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PubMed1987

Amyloidosis complicating idiopathic myelofibrosis.

Three cases of amyloidosis occurring in the later course of idiopathic myelofibrosis were studied at autopsy. In these cases, the amyloid deposition was seen only in the renal glomerulus, which had caused proteinuria. Although their exact nature could not be determined by immunohistochemistry, the amyloid deposits in the three cases were permanganate resistant and exhibited the same organ distribution, indicating a similarity in their character. These cases suggest that amyloidosis might be a complication of idiopathic myelofibrosis, implying that idiopathic myelofibrosis could be a disorder underlying amyloidosis.

Aged↗
PubMed1987

Presence and role of glycosaminoglycans in amyloidosis.

Though the presence of glycosaminoglycans in amyloid deposits has been recognized for a long time their role in the pathogenesis of the disorder has remained elusive. As shown here, liver and spleen of human patients with secondary amyloidosis contain 5 to 10 times the amount of glycosaminoglycans as normal organs. Of the three major glycosaminoglycans measured, the heparan sulfate fraction showed the largest increase. In mice where amyloidosis was induced by the injection of casein and enhancing factor (accelerated model) 35SO4-labeled, or Alcian blue stained glycosaminoglycans appeared as early as and at the same location as proteins detected by Congo red staining which was about 2 days after initiation of the procedure. When glycosaminoglycan synthesis was followed in liver and spleen slices of control and experimental animals a significant increase in rate was found in the spleen of the experimental mice. Though there was an increase in heparan sulfate synthesis the major contribution to the overall increase was made by the chondroitin sulfates in the accelerated as well as in the standard induction model. In addition, unlike in the human disorder the chondroitin sulfates were the major glycosaminoglycans which had accumulated in the spleens of animals which had amyloidosis induced by the long term standard procedure (6 weeks) as measured by isolation and uronic acid analysis. The data presented here show that glycosaminoglycans appear to play an important and perhaps direct role in the process of amyloid deposition in the human disease as well as in the experimentally induced disorder in animals.

Amyloidosis↗
PubMed1987

[Arterial hypertension in renal amyloidosis (a clinico-morphological analysis)].

The incidence of arterial hypertension (AH) in patients with renal amyloidosis varied with its stage. Thus, AH was encountered in 13% of cases with renal amyloidosis at the proteinuric stage, 15% of those at the nephrotic stage, and 53% of those at the azotemic stage. One determinant of AH at the first two of these stages appears to have been damage to the antihypertensive system of the renal medulla, while the increased rate of AH at the azotemic stage was found to be associated with sodium retention in the body. AH in renal amyloidosis is an unfavorable prognostic factor, for it is conducive to amyloid shrinkage of the kidneys and to chronic renal failure.

Adolescent↗
PubMed1987

AA amyloidosis in systemic lupus erythematosus.

A 37-year-old woman with longstanding systemic lupus erythematosus developed cardiac insufficiency, nephrotic syndrome, and azotemia. The findings at echocardiography and cardiac scintigraphy suggested amyloidosis, which was confirmed by rectal biopsy and fine needle biopsy of subcutaneous abdominal fat. Postmortem examination revealed systemic amyloidosis with massive deposits in the heart, spleen and kidneys. She had persistently increased concentration of serum amyloid A protein during the last 4 years of her life, and her amyloidosis was of the secondary (AA) type, as shown by immunohistochemical studies.

Adult↗
PubMed1977

[Macular amyloidosis].

Fifty-five patients with presumptive macular amyloidosis were investigated for the presence of amyloid in dermal tissue. In twenty-six of these patients the diagnosis could be confirmed by the demonstration of amyloid deposits by optical and/or electron microscopy. In this paper attention is called to the frequency of oligosymptomatic forms of macular amyloidosis, which are often overlooked in spite of the usual existence of itching. These inconspicuous forms are termed "minor macular amyloidosis", and they consist of tenuous brown macules on the upper back, irregularly interspersed with the normal skin and with speckles of whitish discoloration. The diagnosis must be verified by the finding of dermal deposits of amyloid. For that purpose the electron microscopy is a much more reliable procedure than conventional methods or thioflavine T.

Amyloid↗
PubMed1986

[Senile amyloidosis of the heart].

