Adult Height in 299 Patients with Turner Syndrome with or without Growth Hormone Therapy: Results and Literature Review

2021 ◽  
pp. 1-8
Author(s):  
Naiara C.B. Dantas ◽  
Adriana F. Braz ◽  
Alexsandra Malaquias ◽  
Sofia Lemos-Marini ◽  
Ivo J.P. Arnhold ◽  
...  
Keyword(s):  
Growth Hormone ◽  
Turner Syndrome ◽  
Adult Height ◽  
Growth Hormone Therapy ◽  
Treated Group ◽  
Untreated Group ◽  
Target Height ◽  
Gh Therapy ◽  
Gh Treatment ◽  
Different Populations

<b><i>Context:</i></b> Treatment with growth hormone (GH) is considered effective in improving adult height (AH) in Turner syndrome (TS). However, there are few studies comparing AH between treated patients and a concurrent untreated group. <b><i>Objective:</i></b> To assess the efficacy of GH treatment in improving AH in TS and to review previous published studies with treated and untreated groups. <b><i>Participants and Methods:</i></b> We retrospectively analyzed clinical data and AH of a large cohort of GH-treated (<i>n</i> = 168) and untreated (<i>n</i> = 131) patients with TS. Data are shown as median and interquartile range (IQR). We assessed pretreatment variables related with AH and compared our results with 16 studies that also included an untreated group. <b><i>Results:</i></b> The GH-treated group was 6.2 cm taller than the untreated group (AH = 149 cm [IQR 144.5–152.5 cm] vs. 142.8 cm [IQR 139–148 cm], <i>p</i> &#x3c; 0.001) after 4.9 years of GH treatment with a dose of 0.35 mg/kg/week. AH SDS corrected for target height (TH) was 7.2 cm higher in GH-treated patients. AH SDS ≥−2 was more frequent in GH-treated patients (43%) than in untreated patients (16%, <i>p</i> &#x3c; 0.001). AH SDS was also more frequently within the TH range in the GH-treated group (52%) than in the untreated group (15%, <i>p</i> &#x3c; 0.001). Height SDS at start of GH therapy and TH SDS were positively correlated with AH (<i>p</i> &#x3c; 0.001; <i>R</i><sup>2</sup> = 0.375). Considering the current result together with previous similar publications, a mean AH gain of 5.7 cm was observed in GH-treated (<i>n</i> = 696) versus untreated (<i>n</i> = 633) patients. <b><i>Conclusions:</i></b> Our study strengthens the evidence for efficacy of GH therapy in patients with TS from different populations.

scholarly journals Final Adult Height after Growth Hormone Treatment in Patients with Turner Syndrome

2019 ◽  
Vol 91 (6) ◽  
pp. 373-379 ◽  
Author(s):  
Jung Min Ahn ◽  
Jung Hwan Suh ◽  
Ah Reum Kwon ◽  
Hyun Wook Chae ◽  
Ho-Seong Kim
Keyword(s):  
Growth Hormone ◽  
Turner Syndrome ◽  
Adult Height ◽  
Final Height ◽  
Early Age ◽  
Gh Therapy ◽  
Gh Treatment ◽  
Height Range ◽  
Final Adult Height ◽  
Height Gain

Aims: This study aimed to evaluate final adult height (AH) after recombinant human growth hormone (GH) treatment of girls with Turner syndrome (TS) and to elucidate the predicting factors for their growth response. Methods: We enrolled 73 patients with TS who underwent GH treatment and reached AH and 14 patients who did not undergo treatment. To assess the effectiveness of GH therapy, we evaluated final AH, height gain over the predicted AH, and height gain over the projected AH. In addition, to analyze the factors affecting final AH, we studied correlations between final AH (or height SDS, height gain) and treatment variables. Results: GH therapy was started at a mean age of 8.87 ± 3.73 years, and the treatment duration was 6.47 ± 3.02 years. The patients in the treated group reached a final AH of 152.03 ± 4.66 cm (final AH SDS for the general population: –1.93 ± 1.03) with a gain over projected AH at the start of treatment of 12.21 ± 4.33 cm. The untreated control subjects had a final AH of 143.57 ± 4.06 cm with a gain over projected AH at the first visit of 3.89 ± 3.80 cm. Final AH and AH SDS were positively correlated to height SDS at the start of treatment. Thirty-five patients out of the 73 GH-treated patients (47.9%) attained to a normal range of height for Korean girls. The patients having attained to a normal height range after GH treatment had shown a higher height SDS at the start of GH treatment, a higher mid-parental height SDS, and a younger age at initiation of estrogen. Conclusions: Our findings demonstrate that GH treatment at an early age is effective in improving the final height SDS and height SDS gain in TS patients. Therefore, GH administration at an early age is important for final height gain.

