ライフサイエンスコーパス: PBC

1 PBC is unrecognized in 13% of those cases.

2 PBC may be another risk factor, perhaps by causing chron

3 PBC should be used as a control arm in future trials of

4 n the NRS (-23%, 95% CI -45 to -1; p=0.037), PBC-40 itch domain, (-14%, -26 to -1; p=0.034), and 5-D

5 e-wide association study that included 1,122 PBC cases and 4,036 controls of Han Chinese descent, wit

6 r results show genome-wide association of 14 PBC risk loci including previously identified 6p21 (HLA-

7 iver-infiltrating T cells from PSC (n = 20), PBC (n = 10), and ALD (n = 10) patients, alongside genom

8 y biliary cirrhosis (PBC), we genotyped 2426 PBC patients and 5731 unaffected controls from three ind

9 Efficacy data were derived from the phase 3 PBC OCA International Study of Efficacy trial, and the n

10 scale (NRS), primary biliary cholangitis-40 (PBC-40) itch domain score and 5-D itch scale, changes in

11 up 28 loci in an additional UK cohort of 620 PBC cases and 2,514 population controls.

12 A total of 130 AIH, 70 PBC, and 81 PSC patients were included contributing to 1

13 The polymorphisms were genotyped in 866 PBC patients and 761 controls from independent US and Ca

14 uent replication in a separate cohort of 907 PBC cases and 2,127 controls.

15 In Lombardia there were 2,970 PBC cases with a female:male ratio of 2.3:1.

16 tant, Th17 skewing was prominent in advanced PBC patients with intensive secretion of IL-23p19 by inf

17 For AIH, PBC, and PSC cohorts, SMRs for all-cause mortality were

18 re the survival and cancer incidence in AIH, PBC, and PSC in the same population.

19 nosorbent assay in 165 serum samples of AIH, PBC, and controls.

20 lignancy on population-based cohorts of AIH, PBC, and PSC in Canterbury, New Zealand.

21 DNA samples from over 500 patients with AIH, PBC, and controls.

22 he presence of AIH-2-specific anti-LKM-1 and PBC-specific AMA was confirmed by indirect immunofluores

23 al Outcomes Study Short Form-36 (SF-36), and PBC-40 questionnaires.

24 netic and immunopathogenic basis for AIH and PBC at the MIF locus.

25 ession was elevated in patients with AIH and PBC versus healthy controls.

26 e in number of transplants for HBV, HCV, and PBC.

27 an clinical trials in patients with NASH and PBC.

28 itor cell activation differs between PSC and PBC and is characterized by a divergent fate commitment

29 In PSC and PBC, disease-associated clonotypes were detected among p

30 ol contents of two clones of cocoa (UIT1 and PBC 140) were removed and the remaining powder was autol

31 t links different pathogenic features of BA, PBC, and PSC.

32 We recently reported an association between PBC and a single nucleotide polymorphism (rs231725) of t

33 (PBC) and investigated associations between PBC incidence and sociodemographic factors and spatial c

34 estigate the genetic variants shared between PBC and BMD.

35 he experimental sequence of NPBC followed by PBC at subsequent relapse or the standard reverse treatm

36 in patients with pancreaticobiliary cancers (PBCs).

37 contribution of the peptide-binding cavity (PBC) to C. albicans adhesion and assessed the adhesive p

38 tDNA) copy number in peripheral blood cells (PBC) has been associated with the risk of developing sev

39 n of the most straight periodic bond chains (PBCs) in metatorbernite structure.

40 oH loss, a finding we termed "minimal change PBC." Ten patients were identified prospectively as havi

41 ormal controls, livers with "minimal change" PBC, CHC, and RSLH showed 9.2 +/- 6.0, 0.44 +/- 0.37 (P

42 Purpose Platinum-based chemotherapy (PBC) for patients with progressing ovarian cancer (OC) i

43 gitis (PSC) and primary biliary cholangitis (PBC) are human primary cholangiopathies characterized by

44 Primary biliary cholangitis (PBC) has been regarded as female-predominant without evi

45 Primary biliary cholangitis (PBC) is a chronic, progressive autoimmune liver disease

46 Primary biliary cholangitis (PBC) is an autoimmune liver disease characterized by the

47 Primary biliary cholangitis (PBC) is an autoimmune liver disease with a strong heredi

48 tant feature of primary biliary cholangitis (PBC) pathogenesis and other cholestatic liver diseases.

