Liver transplantation for homozygous familial hypercholesterolemia: a retrospective analysis from Chinese experience.

Background: Homozygous familial hypercholesterolaemia (HoFH) increases risk of premature cardiovascular events and cardiac death. In severe cases of HoFH, clinical signs and symptoms cannot be controlled well by non-surgical treatments, liver transplantation (LT) currently represents the viable option.

Method: To assess the clinical efficacy, prognosis, and optimal timing of LT for HoFH, a retrospective analysis was conducted on the preoperative, surgical conditions, and postoperative follow-up of children who received an LT for HoFH at the Beijing Friendship Hospital over the period from December 2014 to August 2022.

Parental Living Donor Liver Transplantation as a Solution in Medical Treatment-Resistant Cystathionine-β-Synthase Deficiency: A Single-Center Case Series.

Introduction: Cystathionine-β-synthase deficiency (CBSd) is an inherited metabolic liver disease causing morbidities in eyes, skeleton, brain, and vasculature. Despite its potential lethality due to thromboembolism and liver failure, sole diagnosis of CBSd seemed not to fulfill the enlistment criteria for deceased donor liver transplantation in previous reports.

Methods: We retrospectively reviewed three cases of living donor liver transplantation (LDLT) for pediatric CBSd patients responding poorly to conservative treatment in Beijing Friendship Hospital, and a literature review was performed.

Clinical Application of Metagenomic Next-Generation Sequencing (mNGS) in Patients with Early Pulmonary Infection After Liver Transplantation.

Purpose: To examine the clinical utility of metagenomic next-generation sequencing (mNGS) in individuals with early pulmonary infection following liver transplantation.

Patients And Methods: mNGS and traditional detection results were retrospectively collected from 99 patients with pulmonary infection within one week following liver transplantation. These patients were admitted to the Department of Critical Liver Diseases at Beijing Friendship Hospital from February 2022 to February 2024, along with their general clinical data.

ANP32E promotes esophageal cancer progression and paclitaxel resistance via P53/SLC7A11 axis-regulated ferroptosis.

Esophageal cancer (EC) is associated with high mortality rates and widespread resistance to chemotherapeutic agents, like paclitaxel (PTX), posing a significant global public health challenge. ANP32E is a member of the acidic nuclear phosphoprotein 32 family, its specific biological functions and mechanisms in EC remain unclear. Through bioinformatics analysis and clinical tissue sample studies, we observed a marked upregulation of ANP32E expression in EC tissues.

Synthesis of a Metalla[2]catenane, Metallarectangles and Polynuclear Assemblies from Di(N-Heterocyclic Carbene) Ligands.

The 2,7-fluorenone-linked bis(6-imidazo[1,5-a]pyridinium) salt H-1(PF) reacts with AgO in CHCN to yield the [2]catenane [Ag(1)](PF). The [2]catenane rearranges in DMF to yield two metallamacrocycles [Ag(1)](PF). 2,7-Fluorenone-bridged bis-(imidazolium) salts H-L(PF) (L=2 a, 2 b) react with AgO in CHCN to yield metallamacrocycles [Ag(L)](PF) with interplanar distances between the fluorenone rings too small for [2]catenane formation.

An international, multicenter, survey-based analysis of practice and management of acute liver failure.

Acute liver failure (ALF) is an acute liver dysfunction with coagulopathy and HE in a patient with no known liver disease. As ALF is rare and large clinical trials are lacking, the level of evidence regarding its management is low-moderate, favoring heterogeneous clinical practice. In this international multicenter survey study, we aimed to investigate the current practice and management of patients with ALF.

Favorable Outcomes of Liver Transplantation for Hepatopulmonary Syndrome.

Background: Hepatopulmonary syndrome (HPS) is a pulmonary vascular complication of chronic liver disease, which develops insidiously as a result of chronic liver disease. The prognosis for untreated patients with HPS is extremely poor, and liver transplantation (LT) serves as the only effective means for treating this condition. Here, we performed a retrospective analysis to evaluate the efficacy of LT on the survival and long-term prognosis of patients with HPS.

