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PubMed1996

[Recurrence of AL type primary localized pulmonary amyloidosis].

A 52-year-old female, who was diagnosed primary pulmonary amyloidosis by left open lung biopsy 11 years ago, has been pointed out chest abnormal shadow in the opposite lung for 9 years. Two masses situated in the right S3a and S4 have been growing gradually with suspicion of malignancy. The right open lung biopsy revealed amyloidosis by frozen section and wedge resections were performed. Microscopic findings were compatible with AL type amyloid protein. We reported a rare case of recurrence of localized primary pulmonary amyloidosis.

Amyloidosis↗
PubMed1996

[A case of localized amyloidosis of the ureter].

A case of localized amyloidosis of the ureter is reported. The patient was a 49-year-old female whose chief complaint was macrohematuria. Roentogenographic examination showed left hydronephrosis due to stenosis of left middle ureter. Left nephrouretectomy with cuff was performed with a diagnosis of the left ureteral tumor, and pathological examination revealed localized amyloidosis of the left ureter. Localized amyloidosis of the ureter is a rare lesion, and this is the twenty-first case reported in the Japanese literature. Review of the literature revealed that it is difficult to differentiate this lesion from other ureteral tumors by roentgenographic examination, and it is important to perform preoperative or intraoperative biopsy of ureteral tumors if benign diseases cannot be ruled out.

Amyloidosis↗
PubMed1996

[Familial amyloidosis, Finnish type with marked anhidrosis].

Familial amyloidosis, Finnish type (FAF), is a gelsolin-related systemic amyloidosis that has an autosomal-dominant inheritance pattern and is clinically characterized by progressive cranial neuropathy, corneal lattice dystrophy and skin changes such as cutis laxa, blepharochalasis, and lichen amyloidosis. A 70-year-old Japanese male proband, who was believed to be originally from Fukuoka Prefecture, showed signs and symptoms characteristic of FAF. In addition, he complained of progressive anhidrosis and heat intolerance during the daytime in summer. On examination, perspiration was absent on the almost entire body surface. Molecular genetic studies showed a G-to-A transversion that resulted in the substitution of asparagine for aspartic acid 187 in the gelsolin gene, a mutation found in most patients with FAF. Skin biopsy revealed marked deposition of amyloid, which was positive with anti-gelsolin antibody staining, around eccrine sweat glands and ducts, and around sebaceous glands, outside the basal lamina; slight to mild deposition around small vessels and small nerve fascicles; and very slight deposition in the perineurium and endoneurium. Morphometric evaluation of the nerve terminals and axons of eccrine sweat glands revealed a significant decrease in the number of nerve terminals per transverse profile of the sweat gland. Compared with controls, nerve terminals were further from the secretory epithelial cell owing to deposition of amyloid outside its basal lamina. The proband's sister had almost identical, although much less severe, clinical signs and symptoms with the same mutation of the gelsolin gene. Autonomic signs and symptoms in FAF are reported to be less frequent and less severe than those in familial amyloid polyneuropathy of Andrade type. Findings in our proband suggest that perspiration may be markedly decreased in FAF owing to marked deposition of amyloid around the eccrine sweat gland which causes degeneration of the nerve terminals and disturbs access of the neurotransmitter to the secretory epithelial cell.

Aged↗
PubMed1996

Coexistence of diffuse plane normolipaemic xanthoma and amyloidosis in a patient with monoclonal gammopathy.

Diffuse plane normolipaemic xanthomatosis (DPNX) is a well-defined clinicopathological entity that characteristically presents with yellow-orange plaques in the periorbital areas, the neck, the upper trunk and the flexural folds. DPNX has been reported in association with lymphoproliferative disorders and, occasionally, with miscellaneous, probably coincidental disorders. A case of DPNX in a patient with an IgG lambda monoclonal gammopathy and systemic amyloidosis is reported. Clinical and histopathological findings revealed typical features of DPNX and amyloidosis coexisting in the cutaneous lesions. To our knowledge this is the first reported case showing coexistence of xanthoma and amyloidosis in cutaneous lesions in a patient with a monoclonal gammopathy.

