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At least 991 records · Page 55Linked to original sources
PubMed1975

The effect of beta aminoproprionitrile (BAPN) on experimental amyloidosis.

Experimental amyloidosis was induced in mice with repeated injections of complete Freund's adjuvant (CFA) reinforced with bacterial vaccine. BAPN administered in a mixture with CFA or on its own before the injection of CFA reduced the incidence of amyloidosis. The reduction in the incidence of amyloidosis following the administration of BAPN may be due to its inhibitory effect on the oxidative deamination of amino acids, which presumably inhibit cross-linking of amyloid fibrils or interfere with metabolic pathways which involve the formations of mucopolysaccharide formation. It is suggested that the defective formation of the mucopolysaccharide-amyloid protein complex inhibits amyloid deposition and induces the activity of beta glucuronidase observed in the present study. The reduced incidence of amyloidosis following BAPN adminsitration cannot be due to lysosomal enzyme degradation of the amyloid as the activity of cathepsin D and acid phosphatase is decreased during this process.

Acid Phosphatase↗
PubMed2002

[A case of rheumatoid arthritis with secondary amyloidosis in urinary bladder].

We document a case of 61-year-old woman with a 24 year history of rheumatoid arthritis (RA), who developed severe polyarthralgia, dry cough, paresthesia in the legs, frequent micturition, and severe macrohematuria. We diagnosed as severe RA with extraarticular manifestations based on interstitial pneumonia, mononeuritis multiplex, subcutaneous nodules, and high titer of rheumatoid factor. Ultrasonography demonstrated an intravesical mass lesion. A histological examination of the urinary bladder by endoscopic biopsy revealed marked deposition of AA amyloid. The diagnosis of secondary amyloidosis and bacterial cystitis were made based on histological findings and urine culture. At first, we administered antibiotics by intravenous infusion, which resulted in cure of cystitis and partial improvement of macrohematuria. Then combination therapy of corticosteroids and cyclophosphamide successfully reduced the disease activity of RA. There have only been a few reports published so far on the vesical amyloidosis in patients with RA. However, 5 of 10 patients (50%) in vesical amyloidosis died because of continuous massive hematuria, which induced disseminated intravascular coagulation and multiple organ failure. In conclusion, secondary amyloidosis of the urinary bladder should be considered as a possible cause of hematuria in patients with long-term RA and as an important prognosis factor of RA.

Amyloidosis↗
PubMed1975

[Amyloidosis of the heart].

Two necropsic cases with heart amyloidosis in seninle age are described. The peculiarity in the first case is the parallel affect of myocardium and live with the absence of a disease, which might lead to secondary amyloidosis. The high elevated gamma-globulins (44%) in that case give grounds an autoimmune determined primary idiopathic amyloidosis to be presumed. The second case is with isolated senile myocardial amyloidosis.

Aged↗
PubMed1975

[Generalized secondary amyloidosis in patients with chronic rheumatoid arthritis (author's transl)].

During a period of 6 years (1968 to 1973) 177 patients with rheumatoid arthritis were submitted on one or more occasions to biopsy of the rectal mucosa for the diagnosis of amyloidosis. The indications for biopsy were as follows: 1. proteinuria, even in an intermittent form, 2. progressive type of rheumatoid arthritis with high inflammatory activity, 3. rheumatoid arthritis of longer than 2 years duration with a marked tendency to joint destruction. The histological specimens were stained with Congo red and investigated in polarized light. The biopsy for amyloidosis was positive in 80 patients (45.2%) of whom 14 showed no proteinuria, even of an intermittent nature. Patients with rheumatoid arthritis survived maximally 5 years after amyloidosis had been diagnosed. No successful therapy of amyloidosis has been devised.

Adult↗
PubMedDate not supplied

Amyloidosis in leprosy.

Histopathologic studies were performed on systemic amyloidosis of leprosy patients in two Japanese leprosaria. Amyloid deposits were observed in 23 of 50 cases (46%) autopsied from 1955 to 1959 (Group A), and in 17 of 110 cases (15.4%) from 1962 to 1971 (Group B). These incidences are higher than those of the pre-chemotherapeutic period in Japan. The average age of amyloidal cases was significantly lower than that of nonamyloidal cases in both groups. There was no predilection for either type of leprosy in Group B. Pyelonephritis was frequently observed in both groups; the incidence of amyloidosis in Group B correlated with pyelonephritis. Amyloid deposits may be related to renal disorders associated with lepra reactions or inflammatory lesions. Amyloidosis has been classified into primary and secondary type, but the criteria seem uncertain. We referred to a classification based on immunologic finginds. In the severely affected lesion of the kidney of one case, we observed foci of foreign body type giant cells, suggesting a possibility of resorptive process in human renal amyloidosis.

