ライフサイエンスコーパス: biliary atresia

1 thelium drives the phenotype of experimental biliary atresia.

2 in addition to primary biliary cirrhosis and biliary atresia.

3 nnate immune response in the pathogenesis of biliary atresia.

4 activation of apoptosis in a mouse model of biliary atresia.

5 ths that have furthered our understanding of biliary atresia.

6 poptosis in the pathogenesis of experimental biliary atresia.

7 significant clinical problem in infants with biliary atresia.

8 neonatal mice using an experimental model of biliary atresia.

9 of outcomes, screening, and pathogenesis of biliary atresia.

10 be a contributing factor in the etiology of biliary atresia.

11 ct the ability to induce the murine model of biliary atresia.

12 duct injury and obstruction in experimental biliary atresia.

13 the Kasai portoenterostomy for treatment of biliary atresia.

14 o block disease progression in patients with biliary atresia.

15 ase using a mouse model of rotavirus-induced biliary atresia.

16 or the pathogenesis of the embryonic form of biliary atresia.

17 contributor to the pathogenesis of neonatal biliary atresia.

18 MR cholangiography in depicting extrahepatic biliary atresia.

19 tion genes within the livers of infants with biliary atresia.

20 and 77% specific for depicting extrahepatic biliary atresia.

21 n the etiology, diagnosis, and management of biliary atresia.

22 an diseases, including chronic cirrhosis and biliary atresia.

23 or a failed Kasai operation for extrahepatic biliary atresia.

24 the left lobe was grafted into a child with biliary atresia.

25 he initial surgical therapy for infants with biliary atresia.

26 orthotopic liver transplants for congenital biliary atresia.

27 but not from the HCV-seronegative liver with biliary atresia.

28 etaplasia and proliferation were observed in biliary atresia.

29 itial treatment for children with congenital biliary atresia.

30 with the ductules formed during extrahepatic biliary atresia.

31 tabolism might be developed for treatment of biliary atresia.

32 rs female adults and pediatric patients with biliary atresia.

33 geal varices (EV) in infants with congenital biliary atresia.

34 ion of FGF19 were significantly increased in biliary atresia.

35 tal mice, resulting in an attenuated form of biliary atresia.

36 tion in the early pathogenesis of congenital biliary atresia.

37 11 years after liver transplantation due to biliary atresia.

38 ers and bile ducts of mice with experimental biliary atresia.

39 including sphincter-of-Oddi dysfunction and biliary atresia.

40 t of serious adverse events in children with biliary atresia.

41 lt synthesis and possible therapy for infant biliary atresia.

42 was 0.86 years (IQR 0.58-3.0) and 64.3% had biliary atresia.

43 t, sea lamprey thrives despite developmental biliary atresia.

44 y a key role in pathogenesis in experimental biliary atresia.

45 The cause of liver failure included biliary atresia (11), alpha 1-antitrypsin deficiency (1)

46 y, P < 0.001) and more often diagnosed with biliary atresia (66% versus 41%, P < 0.001).

47 pient survival was obtained in children with biliary atresia (82%, 79%, and 78% at 1, 5, and 10 years

48 Rhesus rotavirus (RRV) can also lead to biliary atresia (a neonatal human disease) in mice.

49 Biliary atresia, a progressive obliterative process invo

50 e influences the newborn's susceptibility to biliary atresia, a severe cholangiopathy of neonates.

51 biliary development and its vulnerability to biliary atresia, a severe pediatric cholangiopathy, we e

52 In children with biliary atresia, a younger age and higher serum bilirubi

53 ed interest in whether rotavirus could cause biliary atresia, an idiopathic, obliterative infantile d

54 ioedema) underwent liver transplantation for biliary atresia, an unrelated condition.

55 Eleven children with end-stage extrahepatic biliary atresia and 11 controls (liver donors) were stud

56 e risk of variceal bleeding in children with biliary atresia and high-risk gastroesophageal varices.

57 st common causes of cholestatic jaundice are biliary atresia and idiopathic neonatal hepatitis (INH).

58 A total of 867 infants were born with biliary atresia and managed between January 1999 and Dec

59 Advances in treatments for biliary atresia and necrotising enterocolitis have been

60 n of interferon gamma in 65% of infants with biliary atresia and no diseased control.

61 er or bile duct remnant in all patients with biliary atresia and only 1 control.

