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At least 685 records · Page 38Linked to original sources
PubMed2005

Macroglossia secondary to systemic amyloidosis: case report and literature review.

Amyloidosis is characterized by an abnormal extracellular deposition of amyloid in different tissues and organs, where it usually causes some type of dysfunction. Its cause is unknown. The two main forms of amyloidosis are systemic and localized; the latter is rare. No satisfactory treatment for systemic amyloidosis has been discovered, and mean survival is poor, ranging from 5 to 15 months depending on the presence or absence of multiple myeloma. We report a case of primary systemic amyloidosis in a 71-year-old man. The diagnosis of amyloidosis was established by tongue biopsy, and its systemic nature was identified by analysis of aspirated abdominal fat. At the 1-year follow-up, the patient's clinical condition had not changed, and he was thereafter lost to follow-up.

Aged↗
PubMed2005

Update for nurse anesthetists--anesthetic considerations for patients with amyloidosis.

Amyloidosis is a rare disease process that results in the deposition of insoluble, fibrous amyloid proteins in extracellular spaces and tissues. Amyloid fibrils can be deposited locally or may involve every organ system of the body. Advancements in the treatment for amyloidosis allow longer survival, and patients are being seen in our operating rooms for diagnostic, interventional, and curative purposes. Amyloidosis has numerous implications for anesthesia providers due to the possibility of systemic involvement. This course describes 2 cases of amyloidosis and discusses the types of amyloidosis and their anesthetic implications.

Amyloidosis↗
PubMed2006

[A case of secondary amyloidosis presenting as massive gastrointestinal bleeding].

Amyloidosis is a disorder characterized by extracellular deposition of amyloid in various tissues and organs. Gastrointestinal manifestations including gastroparesis, constipation, malabsorption, intestinal pseudo-obstruction, and bleeding are common. GI bleeding is a rare initial symptom which can be fatal in some cases. Absence of systemic symptoms and nonspecific endoscopic findings in amyloidosis may make diagnosis difficult. Therefore, amyloidosis-induced GI bleeding should be considered in patients with an obscure hemorrhage. Recently, we experienced a 65-year-old woman who presented with massive hematochezia as a manifestations of amyloidosis. Colonoscopy and SMA angiography showed massive bleeding in the small and large intestine. Colonoscopic biopsy established amyloidosis. We report this case with a review of the relevant literatures.

Aged↗
PubMed2006

[Amyloidosis of familial Mediterranean fever (FMF)--insights to FMF phenotype II].

Amyloidosis is the most grievous manifestation of Familial Mediterranean Fever (FMF), occurring in a high proportion of untreated patients. Continuously elevated serum amyloid A (SAA) levels during remissions, rather than a pulsatile rise during FMF attacks, underlies the development of amyloidosis. FMF phenotype II is one extreme of AA amyloidosis, evolving despite a complete absence of FMF attacks. FMF phenotype II is diagnosed in patients with AA amyloidosis in the context of a family history of FMF. In these patients and in patients with AA amyloidosis without family history of FMF and with unknown precipitating disease, MEFV gene analysis is mandatory. Moreover, since FMF phenotype II is an actual hazard, a cost-benefit analysis suggests that MEFV mutation determination in all first-degree family members of FMF patients is warranted, as it will significantly reduce future patient treatment costs.

Amyloidosis↗
PubMedDate not supplied

Amyloidosis: clinical picture, immunological and biomolecular features, treatment prospects.

Amyloidosis is the name given to a group of clinically protean diseases whose common feature is the tissue accumulation of amyloid fibrils which have specific optical and staining properties, and are both insoluble in physiological solvents and resistant to proteolytic enzymes. Fibril deposition and progressive extracellular infiltration eventually result in atrophy due to compression. The structure of these fibrils embraces a wide range: immunoglobulin light chain or their fragments, acute phase proteins, hormones, protease inhibitors, beta 2-microglobulin, natriuretic peptides, and proteins whose function is still unknown. Despite this heterogeneity, however, they share a common crystallographic beta-pleated sheet structure. The clinical spectrum includes apparently primary forms, amyloidosis of myeloma, forms secondary to familial Mediterranean fever, Alzheimer's disease, forms associated with type 2 diabetes or medullary carcinoma of the thyroid, inherited-familial amyloidosis, and other less common conditions. Two pathogenetic phases are involved: enhanced production of precursor proteins and their abnormal enzyme cleavage, resulting in the formation of intermediate products corresponding to the amyloid fibrils. The results of treatment are still disappointing: alkylating agents and/or cortico-steroids are used in primary forms and for amyloidosis of myeloma; colchicine in familial Mediterranean fever; DMSO in renal amyloidosis; plasmapheresis in inherited-familial forms, together with the supportive management obviously dictated by clinical manifestations.