Adult↗
PubMed2002

Raised circulating interleukin-18 levels in reactive AA-amyloidosis.

OBJECTIVE: To study the circulating levels of interleukin-18 (IL-18), a proinflammtory cytokine implicated in the T helper I response, in patients with rheumatoid arthrtitis (RA) with or without amyloidosis. METHODS: Plasma IL-18 levels were studied by enzyme-linked immusorbent assay in 55 RA patients with reactive amyloidosis and in 55 RA patients without amyloidosis matched with respect to age, sex, seropositivity, disease duration and inflammatory activity, as well as in 55 healthy control subjects. RESULTS: Plasma IL-18 levels were significantly elevated in RA patients as compared with control subjects. Those RA patients who had amyloid had significantly higher circulating level of IL-18 than those without amyloid (418.1 +/- 32.1 ng/l versus 317.0 +/- 21.3 ng/l, P<0.02). This difference was not due to differences in inflammatory activity, nor was it related to renalfunction. CONCLUSION: RA is associated with increased levels of plasma IL-18, the levels being significantly higher in patients with amyloid than in those without amyloid The increased level in the amyloidosis patients may reflect the interaction ofamyloid with cellular meatbolic pathways or, possibly, suggest a direct role of IL-18 in amyloidogenesis.

Amyloid↗
PubMed2002

[Secondary amyloidosis in patients with rheumatoid arthritis(RA)].

The amyloidoses are a group of protein deposition diseases in which amyloid proteins composed of insoluble fibrils are deposited in various organs. Most cases of the secondary amyloidosis(AA amyloidosis) in which amyloid A(AA) protein is deposited followed by uncontrolled, long term RA(duration 7 to 10 years). It has been revealed that the multi-organ dysfunction associated with AA amyloidosis causes the deterioration of RA prognosis. Since the mechanism of amyloid protein deposition is still unknown, the diagnosis of AA amyloidosis is difficult and there is no fundamental therapy for it; there are only supportive therapies for the malfunction of involved organs.

Amyloidosis↗
PubMed2003

[Localized amyloidosis of the cavum].

INTRODUCTION: Amyloidosis is characterized by extracellular deposits of proteins. OBSERVATION: A 66 year-old patient presented with a pseudo-tumoral amyloidosis of the cavum. Clinical and biological examinations confirmed the localized aspect of the disease and immunohistochemical exploration identified a type AL amyloidosis. COMMENTS: The disease may be diffuse involving many organs and leading to various clinical manifestations. It can also be localized and take on a pseudo-tumoral aspect. Localised amyloidosis is a rare lesion of the upper aero-digestive tract, predominating in the larynx. Nasopharyngeal involvement is exceptional.

Aged↗
PubMed2003

[Amyloidosis and oxidative stress].

Recent studies on oxidative stress have revealed that free radical injury appears to be involved in either the amyloid formation process or in post-fibrillar modification in several types of amyloidosis. Here, we report the role of oxidative stress in the pathogenesis of dialysis-related amyloidosis(DRA) and familial amyloidotic polyneuropathy(FAP), and propose radical scavenger treatment for such amyloidosis. For patients under maintenance hemodialysis, EC-SOD Arg213Gly was a risk factor for the progression of DRA, atherosclerosis, and renal failure causing hemodialysis. In FAP patients who had EC-SOD Arg213Gly, massive amyloid deposition which may be related to increased oxidative stress in loco was found especially prominently around blood vessels in the interstitial tissues. Histological and biochemical examinations revealed that oxidative stress is deeply connected with amyloid formation mechanisms in FAP. We started radical scavenger therapy, such as N-acetyl cysteine, vitamin E and vitamin C in 20 Swedish FAP patients for 6 months. Although no improvement was found in the amount of amyloid deposition in biopsy specimens, modified body mass index(mBMI), an index of nutritional status, tended to be increased, suggesting the therapeutic possibility of radical scavenger treatment for amyloidosis.

Amyloidosis↗
PubMed2003

[Amyloidosis localized in the seminal vesicles: clinico-pathological review of 8 cases diagnosed in surgical specimens].

