Focal segmental glomerulosclerosis

Question 1

Focal segmental glomerulosclerosis?

Answer 1

• Focal segmental glomerulosclerosis is cause of nephrotic syndrome and chronic kidney disease.
• It generally presents in young adults.

Question 2

Aetiology?

Answer 2

• Reflux nephropathy: is kidney damage (nephropathy) due to urine flowing backward (reflux) from the bladder toward the kidneys
• Analgesic nephropathy: injury to the kidneys caused by analgesic medications
• HIV infection
• Heroin usage
• Sickle cell disease
• Morbid obesity
• After renal transplant

Question 3

Investigations?

Answer 3

• Dipstick: Blood/Protein
• 24 hour urine collection
• Inc Creatinine
• Renal USS: normal

Question 4

Management?

Answer 4

• Steroids and cyclosporin
• Relapse common, cyclosporin toxic
• Progressive renal failure
• Eventual transplant required

Question 5

Sclerosis of the glomerulus is seen next to Bowman’s capsule

Answer 5

Sclerosis is seen in the perihilar region of the glomerulus

Question 6

You review a 42-year-old woman 8 months following a renal transplant for focal segmental glomerulosclerosis. She is on a combination of tacrolimus, mycophenolate, and prednisolone. She has now presented with a five day history of feeling generally unwell with jaundice, fatigue and arthralgia. On examination she has jaundice, widespread lymphadenopathy and hepatomegaly. What is the most likely diagnosis?

Answer 6

Post transplant lymphoproliferative disorder is most commonly associated with Epstein-Barr virus. It typically occurs 6 months post transplant and is associated with high dose immunosupressant therapy. Remember cytomegalovirus presents within the first 4 weeks to 6 months post transplant.