Failure of partial splenectomy to ameliorate the anemia of pyruvate kinase deficiency
The authors report a case of pyruvate kinase deficiency in a 4-year-old patient who required monthly blood transfusions. A partial splenectomy was performed to decrease the transfusion requirements and at the same time preserve splenic function. After removal of 80% of splenic tissue there was no am...
Gespeichert in:
| Veröffentlicht in: | Journal of pediatric surgery 1997-04, Vol.32 (4), p.641-642 |
|---|---|
| Hauptverfasser: | , , , |
| Format: | Artikel |
| Sprache: | eng |
| Schlagworte: | |
| Online-Zugang: | Volltext |
| Tags: |
Tag hinzufügen
Keine Tags, Fügen Sie den ersten Tag hinzu!
|
| container_end_page | 642 |
|---|---|
| container_issue | 4 |
| container_start_page | 641 |
| container_title | Journal of pediatric surgery |
| container_volume | 32 |
| creator | Sandoval, Claudio Stringel, Gustavo Weisberger, James Jayabose, Somasundaram |
| description | The authors report a case of pyruvate kinase deficiency in a 4-year-old patient who required monthly blood transfusions. A partial splenectomy was performed to decrease the transfusion requirements and at the same time preserve splenic function. After removal of 80% of splenic tissue there was no amelioration of her transfusion needs. She responded well to subsequent total splenectomy performed 6 months later, and is currently well and transfusion independent. Rapid splenic regeneration possibly caused by erythrophagocytosis and extramedullary hematopoiesis is the presumed reason for this patient's failure to respond to partial splenectomy. |
| doi_str_mv | 10.1016/S0022-3468(97)90729-6 |
| format | Article |
| fullrecord | <record><control><sourceid>proquest_cross</sourceid><recordid>TN_cdi_proquest_miscellaneous_79000260</recordid><sourceformat>XML</sourceformat><sourcesystem>PC</sourcesystem><els_id>S0022346897907296</els_id><sourcerecordid>79000260</sourcerecordid><originalsourceid>FETCH-LOGICAL-c389t-842627a2e36e283e82ec94b9ff9b9bd1683310f148e08f8d247189974b3c3ec63</originalsourceid><addsrcrecordid>eNqFkEtLAzEQx4MotVY_QmEPInpYzWObx0mk-IKCB_UcstkJRvdRk91Cv73pg149Dcz8Mv_JD6EpwbcEE373jjGlOSu4vFbiRmFBVc6P0JjMGMlnmIljND4gp-gsxm-MUxuTERopQrkQfIw-n4yvhwBZ57KlCb03dRaXNbRg-65ZZ32XmQZq3wXTQ9Z_QWZaaLzZ8uswrDbtH9-aCFkFzlsPrV2foxNn6ggX-zpJOY8f85d88fb8On9Y5JZJ1eeyoJwKQ4FxoJKBpGBVUSrnVKnKinDJGMGOFBKwdLKihSBSKVGUzDKwnE3Q1W7vMnS_A8ReNz5aqOt0ZDdELVT6MuU4gbMdaEMXYwCnl8E3Jqw1wXqjU2916o0rrYTe6tSbgOk-YCgbqA6v9v7S_HI_N9Ga2gXTWh8PGBWYMyYSdr_DIMlYeQg6bkVB5UMSravO_3PIHxtckUE</addsrcrecordid><sourcetype>Aggregation Database</sourcetype><iscdi>true</iscdi><recordtype>article</recordtype><pqid>79000260</pqid></control><display><type>article</type><title>Failure of partial splenectomy to ameliorate the anemia of pyruvate kinase deficiency</title><source>MEDLINE</source><source>ScienceDirect Journals (5 years ago - present)</source><creator>Sandoval, Claudio ; Stringel, Gustavo ; Weisberger, James ; Jayabose, Somasundaram</creator><creatorcontrib>Sandoval, Claudio ; Stringel, Gustavo ; Weisberger, James ; Jayabose, Somasundaram</creatorcontrib><description>The authors report a case of pyruvate kinase deficiency in a 4-year-old patient who required monthly blood transfusions. A partial splenectomy was performed to decrease the transfusion requirements and at the same time preserve splenic function. After removal of 80% of splenic tissue there was no amelioration of her transfusion needs. She responded well to subsequent total splenectomy performed 6 months later, and is currently well and transfusion independent. Rapid splenic regeneration possibly caused by erythrophagocytosis and extramedullary hematopoiesis is the presumed reason for this patient's failure to respond to partial splenectomy.