Statural Growth in 31 Japanese Patients with SHOX Haploinsufficiency: Support for a Disadvantageous Effect of Gonadal Estrogens

DOI Web Site PubMed 参考文献19件
  • FUKAMI Maki
    Department of Endocrinology and Metabolism, National Research Institute for Child Health and Development
  • NISHI Yoshikazu
    Department of Pediatrics, Hiroshima Red-Cross Hospital
  • HASEGAWA Yukihiro
    Division of Endocrinology and Metabolism, Tokyo Metropolitan Kiyose Children's Hospital
  • MIYOSHI Yoko
    Department of Pediatrics, Osaka University School of Medicine
  • OKABE Takashi
    Department of Pediatrics, Toyama Municipal Hospital
  • HAGA Nobuhiko
    Department of Orthopedics, Shizuoka Children's Hospital
  • NAGAI Toshiro
    Department of Pediatrics, Dokkyo University School of Medicine Koshigaya Hospital
  • TANAKA Toshiaki
    Division of Endocrinology and Metabolism, National Research Center for Child Health and Development
  • OGATA Tsutomu
    Department of Endocrinology and Metabolism, National Research Institute for Child Health and Development

この論文をさがす

抄録

Although gonadal estrogens are known to facilitate the development of skeletal lesion in SHOX haploinsufficiency, controversy exists as to whether gonadal estrogens are disadvantageous to pubertal growth. To clarify this matter, we analyzed growth pattern in 31 Japanese patients with a normal karyotype and molecularly confirmed SHOX haploinsufficiency. The mean height SD score at the diagnosis of SHOX haploinsufficiency was similar between patients identified in childhood and those identified in adulthood (–2.7 ± 0.8 [n = 15] vs. –2.4 ± 0.7 [n = 16], P = 0.36), and was significantly lower in patients identified by the studies for short stature than in those ascertained by the familial studies of the probands both in childhood (–3.0 ± 0.6 [n = 11] vs. –1.8 ± 0.5 [n = 4], P = 0.0051) and in adulthood (–3.0 ± 0.9 [n = 5] vs. –2.2 ± 0.5 [n = 11], P = 0.040). Analysis of longitudinal paired growth data obtained in seven females showed a significantly different mean height SD score between childhood and adulthood (–2.3 ± 0.5 vs. –2.9 ± 0.8, P = 0.0060). The results imply that gonadal estrogens have a deleterious effect on pubertal growth in SHOX haploinsufficiency, and that the growth disadvantage is recognizable by longitudinal rather than cross-sectional growth studies.<br>

収録刊行物

  • Endocrine Journal

    Endocrine Journal 51 (2), 197-200, 2004

    一般社団法人 日本内分泌学会

参考文献 (19)*注記

もっと見る

キーワード

詳細情報 詳細情報について

問題の指摘

ページトップへ