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PubMed1991

A case of primary systemic amyloidosis with AA protein.

We present here a case of young adult-onset primary systemic amyloidosis with AA protein. A 29-year-old male presenting with macrohematuria was transferred to our hospital because of aggravation of renal function. Amyloid protein was detected in the bladder, stomach and thyroid. The amyloid protein proved to be AA type by potassium permanganate histochemical analysis. Since secondary amyloidosis was excluded by the laboratory data and there was no family history of amyloidosis, a diagnosis of primary systemic amyloidosis with AA protein was reached. Although the patient was prescribed dimethyl sulphoxide, his renal function worsened with gastrocolic symptoms and a bleeding tendency. Hemodialysis (HD) was then initiated. After starting the HD, the patient's general condition recovered and subsequently the patient on treatment with maintenance HD was discharged.

Adult↗
PubMed1991

Familial cardiac amyloidosis. Diagnosis by immunocytochemistry.

Familial cardiac amyloidosis is a rare disorder that is difficult to diagnose. There is no specific therapy for this disease, but it is important to distinguish the cardiac and gastrointestinal symptoms of this disease from those of other treatable causes. We have treated a patient with this disorder who presented with cardiac and gastrointestinal symptoms. The diagnosis of amyloidosis was suspected on rectal biopsy and was confirmed by immunocytochemistry and immunoalkaline phosphatase technique. Pre-albumin was demonstrated in the lesion. We concluded that when familial amyloidosis is suspected, a biopsy from the suspected organ system is helpful for the diagnosis. The detection of pre-albumin by immunocytochemistry can elucidate the diagnosis of familial amyloidosis.

Aged↗
PubMedDate not supplied

[Rheumatologic and radiologic symptoms of dialysis-associated beta 2-microglobulin amyloidosis: long-term retrospective study of 175 chronic hemodialysis patients].

beta 2-microglobulin amyloidosis is a major complication in chronic hemodialysis patients. Destructive arthropathy, spondylarthropathy, and carpal tunnel syndrome are clinical manifestations of beta 2M amyloid depositions within the joints, intervertebral discs, and tendon sheets. We have investigated the prevalence of beta 2M amyloidosis associated radiological joint lesions in a population of 175 patients on chronic hemodialysis. In 32 of 175 patients the diagnosis of amyloidosis arthropathy and spondylarthropathy was made by radiomorphological criteria. These 32 patients were asked about rheumatic symptoms (localisation and character of pain, synovitis, carpal tunnel syndrome, influence of dialysis membrane on pain) and examined clinically. Bilateral pain of the shoulders or wrists was complained by most of the patients. 24 of the 32 patients had signs of secondary hyperparathyroidism besides beta 2M-amyloidosis. 29 patients had a carpal tunnel syndrome, 23 of whom had to be operated. beta 2M-amyloid was histochemically demonstrated in all of these 23 cases. Renal transplantation led to immediate pain relief in 3 out of 3 patients, a change of the dialysis membrane (high-flux membrane) improved chronic pain in the majority of patients.

Adult↗
PubMed1991

[Diseases associated with renal amyloidosis. Apropos of 216 cases].

Diseases associated with renal amyloidosis were evaluated in 216 patients observed between 1956 and 1989. The percentage of secondary amyloidosis decreased from 72 to 47%, where it has remained for the past 20 years, despite the clear diminution in the tuberculosis rate; this is due to its increased incidence of association with rheumatoid diseases. Dysglobulinemia was found in more than 75% of the patients with AL amyloidosis. Amyloidosis associated with this dysglobulinemia, of which 30% were myelomatous, represented more than 25% of the cases observed during the past decade and constituted, with rheumatoid arthritis, the two most common etiologies.

Adolescent↗
PubMed1991

Senile cardiac amyloidosis associated with homozygosity for a transthyretin variant (ILE-122).

Senile cardiac amyloidosis, also known as senile systemic amyloidosis, is a sporadic disease of late onset but with increasing incidence with age. Recently it has been shown in one case that amyloid deposits contained a transthyretin variant with an isoleucine for valine substitution at position 122. A second case with the transthyretin isoleucine 122 variant is reported here. This individual, who died with restrictive cardiomyopathy, was found to be homozygous for this transthyretin variant and his son heterozygous for the variant. A brother of the propositus was also homozygous for the variant but died of a cerebral vascular accident without being evaluated for amyloidosis. These studies show genetic transmission of the isoleucine-122 transthyretin variant associated with this form of cardiac amyloidosis.

