Glucose Intolerance and Diabetes Mellitus In Patients with the Prader-Labhart-Willi-Syndrome1

Illig, R; Tschumi, Ariane I.; Vischer, D

doi:10.1159/000400794

Cited by 4 publications

(3 citation statements)

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“…They are also in contradiction with the speculation that insulin levels in PWS were only low before obesity sets in. This was the explanation used in the study in which single children were found to have low insulin levels besides many subjects with high insulin levels [36]. Our data, however, show that not only underweight, but also the overweight prepubertal PWS children have low insulin levels as it is the case in classical GHD [37].…”

Section: Discussionmentioning

confidence: 47%

Low insulin, IGF-I and IGFBP-3 levels in children with Prader-Labhart-Willi syndrome

Eiholzer¹,

Stutz²,

Weinmann³

et al. 1998

European Journal of Pediatrics

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It is well established that insulin-like growth factor I (IGF-I), insulin-like growth factor binding protein-3 (IGFBP-3) and insulin are low in growth hormone deficiency, but due to their dependence on nutrition, they are elevated in healthy obese children. As the presence of growth hormone deficiency in Prader-Labhart-Willi syndrome (PWS) is still controversial, we studied insulin, IGF-I and IGFBP-3 levels in 19 children with PWS (age range 0.5-14.6 years). Serum concentrations of insulin (SDS: -0.7+/-0.9, P = 0.01) and IGF-I (SDS: -0.7+/-0.8, P = 0.002) were low, but IGFBP-3 (SDS: -0.3+/-1.2, P = 0.2) was normal compared to normal weight age-matched children. Since children with PWS are typically obese, insulin, IGF-I and IGFBP-3 levels should be compared to normal obese children who present increased levels of these hormones. In comparison to data of healthy obese children reported in the literature, not only IGF-I, but also IGFBP-3 levels are low and fasting insulin levels even very low, suggesting a growth hormone deficiency.

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Section: Discussionmentioning

confidence: 47%

Low insulin, IGF-I and IGFBP-3 levels in children with Prader-Labhart-Willi syndrome

Eiholzer¹,

Stutz²,

Weinmann³

et al. 1998

European Journal of Pediatrics

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“…The syndrome was described in Switzerland about 25 years ago'-2 and in England 6 years later.3 At least 170 patients have been reported with the syndrome but only about 20 of them have been adults.4-6 Twenty-four patients over age 15 years are described here.…”

mentioning

confidence: 98%

Prader-Willi Syndrome after age 15 years.

Laurance¹,

Brito²,

Wilkinson³

1981

Archives of Disease in Childhood

131

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SUMMARY Twenty-four patients, all of them over 15 years, with the Prader-Willi syndrome are described. Obesity, often extreme, associated with an insatiable appetite, was their principal handicap and this was made worse by educational subnormality and hypogonadism. Three of them developed diabetes. Each attended a special school or an adult training centre. Although most of them were of short stature and had scoliosis, 2 were tall but they were even more severely mentally retarded than is usually the case. Nine other patients died aged between 3 and 23 years. The most common cause of death was cor pulmonale.

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“…46 The insulin resistance found is similar to that of simple obesity,47 and clinically the diabetes behaves as type II nonketotic diabetes responding to weight reduction and/or oral hypoglycaemic agents. If insulin is required a simple once daily long acting preparation is usually effective.…”

Section: Diagnosis Of Prader-willi Syndrome (1) Laboratory Diagnosismentioning

confidence: 72%

The Prader-Willi syndrome.

Donaldson¹,

Chu²,

Cooke³

et al. 1994

Archives of Disease in Childhood

125

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Glucose Intolerance and Diabetes Mellitus In Patients with the Prader-Labhart-Willi-Syndrome1

Cited by 4 publications

References 0 publications

Low insulin, IGF-I and IGFBP-3 levels in children with Prader-Labhart-Willi syndrome

Low insulin, IGF-I and IGFBP-3 levels in children with Prader-Labhart-Willi syndrome

Prader-Willi Syndrome after age 15 years.

The Prader-Willi syndrome.

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