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PubMedDate not supplied

Familial Mediterranean fever (FMF)-associated amyloidosis in childhood. Clinical features, course and outcome.

OBJECTIVE: Familial Mediterranean fever (FMF) is an autosomal recessive disorder of childhood characterized by attacks of fever and serositis. Renal amyloidosis is the most important complication of the disease that determines the prognosis. METHODS: Forty-eight Turkish FMF patients with amyloidosis who have been followed at the two hospitals in Ankara were included in this study. RESULTS: All patients with amyloidosis had been symptomatic for FMF at the time of the diagnosis (Phenotype I), none had received regular colchicine therapy and all presented with proteinuria. Ten of them had asymptomatic proteinuria; 38 had nephrotic syndrome and 8 of them had renal insufficiency (CRI) as well, at the time of the diagnosis. Regular colchicine therapy was commenced to all of the patients. At the end of observation period of 4.5 +/- 2.23 years (range 2-12 yrs) on treatment, nephrotic syndrome resolved in 13 patients and proteinuria was lost in 5 of them. None but 2 of the patients who were diagnosed at proteinuric stage progressed to end stage renal failure (ESRF). Seven MEFV mutations (M694V, M680I, V726A, M694I, K695R, R761H, E148Q) were systematically investigated in 32 patients. Six of the seven studied mutations were found in these patients and clinical diagnosis was confirmed by mutation analysis in 24 patients. Eight patients were found to have mutations on one of the alleles. CONCLUSION: Amyloidosis is the most serious complication of FMF. Colchicine treatment ameliorates the progression of renal disease in the patients who presented with proteinuria and even with nephrotic syndrome. No correlation between the outcome of the patients with nephrotic syndrome and the degree of proteinuria and/or serum albumin levels at the initiation of treatment were noted. Progression to ESRF seems inevitable despite colchicine therapy after the development of CRI in patients with FMF associated amyloidosis.

Adolescent↗
PubMed2002

Management of laryngeal amyloidosis--the experience of Taipei Veterans General Hospital.

Laryngeal amyloidosis is an uncommon disease accounting for 0.68% of benign tumors of the larynx. It can present as a nodular tumor or diffuse subepithelial deposition forming a bulging mass and causing hoarseness, dysphagia or even airway obstruction. We present our experience in the management of laryngeal amyloidosis, and discuss the clinical features and histopathological characteristics of the disease. Reports on benign laryngeal tumor from June 1988 to June 1998 at Taipei VGH were searched to identify and collect amyloid cases for chart review. Five cases of laryngeal amyloidosis were found in the records. The sites of involvement were false cord, ventricle, post-cricoid, and arytenoid-epiglottic fold. No evidence of systemic amyloidosis involvement was observed for any of the five cases. All cases were treated with CO2 laser excision. Recurrence occurred in two patients within one year. More extensive CO2 laser excision was subsequently performed, with no more recurrences noted as of the last follow-ups. Laryngeal amyloidosis is a benign disease; however, surgical management is required when it is symptomatic. Recurrences or large-sized tumors may be a problem; however, advances in laser technology and the cumulative experience of treating benign lesions of the larynx have made CO2 laser endoscopic excision an effective treatment option.

Adult↗
PubMed2002

[Bullous amyloidosis].

INTRODUCTION: The occurrence of skin damage during systemic amyloidosis is common, but the appearance of bullous lesions is rare. Only twenty-seven cases have been reported in the literature. We report our observation of bullous amyloidosis during progression of renal amyloidosis. OBSERVATION: A 61 year-old man, presented with white, soft, palpebral edemas of the lower limbs, without scutulum involvement, associated with a large cubital nerve that had appeared in March 1997. Biological explorations revealed a nephrotic syndrome. Pathologic study of the renal biopsy concluded in amyloidosis. Treatment with colchinine stabilized the renal damage. One year later, a non-pruriginous, papular and bullous eruption occurred, localized essentially in the axillary and inguinal-crural folds of the forearms and legs. In the presence of an amyloidal deposit and intra-epidermal detachment, the cutaneous biopsy was evocative of bullous amyloidosis. The search for concomitant myeloma was negative. Treatment with colchinine was effective. The bullous lesions disappeared after 2 months, and 21 months later, renal damage was still stable. DISCUSSION: These particularities in evolution are exceptional and have never been described. A hypothetical modification in the physico-chemical properties of the amyloidal protein might explain the bullous eruption and stabilization of renal damage.

