多囊性肾脏病变ppt课件.ppt

CYSTIC KIDNEYS Cystic disease of the kidneys in childhood is a confusing and complicated subject. Also the complex and often contradictory nomenclature used to describe cystic kidneys is not an aid to understanding the subject. This section aims to provide the sonographer with a simple approach to cystic kidneys in children. ● Multicystic kidney or multicystic dysplastic kidney—this refers to many cysts in one kidney often containing some dysplastic elements. Not all multicystic kidneys are dysplastic. The condition is still considered to be non-hereditary. If the multicystic kidney is unilateral, the other kidney may be normal, hydronephrotic or dysplastic. If bilateral, it is incompatible with life, and infants die soon after birth with hypoplastic lungs and/or renal failure. Terminology ● Cystic dysplasia—dysplastic kidneys can be unilateral or bilateral, usually contain cysts and are disorganized, containing ectopic tissue such as cartilage and muscle. They may function.Ultrasonically they usually appear small and echogenic with small peripheral cortical cysts. While dysplastic kidneys are often hypoplastic, not all small kidneys are dysplastic. The clinical features are very variable from a normal appearing neonate to a very dysmorphic infant. Dysplastic kidneys are associated with urinary tract obstruction, and many syndromes are associated with cystic dysplastic kidneys. Bilateral renal dysplasia will result in progressive renal failure. ● Polycystic kidney disease refers to two conditions: autosomal recessive polycystic kidney disease and autosomal dominant renal disease. —Autosomal recessive polycystic kidneys were previously known as infantile polycystic kidneys. Confusingly these kidneys appear highly echogenic on ultrasound. There is generalized dilation of the collecting tubules. —Autosomal dominant renal disease was previously known as adult polycystic kidney disease. Cysts develop anywhere along the nephron. The ultimate diagnosis of