49 cal evidence of primary biliary cholangitis (PBC), are largely unknown.

50 pathogenesis of Primary Biliary Cholangitis (PBC), we have analyzed the role of Gal-3 in the murine m

51 term outcome in primary biliary cholangitis (PBC).

52 n comparison to primary biliary cholangitis (PBC).

53 associated with primary biliary cholangitis (PBC).

54 iseases such as primary biliary cholangitis (PBC, previously referred to as primary biliary cirrhosis

55 y atresia [BA], primary biliary cholangitis [PBC], and primary sclerosing cholangitis [PSC]), we buil

56 in patients with primary biliary cirrhosis (PBC) and an incomplete response to ursodeoxycholic acid

57 of patients with primary biliary cirrhosis (PBC) and are present before the onset of clinical diseas

58 the incidence of primary biliary cirrhosis (PBC) and investigated associations between PBC incidence

59 Primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) are infreq

60 stasis, including primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC), results f

61 in patients with primary biliary cirrhosis (PBC) being positive or negative for anti-M2 antibodies r

62 Patients with primary biliary cirrhosis (PBC) characteristically show circulating antimitochondri

63 hort patients and primary biliary cirrhosis (PBC) control patients were genotyped for the CD14 -260C>

64 ed 2,861 cases of primary biliary cirrhosis (PBC) from the UK PBC Consortium and 8,514 UK population

65 Primary biliary cirrhosis (PBC) has a complex clinical phenotype, with debate about

66 understanding of primary biliary cirrhosis (PBC) has been significantly enhanced by the rigorous dis

67 e liver diseases, primary biliary cirrhosis (PBC) has never been reported in early childhood, while t

68 risk factors for primary biliary cirrhosis (PBC) have been identified in recent genome-wide associat

69 Primary biliary cirrhosis (PBC) is a classical autoimmune liver disease for which e

70 Primary biliary cirrhosis (PBC) is a disease with genetic and environmental pathoge

71 Primary biliary cirrhosis (PBC) is a progressive autoimmune liver disease with a lo

72 Primary biliary cirrhosis (PBC) is an autoimmune disease characterized by clinical

73 Primary biliary cirrhosis (PBC) is considered a model autoimmune disease due to the

74 The etiology of primary biliary cirrhosis (PBC) is far from clear.

75 brosis markers in primary biliary cirrhosis (PBC) is needed to facilitate the assessment of its progr

76 A major enigma of primary biliary cirrhosis (PBC) is the selective targeting of biliary cells.

77 AIMS: Studies of primary biliary cirrhosis (PBC) phenotypes largely have been performed using small

78 BA homeostasis in primary biliary cirrhosis (PBC) remains unknown.

79 e models of human primary biliary cirrhosis (PBC) suggest that activated T cells, particularly CD8 T

80 Primary biliary cirrhosis (PBC) was first described in the 1950s as a clinical synd

81 Primary biliary cirrhosis (PBC), a chronic autoimmune liver disease, has been assoc

82 ls from explanted primary biliary cirrhosis (PBC), and control liver using a total of 24 individual s

83 hepatitis (AIH), primary biliary cirrhosis (PBC), and primary sclerosing cholangitis (PSC) are scarc

84 onferring risk to primary biliary cirrhosis (PBC), inflammatory bowel disease (IBD) and celiac diseas

85 In primary biliary cirrhosis (PBC), patients develop a multilineage response to a high

86 ic liver diseases primary biliary cirrhosis (PBC), primary sclerosing cholangitis (PSC), and alcoholi

87 logic hallmark of primary biliary cirrhosis (PBC), the antimitochondrial response to the E2 component

88 genetic basis of primary biliary cirrhosis (PBC), we genotyped 2426 PBC patients and 5731 unaffected

89 ndications except primary biliary cirrhosis (PBC).

90 patitis (AIH) and primary biliary cirrhosis (PBC).

91 iopathogenesis of primary biliary cirrhosis (PBC).

92 f these cases had primary biliary cirrhosis (PBC).

93 susceptibility to primary biliary cirrhosis (PBC).

94 y resembles human primary biliary cirrhosis (PBC).

