Growth Charts for Prader-Willi Syndrome During Growth Hormone Treatment

The purpose of the current study was to develop syndrome-specific standardized growth curves for growth hormone–treated Prader-Willi syndrome (PWS) individuals aged 0 to 18 years. Anthropometric growth-related measures were obtained on 171 subjects with PWS who were treated with growth hormone for at least 40% of their lifespan. They had no history of scoliosis. PWS standardized growth curves were developed for 7 percentile ranges using the LMS method for weight, height, head circumference, weight/length, and BMI along with normative 3rd, 50th, and 97th percentiles plotted using control data from the literature and growth databases. Percentiles were plotted on growth charts for comparison purposes. Growth hormone treatment appears to normalize stature and markedly improves weight in PWS compared with standardized curves for non–growth hormone–treated PWS individuals. Growth chart implications and recommended usage are discussed.
Source: Clinical Pediatrics - Category: Pediatrics Authors: Tags: Articles Source Type: research

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This study’s purpose was to describe the results of SCC as well as GFS for PWS patients with early-onset scoliosis (EOS). Methods: PWS patients were identified from 2 international multicenter EOS databases. Scoliosis, kyphosis, spine height (T1-S1), right/left hemithoracic heights/widths (RHTH, LHTH, RHTW, LHTW) were measured pretreatment, postoperation, and at 2-year follow-up. Complications were recorded. Results: Overall, 23 patients with 2-year follow-up were identified. Pretreatment; patients treated with SCC (n=10) had mean age of 1.8±0.6 years; body mass index (BMI), 16±1.5 kg/m2; s...
Source: Journal of Pediatric Orthopaedics - Category: Orthopaedics Tags: Scoliosis Source Type: research
Rationale: Fusionless techniques for early-onset scoliosis (EOS) have evolved to allow near-normal growth while maintaining the correction achieved during the initial surgery. However, such procedures require repeated surgeries and have increased complication rates. We have developed a 2-stage fusion technique using pedicle screws for EOS to reduce patient burden and complication risk. This series describes the clinical and radiological features of 2 patients with EOS who received 2-stage posterior spinal fusion. This surgical method for EOS represents the first of its kind. Patient concerns: Case 1 was a 10-year-old ...
Source: Medicine - Category: Internal Medicine Tags: Research Article: Clinical Case Report Source Type: research
Conclusions: If the general pediatrician bec omes more familiar with PWS, the diagnosis and treatment will start earlier, which is essential to improve the quality of life and care for these individuals.
Source: Revista Paulista de Pediatria - Category: Pediatrics Source Type: research
Conclusions: Our study demonstrates a higher prevalence of hip dysplasia in patients with PWS than previously documented. The age at which hip dysplasia develops remains unknown; therefore, we recommend an ultrasound screening for all infants with PWS at 6 weeks of age and subsequent radiographic studies at 1, 2, 5, 10, and 15 years of age to allow for early diagnosis and intervention. Level of Evidence: Level III—retrospective comparative study.
Source: Journal of Pediatric Orthopaedics - Category: Orthopaedics Tags: Hip Source Type: research
Aim of this study was to evaluate the role of multimodal intraoperative neuromonitoring and mapping (IONM) for detection of intraoperative and postoperative complications in a series of patients with non-idiopathic scoliosis (NI-S).We reviewed IONM records of 32 patients with NI-S (neurofibromatosis, Duchene muscular dystrophy, spinal muscular atrophy, Haberland, Goldenhair, Prader Willi syndrome, mucopolisaccaridosis, tetraparesis, congenital myopathy and neuropathy, achondroplasia, spinal tumor, diastematomyelia) with a mean age at surgery of 14years (range, 6 –25years), who underwent posterior fusion with instrume...
Source: Clinical Neurophysiology - Category: Neuroscience Authors: Source Type: research
Authors: Nishi Y, Tanaka T Abstract We compiled the major adverse events included in the Annual Research Reports of the Foundation for Growth Research published in and after 2000. We conducted a review of approximately 32,000 patients treated with growth hormone (GH) who subsequently developed leukemia and who were registered with the Foundation for Growth Research (from 1975 to December 31 1997). We performed a literature review and found that GH therapy was not associated with leukemia onset in patients with no risk factors for leukemia. We also reported the onset of diabetes mellitus (DM), scoliosis, and respira...
Source: Pediatric Endocrinology Reviews - Category: Endocrinology Tags: Pediatr Endocrinol Rev Source Type: research
Prader-Willi syndrome is characterized by severe hypotonia in infancy, with decreased lean mass and increased fat mass in childhood followed by severe hyperphagia and consequent obesity. Scoliosis and other orthopaedic manifestations of hypotonia are common in children with Prader-Willi syndrome and cause significant morbidity. The relationships among hypotonia, reduced muscle mass and scoliosis have been difficult to establish. Inactivating mutations in one Prader-Willi syndrome candidate gene, MAGEL2, cause a Prader-Willi-like syndrome called Schaaf-Yang syndrome, highlighting the importance of loss of MAGEL2 in Prader-W...
Source: Human Molecular Genetics - Category: Genetics & Stem Cells Authors: Tags: ARTICLES Source Type: research
Prader- Willi Syndrome (PWS) is a multisystem genetic disorder. In infancy, patients are typically hypotonic and may fail to thrive, but as they grow older, many become obese and develop obstructive sleep apnoea (OSA). We aimed to determine the prevalence of the respiratory complications seen in our cohort of patients referred from a regional PWS clinic in London.We performed a retrospective casenote review of the 49 children with PWS seen from 2010 to 2016, highlighting conditions that could influence their respiratory status.The median age was 5.2[0.4-15.8][range]yrs, 27/49(55%)male, BMI z score 0.7[2.1] mean[SD]. Only 7...
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: 7.1 Paediatric Respiratory Physiology and Sleep Source Type: research
Prader Willi syndrome, resulting from the partial deletion or  lack of expression of a region of genes on the paternal chromosome 15, has a number of phenotypic features which predispose affected patients to ventilatory problems. These include generalised hypotonia, abnormal arousal and ventilatory responses to hypoxia and hypercapnia, scoliosis and fre quently, obesity. The spectrum of the resulting respiratory complications thus runs from sleep disordered breathing, to aspiration and respiratory functional impairment.
Source: Paediatric Respiratory Reviews - Category: Respiratory Medicine Authors: Tags: Review Source Type: research
Prader-Willi syndrome (PWS) is the most frequent type of syndromic obesity and major clinical features include muscular hypotonia, early onset of obesity, short stature, scoliosis, developmental delays, reduced spontaneous physical activity, gait and postural disorders. Improving balance and walking ability in these patients could enhance their health status and quality of life.
Source: International Journal of Osteopathic Medicine - Category: Complementary Medicine Authors: Tags: Original Article Source Type: research
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