PMID- 28854950
OWN - NLM
STAT- MEDLINE
DCOM- 20180521
LR  - 20231112
IS  - 1750-1172 (Electronic)
IS  - 1750-1172 (Linking)
VI  - 12
IP  - 1
DP  - 2017 Aug 30
TI  - Prevalence and risk factors for type 2 diabetes mellitus with Prader-Willi 
      syndrome: a single center experience.
PG  - 146
LID - 10.1186/s13023-017-0702-5 [doi]
LID - 146
AB  - BACKGROUND: Prader-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. METHODS: We 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. RESULTS: Twenty-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/m(2), 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). CONCLUSIONS: The 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.
FAU - Yang, Aram
AU  - Yang A
AD  - Department of Pediatics, Samsung Medical Center, Sungkyunkwan University School 
      of Medicine, 81 Irwon-ro, Gangnam-gu, Seoul, 06351, South Korea.
FAU - Kim, Jinsup
AU  - Kim J
AD  - Department of Pediatics, Samsung Medical Center, Sungkyunkwan University School 
      of Medicine, 81 Irwon-ro, Gangnam-gu, Seoul, 06351, South Korea.
FAU - Cho, Sung Yoon
AU  - Cho SY
AD  - Department of Pediatics, Samsung Medical Center, Sungkyunkwan University School 
      of Medicine, 81 Irwon-ro, Gangnam-gu, Seoul, 06351, South Korea. 
      nadri1217@naver.com.
FAU - Jin, Dong-Kyu
AU  - Jin DK
AD  - Department of Pediatics, Samsung Medical Center, Sungkyunkwan University School 
      of Medicine, 81 Irwon-ro, Gangnam-gu, Seoul, 06351, South Korea. jindk@skku.edu.
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
DEP - 20170830
PL  - England
TA  - Orphanet J Rare Dis
JT  - Orphanet journal of rare diseases
JID - 101266602
SB  - IM
MH  - Adolescent
MH  - Body Mass Index
MH  - Child
MH  - Diabetes Mellitus, Type 2/*epidemiology
MH  - Female
MH  - Humans
MH  - Insulin Resistance/physiology
MH  - Male
MH  - Obesity/epidemiology
MH  - Prader-Willi Syndrome/*epidemiology
MH  - Prevalence
MH  - Retrospective Studies
MH  - Risk Factors
PMC - PMC5577752
OTO - NOTNLM
OT  - Diabetes mellitus
OT  - Insulin, homeostasis model of assessment-insulin
OT  - Obesity
OT  - Prader-Willi syndrome
COIS- ETHICS APPROVAL AND CONSENT TO PARTICIPATE: Written informed consents were 
      obtained from patients and their parents of each patient, and the Institutional 
      Review Board at Samsung Medical Center approved the study (IRB file number: 
      2017-02-144). CONSENT FOR PUBLICATION: Not applicable. COMPETING INTERESTS: The 
      authors declare that they have no competing interests. PUBLISHER'S NOTE: Springer 
      Nature remains neutral with regard to jurisdictional claims in published maps and 
      institutional affiliations.
EDAT- 2017/09/01 06:00
MHDA- 2018/05/22 06:00
PMCR- 2017/08/30
CRDT- 2017/09/01 06:00
PHST- 2017/04/26 00:00 [received]
PHST- 2017/08/23 00:00 [accepted]
PHST- 2017/09/01 06:00 [entrez]
PHST- 2017/09/01 06:00 [pubmed]
PHST- 2018/05/22 06:00 [medline]
PHST- 2017/08/30 00:00 [pmc-release]
AID - 10.1186/s13023-017-0702-5 [pii]
AID - 702 [pii]
AID - 10.1186/s13023-017-0702-5 [doi]
PST - epublish
SO  - Orphanet J Rare Dis. 2017 Aug 30;12(1):146. doi: 10.1186/s13023-017-0702-5.