</description><identifier>ISSN: 0022-3468</identifier><identifier>EISSN: 1531-5037</identifier><identifier>DOI: 10.1016/S0022-3468(97)90729-6</identifier><identifier>PMID: 9126776</identifier><identifier>CODEN: JPDSA3</identifier><language>eng</language><publisher>Philadelphia, PA: Elsevier Inc</publisher><subject>Anemia, Hemolytic - etiology ; Anemia, Hemolytic - therapy ; Biological and medical sciences ; Blood Transfusion ; Carbohydrates (enzymatic deficiencies). Glycogenosis ; Child, Preschool ; Errors of metabolism ; Female ; Humans ; Medical sciences ; Metabolic diseases ; Pyruvate Kinase - deficiency ; Regeneration ; Spleen - physiology ; Splenectomy - methods ; Treatment Failure</subject><ispartof>Journal of pediatric surgery, 1997-04, Vol.32 (4), p.641-642</ispartof><rights>1997</rights><rights>1997 INIST-CNRS</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c389t-842627a2e36e283e82ec94b9ff9b9bd1683310f148e08f8d247189974b3c3ec63</citedby><cites>FETCH-LOGICAL-c389t-842627a2e36e283e82ec94b9ff9b9bd1683310f148e08f8d247189974b3c3ec63</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktohtml>$$Uhttps://dx.doi.org/10.1016/S0022-3468(97)90729-6$$EHTML$$P50$$Gelsevier$$H</linktohtml><link.rule.ids>314,780,784,3549,27923,27924,45994</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=2706337$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/9126776$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Sandoval, Claudio</creatorcontrib><creatorcontrib>Stringel, Gustavo</creatorcontrib><creatorcontrib>Weisberger, James</creatorcontrib><creatorcontrib>Jayabose, Somasundaram</creatorcontrib><title>Failure of partial splenectomy to ameliorate the anemia of pyruvate kinase deficiency</title><title>Journal of pediatric surgery</title><addtitle>J Pediatr Surg</addtitle><description>The authors report a case of pyruvate kinase deficiency in a 4-year-old patient who required monthly blood transfusions. A partial splenectomy was performed to decrease the transfusion requirements and at the same time preserve splenic function. After removal of 80% of splenic tissue there was no amelioration of her transfusion needs. She responded well to subsequent total splenectomy performed 6 months later, and is currently well and transfusion independent. Rapid splenic regeneration possibly caused by erythrophagocytosis and extramedullary hematopoiesis is the presumed reason for this patient's failure to respond to partial splenectomy.</description><subject>Anemia, Hemolytic - etiology</subject><subject>Anemia, Hemolytic - therapy</subject><subject>Biological and medical sciences</subject><subject>Blood Transfusion</subject><subject>Carbohydrates (enzymatic deficiencies). Glycogenosis</subject><subject>Child, Preschool</subject><subject>Errors of metabolism</subject><subject>Female</subject><subject>Humans</subject><subject>Medical sciences</subject><subject>Metabolic diseases</subject><subject>Pyruvate Kinase - deficiency</subject><subject>Regeneration</subject><subject>Spleen - physiology</subject><subject>Splenectomy - methods</subject><subject>Treatment Failure</subject><issn>0022-3468</issn><issn>1531-5037</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>1997</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNqFkEtLAzEQx4MotVY_QmEPInpYzWObx0mk-IKCB_UcstkJRvdRk91Cv73pg149Dcz8Mv_JD6EpwbcEE373jjGlOSu4vFbiRmFBVc6P0JjMGMlnmIljND4gp-gsxm-MUxuTERopQrkQfIw-n4yvhwBZ57KlCb03dRaXNbRg-65ZZ32XmQZq3wXTQ9Z_QWZaaLzZ8uswrDbtH9-aCFkFzlsPrV2foxNn6ggX-zpJOY8f85d88fb8On9Y5JZJ1eeyoJwKQ4FxoJKBpGBVUSrnVKnKinDJGMGOFBKwdLKihSBSKVGUzDKwnE3Q1W7vMnS_A8ReNz5aqOt0ZDdELVT6MuU4gbMdaEMXYwCnl8E3Jqw1wXqjU2916o0rrYTe6tSbgOk-YCgbqA6v9v7S_HI_N9Ga2gXTWh8PGBWYMyYSdr_DIMlYeQg6bkVB5UMSravO_3PIHxtckUE</recordid><startdate>19970401</startdate><enddate>19970401</enddate><creator>Sandoval, Claudio</creator><creator>Stringel, Gustavo</creator><creator>Weisberger, James</creator><creator>Jayabose, Somasundaram</creator><general>Elsevier Inc</general><general>Elsevier</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></search><sort><creationdate>19970401</creationdate><title>Failure of partial splenectomy to ameliorate the anemia of pyruvate kinase deficiency</title><author>Sandoval, Claudio ; Stringel, Gustavo ; Weisberger, James ; Jayabose, Somasundaram</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c389t-842627a2e36e283e82ec94b9ff9b9bd1683310f148e08f8d247189974b3c3ec63</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>1997</creationdate><topic>Anemia, Hemolytic - etiology</topic><topic>Anemia, Hemolytic - therapy</topic><topic>Biological and medical sciences</topic><topic>Blood Transfusion</topic><topic>Carbohydrates (enzymatic deficiencies). Glycogenosis</topic><topic>Child, Preschool</topic><topic>Errors of metabolism</topic><topic>Female</topic><topic>Humans</topic><topic>Medical sciences</topic><topic>Metabolic diseases</topic><topic>Pyruvate Kinase - deficiency</topic><topic>Regeneration</topic><topic>Spleen - physiology</topic><topic>Splenectomy - methods</topic><topic>Treatment Failure</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Sandoval, Claudio</creatorcontrib><creatorcontrib>Stringel, Gustavo</creatorcontrib><creatorcontrib>Weisberger, James</creatorcontrib><creatorcontrib>Jayabose, Somasundaram</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><jtitle>Journal of pediatric surgery</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Sandoval, Claudio</au><au>Stringel, Gustavo</au><au>Weisberger, James</au><au>Jayabose, Somasundaram</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Failure of partial splenectomy to ameliorate the anemia of pyruvate kinase deficiency</atitle><jtitle>Journal of pediatric surgery</jtitle><addtitle>J Pediatr Surg</addtitle><date>1997-04-01</date><risdate>1997</risdate><volume>32</volume><issue>4</issue><spage>641</spage><epage>642</epage><pages>641-642</pages><issn>0022-3468</issn><eissn>1531-5037</eissn><coden>JPDSA3</coden><abstract>The authors report a case of pyruvate kinase deficiency in a 4-year-old patient who required monthly blood transfusions. A partial splenectomy was performed to decrease the transfusion requirements and at the same time preserve splenic function. After removal of 80% of splenic tissue there was no amelioration of her transfusion needs. She responded well to subsequent total splenectomy performed 6 months later, and is currently well and transfusion independent. Rapid splenic regeneration possibly caused by erythrophagocytosis and extramedullary hematopoiesis is the presumed reason for this patient's failure to respond to partial splenectomy.</abstract><cop>Philadelphia, PA</cop><pub>Elsevier Inc</pub><pmid>9126776</pmid><doi>10.1016/S0022-3468(97)90729-6</doi><tpages>2</tpages></addata></record> |
| fulltext | fulltext |
| identifier | ISSN: 0022-3468 |
| ispartof | Journal of pediatric surgery, 1997-04, Vol.32 (4), p.641-642 |
| issn | 0022-3468 1531-5037 |
| language | eng |
| recordid | cdi_proquest_miscellaneous_79000260 |
| source | MEDLINE; ScienceDirect Journals (5 years ago - present) |
| subjects | Anemia, Hemolytic - etiology Anemia, Hemolytic - therapy Biological and medical sciences Blood Transfusion Carbohydrates (enzymatic deficiencies). Glycogenosis Child, Preschool Errors of metabolism Female Humans Medical sciences Metabolic diseases Pyruvate Kinase - deficiency Regeneration Spleen - physiology Splenectomy - methods Treatment Failure |
| title | Failure of partial splenectomy to ameliorate the anemia of pyruvate kinase deficiency |
| url | https://sfx.bib-bvb.de/sfx_tum?ctx_ver=Z39.88-2004&ctx_enc=info:ofi/enc:UTF-8&ctx_tim=2025-01-08T14%3A23%3A05IST&url_ver=Z39.88-2004&url_ctx_fmt=infofi/fmt:kev:mtx:ctx&rfr_id=info:sid/primo.exlibrisgroup.com:primo3-Article-proquest_cross&rft_val_fmt=info:ofi/fmt:kev:mtx:journal&rft.genre=article&rft.atitle=Failure%20of%20partial%20splenectomy%20to%20ameliorate%20the%20anemia%20of%20pyruvate%20kinase%20deficiency&rft.jtitle=Journal%20of%20pediatric%20surgery&rft.au=Sandoval,%20Claudio&rft.date=1997-04-01&rft.volume=32&rft.issue=4&rft.spage=641&rft.epage=642&rft.pages=641-642&rft.issn=0022-3468&rft.eissn=1531-5037&rft.coden=JPDSA3&rft_id=info:doi/10.1016/S0022-3468(97)90729-6&rft_dat=%3Cproquest_cross%3E79000260%3C/proquest_cross%3E%3Curl%3E%3C/url%3E&disable_directlink=true&sfx.directlink=off&sfx.report_link=0&rft_id=info:oai/&rft_pqid=79000260&rft_id=info:pmid/9126776&rft_els_id=S0022346897907296&rfr_iscdi=true |