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PubMed1986

[Amyloidosis of the pancreatic islets and diabetes mellitus].

Pancreas was examined in 136 patients who died at the age of 7 to 89 years of various diseases including 22 with diabetes mellitus. Amyloidosis of its islands was observed in 9 patients (aged 49 and over); 6 out of them suffered from diabetes mellitus. Number of islands with amyloidosis and amyloid quantity were determined morphometrically. Glucagon-producing A-cells and insulin-producing B-cells in the islands not involved in amyloidosis were counted in sections impregnated by Grimelius. It is found that the development of diabetes is determined not only by the islands amyloidosis but by the quantitative domination of A-cells over B-cells in the islands without amyloidosis as well being the manifestation of aging processes.

Adolescent↗
PubMed1987

[A case of amyloidosis associated with a solitary micromolecular plasmocytoma].

Amyloidosis associated with myeloma and its current classification are examined. The clinical example is reported of a patient with isolated micromolecular plasmocytoma in whom the amyloidosis appeared during the remission of the primary disease. The patient was not seen until a late stage when he was admitted to hospital with severe cardiac decompensation. Echocardiography revealed the location to be the cardiac muscle. The increase in left ventricular mass revealed by the ECG was in contrast with the low ECG voltages. Biopsy showed involvement of the oral cavity. The condition resisted all treatment and the patient died a few months after the onset of the amyloidosis. The view that amyloidosis associated with plasma cell dyscrasias should be classified with primary amyloidosis is supported.

Amyloidosis↗
PubMed1987

[Origin of macular amyloidosis. Apropos of 160 cases].

This study is based on clinical findings in 160 patients with skin-limited amyloidosis: 151 macular, 8 biphasic and 1 lichen amyloidosis. 82.5 p. 100 of the patients were women and 17.5 p. 100 were men. 95 p. 100 of the patches were located in the back and 64 p. 100 in the suprascapular region. No less than 88 p. 100 of the patients had pruritus on the patches or on other sites. 75 p. 100 had allergic diseases or a history of allergy, and 45 p. 100 had non-allergic pruriginous diseases. Signs of scratching were noted in most of the patients. The principal causes of pruritus were dermographism, photodermatitis and dermatitis. Macular amyloidosis is now considered as secondary to scratching motivated by pruriginous diseases and not as a primary condition as hitherto described. The term "scratch amyloidosis" is proposed for macular and lichenoid amyloidosis, and the existence of a "preamyloidotic" histological stage is described.

Adult↗
PubMed1986

Amyloidosis presenting in the lower respiratory tract. Clinicopathologic, radiologic, immunohistochemical, and histochemical studies on 48 cases.

We studied 48 cases of amyloidosis localized to the lower respiratory tract. Fourteen cases were classified as tracheobronchial amyloidosis. Twenty-eight cases showed solitary or multiple nodules, and six cases had a diffuse interstitial parenchymal pattern. Almost all patients with tracheobronchial and diffuse interstitial amyloidosis had respiratory symptoms (usually dyspnea), whereas most with nodular amyloidosis were asymptomatic. Nodular parenchymal and, less frequently, tracheobronchial amyloidosis had contiguous cellular infiltrates of plasma cells, lymphocytes, and giant cells. Immunohistochemical staining of specimens from 18 cases showed these plasma cells to be polytypic, except for two tracheobronchial lesions that had a disproportionate number of lambda light chain-bearing plasma cells. Permanganate oxidation of specimens from 19 cases showed patterns in keeping with immunoglobulin-derived amyloid in 12. Local deposits of amyloid in lung may arise from deposition of circulating precursor proteins.

Adult↗
PubMed1986

Nodular primary cutaneous amyloidosis. Isolation and characterization of amyloid fibrils.

A case of nodular cutaneous amyloidosis and Sjögren's syndrome occurred in a 63-year-old woman. Nodules had been seen for the past ten years and Sjögren's syndrome had accompanied amyloidosis for the last three years. The concomitant occurrence of nodular cutaneous amyloidosis and Sjögren's syndrome may not be by chance, since four of 12 cases of nodular cutaneous amyloidosis that have been reported to date in Japan were in patients with both amyloidosis and Sjögren's syndrome. The amyloid deposits in the tissue were stained with anti-lambda light-chain amyloid antibody. Amyloid fibrils were purified from the skin lesions in this patient and were characterized biochemically, immunologically, and ultrastructurally. The results indicated that the amyloid fibrils consisted of 29,000-, 20,000-, and 17,000- dalton peptides, the 29,000-dalton peptide of which was shown to react with the lambda light chain of immunoglobulin by immunoblot study.

