期刊
HORMONE RESEARCH IN PAEDIATRICS
卷 89, 期 3, 页码 200-204出版社
KARGER
DOI: 10.1159/000486336
关键词
IGF-1; rhIGF-1; PAPP-A2; Bone density; Bone structure
资金
- Fondos de Investigacion Sanitaria
- FEDER [PI1302195, PI1600485]
- Ministerio de Ciencia e Innovacion [BFU2014-51836-C2-2-R]
- Centro de Investigacion Biomedica en Red Fisiopatologia de Obesidad y Nutricion (CIBEROBN)
- Instituto de Salud Carlos III
- Fundacion Endocrinologia y Nutricion
Aim: Our objective was to determine changes in bone mineral density (BMD), trabecular bone score (TBS), and body composition after 2 years of therapy with recombinant human insulin-like growth factor-1 (rhIGF-1) in 2 prepubertal children with a complete lack of circulating PAPP-A2 due to a homozygous mutation in PAPP-A2 (p.D643fs25*) resulting in a premature stop codon. Methods: Body composition, BMD, and bone structure were determined by dual-energy X-ray absorptiometry at baseline and after 1 and 2 years of rhIGF-1 treatment. Results: Height increased from 132 to 145.5 cm (patient 1) and from 111.5 to 124.5 cm (patient 2). Bone mineral content increased from 933.40 to 1,057.97 and 1,152.77 g in patient 1, and from 696.12 to 773.26 and 911.51 g in patient 2, after 1 and 2 years, respectively. Whole-body BMD also increased after 2 years of rhIGF-1 from base-line 0.788 to 0.869 g/cm(2) in patient 1 and from 0.763 to 0.829 g/cm(2) in patient 2. After 2 years of treatment, both children had an improvement in TBS. During therapy, a slight increase in body fat mass was seen, with a concomitant increase in lean mass. No adverse effects were reported. Conclusion: Two years of rhIGF-1 improved growth, with a tendency to improve bone mass and bone microstructure and to modulate body composition. (C) 2018 S. Karger AG, Basel.
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