Response to three years of growth hormone therapy in girls with Turner syndrome

Park, Hong Kyu; Lee, Hae Sang; Ko, Jung Hee; Hwang, Il Tae; Hwang, Jin Soon

doi:10.6065/apem.2013.18.1.13

Cited by 8 publications

(8 citation statements)

References 35 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Apart from the previous report of the clinical trial, the only other study to date that has reported final height/ near-adult height data for GH-treated patients with SHOX deficiency found a mean height SDS gain of 0.6 in a group of 5 peripubertal patients who were treated with a gonadotropin-releasing hormone analogue for pubertal suppression and started GH at a mean age of 11.6 years [29] . These findings suggest that, similar to other short stature indications for GH treatment, height gain is greater when GH is initiated early, particularly before onset of puberty [30,31] . However, the use of height SDS for chronological age of the near-adult height measurement in children with a mean age of 15.7 years may not accurately measure the true adult height SDS.…”

Section: Discussionsupporting

confidence: 59%

Safety Outcomes and Near-Adult Height Gain of Growth Hormone-Treated Children with SHOX Deficiency: Data from an Observational Study and a Clinical Trial

Benabbad¹,

Rosilio²,

Child³

et al. 2016

Horm Res Paediatr

View full text Add to dashboard Cite

Background/Aims: To assess auxological and safety data for growth hormone (GH)-treated children with SHOX deficiency. Methods: Data were examined for GH-treated SHOX-deficient children (n = 521) from the observational Genetics and Neuroendocrinology of Short Stature International Study (GeNeSIS). For patients with near-adult height information, GeNeSIS results (n = 90) were compared with a clinical trial (n = 28) of SHOX-deficient patients. Near-adult height was expressed as standard deviation score (SDS) for chronological age, potentially increasing the observed effect of treatment. Results: Most SHOX-deficient patients in GeNeSIS had diagnoses of Leri-Weill syndrome (n = 292) or non-syndromic short stature (n = 228). For GeNeSIS patients with near-adult height data, mean age at GH treatment start was 11.0 years, treatment duration 4.4 years, and height SDS gain 0.83 (95% confidence interval 0.49-1.17). Respective ages, GH treatment durations and height SDS gains for GeNeSIS patients prepubertal at baseline (n = 42) were 9.2 years, 6.0 years and 1.19 (0.76-1.62), and for the clinical trial cohort they were 9.2 years, 6.0 years and 1.25 (0.92-1.58). No new GH-related safety concerns were identified. Conclusion: Patients with SHOX deficiency who had started GH treatment before puberty in routine clinical practice had a similar height gain to that of patients in the clinical trial on which approval for the indication was based, with no new safety concerns.

show abstract

Section: Discussionsupporting

confidence: 59%

Safety Outcomes and Near-Adult Height Gain of Growth Hormone-Treated Children with SHOX Deficiency: Data from an Observational Study and a Clinical Trial

Benabbad¹,

Rosilio²,

Child³

et al. 2016

Horm Res Paediatr

View full text Add to dashboard Cite

show abstract

“…As for the final height, the results were not conclusive. The mean final height was higher in the group who was treated with growth hormone (144.03±4.8) compared with the untreated group (141.08±7.9), but it was lower than the mean final height reported from similar studies in which mean adult height in treated patients varied from 146.8 to 152.2 cm (12,(37)(38)(39)(40)(41)(42). Another weakness of the study is related with the fact that the untreated subgroup of 27 patients of our study missed the GH therapy for non-medical reasons (see above), and the composition of this untreated subgroup was hardly comparable with the selected cases under GH therapy (25 patients).…”

Section: Discussioncontrasting

confidence: 57%

“…Reported studies suggest a growth rate enhancement, or an improvement of the final height of TS patients treated with GH, in a variable measure from 0 to 11.9 cm (12,(38)(39)(40)(41)(42). Differences in the reported results might derive from the age of the therapy initiation, time length of the therapy itself and the hormonal dosages applied.…”

