Polycystic Kidneys CT/MRI head Intracranial aneurysm
Adult PKD: Complications:
Prevalence 1:1000 Renal CRF autosomal dominant; genes on chromosome 16 (PKD1 more severe) or 4 (PKD2) HPT due to increased renin secretion. Thus use ACEI as first-line agents. Polycythemia due to erythropoietin secretion DDx of bilateral renal cysts Cyst infxn, haemorrhage - Multiple simple cysts Haematuria - AR PKD in children proteinuria - Tuberous sclerosis Renal calculi - Von Hipple-Lindau syndrome UTI/ pyelonephritis CVS MVP, MR, AR, TR Presentations CNS Berry aneurysm Hematuria Renal calculi GI Colonic diverticula HPT Renal failure Hernias of anterior abdominal wall UTI SAH a/w berry aneurysm Cysts Liver, spleen, pancreas, testes, epididymis, ovaries, uterus, broad ligament, bladder, Hx lungs, thyroid Symptoms Loin pain Haematuria Mx Abdominal discomfort/pain due to enlarging kidneys Monitor BP & U/E UTI Treat infxns Renal calculi CRF Mx: dialysis, transplant PMHx Stroke (berrys aneurysm) Genetic counseling children and siblings HPT and its complications FMHx PKD Brain aneurysm (ask for death in the family at a young age)
Short Case examination
Peripheral Anaemia 2o to CRF Polycythaemia due to increased erythropoiesis AVF dialysis for CFR Abdominal Hepatomegaly due to cystic dz Palpable kidneys Transplanted kidney Hernias Neurological 3rd nerve palsy & other signs of stroke Request for invxs BP HPT Urine dipstix microscopic haematuria ECG left ventricular hypertrophy