Literature DB >> 26555758

Height outcome of short children with hypochondroplasia after recombinant human growth hormone treatment: a meta-analysis.

Francesco Massart1, Mario Miccoli2, Angelo Baggiani2, Silvano Bertelloni1.   

Abstract

Hypochondroplasia (HCH) is a genetic skeletal dysplasia, characterized by rhizomelic short height (Ht) with facial dysmorphology and lumbar hyperlordosis. Albeit there are concerns that HCH children may not achieve optimal long-term outcome in response to recombinant human growth hormone (rhGH), anecdotal experiences suggested at least short-term Ht improvement. After thorough search of published studies, meta-analysis of rhGH use in HCH children was performed. In 113 HCH children, rhGH administration (median 0.25 mg/kg/week) progressively improved Ht pattern with 12 months catch-up growth (p < 0.0001). Then, Ht improvement resulted constant until 36 months (p < 0.0001), but stature remained subnormal. While bone age chronologically progressed, no serious adverse events were reported. In conclusion, our meta-analysis indicates that rhGH treatment progressively improved Ht outcome of HCH subjects.

Entities:  

Keywords:  IGF-1; growth hormone therapy; height outcome; hypochondroplasia; meta-analysis; skeletal dysplasia

Mesh:

Substances:

Year:  2015        PMID: 26555758     DOI: 10.2217/pgs.15.129

Source DB:  PubMed          Journal:  Pharmacogenomics        ISSN: 1462-2416            Impact factor:   2.533


  4 in total

Review 1.  Achondroplasia: Development, pathogenesis, and therapy.

Authors:  David M Ornitz; Laurence Legeai-Mallet
Journal:  Dev Dyn       Date:  2017-03-02       Impact factor: 3.780

2.  Adult height after spontaneous pubertal growth or GnRH analog treatment in girls with early puberty: a meta-analysis.

Authors:  Silvano Bertelloni; Francesco Massart; Mario Miccoli; Giampiero I Baroncelli
Journal:  Eur J Pediatr       Date:  2017-04-01       Impact factor: 3.183

3.  Clinical management and emerging therapies of FGFR3-related skeletal dysplasia in childhood.

Authors:  Hwa Young Kim; Jung Min Ko
Journal:  Ann Pediatr Endocrinol Metab       Date:  2022-06-30

4.  Evaluation of Efficacy of Long-term Growth Hormone Therapy in Patients with Hypochondroplasia

Authors:  Tuğba Çetin; Zeynep Şıklar; Pınar Kocaay; Merih Berberoğlu
Journal:  J Clin Res Pediatr Endocrinol       Date:  2018-05-09
  4 in total

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