Aged↗
PubMed1990

[Rapid progression of systemic amyloidosis after high-dose corticosteroid therapy in multiple myeloma].

A number of different combination regimens including high-dose corticosteroids (HDCS) have been widely used in an attempt to achieve better results for relapsed or alkylating agent-resistant multiple myeloma (MM). A major complication of these regimens is commonly said to be infection. In addition, we have had occasion to point out that a rapid progression of systemic amyloidosis can be one of serious complications of HDCS therapy in MM. The patient, born in 1940, was diagnosed of having Bence Jones (BJ) type MM in 1987. The conventional therapy including alkylating agents and interferon-alpha induced a partial remission of 22 months' duration. After the relapse, 2 courses of vincristine, adriamycin plus high-dose dexamethasone resulted in a reduction of the excreted amount of urinary BJ proteins with symptomatic improvement. However, the following clinical features indicating systemic amyloidosis occurred in succession within 2 months after beginning the therapy: submandibular swelling, giant tongue, shoulder pad sign, carpal tunnel syndrome, low voltage on ECG and recurrent diarrhea. The biopsy specimens from the submandibular gland revealed amyloid deposition. In the present case, a rapid progression of systemic amyloidosis is supposed to be attributable to the HDCS therapy. The possible mechanism of enhancement of amyloidosis by HDCS therapy is discussed.

Adrenal Cortex Hormones↗
PubMed1990

[Diagnostic difficulties in hepatic amyloidosis--clinical analysis and autopsy data of 3 cases].

Three patients with amyloidosis in whom hepatomegaly was the main sign ara presented. In all cases jaundice coexisted which is regarded as a rare sign in amyloidosis. Attention is called to the diagnostic difficulties of amyloidosis especially without a preceding clinically overt disease process. The presence of a particularly high activity of alkaline phosphatase in the serum, and focal absence of 99Tc uptake by the hepatic macrophage system in liver scintigram suggested liver tumour. However, demonstration of monoclonal protein in blood, urine or ascitic fluid, or raised level of alpha 2 globulin, should call attention to the possibility of liver amyloidosis. The authors stress the usefulness of the method of preincubation of tissue biopsy specimens in potassium hypermanganate during routine staining with Congo red for differentiation of amyloid AA from amyloid AL.

Adult↗
PubMed1990

[Echocardiographic image of cardial amyloidosis].

Heart is involved nearly in 90% of acquired, systemic amyloidosis in a course of plasmocytoma, monoclonal gammapathy, Waldenstrom's disease and others immunological disorders. In classical acquired systemic amyloidosis due to chronic bacterial infection and other inflammatory states heart involvement is observed in 54% of patients. Authors presented a case of cardiac amyloidosis coexisting with hepatomegaly and nephropathy due to plasmocytoma. Diagnosis was based on the echocardiographic examination to which the patient was sent as the unstable postinfarction angina. Echocardiographic image typical of amyloidosis revealed such changes as: concentric both ventricular hypertrophy, generalized contractility impairment, thickness of intraatrial septum and pericardium with minimal effusion. Echocardiographic diagnosis was proved by the anatomopathologic examination.

Amyloidosis↗
PubMedDate not supplied

[Secondary bladder amyloidosis. A rare cause of hematuria].

Secondary vesical amyloidosis is an uncommon pathology and few cases are described in the literature. A suddenly appearing hematuria resistant to treatment in a patient with a diagnosis of systemic amyloidosis is usually the initial sign that guides us to its diagnosis. We present a case of vesical amyloidosis in a patient with systemic amyloidosis secondary to a Rheumatoid Arthritis of length evolution, and which was diagnosed as a result of a pattern of massive incoercible hematuria.

Amyloidosis↗
PubMed1990

A noninvasive screening of systemic reactive (secondary) AA amyloidosis, based on reduced amyloid degrading activity of amyloidotic serum.

