Unidirectional endobronchial valves for management of persistent air-leaks. Results of a multicenter study

Background: To evaluate the efficacy of Endo-Bronchial Valves in the management of persistent air-leaks (PALs) and the procedural cost. Methods: It was a retrospective multicenter study including consecutive patients with PALs for alveolar pleural fistula (APF) undergoing valve treatment. We assessed the efficacy and the cost of the procedure. Results: Seventy-four patients with persistent air leaks due to various etiologies were included in the analysis. In all cases the air leaks were severe and refractory to standard treatments. Sixty-seven (91%) patients underwent valve treatment obtaining a complete resolution of air-leaks in 59 (88%) patients; a reduction of air-leaks in 6 (9%); and no benefits in 2 (3%). The comparison of data before and after valve treatment showed a significant reduction of air-leak duration (16.2±8.8 versus 5.0±1.7 days; P<0.0001); chest tube removal (16.2±8.8 versus 7.3±2.7 days; P<0.0001); and length of hospital stay (LOS) (16.2±8.8 versus 9.7±2.8 days; P=0.004). Seven patients not undergoing valve treatment underwent pneumo-peritoneum with pleurodesis (n=6) or only pleurodesis (n=1). In only 1 (14%) patient, the chest drainage was removed 23 days later while the remaining 6 (86%) were discharged with a domiciliary chest drainage removed after 157±41 days. No significant difference was found in health cost before and after endobronchial valve (EBV) implant (P=0.3). Conclusions: Valve treatment for persistent air leaks is an effective procedure. The reduction of hospitalization costs related to early resolution of air-leaks could overcome the procedural cost

Vitamin D deficiency is not related to eating habits in children with Autistic Spectrum Disorder

Autism Spectrum Disorder (ASD) is characterized by the impairment of communication and social interaction and by repetitive, restricted and stereotyped interests. ASD is often accompanied by comorbidities; eating disorders are frequent and imply important nutritional deficits (i.e. deficiencies of vitamins, minerals and fatty acids). Vitamin D has a critical role in neurodevelopment and serum levels in ASD are reported inadequate. A useful reference for setting up a correct diet in childhood is the food pyramid, which is inspired by the Mediterranean Diet (MD). The MD guarantees an intake of nutrients, considered optimal to maintain an adequate nutritional status. The aim of this study is to explore serum levels of Vitamin D and food habits (through MD adherence) in a sample of children with ASD and evaluate a possible correlation between these factors

Wolfram Syndrome Type 2: A Systematic Review of a Not Easily Identifiable Clinical Spectrum

Background: Wolfram syndrome (WS) is a rare autosomal recessive disorder that is characterized by the presence of diabetes mellitus, optic atrophy and hearing loss, all of which are crucial elements for the diagnosis. WS is variably associated with diabetes insipidus, neurological disorders, urinary tract anomalies, endocrine dysfunctions and many other systemic manifestations. Since Wolfram and Wagener first described WS in 1938, new phenotypic/genotypic variants of the syndrome have been observed and the clinical picture has been significantly enriched. To date, two main subtypes of WS that associated with two different mutations are known: WS type 1 (WS1), caused by the mutation of the wolframine gene (WS1; 606201), and WS type 2 (WS2), caused by the mutation of the CISD2 gene (WS2; 604928). Methods: A systematic review of the literature was describe the phenotypic characteristics of WS2 in order to highlight the key elements that differentiate it from the classic form. Conclusion: WS2 is the rarest and most recently identified subtype of WS; its clinical picture is partially overlapping with that of WS1, from which it traditionally differs by the absence of diabetes insipidus and the presence of greater bleeding tendency and peptic ulcers

Glycemic variability and Time in range are associated with the risk of overweight and high LDL-cholesterol in children and youths with Type 1 Diabetes

Introduction: Reducing cardiovascular risk factors (CVRFs) exposure in children and youths with type 1 diabetes (T1D) is critical for cardiovascular diseases (CVD) prevention. Long-term exposure to hyperglycaemia, measured by HbA1c, had been recognized as the main factor affecting CVRFs profile. To date, the possible association between short-term glycaemic control and variability measured by continuous glucose monitoring (CGM) metrics and CVRFs has not been explored. The aim of this study was to test the hypothesis that CGM metrics independently contribute to CVRFs exposure in children and youths with T1D. Method: BMI, blood pressure (BP), lipid profile, and CGM data of 895 children and youths with T1D were analysed. Binary multivariable logistic regression analyses were performed to test independent associations between CVRFs (BMI percentile&gt;85th, LDL-c&gt;100 mg/dL, BP&gt;90th percentile) and CGM metrics according to sex and adjusting for confounding factors. Results: In both sexes, metrics of hypoglycaemia and glycaemic variability (coefficient of variation [%CV]) positively correlated with BMI percentile. LDL-c positively correlated with mean glucose and metrics of hyperglycaemia. A negative correlation was found between LDL-c and time in range (TIR). No significant correlations were found between CGM metrics and BP percentiles. In both sexes, TIR&lt;70% was significantly associated with LDL-c&gt;100 mg/dL (OR 3.2 in males, 2.1 in females). In females, CV&gt;36% was significantly associated with overweight (OR 2.1). Conclusions: CGM metrics of glycaemic control and variability were significantly associated with the risk of overweight in females and high LDL-c in both sexes