Portacaval shunt in patients with familial hypercholesterolemia
Portacaval shunt was performed in ten patients with homozygous and two with heterozygous familial hypercholesterolemia (FH). Total serum cholesterol was lowered by 20% to 55.4% during follow-up periods of 14 months to almost 9 years, with commensurate decreases in LDL cholesterol. The effect on HDL...
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| Veröffentlicht in: | Annals of surgery 1983-09, Vol.198 (3), p.273-283 |
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| container_title | Annals of surgery |
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| creator | STARZL, T. E CHASE, H. P BENSON, L. N AHRENS, E. H. JR MCNAMARA, D. J BILHEIMER, D. W SCHAEFER, E. J REY, J PORTER, K. A STEIN, E FRANCAVILLA, A |
| description | Portacaval shunt was performed in ten patients with homozygous and two with heterozygous familial hypercholesterolemia (FH). Total serum cholesterol was lowered by 20% to 55.4% during follow-up periods of 14 months to almost 9 years, with commensurate decreases in LDL cholesterol. The effect on HDL cholesterol and triglyceride levels was variable. Tendinocutaneous xanthomas diminished or disappeared. Growth and development in children proceeded or accelerated. There was no detectable emotional or intellectual deterioration. Hepatic failure did not occur, although blood ammonia concentrations and serum alkaline phosphatase levels increased relative to preoperative values. Cardiac symptoms were often improved, but evidence of reversal of cardiovascular lesions was inconclusive. Three patients with pre-existing heart disease died of cardiac complications after 4 months, 18 1/2 months, and 30 months. Portacaval shunt has been effective therapy for patients with FH who were refractory or intolerant to medical treatment; it should be performed before the development of irreversible cardiovascular damage. |
| doi_str_mv | 10.1097/00000658-198309000-00004 |
| format | Article |
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E ; CHASE, H. P ; BENSON, L. N ; AHRENS, E. H. JR ; MCNAMARA, D. J ; BILHEIMER, D. W ; SCHAEFER, E. J ; REY, J ; PORTER, K. A ; STEIN, E ; FRANCAVILLA, A</creator><creatorcontrib>STARZL, T. E ; CHASE, H. P ; BENSON, L. N ; AHRENS, E. H. JR ; MCNAMARA, D. J ; BILHEIMER, D. W ; SCHAEFER, E. J ; REY, J ; PORTER, K. A ; STEIN, E ; FRANCAVILLA, A</creatorcontrib><description>Portacaval shunt was performed in ten patients with homozygous and two with heterozygous familial hypercholesterolemia (FH). Total serum cholesterol was lowered by 20% to 55.4% during follow-up periods of 14 months to almost 9 years, with commensurate decreases in LDL cholesterol. The effect on HDL cholesterol and triglyceride levels was variable. Tendinocutaneous xanthomas diminished or disappeared. Growth and development in children proceeded or accelerated. There was no detectable emotional or intellectual deterioration. Hepatic failure did not occur, although blood ammonia concentrations and serum alkaline phosphatase levels increased relative to preoperative values. Cardiac symptoms were often improved, but evidence of reversal of cardiovascular lesions was inconclusive. Three patients with pre-existing heart disease died of cardiac complications after 4 months, 18 1/2 months, and 30 months. Portacaval shunt has been effective therapy for patients with FH who were refractory or intolerant to medical treatment; it should be performed before the development of irreversible cardiovascular damage.</description><identifier>ISSN: 0003-4932</identifier><identifier>EISSN: 1528-1140</identifier><identifier>DOI: 10.1097/00000658-198309000-00004</identifier><identifier>PMID: 6615051</identifier><identifier>CODEN: ANSUA5</identifier><language>eng</language><publisher>Hagerstown, MD: Lippincott</publisher><subject>Adolescent ; Adult ; Biological and medical sciences ; Cardiovascular Diseases - complications ; Child ; Child, Preschool ; Cholesterol - blood ; Disorders of blood lipids. Hyperlipoproteinemia ; Female ; Follow-Up Studies ; Growth Disorders - etiology ; Hepatic Encephalopathy - etiology ; Humans ; Hyperlipoproteinemia Type II - blood ; Hyperlipoproteinemia Type II - complications ; Hyperlipoproteinemia Type II - surgery ; Intelligence Tests ; Kidney Function Tests ; Liver Function Tests ; Male ; Medical sciences ; Metabolic diseases ; Middle Aged ; Portacaval Shunt, Surgical - adverse effects ; Triglycerides - blood</subject><ispartof>Annals of surgery, 1983-09, Vol.198 (3), p.273-283</ispartof><rights>1984 