Aims and objectives Why is renal so difficult? Demystifying - - PowerPoint PPT Presentation

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Aims and objectives Why is renal so difficult? Demystifying - - PowerPoint PPT Presentation

Sep 23, 2023 438 likes •923 views

Aims and objectives Why is renal so difficult? Demystifying nephrotic and nephritic syndrome Cover general principles with some examples Duration: 70 mins Slides and recordings: www.bitemedicine.com/watch 2 Case-based

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Aims and objectives

  • Why is renal so difficult?
  • Demystifying nephrotic and nephritic syndrome
  • Cover general principles with some examples
  • Duration: 70 mins
  • Slides and recordings: www.bitemedicine.com/watch
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History and examination

A 7-year-old presents to the GP with facial swelling which has got progressively worse. Today, his mum noted his ankles were swollen. He has no past medical history. Urine dip Leucocytes -ve, Protein ++++, Blood -ve, Nitrites -ve

Observations

HR 90, BP 108/72, RR 18, SpO2 95%, Temp 37.0

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Case-based discussion: 1

(1)

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A 7-year-old presents to the GP with facial swelling which has got progressively worse. Today, his mum noted his ankles were swollen. He has no past medical history. Which of the following is consistent with nephrotic syndrome?

Case history

Urinary protein 3.0g in 24 hours Macroscopic haematuria Hypocholesterolaemia Hypercoagulability Albumin > 30g/L app.bitemedicine.com

Q3 Q4 Q5

Observations HR 90, BP 108/72, RR 18, SpO2 95%, Temp 37.0 Urine dip Leucocytes -ve, Protein ++++, Blood -ve, Nitrites –ve

Q1 Q2

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History and examination

A 7-year-old presents to the GP with facial swelling which has got progressively worse. Today, his mum noted his ankles were swollen. Urine dip: Leucocytes -ve, Protein ++++, Blood -ve, Nitrites -ve

Observations

HR 90, BP 108/72, RR 18, SpO2 95%, Temp 37.0

5

Case-based discussion: 1

(1)

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Introduction

Renal failure

Pre-renal Renal Post renal

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Introduction

  • 1. Glomerular
  • 3. Tubular
  • 2. Interstitial
  • 4. Kidney vascular supply

Renal failure

Pre-renal Renal Post renal

(2)

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Introduction

  • 1. Glomerular
  • 3. Tubular
  • 2. Interstitial
  • 4. Kidney vascular supply

Renal failure

Pre-renal Renal Post renal

(2)

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Introduction

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Introduction

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Introduction

Nephrotic Nephritic

Features Proteinuria > haematuria

  • >3.5g per 24hr

Hypoalbuminaemia Oedema Hypercholesterolaemia Hypercoagulable state Increased risk of infection Haematuria > proteinuria Salt retention HTN Oedema Oliguria

Definition: nephrotic and nephritic syndrome are a constellation of symptoms caused by

glomerular damage

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Pathophysiology

Bowman’s capsule

1. 3. 2. Protein and blood

(3)

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Pathophysiology

Bowman’s capsule Glomerular capillaries

(4)

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Pathophysiology: Nephrotic syndrome

Nephrotic

Proteinuria > haematuria Hypoalbuminaemia Oedema Hypercholesterolaemia Hypercoagulable state Increased risk of infection

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Pathophysiology: Nephritic syndrome

Nephritic

Haematuria > proteinuria Salt retention HTN Oedema Oliguria

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Clinical features

Nephrotic Nephritic

Oedema Oedema Frothy urine Haematuria – often macroscopic Weight gain Weight gain +/- HTN HTN

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Clinical features

(5) (6)

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Which of the following is the most likely diagnosis?

Case history

Focal segmental glomerulosclerosis Post-streptococcal glomerulonephritis IgA nephropathy Minimal change disease Membranous nephropathy app.bitemedicine.com

Q3 Q4 Q5

A 7-year-old presents to the GP with facial swelling which has got progressively worse. Today, his mum noted his ankles were swollen. He has no past medical history. Observations HR 90, BP 108/72, RR 18, SpO2 95%, Temp 37.0 Urine dip Leucocytes -ve, Protein ++++, Blood -ve, Nitrites –ve

Q2 Q1

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Aetiology

Nephrotic Nephritic

Minimal change disease IgA nephropathy Focal segmental glomerulosclerosis Post-streptococcal glomerulonephritis Membranous nephropathy Rapidly progressing glomerulonephritis Amyloidosis Alport syndrome Diabetic nephropathy Membranoproliferative glomerulonephritis Diffuse proliferative glomerulonephritis

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Q2

Which of the following would you see on light microscopy in this patient?

