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At least 1,153 records · Page 64Linked to original sources
PubMed1990

Hemodialysis-associated amyloidosis of bone of beta-2 microglobulin origin.

A case of hemodialysis-associated amyloidosis in a patient who had been on hemodialysis for 10 years is described. Bone lesions were found in the humeral heads, carpal bones, and femoral heads and necks. Specimens obtained from the pathologically fractured left femoral neck revealed amyloid deposits histologically. Immunoperoxidase staining for beta-2 microglobulin was positive in the amyloid at the light-microscopic level. We reconfirmed that bone lesions associated with long-term hemodialysis are manifestations of amyloidosis of beta-2 microglobulin origin. Hemodialysis-associated amyloidosis should be considered in the treatment of long-term hemodialysis patients.

Amyloidosis↗
PubMed1989

Antikeratin autoantibodies in the amyloid deposits of lichen amyloidosus and macular amyloidosis.

In order to characterize immunoglobulins found on amyloid deposits of lichen amyloidosus and macular amyloidosis, and elution from cryostat sections was performed with citrate buffer, glycine buffer, NaCl, and PBS. Resulting eluates (mainly IgG) were examined with dot immunoblotting and SDS-PAGE immunoblotting and were found to react with the human epidermal keratin of 50 and 67 kD. Antikeratin autoantibody activities in normal murine and human sera were examined using a dot immunoblotting assay. In murine sera, titers of IgG and IgM autoantibodies were higher in older mice. The human cord blood showed significantly lower IgM autoantibody titers, whereas IgG antibody titers showed no significant differences from adults' sera, probably due to the permeability of IgG through the placental barrier. A stronger antibody activity in older individuals was thought to be due to the repeated exposures to keratin proteins derived from apoptotic keratinocytes. Sera from lichen amyloidosus and macular amyloidosis patients did not show any difference from normal controls in their antikeratin titers. It was concluded that the patients with lichenoid or macular amyloidosis are capable of producing a normal level of antikeratin autoantibodies. However, the removal of opsonized keratin-type amyloid from the skin is slow or deficient due to as yet unknown factors.

Amyloid↗
PubMed1988

Renal amyloidosis. Correlations between morphology, chemical types of amyloid protein and clinical features.

Sixty-one autopsy cases of renal amyloidosis were reviewed to assess the relationship of renal pathology to chemical types of amyloid and clinical features. Glomerular amyloid deposition was divided on the basis of morphological characteristics, into four types: a mesangial nodular type showing nodular mesangial deposits with sparse capillary wall involvement (25 cases), a mesangio-capillary type disclosing diffuse amyloid deposition in the mesangium and along both sides of the glomerular basement membrane (19 cases), a perimembranous type principally involving the subepithelial side of the basement membrane invariably characterized by exuberant spicular arrangement (6 cases), and a hilar type showing amyloid deposits almost exclusively in hilar arterioles (11 cases). Twenty-four of 25 cases of mesangial nodular type (96%) showed amyloid protein of AA type. However, mesangio-capillary and perimembranous types were associated with deposition of AL amyloid protein in 15 of 19 (79%) and all 6 cases, respectively. Nephrotic syndrome was more frequent in patients with AL amyloidosis; notably, all patients with perimembranous type had nephrotic syndrome irrespective of the extent of glomerular amyloid deposits. Chronic renal failure and renal death appeared more common in mesangial nodular type in which the extent of glomerular amyloidosis correlated with that of vascular amyloid deposits. The results obtained suggest that the chemical type of glomerular amyloid protein (AA vs AL) is associated with significant differences in the morphological, clinical and prognostic features of the renal involvement.

Adult↗
PubMed1990

Soft-tissue uptake of 99mTc-diphosphonate and 99mTc-pyrophosphate in amyloidosis.

