Abstract
BackgroundPrader–Willi syndrome (PWS) is often related to severe obesity and type-2 diabetes mellitus (T2DM). However, few studies, and none in Korea, have examined prevalence of T2DM and other variables in PWS. The aim of this study was to identify the prevalence and associated risk factors for T2DM in Korean patients with PWS.MethodsWe performed a retrospective cohort study of the 84 PWS patients aged 10 or over (10.3–35.8 years of age) diagnosed with PWS at Samsung Medical Center from 1994 to 2016. We estimated occurrence of T2DM according to age (10–18 years versus >18 years), body mass index (BMI), genotype, history of growth hormone therapy, homeostasis model of assessment-insulin resistance (HOMA-IR), and the presence of dyslipidemia, hypogonadism, or central precocious puberty. Additionally, we investigated cutoff values of risk factors for development of T2DM.ResultsTwenty-nine of a total 211 patients, diagnosed with PWS over the study period, were diagnosed as having T2DM (13.7%, mean age 15.9 ± 3.6 years). In the >18 years group, obesity, HOMA-IR, and presence of dyslipidemia, hypogonadism, or central precocious puberty were associated with the occurrence of T2DM in univariate analysis. In multivariate logistic regression analysis, only obesity (p = 0.001) and HOMA-IR (p < 0.001) were significant predictive factors for T2DM. Based on the receiver operating a characteristic curve analysis, the cutoff values of HOMA-IR and BMI for predicting T2DM were >2.7 and >28.49 kg/m2, respectively. Of the 29 patients, seven had ≥1 microvascular complication, with non-proliferative diabetic retinopathy in 6 of 7 cases. Advanced age and HOMA-IR were positively correlated with diabetic microvascular complications (p < 0.05, Spearman correlation coefficient 0.393 and 0.434, respectively).ConclusionsThe prevalence of diabetes in Korean PWS was similar to that in previous results. BMI and HOMA-IR were strong predictive factors for the development of T2DM in PWS. We specifically suggest the regular monitoring of glucose homeostasis parameters through a detailed settlement of ethnically specific cutoff values for BMI and HOMA-IR in PWS to prevent progression of T2DM and diabetic microvascular complications.
Highlights
Prader–Willi syndrome (PWS) is often related to severe obesity and type-2 diabetes mellitus (T2DM)
This can lead to severe obesity in childhood [3], which often progressively develops into type-2 diabetes mellitus (T2DM), which is eventually associated with increased morbidity and mortality in PWS
The proportion of patients with severe obesity was higher in PWS-DM group compared to the 84 subjects in total (p < 0.03) (Additional file 1: Fig. S1)
Summary
Prader–Willi syndrome (PWS) is often related to severe obesity and type-2 diabetes mellitus (T2DM). The aim of this study was to identify the prevalence and associated risk factors for T2DM in Korean patients with PWS. Patients with PWS show poor feeding and failure to thrive until nine months of age, they tend to be obese after due to hypothalamic pituitary dysregulation–induced hyperphagia with a lack of satiety. This can lead to severe obesity in childhood [3], which often progressively develops into type-2 diabetes mellitus (T2DM), which is eventually associated with increased morbidity and mortality in PWS. Uncontrolled DM-induced microvascular complications such as diabetic retinopathy, neuropathy, and nephropathy further impair quality of life in PWS.
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