Articles: amyloidosis.
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The authors report the case of a 33-year old male Tunisian who, 7 years after the onset of complete Behçet's disease (diagnosed on international criteria) developed a nephrotic syndrome diagnosed as renal amyloidosis on histological evidence. Attenuation of Congo red staining by potassium permanganate pointed to AA type amyloidosis. Despite treatment with colchicine and cyclophosphamide the patient's condition deteriorated. ⋯ The patient died 3 years after amyloidosis was diagnosed. The authors have reviewed the literature concerning this exceptional association: in all cases where it was typed the amyloidosis was AA. Attention is drawn to the failure of colchicine in this patient.
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Am. Rev. Respir. Dis. · Jul 1985
Case ReportsDiagnosis of pulmonary amyloidosis by transbronchial biopsy.
Previously reported cases of pulmonary parenchymal amyloidosis were diagnosed by open lung biopsy or postmortem examination. We describe 3 patients who were found to have amyloid deposits within the lung parenchyma by flexible fiberoptic bronchoscopy. ⋯ We suggest that a diagnosis of pulmonary amyloidosis can be made by transbronchial biopsy provided the appropriate histologic stains are employed. Special stains for amyloid should be obtained whenever histologic sections from transbronchial biopsy specimens reveal amorphous eosinophilic material within the alveolar septa or within the walls of small vessels.
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Journal of cardiography · Mar 1985
Case Reports[Cardiac involvement in systemic amyloidosis: myocardial scintigraphic evaluation].
To assess the clinical significance of technetium-99m-pyrophosphate (Tc-99m-PYP), -methylene diphosphonate (Tc-99m-MDP) and thallium-201 (Tl-201) myocardial scintigraphy in the diagnosis of cardiac amyloidosis and in the differential diagnosis of cardiac diseases, 12 patients with biopsy-proved systemic amyloidosis (seven with familial amyloid polyneuropathy (FAP) and five with primary amyloidosis) were investigated. The results obtained were as follows: In 10 patients (six with FAP and four with primary amyloidosis) studied by Tc-99m-PYP scintigraphy, two (FAP one, primary amyloidosis one) had diffusely positive myocardial uptake, which was of greater intensity than that of the sternum. Six (four FAP; two primary amyloidosis) also had diffusely positive myocardial uptakes, but the intensity was less than that of the sternum. ⋯ Left ventricular hypertrophy was found in six patients and right ventricular visualization in five. Although electrocardiograms in seven of 10 patients showed QS patterns in the right to mid precordial leads, similar to that seen in antero-septal and extensive anterior myocardial infarctions, neither myocardial perfusion defect nor low uptake on Tl-201 images was detected in nine of them. The scintigrams in another one, which showed low uptake at the apical portion of the left ventricle, were considered normal.(ABSTRACT TRUNCATED AT 400 WORDS)
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Ann Med Interne (Paris) · Jan 1984
Case Reports[Adie's syndrome and polyneuropathy: apropos of a new case and a case of pupillotonia, polyneuropathy and generalized amyloidosis sparing the nerve].
The association of Adie's syndrome and of neuropathy of chronic evolution is not a frequent occurrence; first made known by Togi in 1967, four cases have been described ever since. The authors report two new observations: one concerns a man showing a unilateral tonic pupil associated to sensory neuropathy, raised C. ⋯ The other is that of a woman affected with bilateral pupillotonia, motor neuropathy of the four limbs and generalized amylosis. If the first observation is comparable to the five cases reported, the second is more questionable: the absence of amyloid deposit on three nerve biopsies and at the ciliary ganglion level does not absolutely exclude the possibility of amyloid neuropathy.
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Laryngeal amyloidosis and laryngoceles are uncommon. A unique case of both conditions occurring simultaneously is discussed. ⋯ Gross and histopathologic specimens give full confirmation of the diagnosis. To our knowledge, this is the first case of laryngocele secondary to amyloidosis.