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PubMedDate not supplied

[Heparin tolerance in relation to age and the presence of amyloidosis].

Clotting time under basal conditions and 30', 60' and 120' after 100 I.U. heparin/kg i.v. was determined in four groups:1) 15 healthy subjects aged less than 50 yr; 2) 27 "healthy" subjects over 65 yr; 3) 20 subjects with either myeloma (7 cases), benign monoclonal gammopathy (7) or rheumatoid arthritis (6); 4) 4 subjects with amyloidosis (2 primary, 1 secondary to rheumatoid arthritis, and 1 secondary to myeloma). Rectal biopsy and a histological search for amyloid substance were carried out in all subjects from the 3rd and 4th groups. Heparin tolerance was too widely scattered to enable statistically significantly means to be deduced. Comparison between the arithmetical means of the four groups, however, showed a greater resistance in aged opposed to young subjects, and in patients with amyloidosis as opposed to those in the other three groups. This was constant and marked after 60' and 120', suggesting that this test may offer indirect evidence in support of a diagnosis of amyloidosis.

Adult↗
PubMed1999

[Amyloidosis. A review].

Amyloidosis is a heterogenous group of diseases, all characterized by extracellular deposition of amyloid either systemically or localized. Of wellknown diseases are Alzheimer's dementia, AL-amyloidosis (e.g. in multiple myeloma) and AA-amyloidosis (e.g. in rheumatoid arthritis). Amyloid is composed of three components of which the fibrillary component is the basis of amyloid classification. Many types of amyloid have a systemic distribution and give rise to varying symptoms. The diagnosis is based on biopsy, preferably of abdominal subcutis. The prognosis is poor, however, recent investigations on the three-dimensional structure of the P-component provide hope for future therapy.

Amyloid↗
PubMedDate not supplied

[Fibrinolytic activity of the urine during chronic glomerulonephritis and amyloidosis].

Correlative interconnections between plasminogen activator (PA) activity (fibrin plate method) and level of urokinase antigen (Ag UAP) and tissue PA antigen (Ag TAP) in urine and blood (ELISA) were studied in 60 patients with chronic glomerulonephritis (CGN) and 38 patients with amyloidosis. The high degree of positive correlation between blood and urine initial PA activity and Ag UAP content was found. This suggests the possible leading role of UAP in formation of the basal fluctuations of fibrinolytic activity in blood and urine. High degree of correlation--r = +0.84 and p < 0.001--was found between blood Ag UAP and urine Ag TAP in amyloidosis only. The functional protein loading probe revealed great importance of high urine and blood AP activity in realizing of ultrafiltration renal process--in CGN and amyloidosis.

Amyloidosis↗
PubMed1999

[Multiple nodular pulmonary AA amyloidosis. A case report].

Amyloidosis is a uncommon disease which affect respiratory system with well defined patterns. We describe a case of multiple nodular pulmonary amyloidosis in a 81 year old man mimicking other entities: metastatic disease, tuberculosis and other granulomatose diseases. In our patient amyloide substance was type AA, being type AL the most frequent one in nodular pulmonary amyloidosis.

Aged↗
PubMed1999

Laryngeal amyloidosis with laryngocele.

Both laryngocele and laryngeal amyloidosis are uncommon, and simultaneous occurrences of these entities are extremely rare. A case of laryngeal amyloidosis with laryngocele in which the computed tomography (CT) and magnetic resonance (MR) imaging of the larynx, clearly demonstrating both disease processes, is discussed. Diagnosis is confirmed by histopathologic specimens. Only two cases have been reported in the world literature, and this is the third case of laryngeal amyloidosis associated with laryngocele.

Amyloidosis↗
PubMedDate not supplied

Localized AL amyloidosis of the colon and clinical features of intestinal obstruction. A case report.