scholarly journals SAT-107 Improved Adult Height in Brazilian Turner Syndrome Patients Treated with Growth Hormone

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Renata Da Cunha Scalco ◽  
Adriana Farrant Braz ◽  
Alexsandra C Malaquias ◽  
Sonir Roberto Rauber Antonini ◽  
Gil Guerra-Junior ◽  
...  
Keyword(s):  
Growth Hormone ◽  
Turner Syndrome ◽  
Adult Height ◽  
Delayed Diagnosis ◽  
Bone Age ◽  
Treated Group ◽  
Gh Treatment ◽  
Significant Difference ◽  
Referral Hospitals ◽  
Height Gain

Abstract Background: Short stature is the most frequent clinical manifestation in Turner syndrome (TS), occurring in 98% of these patients. Growth hormone was shown to improve adult height in TS patients from diverse genetic backgrounds. However, there are few studies on adult height in TS patients from developing countries, where the diagnosis is frequently delayed. Objective: To compare adult height between GH-treated and untreated TS patients. Patients and methods: 120 GH-treated and 109 GH-untreated TS patients from 3 referral hospitals in Brazil were evaluated. The most common reasons for not treating TS patients with GH were late diagnosis or GH unavailability. Data on karyotype, parents’ height, puberty development and GH treatment were obtained from their medical records. Adult height was determined when growth velocity was inferior to 1cm/year during a minimum follow-up period of 12 months. Results: The frequency of 45,X karyotype was similar between the groups (48.7% vs. 41.9% in GH-treated vs. GH-untreated TS patients, respectively, P= 0.639). GH-treated TS patients started GH therapy at a chronological age (CA) of 11.2 ± 3.7 yr, bone age of 9.3 ± 3.1 yr, height SDS (British 1965 standards) -3.1 ± 1.1. GH mean dose was 48µg/kg.d and GH treatment duration was 5.4 ± 3.0 yr. Estrogen replacement was started late, at CA of 14.3 ± 2.0 yr in GH-treated and at 14.9 ± 1.9 yr in GH-untreated patients, and the rate of spontaneous puberty was similar between the groups (GH-treated 16.8% vs. GH-untreated 22,8%, P=0.304). Adult height was significantly higher after GH treatment (150.1 ± 5.8 cm vs. 143.3 ± 7.2 cm in GH-treated vs. untreated TS patients, respectively, P &lt; 0.001), even with a small but significant difference in target height between the groups (158.2 ± 4.8 vs. 159.8± 4.5 cm in GH-treated vs. untreated TS patients, respectively, P= 0.015). More than half of the TS GH-treated patients reached normal adult height (equal or higher than 150.2 cm), whereas only 15.6% of GH-untreated patients reached it. Conclusion: Despite the delayed diagnosis of TS patients in our cohort, GH treatment was associated with a significant height gain, and the TS GH-treated group was around 7 cm taller than the GH-untreated group.

The Psychological Consequences of Turner Syndrome and Review of the National Cooperative Growth Study Psychological Substudy

PEDIATRICS ◽  
1998 ◽  
Vol 102 (Supplement_3) ◽  
pp. 488-491 ◽  
Author(s):  
Patricia T. Siegel ◽  
Richard Clopper ◽  
Brian Stabler
Keyword(s):  
Growth Hormone ◽  
Behavior Problems ◽  
Short Stature ◽  
Turner Syndrome ◽  
Child Behavior ◽  
Child Behavior Checklist ◽  
Idiopathic Short Stature ◽  
Gh Therapy ◽  
Gh Treatment ◽  
Control Subjects

Objective. To present longitudinal data on the psychological profile of a cohort of girls with and without Turner syndrome (TS) treated for 3 years with growth hormone (GH). Methods. Among a sample of 283 children with short stature, 37 girls with TS were recruited at 27 US medical centers. Of the original cohort, 22 girls with TS, 13 girls with isolated growth hormone deficiency (GHD), and 12 girls with idiopathic short stature were followed through 3 years of GH therapy. All were school-age, were below the 3rd percentile for height, had low growth rates, and were naive to GH therapy. Psychological tests (the Wide Range Achievement Test and the Slosson Intelligence Test) were administered to the clinical groups within 24 hours of their first GH injection and yearly thereafter. Control subjects were 25 girls with normal stature matched for age and socioeconomic status, who were tested only at baseline. One parent of each subject also completed the Child Behavior Checklist for that subject. Results. At baseline, the clinical groups had more internalizing behavioral problems, had fewer friends, and participated in fewer activities than did the control subjects. The groups did not differ in mean IQ or academic achievement, but the TS group did have more problems in mathematics achievement. Height and growth rate significantly increased in the clinical groups over the 3 years of GH therapy, but IQ and achievement scores did not. Significant linear reductions were noted in both Internalizing and Externalizing Behavior Problems after GH treatment, with the TS group having fewer behavior problems before and after GH treatment than did the GHD–idiopathic short stature group. Decreases in specific Child Behavior Checklist subscales, including attention, social problems, and withdrawal, also were seen in the clinical groups after GH therapy. Conclusions. The comprehensive treatment of girls with TS should include educational and behavioral interventions in addition to traditional medical therapies.

