 Then we come to the next condition of bilateral cis-isolated, that is, artisomal dominant polystick kidney disease. Most common irritable cause of end stage renal disease, incidence is 1 in 400 to 1000, tiny percentage present in the fetal life, which is artisomal dominant. So, as a result scan of parents and grandparents has to be done. It is due to mutation in the PKD1 and PKD2 genes and 85 percent is due to mutation of PKD1 gene. There can be external disease, typically seen as cis in liver, pancreas and spleen, seminal vesicles, which are not seen in the prenatal scans. So, these cis can present as mass, pain, hypertension and hematuria. So, patients result in renal failure in 50 percent by 60 years. So, there is a recent international consensus statement on the diagnosis and management of artisomal dominant polystick kidney in children and young people. So, this article, there is an article. So, this lecture covers this article. Now ADPKD on ultrasound you see enlarged kidneys with multiple cis. It is almost always bilateral. There may be a variable cis size within the kidney or it may be asymmetrical involvement in the, on the right and left side. Extraternal manifestations may also be seen and most commonly include hepatic cis. Cis are less commonly seen in other organs like pancreas, spleen, ovaries and testis. Now, this is an example of ADPKD where you see enlarged kidneys with multiple cis, but the cis size are varying and it is asymmetrical. It is less in the right kidney whereas more in left kidney and there are also hepatic cis. Another example of ADPKD with bilateral cis in the kidneys and you see extraordinary manifestation in the form of multiple hepatic cis is seen in the liver. Now, another example of ADPKD, the polystick disease of the kidneys and as well as cis in the liver as seen here and also rarely there may be cis in the other organs. Here this is a transverse scan of the pancreas showing a cis in the body of the pancreas. It will rare, more common is in the liver. There can be some variations of ADPKD like here it can involve an ectopic kidney. Now this is a coronal scan of the right side showing polystick kidney of the right side, right kidney. Coral scan of the left flank shows that the left renal fossa is empty and the search for an ectopic kidney shows sagittal scan of the bladder and polystick kidney of the ectopic pre-cycle kidney and a little more cranial scan shows that the ectopic pre-cycle kidney is replaced by multiple cis typical of polycystic kidney disease and there is also liver cis. Now, in the Alzheimer dominant polystick kidneys with a family history that is in high risk patients because of history of the family history, there is a pre-raven criteria for a diagnosis. Between the age 15 to 39 years, presence of three renal cis in both kidneys would confirm the diagnosis. In between 40 and 59 years at least two cysts in each kidney then the diagnosis aged 30 years or older at least four cysts in each kidney will give the diagnosis of ADPKD. Ultrasonic 0 or 1 cyst at age 40 years excludes ADPKD. The cysts in ADPKD may show complications of hemorrhage, infection, rupture or nephrolithiasis and the ADPKD results in end stage renal disease about 50% as the age advances. There is no increased risk of renal cell carcinoma. Now, here you see images of ADPKD with bilateral polystick kidneys and cis in the liver. On the left side you see fresh plot in one of the cysts as seen as a soft tissue mass. It may shift in position. Now, fresh clot can also appear as a heterogeneous mass partly filling the cysts or completely filling the cysts as seen in these images. The hemorrhage is subacute will result in appearance of internal echos in the cysts as seen here in these two images. Calculating the collecting system of the polystick kidneys may occur as seen here you see two calculate in the lower calyx of left kidney. This occurs in 16 to 25% of ADPKD. It can result in renal colic, microscopic or gross hematuria or it can result in urinary tract obstruction. So, calculate causing urinary tract obstruction in this left kidney you see polystick kidney and they dilated the ureter medial to the cyst and it trace the ureter you see a calculus in the upper ureter causing obstruction in the patient presenting with ureteric colic. There may be milk of calcium filling the some of the cysts in the polycystic kidneys. Very early onset ADPKD can manifest as hyperechoic kidneys prenatally or in the newborn with ultrasound appearance mimicking that of ARPKD or glomerulocystic disease that is you see bilateral enlarged echogenic kidneys and with or without tiny cysts in the cortex. So, this appearance should give rise to a suspicion of ADPKD and if there is a family history then it is diagnostic otherwise they has to be children suspected of having ADPKD without a genetic diagnosis or clear family history. Confirmatory ultrasound should be performed within 12 months after the initial screening to rule out ADPKD. Now prognosis of ADPKD 50 percent of them end up in renal failure as the age advances. If it is seen prenatally then the prognosis is very poor.