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
Hauptverfasser: LIDAR, M, DORON, A, KEDEM, R, YOSEPOVICH, A, LANGEVITZ, P, LIVNEH, A
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container_end_page 573
container_issue 4
container_start_page 568
container_title Clinical and experimental rheumatology
container_volume 26
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.
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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. 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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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