scholarly journals Adult Height after Growth Hormone Treatment at Pubertal Onset in Short Adolescents Born Small for Gestational Age: Results from a Belgian Registry-Based Study

2018 ◽  
Vol 2018 ◽  
pp. 1-8 ◽  
Author(s):  
M. Thomas ◽  
D. Beckers ◽  
C. Brachet ◽  
H. Dotremont ◽  
M.-C. Lebrethon ◽  
...  
Keyword(s):  
Adult Height ◽  
Hormone Treatment ◽  
Growth Data ◽  
Gh Therapy ◽  
Gh Treatment ◽  
Pubertal Stage ◽  
Pubertal Onset ◽  
Total Height ◽  
Height Gain ◽  
Lower Height

Objectives. Information on the efficacy of GH treatment in short SGA children starting their treatment in adolescence is limited. Therefore, adult height (AH), total height gain, and pubertal height gain were evaluated in short SGA children who started GH treatment at pubertal onset. Patient and Methods. Growth data of 47 short SGA adolescents (22 boys) who started GH treatment at pubertal onset (PUB group) were compared with results from 27 short SGA patients (11 boys) who started GH therapy at least 1 year before pubertal onset (PrePUB group). Results. The PUB group achieved a mean (±SD) total height gain of 0.8 ± 0.7 SDS and an AH of −2.5 ± 0.7 SDS after 4.1 ± 1.1 years of GH treatment with a dosage of 41.8 ± 8.4 μg/kg/day. These results were comparable with those in the PrePUB group, which was treated for a longer duration (5.8 ± 2.1 years), resulting in a total height gain of 1.1 ± 0.7 SDS and an AH of −2.1 ± 1.0 SDS. Multiple regression analysis showed a significantly lower height gain in pubertal patients, females, and patients weighing less at start of GH treatment. An AH above −2 SDS and above the parent-specific lower limit of height was, respectively, reached in 28% and 70% of PUB and 44% and 67% of PrePUB patients (NS). AH SDS was positively correlated with the height SDS at start of GH. Conclusions. Short SGA adolescents starting GH therapy at an early pubertal stage have a modest and variable height gain. A normal AH can be expected in one third of the patients, especially in those with a smaller height deficit at onset of GH treatment.

scholarly journals Is a Two-Year Growth Response to Growth Hormone Treatment a Better Predictor of Poor Adult Height Outcome Than a First-Year Growth Response in Prepubertal Children With Growth Hormone Deficiency?

2021 ◽  
Vol 12 ◽  
Author(s):  
Saartje Straetemans ◽  
Raoul Rooman ◽  
Jean De Schepper
Keyword(s):  
Growth Hormone ◽  
Growth Response ◽  
Adult Height ◽  
Poor Response ◽  
Hormone Treatment ◽  
Hormone Deficiency ◽  
First Year ◽  
Gh Treatment ◽  
Height Gain ◽  
Design And Methods

ObjectiveThe first year response to growth hormone (GH) treatment is related to the total height gain in GH treated children, but an individual poor first year response is a weak predictor of a poor total GH effect in GH deficient (GHD) children. We investigated whether an underwhelming growth response after 2 years might be a better predictor of poor adult height (AH) outcome after GH treatment in GHD children.Design and methodsHeight data of GHD children treated with GH for at least 4 consecutive years of which at least two prepubertal and who attained (near) (n)AH were retrieved from the Belgian Register for GH treated children (n = 110, 63% boys). In ROC analyses, the change in height (ΔHt) SDS after the first and second GH treatment years were tested as predictors of poor AH outcome defined as: (1) nAH SDS <−2.0, or (2) nAH SDS minus mid-parental height SDS <−1.3, or (3) total ΔHt SDS <1.0. The cut-offs for ΔHt SDS and its sensitivity at a 95% specificity level to detect poor AH outcome were determined.ResultsEleven percent of the cohort had a total ΔHt SDS <1.0. ROC curve testing of first and second years ΔHt SDS as a predictor for total ΔHt SDS <1.0 had an AUC >70%. First-year ΔHt SDS <0.41 correctly identified 42% of the patients with poor AH outcome at a 95% specificity level, resulting in respectively 5/12 (4.6%) correctly identified poor final responders and 5/98 (4.5%) misclassified good final responders (ratio 1.0). ΔHt SDS after 2 prepubertal years had a cut-off level of 0.65 and a sensitivity of 50% at a 95% specificity level, resulting in respectively 6/12 (5.5%) correctly identified poor final responders and 5/98 (4.5%) misclassified good final responders (ratio 1.2).ConclusionIn GHD children the growth response after 2 prepubertal years of GH treatment did not meaningfully improve the prediction of poor AH outcome after GH treatment compared to first-year growth response parameters. Therefore, the decision to re-evaluate the diagnosis or adapt the GH dose in case of poor response after 1 year should not be postponed for another year.

