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Potter's syndrome

  1. Potter's syndrome is a rare birth defect. Babies with this condition do not have kidneys or kidneys that are not properly constructed. This causes a shortage of amniotic fluid in the uterus. They often die during pregnancy or shortly after birth.

  1. Another name for Potter's syndrome is Potter sequence. The syndrome is named after Edith Potter, the American pathologist who described it. Sequence means 'consecutive'. The Potter sequence consists of a number of symptoms that occur in sequence, starting with the malfunctioning kidneys.

Symptoms

  1. Because the fetus in Potter's syndrome has abnormal or missing kidneys, various problems arise during pregnancy. A healthy child drinks amniotic fluid and urinates it out again, so that the amount of amniotic fluid is maintained. The malfunctioning kidneys prevent a fetus with Potter's syndrome from producing urine. This creates a shortage of amniotic fluid in the uterus and the mother's abdomen grows less well. Due to the lack of amniotic fluid, the baby becomes oppressed, preventing it from growing or moving properly. As a result, the baby develops stunted growth and abnormalities can develop in the arms, legs and face, such as club feet, a flat nose, a small chin and low-set ears. The shortage of amniotic fluid also prevents the chest and lungs from developing properly.

Diagnosis

  1. The Potter sequence is caused by certain hereditary conditions. The abnormality is rare: it occurs in about 1 in 5000 children, slightly more often in boys than girls. It is often revealed during pregnancy that the fetus has Potter's syndrome. If the mother's abdomen is not growing properly or if an ultrasound shows that there is little amniotic fluid present, this may indicate the syndrome. An ultrasound may also show that the child has a growth delay or has no or poorly developed kidneys. A flake test must then reveal whether the fetus does indeed have Potter's syndrome. Sometimes it is discovered after birth that a baby has the syndrome, because the external abnormalities are only visible and the baby appears to have breathing difficulties. An X-ray shows whether there is something wrong with the kidneys and lungs. If it is unclear what caused a newborn baby to die, an autopsy can reveal whether Potter's syndrome was the cause of death.

Treatment

  1. Potter's syndrome cannot be prevented or treated. If a child with the Potter sequence is born alive, it has serious breathing problems, usually dying within 24 hours. In the future, a baby with the syndrome may be able to have new kidneys during pregnancy through a kidney transplant.

  2. Potter's syndrome cannot be prevented or treated. If a child with the Potter sequence is born alive, it has serious breathing problems, usually dying within 24 hours. In the future, a baby with the syndrome may have new kidneys during pregnancy through a kidney transplant.



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