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PubMed1983

[A case of amyloidosis of the ureter].

This is a case report of a localized amyloidosis of the ureter presented with gross hematuria. Amyloidosis of the ureter is a rare entity and 21 cases have been collected from the Japanese and English literature. It is believed to be difficult to diagnose before surgery and almost all the cases were treated by nephrectomy. After malignancy was denied, autotransplantation combined with psoas hitch procedure was carried out in order to preserve the kidney. This is the second case of ureter amyloidosis treated by autotransplantation. Amyloidosis was confirmed with Congo red stain and electronmicroscopy of which characteristics is discussed in detail.

Amyloidosis↗
PubMed1984

Chronic alveolar hydatidosis and secondary amyloidosis: pathological aspects of the disease in four strains of mice.

Amyloidogenic potentials of alveolar hydatid cyst, the larval stage of Echinococcus multilocularis, were studied in BALB/c, C57BL/6J, CBA/J and A/JAX strains of mice. To determine strain susceptibility and sensitivity of various soft organs to amyloidosis, the intensity of inflammation and mean weights of the larval cyst masses from each host strain were correlated at various time intervals postinfection (p.i.) with the onset and amount of splenic, hepatic and kidney amyloid deposits. The onset and incidence of amyloidosis in these four host strains corresponded with the intensity of hepatic inflammation and not with the load of the parasite biomass. Hydatid mice spleens were more sensitive to amyloid deposition than livers or kidneys. Splenic amyloid deposits appeared at 6 weeks p.i. in BALB/c and C57BL/6J mice and at 8 and 12 weeks p.i. in CBA/J and A/JAX mice, respectively. The latter strain was relatively resistant to hydatid-induced amyloidosis. At 12 weeks p.i., amyloid-mediated changes in the spleens, livers and kidneys of the susceptible host strains were much more severe than in the A/JAX strain. In conclusion, alveolar hydatid cyst is highly amyloidogenic and provides a promising substitute for the study of induction and pathogenesis of secondary amyloidosis.

Amyloidosis↗
PubMed1984

The failure of ascorbic acid therapy to alter the induction or remission of murine amyloidosis.

It has been claimed that ascorbic acid enhances the in vitro degradation of AA amyloid fibrils. This raises the possibility that ascorbic acid may be of benefit in systemic AA amyloidosis, a condition with serious morbidity and mortality for which there is as yet no specific treatment. The effect was therefore tested of oral or injected supplements of ascorbic acid on the induction of AA amyloidosis in mice. Amyloid was induced either by repeated injections of casein or by injection of 'amyloid enhancing factor' and silver nitrate. Mice with established amyloidosis were also treated with additional ascorbic acid. Despite the fact that plasma ascorbic acid levels were significantly higher in orally supplemented mice than in controls there was no demonstrable effect on the induction, the extent and distribution or the progression of amyloidosis.

Amyloid↗
PubMed1984

Senile systemic amyloidosis.

The senile amyloidoses comprise a heterogeneous group of disorders with deposition of amyloid in a variety of tissues. Most of these amyloidoses are localized to one tissue. It has been shown previously that the amyloid fibrils in one form of senile amyloidosis affecting the heart contains a prealbumin-related protein, ASc1. It is shown in this paper by immunohistochemical study using a specific anti-protein ASc1 antiserum that this type of amyloidosis, previously called senile cardiac amyloidosis, is a systemic disease with amyloid deposits in many organs. The designation senile systemic amyloidosis is proposed for this disease, which differs from other systemic amyloidoses in distribution of amyloid deposits.

Aged↗
PubMed1984

[Secondary amyloidosis in white Peking ducks].

In a research institute numerous cases of amyloidosis were observed in 12 of 70 white Peking ducks kept in cages with wire bottoms. All 12 ducks also suffered from chronic inflammed alterations in the sole of foot zone (bumble-foot). A second flock of 40 white Peking ducks supplied with equivalent feed but kept outdoors showed neither signs of bumble foot nor amyloidosis. Likewise, after the healing of the bumble feet per adjustment of the wire bottoms (installing a wider footing area) no new cases of amyloidosis were noticed among the ducks kept in cages. Histologically deposits of amyloid were found in the liver, which was highly affected, the kidney, spleen, stomach, small and large intestines as well as pancreas. The fact that amyloidosis only appeared under certain circumstances indicates the presence of a secondary form of this illness.

