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PubMed2002

[Secondary amyloidosis complicating ulcerative colitis].

We report a case of amyloidosis secondary to ulcerative colitis. A 33-year-old man with total ulcerative colitis was treated by proctocolectomy 3 years and 5 months after the onset of the disease. One year later, a nephrotic syndrome revealed a secondary amyloidosis that led to end-stage renal failure 14 years later. Colectomy could have delayed renal failure. During the 27-year time of follow-up, ulcerative colitis was the only detected disease possibly responsible for secondary amyloidosis.

Adult↗
PubMed2002

Clinical and biochemical outcome of renal amyloidosis.

AA amyloidosis is a relatively rare disease which complicates chronic inflammatory diseases, chronic infections, familial Mediterranean fever (FMF) and malignant diseases. Although amyloid deposition may be found in many organs, renal involvement dominates the clinical picture. We reviewed 63 patients with AA amyloidosis who presented to our nephrology department between 1995 and 2000. Prognostic markers, detailed history, physical examination and laboratory tests were evaluated. The causes of AA amyloidosis were as follows: FMF 42 (66.6%), pulmonary tuberculosis 9 (14.2%), chronic osteomyelitis 4 (6.3%), bronchiectasia 4 (6.3%), rheumatoid arthritis 1 (1.5%), juvenile idiopathic arthritis 1 (1.5%), inflammatory abdominal aortic aneurysm 1 (1.5 %), unknown aetiology 1 (1.5%). The diagnosis was made on renal biopsies in 63.4% of the patients, while the remaining 36.6% were diagnosed as a result of rectal biopsies. Sixteen patients died. A low serum albumin, high creatinine and high 24-hour urine albumin excretion were associated with high mortality.

Adolescent↗
PubMed2002

[Nodular pulmonary amyloidosis with bullae; report of a case].

A 53-year-old male was admitted to the hospital because of progressive paralysis of inferior limb. Chest X-ray film and chest computed tomography (CT) on admission showed a small nodule with bullae in the right lower lung field. After CT-guided marking, partial lung resection including the nodule was performed. The cut surface of the resected specimen showed a tumor (10 mm in diameter) in a bullae measuring 14 mm in diameter. The tumor was diagnosed as amyloidosis. Fourty-six cases of primary localized pulmonary amyloidosis were reported in Japan. However, primary localized pulmonary amyloidosis with bullae is extremely rare, only 3 cases have been reported.

Amyloidosis↗
PubMed2002

Electron and immuno-electron microscopy of abdominal fat identifies and characterizes amyloid fibrils in suspected cardiac amyloidosis.

We evaluated the role of electron microscopy and immuno-electron microscopy studies on abdominal fat fine-needle biopsy samples in diagnosis and characterization of cardiac amyloidosis. The series consists of 15 patients with echocardiographic evidence of "restrictive cardiomyopathy" suspected to be due to amyloidosis. Patients underwent: clinical examination, electrocardiography, 2-D and Doppler echocardiography, immunofixation of serum and urine for detection of monoclonal immunoglobulins, and abdominalfat biopsies that were investigated with polarized light (Congo red), electron and immuno-electron microscopy using specific antibodies to kappa and lambda light chains, apolipoprotein A1, serum amyloid A (SAA), and transthyretin (TTR). Ultrastructural study of abdominal fat samples identified amyloid deposits in 15/15 cases. Immuno-electron microscopy specifically stained amyloid fibrils with antibodies anti-lambda (n = 8), -kappa (n = 2), -apolipoprotein A1 (n = 2) and -TTR (n = 3). Immuno-electron microscopy revealed TTR immuno-labelling in 2 patients with accidental monoclonal components, and a A reaction in I patient without monoclonal components. TTR and apolipoprotein A1 positive cases carried missense mutations in the corresponding genes. Our results demonstrate that amyloid deposits are present in the abdominalfat of patients suspected to have cardiac amyloidosis and that immuno-electron microscopy was able to characterize the amyloid protein in all cases.

Abdomen↗
PubMed2003

99mTc-aprotinin scintigraphy in amyloidosis.

