Neonatal intrahepatic cholestasis caused by citrin deficiency: a histopathologic study of 10 cases / 中华病理学杂志
Chinese Journal of Pathology
;
(12): 452-455, 2012.
Artículo
en Chino
| WPRIM
| ID: wpr-303549
ABSTRACT
<p><b>OBJECTIVE</b>To investigate the diagnostic value of histopathological changes in the liver of patients with neonatal intrahepatic cholestasis caused by citrin deficiency (NICCD).</p><p><b>METHODS</b>Liver specimens from 10 cases of NICCD were evaluated by hematoxylin-eosin stain, histochemistry and immunohistochemistry (EnVision method). SLC25A13 mutation analysis was performed to correlate with histopathology.</p><p><b>RESULTS</b>Most specimens showed varying degrees of fat deposition in hepatocytes, necrotic inflammation, cholestasis and fibrosis (so-called tetralogy). The combination of the above four histological changes was highly characteristic for NICCD. With the progression of the disease, hepatic fibrosis deteriorated and ultimately led to cirrhosis.</p><p><b>CONCLUSIONS</b>NICCD should be suspected in the presence of cholestasis during infancy. A liver biopsy must be performed to rule out other liver diseases. The tetralogy of the hepatic histopathological changes has a highly diagnostic value for NICCD, which is also practical for accurately assessing the degree of inflammation and fibrosis, and similarly the progression of hepatic cirrhosis.</p>
Texto completo:
Disponible
Índice:
WPRIM (Pacífico Occidental)
Asunto principal:
Patología
/
Biopsia
/
Proteínas de Unión al Calcio
/
Colestasis Intrahepática
/
Progresión de la Enfermedad
/
Hepatocitos
/
Transportadores de Anión Orgánico
/
Proteínas de Transporte de Membrana Mitocondrial
/
Genética
/
Hígado
Límite:
Femenino
/
Humanos
/
Lactante
/
Masculino
Idioma:
Chino
Revista:
Chinese Journal of Pathology
Año:
2012
Tipo del documento:
Artículo
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