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OBJECTIVES: The complement system participates in the defense of the body against viral infections through various mechanisms. In the present study conducted on children having Crimean-Congo Hemorrhagic Fever (CCHF), the aim was to evaluate whether the complement system had a role in the pathogenesis of the disease. PATIENTS AND METHODS: Forty-one patients diagnosed with CCHF and 32 healthy controls were included in the study. Serum complement component 3 (C3), 4 (C4) and complement product Bb (Bb) levels were measured in both groups. RESULTS: Compared to the control group, serum C3 levels were lower and Bb levels were higher in CCHF patients (p < 0.01). Moreover, in the patient group, C3 levels were positively correlated with WBC and PLT counts, and Bb levels were positively correlated with AST, ALT and LDH activities. In the patient group, serum Bb levels were negatively correlated with WBC and PLT counts. CONCLUSIONS: The results of the present study suggest that increased activity of the alternative pathway of the complement system in children with CCHF may have a role in the pathogenesis of the disease. [ABSTRACT FROM AUTHOR]

BACKGROUND: Familial Mediterranean Fever (FMF) is an autosomal recessive disease characterized by short lived, febrile serosae inflammatory attacks. FMF has various effects in multiple systems and organs. AIM: In the present study, our aim was to evaluate adrenal steroidogenesis in female FMF patients. PATIENTS AND METHODS: There were 71 women in the study including 41 women with FMF and 30 women as healthy control group (HC group). Of 41 FMF patients, twenty were evaluated in attack period (AP-FMF group) whereas 21 of them were evaluated in attack-free period (AFP-FMF group). In all subjects; serum free testosterone, 17-OHP levels as hormones, IL-1 beta, TNF-alpha, IL-6, IL-18 as proinflammatory cytokines, CRP, fibrinogen, white blood cell (WBC) counts, and erythrocyte sedimentation rate (ESR) as acute phase reactants were measured in samples of venous blood taken in the morning before breakfast. RESULTS: Serum 17-OHP levels in AP-FMF group and AFP-FMF group were higher than in HC group (p < 0.001). A positive correlation was detected between serum levels of 17-OHP and IL-1 beta in FMF patients (p = 0.006; r = 0.486). There was no difference between FMF patients and HC group in terms of free testosterone levels (p > 0.05). CONCLUSIONS: Our results showed an increase in 17-OHP levels in FMF patients. These results may indicate that, regardless to the attack period adrenal steroidogenesis could be affected negatively in FMF patients. [ABSTRACT FROM AUTHOR]

BACKGROUND: The major renal involvement in Familial Mediterranean Fever (FMF) is the occurrence of amyloidosis that primarily affects the kidneys manifested by proteinuria and ending in death from renal failure. AIM: This study aims to investigate whether serum cystatin (cys-C) levels could be used as an early marker of renal involvement in FMF patients. PATIENTS AND METHODS: Forty-six patients with FMF during the attack period (AP), and 41 patients with FMF during attack-free periods (AFP), and 11 patients with FMF associated amyloidosis, and 38 healthy controls were enrolled in the study. We determined cys-C levels in the serum of FMF patients and healthy controls. RESULTS: Serum cys-C levels were significantly increased in patients with FMF and secondary amyloidosis, and serum cys-C is a more accurate and efficient marker for detecting renal involvement than estimated glomerular filtration rate (e-GFR) in patients with FMF. CONCLUSIONS: We propose a cutoff level of the serum cys-C of 876.5 pg/mL for screening renal involvement in patients with FMF, and amyloidosis should be strongly suspected when the serum cys-C reaches 1565.5 pg/mL. [ABSTRACT FROM AUTHOR]