Appendectomy in familial Mediterranean fever : clinical, genetic and pathological findings
Abdominal attacks of familial Mediterranean fever (FMF) may simulate acute appendicitis and bring about considerable uncertainty. The similar presentation of the two clinical entities often leads to an unnecessary appendectomy. 182 consecutive FMF patients were retrospectively reviewed for this stud...
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Veröffentlicht in: | Clinical and experimental rheumatology 2008-07, Vol.26 (4), p.568-573 |
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creator | LIDAR, M DORON, A KEDEM, R YOSEPOVICH, A LANGEVITZ, P LIVNEH, A |
description | Abdominal attacks of familial Mediterranean fever (FMF) may simulate acute appendicitis and bring about considerable uncertainty. The similar presentation of the two clinical entities often leads to an unnecessary appendectomy.
182 consecutive FMF patients were retrospectively reviewed for this study. Clinical and genetic data was compared between those who had undergone an appendectomy (n=71) and those who had not (n=111).
The frequency of appendectomy found in FMF was far above the reported rate in the general population (40% vs. 12-25%). The rate of non-inflamed appendectomies was extremely high (80% vs. 20%) and remained constant over time. Tertiary hospitals and improved therapeutic and diagnostic measures that have evolved over the years did not reduce misdiagnosis of acute appendicitis in FMF. Severe phenotype and homozygosity for M694V were identified as risk factors for appendectomy in FMF. A change from the regular diffuse involvement to right lower quadrant abdominal pain was found to be the best predictor of inflamed appendix in FMF patients undergoing appendectomy for suspected acute appendicitis.
Reliance on clinical parameters should improve diagnostic accuracy of acute appendicitis in the FMF patient population. |
format | Article |
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182 consecutive FMF patients were retrospectively reviewed for this study. Clinical and genetic data was compared between those who had undergone an appendectomy (n=71) and those who had not (n=111).
The frequency of appendectomy found in FMF was far above the reported rate in the general population (40% vs. 12-25%). The rate of non-inflamed appendectomies was extremely high (80% vs. 20%) and remained constant over time. Tertiary hospitals and improved therapeutic and diagnostic measures that have evolved over the years did not reduce misdiagnosis of acute appendicitis in FMF. Severe phenotype and homozygosity for M694V were identified as risk factors for appendectomy in FMF. A change from the regular diffuse involvement to right lower quadrant abdominal pain was found to be the best predictor of inflamed appendix in FMF patients undergoing appendectomy for suspected acute appendicitis.
Reliance on clinical parameters should improve diagnostic accuracy of acute appendicitis in the FMF patient population.</description><identifier>ISSN: 0392-856X</identifier><identifier>EISSN: 1593-098X</identifier><identifier>PMID: 18799086</identifier><language>eng</language><publisher>Pisa: Clinical and Experimental Rheumatology</publisher><subject>Abdominal Pain - etiology ; Abdominal Pain - surgery ; Adult ; Appendectomy - adverse effects ; Appendicitis - diagnosis ; Appendicitis - pathology ; Appendicitis - surgery ; Biological and medical sciences ; Case-Control Studies ; Cytoskeletal Proteins - genetics ; Diagnostic Errors ; Diseases of the osteoarticular system ; Familial Mediterranean Fever - complications ; Familial Mediterranean Fever - genetics ; Familial Mediterranean Fever - pathology ; Female ; Humans ; Inflammatory joint diseases ; Male ; Malformations and congenital and or hereditary diseases involving bones. Joint deformations ; Medical sciences ; Middle Aged ; Mutation ; Pyrin ; Retrospective Studies ; Stomach, duodenum, intestine, rectum, anus ; Surgery (general aspects). Transplantations, organ and tissue grafts. Graft diseases ; Surgery of the digestive system ; Unnecessary Procedures ; Young Adult</subject><ispartof>Clinical and experimental rheumatology, 2008-07, Vol.26 (4), p.568-573</ispartof><rights>2008 INIST-CNRS</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,780,784</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=20687706$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/18799086$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>LIDAR, M</creatorcontrib><creatorcontrib>DORON, A</creatorcontrib><creatorcontrib>KEDEM, R</creatorcontrib><creatorcontrib>YOSEPOVICH, A</creatorcontrib><creatorcontrib>LANGEVITZ, P</creatorcontrib><creatorcontrib>LIVNEH, A</creatorcontrib><title>Appendectomy in familial Mediterranean fever : clinical, genetic and pathological findings</title><title>Clinical and experimental rheumatology</title><addtitle>Clin Exp Rheumatol</addtitle><description>Abdominal attacks of familial Mediterranean fever (FMF) may simulate acute appendicitis and bring about considerable uncertainty. The similar presentation of the two clinical entities often leads to an unnecessary appendectomy.
