Nephritic Sx & Nephrotic Sx

Case report 1
18 yr old man Bilateral loin pain

Macroscopic haematuria
Sore throat started one day earlier BP 140/90; euvolaemic Creatinine 120 mol/l Proteinuria and haematuria on dipstix

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

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

Hypertensive

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 mol/l Cr Cl 140 ml/min

Albumin 18 g/l
24 hr u.protein 10 g

Case Report 5
30 year old man,diabetic
Known hypertensive Ankle oedema Dipstix: ++++ proteinuria Creatinine 124 mol/l (80 120)

Albumin 30 g/l (36 45)

Case Report 6
50 year old obese man
Hypertension 10 years NIDDM 3 years No retinopathy Creatinine 124 mol/l

24 hr urine protein 2 g
HbA1 9.6%

Structure of the filtration barrier

Podocyte Foot processes Fenestrated endothelium

10

Minimal change disease

Glomerular changes in disease

Proliferation Sclerosis Necrosis Increase in mesangial matrix Changes to basement membrane Immune deposits Diffuse vs focal Global vs segmental

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

Features of Glomerular Disease

Proteinuria
Haematuria Renal Failure Salt and Water Retention Loin Pain

Salt and Water Retention

Hypertension
Oedema Oliguria

Loin Pain
Rare

Proteinuria
Marker of renal disease
Risk factor for

cardiovascular disease
Dyslipidaemia Hypertension Something more?

24 hr protein vs urine

protein:creatinine ratio

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

Thromboses
Infection

Learning Points
Clinical features
Commonest types Prognosis Causes Treatments

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

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

Focal Segmental Glomerulosclerosis

Juxtamedullary glomeruli

may be missed due to sampling error Older patients Less sensitive to immunosuppression Hypertension, haematuria, progressive CRF

FSGS:
Familial

VUR
Drug abuse Obesity

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

Rarer Types
Diffuse endocapillary proliferative GN

(post infectious GN) Crescentic GN Membanoproliferative / mesangiocapillary GN

Nephritic Syndrome
Haematuria
Hypertension Oliguria Edema

Rapidly progressive GN
Nephritic or nephrotic onset
ESRF in six months

General Treatment of GN
Control BP
Angiotensin blockade Statin Lose weight Stop smoking

(pneumococcal prophylaxis)
(anticoagulation)

Help!

I need a volunteer!

30

Case report 1
18 yr old man Bilateral loin pain

Macroscopic haematuria
Sore throat started one day earlier BP 140/90; euvolaemic Creatinine 120 mol/l Proteinuria and haematuria on dipstix

Case 1: indicative answers

IgA Disease
Renal failure, proteinuria, haematuria,

oedema, hypertension, oliguria, loin pain

All except oedema and oliguria

Mesangial IgA disease

Classical Bergers

Disease Microscopic haematuria Proteinuria (rarely nephrotic) Hypertension Chronic renal failure ? Failure of hepatic clearance of IgA Association with GI disease No specific treatment

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.

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

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

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

Thin membrane disease

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

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

Hypertensive

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

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)

Acute Post-Infectious GN
Acute onset of gross hematuria (COLA

COLORED) or microscopic hematuria after latent period of 10-14 days. Edema/hypertension RBC casts on U/A Elevated creatinine, increased ASO titer Decreased complement level

Acute Post-Infectious GN
LM Diffuse proliferative and exudative

GN IF IgG and C3 lumpy, bumpy

EM Sub epithelial Hump or Flame

like deposits

Diffuse Endocapillary Proliferative GN (Post Streptococcal GN)

Diffuse endocapillary

proliferative 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

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.

Complications of the Nephritic Syndrome

Hypertensive encephalopathy (seizures, coma)
Heart Failure (pulmonary oedema) Uraemia requiring dialysis

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

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 mol/l Cr Cl 140 ml/min

Albumin 18 g/l
24 hr u.protein 10 g

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

Case Report 5
30year old man,diabetic
Known hypertensive Ankle oedema Dipstix: ++++ proteinuria Creatinine 124 mol/l (80 120)

Albumin 30 g/l (36 45)

Case 5: indicative answers

Nephrotic syndrome secondary to diabetes

/ membranous disease Refer urgently to nephrology

Diabetic glomerulosclerosis
Retinopathy
Hypertension Microalbuminuria

Nephrotic syndrome
Renal failure usually progressive

Poor prognosis on RRT

What wed 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)

Case Report 6
50 year old obese man
Hypertension 10 years NIDDM 3 years No retinopathy Creatinine 124 mol/l

24 hr urine protein 2 g
HbA1 9.6%

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

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

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

Haematuria Urologist or Nephrologist?

Age
Other features proteinuria etc

Urine microscopy for casts

Phase contrast

microscopy

Non-dysmorphic vs dysmorphic

RBC Cast

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

Any Questions?

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