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progressive familial intrahepatic cholestasis (173) 173
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benign recurrent intrahepatic cholestasis (29) 29
salt export pump (27) 27
atp binding cassette subfamily b member 11 (26) 26
disease progression (26) 26
mutations (26) 26
bile salt export pump (25) 25
treatment outcome (25) 25
ursodeoxycholic acid (24) 24
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alagille syndrome (20) 20
hereditary cholestasis (20) 20
transplantation (20) 20
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atp binding cassette transporter, sub-family b - genetics (16) 16
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biliary diversion (16) 16
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intractable pruritus (16) 16
pfic (16) 16
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atp8b1 (15) 15
biliary atresia (15) 15
progressive familial intrahepatic cholestasis type 2 (15) 15
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cholestasis, intrahepatic - metabolism (14) 14
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pregnancy (14) 14
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neonatal cholestasis (13) 13
partial external biliary diversion (13) 13
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bile acids and salts - metabolism (12) 12
biliary tract surgical procedures - methods (12) 12
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Journal of Clinical and Experimental Hepatology, ISSN 0973-6883, 2013, Volume 4, Issue 1, pp. 25 - 36
Journal Article
World journal of hepatology, ISSN 1948-5182, 05/2019, Volume 11, Issue 5, pp. 450 - 463
Progressive familial intrahepatic cholestasis (PFIC) refers to a disparate group of autosomal recessive disorders that are linked by the inability to... 
Bile transport | Intrahepatic cholestasis of pregnancy | Drug induced cholestasis | Progressive familial intrahepatic cholestasis | Bile acids | Systematic Review | Cholestasis | Benign recurrent intrahepatic cholestasis
Journal Article
Best Practice & Research: Clinical Gastroenterology, ISSN 1521-6918, 2010, Volume 24, Issue 5, pp. 541 - 553
Journal Article
Journal Article
Journal of Pediatric Gastroenterology and Nutrition, ISSN 0277-2116, 11/2018, Volume 67, Issue 5, pp. 643 - 648
OBJECTIVES:In patients with progressive familial intrahepatic cholestasis (PFIC), partial external biliary diversion (PEBD), which is associated with a... 
pediatric | CHILDREN | NUTRITION & DIETETICS | bile acids | ATRESIA | neonatal cholestasis | progressive familial intrahepatic cholestasis | PEDIATRICS | HEALTH | OUTCOMES | GASTROENTEROLOGY & HEPATOLOGY | child | BILE | Development and progression | Jaundice, Obstructive | Children | Health aspects | Cholestasis | Quality of life
Journal Article
Journal of Hepatology, ISSN 0168-8278, 07/2019, Volume 71, Issue 1, pp. 153 - 162
Progressive familial intrahepatic cholestasis type 3 (PFIC3), for which there are limited therapeutic options, often leads to end-stage liver disease before... 
Adeno-associated virus | Progressive familial intrahepatic cholestasis | MDR2 | ABCB4 | AAV | PFIC type 3 | PFIC3 | Correction cholestatic phenotype | MDR3 | AAV8 | Gene therapy | HOMOZYGOUS DISRUPTION | IMMUNE-RESPONSE | HEPATOCYTES | ADENOASSOCIATED VIRUS | MDR2 P-GLYCOPROTEIN | BILIARY DIVERSION | IN-VIVO | MOUSE MODEL | DISEASE | GASTROENTEROLOGY & HEPATOLOGY | BILE
Journal Article
JOURNAL OF PEDIATRIC SURGERY, ISSN 0022-3468, 03/2018, Volume 53, Issue 3, pp. 468 - 471
Background: Partial internal biliary diversion (PIBD) is an alternative approach for the treatment of devastating pruritus in patients with progressive... 
SURGERY | LIVER-DISEASE | INTRACTABLE PRURITUS | CHILDREN | Progressive familial intrahepatic cholestasis | SURGICAL APPROACH | Long term results | Internal biliary diversion | PEDIATRICS | MUTATIONS | ILEAL EXCLUSION | Pediatric | BILE
Journal Article
Journal of Pediatrics, The, ISSN 0022-3476, 2014, Volume 164, Issue 5, pp. 1219 - 1227.e3
To examine the effects of 4-phenylbutyrate (4PB) therapy in a patient with progressive familial intrahepatic cholestasis type 2. A homozygous c.3692G>A... 
Pediatrics | TfR | Gamma-glutamyl transferase | Direct bilirubin | Aspartate aminotransaminase | Hemagglutinin | ALT | mRNA | UDCA | Alanine aminotransaminase | D-Bil | Ursodeoxycholic acid | Messenger RNA | T-Bil | cDNA | qPCR | WT | Adenosine triphosphate | G protein-coupled receptor | AST | Empty vector | BSEP | OTCD | P-gp | Quantitative polymerase chain reaction | 4PB | PFIC2 | 4-Phenylbutyrate | Wild type | Progressive familial intrahepatic cholestasis type 2 | Transferrin receptor | P-glycoprotein | Polymerase chain reaction | EV | Complementary DNA | GGT | Ornithine transcarbamylase deficiency | Glyceraldehyde-3-phosphate dehydrogenase | GPCR | HA | ATP | GAPDH | Bile salt export pump | PCR | Total bilirubin | TRANSPORT | SALT EXPORT PUMP | PEDIATRICS | MUTATIONS | EXPRESSION | DEFICIENCY | BILE | Liver Function Tests | Phenylbutyrates - therapeutic use | Cholestasis, Intrahepatic - genetics | Humans | Infant | Cholestasis, Intrahepatic - drug therapy | Genetic Markers | Homozygote | Point Mutation | ATP-Binding Cassette Transporters - genetics | ATP Binding Cassette Subfamily B Member 11 | Pruritus - drug therapy | Gastrointestinal Agents - therapeutic use | Female | Pruritus - etiology | Cholestasis, Intrahepatic - complications | Medical colleges | Care and treatment | Jaundice, Obstructive | Liver | Genetic aspects | Pruritus | Cholestasis
Journal Article
Developmental period medicine, ISSN 1428-345X, 2018, Volume 22, Issue 4, pp. 385 - 389
Journal Article
Hepatology Research, ISSN 1386-6346, 12/2018, Volume 48, Issue 13, pp. 1163 - 1171
Journal Article
Journal of Pediatric Surgery, ISSN 0022-3468, 03/2018, Volume 53, Issue 3, pp. 468 - 471
Partial internal biliary diversion (PIBD) is an alternative approach for the treatment of devastating pruritus in patients with progressive familial... 
Internal biliary diversion | Progressive familial intrahepatic cholestasis | Long term results | Pediatric
Journal Article