Reading Time:
Pathology
Multiple fluid filled cysts develop within kidneys.
Aetiology
Mutation in PKD-1(chromosome 16) or PKD-2 (chromosome 4) lead to a defect in synthesis of polycystin-1 or polycystin-2 .
Signs
May have ballotable large kidneys or an enlarged cystic liver
Symptoms
Haematuria, dysuria, loin pain, polyuria, nocturia, oliguria
Investigations
Bloods: FBC, U&E, Clotting
Imaging: Renal ultrasound – confirms diagnosis
Treatment
Patient Education: Genetic counselling, lifestyle changes
Medical: ACE inhibiters, treatment of CKD complications
Surgical: Transplantation
Prognosis
A significant number never need renal replacement therapy.
Those requiring dialysis generally have a better prognosis than most dialysis patients.
Bleeding into cysts
Infected cysts
Liver and pancreatic cysts
Hypertension
Intra cerebral aneurysms leading to subarachnoid haemorrhage
Cardiac valve disorders: Mitral valve prolapse and aortic regurgitation Diverticular disease
Key Facts
Lorem ipsum dolor sit amet, sapien platea morbi dolor lacus nunc, nunc ullamcorper. Felis aliquet egestas vitae, nibh ante quis quis dolor sed mauris.
Key Images
Lorem ipsum dolor sit amet, sapien platea morbi dolor lacus nunc, nunc ullamcorper. Felis aliquet egestas vitae, nibh ante quis quis dolor sed mauris.
Key References
Lorem ipsum dolor sit amet, sapien platea morbi dolor lacus nunc, nunc ullamcorper. Felis aliquet egestas vitae, nibh ante quis quis dolor sed mauris.