{"content":[{"text":"BODAKÇİ et al. / Turk J Med Sci","bbox":{"left":0.380709,"top":0.052109,"width":0.23858200000000002,"height":0.016381999999999994}},{"text":"and adynamic ileus are often present, a clinical emergency \n scenario quite similar to “acute surgical abdomen”. The most \n common cause of acute surgical abdomen in childhood is \n acute appendicitis with a peak incidence between 10–19 \n years. The lifetime risk of acute appendicitis is 7%–9%, \n which is far beyond the prevalence of FMF [7] which has a \n prevalence of 1/400–1/1000 in endemic countries [8]. \n In clinical practice, appendicitis, ileus/subileus, \n cholecystitis, and renal colic may be perceived as causes \n of abdominal pain before a reliable diagnosis of FMF \n has been made, which may lead to misdiagnosis and \n inappropriate treatments [9]. Heterogeneity of FMF \n attacks, in terms of severity and duration, and their erratic \n recurrence and remission, together with the rare incidence \n and low awareness, explain diagnostic delays of these \n disorders and the high rate of unnecessary laparotomies \n and appendectomies [9]. Therefore, routine preoperative \n screening for a personal or family history of symptoms \n compatible with FMF, should undoubtedly reduce the \n frequency of unwarranted surgical interventions. \n In patients with FMF, appendectomy is usually \n performed in childhood prior to the diagnosis, however \n even confirmation of FMF diagnosis, some patients \n underwent laparotomy due to difficulties in distinguishing \n FMF peritonitis from acute surgical abdomen causes. In \n previous reports abdominal surgery history rate is as high \n as 20% in FMF patients [10]. Our observation is that, \n patients with history of appendectomy or laparotomy \n experience more attacks than those who did not have these \n operations which may give a clue about the clinical course \n of FMF in adulthood. Herein, we aimed to investigate \n clinical features and disease course of FMF patients with \n history of appendectomy in a large cohort.","bbox":{"left":0.064103,"top":0.088472,"width":0.417999,"height":0.514139}},{"text":"assessed with International Severity Scoring System for \n FMF (ISSF) and Autoinflammatory Disease Damage Index \n (ADDI), respectively [12,13]. Having less than one attack \n per 6-months-period is defined as complete response to \n colchicine. Colchicine resistance (crFMF) was defined as \n experiencing more than one attack per month despite the \n maximal tolerated dose of colchicine [14]. \n In ADDI, damage is defined as “persistent or \n irreversible change in structure or function, that is present \n for more than 6 months” [12]. ADDI contains 18 items, \n and these items are categorized by organ systems as \n follows: reproductive, renal/amyloidosis, developmental, \n serosal, neurological, ears, ocular, and musculoskeletal. \n The renal/amyloidosis and neurological damage categories \n were assigned to have the highest number of points \n while serosal damage got the lowest. This index provides \n a universal instrument to measure damage by chronic \n inflammation in FMF [15]. \n ISSF was developed to measure disease severity in \n children and adult FMF patients for research purposes and \n clinical practice. It consists of nine clinical and laboratory \n variables: chronic sequela, organ dysfunction, organ \n failure, attack frequency, increased acute-phase reactants, \n involvement of more than two sites during a single episode, \n more than two different types of attack during the course \n of the disease, duration of attacks, and exertional leg pain \n [13]. The maximum score is 10 and the degree of severity \n was determined as mild (≤2), intermediate (3–5) or severe \n disease (≥6) [12]. \n For statistical analyzes, patients were divided into two \n groups according to their history of appendectomy as \n Appendectomy Performed (AP) or Appendix Intact (AI). \n Differences between attack features, disease severities, \n damage accrual and treatment responses were compared. \n Categorical variables were given as numbers and \n percentages, and continuous variables were presented with \n their mean ± standard deviation (SD) or median (25%– \n 75% interquartile ranges) for descriptive analysis. The \n conformity of continuous variables to normal distribution \n was evaluated by using visual (histogram and probability \n graphs) and analytical methods (Kolmogorov–Smirnov/ \n Shapiro–Wilk tests). If variables were normally distributed \n Student-t-test was used, if not Mann–Whitney U test was \n used for comparison of data sets. The chi-square test was \n used for the comparison of categorical variables. p < 0.05 \n was considered as statistically significant in all analyzes.","bbox":{"left":0.517949,"top":0.088472,"width":0.41799200000000003,"height":0.716353}},{"text":"2. Materials and methods \n In this study, we enrolled 971 adult patients with FMF who \n followed at the outpatient rheumatology clinics of three \n university hospitals: Gazi University, Hacettepe University, \n and Eskişehir Osmangazi University Hospitals which \n admit patients from all over the country. The study was \n conducted between January 2018 and December 2018. The \n diagnosis of FMF was made according to the Tel Hashomer \n criteria [11]. This study was approved by Gazi University \n ethical committee (approval no. 2017-622), conducted in \n accordance with the 1975 Helsinki Declaration. Informed \n consent was obtained from all participants before the \n inclusion of study. \n Demographic data, FMF disease characteristics, \n co-morbid conditions, past medical history including \n surgeries, medication history, colchicine doses, and \n disease complications were meticulously questioned \n and laboratory features were recruited from patient \n files. Disease severity and FMF associated damage were","bbox":{"left":0.064103,"top":0.615966,"width":0.417994,"height":0.297744}},{"text":"3. Results \n The study comprised of 971 FMF patients (597 female, \n mean age 35.3 ± 12.1 years). Appendectomy history was \n evident in 240 (24.7%) subjects. AP group is a bit older \n and had an earlier disease onset (Table 1). In AP group \n mutation negative patients were less and those with two","bbox":{"left":0.517949,"top":0.81818,"width":0.417994,"height":0.09553}},{"text":"2","bbox":{"left":0.064103,"top":0.932515,"width":0.009552000000000005,"height":0.018020000000000036}}],"images_bbox":[],"images_bbox_not_text_overlap":[]}