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PubMedDate not supplied

[Localized amyloidosis of the seminal vesicles].

Amyloidosis of the seminal vesicles is a common finding in autopsies, with increased incidence in older population. It is usually asymptomatic. We report a case of symptomatic localized amyloidosis of the seminal vesicles, with hemospermia and suprapubic pain. Diagnosis was achieved through ultrasound-guided transrectal biopsy. Systemic amyloidosis must be ruled out through proper evaluation. Seminal vesicle enlargement secondary to amyloid deposit may be misdiagnosed as carcinomatous invasion.

Adult↗
PubMed2001

[Good response to colchicine in amyloidosis secondary to inflammatory bowel disease].

Acquired systemic amyloidosis may develop during the course of a number of chronic inflammatory diseases, including inflammatory bowel disease. Amyloidosis, although rare, is life-threatening, especially when it involves the kidneys. It is important to recognize this complication, especially since colchicine has been proposed as a possible treatment.We present a 25-year-old female diagnosed at the age of 13 years with Crohn's disease with involvement of the terminal ileum and right colon. Surgery was performed because of inflammatory activity unresponsive to corticosteroid and immunosuppressive drugs. She subsequently presented nephrotic syndrome due to secondary amyloidosis, which responded well to colchicine treatment.

Adult↗
PubMed1999

[Pseudotumoral adenopathies, an unusual means of detecting systemic amyloidosis].

INTRODUCTION: It is uncommon that lymph node enlargement is diagnostic of systemic amyloidosis as found in the case reported in this study. EXEGESIS: This study examined the case of a 49-year old male with chronic bronchitis in whom in 1990 the presence had been detected of an isolated cervical lymphadenopathy, 2 cm in diameter, and which had previously remained unnoticed. In 1993, a significant number of other peripheral adenopathies also appeared in various locations, i.e., cervical, axillary, inguinal. Chest and abdominal CT-scans revealed several mediastinal and abdominal lymphadenopathies. The histological study with Congo red stain of a cervical lymph node biopsy determined the diagnosis of amyloidosis. The patient was at that time asymptomatic. In September 1997, upon physical examination the following were found: lower limb edema, superior vena cava syndrome, and several cervical lymphadenopathies. Abdominal ultrasonography showed enlarged kidneys, and homogeneous splenomegaly. Biological examination determined the existence of a nephrotic syndrome with renal failure and creatinemia of 350 mumol/L. Due to superior vena cava syndrome worsening, cervical lymph node removal was performed. However, the patient died after rapid renal failure. CONCLUSION: Although it is a rare occurrence, amyloidosis should be taken into consideration in the differential diagnosis of isolated lymphadenopathy. Congo red stain amongst others, and an immunohistochemical study should be performed in cases of uncertain diagnosis.

Amyloidosis↗
PubMed1999

[Value of the biopsy of accessory salivary glands in amyloidosis].

PURPOSE: To assess the value of accessory salivary gland biopsy for the diagnosis of amyloidosis, a study was conducted in the nephrology and hemodialysis department at Ibn Rochd University Hospital from February 1996 to January 1998. METHODS: Renal amyloidosis was confirmed by renal biopsy accompanying accessory salivary gland biopsy. RESULT: The patient's mean age was 39 years old (range 15-80), with a 4:1-male/female ratio. An infectious cause (either tuberculosis or superinfection and dilatation of the bronchi) was the most frequent (70% of the cases) etiology. All the patients presented renal symptomatology. Nephrotic syndrome predominated. Amyloid deposits were observed in 100% of renal needle biopsies and in 80% of accessory salivary gland biopsies. CONCLUSION: Renal biopsy led to more positive cases than the other biopsies. It may be accompanied by severe complications. Furthermore, biopsy of the accessory salivary glands is a simple and very reliable technique for the diagnosis of amyloidosis. It is currently the best diagnostic test.

