Budd-Chiari-like pathology in dolphins.
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ABSTRACT:
SUBMITTER: Fernandez A
PROVIDER: S-EPMC9314369 | biostudies-literature |
REPOSITORIES: biostudies-literature
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Project description:To analyse the risk of pregnancy (a prothrombotic state) in patients with Budd-Chiari Syndrome (BCS).Retrospective study of pregnancy in women with known BCS at single center from January 2001 to December 2015.Out of 53 females with BCS, 7 women had 16 pregnancies. Median age at diagnosis of BCS in these women was 25 years (range 21-34 years). At least one causal factor for BCS was identified in 6 women (86%). Six women had undergone radiological decompressive treatment. All patients had anticoagulation. Six fetuses were lost before 20 wk gestation in 2 women. There were 9 deliveries over 32 wk gestation and one delivery at 27 wk. All infants did well. Seven babies were born by emergency caesarean section. There were no cases of thrombosis. Two patients had notable vaginal (PV) bleeding in 3 pregnancies. None of the patients had variceal haemorrhage. Two patients were diagnosed with pulmonary hypertension, one during pregnancy and the other in the post-partum period. There was no maternal mortality.Maternal outcomes in patients with treated BCS are favourable and fetal outcomes beyond 20 wk gestation are good. There has been increased rate of caesarean section. Pulmonary hypertension is an important finding that needs further validation. These patients should be managed in centers experienced in treating high-risk pregnancies.
| S-EPMC5545139 | biostudies-other
Project description:Gut microbiota is associated with liver diseases. However, gut microbial characteristics of Budd-Chiari syndrome (B-CS) have not been reported. Here, by MiSeq sequencing, gut microbial alterations were characterized among 37 health controls, 20 liver cirrhosis (LC) patients, 31 initial B-CS patients (B-CS group), 33 stability patients after BCS treatment (stability group) and 23 recurrent patients after BCS treatment (recurrence group). Gut microbial diversity was increased in B-CS versus LC. Bacterial community of B-CS clustered with controls but separated from LC. Operational taxonomic units (OTUs) 421, 502 (Clostridium IV) and 141 (Megasphaera) were unique to B-CS. Genera Escherichia/Shigella and Clostridium XI were decreased in B-CS versus controls. Moreover, nine genera, mainly including Bacteroides and Megamonas, were enriched in B-CS versus LC. Notably, Megamonas could distinguish B-CS from LC with areas under the curve (AUCs) of 0.7904. Microbial function prediction revealed that L-amino acid transport system activity was decreased in B-CS versus both LC and controls. Furthermore, OTUs 27 (Clostridium XI), 137 (Clostridium XIVb) and 40 (Bacteroides) were associated with B-CS stability. Importantly, genus Clostridium XI was enriched in stability group versus both recurrence group and B-CS group. Also, PRPP glutamine biosynthesis was reduced in stability group versus recurrence group, but was enriched in stability group versus B-CS group. In conclusion, specific microbial alterations associated with diagnosis and prognosis were detected in B-CS patients. Correction of gut microbial alterations may be a potential strategy for B-CS prevention and treatment.
| S-EPMC5790465 | biostudies-literature
Project description:Myeloproliferative neoplasms (MPNs) are the leading cause of Budd-Chiari syndrome (BCS), and the C allele of JAK2 rs4495487 was reported to be an additional candidate locus that contributed to MPNs. In the present study, we examined the role of JAK2 rs4495487 in the etiology and clinical presentation of Chinese BCS patients. 300 primary BCS patients and 311 healthy controls were enrolled to evaluate the association between JAK2 rs4495487 polymorphism and risk of BCS. All subjects were detected for JAK2 rs4495487 by real-time PCR. Results. The JAK2 rs4495487 polymorphism was associated with JAK2 V617F-positive BCS patients compared with controls (P < 0.01). The CC genotype increased the risk of BCS in patients with JAK2 V617F mutation compared with individuals presenting TT genotype (OR = 13.60, 95% CI = 2.04-90.79) and non-CC genotype (OR = 12.00, 95% CI = 2.07-69.52). We also observed a significantly elevated risk of combined-type BCS associated with CC genotype in the recessive model (OR = 4.44, 95% CI = 1.31-15.12). This study provides statistical evidence that the JAK2 rs4495487 polymorphism is susceptibility factor JAK2 V617F positive BCS and combined BCS in China. Further larger studies are required to confirm these findings.