62 bile duct remnants, and peripheral blood of biliary atresia and other cholestatic disease controls w

63 f cellular and humoral autoimmunity in human biliary atresia and possible interventional strategies t

64 ; new information for assessing prognosis in biliary atresia and primary biliary cirrhosis; and impor

65 Indications for transplantation were biliary atresia and progressive familial intrahepatic ch

66 born procedures such as portoenterostomy for biliary atresia and repair of esophageal atresia and tra

67 ses that affect infants and children-such as biliary atresia and Reye's syndrome; and (3) redefinitio

68 ed based on concepts from the mouse model of biliary atresia and rotavirus vaccination programs.

69 inylated cRNA from livers of 14 infants with biliary atresia and six with neonatal intrahepatic chole

70 actors that affect outcomes of patients with biliary atresia and there are no medical therapies that

71 In general, children <2 years with biliary atresia and those with the most growth delay at

72 inferior long-term native liver survival in biliary atresia and was associated with unsuccessful KPE

73 the rotavirus (RRV)- induced murine model of biliary atresia and whether the T cells are sufficient t

74 Recipient survival was best in children with biliary atresia and worst in adults with malignancy.

75 isease, 4 with cryptogenic cirrhosis, 4 with biliary atresia, and 10 normal subjects.

76 ies, such as primary sclerosing cholangitis, biliary atresia, and cholangiocarcinoma, have limited ex

77 duced liver injury and fibrosis in mice with biliary atresia, and increased survival times.

78 uch as erythroblastosis fetalis, septicemia, biliary atresia, and other causes of hyperbilirubinemia.

79 irrhosis, familial intrahepatic cholestasis, biliary atresia, and primary sclerosing cholangitis, and

80 y cirrhosis, primary sclerosing cholangitis, biliary atresia, and progressive familial intrahepatic c

81 l components of autoimmunity exist in murine biliary atresia, and the progressive bile duct injury is

82 The pathogenesis of biliary atresia appears to involve immune-mediated fibro

83 Infants with biliary atresia are at particularly high risk.

84 conclusion, embryonic and perinatal forms of biliary atresia are distinguished by gene expression pro

85 is of bile duct obstruction in children with biliary atresia are largely unknown.

86 irrhosis, primary sclerosing cholangitis and biliary atresia are thought to be immune-mediated cholan

87 is a genetically programmed animal model for biliary atresia, as it loses its bile ducts and gallblad

88 o underwent hepatoportoenterostomy (HPE) for biliary atresia at 9 U.S. pediatric centers between 1997

89 the Kasai portoenterostomy as treatment for biliary atresia at the region's largest pediatric hepato

90 yonic (n = 5) and perinatal (n = 6) forms of biliary atresia at the time of diagnosis and hybridized

91 in indication for LB was a high suspicion of biliary atresia (BA) [high gamma-glutamyl transferase (G

92 scular complications of portal hypoplasia in biliary atresia (BA) and acute rejection (AR) are still

93 undergoing Kasai portoenterostomy (KPE) for biliary atresia (BA) and to examine associations between

94 Young people (YP) born with biliary atresia (BA) are an emerging population for adul

95 are present in infants with cirrhosis due to biliary atresia (BA) as early as the time of evaluation

96 Biliary atresia (BA) entails an inflammatory sclerosing

97 Children with biliary atresia (BA) have increased maternal cells in th

98 maternal infections with the pathogenesis of biliary atresia (BA) in human offspring are insufficient

99 Biliary atresia (BA) is a chronic neonatal cholangiopath

100 Biliary atresia (BA) is a destructive cholangiopathy of

101 Biliary atresia (BA) is a devastating cholangiopathy of

102 Biliary atresia (BA) is a devastating disease of childho

103 Extrahepatic biliary atresia (BA) is a devastating disease of the neo

104 Biliary atresia (BA) is a devastating neonatal cholangio

105 Biliary atresia (BA) is a fibroinflammatory obstruction

106 Biliary atresia (BA) is a neonatal cholangiopathy of unk

107 Biliary atresia (BA) is a neonatal cholestatic liver dis

108 Biliary atresia (BA) is a neonatal obstructive cholangio

109 Extrahepatic biliary atresia (BA) is a pediatric liver disease with n

110 Biliary atresia (BA) is a progressive fibroinflammatory

111 Biliary atresia (BA) is a progressive fibroinflammatory

112 Biliary atresia (BA) is a progressive inflammatory fibro

113 Biliary atresia (BA) is a progressive, inflammatory chol

114 Biliary atresia (BA) is a rare disease in infants, with

115 Biliary atresia (BA) is a severe pediatric liver disease

116 The etiology of biliary atresia (BA) is not known and is likely multifac

117 gastroesophageal variceal hemorrhage (VH) in biliary atresia (BA) is not well characterized.

