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View article: Late-onset Pompe’s disease in pediatrics: results from an Italian national survey on 38 patients and proposal of a targeted diagnostic algorithm
Late-onset Pompe’s disease in pediatrics: results from an Italian national survey on 38 patients and proposal of a targeted diagnostic algorithm Open
Background Late-onset Pompe’s disease (LOPD) is a progressive treatable metabolic myopathy due to partial acid α-glucosidase (GAA) deficiency, with potential onset during the pediatric age. To date, Pompe’s disease is not widely included i…
View article: Urban environments’ impacts on aging in post-industrial cities: reviewing the pathways to healthy aging
Urban environments’ impacts on aging in post-industrial cities: reviewing the pathways to healthy aging Open
Post-industrial cities are characterized by an aging population, deteriorating urban infrastructure, and a range of socio-environmental challenges, including pollution, economic decline, and out-migration. These conditions, alongside the l…
View article: Portal Vein Obstruction in Pediatric Liver Transplant Patients: An Evaluation of Self‐Reported Management Practices
Portal Vein Obstruction in Pediatric Liver Transplant Patients: An Evaluation of Self‐Reported Management Practices Open
Background and Aims Portal vein obstruction (PVO) is a known complication after pediatric liver transplantation (pLT). Effective management strategies are crucial in improving patient outcomes. This study investigated the various practice …
View article: Lapdoctor: Multicentre Validation of a Scoring System for Preoperative Evaluation of Difficulty of Laparoscopic Donor Nephrectomy
Lapdoctor: Multicentre Validation of a Scoring System for Preoperative Evaluation of Difficulty of Laparoscopic Donor Nephrectomy Open
We previously developed and validated LAPDOCTOR (LAParoscopic-Donor-nephreCTomy-scORe), a novel scoring system for the preoperative assessment of the difficulty of living donor nephrectomy (LDN). To prove its significance, we extended our …
View article: Normothermic Machine Perfusion of Explanted Human Metabolic Livers: A Proof of Concept for Studying Inborn Errors of Metabolism
Normothermic Machine Perfusion of Explanted Human Metabolic Livers: A Proof of Concept for Studying Inborn Errors of Metabolism Open
The human liver plays a central metabolic role; however, its physiology may become imbalanced in inborn errors of metabolism (IEM), a broad category of monogenic disorders. Liver transplantation has been increasingly used to improve patien…
View article: Impairment of Innate Immunity and Depletion of Vaccine‐Induced Memory B and T Cells in the Absence of the Spleen
Impairment of Innate Immunity and Depletion of Vaccine‐Induced Memory B and T Cells in the Absence of the Spleen Open
Splenectomy or congenital asplenia is associated with severe reduction of memory B cells and increased risk of fulminant sepsis by encapsulated bacteria. Current guidelines recommend vaccinations against these pathogens before or after spl…
View article: Fairness and pitfalls of the Italian waiting list for elective liver transplantation: The ECALITA registry study
Fairness and pitfalls of the Italian waiting list for elective liver transplantation: The ECALITA registry study Open
Liver-transplantation waiting-list success-rates have significantly improved in Italy, with patients with hepatocellular-carcinoma and/or HBV-related diseases being favored. High MELD-score, old-age, short-stature, and blood-group O were s…
View article: Waiting list mortality and 5-year transplant survival benefit of patients with MASLD: An Italian liver transplant registry study
Waiting list mortality and 5-year transplant survival benefit of patients with MASLD: An Italian liver transplant registry study Open
The present research addresses the critical need to understand the evolving landscape of liver transplantation indications, mainly focusing on metabolic dysfunction-associated steatotic liver disease (MASLD) in Italy. Given the significant…
View article: Focal adhesion kinase and its epigenetic interactors as diagnostic and therapeutic hints for pediatric hepatoblastoma
Focal adhesion kinase and its epigenetic interactors as diagnostic and therapeutic hints for pediatric hepatoblastoma Open
Background Hepatoblastoma (HB) is the most common pediatric hepatic malignancy. Despite the progress in HB treatment, investigating HB pathomechanisms to optimize stratification and therapies remains a focal point to improve the outcome fo…
View article: Incidence, management and outcomes in hepatic artery complications after paediatric liver transplantation: protocol of the retrospective, international, multicentre HEPATIC Registry
Incidence, management and outcomes in hepatic artery complications after paediatric liver transplantation: protocol of the retrospective, international, multicentre HEPATIC Registry Open
