Scanning Tunneling Spectroscopy Investigation of Au-bis-acetylide Networks on Au(111): The Influence of Metal-Organic Hybridization.

Graphdiyne (GDY) is an appealing two-dimensional carbon material, but the on-surface synthesis of a single layer remains challenging. Demetalation of well-crystalline metal acetylide networks, though in its infancy, provides a new avenue to on-surface synthesized GDY substructures. In spite of the synthetic efforts and theoretical concerns, there are few reports steeped in elaborate characterization of the electronic influence of metalation. In this context, we focused on the surface supported Au-bis-acetylide network, which underwent demetalation after further annealing to form hydrogen-substituted GDY. We made a comprehensive study on the geometric structure and electronic structure and the corresponding demetalized structure on Au(111) through STM, noncontact atomic force microscopy (nc-AFM), scanning tunneling spectroscopy (STS), and density functional theory (DFT) simulations. The bandgap of the Au-bis-acetylide network on Au(111) is measured to be 2.7 eV, while the bandgap of a fully demetalized Au-bis-acetylide network is estimated to be about 4.1 eV. Our findings reveal that the intercalated Au adatoms are positioned closer to the metal surface compared with the organic skeletons, facilitating electronic hybridization between the surface state and unoccupied frontier molecular orbitals of organic components. This leads to an extended conjugation through Au-bis-acetylene bonds, resulting in a reduced bandgap.

Enantioselective Sulfonimidamide Acylation via a Cinchona Alkaloid-Catalyzed Desymmetrization: Scope, Data Science, and Mechanistic Investigation.

Methods to access chiral sulfur(VI) pharmacophores are of interest in medicinal and synthetic chemistry. We report the desymmetrization of unprotected sulfonimidamides via asymmetric acylation with a cinchona-phosphinate catalyst. The desired products are formed in excellent yield and enantioselectivity with no observed bis-acylation. A data-science-driven approach to substrate scope evaluation was coupled to high throughput experimentation (HTE) to facilitate statistical modeling in order to inform mechanistic studies. Reaction kinetics, catalyst structural studies, and density functional theory (DFT) transition state analysis elucidated the turnover-limiting step to be the collapse of the tetrahedral intermediate and provided key insights into the catalyst-substrate structure-activity relationships responsible for the origin of the enantioselectivity. This study offers a reliable method for accessing enantioenriched sulfonimidamides to propel their application as pharmacophores and serves as an example of the mechanistic insight that can be gleaned from integrating data science and traditional physical organic techniques.

Chemical Composition, Toxicity, and Repellency of Essential Oils from Three Hedychium Species Against Stored-Product Insects.

Stored products are constantly infested by insects, so finding eco-friendly bioinsecticides for insect management is important. The work aimed to assess the insecticidal and repellent activity of essential oil (EO) from Hedychium glabrum S. Q. Tong, Hedychium coronarium Koen., and Hedychium yunnanense Gagnep. against Tribolium castaneum, Lasioderma serricorne, and Liposcelis bostrychophila. Results showed that 88 chemical components were identified in the extracted Hedychium EOs, indicating that they exhibited diversity in components. According to principal component analysis (PCA), the composition of the EO from the H. yunnanense stem and leaf (EOHYSL) was significantly different from other EOs due to the different organs and species. The biological activity also varied continuously with plant species and organs. Only the EO of H. yunnanense (EOHY) showed strong fumigant toxicity. While in the contact tests, EOHGR showed the strongest toxicity effect on L. bostrychophila, with a LC50 value of 71.76 µg/cm2, which was closest to the positive control (Pyrethrin). All EOs had remarkable repellent activities against the three target insects, and repellency increased with concentration. According to the results of the comprehensive score, EOHY had the highest potential, which ranged from 0.7999 to 0.8689. Thus, Hedychium EOs possess potential biorational traits to be biological insecticides.

Enhancing the Carrier Transport in Monolayer MoS2 through Interlayer Coupling with 2D Covalent Organic Frameworks.

