Adult height after GH therapy in 188 Ullrich-Turner syndrome patients: results of the German IGLU Follow-up Study 2001

Abstract

Objectives: We aimed to evaluate the factors influencing true adult height (HT) after long-term (from 1987 to 2000) GH treatment in Ullrich -Turner syndrome (UTS) based on modalities conceived in the 1980s. Design: Out of 347 near-adult (.16 years) patients from 96 German centres, whose longitudinal growth was documented within KIGS (Pharmacia International Growth Database), 188 (45, X ¼ 59%; bone age .15 years) were available for further anthropometric measurements. Results: At a median GH dose of 0.88 (10th/90th percentiles: 0.47/1.06) IU/kg per week, a gain of 6.0 (2 1.3/+13) cm above the projected adult height was recorded. Variables were recorded at GH start, after 1 year GH, puberty onset, and last visit on GH therapy. At these visits, the median ages were 11.7, 12.7, 14.2, 16.6 and 18.7 years; and median heights, 0.4, 1.1, 1.7, 1.7 and 1.3 SDS (UTS) respectively. Height gain (DHT) after GH discontinuation was 1.5 cm. Total DHT correlated ðP , 0:001Þ negatively with bone age and HT SDS at GH start, but positively with DHT after the first year, DHT at puberty onset, and GH duration. Final HT correlated ðP , 0:001Þ positively with HT at GH start, first-year DHT, and HT at puberty onset. Body mass index increased slightly ðP , 0:05Þ; with values at start and adult follow-up correlating highly ðR ¼ 0:70; P , 0:001Þ: No major side effects of GH occurred. Conclusions: GH dosages conceived in the 1980s are safe but too low for most UTS patients. HT gain and height are determined by age and HT at GH start. Height gain during the first year on GH is indicative of overall height gain. After spontaneous or induced puberty, little gain in height occurs.

Cardiovascular anomalies in children and young adults with Ullrich-Turner syndrome-the erlangen experience

“…[1][2][3] In recent years, most medical attention has been focused on the improvement of final height by recombinant human growth hormone therapy. [4][5][6][7] Another important therapeutic goal is substitution with estrogens in order to induce and maintain normal sexual development and to achieve optimal skeletal maturation. Thus, health surveillance in children with UTS was primarily conducted by pediatric endocrinologists.…”

Estatura final (EF) em síndrome de Turner (ST): experiência de 76 casos acompanhados na Unidade de Endocrinologia Pediátrica do Hospital de Clínicas da Universidade Federal do Paraná

“…Um grupo de 35 pacientes recebeu concomitantemente oxandrolona, na dose de 0,05 mg/kg/dia, e iniciou estrogênio após idade óssea de 12 anos. Ao final de 4,5 anos de tratamento, observou-se um ganho de 6 cm em relação à estatura final prevista (152,2 vs. 146,3 cm) (19). Segundo Rosenfeld, o tratamento com GH promove um acréscimo de 8,4 cm na estatura final e, quando associado à oxandrolona, esse acréscimo é de 10,3 cm (17).…”

“…Na análise de regressão múltipla, os principais fatores capazes de influenciar a estatura final das pacientes deste estudo foram o tipo de tratamento (GH + OX) e a estatura materna, semelhantemente ao descrito na literatura (9,16,19,21,33,35). Embora a idade cronoló-gica ao início do tratamento esteja correlacionada com uma maior estatura final (15,16,19,21,33,35), neste estudo não observamos essa correlação.…”

“…Embora a idade cronoló-gica ao início do tratamento esteja correlacionada com uma maior estatura final (15,16,19,21,33,35), neste estudo não observamos essa correlação.…”

“…Inúme-ros trabalhos envolvendo essa combinação têm sido publicados desde meados dos anos 1990 (8,(13)(14)(15)(16)(17)(18)(19)(20)(21)(22) …”