Prognosis, with evaluation of general biochemistry, of liver disease in lymphoedema cholestasis syndrome 1 (LCS1/Aagenaes syndrome).
Identifieur interne : 003A17 ( PubMed/Corpus ); précédent : 003A16; suivant : 003A18Prognosis, with evaluation of general biochemistry, of liver disease in lymphoedema cholestasis syndrome 1 (LCS1/Aagenaes syndrome).
Auteurs : Monica Drivdal ; Torleif Trydal ; Tor-Arne Hagve ; Ingunn Bergstad ; Oystein AagenaesSource :
- Scandinavian journal of gastroenterology [ 0036-5521 ] ; 2006.
English descriptors
- KwdEn :
- Adolescent, Adult, Alkaline Phosphatase (blood), Bile Acids and Salts (blood), Case-Control Studies, Child, Child, Preschool, Cholestasis (genetics), Cholestasis (metabolism), Cholestasis (mortality), Humans, Infant, Infant, Newborn, Liver Cirrhosis (etiology), Lymphedema (genetics), Lymphedema (metabolism), Lymphedema (mortality), Middle Aged, Prognosis, Random Allocation, Serum Albumin (analysis), Syndrome, gamma-Glutamyltransferase (blood).
- MESH :
- chemical , analysis : Serum Albumin.
- chemical , blood : Alkaline Phosphatase, Bile Acids and Salts, gamma-Glutamyltransferase.
- etiology : Liver Cirrhosis.
- genetics : Cholestasis, Lymphedema.
- metabolism : Cholestasis, Lymphedema.
- mortality : Cholestasis, Lymphedema.
- Adolescent, Adult, Case-Control Studies, Child, Child, Preschool, Humans, Infant, Infant, Newborn, Middle Aged, Prognosis, Random Allocation, Syndrome.
Abstract
To investigate the prognosis of liver disease in Aagenaes syndrome (lymphoedema cholestasis syndrome 1 (LCS1)), which is an autosomal recessive inherited syndrome consisting of neonatal cholestasis with intermittent cholestatic episodes in childhood into adulthood and development of lymphoedema. Forty Norwegian patients are known to have this condition, 25 of whom are alive. A clinical description of the liver disease is supplied with a case-control study.
DOI: 10.1080/00365520500335183
PubMed: 16635916
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pubmed:16635916Le document en format XML
<record><TEI><teiHeader><fileDesc><titleStmt><title xml:lang="en">Prognosis, with evaluation of general biochemistry, of liver disease in lymphoedema cholestasis syndrome 1 (LCS1/Aagenaes syndrome).</title><author><name sortKey="Drivdal, Monica" sort="Drivdal, Monica" uniqKey="Drivdal M" first="Monica" last="Drivdal">Monica Drivdal</name><affiliation><nlm:affiliation>Department of Nutrition, Institute of Basic Medical Sciences, University of Oslo, Oslo, Norway. monica.drivdal@uus.no</nlm:affiliation></affiliation></author><author><name sortKey="Trydal, Torleif" sort="Trydal, Torleif" uniqKey="Trydal T" first="Torleif" last="Trydal">Torleif Trydal</name></author><author><name sortKey="Hagve, Tor Arne" sort="Hagve, Tor Arne" uniqKey="Hagve T" first="Tor-Arne" last="Hagve">Tor-Arne Hagve</name></author><author><name sortKey="Bergstad, Ingunn" sort="Bergstad, Ingunn" uniqKey="Bergstad I" first="Ingunn" last="Bergstad">Ingunn Bergstad</name></author><author><name sortKey="Aagenaes, Oystein" sort="Aagenaes, Oystein" uniqKey="Aagenaes O" first="Oystein" last="Aagenaes">Oystein Aagenaes</name></author></titleStmt><publicationStmt><idno type="wicri:source">PubMed</idno><date when="2006">2006</date><idno type="RBID">pubmed:16635916</idno><idno type="pmid">16635916</idno><idno type="doi">10.1080/00365520500335183</idno><idno type="wicri:Area/PubMed/Corpus">003A17</idno><idno type="wicri:explorRef" wicri:stream="PubMed" wicri:step="Corpus" wicri:corpus="PubMed">003A17</idno></publicationStmt><sourceDesc><biblStruct><analytic><title xml:lang="en">Prognosis, with evaluation of general biochemistry, of liver disease in lymphoedema cholestasis syndrome 1 (LCS1/Aagenaes syndrome).