The coupling of different 2D materials (2DMs) to form van der Waals heterostructures (vdWHs) is a powerful strategy for adjusting the electronic properties of 2D semiconductors, for applications in opto-electronics and quantum computing. 2D molybdenum disulfide (MoS2 ) represents an archetypical semiconducting, monolayer thick versatile platform for the generation of hybrid vdWH with tunable charge transport characteristics through its interfacing with molecules and assemblies thereof. However, the physisorption of (macro)molecules on 2D MoS2 yields hybrids possessing a limited thermal stability, thereby jeopardizing their technological applications. Herein, the rational design and optimized synthesis of 2D covalent organic frameworks (2D-COFs) for the generation of MoS2 /2D-COF vdWHs exhibiting strong interlayer coupling effects are reported. The high crystallinity of the 2D-COF films makes it possible to engineer an ultrastable periodic doping effect on MoS2 , boosting devices' field-effect mobility at room temperature. Such a performance increase can be attributed to the synergistic effect of the efficient interfacial electron transfer process and the pronounced suppression of MoS2 's lattice vibration. This proof-of-concept work validates an unprecedented approach for the efficient modulation of the electronic properties of 2D transition metal dichalcogenides toward high-performance (opto)electronics for CMOS digital circuits.

A systematic review of the factors significantly influencing the quality of life of living liver donors.

Objectives: This systematic review aimed to synthesize the literature on factors influencing the quality of life in living liver donors post-donation and to provide a reference for developing targeted interventions in clinical practice. Methods: A systematic search guided by the PRISMA 2020 approach was performed on specific databases: PubMed, EMBASE, CINHAL with full text, Web of Science, and ProQuest Dissertations & Theses databases. Peer-reviewed articles published in English from inception to October 2022 covering cross-sectional studies and longitudinal studies on factors affecting the quality of life of living liver donors after donation were included in this systematic review. The methodological quality of the studies was examined using a modified version of the National Institutes of Health Quality Assessment Tool. Results: A total of 6,576 studies were retrieved, and 16 eligible studies were finally included. Four types of independent influencing factors: sociodemographic (gender, donor age, education, ethnicity, and marital status), donation-related (length of hospital stay and number of hospitalizations/hospital visits related to donation surgery, recipient outcome, time from donation, complications, donation decision, ambivalence about donating, donor-recipient relationship), health-related (body mass index and pre-donation physical symptoms), and psychosocial (pre-donation physical and mental score, household income, anxiety, depression), were extracted from the included studies. Several studies consistently identified old age, recipient death, recent donation, postoperative complications experienced by donors, and donor concerns about their well-being as negative influencing factors on physical function. Female donors, low education levels, longer hospital stays, and/or more hospital visits due to donation, poor recipient outcome, recent donation, pre-donation concerns regarding their well-being, and first-degree relative and spouse/partner donors were reported in several studies as negative predictors for psychological status. Factors affecting social function were considered by only two included articles. Conclusions: The quality of life of living liver donors could be affected by both donation surgery and psychosocial factors. Based on the above-influencing factors, clinical nurses can develop targeted interventions to improve the quality of life of living liver donors.

Electroacupuncture ameliorates AOM/DSS-induced mice colorectal cancer by inhibiting inflammation and promoting autophagy via the SIRT1/miR-215/Atg14 axis.