118 Biliary atresia (BA) is notable for marked ductular reac

119 leading theory regarding the pathogenesis of biliary atresia (BA) is that bile duct injury is initiat

120 Biliary atresia (BA) is the end result of a destructive,

121 Biliary atresia (BA) is the most common cause of end-sta

122 The etiology of biliary atresia (BA) is unknown.

123 atal mice, rhesus rotavirus (RRV) can induce biliary atresia (BA), a disease resulting in inflammator

124 Biliary atresia (BA), a progressive fibroinflammatory di

125 mice with rhesus rotavirus (RRV) results in biliary atresia (BA), and this condition is influenced b

126 (2DE) criteria to define CCM associated with biliary atresia (BA), or BA-CCM, and correlate presence

127 In patients with biliary atresia (BA), the extent of intrahepatic biliary

128 Biliary atresia (BA), the most common cause of end-stage

129 of ultrasonography (US) in the diagnosis of biliary atresia (BA), with surgery as the reference stan

130 alloproteinase-7 (MMP-7) have been linked to biliary atresia (BA), with wide variation in concentrati

131 linked to naturally occurring outbreaks of a biliary atresia (BA)-like disease in livestock.

132 e in liver fibrosis, a grave complication of biliary atresia (BA).

133 rhesus rotavirus (RRV)-induced experimental biliary atresia (BA).

134 -3, and MMP-7) was examined in children with biliary atresia (BA; n = 187), alpha-1 antitrypsin defic

135 among the three main human cholangiopathies (biliary atresia [BA], primary biliary cholangitis [PBC],

136 phocytes populate the livers of infants with biliary atresia, but it is unknown whether neonatal lymp

137 ains produces a disease similar to infantile biliary atresia, but previous attempts to correlate reov

138 Biliary atresia can lead to portal hypertension (PH), hi

139 th relevance to multiple diseases, including biliary atresia, choledochal cysts and gallbladder agene

140 -fold (P = .003) and 9.6-fold (P = .0001) in biliary atresia compared with levels in controls.

141 ith either primary sclerosing cholangitis or biliary atresia, compared with only one (4%) of 24 patie

142 ariety of pediatric disorders including AGS, biliary atresia, congenital hepatic fibrosis, sclerosing

143 Biliary atresia continues to represent a major challenge

144 Biopsies of patients with biliary atresia demonstrated increased RhoU/Wrch1 and He

145 A 71/2-month-old girl with a history of biliary atresia developed fevers, hematochezia, tachypne

146 The reference standard was biliary atresia diagnosed at the region's pediatric hepa

147 dren with spina bifida, oesophageal atresia, biliary atresia, diaphragmatic hernia, gastroschisis, an

148 prove overall survival figures by diagnosing biliary atresia earlier based on stool colour charts and

149 Extrahepatic biliary atresia (EHBA) and choledochal cysts (CDC) are i

150 going liver transplantation for extrahepatic biliary atresia (EHBA) and fulminant hepatic failure (FH

151 During and after developmental biliary atresia, expression of cyp7a1 in intestine incre

152 ns for transplantation had been extrahepatic biliary atresia (four patients), Alagille's syndrome (on

153 hotopic liver transplantation for congenital biliary atresia from July 1, 1984 to February 29, 1996 w

154 Participants with biliary atresia from the Childhood Liver Disease Researc

155 Among mice with biliary atresia given injections of antioxidants, only N

156 Discovery of the pathogenic mechanisms of biliary atresia has been limited by the inability to stu

157 Livers of infants with biliary atresia have a coordinated activation of genes i

158 children and neonatal mice with experimental biliary atresia have shown increased expression of proap

159 s were found in 11 independent patients with biliary atresia, hepatitis BC, alcohol, primary biliary

160 Relatively common conditions such as biliary atresia, however, remain largely unexplained and

161 Bile ductular proliferation in biliary atresia, however, was less than that seen in hep

162 Patients with biliary atresia (i.e., obliteration of the biliary tree)

163 samples were obtained from the children with biliary atresia immediately before orthotopic liver tran

164 o treat end-stage liver disease secondary to biliary atresia in a child with polysplenia syndrome.