UNLABELLED: Changes in the amount and distribution of amyloid lesions have been difficult to monitor because they can usually be demonstrated only by evident symptoms or from a biopsy. The recent progress in the treatment of amyloidosis stresses the need for an early diagnosis and the need for noninvasive monitoring during the course of treatment. To validate (99m)Tc-aprotinin scintigraphy, we studied 23 consecutive patients with known or suspected amyloidosis. METHODS: (99m)Tc-Aprotinin (500-700 MBq) was injected intravenously and whole-body scans, regional images, and SPECT tomograms were obtained 90 min after tracer injection. RESULTS: Focal accumulations of (99m)Tc-aprotinin were seen in different organs of 22 patients with a total of 90 lesions, of which 20 were confirmed by biopsy or autopsy. Scintigraphy revealed "silent" amyloid deposits in at least 5 patients who later developed clinical symptoms. Physiologic uptake or excretion in liver and kidneys could not be differentiated from pathologic lesions in those organs. CONCLUSION: (99m)Tc-Aprotinin scintigraphy appears to be a fairly sensitive and specific diagnostic modality in patients with suspected amyloidosis. The technique is noninvasive, and it entails a minimal stress to the patient and is useful for detection of a wide range of lesions.

Adult↗
PubMed1976

Clinical and laboratory findings in primary generalized and multiple-myeloma-related amyloidosis.

Thirty-four patients with primary generalized amyloidosis (PGA) and 14 with multiple-myeloma-related amyloidosis (MRA) were studied. The commonest clinical manifestations in PGA were nephrotic syndrome, hepatomegaly and congestive heart failure, and in MRA, low back pain, plasmacytoma and rheumatoid-arthritis-like syndrome. Eight patients with PGA had limited clinical expression of the disease, such as involvement of only kidneys, joints, parotid glands or gastrointestinal tract; in one patient amyloidosis was limited to lymph nodes. Low serum concentrations of total protein and albumin were common. M components were detected in the serum of 91% of patients with PGA and 92% of patients with MRA: 70% of the M components in PGA and 25% of those in MRA had lambda light chains. Bence Jones proteinemia was detected in 56% of the patients with PGA and in 77% of those with MRA. The serum concentration of immunoglobulins was decreased substantially in more than two thirds of the patients with PGA. Proteinuria (greater than 250 mg/24 h) was observed in 78% of patients with PGA and in 93% of patients with MRA. Bence Jones proteinuria was noted in 75 and 77% of patients, respectively. Plasmacytic infiltration of the bone marrow was found in 90% of the patients with PGA. The mean survival time of the patients with PGA was 28 months and of those with MRA, 29 months from the time of diagnosis.

Adult↗
PubMed2003

[What has become of inflammatory amyloidosis?].

Inflammation associated amyloidosis (AA amyloid) remains a severe complication of chronic inflammatory diseases, namely of rheumatoid arthritis and of juvenile arthritis. Infectious causes of AA amyloid have become uncommon. In the course of chronic inflammatory diseases, amyloid should be regularly screened by proteinuria. The proof of amyloidosis requires tissue analysis examplifying specific apple-green birefringence after staining with Congo red. When amyloidosis is progressive in the course of one chronic disease, powerful anti-inflammatory and immunosuppressive drugs can be used.

Amyloidosis↗
PubMed2003

[A case of solitary nodular pulmonary amyloidosis].

A 64-year-old man without respiratory symptoms was introduced to our hospital because of a nodule of 20 mm in diameter found in the left lung in a periodic health examination. The chest radiograph and CT scan showed a well-defined nodule in the middle of the left lung field, and enlarged mediastinal lymph nodes. Lung cancer was suspected, and transbronchial and CT guided biopsies were performed, but did not lead to a definitive diagnosis. Since the patient refused further examinations, we carefully followed up the nodule in the chest radiograph. After a year and a half, the nodule and the lymph nodes became enlarged, and the patient was admitted to the hospital for a surgical biopsy. Video-assisted thoracoscopic surgery followed by thoracotomy was performed for both a biopsy and nodule resection. Histologically, the nodule tissue was rich in amorphous substances positively stained with Congo Red, which was consistent with amyloidosis. No findings of systemic amyloidosis or secondary amyloidosis were demonstrated.

Amyloidosis↗
PubMed2003

[The clinicopathological features of early renal amyloidosis].