95 in patients with primary biliary cirrhosis (PBC).

96 nt for diagnosing primary biliary cirrhosis (PBC).

97 similar to human primary biliary cirrhosis (PBC).

98 rom patients with primary biliary cirrhosis (PBC).

99 received SLK for primary biliary cirrhosis (PBC, n=76), primary sclerosing cholangitis (n=81), hepat

100 ving first LT for primary biliary cirrhosis (PBC; n=3052), primary sclerosing cholangitis (PSC; n=385

101 ply a novel prediction-based classification (PBC) modeling method.

102 al changes to patients with biopsy confirmed PBC.

103 Patients with confirmed PBCs had a mean of 118.4 +/- 36.8 CTCs/7.5 mL portal vei

104 111 consenting PBC patients, were compared with 115 FDR and 149 control

105 bladder induced phasic bladder contractions (PBC) that were accompanied by multiunit afferent firing.

106 The parent pentabenzo[a,d,g,j,m]coronene (PBC) compound is shown to exhibit a shifted and rotated

107 Finally, cultured PBC cholangiocytes showed decreased AE2 activity, togeth

108 ty-nine untreated and histologically defined PBC patients who had been followed for at least five yea

109 tients with AMAs and normal ALP will develop PBC after 5 years.

110 ng MIF and MIF receptor profiles distinguish PBC from the more inflammatory phenotype of AIH and may

111 cimens from 10 patients with confirmed early PBC, 10 with early stage chronic hepatitis C (CHC), and

112 Familial PBC has been documented in first degree relatives (FDR).

113 Familial PBC was found to be 9.9%.

114 oved by the Food and Drug Administration for PBC treatment, has demonstrated positive effects on bioc

115 l, remained stable for AC, and decreased for PBC, PSC, HCV, CC, and HBV.

116 ease were found as putative risk factors for PBC.

117 Our data indicate for PBC a sex ratio significantly lower than previously cite

118 alidate six previously unknown risk loci for PBC (Pcombined<5 x 10(-8)) and used pathway analysis to

119 f type 2 AIH with serological positivity for PBC-specific anti-mitochondrial antibodies (AMA) in a 3-

120 may show the earliest changes suspicious for PBC, namely, loss of the canals of Hering (CoH).

121 veness of OCA as a second-line treatment for PBC in combination with ursodeoxycholic acid (UDCA) in a

122 the efficacy of ursodiol as a treatment for PBC were published, although it has been clear that urso

123 nd, placebo controlled, cross-over trial for PBC patients with pruritus.

124 Proteomic analysis of BECs from PBC liver compared to normal liver are significantly dif

125 ated from circulating mononuclear cells from PBC patients and healthy controls.

126 of the CD40L promoter in CD4(+) T cells from PBC patients, as compared with controls, and this decrea

127 R-506 is up-regulated in cholangiocytes from PBC patients, binds the 3'UTR region of AE2 mRNA, and pr

128 Whereas no patients died from PBC, the 5-year survival rate was 75%, as compared to 90

129 IL-23p19, IL-17, and IL-23R using liver from PBC (n = 51) and non-PBC (n = 80) control liver disease

130 DR1101 is associated with protection from PBC, and its sequence includes an aspartic acid at beta5

131 Als3 features in the absence of a functional PBC.

132 direct control of sem-2 expression by a Hox-PBC complex.

133 sts a possible more general link between Hox-PBC factors and SoxC proteins in regulating cell prolife

134 The crucial nature of the HOX/PBC factors in directly enhancing expression of this pro

135 l to understand the immunopathology of human PBC.

136 ipyruvate dehydrogenase Abs typical of human PBC.

137 In PBC, DR represents a relevant histologic prognostic mark

138 In PBC, DR was strongly correlated with clinical prognostic

139 characterized progenitor cell activation in PBC versus PSC.

140 putative pathogenic role of decreased AE2 in PBC, miR-506 may constitute a potential therapeutic targ

141 RNA - for the decreased expression of AE2 in PBC.

142 T cells accumulate around the portal area in PBC.

143 The symptom burden in PBC, which is unrelated to disease severity or ursodeoxy

144 novel disease-protective role of B cells in PBC and suggest that B cell depletion therapy in humans

145 ife and impaired health status was common in PBC patients (35% and 46%, respectively) and more common

146 ay a critical role in biliary destruction in PBC.

