Literature DB >> 27054598

Near-final height in 82 Chinese patients with congenital adrenal hyperplasia due to classic 21-hydroxylase deficiency: a single-center study from China.

Lin Juan, Ma Huamei, Su Zhe, Li Yanhong, Chen Hongshan, Chen Qiuli, Zhang Jun, Guo Song, Du Minlian.   

Abstract

BACKGROUND: The objective of this study was to identify variables that might interfere with reaching the near final height (NFH) in Congenital adrenal hyperplasia (CAH) due to classic 21-hydroxylase deficiency (21-OHD).
METHODS: A cross-sectional study of 82 (24 males and 58 females) classic (23 salt-wasting form [SW] and 59 simple-virilizing form [SV]) CAH 21-OHD patients seen in our institution between 1989 and 2015 with 10.6 (0.5~25.5) years of follow-up who reached their NFH was conducted. The variables related to NFH were explored.
RESULTS: NFH (153.35±8.31) cm, (-1.9±1.1) SD was significantly lower than the normal population (p<0.001). The treated patients reached a significantly higher NFH (-1.7±1.1) SD than those untreated (-2.6±1.0) SD (p<0.05). Both of early treatment and late treatment group were taller than untreated group (p<0.001, p=0.013, respectively), and early treatment group had a taller height trend than late treatment group (p=0.089). A better height outcome was observed in patients with advantage in target height, good compliance, and low hydrocortisone dose by multivariate Cox regression analysis in 62 treatment patients. NFH and hydrocortisone dose was negatively correlated (r=-0.23, p=0.078) in treated group. Patients complicated by central precocious puberty (CPP) received gonadotropin-releasing hormone analogue (GnRHa) plus letrozole had increased NFH with height SD for bone age and Ht SD improved after treatment compare to no intervention group (p=0.001, p=0.035).
CONCLUSIONS: Patients with classic 21-OHD have blunted final height, as compared with their target height and the population norm, not-treated even worse. Careful treatment adjustments have a favorable influence on growth. Alternative treatments, such as the use of puberty inhibitors GnRHa in addition to anti-estrogen therapy letrozole can somewhat improve NFH in children with 21-OHD complicated by CPP.

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Year:  2016        PMID: 27054598     DOI: 10.1515/jpem-2015-0406

Source DB:  PubMed          Journal:  J Pediatr Endocrinol Metab        ISSN: 0334-018X            Impact factor:   1.634


  7 in total

1.  Growth Pattern and Clinical Profile of Indian Children with Classical 21-Hydroxylase Deficiency Congenital Adrenal Hyperplasia on Treatment.

Authors:  H Meena; Manisha Jana; Vishwajeet Singh; Madhulika Kabra; Vandana Jain
Journal:  Indian J Pediatr       Date:  2019-01-30       Impact factor: 1.967

2.  Aromatase Inhibitor as Treatment for Severely Advanced Bone Age in Congenital Adrenal Hyperplasia: A Case Report.

Authors:  Wesley J Goedegebuure; Anita C S Hokken-Koelega
Journal:  Horm Res Paediatr       Date:  2019-08-07       Impact factor: 2.852

3.  [Efficacy of letrozole in treatment of children with congenital adrenal hyperplasia due to steroid 21-hydroxylase deficiency].

Authors:  Qian Wang; Shule Zhang; Xue Ma; Guimei Li; Zengmin Wang; Fengxue Wang
Journal:  Zhejiang Da Xue Xue Bao Yi Xue Ban       Date:  2020-05-25

4.  Adult height in pubertal boys with short stature treated with GH/letrozole: a hospital record-based retrospective study.

Authors:  Yaping Ma; Ruofan Jia; Bingyang Xia; Bin Tang; Zhuangjian Xu
Journal:  BMC Pediatr       Date:  2022-06-28       Impact factor: 2.567

Review 5.  Clinical outcomes and characteristics of P30L mutations in congenital adrenal hyperplasia due to 21-hydroxylase deficiency.

Authors:  Mirjana Kocova; Violeta Anastasovska; Henrik Falhammar
Journal:  Endocrine       Date:  2020-05-05       Impact factor: 3.633

6.  Histrelin Implantation and Growth Outcomes in Children With Congenital Adrenal Hyperplasia: An Institutional Experience.

Authors:  Robert A Swendiman; Barbara E Coons; Craig A Alter; Vaneeta Bamba; Michael L Nance; Maria G Vogiatzi
Journal:  J Endocr Soc       Date:  2019-11-13

Review 7.  Should Skeletal Maturation Be Manipulated for Extra Height Gain?

Authors:  Jan M Wit
Journal:  Front Endocrinol (Lausanne)       Date:  2021-12-16       Impact factor: 5.555

  7 in total

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