Adolescent↗
PubMed1990

Beta 2-microglobulin amyloidosis in a patient treated exclusively by continuous ambulatory peritoneal dialysis.

The histological demonstration of beta 2-microglobulin (B2M) amyloidosis in a 75-year-old man treated exclusively by continuous ambulatory peritoneal dialysis (CAPD) for 87 months is reported. Amyloid deposits were detected in all joints from which synovial specimens were obtained at autopsy. It is of note that the patient had never undergone hemodialysis and had suffered only one episode of peritonitis. Both features are of interest, as the few reports of dialysis-related amyloidosis (DRA) in CAPD include patients who have been hemodialyzed and have suffered repeated episodes of peritonitis, both conditions being incriminated in the genesis of B2M amyloidosis. We conclude that DRA may develop in the absence of artificial membranes or frequent peritonitis episodes.

Aged↗
PubMed1988

B2-microglobulin and associated amyloidosis presenting as bilateral popliteal tumors.

Chronic hemodialysis has led to the prolongation of life in many patients with end-stage renal disease, but has also allowed for the development of new diseases that are a consequence of this clinical setting. B2-microglobulin accumulation leading to systemic amyloidosis may be the most recent disease in this category. This case report documents the development of bilateral popliteal tumors in a patient undergoing chronic hemodialysis for 9 years. Removal of one of the tumors and pathological examination demonstrated amyloid that was positive for B2-microglobulin by specific antibody testing. This case adds further support to the suggestion that B2-microglobulin amyloidosis in chronic hemodialysis patients is truly a systemic disorder. The development of popliteal tumors, particularly in proximity to joints, in a chronic hemodialysis patient, must include amyloidosis in the differential diagnosis.

Aged↗
PubMed1996

Extracapillary glomerulonephritis and renal amyloidosis.

We report three patients with well-documented renal amyloidosis who developed rapidly progressive renal failure. Renal biopsies from all three patients showed crescentic glomerulonephritis imposed on renal amyloidosis. All patients were treated with intravenous high-dose methylprednisolone pulses combined with immunosuppressive agents and oral corticosteroids. Partial recovery of renal function was obtained in two patients. For the third patient, treatment had to be stopped after a few days because of a septic arthritis. Renal function continued to deteriorate, and the patient had to be placed on regular hemodialysis. We conclude that extracapillary glomerulonephritis may occasionally complicate a preexisting renal amyloidosis and may be reversible if recognized early and treated appropriately.

Aged↗
PubMed1997

[Cardiac pathology of extracardiac origin (II). The cardiac repercussion of amyloidosis and hemochromatosis].

Although rare, amyloidosis and hemochromatosis are the infiltrative diseases in which the heart is more frequently involved. The most common clinical presentation is heart failure with hemodynamic features of restrictive heart disease in cardiac amyloidosis. The diagnosis is often made because of symptoms of other organ involvement, although sometimes cardiac symptoms may be the initial manifestation. The non-specific clinical presentation and the low prevalence of these cardiomyopathies make the diagnosis difficult if the clinician does not suspect it. Once symptoms develop, the evolution is fast. Usually, the unsatisfactory and ineffective treatment of amyloidosis and hemochromatosis contribute to the poor prognosis. The indication of cardiac transplantation in advanced cases is questionable because of the high recurrence of the illness.

Amyloidosis↗
PubMed2003

Electrophysiological studies on the hippocampus and prefrontal cortex assessing the effects of amyloidosis in amyloid precursor protein 23 transgenic mice.

