2018年多囊性肾脏病变ppt课件-文档资料.ppt
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1、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 i
2、n children., Multicystic kidney or multicystic dysplastic kidneythis 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 ki
3、dney 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 dysplasiadysplastic kidneys can be unilateral or bilateral, usually contain cysts and are disorganized, cont
4、aining 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 norm
5、al 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 condition
6、s: 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 tubule
7、s. Autosomal dominant renal disease was previously known as adult polycystic kidney disease. Cysts develop anywhere along the nephron.,The ultimate diagnosis of the cystic renal disorder is not dependent on any one imaging modality and will depend on many factors. Sources of information when trying
8、to come to the diagnosis should be collated from many areas, such as:, obstetric history of the mother prenatal history and fetal ultrasonography family history information clinical examination of the child radiology of patient and parents laboratory data, for example DNA pathology if a biopsy is ta
9、ken or from any other family members that may have had a biopsy or nephrectomy in the past.,Ultrasound is still the imaging modality of choice in children, and the findings on ultrasound will direct further imaging as required. The ultrasound approach to any cystic renal disease in children must inc
10、lude observations about the following, which should be carefully stated in the ultrasound report., unilateral or bilateral renal cysts (bilateral involvement is more common in the genetically inherited conditions) size of the kidneysare they large or small? localization to one part of the kidney or
11、diffuse involvement of the whole kidney. Is there a capsule around the cysts? extrarenal cysts, in particular in the liver or pancreas liver size and hepatic parenchyma appearance presence of a large spleen and portal hypertension.,Renal cysts are common and may be hereditary,developmental or acquir
12、ed. The classification of cystic renal disease varies according to the perspective from which it is written, and despite a vast amount of literature on the subject, there is still no generally accepted classification in existence. The early Potter classification is of limited value for clinical prac
13、tice because not all types represent clinical entities.,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
14、genetic disease and non-genetic disease:, Genetic disease autosomal recessive polycystic kidney disease (ARPKD) autosomal dominant polycystic kidney disease (ADPKD) juvenile nephronophthisis and medullary cystic disease complex glomerulocystic kidney disease cysts with multiple malformation syndrome
15、s;, Non-genetic disease simple cysts multicystic dysplastic kidney multilocular cysts acquired renal cystic disease (chronic renal failure) caliceal diverticulum medullary sponge kidney.,Genetic disease,Autosomal recessive polycystic kidney disease (ARPKD) This is a generalized cystic dilation of th
16、e renal collecting tubules so that the kidneys are packed to a greater or lesser degree with tiny little cysts (Fig. 3.31). It is much rarer than the autosomal dominant form and occurs in 1 in 50 000 people. Prenatal diagnosis can be made but there are false positive and false negative diagnoses. Co
17、ngenital hepatic fibrosis is a prerequisite for the diagnosis of ARPKD.,Figure 3.31 Autosomal recessive polycystic kidney disease (ARPKD).(A) Cut section of a postmortem specimen in a patient with ARPKD. There are multiple small cysts throughout the whole of the renal substance. It is these multiple
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