Introduction Hepatic artery complications (HACs), such as a thrombosis or stenosis, are serious causes of morbidity and mortality after paediatric liver transplantation (LT). This study will investigate the incidence, current management pr…
View article: Simultaneous or sequential kidney-liver transplantation in primary hyperoxaluria
Simultaneous or sequential kidney-liver transplantation in primary hyperoxaluria Open
Primary hyperoxaluria type 1 is responsible for pediatric kidney failure in 1 to 2% of cases. Novel therapies based on RNA interference are changing the natural history of the disease. However, for those who will progress to kidney failure…
View article: Biliary atresia in preterm infants: a single center experience and review of literature
Biliary atresia in preterm infants: a single center experience and review of literature Open
Introduction The diagnosis of biliary atresia (BA) remains challenging, and there is still uncertainty regarding the optimal time to perform a Kasai portoenterostomy (KPE). Little is known about the difficulties in the diagnosis and outcom…
View article: European Society of Pediatric Radiology survey of perioperative imaging in pediatric liver transplantation: (3) postoperative imaging
European Society of Pediatric Radiology survey of perioperative imaging in pediatric liver transplantation: (3) postoperative imaging Open
Background Liver transplantation is the state-of-the-art curative treatment for end-stage liver disease. Imaging is a key element in the detection of postoperative complications. So far, limited data is available regarding the best radiolo…
View article: Histopathological Spectrum and Molecular Characterization of Liver Tumors in the Setting of Fontan-Associated Liver Disease
Histopathological Spectrum and Molecular Characterization of Liver Tumors in the Setting of Fontan-Associated Liver Disease Open
Purpose: Univentricular heart is corrected with the Fontan procedure (FP). In the long term, so-called Fontan-associated liver diseases (FALDs) can develop. The aim of this study is to analyze the molecular profile of FALDs. Methods: FALDs…
View article: Article Molecular Profile of Liver Tumors in the Setting of Fontan-Associated Liver Disease
Article Molecular Profile of Liver Tumors in the Setting of Fontan-Associated Liver Disease Open
Purpose: To analyze the genetic and molecular profile of liver nodules in the context of Fontan-associated liver disease (FALD) with the aim of identifying possible biomarkers for predictive outcome and personalized therapy. Methods: this …
View article: The Italian data on SARS-CoV-2 infection in transplanted patients support an organ specific immune response in liver recipients
The Italian data on SARS-CoV-2 infection in transplanted patients support an organ specific immune response in liver recipients Open
Introduction The study of immune response to SARSCoV-2 infection in different solid organ transplant settings represents an opportunity for clarifying the interplay between SARS-CoV-2 and the immune system. In our nationwide registry study…
View article: Prevalence, management and efficacy of treatment in portal vein obstruction after paediatric liver transplantation: protocol of the retrospective international multicentre PORTAL registry
Prevalence, management and efficacy of treatment in portal vein obstruction after paediatric liver transplantation: protocol of the retrospective international multicentre PORTAL registry Open
Introduction Portal vein obstruction (PVO) consists of anastomotic stenosis and thrombosis, which occurs due to a progression of the former. The aim of this large-scale international study is to assess the prevalence, current management pr…
View article: Differences in Physical Activity Levels between Healthy and Transplanted Children: Who Needs More Tips?
Differences in Physical Activity Levels between Healthy and Transplanted Children: Who Needs More Tips? Open
Background: Advances in the medical-surgical field have significantly increased the life expectancy of patients undergoing solid organ transplantation but this exposes patients to long-term complications due to chronic therapies and change…
View article: Biomarkers to predict disease progression and therapeutic response in isolated methylmalonic acidemia
Biomarkers to predict disease progression and therapeutic response in isolated methylmalonic acidemia Open
Methylmalonic Acidemia (MMA) is a heterogenous group of inborn errors of metabolism caused by a defect in the methylmalonyl‐CoA mutase (MMUT) enzyme or the synthesis and transport of its cofactor, 5′‐deoxy‐adenosylcobalamin. It is characte…
View article: Systematic use of magnetic double J stent in pediatric kidney transplantation: A single‐center experience
Systematic use of magnetic double J stent in pediatric kidney transplantation: A single‐center experience Open
Background The intraoperative insertion of a double J stent (DJS) is known to reduce urological complications and is broadly accepted in kidney transplant (KTx) patients. The magnetic ureteral DJS (mDJS) represents a valid alternative devi…