Characterization of Diabetes Mellitus in Japanese Prader-Willi Syndrome

Abstract

Prader-Willi syndrome (PWS) is frequently associated with marked obesity and diabetes mellitus (DM). Although the overall frequency of DM in PWS ranges from 7–20%, there is only limited data available on Japanese patients. This study evaluated five factors associated with DM in PWS: 1) frequency, 2) age of onset, 3) risk factors, 4) long-term complications and 5) treatment. Sixty-five patients, ranging in age from 10 to 53 yr, were studied retrospectively. The frequency of DM in patients over 10 yr of age was 26.2% (17/65 patients). The age of DM onset ranged from 10 to 29 yr with a median age of 15 yr. The body mass index (BMI) was significantly higher in the DM group in comparison with the non-DM group. The number of patients using growth hormone (GH) in the DM group was significantly lower than the number that did not. Proteinuria (urinary excretion of albumin/creatinine at spot collection: U-Alb/Cr ≥300 mg/gCr) was observed in 1/17 patients (5.9%), microalbuminuria (U-Alb/Cr 30–300 mg/gCr) was observed in 4/17 patients (23.5%) and nonproliferative retinopathy was observed in 2/17 patients (11.8%). Among oral hypoglycemic agents, alpha-glucosidase inhibitors (α-GI) were most often used in our patients (10/17, 58.8%). Eleven out of 17 patients (64.7%) had been treated with insulin.

Prevalence and risk factors for type 2 diabetes mellitus with Prader–Willi syndrome: a single center experience

“…The prevalence of T2DM in the present study was 13.7% (29/211). This proportion is similar to that of previous studies (7–25%) [ 13 – 16 ]. Considering the prevalence of T2DM in adults (6.1–6.9%) [ 17 ] and 0.2% in children younger than 18 years in Korea [ 18 ], the prevalence rate of T2DM in Korean PWS is sufficiently high to warrant attention.…”

“…A recent Italian cohort study revealed a 13.5% prevalence of T2DM, which was similar to our results [ 15 ]. This proportion is relatively lower than the results of a Japanese study, which were 26.2% [ 16 ], but higher than in a French study, 0% [ 19 ]. The differences in the described frequencies of T2DM have presumably resulted from various sizes of the PWS base population, the diagnostic approach to T2DM, the wide range of the age group, and the study period.…”

“…Several studies have reported that insulin resistance would not be significantly involved in the causes of T2DM in PWS due to the better insulin sensitivity of patients compared to obese individuals with PWS [ 5 , 33 ]. However, according to recent studies including our results, although patients with PWS may have relative insulin sensitivity, insulin resistance and obesity are considered the most important factors in the occurrence of T2DM through the destruction of β-cell function [ 15 , 16 , 37 ].…”

Efficacy of sodium-glucose cotransporter 2 inhibitor with glucagon-like peptide-1 receptor agonist for the glycemic control of a patient with Prader-Willi syndrome: a case report

“…GH may decrease insulin sensitivity, whereas the improvement of body composition by GH treatment may lower the risk of DM. Tsuchiya et al (3) reported that the frequency of DM in PWS was 9.7% among the patients treated with GH, while 41.2% in the patient who did not receive GH treatment developed to DM. Among the Korean patients with PWS, 72.4% in the DM group and 90.9% in the non-DM group had a history of GH treatment (10).…”

Successful peritoneal dialysis for the end-stage kidney disease associated with Prader–Willi syndrome: a case report

“…PWS patients become overweight in early childhood due to an insatiable appetite and, therefore, onset of DM is generally during adolescence at the earliest [ 3 ]. Prevalence of DM was reported 22% in 68 patients older than 13 years (age of youngest person with DM) in United Kingdom [ 3 ], whereas 26.2% in 65 Japanese patients older than 10 years (age of youngest person with DM) [ 4 ]. The type of DM was mostly non-insulin dependent in both cohorts [ 3 , 4 ].…”