147 round the damaged interlobular bile ducts in PBC.

148 by K19 immunostaining is an early feature in PBC.

149 rrent surrogate markers of liver fibrosis in PBC.

150 to PBC, whereas lower frequency was found in PBC, compared to both AIH and healthy controls.

151 rsely correlated with levels of serum IgM in PBC patients.

152 in the pathogenesis of elevated serum IgM in PBC.

153 ing enhanced expression of IL21 and IL21R in PBC livers (particularly in the hepatic portal tracks) s

154 ion of IL-12/Th1-mediated immunopathology in PBC.

155 ne-related loci not previously implicated in PBC and confirmed associations at 19 of 22 established r

156 owed that miR-506 expression is increased in PBC livers versus normal liver specimens.

157 T-cell receptor stimulation are increased in PBC.

158 ier, and had a higher proliferation index in PBC compared with PSC.

159 rdiovascular disease was significant only in PBC and alcoholic liver disease, owing to high mortality

160 er fibrosis stage and disease progression in PBC.

161 sed DNA methylation of the CD40L promoter in PBC patients, suggests that environmental factors, rathe

162 ve been proposed as potential pruritogens in PBC.

163 , a novel class of drug to treat pruritus in PBC.

164 gest a sustained rigorous B-cell response in PBC, likely activated and perpetuated by cognate autoant

165 However, its role in PBC has not been addressed.

166 repertoire of genes with potential roles in PBC pathogenesis that need to be explored by follow-up b

167 Progression of liver stiffness in PBC is predictive of poor outcome.

168 Strikingly, in PBC, although there were no significant differences in B

169 In Denmark there were 722 cases of incident PBC, female:male ratio was 4.2:1, and the annual inciden

170 years from North East England with incident PBC diagnosed during 1987-2003.

171 oss-sectional study using the United Kingdom-PBC, patient cohort.

172 hepatic and immunological features mimicking PBC in Ae2-deficient mice strongly suggest that decrease

173 ion of B cells in the pathogenesis of murine PBC.

174 Pase occur in anti-M2 positive and -negative PBC but do not have any relevance with respect to clinic

175 -M2 positive and 50% of the anti-M2 negative PBC patients had anti-beta- and/or anti-gamma-antibodies

176 ed that prolonging PFI with single-agent non-PBC (NPBC) may offer a strategy to improve overall outco

177 IL-23R using liver from PBC (n = 51) and non-PBC (n = 80) control liver disease patients.

178 tiffness appears stable in most noncirrhotic PBC patients, whereas it significantly increases in pati

179 In conclusion, our study identified a novel PBC susceptibility variant that has been shown to be str

180 8/CTLA4/ICOS, CD58, ARID3A and IL16 as novel PBC risk loci.

181 (2mM) which did not change the amplitude of PBC significantly decreased peak afferent firing from 79

182 our knowledge of the genetic architecture of PBC.

183 -E2), the major mitochondrial autoantigen of PBC and xenobiotic cross reactive chemicals.

184 pe of the major mitochondrial autoantigen of PBC, 2-octynoic acid (2-OA) coupled to BSA (2OA-BSA) and

185 We focused on the incident cases of PBC, including gender and outcome, among 9.7 million inh

186 a7, in peripheral blood mononuclear cells of PBC.

187 (IgM) and using sera from a large cohort of PBC patients and controls (n = 516), we examined in deta

188 The lifetime cost of PBC treatment would increase from $63,000 to $902,000 (1

189 cal model to simulate the lifetime course of PBC patients treated with OCA+UDCA versus UDCA alone.

190 enetic factors at play in the development of PBC.

191 re critical components in the development of PBC.

192 tal-based field-effect transistor devices of PBC exhibited efficient charge transport behavior, givin

193 = 216), or with nonestablished diagnosis of PBC (n = 229).

194 feature may support a clinical diagnosis of PBC even in the absence of characteristic, granulomatous

195 cal practice does not lead to a diagnosis of PBC.

196 -regulated in the intrahepatic bile ducts of PBC livers, compared with normal and primary sclerosing

197 ntal agent may contribute to the etiology of PBC, we have analyzed seasonal variation with respect to

198 rying environmental agent in the etiology of PBC.