Amyloid↗
PubMed1985

Systemic amyloidosis: a review with emphasis on pathogenesis.

Our knowledge about the composition of the deposits in amyloidosis has increased considerably during the last decade. Three different protein groups have been shown to form fibrils in systemic amyloidosis, namely monoclonal immunoglobulin light chains in primary and myeloma-associated amyloidosis, protein AA in secondary amyloidosis and prealbumin in the familial and senile forms of systemic amyloidosis. This review deals with known and postulated pathogenetic mechanisms involved in the creation of fibrils from these proteins.

Aging↗
PubMed1986

[Value of aspiration biopsy of subcutaneous fat in amyloidosis].

Fine-needle aspiration of subcutaneous fat (FNAF) was performed in 24 patients, 12 with previously diagnosed amyloidosis presenting with proteinuria or nephrotic syndrome, and 12 presenting a nephrotic syndrome without amyloidosis on renal biopsy. FNAF was positive in 10 of 12 patients with amyloidosis (sensitivity: 83%) and negative in 12 of 12 patients with non-amyloid nephrotic syndrome (specificity: 100%). Considering a 2.5 to 10% prevalence of amyloidosis in adult patients with proteinuria or nephrotic syndrome, a positive FNAF is diagnostic of amyloidosis, and a negative FNAF rules out the diagnosis with a probability of 98 to 99%. FNAF is a simple and safe method which can be useful in patients who cannot undergo a renal biopsy.

Adipose Tissue↗
PubMed1978

[Electrophysiological study of a Portuguese case of amyloidosis with conduction disorders].

Cardiac involvement in the course of familial Portuguese amyloidosis, as apart from the other primary amyloidosis, is characterised by the early and wide-spread intracardiac conduction disorders, and the contrasting late presentation of the clinical signs. A case is presented of Portuguese amyloidosis which was typical from the viewpoint of the neurological disorders, the familial characteristics, and the positive biopsy; the main conduction defects found in primitive amyloidoses are also recalled. The patient described had for many years suffered from first degree heart block, and then presented with lipid changes with syncopal attacks which led to electrophysiological investigation of the conduction defect, no similar example of which has been found in the literature. The severity and widespread nature of the disorders which were found, together with the localisation of a sub-His block led us to implant the pacemaker. We have only found two other patients who had implants for disorders of conduction secondary to cardiac amyloidosis. Emphasis has been laid on the importance of this investigation which, when it leads to the positioning of a pacemaker, should avoid the onset of syncopal attacks and sudden death which together constitute one of the primary causes of mortality in primary amyloidosis. The length of follow-up in our case has been 14 months (April 1977).

Adult↗
PubMed1977

Incidence of renal amyloidosis in pulmonary tuberculosis.

Incidences of renal amyloidosis were studied in patients who were in various stages of pulmonary tuberculosis and a three year follow-up gave some opportunity to study the effectiveness of anti-tuberculosis treatment on the course of renal amyloidosis. It was concluded that 9 to 11 per cent of all patients with pulmonary tuberculosis will eventually develop proteinuria due to renal amyloidosis after a certain period of time. It has been postulated that once amyloidosis has extensively involved the kidneys, anti-tuberculous treatment will not cause any regression in the course of renal amyloidosis.

Adolescent↗
PubMed1984

[M-mode and two-dimensional echocardiography of 7 cases of cardiac amyloidosis].

Although rare, cardiac amyloidosis is the commonest cause of infiltrative myocardiopathy. The diagnosis may be suspected clinically in patients with mainly right ventricular failure of sudden onset. The aim of this study was to assess the diagnostic value of M-Mode and 2D echocardiography in this condition. Seven cases of cardiac amyloidosis were studied. Biventricular hypertrophy, usually more severe on the left side with reduction in size of the left ventricular chamber, was observed in all cases. Parameters of systolic and diastolic function were abnormal. A significant pericardial effusion was demonstrated in 3 patients. 2D echocardiography also allows evaluation of the myocardial structure: in 3 cases the whole of the left ventricular myocardium seemed granular, sparkling and abnormally echogenic. In patients with cardiac failure these appearances are very suggestive of amyloidosis, especially when the ECG shows low voltage complexes and pathological Q waves. In 3 other patients, this abnormal echogenic myocardial appearance was observed only in the interventricular septum, which is much less suggestive of cardiac amyloidosis. In conclusion, in patients with cardiac failure with cardiomegaly and a low voltage ECG, echocardiographic findings of hypertrophic cardiomyopathy (only rarely with dilatation) and hypokinetic wall motion are suggestive of cardiac amyloidosis, especially when the myocardium has a granular, sparkling appearance.