INIST-CNRS</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c376t-c03470b40e8a2446b6d7586789d6f294024ff09c596c3898127ebe907df22c333</citedby></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC1353292/pdf/$$EPDF$$P50$$Gpubmedcentral$$H</linktopdf><linktohtml>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC1353292/$$EHTML$$P50$$Gpubmedcentral$$H</linktohtml><link.rule.ids>230,314,723,776,780,881,27901,27902,53766,53768</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=9313105$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/6615051$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>STARZL, T. E</creatorcontrib><creatorcontrib>CHASE, H. P</creatorcontrib><creatorcontrib>BENSON, L. N</creatorcontrib><creatorcontrib>AHRENS, E. H. JR</creatorcontrib><creatorcontrib>MCNAMARA, D. J</creatorcontrib><creatorcontrib>BILHEIMER, D. W</creatorcontrib><creatorcontrib>SCHAEFER, E. J</creatorcontrib><creatorcontrib>REY, J</creatorcontrib><creatorcontrib>PORTER, K. A</creatorcontrib><creatorcontrib>STEIN, E</creatorcontrib><creatorcontrib>FRANCAVILLA, A</creatorcontrib><title>Portacaval shunt in patients with familial hypercholesterolemia</title><title>Annals of surgery</title><addtitle>Ann Surg</addtitle><description>Portacaval shunt was performed in ten patients with homozygous and two with heterozygous familial hypercholesterolemia (FH). Total serum cholesterol was lowered by 20% to 55.4% during follow-up periods of 14 months to almost 9 years, with commensurate decreases in LDL cholesterol. The effect on HDL cholesterol and triglyceride levels was variable. Tendinocutaneous xanthomas diminished or disappeared. Growth and development in children proceeded or accelerated. There was no detectable emotional or intellectual deterioration. Hepatic failure did not occur, although blood ammonia concentrations and serum alkaline phosphatase levels increased relative to preoperative values. Cardiac symptoms were often improved, but evidence of reversal of cardiovascular lesions was inconclusive. Three patients with pre-existing heart disease died of cardiac complications after 4 months, 18 1/2 months, and 30 months. Portacaval shunt has been effective therapy for patients with FH who were refractory or intolerant to medical treatment; it should be performed before the development of irreversible cardiovascular damage.</description><subject>Adolescent</subject><subject>Adult</subject><subject>Biological and medical sciences</subject><subject>Cardiovascular Diseases - complications</subject><subject>Child</subject><subject>Child, Preschool</subject><subject>Cholesterol - blood</subject><subject>Disorders of blood lipids. Hyperlipoproteinemia</subject><subject>Female</subject><subject>Follow-Up Studies</subject><subject>Growth Disorders - etiology</subject><subject>Hepatic Encephalopathy - etiology</subject><subject>Humans</subject><subject>Hyperlipoproteinemia Type II - blood</subject><subject>Hyperlipoproteinemia Type II - complications</subject><subject>Hyperlipoproteinemia Type II - surgery</subject><subject>Intelligence Tests</subject><subject>Kidney Function Tests</subject><subject>Liver Function Tests</subject><subject>Male</subject><subject>Medical sciences</subject><subject>Metabolic diseases</subject><subject>Middle Aged</subject><subject>Portacaval Shunt, Surgical - adverse effects</subject><subject>Triglycerides - blood</subject><issn>0003-4932</issn><issn>1528-1140</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>1983</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNpVUMtOwzAQtBColMInIOWAuAXWj_hxAaGKl4QEBzhbrusQozyKnRT173FpqWAvq52ZHa8HoQzDBQYlLmFdvJA5VpKCSkO-RtgeGuOCJBgz2EfjBNGcKUoO0VGMHwCYSRAjNOIcF1DgMbp-6UJvrFmaOovV0PaZb7OF6b1r-5h9-b7KStP42ie-Wi1csFVXu9i7kFrjzTE6KE0d3cm2T9Db3e3r9CF_er5_nN485ZYK3ucWKBMwY-CkIYzxGZ-LQnIh1ZyXRDEgrCxB2UJxS6WSmAg3cwrEvCTEUkon6GrjuxhmjZvbdF4wtV4E35iw0p3x-j_T-kq_d0uNaUGJIsngfGsQus8h_UA3PlpX16Z13RC1BM4UEyIJ5UZoQxdjcOXuEQx6Hb7-DV_vwv-BWFo9_XvkbnGbduLPtryJ1tRlMK31cSdTFFMMBf0GDbOMxA</recordid><startdate>19830901</startdate><enddate>19830901</enddate><creator>STARZL, T. E</creator><creator>CHASE, H. P</creator><creator>BENSON, L. N</creator><creator>AHRENS, E. H. JR</creator><creator>MCNAMARA, D. J</creator><creator>BILHEIMER, D. W</creator><creator>SCHAEFER, E. J</creator><creator>REY, J</creator><creator>PORTER, K. A</creator><creator>STEIN, E</creator><creator>FRANCAVILLA, A</creator><general>Lippincott</general><scope>IQODW</scope><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope><scope>5PM</scope></search><sort><creationdate>19830901</creationdate><title>Portacaval shunt in patients with familial hypercholesterolemia</title><author>STARZL, T. E ; CHASE, H. P ; BENSON, L. N ; AHRENS, E. H. JR ; MCNAMARA, D. J ; BILHEIMER, D. W ; SCHAEFER, E. J ; REY, J ; PORTER, K. A ; STEIN, E ; FRANCAVILLA, A</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c376t-c03470b40e8a2446b6d7586789d6f294024ff09c596c3898127ebe907df22c333</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>1983</creationdate><topic>Adolescent</topic><topic>Adult</topic><topic>Biological and medical sciences</topic><topic>Cardiovascular Diseases - complications</topic><topic>Child</topic><topic>Child, Preschool</topic><topic>Cholesterol - blood</topic><topic>Disorders of blood lipids. Hyperlipoproteinemia</topic><topic>Female</topic><topic>Follow-Up Studies</topic><topic>Growth Disorders - etiology</topic><topic>Hepatic Encephalopathy - etiology</topic><topic>Humans</topic><topic>Hyperlipoproteinemia Type II - blood</topic><topic>Hyperlipoproteinemia Type II - complications</topic><topic>Hyperlipoproteinemia Type II - surgery</topic><topic>Intelligence Tests</topic><topic>Kidney Function Tests</topic><topic>Liver Function Tests</topic><topic>Male</topic><topic>Medical sciences</topic><topic>Metabolic diseases</topic><topic>Middle Aged</topic><topic>Portacaval Shunt, Surgical - adverse effects</topic><topic>Triglycerides - blood</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>STARZL, T. E</creatorcontrib><creatorcontrib>CHASE, H. P</creatorcontrib><creatorcontrib>BENSON, L. N</creatorcontrib><creatorcontrib>AHRENS, E. H. JR</creatorcontrib><creatorcontrib>MCNAMARA, D. J</creatorcontrib><creatorcontrib>BILHEIMER, D. W</creatorcontrib><creatorcontrib>SCHAEFER, E. J</creatorcontrib><creatorcontrib>REY, J</creatorcontrib><creatorcontrib>PORTER, K. A</creatorcontrib><creatorcontrib>STEIN, E</creatorcontrib><creatorcontrib>FRANCAVILLA, A</creatorcontrib><collection>Pascal-Francis</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><collection>PubMed Central (Full Participant titles)</collection><jtitle>Annals of surgery</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>STARZL, T. E</au><au>CHASE, H. P</au><au>BENSON, L. N</au><au>AHRENS, E. H. JR</au><au>MCNAMARA, D. J</au><au>BILHEIMER, D. W</au><au>SCHAEFER, E. J</au><au>REY, J</au><au>PORTER, K. A</au><au>STEIN, E</au><au>FRANCAVILLA, A</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Portacaval shunt in patients with familial hypercholesterolemia</atitle><jtitle>Annals of surgery</jtitle><addtitle>Ann Surg</addtitle><date>1983-09-01</date><risdate>1983</risdate><volume>198</volume><issue>3</issue><spage>273</spage><epage>283</epage><pages>273-283</pages><issn>0003-4932</issn><eissn>1528-1140</eissn><coden>ANSUA5</coden><abstract>Portacaval shunt was performed in ten patients with homozygous and two with heterozygous familial hypercholesterolemia (FH). Total serum cholesterol was lowered by 20% to 55.4% during follow-up periods of 14 months to almost 9 years, with commensurate decreases in LDL cholesterol. The effect on HDL cholesterol and triglyceride levels was variable. Tendinocutaneous xanthomas diminished or disappeared. Growth and development in children proceeded or accelerated. There was no detectable emotional or intellectual deterioration. Hepatic failure did not occur, although blood ammonia concentrations and serum alkaline phosphatase levels increased relative to preoperative values. Cardiac symptoms were often improved, but evidence of reversal of cardiovascular lesions was inconclusive. Three patients with pre-existing heart disease died of cardiac complications after 4 months, 18 1/2 months, and 30 months. Portacaval shunt has been effective therapy for patients with FH who were refractory or intolerant to medical treatment; it should be performed before the development of irreversible cardiovascular damage.</abstract><cop>Hagerstown, MD</cop><pub>Lippincott</pub><pmid>6615051</pmid><doi>10.1097/00000658-198309000-00004</doi><tpages>11</tpages><oa>free_for_read</oa></addata></record> |
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| ispartof | Annals of surgery, 1983-09, Vol.198 (3), p.273-283 |
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| source | MEDLINE; EZB-FREE-00999 freely available EZB journals; PubMed Central; Journals@Ovid Complete |
| subjects | Adolescent Adult Biological and medical sciences Cardiovascular Diseases - complications Child Child, Preschool Cholesterol - blood Disorders of blood lipids. Hyperlipoproteinemia Female Follow-Up Studies Growth Disorders - etiology Hepatic Encephalopathy - etiology Humans Hyperlipoproteinemia Type II - blood Hyperlipoproteinemia Type II - complications Hyperlipoproteinemia Type II - surgery Intelligence Tests Kidney Function Tests Liver Function Tests Male Medical sciences Metabolic diseases Middle Aged Portacaval Shunt, Surgical - adverse effects Triglycerides - blood |
| title | Portacaval shunt in patients with familial hypercholesterolemia |
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