OBJECTIVES: Clinical and pathological study of eight cases of localized amyloidosis of the seminal vesicles. Review of the literature. METHODS: Immunohistochemical and histochemical study in surgical specimens. RESULTS: Two of the eight cases studied for amyloidosis in seminal vesicles, were obtained from radical prostatectomy specimens; patients had prior androgen deprivation therapy for two months. Four cases were obtained from radical cistoprostatectomy specimens because of urothelial carcinoma and the last two cases were diagnosed by transrectal prostatic needle biopsy which including a portion of seminal vesicle. Amyloidosis of seminal the vesicle is permanganate-sensitive; A-Amyloid, laminin, amyloid P protein and collagen IV negative. CONCLUSION: Localized amyloidosis ot the seminal vesicle is not an unusual finding. The incidence increases with age. The histochemical and immunohistochemical features are different from other amyloid deposits.

Adenocarcinoma↗
PubMedDate not supplied

Familial Mediterranean fever (FMF) and renal AA amyloidosis--phenotype-genotype correlation, treatment and prognosis.

Familial Mediterranean fever (FMF) is an autosomal recessive disease, which primarily affects the population surrounding the Mediterranean basin. It is characterized by recurrent attacks of fever and peritonitis, pleuritis, arthritis or erysipelas-like erythema. Amyloidosis, causing renal failure, is one of the most severe complications of the disease. The gene associated with FMF (MEFV) has been recently isolated. Phenotype-genotype correlation studies revealed that amyloidosis was more common in FMF patients originating from North-Africa who were homozygous for the M694V mutation. Such a correlation was not found in Turkish patients. The risk of amyloidosis is increased in male FMF patients and in patients bearing polymorphism a/a in the SAA1 gene. Colchicine is the chosen drug for the treatment of FMF and can prevent amyloidosis.

Amyloidosis↗
PubMed2003

[Is the current treatment of primary and secondary amyloidosis effective?].

BACKGROUND: Retrospective study about results of treatment of patients (pts) with primary (AL) and secondary (AA) amyloidosis is given. 31 pts with systemic forms of amyloidosis have been treated and followed-up in our department since 1993. METHODS AND RESULTS: 6 men and 11 women were in the AL group with the mean age of 59 years. Multiple myeloma was diagnosed in 9 pts, monoclonal gammapathy of undetermined significance (MGUS) was found in 8 pts. The kidneys were affected in all pts, heart in 59% of pts, liver, joints and skin in 26% of pts and polyneuropathy was detected only in 1 pt. Progression of renal insufficiency with decrease of glomerular filtration rate (GFR) was detected in the AL group at the end of follow-up period compared with the initial level (p < 0.05) despite the intensive treatment. The difference did not reach statistical significance in other investigated parameters. Median of survival was 13 months from the assessment of diagnosis. Partial remission of amyloidosis was achieved in 9 pts, stable disease was in 5 pts and in 3 pts the disease progressed. 4 men and 10 women were in the AA group with mean age of 58 years. Underlying disease was rheumatoid arthritis in 7 pts, ankylosing spondylitis in 2 pts, juvenile chronic arthritis in 1 pt, Crohn's disease in 2 pts, eosinophilic fasciitis in 1 pt and chronic abscesses in NK cell deficiency in 1 pt. The kidneys were affected in all pts, bowels and heart in 36% of pts. GFR (p < 0.05) and plasma creatinine (p < 0.01) significantly decreased at the end of follow-up period compared with initial levels. Median of survival was 30 months. Partial remission was achieved in 2 pts, stable disease was in 3 pts and progression was detected in 9 pts despite the use of various treatment regimens. CONCLUSIONS: Both forms of systemic amyloidosis represent severe disease with limited response to treatment. The use of new drugs is promising and could lead to better response to treatment.

Adult↗
PubMed1992

[Ulcerative colitis and amyloidosis. Presentation of a case and review of the literature].

A case of ulcerative colitis associated with secondary amyloidosis in a 62-year-old man who died from septic shock and pneumonia complicating head injury is reported. Amyloid deposition was incidentally found at autopsy. Proteinuria and hepatomegaly discovered a few days before his death were the only signs of amyloidosis. The postmortem examination showed chronic ulcerative colitis (remitting form) with pseudo-polyps and amyloid deposition in the liver, spleen, pancreas, rectum, adrenals and kidneys. Although secondary amyloidosis complicating with Crohn's disease has been frequently reported, amyloidosis associated with ulcerative colitis has been exceptionally described and only 10 cases have been collected from the literature.