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.

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 Pubertal growth in normal Thai children: a longitudinal study

2010 ◽  
Vol 4 (5) ◽  
pp. 793-795
Author(s):  
Suttipong Wacharasindhu ◽  
Vichit Supornsilchai ◽  
Suphab Aroonparkmongkol ◽  
Thaninee Sahakitrungrueng
Keyword(s):  
Longitudinal Study ◽  
Growth Parameters ◽  
Longitudinal Growth ◽  
Compensatory Mechanism ◽  
Growth Data ◽  
Clinical Practices ◽  
Normal Children ◽  
Pubertal Onset ◽  
Pubertal Growth ◽  
Height Gain

Abstract Background: Pubertal growth data in Thai children has been reported as cross-sectional studies. There is no longitudinal study in Thai children. Objective: Investigate the longitudinal growth data in normal Thai children including the relationship between age at pubertal onset and other growth parameters. Material and method: Eighty-eight normal children (44 boys, 44 girls) were longitudinally assessed for the growth and puberty until they reached their final adult height. Pubertal staging was assessed by the Tanner method. Results: Mean age of pubertal onset was 10.2 ± 1.2 years for girls and 12.2 ± 1.0 years for boys. Total pubertal height gain was 18.3 ± 4.0 cm for girls and 22.3 ± 4.4 cm for boys. Total pubertal height gain had a negative correlation with age at pubertal onset for girls, but not for boys. Conclusion: The onset of puberty was not much changed from previous studies. Girls with early puberty had a higher pubertal height gain. This might be due to a compensatory mechanism. These longitudinal growth data can be used as a reference in clinical practices for Thai children.

scholarly journals IGF-1 and Growth Response to Adult Height in a Randomized GH Treatment Trial in Short Non-GH-Deficient Children

2014 ◽  
Vol 99 (8) ◽  
pp. 2917-2924 ◽  
Author(s):  
Berit Kriström ◽  
Elena Lundberg ◽  
Björn Jonsson ◽  
Kerstin Albertsson-Wikland ◽  
Keyword(s):  
Clinical Trial ◽  
Growth Response ◽  
Adult Height ◽  
Bone Age ◽  
Controlled Clinical Trial ◽  
Dependent Manner ◽  
Gh Treatment ◽  
Randomized Controlled ◽  
Short Children ◽  
Height Gain

Context: GH treatment significantly increased adult height (AH) in a dose-dependent manner in short non-GH-deficient children in a randomized, controlled, clinical trial; the mean gain in height SD score (heightSDS) was 1.3 (range 0–3), compared with 0.2 in the untreated group. Objective: The objective of the study was to analyze the relationship between IGF-1SDS, IGF binding protein-3 SDS (IGFBP3SDS), and their ratioSDS with a gain in the heightSDS until AH in non-GH-deficient short children. Design and Setting: This was a randomized, controlled, multicenter clinical trial. Intervention: The intervention included GH treatment: 33 or 67 μg/kg · d plus untreated controls. Subjects: One hundred fifty-one non-GH-deficient short children were included in the intent-to-treat (ITT) population and 108 in the per-protocol (PP) population; 112 children in the ITT and 68 children in the PP populations had idiopathic short stature (ISS). Main Outcome Measures: Increments from baseline to on-treatment study mean IGF-1SDS (ΔIGF-1SDS), IGFBP3SDS, and IGF-1 to IGFBP3 ratioSDS were assessed in relationship to the gain in heightSDS. Results: Sixty-two percent of the variance in the gain in heightSDS in children on GH treatment could be explained by four variables: ΔIGF-1SDS (explaining 28%), bone age delay, birth length (the taller the better), and GH dose (the higher the better). The lower IGF-1SDS was at baseline, the higher was its increment during treatment. For both the AllPP- and the ISSPP-treated groups, the attained IGF-1SDS study level did not correlate with height gain. Conclusion: In short non-GH-deficient children, the GH dose-related increment in IGF-1SDS from baseline to mean study level was the most important explanatory variable for long-term growth response from the peripubertal period until AH, when IGF-1SDS, IGFBP3SDS, and their ratioSDS were compared concurrently.