This study describes the results of scintigraphy with 99mTc-labeled methylene-diphosphonate (99mTc-MDP) and pyrophosphate (99mTc-PYP) as a noninvasive test for the distribution of organ involvement in five different types of amyloidosis. Scintigraphy with 99mTc-labeled phosphates appeared to be a sensitive noninvasive screening test for the extent and the distribution of organ involvement in systemic AA and systemic AL amyloidosis as well as in local bronchial amyloid, local dermal amyloid, and familial amyloidotic polyneuropathy. Echocardiography, however, was more sensitive for demonstrating cardiac involvement in systemic amyloidosis than 99mTc-MDP or 99mTc-PYP scintigraphy. 99mTc-MDP images showed a better contrast than 99mTc-PYP images, although there was no difference in the extent or the intensity of soft-tissue uptake.

Adult↗
PubMed1977

The influence of glomerular and interstitial factors on the serum creatinine concentration in renal amyloidosis.

48 cases of renal amyloidosis (grade I-IV) were investigated morphologically (point-counting method). Statistically significant positive correlations exist between relative interstitial volume and serum creatinine concentration at the time of biopsy. In amyloidosis there are furthermore connections (positive rank correlations) between the extent of glomerular amyloidosis and renal insufficiency, although single cases of grade III show normal serum creatinine concentrations if the interstitium is not enlarged. With regard to interstitial alterations only fibrosis, not amyloid masses, seems to influence renal function, especially in the case of predamaged glomeruli (grade II to IV). The diminished renal function - reduction of the glom. filtr. rate - may be explained in the case of interstitial fibrosis by slowing of renal blood flow caused by a decreasing cross-sectional area of postglomerular vessels. Renal insufficiency seems to depend upon both the glomerular and the interstitial factor, but in grades II-IV the interstitial fibrosis is thought to be of more importance for renal function.

Amyloidosis↗
PubMed1979

Ultrastructural studies on the evolution of amyloidosis in the cyclic hematopoietic (CH) dog.

Electron microscopy studies were made on tissues of cyclic hematopoietic (CH) dogs of various ages presenting a high incidence of spontaneous amyloidosis. The distribution and morphologic characteristics of amyloidosis in this animal model closely correspond to the secondary and familial forms of the disease in humans. Plasma cells and, particularly, macrophages presented marked changes during the evolution of amyloid deposition. Residual bodies in the macrophages contained abundant cell debris, a result of both endocytic and autophagocytic activities. Intracellular amyloid fibrils were not observed by conventional electron microscopy. A few reticular cells contained intracytoplasmic fibrils which were morphologically different from amyloid. There was no correlation between the amount of intracellular fibrils and the size of the extracellular amyloid deposits. On the contrary, a temporal association between the magnitude of the amyloid deposits and cytoplasmic changes in the macrophages at sequential stages of the evolution of the disease was evident. It is suggested that the hematopoietic defect in the CH dog could play an important role in the production of amyloidosis, making this animal an excellent experimental model for studies of that disease.

Amyloidosis↗
PubMed1977

Tumors and amyloidosis in mice painted with crude oil found on bathing beaches.

Oil lumps collected on the beaches of Israel in 1970, 1971 and 1973 were extracted with pure acetone and the extracts were used to paint the skin of mice twice weekly for 12 months. The oil lumps originated from crude oil spilled from tankers. The less recently collected oils induced papillomata and lymphomata in some animals. They were also more active than the recent oil in the induction of generalized amyloidosis. Mice painted for 12 months with acetone alone developed amyloidosis to a similar extent as those painted with the oldest oil. In previously reported experiments, however, acetone was much less active than the oil in producing amyloidosis after 5 months of painting. The possibility that acetone and oil might act both synergistically or to be antagonistic at different phases of amyloidogenesis is discussed.

Amyloidosis↗
PubMed1990

Is technetium-99 m-pyrophosphate scintigraphy valuable in the diagnosis of cardiac amyloidosis?