Standardization of the measurement of amyloid degrading activity (ADA) by diffusion of serum in amyloid-impregnated agar plates may either indicate or exclude with reasonable certainty the presence of systemic AA amyloidosis. In certain cases, it may obviate the need for a diagnostic biopsy. The sera of 38 patients with systemic amyloidosis were tested and compared with sera of 38 controls matched for age, serum creatinine and albumin blood levels, and with sera of 48 additional controls with the same basic diseases as the amyloidotic patients but without amyloidosis. The difference between ADA of amyloidotic and control patients was significant, with no overlap in the range of activity between the two groups. A positive correlation was found between ADA and serum albumin concentration in the nonamyloidotic matched controls but not in patients with amyloidosis. Our data do not support the view that the decline in ADA of sera of amyloidotic patients is due to hypoalbuminemia.

Adipose Tissue↗
PubMed1990

[Secondary amyloidosis in chronic polyarthritis].

Systemic secondary amyloidosis was a post-mortem finding recorded from 24% of patients with classical chronic polyarthritis. In 80 to 100% of all cases, amyloidosis was present in heart, thyroid gland, kidneys, adrenal gland, pancreas, spleen, and gastro-intestinal canal. It was recordable, in 50 to 70% of all cases, from liver, aorta, lungs, and lymph nodes. Nerves, muscles, skin, and synovial membrane were less often affected (10 to 40% of all cases). No amyloidosis was recorded at all from the brain. Amyloid depositions differed greatly in intensity by organs. The most massive deposits were found in the gastrointestinal tract, kidneys, adrenal glands, thyroid gland, and spleen. Amyloid depositions were moderate in heart, pancreas, lungs, and liver. They were extremely low in lymph nodes, muscles, nerves, synovial membrane, and skin. The author assumes amyloid deposition to be associated with increase in arterial or venous concentrations of circulating amyloid precursors. Frequency and intensity of amyloid depositions in different organs may be linked to blood supply to the latter. The higher the minute volume of a given organ, the more strongly pronounced is the amyloidosis in it. In the context of amyloidogenesis, consideration should be given, as well, to locally delimited factors, such as organ motility.

Adult↗
PubMed1989

[Noninvasive diagnosis of cardiac involvement by technetium-99m-pyrophosphate (Tc-99m PYP) myocardial scintigraphy in 2 cases of familial amyloid polyneuropathy and 1 case of secondary amyloidosis].

To validate the significance of technetium-99m-pyrophosphate (Tc-99m PYP) myocardial scintigraphy in diagnosing cardiac amyloidosis, 2 patients with familial amyloid polyneuropathy (FAP) and 1 patient with amyloidosis secondary to chronic rheumatic arthritis were studied. All three patients had echocardiographic abnormalities, which were increased wall thickness of the interventricular septum and the left ventricular posterior wall, and granular sparkling appearance in the septum. In 2 patients with FAP, abnormal myocardial uptake of Tc-99m PYP was diffusely detected in Tc-99m PYP SPECT. In the remaining 1 patient with secondary amyloidosis, however, Tc-99m PYP SPECT showed no abnormality, although we had confirmed the presence of myocardial amyloid deposits (type AA amyloid protein) with high amount in the histological examination. Thus, these results indicate that Tc-99m PYP scintigraphy may have a limitation in detecting cardiac involvement in secondary amyloidosis although it is useful in FAP.

Adult↗
PubMed1989

[Amyloidosis: new facts, disputable and unresolved problems].

The proofs of the heterogeneity of both the amyloid fiber protein and its precursor (serum and local) are, in the author's opinion, the most important among new advances in the amyloidosis problem. These proofs should become a basis for the study of disputable and unsolved problems in the pathogenesis of various types and forms of amyloidosis and development of its classification. The author suggests the schemes of the pathogenesis of AA- and AL-amyloidosis, indicated main and secondary features of a tentative amyloidosis classification.

Amyloidosis↗
PubMed1989

Endobronchial appearance of tracheobronchial amyloidosis. A case report and suggested classification.

Tracheobronchial amyloidosis with the co-existence of submucosal plaques and tumour-like masses is reported. The subdivision of tracheobronchial amyloidosis into submucosal plaques and tumour-like masses is questioned since there appears to be no difference in their clinical presentation and pathological appearance. It is suggested that lower respiratory tract amyloidosis should be classified into tracheobronchial, nodular parenchymal and diffuse alveolar septal amyloidosis.