OBJECTIVE: To investigate the clinicopathological manifestations of early renal amyloidosis (AL) and its diagnostic criteria. METHODS: Fifteen cases with early renal amyloidosis admitted from 1994 to 2001 were collected from the hospital, and their clinical and pathological features were reviewed. Of them, the initial diagnoses were not made by depending findings from the light microscopy (LM) and immunofluorescense (IF), but confirmed by electron microscopy (EM) afterwards. Immuno-electron microscopy (IEM) were applied for amyloidosis typing. RESULTS: Most patients of early renal AL were in the middle to old age. Nephrotic syndrome was the most prominent symptoms and signs accompanying with rare microscopic hematuria and hypertension. Most of them had a normal renal function. Pathological examinations of renal biopsies using LM and IF showed mild mesangial proliferation and mild thickening of glomerular basement membrane (GBM). Immunoglobulins and complements were negative or only scanty in certain cases, but in all cases there was a light chain protein deposition homogeously. There were 4 cases of minimal change glomerulopathy, 5 cases of mild mesangial proliferative glomerulonephritis, 5 cases of stage I membranous nephropathy, and 1 case of cast nephropathy diagnosed with LM. The amyloid fibrils (diameter 8 - 10 nm) were randomly distributed in the mesangium, along GBM and at the arteriolar wall under EM. Additionally, Congo red staining was positive. IEM demonstrated that amyloid fibrils labeled with colloid gold was combined with a kind of light chain protein which was confirmed as the light chain type of AL. CONCLUSIONS: The diagnosis of early renal AL was occasionally neglected by depending only findings of LM and LF. However, special amyloid fibrils can be detected using EM. EM observation is an indispensable technique for the diagnosis of early renal AL and the typing of AL may further be determined by using IEM.

Adult↗
PubMed2003

[Cardiac manifestations of amyloidosis by deposits of transthyretin and apolipoprotein A1. Report of 3 families].

Amyloidosis is characterised by extracellular deposits of a heterogenous protein. Compared with secondary forms, hereditary amyloidosis due to genetic mutations is rare. The authors report the cardiac manifestations in a French family of 5 sisters and 1 brother, three of whom presented with amyloidosis with deposits of transthyretin and apolipoprotein A1 due to a new genetic mutation.

Adult↗
PubMed1992

Recurrent, isolated factor X deficiency in myeloma: repeated normalization of factor X levels after cytostatic chemotherapy followed by late treatment failure associated with the development of systemic amyloidosis.

We describe the case of a 64-year-old woman with isolated severe factor X deficiency associated with kappa light chain myeloma. At the time of diagnosis there was no evidence for amyloidosis. Complete remission (CR) of myeloma as well as normalization of factor X levels were achieved after cytostatic chemotherapy. Subsequently, factor X deficiency recurred twice without any evidence for relapse of myeloma. The first time factor X normalized again following cytostatic treatment, the second time, however, factor X deficiency was refractory to chemotherapy. Finally, relapse of myeloma became evident associated with rapidly progressing, systemic amyloidosis, which was fatal within a few months. Initially, factor X infusion studies showed a normal recovery, but when amyloidosis became overt the recovery decreased to 0%. We assume that factor X deficiency was due to a binding of factor X to kappa light chains associated with the proliferation of the malignant myeloma cell clone.

Amyloidosis↗
PubMed2003

[Analyses of pathogenesis and therapeutic approaches for hereditary amyloidosis].

Amyloidosis is a disorder of protein metabolism in which normally soluble autologous proteins are deposited in tissues as abnormal insoluble fibrils, causing structural and functional disruptions. We have recently identified the novel localized amyloidosis accompanied by trichiasis. The precursor protein of amyloid deposits was mutated lactoferrin and all the patients had lactoferrin Glu561Asp. The disease was classified hereditary amyloidosis whose risk factor is trichiasis. We examined the therapeutic possibilities for mutated transthyretin(ATTR) related familial amyloidotic polyneurpathy(FAP), one of the systemic amyloidoses. Cr3+ suppressed amyloid formation by stabilizing ATTR structure in vitro. BSB is a useful new diagnostic tool to detect amyloid deposits both in in vitro and in vivo and may have therapeutic potential for preventing amyloid deposition. Gene therapy using single-stranded oligonuclotides(SSOs) may become a promising tool for therapy instead of liver transplantation. SSOs with athrocollagen effectively replaced the TTR gene both in vitro and in vivo.

Amyloidosis↗
PubMedDate not supplied

Cutaneous and lingual papules as a sign of beta 2 microglobulin-derived amyloidosis in a long-term hemodialysis patient.

Although hemodialysis has permitted long-term survival of patients with renal failure, beta 2 microglobulin-derived amyloidosis is a serious complication occurring most commonly in long-term hemodialysis patients. Orthopedic manifestations are quite common, but cutaneous and lingual manifestations are relatively uncommon. We report a 56-year-old patient with lichenoid plaque type of skin eruptions and lingual papules caused by beta 2 microglobulin-derived amyloidosis. Immunohistochemical study showed that amyloid deposits were positive for anti-beta 2 microglobulin antibody, but negative for anti-advanced glycation end products antibody (anti-CML and CLE antibody). We discuss the histological and the clinical features of skin manifestations of beta 2 microglobulin-derived amyloidosis.

Amyloidosis↗
PubMedDate not supplied

Cardiac amyloidosis: hemodynamic, echocardiographic and endomyocardial biopsy studies.

