| Autor: |
P, Kasparian, J, Chebat, G, Feldmann, C, Degott, F, Seigneur |
| Jazyk: |
francouzština |
| Rok vydání: |
1978 |
| Předmět: |
|
| Zdroj: |
La Nouvelle presse medicale. 7(14) |
| ISSN: |
0301-1518 |
| Popis: |
Two unreleated adult males were found to be suffering from an association of pan-lobular severe emphysema and hepatomegally of unknown origin which led to the discovery of a marked deficit in alpha-1 antitrypsin (A1-AT) in relation to a PiZ phenotype. Liver biopsy revealed cirrhosis with portal fibrosis in one case and in both cases fatty infiltration with the accumulation of a glycoprotein antigenically identical to A1-AT. Electron microscopy showed this protein to be situated within the dilated lumina of the endoplasmic reticulum of the hepatocytes. A1-AT deficiency is usually associated with pulmonary involvement only in the adult and liver involvement only in the child. The association of the two remains rare--hence the interest of the two cases reported. |
| Databáze: |
OpenAIRE |
| Externí odkaz: |
|