Colorectal cancer (CRC) is one of the most common tumors of the digestive tract, with the third-highest incidence and the second-highest mortality rate among all malignant tumors worldwide. However, treatment options for CRC remain limited. As a complementary therapy, acupuncture or electro-acupuncture (EA) has been widely applied in the treatment of various inflammation-related diseases, such as obesity, ulcerative colitis and tumors. Although numerous pre-clinical and clinical studies have investigated the beneficial effects of acupuncture on CRC, the mechanism underlying the therapeutic action of EA is largely unknown. Evidence from previous studies has revealed that SIRT1 participates in CRC progression by activating autophagy-related miRNAs. Using azoxymethane/dextran sulfate sodium- (AOM/DSS-) induced colorectal cancer model in mice, we explored whether EA treatment can inhibit inflammation and promote autophagy via the SIRT1/miR-215/Atg14 axis. Our results showed that EA notably alleviated the CRC in mice, by decreasing the tumor number and DAI scores, inflammation, and increasing body weight of mice. Besides, EA increased the expression of SIRT1 and autophagy. Further experiments showed that SIRT1 overexpression downregulated miR-215, and promoted the expression of Atg14, whereas SIRT1 knockdown induced opposite results. In conclusion, EA can ameliorate AOM/DSS-induced CRC through regulating the SIRT1-mediated miR-215/Atg14 axis by suppressing inflammation and promoting autophagy in mice. These findings reveal a potential molecular mechanism underlying the anti-CRC effect of EA indicating that EA is a promising therapeutic candidate for CRC.

OER catalytic performance of a composite catalyst comprising multi-layer thin flake Co3O4 and PPy nanofibers.

The oxygen evolution reaction (OER) plays a crucial role in energy conversion and storage processes, highlighting the significance of searching for efficient and stable OER catalysts. In this study, we have developed a composite catalyst, PPy@Co3O4, with outstanding catalytic performance for the OER. The catalyst was constructed by integrating multi-layer thin flake Co3O4 with attached PPy nanofibers, utilizing the rich active sites of Co3O4 and the flexibility and tunability of PPy nanofibers to optimize the catalyst structure. Through comprehensive characterization and performance evaluation, our results demonstrate that the PPy@Co3O4 (0.1 : 1) catalyst exhibits remarkable OER catalytic activity and stability. This research provides new strategies and insights for the development of efficient and stable OER catalysts, holding promising prospects for energy conversion and storage applications in relevant fields.

Liver retransplantation: Timing is equally important.

BACKGROUND AND AIM: To evaluate the effect of transplantation interval on patient and graft survival in liver retransplantation (reLT) using meta-analytical techniques. METHODS: Literature search was undertaken until January 2022 to identify comparative studies evaluating patient survival rates, graft survival rates, and the interval time. Pooled hazard ratio (HR) or risk ratio (RR) and 95% confidence intervals (95% CI) were calculated with either the fixed or random effect model. RESULTS: The 12 articles were included in this meta-analysis. The late reLT survival rate is better than the early reLT in the 30 days group, and there is no statistical significance in other time groups. The patient survival was significantly higher in late reLT than early reLT at 1 and 5 years (respectively: RR, 0.81 [95% CI, 0.73-0.89]; RR, 0.64 [95% CI, 0.46-0.88]). The graft survival was significantly higher in late reLT than early reLT at 1 year (RR, 0.75 [95% CI, 0.63-0.89]). The risk of death after reLT in early group was 1.43 times higher than that in late group (HR, 1.43 [95% CI, 1.21-1.71]). CONCLUSIONS: Late reLT had significantly better survival rates than early reLT, and the transplantation interval was more reasonable to divide the early or late groups by 30 days.

Data Science-Enabled Palladium-Catalyzed Enantioselective Aryl-Carbonylation of Sulfonimidamides.

New methods for the general asymmetric synthesis of sulfonimidamides are of great interest due to their applications in medicinal chemistry, agrochemical discovery, and academic research. We report a palladium-catalyzed cross-coupling method for the enantioselective aryl-carbonylation of sulfonimidamides. Using data science techniques, a virtual library of calculated bisphosphine ligand descriptors was used to guide reaction optimization by effectively sampling the catalyst chemical space. The optimized conditions identified using this approach provided the desired product in excellent yield and enantioselectivity. As the next step, a data science-driven strategy was also used to explore a diverse set of aryl and heteroaryl iodides, providing key information about the scope and limitations of the method. Furthermore, we tested a range of racemic sulfonimidamides for compatibility of this coupling partner. The developed method offers a general and efficient strategy for accessing enantioenriched sulfonimidamides, which should facilitate their application in industrial and academic settings.

