Outcomes of pregnancy in patients with known Budd-Chiari syndrome.
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ABSTRACT:
SUBMITTER: Khan F
PROVIDER: S-EPMC5545139 | biostudies-other | 2017 Jul
REPOSITORIES: biostudies-other
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Project description:ObjectiveTo evaluate current practice and outcomes of pregnancy in women previously diagnosed with Budd-Chiari syndrome and/or portal vein thrombosis, with and without concomitant portal hypertension.Design and settingMulticentre retrospective cohort study between 2008 and 2021.PopulationWomen who conceived in the predefined period after the diagnosis of Budd-Chiari syndrome and/or portal vein thrombosis.Methods and main outcome measuresWe collected data on diagnosis and clinical features. The primary outcomes were maternal mortality and live birth rate. Secondary outcomes included maternal, neonatal and obstetric complications.ResultsForty-five women (12 Budd-Chiari syndrome, 33 portal vein thrombosis; 76 pregnancies) were included. Underlying prothrombotic disorders were present in 23 of the 45 women (51%). Thirty-eight women (84%) received low-molecular-weight heparin during pregnancy. Of 45 first pregnancies, 11 (24%) ended in pregnancy loss and 34 (76%) resulted in live birth of which 27 were at term (79% of live births and 60% of pregnancies). No maternal deaths were observed; one woman developed pulmonary embolism during pregnancy and two women (4%) had variceal bleeding requiring intervention.ConclusionsThe high number of term live births (79%) and lower than expected risk of pregnancy-related maternal and neonatal morbidity in our cohort suggest that Budd-Chiari syndrome and/or portal vein thrombosis should not be considered as an absolute contraindication for pregnancy. Individualised, nuanced counselling and a multidisciplinary pregnancy surveillance approach are essential in this patient population.Tweetable abstractBudd-Chiari syndrome and/or portal vein thrombosis should not be considered as an absolute contraindication for pregnancy.
| S-EPMC9293458 | biostudies-literature
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: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:Chiari Type I Malformation (CMI) is characterized by displacement of the cerebellar tonsils below the base of the skull, resulting in significant neurologic morbidity. Although multiple lines of evidence support a genetic contribution to disease, no genes have been identified. We therefore conducted the largest whole genome linkage screen to date using 367 individuals from 66 families with at least two individuals presenting with nonsyndromic CMI with or without syringomyelia. Initial findings across all 66 families showed minimal evidence for linkage due to suspected genetic heterogeneity. In order to improve power to localize susceptibility genes, stratified linkage analyses were performed using clinical criteria to differentiate families based on etiologic factors. Families were stratified on the presence or absence of clinical features associated with connective tissue disorders (CTDs) since CMI and CTDs frequently co-occur and it has been proposed that CMI patients with CTDs represent a distinct class of patients with a different underlying disease mechanism. Stratified linkage analyses resulted in a marked increase in evidence of linkage to multiple genomic regions consistent with reduced genetic heterogeneity. Of particular interest were two regions (Chr8, Max LOD = 3.04; Chr12, Max LOD = 2.09) identified within the subset of "CTD-negative" families, both of which harbor growth differentiation factors (GDF6, GDF3) implicated in the development of Klippel-Feil syndrome (KFS). Interestingly, roughly 3-5% of CMI patients are diagnosed with KFS. In order to investigate the possibility that CMI and KFS are allelic, GDF3 and GDF6 were sequenced leading to the identification of a previously known KFS missense mutation and potential regulatory variants in GDF6. This study has demonstrated the value of reducing genetic heterogeneity by clinical stratification implicating several convincing biological candidates and further supporting the hypothesis that multiple, distinct mechanisms are responsible for CMI.
