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PubMed1990

[A case of amyloidosis of peripheral arteries of the heart and liver in an elderly patient].

Cases of isolated cardiac amyloidosis and amyloidosis of the liver are seldom mentioned in the literature (Pitkänen et al., 1984). At the autopsy of a 85 years old woman we found a severe common arteriosclerosis, especially of the brain and heart. A pacemaker was regularly implanted. By staining with Kongo red there was histologically shown an isolated amyloidosis of the peripheral heart and liver arteries.

Aged↗
PubMed1990

Systemic amyloidosis in a mare.

A mare with chronic cachexia had multiple skin nodules, abdominal masses (attached and free floating), and large lymph nodes. Fine-needle aspiration cytologic evaluation of a skin mass revealed multinucleated giant cells surrounding eosinophilic material. Histologic evaluation revealed extensive amyloid deposits within the masses, lymph nodes, and the interstitium of many organs. The presence of systemic (visceral) and organ-limited (cutaneous) forms of amyloid is rare in horses. Amyloid congophilia was retained after potassium permanganate oxidation. The fibrils were thus distinct from the AA (secondary) fibrils that are found in most cases of equine amyloidosis, suggesting that this mare may have had primary amyloidosis. Regardless of fibril type, the presence of multinucleated giant cells in association with eosinophilic material in cytologic preparations of skin nodules may suggest a differential diagnosis of amyloidosis in horses.

Amyloidosis↗
PubMed1990

[Subacute kidney insufficiency in exclusively vascular kidney amyloidosis].

Two patients are reported with secondary amyloidosis in whom renal biopsy disclosed massive amyloid deposition with exclusively extravascular localization. In one case, autopsy showed selective amyloid deposition in the vessel walls of most examined organs. In both patients, the clinical presentation consisted of subacute renal failure without proteinuria. A possible reduction of renal perfusion, secondary to a depleted blood volume in one case and to pharmacological inhibition of prostaglandin synthesis in the other, could enhance renal dysfunction in both patients, who, on the other hand, previously had mild renal failure. The clinical presentation of renal amyloidosis basically depends on the distribution and severity of amyloid deposits. Vascular localization represents an uncommon pattern of renal amyloidosis, generally associated with chronic renal failure with minimal or no proteinuria.

Acute Kidney Injury↗
PubMed1990

[The potentiating action of artificial immunodeficiency on the development of amyloidosis in plague in great gerbils].

Cyclophosphamide injection causes amyloidosis development in the spleen of great gerbils both intact and plague-infected after immunization. The amyloid deposits are observed irrespective of acquired antiplague resistance level. Alterations observed may be considered as acquired (secondary) AA-amyloidosis. Results indicate the significance of immunodepression in amyloidogenesis and a possibility to use the cyclophosphamide-treated great gerbils as a convenient experimental model of amyloidosis.

Amyloidosis↗
PubMedDate not supplied

Successful intermediate-term outcome for patients with cardiac amyloidosis undergoing heart transplantation: results of a multicenter survey.

Amyloid heart disease has been considered a contraindication for heart transplant on the basis of the hypothesis that it is a systemic disease and that amyloid deposition would occur in the cardiac allograft. Despite these concerns, several centers have performed heart transplantation for amyloidosis in a limited number of cases. A survey was sent to all of the U.S. centers and a limited number of European centers listed with the Registry of the International Society for Heart Transplantation. Nineteen centers responded, and data were provided for a total of seven patients (three men and four women, with a mean age of 46 years, range, 30 to 60 years) who had transplants for cardiac amyloid. The diagnosis of cardiac amyloidosis was made histologically on endomyocardial biopsy and/or examination of the explanted heart. Additional organ involvement included liver (two cases), rectal (two cases), renal (one case), gingiva (one case), and tongue (one case). A specific amyloid protein was identified in five patients (four lambda, one kappa Ig light chain). Two patients developed recurrent amyloid in their allograft seen by electron microscopy at 3 1/2 and 4 months, respectively. One patient developed progressive diastolic dysfunction, but systolic function was preserved. This patient died 13 months after transplantation as a result of progressive liver infiltration with amyloid. One patient died immediately after operation. Five patients are alive and fully rehabilitated 32 +/- 12 months after transplant. On the basis of this small series, some patients with cardiac amyloidosis can undergo heart transplantation with good intermediate-term results.

