多囊性肾脏病变PPT课件

,单击此处编辑母版文本样式,第二级,第三级,第四级,第五级,*,单击此处编辑母版标题样式,单击此处编辑母版文本样式,第二级,第三级,第四级,第五级,单击此处编辑母版标题样式,*,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 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 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 dysplasiadysplastic 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 the cystic,renal disorder is not dependent on any,one imaging modality and will depend on,many factors.Sources of information when,trying 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 taken 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,include 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 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 acquired.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 practice 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,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 syndromes;,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 the 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.,Congenital hepatic fibrosis is a prerequisite for,the diagnosis of ARPKD.,Figure 3.31,Autosomal recessive polycystic kidney disease(ARPKD).(A)C