182 consecutive FMF patients were retrospectively reviewed for this study. Clinical and genetic data was compared between those who had undergone an appendectomy (n=71) and those who had not (n=111).
The frequency of appendectomy found in FMF was far above the reported rate in the general population (40% vs. 12-25%). The rate of non-inflamed appendectomies was extremely high (80% vs. 20%) and remained constant over time. Tertiary hospitals and improved therapeutic and diagnostic measures that have evolved over the years did not reduce misdiagnosis of acute appendicitis in FMF. Severe phenotype and homozygosity for M694V were identified as risk factors for appendectomy in FMF. A change from the regular diffuse involvement to right lower quadrant abdominal pain was found to be the best predictor of inflamed appendix in FMF patients undergoing appendectomy for suspected acute appendicitis.
Reliance on clinical parameters should improve diagnostic accuracy of acute appendicitis in the FMF patient population.</description><subject>Abdominal Pain - etiology</subject><subject>Abdominal Pain - surgery</subject><subject>Adult</subject><subject>Appendectomy - adverse effects</subject><subject>Appendicitis - diagnosis</subject><subject>Appendicitis - pathology</subject><subject>Appendicitis - surgery</subject><subject>Biological and medical sciences</subject><subject>Case-Control Studies</subject><subject>Cytoskeletal Proteins - genetics</subject><subject>Diagnostic Errors</subject><subject>Diseases of the osteoarticular system</subject><subject>Familial Mediterranean Fever - complications</subject><subject>Familial Mediterranean Fever - genetics</subject><subject>Familial Mediterranean Fever - pathology</subject><subject>Female</subject><subject>Humans</subject><subject>Inflammatory joint diseases</subject><subject>Male</subject><subject>Malformations and congenital and or hereditary diseases involving bones. Joint deformations</subject><subject>Medical sciences</subject><subject>Middle Aged</subject><subject>Mutation</subject><subject>Pyrin</subject><subject>Retrospective Studies</subject><subject>Stomach, duodenum, intestine, rectum, anus</subject><subject>Surgery (general aspects). Transplantations, organ and tissue grafts. Graft diseases</subject><subject>Surgery of the digestive system</subject><subject>Unnecessary Procedures</subject><subject>Young Adult</subject><issn>0392-856X</issn><issn>1593-098X</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2008</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNo90E1Lw0AQBuBFFFurf0H2oicDm2z2y1spfkHFi0LxUia7s3Ul2cRsKvTfm2L1NDDzMMw7R2SaC8MzZvTqmEwZN0WmhVxNyFlKn4wVUkh1Sia5VsYwLafkfd51GB3aoW12NETqoQl1gJo-owsD9j1EhLGN39jTW2rrEIOF-oZuMOIQLIXoaAfDR1u3m_2E-hBdiJt0Tk481AkvDnVG3u7vXheP2fLl4WkxX2Zdwc2QVVZxLkQJaF2Z-4oXBorSOfB5qbVTvvICmC5yjyWz4CsAjqoUklcqr4TgM3L9u7fr268tpmHdhGSxrsfL221aSyO0FGwPLw9wWzXo1l0fGuh3679vjODqACCNSfyY3Yb07womtVJM8h_Idmse</recordid><startdate>20080701</startdate><enddate>20080701</enddate><creator>LIDAR, M</creator><creator>DORON, A</creator><creator>KEDEM, R</creator><creator>YOSEPOVICH, A</creator><creator>LANGEVITZ, P</creator><creator>LIVNEH, A</creator><general>Clinical and Experimental Rheumatology</general><scope>IQODW</scope><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>7X8</scope></search><sort><creationdate>20080701</creationdate><title>Appendectomy in familial Mediterranean fever : clinical, genetic and pathological findings</title><author>LIDAR, M ; DORON, A ; KEDEM, R ; YOSEPOVICH, A ; LANGEVITZ, P ; LIVNEH, A</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-p239t-bc733554aecd41fb329a24ddaf1488d7fbf5a0821fe40cafbaa3e74563b71b553</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2008</creationdate><topic>Abdominal Pain - etiology</topic><topic>Abdominal Pain - surgery</topic><topic>Adult</topic><topic>Appendectomy - adverse effects</topic><topic>Appendicitis - diagnosis</topic><topic>Appendicitis - pathology</topic><topic>Appendicitis - surgery</topic><topic>Biological and medical sciences</topic><topic>Case-Control Studies</topic><topic>Cytoskeletal Proteins - genetics</topic><topic>Diagnostic Errors</topic><topic>Diseases of the osteoarticular