Tyrosinemia with acute intermittent porphyria: Aminolevulinic acid dehydratase deficiency related to elevated urinary aminolevulinic acid levels
A patient who had hereditary tyrosinemia was observed during two illnesses to have characteristics ofacute intermittent porphyria with associated hypertension. Metabolic studies revealed elevated levels of urinary aminolevulinic acid but normal levels of porphyrin metabolites associated with, and po...
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| Veröffentlicht in: | The Journal of pediatrics 1977-03, Vol.90 (3), p.400-404 |
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| container_issue | 3 |
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| container_title | The Journal of pediatrics |
| container_volume | 90 |
| creator | Strife, C. Frederic Zuroweste, Edward L. Emmett, Edward A. Finelli, Vincent N. Petering, H.G. Berry, Helen K. |
| description | A patient who had hereditary tyrosinemia was observed during two illnesses to have characteristics ofacute intermittent porphyria with associated hypertension. Metabolic studies revealed elevated levels of urinary aminolevulinic acid but normal levels of porphyrin metabolites associated with, and possibly explained by, decreased red blood cell activity of the zinc-dependent enzyme, aminolevulinic acid dehydratase. Zinc deficiency could not be directly associated with the diminished enzyme activity. The patient's hypertension appeared to be related to increased urinary excretion of catecholamines and to elevated renin activity in peripheral venous blood. |
| doi_str_mv | 10.1016/S0022-3476(77)80701-4 |
| format | Article |
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Frederic ; Zuroweste, Edward L. ; Emmett, Edward A. ; Finelli, Vincent N. ; Petering, H.G. ; Berry, Helen K.</creator><creatorcontrib>Strife, C. Frederic ; Zuroweste, Edward L. ; Emmett, Edward A. ; Finelli, Vincent N. ; Petering, H.G. ; Berry, Helen K.</creatorcontrib><description>A patient who had hereditary tyrosinemia was observed during two illnesses to have characteristics ofacute intermittent porphyria with associated hypertension. Metabolic studies revealed elevated levels of urinary aminolevulinic acid but normal levels of porphyrin metabolites associated with, and possibly explained by, decreased red blood cell activity of the zinc-dependent enzyme, aminolevulinic acid dehydratase. Zinc deficiency could not be directly associated with the diminished enzyme activity. The patient's hypertension appeared to be related to increased urinary excretion of catecholamines and to elevated renin activity in peripheral venous blood.</description><identifier>ISSN: 0022-3476</identifier><identifier>EISSN: 1097-6833</identifier><identifier>DOI: 10.1016/S0022-3476(77)80701-4</identifier><identifier>PMID: 839332</identifier><language>eng</language><publisher>United States: Mosby, Inc</publisher><subject>Adolescent ; Amino Acid Metabolism, Inborn Errors - complications ; Aminolevulinic Acid - urine ; Catecholamines - urine ; Female ; Humans ; Hydro-Lyases - deficiency ; Levulinic Acids - urine ; Porphobilinogen Synthase - deficiency ; Porphyrias - complications ; Renin - blood ; Tyrosine - metabolism</subject><ispartof>The Journal of pediatrics, 1977-03, Vol.90 (3), p.400-404</ispartof><rights>1977 The C. V. 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Frederic</creatorcontrib><creatorcontrib>Zuroweste, Edward L.</creatorcontrib><creatorcontrib>Emmett, Edward A.</creatorcontrib><creatorcontrib>Finelli, Vincent N.</creatorcontrib><creatorcontrib>Petering, H.G.</creatorcontrib><creatorcontrib>Berry, Helen K.</creatorcontrib><title>Tyrosinemia with acute intermittent porphyria: Aminolevulinic acid dehydratase deficiency related to elevated urinary aminolevulinic acid levels</title><title>The Journal of pediatrics</title><addtitle>J Pediatr</addtitle><description>A patient who had hereditary tyrosinemia was observed during two illnesses to have characteristics ofacute intermittent porphyria with associated hypertension. Metabolic studies revealed elevated levels of urinary aminolevulinic acid but normal levels of porphyrin metabolites associated with, and possibly explained by, decreased red blood cell activity of the zinc-dependent enzyme, aminolevulinic acid dehydratase. Zinc deficiency could not be directly associated with the diminished enzyme activity. The patient's hypertension appeared to be related to increased urinary excretion of catecholamines and to elevated renin activity in peripheral venous blood.