Aged↗
PubMed1996

[A case of primary localized amyloidosis of the urinary bladder].

A case of primary localized amyloidosis of the urinary bladder is reported. The patient was a 46-year-old female who suffered from macrohematuria and consulted our department. A yellowish non-papillary tumor at the retrotrigone of the bladder was observed on cystoscopy. Histological diagnosis was amyloidosis of the bladder. Since there was no evidence of disease in other organs, this patient was diagnosed with primary localized amyloidosis of the bladder and transurethral resection was performed. The clinical course was uneventful and she has been well without recurrence for 6 months postoperatively. This case was cured by transurethral resection, but close follow-up of the patients is necessary because of the recurrence.

Amyloidosis↗
PubMed1996

Immunohistochemical localization of apolipoprotein E in renal amyloidosis.

To clarify the role of apolipoprotein E (apo E) in the formation of amyloid deposits, we examined specimens from 11 patients with renal amyloidosis who underwent renal biopsy by an immunohistochemical method using a monoclonal antibody (murine IgG1). Apo E was distributed in the amyloid deposits of all patients in a pattern similar to that obtained with Congo red staining. Strong positive staining for apo E was found on the amyloid deposits in the glomeruli. These results suggest that apo E is a common constituent of amyloid fibrils and that it may be a useful marker for immunohistochemical studies of systemic amyloidosis including renal amyloidosis.

Adult↗
PubMed1996

[Type AA renal amyloidosis in sarcoidosis].

Sarcoidosis is an uncommon cause of secondary amyloidosis. We describe in this paper the case of a 39 years old patient, with a pulmonary and hepatosplenic sarcoidosis. A nephrotic syndrome led to a renal biopsy which showed AA type amyloidosis. As no other cause of amyloidosis has been found we admitted that it was a result of sarcoidosis which was associated with the unusual inflammatory syndrome.

Adult↗
PubMed1996

[Cardiac amyloidosis: review of the literature].

The authors review the clinical impact of cardiac amyloidosis and new aspects concerning the management of this disease in the literature. Cardiac amyloidosis accounts for 5-10% of non-coronary myocardial disorders. It is a distinct form of cardiomyopathy with a severe prognosis. The authors review the role of endomyocardial biopsy and the relative sensitivity of non-invasive diagnostic methods. Chemical and genetic typing of amyloid, which is of considerable clinical value, is also reviewed due to recent evidence of treatment for different types of systemic and cardiac amyloidosis, such as the most common hereditary form caused by transthyretin gene mutation.

Amyloidosis↗
PubMed1997

Systemic amyloidosis with temporal artery involvement mimicking temporal arteritis.

A 77-year-old man presented with jaw claudication, arthralgias and myalgias, weight loss, marked fatigue, and thickened temporal arteries. No vasculitis was seen on the temporal artery biopsy specimen, but amyloidosis was suspected and confirmed with Congo red staining. Subsequent bone marrow biopsy revealed multiple myeloma. Although the patient initially was thought to have temporal arteritis, the results of temporal artery biopsy directed further investigations that led to the diagnosis of systemic amyloidosis. Systemic amyloidosis should be considered in the differential diagnosis when patients, especially men, present with clinical findings suggestive of temporal arteritis but without evidence of vasculitis in temporal artery biopsy specimens.

Aged↗
PubMed1997

[Amyloidosis of a coral reef infrarenal aorta].

This case concerns a 37-year-old woman with a cruro-gluteal claudication, from which she had been suffering since the age of 33, and which prevented her from walking more than 50 metres. The arteriographic examination revealed preocclusive coralliform proliferations of the infrarenal aorta, with a 30% stenosis of the right internal carotid artery. After 18 months, a straight aorto-aortic tube yielded excellent results. The results of the pathological examination led to the conclusion that this was a secondary aortic amyloidosis with no specific lesions of the aortic wall. This coral reef aorta is distinguished by its infrarenal location (fourth case worldwide), as well as by the major amylotic infiltrations of the endoaortic proliferations and of the aortic wall. In the absence of generalized amyloidosis, we suspect massive localized amyloidosis subsequent to an old inflammatory aortic process compatible with a juvenile atheroma opened in the aortic lumen or more probably a sequel of Takayasu's disease.

