[Caroli's disease, congenital hepatic fibrosis and renal polycystosis: a little frequent association]. 1996

E Rodríguez Rodríguez, and F Santolaria Fernández, and E González Reimers, and H Alvarez Argüelles, and M Cruz Díaz, and F Rodríguez Moreno, and J Oramas Rodríguez
Servicio de Medicina Interna. Hospital Universitario de Canarias. La Laguna, Tenerife.

We report the case of a woman with Caroli's syndrome (Caroli's disease and congenital hepatic fibrosis) and adult polycystic kidney disease. Whereas the association of these two entities with autosomal-recessive-polycystic kidney disease has been widely reported, its association with adult-polycystic kidney disease is very infrequent.

UI MeSH Term Description Entries
D007690 Polycystic Kidney Diseases Hereditary diseases that are characterized by the progressive expansion of a large number of tightly packed CYSTS within the KIDNEYS. They include diseases with autosomal dominant and autosomal recessive inheritance. Kidney, Polycystic,Polycystic Kidney,Polycystic Kidney Disease,Polycystic Kidneys,Polycystic Renal Disease,Disease, Polycystic Kidney,Disease, Polycystic Renal,Diseases, Polycystic Kidney,Diseases, Polycystic Renal,Kidney Disease, Polycystic,Kidney Diseases, Polycystic,Kidneys, Polycystic,Polycystic Renal Diseases,Renal Disease, Polycystic,Renal Diseases, Polycystic
D008103 Liver Cirrhosis Liver disease in which the normal microcirculation, the gross vascular anatomy, and the hepatic architecture have been variably destroyed and altered with fibrous septa surrounding regenerated or regenerating parenchymal nodules. Cirrhosis, Liver,Fibrosis, Liver,Hepatic Cirrhosis,Liver Fibrosis,Cirrhosis, Hepatic
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults
D014057 Tomography, X-Ray Computed Tomography using x-ray transmission and a computer algorithm to reconstruct the image. CAT Scan, X-Ray,CT Scan, X-Ray,Cine-CT,Computerized Tomography, X-Ray,Electron Beam Computed Tomography,Tomodensitometry,Tomography, Transmission Computed,X-Ray Tomography, Computed,CAT Scan, X Ray,CT X Ray,Computed Tomography, X-Ray,Computed X Ray Tomography,Computerized Tomography, X Ray,Electron Beam Tomography,Tomography, X Ray Computed,Tomography, X-Ray Computer Assisted,Tomography, X-Ray Computerized,Tomography, X-Ray Computerized Axial,Tomography, Xray Computed,X Ray Computerized Tomography,X Ray Tomography, Computed,X-Ray Computer Assisted Tomography,X-Ray Computerized Axial Tomography,Beam Tomography, Electron,CAT Scans, X-Ray,CT Scan, X Ray,CT Scans, X-Ray,CT X Rays,Cine CT,Computed Tomography, Transmission,Computed Tomography, X Ray,Computed Tomography, Xray,Computed X-Ray Tomography,Scan, X-Ray CAT,Scan, X-Ray CT,Scans, X-Ray CAT,Scans, X-Ray CT,Tomographies, Computed X-Ray,Tomography, Computed X-Ray,Tomography, Electron Beam,Tomography, X Ray Computer Assisted,Tomography, X Ray Computerized,Tomography, X Ray Computerized Axial,Transmission Computed Tomography,X Ray Computer Assisted Tomography,X Ray Computerized Axial Tomography,X Ray, CT,X Rays, CT,X-Ray CAT Scan,X-Ray CAT Scans,X-Ray CT Scan,X-Ray CT Scans,X-Ray Computed Tomography,X-Ray Computerized Tomography,Xray Computed Tomography
D016767 Caroli Disease Congenital cystic dilatation of the intrahepatic bile ducts (BILE DUCTS, INTRAHEPATIC). It consists of 2 types: simple Caroli disease is characterized by bile duct dilatation (ectasia) alone; and complex Caroli disease is characterized by bile duct dilatation with extensive hepatic fibrosis and portal hypertension (HYPERTENSION, PORTAL). Benign renal tubular ectasia is associated with both types of Caroli disease. Caroli's Disease,Caroli's Syndrome,Caroli Syndrome,Carolis Disease,Carolis Syndrome,Disease, Caroli,Disease, Caroli's,Syndrome, Caroli's

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