Amyloidosis↗
PubMed1975

Studies of catecholamines in amyloidosis.

Amyloidosis was induced in 21 mice by daily injections of casein solution for four weeks. Thioflavine T staining showed that the induction of amyloidosis took place in all mice. The catecholamine (CA) content was estimated in the spleen, proximal colon and kidney. In spleen the noradrenaline (NA) content per wet weight of tissue in casein-induced amyloidosis was significantly (p less than 0.01) lower than in the controls but this reduction was not apparent because of the huge increase in the weight of amyloid spleens. In the proximal colon and kidney the NA content did not differ from the control values. By fluorescence histochemistry the adrenergic fibres appeared to be well maintained in the spleen, duodenum and proximal colon in casein-induced amyloidosis. The CA content of rectal mucosal biopsies from five patients suffering from amyloidosis was compared with that from seven control persons. There was no significant difference in NA content. The present results suggest that in amyloid disease detectable alterations in the CA content or such morphological changes in adrenergic nerve fibres, which can be detected by fluorescence histochemistry take place only in the most advanced disease, if at all.

Amyloidosis↗
PubMed2002

[Amyloidosis associated with chronic granulomatous disease in a patient with a renal transplant and recurrent urinary tract infections].

Chronic granulomatous disease is a group of syndromes which share a defect in a component of the phagocyte NADPH-oxidase complex. Without this enzyme activity, phagocytic cells cannot produce superoxide, peroxide, and other potent microbicidal radicals, and are less able to kill ingested pathogens. The clinical picture is characterised by recurrent life-threatening bacterial and fungal infections and abnormal tissue granuloma formation. On the other hand, amyloidosis is a systemic disease with renal involvement occurring in the majority of cases. Recurrent amyloidosis is a rare but well documented event in renal transplant recipients. However, graft loss secondary to amyloidosis has been noted infrequently. In addition, de novo amyloidosis has not been previously associated with graft loss. We report here a renal transplant recipient with chronic granulomatous disease and history of recurrent urinary tract infections, who developed nephrotic syndrome and progressive renal insufficiency secondary to de novo AA amyloidosis leading to graft loss 66 months after transplantation.

Amyloidosis↗
PubMed1992

[Clinical characteristics and prognosis of secondary amyloidosis in patients with rheumatoid arthritis--renal involvement and therapy].

Secondary amyloidosis is an important complication that may have a strong influence on the prognosis of patients with rheumatoid arthritis (RA). We studied 21 RA patients with secondary amyloidosis. The two major initial signs were gastrointestinal symptoms and renal involvement. When 15 of the 21 patients were diagnosed as having secondary amyloidosis, they displayed renal involvement including proteinuria, hematuria and hypercreatininemia. The 15 patients with amyloidosis were either subjected to dialysis or died within 35 months on the average. The causes of death in 13 patients were cardiac failure, gastrointestinal bleeding and infection, which were strongly implicated with renal failure. Dialysis was applied to seven patients. Three of them were maintained with chronic dialysis. We discussed the induction-time and the method of dialysis in patients with amyloidosis secondary to RA.

Adolescent↗
PubMed2004

Severe intrahepatic cholestasis in an elderly patient with primary amyloidosis and colon adenocarcinoma.

Hepatic involvement in primary amyloidosis (AL type) is not rare but is often clinically silent. However, presentation with jaundice in AL-type amyloidosis is rare, with an incidence of less than 5% reported in the literature. It is considered to be a preterminal sign. We herein report on a case of primary hepatic amyloidosis presenting with severe intrahepatic cholestasis. Viral, drug, alcohol, and autoimmune etiologies were all excluded. A liver biopsy was performed because of unexplained cholestatic jaundice for 3 months. The pathology showed hepatic amyloidosis with extensive amyloid deposition in the expanded portal tracts and sinusoidal space. The patient received supportive treatment only, because of persistent jaundice, coexistent colon cancer with para-aortic lymph node metastasis, and possibly peritoneal carcinomatosis. Unfortunately, the patient died of sepsis 10 months after the onset of jaundice. We suggest that hepatic amyloidosis must be considered in the differential diagnosis of unexplained cholestatic jaundice.

