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1.
Front Med (Lausanne) ; 8: 704328, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34646837

RESUMO

Background and Aims: Biliary atresia (BA) is the most common cholestatic liver disease in neonates. Although the Kasai procedure can improve temporary biliary drainage in some cases, complications and liver fibrosis still develop. Liver transplantation is the ultimate treatment. The current study aimed to investigate the effect of previous Kasai surgery on gut microbiota and bile acid in BA with end-stage liver disease. Methods: Patients with BA with end-stage liver disease were divided into two groups according to whether they had previously undergone Kasai surgery (non-Kasai: n = 8, post-Kasai: n = 8). Metagenomic sequencing and ultraperformance liquid chromatography/tandem mass spectrometry were performed to identify the gut microbiota and bile acid. Results: Previous Kasai surgery had some effects on gut microbiota and bile acid in BA with end-stage liver disease. In the gut microbiome, the differential species were mainly distributed at the species level. Veillonella atypica had a significant increase in the non-Kasai group (P < 0.05). Bacteroides spp., Prevotella spp., Barnesiella spp., Parabacteroides spp., Heliobacterium spp., Erysipelatoclostridium spp. and Diaporthe spp. were increased in the post-Kasai group (P < 0.05). Concerning functional profiles, methionine biosynthesis was enriched in the non-Kasai group, while pyridoxal biosynthesis and riboflavin biosynthesis were enriched in the post-Kasai group (linear discriminant analysis > 2, P < 0.05). In stools, 17 bile acids were distinctly elevated in the post-Kasai group, such as cholic acid, chenodeoxycholic acid, ß-muricholic acid and tauro α-muricholate (P < 0.05). Spearman correlation test showed that V. atypica had an enormously positive correlation with liver enzymes. Faecalibacterium prausnitzii and Escherichia coli were associated with derivatives of the alternative pathway of bile acid metabolism. Conclusion: Previous Kasai surgery can improve the gut microbiota and bile acid in patients with BA with end-stage liver disease. This improvement contributes to maintaining the intestinal barrier.

2.
Front Immunol ; 12: 698900, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34630385

RESUMO

Background and Aims: Biliary atresia is the most common cause of liver disease and liver transplantation in children. The accumulation of bile acids in hepatocytes and the stimulation of the intestinal microbiome can aggravate the disease progression. This study investigated changes in the composition of the gut microbiota and its metabolites in biliary atresia and the possible effects of these changes on disease progression. Methods: Stool samples of biliary atresia at different disease stages and matched control individuals were collected (early stage: 16 patients, 16 controls; later stage: 16 patients, 10 controls). Metagenomic sequencing was performed to evaluate the gut microbiota structure. Untargeted metabolomics was performed to detect and analyze the metabolites and bile acid composition. Results: A disturbed gut microbiota structure occurred in the early and later stages of biliary atresia. Klebsiella, Streptococcus, Veillonella, and Enterococcus have always been dominant. The abundance of V. atypica displayed significant changes between the early and later stages of biliary atresia. Combined with clinical indicators, Spearman's analysis showed that Klebsiella and Veillonella atypica strongly correlated with liver enzymes. Enterococcus faecium had an enormously positive relationship with lithocholic acid derivatives. Metabolites involved in tryptophan metabolism were changed in the patients with biliary atresia, which had a significant association with stool V. atypica and blood total bilirubin (p < 0.05). Conclusions: The liver damage of biliary atresia was directly or indirectly exacerbated by the interaction of enriched Klebsiella (K. pneumoniae), Veillonella (V. atypica), and Enterococcus (E. faecium) with dysmetabolism of tryptophan and bile acid.

