Renal agenesis occurs when a baby is born with only one kidney or no kidneys.
Condition Description. Renal agenesis is the name given to a condition that is present at birth that is an absence of one or both kidneys. The kidneys develop between the 5th and 12th week of fetal life, and by the 13th week they are normally producing urine.
In Potter syndrome, the primary problem is kidney failure. The kidneys fail to develop properly as the baby is growing in the womb. The kidneys normally produce the amniotic fluid (as urine). Potter phenotype refers to a typical facial appearance that occurs in a newborn when there is no amniotic fluid.
Morbidity/mortality
Potter syndrome is usually fatal in the first few days of the patient's life; most often, the cause is pulmonary failure. Bilateral renal agenesis is incompatible with extrauterine life, and 33% of fetuses die in utero.
Can you live without kidneys? Because your kidneys are so important, you cannot live without them. But it is possible to live a perfectly healthy life with only one working kidney.
Without dialysis or a kidney transplant, kidney failure is fatal. You may survive a few days or weeks without treatment.
Without life-sustaining dialysis or a kidney transplant, once a person with kidney disease reaches stage 5 (end stage renal disease or ESRD), toxins build up in the body and death usually comes within a few weeks.
Potter syndrome due to other causes is also often fatal at or shortly after birth, but there is an increased chance for survival. Infants who do survive the newborn period generally experience chronic lung disease and chronic kidney failure.
Potter syndrome, also known as Potter sequence, is a rare condition that affects how a fetus develops in the uterus. The condition is the result of abnormal kidney growth and function, which affects how much amniotic fluid surrounds the baby during pregnancy.
The mean survival for patients with Werner syndrome (WS) is 46 years. Death usually occurs when patients are aged 30-50 years because of atherosclerosis or malignant tumors.
Bilateral renal agenesis (BRA) is a rare and life-limiting condition in which the baby's kidneys never develop. The role of the kidneys is to clean waste out of the body and control fluid balance.
Bilateral renal agenesis is a lethal condition – the fetus may be stillborn or die shortly after delivery.
Bilateral renal agenesis, or absence of both kidneys, is commonly referred to as a “lethal” condition.
The most common disorders seen with horseshoe kidney include: Turner syndrome: a genetic disorder seen in girls that causes them to be shorter than others and to not mature sexually as they grow into adulthood. Sixty percent of girls with Turner syndrome have horseshoe kidneys.
A horseshoe kidney, also called renal fusion, is a condition a child is born with. The two kidneys fuse before birth. Many people don't experience symptoms, but complications from horseshoe kidney can occur. Most often, people have frequent UTIs and kidney stones.
Symptoms characteristic of Doege–Potter syndrome
Hypoglycemia (diaphoresis, tremor, anxiety, and lost consciousness) was the predominant symptom of Doege–Potter syndrome. Hypoglycemia can be the initial indicator that leads to the diagnosis of SFTs.
In general, classic Potter syndrome is terminal. The other types of Potter syndrome often cause death at the time of birth or shortly after. Infants who do survive typically experience severe long-term outcomes, such as respiratory distress and chronic renal failure.
Renal failure is the main defect in Potter's sequence. Other characteristic features include premature birth, breech presentation, atypical facial appearance, and limb malformations. Severe respiratory insufficiency leads to a fatal outcome in most infants.
Potter sequence and Potter syndrome are used interchangeably because the sequence of events leading to oligohydramnios is consistent. But the Potter sequence more specifically describes the decreased amniotic fluid irrespective of the cause.
Children born with one kidney (unilateral renal agenesis) are often healthy, don't have symptoms and don't need treatment. However, they may develop problems like high blood pressure and kidney disease in adulthood.
Bilateral renal agenesis, the absence of both kidneys, is rare, occurring in an estimated 1 out of every 4,000 to 5,000 newborns. The defect is more common in males. In some cases there is a family history.
If you have both kidneys completely removed, you will not make any urine. You will need to have kidney dialysis. This is a way of getting rid of waste products and excess water that the kidneys normally filter out of your blood. Dialysis means you can lead a more or less normal life without a working kidney.
When your kidneys are failing, a high concentration and accumulation of substances lead to brown, red, or purple urine. Studies suggest the urine color is due to abnormal protein or sugar as well as high numbers of cellular casts and red and white blood cells.
End-stage kidney disease (ESKD) is the last stage of long-term (chronic) kidney disease. This is when your kidneys can no longer support your body's needs. End-stage kidney disease is also called end-stage renal disease (ESRD).