</title><author><name sortKey="Drivdal, Monica" sort="Drivdal, Monica" uniqKey="Drivdal M" first="Monica" last="Drivdal">Monica Drivdal</name><affiliation><nlm:affiliation>Department of Nutrition, Institute of Basic Medical Sciences, University of Oslo, Oslo, Norway. monica.drivdal@uus.no</nlm:affiliation></affiliation></author><author><name sortKey="Trydal, Torleif" sort="Trydal, Torleif" uniqKey="Trydal T" first="Torleif" last="Trydal">Torleif Trydal</name></author><author><name sortKey="Hagve, Tor Arne" sort="Hagve, Tor Arne" uniqKey="Hagve T" first="Tor-Arne" last="Hagve">Tor-Arne Hagve</name></author><author><name sortKey="Bergstad, Ingunn" sort="Bergstad, Ingunn" uniqKey="Bergstad I" first="Ingunn" last="Bergstad">Ingunn Bergstad</name></author><author><name sortKey="Aagenaes, Oystein" sort="Aagenaes, Oystein" uniqKey="Aagenaes O" first="Oystein" last="Aagenaes">Oystein Aagenaes</name></author></analytic><series><title level="j">Scandinavian journal of gastroenterology</title><idno type="ISSN">0036-5521</idno><imprint><date when="2006" type="published">2006</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Adolescent</term><term>Adult</term><term>Alkaline Phosphatase (blood)</term><term>Bile Acids and Salts (blood)</term><term>Case-Control Studies</term><term>Child</term><term>Child, Preschool</term><term>Cholestasis (genetics)</term><term>Cholestasis (metabolism)</term><term>Cholestasis (mortality)</term><term>Humans</term><term>Infant</term><term>Infant, Newborn</term><term>Liver Cirrhosis (etiology)</term><term>Lymphedema (genetics)</term><term>Lymphedema (metabolism)</term><term>Lymphedema (mortality)</term><term>Middle Aged</term><term>Prognosis</term><term>Random Allocation</term><term>Serum Albumin (analysis)</term><term>Syndrome</term><term>gamma-Glutamyltransferase (blood)</term></keywords><keywords scheme="MESH" type="chemical" qualifier="analysis" xml:lang="en"><term>Serum Albumin</term></keywords><keywords scheme="MESH" type="chemical" qualifier="blood" xml:lang="en"><term>Alkaline Phosphatase</term><term>Bile Acids and Salts</term><term>gamma-Glutamyltransferase</term></keywords><keywords scheme="MESH" qualifier="etiology" xml:lang="en"><term>Liver Cirrhosis</term></keywords><keywords scheme="MESH" qualifier="genetics" xml:lang="en"><term>Cholestasis</term><term>Lymphedema</term></keywords><keywords scheme="MESH" qualifier="metabolism" xml:lang="en"><term>Cholestasis</term><term>Lymphedema</term></keywords><keywords scheme="MESH" qualifier="mortality" xml:lang="en"><term>Cholestasis</term><term>Lymphedema</term></keywords><keywords scheme="MESH" xml:lang="en"><term>Adolescent</term><term>Adult</term><term>Case-Control Studies</term><term>Child</term><term>Child, Preschool</term><term>Humans</term><term>Infant</term><term>Infant, Newborn</term><term>Middle Aged</term><term>Prognosis</term><term>Random Allocation</term><term>Syndrome</term></keywords></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">To investigate the prognosis of liver disease in Aagenaes syndrome (lymphoedema cholestasis syndrome 1 (LCS1)), which is an autosomal recessive inherited syndrome consisting of neonatal cholestasis with intermittent cholestatic episodes in childhood into adulthood and development of lymphoedema. Forty Norwegian patients are known to have this condition, 25 of whom are alive. A clinical description of the liver disease is supplied with a case-control study.