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PubMed1977

Occurrence of amyloidosis secondary to the induction of experimental hypertension in mice.

Following the induction of three different forms of experimental hypertension, deposits of amyloid were found in the spleens of 5-20 per cent of the mice late in the course of the hypertension, Amyloidosis was found in nude (with genetical aplasia of the thymus) as well as in haired (normal) mice. The highest frequency of amyloidosis was observed in mice with hypertension due to partial infarction of one kidney and contralateral nephrectomy. The hypertensive vascular disease, involving lesion of the vessels and of the organs supplied by the affected vessels, is believed, to represent a stimulus for the reticulo endothelial system (RES) with development of amyloidosis as a secondary event.

Amyloidosis↗

PubMed1981

Systemic amyloidosis and non-hematologic malignancy in a large autopsy series.

A series of 24,388 consecutive autopsies yielded 148 cases of amyloidosis, for which the associated diseases were tabulated. 13 solid primary malignancies were found in eleven patients, including one mucoepidermoid cancer of the parotid and one thymoma; two out of eleven had evidence of pyelonephritis. Five patients suffered from past or present renal adenocarcinoma, the most common carcinomatous cause of amyloidosis. Systemic amyloid deposits thus occurred in 2.1% of autopsied patients with renal carcinoma, but showed no obvious correlation with tumor stage or histologic type. Details are presented of an unusual case of hypernephroma producing a stable bronchial metastasis cuffed by nodular amyloid and dissociated by a lymphoid infiltrate containing plasma cells. The occurrence of systemic amyloidosis in non-hematologic malignancy thus appears to be a rare event, which, especially in the case of renal cancer, is assumed to be due to amyloid-fibril protein AA.

Aged↗

PubMed1998

Generalised amyloidosis in two Siamese cats: spontaneous liver haemorrhage and chronic renal failure.

Two cases are reported, illustrating the antemortem diagnosis of systemic amyloidosis in Siamese cats. A cat presenting with inappetence and depression was diagnosed as having systemic amyloidosis with spontaneous haemorrhage from the liver. In another cat from the same breeding cattery, chronic renal failure due to systemic amyloidosis was an incidental finding. Little treatment was possible in either case and both were later euthanased. The two cats had similar renal and hepatic pathology but different signs of disease.

Amyloidosis↗

PubMed1968

Amyloidosis induced in mice by Escherichia coli endotoxin.

Amyloidosis was produced in mice by repeated subcutaneous injections of 0.5-or 0.005-milligram amounts of Escherichia coli endotoxin. Of the two strains of mice examined, amyloidosis was induced more readily in one than in the other. The ability of endotoxin to induce amyloidosis lends support to the view that stimulation of reticuloendothelial cells leads to amyloid formation.

Amyloidosis↗

PubMed1975

Systemic amyloidosis in mice inoculated with lyophilized Candida cells.

Injection of lyophilized Candida albicans or C. parapsilosis cells induced amyloidosis in outbred CFW mice. The disease process was irreversible, and a significant number of animals died of systemic amyloidosis within 400 days after the last treatment. The techniques described may represent a model system for the study of systemic amyloidosis.

Amyloid↗

PubMed1983

Amyloidosis and the serum amyloid A protein response to muramyl dipeptide analogs and different mycobacterial species.

Serum amyloid A protein (SAA) elevation accompanies induction of secondary amyloidosis in mice given Mycobacterium butyricum in Freund adjuvant. The synthesis of SAA by cultured hepatocytes is induced by a macrophage-derived mediator, which has been identified as interleukin 1. In these studies, SAA synthesis has been used as an index of macrophage activation to examine the in vivo response of mice to challenge with seven different mycobacteria and with synthetic analogs of the immunoadjuvant N-acetylmuramyl-L-alanyl-D-isoglutamine [MDP(L-D)]. SAA synthesis was stimulated by administration (by the intraperitoneal route) of the mycobacteria dissolved in saline, with Mycobacterium vaccae being the most active and Mycobacterium leprae being the least stimulatory. MDP(L-D), which is the minimal structure (molecular weight, 492) able to substitute for mycobacteria in Freund adjuvant, stimulated SAA synthesis, whereas the MDP(D-D) isomer was inactive. The butyl ester of MDP, which induces no detectable pyrogenicity but retains adjuvanticity, required a 100-fold greater dosage than MDP(L-D) in stimulating SAA synthesis. Amyloidosis was detected histologically only when active SAA inducers MDP(L-D), M. vaccae, and M. butyricum, were administered in incomplete Freund adjuvant, with amyloid-enhancing factor. These studies demonstrated that SAA elevation was a sensitive in vivo marker of the capacity of antigens to stimulate macrophages to produce interleukin 1. A point of considerable relevance to the human use of MDP was the observation that repeated injections of the adjuvant MDP in saline did not induce secondary amyloidosis.