Amyloidosis is a systemic disease frequently involving the myocardium and leading to functional disturbances of the heart. Amyloidosis can mimic other cardiac diseases. A conclusive clinical diagnosis of cardiac involvement can only be made by a combination of different diagnostic methods. In 7 patients with myocardial amyloidosis we used a combined first-pass and static scintigraphy with technetium-99 m-pyrophosphate. There was only insignificant myocardial uptake of the tracer. The first-pass studies however revealed reduced systolic function in 4/7 patients and impaired diastolic function in 6/7 patients. Therefore, although cardiac amyloid could not be demonstrated in the static scintigraphy due to amyloid fibril amount and composition, myocardial functional abnormalities were seen in the first-pass study.

Amyloidosis↗
PubMed1990

Barium meal study for amyloidosis of the small intestine: measurements on radiograph.

In order to determine barium meal radiographic findings characteristic of amyloidosis, we measured the jejunal diameter, valvular width, and intervalvular distance in 25 patients with small bowel amyloidosis and in 30 control individuals, and compared the two groups with each other. As a result, jejunal diameter demonstrated no difference between amyloidosis cases and controls, while there was a significant difference in valvular width or intervalvular distance between the two groups. Our results indicate that objective estimates of the thickening of the valvulae and the shortening of the intervalvular distance by means of measurements of the given roentgenograms greatly contribute to the clinical diagnosis of this disease.

Adult↗
PubMed1990

Secondary amyloidosis in glycogen storage disease type Ib.

We observed the development of generalized amyloidosis in a girl with glycogen storage disease type Ib (GSD-Ib) who showed neutropenia, neutrophil dysfunction and recurrent infections. Renal and thyroid biopsies showed secondary amyloidosis, characterized by the presence of potassium permanganate sensitive Dylon positive deposits in glomeruli, renal vessels and thyroid interstitium. Immunohistochemistry showed that the deposits were composed of amyloid A (AA) protein. Possibly neutrophil abnormalities are involved in the pathogenesis of amyloidosis.

Amyloidosis↗
PubMed1986

Generalized amyloidosis secondary to xanthogranulomatous pyelonephritis.

Unilateral nephrectomy in an 8-year-old male with chronic renal failure and urosepsis revealed a combined histologic lesion of xanthogranulomatous pyelonephritis and amyloidosis. Amyloid deposits were found also in the rectum and liver. On clinical grounds xanthogranulomatous pyelonephritis was also suspected in the remaining kidney. Common causes of systemic amyloidosis were excluded. After operation the signs of amyloidosis diminished, probably due to removal of the infected kidney, but renal function deteriorated rapidly.

Amyloidosis↗
PubMed1986

The incidence of amyloidosis complicating inflammatory bowel disease. A prospective survey of 177 patients.

Three cases of secondary amyloidosis are reported, two in patients who had unequivocal Crohn's disease and one in whom the clinical course was that of Crohn's disease, but with histologic findings that were more suggestive of ulcerative colitis. All had evidence of renal failure. A prospective study of 177 patients with inflammatory bowel disease of greater than five years' duration was carried out in an attempt to establish the incidence of secondary amyloidosis, using rectal biopsy and simple renal function tests. No new cases were found. Neither was there evidence of renal failure due to other conditions. In the absence of renal dysfunction, a search for secondary amyloidosis probably is not justified.

Adult↗
PubMed1995

Juvenile rheumatoid arthritis and renal amyloidosis (case report).

Clinical renal abnormalities, including haematuria, proteinuria, abnormal urinary sediment, decreased renal functions and hypertension are relatively common in children with juvenile rheumatoid arthritis (JRA). These findings may be due to renal amyloidosis or administration of drugs that are potentially nephrotoxic. The case of an 11 years old boy diagnosed as JRA at 4.5 months of age and treated with steroids for 10 years is presented. In his history he had hypertension for 5 years and cataract for one year. Renal biopsy was done to evaluate the aetiology for proteinuria, which was overlooked before his admission to our Department. Secondary renal amyloidosis due to JRA was found at biopsy. The importance of investigation for amyloidosis during the long-term follow-up of JRA is reemphasized.

Amyloidosis↗
PubMed1989

Portal hypertension secondary to intrahepatic arterio-portal shunt in primary amyloidosis: a case report.

