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At least 163 records · Page 9Linked to original sources
PubMed1993

[Dialysis-associated amyloidosis. Part 2: Incidence and site of osseous amyloidosis. Dialysis characteristics and clinical findings].

In a conventional radiographic skeletal survey of 90 hemodialyzed patients, 28% (25 patients) showed renal osteopathy; 27 patients (30%) had periarticular bone cysts and 3 patients (3%) presented radiological evidence of destructive spondylarthropathy. The bone cysts were most commonly identified in the carpal bones and around the hips and shoulders; destructive spondylarthropathy was seen in the cervical and lumbar spine. Whereas characteristic radiographic changes of renal osteodystrophy could already be identified in the 1st year of dialysis, periarticular bone cysts occurred at the earliest after 2 years, most frequently after 5 years of dialysis. Destructive spondylarthropathy was seen after more than 10 years of dialysis. A positive correlation between these bone lesions and secondary hyperparathyreoidism, dialysis membranes or renal diseases was not found.

Adult↗
PubMed1997

Familial Mediterranean fever in children: report of a large series and discussion of the risk and prognostic factors of amyloidosis.

UNLABELLED: Familial Mediterranean fever (FMF) is a genetically transmitted disease characterized by recurrent attacks of fever and serositis. The most important complication of this disease is the development of amyloidosis. We present our analysis of 425 FMF patients without and 180 with amyloidosis (123 FMF having amyloidosis type 1 and 57 FMF having amyloidosis type II). The male female ratio was higher in the amyloidosis population (111/69) when compared to the FMF population (225 200) (P = 0.048). Consanguinity rate was the same among FMF and amyloidosis groups. However, a family history of amyloidosis was significantly more frequent in the amyloidosis group (P = 0.00001). Multivariate analysis has revealed that in FMF patients, the presence of a family history of amyloidosis plus consanguinity has a 6.04 fold increased risk of amyloidosis (P < 0.0001). The 5-year chronic renal failure free survival was 43.1% and 18.7% in type I and type II amyloidosis, respectively. The time interval to develop chronic renal failure after the development of amyloidosis was 4.8 in type I and 3.0 years in type II. respectively. We found ten cases of Henoch-Schönlein Purpura and nine of polyarteritis nodosa among our patients. The significance of the association between FMF and vasculitis awaits to be clarified. Among the FMF patients put on colchicine therapy (435), only 10 (2.3%) have developed amyloidosis confirming that this drug protects from amyloidosis. CONCLUSION: Since the presence of a familial history of amyloidosis has been defined as the most important risk factor in the development of amyloidosis, we suggest that additional genetic factors may be operative in the development of amyloidosis.

Amyloidosis↗
PubMedDate not supplied

Serum amyloid A1 -13 T/C alleles in Turkish familial Mediterranean fever patients with and without amyloidosis.

Previously reported studies concerning the effect of homozygosity of the 1.1 allele of the SAA gene found a correlation between this haplotype and susceptibility to amyloidosis in FMF patients. Another report revealed a strong association between SAA1 -13T/C and secondary amyloidosis in the rheumatoid arthritis patient group. In this study, we aimed to determine the effect of SAA1 -13T/C in FMF patients with and without amyloidosis. The study cohort, consisting of 166 patients with FMF was divided into two groups, according to the presence (n=66) or absence (n=100) of renal amyloidosis at study entry. MEFV gene mutation analysis and allelic variant of SAA1 gene -13 T/C was analyzed according to the previously described techniques. SAA1 -13 T allele frequencies were 0.5816, 0.23 and 0.4242 in controls, FMF patients and FMF-amyloidosis patients respectively. The difference between controls vs. FMF patients and FMF-amyloidosis patients were 0.0002 and 0.1673 respectively. It was 0.0071 for FMF-patients vs. FMF-amyloidosis. When 694 M/V homozygous nonamyloid-FMF group was compared with 694 M/V carriers of the FMF-amyloidosis group, the difference was 0.049. When carrying TT allele was considered, the difference between controls vs. FMF patients and FMF-amyloidosis patients were 0.0001 and 0.58. It was 0.0003 for FMF patients vs. FMF-amyloidosis. When 694 M/V homozygous nonamyloid-FMF group was compared with 694 M/V carriers of the FMF-amyloidosis group, the difference was 0.03. Carrying SAA -13T in homozygote state revealed a 7.9 (95% CI 3.6 -17.5) fold risk for the occurrence of amyloidosis when compared with FMF patients without amyloidosis. This was 8.75 (95% CI 3.0 - 25.1) when 694 M/V homozygotes were taken into consideration. Our data revealed that the genotype SAA1 -13T has at least an effect on the development of amyloidosis. As more data on this polymorphism accumulate, we will understand its effect on the pathogenesis of amyloidosis in FMF.

Amyloidosis↗
PubMed2005

[Multisystemic amyloidosis. Clinical study of 39 patients in Lebanon].

OBJECTIVE: Clinical analysis of multisystem amyloidosis in Lebanon, by histological type. METHOD: Retrospective study of 39 cases of multisystem amyloidosis diagnosed histologically in a university hospital center between 1991 and 2002. It analyzed the following clinical data: age, gender, type of presentation, time from symptom onset to diagnosis, clinical features, concomitant diseases, family history of amyloidosis, biopsy sites, presence of urinary or serum monoclonal gammopathy, immunohistochemical type, prognosis and treatment. RESULTS: Median age at diagnosis was 56+/-18 years. The overall ratio of men to woman was 1.4. AL amyloidosis (amyloid light chain) accounted for 54% (21/39) of the cases, AA (amyloid-associated) amyloidosis 36% (14/39), while 10% (4/39) were not typed. Among the 21 cases of AL amyloidosis, 12 were idiopathic (57%) and 9 (43%) were associated with multiple myeloma; among the 14 cases of AA amyloidosis, 7 were associated with familial Mediterranean fever and 5 with chronic disorders. Proteinuria was often the first symptom. The initial manifestations in AL amyloidosis patients with myeloma were more often related to amyloidosis than to myeloma. Renal involvement was seen in 95% (37/39) of all cases (95% of AL versus 93% of AA), proteinuria in 87% of cases and renal failure in 72%. Cardiac amyloidosis (57% of AL versus 7% of AA; p>0.05), infiltration of the tongue (19% of AL versus 0% of AA; p>0.05) and neurological manifestations (24% of AL versus 7% of AA; p>0.05) were more frequent in AL amyloidosis. The 7 patients who died (18%) had AL amyloidosis (5 of them had myeloma). Heart failure was the most frequent cause of death related to amyloid. CONCLUSION: Multisystem amyloidosis is frequent in Lebanon and familial Mediterranean fever is still frequently associated with the secondary type. Accurate diagnosis and classification are essential for the prognosis and treatment of the disease. Poor prognosis was associated with the AL type, especially when accompanied by myeloma, and with cardiac amyloidosis.

Adult↗