A morphologic study of 376 hearts from individuals who died between 75 and 98 years of age demonstrated senile amyloidosis of the heart in 307 (82%) of the cases, using the Congo red stains for polarized light studies and the thiosine red for luminescent microscopy. The frequency of cardiac amyloidosis detection increased with age, and reached 100% in those above 90. Clinico-anatomical correlations showed repolarization changes to be the most common findings (80%), followed by arrhythmia (over 50%), conductivity disorders and reduced voltage of electrocardiographic waves. Chronic heart failure was less common (about 10%). In most cases, moderately marked coronary atherosclerosis was also found at autopsy. Amyloidosis was thought to be making a more significant contribution towards myocardial lesions, as compared to atherosclerosis, in elderly people above 85 years of age.

Age Factors↗
PubMed1986

[Amyloidosis in patients with rheumatoid arthritis].

Of 467 patients followed-up for 15 yrs, rheumatoid arthritis was complicated by amyloidosis in 47 (10%). The relationship of amyloidosis development with a high or constantly moderate activity and expression of rheumatoid arthritis and its severity was brought to light. The results of biopsies of the mucosa, kidneys and liver, autopsy and clinical findings were analyzed and correlated. An increase in the frequency of amyloidosis development was noted in patients treated with glucocorticoids whereas in patients receiving cytostatics there was not such an increase.

Adolescent↗
PubMedDate not supplied

HLA antigen frequencies among patients with juvenile chronic arthritis and amyloidosis: a brief report.

Amyloidosis is seen in a small number of patients with juvenile chronic arthritis (JCA). In order to determine whether particular HLA markers might predispose to the development of amyloid in JCA a group of 45 patients with amyloidosis confirmed by biopsy was typed for the HLA-A, B, C and DR loci. The results confirmed previous smaller studies that no HLA antigen detected by standard serological techniques was associated specifically with the development of amyloidosis. Those antigens which showed an altered frequency (ie. DR4 and DRw8) were known to be associated with the different types of JCA onset represented in this group.

Amyloidosis↗
PubMed1986

[Osteomedullary amyloidosis with osteolytic lesions. Apropos of 4 cases].

Amyloidosis of bone and bone marrow is rarely symptomatic and is generally limited to deposits diagnosed by histological examination. Four cases with lytic bone lesions are reported. In 2 cases, amyloidosis was associated with Waldenstroem's Macroglobulinemia, in one case with multiple myeloma and in 1 case with solitary plasmocytoma. These 4 cases are compared to 14 cases already published in the literature, including 2 cases of plasmocytic dyscrasias. The role of amyloidosis in the genesis of bone lesions is discussed.

Aged↗
PubMed1986

Chemical classification of hereditary amyloidosis in Brazilian families and identification of gene carriers.

Plasma prealbumin was isolated from individuals in 3 kindreds from Brazil with biopsy proven heredofamilial amyloidosis as well as from a number of asymptomatic family members. The prealbumin samples were cleaved with cyanogen bromide and the resulting peptide mixtures separated by reverse phase high performance liquid chromatography. The peptide elution pattern seen for the individuals with confirmed amyloidosis is consistent for the presence of a prealbumin variant with a methionine for valine at position 30 of the molecule. Sequence analysis of the isolated peptides confirms this observation and shows that the 3 Brazilian families investigated in our study have the same prealbumin variant as individuals with amyloidosis of Swedish/American, Portuguese and Japanese origins.

Adult↗
PubMed1985

Renal adenocarcinoma and systemic amyloidosis. Immunohistochemical and histochemical studies.

We present six patients with associated renal adenocarcinoma and systemic amyloidosis. In five of the patients amyloidosis showed clinical and organ distribution characteristics of reactive (secondary) amyloidosis. The amyloid deposits contained amyloid A protein as defined by potassium permanganate sensitivity and by reactivity with anti-amyloid A antiserum. Four of these patients had suffered from additional inflammatory diseases. In the sixth patient the amyloid disease had different clinicopathologic characteristics and was evidently of an immunoglobulin light-chain-derived type related to a concomitant multiple myeloma. Although renal carcinoma seems to be an effective stimulator of amyloid formation, the possible contributory influence of associated chronic inflammation deserves more careful appraisal.

Aged↗
PubMed1985

[Peritoneal amyloidosis in Waldenström's macroglobulinemia. X-ray computed tomographic aspects].

A 74 years old man, know to have Waldenström's macroglobulinemia was hospitalized for intestinal obstruction. Upper gastrointestinal barium study revealed an extrinsic stenosis of duodenum. Ultrasonography and abdominal computed tomography revealed a diffuse mesenteric infiltration with medium level echoes and encasing of mesenteric vessels. Histologic examination of biopsies taken at exploratory laparotomy disclosed amyloidosis of the epiploon and mesentery. The review of amyloidosis in macroglobulinemia shows peritoneal amyloidosis to be a very rare event. Sonographic, computed tomographic and pathologic findings of this unusual case are discussed.