Feasibility of caffeic acid as a crosslinking agent in modifying acellular extracellular matrices.

Acellular extracellular matrices (aECM) are commonly utilized, both experimentally and clinically, in the regenerative medicine field. However, some disadvantages such as rapid degradation, poor mechanical properties, chronic inflammatory reactions and low antioxidant activity have limited their further application. In this study the feasibility of caffeic acid as a crosslinking agent in fixing small intestinal submucosa (SIS) was evaluated. The ninhydrin assay, swelling ratio and FTIR spectra indicated that caffeic acid can efficiently react with free amino groups to crosslink SIS and the highest crosslinking index reached 21.60 ± 1.37%. Moreover, the shrinkage temperature of SIS remarkably increased from 59 °C to about 80 °C and the degradation rate of CA-SIS was all lower than 6%, demonstrating their improved biostability and hydrothermal stability. Importantly, the antioxidant activity of CA-SIS ranged from 55% to 90%, statistically higher than that of native SIS (37.33 ± 2.94%). Additionally the cytotoxicity test presented that the cytotoxicity grade of CA-SIS was 1 or 0, whilst large numbers of living HUVECs were attached to the surface of the material and exhibited high cell viability. These results indicated their excellent cytocompatibility. The data of subcutaneous implant displayed that the number of inflammatory cells in 2%- and 2.5%CA-SIS groups remained at a low level (below 100 cells/field) while that of the native SIS group continued increasing, finally reaching 142.33 ± 30.92 cells/field. In conclusion, caffeic acid is a promising candidate for modifying aECM and may play a vital role in the design and fabrication of tissue engineering scaffolds.

Prevalence and factors associated with depression and anxiety in living liver donors: A systematic review and meta-analysis.

OBJECTIVE: This study aimed to systematically review the prevalence and factors associated with depression and anxiety in living liver donors after liver transplantation. METHODS: Five English language electronic databases and four Chinese language electronic databases were searched from inception to February 2023. Two investigators independently extracted the data and assessed the study quality. The pooled prevalence was calculated using STATA software (version 14.0). We performed a narrative review to summarize the factors associated with depression and anxiety in living liver donors after liver transplantation. The protocol of this study was registered with the International Prospective Register of Systematic Reviews (PROSPERO) database, registration number: CRD42021290071. RESULTS: This study included 39 articles involving 18,577 participants. The pooled prevalence was 6.3% [95% confidence interval (CI) (4.1-8.9%)] for depression in living liver donors, and 10.0% [95% CI (4.0-18.1%)] for anxiety. Prevalence of clinically significant depressive or anxiety symptoms was higher (15.7% and 17.4%) compared with disorders (2.7% and 2.2%). The prevalence of depression and anxiety was highest within 3 months post-donation. Specifically, female donors, serious postoperative complications, and recipients' poor health/death have been reported as factors having a negative influence on depression and anxiety. CONCLUSIONS: Collected evidence showed that the overall prevalence of depression or anxiety of living liver donors was high. Therefore, early detection and timely treatment of psychological disorders are crucial to promote positive psychiatric health outcomes and ensure the quality of life of living liver donors.

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. Cox regression models were used to analyze risk factors associated with LAF, and landmark analysis was used to evaluate the impact of IS level on LAF. Longitudinal analysis was also conducted in patients with paired biopsies. RESULTS: A total of 139 patients involving 174 liver biopsies were included. With 2.3 to 5.9 years of follow-up, LAF was detected in 91.4% of patients (7.9% were significant), up to 88.2% of whom showed normal liver function. Episodes of acute rejection, biliary complications, cytomegalovirus infection, and prolonged cold ischemia time were independent risk factors. Besides, the risk of LAF in patients with relatively low IS levels at postoperative 1-3, 3-6, 6-12, and 12-36 months was higher than the counterparts. Especially, in patients with relatively high IS levels (mean tacrolimus trough concentration ≥5.1 ng/ml) during postoperative 12-36 months, the risk of LAF was 67% lower in the short future ( P =0.006). In paired analysis, patients with increased IS levels were more likely to achieve fibrosis-reduction (HR=7.53, P =0.025). CONCLUSIONS: Mild to moderate LAF is common among pediatric LT recipients and can appear early and silently. Maintaining adequate levels of IS during 1-3 years after LT seems crucial to ensure protection against LAF.