Adult↗
PubMed1997

[Primary amyloidosis of the gastrointestinal tract and liver--two case reports].

In primary amyloidosis the gastrointestinal tract and the liver are commonly involved, but clinical features and prognosis are mainly determined by the extent of cardiac and renal involvement. We review two cases with primary amyloidosis of the gastrointestinal tract and the liver. The predominant symptoms were malabsorption and hepatomegaly with cholestasis. The clinical aspects, diagnosis, treatment and prognosis of primary amyloidosis of the gastrointestinal tract and the liver are discussed in the context of the current literature.

Amyloidosis↗
PubMed1997

Increased acute-phase response and renal amyloidosis in aged CD2-fas-transgenic mice.

We previously demonstrated that increased Fas expression in T cells of aged CD2-fas transgenic (Fas-Tg) CD-1 mice results in an increased immune response and T cell apoptosis. Surprisingly, despite prevention of T cell immune senescence, the average life span of Fas-Tg mice is comparable with that of nontransgenic (non-Tg) mice. Histopathologic evaluation of tissue sections showed that nearly 50% of the aged (>18-mo-old) Fas-Tg mice developed renal amyloid A amyloidosis, whereas no amyloid deposition was observed in aged non-Tg mice. The amyloid A deposition was observed primarily in glomeruli by using immunohistochemical stains and electron microscopy. The full-length amino acid coding sequence of serum amyloid A2 cDNA in CD-1 mice was identical to that of amyloid A amyloidosis-susceptible BALB/c mice. Although there was no significant difference in steady-state serum amyloid A level in the serum of aged non-Tg and Fas-Tg mice, challenging mice with staphylococcal enterotoxin B resulted in significantly higher serum levels of serum amyloid A on day 2 and IL-6 on days 1 and 2 and a higher magnitude of weight loss on day 7 in aged Fas-Tg mice compared with young mice. These parameters, at the indicated time points, were equivalent between young and aged non-Tg mice. Taken together, our data suggest that prevention of T cell senescence in Fas-Tg mice may be a factor in induction of an excessive acute-phase response triggered by T cell activation. The Fas-Tg mice are a novel model for understanding the immunologic mechanisms leading to secondary amyloidosis.

Acute-Phase Reaction↗
PubMed1996

[Laryngeal amyloidosis: a case report].

Laryngeal amyloidosis is a rare, benign disease of the larynx the histology and clinical progression are uncertain. The present work reports a personal case of systemic amyloidosis principally involving the larynx. After a brief review of the literature regarding the classification of amyloidosis, its pathology and clinical implications are updated; in addition the present treatment modalities, diagnostic elements and prognosis are taken into consideration. In particular, the paper views the importance of a multidisciplinary approach in dealing with all patients presenting an organ-limited form of this disease as well as the application and advantages of carbon dioxide laser during the course of endoscopic surgery.

Amyloidosis↗
PubMed1997

Spontaneous rupture of the liver in amyloidosis.

Hepatic rupture is an unusual complication of hepatic amyloidosis. We report the case of a 59-yr-old man with a subcapsular hematoma of the liver with hepatic rupture secondary to amyloidosis. The patient died during the surgery. The etiology, pathology, diagnosis, and treatment of this complication are discussed. Previously reported cases of subcapsular hematoma of the liver secondary to amyloidosis are reviewed. This knowledge and an awareness of the possible diagnosis will help to decrease the high mortality rate associated with this complication.

Adolescent↗
PubMed1997

[A case of nephrotic syndrome associated with minimal renal amyloidosis--a four-year follow-up].

