FAMILIAL MEDITERRANEAN FEVER AMONG KURDISH CHILDREN; A SINGLE CENTER STUDY 

Bakhtyar Qadr H. Khurshid a, Jamal Ahmed Rashid b, Khalid Hama Salih b and Dlnya Assad Muhamad c



a KBMS Trainee, Sulaimani Pediatric Teaching Hospital, Sulaimani, Kurdistan Region, Iraq. 
Department of Pediatircs, College of Medicine, University of Sulaimani. 
c College of Science, Department of Biology, University of Sulaimani, Sulaimani


Submitted: 15/7/2018; Accepted: 27/2/2019; Published 21/3/2019

ABSTRACT


Background 

Familial Mediterranean Fever is recessively inherited auto inflammatory disease. It shows an obvious ethnic distribution, which is mainly prevalence among Eastern Mediterranean people. 


Objectives 

The aim of the present study is an accurate diagnosis of FMF in Kurdish children by detection of genetic mutations in addition to clinical features. 


Patients and Methods

In the current study, clinical data for 20 patients, aged 18 years or younger, affected by FMF was analyzed and discussed. Those diagnosed as FMF, according to Tel-Hashomer criteria and sent for genetic confirmation were included in the study. The cases were collected from different hospital and Pediatric clinics in Sulaimani, Kurdistan Region, Iraq between December 2017 to May 2018.


Results

Among 20 patients who were diagnosed with FMF, (60%, n=12) of them were male and (40%, n=8) were female, leading to a male to female ratio of 1.5:1. The mean age of onset of symptoms was 3 ± 2.6 years, and the mean delay in diagnosis was 3.17 ± 2.02 years. The main clinical features were as follows: fever which counted for (95%), followed by abdominal pain (75%), joint pain (45%), and skin rash (15%). Family history of FMF was presented in 10% of the cases. Regarding Genetic analysis, the most common type of mutation was found to be M694V (36.8%), followed by M680I (23.7%), M694I (18.4%), V726A (15.8%), E148Q (2.6%) and A744S (2.6%). Only 5% of the cases had undergone an appendectomy before diagnosis. Regarding using of colchicine, it has been noticed that 5.3% of the cases were non-responder. None of the patients was found to have renal amyloidosis. 


Conclusion

Clinical criteria with genetic study are the most preferred methods in diagnosis of FMF cases. Genotype results were found to be similar to the FMF patients of ethnic Arabs and, to a degree, to those of Turkish patients. 



KEYWORDS

Familial Mediterranean Fever, Genetic analysis, Children, Kurdish.