Aged↗
PubMed1991

Immunoglobulin-related amyloidosis presenting as recurrent isolated lymph node involvement.

Up to 37% of cases of generalized primary and secondary amyloidosis demonstrate lymph node involvement. Lymph node involvement as the presenting feature of generalized amyloidosis is uncommon. Isolated lymph node amyloidosis (that is, with no extranodal amyloidosis) is exceedingly rare; review of the literature reveals only two reported cases. A case of recurrent isolated lymph node amyloidosis is presented, with review of the literature.

Amyloidosis↗
PubMed1991

[Histopathological and immunohistochemical studies of generalized amyloidosis].

Histopathological and immunohistochemical studies were performed on 10 autopsy cases of generalized amyloidosis. The results showed that there were 3 cases of secondary amyloidosis (AA protein), 4 cases of primary amyloidosis (AL protein) and 3 cases of amyloidosis associated with multiple myeloma (AL protein); no familial amyloidosis (AF protein) was identified. Amyloid substances detected in all of the cases were similar in appearance morphologically, and differentiation of different types of amyloid proteins could not be depended on whether the disease is primary in nature or there is amyloid deposition in various organs or tissues. Anyhow, the differentiation could be made by pre-treatment with KMnO4 in Congo red stain or by immunoperoxidase stain, and the latter one is considered to be more reliable in identifying amyloid protein types.

Adult↗
PubMed1990

[Sicca syndrome in amyloidosis].

We report on two patients with rheumatoid arthritis and sicca syndrome due to secondary amyloidosis (A-amyloid) with involvement of the kidneys and gut. At autopsy, generalized amyloidosis was found with deposits in the salivary and lacrimal glands. Two other patients developed sicca syndrome due to primary systemic L-amyloidosis with Bence-Jones paraproteinuria, renal insufficiency and amyloid cardiomyopathy; both died of cardiac failure. Although sicca syndrome with amyloidosis has been described only occasionally, it is possible that this association is more frequent. These observations suggest that not only Sjögren's syndrome but also amyloidosis should be considered as a possible cause of sicca syndrome.

Amyloidosis↗
PubMed1990

[Chemotactic function of skin fibroblasts in patients with amyloidosis].

Chemotaxis of cultivated fibroblasts, obtained from patients with amyloidosis, chronic glomerulonephritis and healthy volunteers, was investigated. Fibroblast migration toward donor serum and serum from patients with amyloidosis was measured using Boyden chamber's technique. As "zero" chemoattractant Hank's solution was used. It was shown, that chemotactic index (CI) was independent from cell density. Significant CI depression of fibroblasts from patients with amyloidosis toward donor serum in contrast to fibroblasts from patients with chronic glomerulonephritis and healthy volunteers was shown. The depression of chemotactic function was the same with fibroblasts from patients with different variants of amyloidosis and different stages of amyloid nephropathy and was stable in several cell generations. The results obtained suggest the existence of primary hereditary variant (variants) of chemotactic function, which may lead to the development of amyloidosis in certain conditions.

Adolescent↗
PubMed1986

Systemic amyloidosis complicating cystic fibrosis. A retrospective pathologic study.

A retrospective autopsy study during 1957 to 1983 of patients with clinically documented cystic fibrosis (CF) who were at least 15 years old at the time of death (33 patients) revealed that 11 (33%) had amyloid deposits in multiple organs. The spleen, liver, and kidneys were the principally affected organs, with microscopic deposits mainly restricted to blood vessels. Only one patient had overt clinical manifestations of organ dysfunction secondary to the presence of amyloid. No differences were noted between the groups with (11 patients) and without (22 patients) amyloidosis with respect to age at diagnosis of CF; number, severity, and types of infections; and longevity. Our data demonstrate that amyloidosis is more common in patients with CF than previously reported, and this purported increase parallels the longer life span of these patients. Those patients who are older than 15 years of age constitute the particular group at risk, and they should be evaluated for amyloidosis if unusual clinical findings emerge to suggest it. Clinically evident amyloidosis is uncommon, however, in patients with CF and amyloid deposits at this time. It is likely that clinically relevant amyloidosis will become more of a complicating factor in the future as the life span of these patients continues to increase.