Adenocarcinoma↗
PubMed1992

[Amyloidosis in systemic lupus erythematosus].

Based on the authors' experience from 1982 to 1990 it was noted that out of 26 cases of renal amyloidosis in the presence of nonpurulent and purulent conditions 2 patients (7%) demonstrated the association with systemic lupus erythematosus (SLE). It was also noted that amyloidosis developed in the patients with a long history of the disease. Long-course immunosuppression treatment could be regarded as the other factor-of-risk for amyloidosis development. Histochemical examination of both patients demonstrated that amyloid deposits in the renal glomeruli were resistant to the potassium permanganate effect and consisted of AL-protein. The results obtained indicated the possibility of appearance of immunoglobulins AL--the proteins of the primary amyloidosis--synthesized in the spectrum in the SLE presence as well as their deposition in the renal glomeruli. As a possible cause of proteinuria and the nephrotic syndrome in SLE patients amyloidosis should be diagnosed in the life time and be regarded in the choice of therapeutic policy as well as in the assessment of pulse immunosuppressive therapy practicability.

Adolescent↗
PubMedDate not supplied

Evaluation of the hypercoagulable state by measuring protein C and antithrombin III levels in nephrotic syndrome and in familial Mediterranean fever-related amyloidosis.

The levels of protein C (PC) and antithrombin III (AT III) antigens (ag) were measured in the plasma of 39 patients with various histologic types of primary nephrotic syndrome (NS) and in 12 patients with amyloidosis secondary to familial Mediterranean fever (FMF). The controls comprised 15 healthy children. Normal or elevated PC levels were observed in primary NS patients (mean 64%, range 36-98%) and in amyloidosis patients (mean 58%, range 48-70%). There was no difference found between PC ag levels in primary NS and in amyloidosis patients. In addition, no correlation existed between protein selectivity and the PC ag levels in the primary NS patients. Normal and decreased levels of AT III were observed (mean 29 mg/dl, range 11.1-39 mg/dl) in the patients with primary NS and amyloidosis (mean 31 mg/dl range, 21-39 mg/dl). The AT III ag levels of these two groups did not differ and no correlation was found between protein selectivity and AT III levels in primary NS patients. These results suggest that in patients with primary NS, or amyloidosis secondary to FMF, hypercoagulability is not related to a deficiency in PC ag levels due to a dynamic balance between urinary losses, increased rate of hepatic synthesis, catabolism and the distribution of PC in the body compartments. Patients with low AT III levels may be more susceptible to thromboembolic complications than patients with normal levels.

Adolescent↗
PubMed2004

[A comparative description of amyloidosis in monkeys].

The paper contains the results of a postmortem study of 461 cases of amyloidosis in monkeys of 4 species (Papio hamadryas, Macaca mulatto, Macaca fascicularis and Macaca nemestrina) from the Sukhumi and Adler primatological facilities. Emergence of amyloidosis was found to be dependent on age; the frequency ratio at which separate organs are affected is presented; the specific disease signs are described for different monkey species. Generalized and isolated cases of amyloidosis are elucidated with the liver being the most vulnerable organ in macaques (64%) and the kidneys--in baboons (94%). A comparative description of different forms of amyloidosis in monkey and man was made use of to suggest using the amyloidosis in monkeys as a model process for studying the pathology in man.

Aging↗
PubMed2004

Anti-interleukin 6 receptor antibody inhibits murine AA-amyloidosis.