Aged↗
PubMed1986

Cardiac amyloidosis. Therapeutic and diagnostic difficulties with reference to two different forms of the disease.

Two male patients with primary cardiac amyloidosis are described. Patient 1 presented with typical effort angina pectoris with no ischemic electrocardiographic changes and a normal coronary angiogram. At necropsy, a severe diffuse, intravascular amyloid deposition was observed in the intramural coronary arteries. In patient 2 the presenting symptom was congestive heart failure with echocardiographic evidence of asymmetric septal hypertrophy and pericardial effusion. Technetium-99m pyrophosphate scintigraphy showed diffuse myocardial uptake, and the diagnosis of cardiac amyloidosis was confirmed in the postmortem examination. The diagnostic and therapeutic problems associated with cardiac amyloidosis are discussed in the light of these case reports.

Adult↗
PubMed1986

Amyloidosis complicating the rehabilitation of a patient with a right cerebrovascular accident.

Physiatrists are rarely presented with a case of amyloidosis. Its unrelenting course must be appreciated in order to speed the process of rehabilitation and help in setting appropriate and rapidly achievable goals. These rehabilitation challenges are discussed with respect to a patient with amyloidosis and a right cerebrovascular accident. The need for medical management of the systemic symptoms of amyloidosis is also addressed. It is felt that the addition of such a progressive terminal disease to the diagnosis of stroke leads to unique interactions among the rehabilitation staff, the patient, and the patient's family, and the need for an aggressive approach to goal-setting and discharge planning.

Amyloidosis↗
PubMed1985

Generalized amyloidosis in cystic fibrosis.

A boy suffering from cystic fibrosis (CF) complicated by generalized amyloidosis is reported. His condition was fairly good during the first 10 years of life, but after this time he had repeated pulmonary infections caused by Staphylococcus aureus. From the age of 14, he developed increasing hepato- and splenomegaly and a liver biopsy revealed massive amyloidosis. At the age of 16 he died of respiratory and cardiac failure. Post-mortem examination revealed wide-spread amyloidosis in addition to pulmonary and pancreatic findings characteristic of CF.

Adolescent↗
PubMed1977

Murine amyloid protein AA in casein-induced experimental amyloidosis.

Amyloidosis was induced in mice by 25 subcutaneous injections of casein. The splenic amyloid fibrils were identified by electron microscopy to be closely associated with reticular cells. After isolation of the fibrils by simple physical techniques, their ultrastructure revealed single filaments of 80 to 100 A width, which were rigid, nonbranching, and of indeterminate length. This is comparable to previous studies on human preparations. The amyloid fibrils were dissociated by solution in guanidine and chromatography. The resultant amyloid fibril protein was characterized as to its molecular weight, amino acid analysis, and amino-terminal sequence. It was thus definitely identified as protein AA, the major component of secondary amyloidosis. An antibody to this protein, murine AA, identified a cross-reacting mouse serum protein SAA and indicated a species specificity when tested against human preparations. A comparison is made with the AA protein in another murine model as well as AA proteins from human, guinea pig, monkey, and mink amyloidosis.

Amino Acid Sequence↗
PubMed1985

Renal amyloidosis in Jordan.

Twenty cases of renal amyloidosis were observed among 182 patients who underwent kidney biopsy at Jordan University Hospital (JUH) during the period 1979-1983. Eight cases were classified as primary amyloidosis while the remaining 12 were secondary to various underlying diseases, the most significant one was familial Mediterranean fever (FMF). Comparison of incidence of renal amyloidosis in kidney biopsy series from neighbouring countries is presented.

Adolescent↗
PubMed1985

[Morphometric characteristics of thrombocytes in renal amyloidosis].

Forty-three patients with primary, secondary and hereditary amyloidosis at different stages of renal lesions (proteinuric, nephrotic, chronic renal failure) were examined for the count and size of peripheral blood platelets. All the patients manifested a diminution of the mean diameter of platelets caused by predominance in the circulation of their minor forms. In half of the cases these alterations combined with thrombocytosis. A direct non-linear relationship was revealed between the size of platelets and their count in patients with primary and secondary amyloidosis. The possibility of primordial functional deficiency of platelets in patients with amyloidosis is discussed.

Adolescent↗