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      A Retrospective Analysis of the Growth Pattern in Patients with Salt-wasting 21-Hydroxylase Deficiency

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          Abstract

          The objective of this study was to investigate the growth pattern of children with the salt-wasting form of congenital adrenal hyperplasia caused by 21-hydroxylase deficiency (21-OHD). We reviewed the medical records of 13 patients in whom salt-wasting 21-OHD was diagnosed during the first 2 mo of life at our hospital from 1980 through 2008. Six reached adult height. Growth patterns, bone age, biochemical data, and the hydrocortisone dose at each growth stage were analyzed retrospectively. The mean adult height was 155.1 ± 6.5 cm (mean ± SD) in females and 158.1 ± 7.1 cm in males. Although length at birth was normal or longer than the national mean in almost all patients, the mean height SD score of both boys and girls decreased to below 0 SD during infancy. Subsequently, both boys and girls transiently showed growth acceleration and reached their peak growth velocity at 3–10 yr of age. In conclusion, in addition to suppression of growth during infancy, there was inappropriate growth acceleration during childhood. Especially from 3 mo to 3 yr of age, decreasing the hydrocortisone dose in patients who exhibit slower growth may lead to satisfactory height outcomes. Also, strict adjustment of the hydrocortisone dose to avoid accelerated growth from childhood to adolescence might improve adult height outcomes of patients with 21-OHD.

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          Congenital adrenal hyperplasia.

          Deborah P. Merke, Stefan R. Bornstein (2005)
          Congenital adrenal hyperplasia (CAH) due to deficiency of 21-hydroxylase is a disorder of the adrenal cortex characterised by cortisol deficiency, with or without aldosterone deficiency, and androgen excess. Patients with the most severe form also have abnormalities of the adrenal medulla and epinephrine deficiency. The severe classic form occurs in one in 15,000 births worldwide, and the mild non-classic form is a common cause of hyperandrogenism. Neonatal screening for CAH and gene-specific prenatal diagnosis are now possible. Standard hormone replacement fails to achieve normal growth and development for many children with CAH, and adults can experience iatrogenic Cushing's syndrome, hyperandrogenism, infertility, or the development of the metabolic syndrome. This Seminar reviews the epidemiology, genetics, pathophysiology, diagnosis, and management of CAH, and provides an overview of clinical challenges and future therapies.
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            Consensus statement on 21-hydroxylase deficiency from the Lawson Wilkins Pediatric Endocrine Society and the European Society for Paediatric Endocrinology.

            Lourdes Ibañez, Peter Clayton (2002)
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              Reduced final height outcome in congenital adrenal hyperplasia under prednisone treatment: deceleration of growth velocity during puberty.

              Susanne Pozza, Dietrich Knorr, Maximilian Schmidt … (2007)
              Normal to decreased final height (FH) has been reported in patients with congenital adrenal hyperplasia (CAH). The objective was to determine FH outcome and influences of steroid treatment. The effects of glucocorticoid treatment for classical CAH were retrospectively studied in 125 patients (77 females). Growth pattern, FH, and pubertal development were recorded. Corrected FH was in the lower range of genetic potential [females with simple virilizing (SV)-CAH, -0.6 +/- 1.0 sd score (SDS) vs. females with salt-wasting (SW)-CAH, -0.6 +/- 0.9 SDS; males with SV-CAH, -1.1 +/- 0.9 SDS vs. males with SW-CAH, -0.9 +/- 0.9 SDS]. Total pubertal growth was significantly reduced in comparison with a reference population (females with SV-CAH, 11.9 +/- 6.5 cm, and females with SW-CAH, 13.8 +/- 7.6 cm vs. reference 20.3 +/- 6.8 cm, P < 0.01; and males with SV-CAH, 15.4 +/- 6.6 cm, and males with SW-CAH, 18.5 +/- 6.9 cm vs. reference 28.2 +/- 8.2 cm, P < 0.01). Thirty-three patients had been treated with prednisone, which resulted in reduced FH compared with patients (n = 92) treated with hydrocortisone (-1.0 +/- 0.9 SDS vs.-0.6 +/- 0.9 SDS; P < 0.05). FH correlated negatively with hydrocortisone dose given at the start of puberty (r = -0.3; P < 0.05). Pubertal development started early in boys [9.8 +/- 2.3 yr (SV) and 10.6 +/- 1.9 yr (SW)] and was timely in girls [9.8 +/- 1.9 yr (SV) and 10.3 +/- 1.5 yr (SW), menarche at 13.3 +/- 1.7 yr (SV) and 13.7 +/- 1.5 yr (SV)]. Patients with CAH are able to achieve adequate FH with conventional therapy. Total pubertal growth is significantly decreased, and treatment with prednisone results in decreased FH. In addition to biochemical analysis, treatment should be adjusted to normal growth velocity, especially during puberty.
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                Author and article information

                Journal
                Journal ID (nlm-ta): Clin Pediatr Endocrinol
                Journal ID (iso-abbrev): Clin Pediatr Endocrinol
                Journal ID (publisher-id): CPE
                Title: Clinical Pediatric Endocrinology
                Publisher: The Japanese Society for Pediatric Endocrinology
                ISSN (Print): 0918-5739
                ISSN (Electronic): 1347-7358
                Publication date (Electronic): 22 April 2014
                Publication date (Print): April 2014
                Volume: 23
                Issue: 2
                Pages: 27-34
                Affiliations
                [1 ] Department of Endocrinology and Metabolism, Fukuoka Children’s Hospital, Fukuoka, Japan
                Author notes
                Corresponding author: Dr. Atsuko Kawano, Department of Endocrinology and Metabolism, Fukuoka Children’s Hospital, 2-5-1 Tojin-machi, Chuoku, Fukuoka 810-0063, Japan. E-mail: sasaki.a@ 123456fcho.jp
                Article
                Publisher ID: 9975
                DOI: 10.1297/cpe.23.27
                PMC ID: 4004995
                PubMed ID: 24790384
                SO-VID: eef2bd06-69ec-4df9-b796-1b8523554a16
                Copyright © 2014©The Japanese Society for Pediatric Endocrinology
                License:

                This is an open-access article distributed under the terms of the Creative Commons Attribution Non-Commercial No Derivatives (by-nc-nd) License.

                History
                Date received : 17 July 2013
                Date accepted : 12 December 2013
                Categories
                Subject: Original Article

                Keywords: growth,congenital adrenal hyperplasia,21-hydroxylase deficiency,childhood
                Data availability:
                Keywords: growth, congenital adrenal hyperplasia, 21-hydroxylase deficiency, childhood

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