Portal hypertension is a rare complication in hepatic amyloidosis. We experienced a case of a 40-year-old man with primary amyloidosis and advanced esophageal varices. Angiographic procedures clearly demonstrated portal hypertension secondary to intrahepatic arterio-portal shunting. Hepatic arterial embolization brought about a disappearance of the A-P shunt and portal hypertension, though rebleeding occurred. To our knowledge, this is the first case report in which the pathogenesis of portal hypertension in hepatic amyloidosis was elucidated.

Adult↗
PubMed1986

Spontaneous rupture of the spleen and liver in amyloidosis--a case report and review of the literature.

A 52 year-old woman with systemic amyloidosis complicated with multiple myeloma died suddenly of intraperitoneal hemorrhage due to spontaneous rupture of the spleen and liver. Autopsy revealed multiple myeloma involving the bone marrow and diffuse amyloidosis involving the liver, spleen, kidneys, heart, bone marrow, lymph nodes, lungs, gastrointestinal tract, thyroid, skin and adrenal glands. The splenic red pulp and the hepatic parenchyma were replaced by masses of amyloid. Amyloid deposits were also numerous in the walls of blood vessels and linearly in the intracapsular regions of both the liver and spleen. This is the eighth case of spontaneous rupture of the spleen and the second case of spontaneous rupture of the liver in association with systemic amyloidosis.

Amyloidosis↗
PubMed1982

Gastroscopic biopsy of the stomach for the diagnosis of amyloidosis.

1. Amyloid deposition was investigated in the biopsied specimens obtained from the stomachs of suspected cases of amyloidosis. The subjects were seven cases of suggestive amyloidosis with clinical symptoms of muscle rigidity, masked face, macroglossia and B-J proteinuria. The stomach of an autopsied amyloidosis patient was also investigated. 2. Mucosal tissue was obtained from fundus, corpus and antrum of the stomach using gastrofiberscope, Olympus GFB2. And amyloid in the tissue was detected by H & E Congo red staining and polarization microscopy. Rectal biopsy was also done in the majority of the cases to compare with stomach biopsy. 3. Positive results were obtained with gastric biopsy in all seven cases and five of the 5 cases by rectal biopsy. Thus, gastric and rectal biopsy yielded the same results so far as detection of amyloid deposition was concerned. 4. Biopsy specimens from several sites of the stomach gave similar results in regard to the amyloid deposition. Tissue deposition of amyloid in the stomach wall showed a somewhat high incidence in the walls of intramucosal capillaries and in the walls of blood vessels of lamina muscularies mucosa. Gastroscopic biopsy of the stomach proved to be useful as a routine technique for the definite diagnosis of the disease.

Aged↗
PubMed1999

Phase II trial of high-dose dexamethasone for untreated patients with primary systemic amyloidosis.

Primary systemic amyloidosis is the disorder that results from the deposition of insoluble immunoglobulin light chain fragments. Patients seen within 30 days of diagnosis have a median survival of 13 months. Patients treated with melphalan and prednisone have a median survival of 17 months. There is a need for new therapies. A prospective study was undertaken of high-dose dexamethasone in the treatment of 25 patients with previously untreated primary systemic amyloidosis. Treatment was similar to that given to patients with multiple myeloma. In this cohort, three patients showed objective regression with organ-specific improvement of the disease. The median survival of the entire group was 13.8 months. High-dose dexamethasone is of occasional benefit in patients with amyloidosis but does not appear to be superior to melphalan and prednisone chemotherapy.

Adult↗
PubMed1989

MR and CT imaging of perirenal amyloidosis.

The radiographic findings of perirenal amyloidosis have not previously been described. A patient with multiple myeloma and primary amyloidosis (AL) with secondary hydronephrosis as examined on plain film, retrograde ureteropyelography, computed tomography, and magnetic resonance imaging is presented. This case illustrates perirenal amyloidosis and demonstrates the utility of CT and MRI in patient management and surgical treatment.

Aged↗