Amyloidosis↗
PubMedDate not supplied

[AA-amyloidosis in inflammatory rheumatic diseases. A report of clinical experiences].

A review of the literature on rheumatic diseases with secondary amyloidosis and our diagnostic and therapeutic experiences in 33 own cases were reported. The place of subcutaneous fat biopsy in the diagnosis of secondary amyloidosis was shown included our own results and the corresponding literature. We saw amyloidosis type AA mostly in patients with high disease activity and progressive stage. There is no sure relationship to the duration of the disease. The renal involvement is clinical most significantly and also most frequently. The renal amyloidosis type AA can be influenced therapeutically however only in normal or unimportant restricted renal function. The therapeutic success is not depended on the choice of the long acting antirheumatic drug. The results were discussed in respect with the current literature.

Adipose Tissue↗
PubMed1955

Amyloidosis in paraplegia correlation with decubitus ulcers.

Six patients died with amyloidosis and four patients are living with amyloidosis out of 1,000 with spinal cord injuries who were studied.No correlation was found between Congo red retention and the length of time since injury or the site of the spinal cord lesion. However, it is felt that the longer decubitus ulcer persists, the greater is the probability of amyloidosis. It is believed that decubitus ulcers with secondary osteomyelitis are of primary importance as etiologic factors in amyloidosis associated with spinal cord injuries, and that pyelonephritis plays a very minor secondary role.

Amyloidosis↗
PubMed2003

[Amyloidosis and pleural calcification].

INTRODUCTION: Pulmonary diseases during the course of generalised amyloidosis are principally represented by tracheobronchial involvement and diffuse parenchymal localizations. CASE REPORT: The authors report the case of a 66-year-old woman presenting with pleural amyloidosis in the context of generalised amyloidosis. Thoracoscopy performed in the investigation of recurrent transudative pleural effusions found evidence of an inflamed parietal pleura with areas of calcification. Pleural biopsies confirmed amyloid infiltration pleural ossification. Talc pleurodesis was performed. CONCLUSIONS: The authors' conclusion is that, meeting an unexplained pleural effusion even transudative during the course of a generalised amyloidosis, the thoracoscopy is the diagnosis key test as it allows moreover a pleural pleurodesis to be performed.

Aged↗
PubMedDate not supplied

[Secondary amyloidosis (AA) and renal disease].

Amyloidosis is a disease resulting from extracellular deposition of fibrillar protein in various organs. AA amyloidosis may complicate chronic inflammatory diseases, chronic infections and another chronic diseases. We review 31 patients (13 males and 18 females) with biopsy proven renal or rectal AA amyloidosis, referred to out hospital between january 1999 and november 2002. Renal failure was defined as serum creatinine > or = 1.5 mg/dl. Mean age was 58.4 +/- 15.7 years. The causes of AA amyloidosis were an underlying chronic rheumatologic disease (51.6%), chronic infection (41.9%) and a chronic inflammatory intestinal disorder (6.5%). Renal failure (RF) was detected in 20 patients (61.2%) and proteinuria and hematuria were found in 90.3% and 45.5 respectively. Proteinuria at diagnosis was 5.2 +/- 3.9 g/24 h and mean serum creatinine 3.5 +/- 3.7 mg/dl. Survival of patients without dialysis was 66.8 (51.1% RF, 90.9% non-RF) and 53.4% (38.2 RF, 77.9% non-RF) at 12 and 24 months respectively (p = 0.017). End-stage renal disease developed in 13 patients (41.9%). Ten patients were maintained on hemodialysis and 3 on CAD. Survival in dialysis at 6 and 12 months was 68.3% and 42.7% respectively. Fifteen patients died and the main causes of death were: infections (46.6) haemorrhagic complications (33.3%), cardiovascular events (13.3%) and cachexia (6.6%).

Amyloidosis↗
PubMed2003

[Two cases of systemic amyloidosis presenting with abnormalities in liver function tests].

Systemic amyloidosis results from the deposition of insoluble, fibrous amyloid proteins. It occurs mainly in the extracellular spaces of multiple organs and tissues including the kidney, heart, and liver. Although amyloid deposition in the liver is common in patients with systemic amyloidosis, clinically apparent liver disease is relatively rare. Indeed, most patients with systemic amyloidosis manifest only minimal to moderate hepatomegaly and trivial abnormalities in liver function tests. Recently, we experienced two cases of patients who presented with abnormalities in liver function tests and hepatomegaly as manifestations of systemic amyloidosis. We report these cases with a review of the relevant literatures.

Adult↗