scholarly journals Childhood growth hormone treatment in women with Turner syndrome - benefits and adverse effects

2019 ◽  
Vol 9 (1) ◽  
Author(s):  
Tomasz Irzyniec ◽  
Wacław Jeż ◽  
Katarzyna Lepska ◽  
Izabela Maciejewska-Paszek ◽  
Jakub Frelich
Keyword(s):  
Growth Hormone ◽  
Adverse Effects ◽  
Turner Syndrome ◽  
Sex Chromosome ◽  
Initial Period ◽  
Growth Failure ◽  
Hormone Treatment ◽  
Treated Group ◽  
Gh Treatment ◽  
Childhood Growth

Abstract Turner syndrome (TS) is characterized by the partial or complete loss of one sex chromosome and results in growth failure, gonadal insufficiency and cardiac anomalies. Treatment with growth hormone (GH) during childhood has indisputable benefits when taking into account the low stature of TS women. Medical records and biochemical findings of 33 TS women treated with GH in childhood (GH+) were compared to those of 124 TS women who did not receive GH (GH−). It seems that the GH-treated group might have had a more severe initial phenotype than the untreated group, as evidenced by higher FSH, more feeding issues in infancy, more lymphedema cases and urinary system malformations. GH+ women were significantly taller and had a better lipid profile and lower prevalence of arterial hypertension than GH− . However, they also had lower thrombocyte counts, a greater prevalence of retrognathism and nail anomalies, especially when the GH treatment was delayed. Long-term GH use was not as effective for growth as GH treatment during the initial period and seemed to have resulted in elevated creatinine levels. GH treatment in childhood has benefits in adulthood; however, adverse effects may occur, especially in individuals with treatment that is delayed or is too long.

Growth and Adult Height in Girls With Turner Syndrome Following IGF-1 Titrated Growth Hormone Treatment

2020 ◽  
Vol 105 (8) ◽  
pp. 2566-2574
Author(s):  
Amanda Cleemann Wang ◽  
Casper P Hagen ◽  
Leila Nedaeifard ◽  
Anders Juul ◽  
Rikke Beck Jensen
Keyword(s):  
Growth Hormone ◽  
Turner Syndrome ◽  
Real World ◽  
Adult Height ◽  
Standard Deviation Score ◽  
Fixed Dose ◽  
Dose Titration ◽  
Gh Treatment ◽  
Real World Evidence ◽  
Tertiary Center

Abstract Context Girls with Turner syndrome (TS) suffer linear growth failure, and TS is a registered indication for growth hormone (GH) treatment. GH is classically dosed according to body weight, and serum insulin-like growth factor-1 (IGF-1) concentrations are recommended to be kept within references according to international guidelines. Objective To assess the effect of long-term GH treatment in girls with TS following GH dosing by IGF-1 titration. Design and setting A retrospective, real-world evidence, observational study consisting of data collected in a single tertiary center from 1991 to 2018. Patients A cohort of 63 girls with TS treated with GH by IGF-1 titration with a median duration of 6.7 years (interquartile range [IQR]: 3.4-9.7 years). Main outcome measures Longitudinal measurements of height, IGF-1, and adult height (AH) following GH treatment were evaluated and compared between the different karyotypes (45,X, 45,X/46,XX, or miscellaneous). Results Using GH dose titration according to IGF-1, only 6% of girls with TS had supranormal IGF-1 levels. Median dose was 33 µg/kg/day (IQR: 28-39 µg/kg/day) with no difference between the karyotype groups. AH was reached for 73% who attained a median AH of 1.25 standard deviation score (SDS) for age specific TS references (IQR: 0.64-1.50 SDS), and a median gain in height (ΔHSDS: AH SDS minus baseline height SDS of TS references) of 0.50 SDS, equal to 3.2 cm (SD 7.68) for all karyotypes. Conclusion Our real-world evidence study suggested that titration of GH dose to keep IGF-1 levels within the normal range resulted in a lower AH gain than in studies where a fixed dose was used.

