ライフサイエンスコーパス: intrahepatic cholestasis

1 gands may be potential therapeutic drugs for intrahepatic cholestasis.

2 lation may result in bile acid retention and intrahepatic cholestasis.

3 Fic1 [Byler's disease]) and benign recurrent intrahepatic cholestasis.

4 gene of a patient with neonatal progressive intrahepatic cholestasis.

5 ysis of nontransplant surgical approaches to intrahepatic cholestasis.

6 inherited liver disease progressive familial intrahepatic cholestasis.

7 3R2 may contribute to the pathophysiology of intrahepatic cholestasis.

8 ATPase disrupted in three inherited forms of intrahepatic cholestasis.

9 d dysfunction despite increased fibrosis and intrahepatic cholestasis.

10 -null mice that are resistant to LCA-induced intrahepatic cholestasis.

11 ific beta-catenin knockout (KO) mice exhibit intrahepatic cholestasis.

12 cular morphology, bile secretory defect, and intrahepatic cholestasis.

13 SEP), ABCB11, is mutated in several forms of intrahepatic cholestasis.

14 eral and canalicular membranes, resulting in intrahepatic cholestasis.

15 She was diagnosed with acute sickle cell intrahepatic cholestasis.

16 irrhosis, primary sclerosing cholangitis and intrahepatic cholestasis.

17 ver damage in rat models of extrahepatic and intrahepatic cholestasis.

18 GS patients, and PFIC patients with familial intrahepatic cholestasis 1 (FIC1) genotype, responded be

19 at ATPase class I type 8B member 1 [familial intrahepatic cholestasis 1 (FIC1)] posttranslationally a

20 Progressive familial intrahepatic cholestasis 1 (PFIC1) results from mutation

21 h Alagille syndrome (ALGS), 16 with familial intrahepatic cholestasis-1 (FIC1), 18 with bile salt exp

22 of the FXR abrogated the effect of familial intrahepatic cholestasis-1 antisense oligonucleotides.

23 echanisms by which mutations in the familial intrahepatic cholestasis-1 gene cause Byler's disease (p

24 Loss of familial intrahepatic cholestasis-1 leads to diminished nuclear t

25 e-mediated knock-down of endogenous familial intrahepatic cholestasis-1 led to up-regulation of apica

26 the farnesoid X receptor (FXR), and familial intrahepatic cholestasis-1 were studied in the ileum of

27 In familial intrahepatic cholestasis-1-negative Caco-2 cells, the ac

28 r but not cytoplasmic extracts from familial intrahepatic cholestasis-1-negative Caco-2 cells.

29 Sickle cell intrahepatic cholestasis, a potentially fatal complicati

30 We report a patient who presented with intrahepatic cholestasis, a rare and potentially lethal

31 Mutations in BSEP result in progressive intrahepatic cholestasis, a severe liver disease that im

32 r, especially in enhancing the resolution of intrahepatic cholestasis after DDC injury.

33 liver transplantation for acute sickle cell intrahepatic cholestasis and a novel case of transplanta

34 autosomal-dominant disorder characterized by intrahepatic cholestasis and abnormalities of heart, eye

35 ids prolonged postnatal survival, alleviated intrahepatic cholestasis and intestinal malabsorption, r

36 holangiopathies such as progressive familial intrahepatic cholestasis and primary sclerosing cholangi

37 n after the development of acute sickle cell intrahepatic cholestasis and renal failure requiring con

38 s, biliary atresia, and progressive familial intrahepatic cholestasis, and clinical trials of therapi

39 is used to treat primary biliary cirrhosis, intrahepatic cholestasis, and other cholestatic conditio

40 adult B-cell development, the prevention of intrahepatic cholestasis, and parturition, and is a new

41 diagnosis, 14 age-appropriate subjects with intrahepatic cholestasis as diseased controls and seven

42 Coincidently, we observed an improvement in intrahepatic cholestasis as seen by decreases in both se

43 presenting with features of benign recurrent intrahepatic cholestasis associated with a heterozygous

