Inherited, chronic, progressive hepatic degeneration in Bedlington terriers with increased liver copper concentrations: clinical and pathologic observations and comparison with other copper-associated liver diseases.

摘要: One hundred nineteen hepatic tissue samples from 117 Bedlington Terriers were divided into 6 groups depending on the severity of histopathologic changes. Group 0 comprised dogs with microscopically normal livers. I had copper-positive, lipofuscin-containing lysosomes present in centrilobular hepatocytes. Microfoci necrosis, addition to increased numbers and periportal hepatocytes, group II dogs. III more translobularly morphologic changes consistent chronic active hepatitis. Mixed micro- or macronodular cirrhosis translobular presence characterized IV Dogs V massive necrosis that Histochemical staining for copper was useful making microscopic diagnosis this disease shown be necessary early (group I) when other clinical pathologic values associated syndrome not consistently abnormal. Copper histochemical stains varied sensitivity. Timm's silver sulfide sensitive than rubeanic acid, which rhodanine staining. The brown pigment lipofuscin aid orcein, Prussian blue, periodic acid-Schiff, acid-fast together fluorescent microscopy (excitation maxima: 365 nm; emissions: 420 + nm). Since these positive only later stages disease, they especially its diagnosis. increase age increasing concentration. These observations illustrate inherited, degeneration Terrier is progressive. Clinical chemical tests diagnostically disease. Alanine transaminase activity most value, but always abnormal, even severe damage present. signs seen III, IV, V. Death due failure occurred Hemosiderin amounts liver, bone marrow, spleen, lymph nodes affected Terriers, indicating a possible defect iron metabolism and/or an RBC turnover existed.(ABSTRACT TRUNCATED AT 400 WORDS)