Amyloidosis↗
PubMed1986

[Amyloidosis and familial Mediterranean fever].

Familial Mediterranean Fever (F. M. F.) is an autosomal recessive disorder occurring most commonly in Sepharadi Jews and Armenians. Two phenotypic features characterize the disease: brief episodic febrile attacks of peritonitis, pleuritis or synovitis recurring from childhood or adolescence and the development of systemic amyloidosis. Attacks are accompanied by striking elevations of acute phase proteins, including serum amyloid A protein. The amyloidosis of Familial Mediterranean Fever is of the AA type, and manifest clinically as a nephropathy that passes through proteinuria, nephrotic and uremic stages to renal death. Although there is ethnic variation in the incidence of amyloidosis of F. M. F. in our patient population--predominantly Sepharadi Jews of North African extraction--an amyloidotic death at an early age is their genetic destiny. Since the introduction in 1972 of colchicine to prevent the febrile attacks, the drug has been proven and become the main stay of therapy. Today, colchicine has been shown to be effective in preventing amyloidosis as well as the febrile attacks in Familial Mediterranean Fever. End stage renal disease is not the end of the road for patients with F.M.F. because of improving outlook for dialysis and renal transplantation in these patients.

Amyloidosis↗
PubMed1987

[Amyloidosis in hemodialysis patients].

Amyloidosis in long-term haemodialysis patients mainly involves the osteo-articular system. It is held responsible for carpal tunnel syndrome, chronic arthralgia and various types of arthropathy, chronic synovitis and tenosynovitis, haemarthrosis, subacute polyarthritis and destructive arthropathies of the limbs and spine. Radiologically, amyloidosis may appear as bone cavities, particularly visible in the hips and wrists. Its frequency increases with the duration of haemodialysis. Biochemically, amyloidosis consists of beta 2-microglobulin (beta 2-M). This protein accumulates in uraemic patients under dialysis and seems to play a major role in the pathogenesis of amyloid deposits. The accumulation is due to renal impairment, being maximum in anuric patients. However, the unsatisfactory clearance of beta 2-M by dialysis methods also contributes to its retention: the production and elimination of beta 2-M seems to vary according to the extrarenal clearing technique. These data suggest that improvements in clearing techniques will eventually prevent dialysis amyloidosis.

Amyloidosis↗
PubMed1988

[Ankylosing spondylitis with type AA amyloidosis. 6 cases].

The authors have studied 6 cases of systemic AA amyloidosis associated with ankylosing spondylitis. Renal failure occurred in all patients a mean of 19 years after the clinical onset of the rheumatic disease. Three patients progressed rapidly (between 3 months and 3 years) to end-stage renal failure. Such an outcome did not depend upon early onset of the renal impairment, degree of inflammation or treatment with colchicine. All patients were alive 2 to 10 years later, and this confirms a better prognosis than with AL amyloidosis. The utility of combining Wright's permaganate reaction with immunological methods to characterize the amyloid deposits was also confirmed. It is concluded that amyloidosis is a rare complication of ankylosing spondylitis and probably depends on a genetic predisposition. The possibility of amyloidosis should be kept in mind when proteinuria or renal failure appear in the course of ankylosing spondylitis.

Adolescent↗
PubMed1987

Secondary amyloidosis in normal and immunocompromised mice infected with Schistosoma japonicum.

Secondary amyloidosis developed in various tissues of immunologically intact and T-cell deprived CBA mice infected with Schistosoma japonicum as indicated by histological, histochemical and immunofluorescence techniques. Mice deprived of T-cells were most susceptible both in terms of the timing of the onset of amyloidosis, first noted 6 weeks post-infection, and in terms of the incidence, reaching 100% 12 weeks post-infection. Amyloidosis developed later in the infection in immunologically intact mice, the incidence was lower and there was less extensive replacement of normal tissue compared with that seen in T-cell deprived mice. Amyloid-related changes were most noticeable in the spleen where there was replacement of both follicular and non-follicular tissue. In the liver, amyloid deposits caused hepatocellular atrophic changes and in the kidney, glomerular and interstitial deposits were observed. The relationships between immunological reactivity and amyloidosis during schistosome infection is discussed.

Amyloidosis↗