165 eings, we searched for genomic signatures of biliary atresia in affected infants.

166 We induced biliary atresia in BALB/c mice by intraperitoneal admini

167 most common indications for transplant were biliary atresia in children (56%) and hepatitis C in adu

168 aches to re-examine whether rotavirus causes biliary atresia in children are discussed based on conce

169 Since 1999, all infants with suspected biliary atresia in England and Wales, UK, have been refe

170 were highest in group A and those <5 kg; and biliary atresia in group C (72.8%).

171 pects of the intrahepatic pathophysiology of biliary atresia in humans including bile duct dysmorphog

172 ducts, pathology analogous to that found in biliary atresia in humans, and high levels of T3SA+ anti

173 relationship between rotavirus infection and biliary atresia in humans.

174 e 4, are important for biliary infection and biliary atresia in mice.

175 a plant toxin, biliatresone, responsible for biliary atresia in naturally-occurring animal models, th

176 and suppressed the phenotype of experimental biliary atresia in neonatal mice.

177 in the serum of mice and children and causes biliary atresia in neonatal mice.

178 Using a model of rotavirus-induced biliary atresia in newborn mice, we found that activated

179 Treating biliary atresia in newborns earlier can delay or prevent

180 and the indications for transplantation were biliary atresia in seven, fulminant hepatic failure in s

181 p between group C rotavirus and extrahepatic biliary atresia in the 10 patients in whom virus RNA was

182 measurements detected all known infants with biliary atresia in the study population, although the 95

183 outcome of a 2-year cohort of children with biliary atresia in the UK and Ireland was assessed to fi

184 bile salts in intestine after developmental biliary atresia, in addition to known mechanisms, such a

185 Studies in the rotavirus mouse model of biliary atresia indicate that infection of biliary epith

186 ve familial intrahepatic cholestasis type 1, biliary atresia, intrahepatic cholestasis of pregnancy,

187 Biliary atresia is a devastating disorder of the newborn

188 Biliary atresia is a fibro-inflammatory cholangiopathy t

189 Biliary atresia is a neonatal liver disease with extrahe

190 Biliary atresia is a neonatal obstructive cholangiopathy

191 Biliary atresia is a rare disease and reported outcomes

192 Biliary atresia is a rare disease of infancy, which has

193 Biliary atresia is an inflammatory fibrosclerosing lesio

194 Biliary atresia is an inflammatory, fibrosclerosing neon

195 Biliary atresia is an obliterative cholangiopathy of inf

196 Biliary atresia is associated with oligoclonal expansion

197 in the majority of children with congenital biliary atresia is definitive correction with orthotopic

198 The outcome after portoenterostomy for biliary atresia is determined by age at surgery and anat

199 er, treatment typically occurs later because biliary atresia is difficult to detect during its early

200 hotopic liver transplantation for congenital biliary atresia is excellent and is independent of recip

201 he embryonic and perinatal clinical forms of biliary atresia is largely undefined.

202 The pathogenesis of biliary atresia is not known; one theory is that of a vi

203 The outcome of children with biliary atresia is related to the caseload of the surgic

204 Biliary atresia is the commonest cause of pathological j

205 Biliary atresia is the leading cause of cholestasis in i

206 Biliary atresia is the most common cause of end-stage li

207 Biliary atresia is the most common cause of end-stage li

208 Biliary atresia is the most common cholangiopathy of chi

209 Biliary atresia is the most common indication for liver

210 e loops and are associated with extrahepatic biliary atresia, lead to a loss of membrane recognition,

211 Sox17 haploinsufficiency causes biliary atresia-like phenotypes and hepatitis in late or

212 epatobiliary disease, including extrahepatic biliary atresia, liver disease and transplantation in cy

213 Although much is known about management of biliary atresia, many aspects are poorly understood, inc

214 In biliary atresia (n = 6) and 1, anti-trypsin deficiency (

215 n = 1,562], oesophageal atresia [n = 6,303], biliary atresia [n = 3,877], diaphragmatic hernia [n = 6

216 ons; two were abnormal but not suggestive of biliary atresia (one false-negative finding); 12 were co

217 Samples from 32 subjects (20 with biliary atresia or choledochal cyst and 12 controls) wer

218 ction, and 123 (39.9%) an operation to treat biliary atresia or choledochal cyst in the preceding yea

219 of common bile duct (CBD) disorders, such as biliary atresia or ischemic strictures, is restricted by

220 However, in contrast to patients with biliary atresia or other forms of cholestasis who develo

221 of five patients (one lost to follow-up) had biliary atresia or other surgical lesions; two were abno

222 ession in fibrosing cholangiopathies such as biliary atresia or primary sclerosing cholangitis.