system</topic><topic>Familial Mediterranean Fever - complications</topic><topic>Familial Mediterranean Fever - genetics</topic><topic>Familial Mediterranean Fever - pathology</topic><topic>Female</topic><topic>Humans</topic><topic>Inflammatory joint diseases</topic><topic>Male</topic><topic>Malformations and congenital and or hereditary diseases involving bones. Joint deformations</topic><topic>Medical sciences</topic><topic>Middle Aged</topic><topic>Mutation</topic><topic>Pyrin</topic><topic>Retrospective Studies</topic><topic>Stomach, duodenum, intestine, rectum, anus</topic><topic>Surgery (general aspects). Transplantations, organ and tissue grafts. Graft diseases</topic><topic>Surgery of the digestive system</topic><topic>Unnecessary Procedures</topic><topic>Young Adult</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>LIDAR, M</creatorcontrib><creatorcontrib>DORON, A</creatorcontrib><creatorcontrib>KEDEM, R</creatorcontrib><creatorcontrib>YOSEPOVICH, A</creatorcontrib><creatorcontrib>LANGEVITZ, P</creatorcontrib><creatorcontrib>LIVNEH, 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>MEDLINE - Academic</collection><jtitle>Clinical and experimental rheumatology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>LIDAR, M</au><au>DORON, A</au><au>KEDEM, R</au><au>YOSEPOVICH, A</au><au>LANGEVITZ, P</au><au>LIVNEH, A</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Appendectomy in familial Mediterranean fever : clinical, genetic and pathological findings</atitle><jtitle>Clinical and experimental rheumatology</jtitle><addtitle>Clin Exp Rheumatol</addtitle><date>2008-07-01</date><risdate>2008</risdate><volume>26</volume><issue>4</issue><spage>568</spage><epage>573</epage><pages>568-573</pages><issn>0392-856X</issn><eissn>1593-098X</eissn><abstract>Abdominal attacks of familial Mediterranean fever (FMF) may simulate acute appendicitis and bring about considerable uncertainty. The similar presentation of the two clinical entities often leads to an unnecessary appendectomy.
182 consecutive FMF patients were retrospectively reviewed for this study. Clinical and genetic data was compared between those who had undergone an appendectomy (n=71) and those who had not (n=111).
The frequency of appendectomy found in FMF was far above the reported rate in the general population (40% vs. 12-25%). The rate of non-inflamed appendectomies was extremely high (80% vs. 20%) and remained constant over time. Tertiary hospitals and improved therapeutic and diagnostic measures that have evolved over the years did not reduce misdiagnosis of acute appendicitis in FMF. Severe phenotype and homozygosity for M694V were identified as risk factors for appendectomy in FMF. A change from the regular diffuse involvement to right lower quadrant abdominal pain was found to be the best predictor of inflamed appendix in FMF patients undergoing appendectomy for suspected acute appendicitis.
Reliance on clinical parameters should improve diagnostic accuracy of acute appendicitis in the FMF patient population.</abstract><cop>Pisa</cop><pub>Clinical and Experimental Rheumatology</pub><pmid>18799086</pmid><tpages>6</tpages></addata></record> |
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subjects | Abdominal Pain - etiology Abdominal Pain - surgery Adult Appendectomy - adverse effects Appendicitis - diagnosis Appendicitis - pathology Appendicitis - surgery Biological and medical sciences Case-Control Studies Cytoskeletal Proteins - genetics Diagnostic Errors Diseases of the osteoarticular system Familial Mediterranean Fever - complications Familial Mediterranean Fever - genetics Familial Mediterranean Fever - pathology Female Humans Inflammatory joint diseases Male Malformations and congenital and or hereditary diseases involving bones. Joint deformations Medical sciences Middle Aged Mutation Pyrin Retrospective Studies Stomach, duodenum, intestine, rectum, anus Surgery (general aspects). Transplantations, organ and tissue grafts. Graft diseases Surgery of the digestive system Unnecessary Procedures Young Adult |
title | Appendectomy in familial Mediterranean fever : clinical, genetic and pathological findings |
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