| S-EPMC3631233 | biostudies-literature
Project description:For management of Budd-Chiari syndrome (BCS), a step-wise therapeutic approach starting with medical treatment, followed by endovascular recanalization, transjugular intrahepatic portosystemic shunt, and finally liver transplantation has been adopted. We retrospectively analyzed 51 patients with symptomatic short segment (≤ 30 mm) hepatic vein (HV)-type BCS who underwent percutaneous transluminal balloon angioplasty (PTBA) with/without stenting to determine the feasibility, clinical effectiveness, and long-term outcomes. The intervention was technically successful in 94.1% of cases (48/51)-32 patients underwent PTBA and 16 patients underwent HV stenting. Procedure-related complications occurred in 14 patients (29.1%). The clinical success rate at 4 weeks was 91.7% (44/48). Nine patients underwent reintervention, six patients due to restenosis/occlusion and three patients with clinical failure. The mean primary patency duration was 64.6 ± 19.9 months (CI, 58.5-70.8; range, 1.2-81.7 months). The cumulative 1-, 2-, and 5-year primary patency rates were 85.4, 74.5, and 58.3%, respectively. The cumulative 1-, 2-, and 5-year secondary patency rates were 93.8, 87.2, and 75%, respectively. The cumulative 1-, 2-, and 5-year survival rates were 97.9, 91.5, and 50%, respectively. Percutaneous transluminal angioplasty with and without stenting is effective and achieves excellent long-term patency and survival rates in patients with symptomatic HV-type BCS. With its lower incidence of re-occlusion and higher clinical success rate, HV angioplasty combined with stenting should be the preferred option especially in patients with segmental HV-type BCS.
| S-EPMC9388522 | biostudies-literature
Project description:ObjectiveTo investigate the results of in vitro fertilization among polycystic ovary syndrome (PCOS) patients using the long-acting long protocol regarding the relationship between menstrual patterns and adverse pregnancy outcomes.DesignRetrospective cohort study.SettingUniversity-affiliated reproductive medical center.BackgroundThe menstrual patterns of patients with PCOS is considered related to metabolism; however, no study has analyzed the outcome of in vitro fertilization/intracytoplasmic sperm injection (IVF/ICSI) in patients with PCOS who have different menstrual patterns. This study aimed to observe the outcomes of IVF/ICSI in patients with PCOS with different menstrual patterns who used the long-acting long protocol.MethodsThis was a retrospective analysis in the first cycle of IVF/ICSI at the Reproductive Medicine Center of the First Affiliated Hospital of Zhengzhou University from January 2017 to December 2019. In total, 1834 patients with PCOS were classified into the regular menstruation group (n=214), the oligomenorrhea group (n=1402), and the amenorrhea group (n=218).ResultsPCOS patients who used the long-acting long protocol of IVF/ICSI had similar clinical pregnancy rates and live birth rates despite having different menstrual patterns. The overall incidence of adverse pregnancy outcomes, including abortion, spontaneous preterm birth (sPTB), gestational diabetes(GDM), hypertensive disorder inpregnancy (HDP), and premature rupture of membranes(PROM, was significantly higher in the amenorrhea group than in the regular menstrual and oligomenorrhea groups (25.88% vs. 30.41% vs. 43.69%; P = 0.013). Additionally, the rates of GDM (2.35% vs. 6.10% vs. 13.79%; P=0.015) and macrosomia (5.26% vs. 10.94% vs. 18.39%; P=0.026) in the amenorrhea group were significantly higher than those in the other two groups. Correction for confounding factors showed that menstrual patterns are related to the occurrence of adverse pregnancy outcomes. Amenorrhea is an independent risk factor for adverse pregnancy outcome (OR [odds ratio]: 2.039, 95% CI [confidence interval]: 1.087-3.822), GDM (OR: 5.023, 95% CI: 1.083-23.289), and macrosomia (OR: 4.918, 95% CI: 1.516-15.954).ConclusionsIVF/ICSI can achieve similar pregnancy and live birth rates in PCOS patients with different menstrual patterns. However, the overall incidence of adverse pregnancy outcomes in PCOS patients with amenorrhea is higher than that in patients with regular menstruation or oligomenorrhea.
