The Evaluation of Flow-Mediated Vasodilation in the Brachial Artery Correlates With Endothelial Dysfunction Evaluated by Nitric Oxide Synthase Metabolites in Marfan Syndrome Patients.

Marfan syndrome (MS) is of the most common connective tissue disorders. Although most patients have mutations in the fibrillin-1 gene (FBN1) and more than 1,700 mutations have been described, there are no mutations in less than 10% of patients. Aortic dilation is the most important complication; it involves chronic inflammatory processes and endothelial dysfunction. Prospective study from March 2015 to January 2017, in a cohort of 32 patients of MS confirmed by Ghent criteria and 35 controls of both genders, with a median age of 26 years (18-56). Patients had no comorbidities such as diabetes, hypertension, and/or neoplasms. They were not being treated with statin, NSAIDs, calcium antagonists, oral nitrates, and/or beta-blockers during 7 days prior to the study and patients with smoking history in the last 4 years. Controls were matched by age and gender. We analyzed endothelial dysfunction by flow-mediated vasodilation in the brachial artery, determining the maximum peak flow in the reactive hyperemia phase with a Philips Envisor device with Doppler capability. Its correlation with serum levels of biological markers that could participate in endothelial dysfunction pathways such as NO3-/NO2- ratio, NO2- , citrulline, TNFα, IL-1, IL-6, IL-10, IL-8, osteopontin, ICAM, VCAM, and NO3-/NO2- was determined. Endothelial dysfunction was found in 21 MS patients (65%). The aortic annulus (AAo) was of 27 mm (22-40) and 24 mm (22-30) (p = 0.04) in MS patients with and without dysfunction. The level of NO3-/NO2- ratio, was of 108.95 ± 12.05 nM/ml in controls vs. 170.04 ± 18.76 nM/ml in MS (p = 0.002), NO2- was of 33.78 ± 3.41 vs. 43.95 ± 2.59 nM/ml (p = 0.03), citrulline 62.65 ± 3.46 vs. 72.81 ± 4.35 µMol/ml (p = 0.06). VCAM median was 39 pg/ml (0-86) vs. 32 pg/ml (11-66) (p = 0.03), respectively. The correlation of VCAM with triglycerides (TG) was of 0.62 (p = 0.005). There were no differences in TNFα, IL-1, IL-6, IL-8, IL-10, and osteopontin. MS endothelial dysfunction is related to aortic diameters, and increased levels of VCAM, L-citrulline and NO3-/NO2- ratio, NO2- . VCAM-1 has a significant correlation with TG and could play a significant role in endothelial dysfunction.

Clinical impact of a 6-week preoperative very low calorie diet on body weight and liver size in morbidly obese patients.

BACKGROUND: Laparoscopic Roux-en-Y gastric bypass (LRYGB) may be a technically challenging surgical technique when features such as thick abdominal wall and increased liver volume are present. Very low calorie diets in the form of liquid meal replacements given 6 weeks prior to surgery have proven to decrease liver volume. The aim of our study was to assess the effect of a 6-week preoperative low calorie/regular diet on liver volume and body weight in morbidly obese patients. METHODS: A pilot study was carried out in 20 morbidly obese patients with an 800-kcal diet for 6 weeks. They were followed weekly to ensure proper compliance. CT scan was used for determining liver volume every 2 weeks in order to assess the impact of the diet. Baseline values were taken as controls for every patient as tied measures. Statistical analysis was suitable to variable scaling and performed using SPSS v. 20.0. Parametric and non-parametric test for tied measures were done. Any p value lesser than 0.05 or 5% was considered as statistically significant. RESULTS: Twenty patients adhered to the diet intervention for 6 weeks prior to LRYGB. Median ± SD age was 34.5 ± 11.5 years and 17 were female (85%). Heart rate and blood pressure did not vary across the study. Initial median ± SD BMI was 46.02 ± 5.29 kg/m2 (range 38.7-54.8). Repeated and tied measurements across the 6 weeks of treatment within individuals resulted statistically significant for reducing BMI (p < 0.0001). CT scan assessed liver volume initially as a control and in weeks 2, 4 and 6. Parametric and non-parametric assessment for multiple measurements also showed statistical significance among these values (p < 0.0001). Diet tolerability was additionally evaluated with a questionnaire showing more than 80% of acceptability with discrete rates of nausea (15%) and diarrhoea (15%). CONCLUSIONS: Based on our results, we demonstrated that a very low calorie diet with home ingredients is capable for effectively reducing body weight and liver size in morbidly obese patients. This relatively short intervention (4 to 6 weeks) was accomplished in all our patients with a high frequency of compliance and a low rate of secondary effects.

Systemic lupus erythematosus risk factors for coronary artery calcifications.

OBJECTIVE: Premature atherosclerosis in patients with SLE is partially explained by traditional risk factors; therefore, we aimed to identify lupus-related risk factors for coronary artery calcifications. METHODS: An inception cohort of 139 lupus patients (93% females) was screened for coronary artery calcifications using Multidetector CT, after 5.1 years of follow-up. Clinical and immunological variables and cardiovascular risk factors were assessed longitudinally. Also, 100 age- and sex-matched healthy subjects were studied. Correlates for calcifications were analysed in lupus patients, including levels of lipids and inflammatory molecules in samples obtained at enrolment, mid-term follow-up and at screening. RESULTS: At enrolment, lupus patients were 27.2 (9.1) years of age and with a disease duration of 5.4 (3.8) months. Calcifications were detected in 7.2% of patients and 1% of controls [unadjusted odds ratio (OR) 7.7, 95% CI 1.05, 336.3, P = 0.02]. In lupus, calcifications were detected since the age of 23 years and from 3 years of diagnosis. Patients with calcifications were older, post-menopausal, and had higher levels of serum apolipoprotein B and Framingham risk scores (P < 0.05). Lupus-related factors identified included age at diagnosis, IgG aCLs, cumulative lupus activity, length of moderate/severe activity and cumulative dose of prednisone and CYC (P < 0.05). Use of anti-malarials was protective (P = 0.006). Logistic regression analysis showed as predictors of calcification: disease duration (OR 15.1, 95% CI 2.6, 87.2), age at enrolment (OR 8.5, 95% CI 1.7, 43.0) and SLEDAI 2000 update (SLEDAI-2K) mean area under the curve (OR 12.3, 95% CI 2.5, 61.8). Longitudinal analyses of lipids and inflammatory molecules did not differ between patients. CONCLUSIONS: Disease activity is a potentially modifiable risk factor for coronary artery calcifications in SLE. Therefore, management of traditional risk factors plus tight control of lupus activity, including the use of anti-malarials, is recommended.

Wallenberg's Syndrome: An Unusual Case of Dysphagia.

A 56-year-old man presented with sudden-onset oropharyngeal dysphagia and vomiting of central etiology. Neurological evaluation showed uvula deviation to the left, paresis of the mid-right portion of the soft palate, lateralization of gaze to the right side, and dysphonia. Magnetic resonance imaging (MRI) showed an infarction in the left lateral medullary region, therefore the diagnosis of Wallenberg's syndrome was established. The neurological issues along with the dysphagia gradually improved and the patient was discharged.