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Impaired glucose metabolism in subjects with the Williams-Beuren syndrome: A five-year follow-up cohort study.

ABSTRACT: OBJECTIVE:The Williams-Beuren syndrome (WS) is associated with impaired glucose metabolism (IGM) early in adulthood. However, the pathophysiology of IGM remains poorly defined, due to the lack of longitudinal studies investigating the contribution of ?-cell dysfunction and impaired insulin sensitivity. This study aimed at assessing incidence of IGM and the underlying mechanisms in WS adults. METHODS:This observational, longitudinal (5-year), cohort study enrolled thirty-one consecutive WS subjects attending a tertiary referral center. An oral glucose tolerance test (OGTT) was performed yearly and used to classify patients as normal or IGM, including impaired fasting glucose (IFG) and/or impaired glucose tolerance (IGT) and diabetes mellitus (DM), and to calculate surrogate measures of insulin secretion and/or sensitivity. RESULTS:IGM patients were 18 (58.1%, three DM) at baseline and 19 (61.3%, five DM) at end-of-follow-up. However, 13 individuals changed category of glucose homeostasis in both directions during follow-up (8 progressors, 5 regressors) and 18 did not (8 non-progressors, 10 non-regressors). New cases of IGM and DM were 11.1 and 2.53 per 100 persons-year, respectively, and were treated non-pharmacologically. In the whole cohort and, to a higher extent, in progressors, indices of early-phase insulin secretion and insulin sensitivity decreased significantly from baseline to end-of-follow-up, with concurrent reduction of the oral disposition index and insulin secretion-sensitivity index-2 (ISSI-2), compensating insulin secretion for the level of insulin resistance. No baseline measure independently predicted progression, which correlated with change from baseline in ISSI-2. Compared with patients with normal glucose homeostasis, IGT subjects had impaired insulin sensitivity, whereas insulin secretion was reduced only in those with IFG+IGT or DM. CONCLUSIONS:IGM incidence is high in young adults with WS, suggesting the need of early screening and timed intervention. As in classical type 2 diabetes, impaired insulin sensitivity and ?-cell dysfunction contribute, in this sequence, to progression to IGM and DM.

SUBMITTER: Lunati ME

PROVIDER: S-EPMC5650138 | biostudies-literature | 2017

REPOSITORIES: biostudies-literature

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Impaired glucose metabolism in subjects with the Williams-Beuren syndrome: A five-year follow-up cohort study.

Lunati Maria Elena ME Bedeschi Maria Francesca MF Resi Veronica V Grancini Valeria V Palmieri Eva E Salera Simona S Lalatta Faustina F Pugliese Giuseppe G Orsi Emanuela E

PloS one 20171020 10

<h4>Objective</h4>The Williams-Beuren syndrome (WS) is associated with impaired glucose metabolism (IGM) early in adulthood. However, the pathophysiology of IGM remains poorly defined, due to the lack of longitudinal studies investigating the contribution of β-cell dysfunction and impaired insulin sensitivity. This study aimed at assessing incidence of IGM and the underlying mechanisms in WS adults.<h4>Methods</h4>This observational, longitudinal (5-year), cohort study enrolled thirty-one consec ...[more]

PMID: 29053727

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Project description:PURPOSE: To assess longitudinal changes in parapapillary atrophy in the adult population of Greater Beijing. METHODS: The population-based Beijing Eye Study 2006 included 3251 subjects who had participated in the Beijing Eye Study 2001 and returned for re-examination. The mean age was 60.4 ± 10.1 years. Using optic disc photographs, we measured parapapillary atrophy which was divided into alpha zone and beta zone. RESULTS: Overall progression rate of alpha zone was seen in 0.6 ± 0.1% (95% confidence interval (CI):0.3,0.9) of the subjects and of beta zone in 8.2 ± 0.5% (95%CI:7.2,9.1) of the subjects. In binary regression analysis, rate of progression of alpha zone was significantly associated higher age (P = 0.04) and the co-progression of zone Beta (P<0.001). Rate of progression of beta zone was significantly associated with higher age (P<0.001; odds ratio (OR):1.11;95%CI:1.10,1.14), higher intraocular pressure (P<0.001;OR:1.10;95%CI:1.05,1.14), higher myopic refractive error (P<0.001;OR:0.71; 95%CI:0.67,0.75), rural region of habitation (P = 0.002;OR: 0.58; 95%CI:0.41,0.82), presence of glaucomatous optic nerve damage (P<0.001;OR:2.89; 95%CI:1.62,5.14), co-progression of alpha zone (P<0.001;OR:7.13;95%CI:2.43,20.9), absence of arterial hypertension (P = 0.03;OR: 0.70; 95%CI:0.51,0.96), and thicker central corneal thickness (P = 0.02;OR:1.01;95%CI:1.00,1.01). Subjects with a non-glaucomatous optic nerve damage (n = 22) as compared to the remaining subjects did not vary in the progression rate of alpha zone (0.0% versus 0.6 ± 0.1%; P = 1.0) and beta zone (8.2 ± 0.5% versus 6.3 ± 0.6%;P = 1.0). CONCLUSIONS: In adult Chinese in Greater Beijing, the 5-year progression rate of beta zone of parapapillary atrophy (seen in 8.2 ± 0.5% of subjects) was significantly correlated with higher age, rural region of habitation, absence of arterial hypertension, higher intraocular pressure, higher myopic refractive error, thicker central corneal thickness, and presence of glaucoma. It was not associated with non-glaucomatous optic nerve damage.

Project description:IntroductionThere is increasing evidence that the in utero environment affects the health and disease risk of offspring throughout their lives. The long-term effect of maternal hyperglycaemia on offspring glucose metabolism is of interest in a public health perspective. The aim of this study was to examine the association between in utero exposure to maternal glycaemia and offspring glucose metabolism.MethodsMother-child pairs were recruited from an RCT to prevent gestational diabetes mellitus where 855 healthy pregnant women were randomised to exercise or standard antenatal care. The original RCT detected no group differences in gestational diabetes mellitus prevalence or insulin resistance. The two groups were analysed as one group in the present study. Maternal glucose levels were assessed after 2-hour 75-gram oral glucose tolerance tests in pregnancy week ~34. Offspring outcomes were evaluated at ~9 years of age and included fasting glucose and homeostatic model assessment of insulin resistance. Multivariable regression models were performed, controlling for potential hereditary and lifestyle confounding factors.ResultsComplete data were available for 105 mother-child pairs. The regression analysis showed a positive association between maternal and offspring fasting glucose that was borderline significant (beta=0.18, 95% CI [-0.00027, 0.37], p=0.050). We did not find significant associations between maternal fasting glucose and offspring insulin resistance (beta=0.080, 95% CI [-0.087, 0.25], p=0.34), or between maternal 2-hour glucose and offspring fasting glucose (beta=0.016, 95% CI [-0.038, 0.070], p=0.56) or insulin resistance (beta=0.017, 95% CI [-0.032, 0.065], p=0.49).ConclusionsAssessing a homogeneous group of healthy mother-child pairs, we found a borderline significant positive association between maternal and offspring fasting glucose, which persisted after adjustment for potential hereditary and lifestyle confounding factors. Our findings support other similar studies and highlight that improving the metabolic health of pregnant women, and women in childbearing age, should remain a key public health priority.Clinical trial registrationClinicalTrials.gov, identifier NCT00476567.

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Impaired glucose metabolism in subjects with the Williams-Beuren syndrome: A five-year follow-up cohort study.

Publications

Impaired glucose metabolism in subjects with the Williams-Beuren syndrome: A five-year follow-up cohort study.

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