Resting energy expenditure in argininosuccinic aciduria and in other urea cycle disorders.

No data are available on the specific energy needs of patients affected with Urea Cycle disorders (UCD) and especially argininosuccinic aciduria (ASA). In our experience, ASA patients tend to develop central adiposity and hypertriglyceridemia when treated with apparently adequate energy intake, while the other UCD do not. The aim of this study was to evaluate anthropometric parameters, body composition, risk of metabolic syndrome (MS) and resting energy expenditure (REE), both by indirect calorimetry (IC) and predictive equations, in UCD patients.

Quality of Life of Adult Survivors After Extra Corporeal Membrane Oxygenation (ECMO): A Quantitative Study.

Introduction: Extracorporeal Membrane Oxygenation (ECMO) is a technique used in the treatment of patients with severe respiratory failure. A lot of studies in the literature investigated the survival rate of patients treated with ECMO, but few are those that assess the long-term outcomes and the quality of life (QoL) of survivors.

Materials And Methods: A retrospective observational study with prospective QoL assessment through administration of the questionnaire EuroQol-5 Dimension was conducted in acute respiratory distress syndrome patients who h ad ECMO as a rescue therapy for reversible refractory hypoxemia from January 2013 until December 2015 in an Italian referral ECMO center.

[Risk factors for delirium in intensive care unitin liver transplant patients].

Unlabelled: . Risk factors for delirium in intensive care unit in liver transplant patients.

Introduction: Delirium is a disorder of the state of consciousness characterized by acute onset and temporal variability of cognitive abilities.

Enzymatic replacement therapy for Hunter disease: Up to 9 years experience with 17 patients.

Hunter disease is an X-linked lysosomal storage disorder characterized by progressive storage of glycosaminoglycans (GAGs) and multi-organ impairment. The central nervous system (CNS) is involved in at least 50% of cases. Since 2006, the enzymatic replacement therapy (ERT) is available but with no effect on the cognitive impairment, as the present formulation does not cross the blood-brain barrier.

Improvement of Cardiomyopathy After High-Fat Diet in Two Siblings with Glycogen Storage Disease Type III.

Glycogenosis type III (GSD III) is an autosomal recessive disorder due to amylo-1,6-glucosidase deficiency. This disease causes limit dextrin storage in affected tissues: liver, skeletal muscles, and heart in GSD IIIa and only liver in GSD IIIb. Cardiomyopathy is quite frequent in GSD IIIa with variable severity and progression of manifestations.

Is the prevalence of overweight reducing at age 5-6 years? Ten years data collection in ASL Milano 2.

Background: Prevalence of overweight and obesity has been reported as high even in preschool age children. However, recent international reports suggest that prevalence is now plateauing in pediatric age. Up to now no data are available on prevalence changes in Italy in the new Millennium.

NAVIGATED VERSUS MECHANICAL KNEE REPLACEMENT: COMPARISON BETWEEN INITIAL NAVIGATED AND CONVENTIONAL CASES PERFORMED BY THE SAME SURGEON.

Objective: The postoperative mechanical axis in 36 cases of total knee arthroplasty using navigated and mechanical alignment systems was evaluated and compared. All the operations were performed over the same period by the same surgeon and team, who were beginners in the navigated technique and experienced in the mechanical technique.

Methods: Between 2008 and 2010, 36 total knee prostheses were performed and compared: 25 navigated and 11 mechanical procedures.