Polycystic Kidney Disease

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General Inspection

• Hypertensive
• Plethoric facies secondary to overproduction of EPO by polycystic kidneys
• May have signs of anaemia if CKD
• Presence of neurological deficits secondary to aneurysm
  • old stroke
  • third nerve palsy secondary to berry aneruysm
• Signs of AV fistula or lines for renal replacement therapy
• Check volume status
• Check for encephalopathy with asterixis
• terry's nails

Abdomen

• fullnes to flanks
• Liver may be enlarged - hepatic cysts occur in 70% of patients
• Nephrectomy scars - may be unilateral or bilateral, often necessary in context of trauma and haemorrhage
• Scars of renal transplant
• spleen may be enlarged with splenic cysts (5% of patients)
• Presence of bilateral renal masses

Other

• Examine for MVP/ AR

Differential diagnosis

1. Autosomal dominant polycystic kidney disease - mostly chromosome 16, sometimes chromosome 4
2. Autosomal recessive polycystic kidney disease - presenting in infancy, producing severe liver and renal disease
3. Von Hippel Lindau syndrome, A/D chromosome 3, haemangioblastomas in cerebellem, retina, spine + cysts in kidney, liver, spleen, risk of renal cell carcinoma and phaemochromocytoma
4. Tuberous sclerosis
5. trisomies 13/ 18, 21
6. Bilateral renal cysts (benign)
7. Bilateral hydronephrosis
8. Amyloidosis
9. Bilateral Renal Cell Carcinoma

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Renal Replacement Therapy and Transplantation

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Establish mode of renal replacement therapy

• ​AV fistula - mature vs immature - check via palpable thrill and auscultated bruit
• tunneled vascath - usually used whilst an AV fistula matures
• presence of peritoneal dialysis - look for evidence of peritoneal dialysis catheter, signs of infection, presence of peritonitis
• renal transplant - scars in right iliac fossa, palpate mass (should be smooth), percuss mass, auscultate for bruit, determine tenderness (may signify infection, rejection etc)
• Previous scars suggestive of past attempts at AV fistula access

Is the renal replacement therapy adequate

• Comment on fluid status (JVP)
• auscultate for pericardial rub
• look for evidence of metabolic uraemic encephalopathy (check orientation, check asterixis)
• excoriation marks
• tachypnoea

Complications of CKD

• parathyroidectomy scars
• anaemia

Complications of immunosupressive therapy

• Prednisolone side effects
• stigmata of infection
• Presence of SCC/ BCC
• evidence of use of cyclosporin - gum hypertrophy, hirsutism, coarse tremor, hypertension, DM

Evidence of aetiology

• ​Diabetes - glucose finger-prick testing, lipohypertrophy from s/c insulin, diabetic retinopathy, neuropathy
• hypertension - hypertensive changes such as silver wiring, AV nicking
• Polycystic kidney disease
• vasculitis - livido reticularis, mononeuritis multiplex, stigmata of rheumatological disease