In vitro and in vivo electrophysiological studies were done to investigate the neuronal function of the hippocampus and prefrontal cortex in the amyloid precursor protein (APP) 23 transgenic mouse model for amyloidosis developed by Sturchler-Pierrat et al. [Proc Natl Acad Sci USA 94 (1997) 13287]. Brain slices were taken from 3, 6, 9, 12, 18 and 24 month old wildtype and hemizygous type APP23 mice. Extracellular field potentials were recorded from the CA1 region of the hippocampus while stimulating the Schaffer collaterals. In addition, extracellular field potentials were elicited from areas within layer V/VI of the prefrontal cortex by stimulating the same layer V/VI. Basic synaptic function in the hippocampus was reduced in hemizygous APP23 mice compared with their wildtype littermates at 12 and 18 months of age, whereas, it was unaltered within the prefrontal cortex. Long-term potentiation in the hippocampus and the prefrontal cortex of hemizygous APP23 mice was similar compared with their wildtype littermates. In vivo electrophysiological experiments were done in 3, 9, 18 and 24 month old wildtype and hemizygous APP23 mice. No differences were observed in the number of single spontaneously active units recorded within the prefrontal cortex of hemizygous APP23 mice compared with their wildtype littermates. Field potentials elicited during stimulation of cortico-cortical pathways to assess synaptic transmission and short-term synaptic plasticity were also unchanged in hemizygous APP23 mice. Furthermore, presumable antidromic field potentials recorded in the prefrontal cortex during stimulation of the striatum were similar between the hemizygous APP23 and wildtype mice at each age. The present study shows that amyloidosis impairs basic synaptic function but not long-term potentiation in the hippocampus, however, does not alter any of the neurophysiological functions measured within the prefrontal cortex. These findings suggest that amyloidosis may be involved in altering some neurophysiological functions within only certain brain structures. Although APP23 mice have impaired cognitive performance, long-term plasticity, a cellular model for memory, is not affected, raising the question on the relationship between these processes.

Action Potentials↗
PubMed2001

CT and MR imaging of localized amyloidosis.

The localized form of amyloidosis affecting the head and neck region is rare. The characteristic features of localized amyloidosis appeared as multiple nodules on the tongue, lip, and cheek on computed tomography (CT) and magnetic resonance imaging (MRI). Contrast-enhanced CT scans represented this lesion as a marked nodular enhancement. MR features of this lesion appeared slightly low on T1-weighted images and slightly high on T2-weighted images compared with T1-T2 images of residual normal tongue. Time contrast intensity curves obtained from dynamic MRI rapidly increased to reach a plateau and gradually decreased during the late and delayed phases. MR findings suggest that such lesions might be comprised of fibrous tissue with abundant vessels. This report suggests that dynamic MRI might be helpful for diagnosing localized amyloidosis.

Aged↗
PubMed2002

Primary diffuse tracheobrochial amyloidosis: case report.

Respiratory amyloidosis is a rare disease that occurs in three forms: tracheobronchial, nodular parenchymal and diffuse parenchymal involment. Tracheobronchial amyloidosis is characterized by focal or diffuse deposition of amyloid in the submucosa of the trachea and proximal bronchi. Herein, we report a case of diffuse tracheobronchial amyloidosis with plain radiography and thorax computed tomography findings.

Amyloidosis↗
PubMed1984

Cardiac arrhythmias in systemic amyloidosis: correlation with echocardiographic abnormalities.

To determine the prevalence of cardiac arrhythmias in patients with systemic amyloidosis 24 hour electrocardiographic monitoring was performed in 27 patients with primary amyloidosis and in 6 patients with familial amyloid polyneuropathy. All patients underwent echocardiographic studies. Despite a high prevalence of conduction disturbances on standard electrocardiogram, clinically significant bradyarrhythmias were rare (one patient). Complex ventricular arrhythmias (multiform, paired or repetitive beats) occurred in 14 patients (47%) with primary amyloid and 3 patients (50%) with familial amyloid polyneuropathy. The presence of cardiac arrhythmia correlated with heart failure and, more strongly, with an abnormal echocardiogram. There were four sudden deaths, all in patients with abnormal echocardiograms and complex ventricular arrhythmias. These findings suggest that complex ventricular arrhythmia on Holter monitoring is common in cardiac amyloidosis and may be a harbinger of subsequent sudden cardiac death.

