Familial Mediterranean fever (FMF) is an autosomal recessive autoimmune disorder characterized by recurrent bouts of fever and serosal inflammation. FMF may be complicated by AA-type amyloidosis, worsening the prognosis, with associated renal failure in some patients. Complication rate varies with race, being as high as 60% in Turks and as low as 2% in Armenians. In a few cases of patients with FMF (phenotype 2), amyloid nephropathy may be the presenting manifestation. This study included 420 patients who were admitted to the Nephrology and Rheumatology Departments of Atatürk Education and Research Hospital with unexplained proteinuria/nephrotic syndrome. The initial screening test for amyloidosis was the presence of significant proteinuria (300 mg/24 h). All MEFV gene exons were screened for causative mutations by direct DNA sequencing to check for any mutations. There were 22 phenotype 2 FMF patients with 27 allelic variants. The most prevalent allelic variants were M694V (10/27, 37%) and E148Q (7/27, 26%). Phenotype 2 FMF is not as rare as it was thought before; this should be kept in mind for all patients with unexplained proteinuria and/or acute phase response in high-risk ethnic groups for FMF.
OBJectIVe: We evaluated the incidence and risk factors for acute renal failure (ARF) and also the associated hazard of death in recipients of cardiac transplants.
MAterIAl and MetHODS:We included 25 patients in the study; 18 patients developed ARF (72%) and underwent continuous venovenous hemodiafiltration (Group I) and 7 patients had stable renal function (28%) (Group II). We retrospectively retrieved demographic variables; clinical, perioperative, postoperative complications and echocardiographic data; and biochemical parameters at the time of the surgery and six months later.
reSultS:Cumulative survival was 72.2% after 6 months, 64.2% after 24 months, and 51.4% after 32 months for Group I and 50% after 32 months for Group II (p>0.05). A total of 8 patients died (32%); 1 (5.5%) from Group I and 7 (87.5%) from Group II. Risk factors for ARF were preoperative serum BUN, creatinine levels, and cardiopulmonary bypass time (p<0.05). Only one patient underwent chronic hemodialysis because of chronic renal dysfunction in Group I while there was no such patient in group II.cOncluSIOn: Preoperative serum BUN, creatinine value, and cardioopulmonary bypass time were found to be risk factors for ARF after cardiac transplantation. Postoperative renal dysfunction did not affect long-term renal function and survival.
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