3.
Front Med (Lausanne) ; 8: 687028, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34671611

RESUMO

Objectives: Invasive fungal infection (IFI) remains an important cause of mortality in liver transplantation (LT). The objective of this meta-analysis was to identify the risk factors for IFI after LT. Methods: We searched for relevant studies published up to June 2020 from PubMed, Web of Science, Embase, and the Cochrane Library. Odds ratios (ORs) and their corresponding 95% CIs were used to identify significant differences in the risk factors. Heterogeneity between studies was evaluated by the I 2 test, and potential publication bias was assessed with Egger's test. The quality of included studies was evaluated with the Newcastle-Ottawa Scale (NOS). Results: A total of 14 studies enrolling 4,284 recipients were included in the meta-analysis. Reoperation (OR = 2.18, 95% CI: 1.61-2.94), posttransplantation dialysis (OR = 2.03, 95% CI: 1.52-2.72), bacterial infection (OR = 1.81, 95% CI: 1.33-2.46), live donor (OR = 1.78, 95% CI: 1.20-2.63), retransplantation (OR = 2.45, 95% CI: 1.54-3.89), and fungal colonization (OR = 2.60, 95% CI: 1.99-3.42) were associated with the risk factors of IFI after LT. Conclusions: Despite some risk factors that have been identified as significant factors for IFI post-LT, which may inform prevention recommendations, rigorous and well-designed studies with adequate sample sizes should be conducted to solve the limitations of this study.

4.
Ying Yong Sheng Tai Xue Bao ; 32(8): 2886-2894, 2021 Aug.
Artigo em Chinês | MEDLINE | ID: mdl-34664462

RESUMO

Rainfall intensity, slope length, and slope gradient are the important factors affecting runoff and sediment yield. In order to quantitatively analyze the effects of rainfall intensity, slope length, and slope gradient on the erosion process of Ansai loess slope in loess hilly and gully region, we analyzed the variation of runoff and sediment yield on Ansai loess with two slope lengths (5, 10 m), three slopes (5°, 10°, 15°) and two rainfall intensities (60, 90 mm·h-1) in an indoor simulated rainfall experiment. The results showed that the initial runoff generation time decreased with the increases of slope length, though the overall change was not significant. The initial runoff generation time decreased with the increases of rainfall intensity. Compared with the intensity of 60 mm·h-1, the initial runoff generation time decreased by 5.7-18 min under the intensity of 90 mm·h-1. Among them, the runoff initiation time on the slope of 10° was the fastest. With the duration of rainfall, runoff yield rate increased rapidly at first, and then gradually fluctuated around a certain value. The sediment yield rate increased rapidly in a short period of time at the initial stage of runoff generation, and then decreased after reaching the maximum, and being gradua-lly stable. The rates of runoff and sediment yield increased with the increases of slope length and rainfall intensity, but the law of change with slope was not obvious. With the increases of rainfall intensity, slope length and gradient, the total sediment yield increased accordingly. Under the rainfall intensity of 90 mm·h-1, the slope surface with the length of 10 m and slope of 15° generated rill, leading to the highest total erosion amount (11885.66 g). Under the rainfall intensity of 60 mm·h-1, the erosion amount per unit area decreased with the increases of slope length, and there was a critical erosion slope length in 5-10 m slope section. Slope length, slope and rainfall intensity all played a promoting role in runoff process. Rainfall intensity, slope length, and their interaction contributed more to runoff yield rate and total erosion amount. Rainfall intensity contributed the most to runoff yield rate, with a contribution rate of 49.8%. The contribution rate of slope length to the total erosion was the largest, which reached 37.8%.


