多囊性肾脏病变.pptxVIP

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.;;;;;;;;; The following classification is by no means all inclusive but aims to emphasize the important clinical cystic disorders likely to be encountered by the sonographer. Broadly speaking, cystic disease of the kidneys can be divided into two groups genetic disease and non-genetic disease: ;;;Genetic disease;; There are two types of presentation of ARPKDdepending on the age of the patient and the dominance of the renal or the hepatic disease. In those children who present at birth or during the neonatal period the renal disease dominates, while in those who present later in childhood or adolescence the liver disease dominates, with much milder renal manifestations. The hepatic disease in these children is called congenital hepatic fibrosis with renal tubular ectasia and comes to medical attention because of theproblems associated with hepatic fibrosis, such as splenomegaly, portal hypertension, varices and bleeding.;;Sonographic Appearances ● There is a wide spectrum of appearances from birth and with increasing age throughout childhood. ● There is bilateral equal involvement of the kidneys, and the reniform shape is usually maintained. ● The kidneys must be big and are g