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<jats:title>Abstract</jats:title><jats:sec><jats:title>Introduction</jats:title><jats:p>Liver transplantation (LT) is the choice of therapeutic option for end‐stage hepatic GSD patients; however, reports about the long‐term outcome of LT in these patients have remained controversial.</jats:p></jats:sec><jats:sec><jats:title>Methods</jats:title><jats:p>We performed a systematic review and meta‐analysis of observational studies published until Dec 31, 2021, that investigated the long‐term outcome of LT in hepatic GSD patients. A literature search in the MEDLINE/PubMed, EMBASE,Cochrane Library, Scopus and Web of Science Core Collection databases was performed.</jats:p></jats:sec><jats:sec><jats:title>Results</jats:title><jats:p>14 studies with 210 patients were included in our analysis. As the results showed, the pooled proportion of GSD patients with complications after liver transplant (e.g., hemorrhagic shock, biliary complications, tacrolimus encephalopathy, chronic hepatitis, hepatic artery thrombosis, hepatic adenoma, sepsis, liver dysfunction, chronic rejection, acute cellular rejection, and CMV infection) was 27.7% (95% CI: 20.42–35.67) without heterogeneity (<jats:italic>I<jats:sup>2</jats:sup></jats:italic> = 24.04%), as calculated by the random‐effect model. The pooled proportion of GSD patients with complications related to GSD after LT, including HCC (Hepatocellular carcinoma), renal complication, muscle problems, delayed menarche, persistent neutropenia, pneumonitis, renal failure, and hepatic adenoma was 22.2% (95% CI: 7.97–40.01) with high heterogeneity (<jats:italic>I<jats:sup>2</jats:sup></jats:italic> = 82.47%). Subgroup analysis including the age of patients (adult/pediatric), duration of follow‐up, and type of donor was conducted to investigate the resources of heterogeneity.</jats:p></jats:sec><jats:sec><jats:title>Conclusion</jats:title><jats:p>According to our investigation and review analysis, most GSD patients showed significant outcome improvement after liver transplantation. Overall, our findings showed an excellent outcome of liver transplantation in GSD patients; however, it needs further investigations to be confirmed.</jats:p></jats:sec>

<jats:title>Abstract</jats:title><jats:sec><jats:title>Introduction</jats:title><jats:p>Bronchial anastomotic dehiscence (AD) is an uncommon complication following lung transplantation that carries significant morbidity and mortality. The objective of this study was to characterize fungal and bacterial infections in ADs, including whether infections following AD were associated with progression to bronchial stenosis.</jats:p></jats:sec><jats:sec><jats:title>Methods</jats:title><jats:p>This was a single‐center study of 615 lung transplant recipients between 6/1/2015 and 12/31/2021. Airway complications were defined according to ISHLT consensus guidelines.</jats:p></jats:sec><jats:sec><jats:title>Results</jats:title><jats:p>22 of the 615 recipients (3.6%) developed an AD. Bronchial ischemia or necrosis was common prior to dehiscence (68.1%). Fourteen (63.6%) recipients had bacterial airway infections, most commonly with Gram‐negative rods, prior to dehiscence. Thirteen (59.1%) recipients had an associated pleural infection, most commonly with Candida species (30.8%). Post‐dehiscence Aspergillus species were isolated in 4 recipients, 3 of which were de novo infections. Eleven had bacterial infections prior to dehiscence resolution, most commonly with <jats:italic>Pseudomonas aeruginosa</jats:italic>. Eleven recipients developed airway stenosis requiring dilation and/or stenting. Development of secondary infection prior to AD resolution was not associated with progression to stenosis (OR = .41, 95% CI = .05‐3.30, <jats:italic>p</jats:italic> = .41).</jats:p></jats:sec><jats:sec><jats:title>Conclusions</jats:title><jats:p>Gram‐negative bacterial infections are common before and after AD. Pleural infection should be suspected in most cases. Infections prior to healing were not associated with subsequent development of airway stenosis.</jats:p></jats:sec>

<jats:title>Abstract</jats:title><jats:sec><jats:title>Introduction</jats:title><jats:p>The data on post‐transplant cyclophosphamide (PTCy) in pediatric acute leukemia after matched allo‐HSCT are limited to case series. The present study aimed to assess the results of PTCy‐based GVHD prophylaxis in a large cohort of children with acute leukemia after matched allo‐HSCT.</jats:p></jats:sec><jats:sec><jats:title>Methods</jats:title><jats:p>A retrospective analysis of 190 pediatric patients with acute leukemia who had a first allograft between 2008 and 2020 from a matched sibling donor (MSD) or matched unrelated donor (MUD) was carried out. In the MSD setting, GVHD prophylaxis consisted of PTCy alone (<jats:italic>n</jats:italic> = 28) for the study group, and calcineurin inhibitor (CNI) ± antimetabolite (<jats:italic>n</jats:italic> = 30) for the control group. In MUD setting, most patients in the study group received GVHD prophylaxis with PTCy+CNI+mycophenolate mofetil (<jats:italic>n</jats:italic> = 42, 66.7%) or PTCy+CNI+sirolimus (<jats:italic>n</jats:italic> = 12, 19%). All patients (<jats:italic>n</jats:italic> = 69) in the control group received ATG+CNI+antimetabolite.</jats:p></jats:sec><jats:sec><jats:title>Results</jats:title><jats:p>After MUD allo‐HSCT, the incidences of acute GVHD grade III‐IV and moderate/severe chronic GVHD were significantly lower in the PTCy group compared to control (6.6% vs. 35.0% and 12.7% vs. 47.1%, respectively, <jats:italic>p</jats:italic> &lt; .0001). Five‐year GVHD‐free, relapse‐free survival (GRFS) after MUD allo‐HSCT was higher in the PTCy group compared to control (35.1% vs. 7.3%, <jats:italic>p</jats:italic> &lt; .0001). At the same time, there was no significant difference between both groups after MSD allo‐HSCT.</jats:p></jats:sec><jats:sec><jats:title>Conclusions</jats:title><jats:p>In pediatric acute leukemia, PTCy‐based GVHD prophylaxis for MUD allo‐HSCT is a feasible and effective option that results in a low incidence of GVHD. Compared to the ATG‐based approach, PTCy provides better control of GVHD in children. In pediatric allo‐HSCT from MSD, PTCy demonstrates comparable effectiveness to conventional GVHD prophylaxis.</jats:p></jats:sec>