Section: Discussionmentioning

confidence: 98%

Turner syndrome in Albania and the efficacy of its treatment with growth hormone

Hoxha

Babameto-Laku

Vyshka

et al. 2015

Journal of Pediatric Endocrinology and Metabolism

View full text Add to dashboard Cite

The aim of this study was the evaluation of Turner syndrome inside the Albanian population, its clinical, cytological and genetic characteristics, the accompanying pathologies, and the efficacy of the treatment with the growth hormone. We performed a retrospective analysis of 59 patients suffering from this syndrome (aging from 5 to 23 years old). The diagnosis of female patients suffering from Turner syndrome is delayed, with a mean age at the moment of diagnosis of 13.74 years (5-23 years). The main reason for seeking medical advice was the growth retardation or a delayed puberty. Available data for 52 patients showed that the most frequent accompanying pathologies were the following: thyroid autoimmune disorders (59%), cardiovascular anomalies (43%), renal pathologies (41%), hearing impairment (4.3%) and hypertension (3.3%). Follow-up for the growth rate was possible for 52 patients out of the total of 59 patients. Twenty-five of the female patients suffering Turner syndrome and forming part of our study sample were treated with growth hormone for a period averaging 3 years and 4 months. A variety of reasons was identified as responsible for the missed treatment in 27 patients. We saw an enhanced growth (in terms of body height) within the treated subgroup, when compared with the untreated subgroup (27 patients), especially during the first 3 years of the follow-up. No side effects of this treatment were reported. Both groups of patients initiated as well a sexual hormone therapy (estrogens and progesterone) for inducing puberty at the age of 12 years. Further work is needed for an early diagnosis of this syndrome, the prompt treatment with growth hormone and the monitoring of accompanying disorders. This will ensure a better quality of life and an improvement of the longevity of patients suffering from the Turner syndrome.

show abstract

“…The new indications include Small for Gestational Age (SGA), Idiopathic Short Stature (ISS) and Turner Syndrome (TS) in addition to other indications [ 1 – 3 ]. Since rGH was introduced, there have been several observational studies that described its effectiveness among children [ 4 – 7 ]. In most of the indications listed above, rGH therapy has been shown to improve final adult height [ 5 , 7 – 9 ].…”

Section: Introductionmentioning

confidence: 99%

“…Since rGH was introduced, there have been several observational studies that described its effectiveness among children [ 4 – 7 ]. In most of the indications listed above, rGH therapy has been shown to improve final adult height [ 5 , 7 – 9 ]. Despite the increasing body of international literature on rGH use, there is paucity of data on rGH use in Kuwait and the broader Middle-East which share unique ethnic and socio-cultural backgrounds.…”

Section: Introductionmentioning

confidence: 99%

Recombinant growth hormone therapy in children with short stature in Kuwait: a cross-sectional study of use and treatment outcomes

et al. 2015

View full text Add to dashboard Cite

BackgroundRecombinant Growth hormone (rGH) therapy is approved in many countries for treatment of short stature in a number of childhood diagnoses. Despite the increasing body of international literature on rGH use, there is paucity of data on rGH use in Kuwait and the broader Middle-East which share unique ethnic and socio-cultural backgrounds. This study aimed to describe the pattern of use and treatment outcomes of rGH therapy in Kuwait.MethodsThis is a cross-sectional retrospective review of children treated with rGH in the Department of Pediatrics, in a major hospital in Kuwait between December 2013 and December 2014. Data were extracted using standard data extraction form and the response to rGH therapy was defined as a gain of ≥ 0.3 standard deviation score (SDS) of height per year.ResultsA total of 60 children were treated with rGH in the center. Their Median (Interquartile) age at rGH initiation was 9.0 (6.2, 10.7) years. The most common indications for rGH therapy were Growth Hormone Deficiency (GHD) 23 (38.3 %), Idiopathic Short Stature (ISS) 12 (20.0 %) and Small for Gestational Age (SGA) 9 (15.0 %). After excluding patients with TS, no significant differences were found in gender of those who received rGH therapy in all indications combined or in each group (p ≥ 0.40). At 1-year follow-up, children in all groups had median height SDS change of ≥ 0.3 SDS except for children with ISS. Age at rGH initiation was negatively associated with 1-year treatment response, Adjusted odds ratio (AOR) 0.56 (95 % CI: 0.04–1.49); p = 0.011).ConclusionsGHD is the most common indication of rGH therapy. All indications except for ISS showed significant 1-year treatment response to therapy. Treatment outcomes in patients with ISS should be further investigated in Kuwait. Younger age at initiation of rGH therapy was independently associated with significant response to therapy suggesting the importance of identifying children with short stature and prompt initiation of rGH therapy.

show abstract

Response to three years of growth hormone therapy in girls with Turner syndrome

Cited by 8 publications

References 35 publications

Safety Outcomes and Near-Adult Height Gain of Growth Hormone-Treated Children with SHOX Deficiency: Data from an Observational Study and a Clinical Trial

Safety Outcomes and Near-Adult Height Gain of Growth Hormone-Treated Children with SHOX Deficiency: Data from an Observational Study and a Clinical Trial

Turner syndrome in Albania and the efficacy of its treatment with growth hormone

Recombinant growth hormone therapy in children with short stature in Kuwait: a cross-sectional study of use and treatment outcomes

Contact Info

Product

Resources

About