RESUMEN
Familial Mediterranean fever [FMF] is an autosomal recessive disorder characterized by sporadic, paroxysmal attacks of fever and serosal inflammation. Although the disease usually begins before the age of 20 years, we aimed to evaluate the demography, clinical features and treatment outcome of familial Mediterranean fever in Iranian adult patients above 20 years old. In this cross-sectional study, adult patients [first attack at the age of > 20 years] with a diagnosis of FMF who referred to the gastroenterology and rheumatology clinics of Ardebil University of Medical Science [situated in north west of Iran] over the period of 2004-2009 were enrolled. FMF diagnosis was based on clinical criteria. Forty four FMF patients [30 male and 14 female] with the mean [ +/- Standard Deviation [SD]] age of first attack of 29 +/- 7.8 years were enrolled. Abdominal pain [95.5%] and fever [91%] were the most common clinical findings. All of the patients had satisfactorily responded to therapy. Response was complete in 76.7% and partial in 23.3% of the patients. There was no clinical or laboratory evidence of amloidosis at the time of diagnosis or during follow-up. Our findings demonstrated that adult-onset FMF in Iran has different characteristics [more common in males, lesser prevalence of arthritis and erysipelas-like erythema, less delay in diagnosis] and treatment outcome [favorable response even to low-dose colchicines] in comparison with the previous data on early onset patients
RESUMEN
There is a relationship between specific genotypes of Helicobacter pylori cagA and vacA genes and the increased risk of peptic ulcer diseases and gastric cancer. These genes also possess strong patterns of geographical differentiation. The present study aims to determine the patterns of variation of the virulence genes in Iran and their association with clinical status. Sequence fragments for cagAand vacA were obtained from a total of 147 H. pylori isolates from diverse geographical and ethnic sources within Iran. We used phylogenetic methods to determine the patterns of allelic diversity, and the relationship between evolutionary lineages and clinical status. Phylogenetic analyses of Iranian cagA gene disclosed four lineages, whereas the vacA gene had two distinct lineages. The cagA lineage II showed extensive genetic diversity compared with lineage I. cagA lineages III and IV disclosed mixed ancestries with recombinant nucleotides that originated from lineages I and H Iranian strains with vac A lineage II genotype were significantly cagA+ [> 90%, p = 0.0] and correlated with a higher rate of peptic ulcers in infected individuals [p =0.003]. Most strains in the cagA lineage I showed a vacA lineage II genotype [p = 0.003] and significantly correlated with an increased risk of peptic ulcers in infected individuals [p = 0.022]. Strains with cagA lineage III genotype significantly correlated with gastritis [p = 0.0]. The increased level of allelic diversity in the virulence genes shows strong evolutionary dynamics, resulting in the emergence of new clonal genealogies of the cagA gene within Iran. Particular lineages of the Iranian cagA and vac A genes correlate with peptic ulcer diseases