199 of AMA-positive patients without evidence of PBC was 16.1 per 100,000.

200 IL-12/Th1 and IL-23/Th17 in the evolution of PBC.

201 gnostic or suggestive histologic features of PBC, but with near complete CoH loss; six had available

202 ient mice, capturing several key features of PBC, including liver-specific inflammation focused on po

203 and immunologically, reflect key features of PBC, providing a useful generic model to understand the

204 r was AMA positive without other features of PBC.

205 f Efficacy trial, and the natural history of PBC was informed by published clinical studies.

206 e findings confirm that overall incidence of PBC did not rise over time, but sociodemographic variati

207 The age/sex-adjusted annual incidence of PBC was 16.7 per million.

208 ons have been implicated in the induction of PBC.

209 Loss of PBC function resulted in an adhesion phenotype that was

210 clusion, we developed a novel mouse model of PBC that may help to elucidate the detailed mechanism of

211 dvantage of our well-defined murine model of PBC, in which mice are immunized with 2-octynoic acid co

212 Fifth, several mouse models of PBC highlight the importance of loss of tolerance to PDC

213 rove existing long-term prognostic models of PBC using data from the UK-PBC Research Cohort.

214 The reported high familial occurrence of PBC could imply screening with AMA of FDR with at least

215 stantially improve the long-term outcomes of PBC patients, but at its current annual price of $69,350

216 ave been used to clarify the pathogenesis of PBC and are generally considered reflective of an autoim

217 The pathogenesis of PBC is thought to be orchestrated by Th1 and/or Th17.

218 ntribution of B cells to the pathogenesis of PBC is unclear.

219 ion might be involved in the pathogenesis of PBC.

220 patient cohort to obtain a better picture of PBC phenotypes.

221 ous and geographically defined population of PBC patients.

222 verall survival, incidence and prevalence of PBC in two well defined population-based studies over a

223 The prognosis of PBC patients can be accurately evaluated using the UK-PB

224 acid (UDCA), which slows the progression of PBC, particularly in stage I and II of the disease.

225 e of cirrhosis, the 5-year incidence rate of PBC was 16%.

226 PBC is increased irrespective of the risk of PBC development.

227 Risk of PBC increased in areas with higher levels of socioeconom

228 regulation resulting in an increased risk of PBC is amplified by overexpression of an important proin

229 We submit that the biliary specificity of PBC is secondary to the unique processes of biliary apop

230 The study of PBC in the 2000s has been buoyed by two basic science ad

231 al human cholangiocytes, and transfection of PBC cholangiocytes with anti-miR-506 was able to improve

232 s have greatly advanced our understanding of PBC.

233 Our results support the use of PBC modeling as a triage point at the laboratory, lessen

234 an be used for molecular characterization of PBCs and share features of metastatic tissue.

235 to study the pathogenesis and progression of PBCs, as well as a diagnostic or prognostic tool to stra

236 Structural analysis of purified loss-of-PBC-function mutant proteins showed that the mutations d

237 the opposing effects of these HLA alleles on PBC susceptibility, we compared the features of epitopes

238 4(+) helper T subsets and their cytokines on PBC using our previous established murine model of 2-OA-

239 olymorphisms (SNPs) and their interaction on PBC risk was assessed by logistic regression.

240 measured using quantitative real-time PCR on PBC DNA samples from participants in the UK-based Breakt

241 = 5) and from patients with PSC (n = 20) or PBC (n = 20).

242 luated on a numerical rating scale and other PBC symptoms in an electronic diary completed twice dail

243 static, resectable, or borderline-resectable PBCs had a mean of 83.2 CTCs/7.5 mL portal vein blood (m

244 ared to patients with diagnostic early-stage PBC biopsies, showed identical treatment responses to ur

245 In both early and late-stage PBC, the predominant Ig isotype to SAc is IgM, with tite

246 to rPDC-E2 in early-stage versus late-stage PBC.

247 eloped that are widely used to risk stratify PBC patients and guide their management.

248 udy, we evaluated 18 patients with suspected PBCs.

249 isease risk does not vary spatially and that PBC cases occur independently.

250 We conclude that PBC induces characteristic changes in liver expression o

251 sion MITO-8 supports the recommendation that PBC not be delayed in favor of an NPBC in patients with

252 The PBC mutant phenotype was evaluated in assays using monol

253 dated symptom assessment tools including the PBC-40 was also undertaken and is reported here.