</description><subject>Adolescent</subject><subject>Amino Acid Metabolism, Inborn Errors - complications</subject><subject>Aminolevulinic Acid - urine</subject><subject>Catecholamines - urine</subject><subject>Female</subject><subject>Humans</subject><subject>Hydro-Lyases - deficiency</subject><subject>Levulinic Acids - urine</subject><subject>Porphobilinogen Synthase - deficiency</subject><subject>Porphyrias - complications</subject><subject>Renin - blood</subject><subject>Tyrosine - metabolism</subject><issn>0022-3476</issn><issn>1097-6833</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>1977</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNqFUctuFDEQtBCvJfAHIPmE4DDQHjtjOxcURbykSBwIZ8tr92gbzWOxPUHzF3wy3t0oJyRO3a2u7lJVMfZKwDsBonv_HaBtG6l090brtwY0iEY9YBsBVjedkfIh29xDnrJnOf8EAKsAnrDHRlop2w37c7OmOdOEI3n-m8qO-7AU5DQVTCOVglPh-zntd2sif8EvR5rmAW-XgSYKFUyRR9ytMfniM9a-p0A4hZUnHHzByMvMsV4c-yXR5NPK_T_e1BGH_Jw96v2Q8cVdPWM_Pn28ufrSXH_7_PXq8roJ8tyWpgti20lrtVGtFcpK2LbSix6s7frYKemtFdFKbWUMsYt9FEabbfRgwVil5Bl7ffq7T_OvBXNxI-WAw-AnnJfsjDRKtdpU4PkJGKpROWHv9onGKsIJcIcg3DEId3DZae2OQbgDwcs7gmU7Yry_Ojlf1x9O66oZbwmTy0ffMFLCUFyc6T8EfwGpV5to</recordid><startdate>197703</startdate><enddate>197703</enddate><creator>Strife, C. Frederic</creator><creator>Zuroweste, Edward L.</creator><creator>Emmett, Edward A.</creator><creator>Finelli, Vincent N.</creator><creator>Petering, H.G.</creator><creator>Berry, Helen K.</creator><general>Mosby, Inc</general><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></search><sort><creationdate>197703</creationdate><title>Tyrosinemia with acute intermittent porphyria: Aminolevulinic acid dehydratase deficiency related to elevated urinary aminolevulinic acid levels</title><author>Strife, C. Frederic ; Zuroweste, Edward L. ; Emmett, Edward A. ; Finelli, Vincent N. ; Petering, H.G. ; Berry, Helen K.</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c359t-6c1b63997842914930b23a1f0996fd643a991d93793dcd6dfd1878bda09089443</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>1977</creationdate><topic>Adolescent</topic><topic>Amino Acid Metabolism, Inborn Errors - complications</topic><topic>Aminolevulinic Acid - urine</topic><topic>Catecholamines - urine</topic><topic>Female</topic><topic>Humans</topic><topic>Hydro-Lyases - deficiency</topic><topic>Levulinic Acids - urine</topic><topic>Porphobilinogen Synthase - deficiency</topic><topic>Porphyrias - complications</topic><topic>Renin - blood</topic><topic>Tyrosine - metabolism</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Strife, C. Frederic</creatorcontrib><creatorcontrib>Zuroweste, Edward L.</creatorcontrib><creatorcontrib>Emmett, Edward A.</creatorcontrib><creatorcontrib>Finelli, Vincent N.</creatorcontrib><creatorcontrib>Petering, H.G.</creatorcontrib><creatorcontrib>Berry, Helen K.</creatorcontrib><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><jtitle>The Journal of pediatrics</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Strife, C. Frederic</au><au>Zuroweste, Edward L.</au><au>Emmett, Edward A.</au><au>Finelli, Vincent N.</au><au>Petering, H.G.</au><au>Berry, Helen K.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Tyrosinemia with acute intermittent porphyria: Aminolevulinic acid dehydratase deficiency related to elevated urinary aminolevulinic acid levels</atitle><jtitle>The Journal of pediatrics</jtitle><addtitle>J Pediatr</addtitle><date>1977-03</date><risdate>1977</risdate><volume>90</volume><issue>3</issue><spage>400</spage><epage>404</epage><pages>400-404</pages><issn>0022-3476</issn><eissn>1097-6833</eissn><abstract>A patient who had hereditary tyrosinemia was observed during two illnesses to have characteristics ofacute intermittent porphyria with associated hypertension. Metabolic studies revealed elevated levels of urinary aminolevulinic acid but normal levels of porphyrin metabolites associated with, and possibly explained by, decreased red blood cell activity of the zinc-dependent enzyme, aminolevulinic acid dehydratase. Zinc deficiency could not be directly associated with the diminished enzyme activity. The patient's hypertension appeared to be related to increased urinary excretion of catecholamines and to elevated renin activity in peripheral venous blood.</abstract><cop>United States</cop><pub>Mosby, Inc</pub><pmid>839332</pmid><doi>10.1016/S0022-3476(77)80701-4</doi><tpages>5</tpages></addata></record> |
| fulltext | fulltext |
| identifier | ISSN: 0022-3476 |
| ispartof | The Journal of pediatrics, 1977-03, Vol.90 (3), p.400-404 |
| issn | 0022-3476 1097-6833 |
| language | eng |
| recordid | cdi_proquest_miscellaneous_83844278 |
| source | MEDLINE; Elsevier ScienceDirect Journals Complete |
| subjects | Adolescent Amino Acid Metabolism, Inborn Errors - complications Aminolevulinic Acid - urine Catecholamines - urine Female Humans Hydro-Lyases - deficiency Levulinic Acids - urine Porphobilinogen Synthase - deficiency Porphyrias - complications Renin - blood Tyrosine - metabolism |
| title | Tyrosinemia with acute intermittent porphyria: Aminolevulinic acid dehydratase deficiency related to elevated urinary aminolevulinic acid levels |
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