Acromesomelic Dysplasia, Type Maroteaux: Impact of Long-Term (8 Years) High-Dose Growth Hormone Treatment on Growth Velocity and Final Height in 2 Siblings

2020 ◽  
Vol 93 (5) ◽  
pp. 335-342
Author(s):  
Ved Bhushan Arya ◽  
Meena Raj ◽  
Maha Younes ◽  
Simon Chapman ◽  
Melita Irving ◽  
...  
Keyword(s):  
Growth Hormone ◽  
Serum Insulin ◽  
Final Height ◽  
Hormone Treatment ◽  
High Dose ◽  
Childhood And Adolescence ◽  
Loss Of Function ◽  
Gh Treatment ◽  
Height Gain

<b><i>Introduction:</i></b> Acromesomelic dysplasia, type Maroteaux (AMDM) is a rare autosomal recessive skeletal dysplasia, characterized by severe dwarfism and disproportionate limb shortening. It results from loss-of-function <i>NPR2</i> mutations affecting the C-type natriuretic peptide receptor. Resistance to growth hormone (GH) action has previously been suggested. We describe outcomes of 2 siblings with AMDM after prolonged high-dose GH treatment. <b><i>Patients/Methods:</i></b> Two siblings (Pt-A and Pt-B; consanguineous parents) presented in early childhood with severe disproportionate short stature and radiological features of AMDM. Subsequent genetic testing identified a novel homozygous <i>NPR2</i> mutation. GH provocation testing showed relatively high GH levels. Serum insulin-like growth factor 1 (IGF-1) was ∼2 SD below age/sex-specific mean. High-dose GH (0.075 mg/kg/day) was started. Pre-GH height velocities were 3.7 (Pt-A) and 4.5 (Pt-B) cm/year. GH dose was adjusted to sustain serum IGF-1 towards +3 SDS for age/sex. Annualized height velocities for first 3 years on GH were 7.0, 5.4, and 4.7 cm/year for patient A and 9.4, 8.0, and 5.9 cm/year for patient B. Height gain during puberty was 10.6 (Pt-A) and 5.9 (Pt-B) cm. Final heights after 8.5 years of GH treatment were 130.5 cm (−6.57 SDS, Pt-A) and 134 cm (−4.58 SDS, Pt-B). <b><i>Conclusions:</i></b> To the best of our knowledge, this is the first report of final height in patients with AMDM after long-term GH treatment. Our results confirm the finding of relative GH resistance in AMDM, which when overcome with high-dose GH treatment resulted in improved height SDS during childhood and adolescence and associated quality of life. The final height of our patients was significantly higher than average reported final height (120 cm) of AMDM patients.

scholarly journals Growth in Children With Noonan Syndrome and Effects of Growth Hormone Treatment on Adult Height

2021 ◽  
Vol 12 ◽  
Author(s):  
Annachiara Libraro ◽  
Vito D’Ascanio ◽  
Marco Cappa ◽  
Mariangela Chiarito ◽  
Maria Cristina Digilio ◽  
...  
Keyword(s):  
Noonan Syndrome ◽  
Adult Height ◽  
Standard Dose ◽  
Genetic Diagnosis ◽  
Hormone Treatment ◽  
Specific Response ◽  
Multicenter Cohort Study ◽  
Pubertal Onset ◽  
Significant Difference ◽  
Rhgh Treatment

ObjectivesGrowth impairment is a common manifestation in Noonan syndrome (NS). Recombinant human GH (rhGH) treatment has been shown to increase growth and adult height (AH) in a few studies. We aimed to evaluate the growth trajectory towards the AH, and the effects of rhGH treatment in a large cohort of NS children.MethodsRetrospective, multicenter, cohort study including subjects with genetic diagnosis of NS. A total of 228 NS patients, 154 with PTPN11 mutations, 94 who reached AH, were recruited. Auxological data were collected at 2, 5, and 10 years, at pubertal onset, at AH. Sixty-eight NS subjects affected with GH deficiency (GHD) were treated with rhGH at a mean dose of 0.24 mg/kg per week until AH achievement.ResultsANOVA analysis showed a significant difference between birth length and height standard deviation scores (HSDS) at the different key ages (p&lt;0.001), while no significant differences were found between HSDS measurements at 2, 5, and 10 years, at pubertal onset, and at AH. HSDS increased from −3.10 ± 0.84 to −2.31 ± 0.99 during rhGH treatment, with a total height gain of 0.79 ± 0.74, and no significant difference between untreated and treated NS at AH.ConclusionsrhGH treatment at the standard dose used for children with GH idiopathic deficiency is effective in improving growth and AH in NS with GHD. Further studies are needed to assess genotype-specific response to rhGH treatment in the different pathogenic variants of PTPN11 gene and in the less common genotypes.

