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PubMed1992

Amyloidosis.

Amyloidosis is a heterogenous group of diseases characterized by deposition of a fibrillar, proteinaceous material, amyloid, in various tissues and organs. Increasing knowledge about the different proteins that constitute the amyloid fibrils has made it possible to classify amyloidosis by the fibril protein, which appears more rational than the traditional classification by its clinical expression. A serum protein is the precursor of the amyloid fibril protein in the various systemic forms of amyloidosis. Although the chemical composition of amyloid is presently well known, the pathogenetic processes that convert such proteins into a fibrillar form and lay them down in the tissues are far from clarified. This review describes the amyloid deposits, some putative pathogenetic mechanisms, and the clinical, therapeutic, and prognostic aspects of the most important forms of amyloid disease.

Amyloid↗
PubMed1978

Solitary primary amyloidosis of urinary bladder. Light and electron microscopic study.

A fifty-four-year-old white woman with a history of episodic gross hematuria was diagnosed as having solitary primary amyloidosis of the urinary bladder involving a large segment of the dome and anterior wall. A segmental resection of the bladder was performed with good results. Electron microscopic examination of the lesion revealed evidence to indicate that amyloid in this case was produced by the fibroblasts within the lamina propria of the bladder. The mode of amyloidogenesis is identical to that noted in some of the previously published electron microscopic studies on localized amyloidosis of skin. Solitary primary amyloidosis of the urinary bladder is a rare lesion with only 46 cases having been previously reported in the literature.

Amyloid↗
PubMed1988

Primary amyloidosis of lower urinary tract.

Primary amyloidosis of the lower urinary tract is a rare condition with an excellent prognosis in most cases. Three patients with this condition are described. In the cases of localized amyloidosis of the urethra and urinary bladder, the clinical presentation mimicked cancer of the respective sites. This was also true in the case of primary systemic amyloidosis involving the bladder. If significant associated systemic or local disease can be excluded, management is symptomatic and expectant.

Aged↗
PubMed1982

Localized laryngeal amyloidosis: partial characterization of an amyloid fibril protein AL.

Amyloid fibrils were extracted from a patient Wr with more than 10 yr history of localized laryngeal amyloidosis. Degraded amyloid fibrils reacted in immunodiffusion with an antiserum against an amyloid protein of immunoglobulin kappa light chain origin, showing a line of identity with a kappa I amyloid protein. The protein Wr had a blocked aminoterminal, previously only reported in lambda chains. Amino acid sequence analysis of a fragment of the protein showed it to be an immunoglobulin light chain protein of V kappa I or V kappa III subgroup. The protein had a few unusual amino acid residues as compared to other kappa light chains. The findings support the view that the fibrils in localized, tumour-like amyloidosis are composed by homogeneous immunoglobulin light chain proteins in the same way as is seen in primary and myeloma associated systemic amyloidosis. It is possible that unusual light chains are over-represented in amyloid fibrils.

Amino Acid Sequence↗
PubMed1991

Senile systemic amyloidosis: a clinico-pathological study of twelve patients with massive amyloid infiltration.

Hearts from 12 patients, 82-92 years old, with immunohistochemically verified pronounced senile systemic amyloidosis, were studied postmortem. Sections from the left ventricle, atrioventricular and sinus nodes and atrioventricular bundle were studied using the light microscope. The weights of the hearts varied between 310 and 870 grams. In the studied parts of the conduction system, the amounts of amyloid were considerably smaller than in the surrounding myocardium. Clinical data were taken from available case records. Eight of the patients had a history of uncomplicated congestive heart failure, but only two had been in hospital with cardiac symptoms prior to their last admission. The electrocardiogram showed atrial fibrillation in nine cases, left bundle branch block in three, right bundle branch block in two and left anterior hemiblock in one. No evidence of atrioventricular dissociation was seen and, in no case, was death considered to be due to conduction disturbances. Amyloidosis was a contributing cause of death in 7 patients, while the remaining patients died of other diseases. Amyloidosis is rarely diagnosed before death and was not suspected during life in any of our patients.

Aged↗
PubMed1991

Sipple syndrome with lichen amyloidosis as a paracrinopathy: pleiotropy, heterogeneity, or a contiguous gene?

