Cystic kidney disease

Cystic kidney disease
Classification and external resources
Specialty	medical genetics
ICD-10	Q61
ICD-9-CM	753.1
eMedicine	med/3189
MeSH	D052177
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Cystic kidney disease refers to a wide range of hereditary, developmental, and acquired conditions. With the inclusion of neoplasms with cystic changes, over 40 classifications and subtypes have been identified. Depending on the disease classification, the presentation of disease may be from birth, or much later into adult life. Cystic disease may involve one or both kidneys and may or may not occur in the presence of other anomalies. A higher incidence of cystic kidney disease is found in the male population and prevalence increases with age. Renal cysts have been reported in more than 50% of patients over the age of 50. Typically, cysts grow up to 2.88 mm annually and cause related pain and/or hemorrhage.

Of the cystic kidney diseases, the most common is Polycystic kidney disease; having two prevalent sub-types: autosomal recessive and autosomal dominant polycystic kidney disease.Autosomal Recessive Polycystic Kidney Disease (ARPKD) is primarily diagnosed in infants and young children. Autosomal dominant polycystic kidney disease (ADPKD) is most often diagnosed in adulthood.

Another example of cystic kidney disease is Medullary sponge kidney.

Many forms of cystic kidney disease can be detected in children prior to birth. Abnormalities which only affect one kidney are unlikely to cause a problem with the healthy arrival of a baby. Abnormalities which affect both kidneys can have an effect on the baby's amniotic fluid volume which can in turn lead to problems with lung development. Some forms of obstruction can be very hard to differentiate from cystic renal disease on early scans.

Multicystic dysplastic kidney - affects only one side; the other kidney is usually healthy.

Cystic dysplasia - can affect one or both kidneys

Autosomal recessive polycystic kidney disease

Autosomal dominant polycystic kidney disease

Posterior urethral valves - easily mistaken for cystic renal disease on early scans; appearances are due to obstruction not cysts

High grade vesico-ureteric reflux - easily mistaken for cystic renal disease on early scans

Pelvi-ureteric junction obstruction - easily mistaken for cystic renal disease on early scans

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