Adult↗
PubMedDate not supplied

Amyloidosis in children with familial Mediterranean fever.

In this survey 113 children with secondary amyloidosis due to familial Mediterranean fever are reviewed in regard to their respective histories, and physical and laboratory findings. The beneficial effects of colchicine in the treatment of this condition are evaluated. The number of children presented with amyloidosis secondary to familial Mediterranean fever was considerable. The male-female ratio was 4/3. It was observed that the number of patients with amyloidosis increased through the adolescent period, and that most of the cases demonstrated phenotype I (74.33%). Another important finding was the increase of partial thromboplastin time in 96 out of 113 cases (84.95%). All the symptoms of the periodic attacks were relieved by colchicine. A significant difference was found between the serum total protein and albumin values before and after colchicine therapy.

Adolescent↗
PubMed1989

[An elderly patient with IgD myeloma associated with renal amyloidosis and acute renal failure].

A 83-year-old male patient with IgD myeloma associated with renal amyloidosis and acute renal failure is described. We also reviewed the clinicopathological findings of IgD myeloma in the literature. Although hemodialysis was performed 36 times for acute renal failure, he died of severe gastrointestinal bleeding. Renal necropsy specimens revealed typical features of amyloidosis in light microscopic, electron microscopic and immunofluorescent examinations. The levels of serum IgD (755 mg/dl), immunoelectrophoresis, and immunofluorescence of bone marrow and renal specimens were consistent with IgD myeloma (lambda type). IgD myeloma is generally considered to be a rare disease of juvenile onset and is complicated with extramyelogenic tumors according to previous reports. However, IgD myeloma without extramyelogenic tumors occurred in a 83-year-old patient reported here. It appears that advanced age onset IgD myeloma associated with renal amyloidosis and acute renal failure is rare. This was the oldest case of IgD myeloma we examined.

Acute Kidney Injury↗
PubMed1989

Peripheral neuropathy as an early marker of AL amyloidosis.

Fifty-one of 146 patients with primary amyloid deposits of light-chain origin (AL) examined between 1972 and 1986 were found to have peripheral neuropathy as part of their systemic amyloidosis. Peripheral neuropathy was the presenting symptom in 11 patients, and it was a later symptom or an incidental physical examination finding at the time of amyloid workup in 40 additional patients. The median duration of time from first symptom to the diagnosis of primary amyloidosis was longer in those whose first symptom was neuropathy (48 months vs 12 months). In addition, survival time after diagnosis for these patients was significantly longer (at least 35 months vs 16 months). Peripheral neuropathy is not an uncommon symptom of primary amyloidosis and may mark the onset of the disease process years before infiltration of vital structures of the heart, kidneys or gastrointestinal tract leads to failure and a subsequent downhill course. Appreciating amyloid disease at this early stage may widen the potential therapeutic window.

Amyloidosis↗
PubMed1989

Genetic amyloidosis: recent advances.

The hereditary amyloidoses are usually associated with peripheral neuropathy and, for this reason, they have been called familial amyloidotic polyneuropathy (FAP). The neuropathy usually involves both the peripheral sensory motor and autonomic nervous systems, resulting in not only loss of sensation and motor function but also bowel dysfunction and orthostatic hypotension. In addition, most of the FAP syndromes involve other organ systems, in particular the renal and cardiac systems. Renal amyloidosis usually presents as nephrotic syndrome and later terminates in azotemia. Cardiac amyloidosis may present as congestive heart failure or arrhythmia. Whereas the neuropathy of hereditary amyloidosis may be the most prominent clinical feature, it is renal or cardiac disease that often dictates the length of survival.

Alanine↗
PubMed1989

Amyloidosis in pigtailed macaques (Macaca nemestrina): pathologic aspects.