44 osis of a third type of neonatal progressive intrahepatic cholestasis associated with impaired bile a

45 nesis of primary biliary cirrhosis, familial intrahepatic cholestasis, biliary atresia, and primary s

46 s of inherited cholestasis, benign recurrent intrahepatic cholestasis (BRIC) and progressive familial

47 spectrum from intermittent (benign recurrent intrahepatic cholestasis; BRIC) to progressive familial

48 hildhood liver disease, progressive familial intrahepatic cholestasis, but cause and effect is less c

49 fferent genes can cause progressive familial intrahepatic cholestasis, but known genes cannot account

50 ous and metabolic causes, including neonatal intrahepatic cholestasis caused by citrin deficiency (NI

51 Inherited syndromes of intrahepatic cholestasis commonly result from mutations

52 s with biliary atresia and six with neonatal intrahepatic cholestasis (diseased controls) and hybridi

53 apies for children and adults suffering from intrahepatic cholestasis due to BSEP deficiency.

54 accumulating toxic bile acids and developing intrahepatic cholestasis during the early stage of liver

55 Benign recurrent intrahepatic cholestasis emanates from a partially funct

56 idered as an additional progressive familial intrahepatic cholestasis gene.

57 constitute the molecular defect in familial intrahepatic cholestasis I (Fic1 [Byler's disease]) and

58 aggravating factor in the early CPZ-induced intrahepatic cholestasis in human hepatocytes.

59 Disorders characterized by intrahepatic cholestasis in particular have now been sub

60 ns are known to cause hepatotoxicity such as intrahepatic cholestasis in susceptible women during pre

61 Sickle cell intrahepatic cholestasis is a potentially catastrophic c

62 Sickle cell intrahepatic cholestasis is a potentially fatal end-orga

63 Our study demonstrates that intrahepatic cholestasis leading to hepatocyte exposure

64 asis characterized by a progressive familial intrahepatic cholestasis-like phenotype with normal seru

65 s in five patients with progressive familial intrahepatic cholestasis-like phenotype with normal seru

66 estasis (PFIC) is characterized by pruritus, intrahepatic cholestasis, low serum gamma-glutamyltransf

67 In a mouse model of intrahepatic cholestasis, metformin treatment induced FX

68 not activate BSEP, whereas benign recurrent intrahepatic cholestasis mutants partially activated BSE

69 Progressive familial intrahepatic cholestasis mutations were introduced into

70 In sepsis, intrahepatic cholestasis occurs frequently, suggesting i

71 Intrahepatic cholestasis of pregnancy (ICP) affects 1/14

72 nagement and pregnancy outcome in women with intrahepatic cholestasis of pregnancy (ICP) and treatmen

73 Intrahepatic cholestasis of pregnancy (ICP) is a liver d

74 Intrahepatic cholestasis of pregnancy (ICP) is a liver d

75 Intrahepatic cholestasis of pregnancy (ICP) is a pregnan

76 Intrahepatic cholestasis of pregnancy (ICP) is a pregnan

77 Intrahepatic cholestasis of pregnancy (ICP) is associate

78 Intrahepatic cholestasis of pregnancy (ICP) is the most

79 Intrahepatic cholestasis of pregnancy (ICP) is the most

80 Intrahepatic cholestasis of pregnancy (ICP) is the most

81 pruritus of the skin is an early symptom of intrahepatic cholestasis of pregnancy (ICP) or due to be

82 Intrahepatic cholestasis of pregnancy (ICP), marked by e

83 s, and outcomes of babies born to women with intrahepatic cholestasis of pregnancy (ICP).