Updated epithelial barrier dysfunction in chronic rhinosinusitis: Targeting pathophysiology and treatment response of tight junctions.

Tight junction (TJ) proteins establish a physical barrier between epithelial cells, playing a crucial role in maintaining tissue homeostasis by safeguarding host tissues against pathogens, allergens, antigens, irritants, etc. Recently, an increasing number of studies have demonstrated that abnormal expression of TJs plays an essential role in the development and progression of inflammatory airway diseases, including chronic obstructive pulmonary disease, asthma, allergic rhinitis, and chronic rhinosinusitis (CRS) with or without nasal polyps. Among them, CRS with nasal polyps is a prevalent chronic inflammatory disease that affects the nasal cavity and paranasal sinuses, leading to a poor prognosis and significantly impacting patients' quality of life.

Unravelling the Roles of Solvophobic Effects and π⋅⋅⋅π Stacking Interactions in the Formation of [2]Catenanes Featuring Di-(N-Heterocyclic Carbene) Building Blocks.

A series of [2]catenanes has been prepared from di-NHC building blocks by utilizing solvophobic effects and/or π⋅⋅⋅π stacking interactions. The dinickel naphthobiscarbene complex syn-[1] and the kinked biphenyl-bridged bipyridyl ligand L yield the [2]catenane [2-IL](OTf) by self-assembly. Solvophobic effects are pivotal for the formation of the interlocked species.

The P1 protein of wheat yellow mosaic virus exerts RNA silencing suppression activity to facilitate virus infection in wheat plants.

Wheat yellow mosaic virus (WYMV) causes severe wheat viral disease in Asia. However, the viral suppressor of RNA silencing (VSR) encoded by WYMV has not been identified. Here, the P1 protein encoded by WYMV RNA2 was shown to suppress RNA silencing in Nicotiana benthamiana.

Anesthesia and Critical Care for the Prediction and Prevention for Small-for-size Syndrome: Guidelines from the ILTS-iLDLT-LTSI Consensus Conference.

Background: During the perioperative period of living donor liver transplantation, anesthesiologists and intensivists may encounter patients in receipt of small grafts that puts them at risk of developing small for size syndrome (SFSS).

Methods: A scientific committee (106 members from 21 countries) performed an extensive literature review on aspects of SFSS with proposed recommendations. Recommendations underwent a blinded review by an independent expert panel and discussion/voting on the recommendations occurred at a consensus conference organized by the International Liver Transplantation Society, International Living Donor Liver Transplantation Group, and Liver Transplantation Society of India.

Impact of immunosuppression level on liver allograft fibrosis after pediatric liver transplantation: a retrospective cohort study.

Background: Liver allograft fibrosis (LAF) is prevalent among children with long-term survival after liver transplantation (LT). The authors aimed to identify clinical risk factors, with a focus on the impact of immunosuppression (IS) level in the early post-transplant period on LAF.

Methods: A retrospective study was conducted on pediatric LT recipients with at least 1-year of follow-up.

Impact of pure laparoscopic surgery on bile duct division of living donor left lateral section procurement.

Background: Although laparoscopic living donor left lateral section liver procurements represents an established and safe procedure, there remains much discussion on this topic. In particular, the issue of whether laparoscopic living donor liver procurement increases the difficulty of the surgery and potential complications for recipients continue to confound experts in this field.

Methods: In this report, data from 180 cases of living donor left lateral section liver transplantation patients were analyzed retrospectively.

Epithelial Tight Junction Anomalies in Nasal Inverted Papilloma.

Objectives: As a critical component of the epithelial barrier, tight junctions (TJs) are essential in nasal mucosa against pathogen invasion. However, the function of TJs has rarely been reported in nasal inverted papilloma (NIP). This study aims to investigate the potential factors of TJs' abnormality in NIP.

Outcomes from a Single Transplant Center of 5 Pediatric Cases of Domino Liver Transplantation from Live Donors with Maple Syrup Urine Disease.

