Update on the etiologies and management of neonatal cholestasis

Clinics in Perinatology

Volumn 29, Issue 1, 2002, Pages 159-180

  Karpen, Saul J ^a  

^a BAYLOR COLLEGE OF MEDICINE   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

ALPHA TOCOPHEROL; MULTIVITAMIN; URSODEOXYCHOLIC ACID; VITAMIN K GROUP;

BILE FLOW; BILE SECRETION; CHOLESTASIS; CLINICAL EXAMINATION; DISEASE CLASSIFICATION; DISEASE PREDISPOSITION; HUMAN; INTRAHEPATIC BILE DUCT; NEWBORN CARE; NEWBORN JAUNDICE; PATHOPHYSIOLOGY; PRIORITY JOURNAL; REVIEW; SYNDROME DELINEATION; TOTAL PARENTERAL NUTRITION; VITAMIN SUPPLEMENTATION;

EID: 0036127891     PISSN: 00955108     EISSN: None     Source Type: Journal    
DOI: 10.1016/S0095-5108(03)00069-1     Document Type: Review

References (112)

1. Identification of a novel gene family encoding human liver-specific organic anion transporter LST-1
2. Syndromic paucity of interlobular bile ducts (Alagille syndrome or arteriohepatic dysplasia): Review of 80 cases
3. Hepatic ductular hypoplasia associated with characteristic facies, vertebral malformations, retarded physical, mental, and sexual development, and cardiac murmur
4. A multivariable risk factor analysis of the portoenterostomy (Kasai) procedure for biliary atresia: Twenty-five years of experience from two centers
5. Bile acid therapy in pediatric hepatobiliary disease: The role of ursodeoxycholic acid
6. Fetal and neonatal bile acid synthesis and metabolism-clinical implications
7. Inborn errors of bile acid biosynthesis and transport: Novel forms of metabolic liver disease
8. Neonatal cholestasis
9. Biliary atresia: Current concepts and research directions. Summary of a symposium
10. Pathologic versus physiologic cholestasis: Elevated serum concentration of a secondary bile acid in the presence of hepatobiliary disease
11. Biliary atresia: Pathogenesis and treatment
12. Physiologic cholestasis: II. Serum bile acid levels reflect the development of the enterohepatic circulation in rats
13. Resolution of nonsyndromic paucity of intrahepatic bile ducts in infancy
14. Cholestatic syndromes of infancy and childhood
15. The intrahepatic cholangiopathies
16. Liver disease caused by disorders of bile acid synthesis
17. Nonsyndromic paucity of intrahepatic bile ducts in infancy and idiopathic ductopenia in adulthood: The same syndrome?
18. Fine-resolution mapping by haplotype evaluation: The examples of PFIC 1 and BRIC
19. A gene encoding a P-type ATPase mutated in two forms of hereditary cholestasis
20. Extrahepatic biliary atresia and associated anomalies: Etiologic heterogeneity suggested by distinctive patterns of associations
21. Is the Kasai operation still indicated in children older than 3 months diagnosed with biliary atresia?
22. Counts DR: Cholestasis and hypoglycemia: Manifestations of congenital anterior hypopituitarism
23. Liver and biliary problems in cystic fibrosis
24. Hepatic uptake of bilirubin and its conjugates by the human organic anion transporter SLC21A6
25. Biliary atresia: The King's College Hospital experience, 1974- 1995
26. Neonatal hyperbilirubinemia
27. Interleukin-1 beta suppresses retinoid trans-activation of two hepatic transporter genes involved in bile formation
28. Destructive intrahepatic bile duct diseases
29. Intrahepatic bile ducts under the lens
30. Proliferation to paucity: Evolution of bile duct abnormalities in a case of Alagille syndrome
31. Mutations in the MDR 3 gene cause progressive familial intrahepatic cholestasis
32. Septicemia in the newborn
33. Cholestatic jaundice and congenital hypopituitarism
34. Diagnostic approach to the child with hepatobiliary disease
35. FIC1, the protein affected in two forms of hereditary cholestasis, is localized in the cholangiocyte and the canalicular membrane of the hepatocyte
36. Disorders in protein glycosylation and potential therapy: Tip of an iceberg?
37. US approach to jaundice in infants and children
38. Diagnosis of biliary atresia: Relative accuracy of percutaneous liver biopsy, open liver biopsy, and operative cholangiography
39. Neonatal cholestasis and hypopituitarism
40. Bile acid secretion, bile flow and biliary lipid secretion in humans
41. Measurement of bile and acid kinetics by isotope dilution in man
42. Jaundice at 14 days of age: Exclude biliary atresia
43. Progressive familial intrahepatic cholestasis: Genetic basis and treatment
44. The wide spectrum of multidrug resistance 3 deficiency: From neonatal cholestasis to cirrhosis of adulthood
45. Genetic cholestasis: Lessons from the molecular physiology of bile formation
46. The molecular genetics of familial intrahepatic cholestasis
47. Hepatocanalicular bile salt export pump deficiency in patients with progressive familial intrahepatic cholestasis
48. Paucity of interlobular bile ducts: Arteriohepatic dysplasia and nonsyndromic duct paucity
49. Nonsyndromatic paucity of interlobular bile ducts: Light and electron microscopic evaluation of sequential liver biopsies in early childhood