Assuntos
Chuva , Movimentos da Água , China , Sedimentos Geológicos , Solo
5.
Arch Virol ; 2021 Sep 28.
Artigo em Inglês | MEDLINE | ID: mdl-34581864

RESUMO

Here, we describe the full-length genome sequence of a novel ourmia-like mycovirus, tentatively named "Botryosphaeria dothidea ourmia-like virus 1" (BdOLV1), isolated from the phytopathogenic fungus Botryosphaeria dothidea strain 8A, associated with apple ring rot in Shanxi province, China. The complete BdOLV1 genome is comprised of a 2797-nucleotide positive-sense (+) single-stranded RNA (ssRNA) with a single open reading frame (ORF). The ORF putatively encodes a 642-amino-acid polypeptide with conserved RNA-dependent RNA polymerase (RdRp) motifs related to those of viruses of the family Botourmiaviridae. Phylogenetic analysis based on RdRp amino acid sequences showed that BdOLV1 is grouped with unclassified oomycete-infecting viruses closely related to members of the genus Botoulivirus in the family Botourmiaviridae. This is the first report of a novel (+)ssRNA virus in B. dothidea related to members of the genus Botoulivirus in the family Botourmiaviridae.

6.
Liver Transpl ; 2021 Sep 05.
Artigo em Inglês | MEDLINE | ID: mdl-34482616

RESUMO

The aim is to explore the impact of the Kasai procedure (KP) and the length of native liver survival time (NLST) on outcomes of liver transplantation (LT). Patients with biliary atresia (BA), who underwent LT in Beijing Friendship Hospital from January 2017 to December 2019, were enrolled and divided into non-KP (N-KP) and post-KP (P-KP) groups. The patients in the P-KP group were further divided into early failure (KP-EF) defined by NLST <1 year, medium failure (KP-MF, NLST 1-5 years), and late failure (KP-LF, NLST >5 years) subgroups. Clinical data at baseline and during follow-up were collected. The inverse probability of treatment weighting method was used to evaluate the independent effect of KP and the length of NLST on clinical outcomes. Among 197 patients with BA, the N-KP group accounted for 43 (21.8%), KP-EF 71 (46.1%), KP-MF 59 (38.3%), and KP-LF 24 (15.6%) cases, respectively. The N-KP and KP-EF groups had significantly longer hospitalization and intensive care unit stays after LT. Graft and overall survival rates were 93.0% in the N-KP group and 97.4% in P-KP group, respectively. The mortality rate in the P-KP group were significantly lower compared with that of the N-KP group with a hazard ratio (HR) of 0.2 (P = 0.02). The risks of biliary and vascular complications and cytomegalovirus (CMV) infection after LT were significantly higher in KP-EF group than those in the KP-MF and KP-LF groups (HRs = 0.09, 0.2, and 0.3, respectively; all P < 0.001). The KP significantly improved after LT overall survival. Patients with early native liver failure after KP have significantly higher risks for biliary and vascular complications and CMV infection.

7.
Int J Gen Med ; 14: 5563-5571, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34539186

RESUMO

Purpose: This study was performed to analyze the risk factors for hemorrhagic complications after ultrasound-guided liver biopsies. Patients and Methods: In this retrospective study, we reviewed 1193 ultrasound-guided percutaneous liver biopsies performed in our hospital from January 2018 to December 2020. Relevant patient characteristics, indications for biopsy, laboratory findings, biopsy technique, hemorrhagic complications, and pathologic outcomes were collected. Results: We analyzed 834 procedures performed on 807 patients with complete data. The bleeding group comprised 45 patients with post-procedure bleeding, and non-bleeding group comprising the remaining 789 patients. Univariate analysis showed that age (p < 0.001), number of needle passes (p = 0.009), platelet count (p = 0.002), prothrombin time (p < 0.001), and international normalized ratio (p < 0.001) were associated with post-procedure bleeding. Multivariable regression analysis showed that age under 18 years (p < 0.001), low platelet count (p = 0.001), and increased needle passes (p = 0.025) were independent risk factors for bleeding complications. Conclusion: Sex and focal liver lesions did not affect the risk of post-procedure bleeding. The international normalized ratio and prothrombin time were associated with an increased incidence of bleeding; however, they had no predictive value. Age, number of needle passes, and platelet count were identified as reliable predictors of bleeding.

