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A explanation autosomal recessive polycystic kidney disease

May 08, 2017

Polycystic kidney disease is pided into autosomal dominant and recessive genetic diseases, and the majority of polycystic kidney patients are autosomal dominant polycystic kidney disease.Why is the number of patients with autosomal recessive polycystic kidney disease and autosomal dominant polycystic kidney disease in patients with such a large gap?

Understand autosomal recessive polycystic kidney disease and find out why.

Autosomal recessive polycystic kidney disease (ARPKD) with autosomal recessive inheritance.Almost all parents don't have the same history.Autosomal recessive polycystic kidney disease also known as infantile polycystic kidney, for the rare type of polycystic kidney.Often die shortly after birth, with only a few the lighter types that can survive to childhood or even adulthood.

 ARPKD patients often die shortly after birth, with only a few the lighter types that can survive to childhood or even adulthood.Tongshantang experts for you to resolve autosomal recessive polycystic kidney disease classification and characteristics:  1.Perinatal type:When there are serious perinatal renal cystic diseases,involving 90% collection tube, at the same time there is a small amount of portal vein around the fiber proliferation, in perinatal death.  2.Neonatal type:Involving 60% collection tube, with mild portal vein around fibrous proliferation.Was born a month after the onset of symptoms, when a few months died of kidney failure.  3.Infantile type:The baby type showed bilateral renal enlargement,involving 25% renal,liver, splenomegaly and with mild portal vein around fibrous proliferation.3 ~ 6 months after birth appeared symptoms,died of renal failure in childhood.  4.Juvenile type:Juvenile onset symptoms in 13~19 years old.Renal damage was relatively mild,and only less 10% of the renal tubules showed cystic degeneration and occasionally developed renal failure.Severe fibrosis of the hepatic portal vein.Generally around 20 years of age due to liver complications, portal hypertension death.Autosomal recessive polycystic kidney main cause double kidney seeper Polycystic renal dysplasia Congenital liver fiber hyperplasia with shaofu zhuyu tang.Tongshantang experts pointed out that the current medicine for autosomal recessive polycystic kidney disease without special treatment, the prognosis is bad.When renal insufficiency, due to hepatic lesions often cannot accept dialysis or transplantation.Portal hypertension upper gastrointestinal bleeding is often life-threatening.Due to renal failure and infection, should not shunt operation.Kidney and liver damage increased the difficulty of treatment.
Clear understanding of autosomal recessive polycystic kidney disease, convenient for everyone to prescribe the right medicine, understand the condition, make a reasonable judgment.

 

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