The term amyloidosis refers to a group of disorders characterized by the extracellular accumulation of insoluble, fibrillar proteins (i.e. amyloid) in various organs and apparatuses. Local deposition of amyloid without systemic involvement is a rather uncommon form that gives rise to an amyloid pseudo-tumor or "amyloidoma". This paper describes the clinical features of an intestinal subocclusion observed in an elderly patient with localized primary amyloidosis of the transverse colon. The endoscopic picture indicated a stenosing neoplasm. Segmentary colectomy, however, followed by histological examination (characteristic green color in polarized light after staining with Congo red) and immunohistochemical analysis of the resected tissue, revealed massive deposits of amyloid composed of lambda light chains in the interstitial connective, and perivascular tissue and muscular tunic, and resulted in a diagnosis of AL amyloidosis. This was successfully treated with a combination of melphalan and prednisone.

Aged↗
PubMed1999

[Corneal amyloidosis complicated by trichiasis--immunohistochemical identification of the amyloid light chain protein].

PURPOSE: To identify the amyloid protein of the corneal amyloidosis complicated by trichiasis. METHODS: The two patients were 41-year-old and 38-year-old women with trichiasis. They had gelatinous drop-like corneal change in hemilateral eye. The lesion was excised and examined by light and electron microscopy. Additionally, we performed an immunohistochemical study with immunofluorescence techniques using cryosections. RESULTS: The amyloid deposits were confirmed with light and electron microscopy. Congo red positive staining was not reduced following pretreatment with potassium permanganate. Immunohistochemically, amyloid deposits in the cornea stained positively with serum human light chain kappa and lambda. Pretreatment of the section with 0.05% Tween-20 did not decrease the staining with fluorescence. The deposits stained negatively with serum prealbumin and keratin antibodies. CONCLUSIONS: These findings indicate the protein of the corneal amyloidosis complicating trichiasis to be an amyloid light chain (AL) protein that has never been identified in this kind of corneal amyloidosis.

Adult↗
PubMed1999

[Treatment with chlorambucil of renal amyloidosis secondary to rheumatoid arthritis. Study of 6 cases].

BACKGROUND: Amyloidosis is a common complication of rheumatoid arthritis. Renal disease is the main manifestation and its usual outcome is the lost of renal function. Some clinical evidences suggest that low-dose treatment with chlorambucil may be effective as therapy of this complication. PATIENTS AND METHODS: The effect of chlorambucil treatment in a group of six patients diagnosed by renal biopsy of renal amyloidosis secondary to rheumatoid arthritis was evaluated. Patients were treated with 0.1 mg/kg body weight/day for a time above a year until reduction of proteinuria. RESULTS: 3 out of 6 patients showed decrease of proteinuria below 500 mg/day, two patients have started renal replacement therapy and one died without response to treatment. It was detected non reversible azoospermia in one patient. Mean follow-up time has been sixty months. CONCLUSIONS: Chlorambucil may be effective in the treatment of renal amyloidosis secondary to rheumatoid arthritis when treatment starts before renal function is impaired. Long term remissions can be obtained even after suspending treatment.

Adult↗
PubMed1999

Pulmonary alveolar-septal amyloidosis presenting as heart failure with unilateral pleural effusion: a case report.

We report a case of pulmonary alveolar-septal amyloidosis associated with chylothorax and paraproteinemia initially presenting as congestive heart failure with unilateral pleural effusion. A 72-year-old man was initially diagnosed with congestive heart failure based on the correlation of clinical manifestation and chest radiography. Concentric left ventricular hypertrophy with mild hypokinesis of the left ventricle was found on cardiac echography. Thoracocentesis was performed for right-sided pleural effusion, which persisted despite medical treatment; chylous exudate was aspirated. Because the etiology of the exudative pleural effusion was undetermined, the patient underwent a thoracotomy that showed pulmonary alveolar-septal amyloidosis. Immunoglobulin M paraproteinemia was identified by serum immunoelectrophoresis. We conclude that it is imperative to search for the cause of an undetermined exudative pleural effusion, with particular attention to chylothorax and amyloidosis as the differential diagnoses.

Aged↗
PubMed1999

[Amyloidosis and renal failure].