OBJECTIVE: AA-amyloidosis is a severe complication in chronic inflammatory diseases. AA-amyloidosis is caused by the deposition of insoluble fibrils containing AA amyloid protein derived from serum amyloid A (SAA), which is synthesized by inflammatory cytokine stimulation. We examined whether anti-interleukin 6 receptor (IL-6R) antibody prevented the development of AA-amyloidosis in mouse models. METHODS: A transient model was induced by the injection of amyloid enhancing factor (AEF) and adjuvant treatment in C57BL/6 mice. Monoclonal IgG1 antibody, MR16-1, was injected intraperitoneally just once before the injection of AEF and adjuvant. After 2 and 5 weeks, mice were sacrificed and histologically examined. In contrast, a chronic model was induced by AEF injection into IL-6 transgenic mice. One week later, in order to avoid neutralizing antibody production, MR16-1 (200 mg/kg) was injected intravenously. MR16-1 (5 mg/kg) was injected subcutaneously twice a week from the next week. Three and 6 weeks after AEF injection, mice were sacrificed and histologically examined. RESULTS: In the transient model, amyloid deposition was observed in the spleen, liver, and kidney as early as 2 weeks after treatment. MR16-1 completely prevented amyloid deposition. Although IL-6 production was not suppressed, SAA production was significantly suppressed. In the chronic model, substantial amyloid deposition was seen in multiple organs and tissues as well as the spleen, liver, and kidney. MR16-1 suppressed amyloid deposition in many organs, even when injected one week after AEF injection; it showed a tendency to decrease SAA and IL-6 levels were decreased. CONCLUSION: IL-6 is a key cytokine for the induction of AA-amyloidosis, and anti-IL-6R therapy appears promising for the treatment of AA-amyloidosis.

Amyloidosis↗
PubMed1992

Renal amyloidosis in a family of beagles.

Renal amyloidosis was confirmed in 6 related male and female Beagles, ranging in age from 5 to 11 years. The most commonly reported signs of illness included lethargy, anorexia, vomiting, and weight loss. Common clinicopathologic abnormalities were normocytic, normochromic anemia; hypoalbuminemia; azotemia; hypercholesterolemia; proteinuria; and urine specific gravity values below the normal range. Histologic examination of renal tissue from the 6 Beagles revealed moderate to severe glomerular amyloidosis with inconsistently observed mild medullary interstitial amyloidosis. Congo red-stained kidney sections from 4 of 4 affected dogs were potassium permanganate-sensitive, suggestive of reactive amyloidosis. Hereditary predisposition for renal amyloidosis was suspected in these Beagles.

Amyloidosis↗
PubMed2004

Diagnostic and therapeutic approach of systemic amyloidosis.

Amyloidosis is a group of diseases, all characterised by deposition of protein fibrils with a beta-sheet structure. This structure generates affinity of amyloid for Congo red dye and is resistant to proteolysis. Three types of systemic amyloidosis are important for the clinician: AA (related to underlying chronic inflammation), AL (related to underlying monoclonal light chain production) and ATTR amyloidosis (related to old age or underlying hereditary mutations of transthyretin). Signs and symptoms vary considerably among the three types and the choice of treatment differs completely. A stepwise approach in diagnosis and therapy is presented. When amyloidosis is suspected the first step is histological proof of amyloid and the second is proof of systemic involvement. The next two steps are determination of the type of amyloid followed by detection of the precursor protein. The fifth step is a thoughtful clinical evaluation, necessary for assessment of prognosis and therapy. Subsequently, the choice of therapy is based on the 'precursor-product' concept. In the final step, the effects of therapy on the underlying disease as well as on the amyloidosis are assessed during follow-up. In this evaluation serum amyloid P component (SAP) scintigraphy helps to show organ involvement and therapy response.

Amyloid↗
PubMed1992

Amyloidosis. Part II. Implications for neuroscience nurses: Alzheimer's disease.

UNLABELLED: Amyloidosis describes a group of diseases characterized by deposition of abnormal fibrous proteins in body organs and soft tissues. This disease complex is classified under the broad headings primary and secondary based on whether or not chronic infections or inflammatory conditions coexist. In addition, localized versus systemic involvement and heredofamilial considerations are used to subclassify amyloidosis. Many primary and secondary amyloid diseases involve the central, peripheral and autonomic nervous systems. This paper will focus on the latest information concerning systemic and localized forms of amyloidosis involving the nervous system with special emphasis on Alzheimer's disease and amyloidosis secondary to spinal cord injury. The roles of the nurse related to diagnosis, patient care, patient and family education and identification of support systems in the community will be covered. Patient care will be discussed from the standpoint of process and outcome criteria for specific nursing diagnoses. OBJECTIVES: 1. Describe the pathophysiological processes of amyloidosis. 2. Relate four aspects of nursing care to nursing diagnoses. 3. List four critical elements to include in patient/family education.

