Search results for "AMYLOIDOSIS"
showing 10 items of 79 documents
Psoriasis arthropathica und Amyloidose
1972
Von insgesamt 5 Psoriasis arthropathica-Kranken wurden 4 rectumbioptisch, einer autoptisch auf das Vorliegen einer Amyloidose hin untersucht. Bei einem Patienten konnte ein positiver Amyloidnachweis gefuhrt werden. Es handelte sich dabei wie bei allen in der Literatur aufgefuhrten Fallen um die sekundare bzw. periretikulare Form der Amyloidablagerung. Anhand des vorliegenden Schrifttums wird die Haufigkeit einer Amyloidose bei Psoriatikern diskutiert, es werden Unterschiede zur Amyloidose bei primar chronischen Polyarthritis-Kranken aufgezeigt sowie Moglichkeiten der Amyloidgenese bei Psoriasis erortert.
Glomerul�re Amyloidose des Menschen ? unter besonderer Ber�cksichtigung der Proteinurie und der Amyloidogenese
1980
Light and electron microscopic investigations were carried out on kidney biopsies in renal amyloidosis cases. Particular attention was paid to the relationships between glomerular amyloidosis and proteinuria.
Evaluation of domino liver transplantations in Germany.
2013
Summary A retrospective multicenter study has been conducted to evaluate domino liver transplantations (DLTs) in Germany. The study provides insight into survival and features having an impact on the assessment of neuropathy after DLT. In addition, a neurologic follow-up program with a scheme to estimate the likelihood of de novo amyloidosis is presented. A series of 61 DLTs at seven transplant centers in Germany was enrolled. The mean age of domino recipients at the time of transplantation was 58 years, 46 of them being men, and 15 being women. The median follow-up was 46 months. The overall 1-, 3-, and 5-year survival of domino recipients was 81.6%, 70.8% and 68.8%, respectively. Causes o…
The Influence of Insulin on Local Amyloidosis of the Islets of Langerhans and Insulinoma
1980
Summary Amyloid-containing islets of Langerhans from the pancreas of 75 diabetics and one case of an amyloid-containing insulinoma were investigated. By light and electron microscopy, amyloid deposits were observed between the B cells and adjacent capillaries. The cytoplasm of B cells and extracellular amyloid display immunohistological binding of anti-insulin antibody. Correspondingly, ferritin-labeled anti-insulin antibody was found by electron microscopy on and between the amyloid fibrils. Insulin or proinsulin (or a protein closely related to insulin) thus appears to be a.component of the protein which constitutes the amyloid. The molecular weight and mode of deposition of this form of …
Asymptomatic immunoglobulin light chain amyloidosis (AL) at the time of diagnostic bone marrow biopsy in newly diagnosed patients with multiple myelo…
2011
The rate of asymptomatic amyloidosis (AL) among patients with newly diagnosed multiple myeloma (MM) or smoldering multiple myeloma (SMM) is unknown. We evaluated number and clinical significance of asymptomatic AL in consecutive MM and SMM patients, not having recognition of symptomatic AL at the time of their diagnostic bone marrow biopsy. Bone marrow biopsies were stained with Congo red and considered diagnostic for AL in case of positive Congo red staining with apple-green birefringence. Biopsies from 144 patients were evaluated: 77 had a diagnosis of MM and 67 of SMM. The median age was 59 (range 26–84) years; the median follow-up was 76 months (range 0–216). Immunoglobulin isotypes wer…
Amyloidoma of the gasserian ganglion as a cause of symptomatic neuralgia of the trigeminal nerve: report of three cases.
1993
Three cases of symptomatic neuralgia of the trigeminal nerve due to an amyloidoma in the gasserian ganglion are described. The correct diagnosis was not made prior to histological examination of the surgical biopsy specimens. Medical history and clinical observation led to the diagnosis of a malignant process of the nasal cavities in the first patient; of an inflammatory dental focus in the second patient; and of multiple sclerosis in the third patient. CT findings were normal in cases 1 and 2; in case 3, a schwannoma was suspected from the CT appearances. In case 1, MRI had not been performed; in cases 2 and 3, MRI revealed a tumour mass which was also considered to be a schwannoma. Histol…
Therapy of ATTR Cardiac Amyloidosis: Current Indications
2023
Transthyretin cardiac amyloidosis is a restrictive cardiomyopathy caused by extracellular deposition in the heart of amyloid fibrils derived from plasma transthyretin (ATTR), either in its hereditary (ATTRh) or acquired (ATTRwt) forms. Cardiac amyloidosis has a very poor prognosis if therapy is not started promptly. Therefore, it is very important to recognize cardiac amyloidosis early in order to immediately start a treatment capable of modifying the prognosis. Treatment of cardiac amyloidosis is not easy, often requiring a multidisciplinary team. New RNA-interfering drugs (such as patisiran) have been devised and are effective in the treatment of ATTRh amyloidosis. Tafamidis (a stabilizer…
Familial Amyloid Polyneuropathy
2013
Familial amyloid polyneuropathy (FAP; also known as familiar amyloidosis and hereditary amyloidosis) is an autosomal dominant inherited disease due to mutations of the transthyretin (TTR) gene coding for the corresponding protein, consisting of 127 amino acids. The gene is located on chromosome 18q. More than 100 different mutations are known. Other mutant precursor proteins produced in the liver, such as apolipoprotein I and II, lysozyme and fibrinogen Aα, may be of etiological importance as well. Amyloidogenic mutations of the TTR gene lead to decreased stability of the corresponding protein and subsequently to extracellular deposition of amyloid in several tissues (peripheral and autonom…