Experience in the diagnosis and treatment of pneumonia caused by infection with Tropheryma whipplei: A case series.

Tropheryma whipplei (TW) is the root cause of Whipple's disease (WD), a rare infectious illness leading to multi-organ impairment. A prominent feature of WD is acute pneumonia, which can be exceedingly challenging to diagnose clinically due to the pathogen's surreptitious nature. However and significantly, with the advent of metagenomic next-generation sequencing (mNGS) of bronchoalveolar lavage fluid (BALF), it offers clinicians a potent tool at their disposal to detect TW infections. The present study conducted a retrospective analysis of clinical data gleaned from five patients in Hunan Province in China. Findings in this study demonstrated the potential of BALF-mNGS in diagnosing pneumonia caused by TW infection.

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. Of these 180 cases, 106 grafts were procured by open surgery and 74 by pure laparoscopic surgery. Results: While surgery durations and blood loss were decreased in donors from the laparoscopic surgery group, increased biliary openings of grafts and relatively high peak aspartate aminotransferase (AST) levels were present in both donors and recipients with this procedure. Conclusions: Laparoscopic living donor left lateral section liver procurement represents a safe and effective procedure for both donors and recipients. However, laparoscopic surgery can more frequently lead to multiple biliary tracts in the graft and its impact on the prognosis of recipients remains uncertain. Use of routine X-ray based intraoperative cholangiography may help to reduce this problem.

Second-Generation Atroposelective Synthesis of KRAS G12C Covalent Inhibitor GDC-6036.

A chromatography-free asymmetric synthesis of GDC-6036 (1) was achieved via a highly atroposelective Negishi coupling of aminopyridine 5 and quinazoline 6b catalyzed by 0.5 mol % [Pd(cin)Cl]2 and 1 mol % (R,R)-Chiraphite to afford the key intermediate (Ra)-3. An alkoxylation of (Ra)-3 with (S)-N-methylprolinol (4) and a global deprotection generates the penultimate heterobiaryl intermediate 2. A controlled acrylamide installation by stepwise acylation/sulfone elimination and final adipate salt formation and crystallization delivered high-purity GDC-6036 (1).

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. Serum leucine and valine concentrations were significantly higher than normal values. Recipients ranged in age from 0.75 to 9 years old. Three patients underwent auxiliary liver transplantation, and the other children all underwent liver or partial liver transplantation. This case report was followed up for 25 to 79 months. The prognosis, growth, and development of patients were followed up. By the end of the last follow-up, all children had survived. All patients had normal serum leucine and valine concentrations after surgery. In case 1, portal vein stenosis post-operatively. In case 2, stenosis of hepatic artery and bile duct occurred. In case 5, hepatic artery and portal vein stenosis occurred, resulting in graft loss.   CONCLUSIONS The findings from our center support the findings from other pediatric liver transplant centers that liver transplantation using MSUD donors can have successful outcomes without the development of MSUD in the recipient.

Correction to: Autophagic activation of IRF-1 aggravates hepatic ischemia-reperfusion injury via JNK signaling.

[This corrects the article DOI: 10.1002/mco2.58.].

Significance of pyroptosis-related gene in the diagnosis and classification of rheumatoid arthritis.