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

2002 ◽  
pp. 625-633 ◽  
Author(s):  
MB Ranke ◽  
CJ Partsch ◽  
A Lindberg ◽  
HG Dorr ◽  
M Bettendorf ◽  
...  
Keyword(s):  
Turner Syndrome ◽  
Adult Height ◽  
Bone Age ◽  
First Year ◽  
Gh Therapy ◽  
Gh Treatment ◽  
Height Gain ◽  
Puberty Onset

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=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 (-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 (DeltaHT) after GH discontinuation was 1.5 cm. Total DeltaHT correlated (P<0.001) negatively with bone age and HT SDS at GH start, but positively with DeltaHT after the first Year, DeltaHT at puberty onset, and GH duration. Final HT correlated (P<0.001) positively with HT at GH start, first-Year DeltaHT, and HT at puberty onset. Body mass index increased slightly (P<0.05), with values at start and adult follow-up correlating highly (R=0.70, P<0.001). 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.

Growth Hormone Treatment of Girls With Turner Syndrome: The National Cooperative Growth Study Experience

PEDIATRICS ◽  
1998 ◽  
Vol 102 (Supplement_3) ◽  
pp. 479-481
Author(s):  
Leslie Plotnick ◽  
Kenneth M. Attie ◽  
Sandra L. Blethen ◽  
Judy P. Sy
Keyword(s):  
Growth Hormone ◽  
Turner Syndrome ◽  
Adult Height ◽  
Standard Deviation Score ◽  
Response To Treatment ◽  
Growth Study ◽  
Duration Of Treatment ◽  
Gh Treatment ◽  
Recombinant Growth Hormone ◽  
National Cooperative

Objective. To evaluate growth rate and adult height with recombinant growth hormone (GH) treatment in girls with Turner syndrome (TS) and predictors of their growth response. Methods. Data on girls with TS who were treated with GH in the National Cooperative Growth Study (NCGS) were evaluated. As of January 1997, there were 2798 girls with TS in the NCGS database, 2652 of whom had not previously received GH. Follow-up data on growth were available for 2475 subjects, and data on adult height were available for 622. Results. The average age of girls with TS at enrollment in the NCGS was 10.1 ± 3.6 years. These patients had severely short stature compared with that of unaffected American girls (height, 118.5 ± 16.5 cm; height standard deviation score [SDS], −3.1 ± 0.9), but their heights were typical of those of American girls with TS (TS-specific height SDS, 0.01 ± 0.9). Treatment with GH for an average duration of 3.2 ± 2.0 years resulted in an increase in height SDS of 0.8 ± 0.7 compared with unaffected girls and of 1.2 ± 0.8 compared with TS standards. Growth rates increased from 4.0 ± 2.3 cm/year before treatment to 7.5 ± 2.0 cm/year after 1 year of treatment. Duration of treatment with GH was the strongest predictor of change in height SDS. After 6 to 7 years of treatment with GH, there was a cumulative change of 2.0 in mean height SDS. The 622 girls who reached adult height were older when they began taking GH. Their mean height gain over pre-GH projected height was 6.4 ± 4.9 cm after 3.7 ± 1.9 years of treatment. Their adult height was 148.3 ± 5.6 cm. Conclusions. Although the response to treatment with GH varied, it was associated with highly significant gains in growth and adult height in girls with TS. Duration of treatment with GH was the most important variable predicting adult height.

scholarly journals X-chromosome gene dosage as a determinant of impaired pre and postnatal growth and adult height in Turner syndrome

2016 ◽  
Vol 174 (3) ◽  
pp. 281-288 ◽  
Author(s):  
Elodie Fiot ◽  
Delphine Zenaty ◽  
Priscilla Boizeau ◽  
Jeremy Haigneré ◽  
Sophie Dos Santos ◽  
...  
Keyword(s):  
Turner Syndrome ◽  
X Chromosome ◽  
Multiple Testing ◽  
Adult Height ◽  
Gene Dosage ◽  
Postnatal Growth ◽  
Target Height ◽  
Anthropometric Parameters ◽  
Gh Treatment ◽  
Chromosome Gene