| S-EPMC4628667 | biostudies-literature
Project description:BackgroundBudd-Chiari syndrome (BCS) results when the outflow of the hepatic vein (HV) is obstructed. BCS patients exhibiting an accessory HV (AHV) that is dilated but obstructed can achieve significant alleviation of liver congestion after undergoing AHV recanalization. This meta-analysis was developed to explore the clinical efficacy of AHV recanalization in patients with BCS.Materials and methodsPubMed, Embase, and Wanfang databases were searched for relevant studies published as of November 2022, and RevMan 5.3 and Stata 12.0 were used for pooled endpoint analyses.ResultsTwelve total studies were identified for analysis. Pooled primary clinical success, re-stenosis, 1- and 5-year primary patency, 1- and 5-year secondary patency, 1-year overall survival (OS), and 5-year OS rates of patients in these studies following AHV recanalization were 96%, 17%, 91%, 75%, 98%, 91%, 97%, and 96%, respectively. Patients also exhibited a significant reduction in AHV pressure after recanalization relative to preoperative levels (P < 0.00001). Endpoints exhibiting significant heterogeneity among these studies included, AHV pressure (I2 = 95%), 1-year primary patency (I2 = 51.2%), and 5-year primary patency (I2 = 62.4%). Relative to HV recanalization, AHV recanalization was related to a lower rate of re-stenosis (P = 0.002) and longer primary patency (P < 0.00001), but was not associated with any improvements in clinical success (P = 0.88) or OS (P = 0.29) relative to HV recanalization.ConclusionsThe present meta-analysis highlights AHV recanalization as an effective means of achieving positive long-term outcomes in patients affected by BCS, potentially achieving better long-term results than those associated with HV recanalization.
| S-EPMC10546748 | biostudies-literature
Project description:Budd-Chiari syndrome (BCS) results in sinusoidal congestion and hepatocyte apoptosis, which can further progress to liver fibrosis, cirrhosis, and hepatocellular carcinoma. Endovascular (EV) treatment has been the primary therapeutic method and achieved excellent outcomes. However, whether EV treatment could reverse liver cirrhosis in patients with BCS is still unclear. To investigate the effect of endovascular treatment on liver cirrhosis in patients with Budd-Chiari syndrome, we performed gene expression profiling analysis of the liver from patients with or without EV treatment.
2023-03-26 | GSE228205 | GEO
Project description:Women during pregnancy or puerperium are likely to develop Budd-Chiari syndrome (BCS). However, the reported prevalence of pregnancy-related BCS varied considerably among studies. Our study aims to systematically review this issue. Overall, 817 papers were initially identified via the PubMed, EMBASE, China National Knowledge Infrastructure, and Chinese Scientific and Technological Journal databases. Twenty of them were eligible. The prevalence of pregnancy-related BCS varied from 0% to 21.5%. The pooled prevalence was 6.8% (95% CI: 3.9-10.5%) in all BCS patients, 6.3% (95% CI: 3.8-9.4%) in primary BCS patients, and 13.1% (95% CI: 7.1-20.7%) in female BCS patients. Among them, one study was carried out in Africa with a prevalence of 10.6%; 14 studies in Asian countries with a pooled prevalence of 7.1% (95% CI: 3.1-12.6%); and 5 studies in European countries with a pooled prevalence of 5.0% (95% CI: 3.1-7.3%). The pooled prevalence was 6.7% (95% CI: 2.6-12.3%) in studies published before 2005 and 7.3% (95% CI: 4.2-12.5%) in those published after 2005. In conclusion, pregnancy is a relatively common risk factor for BCS, but there is a huge variation in the prevalence among studies. Physicians should be aware of pregnancy-related BCS.
| S-EPMC4592727 | biostudies-literature
Project description:Background & Aims:Hepatobiliary phase (HBP) images can discriminate between benign and malignant liver lesions, but it is unclear if this approach can be used in patients with Budd-Chiari syndrome (BCS). Thus, we aimed to assess the diagnostic utility of HBP images in patients with BCS. Methods:This retrospective study included all patients admitted to our institution with a diagnosis of BCS and focal liver lesions on hepatobiliary contrast agent-enhanced MR imaging (HBCA-MRI) from 2000 to 2019. MR images were reviewed by 2 radiologists blinded to the diagnosis of the lesions. Patient and lesion characteristics were recorded, focusing on HBP imaging features. Results:Twenty-six patients (mean 35 ± 11 years old [13-65]; 21 women [81%] 35 ± 12 years old [13-65]; 5 men [19%] 36 ± 10 years old [19-44]) with 99 benign liver lesions and 12 hepatocellular carcinomas (HCCs) were analyzed. Patients with HCC were significantly older than those with benign lesions (mean 50 ± 10 vs. 33 ± 9 years old, p = 0.003), with higher alpha-fetoprotein (AFP) levels (3/4 [75%] vs. 1/22 [5%] with AFP >15 ng/ml, p <0.001). Homogeneous hypointense signals were identified on HBP in 14 lesions, including 12/12 (100%) HCCs, and 2/99 (2%) benign lesions (p <0.001). Most benign liver lesions showed either peripheral (n = 52/99 [53%]) or homogeneous hyperintensity (n = 23/99 [23%]) on HBP. Lesions with signal hypointensity on HBP in patients with AFP serum levels >15 ng/ml were all HCCs. Conclusion:Most benign lesions showed homogeneous or peripheral hyperintensity on HBP images while all HCCs were homogeneously hypointense. HBP images are helpful to differentiate between benign lesions and HCCs and outperform other sequences. They should be systematically acquired for the characterization of focal lesions in patients with BCS. Lay summary:Hepatobiliary phase imaging is an approach that has recently been shown to discriminate between benign and malignant lesions in the liver. However, it was not known whether this imaging approach could be used effectively in patients with Budd-Chiari syndrome. Herein, we have shown that hepatobiliary phase imaging appears to be useful for differentiating between benign and malignant liver lesions in patients with Budd-Chiari syndrome.