A five-generation white family with multiple endocrine neoplasia type 2A had six affected members. Two, a mother and her daughter, had interscapular cutaneous pruritic lesions resembling macular/lichen amyloidosis. In the daughter, light microscopy showed homogeneous aggregates in the papillary dermis. Crystal violet staining showed metachromasia and indicated that the deposits were amyloid. This is the fourth family with familial medullary thyroid carcinoma with cutaneous amyloidosis and as such it allowed comparative analysis. Genetic heterogeneity, a contiguous gene, and pleiotropy were considered. It appears that multiple endocrine neoplasia type 2A/familial medullary thyroid carcinoma with cutaneous amyloidosis represents the phenotypic variability of the expression of a pleiotropic gene. The condition is one of the predominantly ectodermal autosomal dominant phakomatoses and is most likely a paracrinopathy.

Adolescent↗
PubMed1989

Amino acid sequence variations in protein AA of cats with high and low incidences of AA amyloidosis.

1. Amyloid isolated from the liver of a domestic short-haired (DSH) cat was dissolved and purified by gel filtration for amino acid sequence analysis. 2. Sequences of two major peptides corresponding to positions 18-23 and 25-75 of human amyloid protein AA were obtained when cyanogen bromide-cleaved protein was applied to an amino acid sequenator. 3. Comparison of these regions of amyloid protein from the Abyssinian cat (high incidence of AA amyloidosis) and DSH cat (low incidence of AA amyloidosis) revealed three amino acid differences, two of which occurred within regions that are completely conserved in the Abyssinian cat and all other species. 4. Secondary prediction plots showed less potential for amyloidogenicity (i.e., less beta-sheet conformation) in protein AA of the DSH cat as compared to the Abyssinian cat and other animal species. 5. These differences in protein AA of the DSH cat may, therefore, be linked to the comparatively uncommon occurrence of AA amyloidosis in the DSH cat as compared to the Abyssinian cat and other animals species.

Amino Acid Sequence↗
PubMed1990

Comprehensive Doppler assessment of right ventricular diastolic function in cardiac amyloidosis.

To assess right ventricular diastolic function in cardiac amyloidosis, pulsed wave Doppler ultrasound measurements of right ventricular inflow velocities and superior vena cava and hepatic vein flow velocities with respiratory monitoring were performed in 41 patients with primary systemic amyloidosis and two-dimensional echocardiographic features of cardiac involvement. Right ventricular diastolic function was abnormal in 31 (76%) of these patients, the major abnormality being a short deceleration time (less than 150 ms) in 21 (68%), suggesting restriction. In contrast, 7 (23%) of the 31 patients had a decreased ratio of early (E) and late (A) diastolic peak flow velocities and a prolonged deceleration time (greater than 240 ms), suggesting abnormal relaxation. The patients were classified into two groups on the basis of right ventricular free wall thickness: group 1, less than 7 mm and group 2, greater than or equal to 7 mm. Compared with normal values, group 1 showed an increased peak late flow velocity (44 +/- 19 versus 39 +/- 6 cm/s; p less than 0.01) and a decreased E/A velocity ratio (1.1 +/- 0.4 versus 1.5 +/- 0.3; p less than 0.01). Group 2 showed a markedly shortened deceleration time (151 +/- 37 versus 225 +/- 28 ms; p less than 0.01), characteristic of restriction. In the overall group, superior vena cava peak flow velocity was decreased in systole and increased in diastole and flow reversals during inspiration were increased compared with normal values. Hepatic venous flow velocities were similar to those in the superior vena cava except for larger flow reversals in the hepatic vein. Thus, in cardiac amyloidosis, right ventricular diastolic function is abnormal. There is a spectrum of right ventricular filling abnormalities and the restrictive filling pattern is seen only in the advanced stages of the disease.

Amyloidosis↗
PubMedDate not supplied

Primary amyloidosis of the sternum. Imaging findings.

A rare case of focal primary amyloidosis is presented with CT and MR correlation. Imaging findings demonstrate an expansile, mixed sclerotic and lytic lesion in the sternum, with MR signal characteristics similar to those previously described for diffuse primary amyloidosis and hemodialysis-related amyloidosis.

Adult↗
PubMed1994

Cacogeusia in amyloidosis associated with plasma cell dyscrasia.

Cacogeusia, being the patients' main complaint, is reported as a new symptom of amyloidosis, probably caused by a peripheral sensory neuropathy. A case is presented of a patient with weight loss, dysphagia, macroglossia and taste disturbances, due to amyloidosis associated with a plasma cell dyscrasia. The importance of oral manifestations in amyloidosis is discussed.

Aged↗
PubMedDate not supplied

Localized amyloidosis of the tongue: a review.