Adult↗
PubMed2004

[Renal AA amyloidosis secondary to bronchiectasis: a report of two cases (including one with Mounier-Kuhn syndrome)].

INTRODUCTION: We describe 2 patients with AA renal amyloidosis secondary to bronchiectasis (one patient had tracheobronchomegaly or Mounier-Kuhn syndrome). CASE REPORTS: The time period between the diagnosis of bronchiectasis and the development of renal amyloidosis was 40 years and 30 years respectively. Both patients evolved to end-stage renal failure requiring dialysis. CONCLUSION: Although the incidence of renal amyloidosis secondary to bronchectasis has declined, it remains a dreadful complication.

Aged↗
PubMed1998

A simple screening test for variant transthyretins associated with familial transthyretin amyloidosis using isoelectric focusing.

Variant forms of the plasma protein transthyretin (TTR) are associated with the most frequently occurring type of familial systemic amyloidosis. Organ system involvement in transthyretin type amyloidosis (ATTR) is often similar to that which occurs in light chain amyloid disease (AL). The proper diagnosis of ATTR is important since treatment (liver transplantation) differs from that in AL (chemotherapy). We present a two-step test to screen sera for variant TTRs using non-denaturing gel electrophoresis performed in 7.5% acrylamide (PAGE) followed by isoelectric focusing (IEF) between pH 4.0 and 7.0 in 2.5 M urea. Serum samples from 110 patients with amyloidosis and their relatives were tested using this IEF technique and compared to genetic mutation results. Sera from patients with ATTR who underwent liver transplantation were also examined prior to and following surgery. IEF analysis showed the presence of both wild-type and variant TTR in 74 of the 110 serum samples tested. Genomic DNA from peripheral blood was used to identify TTR gene mutations in 77 of the 110 patients. Fifteen variants including Val122Ile, preponderant in the African-American population, could be demonstrated by IEF. The sensitivity of IEF was 96% (74/77) and the specificity was 100% (33/33). The predictive values for a positive or negative result were 100% (74/74) and 92% (33/36), respectively. There were no false-positive results and 4% (3/77) false-negative results. In sera from patients with ATTR who underwent liver transplantation, variant TTR was detected by IEF before, but not after, surgery. A simple, accurate, sensitive method is presented as a useful screening test for variant transthyretins associated with ATTR.

Amyloidosis↗
PubMed1998

[Secondary kidney amyloidosis and mucoviscidosis].

BACKGROUND: Due to the improvement in cystic fibrosis management, life expectancy has risen; on the other hand, longer survival has led to new complications, including secondary renal amyloidosis, which has been so far very uncommonly reported. CASE REPORTS: Secondary nephropathic amyloidosis was seen in two 25-year and 22-year-old adults with cystic fibrosis. Both had developed recurrent pulmonary infections due to Pseudomonas aeruginosa over several years. One patient died after 2 years of progressive kidney failure. CONCLUSION: Severe renal insufficiency due to secondary amyloidosis may complicate the course of cystic fibrosis and become the main prognosis factor in adults.

Adolescent↗
PubMed1994

Animal models for reactive amyloidosis.

