[Polycystic liver disease without autosomal dominant polycystic kidney disease]. 2003

R Peces, and P González, and J L Venegas
Sección de Nefrología, Hospital General La Mancha-Centro, Avda. de la Constitución, 3, 13600 Alcázar de San Juan, Ciudad Real. cpeces@varnet.com

Polycystic liver disease is characterized by the presence of multiple bile duct-derived epithelial cysts scattered in the liver parenchyma. The natural history and clinical manifestations of polycystic liver disease are based on the disease as it manifests in patients with autosomal dominant polycystic kidney disease (ADPKD). The occurrence of polycystic liver disease independently from polycystic kidney disease has been known for a long time. More recently, a gene for autosomal dominant polycystic liver disease has been identified on chromosome 19p 13.2-13.1. Isolated polycystic liver disease is underdiagnosed and genetically distinct from polycystic liver disease associated with ADPKD but with similar pathogenesis and clinical manifestations. We report here two men with polycystic liver disease no associated with ADPKD. Ultrasound and computed tomography imaging were effective in documenting the underlying lesions non-invasively.

UI MeSH Term Description Entries
D008107 Liver Diseases Pathological processes of the LIVER. Liver Dysfunction,Disease, Liver,Diseases, Liver,Dysfunction, Liver,Dysfunctions, Liver,Liver Disease,Liver Dysfunctions
D008297 Male Males
D011859 Radiography Examination of any part of the body for diagnostic purposes by means of X-RAYS or GAMMA RAYS, recording the image on a sensitized surface (such as photographic film). Radiology, Diagnostic X-Ray,Roentgenography,X-Ray, Diagnostic,Diagnostic X-Ray,Diagnostic X-Ray Radiology,X-Ray Radiology, Diagnostic,Diagnostic X Ray,Diagnostic X Ray Radiology,Diagnostic X-Rays,Radiology, Diagnostic X Ray,X Ray Radiology, Diagnostic,X Ray, Diagnostic,X-Rays, Diagnostic
D003560 Cysts Any fluid-filled closed cavity or sac that is lined by an EPITHELIUM. Cysts can be of normal, abnormal, non-neoplastic, or neoplastic tissues. Cyst
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
D000368 Aged A person 65 years of age or older. For a person older than 79 years, AGED, 80 AND OVER is available. Elderly
D015898 Tomography Scanners, X-Ray Computed X-ray image-detecting devices that make a focused image of body structures lying in a predetermined plane from which more complex images are computed. CAT Scanners, X-Ray,CT Scanner, X-Ray,Computed Tomography Scanners, X-Ray,Tomography, Computed, Scanners,Tomography, X-Ray Computed, Scanners,CAT Scanner, X-Ray,Computed Tomography Scanner, X-Ray,Tomography, X-Ray Computed, Scanner,X-Ray Computed Tomography Scanner,X-Ray Computed Tomography Scanners,CAT Scanner, X Ray,CAT Scanners, X Ray,CT Scanner, X Ray,CT Scanners, X-Ray,Computed Tomography Scanner, X Ray,Computed Tomography Scanners, X Ray,Scanner, X-Ray CAT,Scanner, X-Ray CT,Scanners, X-Ray CAT,Scanners, X-Ray CT,Tomography Scanners, X Ray Computed,X Ray Computed Tomography Scanner,X Ray Computed Tomography Scanners,X-Ray CAT Scanner,X-Ray CAT Scanners,X-Ray CT Scanner,X-Ray CT Scanners
D016891 Polycystic Kidney, Autosomal Dominant Kidney disorders with autosomal dominant inheritance and characterized by multiple CYSTS in both KIDNEYS with progressive deterioration of renal function. Autosomal Dominant Polycystic Kidney,Kidney, Polycystic, Autosomal Dominant,ADPKD,Adult Polycystic Kidney Disease,Adult Polycystic Kidney Disease Type 1,Adult Polycystic Kidney Disease Type 2,Polycystic Kidney Disease 2,Polycystic Kidney Disease, Adult,Polycystic Kidney Disease, Adult Type 2,Polycystic Kidney Disease, Adult, Type II,Polycystic Kidney Disease, Autosomal Dominant,Polycystic Kidney Disease, Type 2,Polycystic Kidney, Type 1 Autosomal Dominant Disease,Polycystic Kidney, Type 2 Autosomal Dominant Disease

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