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Background: Familial Mediterranean fever (FMF) is an autosomal recessive inherited disease characterized by recurrent attacks of fever and pain secondary to polyserositis, mainly of the abdomen and joints. The symptoms start abruptly, occur before the age of 20 in 90% of the patients and generally decrease spontaneously within 1-4 days. Because of similar clinical and laboratory settings, it is difficult to differentiate an attack of FMF from acute appendicitis and other acute abdominal conditions. Therefore preoperative evaluation is very important to prevent unnecessary surgeries in FMF patients with abdominal attacks. Objective: This study aims to describe the situation regarding incorrectly performed laparatomies for acute abdomen among FMF patients with abdominal attacks in Yerevan, Armenia. It further aims to assess potential contributing factors to the incorrectly performed laparatomies in these patients. Methods: Cross-sectional conventional content analysis approach was employed for the proposed study to gain deep insights into the study questions. Qualitative study design was used to conduct in-depth and focus group interviews with the health care providers (surgeons, physicians who deal with the FMF patients in general and rheumatologists and FMF patients who had surgeries), to understand their experience in this field. In-depth interviews with FMF patients who had undergone surgery (laparotomies/laparoscopies) helped us identify the scope of the problem and reveal patients’ experience within the health care system. Results: Two focus groups with surgeons and thirteen in-depth interviews with six health care providers (experts) and seven FMF patients who had undergone surgeries were completed. Three prospective focus group members refused to participate due to lack of time. Five surgeons participated in both focus group discussions. All participants were males. Work experience varied between 8 and more than 30 years. Age varied between 27 to 63 years old. Among physicians who deal with FMF patients in their practice all experts were females except for two. Work experience varied from 8 to 35 years and the age range was from 35 to 67 years old. Among FMF patients, who underwent surgeries before their disease was diagnosed, 4 were females and 3 were males. The age range varied between 23 and 62 years. Eight categories were revealed from in-depths interviews and focus group discussions that describe the proposed research questions. The main categories are (1) Causes of the acute abdomen (2) Acute abdominal pain in FMF patients (3) First experience of FMF patients in medical settings and potential contributing factors to misdiagnosis (4) Diagnosis of FMF abdominal pains vs. acute abdomen of other causes (5) Management of acute abdominal pains/FMF abdominal attacks (6) Changes in practice (7) Patients’ attitude towards unnecessary surgeries (8) Participants’ further suggestions. Conclusion: The study findings revealed that situation in Armenia regarding FMF patients who had undergone surgeries due to misdiagnosis is more satisfactory than we expected. It was explained that as Armenia is considered an endemic zone for FMF so the doctors, especially in the Yerevan, are more informed about the disease and they very often can differentiate FMF abdominal attacks from other causes. However, there is still misdiagnosis of FMF abdominal attacks. So, we developed an algorithm for differential diagnosis of acute appendicitis and FMF abdominal attacks which will help surgeons avoid misdiagnosis and unnecessary surgeries in FMF patients and also not miss acute appendicitis in these patients. |
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