Case history

Normal glomerulus Hypercellularity Fusion of podocytes Thickened basement membrane Segmental glomerulosclerosis app.bitemedicine.com

Q3 Q1 Q4 Q5

A 7-year-old presents to the GP with facial swelling which has got progressively worse. Today, his mum noted his ankles were swollen. He has no past medical history. Observations HR 90, BP 108/72, RR 18, SpO2 95%, Temp 37.0 Urine dip Leucocytes -ve, Protein ++++, Blood -ve, Nitrites –ve

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Nephrotic syndrome

Nephrotic syndrome Minimal change disease Focal segmental glomerulosclerosis Membranous nephropathy Membranoproliferative glomerulonephritis Amyloidosis Diabetic nephropathy

Risk factors and comments Most common cause in children

  • Usually Idiopathic

No change on light microscopy Electron microscopy

  • Fusion of podocytes
  • Effacement of foot processes

Responds well to steroids

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Nephrotic syndrome

Nephrotic syndrome Minimal change disease Focal segmental glomerulosclerosis Membranous nephropathy Membranoproliferative glomerulonephritis Amyloidosis Diabetic nephropathy

(4)

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Nephrotic syndrome

Nephrotic syndrome Minimal change disease Focal segmental glomerulosclerosis Membranous nephropathy Membranoproliferative glomerulonephritis Amyloidosis Diabetic nephropathy

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Nephrotic syndrome

Nephrotic syndrome Minimal change disease Focal segmental glomerulosclerosis Membranous nephropathy Membranoproliferative glomerulonephritis Amyloidosis Diabetic nephropathy

Risk factors and comments Most common cause in adults

  • HIV
  • Sickle cell anaemia
  • Heroin
  • SLE

Light microscopy

  • Segmental sclerosis
  • <50% of glomeruli affected

Variable response to steroids

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Nephrotic syndrome

Nephrotic syndrome Minimal change disease Focal segmental glomerulosclerosis Membranous nephropathy Membranoproliferative glomerulonephritis Amyloidosis Diabetic nephropathy

(7)

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Nephrotic syndrome

Nephrotic syndrome Minimal change disease Focal segmental glomerulosclerosis Membranous nephropathy Membranoproliferative glomerulonephritis Amyloidosis Diabetic nephropathy

Risk factors and comments Most common cause in caucasian adults

  • Malignancy
  • Hepatitis B and C
  • NSAIDs
  • SLE

Light microscopy

  • Thickened basement membrane

Electron microscopy

  • Subepithelial immune complex deposition
  • Spike and dome pattern

Variable response to steroids

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Nephrotic syndrome

Nephrotic syndrome Minimal change disease Focal segmental glomerulosclerosis Membranous nephropathy Membranoproliferative glomerulonephritis Amyloidosis Diabetic nephropathy

(8)

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Nephrotic syndrome

Nephrotic syndrome Minimal change disease Focal segmental glomerulosclerosis Membranous nephropathy Membranoproliferative glomerulonephritis Amyloidosis Diabetic nephropathy

(9)

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History and examination

A 20-year-old male presents to the GP complaining

  • f red urine. He is recovering from a sore throat

which started 2 days ago. Examination reveals erythematous tonsils. He has no past medical history. Urine dip Leucocytes -ve, Protein +, Blood ++++, Nitrites -ve

Observations

HR 90, BP 128/85, RR 18, SpO2 95%, Temp 37.0

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Case-based discussion: 2

(10)

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Which of the following suggests nephritic syndrome?

Case history

Urinary protein 3.7g in 24 hours Red cell casts Lipid casts White cell casts Brown granular casts app.bitemedicine.com

Q2 Q4 Q3 Q1 Q5

A 20-year-old male presents to the GP complaining of red urine. He is recovering from a sore throat which started 2 days ago. Examination reveals erythematous tonsils. He has no past medical history. Observations HR 90, BP 128/85, RR 18, SpO2 95%, Temp 37.0 Urine dip Leucocytes -ve, Protein +, Blood ++++, Nitrites -ve

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Which of the following is the most likely diagnosis?

Case history

Rapidly progressive glomerulonephritis Post-streptococcal glomerulonephritis IgA nephropathy

Buerger's disease

Alport syndrome app.bitemedicine.com A 20-year-old male presents to the GP complaining of red urine. He is recovering from a sore throat which started 2 days ago. Examination reveals erythematous tonsils. He has no past medical history. Observations HR 90, BP 128/85, RR 18, SpO2 95%, Temp 37.0 Urine dip Leucocytes -ve, Protein +, Blood ++++, Nitrites -ve

Q2 Q1 Q3 Q4 Q5

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Nephritic syndrome

Nephritic syndrome IgA nephropathy Post-streptococcal glomerulonephritis Rapidly progressive glomerulonephritis Alport syndrome

Risk factors and comments Most common glomerulonephritis worldwide Macroscopic haematuria 1-2 days post viral infection Immunofluorescence microscopy:

  • IgA complex deposition

30% progress to ESRF Steroid not particularly effective

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Nephritic syndrome

Nephritic syndrome IgA nephropathy Post-streptococcal glomerulonephritis Rapidly progressive glomerulonephritis Alport’s syndrome

