Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0024523 (malabsorption)
 7,319 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The clinical outcome of 61 patients with renal amyloidosis treated with chronic dialysis was reviewed. Eighteen patients, 4 with primary or AL amyloidosis and 14 with reactive or AA amyloidosis, died within one month from starting treatment. The other 43 patients were treated with dialysis for 3 to 199 months and are the object of this study. Sixteen patients had AL amyloidosis and 27 had AA amyloidosis. Thirty-five patients were treated with hemodialysis (HD) for a mean period of 40 +/- 47 months and 8 were treated with continuous ambulatory peritoneal dialysis (CAPD) for 20 +/- 15 months. Patient survival rate at 1 and 5 years was 68% and 30% respectively. There was no difference in survival rate between patients treated with HD and those treated with CAPD, while patients younger than 45 had a better 5-year survival rate. Twenty four (60%) patients achieved a satisfactory rehabilitation with dialysis. At the last follow-up, 15 patients (14 on HD, 1 on CAPD) were alive 61 +/- 58 months after starting dialysis. Twenty-eight patients died after 30 +/- 20 months. The main causes of death were: cardiovascular accident (11), stroke (3), sepsis (5) and cachexia (5). The most important extra-renal complications of amyloidosis were related to cardiovascular involvement (heart failures, arrhythmias, hypotension) and gastrointestinal involvement (malabsorption). Intra-dialytic hypotension in patients on HD and peritonitis in patients on CAPD were the main problems related to dialytic procedure. his study confirms that life expectancy and the quality of life of dialysis patients with systemic amyloidosis are poorer than those of general dialysis population.(ABSTRACT TRUNCATED AT 250 WORDS)
 ...
PMID:Chronic dialysis in patients with systemic amyloidosis: the experience in northern Italy. 151 84

The progression of familial Mediterranean fever is marked by the recurrence, at varying intervals, of acute flares that regress spontaneously. Prognosis, which depends on the occurrence of amyloidosis, has been transformed by colchicine treatment. Incidence of amyloidosis is higher in certain ethnic groups (Jews from North Africa, Turks) and depends on by the specific MEFV mutation. Amyloid is composed of clusters of protein strands identical to the AA protein of secondary amyloidosis and infiltrates the walls of all arterioles except those of the central nervous system. The earliest and most consistent localization is in the kidney, where it develops over several years and in 4 stages--preclinical (latency), proteinuric, nephrotic and uremic--before concluding in end-state renal failure. Before the advent of colchicine, dialysis and transplantation, only renal amyloidosis caused clinical manifestations and lethal complications; any amyloidosis at any other sites remained latent. Prolonged survival with hemodialysis and kidney transplantation now leaves time for manifestation of these other localizations, such as infiltration into the intestines causing malabsorption, or potentially lethal cardiac lesions. Treatment of familial Mediterranean fever is based on the continuous administration of colchicine, which at the average dose of 1 to 2 mg per day can prevent flares or at least reduce their frequency or intensity. Systematic use of colchicine also prevents the onset of amyloidosis, even in the rare cases where it cannot prevent flares. These data fully justify the systematic use of colchicine for continuous prophylactic treatment from diagnosis and even after kidney transplantation, to prevent recurrence of the grafted kidney or extension to other organs. The curative efficacy of colchicine on flares is debatable, although several studies report positive results against progression of early amyloidosis.
 ...
PMID:[From familial Mediterranean fever to amyloidosis]. 1614 55