Aged↗
PubMed1983

AA protein-related renal amyloidosis in drug addicts.

Reports of renal amyloidosis occurring among narcotic addicts have been limited, for the most part, to case reports. In a prospective survey of 150 addicts examined at autopsy in the Office of the Chief Medical Examiner of the City of New York, 7 cases of renal amyloidosis were found. Immunohistologic examination demonstrated that in all of the 7 cases, the amyloid was AA protein-related. The amyloid extracted from the kidneys of two addicts and analyzed biochemically did not differ from the AA amyloid secondary to chronic infectious and inflammatory diseases. The combined data of previous reports and the present survey demonstrate that addicts who are subcutaneous users with skin infections most frequently develop amyloidosis. Our data demonstrating renal amyloidosis in 26% of addicts with chronic suppurative skin infections suggest that such addicts are at high risk for the development of amyloidosis.

Adult↗
PubMed1982

Gastrointestinal bleeding and amyloidosis.

Although gastrointestinal bleeding is well known in patients with amyloidosis, when it occurs as the initial or sole overt manifestation of the disease it is much rarer and frequently causes diagnostic difficulty. This subject is reviewed in the context of a patient who underwent multiple invasive and noninvasive examinations to achieve this diagnosis. Both upper and lower gastrointestinal bleeding of all degrees has been shown to occur with amyloidosis. It appears that diffuse involvement of the gastrointestinal tract is much more common than a specific lesion. Our patient was ultimately found to have multiple waxy amyloid plaques and extremely friable jejunal mucosa at endoscopy. Radiographically, amyloidosis may cause diffuse thickening of the mucosal folds, a finding that carries a broad differential diagnosis. Arteriography, although not used frequently as a diagnostic modality in amyloidosis, may reveal luminal irregularities, truncation, and diffuse attenuation of the vasculature. The treatment of amyloidosis thus far has been unrewarding and difficult. Several new therapeutic measures which appear promising are discussed.

Aged↗
PubMed1981

Relationship of hypothyroidism to diabetes mellitus, renal amyloidosis, and thrombosis in purebred beagles.

Review of 484 records for colony Beagles revealed an association between hypothyroidism and diabetes mellitus. The average time between first notation of hypothyroidism and later development of diabetes mellitus was 2.8 years. An association was also made with hypothyroidism, renal amyloidosis, and thrombosis. Hypothyroidism was significantly related to thrombosis, thrombosis was significantly related to renal amyloidosis, but hypothyroidism and renal amyloidosis were not significantly related. Of 62 hypothyroid dogs, 11 were diabetic and 7 others had thrombosis. Six hypothyroid dogs had renal amyloidosis, 4 of which had thrombosis. One dog had renal amyloidosis and thrombosis in the absence of hypothyroidism. There does not appear to be an association with any of the lesions and previous low-dose, whole-body gamma, or sham irradiation.

Amyloidosis↗
PubMed1982

Amyloidosis masquerading as inflammatory bowel disease with a mass lesion simulating a malignancy.

Our patient, who was known to have multiple myeloma, presented with weight loss, rectal bleeding, and a barium enema study suggestive of a colitis with a mass lesion. Colonoscopy with biopsy revealed the mass to be large mucosal folds infiltrated with amyloidosis. Amyloidosis has been reported to mimick malignancy, mainly by tumorous deposits in the stomach and less commonly in the small and large bowels. Gastrointestinal surgery in patients with amyloidosis potentially may have undesirable consequences due to failure of anastomotic suture lines and subsequent sepsis (6, 11, 17, 18). The knowledge that amyloidosis may be associated with multiple myeloma and an appreciation of the wide range of gastrointestinal roentgenographic findings in patients with amyloidosis should prompt the clinician to obtain endoscopic and biopsy evaluation of these patients.

Adult↗
PubMed1995

Outcomes of thirty-four rheumatoid arthritis patients with renal amyloidosis, including twelve given alkylating agents.

