PW01-036 – Renal replacement therapy in patients with FMF
نویسندگان
چکیده
Results Hemodialysis: Of the 28 patients not undergoing renal transplantation (Tx) in Armenia, 9 died (systemic amyloidosis 5, heart attack 2, stroke -2); 15 moved to another country and 4 remained on dialysis. Over half of the 25 pts with minimum observation period of 6 mo were resistant to EPO. One third died, mainly due to cardiovascular complications and systemic amyloidosis. Living related donor Tx was done after 8 mo (median) of HD in 12 patients aged 38 ± 11.6 years, i.e. 12.5% of all Tx (96) done at the same period. In addition to standard immunosuppression all received low dose colchicine (0.6-1.2 mg/day). Main complications were rejection (8), delayed graft function for tubular necrosis (2), lymphocele (2), CMV disease (2) and tuberculosis (1). Additional problems included diarrhea (colchicine, MMF, generalized amyloidosis; 9) and severe neuropathy due to interaction of cyclosporine with colchicine (1). Interaction of colchicine with CNI/MMF (neuropathy,diarrhea) required reduction of immunosuppression in some patients, resulting in higher rejection rate. One patient died of generalized amyloidosis and 1 kidney was lost after reduction of immunosuppression due to tuberculosis. Ten patients have good renal function.
منابع مشابه
PW01-031 – Treatment of FMF in middle and old age
Introduction Current recommendations for the treatment of familial Mediterranean fever (FMF) are based largely on the observation of FMF patients receiving colchicine therapy in childhood and young age. The adequate colchicine therapy led to more and more patients survive to that age. In addition, there are national peculiarities of FMF. For example, in Armenia, even before the massive use of c...
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Results Heterozygote carriers associated with abortive and mild FMF features is 18,72%, and 1.29% of patients with clinical features of FMF are without mutations. In some FMF patients “mild” MEFV mutations are associated with inflammatory attacks (P369S: 0.49%; E148Q: 5.09%; A744S: 0.74%). Genotypes E148Q/A744S and E148Q/P369S are found rarely. We have revealed the complex FMF cases with follow...
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Cem Sungur, MD, PK (PO Box) 272, 06693 Kavaklidere, TR-Ankara (Turkey) with FMF were compared to 5 chronic hemodialysis patients with different renal disease. None of these patients had active inflammatory disease (e.g. active SLE) or infectious complications. All of the patients were dia-lyzed with cuprophane membranes and acetate-containing dialysate. The duration of renal replacement therapy...
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Introduction Familial Mediterranean fever (FMF) is an inherited autosomal recessive disorder, ethnically restricted and commonly found among individuals of Mediterranean descent, caused by MEditerranean FeVer gene (MEFV) mutations on the chromosome 16. It is the most frequent periodic febrile syndrome among autoinflammatory syndromes. Eighty % of patients with FMF have MEFV mutations, while aro...
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Introduction MEFV is the first identified autoinflammatory gene related to Familial Mediterranean Fever (FMF) disease. We previously the tested the hypothesize of epigenetic involvement in FMF, mainly based on the occurrence of FMF in patients without mutations and decreased MEFV transcripts in leukocyte samples independent from mutations. Our study showed that higher methylation level of MEFV ...
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