jsmc-10188

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. 

b 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

DOI Link: https://doi.org/10.17656/jsmc.10188 

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.

References 

1. Özen S, Batu ED, Demir S. Familial mediterranean fever: recent developments in pathogenesis and new recommendations for management. Frontiers in immunology. 2017 Mar 23;8:253. 

2. Turkish FM. Familial mediterranean fever (FMF) in Turkey. Medicine. 2005 Jan;84(1):1-1. 

3. Kucuk A, Gezer IA, Ucar R, Karahan AY. Familial mediterranean fever. Acta Medica (Hradec Kralove). 2014;57(3):97-104. 

4. Sarı İ, Birlik M, Kasifoğlu T. Familial Mediterranean fever: an updated review. European journal of rheumatology. 2014 Mar;1(1):21. 

5. Settin A, El-Baz R, Rasool MA, El-Khalegy H, El-Sayed O, El-Bendary M, Al-Nagar A. Clinical and molecular diagnosis of Familial Mediterranean Fever in Egyptian children. Journal of Gastrointestinal and Liver Diseases. 2007 Jun 1;16(2):141.

6. Khan S, Karjoo M, Karjoo S. Familial Mediterranean Fever: Review of Literature and Report of Two Cases. International Journal of Pediatrics. 2017;5(12):6469-84. 

7. Rigante D, La Torraca I, Ansuini V, Compagnone A, Sallì A, Stabile A. The multi-face expression of familial Mediterranean fever in the child. European Review for Medical and Pharmacological Sciences. 2006;10(4):163. 

8. Shohat M, Halpern GJ. Familial Mediterranean fever—a review. Genetics in Medicine. 2011 Jun;13(6):487. 

9. Yalçınkaya F, Özen S, Özçakar ZB, Aktay N, Çakar N, Düzova A, Kasapçopur Ö, Elhan AH, Doğanay B, Ekim M, Kara N. A new set of criteria for the diagnosis of familial Mediterranean fever in childhood. Rheumatology. 2009 Feb 4;48(4):395-8. 

10. Bashardoust B. Familial Mediterranean fever; diagnosis, treatment, and complications. Journal of nephropharmacology. 2015;4(1):5.

11. Ben-Chetrit E, Touitou I. Familial Mediterranean fever in the world. Arthritis Care & Research. 2009 Oct 15;61(10):1447-53. 

12. Soriano A, Manna R. Familial Mediterranean fever: new phenotypes. Autoimmunity reviews. 2012 Nov 1;12(1):31-7. 

13. Jarjour RA. Familial Mediterranean fever in Syrian patients: MEFV gene mutations and genotype–phenotype correlation. Molecular biology reports. 2010 Jan 1;37(1):1-5. 

14. Hentgen V, Grateau G, Stankovic-Stojanovic K, Amselem S, Jéru I. Familial Mediterranean fever in heterozygotes: are we able to accurately diagnose the disease in very young children?. Arthritis & Rheumatology. 2013 Jun 1;65(6):1654-62. 

15. Manna R, Cerquaglia CL, Curigliano V, Fonnesu C, Giovinale M, Verrecchia EL, Montalto MA, De Socio G, Soriano AL, La Regina MI, Gasbarrini G. Clinical features of familial Mediterranean fever: an Italian overview. Eur Rev Med Pharmacol Sci. 2009 Mar 1;13(Suppl 1):51-3. 

16. Salehzadeh F. Familial Mediterranean fever in Iran: a report from FMF registration center. International journal of rheumatology. 2015;2015. 

17. Rawashdeh MO, Majeed HA. Familial Mediterranean fever in Arab children: the high prevalence and gene frequency. European journal of pediatrics. 1996 Jul 1;155(7):540-4. 

18. Al-Wahadneh AM, Dahabreh MM. Familial Mediterranean fever in children: a single centre experience in Jordan. Eastern Mediterranean Health Journal. 2006 Nov 1;12(6):818. 

19. Mneimneh S, Naous A, Naja Z, Naja Z, Naja AS, Megarbane A, Rajab M. Familial Mediterranean Fever: Clinical and Genetic Characteristics among Lebanese Pediatric Population. Open Journal of Rheumatology and Autoimmune Diseases. 2016 Jul 9;6(03):63. 

Full Text

 © The Authors, published by University of Sulaimani, College of Medicine

This work is licensed under a Creative Commons Attribution 4.0 International License.