8.
Front Med (Lausanne) ; 8: 726502, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34513885

RESUMO

Auxiliary grafts have a high risk of Hepatitis B virus (HBV) infection in patients with chronic HBV-related diseases. Hepatitis B virus-related auxiliary partial orthotopic liver transplantation (APOLT) cases were reviewed to show the results of current methods to block native-to-graft HBV transmission. Three patients received APOLT for HBV-related liver cirrhosis and a recurrent upper gastrointestinal hemorrhage between April 2015 and January 2017 by the liver transplant team of Beijing Friendship Hospital affiliated with Capital Medical University. All three patients were positive for HBV surface antigen (HBsAg) and had a negative HBV DNA test result before transplantation. After auxiliary transplantations, HBsAg was found to be positive in two patients and negative in one patient. To avoid graft infection of HBV, entecavir-based therapy was employed and the remnant native livers of the recipients were removed 51-878 days after liver transplantation. Then, serum conversions of HBsAg were found in all three cases. For the first time, this case series shows the possibility of blocking the transmission of HBV from a native liver to a graft in auxiliary transplantation by entecavir-based therapy. Among the cases, a left lobe graft was successfully implanted as a replacement of the right lobe of the recipient, which is also discussed.

9.
Transpl Int ; 2021 Sep 12.
Artigo em Inglês | MEDLINE | ID: mdl-34510581

RESUMO

BACKGROUND: The use of Epstein-Barr Virus specific cytotoxic T lymphocytes (EBV-CTLs) in adoptive immunotherapy in hematopoietic stem cell transplantation (HSCT) patients with post-transplantation lymphoproliferative disorder (PTLD) has demonstrated safety and effectiveness. EBV-CTLs might also be the effective treatment of refractory PTLD of solid organ transplantation (SOT) recipients. METHODS: Two independent assessors searched Pubmed, Embase, Cochrane Library, and Web of Science from their inception to November 2020. Eleven studies with 76 patients (42, 55% male) were included. We extracted the data and completed quality assessments. RESULTS: Most of the studies were from Europe and the USA. Liver and kidney transplantation accounted for most of the transplant types. Thirty-five (46.1%) patients were diagnosed with monomorphic PTLD, and B lymphocyte type was the most common. All the patients received primary treatment for PTLD while it was ineffective. CTLs included autologous EBV-CTLs (15/76, 22%) and HLA-matched third-party EBV-CTLs (61/76, 78%). The response rate for EBV-CTL treatment of refractory PTLD was 66%. Of fifty patients, 36 achieved complete remission and 14 achieved partial remission. EBV-DNA level decreased in 39 patients. Adverse reactions were rare and mild. CONCLUSIONS: We conclude that adoptive therapy with EBV-specific CTLs is safe, well-tolerated and effective in PTLD.

10.
Front Cell Infect Microbiol ; 11: 695134, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34368015

RESUMO

The objective of this study was to evaluate the value of molecular methods in the management of community-acquired pneumonia (CAP) in children. Previously developed mass spectrometry (MS)-based methods combined with quantitative real-time PCR (combined-MS methods) were used to describe the aetiology and evaluate antibiotic therapy in the enrolled children. Sputum collected from 302 children hospitalized with CAP were analyzed using the combined-MS methods, which can detect 19 viruses and 12 bacteria related to CAP. Based on the results, appropriate antibiotics were determined using national guidelines and compared with the initial empirical therapies. Respiratory pathogens were identified in 84.4% of the patients (255/302). Co-infection was the predominant infection pattern (51.7%, 156/302) and was primarily a bacterial-viral mixed infection (36.8%, 111/302). Compared with that using culture-based methods, the identification rate for bacteria using the combined-MS methods (61.8%, 126/204) increased by 28.5% (p <0.001). Based on the results of the combined-MS methods, the initial antibiotic treatment of 235 patients was not optimal, which mostly required switching to ß-lactam/ß-lactamase inhibitor combinations or reducing unnecessary macrolide treatments. Moreover, using the combined-MS methods to guide antibiotic therapy showed potential to decrease the length of stay in children with severe CAP. For children with CAP, quantitative molecular testing on sputum can serve as an important complement to traditional culture methods. Early aetiology elucidated using molecular testing can help guide the antibiotic therapy.


