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Journal of Pediatric Endocrinology and Metabolism

Editor-in-Chief: Kiess, Wieland

Ed. by Bereket, Abdullah / Darendeliler, Feyza / Dattani, Mehul / Gustafsson, Jan / Luo, Fei Hong / Mericq, Veronica / Toppari, Jorma


IMPACT FACTOR 2018: 1.239

CiteScore 2018: 1.22

SCImago Journal Rank (SJR) 2018: 0.507
Source Normalized Impact per Paper (SNIP) 2018: 0.562

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2191-0251
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Volume 29, Issue 7

Issues

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
  • Department of Pediatrics, The First Affiliated Hospital, Sun Yat-Sen University, Guangzhou, P.R.China
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/ Ma Huamei
  • Corresponding author
  • Department of Pediatrics, The First Affiliated Hospital, Sun Yat-Sen University, No. 58, Zhongshan II Rd., Guangzhou 510080, P.R. China, Phone: +86 20 87755766 8310, Fax: +86 20 87750632
  • Email
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/ Su Zhe / Li Yanhong
  • Department of Pediatrics, The First Affiliated Hospital, Sun Yat-Sen University, Guangzhou, P.R.China
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/ Chen Hongshan
  • Department of Pediatrics, The First Affiliated Hospital, Sun Yat-Sen University, Guangzhou, P.R.China
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/ Chen Qiuli
  • Department of Pediatrics, The First Affiliated Hospital, Sun Yat-Sen University, Guangzhou, P.R.China
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/ Zhang Jun
  • Department of Pediatrics, The First Affiliated Hospital, Sun Yat-Sen University, Guangzhou, P.R.China
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/ Guo Song
  • Department of Pediatrics, The First Affiliated Hospital, Sun Yat-Sen University, Guangzhou, P.R.China
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/ Du Minlian
  • Department of Pediatrics, The First Affiliated Hospital, Sun Yat-Sen University, Guangzhou, P.R.China
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Published Online: 2016-04-07 | DOI: https://doi.org/10.1515/jpem-2015-0406

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.

Keywords: congenital adrenal hyperplasia; 21-hydroxylase deficiency; near final height; target height

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About the article

Received: 2015-10-12

Accepted: 2016-01-22

Published Online: 2016-04-07

Published in Print: 2016-07-01


Author contributions: All the authors have accepted responsibility for the entire content of this submitted manuscript and approved submission.

Research funding: None declared.

Employment or leadership: None declared.

Honorarium: None declared.

Competing interests: The funding organization(s) played no role in the study design; in the collection, analysis, and interpretation of data; in the writing of the report; or in the decision to submit the report for publication.


Citation Information: Journal of Pediatric Endocrinology and Metabolism, Volume 29, Issue 7, Pages 841–848, ISSN (Online) 2191-0251, ISSN (Print) 0334-018X, DOI: https://doi.org/10.1515/jpem-2015-0406.

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