254 omplexes that contain one member each of the PBC and MEIS/PREP subclasses.

255 rate the essential and principal role of the PBC in Als3 adhesion.

256 Expression of the PBC protein PBX1 in the SVZ has been reported, but its f

257 analyses are needed to demonstrate that the PBC model can be effectively applied in clinical setting

258 Model outcomes were validated using the PBC Global Study.

259 ults, and symptom impact (assessed using the PBC-40 and other related measures).

260 the average overlap of 0.77% seen within the PBC (P = 0.024) and ALD groups (0.40%, P < 0.0001).

261 +/- standard error of the mean) compared to PBC samples (1.13 +/- 0.17, P < 0.0001) and ALD samples

262 Compared to PBC, 5-year outcomes were worse for NASH, HCV, and HCC f

263 ession -794 CATT7 allele in AIH, compared to PBC, whereas lower frequency was found in PBC, compared

264 tory IFN-gamma production that contribute to PBC pathology.

265 which has the closest serologic features to PBC is a mouse with a T-cell-restricted expression of th

266 Intriguingly, two PBC susceptibility loci identified through genome-wide a

267 ied out a GWAS using 1,840 cases from the UK PBC Consortium and 5,163 UK population controls as part

268 primary biliary cirrhosis (PBC) from the UK PBC Consortium and 8,514 UK population controls across 1

269 gnostic models of PBC using data from the UK-PBC Research Cohort.

270 nts can be accurately evaluated using the UK-PBC risk scores.

271 literature of AIH type 2 with an unexpected PBC-specific AMA positivity in a young child.

272 ll subsets in 59 subjects, including 28 with PBC, 13 with primary sclerosing cholangitis (PSC), and 1

273 ery 8 weeks through week 20), in adults with PBC and an inadequate response to ursodeoxycholic acid t

274 We identified 3 loci newly associated with PBC (at P<5x10(-8)), increasing the number of known susc

275 = 0.044) were significantly associated with PBC.

276 de significant BMD SNPs for association with PBC using two European GWAS data sets (n = 8392), with r

277 Compared with PBC, 5-year graft and patient survival were (a) similar

278 e progenitor cell niche in PSC compared with PBC.

279 ith PBC (n = 275), previously diagnosed with PBC (n = 216), or with nonestablished diagnosis of PBC (

280 e categorized as either newly diagnosed with PBC (n = 275), previously diagnosed with PBC (n = 216),

281 Compared to those newly diagnosed with PBC, the patients were slightly younger, had lower AMA t

282 that B cell depletion therapy in humans with PBC should be approached with caution.

283 intriguing as AMA and indeed an overlap with PBC are virtually absent in Type 2 AIH, a pediatric form

284 rom 132 subjects, including 76 patients with PBC and 56 controls, and report a higher frequency of T(

285 A-positive and 19 AMA-negative patients with PBC and 76 controls, by testing for reactivity against t

286 In liver tissues from patients with PBC and dnTGF-betaRII mice, a model of autoimmune cholan

287 open-label study enrolled six patients with PBC and incomplete responses to UDCA to be treated with

288 uld be able to determine which patients with PBC are likely to progress despite treatment with ursodi

289 ion medicine may be needed for patients with PBC at various stages of their disease process.

290 rophages (MDMphis) from either patients with PBC or controls in the presence or absence of anti-mitoc

291 r levels of CD74 were found in patients with PBC versus AIH and controls.

292 ial mechanism for treatment of patients with PBC with an incomplete response to UDCA.

293 ty for osteoporosis therapy in patients with PBC, although adherence is higher with the monthly regim

294 BEC apotopes, macrophages from patients with PBC, and AMAs.

295 Among patients with PBC, response to UDCA, treatment and symptoms are relate

296 estigative therapeutic tool in patients with PBC.

297 d from 90% among patients who presented with PBC when they were older than age 70, to less than 50% f

298 2-year study in 42 postmenopausal women with PBC and osteoporosis.

299 portal vein blood samples from patients with PBCs, but less than 25% of peripheral blood samples.

300 e mortality of AMA-positive patients without PBC is increased irrespective of the risk of PBC develop