Acetylmuramyl-Alanyl-Isoglutamine↗

PubMed1981

Amyloidosis in children with familial Mediterranean fever.

The clinical and laboratory findings of 35 children with familial Mediterranean fever who developed amyloidosis are described. The types, frequency, and severity of attacks of familial Mediterranean fever in these children were no different from patients with this disease without amyloidosis. Although amyloid was widely deposited in all tissues, the major clinical manifestations of the amyloidosis were proteinuria, the nephrotic syndrome, and progressive renal failure. Only 20% of the patients were alive 5 years after the first appearance of proteinuria.

Adolescent↗

PubMed2005

"Periodic fever" without fever: two cases of non-febrile TRAPS with mutations in the TNFRSF1A gene presenting with episodes of inflammation or monosymptomatic amyloidosis.

BACKGROUND: Tumour necrosis factor (TNF) receptor associated periodic syndrome (TRAPS) is caused by dominant mutations in the TNFRSF1A gene. In typical cases TRAPS begins early in childhood and is characterised by high and remittent fever over a period of 1-4 weeks or longer, accompanied by systemic and local inflammation. CASE REPORTS: Patient 1 presented with recurrent episodes of weakness, migrating myalgias, arthralgias, exanthema, and chest pain lasting for 1-4 weeks, but without any fever over an initial period of 4 years at least. Diagnosis of TRAPS was confirmed by the heterozygous mutation Y20H in TNFRSF1A. Patient 2, a 23 year old woman never had any symptoms indicative of TRAPS. Genetic evaluation of all members of her family with a TRAPS index patient disclosed the T50M mutation in TNFRSF1A. A medical check up showed proteinuria, and renal biopsy disclosed AA amyloidosis. CONCLUSIONS: TRAPS associated mutations can induce considerable inflammation that is not necessarily accompanied by fever. Even monosymptomatic severe amyloidosis can occur in these patients. Genetic counselling and appropriate management to prevent or mitigate amyloidosis may be necessary.

Adult↗

PubMed1991

Adult onset Still's disease and related renal amyloidosis.

A 57 year old woman presented with clinical and biological features of adult onset Still's disease. A kidney biopsy was performed at the onset of the condition and was found to be normal. Four years later, in view of a nephrotic syndrome, histopathological examination of a new kidney biopsy specimen was made and showed typical amyloid deposition. Renal amyloidosis was suspected in five previously reported cases but demonstrated by kidney biopsy in only two of them. In the present case renal amyloidosis was recorded after disease of four years' duration. The normality of the first renal biopsy specimen suggests the possibility of a direct relation between amyloidosis and adult onset Still's disease.

Amyloidosis↗

PubMed2005

Lactoferrin Glu561Asp facilitates secondary amyloidosis in the cornea.

AIM: To elucidate the pathogenic mechanism of amyloid formation in corneal amyloidosis with trichiasis. METHODS: Ophthalmological examination was performed in nine patients to determine secondary corneal amyloidosis with trichiasis. Congo red staining and immunohistochemistry using anti-human lactoferrin antibody were used for biopsied corneal samples. For genetic analyses, single strand conformation polymorphism (SSCP), direct DNA sequence analysis, and polymerase chain reaction (PCR) induced mutation restriction analysis (IMRA) were employed to detect lactoferrin gene polymorphism. RESULTS: All patients had had trichiasis at least for 1 year, and all amyloid-like deposits were found in one eye with trichiasis. Ophthalmological examination revealed that eight patients showed gelatinous type of amyloid deposition and one showed lattice type of amyloid deposition. Studies of biopsied corneal samples with Congo red stain revealed positive staining just under the corneal epithelial cells. Immunoreactivity of anti-human lactoferrin antibodies was recognised in all tissues with positive Congo red staining. Lactoferrin gene analysis revealed that seven patients were heterozygotic and two were homozygotic for lactoferrin Glu561Asp. The frequency of the polymorphism in the patients was significantly different from that in 56 healthy control subjects. CONCLUSION: Lactoferrin Glu561Asp is a key polymorphism related to facilitating amyloid formation in corneal amyloidosis with trichiasis.

