Comparison of diagnostic criteria in children with family mediterranean fever

Introduction Familial Mediterranean Fever (FMF) is an autoinflammatory autosomal recessive disease characterised with recurrent attacks of fever and inflammation of serous membranes (peritonitis, pleuritis, pericarditis, arthritis). The diagnosis is made according to clinical findings and supported by genetic analysis. The most used diagnostic criteria is Tel Hashomer criteria. New FMF diagnostic criteria for pediatric patients was identified by Yalçınkaya et al. in 2009. But this criteria should be supported with further studies. In this study we planned to compare Yalçınkaya criteria and Tel Hashomer criteria in FMF patients. Material and Method 135 patients diagnosed as FMF were included to our study. 165 children admitted to our pediatric nephrology unit with FMF like symptoms, were reviewed as control group. Demografic features and laboratory findings were evaluated retrospectively. The 5 Yalçınkaya criterias were used to compare the Yalçınkaya and Tel Hashomer criteria. The relation between genetic results and these criterias was evaluated. Results Mean age of the patients was 14,02± 5,32 years (min-max.: 1,1-18). Mean age at the onset of the symptoms was 6,98±4,65 years (min.-max.: 0,6-17), mean age of diagnosis was detected as 8,76±4,58 years (min.-max.: 1,1-18). When ages at the onset of the complaints and mean age at diagnosis were evaluated, mean time of delay for diagnosis was detected as 1,8 years. Five new criteria consisting of recurrent abdominal pain, fever, arthritis, chest pain and family history of FMF were significantly higher in patients' group than controls' (p<0,05). It is found that Yalçınkaya criteria is as sensitive and spesific as Tel Hashomer criteria. The presence of at least 2 criteria of Yalçınkaya criteria was seen adequate to diagnose FMF. When we evaluated the relation between genetic features and Yalçınkaya criteria, the sensitivity and specificity of Yalçınkaya criteria for homozygous, compound heterozygous, heterozygous patients and patients with no mutations were similar. It is concluded that our study results supports Yalçınkaya criteria.