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Cystic Kidney Disease

more about Cystic Kidney Disease

Polycystic kidney disease, multicystic kidney disease, malformation of the kidney

  • Fluid-filled sacs form in the kidneys.  This is usually present from the time an individual is a fetus, but sometimes the sacs form later in life.
  • The fluid-filled sacs might be small or large.  In the normal kidneys, there are no sacs.
  • Polycystic kidney disease (PKD) has cysts in both the right and left kidneys, and has a familial tendency.  The polycystic kidney retains the shape of a kidney even though there are numerous cysts in it; and it is able to perform the kidney's function of filtering waste products out of the blood.  It may not perform this function perfectly.  PKD can be discovered at any age.  The younger the age when symptoms develop, the more serious the kidney impairment is likely to be.
  • In multicystic kidney disease (MKD), the affected kidney has cysts, often quite large, and the kidney looks like a cluster of grapes instead of the normal "kidney bean" shape.  The affected kidney does not function.  MKD almost always affects just 1 of the 2 kidneys and has no familial tendency.  The opposite kidney of the pair has to be evaluated though, because it frequently will have structural abnormalities.
  • Some pediatric patients have a solitary cyst in the kidney.  It does not interfere with kidney function unless it is quite large.

  • Abdominal fullness, due to large kidneys
  • Large urine output, due to inability to make concentrated urine
  • Large lumps in the right and/or left flank, (flank masses) which might be noted at birth.

  • Inherited conditions that run in families
  • Unknown causes

  • Symptoms as noted above
  • Very large kidneys, seen on ultrasound
  • Cysts in the kidneys, seen on ultrasound
  • A special X-ray of the kidneys (called an IVP) may show the cysts.  This X-ray is done with the assistance of dye injected into an arm vein.  The dye then concentrates in the kidneys, outlining the kidney anatomy.  In this disease, on the IVP the kidneys look like the spokes of a wheel.
  • Sometimes this is diagnosed by CAT scan (3-dimensional X-ray) of the abdomen and kidneys, or by biopsy, during surgery.

  • Unknown

  • The polycystic kidneys in PKD cannot be repaired, but physicians treat the complications.
  • The multicystic kidney (MKD) is a non-functioning kidney.  If its bulky mass is not causing any discomfort, surgeons differ in their opinions whether this kidney needs to be removed.  It is more important to repair any critical anatomic defect in the patient's other kidney.
  • The solitary cyst condition: this cyst is usually small and not likely to need treatment.

  • Kidney failure can occur in PKD, leading to dialysis and possible kidney transplant.
  • In kidney failure, the body may have much swelling (edema) and may have too much potassium in the bloodstream.  There is inability to tolerate a lot of protein in the diet.  The patient may need to limit the amount of protein that is eaten, for this reason.
  • Hypertension (high blood pressure) and congestive heart failure (severe fluid retention because of heart failure) can occur in patients with PKD, and are treated with medication and salt restriction.
  • Various forms of abnormal liver anatomy occur in many patients with PKD.  These range from minor to serious.  A very small liver cyst might be an incidental finding, and not cause illness.  A serious defect in liver anatomy could lead to liver failure.
  • Under-developed lungs can occur in PKD, leading to impaired breathing.
  • If kidney failure or impaired breathing occurs in a neonate with PKD or an infant with PKD, this is very serious and may not be correctible.

  • Urgently consult your physician in the case of abdominal mass, flank mass, abdominal fullness, very large or very small urine output, breathing difficulty or body swelling, as all could be signs of cystic kidney.  A blood test, urine test, ultrasound, and X-rays might be ordered.  The blood test (creatinine) shows whether kidney function has started to deteriorate.  The other tests find a range of kidney abnormalities.
  • There is a condition in neonates and infants in which the kidney size is transiently enlarged for unknown reasons, and then returns to normal.  This finding on an ultrasound or X-ray might make a parent worry until the physician interprets it and explains that it might return to normal.

  • PKD is the condition that has the most potential to be serious, especially if symptoms start in infancy.
  • The MKD patient basically has problems with the kidney only if the enlarged kidney is bothersome to the patient, and if the opposite kidney (the one not affected by MKD) has an important anatomic defect that needs surgical repair.
  • The MKD patient will have to be concerned that he has only 1 working kidney.
  • The solitary cyst condition: the kidney with a solitary cyst is likely to be only an incidental finding in a normal child.

more about Cystic Kidney Disease

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