Table 1 Comparison of typical clinical features of ARPKD and ADPKD
From: Translational research approaches to study pediatric polycystic kidney disease
| Â | 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 |