84 severity of acute and chronic complications, intrahepatic cholestasis of pregnancy [ICP]) were simila

85 Hyperemesis gravidarum and intrahepatic cholestasis of pregnancy have no relationsh

86 Intrahepatic cholestasis of pregnancy is pruritus and el

87 Recurrent prolonged intrahepatic cholestasis of pregnancy was followed by pe

88 hepatic cholestasis type 1, biliary atresia, intrahepatic cholestasis of pregnancy, and primary bilia

89 y cirrhosis, primary sclerosing cholangitis, intrahepatic cholestasis of pregnancy, or hereditary ped

90 cirrhosis; and important clinical trials in intrahepatic cholestasis of pregnancy, primary biliary c

91 ngitis, and clinical trials of therapies for intrahepatic cholestasis of pregnancy, primary biliary c

92 ow-phospholipid-associated cholelithiasis or intrahepatic cholestasis of pregnancy.

93 to treat children with Progressive Familial Intrahepatic Cholestasis (PFIC) and Alagille syndrome (A

94 Progressive familial intrahepatic cholestasis (PFIC) is characterized by prur

95 PFIC II is a subtype of progressive familial intrahepatic cholestasis (PFIC) that is associated with

96 of autosomal recessive progressive familial intrahepatic cholestasis (PFIC) with raised serum gamma-

97 encoding BSEP, underlay progressive familial intrahepatic cholestasis (PFIC)--or "neonatal hepatitis"

98 c cholestasis; BRIC) to progressive familial intrahepatic cholestasis (PFIC).

99 c liver disease akin to progressive familial intrahepatic cholestasis (PFIC).

100 yD and ByS (subtypes of progressive familial intrahepatic cholestasis [PFIC]) represent one clinicopa

101 uestration, viral hepatitis, and sickle cell intrahepatic cholestasis (SCIC).

102 ing bile salt transport problems in familial intrahepatic cholestasis syndromes were some of the majo

103 PEX2 mutant liver has severe but transient intrahepatic cholestasis that abates in the early postna

104 ere biliary atresia and progressive familial intrahepatic cholestasis (three patients each).

105 cholestasis (BRIC) and progressive familial intrahepatic cholestasis type 1 (PFIC1), were previously

106 ille syndrome), ATP8B1 (progressive familial intrahepatic cholestasis type 1 [PFIC1]), ABCB11 (PFIC2)

107 ileum of children with progressive familial intrahepatic cholestasis type 1 and in Caco-2 cells.

108 cause Byler's disease (progressive familial intrahepatic cholestasis type 1) are unknown.

109 nto the pathogenesis of progressive familial intrahepatic cholestasis type 1, biliary atresia, intrah

110 cted in 3 patients with progressive familial intrahepatic cholestasis type 1.

111 Progressive familial intrahepatic cholestasis type 2 (PFIC2) is a result of m

112 atic liver diseases such as primary familial intrahepatic cholestasis type 2 (PFIC2).

113 f clinical evolution in progressive familial intrahepatic cholestasis type 2 patients and how they re

114 cluded 22 children with progressive familial intrahepatic cholestasis type 2.

115 rea cycle disorders and progressive familial intrahepatic cholestasis type 2.

116 ously in a patient with progressive familial intrahepatic cholestasis type 3 (PFIC-3).

117 ary diseases, including progressive familial intrahepatic cholestasis type 3 (PFIC3), a rare disease

118 Progressive familial intrahepatic cholestasis type 3 is caused by biallelic v

119 el therapeutic tool for progressive familial intrahepatic cholestasis type 3 patients.

120 ariations identified in progressive familial intrahepatic cholestasis type 3 patients.

121 kout mouse, a model for progressive familial intrahepatic cholestasis type 3.

122 CB4 gene sequence cause progressive familial intrahepatic cholestasis type 3.

123 to the pathogenesis of progressive familial intrahepatic cholestasis type II (PFIC II).

124 deficiencies result in progressive familial intrahepatic cholestasis type III.

125 tations in ATP8B1 cause progressive familial intrahepatic cholestasis type1 in humans, which is chara

126 a neuroleptic drug known for years to induce intrahepatic cholestasis, were investigated using the di

127 We describe a patient with sickle cell intrahepatic cholestasis who underwent liver transplanta