A heterogeneous group of disorders associated with abnormal extracellular deposition of fibrillar proteins is defined amyloidosis. The renal involvement may occur in the absence of a recognized underlying disease or the kidney is affected as a result of systemic amyloidosis. Even if the diagnosis can only be confirmed by demonstrating the presence of amyloid deposits in the tissues, the development of a radiolabelled serum amyloid P component as a diagnostic nuclear tracer and the reduced urinary excretion of glycosaminoglycans as a decrease in the synthesis of functioning glomeruli and trapping by amyloid fibrils allow new diagnostic insight for the future. Patients maintained on haemodialysis or continuous ambulatory peritoneal dialysis for long develop amyloid deposits composed of beta 2 microglobulin that are predominantly osteoarticular, associated with carpal tunnel syndrome, large-joint pain, stiffness and pathological fractures. Systemic amyloidosis and some local forms are progressive and no treatment specifically resolves the amyloid deposits but therapy may reduce amyloid precursor proteins and improve survival.

Amyloidosis↗
PubMed1999

Secondary amyloidosis associated with giant cell arteritis/polymyalgia rheumatica.

Although giant cell arteritis (GCA) is characterized by chronic inflammation, secondary (AA) amyloidosis appears to be an exceptionally rare complication of this disorder. We describe an 84-year-old man with biopsy proven GCA and polymyalgia rheumatica (PMR) who was found at autopsy to have AA amyloid deposition in numerous organs, 9 years after his diagnosis of GCA. Persistent musculoskeletal symptoms, attributed to refractory PMR during the patient's life, were likely due to AA amyloidosis. This unrecognized complication of GCA/PMR confounded his therapy, leading to excessive treatment with corticosteroids and methotrexate. This case shows that the occurrence of AA amyloidosis should be considered in patients with "refractory PMR" developing after a period of treatment, and that autopsies play a vital role in enigmatic cases.

Aged↗
PubMed1999

[Amyloidosis of the larynx].

The paper presents three cases of localised amyloidosis of the larynx. The study was focused on case histories, findings of larynx examination and method of treatment was discussed. Amyloidosis is a disease characterised by gathering of amyloid deposits in extracellular space. The amyloid fibres consist of different kinds of proteins which have the ability of forming laminae. This disease may be secondary to numerous general processes in which amyloid proteins forms. Etiopathogenesis of this disease has not been explained yet. In the following paper the latest theories and classification of amyloidosis are presented.

Adult↗
PubMed2000

Clotting alterations in primary systemic amyloidosis.

BACKGROUND AND OBJECTIVE: The bleeding manifestations frequently observed in patients with immunoglobulin light chain amyloidosis (AL) have been attributed to different pathogenetic factors: amyloid deposits in several organs and systems leading to failures of these latter, the affinity of amyloid for some clotting factors, and the presence of plasma components interfering with fibrin formation could all induce alterations of clotting tests. This investigation was aimed at defining the prevalence of clotting abnormalities and their clinical manifestations in patients with AL. DESIGN AND METHODS: Thirty-six consecutive patients with biopsy proven amyloidosis and documented monoclonal gammapathy were enrolled within one year. The following clotting tests were considered in the study: activated partial thromboplastin time (aPTT), prothrombin time (PT), thrombin time (TT), reptilase time (RT), Russell's viper venom time (RVTT), fibrinogen, factor X and alpha-2 antiplasmin. RESULTS: Hemorrhagic manifestations were mild to moderate in nine patients, but severe and untractable in one. The most frequent clotting anomaly was defective fibrinogen conversion to fibrin, as demonstrated by prolongation of both TT (85% of cases) and RT (90% of cases). Low levels of factor X activity were observed in about 1 out of 4 samples, while fibrinogen and alpha2 antiplasmin levels were distributed over a wide range of values. PT was prolonged in 8 and aPTT in 25 patients. The search for lupus anticoagulant was negative in samples showing a prolongation of aPTT and/or RVVT. INTERPRETATION AND CONCLUSIONS: The prolongation of TT and RT is not dependent on either the presence of a heparin-like substance in the plasma or on fibrinogen levels; furthermore, the prolongation of RVVT is not related to factor X level. The hypothesized presence in the plasma of an inhibitor of fibrin formation could also affect factor X activation by Russell viper venom. The prolongation of TT and RT represents a peculiar feature of amyloidosis. The variability in the behavior of the other clotting times and hemostatic factors studied is mirrored in the heterogeneity of the clinical features observed in this disease.

