Adult height in girls with Turner syndrome treated from before 6 years of age with a fixed per kilogram GH dose

Abstract

Objective: To evaluate adult height (AH) in 25 girls with Turner syndrome (TS) who were treated from before 6 years of age for 10.0G1.7 years with a fixed GH dose of 0.33 mg/kg per week. Patients and design: After a 6-month pretreatment assessment all patients were measured 6-monthly under therapy to assess height SDS (H-SDS) and height velocity (HV) until AH achievement. Results: Following initial acceleration, HV declined after the first 4 years of therapy. At the end of the sixth year of therapy, H-SDS gain was 1.9G1.1. Thereafter, H-SDS gain from baseline decreased, becoming 0.9G0.9 SDS at AH achievement. Bone maturation velocity did not significantly change throughout the prepubertal period. According to Lyon standards for TS, mean AH SDS was significantly higher than pretreatment H-SDS (P!0.0001), with a mean H-SDS change of 0.9G0.9. However, the prevalence of patients with AH !K2 SDS (according to Sempé standards) was close to those recorded at the start of therapy (16/25 vs 18/25). No significant differences in terms of AH were found between patients with either X monosomy or X-chromosomal abnormalities and between girls with either spontaneous or induced puberty. Conclusions: We infer that the therapeutic regimen adopted in this prospective study is sufficient to induce a significant growth acceleration during the first year, but the response waned after 6 years of treatment.

Evaluation of the results of Turner syndrome patients during the first three years of recombinant human growth hormone therapy

“…Methods to improve the growth of women with Turner syndrome are still being sought. There is a noteworthy study in which a group of patients took growth hormone together with children's low-dose estrogen [4,6,[18][19][20][21]. Greater height gain values were observed in these girls than in patients who received rHGH alone.…”

“…The authors explain this dependence with the ability of estrogens to directly increase the response of the skeletal growth plates to IGF-1 or growth hormone. Currently, low dose estrogen therapy is implemented at the age at which the puberty process is supposed to begin, and after the next two years, progesterone is added [18][19][20][21]. Promising effects are observed with the use of growth hormone and oxandrolone (an anabolic steroid) at a dose of 0.03 and 0.05 mg / kg / day.…”

Diagnostic and therapeutic considerations in Turner syndrome

“…Skeletal disproportion in TS includes the following phenotypes: micrognathia, high-arched palate, short fourth metacarpals, genu valgum, Madelung wrist deformities, and short limbs. Short stature, recognized by Lemli and Smith [ 3 ] as a ubiquitous abnormality, is now effectively treated with early initiation of recombinant growth hormone (GH) therapy [ 17 - 19 ]. Achieving as normal a height as possible through GH therapy has become standard [ 20 ].…”

“…Individual sensitivity to recombinant human GH is known to be variable28). GH induces significant growth acceleration during the first year, but the response wanes over time [ 18 , 19 , 27 ]. Specific growth-related genetic markers are associated with growth response in TS [ 29 , 30 ].…”

Treatment of children with Turner’s syndrome with recombinant growth hormone

“…Існує значна індивідуальна мінливість відповіді на терапію рГР. Низкою досліджень показано, що варіативність ростової відповіді у дівчат із СШТ на лікування рГР залежить від безлічі чинників: генетично детермінованого зросту, ступеня затримки росту, своєчасного початку і тривалості лікування, наявності самостійного статевого розвитку, а також від генетичних маркерів і профілю експресії генів [2,8,9,10]. Рекомендована початкова доза рГР становить 0,05 мг/кг на добу (0,375 мг/кг на тиж) [1,5].…”