Cystinuria with hyperuricemia and methioninuria: Biochemical study of a case
An otherwise typical cystinuric subject manifested several seemingly innocuous additional biochemical abnormalities: hyperuricemia, methioninuria and several serum amino acids in abnormal amounts. In contrast to findings reported in classic cystinuria, urinary cystine was decreased by homocysteine l...
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| Veröffentlicht in: | The American journal of medicine 1967-01, Vol.43 (1), p.125-130 |
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| container_title | The American journal of medicine |
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| creator | King, J.Stanton Wainer, Arthur |
| description | An otherwise typical cystinuric subject manifested several seemingly innocuous additional biochemical abnormalities: hyperuricemia, methioninuria and several serum amino acids in abnormal amounts. In contrast to findings reported in classic cystinuria, urinary cystine was decreased by homocysteine loading and temporarily decreased, followed by only a small increase after methionine loading. The response to taurine loading was atypical. Pyridoxine hydrochloride administration tended to correct some of the serum amino acid aberrations. Radiomethionine and radiotaurine administration revealed no absolute defect in the pathway between methionine and the metabolites of taurine. The response to penicillamine treatment was like that of the usual cystinuric patient. During methionine loading, urinary methionine was decreased, despite elevated serum levels, indicating that the methioninuria is not the result of a tubular defect. |
| doi_str_mv | 10.1016/0002-9343(67)90153-2 |
| format | Article |
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In contrast to findings reported in classic cystinuria, urinary cystine was decreased by homocysteine loading and temporarily decreased, followed by only a small increase after methionine loading. The response to taurine loading was atypical. Pyridoxine hydrochloride administration tended to correct some of the serum amino acid aberrations. Radiomethionine and radiotaurine administration revealed no absolute defect in the pathway between methionine and the metabolites of taurine. The response to penicillamine treatment was like that of the usual cystinuric patient. During methionine loading, urinary methionine was decreased, despite elevated serum levels, indicating that the methioninuria is not the result of a tubular defect.</description><identifier>ISSN: 0002-9343</identifier><identifier>EISSN: 1555-7162</identifier><identifier>DOI: 10.1016/0002-9343(67)90153-2</identifier><identifier>PMID: 4951411</identifier><language>eng</language><publisher>United States: Elsevier Inc</publisher><subject>Adult ; Amino Acids - urine ; Cystinuria - complications ; Cystinuria - drug therapy ; Cystinuria - urine ; Female ; Homocysteine - therapeutic use ; Humans ; Kidney Calculi - complications ; Kidney Calculi - metabolism ; Methionine - urine ; Penicillamine - adverse effects ; Penicillamine - therapeutic use ; Taurine - metabolism ; Threonine - blood ; Uric Acid - blood</subject><ispartof>The American journal of medicine, 1967-01, Vol.43 (1), p.125-130</ispartof><rights>1967</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktohtml>$$Uhttps://dx.doi.org/10.1016/0002-9343(67)90153-2$$EHTML$$P50$$Gelsevier$$H</linktohtml><link.rule.ids>314,776,780,3536,27903,27904,45974</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/4951411$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>King, J.Stanton</creatorcontrib><creatorcontrib>Wainer, Arthur</creatorcontrib><title>Cystinuria with hyperuricemia and methioninuria: Biochemical study of a case</title><title>The American journal of medicine</title><addtitle>Am J Med</addtitle><description>An otherwise typical cystinuric subject manifested several seemingly innocuous additional biochemical abnormalities: hyperuricemia, methioninuria and several serum amino acids in abnormal amounts. In contrast to findings reported in classic cystinuria, urinary cystine was decreased by homocysteine loading and temporarily decreased, followed by only a small increase after methionine loading. The response to taurine loading was atypical. Pyridoxine hydrochloride administration tended to correct some of the serum amino acid aberrations. Radiomethionine and radiotaurine administration revealed no absolute defect in the pathway between methionine and the metabolites of taurine. The response to penicillamine treatment was like that of the usual cystinuric patient. During methionine loading, urinary methionine was decreased, despite elevated serum levels, indicating that the methioninuria is not the result of a tubular defect.