Orphanet J Rare Dis
December 2024
Background: Mucopolysaccharidosis (MPS) type 1 S and type 2 are rare lysosomal storage disorders characterized by impaired enzyme production, resulting in glycosaminoglycans accumulation within lysosomes. Enzyme Replacement Therapy (ERT) with laronidase and idursulfase are first line treatments, respectively. However, infusion-related hypersensitivity reactions (HR) may lead to ERT discontinuation.
View Article and Find Full Text PDFPatients with esophageal cancer and concomitant liver cirrhosis (LC) pose a surgical challenge because of the increased risk of postoperative complications and mortality. Purpose of this study was to review the existing literature and estimate perioperative short-term outcomes of esophagectomy in this patient population. Systematic review and meta-analysis.
View Article and Find Full Text PDFBackground: Minimally invasive distal gastrectomy (MIDG) has been shown to be associated with improved short-term outcomes compared to open distal gastrectomy (ODG) in patients with locally advanced gastric cancer (LAGC). The impact of MIDG on long-term patient survival remains debated. Aim was to compare the MIDG vs.
View Article and Find Full Text PDFBackground: Minimally invasive distal gastrectomy (MIDG) has been shown to improve short-term outcomes compared with open distal gastrectomy (ODG) in patients with early (EGC) and locally advanced gastric cancer (LAGC). The impact of MIDG on patient survival remains debated. This study aimed to compare the effect of MIDG versus ODG on long-term survival.
View Article and Find Full Text PDFBackground: In the realm of allergen immunotherapy (AIT), the quality of evidence varies across different products, making it unjustifiable to extend overall conclusions to all AIT products, as highlighted by WAO and EAACI.
Objective: To confirm the efficacy of the 300 IR 5-grass pollen sublingual AIT (SLIT)-tablet through a specific meta-analysis of randomized controlled trials (RCTs) involving patients with allergic rhino-conjunctivitis (ARC) with/without mild/intermittent asthma.
Methods: Data from published RCTs on the 300 IR 5-grass SLIT-tablet were gathered from electronic databases (MEDLINE, ISI Web of Science, LILACS, the Cochrane Library and ClinicalTrial.
The increasing interest in Waste-to-Chemical (WtC) technologies operating with Solid Recovered Fuels (SRF) from non-recyclable plastic streams requires a quantitative analysis on the actual convenience of this alternative valorization pathway. This study assesses SRF in selected WtC technologies for hydrogen and methanol production and compares it with the well-established practice of co-combustion in the cement industry. Two case studies are considered: the first one represents the current scenario where SRF is used in co-combustion for cement production meanwhile the chemical is produced by steam reforming; in the second scenario, the cement plant is fed with pet-coke only, leaving SRF as a feedstock for WtC.
View Article and Find Full Text PDFThis systematic review aims to evaluate the current evidence regarding safety and efficacy of magnetic sphincter augmentation (MSA) for the treatment of gastroesophageal reflux disease (GERD) after laparoscopic sleeve gastrectomy (LSG). Conversion to Roux-en-Y gastric bypass (RYGB) carries the risk of surgical and metabolic complications and may be contraindicated in patients with normalized or near-normalized body mass index. The LINX™ procedure aims to restore LES competency and to repair the crura.
View Article and Find Full Text PDFThe optimal treatment for paraesophageal hiatus hernia (PEH) is controversial. While crural buttressing with mesh shows promises in reducing recurrences, the decision to use mesh during minimally invasive PEH repair is largely subjective. Due to these uncertainties, we conducted a survey to examine current clinical practices among surgeons and to assess which are the most important determinants in the decision-making process for mesh placement.
View Article and Find Full Text PDFPurpose: Anastomotic leak (AL) remains the most important complication after left-sided colic anastomoses and technical complications during anastomotic construction are responsible of higher leakage incidence. Powered circular stapler (PCS) in colorectal surgery has been introduced in order to reduce technical errors and post-operative complications due to the manual circular stapler (MCS).
Methods: A systematic review and meta-analysis were performed.
Introduction: Surgical repair of hiatal hernia (HH) is plagued by high recurrence rates. Hiatoplasty failure has been identified as a major determinant of recurrent symptoms and HH, but there is no consensus on the optimal surgical approach to minimize this complication and hiatal mesh reinforcement remains controversial. The use of the falciform ligament as an autologous rotational flap to support crural repair has been proposed as a potential solution.
View Article and Find Full Text PDFBackground: Biological therapies, such as mepolizumab, have transformed the treatment of severe eosinophilic asthma. Although mepolizumab's short-term effectiveness is established, there is limited evidence on its ability to achieve long-term clinical remission.
Objective: To evaluate the long-term effectiveness and safety of mepolizumab, explore its potential to induce clinical and sustained remission, and identify baseline factors associated with the likelihood of achieving remission over 24 months.
J Laparoendosc Adv Surg Tech A
August 2024
Biomedical devices implanted transabdominally have gained popularity over the past 50 years in the treatment of gastroesophageal reflux disease, paraesophageal hiatal hernia, and morbid obesity. Device-related foregut erosions (FEs) represent a challenging event that demands special attention owing to the potential of severe postoperative complications and death. The aim was to provide an overview of full-thickness foregut injury leading to erosion associated with four types of biomedical devices.
View Article and Find Full Text PDFBackground: Radical esophagectomy, including thoracic duct resection (TDR), has been proposed to improve regional lymphadenectomy and possibly reduce the risk of locoregional recurrence. However, because of its impact on immunoregulation, some authors have expressed concerns about its possible detrimental effect on long-term survival. The purpose of this review was to assess the influence of TDR on long-term survival.
View Article and Find Full Text PDFIntroduction: Traditionally, radical prostatectomy (RP) has been considered a contraindication to minimally invasive inguinal hernia repair. Purpose of this systematic review was to examine the current evidence and outcomes of minimally invasive inguinal hernia repair after RP.
Materials And Methods: Web of Science, PubMed, and EMBASE data sets were consulted.
Asthma is a disease characterized by chronic airway hyperresponsiveness and inflammation, with signs of variable airflow limitation and impaired lung function leading to respiratory symptoms such as shortness of breath, chest tightness and cough. Eosinophilic asthma is a distinct phenotype that affects more than half of patients diagnosed with severe asthma. It can be effectively treated with monoclonal antibodies targeting specific immunological signaling pathways that fuel the inflammation underlying the disease, particularly Interleukin-5 (IL-5), a cytokine that plays a crucial role in asthma.
View Article and Find Full Text PDF: Thoracic duct ligation (TDL) during esophagectomy has been proposed to reduce the risk of postoperative chylothorax. Because of its role in immunoregulation, some authors argued that it had an unfavorable TDL effect on survival. The aim of this study was to analyze the effect of TDL on overall survival (OS).
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