Amyloidosis↗
PubMed1984

[Differential diagnosis of round lung lesions--isolated tumor-like amyloidosis].

One case of isolated tumorlike amyloidosis of the lung is reported. The patient was found by mass radiographic survey. Before operation the histological diagnosis was unknown. The specimen of lung from thoracotomia showed tumorlike amyloidosis and independent from amyloidosis sarcoid granulomatosis of uncertain cause. Isolated tumorlike amyloidosis of the lung is a very rear diagnosis. Thus diagnosis before operation is very difficult. In case of circular focus of undetermined etiology surgical intervention is indicated to avoid delayed resection of bronchial carcinoma.

Amyloidosis↗
PubMed1983

[Amyloidosis and its bronchopulmonary localizations. Apropos of 3 cases].

Amyloidosis of the lower respiratory tract is a rare condition. We report three new cases and we review the literature. Tracheobronchial amyloidosis is the most frequent form. The diagnostic is made by fiberoscopy and biopsies with a risk of bleeding. Our patients were usually treated with intermittent bronchoscopic resections. In one of the three cases reported, desobstruction of the large airway was performed with laser. Nodular pulmonary amyloidosis is usually asymptomatic, and surgical resection results in cure in all cases. Diffuse pulmonary amyloidosis is very unusual and fatal.

Adult↗
PubMedDate not supplied

[Cardiac amyloidosis. Report of 11 autopsy cases and review of the literature].

The authors report 11 cases cardiac amyloidosis discovered on autopsy. The routine examination of the artrial of 20 patients over the age of 60 revealed the presence of these deposits in 10 per cent of cases. Three cases of secondary amyloidosis were discovered with minimal cardiac involvement. There was one case of senile amyloidosis and five cases of primary amyloidosis, two of which were associated with multiple myeloma. The clinical manifestations of these last six cases were cardiac decompensation (congestive in one case) and angina with normal coronary arteries, but involvement of the small vessels. Two cases of anterior pseudo-sequelae are reported and there were 4 cases of disturbances of intraventricular conduction and one case of atrial fibrillation. Amyloid deposits are rarely localized to the heart alone. Associated immunological disorders were present in 3 of the 6 cases. The authors compare this series to a review of the literature.

Adult↗
PubMed1983

[Primary amyloidosis associated with early gastric carcinoma(IIb like IIa type) diagnosed by preoperative gastric biopsy--a case report].

A case of primary amyloidosis associated with intramucosal adenocarcinoma of the stomach, diagnosed by preoperative biopsy, is reported. A 77-year-old man was admitted to our hospital with complaints of vertigo and general fatigue. X-ray and endoscopic examination revealed a IIb-like IIa type protruded lesion on the cardia of the stomach. A biopsy specimen from the protruded lesion disclosed well differentiated tubular adenocarcinoma and amyloid deposition. Partial gastrectomy was performed on August, 18, 1981. Upon histological study, a diagnosis of primary amyloidosis associated with intramucosal well differentiated tubular adenocarcinoma was made. We suggest that the histogenesis of this gastric carcinoma was related to the gastric lesion due to primary amyloidosis. Careful review of the Japanese literature disclosed that ours is the first case report of primary amyloidosis associated with early gastric carcinoma diagnosed by pre-operative gastric biopsy.

Adenocarcinoma↗
PubMed1978

Amyloidosis secondary to drug abuse and chronic skin suppuration.

Amyloidosis has not been included in the wide spectrum of medical complications associated with drug abuse. Chronic visceral infection is a recognized cause of amyloidosis, but chronic suppuration of the skin is not a well-appreciated cause of this condition. Furthermore, to our knowledge, chronic skin suppuration secondary to parenteral drug abuse has not been reported as a cause for systemic amyloidosis. We report a case in which subcutaneous injections of narcotic tablets led to chronic skin suppuration and systemic amyloidosis.

Abscess↗
PubMed1978

The cyclic hematopoietic dog: a model for spontaneous secondary amyloidosis. A morphologic study.