50. Neonatal cholestasis
51. A missense mutation in FIC 1 is associated with greenland familial cholestasis
52. Neonatal hemochromatosis
53. Progressive familial intrahepatic cholestasis: A personal perspective
54. Oropharyngeal and upper respiratory tract mucosal-gland siderosis in neonatal hemochromatosis: An approach to biopsy diagnosis
55. Nonsyndromic paucity of interlobular bile ducts: Clinical and laboratory findings of 10 cases
56. Molecular mechanisms of cholestasis: Causes and consequences of impaired bile formation
57. Evaluation of the triangular cord sign in the diagnosis of biliary atresia
58. Alagille syndrome
59. Clinical and molecular genetics of Alagille syndrome
60. Biliary atresia
61. Clinical spectrum of X-linked hyper-IgM syndrome
62. Alagille syndrome is caused by mutations in human Jagged1, which encodes a ligand for Notch1
63. Outcome of liver disease in children with Alagille syndrome: A study of 163 patients
64. Anomalous development of the hepatobiliary system in the Inv mouse
65. Late referral for biliary atresia: Missed opportunities for effective surgery
66. Sclerosing cholangitis in the paediatric patient
67. Sepsis-associated cholestasis
68. Molecular aspects of hepatobiliary transport
69. The secretory function of the liver: New aspects of hepatobiliary transport
70. Biliary atresia: Current management and outcome
71. Neonatal hemochromatosis
72. Mechanisms and regulation of bile secretion
73. The outcome of surgery for biliary atresia and the current status of long-term survivors
74. Mutations in the human Jagged 1 gene are responsible for Alagille syndrome
75. MDR3 mutations: A glimpse into pandora's box and the future of canalicular pathophysiology
76. The ultrasonographic 'triangular cord' coupled with gallbladder images in the diagnostic prediction of blliary atresia from infantile intrahepatic cholestasis
77. A new diagnostic approach to blliary atresia with emphasis on the ultrasonographic triangular cord sign: Comparison of ultrasonography, hepatobiliary scintigraphy, and liver needle biopsy in the evaluation of infantile cholestasis
78. Neonatal cholestasis: A red alert for the jaundiced newborn
79. Alpha-1-antitrypsin deficiency
80. Primary sclerosing cholangitis in children
81. MDR 3 gene defect in adults with symptomatic intrahepatic and gallbladder cholesterol cholelithiasis
82. Bile acid biosynthesis
83. Biliary atresia: Surgical managementand treatment options as they relate to outcome
84. Prognosis of nonsyndromic paucity of intrahepatic bile ducts
85. Neonatal cholestasis, hypoglycemia, and congenital hypopituitarism
86. Long-term survivors in biliary atresia: Findings for a 20-year survival group
87. Neonatal hemochromatosis: Outcomes of pharmacologic and surgical therapies
88. Total parenteral nutrition-related liver disease
89. Multicenter trial of d-alpha-tocopheryl polyethylene glycol 1000 succinate for treatment of vitamin E deficiency in children with chronic cholestasis
90. Jagged 1 mutations in alagille syndrome
91. Langerhans' cell histiocytosis presenting with hepatic dysfunction
92. Extrahepatic biliary atresia with laterality sequence anomalies
93. A gene encoding a liver-specific ABC transporter is mutated in progressive familial intrahepatic cholestasis
94. Physiologic cholestasis: Elevation of the primary serum bile acid concentrations in normal infants
95. Making the diagnosis of biliary atresia using the triangular cord sign and gallbladder length
96. Parenteral nutrition-associated cholestasis
97. Genetic defects in hepatocanalicular transport
98. Hepatocellular bile salt transport: Lessons from cholestasis
99. Inflammation-induced cholestasis
100. Genetic disorders and molecular mechanisms in cholestatic liver disease: A clinical approach
101. Molecular pathogenesis of cholestasis
102. Molecular regulation of hepatocellular transport systems in cholestasis
103. Recurrent familial intrahepatic cholestasis in the Faeroe Islands: Phenotypic heterogeneity but genetic homogeneity
104. Detection of reovirus RNA in hepatobiliary tissues from patients with extrahepatic biliary atresia and choledochal cysts
105. The development of the intrahepatic bile ducts in man: A keratin- immunohistochemical study
106. Neonatal hemochromatosis: The importance of early recognition of liver failure
107. Bile acid metabolism and fat absorption in newborn infants
108. Bile-salt metabolism in the newborn: Measurement of pool size and synthesis by stable isotope technic
109. Neonatal hepatitis: A diagnostic approach
110. Bile salt metabolism in the human premature infant: Preliminary observations of pool size and synthesis rate following prenatal administration of dexamethasone and phenobarbital
111. Congenital disorders of glycosylation: Have you encountered them?
112. Liver biopsy in neonatal cholestasis: A review on statistical grounds