introduction: autosomal dominant polycystic kidney disease (adpkd) is one of the most common genetic kidney disorders with the incidence of 1 in 1,000 births. adpkd is genetically heterogeneous with two genes identified: pkd1 (16p13.3, 46 exons) and pkd2 (4q21, 15 exons). eighty five percent of the patients with adpkd have at least one mutation in the pkd1 gene. genetic studies have demonstrate...

Polycystic kidney disease is an inherited condition, characterized by the development of cysts in the kidney, as well as in other organs. Patients with polycystic kidney can suffer from the same causes of acute kidney injury as the general population. Nephritic syndrome is an uncommon cause of acute kidney injury in the general population and less common in patients with polycystic kidney disea...

Polycystic kidney disease is a multisystemic familial inherited disease. A symptomatic treatment approach the standard modality for autosomal dominant polycystic (ADPKD) complications and life-long 50% chronic renal failure risk, as transplantation only definitive treatment.

congenital hepatic fibrosis (chf) is a rare disease that affects both the liver and kidneys.  congenital hepatic fibrosis (chf) is an autosomal recessive inherited malformation defined pathologically by a variable degree of periportal fibrosis and irregularly shaped proliferating bile ducts. affected individuals also have impaired renal function, usually caused, in children and teenagers, by an...

et al. Caspase inhibition reduces tubular apoptosis and proliferation and slows disease progression in polycystic kidney disease. Development of polycystic kidney disease in juvenile cystic kidney mice: insights into pathogenesis, ciliary abnormalities, and common features with human disease. Magenheimer BS et al. Calcium restriction allows cAMP activation of the B-Raf/ERK pathway, switching ce...

Background Autosomal recessive polycystic kidney disease (ARPKD; OMIM number 263200) is a severe early onset hereditary form of polycystic kidney and liver disease. Case Report In the current study, we present a consanguineous couple with a history of an affected son with polycystic kidney disease (PKD), hepatic failure and epileptic seizures who died at the age of 8 months. Both parents were h...

autosomal dominant polycystic kidney disease (adpkd) is an inherited disorder associated with multiple cyst formation in the different organs. development of pancreatic cyst in adpkd is often asymptomatic and is associated with no complication. a 38‑year‑old man with adpkd was presented with six episodes of acute pancreatitis and two episodes of cholangitis in a period of 12 months. various ima...

BACKGROUND Kidney stones in patients with autosomal dominant polycystic kidney disease are common, regarded as the consequence of the combination of anatomic abnormality and metabolic risk factors. However, complete staghorn calculus is rare in polycystic kidney disease and predicts a gloomy prognosis of kidney. For general population, recent data showed metabolic factors were the dominant caus...