BACKGROUND Maple syrup urine disease (MSUD) is a rare genetic deficiency of the branched-chain alpha-keto acid dehydrogenase (BCKAD) complex that breaks down amino acids, resulting in multi-organ failure. This report is of 5 pediatric cases of domino liver transplantation (DLT) from live donors with MSUD from a single transplant center in Beijing. CASE REPORT All MSUD donors were confirmed to have disease-causing mutations in BCKDHA (branched-chain keto acid dehydrogenase E1, alpha polypeptide) or BCKDHB (branched-chain keto acid dehydrogenase E1, ß polypeptide) genes by peripheral blood whole-exon sequencing.

Prophylactic terlipressin infusion for severe postreperfusion syndrome in patients undergoing deceased donor liver transplantation: the TIPS-DDLT randomized controlled trial.

Background: Severe postreperfusion syndrome (PRS) is a critical and potentially catastrophic event during deceased donor liver transplantation (LT). Terlipressin has been widely used as a renoprotective agent during the perioperative period of LT. This study was designed to evaluate whether prophylactic terlipressin would reduce the occurrence of severe PRS in deceased donor LT.

[Evaluation of the efficacy of Nd:YAG laser in the treatment of 80 patients with oral mucosal venous malformation].

Purpose: To evaluate the efficacy of Nd:YAG laser treatment for venous malformations.

Methods: Eighty patients with oral mucosal venous malformations underwent one or more Nd:YAG laser treatments, and photographs of the lesions before and after laser treatment were collected and compared, patients' satisfaction were assessed using visual analog scale (VAS). SPSS 22.

Predictive Significance of Claudin-3 for Epithelial Barrier Dysfunction in Chronic Rhinosinusitis With Nasal Polyps.

Purpose: The abnormal expression of tight junction (TJ) plays a vital role in the pathogenesis of chronic rhinosinusitis with nasal polyps (CRSwNP). However, there is no appropriate tool to distinguish and diagnose epithelial barrier defects in clinical practice. This study aimed to evaluate the predictive value of claudin-3 for epithelial barrier dysfunction in CRSwNP.

Construction and Hierarchical Self-Assembly of Multifunctional Coordination Cages with Triangular Metal-Metal-Bonded Units.

Herein, a series of face-capped (TrM)L (Tr = cycloheptatrienyl cationic ring; M = metal; L = organosulfur ligand) tetrahedral cages - functionalized with 12 appended crown ether moieties were designed and synthesized. The reversible binding of ammonium cations with peripheral crown ether moieties to adjust internal guest-binding was realized. Combination of a bisammonium linker and cage led to the formation of a supramolecular gel via host-guest interactions between the crown ether moieties and ammonium salts.

Effect of liver transplantation with primary hyperoxaluria type 1: Five case reports and review of literature.

Background: Primary hyperoxaluria type 1 (PH1) is a rare autosomal recessive disease stemming from a deficiency in liver-specific alanine-glyoxylate aminotransferase, resulting in increased endogenous oxalate deposition and end-stage renal disease. Organ transplantation is the only effective treatment. However, its approach and timing remain controversial.

Solvato-Controlled Assembly and Structural Transformation of Emissive Poly-NHC-Based Organometallic Cages and Their Applications in Amino Acid Sensing and Fluorescence Imaging.

Stimuli-induced structural transformation of supramolecular cages has drawn increasing attention because of their sensitive feature to external variations as model systems to simulate biological processes. However, combining structural transformation and useful functions has remained a difficult task. This study reports the solvato-controlled self-assembly of two unique topologies with different emission characteristics, a water-soluble Ag L cage (A) and an Ag L cage (B), produced from the same sulfonate-pendant tetraphenylethene (TPE) bridged tetrakis-(1,2,4-triazolium) ligand.

Synthesis of triarylborane-centered N-heterocyclic carbene cages with tunable photophysical properties.

Triarylborane-based discrete metal-carbene supramolecular cages [M(1)](PF) (M = Ag, Au) were synthesized and characterized. The new hexacarbene assemblies show a significant solvatochromic effect in solvents of different polarity. Furthermore, the reversible fluoride binding property of [Au(1)](PF) was investigated by UV-vis absorption and fluorescence titrations.