We reviewed 34 cases of rheumatoid arthritis with biopsy-proven renal amyloidosis. Mean age was 57.1 +/- 13 years and mean duration of rheumatoid arthritis was 13.7 +/- 8.2 years. Renal function tests done at the time of diagnosis of amyloidosis were available for 32 patients and showed renal failure in 16. Twelve patients received an alkylating agent. Twenty patients (59%) died, after a mean interval of 25.7 +/- 25.1 months since the diagnosis of renal amyloidosis; 19 of these patients (95%) had renal failure. Seven of the 14 survivors and four of the 11 survivors with impaired renal function were given an alkylating agent; mean follow-up since the diagnosis of renal amyloidosis was 77.2 +/- 58.7 months. At completion of the study, renal function was normal in only three patients, all of whom received an alkylating agent. Our data confirms the bleak prognosis of renal amyloidosis in rheumatoid arthritis patients and suggests a need for randomized trials designed to evaluate the efficacy of alkylating agents in this condition.

Adult↗
PubMed1995

Hyposplenic blood picture in systemic amyloidosis. Its absence is not a predictable sign for absence of splenic involvement.

We studied a total of 61 cases of systemic and senile amyloidosis to evaluate the significance of a "hyposplenic blood picture" (the presence of numerous Howell-Jolly bodies) in these patients and to correlate its presence with the pattern and severity of the splenic involvement. To ascertain whether this peripheral blood picture is prevalent with a certain type of amyloidosis, we classified all cases by immunostaining with a panel of antibodies against AL amyloid (kappa and lambda light chains), serum amyloid-associated protein, prealbumin, beta-2 microglobulin, and amyloid p component. Based on immunostaining results, all cases were classified as AL (31 cases), AA (8 cases), or senile (prealbumin-positive, 22 cases) amyloidosis. Howell-Jolly bodies were identified in six patients with amyloid L amyloidosis; of these, four cases had diffuse splenic cord involvement, one had a follicular pattern, and one had a vascular pattern. Only one of these patients had a typical hyposplenic blood picture. This patient had far-advanced diffuse splenic involvement. The remaining five patients had rare to few Howell-Jolly bodies. In addition, 12 other patients had diffuse splenic involvement with no Howell-Jolly bodies present. This study concludes that even when there is advanced diffuse replacement of splenic cords with amyloid on light microscopy, the "pitting" function of the spleen appears to be preserved in most cases. The absence of a hyposplenic blood picture cannot be equated with normal splenic cord histology in patients with systemic amyloidosis.

Amyloidosis↗
PubMed1993

Primary amyloidosis--involving principally intrarenal blood vessels.

We analysed renal biopsies from 34 nephrotic patients with renal amyloidosis, seven with primary form kappa chain (AL amyloidosis) and 27 with secondary amyloidosis associated with the other chronic diseases. Renal biopsy specimens were analysed using optical and immunofluorescence microscopy. The extent of amyloid deposits was graded from 0 to + + + +. Intrarenal blood vessel deposits were more prominent than intraglomerular in five of seven patients with AL amyloidosis, while they were identical in one, and in one, intraglomerular amyloid deposits were dominant. The results were different in the group of patients with secondary amyloidosis: a lower degree of intrarenal blood vessels deposition than glomerular was noted in 22 of 27 cases, the degree of deposition was identical in 4 of 27 cases and more expressed blood vessel deposition was present in only one case. Granular or combined deposits (granular+linear) were found on immunofluorescence microscopy in primary form, but the dominant form of deposition was amorphous in secondary amyloid.

Adolescent↗
PubMed1993

Survey on spontaneous systemic amyloidosis in aging mice.

The incidence of systemic amyloidosis in CD-1 mice (Charles River, caesarian derived) obtained from long term studies over more than a 15-year period is reported. The survey included samples of visceral organs, peripheral and central nervous tissues, bone and bone marrow. The total incidence in all mice of this survey did not show any clear evidence of a difference between males and females. Amyloidosis deposits were mainly seen in the stomach (glandular), heart, small intestines, kidney, liver, spleen, thyroid, parathyroid, adrenals, salivary glands and ovaries, but not in the brain, spinal cord, bone or bone marrow. The survey showed that amyloidosis in CD-1 mice was spontaneous, systemic and it is age-related. Amyloid deposition was extracellular and it stained positively with Congo Red and also stained positively with Oil Red O and Alcian blue. In general, amyloidosis in CD-1 mice, was higher in comparison with B6C3F (cross between C57BL/C6 NCRLB and C3H/HEN NCRLB, (bred by Charles River), CFLP strain (hysterectomy derived strain of Swiss origin) and MAGF: TIF (SPF). This survey also showed that spontaneous systemic amyloidosis in CD-1 mice, was one of the major factors contributory to death in aging CD-1 mice.

Aging↗