A 61-year-old male had nephrotic syndrome in association with minimal renal amyloidosis. The amyloid deposits were inconspicuous and had been initially overlooked, and the biopsy specimen was thought to show minimal glomerular changes. Accordingly he was diagnosed as having minimal change nephrotic syndrome (MCNS). Thereafter, administration of 40 mg/day of prednisolone was started. A few weeks after treatment, proteinuria decreased, but did not disappear. Four years later, he was readmitted for the treatment of the nephrotic syndrome. The second biopsy at 65 years of age revealed typical renal amyloidosis by light microscopy. Congo red staining and electron microscopy confirmed the presence of amyloid deposits. The serum A protein (SAA) level was 328 micrograms/ml. Furthermore, the first biopsy specimen revealed minimal renal amyloidosis by Congo red staining. At the time, 40 mg/day of prednisolone proved ineffective for the proteinuria. However, after methylprednisolone pulse therapy, the proteinuria decreased and the nephrotic syndrome improved. After discharge, administration of 20 mg/day of prednisolone was maintained. One year later, the patient showed no evidence of recurrence of the nephrotic syndrome and the SAA level decreased (from 328 micrograms/ml to 74.4 micrograms/ml). Prednisolone proved to have a beneficial effect on the reduction of proteinuria and SAA levels. We strongly recommend careful examination for amyloid deposits in all kidney biopsy specimens with the appearance of MCNS on older patients whose proteinuria does not respond to the administration of prednisolone.

Amyloidosis↗
PubMed1996

[Generalized amyloidosis associated with Hashimoto's thyroiditis and hypothyroidism].

Involvement of the thyroid gland in systemic amyloidosis is known since 1855, most frequently histologic than clinical. We report here a case of systemic amyloidosis associated with myxedema and Hashimoto's thyroiditis which has been rarely reported in the literature, and may represent a new example of auto-immune diseases associated with systemic amyloidosis.

Amyloidosis↗
PubMed1996

[Localized amyloidosis of the larynx].

Localized laryngeal amyloidosis is a rare laryngeal disease which accounts for less than 1% of all benign laryngeal tumors. The symptoms depend on where the amyloid deposit is located in the larynx. When the vocal cords are involved there may be some hoarseness; pain or increasing difficulty in inspiration may arise when, respectively, the aryepiglottic fold or subglottic space are involved. The present paper reports a case of laryngeal amyloidosis without any sign of systemic disease. It also deals with the principle diagnostic procedures to follow. After a review of the literature, emphasis is placed on how important it is to recognize laryngeal amyloidosis in order to achieve an appropriate diagnosis and plan therapy properly. Surgery is the main treatment using either endoscopy or an external neck approach. Recently CO2 laser surgery has been used successfully. Prognosis depends on both the size of the amyloid deposit and whether there is some simultaneous overall involvement.

Adult↗
PubMed1997

Serum amyloid P component enhances induction of murine amyloidosis.

Serum amyloid P component (SAP), a common component of all known types of amyloid fibrils, protects amyloid fibrils from proteolysis in vitro. It is therefore speculated to contribute to the deposition of amyloid fibrils in various types of amyloidoses. However, a role for SAP in amyloid deposition is not yet known. To investigate the relationship between SAP and amyloid deposition, we used gene targeting techniques to generate a unique strain of mice carrying a null mutation at the sap locus. The resultant SAP-deficient mice displayed no obvious phenotypic abnormalities. We asked whether experimental amyloid A (AA) amyloidosis could be induced in the SAP-deficient mice. The wild-type and SAP-deficient mice did not differ in their synthesis of serum amyloid A, the precursor protein of AA amyloid fibril, in response to acute inflammation. The induction of AA amyloidosis, however, was significantly retarded in the SAP-deficient mice relative to wild-type mice. Our experiments present, for the first time, compelling evidence that, although not essential in the deposition of AA amyloid, SAP significantly accelerates this reaction. Thus, SAP enhances the induction of murine amyloidosis and may play an important role in the pathogenesis of human amyloidoses, including Alzheimer's disease.

Acute-Phase Reaction↗