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by ebt0125
Last updated 6 years ago

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ARPKD Kidney

Healthy Kidney

Healthy Liver


Children with ARPKD also have the liver abnormality called congenital hepatic fibrosis (CHF) that may lead eventually to enlargement of the liver and spleen. In the liver, the abnormality can impede the return of blood from the intestine to the liver. This condition, called portal hypertension, can lead to distention (varices) and increased pressure in the veins around the esophagus, the stomach, and the intestine. These varices can rupture, leading sometimes to life-threatening gastro-intestinal bleeding (inside the body bleeding in an organ). In addition, portal hypertension can cause splenic enlargement or Splenomegaly which is a larger-than-normal spleen, and can cause Hypersplenism. Hypersplenism is an overactive spleen. The spleen is an organ found in the upper left side of your belly area. The spleen helps filter old and damaged cells from your bloodstream. If your spleen is overactive, it removes the blood cells too early and too quickly, with resulting low red blood cell, white blood cell and platelet counts.

The kidneys can become quite enlarged. In addition, the normal function of the collecting (a liquid that keeps it alive) tubule is disrupted. In the normal kidney, the collecting tubule makes the amount of water and acid in the tubular fluid so that the body keeps an appropriate amount of water and eliminates excess amounts of acid. In ARPKD patients, the cystic collecting ducts cannot retrieve water efficiently, causing much more urine than in children with normal kidneys.

ARPKDBy Ethan Teige

This Video Explains the treatment, symptoms, and causes of ARPKD.

Autosomal recessive polycystic kidney disease, ARPKD, is a rare disorder that is Recessive, and there are no other types of this disease (Ex: type one diabetes). Approximately 1 in 20,000 people have ARPKD. It affects boys and girls equally and can cause death in the first month after birth. If the child survives the first month, the chances of survival are good, but they will have a rough life. There are no treatments to slow or cure ARPKD.In ARPKD patients, small cysts form. A cyst is a bubble-like thing that grows under the skin, and can pop through the skin at any time. In ARPKD, both kidneys are affected, and liver with these cysts.


once you get past the first couple years of ARPKD, you are very likely to survive. But your life is limited because the side affects still occur. you wouldn't be able to eat hard, and big foods. you also cannot eat the normal amount of food like everyone else. and on a side note, to be safe you might need to bring portable toilet on even an hour trip becuase you have to urinate almost every 30 minutes. And last, but certainly not least, you're kidneys and liver can fail on you at any moment. it's very unlikely becuase there has only been about 300 known records of this ever happening. you have to have about 7 visits to the doctor a year.


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