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Autosomal Dominant Polycystic Kidney Disease P012

Last amended 
1 April 2021
Current RMA Instruments
Reasonable Hypothesis SOP
39 of 2015
Balance of Probabilities SOP
40 of 2015
Changes from previous Instruments

SOP Bulletin 179

ICD Coding
  • ICD-9-CM Codes: 753.13
  • ICD-10-AM Codes: Q61.2
Brief description

This is a genetic disorder of the kidney resulting in the development of multiple bilateral renal cysts, enlarged kidneys and renal damage.  

Confirming the diagnosis

This diagnosis is based on imaging (MRI scan, CT scan or ultrasound of the kidneys), renal function testing, and genetic testing.

The relevant medical specialist is a nephrologist.

Additional diagnoses covered by SOP

Conditions not covered by SOP
  • acquired cystic disease of kidney
  • autosomal recessive polycystic kidney disease
  • medullary cystic kidney disease
  • medullary sponge kidney disease

(all non-SOP)

Clinical onset

The genetic abnormality is present from birth.  The condition is typically asymptomatic early in life, with clinical manifestations typically appearing around the ages of 30 to 40 years.  

Clinical worsening

The only SOP worsening factor is for inability to obtain appropriate clinical management.  Natural progression of the disease varies with the phenotype.  Worsening beyond the normal progression of the disease needs to be evident before a clinical worsening factor can be considered.

Treatment is aimed at slowing the rate of progression of kidney function loss and limiting complications  Measures include strict blood pressure control, dietary protein restriction, a low-salt diet, and statins.

In most patients, renal function remains intact until the fourth decade of life but once the glomerular filtration rate (GFR) starts to decline, the average reduction is 4.4 to 5.9 mL/min per year.  Most patients with ADPKD die from cardiac causes or kidney failure.