Perspectives on growth promoting treatment for patients with Turner syndrome in Japan

Clinical Pediatric Endocrinology : Case Reports and Clinical Investigations : Official Journal of the Japanese Society for Pediatric Endocrinology
Toshiaki Tanaka

Abstract

In Japan, anabolic steroid hormone (ASH) treatment for Turner syndrome (TS) to promote growth had been provided before GH therapy for TS was approved. ASH effectively improved the adult height (AH) of TS patients without spontaneous puberty but decreased the AH of TS patients with spontaneous puberty. Although GH therapy for TS was approved in 1991, the approved dosage remained 0.5 IU/kg/wk for GH-deficient TS patients and improved AH by approximately 7 cm. However, AH did not reach -2 standard deviations in healthy girls. In 1999, the requirement of GH deficiency was removed and a dose of 1.0 IU/kg/wk was approved. Although an increase in AH was expected, no reports showed significant improvements in AH at a high dose of GH. GH + ASH combination therapy was reevaluated and recommended for TS patients with gonadal failure and an extremely short stature or those who respond poorly to GH therapy. Although early estrogen replacement therapy is recommended to improve psychological quality of life and prevent osteoporosis, it lowered AH even at a low dose of ethinyl estradiol (25 ng/kg/d). The initiation of ethynyl estradiol at an extremely low dose (1-5 ng/kg/d) at a relatively young age successfully improved AH.

References

Apr 1, 1992·Acta Paediatrica Japonica; Overseas Edition·S Suwa
Mar 1, 1991·Jinrui idengaku zasshi. The Japanese journal of human genetics·T MaedaN Yabe
Oct 20, 1994·The New England Journal of Medicine·E P SmithK S Korach
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Sep 29, 2005·The Journal of Clinical Endocrinology and Metabolism·Robert L RosenfieldAllen W Root
Oct 19, 2006·The Journal of Clinical Endocrinology and Metabolism·Carolyn A Bondy, UNKNOWN Turner Syndrome Study Group
Jan 12, 2010·The Journal of Clinical Endocrinology and Metabolism·Leonie A MenkeJan M Wit
Apr 1, 2011·The New England Journal of Medicine·Judith L RossGordon B Cutler
Apr 16, 2011·BMJ : British Medical Journal·Emma Jane GaultUNKNOWN British Society for Paediatric Endocrinology and Diabetes
May 17, 2013·The Journal of Clinical Endocrinology and Metabolism·L Torres-SantiagoN Mauras
Jul 1, 2010·Clinical Pediatric Endocrinology : Case Reports and Clinical Investigations : Official Journal of the Japanese Society for Pediatric Endocrinology·Keinosuke FujitaToshiaki Tanaka
Jan 1, 2008·Clinical Pediatric Endocrinology : Case Reports and Clinical Investigations : Official Journal of the Japanese Society for Pediatric Endocrinology·Toshiaki TanakaMari Satoh
Nov 17, 2015·Clinical Pediatric Endocrinology : Case Reports and Clinical Investigations : Official Journal of the Japanese Society for Pediatric Endocrinology·Toshiaki TanakaSusumu Yokoya
May 21, 2016·Frontiers in Endocrinology·Maria Felicia FaienzaGiacomina Brunetti
Jun 6, 2019·Hormone Research in Pædiatrics·Malcolm DonaldsonUNKNOWN on behalf of the European Society for Paediatric Endocrinology Turner Syndrome Working Group

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