Author/Editor     Knehtl, Maša; Ćeranić, Davorin; Urlep, Dejan; Glumbić, Ivana; Kavalar, Rajko; Skalicky, Marjan; Skok, Pavel
Title     Jetrna amiloidoza - predstavitev bolnika in pregled literature
Translated title     Hepatic amyloidosis - case report and literature review
Type     članek
Source     Zdrav Vestn
Vol. and No.     Letnik 77, št. 2
Publication year     2008
Volume     str. 135-9
Language     slo
Abstract     Amyloidosis is a disorder of protein metabolism that leads to extracellular deposition of insoluble proteinaceous material. Clinically important are amyloidosis AL and AA. Primary or AL form is associated with immunocyte dyscrasia, multiple myeloma, macroglobulinemia or monoclonal gammopathy, the precursor protein are immunoglobulin light chains. Amyloidosis AA or reactive amyloidosis is associated with chronic inflammatory disease. The authors are presenting a52 year old man who was transferred to their departmentf or diagnostic proceedings of anaemia and liver disease from the department o f o rthopaedics, where he was treated for compression fractures of the spine. tlnfortunately, during the first hospitalisation the patient refused liver biopsy. The patient died due to spleenic rupture and exsanguination into abdominal cavity. At autopsy, amyloidosis was ,found with amyloid deposits in the liver and other organs. In the article, different forms of this disease with grim prognosis are described. The authors presented a patient with primary amyloidosis which led to, fatal complication. According to the literature, patients with this form of amyloidosis have median survival of 1-2 years from establishing the diaguosis.
Descriptors     LIVER DISEASES
AMYLOIDOSIS
MIDDLE AGE