For over 70 years animal experiments have been performed to elucidate the pathogenesis of reactive amyloidosis and to investigate the formation of the beta-pleated sheet-rich amyloid fibrils in general. In appropriate species, primarily rodents like mouse and hamster, amyloid is formed after stimulation with amyloid-inducing injections after a lag phase (secondary or reactive amyloid, AA amyloid). For the formation of this AA amyloid, elevated values in blood of its precursor protein, SAA, is the first prerequisite. SAA is an acute phase protein of hepatic origin, released after stimulation by cytokines, and is associated in serum with high-density lipoprotein (apoSAA). In mouse, hamster and mink amyloidogenic subtypes of SAA are found. In the rat SAA is absent, although its mRNA is transcribed. Evidence is increasing that SAA crystallizes to fibrils first, whilst loss of its C-terminal end can be a post-fibrillogenic phenomenon. Glycoproteins, proteoglycans, glycosaminoglycans and lipids are reintroduced in experimental amyloid research. Basement membrane heparan sulphate proteoglycans (perlecans) are attributed to have a primary role. The pentraxin serum amyloid P-component is a calcium-dependent secondary phenomenon. Membrane-bound, lipid-rich vesicles are found amongst the newly deposited pericellular amyloid fibrils. These vesicles probably have to be interpreted as indicators of primary membrane alteration during amyloid fibril crystallization. The vesicles will be formed after rupture of the membranes caused by the stiff intramembranously crystallized protein fibrils. Morphological evidence supporting this hypothesis has been found in immunoelectron microscopical studies. Accumulation of intramembranous SAA preceded amyloid fibril deposition. Fibril formation then might be related to conformational change of the intramembranous SAA. The lag phase for amyloid deposition is shortened after a single injection of a fraction of amyloid, the AEF. It is a low-molecular-weight glycoprotein that easily associates with other molecules. When isolated from amyloid fibrils, the (F)AEF contains a large proportion of beta-pleated sheet molecular structure. It is probable that this structure holds an explanation for its enhancing potency: forming a nidus for physical crystallization. The major substances and animal species used in animal experiments on amyloidosis, are mentioned. Overlooked by-effects of amyloidogenic stimuli are discussed. Polyarthritis after systemic endotoxin injections found in the hamster acts as a source of cytokines, further triggering the reactive amyloidosis.(ABSTRACT TRUNCATED AT 400 WORDS)

Amyloidosis↗
PubMed1994

Clinical and therapeutic aspects of AA amyloidosis.

Approach to the management of AA amyloidosis complicating RA. (A) In case of proteinuria or loss of renal function a rectal biopsy or a subcutaneous fat biopsy is a suitable screening method for the detection of amyloidosis. If in any doubt, try to ascertain the diagnosis by renal biopsy. Adequate staining with alkaline Congo red and preferably immunohistochemical staining with anti-AA antibodies should be performed. Beware of renal pathology other than amyloidosis even in the presence of a positive rectal biopsy. (B) A vigorous attempt to control disease activity of the RA should be made in order to eliminate the production of SAA, an acute phase protein. The response to treatment should be monitored by serial measurements of CRP and preferably SAA. (C) The function of some vital organs should be evaluated: (a) endogenous creatinine clearance and the extent of proteinuria; (b) electrocardiogram and optional echocardiography; (c) thyroid function and adrenocortical function; (d) intestinal absorption tests; (e) optional--SAP scintigraphy and turnover studies. (D) Attention should be given to adequate supportive treatment: (a) blood pressure control; (b) treatment of intercurrent infections; (c) corticosteroids during major surgical procedures; (d) pay attention to the possible effect of NSAID on proteinuria and renal function. (E) In case of total renal failure or uncontrollable proteinuria: (a) consider the possibility of primary renal transplantation; (b) otherwise regular haemodialysis is indicated.

Amyloidosis↗
PubMed1999

A reaction to tape after tracheal extubation in a patient with systemic amyloidosis.

A reaction to tape after tracheal extubation in a patient with systemic amyloidosis is reported. A patient underwent a right thoracotomy with general anesthesia. A double-lumen tube secured with 1-inch adhesive tape (3M Blenderm, 3M Health Care, St Paul, MN) was used. The same kind of tape was used to cover the eyelids. On removal of the tape, hemorrhagic and purpuric lesions appeared on the skin in regions corresponding to the tape contact area. A diagnosis of amyloidosis was made based on large tongue, lip edema, purpuric and hemorrhagic spots on the skin, congestive heart failure, and skin biopsy. Amyloidosis and its anesthetic implications are discussed.

Adhesives↗