Adolescent↗

PubMed1996

Monoclonal origin of localised orbital amyloidosis detected by molecular analysis.

AIMS: Primary localised orbital amyloidosis is a rare disease. The purpose of this study was to describe two cases of primary orbital amyloidosis and emphasise the value of molecular analysis of immunoglobulin gene rearrangement in identifying a monoclonal population of cells responsible for the amyloid production. METHODS: Charts and biopsy specimens of each case were reviewed. Conventional light microscopy, immunohistochemistry, and polymerase chain reaction (PCR) analysis for immunoglobulin gene rearrangement were performed in both cases. RESULTS: An unusual presentation of localised primary amyloidosis with bilateral and extensive enlargement of multiple extraocular muscles was seen in case 1. The presence of amyloid deposits was confirmed by biopsy in both cases. Evidence of a monoclonal population of plasma cells was shown by immunohistochemical analysis in case 2 only. The monoclonal origin of the cells responsible for the amyloid deposition was determined by PCR analysis demonstrating immunoglobulin heavy chain gene rearrangement in both cases. CONCLUSIONS: A monoclonal population of plasma cells responsible for the amyloid deposition was present in these two cases. PCR analysis is extremely helpful in determining monoclonality, a finding that may have important therapeutic and prognostic implications.

Adult↗

PubMed1982

Reduced amyloid-A-degrading activity in serum in amyloidosis associated with rheumatoid arthritis.

The ability to degrade amyloid A fibrils was studied in the serum of 31 patients with amyloidosis associated with rheumatoid arthritis, 33 patients with rheumatoid arthritis without amyloidosis, and 47 healthy controls. Fibrillar amyloid A protein and the radial diffusion method were used. The mean degrading activity in serum was significantly lower in patients with rheumatoid arthritis complicated by amyloidosis (58 +/- 19% SD of the activity in a pooled sample of sera from 100 healthy blood donors used as standard) than in patients with rheumatoid arthritis alone (78 +/- 14%; p less than 0.001) or controls (99 +/- 19%; p less than 0.001). Alpha 1-antitrypsin, concentrations of which were raised in both groups of patients, inhibited the degrading activity in serum even in low concentrations. A negative correlation between degrading activity and alpha 1-antitrypsin concentrations was observed. These findings suggest that reduced amyloid-A-degrading activity is due to inhibition rather than to deficiency of enzyme.

Adult↗

PubMed1970

Intestinal pseudo-obstruction in systemic amyloidosis.

The occurrence of clinical manifestations of mechanical intestinal obstruction in eight cases of systemic amyloidosis is reported. Seven similar cases have been recorded in the literature and are briefly reviewed. Correct diagnosis is important in these cases if useless surgical treatment or prolonged diagnostic investigations are to be avoided. Gastrointestinal involvement by systemic amyloidosis should be considered in a patient presenting with clinical manifestations of mechanical obstruction but paralytic ileus seen on plain films. In these cases an attempt should be made, by means of a barium enema, to exclude an obstructing lesion of the colon or distal small bowel. Once amyloidosis is considered, it can be confirmed easily and safely by biopsy of the rectal or small-intestinal mucosa.

Aged↗

PubMed1991

Sinusoidal portal hypertension in hepatic amyloidosis.