Assuntos
Infecções Comunitárias Adquiridas , Pneumonia , Antibacterianos/uso terapêutico , Bactérias/genética , Criança , Infecções Comunitárias Adquiridas/tratamento farmacológico , Humanos , Espectrometria de Massas , Pneumonia/tratamento farmacológico
11.
Transl Pediatr ; 10(7): 1947-1951, 2021 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-34430444

RESUMO

Argininemia is a rare inherited disorder characterized by progressive spastic paraplegia, leading by mutation of the ARG1 gene. Liver transplantation (LT) had been reported to prevent symptoms progression, while its pathophysiology is still unclear. A 13-year-old male patient with argininemia for progressive neurological impairment was admitted to our center. Plasma amino acid screening showed a high concentration of arginine, and gene sequencing showed heterozygous mutation of the ARG1 gene. Spastic Paraplegia Rating Scale (SPRS), motor evoked potentials (MEPs), somatosensory evoked potentials (SEPs), F-wave, electromyography, nerve conduction velocity (NCV), and brain MRI were used to evaluate the patient. Herein, we describe the clinical characteristics of this patient, attempting a correlation between clinical, neurophysiological, and neuroimaging data in argininemia. Pyramidal tract dysfunction of lower limbs affected him, while only MEPs showed abnormalities among all neurophysiological evaluations, and mild cerebellum atrophy was observed. He responded poorly to traditional treatment such as a protein restriction diet and sodium benzoate. The symptoms of speech disorder, irritability, and dyskinesia were gradually deteriorating, so living-donor LT (LDLT) was done to prevent the progression. The symptoms improved significantly six months after LT, and the spasticity severity score decreased 50%. The findings suggest that LDLT is effective to argininemia, and the phenotypical similarities to other disorders that affect the urea cycle (HHH syndrome and pyrroline-5-carboxylate synthetase deficiency) suggest a common mechanism may contribute to maintaining the integrity of the corticospinal tract.

12.
Chem Commun (Camb) ; 57(69): 8584-8587, 2021 Sep 07.
Artigo em Inglês | MEDLINE | ID: mdl-34355228

RESUMO

A facile synthetic method to form cage-annulated crown ether with anchored imidazolium units was developed. The present work verified the potential application of the metal-carbene template approach (MCTA) in the preparation of novel flexible polyimidazolium cages by photochemical [2+2] cycloaddition reactions and may provide a new method for the preparation of flexible pure organic cages with incorporation of a variety of functional sites.

14.
Front Mol Biosci ; 8: 690151, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34307456

RESUMO

The actin-related protein 2/3 complex (Arp2/3) is a major actin nucleator that has been widely reported and plays an important role in promoting the migration and invasion of various cancers. However, the expression patterns and prognostic values of Arp2/3 subunits in hepatocellular carcinoma (HCC) remain unclear. In this study, The Cancer Genome Atlas (TCGA) and UCSC Xena databases were used to obtain mRNA expression and the corresponding clinical information, respectively. The differential expression and Arp2/3 subunits in HCC were analyzed using the "limma" package of R 4.0.4 software. The prognostic value of each subunit was evaluated using Kaplan-Meier survival analysis and Cox proportional hazards regression analyses. The results revealed that mRNA expression of Arp2/3 members (ACTR2, ACTR3, ARPC1A, APRC1B, ARPC2, ARPC3, ARPC4, ARPC5, and ARPC5L) was upregulated in HCC. Higher expression of Arp2/3 members was significantly correlated with worse overall survival (OS) and shorter progression-free survival (PFS) in HCC patients. Cox proportional hazards regression analyses demonstrated that ACTR3, ARPC2, and ARPC5 were independent prognostic biomarkers of survival in patients with HCC. The relation between tumor immunocyte infiltration and the prognostic subunits was determined using the TIMER 2.0 platform and the GEPIA database. Gene set enrichment analysis (GSEA) was performed to explore the potential mechanisms of prognostic subunits in the carcinogenesis of HCC. The results revealed that ACTR3, ARPC2, and ARPC5 were significantly positively correlated with the infiltration of immune cells in HCC. The GSEA results indicated that ACTR3, ARPC2, and ARPC5 are involved in multiple cancer-related pathways that promote the development of HCC. In brief, various analyses indicated that Arp2/3 complex subunits were significantly upregulated and predicted worse survival in HCC, and they found that ACTR3, ARPC2, and ARPC5 could be used as independent predictors of survival and might be applied as promising molecular targets for diagnosis and therapy of HCC in the future.

