Prader-Willi syndrome, diabetes mellitus and hypogonadism

Nagai, Toshiro; Mori, Masataka

doi:10.1016/s0753-3322(00)88102-0

Cited by 22 publications

(15 citation statements)

References 16 publications

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“…Finally, six patients (25%) showed a partial central hypogonadism for LH/testosterone axis (normal LH despite low testosterone), associated with peripheral gonadal dysfunction for FSH/inhibin B axis (high FSH and low InhB): a hormonal pattern that we consider as a combined hypogonadism. On the other hand, none of our older patients showed the classical hypergonadotropic hypogonadism (high LH/low testosterone associated with high FSH/low inhibin B), in contrast to what previously reported 17,18,24 …”

Section: Discussioncontrasting

confidence: 99%

“…On the other hand, none of our older patients showed the classical hypergonadotropic hypogonadism (high LH/low testosterone associated with high FSH/low inhibin B), in contrast to what previously reported. 17,18,24 As other authors have shown, [10][11][12] we found that most PWS patients are able to start puberty, but successively they have a pubertal arrest. In fact, none of our PWS participants proceeded beyond pubertal stage 3, and some remained prepubertal despite being older than 13AE5 years.…”

Section: Discussionsupporting

confidence: 75%

“…2,4,9,10,16 Other authors have suggested that hypogonadism may be related to a primary gonadal disorder, with elevated basal and stimulated FSH and LH levels. 12,17,18 Recently, it has been suggested that children with PWS display a specific form of combined hypothalamic and peripheral hypogonadism. 11 To better understand the pathophysiology of pubertal development in PWS males, we investigated the pituitary-gonadal axis function in a sample of children, adolescents and adults with genetically confirmed PWS in comparison with a group of healthy controls.…”

Section: Introductionmentioning

confidence: 99%

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Multiple forms of hypogonadism of central, peripheral or combined origin in males with Prader–Willi syndrome

Radicioni

Giorgio

Grugni

et al. 2011

Clinical Endocrinology

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Section: Discussioncontrasting

confidence: 99%

Section: Discussionsupporting

confidence: 75%

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Multiple forms of hypogonadism of central, peripheral or combined origin in males with Prader–Willi syndrome

Radicioni

Giorgio

Grugni

et al. 2011

Clinical Endocrinology

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“…4 Severity levels for ID are typically categorised by broad intelligence quotient (IQ), alongside the required deficits in independent living skills, into mild (IQ [50][51][52][53][54][55][56][57][58][59][60][61][62][63][64][65][66][67][68][69], moderate (IQ [35][36][37][38][39][40][41][42][43][44][45][46][47][48][49], severe and profound (IQ < 20) ID. 5 Acknowledging the wide variation that exists between individuals with ID, typical abilities suggested for each category are outlined in Table 1 [based on World Health Organization (WHO) International Classification of Diseases, Tenth Edition (ICD-10)].…”

Section: Definition Of Intellectual Disability and Case Identificationmentioning

confidence: 99%

“…Prader-Willi syndrome). 46 Physical inactivity and sedentary behaviour are both common among people with ID, with only a minority (18-33%) achieving the recommended 30 minutes of moderate to vigorous physical activity (MVPA) daily, 47,48 and < 15% of people with ID complete the recommended 10,000 steps per day. 49 Furthermore, < 10% of adults with ID who live in supported accommodation have an intake of fruit and vegetables that is sufficient for a balanced diet.…”

Section: Type 2 Diabetes and Impaired Glucose Regulationmentioning

confidence: 99%

Screening for glucose intolerance and development of a lifestyle education programme for prevention of type 2 diabetes in a population with intellectual disabilities: the STOP Diabetes research project

Dunkley

Tyrer

Spong

et al. 2017

Programme Grants Appl Res

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BackgroundThe prevalence of type 2 diabetes mellitus (T2DM) and of cardiovascular disease (CVD) is believed to be higher among people with intellectual disability (ID) than in the general population. However, research on prevalence and prevention in this population is limited.ObjectivesThe objectives of this programme of work were to establish a programme of research that would significantly enhance the knowledge and understanding of impaired glucose regulation (IGR) and T2DM in people with ID; to test strategies for the early identification of IGR and T2DM in people with ID; and to develop a lifestyle education programme and educator training protocol to promote behaviour change in a population with ID and IGR (or at a high risk of T2DM/CVD).SettingLeicestershire, UK.ParticipantsAdults with ID were recruited from community settings, including residential homes and family homes. Adults with mild to moderate ID who had an elevated body mass index (BMI) of ≥ 25 kg/m2and/or IGR were invited to take part in the education programme.Main outcome measuresThe primary outcome of the screening programme was the prevalence of screen-detected T2DM and IGR. The uptake, feasibility and acceptability of the intervention were assessed.Data sourcesParticipants were recruited from general practices, specialist ID services and clinics, and through direct contact.ResultsA total of 930 people with ID were recruited to the screening programme: 58% were male, 80% were white and 68% were overweight or obese. The mean age of participants was 43.3 years (standard deviation 14.2 years). Bloods were obtained for 675 participants (73%). The prevalence of previously undiagnosed T2DM was 1.3% [95% confidence interval (CI) 0.5% to 2%] and of IGR was 5% (95% CI 4% to 7%). Abnormal IGR was more common in those of non-white ethnicity; those with a first-degree family history of diabetes; those with increasing weight, waist circumference, BMI, diastolic blood pressure or triglycerides; and those with lower high-density lipoprotein cholesterol. We developed a lifestyle educational programme for people with ID, informed by findings from qualitative stakeholder interviews (health-care professionals,n = 14; people with ID,n = 7) and evidence reviews. Subsequently, 11 people with ID (and carers) participated in pilot education sessions (two groups) and five people attended education for the feasibility stage (one group). We found that it was feasible to collect primary outcome measures on physical activity and sedentary behaviour using wrist-worn accelerometers. We found that the programme was relatively costly, meaning that large changes in activity or diet (or a reduction in programme costs) would be necessary for the programme to be cost-effective. We also developed a quality development process for assessing intervention fidelity.LimitationsWe were able to screen only around 30% of the population and involved only a small number in the piloting and feasibility work.ConclusionsThe results from this programme of work have significantly enhanced the existing knowledge and understanding of T2DM and IGR in people with ID. We have developed a lifestyle education programme and educator training protocol to promote behaviour change in this population.Future workFurther work is needed to evaluate the STOP Diabetes intervention to identify cost-effective strategies for its implementation.Trial registrationClinicalTrials.gov NCT02513277.FundingThe National Institute for Health Research Programme Grants for Applied Research programme and will be published in full inHealth Research Programme Grants for Applied Research; Vol. 5, No. 11. See the NIHR Journals Library website for further project information.

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Gastrointestinal System, Obesity, and Body Composition

Scheimann

Lee²,

Ellis

Management of Prader-Willi Syndrome

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Prader-Willi syndrome, diabetes mellitus and hypogonadism

Cited by 22 publications

References 16 publications

Multiple forms of hypogonadism of central, peripheral or combined origin in males with Prader–Willi syndrome

Multiple forms of hypogonadism of central, peripheral or combined origin in males with Prader–Willi syndrome

Screening for glucose intolerance and development of a lifestyle education programme for prevention of type 2 diabetes in a population with intellectual disabilities: the STOP Diabetes research project

Gastrointestinal System, Obesity, and Body Composition

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