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[Periodic disease and periarteritis nodosa in the same patient: coincidence?].

Familial Mediterranean fever (FMF) chiefly affects patients of Arabic, Jewish, Armenian or Turkish origin and takes the form of recurrent episodes of peritonitis, arthritis or pleurisy. Periarteritis nodosa (PAN) is a vasculitis affecting elderly people and manifested by a general deterioration, unexplained fever and peripheral neuropathy or muscular weakness. We describe a patient presenting both diseases. Ours is the seventh reported case associating these two affections. This association was suspected by SACHS and co-workers who discovered an increased frequency of PAN in patients with FMF compared to the expected rate for the whole population (7). These observations warrant a search for PAN in young patients affected by FMF and showing signs of vasculitis.

Adult↗

[Immunogenetic blood markers as a risk factor of development of periodic disease in Armenian population].

AIM: To reveal characteristic associations between various markers of immunogenetic (HLA--A, B, C, DR), erythrocytic (ABO, Rh-Hr, MNSs, Pp, Kell-Chellano, Lewis), serum (Gm, Inv) systems and familial Mediterranean fever (FMF) in Armenian population. MATERIAL AND METHODS: From 41 to 125 patients (depending on systems studied) were examined. HLA-antigens of A-, B-, C-classes were detected by the microcytotoxic test in a total population of lymphocytes, HLA-DR antigens--by a prolonged test in B-lymphocytes, erythrocytic antigens--by hemagglutination technique and indirect Coombs' reaction, serum antigens--by the method of hemagglutination suppression. RESULTS: Confidential positive associative relations between antigens HLA-A1 and A9 (RR = 2.2 and 2.4), HLA B5 and B35 (RR = 3.03 and 3.2), HLA-Cw4 (RR = 4.3), HLA-DR3 (RR = 2.6), phenotypes AB (RR = 2.86) and MN (RR = 2.2), serum antigen Gmx+ (RR = 2.87) were demonstrated. Simultaneous expression of phenotype MN and antigen Gmx+ is a marker of high predisposition to the disease (RR = 4.7). Negative associative relations were found between FMF and antigens HLA-B12 and B18 (RR = 0.6 and 0.1), HLA DR4 (RR = 0.1) and phenotype MM (RR = 0.37). CONCLUSION: A simultaneous complex investigation of the markers of various immunogenetic systems allows detection of genetic markers of predisposition to FMF (HLA-B5, B35, Cw4, DR3, AB, Gmx+, MN) and the resistance to this disease (HLA-B12, B18, DR4) in a population of Armenians.

Adolescent↗

[Periodic disease and pregnancy].

The authors report a case of familial Mediterranean fever in a pregnant woman treated with Colchicine. She delivered normally at term. A review of the literature shows that colchicine does not have a teratogenic effect which it was long thought to have. All the same it is best to carry out fetal karyotype examination using early amniocentesis. Furthermore, colchicine improves fertility which is disturbed in these patients and pregnancy has a good effect on the disease.

Adult↗

[Level of leukotrienes B4 and C4 in the blood of patients with periodic disease].

The level of LTB4 and LTC4 in blood plasma of patients with familial mediterranean fever is significantly higher than in healthy donors: 53 + 10 pg/ml for LTB4 (normal - 25 + 5 pg/ml) and 175 + 22 pg/ml for LTC4 (normal 67 + 19 pg/ml). The more increase of the LTB4 and LTC4 content in plasma is observed during attacks of fever - 107 + 21 pg/ml (LTB4) and 249 + 34 pg/ml (LTC4). Hyperbaric oxygenation of patients, used to relieve pain and fever, reduces the level of leukotrienes.

Colchicine↗

[Encapsulating peritonitis in periodic disease. Apropos of a case studied by x-ray computed tomography].

A case of encapsulating peritonitis complicating the course of familial Mediterranean fever is reported. This encapsulating peritonitis was responsible for abdominal pain and had a "pseudocystic" appearance on ultrasonography and computed tomography. Ultrasound guided aspiration produced a yellowish fluid rich in proteins and poor in cells. Surgical operation revealed a congested appearance of the peritoneum associated with a richly fibrinous appearance of the external wall of the mass. The differential diagnosis is discussed.

Adult↗