Skeletal maturity of children with multiple osteochondromas: is diminished stature due to a systemic influence?

Journal of Children's Orthopaedics
Heleen M StaalLodewijk W van Rhijn

Abstract

Multiple ostechondromas (MO) is an autosomal dominant inherited disease caused by mutated exostosin genes. It mostly affects the long bones and can lead to growth disturbances, especially disproportionate short stature. Both the local effect on growth plates and the systemic influence of the gene disorder on growth mechanisms might explain the diminished stature. The hypothesis of this study is that the diminished stature in adults with MO is due to a systemic influence, leading to early skeletal maturation and early closure of the growth plate. Therefore, in these patients the skeletal age in adolescence is hypothesized to be higher than the calendar age. Radiographs of the left hand were collected from 50 MO-affected children. The skeletal age was calculated using these radiographs according to the Greulich-Pyle bone scale and was compared to the calendar age at the time of radiography. Children aged 3-12 years had a significantly lower skeletal age compared to their calendar age (p = 0.030). Children aged 12-17 years had a significantly higher skeletal age (p = 0.019), especially boys. Skeletal maturation in children with MO therefore differs from their peers. In this study, the skeletal age in younger children with MO is lo...Continue Reading

References

Apr 1, 1991·Journal of Medical Genetics·R C Hennekam
Jan 2, 1995·American Journal of Medical Genetics·C L WicklundJ T Hecht
Jul 1, 1994·The Journal of Bone and Joint Surgery. American Volume·G A SchmaleW H Raskind
May 18, 1999·Developmental Biology·S PathiA Vortkamp
Sep 8, 2000·Journal of Bone and Mineral Research : the Official Journal of the American Society for Bone and Mineral Research·M Enomoto-IwamotoM Iwamoto
Nov 1, 1961·The Journal of Bone and Joint Surgery. British Volume·L SOLOMON
Sep 28, 2004·The Journal of Bone and Joint Surgery. British Volume·D E PorterA H R W Simpson
Jun 14, 2005·The Journal of Clinical Investigation·Tatsuya KobayashiHenry M Kronenberg
Apr 6, 2007·Proceedings of the National Academy of Sciences of the United States of America·Yukiko MaedaBeate Lanske
Dec 22, 2010·Proceedings of the National Academy of Sciences of the United States of America·Takao HiraiHenry M Kronenberg
Dec 6, 2011·Journal of Pediatric Orthopedics. Part B·Nicholas David ClementDaniel E Porter
Jan 20, 2012·The Journal of Bone and Joint Surgery. American Volume·Elena PedriniLuca Sangiorgi
Nov 30, 2012·Journal of Orthopaedic Research : Official Publication of the Orthopaedic Research Society·Kevin B JonesMario R Capecchi

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Citations

Jun 12, 2020·Italian Journal of Pediatrics·Andrea TrombettaGianluca Tornese

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