15.
Front Physiol ; 12: 704313, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34262484

RESUMO

Background and Aims: Biliary atresia (BA) is an idiopathic neonatal cholestasis and is the most common indication in pediatric liver transplantation (LT). Previous studies have suggested that the gut microbiota (GM) in BA is disordered. However, the effect of LT on gut dysbiosis in patients with BA has not yet been elucidated. Methods: Patients with BA (n = 16) and healthy controls (n = 10) were recruited. In the early life of children with BA, Kasai surgery is a typical procedure for restoring bile flow. According to whether BA patients had previously undergone Kasai surgery, we divided the post-LT patients into the with-Kasai group (n = 8) and non-Kasai group (n = 8). Fecal samples were collected in both the BA and the control group; among BA patients, samples were obtained again 6 months after LT. A total of 40 fecal samples were collected, of which 16 were pre-LT, 14 were post-LT (8 were with-Kasai, 6 were non-Kasai), and 10 were from the control group. Metagenomic sequencing was performed to evaluate the GM. Results: The Kruskal-Wallis test showed a statistically significant difference in the number of genes between the pre-LT and the control group, the pre-LT and the post-LT group (P < 0.05), but no statistical difference between the post-LT and the control group. Principal coordinate analysis also showed that the microbiome structure was similar between the post-LT and control group (P > 0.05). Analysis of the GM composition showed a significant decrease in Serratia, Enterobacter, Morganella, Skunalikevirus, and Phifllikevirus while short chain fatty acid (SCFA)-producing bacteria such as Roseburia, Blautia, Clostridium, Akkermansia, and Ruminococcus were increased after LT (linear discriminant analysis > 2, P < 0.05). However, they still did not reach the normal control level. Concerning functional profiles, lipopolysaccharide metabolism, multidrug resistance, polyamine biosynthesis, GABA biosynthesis, and EHEC/EPEC pathogenicity signature were more enriched in the post-LT group compared with the control group. Prior Kasai surgery had a specific influence on the postoperative GM. Conclusion: LT partly improved the GM in patients with BA, which provided new insight into understanding the role of LT in BA.

