4 y (Study period December 2002 through December 2016) was performed. Height Z scores, target height Z scores, height velocities and laboratory parameters were analysed.
Results:
Children were treated with hydrocortisone in a mean dose of 15.7 ± 3.3 mg/m2/d. Mean 17-hydroxy progesterone in boys and girls were 10.8 ± 6.7 ng/ml and 11.3 ± 9.3 ng/ml respectively. Fifteen children (7 boys) developed central precocious puberty at mean age of 7.6 ± 1.8 y and 13 were treated with GnRH analogues for 3.5 y. Of all patients, 18 (10 girls, 8 boys) reached final height at a mean age of 14.2 ± 1.6 y. Mean final height achieved was 158.0 ± 8.5 cm in boys [target height (TH) -165.5 ± 3.8 cm] and in girls it was 149.9 ± 6.7 cm [target height (TH) 154.7 ± 6.4 cm]. Final height standard deviation scores (SDS) for boys and girls were - 2.06 ± 1.1 (TH-SDS -1.06 ± 0.5) and - 1.47 ± 1.1 (TH-SDS -0.56 ± 1.2) respectively and were not significantly different from target height Z scores (p > 0.05). Growth velocity was attenuated during pubertal years.
Conclusions:
Monitoring growth and puberty in children with CAH is critical for optimizing final height.
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