“… Fig. 2 a Comparison of adjusted estimated means of Turner syndrome (TS) height standard deviation score (HSDS) references (± SE): target height (TH)-corrected HSDS values, normal-height standard deviation score values, and HSDS values based on the reference populations of Ranke [ 19 ], Cabrol [ 20 ] and Westerlaken [ 21 ]. b Comparison of adjusted estimated means of change in TS HSDS references (± SE): TH-corrected and normal ΔHSDS, and ΔHSDS values based on the reference populations of Ranke [ 19 ], Cabrol [ 20 ] and Westerlaken [ 21 ] …”
Section: Resultsmentioning
confidence: 99%
“…NS and TS children’s growth responses, from start through 4 years of GH treatment, were compared with national reference growth charts and the untreated disease-specific references for NS [ 7 ] and TS children [ 19 – 21 ]. The latter are hereafter referred to as the ‘Ranke’ [ 7 , 19 ], ‘Cabrol’ [ 20 ] and ‘Westerlaken’ [ 21 ] reference populations.…”
BackgroundTurner syndrome (TS) and Noonan syndrome (NS) are distinct syndromes associated with short stature and other similar phenotypic features. We compared the responses to growth hormone (GH) therapy of TS and NS patients enrolled in the NordiNet® International Outcome Study (IOS) or the American Norditropin Studies: Web-Enabled Research (ANSWER) Program, which collect information on GH therapy in clinical practice.MethodsRepeated-measures regression analysis was performed on change in height standard deviation score (HSDS) and target-height-corrected HSDS, based on national normal references and treatment-naïve disease-specific references. Models were adjusted for baseline age and HSDS, and average GH dose. The study population was paediatric patients with TS and NS in the NordiNet® IOS and ANSWER Program. Longitudinal growth responses over 4 years were evaluated.ResultsIn 30 NS patients (24 males; baseline age 8.39 ± 3.45 years) and 294 TS patients (7.81 ± 3.22 years), 4-year adjusted ΔHSDS were +1.14 ± 0.13 and +1.03 ± 0.04, respectively (national references). Based on untreated, disease-specific references, 4-year adjusted ΔHSDS for NS and TS were +1.48 ± 0.10 and +1.79 ± 0.04. The analyses showed a significant increase in HSDS over time for both NS and TS (P < 0.0001). ΔHSDS in NS was higher with younger baseline age; ΔHSDS in TS was higher for patients with younger baseline age and higher GH dose.ConclusionsNS and TS patients responded well and similarly over 4 years of GH treatment.Electronic supplementary materialThe online version of this article (doi:10.1186/s13633-015-0015-1) contains supplementary material, which is available to authorized users.
“… Fig. 2 a Comparison of adjusted estimated means of Turner syndrome (TS) height standard deviation score (HSDS) references (± SE): target height (TH)-corrected HSDS values, normal-height standard deviation score values, and HSDS values based on the reference populations of Ranke [ 19 ], Cabrol [ 20 ] and Westerlaken [ 21 ]. b Comparison of adjusted estimated means of change in TS HSDS references (± SE): TH-corrected and normal ΔHSDS, and ΔHSDS values based on the reference populations of Ranke [ 19 ], Cabrol [ 20 ] and Westerlaken [ 21 ] …”
Section: Resultsmentioning
confidence: 99%
“…NS and TS children’s growth responses, from start through 4 years of GH treatment, were compared with national reference growth charts and the untreated disease-specific references for NS [ 7 ] and TS children [ 19 – 21 ]. The latter are hereafter referred to as the ‘Ranke’ [ 7 , 19 ], ‘Cabrol’ [ 20 ] and ‘Westerlaken’ [ 21 ] reference populations.…”
BackgroundTurner syndrome (TS) and Noonan syndrome (NS) are distinct syndromes associated with short stature and other similar phenotypic features. We compared the responses to growth hormone (GH) therapy of TS and NS patients enrolled in the NordiNet® International Outcome Study (IOS) or the American Norditropin Studies: Web-Enabled Research (ANSWER) Program, which collect information on GH therapy in clinical practice.MethodsRepeated-measures regression analysis was performed on change in height standard deviation score (HSDS) and target-height-corrected HSDS, based on national normal references and treatment-naïve disease-specific references. Models were adjusted for baseline age and HSDS, and average GH dose. The study population was paediatric patients with TS and NS in the NordiNet® IOS and ANSWER Program. Longitudinal growth responses over 4 years were evaluated.ResultsIn 30 NS patients (24 males; baseline age 8.39 ± 3.45 years) and 294 TS patients (7.81 ± 3.22 years), 4-year adjusted ΔHSDS were +1.14 ± 0.13 and +1.03 ± 0.04, respectively (national references). Based on untreated, disease-specific references, 4-year adjusted ΔHSDS for NS and TS were +1.48 ± 0.10 and +1.79 ± 0.04. The analyses showed a significant increase in HSDS over time for both NS and TS (P < 0.0001). ΔHSDS in NS was higher with younger baseline age; ΔHSDS in TS was higher for patients with younger baseline age and higher GH dose.ConclusionsNS and TS patients responded well and similarly over 4 years of GH treatment.Electronic supplementary materialThe online version of this article (doi:10.1186/s13633-015-0015-1) contains supplementary material, which is available to authorized users.
“…À l'inverse, dans le ST, une inflexion progressive est noté e aprè s 9 ans pour des tailles finales moyennes de l'ordre de 142 cm, soit À20 cm des tailles moyennes fé minines selon l'ethnie. Cette taille est é troitement corré lé e à la taille parentale [12].…”
Section: La Croissance Et La Taille Adulte Des Sk Et St Sous L'influeunclassified
“…Several methods are used [12,13]: projected height using appropriate growth charts: predic tion methods, such as the method of Lyon using height SD score and a correction factor [14]: or methods based on bone age [15]. In France, Lyon's method has recently been validated by Cabrol et al [6] on a group of 38 untreated patients with a good correlation coefficient (r = 0.8) and satisfactory accuracy. In the USA, Attie [ 16] has evaluated several prediction methods in 56 patients.…”
Section: Karg F Rmentioning
confidence: 99%
“…For example, in France, FH of 141.1 ± 6.4 cm [4], 142.1 ± 5.6 cm [5], and 142.5 ± 4.9 cm [6] have been reported, corresponding to -3.4 to -3.7 SD score for the general population [7]. Several reports have indicated that growth hormone (GH) in creases growth velocity (GV) in patients with TS [1.…”
Growth hormone (GH) is registered for children with Turner syndrome (TS) in several countries. Improving the final heights (FH) is certainly the most worthy goal of therapy, but evaluation of treatment effect is complicated by methodological difficulties. Several series of FH results have now been published, with estimated benefits ranging from 0-9.3 cm, as compared to predicted height before treatment. The majority of studies report height gains of less than 5 cm, but in these studies, GH was started at a relatively late age and used at low doses. Several approaches can be utilized to improve FH results in TS, including early initiation of GH therapy, increased or optimized GH dose regimens, or optimization of sexual steroid utilization.
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