Adolescent↗
PubMed1988

Utility of subcutaneous fat aspiration for the diagnosis of systemic amyloidosis (immunoglobulin light chain).

To our knowledge, this is the first blind and controlled analysis of subcutaneous fat aspiration for the diagnosis of primary systemic amyloidosis. The procedure was performed on 82 patients with biopsy-proved systemic amyloidosis and 72 normal adult volunteers. Slides from 71 of the 72 controls were read as negative. Slides from 59 (72%) of the 82 patients with amyloidosis were read as positive or weakly positive after staining with alkaline Congo red. Subcutaneous fat aspiration was as sensitive as rectal biopsy and substantially more sensitive than bone marrow biopsy in diagnosing amyloidosis. In six instances fat aspiration would have obviated the need for a more invasive diagnostic biopsy. Subcutaneous fat aspiration is sensitive (72%) and specific (99%) for amyloidosis. It is technically simpler and less expensive than rectal biopsy and permits immediate assessment of specimen adequacy. The concordance rate for two independent pathologists was 95%. Equivocally positive stains should be interpreted with caution because weak nonspecific staining may be seen.

Adipose Tissue↗
PubMedDate not supplied

Abdominal fat tissue aspirate in amyloidosis of familial Mediterranean fever.

Abdominal fat tissue aspirates from 20 patients with biopsy-proved amyloidosis were investigated by polarized microscopy after staining with Congo-red. Positive results were obtained in 4 of 5 patients with primary amyloidosis (AL) and in none of 15 with amyloidosis (AA) of Familial Mediterranean Fever (FMF). We suggest that although this technique is simple, safe and effective in other forms of amyloidosis, it cannot be used as a diagnostic tool in FMF patients suffering from amyloidosis.

Abdomen↗
PubMed1989

[Prostatic amyloidosis].

The case of amyloidosis of infrequent localization is presented. Amyloid deposit was found in the prostate gland which was removed transvesically owing to dysurial complaints. The amyloid was found to be of type AA with Wright's modification of Romhányi's technique which referred to a secondary amyloidosis. Clinical and laboratory examinations failed to prove the existence of secondary amyloidosis thus local amyloidosis or the first organic manifestation of a secondary amyloidosis was assumed.

Amyloidosis↗
PubMed1989

Familial cutaneous lichen amyloidosis in association with multiple endocrine neoplasia type 2A: a new variant.

Multiple endocrine neoplasia type 2A (MEN 2A) is a rare hereditary disease transmitted in families as an autosomal dominant trait. We have identified a family in which the expression of a rare autosomal dominant form of cutaneous lichen amyloidosis appears to cosegregate with MEN 2A. In this family the skin lesion presented as multiple infiltrated papules overlying well demarcated plaques over the scapular area (right or left). Immunohistochemical studies demonstrated amyloid which stained for keratin but not calcitonin. A total of 19 members were screened. Three members of the family have the characteristic skin lesion and MEN 2A; two additional members have MEN 2A but have not manifested observable skin changes of lichen amyloidosis. Another unrelated Italian family with a similar type of pruritic skin rash and MEN 2A has been reported recently. Although the initial skin biopsies were negative for amyloidosis, subsequent biopsy established the association of MEN 2A with amyloidosis in this family also. When these kindreds are combined, several conclusions can be drawn. First, the syndrome of cutaneous amyloidosis and MEN 2A appears to be a clearly defined autosomal dominant hereditary syndrome. Whether this syndrome can be linked to chromosome 10 is not yet known. Second, the dermal amyloid appears to be caused by deposition of keratin-like peptides rather than calcitonin-like peptides. Third, we believe that patients with the hereditary form of cutaneous amyloid should be screened for medullary thyroid carcinoma to determine the true frequency of this syndrome.

Amyloidosis↗
PubMedDate not supplied

Subcutaneous fat biopsy in the diagnosis of amyloidosis secondary to chronic arthritis.