Six patients with cardiac amyloidosis (four males, two females; age 27-60 years) were evaluated by us. Four patients presented with congestive heart failure, while one patient each presented with effort angina and giddiness. Extracardiac clues to the diagnosis in the form of involvement of other systems were present in only two patients. The electrocardiogram was abnormal in four patients while three exhibited roentgenographic evidence of cardiomegaly or pulmonary venous hypertension. Echocardiography suggested the diagnosis of amyloidosis in only two patients, restrictive cardiomyopathy in two other patients and dilated and hypertrophic cardiomyopathy in one patient each. Cardiac catheterisation and angiography suggested restrictive heart disease in four patients and hypertrophic cardiomyopathy in one. One patient, whose initial haemodynamic study was normal, had features of dilated cardiomyopathy at repeat study after 11 months. Endomyocardial biopsies showed amyloid deposits in all patients. We emphasise the varied clinical manifestation of cardiac amyloidosis and the need for a high index of suspicion. The diagnosis can be safely and reliably confirmed by endomyocardial biopsy.

Adult↗
PubMed1992

[Immunosuppressive therapy of secondary amyloidosis associated with rheumatoid arthritis or Still's disease].

Ten cases of renal amyloidosis associated with rheumatoid arthritis (n = 9) or Still's disease (n = 1) were studied retrospectively. One patient had end-stage renal failure at first presentation, whereas the nine others had nephrotic syndrome without renal failure as the initial manifestation of amyloidosis. In the three untreated patients, renal function deteriorated steadily and dialysis was rapidly necessary. In contrast, among the six patients given chemotherapy, four had normal renal function under therapy. These favorable therapeutic results are analyzed and compared with previously published data on the treatment of secondary amyloidosis.

Adult↗
PubMed1992

[Amyloidosis in the autopsy data from a general hospital of Leningrad].

Over the recent 20 years the incidence of amyloidosis did not undergo any noticeable changes, accounting for 1.48% of the total number of autopsies in 1964-1968 and for 1.52% in 1984-1988 (P less than 0.5). The number of cases of the clinically unrecognized amyloidosis increased from 37.5% in the first period to 52.18% in the second one. In most cases amyloidosis affects the kidneys (94.9%), spleen (58.2%), liver (48%) and then, in the descending order, there follow adrenals, intestine, heart, pancreas and other organs (the total data for both the periods).

Age Factors↗
PubMed1992

[The effect of a protein loading test on the fibrinolytic system in patients with chronic glomerulonephritis and amyloidosis].

Investigation of the reserves of the fibrinolytic system with the aid of protein stimulation was carried out in 10 patients with chronic glomerulonephritis and in 10 patients suffering from amyloidosis. All the patients manifested proteinuria exceeding 3.5 g/day and other symptoms of nephrotic syndrome of varying intensity. Renal function was preserved in all the patients. The reserves of the fibrinolytic system were measured by analyzing blood plasma and urine before and after beef protein stimulation. The data revealed reciprocal responses of activator activity in blood plasma and urine in patients suffering from chronic glomerulonephritis and amyloidosis. In patients with amyloidosis, the test revealed complete depletion of activator activity in urine while its considerable reserves were preserved in blood plasma of the systemic channel.

Amyloidosis↗
PubMed2004

Relationship of intercellular adhesion molecule-3 and hepatocyte growth factor with amyloidosis A in chronic renal-failure patients.

BACKGROUND: Patients with chronic renal failure have elevated serum levels of intercellular adhesion molecules and hepatocyte growth factor. The aim of this study was to investigate immunohistochemically whether these substances are associated with amyloidosis A in this patient group. MATERIAL/METHODS: Liver needle-biopsy specimens from 40 chronic renal-failure patients, 20 with amyloidosis A (Group I) and 20 without this disease (Group II), were investigated. Twenty control liver biopsies were obtained from healthy individuals (Group III). All specimens were immunohistochemically stained to determine the presence and density of hepatocyte growth factor and intercellular adhesion molecule-3. RESULTS: The levels of positivity for hepatocyte growth factor and intercellular adhesion molecule-3 in Groups I and II were significantly higher than the levels in the control group. Also, the Group I sections had significantly more cells that were positive for intercellular adhesion molecule-3 than the Group II sections (p<0.016). There was no significant difference between Groups I and II regarding the level of positivity for hepatocyte growth factor. CONCLUSIONS: The fact that there was a higher density of intercellular adhesion molecule-3-positive cells in the patients with amyloidosis A indicates that this substance may be a microenvironmental factor in this disease. If this is true, regulating the serum level of this molecule may prevent amyloid A in chronic renal-failure patients.

Amyloidosis↗