Hepatic venous catheterisation and transvenous liver biopsy were performed in five patients with hepatic amyloidosis. In three patients, hepatic venous pressures were normal and histological examination of the liver biopsy specimen showed discrete and sparse perisinusoidal amyloid deposits. In the other two, however, the gradient between wedged and free hepatic venous pressures was increased (12 and 16 mmHg; normal 1-4 mmHg) and amyloid deposits were abundant and diffuse in the Disse's space. This study shows that portal hypertension in patients with hepatic amyloidosis is of the sinusoidal type and is related to the reduction of vascular space of hepatic sinusoids by massive perisinusoidal amyloid deposits. Furthermore, portal hypertension is associated with a poor prognosis in patients with hepatic amyloidosis.

Aged↗

PubMed1992

Duodenal perforation in primary systemic amyloidosis.

Spontaneous duodenal perforation in two patients with primary systemic amyloidosis associated with multiple myeloma is described. Bowel perforation is a rare and often fatal complication of amyloidosis, and duodenal perforation has not been previously described. Both patients survived the bowel perforation with conservative management. Bowel perforation should be suspected in patients with amyloidosis presenting with acute abdominal pain, and active non-surgical management can be associated with prolonged survival.

Aged↗

PubMed1984

Computed tomographic appearances of cardiac amyloidosis.

Computed tomography was used to identify the characteristic appearances of histologically confirmed cardiac amyloidosis in two patients. Mean myocardial density and 95% confidence limits in one of these patients (30.6 +/- 3.4 Hounsfield units (HU) ) were significantly lower than in patients with diffuse hypertrophic cardiomyopathy (range from 38.8 +/- 5.7 HU to 45.9 +/- 4.4 HU) and normal myocardium (range from 41.9 +/- 4.3 HU to 44.8 +/- 4.4 HU) on pre-contrast computed tomograms. Although only an approximate myocardial density was obtained in the second patient with amyloidosis, a similar result (30 HU) was noted on pre-contrast tomograms. Diffuse thickening of the interventricular septum and left ventricular free wall was found in both patients. Myocardial density on post-contrast computed tomograms was 102.8 +/- 5.2 HU in one patient and approximately 100 HU in the other. A pericardial effusion was noted in the first patient. A low myocardial density on pre-contrast tomograms and diffuse myocardial thickening on post-contrast tomograms are considered to be important features of cardiac amyloidosis.

Alcoholism↗

PubMed1984

Disturbances of cardiac rhythm and conduction in familial amyloidosis with polyneuropathy.

Sixteen consecutive patients with familial amyloidosis with polyneuropathy of varying duration and severity underwent 24 hour ambulatory electrocardiographic monitoring. A high incidence of sinus node dysfunction, supraventricular and ventricular arrhythmias, and disturbances of atrioventricular conduction was found. Considerably more arrhythmias and disturbances of conduction were detected by long term electrocardiographic monitoring than by conventional 12 lead electrocardiograms. During a follow up period of three to 14 months five patients needed treatment by a pacemaker, three of them because of symptomatic complete heart block, one because of second degree heart block with heart failure, and one because of symptomatic dysfunction of the sinus node. The tachyarrhythmias did not require specific treatment. Long term electrocardiographic monitoring is a useful adjunct in the evaluation of patients with familial amyloidosis with polyneuropathy as it may detect otherwise unrecognised symptomatic disturbances of heart rhythm. The results may be valid also for other forms of amyloidosis involving the heart.

Adult↗

PubMed1999

Hereditary cardiac amyloidosis associated with the transthyretin Ile122 mutation in a white man.

An 83 year old white man with atrial fibrillation was admitted to hospital after a cerebral infarct. Echocardiography was characteristic of cardiac amyloid deposition and subsequent tests confirmed amyloidosis of transthyretin (TTR) type, in association with the Ile122 mutation of the TTR gene; this has only been reported previously in African Americans in whom it occurs with an allele frequency of 2%. Haplotype analysis did not suggest a different founder than for the African Ile122 mutation. Cardiac amyloidosis should be considered among elderly patients presenting with cardiac failure and/or arrhythmia, particularly if they are resistant to conventional treatment; if confirmed, it should be followed by precise characterisation of amyloid fibril type. The prevalence of autosomal dominant cardiac TTR amyloidosis in elderly white people is unknown but early diagnosis and supportive treatment may prevent complications among affected family members.

Aged↗