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Autosomal Dominant Polycystic Kidney Disease

Title               

Ñ    Autosomal Dominant Polycystic Kidney Disease

Definition

Autosomal dominant polycystic kidney disease is an:

Ñ    Inherited disorder

Ñ    Usually presents in adult life

Ñ    Manifest by multiple renal cysts

Ñ    Variably associated with extra-renal manifestations.

Aetiology

Ñ    Autosomal dominant inheritance

Pathogenesis

Ñ    Bilateral progressive cystic dilatation of the renal tubules

Ñ    Stretch parenchyma and cause atrophy with resultant renal failure

Clinical Features

History

Ñ    Presents at any age after the 2^nd decade

KUS

Ñ    Abdominal pain

Ñ    Haematuria

Ñ    Uraemia

Examination

CVS

Ñ    Hypertension

Ñ    Mitral valve prolapse

GIT

Ñ    Hepatic cysts

KUS

Ñ    Renal cysts

Ñ    Renal calculi

Ñ    Progressive renal failure

CNS

Ñ    Intracranial aneurysm

Ñ    Subarachnoid haemorrhage

Complications

GIT

Ñ    Pain

Ñ    Infection of cysts

Ñ    Compression:

Bile ducts

Portal vein

Hepatic venous outflow

KUS

Ñ    Haemorrhage into cyst

Ñ    Infection of cysts

Investigations

Fluids

Blood

Biochemistry

Ñ    Evidence of renal dysfunction

Imaging

Ultrasound

Ñ    Identify cysts

CT scan

Ñ    More sensitive

Management 

Control

Drugs

Ñ    Antihypertensives

Surgery

Ñ    Surgical decompression for complications

Support

Renal failure

Ñ    Conservative management

Ñ    Dialysis

Ñ    Transplantation

Prevention

Lifestyle Adjustments

Ñ    Screening of children and genetic counselling

Prognosis

Ñ    Approximately 30 % are alive without renal replacement therapy at 70 years

Ñ    Survival on haemodialysis or following transplantation is the same as for other renal disease

 

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