</div></front></TEI><pubmed><MedlineCitation Status="MEDLINE" Owner="NLM"><PMID Version="1">16635916</PMID><DateCreated><Year>2006</Year><Month>04</Month><Day>25</Day></DateCreated><DateCompleted><Year>2006</Year><Month>08</Month><Day>18</Day></DateCompleted><DateRevised><Year>2006</Year><Month>11</Month><Day>15</Day></DateRevised><Article PubModel="Print"><Journal><ISSN IssnType="Print">0036-5521</ISSN><JournalIssue CitedMedium="Print"><Volume>41</Volume><Issue>4</Issue><PubDate><Year>2006</Year><Month>Apr</Month></PubDate></JournalIssue><Title>Scandinavian journal of gastroenterology</Title><ISOAbbreviation>Scand. J. Gastroenterol.</ISOAbbreviation></Journal><ArticleTitle>Prognosis, with evaluation of general biochemistry, of liver disease in lymphoedema cholestasis syndrome 1 (LCS1/Aagenaes syndrome).</ArticleTitle><Pagination><MedlinePgn>465-71</MedlinePgn></Pagination><Abstract><AbstractText Label="OBJECTIVE" NlmCategory="OBJECTIVE">To investigate the prognosis of liver disease in Aagenaes syndrome (lymphoedema cholestasis syndrome 1 (LCS1)), which is an autosomal recessive inherited syndrome consisting of neonatal cholestasis with intermittent cholestatic episodes in childhood into adulthood and development of lymphoedema. Forty Norwegian patients are known to have this condition, 25 of whom are alive. A clinical description of the liver disease is supplied with a case-control study.</AbstractText><AbstractText Label="MATERIAL AND METHODS" NlmCategory="METHODS">In this paper we review the course of the liver disease in the Norwegian cohort of patients and present results from a case-control study in the patients above 10 years of age. The case-control study was performed on 15 patients without clinical cholestasis (itching and sometimes jaundice) at the time of the study. An evaluation of 11 patients above 15 years of age without chronic biochemical cholestasis (increased alkaline phosphatase (ALP), gamma-glutamyl transferase (GGT) and/or serum bile acids) was also carried out. For each patient one randomly identified control person was included (15 in one study, 11 in the other).</AbstractText><AbstractText Label="RESULTS" NlmCategory="RESULTS">Cirrhosis with either transplantation or death in infancy or early childhood occurred in six patients; slowly developing cirrhosis occurred in three patients. Two patients may be in the process of developing cirrhosis. Significantly increased ALP and GGT levels were found in patients with normal liver biochemistry in the preceding years when compared with the case control group. Additionally, albumin was found to be lower in older patients.</AbstractText><AbstractText Label="CONCLUSIONS" NlmCategory="CONCLUSIONS">Compared with that for other types of hereditary neonatal cholestasis, patients with LCS1 have a relatively good prognosis. More than 50% can expect a normal life span.</AbstractText></Abstract><AuthorList CompleteYN="Y"><Author ValidYN="Y"><LastName>Drivdal</LastName><ForeName>Monica</ForeName><Initials>M</Initials><AffiliationInfo><Affiliation>Department of Nutrition, Institute of Basic Medical Sciences, University of Oslo, Oslo, Norway. monica.drivdal@uus.no</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Trydal</LastName><ForeName>Torleif</ForeName><Initials>T</Initials></Author><Author ValidYN="Y"><LastName>Hagve</LastName><ForeName>Tor-Arne</ForeName><Initials>TA</Initials></Author><Author ValidYN="Y"><LastName>Bergstad</LastName><ForeName>Ingunn</ForeName><Initials>I</Initials></Author><Author ValidYN="Y"><LastName>Aagenaes</LastName><ForeName>Oystein</ForeName><Initials>O</Initials></Author></AuthorList><Language>eng</Language><PublicationTypeList><PublicationType UI="D016428">Journal Article</PublicationType><PublicationType UI="D013485">Research Support, Non-U.S. Gov't</PublicationType></PublicationTypeList></Article><MedlineJournalInfo><Country>England</Country><MedlineTA>Scand