Spontaneous amyloidosis was found in dogs affected with hereditary cyclic hematopoiesis (CH dogs). Early perifollicular deposits of amyloid were observed in the spleens of 15-week-old CH dogs. By the 24th week, amyloid deposits were also found in the liver, kidneys, pancreas, adrenals, and small intestine; the incidence of the condition rose to more than 90%. The visceral involvement and the histologic characteristics of amyloid deposition closely resemble those of the secondary form in humans. A transient lymphoid hypoplasia was noted in the spleens of neonates and pups. This abnormality did not appear to be related to exogenous conditions. In young adult dogs, the initial hypoplastic characteristics were replaced by enlarged marginal zones in the follicles of the spleen, composed of pyroninophilic cells and, in a later stage, of PAS-positive cells. These cellular changes preceded the amyloid deposition. Due to the characteristic cyclic neutropenia of the hereditarily transmitted hematologic syndrome, most CH dogs experience episodes of infectious diseases, although the episodes of infection may be separated by long periods of relatively good health. This may provide the underlying antigenic stimulation which triggers the process of amyloid deposition. However, the lag period for the onset of amyloidosis is extremely short and the type of infections is not considered a predisposing factor for amyloid deposition. It is possible that a peculiar sensitivity of the lymphoid system in the CH dog would facilitate the development of widespread amyloidosis. Since the sequence of splenic lymphoid hypoplasia, follicular activation, and amyloid deposition associated with age are consistently repeated, the CH dog may be a suitable animal model for the study of the pathogenesis of secondary form of amyloidosis in humans.

Age Factors↗
PubMed1980

Amyloidosis in immunoblastic lymphadenopathy.

A case of immunoblastic lymphadenopathy and systemic amyloidosis in a 21-year-old man is presented. The basic defect in immunoblastic lymphadenopathy is believed to be an abnormal immune reaction, most probably of the B cell system. Although systemic amyloidosis is common in some disorders of the B cell system, no cases of systemic amyloidosis complicating immunoblastic lymphadenopathy have been previously reported. Immunoblastic lymphadenopathy includes elements known to be associated with amyloidosis and we believe that this association is not merely coincidental.

Adult↗
PubMed1981

Bullous amyloidosis.

The distribution pattern of amyloid deposition varies greatly in systemic amyloidosis. In primary and myeloma-associated amyloidosis, skin manifestations are common. Three patients with myeloma-associated amyloidosis had bullae or signs of increased skin fragility caused by cleavage in extensive dermal amyloid deposits. The bullae in bullous amyloidosis are, therefore, unique since they are intradermal, not subepidermal.

Aged↗
PubMed1977

[New method of differential histological diagnosis of different forms of amyloidosis].

A new method for morphological diagnosis of various forms of amyloidosis based on the use of a hot buffered hexamine-silver solution is suggested. Its potentials are illustrated on 37 cases of primary, hereditary, and secondary human amyloidosis as well as experimental amyloidosis. The proposed method permits morphological differential diagnosis of amyloidosis in those cases where other methods failed to do it.

Amyloidosis↗
PubMed1981

[Dramatic aggravation of renal amyloidosis after surgery. Three cases (author's transl)].

A few cases of improvement in secondary renal amyloidosis following surgery (in particular, removal of the amylogenic foci) have been published, but cases of aggravation are much more numerous. The authors report on three patients whose renal function deteriorated dramatically after extra-renal surgery (pneumonectomy, aortic valve replacement, mitral valve replacement). None of the usual precipitating factors, such as DIC, cardiovascular collapse, sepsis or renal vein thrombosis, could be detected, but two patients had been under extracorporeal circulation. Such accidents appear to be unpredictable and irreversible. They can be seen in primary or secondary amyloidosis and whether or not surgery involves an amylogenic focus. Indeed, in two of their patients the diagnosis of amyloidosis was unknown before the operation. This suggests that in patients with suspected amyloidosis no major surgical operation should be undertaken without prior renal biopsy.

Adult↗
PubMed1980

Primary cardiac amyloidosis: A case presentation.

