[Polycystic kidney disease in children].
Polycystic renal diseases in children include 2 pathologically and genetically distinct diseases: autosomal recessive polycystic kidney disease (ARPKD), incorrectly called the "infantile form", characterized by a constant hepato-renal involvement, and autosomal dominant polycystic kidney disease, often termed the "adult form", which is more and more frequently detected in children as a result of advances in renal imaging techniques. The differential diagnosis is not based on age at discovery, clinical symptoms or renal imaging, which may all be similar in the 2 diseases, but on the presence, detected by either pathology or ultrasonography, of the biliary dysgenesis specific to ARPKD, and mainly on the mode of inheritance, confirmed by the results of ultrasonography in parents and grandparents.
Gagnadoux
M F
MF
Service de néphrologie pédiatrique, INSERM U 192, hôpital Necker-Enfants-Malades, Paris, France.
Habib
R
R
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005260:Female; D005799:Genes, Dominant; D005808:Genes, Recessive; D006530:Hepatorenal Syndrome / Q000209:etiology; D006801:Humans; D007223:Infant; D008297:Male; D010375:Pedigree; D007690:Polycystic Kidney Diseases / Q000150:complications / Q000175:diagnosis / Q000235:genetics* / Q000473:pathology; D014463:Ultrasonography
Les polykystoses rénales de l'enfant.