Polycystic Kidney

Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary renal disorder and has no gender predilection. This results in a large number of bilateral cortical and medullary renal cysts and accounts for 10% to 15% of patients receiving dialysis. On the other hand, Autosomal recessive polycystic kidney disease (ARPKD) is characterized pathologically as a spectrum of dilated renal collecting tubules, hepatic cysts, and periportal fibrosis.

Clinical presentation

Patients with ADPKD usually presents with signs and symptoms of;

Palpable mass in the flank
Flank pain
Hematuria
Hypertension
Recurrent UTIs

ARPKD is divided into four types; perinatal, neonatal, infantile, and juvenile depending on the patient's age at the onset of clinical manifestations.

Patients with perinatal disease (perinatal) have massively enlarged kidneys, hypoplastic lungs, and oligohydramnios.
Younger patients (neonatal) present predominantly with renal insuiciency.
Older children (infantile) will present with manifestations of Portal Hypertension.
Hepatic involvement is typically dominant in older patients (juvenile)

Ultrasound features

ADPKD
- At sonography, the kidneys are enlarged and replaced by multiple bilateral, asymmetric cysts of varying size.
- Cysts complicated by hemorrhage or infection have thick walls, internal echoes, and/or fluid-debris levels.
- Dystrophic calciication within cyst walls or stones may be seen as echogenic foci with sharp, distal acoustic shadowing.
ARPKD
- The kidneys are massively enlarged echogenic with a lack of corticomedullary diferentiation
- Occasionally, macroscopic cysts will be noted.

Enlarged kidneys with innumerable cysts of varying sizes — **ADPKD.** Figure 1: *The kidneys are of enlarged sizes and contains multiple cysts or ramdom sizes*. Figure 2: End-stage disease. *The kidneys are massively enlarged and completely replaced by cysts of varying sizes*.

kidneys with cysts of varyying sizes and random distribution in the cortex and medullar — **ADPKD.** Figure 1: *The kidneys are of enlarged sizes and contains multiple cysts or ramdom sizes*. Figure 2: End-stage disease. *The kidneys are massively enlarged and completely replaced by cysts of varying sizes*.

enlarged kidney with innumerable microcyst in patient with ARPKD — **ARPKD.** Figure 1: *Two year- old patient with renal insuficiency. Sagittal sonogram shows marked renal enlargement and innumerable microcysts*. Figure 2: *Sagittal sonogram of the liver for the same patient containing innumerable cysts*.

Multiple liver cysts in patient with polycystic kidney disease — **ARPKD.** Figure 1: *Two year- old patient with renal insuficiency. Sagittal sonogram shows marked renal enlargement and innumerable microcysts*. Figure 2: *Sagittal sonogram of the liver for the same patient containing innumerable cysts*.