223 patients with primary biliary cirrhosis and biliary atresia or with Alagille syndrome, two major ped

224 diatric cases for which 39 (93%) were due to biliary atresia (P<0.001).

225 Th2-activating cytokine IL-33 is elevated in biliary atresia patient serum and in the livers and bile

226 Serum and liver specimens, obtained from biliary atresia patients (N=87) at KPE or age-matched ch

227 a group of 23 pre- and postportoenterostomy biliary atresia patients were inversely related to total

228 Ten of 20 biliary atresia patients were positive for group C rotav

229 In the murine model of biliary atresia, perinatal exposure to rhesus rotavirus

230 Senescence may play a role in biliary atresia, primary sclerosing cholangitis, cellula

231 Recent advances in the understanding of biliary atresia published between December 1995 and Nove

232 S: The multicenter, double-blind Steroids in Biliary Atresia Randomized Trial (START) was conducted i

233 romol/L) as an early outcome in all cases of biliary atresia referred from one of the three centres.

234 comes after Kasai portoenterostomy (KPE) for biliary atresia remain highly variable for unclear reaso

235 The etiology of biliary atresia remains poorly understood.

236 lantation, neonatal hemochromatosis, and the Biliary Atresia Research Consortium have been summarized

237 In biliary atresia, serum bilirubin is commonly used to pre

238 Children with biliary atresia should be managed in surgical centres wi

239 inctive subgroup commonly referred to as the biliary atresia splenic malformation (BASM) syndrome.

240 ecent multicenter genetic exploration of the biliary atresia splenic malformation syndrome identified

241 ants with Cholestasis [PROBE]) or prevalent (Biliary Atresia Study of Infants and Children [BASIC]) c

242 ological networks previously unrecognized in biliary atresia, such as the complement components C3ar-

243 5% CI 0.95-1.01, p = 0.19) and children with biliary atresia surviving with native liver (OR = 0.96,

244 ic transplantation, done more frequently for biliary atresia than for any other cause of liver failur

245 In human neonates with biliary atresia, the fecal microbiome signature of these

246 Biliary atresia, the most common cause of childhood cirr

247 ogenesis; however, a subset of patients with biliary atresia, the most common childhood cholangiopath

248 This first genetically linked model of biliary atresia, the Pkd1l1 LKO mouse, may allow researc

249 IGFBP-1 levels and reduced IGFBP-3 levels in biliary atresia, there was no change in either IGFBP-1 o

250 e-negative finding); 12 were consistent with biliary atresia (three false-positive findings); four de

251 We discovered that the sea lamprey adapts to biliary atresia through a unique mechanism of de novo sy

252 al disorders such as oesophageal atresia and biliary atresia through clinical trials because of the r

253 lium, and drive the phenotypic expression of biliary atresia, thus constituting a potential therapeut

254 We hypothesized that the T cells in biliary atresia tissue expressed related TCRs, suggestin

255 Here, we used a rotavirus-induced model of biliary atresia to investigate the entire biliary transc

256 ctors for failure after portoenterostomy for biliary atresia using univariate and multivariable metho

257 ly transplantation or death in children with biliary atresia was determined.

258 Biliary atresia was the most common cause (50.4%) of end

259 1999, infants born in the UK with suspected biliary atresia were investigated in regional centres, a

260 148 infants with biliary atresia were treated between January, 1999, and

261 athway of cholangiocyte injury in a model of biliary atresia, which is relevant to human BA and may s

262 ease, and chronic biliary disorders, such as biliary atresia, which remains the most common paediatri

263 Among infants with biliary atresia who have undergone hepatoportoenterostom

264 servational study, we included patients with biliary atresia who underwent a successful KP, defined a

265 creening identified the 7 known infants with biliary atresia with a sensitivity of 100% (95% CI, 56.1

266 of a 15-month-old female infant with type I biliary atresia with jaundice (total serum bilirubin, 22

267 In patients with biliary atresia with successful KP, sBA is an early biom

268 (10 of 13) for the detection of extrahepatic biliary atresia, with a positive predictive value of 75%

269 nd the phenotypic expression of experimental biliary atresia, with glutamine promoting survival of bi