Birth defects .... Cystic Diseases Kidney problem 4

Birth defects .... Cystic Diseases Kidney problem 4



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Birth defects .... Cystic Diseases Kidney problem 4
Posted in in 2013


ADPKD is a fairly common cause of kidney failure in adults.    The cysts of the disease may be seen
on a prenatal ultrasound of the developing fetus, but in most cases, the number of cysts in the
kidneys increases slowly and kidney function is normal for most or all of childhood and
into adulthood.   The cysts can start to form in the kidneys in the fetus, or the kidneys may be

normal without cysts until as late as 30 years of age.    At some age cysts start to form and more
and more cysts progressively form in both kidneys.   There is a tendency of the pattern
of development of cysts to be the same in members of a family, but it is a tendency and not a rule.

As more and more cysts form often hypertension becomes a problem, and then after hypertension,
gradual decrease in kidney function.   The age at which kidney failure occurs and dialysis and
transplantation are needed varies from infancy to old age.  A few people die in old age with cysts in
the kidneys but with the kidneys working well enough that they do not need dialysis or
transplantation.   The average age of kidney failure is about 50 years old.
ARPKD is a very uncommon disease.   The baby with ARPKD has many small cysts all throughout
the kidneys right from the time that they form in utero.   The kidneys are larger than normal because
of the cysts, sometimes very large.   In the most severely affected children the kidneys never work
and never make urine.    This leads to a lack of normal lung development, since lung development is
dependent on breathing amniotic fluid, and amniotic fluid comes from baby urine. Without lung
development the baby dies of lack of oxygen soon after birth.   In a few cases, fluid has been
successfully injected into the uterus around the baby every few days for many weeks during
pregnancy, resulting in lung development.    This is tricky and may also result in stimulating labor, so
that the baby is born quite prematurely with poor lung function and dies
Among the infants with the disease who survive, the severity of the kidney disease and the age at
which dialysis and transplantation are needed varies between infancy and adolescence.
Most children with ARPKD also have a condition known as congenital hepatic fibrosis, which means
that there are threads of scar tissue through the liver.  This usually doesn't interfere with liver function
but may obstruct the flow of blood to the liver through the portal vein from the intestines.    This leads

to back up of blood in the spleen and in veins of the esophagus and can cause serious problems, too. 
Backup of blood in the spleen means that platelets get trapped in the spleen and so are not in the
general blood circulation.   Platelets are important in the circulation as agents of clotting.   Meanwhile
the blood in the veins of the esophagus is under increased pressure, so that the veins often burst and
bleed.   With low platelets, the esophagus may bleed and bleed if the veins burst.    To prevent this
kind of bleeding, some children with ARPKD need surgery to shunt blood flow away from the
esophagus while those with a more severe form of hepatic fibrosis also need a liver transplant as well

as a kidney transplant.


My advise
1.....  If you are suffering from (either husband or wife) ARPKD you should take care before conceiving.
2...... Drink sufficient water and fruit juices

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