16.
J Clin Lab Anal ; 35(8): e23890, 2021 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-34263944

RESUMO

BACKGROUND: Perinatal depression (PD) is one of the most common complications of pregnancy, and timely diagnosis and treatment are still challenging in China due to the scarcity of psychiatrists. This study aimed to investigate whether long noncoding RNAs (lncRNAs) are potential diagnostic biomarkers of PD. METHODS: Using RT-PCR, six downregulated major depressive disorder (MDD)-associated lncRNAs (NONSUSG010267, NONHSAT140386, NONHSAG004550, NONHSAT125420, NONHSAG013606, and NONMMUG014361) were assessed in 39 pregnant women with PD (PD group), 20 PD patients undergoing mindfulness-integrated cognitive behavior therapy (MiCBT) (treatment group (TG)), and 51 normal pregnant women (normal control (NC) group) to identify significantly differentially expressed lncRNAs during the second trimester and at 42 days postpartum. RESULTS: Compared with the NC group, the six lncRNAs were significantly downregulated in the PD group during the second trimester and at 42 days postpartum (p<0.01~0.001). Expression of NONHSAG004550 and NONHSAT125420 was significantly upregulated after MiCBT therapy in TG (p<0.01~0.001), and no significant differences were observed between TG and the NC group at 42 days postpartum (p>0.05). NONHSAG004550 and NONHSAT125420 were significantly differentially expressed in the PD group, and this expression was altered according to the amelioration of depressive symptoms. Receiver operating characteristic (ROC) curve analysis revealed that the two lncRNAs combined had a good value in predicting PD, with an area under the curve (AUC) of 0.764 (95% confidence interval (CI): 0.639-0.888). CONCLUSION: The combination of lncRNAs NONHSAG004550 and NONHSAT125420 is a novel potential diagnostic biomarker of PD.

17.
Mol Ther Nucleic Acids ; 24: 961-970, 2021 Jun 04.
Artigo em Inglês | MEDLINE | ID: mdl-34094714

RESUMO

Congenital limb malformations (CLMs) affect 1 in 500 live births. However, the value of exome sequencing (ES) for CLM is lacking. The purpose of this study was to decipher the mutational signature of CLM on an exome level. We enrolled a cohort of 66 unrelated probands (including 47 families) with CLM requiring surgical correction. ES was performed for all patients and available parental samples. A definite molecular diagnosis was achieved in 21 out of 66 (32%) patients. We identified 19 pathogenic or likely pathogenic single-nucleotide variants and three copy number variants, of which 11 variants were novel. We identified four variants of uncertain significance. Additionally, we identified RPL9 and UBA2 as novel candidate genes for CLM. By comparing the detailed phenotypic features, we expand the phenotypic spectrum of diastrophic dysplasia and chromosome 6q terminal deletion syndrome. We also found that the diagnostic rate was significantly higher in patients with a family history of CLM (p = 0.012) or more than one limb affected (p = 0.034). Our study expands our understanding of the mutational and phenotypic spectrum of CLM and provides novel insights into the genetic basis of these syndromes.

18.
World J Surg Oncol ; 19(1): 168, 2021 Jun 10.
Artigo em Inglês | MEDLINE | ID: mdl-34112190

RESUMO

BACKGROUND: Sorafenib was reported as a useful adjuvant treatment in patients with hepatocellular carcinoma who underwent surgical resection. However, its therapeutic value remains controversial. This meta-analysis examined the available data regarding the efficacy and safety of sorafenib in patients with hepatocellular carcinoma after radical surgery. METHODS: The meta-analysis was conducted according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. The protocol was registered in advance with PROSPERO (CRD42021233868). We searched PubMed, Embase, Cochrane Library, and Web of Science to identify eligible studies. Overall survival, recurrence-free survival, and recurrence rates were analyzed, and adverse events were reviewed. Hazard ratios or pooled risk ratios with 95% CIs were collected and analyzed using STATA version 12.0 in a fixed-effects or random-effects meta-analysis model. RESULTS: In total, 2655 patients from 13 studies were ultimately included in this meta-analysis. The combined results illustrated that sorafenib was associated with better overall survival than the control (hazard ratio = 0.71, 95% CI = 0.59-0.86; P < 0.001). Similarly, the drug also improved recurrence-free survival (hazard ratio = 0.68, 95% CI = 0.54-0.86, P = 0.001). Combined data revealed that patients treated with sorafenib after resection had a lower recurrence rate (pooled risk ratio = 0.78, 95% CI = 0.68-0.90, P < 0.001). The primary adverse events were hand-foot skin reaction, fatigue, and diarrhea of mild-to-moderate severity, whereas grade 4 adverse events were rare (< 1%). CONCLUSIONS: This meta-analysis demonstrated that adjuvant sorafenib therapy after resection in patients with hepatocellular carcinoma could prolong overall survival and recurrence-free survival and reduce recurrence rates without intolerable side effects. However, more evidence is needed before reaching a definitive conclusion.


