Autosomal Recessive Polycystic Kidney Disease (ARPKD) is a genetic disorder
in which there are many fluid-filled cysts in kidneys of unborn children. The
children usually can not live out their childhood. Some of them may grow up with
those cysts in their kidneys, but many severe symptoms and complications are
torturing them all the time. There are some characters of Autosomal Recessive
Polycystic Kidney Disease (ARPKD).
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Fetal stage
At this stage, if the mother takes CT, the cysts will increase parenchymal
echogenicity in her baby’s kidneys. Moreover, amniotic fluid of the mother is
usually less than other mothers, because her baby’s kidneys fail in draining out
the normal excrement. With this condition, the baby will have the undergrown
lung and the malformation of spine. What’s worse, its big kidneys and spleen
will make its mother dead for dystocia.
Neonatal stage
Neonate with ARPKD is usually dead several hours later after she or he was
born mainly because of respiratory insufficiency or respiratory failure which is
attributed to lung malformation, aerothorax and congestive heart failure.
However, some of them will survive after their respiratory function return
closely normal. With decreasing glomeruli filtrate rate (GFR), their renal
function as well as urine is affected, but they are rarely dead from Kidney
Failure at this stage.Relationship between hyperlipidemia and Polycystic Kidney Disease
Infancy
The surviving neonates are destined to be troubled by high blood pressure and
renal insufficiency. High blood pressure should take the main responsibility for
their death in their period of infancy, because it can cause severe
complications such as cardiomegaly and congestive heart failure which can bring
sudden death to them.
Adolescence and adult
As time goes, more and more complications occur among those people with
ARPKD, who mainly show geneogenous hepatic fibrosis, expansion of renal
parenchyma, portal hypertension and cholangitis. Portal hypertension can cause
swollen spleen and hypersplenism which reduce complete blood cells. The most
severe complication is cholangitis which make liver dead suddenly.
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In other word, Autosomal Recessive Polycystic Kidney Disease (ARPKD) is a
disease which can damage all organs of human beings. At present, the treatments
mainly emphasize on easing their symptoms and complications.