| S-EPMC8637325 | biostudies-literature
Project description:Compressive cervical myelopathy is a well-known life-threatening complication in mucopolysaccharidosis (MPS) patients. Glycosaminoglycan accumulation in the growing cartilage results in dens dysplasia, atlanto-axial instability, and subsequent periodontoid fibrocartilaginous tissue deposition with upper cervical stenosis.Chiari malformation type 1 (CM1) is a congenital downward cerebellar tonsil ectopia determined by clivus and posterior cranial fossa underdevelopment, possibly leading to progressive spinal cord cavitation (syringomyelia) and severe neurological impairment.We present a boy affected with Hunter syndrome (MPS II) and cerebellar tonsil ectopia who developed a holocord syringomyelia at the age of 6 years. The child underwent atlanto-occipital decompressive surgery with rapid clinical and neuroimaging improvement.Sharing a primary mesenchymal involvement of the cervical-occipital region, the coexistence of CM1 in MPS might be not unexpected and complicate further the disease course. In these patients, strict monitoring and prompt treatment might be of foremost importance for preventing major neurological complications.
| S-EPMC3897861 | biostudies-literature
Project description:Study questionAmong babies born by ART, do singleton survivors of a vanishing twin have lower birth weight than other singletons?Summary answerVanishing twin syndrome (VTS) was associated with lower birth weight among ART singletons; a sibship analysis indicated that the association was not confounded by maternal characteristics that remain stable between deliveries.What is known alreadyPrevious studies indicate that ART singletons with VTS have increased risk of adverse pregnancy outcomes, compared with other ART singletons. The potential contribution of unmeasured maternal background characteristics has been unclear.Study design, size and durationThis was a Norwegian population-based registry study, including 17 368 mothers with 20 410 ART singleton deliveries between January 1984 and December 2013.Participants/materials, setting, methodsThe study population included 17 291 ART singletons without VTS, 638 ART singletons with VTS and 2418 ART singletons with uncertain vanishing twin status. We estimated differences in birth weight and gestational age comparing ART singletons with VTS first to all ART singletons without VTS, and subsequently to their ART siblings without VTS, using random- and fixed-effects linear regression, respectively. The corresponding comparisons for the associations with preterm birth and small for gestational age (SGA) were conducted using random-and fixed-effects logistic regression. The sibling analysis of preterm birth included 587 discordant siblings, while the sibling analysis of SGA included 674 discordant siblings.Main results and the role of chanceART singletons with VTS had lower birth weight when compared to all ART singletons without VTS, with an adjusted mean difference (95% CI) of -116 g (-165, -67). When we compared ART singletons with VTS to their ART singletons sibling without VTS, the adjusted mean difference was -112 g (-209, -15). ART singletons with VTS also had increased risk of being born SGA, with an adjusted odds ratio (OR) (95% CI) of 1.48 (1.07, 2.03) compared to all ART singletons without VTS, and 2.79 (1.12, 6.91) in the sibship analyses. ART singletons with VTS were also more likely to be born preterm, although this difference did not reach statistical significance.Limitations reasons for cautionWe did not have information on maternal socio-economic status, but this factor is accounted for in the sibship analyses. We also had no information on whether fresh or frozen embryos were replaced.Wider implications of the findingsThe reduction in birth weight and increased risk of SGA in ART singletons with VTS may suggest the presence of harmful intrauterine factors with long-term health impact. While vanishing twins are not routinely observed in naturally conceived pregnancies, loss of a twin is potentially a risk factor for the surviving foetus in any pregnancy. This could be further explored in large samples of naturally conceived pregnancies with the necessary information.Study funding/competing interest(s)The authors of this study are supported in part by the UK Medical Research Council, US National Institute of Environmental Health Sciences and the Norwegian Research Council. The authors have no conflicts of interest.Trial registration numberN/A.
| S-EPMC5850786 | 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