1. Nephritic Sx & Nephrotic Sx

2. Case report 1 18 yr old man Bilateral loin pain Macroscopic haematuria
Sore throat started one day earlier
BP 140/90; euvolaemic
Creatinine 120 μmol/l
Proteinuria and haematuria on dipstix

3. Case Report 2 20 yr old lady Completely well Haematuria on dipstix
No proteinuria
Normotensive

4. Case Report 3 12 year old boy Impetigo two weeks earlier Headache
Oliguric
Frothy dark coloured urine
Hypertensive

5. Case report 4 15yr old woman
3/12 ankle swelling; face and fingers swollen in the am
BP 130/80; JVP normal; Leg oedema
Creatinine 54 μmol/l
Cr Cl 140 ml/min
Albumin 18 g/l
24 hr u.protein 10 g

6. Case Report 5 30 year old man,diabetic Known hypertensive Ankle oedema
Dipstix: ++++ proteinuria
Creatinine 124 μmol/l (80 – 120)
Albumin 30 g/l (36 – 45)

7. Case Report 6 50 year old obese man Hypertension 10 years
NIDDM 3 years
No retinopathy
Creatinine 124 μmol/l
24 hr urine protein 2 g
HbA1 9.6%

10. Structure of the filtration barrier
Podocyte
Foot processes
Fenestrated
endothelium

11. Minimal change disease

12. Glomerular changes in disease
Proliferation
Sclerosis
Necrosis
Increase in mesangial matrix
Changes to basement membrane
Immune deposits
Diffuse vs focal
Global vs segmental

13. Common Syndromes Nephrotic Syndrome Nephritic Syndrome
Rapidly Progressive GN
Loin Pain Haematuria Syndrome

14. Features of Glomerular Disease
Proteinuria
Haematuria
Renal Failure
Salt and Water Retention
Loin Pain

15. Salt and Water Retention
Hypertension
Oedema
Oliguria

16. Loin Pain
Rare

17. Proteinuria Marker of renal disease
Risk factor for cardiovascular disease
Dyslipidaemia
Hypertension
Something more?
24 hr protein vs urine protein:creatinine ratio

18. Nephrotic syndrome Proteinuria > 40 mg/m2*hr
Hypoalbuminaemia (<2.5mg/dl)
Oedema
Hyperlipidemia
Thromboses
Infection

19. Learning Points Clinical features Commonest types Prognosis Causes
Treatments

20. Nephrotic Syndrome Causes of primary idiopathic NS
Minimal change disease
Mesangial proliferation
Focal segmental glomerulosclerosis

21. Minimal Change Disease
Usually children
Nephrotic syndrome with highly selective proteinuria and generalised oedema
Rarely hypertension or ARF
T cell mediated – VPF
Steroid sensitive usually
Spectrum of disease to FSGS

22. Focal Segmental Glomerulosclerosis
Juxtamedullary glomeruli – may be missed due to sampling error
Older patients
Less sensitive to immunosuppression
Hypertension, haematuria, progressive CRF

23. FSGS:
Familial
VUR
Drug abuse
Obesity

24. Common types of GN Primary Thin membrane disease IgA disease
Minimal Change / FSGS spectrum
Membanous Nephropathy
Secondary
PSGN & Diabetic Glomerulosclerosis

25. Rarer Types
Diffuse endocapillary proliferative GN (post infectious GN)
Crescentic GN
Membanoproliferative / mesangiocapillary GN

26. Nephritic Syndrome
Haematuria
Hypertension
Oliguria
Edema

27. Rapidly progressive GN
Nephritic or nephrotic onset
ESRF in six months

28. General Treatment of GN
Control BP
Angiotensin blockade
Statin
Lose weight
Stop smoking
(pneumococcal prophylaxis)
(anticoagulation)

29. Help!
I need a volunteer!

31. Case report 1 18 yr old man Bilateral loin pain Macroscopic haematuria
Sore throat started one day earlier
BP 140/90; euvolaemic
Creatinine 120 μmol/l
Proteinuria and haematuria on dipstix

32. Case 1: indicative answers
IgA Disease
Renal failure, proteinuria, haematuria, oedema, hypertension, oliguria, loin pain
All except oedema and oliguria

33. Mesangial IgA disease Classical Berger’s Disease
Microscopic haematuria
Proteinuria (rarely nephrotic)
Hypertension
Chronic renal failure
? Failure of hepatic clearance of IgA
Association with GI disease
No specific treatment

34. Ig A Nephropathy
Ig A nephropathy is the most common primary GN worldwide
Usually present with hematuria
Episodes of gross hematuria are precipitated by flu like illness, exercise
Urinary protein excretion usually non-nephrotic
Associated with chronic liver ds, psoriasis, IBD and HIV disease.