J Gastroenterol</MedlineTA><NlmUniqueID>0060105</NlmUniqueID><ISSNLinking>0036-5521</ISSNLinking></MedlineJournalInfo><ChemicalList><Chemical><RegistryNumber>0</RegistryNumber><NameOfSubstance UI="D001647">Bile Acids and Salts</NameOfSubstance></Chemical><Chemical><RegistryNumber>0</RegistryNumber><NameOfSubstance UI="D012709">Serum Albumin</NameOfSubstance></Chemical><Chemical><RegistryNumber>EC 2.3.2.2</RegistryNumber><NameOfSubstance UI="D005723">gamma-Glutamyltransferase</NameOfSubstance></Chemical><Chemical><RegistryNumber>EC 3.1.3.1</RegistryNumber><NameOfSubstance UI="D000469">Alkaline Phosphatase</NameOfSubstance></Chemical></ChemicalList><CitationSubset>IM</CitationSubset><MeshHeadingList><MeshHeading><DescriptorName UI="D000293" MajorTopicYN="N">Adolescent</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D000328" MajorTopicYN="N">Adult</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D000469" MajorTopicYN="N">Alkaline Phosphatase</DescriptorName><QualifierName UI="Q000097" MajorTopicYN="N">blood</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D001647" MajorTopicYN="N">Bile Acids and Salts</DescriptorName><QualifierName UI="Q000097" MajorTopicYN="N">blood</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D016022" MajorTopicYN="N">Case-Control Studies</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D002648" MajorTopicYN="N">Child</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D002675" MajorTopicYN="N">Child, Preschool</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D002779" MajorTopicYN="N">Cholestasis</DescriptorName><QualifierName UI="Q000235" MajorTopicYN="N">genetics</QualifierName><QualifierName UI="Q000378" MajorTopicYN="Y">metabolism</QualifierName><QualifierName UI="Q000401" MajorTopicYN="Y">mortality</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D006801" MajorTopicYN="N">Humans</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D007223" MajorTopicYN="N">Infant</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D007231" MajorTopicYN="N">Infant, Newborn</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D008103" MajorTopicYN="N">Liver Cirrhosis</DescriptorName><QualifierName UI="Q000209" MajorTopicYN="N">etiology</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D008209" MajorTopicYN="N">Lymphedema</DescriptorName><QualifierName UI="Q000235" MajorTopicYN="N">genetics</QualifierName><QualifierName UI="Q000378" MajorTopicYN="Y">metabolism</QualifierName><QualifierName UI="Q000401" MajorTopicYN="Y">mortality</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D008875" MajorTopicYN="N">Middle Aged</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D011379" MajorTopicYN="N">Prognosis</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D011897" MajorTopicYN="N">Random Allocation</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D012709" MajorTopicYN="N">Serum Albumin</DescriptorName><QualifierName UI="Q000032" MajorTopicYN="N">analysis</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D013577" MajorTopicYN="N">Syndrome</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D005723" MajorTopicYN="N">gamma-Glutamyltransferase</DescriptorName><QualifierName UI="Q000097" MajorTopicYN="N">blood</QualifierName></MeshHeading></MeshHeadingList></MedlineCitation><PubmedData><History><PubMedPubDate PubStatus="pubmed"><Year>2006</Year><Month>4</Month><Day>26</Day><Hour>9</Hour><Minute>0</Minute></PubMedPubDate><PubMedPubDate PubStatus="medline"><Year>2006</Year><Month>8</Month><Day>19</Day><Hour>9</Hour><Minute>0</Minute></PubMedPubDate><PubMedPubDate 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