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Table 1 Comparison of typical clinical features of ARPKD and ADPKD

From: Translational research approaches to study pediatric polycystic kidney disease

  ARPKD OPRHA:731 ADPKD OPRHA:730
Incidence 1:20.000 1:500-1:1000
Main clinical kidney manifestations Prenatal enlarged kidneys, cystic kidneys, oligo-/anhydramnios
Chronic kidney disease
Hyponatremia
Hypertension
Increased TKV, cystic kidneys
Hypertension
Proteinuria
Hematuria
Chronic kidney disease
Kidney Ultrasound Increased echogenicity of kidney parenchyma. „Salt-and-pepper“-pattern. Small, sometimes invisible cysts (<2mm).
More ADPKD-like pattern with advancing age
Cysts of different sizes in cortex and medulla.
Usually several large cysts.
Usually bilateral cysts
Hepatic Pathology Mandatory: Ductal plate malformation/congenital hepatic fibrosis with hyperplastic biliary ducts and portal fibrosis
Dilated bile ducts (Caroli syndrome)
Portal hypertension
Increased risk of cholangitis
Occasionally ductal plate malformation/congenital hepatic fibrosis
Liver cysts: Common in adults, rare in children.
Associated anomalies Neonatal respiratory distress/failure due to pulmonary hypoplasia
Rarely pancreatic cysts.
Single case reports of intracranial aneurysms.
Pancreatic cysts and/or cysts in other epithelial organs
Colon diverticula and hernia
Cardiovascular anomalies, and familiarly clustered intracranial aneurysms, abdominal Aorta aneurysms
Bronchiectasis
Pain