| S-EPMC7232085 | biostudies-literature
Project description:We investigated the effects of gut microbiota and serum metabolite levels in patients with Budd-Chiari syndrome (B-CS) and their importance for guiding clinical management strategies. In total, 214 B-CS patients (93 untreated and 121 treated) and 41 healthy controls were enrolled. Gut microbiota and serum metabolome were analysed using shotgun metagenomics and liquid chromatography-mass spectrometry. The gut microbiota of the patients showed abundance of Campylobacter and low levels of Saccharomyces, Deinococcus, and Thiomonas (P < 0.05). Thirty metabolites, including taurocholate and (R)-3-hydroxybutyric acid, were identified in the patients (VIP > 1, P < 0.05 and FC > 1.2 or FC < 0.83). Random forest (RF) models showed that serum metabolome could effectively identify B-CS from healthy controls and RF-metabolomics exhibited perfect discrimination (AUC = 100%, 95% CI: 100% – 100%), which was significantly higher than that achieved by RF-metagenomics (AUC = 58.48%, 95% CI: 38.46% – 78.5%). Campylobacter concisus and taurocholate showed significant positive correlation in patients with clinical manifestations (P < 0.05). Actinobacteria levels were significantly higher in untreated patients than in treated patients (P < 0.05). Campylobacter and Veillonella levels were significantly higher in treated patients than in healthy controls (P < 0.05). We identified major alterations in the gut microbiota and serum metabolome of patients with B-CS. Faecal metagenomics- and serum metabolomics-guided management strategies are required for patients with B-CS.
| S-EPMC8567795 | biostudies-literature
Project description:Background. Thousands of Budd-Chiari syndrome (BCS) studies have been published in China, and yet no one has explored its incidence or prevalence in the whole country. Methods. Three most commonly used Chinese language electronic databases were searched, and epidemiological data were extracted from the selected articles. Results. By the end of 2013, 20191 BCS cases were reported in China. The mean age of BCS patients was 36.29 ± 1.28 years, and ratio of male to female was 150/100. About 80% BCS patients were distributed in Henan, Shandong, Beijing, Jiangsu, and Anhui, and all of them except for Beijing were located in the downstream areas of Yellow River and the whole Huai River basin. The incidence and prevalence of BCS in China with and without the top 5 high-prevalence areas were estimated to be 0.88/million per year and 7.69/million and 0.28/million per year and 2.21/million, respectively. Conclusions. Most BCS patients in China are distributed in the downstream areas of Yellow River and the whole Huai River basin. The incidence and prevalence are comparable to those of Western countries without taking into account the top 5 high-prevalence areas.
| S-EPMC4609452 | biostudies-literature
Project description:BackgroundVarious studies have demonstrated that factor V Leiden (FVL) and G20210A prothrombin mutation contribute to the risk of Budd-Chiari syndrome (BCS), while other studies provided conflicting findings. In order to derive more precise estimations of the relationships, a meta-analysis was performed.MethodsEligible articles were identified through search of databases including Pubmed, Chinese Biomedical Database (CBM, Chinese), and Chinese National Knowledge Infrastructure (CNKI, Chinese). Odd ratios (ORs) with 95% confidence intervals (CIs) were calculated using random- or fixed- model.ResultsFinally, twelve studies were included for FVL and nine studies were included for G20210A prothrombin mutation. With respect to FVL, significantly increased BCS risk was found in the overall population (OR?=?6.29, 95%CI?=?4.23-9.36). Subgroup analyses suggested that FVL was associated with an increased risk of BCS in the population with high background mutation prevalence (>1% in the normal population). No significant association was found between BCS and G20210A prothrombin mutation (OR?=?1.78, 95%CI?=?0.77-4.11).ConclusionThe presence of FVL should be evaluated in patients with BCS. Conversely, G20210A prothrombin mutation is not significantly associated with risk of BCS. Large-scale well designed studies are necessary to be conducted to further confirm or refute the observed association.
| S-EPMC3995749 | biostudies-literature