A father and son with cholestasis and peripheral pulmonic stenosis
Identifieur interne : 010F96 ( Main/Merge ); précédent : 010F95; suivant : 010F97A father and son with cholestasis and peripheral pulmonic stenosis
Auteurs : Caroline A. Riely [États-Unis] ; Douglas R. Labrecque [États-Unis] ; Cameron Ghent [États-Unis] ; Arthur Horwich [États-Unis] ; Gerald Klatskin [États-Unis]Source :
- The Journal of Pediatrics [ 0022-3476 ] ; 1978.
Abstract
We report a father and son with the syndrome of cholestasis and peripheral pulmonic stenosis. These cases demonstrate the clinical and biochemical features noted in previous reports of this entity and allow us to differentiate clearly this syndrome, with its benign course, from other more progressive forms of intrahepatic cholestasis. The vertical transmission supports a genetic etiology for this disease. Although serum bile acid levels are elevated in these patients, the individual bile acids do not display a distinctive pattern and no abnormal bile acids are identified.
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DOI: 10.1016/S0022-3476(78)80428-4
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Labrecque</name></author><author><name sortKey="Ghent, Cameron" sort="Ghent, Cameron" uniqKey="Ghent C" first="Cameron" last="Ghent">Cameron Ghent</name></author><author><name sortKey="Horwich, Arthur" sort="Horwich, Arthur" uniqKey="Horwich A" first="Arthur" last="Horwich">Arthur Horwich</name></author><author><name sortKey="Klatskin, Gerald" sort="Klatskin, Gerald" uniqKey="Klatskin G" first="Gerald" last="Klatskin">Gerald Klatskin</name></author></titleStmt><publicationStmt><idno type="wicri:source">ISTEX</idno><idno type="RBID">ISTEX:BF5FDF593FEC4ACD25590B68F2BC7076609080A2</idno><date when="1978" year="1978">1978</date><idno type="doi">10.1016/S0022-3476(78)80428-4</idno><idno type="url">https://api.istex.fr/document/BF5FDF593FEC4ACD25590B68F2BC7076609080A2/fulltext/pdf</idno><idno type="wicri:Area/Istex/Corpus">005996</idno><idno type="wicri:explorRef" wicri:stream="Istex" wicri:step="Corpus" wicri:corpus="ISTEX">005996</idno><idno type="wicri:Area/Istex/Curation">005996</idno><idno type="wicri:Area/Istex/Checkpoint">006843</idno><idno type="wicri:explorRef" wicri:stream="Istex" wicri:step="Checkpoint">006843</idno><idno type="wicri:doubleKey">0022-3476:1978:Riely C:a:father:and</idno><idno type="wicri:Area/Main/Merge">010F96</idno></publicationStmt><sourceDesc><biblStruct><analytic><title level="a" type="main" xml:lang="en">A father and son with cholestasis and peripheral pulmonic stenosis</title><author><name sortKey="Riely, Caroline A" sort="Riely, Caroline A" uniqKey="Riely C" first="Caroline A." last="Riely">Caroline A. 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These cases demonstrate the clinical and biochemical features noted in previous reports of this entity and allow us to differentiate clearly this syndrome, with its benign course, from other more progressive forms of intrahepatic cholestasis. The vertical transmission supports a genetic etiology for this disease. Although serum bile acid levels are elevated in these patients, the individual bile acids do not display a distinctive pattern and no abnormal bile acids are identified.</div></front></TEI></record>Pour manipuler ce document sous Unix (Dilib)
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