The pathologic aspects of 248 cases of amyloidosis in pigtailed macaques (Macaca nemestrina) at the Washington Regional Primate Research Center from 1971 through 1985 were studied. Amyloid was present in the spleen, liver and gastrointestinal (GI) tract, either alone or in combination, in nearly 75% of the monkeys. Its occurrence declined with age in the spleen and the GI tract, but increased with age in the liver. Both intestinal inflammation and retroperitoneal fibromatosis were strongly associated with amyloid deposition in the GI tract. Monkeys with histopathologic findings of enteritis or enterocolitis and glomerulonephritis were at increased risk of developing amyloidosis. Forty cases of amyloidosis with a history of chronic diarrhea had type AA amyloid by histochemical tests.

Age Factors↗
PubMed1989

Intestinal pseudo-obstruction due to dialysis amyloidosis.

Marked gastric dilatation and the carpal tunnel syndrome developed concurrently in a 58-year-old man treated with hemodialysis for 14 years. Despite extensive examinations, no attributable organic lesion was demonstrated in the gastrointestinal tract. However, amyloid deposition, which was immunohistochemically identified as beta-2 microglobulin, was demonstrated in the stomach and the synovia excised from the right carpal tunnel. Intestinal pseudo-obstruction due to dialysis amyloidosis was considered to be the cause of the gastric dilatation. There have been a few recently reported cases with dialysis amyloidosis in which extra-articular amyloid deposition resulted in overt clinical problems. The present case provides additional evidence that dialysis amyloidosis can develop serious extra-articular complications; intestinal pseudo-obstruction is a very rare gastrointestinal manifestation.

Amyloidosis↗
PubMed1989

[Amyloidosis lesions of the digestive organs in patients with tuberculosis and chronic nonspecific lung diseases].

Histological postmortem examination of 30 persons who had died of pulmonary tuberculosis complicated by renal amyloidosis revealed amyloid affections in the rectum, stomach and liver of 76.3, 60 and 56.7 per cent of the dead, respectively. Histological examination of biopsy specimens of the gastric and duodenal mucosa from 20 patients with pulmonary tuberculosis revealed amyloid affections in 17 of them (85 per cent). An analogous examination of 18 patients with chronic nonspecific diseases of the lungs revealed amyloidosis of the organs in 10 persons (55.6 per cent). Efficiency of using proteinase inhibitors such as amben, epsilon-aminocapronic acid and contrical in the prevention and treatment of secondary amyloidosis of the digestive organs was studied experimentally.

Adult↗
PubMed1989

Bullous amyloidosis.

Amyloidosis may present with involvement of a variety of organ systems. Cutaneous involvement is a relatively common finding in patients with systemic amyloidosis. The occurrence of bullous skin lesions, however, is rare; only a few such cases have been previously reported. We describe a patient who presented with a subepidermal bullous skin disease initially thought to be bullous pemphigoid based on both clinical and histologic appearances. The patient subsequently developed the nephrotic syndrome. Biopsy specimens of the skin and kidney showed involvement of both organs with amyloid, and amyloid was later found in the spleen, heart, and nervous system. No subsequent evidence of myeloma was found in this patient. The clinical, histopathologic, immunofluorescent, and electron microscopic findings of systemic amyloidosis are discussed.

Amyloidosis↗
PubMed1989

[Imaging technics in extra-cardiac thoracic amyloidosis].

This report deals with five observations of tracheobronchial, pleuropulmonary and mediastinal amyloidosis. Exploration was performed with plain radiographs, computed tomography in three cases, and magnetic resonance imaging in one case of tracheobronchial and interstitial amyloidosis. After their review of literature, the authors describe the main features of extracardiac thoracic amyloidosis observed with imaging and establish the contribution of the various techniques to the diagnosis and follow-up of the disease.

Aged↗
PubMed1989

[Localized amyloidosis of urinary bladder: a case report].

