Familial Mediterranean fever

Familial Mediterranean fever (FMF) is the most frequent hereditary inflammatory disease characterized by self-limited recurrent attacks of fever and serositis. It is transmitted in an autosomal recessive pattern and affects certain ethnic groups mainly Jews, Turks, Arabs, and Armenians. FMF is caused by mutations in MEFV gene, which encodes pyrin. This protein is expressed mainly in myeloid/monocytic cells and modulates IL-1 beta processing, NF-kappa B activation, and apoptosis. A mutated pyrin probably results in uncontrolled inflammation. The most devastating complication of FMF is amyloidosis, leading to chronic renal failure. M694V homozygocity, male gender and the alpha/alpha genotype of serum amyloid A1 gene are the currently established risk factors for development of amyloidosis. Daily colchicine is the mainstay of the therapy for the disease, resulting in complete remission or marked reduction in the frequency and duration of attacks in most patients. It is also effective in preventing and arresting renal amyloidosis

Effects of rhG-CSF on neutrophil functions and survival in sepsis induced diabetic rats

Diabetic patients are more prone to infection and evidence for an immunologic defect superimposed upon the metabolic abnormalities of diabetes is convincing. Neutrophils play a critical role in the host defense mechanism against various bacterial infections, and it is suggested that impaired neutrophil functions cause susceptibility to infections in diabetic patients. To explore the possibility that Granulocyte colony-stimulating factor (G-CSF) may be useful to prevent the morbidity and mortality caused by infections in diabetics. We studied the effect of G-CSF against septicemia in diabetic rats

The relations between attacks and menstrual periods and pregnancies of familial Mediterranean fever patients

Familial Mediterranean fever (FMF) is an autosomal recessive disease characterized by short lived, febrile serosal inflammatory attacks. Although majority of patients have random pattern of attacks, some reports described precipitating factors. There are also contradictory reports relating FMF attacks with menstruation and the natural course of their pregnancies. Seventy-two female patients with FMF with a mean age of 34.9 +/- 12.4 were interviewed. A standardized questionnaire was used inquiring any associations of FMF attacks of the patients with their menstruations and pregnancies. Thirty-eight patients (53%) reported that their attacks frequently coincided with their menstrual cycles and 17 patients noticed pleuritic chest pain in addition to their abdominal attacks. One patient experienced only febrile pleural attacks during her menstrual cycles. Unlike dysmenorrhoea, none of these patients' attacks responded to non-steroidal anti-inflammatory drugs. All of the patients could correctly differentiate their FMF attacks from dysmenorrhoea. Forty patients could give detailed information about the frequency and severity of their FMF attacks during 73 pregnancies: 25 patients (62.5%) experienced complete symptomatic remissions; the attacks were aggravated (7 patients), ameliorated (6 patients) or did not change (2 patients) in the rest of the pregnancies. Four patients continued to use colchicine during their pregnancies and delivered healthy babies. One patient gave birth to a child with Down's syndrome although she was not on colchicine therapy. Although FMF attacks and discomforts of menstrual cycles do overlap frequently, patients can easily differentiated them. Patients can be reasonably assured that the period of pregnancy will be comfortable but abstaining from colchicine should not be recommended. Gynecologists must be aware of FMF in the differential diagnosis of dysmenorrhoea or endometriosis

A case with recurrent calf pain and swelling: recurrent spontaneous calf haematoma.

The differential diagnosis of unilateral calf swelling and pain includes deep venous thrombosis, haematoma, ruptured popliteal cyst, infection, lymph oedema, and sarcoma. It is important to distinguish between these entities, as the treatment of each is different. We present a case of recurrent calf swelling due to spontaneous calf haematoma diagnosed with ultrasound and MRI and subsequently treated conservatively

Seroprevalence of Borrelia burgdorferi in patients with Behçet's disease.

Objective. Turkey is one of the countries where Behcet's disease is most prevalent. Although its pathogenesis is not defined clearly, infectious agents are thought to play a role in the etiology. In one study of a group of uveitis patients, including those with Behcet's disease, increased seropositivity to B. burgdorferi was reported by enzyme-linked immunosorbent assay (ELISA). The seroprevalence of B. burgdorferi has been found to be as high as 36% in some rural areas of Turkey, although Lyme disease caused by B. burgdorferi is quite rare. In this study, we investigated the seroreactivity to B. burgdorferi antigens in patients with Behcet's disease and compared it with that of healthy and disease controls. Materials and methods. This study was conducted in Izmir in western Turkey. B. burgdorferi immunoglobulin (Ig)M and IgG antibodies were tested by ELISA in the sera of patients with Behcet's disease (n=30), rheumatoid arthritis patients as disease controls (n=31), and healthy controls (n=31). Positive results were confirmed by Western blotting. Results. The difference in B. burgdorferi seropositivity between the groups was not significant by any method. Seroreactivity to B. burgdorferi antigens by ELISA was detected in 26.7% of the patients with Behcet's disease, 35.5% of those with rheumatoid arthritis, and 19.4% of the healthy controls. Immunoblots were positive in 13.3% of the Behcet's disease patients, 22.6% of the rheumatoid arthritis patients, and 12.9% of healthy controls. Conclusion. These results suggest no association between Behcet's disease and B. burgdorferi infection