KIGS Highlights: Growth Hormone Treatment in Prader-Willi Syndrome

Tauber, Maïthé; Cutfield, Wayne S.

doi:10.1159/000110475

Cited by 25 publications

(28 citation statements)

References 5 publications

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“…According to cross-sectional, prospective and retrospective studies, GHD prevalence ranged from 58% to 100% [24,25]. Similar prevalence rates (74%) were observed in the international, observational KIGS database [26].…”

Section: Introductionsupporting

confidence: 60%

GH treatment, BMI and different genotypes in patients with Prader-Willi syndrome and scoliosis: Is there any relationship?

Greggi¹,

Pipitone²,

Martikos³

et al. 2013

JBiSE

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The purpose of this study is to try to find a protocol defining a clinical diagnostic procedure for the patients to be admitted to the authors' Institute to receive treatment for either suspected or confirmed diagnosis of spine deformity in Prader-Willi syndrome (PWS). The aim is to evaluate every subject from the diagnostic point of view, assessing variability of clinical expression and evolution of spinal deformity in the light of the related genetic aspects, thus providing a univocal protocol. The present series only includes patients (18 cases) with PWS, 7 hospitalized for surgical treatment of scoliosis, 11 followed-up at the authors' institute only for conservative treatment of scoliosis. Both BMI tracks (weight/height 2 ) and BMI Z-score (only for children older than 2 years) were assessed. Moreover, the GH treatment was evaluated for each group of patients as follows: being administered, suspended or no treatment. Finally, the gene was compared with BMI. No relationship was observed either between GH treatment and mean BMI or between genetics and mean BMI. More patients should be seen by the authors to confirm or refute the current findings.

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Section: Introductionsupporting

confidence: 60%

GH treatment, BMI and different genotypes in patients with Prader-Willi syndrome and scoliosis: Is there any relationship?

Greggi¹,

Pipitone²,

Martikos³

et al. 2013

JBiSE

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show abstract

“…commun.] and the International KIGS Databases (6.4 years) [21,29]. Growth velocity during the first year of treatment and the decrease of BMI were quite comparable in our database compared to KIGS data [29].…”

Section: Discussionmentioning

confidence: 56%

Endocrine Disorders in Children with Prader-Willi Syndrome – Data from 142 Children of the French Database

et al. 2010

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Aim: The first results from the French National Prader-Willi pediatric database in a cohort of 142 children aged 0.2–18.8 years are reported. This database gathers information about the endocrine dysfunctions traditionally described in Prader-Willi patients. Methods: Questionnaires were filled in by the patients’ practitioners. The coordination team of the reference center performed the statistical analysis. Results: Median BMI Z-score was +1.3 for a median age of 7.1 years, and 40% of the population were overweight or obese (International Obesity Task Force 2000 criteria). Growth hormone deficiency was present in 80% of patients and 86.7% were treated, with a height gain of +1 SD and a BMI reduction of –0.8 Z-score achieved in the first year of treatment. Hypogonadism was present in 49% of patients, and hypothyroidism in 24.4%. Glucose intolerance was found in 4% of patients, but no diabetes mellitus was detected in the 74 patients explored. Conclusion: Our report gives an overview of endocrine dysfunctions recorded in a large registry database of French children and adolescents with Prader-Willi syndrome. The database, which now encompasses six southern regions of France, will be further extended to the whole country and to adult patients.

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“…In the international observational KIGS database, 74% of 424 children with PWS tested for GHD had low GH levels. IGF-1 levels were also low in nearly all children [12]. Nevertheless, GH peak to provocative stimulus during childhood has been demonstrated to be heterogeneous.…”

Section: Discussionmentioning

confidence: 99%

“…However, data about the prevalence of GH deficiency (GHD) in very young subjects with PWS are still unclear. Additionally, the percentage of children with PWS that fulfill the criteria for GHD varies from 40 to 100% [10,12]. This discrepancy may be attributed to the different stimulation tests used, as well as to poor GH assay standardization and/or to the presence of obesity.…”

Section: Introductionmentioning

confidence: 99%

Growth Hormone Response to Standard Provocative Stimuli and Combined Tests in Very Young Children with Prader-Willi Syndrome

et al. 2014

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Background: In Prader-Willi syndrome (PWS) a reduced growth hormone (GH) response to several stimulators has been documented in many studies, but none have focused on very young children. We evaluated the pattern of GH secretion in very young PWS patients. Patients and Methods: Twenty-seven genetically confirmed PWS children (10 females, aged 0.4-5 years, mean: 2.2 ± 1.4 years) were included. All subjects underwent standard provocative tests (clonidine, CLO; and arginine, ARG) and one combined test [growth hormone-releasing hormone (GHRH) plus pyridostigmine (13 patients) or GHRH plus arginine (14 patients)]. Insulin-like growth factor-1 (IGF-1) levels were also measured. Results: While standard tests (CLO and ARG) showed low GH peak in 85.2 and 70.4% of the patients, respectively, the combined test was found to be normal in 85.2%. IGF-1 was low in 66.7% of patients. Out of 27 patients, 3 (11%) showed a normal GH peak with both standard tests (group A), 6 (22%) to one of the standard tests (group B) and 18 (67%) presented a low response to both standard tests (group C). Four subjects showed low response to both the combined and standard tests and reduced IGF-1. Conclusion: Our data suggest that very young PWS children seem to have impaired hypothalamic GHRH secretion with a normal GH pituitary reserve.

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KIGS Highlights: Growth Hormone Treatment in Prader-Willi Syndrome

Cited by 25 publications

References 5 publications

GH treatment, BMI and different genotypes in patients with Prader-Willi syndrome and scoliosis: Is there any relationship?

GH treatment, BMI and different genotypes in patients with Prader-Willi syndrome and scoliosis: Is there any relationship?

Endocrine Disorders in Children with Prader-Willi Syndrome – Data from 142 Children of the French Database

Growth Hormone Response to Standard Provocative Stimuli and Combined Tests in Very Young Children with Prader-Willi Syndrome

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