Major determinants of height development in Turner syndrome (TS) patients treated with GH:: Analysis of 987 patients from KIGS

被引:63
作者
Ranke, Michael B.
Lindberg, Anders
Ferrandez Longas, Angel
Darendeliler, Feyza
Albertsson-Wikland, Kerstin
Dunger, David
Cutfield, Wayne S.
Tauber, Maithe
Wilton, Patrick
Wollmann, Hartmut A.
Reiter, Edward O.
机构
[1] Univ Tubingen, Childrens Hosp, Paediat Endocrinol Sect, D-72076 Tubingen, Germany
[2] Pfizer Inc, S-11287 Stockholm, Sweden
[3] Univ Zaragoza, Childrens Hosp Miguel Servet, Dept Pediat, ESP-50009 Zaragoza, Spain
[4] Istanbul Fac Med, Cocuk Klin, Dept Paediat, TR-34390 Istanbul, Turkey
[5] Univ Gothenburg, Sahlgrenska Acad, Queen Silvia Childrens Hosp, Dept Pediat,Pediat Growth Res Ctr, S-41685 Gothenburg, Sweden
[6] Univ Cambridge, Dept Pediat, Cambridge CB2 2QQ, England
[7] Univ Auckland, Dept Pediat, Auckland 92019, New Zealand
[8] Univ Hosp, Serv Pediat Endocrinol, F-31059 Toulouse, France
[9] Tufts Univ Med, Baystate Med Ctr, Childrens Hosp, Springfield, MA 01199 USA
关键词
D O I
10.1203/01.pdr.0000250039.42000.c9
中图分类号
R72 [儿科学];
学科分类号
100202 ;
摘要
Little is known about factors determining height outcome during GH treatment in Turner syndrome (TS). We investigated 987 TS children within the Kabi International Growth Study (KIGS) who had reached near adult height (NAH) after > 4 y GH treatment (including > 1 y before puberty). Through multiple regression analysis we developed a model for NAH and total gain. Our results were as follows (median): 1) At start, age 9.7 yrs, height (HT) 118.0 cm (0.0 TS SDS), projected adult height 146.1 cm, GH dose 0.27 mg/kg wk; 2) NAH HT 151.0 cm (1.5 TS SDS); 3) Prepubertal gain 21.2 cm (1.6 TS SDS); 4) Pubertal gain 9.4 cm (0.0 TS SDS). NAH correlated (r(2) = 0.67) with (ranked) HT at GH start (+), Is' year responsiveness to GH (+), MPH (+), age at puberty onset (+), age at GH start (-), and dose (+). The same factors explained (R-2 = 0.90) the total HT gain. However, HT at GH start correlated negatively. Karyotype had no influence on outcome. Evidently, height at GH start (the taller, the better), age at GH start (the younger, the better), the responsiveness to GH (the higher, the better) and age at puberty (the later, the better) determine NAH.
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页码:105 / 110
页数:6
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