</description><subject>Adult</subject><subject>Amino Acids - urine</subject><subject>Cystinuria - complications</subject><subject>Cystinuria - drug therapy</subject><subject>Cystinuria - urine</subject><subject>Female</subject><subject>Homocysteine - therapeutic use</subject><subject>Humans</subject><subject>Kidney Calculi - complications</subject><subject>Kidney Calculi - metabolism</subject><subject>Methionine - urine</subject><subject>Penicillamine - adverse effects</subject><subject>Penicillamine - therapeutic use</subject><subject>Taurine - metabolism</subject><subject>Threonine - blood</subject><subject>Uric Acid - blood</subject><issn>0002-9343</issn><issn>1555-7162</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>1967</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNo9kFtLxDAQhYMo67r6DxTyqA_VXNqk8UFYizdY8EWfQzaZ0Mi2XZqu0n9v1i7CwHDmDMOcD6FLSm4poeKOEMIyxXN-LeSNIrTgGTtCc1oURSapYMdo_r9yis5i_EqSqELM0CxXBc0pnaNVNcYhtLs-GPwThhrX4xb6JC00aWRahxsY6tC109I9fgydrZNpzQbHYedG3HlssDURztGJN5sIF4e-QJ_PTx_Va7Z6f3mrlqsMmKBDJvO19NTI0jHvFHWuzD1nXinOnBfOeGFcKcukpExBc-PBgpRKGLCpgC_Q1XR3u1s34PS2D43pR31IlfyHyYf0xHeAXkcboLXgQg920K4LmhK9p6j3iPQekRZS_1HUjP8CcVtkxA</recordid><startdate>19670101</startdate><enddate>19670101</enddate><creator>King, J.Stanton</creator><creator>Wainer, Arthur</creator><general>Elsevier Inc</general><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope></search><sort><creationdate>19670101</creationdate><title>Cystinuria with hyperuricemia and methioninuria: Biochemical study of a case</title><author>King, J.Stanton ; Wainer, Arthur</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-e261t-74b7f1a78d2fd91dd84f32f9932df6daf6ad87832d770164afece7796aecaece3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>1967</creationdate><topic>Adult</topic><topic>Amino Acids - urine</topic><topic>Cystinuria - complications</topic><topic>Cystinuria - drug therapy</topic><topic>Cystinuria - urine</topic><topic>Female</topic><topic>Homocysteine - therapeutic use</topic><topic>Humans</topic><topic>Kidney Calculi - complications</topic><topic>Kidney Calculi - metabolism</topic><topic>Methionine - urine</topic><topic>Penicillamine - adverse effects</topic><topic>Penicillamine - therapeutic use</topic><topic>Taurine - metabolism</topic><topic>Threonine - blood</topic><topic>Uric Acid - blood</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>King, J.Stanton</creatorcontrib><creatorcontrib>Wainer, Arthur</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><jtitle>The American journal of medicine</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>King, J.Stanton</au><au>Wainer, Arthur</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Cystinuria with hyperuricemia and methioninuria: Biochemical study of a case</atitle><jtitle>The American journal of medicine</jtitle><addtitle>Am J Med</addtitle><date>1967-01-01</date><risdate>1967</risdate><volume>43</volume><issue>1</issue><spage>125</spage><epage>130</epage><pages>125-130</pages><issn>0002-9343</issn><eissn>1555-7162</eissn><abstract>An otherwise typical cystinuric subject manifested several seemingly innocuous additional biochemical abnormalities: hyperuricemia, methioninuria and several serum amino acids in abnormal amounts. In contrast to findings reported in classic cystinuria, urinary cystine was decreased by homocysteine loading and temporarily decreased, followed by only a small increase after methionine loading. The response to taurine loading was atypical. Pyridoxine hydrochloride administration tended to correct some of the serum amino acid aberrations. Radiomethionine and radiotaurine administration revealed no absolute defect in the pathway between methionine and the metabolites of taurine. The response to penicillamine treatment was like that of the usual cystinuric patient. During methionine loading, urinary methionine was decreased, despite elevated serum levels, indicating that the methioninuria is not the result of a tubular defect.</abstract><cop>United States</cop><pub>Elsevier Inc</pub><pmid>4951411</pmid><doi>10.1016/0002-9343(67)90153-2</doi><tpages>6</tpages></addata></record> |
| fulltext | fulltext |
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| ispartof | The American journal of medicine, 1967-01, Vol.43 (1), p.125-130 |
| issn | 0002-9343 1555-7162 |
| language | eng |
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| source | MEDLINE; Elsevier ScienceDirect Journals |
| subjects | Adult Amino Acids - urine Cystinuria - complications Cystinuria - drug therapy Cystinuria - urine Female Homocysteine - therapeutic use Humans Kidney Calculi - complications Kidney Calculi - metabolism Methionine - urine Penicillamine - adverse effects Penicillamine - therapeutic use Taurine - metabolism Threonine - blood Uric Acid - blood |
| title | Cystinuria with hyperuricemia and methioninuria: Biochemical study of a case |
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