Background: Rheumatoid arthritis (RA), a chronic autoimmune inflammatory disease, is often characterized by persistent morning stiffness, joint pain, and swelling. Early diagnosis and timely treatment of RA can effectively delay the progression of the condition and significantly reduce the incidence of disability. In the study, we explored the function of pyroptosis-related genes (PRGs) in the diagnosis and classification of rheumatoid arthritis based on Gene Expression Omnibus (GEO) datasets. Method: We downloaded the GSE93272 dataset from the GEO database, which contains 35 healthy controls and 67 RA patients. Firstly, the GSE93272 was normalized by the R software "limma" package. Then, we screened PRGs by SVM-RFE, LASSO, and RF algorithms. To further investigate the prevalence of RA, we established a nomogram model. Besides, we grouped gene expression profiles into two clusters and explored their relationship with infiltrating immune cells. Finally, we analyzed the relationship between the two clusters and the cytokines. Result: CHMP3, TP53, AIM2, NLRP1, and PLCG1 were identified as PRGs. The nomogram model revealed that decision-making based on established model might be beneficial for RA patients, and the predictive power of the nomogram model was significant. In addition, we identified two different pyroptosis patterns (pyroptosis clusters A and B) based on the 5 PRGs. We found that eosinophil, gamma delta T cell, macrophage, natural killer cell, regulatory T cell, type 17 T helper cell, and type 2 T helper cell were significant high expressed in cluster B. And, we identified gene clusters A and B based on 56 differentially expressed genes (DEGs) between pyroptosis cluster A and B. And we calculated the pyroptosis score for each sample to quantify the different patterns. The patients in pyroptosis cluster B or gene cluster B had higher pyroptosis scores than those in pyroptosis cluster A or gene cluster A. Conclusion: In summary, PRGs play vital roles in the development and occurrence of RA. Our findings might provide novel views for the immunotherapy strategies with RA.

Quality of life, anxiety and depression symptoms in living related kidney donors: a cross­sectional study.

OBJECTIVES: Few studies have examined the quality of life of living kidney donors in mainland China. The data on anxiety and depression of living kidney donors was also scant. This study aimed to investigate quality of life, anxiety, and depression and identify their influencing factors among living kidney donors in mainland China. METHODS: A cross-sectional study included 122 living kidney donors from a kidney transplantation center in China. The abbreviated World Health Organization Quality of Life questionnaire, Generalized Anxiety Disorder 2-item and Patient Health Questionnaire 2-item were used to assess the quality of life, anxiety and depression symptoms, respectively. RESULTS: Our study found that the physical related quality of life of our donors was poorer than that of the domestic general population. Among 122 donors, 43.4% and 29.5% of them were found to have anxiety and depression symptoms, respectively. Poor health condition of recipient was identified as not only negative factors affecting all domains of quality of life, but also closely related to anxiety and depression of kidney donors. Donors with proteinuria were more likely to have a poor psychological, social related quality of life, as well as anxiety and depression symptoms. CONCLUSIONS: Living kidney donation has an impact on the physical and mental health of donors. Both the physical and mental health of living kidney donors should not be ignored. More attention and support should be given to donors with proteinuria and donors whose relative recipient suffering poor health condition.

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. CASE SUMMARY: We retrospectively analyzed 5 patients diagnosed with PH1 from the Liver Transplant Center of the Beijing Friendship Hospital from March 2017 to December 2020. Our cohort included 4 males and 1 female. The median age at onset was 4.0 years (range: 1.0-5.0), age at diagnosis was 12.2 years (range: 6.7-23.5), age at liver transplantation (LT) was 12.2 years (range: 7.0-25.1), and the follow-up time was 26.3 mo (range: 12.8-40.1). All patients had delayed diagnosis, and 3 patients had progressed to end-stage renal disease by the time they were diagnosed. Two patients received preemptive LT; their estimated glomerular filtration rate was maintained at > 120 mL/min/1.73 m2, indicating a better prognosis. Three patients received sequential liver and kidney transplantation. After transplantation, serum and urinary oxalate decreased, and liver function recovered. At the last follow-up, the estimated glomerular filtration rates of the latter 3 patients were 179, 52 and 21 mL/min/1.73 m2. CONCLUSION: Different transplantation strategies should be adopted for patients based on their renal function stage. Preemptive-LT offers a good therapeutic approach for PH1.