Assuntos
Antineoplásicos , Carcinoma Hepatocelular , Neoplasias Hepáticas , Antineoplásicos/efeitos adversos , Carcinoma Hepatocelular/tratamento farmacológico , Carcinoma Hepatocelular/cirurgia , Humanos , Neoplasias Hepáticas/tratamento farmacológico , Neoplasias Hepáticas/cirurgia , Recidiva Local de Neoplasia/tratamento farmacológico , Niacinamida/efeitos adversos , Compostos de Fenilureia/efeitos adversos , Prognóstico , Sorafenibe/uso terapêutico , Resultado do Tratamento
19.
Artigo em Inglês | MEDLINE | ID: mdl-34166822

RESUMO

STUDY OBJECTIVE: To study the bacteria and fungi causing vulvovaginitis in prepubertal girls. DESIGN: Swabs from vaginal introitus were collected from patients with vulvovaginitis in 2018, and cultured for the identification of microorganisms with standard microbiological techniques. SETTING: A children's hospital in Hangzhou, East China. PARTICIPANTS: A total of 1235 Chinese prepubertal girls diagnosed with vulvovaginitis. MAIN OUTCOME MEASURE: Bacteria or fungi in pure cultures or as the predominant organism were defined as pathogens. RESULTS: A total of 1235 cases were diagnosed as vulvovaginitis, and 515 isolates were identified from 494 patients (40%, 494/1235). The most common pathogen was Streptococcus pyogenes (27.6%,142/515), followed by Haemophilus influenzae (27.2%,140/515), Candida albicans (22.3%,115/515), Staphylococcus aureus (5.8%, 30/515) and Escherichia coli (4.7%,24/515). All S pyogenes isolates were sensitive to penicillin, whereas 53.7% (73/136) of H influenzae isolates were sensitive to ampicillin, and 70.4% (19/27) of S aureus isolates were sensitive to oxacillin. CONCLUSION: S pyogenes and H influenzae were the 2 most commonly identified pathogenic bacteria found in prepubertal girls with vulvovaginitis. Vulvovaginitis in prepubertal girls caused by C albicans may be more common in older children.

20.
Front Microbiol ; 12: 659210, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34113326

RESUMO

Mycovirus infection has been widely shown to attenuate the virulence of phytopathogenic fungi. Valsa mali is an agriculturally important fungus that causes Valsa canker disease in apple trees. In this study, two unrelated mycoviruses [Cryphonectria hypovirus 1 (CHV1, genus Hypovirus, and single-stranded RNA) and Mycoreovirus 1 (MyRV1, genus Mycoreovirus, double-stranded RNA)] that originated from Cryphonectria parasitica (chestnut blight fungus) were singly or doubly introduced into V. mali via protoplast fusion. CHV1 and MyRV1 stably infected V. mali and caused a reduction in fungal vegetative growth and virulence. Co-infection of both viruses further reduced the virulence of V. mali but compromised the stability of CHV1 infection and horizontal transmission through hyphal anastomosis. Infections of MyRV1 and, to a lesser extent, CHV1 up-regulated the transcript expression of RNA silencing-related genes in V. mali. The accumulation of CHV1 (but not MyRV1) was elevated by the knockdown of dcl2, a key gene of the RNA silencing pathway. Similarly, the accumulation of CHV1 and the efficiency of the horizontal transmission of CHV1 during co-infection was restored by the knockdown of dcl2. Thus, CHV1 and MyRV1 are potential biological control agents for apple Valsa canker disease, but co-infection of both viruses has a negative effect on CHV1 infection in V. mali due to the activation of antiviral RNA silencing by MyRV1 infection.

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