Renal exam

Question 1

Complications nephrotic syndrome

Answer 1

Hypovolaemia: protein loss from kidneys

tx: isotonic saline, 20% albumin

Infection: urinary loss of immunoglobulin and complement

• prone to pneumococcal and HiB infections (peritonitis, sepsis)
  tx: prophylactic penicillin for duration of proteinuria
• pneumococcal, influenza vaccination, chickenpox
• chickenpox: treat with aciclovir and ZIG

*steroids may mask infection

Thrombosis

Question 2

Renal mass

Answer 2

genetic: ADPKD, multicystic dysplastic kidney

infiltrative: Wilm’s, NB, storage disorder

inflammatory: cyst, hydronephrosis, hypertrophy secondary to unilateral agensis, renal vein thrombosis

infection: sepsis, abscess

Question 3

ADPCKD

Answer 3

pathophysiology: dilated nephrons which enlarge causing cysts

clinical:

• onset from perinatal period
• asymptomatic renal mass in childhood
• abdominal pain
• haematuria/proteinuria
• HTN
• chronic renal failure
• IC aneurysms

Question 4

ARPCKD

Answer 4

pathophysiology: cysts in the CD of nephrons

clinical:

• neonatal period with bilateral renal masses
• pulmonary hypoplasia
• congenital hepatic fibrosis and cysts
• portal HTN

Question 5

Nephrotic syndrome

Answer 5

clinical:

• oedema
• pallor
• tachypnoea/dyspnoea
• pleural effusion/ascites
• peripheral oedema
• HTN
• poor circulating volume

complications:

• HTN
• acute/chronic renal failure
• infection
• thrombosis
• malnutrition
• hyperlipidaemia

management:

• fluid balance
• steroids: prednisone 60mg/m2 for 4 weeks then 40mg/m2 alternate days for 4 weeks
• cyclophosphamide or cyclosporin if steroid resistant

referral:

• steroid resistance 28 days
• age<12 mo, >12yrs
• persistent haematuria
• HTN
• renal failure

Question 6

Nephrotic syndrome

causes

Answer 6

Differential diagnosis:

• minimal change disease
• focal segmental glomerulosclerosis
• membranoproliferative GN
• membranous nephropathy
• HSP with renal involvement
• SLE
• congenital nephrotic syndrome

Question 7

steroid induced toxicity

Answer 7

causes:

• asthma
• JIA
• CF
• dermatomyositis
• Crohn’s
• post renal/cardiac transplant
• nephrotic syndrome

Question 8

BWS

Answer 8

genetics: 11q15.5 sporadic

major features:

• pre/post natal overgrowth >90th centile
• macroglossia
• abdominal wall defect

minor features:

• ear signs: ear lobe crease, posterior helical pits
• facial naevus flammeus
• hypoglycaemia
• organomegaly
• hemihypertrophy

Question 9

Wilm’s tumour

Answer 9

risk factors:

• BWS
• hemihypertrophy
• aniridia
• DRASH syndrome: ambiguous genitalia, nephropathy, Wilm’s