Primary sclerosing cholangitis as an intractable disease

Tamura, Sumihito; Sugawara, Yasuhiko; Kokudo, Norihiro

doi:10.5582/irdr.2012.v1.1.13

Cited by 2 publications

(2 citation statements)

References 47 publications

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“…ER expression on cholangiocytes is increased in cholestatic liver diseases but is absent in healthy individuals[ 44 ]. Taking into account the fact that the prevalence of PSC is much decreased in women and Asian populations[ 45 ] (and presumably in populations with higher than average consumption of isoflavones), we could speculate upon potential pathogenetic pathways with significant impact on disease development that are influenced by dietary habits and endocrine signaling.…”

Section: Discussionmentioning

confidence: 99%

Distinct gut microbiota profiles in patients with primary sclerosing cholangitis and ulcerative colitis

Bajer¹,

Kverka²,

Kostovčík³

et al. 2017

WJG

284

203

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AIMTo characterize the gut bacterial microbiota of patients with primary sclerosing cholangitis (PSC) and ulcerative colitis (UC).METHODSStool samples were collected and relevant clinical data obtained from 106 study participants, 43 PSC patients with (n = 32) or without (n = 11) concomitant inflammatory bowel disease, 32 UC patients, and 31 healthy controls. The V3 and V4 regions of the 16S ribosomal RNA gene were sequenced on Illumina MiSeq platform to cover low taxonomic levels. Data were further processed in QIIME employing MaAsLin and LEfSe tools for analysis of the output data.RESULTSMicrobial profiles in both PSC and UC were characterized by low bacterial diversity and significant change in global microbial composition. Rothia, Enterococcus, Streptococcus, Veillonella, and three other genera were markedly overrepresented in PSC regardless of concomitant inflammatory bowel disease (IBD). Rothia, Veillonella and Streptococcus were tracked to the species level to identify Rothia mucilaginosa, Streptococcus infantus, S. alactolyticus, and S. equi along with Veillonella parvula and V. dispar. PSC was further characterized by decreased abundance of Adlercreutzia equolifaciens and Prevotella copri. Decrease in genus Phascolarctobacterium was linked to presence of colonic inflammation regardless of IBD phenotype. Akkermansia muciniphila, Butyricicoccus pullicaecorum and Clostridium colinum were decreased in UC along with genus Roseburia. Low levels of serum albumin were significantly correlated with enrichment of order Actinomycetales.CONCLUSIONPSC is associated with specific gut microbes independently of concomitant IBD and several bacterial taxa clearly distinguish IBD phenotypes (PSC-IBD and UC).

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Section: Discussionmentioning

confidence: 99%

Distinct gut microbiota profiles in patients with primary sclerosing cholangitis and ulcerative colitis

Bajer¹,

Kverka²,

Kostovčík³

et al. 2017

WJG

284

203

View full text Add to dashboard Cite

show abstract

“…Moreover, these studies have revealed different age distribution at diagnosis and a lower prevalence of inflammatory bowel disease (IBD) as a comorbidity in Japan and Singapore than in Europe and North America [21][22][23][24][25]. Aside from a relatively large retrospective multicenter study performed in Japan [25], overall longitudinal data for the Asian population remain limited [2,[23][24][25][26][27]. The association between ethnicity and the clinical features of PSC remains unclear.…”

Section: Introductionmentioning

confidence: 99%

Longitudinal analysis of transplant candidates with primary sclerosing cholangitis in an Asian liver transplant center

Hsiao

et al. 2023

European Journal of Gastroenterology &Amp; Hepatology

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Background Primary sclerosing cholangitis (PSC) is a rare disease in Asia, and few studies have investigated the disease in this ethnicity, particularly in wait-listed patients for liver transplantation (LT). We aimed to investigate the prognostic factors and outcomes of wait-listed patients with PSC in an Asian transplant center. Methods Survival was retrospectively analyzed. Results Eighteen (10 male and 8 female) wait-listed patients with PSC, with a median age at diagnosis of 44.5 years, were included. Compared with men, women had significantly higher aspartate aminotransferase to platelet ratio index scores (3.28 vs. 1.13; P = 0.012) and bilirubin levels (7.68 vs. 4.03 mg/dl; P = 0.043) and more often presented with decompensating events, including ascites [5 (63%) vs. 1 (10%); P = 0.043] and splenomegaly [8 (100%) vs. 4 (40%); P = 0.013]. Compared with the non-LT group, the LT group exhibited a superior survival rate for women (P = 0.004) but not for men. In the univariable analysis, significant risk factors associated with overall survival included malignancies with a hazard ratio (95% confidence interval) of 5.53 (1.00-30.51) and esophageal varices (EV) [4.18 (1.05-16.61)], whereas female gender [25.00 (1.49-500.00)], LT [0.09 (0.01-0.80)] and ] were indicated in the multivariable analysis. Conclusions For Asian wait-listed patients with PSC, EV and female gender were the risk factors related to overall survival, and LT was the protective factor. Our experiences suggested that LT brings more benefits in female patients. Strategies are needed to provide equivalent transplant benefits.

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Primary sclerosing cholangitis as an intractable disease

Cited by 2 publications

References 47 publications

Distinct gut microbiota profiles in patients with primary sclerosing cholangitis and ulcerative colitis

Distinct gut microbiota profiles in patients with primary sclerosing cholangitis and ulcerative colitis

Longitudinal analysis of transplant candidates with primary sclerosing cholangitis in an Asian liver transplant center

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