scholarly journals Individualized Growth Hormone Treatment - Using Two Different Prediction Models in a Clinical Setting

2022 ◽  
Author(s):  
Helena-Jamin Ly ◽  
Anders Lindberg ◽  
Hans Fors ◽  
Jovanna Dahlgren
Keyword(s):  
Growth Hormone ◽  
Prediction Models ◽  
Pearson Correlation ◽  
Hormone Treatment ◽  
Hormone Deficiency ◽  
Study Cohort ◽  
Growth Data ◽  
Gh Treatment ◽  
Predicted Height ◽  
One Year

Abstract BackgroundDiagnosing growth hormone deficiency (GHD) can be challenging; hence, prediction models on growth outcome from growth hormone (GH) treatment have shown to be useful. We aim to compare the accuracy of the more readily available KIGS (Pfizer International Growth Study) prediction model to the previously clinically validated Gothenburg model.MethodsPrepubertal children with GHD who started GH treatment at Queen Silvia Children’s Hospital between 2004 and 2016 were considered for the study. Exclusion criteria were short stature due to syndrome, chronic disease, oncology disease, or known bad adherence. Growth predictions were made according to the Gothenburg model and the KIGS model. Growth data from birth until one year after start of GH treatment were collected from medical charts. Predicted height and observed height were then compared. ResultsA total of 123 children, 47 girls (38%) and 76 boys (62%) were included, with a mean age of 5.71 (±1.81 SD) years at start of GH treatment. The Pearson correlation of predicted first-year growth versus growth outcome were r = 0.990 for the Gothenburg model and r = 0.991 for the KIGS model. Studentized residuals were 0.10 ± 0.81 SD and 0.03 ± 0.96 SD, respectively, for the models. The comparison between the two models showed r = 0.995.ConclusionThe Gothenburg model and the KIGS model are equally accurate at predicting height outcome from GH treatment for our study cohort. We therefore promote the use of either model in clinical settings.

scholarly journals Factors Predicting the Near-Final Height in Growth Hormone-Treated Children and Adolescents with Chronic Kidney Disease

2008 ◽  
Vol 93 (4) ◽  
pp. 1359-1365 ◽  
Author(s):  
Richard Nissel ◽  
Anders Lindberg ◽  
Otto Mehls ◽  
Dieter Haffner
Keyword(s):  
Chronic Kidney Disease ◽  
Kidney Disease ◽  
Conservative Treatment ◽  
Outcome Measures ◽  
Growth Response ◽  
Adult Height ◽  
Final Height ◽  
Delayed Puberty ◽  
Gh Therapy ◽  
Gh Treatment

Abstract Context: GH therapy is an accepted measure to increase adult height in young prepubertal patients suffering from growth failure related to chronic kidney disease (CKD). The impact of GH therapy on final height (FH) in CKD patients of pubertal age is unclear. Objective: This study set out to analyze near-FH in a cohort of GH-treated CKD patients. Design, Settings, and Patients: Of 240 evaluable patients in the Pfizer International Growth Database (KIGS) with CKD, 39% were prepubertal and 61% were pubertal at baseline; 45% were on conservative treatment for CKD, 28% were on dialysis, and 27% were in the period after renal transplantation. Main Outcome Measures: Near-FH, relation to pubertal stage, and factors predictive of growth response were the main outcome measures. Results: Mean height sd scores increased continuously during GH treatment until near-FH by 1.2 and 1.6 in boys and girls, respectively. Mean near-FH differed significantly from prepubertal patients showing severely delayed puberty (−3.6), late pubertal patients (−2.9), early pubertal patients (−2.2), and prepubertal patients with normal onset of puberty (−2.0). The initial degree of stunting, degree of bone age retardation, duration of GH therapy, time spent on conservative treatment/dialysis, pubertal delay (&gt;2 sd), gender, and age at start of GH treatment were significant predictors of growth response to GH therapy, explaining between 33 and 61% of the overall variability. Conclusions: Long-term GH therapy of CKD patients in prepubertal and pubertal age results in an increased adult height, but response is diminished in patients on dialysis and/or with severely delayed puberty.

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