Subcutaneous fat biopsy was investigated for its sensitivity in giving a diagnosis in 44 consecutive patients with rheumatoid arthritis or ankylosing spondylitis suspected of systemic amyloidosis. In 26 of these patients amyloidosis could be demonstrated by fat or rectal biopsy or biopsies from organs suspected of amyloid deposition. Fourteen of the 26 (54%) fat biopsy specimens of the patients with amyloidosis were positive after staining with Congo red and 22 (85%) of the rectal biopsy specimens were positive. All 12 kidney biopsy specimens and 4 biopsy specimens from other organs of these 26 patients were positive for amyloidosis. In 2 patients with a negative rectal biopsy specimen, fat biopsy would have obviated the need for a more invasive biopsy. All patients experienced fat biopsy as less demanding compared to other biopsy procedures. These results imply that in patients with chronic arthritis subcutaneous fat biopsy is a useful screening procedure. In this patient group fat biopsy is less sensitive for the diagnosis of amyloidosis compared to rectal biopsy.

Adipose Tissue↗
PubMed1989

[A study of rheumatoid arthritis patients associated with biopsy-proven secondary amyloidosis].

Reactive systemic amyloidosis associated with rheumatoid arthritis (RA) was studied clinically in 28 patients (2 men and 26 women). The diagnosis of amyloidosis was established by histological examination of biopsy materials. Upper gastrointestinal tract biopsy was performed in 14 patients, and renal and rectal biopsy in 8 and 4 respectively. The mean age and duration of RA at diagnosis of amyloidosis were 58.6 (range 35-72) years and 15.5 (range 4-44) years respectively. Almost all patients had intractable and progressive courses of RA. Serological activities determined by C-reactive protein (CRP) and erythrocyte sedimentation rates were moderate to high in over 80% of the cases. Renal abnormalities were noticed in 19 cases, and gastrointestinal disorders in 10. Eight patients died from 1 to 54 (mean 15.3) months after the diagnosis of amyloidosis; 5 died of renal failure and 2 of gastrointestinal involvements. Renal impairments progressed frequently and serum creatinine elevated over 1.5 mg/dl in another 8 cases. Five patients progressing to renal failure were treated with hemodialysis. Three died within several weeks after the induction of hemodialysis, although 2 were treated for more than 2 years. Intractable hypotension and pulmonary congestion were frequently observed in these cases. A close relationship was found between serum amyloid A protein (SAA) and CRP concentration, so that the measurement of SAA seemed to be valuable in assessing disease activity. Concerning the treatment of amyloidosis, cyclophosphamide and corticosteroids seemed to be effective in several cases, although it had been unsatisfactory in most cases.

Adult↗
PubMed1989

[Treatment of amyloidosis with dimethyl sulfoxide (DMSO)].

In this study we have investigated the role of oral dimethylsulfoxide (DMSO) therapy in 2 patients with primary amyloidosis (AL) and in 2 patients with secondary amyloidosis (AA) to long-standing rheumatoid arthritis. DMSO treatment produced no beneficial effects in the patients with idiopathic amyloidosis. Instead the patients with secondary amyloidosis experienced a subjective improvement, a decrease of inflammatory activity of the rheumatoid arthritis and an unequivocal improvement of renal function following 3-6 months of DMSO therapy. No serious side effects of DMSO were observed except for unpleasant breath odour. We conclude that a treatment with oral DMSO may prolong life of patients with secondary amyloidosis.

Administration, Oral↗
PubMed1988

Does immunodeficiency in uremic patients promote dialysis-related amyloidosis?

The present study was undertaken to evaluate the immune function of 5 hemodialysis patients with dialysis-related amyloidosis as compared to 6 without, both groups having a dialytic age from 7 to 21 years, and 5 healthy controls. We investigated serum levels of immunoglobulins (IgG, IgA, IgM), made skin tests and measured peripheral lymphocyte subsets using monoclonal antibodies. 1) The absolute numbers of T3, T4 and T8 cells were significantly lower in hemodialysis patients than controls and T3, T4 cells were lower in patients with amyloidosis than in those without: T3 (m +/- SD/microliter), 387 +/- 253 vs 744 +/- 207 (p = 0.03); T4, 262 +/- 115 vs 589 +/- 297 (p = 0.04). 2) Serum levels of IgG and IgM were significantly lower in patients with amyloidosis than in the others: IgG (m +/- SD, g/l), 10.2 +/- 1.4 vs 15.4 +/- 3.2 (p less than 0.001); IgM, 0.65 +/- 0.28 vs 1.65 +/- 0.37 (p less than 0.001). 3) Delayed hypersensitivity studied by skin tests showed less than 3 positive antigens in 4/5 patients with amyloidosis against 1/6 patients without. These data suggest there is a marked defect of T-cell help in uremic patients with dialysis-related amyloidosis.

Adult↗