ObjectiveShort stature is a key aspect of the phenotype of patients with Turner syndrome (TS). SHOX haploinsufficiency is responsible for about two-thirds of the height deficit. The aim was to investigate the effect of X-chromosome gene dosage on anthropometric parameters at birth, spontaneous height, and adult height (AH) after growth hormone (GH) treatment.DesignWe conducted a national observational multicenter study.MethodsBirth parameter SDS for gestational age, height, and AH before and after GH treatment respectively, and height deficit with respect to target height (SDS) were classified by karyotype subgroup in a cohort of 1501 patients with TS: 45,X (36%), isoXq (19%), 45,X/46,XX (15%), XrX (7%), presence of Y (6%), or other karyotypes (17%).ResultsBirth weight, length (P<0.0001), and head circumference (P<0.001), height and height deficit with respect to target height (SDS) before GH treatment, at a median age of 8.8 (5.3–11.8) years and after adjustment for age and correction for multiple testing (P<0.0001), and AH deficit with respect to target height at a median age of 19.3 (18.0–21.8) years and with additional adjustment for dose and duration of GH treatment (P=0.006), were significantly associated with karyotype subgroup. Growth retardation tended to be more severe in patients with XrX, isoXq, and, to a lesser extent, 45,X karyotypes than in patients with 45,X/46,XX karyotypes or a Y chromosome.ConclusionThese data suggest that haploinsufficiency for an unknown Xp gene increases the risk of fetal and postnatal growth deficit and short AH with respect to target height after GH therapy.

Adult height in sixty girls with Turner syndrome treated with growth hormone matched with an untreated group

2005 ◽  
Vol 28 (6) ◽  
pp. 350-356 ◽  
Author(s):  
A. M. Pasquino ◽  
I. Pucarelli ◽  
M. Segni ◽  
L. Tarani ◽  
V. Calcaterra ◽  
...  
Keyword(s):  
Growth Hormone ◽  
Turner Syndrome ◽  
Adult Height ◽  
Untreated Group

scholarly journals SAT-106 Growth Hormone Treatment Response in Children

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Mitchell Rath ◽  
Daniele Pacaud ◽  
Karin Winston ◽  
Josephine Ho ◽  
Jonathan M Dawrant ◽  
...  
Keyword(s):  
Growth Hormone ◽  
Turner Syndrome ◽  
Tertiary Care ◽  
Stimulation Test ◽  
Height Velocity ◽  
First Year ◽  
Gh Therapy ◽  
Gh Treatment ◽  
Common Diagnosis ◽  
The Mean

Abstract OBJECTIVES: Growth hormone (GH) therapy is an effective treatment in addressing growth failure in children with GH deficiency (GHD). It has also been increasingly used in non-GH deficient (nGHD) conditions. We sought to report the growth response of GHD and nGHD patients who received GH therapy at a tertiary care center. METHODS: Data was collected from health records of patients followed in the endocrinology clinic at Alberta Children’s Hospital, Calgary, Canada, from 2005 to 2019, and used to analyze clinical responses based on indication for GH treatment. RESULTS: A total of 167 patient records (87 males and 80 females) were used for analysis. The average age at the start of GH therapy was 7.3 years (range 0.25 to 16.98 yrs). 74 patients were in the GHD group while 93 were nGHD. Of the patients in the nGHD group, the most common diagnosis were: idiopathic short stature (ISS)(n=45), Turner syndrome (TS)(n=26), and Prader Willi Syndrome (PWS)(n=8). The mean height velocity (HV) in year 1 was highest in the GHD group at 11.68 cm/year (n= 62, sd = 5.93), followed by ISS at 9.41cm/year (n = 52, sd = 4.34). The mean first year HV of those who had received chemotherapy (n= 5, mean = 5.48, sd = 1.92) or had Turner syndrome (n= 24, mean = 7.20, sd = 2.15) was significantly lower than both the GHD and ISS groups. GH peak during a GH stimulation test at baseline was not correlated to the first year height velocity while on GH treatment. However there was a negative linear correlation between baseline IGF1 level and first year height velocity (Spearman’s rho = 0.312216, p-value= 0.01516). Age at GH initiation was negatively correlated with height velocity during GH treatment. Height velocity over time decreased sharply from year 1 to year 3, and became stable for the remaining years of GH therapy. For the entire group, HV for years 1-5 was 9.81 (sd=4.83), 7.40 (sd=2.89), 6.29 (sd=2.38), 5.92 (sd=2.56), 5.66 (sd=2.51). There is no significant correlation between GH dose and height velocity response after adjusting for diagnosis. CONCLUSION: In our population, the response to GH therapy was consistent with those reported in the literature. Response to GH therapy was not associated with GH peak on stimulation but rather to baseline IGF-1 level and age at initiation. Although peak GH to stimulation is required to obtain public funding for GH therapy, these findings demonstrate that GH stimulation test results may not indicate which patients may benefit the most from GH therapy. Follow-up until final adult height will allow us to have a better understanding of the efficacy of GH therapy in patients with both GHD and nGHD conditions.

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