Alzheimer Disease↗
PubMed1992

Induction in mice of human light-chain-associated amyloidosis.

Primary (idiopathic) or multiple myeloma-associated amyloidosis is characterized by the deposition in tissue of monoclonal light chains or light-chain fragments (AL amyloidosis). In contrast to other types of amyloidosis, information regarding the pathogenesis of light-chain-related amyloid has heretofore been limited due to the lack of a suitable in vivo model. The authors report the successful experimental induction of human AL amyloid deposits. The repeated injection into mice of Bence Jones proteins obtained from two patients with AL amyloidosis produced the histopathologic lesions characteristic of this disease. Partial dehydration of animals before protein injection resulted in the acceleration of amyloid formation. The human proteins were deposited as amyloid within the mouse renal blood vessel walls and parenchymal tissue, as well as in other organs. The deposits were Congo red-positive, exhibited green birefringence, and had a fibrillar ultrastructure. As evidenced immunohistochemically, the experimentally induced amyloid deposits consisted of the injected human light chains, and in addition, contained mouse amyloid P component (AP); mouse immunoglobulin (Ig) or inflammatory-associated amyloid A protein was not detected. Extraction and characterization of the amyloid deposits found within the mouse kidney revealed the presence of a predominantly intact human light polypeptide chain. Mice injected in identical manner with a non-amyloid-associated Bence Jones protein had no or only rare amyloid deposits. The experimental mouse model provides a means to ascertain the amyloidogenic potential of human monoclonal light chains and to study further the pathogenesis of AL amyloidosis.

Amyloid↗
PubMed1992

Extended survival of patients with primary systemic amyloidosis.

Systemic amyloidosis can produce a wide variety of clinical manifestations, including characteristic cutaneous findings. Large series of patients with primary systemic amyloidosis have shown that systemic amyloidosis, with or without associated myeloma, has a median survival of no more than twenty-four months. We present a case of systemic amyloidosis that has been present in a woman for eighteen years, as manifested by periorbital purpura and an immunoglobulin G kappa light chain paraproteinemia. She was otherwise healthy; results of bone marrow examination showed no overt myeloma. We speculate that kappa light chain paraproteinemia could prove to be a marker for a more benign type of systemic amyloidosis.

Aged↗
PubMed1992

[Amyloidosis in rheumatoid arthritis].

Complication of secondary amyloidosis is becoming an important problem in the management of rheumatoid arthritis in Japan, because patients with rheumatoid arthritis, complicated by amyloidosis, have been increasing recently and the prognosis is poor in most cases. Renal and gastrointestinal symptoms are cardinal features of amyloidosis in rheumatoid arthritis, and most of these patients die of renal failure or cachexia, based on gastrointestinal involvement, within several years. Diagnosis in the early stage is recommended for good management of amyloidosis in rheumatoid arthritis, and gastroscopic biopsy appears to be the most available method for early diagnosis of amyloidosis.

Amyloidosis↗
PubMed1992

Amyloidosis: current approaches for diagnosis and treatment.

Amyloidosis is a complex disease resulting from the extracellular deposition of fibrous degradation proteins in body tissues. The type of protein deposited determines the class of amyloidosis present. Morphological, cytochemical, and immunohistochemical studies allow one to precisely identify the class of amyloid present and, therefore, more appropriately select a therapy. Rectal biopsy is the preferred initial procedure for obtaining tissue for study; however, subcutaneous fat pad, gingival, skin, and bone marrow biopsies are alternative noninvasive biopsy sites. We have presented two patients with multiple myeloma and amyloidosis of the AL type. One patient presented with multi-organ failure of undetermined etiology and the other with asymptomatic hepatosplenomegaly. The diagnosis of amyloidosis was made by bone marrow biopsy in the first patient and rectal biopsy in the second patient using morphological studies and confirmed cytochemically with the congo red stain. The type of amyloidosis was determined with immunohistochemical studies. Their clinical course and response to therapy will be assessed by repeated immunoglobulin studies, liver and spleen size, and studies for organ dysfunction such as 2-D echo and level of proteinuria.

Amyloidosis↗