Insulin Sensitivity and Pancreatic ß-Cell Function in Ecuadorian Women With Turner Syndrome.

Objective: To assess insulin sensitivity and pancreatic ß-cell function in an adult population of Ecuadorian individuals with Turner syndrome (TS). Design and Methods: This was a cross-sectional correlational study conducted in TS subjects (>20 years old; n = 38). A standard 2-h oral glucose tolerance test was performed in both women with TS and the reference group. Glucose, lipids, insulin, and C-peptide concentrations were measured. Homeostasis Model Assessment (HOMA) of Insulin Resistance, Quantitative Insulin Sensitivity Check Index, McAuley, Matsuda, and Belfiore indices were calculated to evaluate the degree of insulin resistance (IR). The pancreatic ß-cell function was assessed using HOMA-ß, basal C-Peptide Index (CPI), and CPII at 120'. Results: A higher prevalence of impaired glucose tolerance was found in TS subjects compared with the reference group. Although significant differences were found for glucose concentrations at 60' and 120' (but not at 0'), only the baseline insulin concentrations differed significantly between the two groups. The values of the IR indices were statistically different between study and reference groups. A significant number of TS subjects diagnosed with IR were differently classified according to the index applied. The concentrations of C-peptide at 0' and 120' of TS subjects were similar to those of the control group. In contrast, the CPI and CPII values in the study group were significantly lower than those in the control group. Conclusion: It is impossible to select the best surrogate method for the assessment of IR in women with TS. The CPI and CPII values could be preferable to other indices to assess the pancreatic ß-cell function in TS subjects. Our findings suggest that IR and pancreatic ß-cell dysfunction could be independent events in women with TS, and both conditions seem to be caused by the disease per se. Our results imply that early screening and intervention for TS would be therapeutic for TS women.

Metabolic Syndrome as a Risk Factor for Sensorineural Hearing Loss in Adult Patients with Turner Syndrome.

BACKGROUND AND PURPOSE: Metabolic syndrome (MetS) is a disorder associated with an increased risk of cardiovascular disease. The frequency of each component of MetS in Turner syndrome (TS) subjects is high. An elevated incidence of hearing loss has also been reported in TS. Sensorineural hearing loss (SNHL) affects at least half of young women with TS. The association between MetS and SNHL has not been previously considered in TS. The aim of this study is to evaluate the association between these two conditions. PATIENTS AND METHODS: Cross-sectional anthropometric, cardio-metabolic and audiological data were obtained from a cohort consisting of unrelated TS subjects (>20 years of age; n = 93). Metabolic syndrome was defined according to the International Diabetes Federation criteria. Types and severity of hearing loss were based on the American Speech Hearing Association guidelines. RESULTS: Hearing loss was detected in 74% of ears from adult TS subjects and SNHL was observed in half of our TS subjects. The prevalence of MetS in TS subjects with or without SNHL was 64% and 11%, respectively (P < 0.05). After adjusting for age, MetS was related to a ninefold increase in the odds of SNHL. This odds increased in a stepwise manner as the number of MetS components increased. CONCLUSION: MetS and its individual components were associated factors for SNHL in TS subjects. A reduction in the number and severity of the components of MetS might potentially contribute to decreasing the progression of SNHL at younger ages, but further studies will be needed to explain the underlying pathological mechanism connecting MetS and SNHL.

Anthropometric variables as cardiovascular risk predictors in a cohort of adult subjects with Turner syndrome.

BACKGROUND AND PURPOSE: Excessive adiposity is associated with cardiometabolic complications in Turner syndrome (TS) subjects. Reference data for predictive anthropometric indices of overweight/obesity and metabolic syndrome (MetS) are lacking for subjects with TS. The purpose of this study was to identify the best anthropometric predictor of cardiometabolic risk in a Latin-American cohort of TS subjects. PATIENTS AND METHODS: This was a cross-sectional correlational study conducted in adult TS subjects (n=88) over the past seven years. Anthropometric parameters, body composition and biochemical variables were evaluated in a study and in a reference (n=57) group. Overweight/obesity and MetS were diagnosed using international consensus. The area under the ROC curve (AUC-ROC) was then used to determine the value of each anthropometric variable in predicting MetS or overweight/obesity. RESULTS: The prevalence of MetS and overweight/obesity in TS subjects was 40% and 48%, respectively. All anthropometric and cardiometabolic variables were significantly increased in TS subjects when compared to the reference group, except for body mass index (BMI) and HDL-c. To detect MetS and overweight/obesity, waist to height ratio (WHtR) was found to have a higher correlation with cardiometabolic variables (TC, LDL-c, HDL-c levels and the LDL-c/HDL-c ratio), and to have a higher AUC-ROC and odds ratio than BMI, waist circumference (WC) and the waist to hip ratio (WHR). CONCLUSION: The prevalence of MetS and overweight/obesity is elevated in TS subjects. WHtR was the most useful variable in predicting the presence of MetS and overweight and obesity in this TS cohort. A combination of WHtR with BMI or with WC could have the best clinical utility in identifying adult TS subjects with overweight/obesity and MetS, respectively.

EMG biofeedback technique improves muscular activity in patietns with hypoglossal-facial anastomosis

En el presente estudio se evaluó la actividad electromiográfica (EMG) facial en tres pacientes (un hombre y dos mujeres) sometidos a una anastomosis nerviosa hipogloso-facial, con parálisis facial posquirúrgica y a quienes se aplicó un programa de entrenamiento con biorretroalimentación. Los pacientes se sentaron frente a un electromiógrafo Autogenic 1 700, de manera que la señal analógica visual de retroalimentación fue un parámetro de la actividad muscular del lado afectado de su cara. Se colocaron electrodos superficiales en grupos musculares especificos teniéndose cuidado de colocarlos en los mismos sitios en los tres pacientes y en todas las sesiones. El entrenamiento costó de dos fases. En la primera: los pacientes realizaron los movimientos de: sonreír, fruncir la nariz y de profusión de los labios con su máximo esfuerzo, mientras mantenían su lengua relajada. En la segunda: activaron su lengua intentando mantener los músculos faciales relajados. Los resultados mostraron un incremento significativo de la actividad muscular del lado afectado de la cara al realizar los movimientos faciales sin la activación de la lengua, y una relajación importante en toda la cara al activar la lengua. Los pacientes también mostraron una mejoría moderada en el aspecto clínico. En menos de nueve semanas de entrenamiento usando la aproximación de biorretroalimentación, se observaron los primeros cambios significativos, lo que sugiere una transferencia exitosa de actividad neuronal en la anastomosis hipogloso-facial, de acurdo con otros reportes