Abstract：
:Bile acid synthetic defects are uncommon disorders that cause progressive cholestatic liver disease that is often lethal in infancy or early childhood. Five specific primary defects have been described. Diagnosis is based on mass spectrometry of urine and serum. Pathogenesis of liver injury is related to persistent reduction in levels of normal bile acids and accumulation of abnormal, potentially hepatotoxic, intermediaries. Sites of injury are the liver cell, the bile canaliculus, and the smallest bile ductules. The interlobular bile ducts are normal. The liver lesion is progressive chronic hepatitis with an especially high incidence of GCT in patients who present in infancy. Bile acid replacement therapy is usually effective in arresting the liver injury. Regression of liver damage has been documented during treatment of patients who were diagnosed early in life. Because bile acid synthetic disorders are the only cholestatic diseases of infancy in which GCT of hepatocytes is consistently present, the author suggest that the injury responsible for GCT may be specific for toxic bile acids. Accordingly, immaturity of the bile acid synthetic pathway may render many otherwise normal infants vulnerable to transient "neonatal hepatitis" with GCT in a broad range of cholestatic disorders.
journal_name
Clin Liver Disjournal_title
Clinics in liver diseaseauthors
Bove KEdoi
10.1016/s1089-3261(05)70144-6subject
Has Abstractpub_date
2000-11-01 00:00:00pages
831-48issue
4eissn
1089-3261issn
1557-8224pii
S1089-3261(05)70144-6journal_volume
4pub_type
杂志文章,评审abstract：:Newer noninvasive tests have begun to replace liver biopsy for staging purposes. The clinician must evaluate these tools and apply them to individual patients. None of these modalities give the exact same staging of fibrosis as a liver biopsy, but they are excellent tools for risk stratification. Still, it should be r...
journal_title：Clinics in liver disease
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journal_title：Clinics in liver disease
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journal_title：Clinics in liver disease
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journal_title：Clinics in liver disease
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journal_title：Clinics in liver disease
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journal_title：Clinics in liver disease
pub_type： 杂志文章,评审
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pub_type： 共识发展会议,杂志文章,评审
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journal_title：Clinics in liver disease
pub_type： 杂志文章,评审
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journal_title：Clinics in liver disease
pub_type： 杂志文章,评审
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journal_title：Clinics in liver disease
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journal_title：Clinics in liver disease
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journal_title：Clinics in liver disease
pub_type： 杂志文章,评审
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journal_title：Clinics in liver disease
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journal_title：Clinics in liver disease
pub_type： 杂志文章,评审
doi：10.1016/j.cld.2008.02.006
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