Amyloidosis is a disease entity defined by the presence of extracellular deposits of proteinacious material. These deposits have a characteristic apple-green birefringence with polarized light after staining with Congo red. Amyloid involvement of the tongue is almost universally secondary to systemic disease. The mean survival of patients with a systemic form of amyloidosis is between 5 to 15 months, whereas those with the localized form have an excellent prognosis. This article presents a case of the more unusual localized form. A thorough evaluation, including abdominal fat or rectal biopsy, is essential in every patient to identify any systemic involvement. The absence of systemic amyloidosis offers a much more favorable prognosis and may be treated with simple surgical excision.

Amyloidosis↗
PubMedDate not supplied

Primary cardiac amyloidosis with 20-year survival.

BACKGROUND: The natural history of primary amyloidosis is poor, and for patients with symptomatic cardiac involvement, survival is generally less than 6 months. Even among treated patients with amyloid heart disease, survival beyond 5 years is rare. CASE REPORT: We report a patient with primary cardiac amyloidosis who is currently alive 20 years after his initial diagnosis. The extent and subtype of amyloid were documented by endomyocardial biopsy both at the time of initial diagnosis and 20 years later. To our knowledge, this is the longest survival ever reported for a patient with cardiac involvement by primary amyloidosis. CONCLUSION: The remarkably long stabilization of amyloid deposition in this patient may be attributed to early diagnosis, early institution of therapy, and, possibly, favorable genetic factors.

Adult↗
PubMed2006

Isolated left atrial amyloidosis: acute premitral stenosis secondary to spontaneous intramural left atrial hemorrhagic dissection.

This case presentation describes the very rare condition of isolated atrial amyloidosis and it's associated complications. A 53-year-old male presented to hospital in severe pulmonary edema secondary to spontaneous intramural left atrial hemorrhage and subsequent wall dissection causing acute "pre-mitral valve" obstruction. The patient was subsequently found to have AL subtype amyloid, which has never before been reported in isolated atrial amyloidosis. The prognosis is poor in AL type amyloidosis with cardiac involvement.

Acute Disease↗
PubMed2006

Pulmonary hypertension as a dominant clinical picture in a case of amyloidosis and smoldering multiple myeloma.

A 48-year-old male patient presented with dyspnea on exertion. Patient was found to have pulmonary hypertension. Myocardial biopsy showed amyloidosis and further work-up revealed Salmon-Durie stage 1A multiple myeloma. Patient had no other clinical manifestations of amyloidosis. It is possible that the pulmonary hypertension is caused by amyloid deposition into pulmonary arteries as the arterial amyloid deposition is common in AL amyloidosis. Treatment with sildenafil led to hemodynamic and symptomatic improvement.

Amyloidosis↗
PubMed2006

Nail dystrophy and blisters as sole manifestations in myeloma-associated amyloidosis.

We report the case of a 61-year-old Japanese man with IgG lambda-type multiple myeloma, who presented with nail dystrophy as the initial manifestation of systemic amyloidosis. Subsequently he developed bullous amyloidosis. This report documents these two rare signs of systemic amyloidosis and demonstrates the precise location of cutaneous blister formation and amyloid deposition by fluorescence antigen mapping and electron microscopy.

Amyloidosis↗
PubMed2005

Amyloidosis in cystic fibrosis: a case series.

As the life expectancy of patients with cystic fibrosis increases, unusual complications including amyloidosis are increasingly recognised. We report three cases of amyloidosis including a unique case presenting with a hemorrhagic and thrombotic diatheisis. This complication of amyloidosis has never been reported in cystic fibrosis.

Adult↗
PubMed2005

Localized orbital amyloidosis involving the lacrimal sac and nasolacrimal duct.

PURPOSE: To report the case of a 70-year-old man who presented with tearing in his left eye and a firm palpable lump in the area overlying his left lacrimal sac. DESIGN: Retrospective interventional case report. METHODS: Noninvasive diagnostic evaluation followed by external dacryocystorhinostomy, histopathologic studies, and systemic evaluation. RESULTS: The patient was found to have idiopathic localized amyloidosis limited to the lacrimal sac and nasolacrimal duct. CONCLUSION: The localized form of amyloidosis is rare, typically involves the head and neck without systemic manifestations, and carries an excellent prognosis. Previous reports of orbital amyloidosis have described involvement of the lacrimal gland, extraocular muscles, and the cranial nerves. To our knowledge, this is the first report of a patient with nasolacrimal duct obstruction secondary to amyloid deposition in the lacrimal sac and fossa.

Aged↗