Adult↗
PubMed2000

[Prevalence of subcutaneous, labial and rectal amyloid lesions in patients wi histologically confirmed renal amyloidosis].

Our work is to evaluate the biopsies rectal (RB), cutaneous and one about the accessory salivary glands (ASGB) in the diagnosis of amyloidosis renal through 20 cases of renal amyloidosis confirmed by renal biopsy in unit of nephrology and hemodialysis in UHC lbn Rochd from February 1996 to January 1998. The mean age of our patients was 39 years old (15-80) with a male predominance of (4/1). The infectious pathology has been the most frequent cause (70%) which consisted essentially in the tuberculosis and the surinfection on dilatation of the bronchi. All the patients had nephrologic symptomatology dominated by the nephrotic syndrome. The 4 biopsies were practised in the 20 patients. Wright's reaction practised in all renal biopsy confirmed the AA nature of the amyloidosis. The amyloïd deposits were noticed in 100% of renal needle biopsy, 80% of accessory gland biopsy, 75% of rectal biopsy and in 35% of cutaneous biopsy. The association of RB and ASGB was positive in 90% of the cases. If the renal biopsy gave more positivity than the other biopsies, it caused complications that were severe sometimes. Also, the biopsy of the ASG, the simple technique is very reliable in the recognition of the amylosis and seems to be the best diagnostic test for this affection.

Adolescent↗
PubMed2000

Altered platelet shape change in hereditary gelsolin Asp187Asn-related amyloidosis.

Hereditary gelsolin-related amyloidosis (AGel amyloidosis) is a systemic disorder caused by a G654A or G654T mutation in the gene coding for gelsolin, an actin-modulating protein. Altered platelet shape change has been demonstrated in gelsolin-deficient knock-out mice, but this has not been studied in humans with gelsolin deficiency. We measured platelet shape change, characterized by maximal decrease in light transmission (D) and reaction time (T), and aggregation, associated with stimulation of platelets with different agonists in platelet rich plasma, as well as coagulation factor VIII and ristocetin cofactor activities in 20 patients, 10 healthy sibs and 20 healthy control subjects. Statistically significant alterations of parameters describing platelet shape change (D, T) were observed after stimulation with adenosine diphosphate and collagen in patients when compared to healthy subjects, but not in maximal aggregation responses, platelet counts, coagulation factor VIII or ristocetin cofactor activity levels. Patients had more haemostatic derangements. Our results suggest that, in addition to amyloid deposition, the G654A gelsolin gene defect causes altered gelsolin-mediated cellular mechanisms, which may contribute, e.g., to bleeding tendency in AGel amyloidosis patients.

Adult↗
PubMed1999

Renal transplantation in systemic amyloidosis.

Renal transplantation was performed in a 24-yr-old woman with type AA systemic amyloidosis. Renal biopsy at 11 yr post-transplantation showed no amyloid recurrence, but the presence of mesangial proliferative glomerulonephritis and focal segmental glomerular sclerosis. Renal function was satisfactory with the exception of mild proteinuria. Symptoms related to systemic amyloidosis including goiter and cardiac deposition, improved post-transplantation. Renal transplantation is the recommended therapy for type AA systemic amyloidosis.

Adult↗
PubMed2000

[Cardiac amyloidosis].

In our experience, QS pattern of poor R wave progression and atrio-ventricular block of varying degrees on electrocardiogram, cardiomegaly with pleural effusion on chest X-ray film, left ventricular wall thickening, pericardial effusion and findings suggesting left ventricular diastolic dysfunction on echocardiogram and increased right ventricular end-diastolic pressure in cardiac catheterization were frequently observed in patients with cardiac amyloidosis. Though none of these findings are specific, we should suspect cardiac amyloidosis as a possibility when some of these signs are observed in patients with chronic cardiac failure of unknown etiology. Left ventricular mass obtained from echocardiography could be useful predictive parameter of prognosis in patients with cardiac amyloidosis.

Amyloidosis↗