A young man with primary cardiac amyloidosis presented clinically as a case of 'restrictive' cardiomyopathy with an initial diagnosis of constrictive pericarditis, probably of tuberculous origin. Cardiac catheterization showed features compatible with restrictive cardiomyopathy or constrictive pericarditis but pericardial biopsy was negative. Percutaneous right ventricular endomyocardial biopsies established amyloid infiltration of the myocardium, but rectal, tongue and liver biopsies were all negative for amyloidosis. Bence-Jones protein was absent from the urine. Serum electrophoresis, immunophoresis, and bone marrow examination all failed to detect possible multiple myelomatosis as a cause of secondary amyloidosis. Respiratory function tests showed a restrictive picture, probably secondary to chronic congestive cardiac failure. Thus, this case is the second example of primary cardiac amyloidosis in the world literature which has been dianosed by right ventricular endomyocardial biopsy. Phonocardiographic features of early aortic valve closure are reported for the first time in amyloid restrictive cardiomyopathy. Since tuberculous constrictive pericarditis is an important cause of cardiac disease in this country, with effective therapy, the authors stress more frequent use of endomyocardial biopsy as a safe diagnostic tool to exclude other similar disease states. The patient was offered the possibility of a cardiac transplantation in view of the localized nature of his disease, but refused. He now continues to respond unsatisfactorily to antifailure therapy with, presumably, a poor prognosis.

Adult↗
PubMed1995

[Amyloidosis associated with long-term dialysis].

Dialysis amyloidosis is a frequent complication encountered in patients receiving long-term hemodialysis. These amyloid deposits are composed mainly of an insoluble fibrillar material that consists of beta 2-microglobulin (beta 2-m). While this fibrillar protein is a major component of these deposits, numerous other substances have been identified in the amyloid deposits; e.g., amyloid P component, calcium, glycosaminoglycans, chondroitin sulfate, hyaluronic acid, collagen, protease inhibitors, k-chain protein, ubiquitin, apolipoprotein E and macrophages. Hypotheses on the pathogenesis of amyloidosis have suggested roles for each of these factors. The pathogenesis of beta 2-m-related amyloidosis is probably multifactorial, with the retention of beta 2-m presumed to be the basic requirement for its initiation. It has been demonstrated in vivo that radiolabeled beta 2-m accumulates at the site of amyloid deposits. Our autoradiographic study of synovial tissue demonstrated that the cells had taken up radiolabeled beta 2-m, indicating that circulating radiolabeled-beta 2-m could be detected as an accumulation because it is taken up by the cells around the amyloid deposits. At present it can not be said that any basic treatment for beta 2-m-related amyloidosis has been established. It has been reported that the administration of a low dose of a corticosteroid may be effective in treating beta 2-m amyloid-related arthropathy. The articular symptoms resolved in most patients with corticosteroid. However, it should be borne in mind that corticosteroid may induce some adverse effects. Corticosteroid should be used only in patients with severe articular symptoms.

Adult↗
PubMed1994

[Lack of therapeutic effect on primary amyloidosis by interferon-alpha].

The therapy of primary amyloidosis is still unsatisfactory. The response rate after cytostatics, dimethylsulphoxide, colchicin and vitamin E is usually low. None of these treatment modalities prolongs significantly the survival in the majority of treated patients. The success of interferon alpha in the maintenance therapy of follicular non-Hodgkin's lymphoma and in the remission of multiple myeloma, as well as successful treatment of primary cryoglobulinemia, brought us to the idea to test interferon alfa in the therapy of primary amyloidosis. Interferon alpha-2b was administered to a patient with three years history of primary amyloidosis. Interferon alpha was used in the dose of 3 x 10(6) i. V. daily for a treatment period of 10 weeks. The evaluation of the response was based on the weekly assessment of the light chain lambda concentration in the morning spot of urine. No significant decrease of the light chain concentration during the course of the therapy was observed. The administration of interferon alpha-2b was interrupted in the 10th week of the therapy because of manic psychosis. The question is, whether a higher dose than 3 x 10(6) IU daily would be able to decrease the light chain production, or if this disease is resistant to interferon alpha therapy. Because of the low incidence of primary amyloidosis, the experiences will be collected on the base of small groups of case reports.

Amyloidosis↗