35. Ig A Nephropathy
Only 30% of patients with IgA nephropathy has progressive disease.
In progressive disease, use of fish oil may be beneficial.
Immunosuppressive therapy in patients with Ig A nephropathy has not consistently shown to be of benefit

36. Case Report 2 20 yr old lady Completely well Haematuria on dipstix
No proteinuria
Normotensive

37. Case 2: indicative answers
Exclude menstruation!
Thin membrane disease (possibly IgA disease)
Commonest cause of isolated microscopic haematuria in this age group.
At this age, urological cause unlikely; nil to suggest infection / urolithiasis

38. Thin membrane disease Most common GN Microscopic haematuria Familial
Benign
No treatment needed
Most young people with isolated microscopic haematuria have thin membrane disease

39. Case Report 3 12 year old boy Impetigo two weeks earlier Headache
Oliguric
Frothy dark coloured urine
Hypertensive

40. Case 3: indicative answers
Acute nephritic syndrome
Post-streptococcal glomerulonephritis
Diffuse proliferative endocapillary glomerulonephritis
Due to salt and water retention, so salt restriction or loop diuretic

41. Acute Post-Infectious GN
Usually occur in children
Post-streptococcal GN is the most common cause of post infectious GN
Occurs after a streptococcal sore throat or impetigo
Caused by Group A, beta-hemolytic streptococci, particularly nephritogenic strains – Type 1,4,12 (throat) and 2,49(skin)

42. Acute Post-Infectious GN
Acute onset of gross hematuria (COLA COLORED) or microscopic hematuria after latent period of days.
Edema/hypertension
RBC casts on U/A
Elevated creatinine, increased ASO titer
Decreased complement level

43. Acute Post-Infectious GN
LM – Diffuse proliferative and exudative GN
IF – IgG and C3 “lumpy, bumpy”
EM – Sub epithelial “Hump” or “Flame” like deposits

44. Diffuse Endocapillary Proliferative GN (Post Streptococcal GN)
Post infectious; usually Gp A Strep
Acute nephritic syndrome
Uraemia rare
Self-limited; rarely death from BP
Abnormal RUA for up to 2 yrs
Circulating immune complex mediated

45. Acute Post-Infectious GN
Renal biopsy is generally not required.
Treatment is supportive and consist of sodium restriction, control of BP and dialysis if this become necessary.

46. Complications of the Nephritic Syndrome
Hypertensive encephalopathy (seizures, coma)
Heart Failure (pulmonary oedema)
Uraemia requiring dialysis

47. Prognosis in the Nephritic Syndrome
More than 95% of children make a complete recovery
Chronic renal impairment in the longer term is uncommon in children
Bad prognostic features include severe renal impairment at presentation and continuing heavy proteinuria and hypertension
Adults more likely to have long term sequellae than children

48. Case report 4
15 yr old girl
3/12 ankle swelling; face and fingers swollen in the am
BP 130/80; JVP normal; Leg oedema
Creatinine 54 μmol/l
Cr Cl 140 ml/min
Albumin 18 g/l
24 hr u.protein 10 g

49. Case 4: indicative answers
Minimal change – focal segmental glomerulosclerosis spectrum
Very nephrotic
Age and borderline BP make FSGS more likely than MCN
Effect of loss of colloid osmotic pressure gradient across glomerulus causing hyperfiltration

50. Case Report 5 30year old man,diabetic Known hypertensive Ankle oedema
Dipstix: ++++ proteinuria
Creatinine 124 μmol/l (80 – 120)
Albumin 30 g/l (36 – 45)

51. Case 5: indicative answers
Nephrotic syndrome secondary to diabetes / membranous disease
Refer urgently to nephrology

52. Diabetic glomerulosclerosis
Retinopathy
Hypertension
Microalbuminuria
Nephrotic syndrome
Renal failure – usually progressive
Poor prognosis on RRT

53. What we’d like! Demography including tel no and occupation
Reason for referral: presenting complaint, expectations
Co-morbidities, incl other diagnoses, smoking, alcohol and BMI, social care needs
Examination
Medications (incl recently stopped), allergies etc
Treatment and investigations to date
Special requirements (eg interpreter)

54. Case Report 6 50 year old obese man Hypertension 10 years
NIDDM 3 years
No retinopathy
Creatinine 124 μmol/l
24 hr urine protein 2 g
HbA1 9.6%

55. Case 6: indicative answers
Obesity-related FSGS more likely than diabetic nephropathy (duration diabetes, absence of retinopathy)
Worsening nephrotic syndrome and progressive renal failure; Death from cardiovascular cause before reaches ESRF
Stop smoking, lose weight, improve glycaemic control, regular exercise, healthy diet, moderate alcohol in that order

56. Case 6: indicative answers contd
Lack of ownership of responsibility for own health
Withdrawal symptoms (smoking)
Denial of calorie intake
Difficulty exercising due to immobility
No!
Problems with MDRD equation
No evidence of benefit of ACE inhibitors in absence proteinuria
Dangers of ACE inhibitors in patients with angioneurotic oedema, hypotension or bilateral renal artery stenosis

57. Lessons Not all abnormal urinalysis is a UTI
Acute pyelonephritis is very rarely bilateral

58. Haematuria Urologist or Nephrologist?
Age
Other features – proteinuria etc
Urine microscopy for casts
Phase contrast microscopy

59. Non-dysmorphic vs dysmorphic

60. RBC Cast

61. AntiGBM disease RPGN + Lung haemorrhage
Destructive process – medical emergency!
Antibody-mediated
One hit
High dose immunosuppression
Plasma exchange

62. Any Questions?

63. Whoopee! It’s
….Coffee Time