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    Effect of Genotype on Plasma and Urinary Adrenomedullin Levels in Children with Familial Mediterranean Fever
    (Ortadogu Ad Pres & Publ Co, 2012) Kalman, Suleyman; Sakallioglu, Onur; Dogru, Ilker; Gul, Davut; Gok, Faysal
    Objective: Familial Medeitarrenean Fever (FMF) is an autosomal recessive disease characterized with fever and serositis episodes. Renal amyloidosis which is the most important complication of the disease also determines prognosis. Although it is still conflicting, M694V homozygosity is a risk factor for amyloidosis. Adrenomedullin (ADM) is a potent vasodilator and a cytoprotective peptide. It has been proposed that it may have a role in pathogenesis of inflammatory diseases like Behcet's disease, Henoch-Schonlein purpura and its plasma levels are high in pediatric patients with FMF. The aim of this study is to compare plasma and urinary ADM levels of FMF patients who exhibit M694V homozygosity and the patients who have other genotypes. Material and Methods: A total of 37 patients with a mean age of 9.2 +/- 4.7 years were included in the study. Sixteen patients had M694V homozygous mutation (Group I), 10 patients had M694V compound heterozygous (Group II), and 11 patients had other/other mutations (Group III). No patients had an FMF attack at the time of evaluation. Results: Plasma ADM levels in Group 1(26.83 +/- 5.71 pmol/mL) were higher than Group II and Group III (23.25 +/- 3.28 pmol/mL, 22.80 +/- 3.90 pmol/mL; p=0.032, p=0.026, respectively). There were no differences between Group II and Group III (p=0.228). A difference could not be found between three groups in terms of urinary ADM levels (Group I: 18.58 +/- 2.37, Group II: 17.84 +/- 3.36, Group III: 17.96 +/- 3.46 pmol/mg creatinine). White blood cell count, erythrocyte sedimentation rate, C-reactive protein, fibrinogen levels were also similar among the groups. Conclusion: It was concluded that further studies including more cases were needed to determine the role of adrenomedullin in immunoinflammatory process of FMF and to prove coexistence of subclinical inflammation with M694V homozygot mutation and high ADM levels.