A case of localized amyloidosis of the urinary bladder is reported. A 82-year-old woman visited our hospital with the chief complaint of miction pain and residual urine sensation. Cystoscopic examination revealed a broad-based and nonpapillary tumor without bleeding on the right lateral wall. A transurethral biopsy of this tumor was performed. A histopathological examination with H.E. and Congo red stains demonstrated amyloid deposition in the submucosal layers of the vesical wall. Rectal biopsy and other findings suggested no deposition of amyloid in other organs. On the basis of these findings, we made a diagnosis of localized amyloidosis of the urinary bladder. To the best of our knowledge, the present case is the 23rd of localized amyloidosis of urinary bladder in Japan. The patient was asymptomatic after biopsy. We discuss the clinical features and management of this disease.

Aged↗
PubMed1989

[Primary amyloidosis of the bladder: a case report].

A case of primary amyloidosis of the bladder, which developed 4 years after primary treatment, is described. The patient is a 55-year-old male, who consulted our clinic with a complaint of asymptomatic gross hematuria on October 1, 1983. Cystoscopic examination showed a tumorous lesion involving right lateral wall of the bladder, which was resected transurethrally. No malignancy was found by histopathological examination. About 4 years later, he consulted our clinic again with a complaint of asymptomatic gross hematuria. There was a tumorous lesion involving left lateral wall of the bladder on cystoscopy. Transurethral resection of bladder tumor was performed under a diagnosis of bladder tumor. Histological examination revealed amyloidosis of the bladder, AL-type amyloid. Re-examination of the specimens resected 4 years ago showed the same findings. Biopsies from the tongue, skin and rectum disclosed no amyloid deposition. During these 19 months, he had no recurrence. This case was thought to be the first one of primary amyloidosis of the bladder showing new recurrence reported in Japanese literature.

Amyloidosis↗
PubMed1989

[A case of localized amyloidosis of the bladder manifesting post-renal failure].

We report a case of primary localized amyloidosis of the bladder which manifested post-renal failure. A 79-year-old woman with diabetes mellitus complained of anorexia and oliguria. Computed tomographic (CT) scan showed bilateral hydronephrosis. Cystoscopic examination revealed a broad-based nonpapillary tumor in the trigonum of the bladder and CT scan demonstrated thickening of the posterior wall of the bladder. Pathological examination of the transurethral biopsy specimen revealed amyloid deposits in the submucosa, but no malignant changes were found. Cytodiagnosis of washing fluid of the bladder revealed amyloid deposits around the exfoliative cells. Serum electrophoresis showed a normal pattern. Urinary Bence-Jones protein was not detected. Amyloid deposits were not found in rectal mucosa. Systemic or secondary amyloidosis was ruled out from these findings, and primary localized amyloidosis of the bladder was diagnosed. The mass of the bladder was transurethrally resected and pig-tail stents were indwelt. These procedures gave a satisfactory result.

Acute Kidney Injury↗
PubMed1989

Morphological and immunohistochemical findings in dialysis-related amyloidosis. An analysis of 16 cases.

The morphologic features of amyloidosis associated with long-term hemodialysis for chronic renal failure were studied in a series of 16 patients in all of whom immunohistochemical evidence of beta 2-microglobulin-related amyloidosis was obtained. Osteoarticular involvement was present in 15 cases, as demonstrated in synovial biopsies, articular fluid sediments, three surgically obtained femoral heads, and 1 autopsied case. Amyloid was found in synovial membrane, articular cartilage and capsule. Osseous lesions due to amyloid infiltration were found in 2 cases with spontaneous fracture of the femoral neck. Thirteen patients had also amyloid in tissues obtained from carpal tunnel. Systemic involvement was demonstrated in 8 patients by means of rectal biopsies, abdominal fat aspiration biopsies, surgical specimens and autopsy. Deposition in the muscular layer of the gastrointestinal tract was found in 3 cases. Nodular subendothelial deposits in vessels of multiple sites seem to be characteristic of this type of amyloidosis. Immunohistochemical reactions for beta 2-microglobulin and P component were strongly positive in amyloid deposits of all cases. According to our results, abdominal fat aspiration biopsy has little value for screening purposes, whereas the study of articular fluid sediment is highly sensitive as a diagnostic method in patients with articular effusions.

Acute Kidney Injury↗