(11)

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Nephritic syndrome

Nephritic syndrome IgA nephropathy Post-streptococcal glomerulonephritis Rapidly progressive glomerulonephritis Alport’s syndrome

Risk factors and comments Occurs after Group A strep infection Macroscopic haematuria 2 weeks post infection Light microscopy:

  • Hypercellular glomeruli

Electron microscopy

  • Subepithelial immune complex deposits

Self-limiting but may progress to RPGN

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Nephritic syndrome

Nephritic syndrome IgA nephropathy Post-streptococcal glomerulonephritis Rapidly progressive glomerulonephritis Alport’s syndrome

(12)

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Nephritic syndrome

Nephritic syndrome IgA nephropathy Post-streptococcal glomerulonephritis Rapidly progressive glomerulonephritis Alport’s syndrome

Risk factors and comments Multiple causes:

  • Post-streptococcal glomerulonephritis
  • Goodpasture syndrome
  • Granulomatosis with polyangiitis
  • Churg-Strauss syndrome
  • Microscopic polyangiitis

Light microscopy

  • Inflammatory crescents in Bowman’s space

Immunofluorescence microscopy:

  • Can help determine aetiology

Progresses to renal failure in weeks to months

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Nephritic syndrome

Nephritic syndrome IgA nephropathy Rapidly progressive glomerulonephritis Alport’s syndrome Nephritic syndrome IgA nephropathy Post-streptococcal glomerulonephritis Rapidly progressive glomerulonephritis Alport’s syndrome

(13)

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Investigations: General principles

Bedside

  • Urinalysis:
  • Proteinuria vs haematuria
  • Casts: lipid (nephrotic) vs red cell (nephritic)
  • 24-hour urinary protein collection: >3.5g/24hr is suggestive of nephrotic syndrome
  • Urine albumin:creatinine ratio raised due to proteinuria

Bloods

  • U&Es: monitor eGFR and creatinine to assess for renal failure
  • LFTs: hypoalbuminemia < 25-30 g/L in nephrotic syndrome
  • Lipid profile: hypercholesterolaemia and hypertriglyceridemia
  • Complement: may be depleted depending on cause e.g. PSGN

Special tests

  • Renal biopsy: light and electron microscopy to assess glomerular damage
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Management: General principles

Lifestyle

  • Improve cardiovascular risk factors

Immunosuppression

  • Steroids
  • DMARDs
  • Ciclosporin
  • Cyclophosphamide

Adjunctive treatment

  • ACEi: reduces proteinuria
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Complications & Prognosis

Complication

  • Renal failure
  • CV risk
  • Infection
  • Thrombosis

Prognosis:

  • MCD: good prognosis and responds to steroids
  • FSGS: variable
  • IgA: 25-30% progress to end-stage renal failure
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Top-decile question

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Recap

  • Glomerular disease leads to nephrotic and nephritic syndrome
  • Nephrotic: protein > blood
  • Nephritic: blood > protein
  • Many causes of which the commonest are MCD, FSGS, and IgA

nephropathy

  • Investigate with urinalysis, bloods, and biopsy
  • Management may require immunosuppression

(1) (10)

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References

1. Polarlys / CC BY-SA (http://creativecommons.org/licenses/by-sa/3.0/) 2.

  • Modified. CKRobinson / CC BY-SA (https://creativecommons.org/licenses/by-sa/4.0)

3. Pharmattila / CC BY-SA (https://creativecommons.org/licenses/by-sa/4.0) 4.

  • Modified. Ed Uthman (Pathologist), website: [2] / CC BY-SA (https://creativecommons.org/licenses/by-sa/2.0)

5. Nephrotic syndrome / CC BY-SA (https://creativecommons.org/licenses/by-sa/3.0) 6. James Heilman, MD / CC BY-SA (https://creativecommons.org/licenses/by-sa/3.0) 7. Nephron / CC BY-SA (https://creativecommons.org/licenses/by-sa/3.0) 8. Nephron / CC BY-SA (https://creativecommons.org/licenses/by-sa/3.0) 9. Renal_corpuscle.svg: M•Komorniczak -talk- (polish Wikipedist)derivative work: Huckfinne / CC BY-SA (https://creativecommons.org/licenses/by-sa/3.0) 10. James Heilman, MD, Attribution-Share Alike 4.0 International license. 11. Lazarus Karamadoukis, Linmarie Ludeman and Anthony J Williams / CC BY (https://creativecommons.org/licenses/by/2.0) 12. Nephron / CC BY-SA (https://creativecommons.org/licenses/by-sa/3.0) 13.

  • Modified. Nephron / CC BY-SA (https://creativecommons.org/licenses/by-sa/3.0)

Pomegranate image: Unsplash

All other images were made by BiteMedicine or under basic license from Shutterstock and not suitable for redistribution.

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