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Congenital Cystic Dysplasia of the Kidneys

(Congenital Renal Cystic Dysplasia)

By

Enrica Fung

, MD, MPH, Loma Linda University School of Medicine

Last full review/revision Apr 2021| Content last modified Apr 2021
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Congenital cystic dysplasia of the kidneys is a broad category of birth defects involving the kidneys and/or urinary tract that may cause blockage of the flow of urine.

Congenital cystic dysplasia affects one or both kidneys. Cystic dysplasia of the kidneys may be the only birth defect, or it may be part of a syndrome and occur together with other abnormalities. Abnormalities may affect other parts of the urinary tract, including the ureter, bladder, or rarely the urethra, testes, or abdominal wall muscles. (See figure Viewing the Urinary Tract.)

If enough kidney tissue is damaged, chronic kidney disease can result, sometimes causing symptoms, often with slowed growth. Congenital cystic dysplasia of the kidneys is commonly discovered when routine ultrasonography is done before birth or is done during early childhood for a reason unrelated to the kidneys.

Prognosis is highly unpredictable because it may be difficult for doctors to tell how much kidney tissue is unaffected and functions normally.

Treatment is surgical correction if needed for any associated defects of the urinary tract. If chronic kidney disease progresses to end-stage renal disease (ESRD, or end-stage kidney failure), dialysis or kidney transplantation may be needed.

More Information

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Nephrotic Syndrome
Glomeruli are clusters of tiny blood vessels in the kidneys containing pores that filter blood. In patients with nephrotic syndrome the glomeruli do not function properly and cause excessive protein excretion into the urine. Excessive protein excretion in turn may lead to fluid accumulation, low levels of the protein albumin in the blood, and high levels of blood fats. There are several causes of nephrotic syndrome, including which of the following?
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