Nephritic & Nephrotic Syndrome Flashcards

1
Q

What are the 3 layers of the Glomerular Filtration Barrier?

A

Endothelial Cell layer - fenestrated
GBM - Type 4 collagen & laminin with embedded mesangial cells (smooth muscle)
Podocytes (Podocin & nephrin proteins)

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2
Q

Define Nephritic vs Nephrotic Syndrome?

A

Nephritic = High haematuria & intravascular overload

Nephrotic = High proteinuria & intravascular depletion

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3
Q

What would Nephrotic syndrome look like?

A

OEdema in face, legs, ascites and pleural effusions

~Raised BP

Frothy Urine

~Pale but mostly well

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4
Q

What causes Nephrotic Syndrome?

A

85% are Minimal Change Disease

15% are termed “Steroid REsistant nephrotic syndrome” which includes:

  • Congenital NPHS1 or 2
  • Acquired FSGS (Focal segmental glomerular sclerosis)
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5
Q

How do we confirm minimal change disease?

A

U&Es + Creatinine = Renal function should be normal
BP = Generally Normal
Urine Dipstix = high Protein & maybe haematuria
FBC: albumin should be low

Trial Prednisalone

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6
Q

Why don’t we biopsy in most nephrotic syndromes?

A

Most it’s almost always minimal change disease so we just trial treatment

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7
Q

Nephrotic Syndrome is treated with 8wks of prednisolone. what are the major SEs?

A
Commonest:
GI due to high acid
Behaviour (irritable/moody/don't sleep)
HTN
Weight Gain

Uncommon:
Glucose intolerance
Adrenal crisis

Growth problems with recurrent courses

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8
Q

Describe the outcome of MCD?

A

95% go into remission in 2-4wks

80% will relapse

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9
Q

How do you treat Steroid Resistant Nephrotic Syndromes e.g. FSGS?

A

Second line immunosuppression

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10
Q

Try to list 8 major causes of childhood haematuria?

A

UTI
Trauma
Stones

Glomerulonephritis due to:

  • Post-infective GN
  • IgA nephropathy / HSP
  • Membranoproliferative GN
  • SLE
  • ANCA +ve vasculitis e.g. GPA
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11
Q

What’s the classic presentation of Nephritic Syndrome?

A
  • Haematuria (frank or microscopic)
  • Proteinuria
    Reduced GFR:
    –> Oligura
    –> Fluid overload (JVP/oedema)
    –> HTN
    –> High creatinine

Often comes post-infection, particularly throat

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12
Q

What’s the most common nephritic syndrome in kids vs adults?

A

Kids - Post-infective Glomerulonephritsi

Adults – IgA Nephropathy

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13
Q

What causes Post-infective Glomerulonephritis?

A

Group A strep
Throat infections 7-10days later
skin 2-4wks later

Nephrogenic strep antigens bind to sites in glomeruli & form immune complexes which are deposited there

This triggers Alternative Complement activation & Adaptive immune response leading to the nephritis

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14
Q

How can you diagnose Post-infective Glomerulonephritis?

A

A bacterial culture & throat swab for Strep A

+ve ASO Titre

Low C3 (used up)

Normal Renal US

ANA (SLE) + ANCA (GPA) to rule out autoimmune

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15
Q

So child with nephritic syndrome presents a week after having a throat infection.
Tests show Strep A culture, +ve ASO titre, low C3
Renal US, ANA &; ANCA were all -ve
How would you treat the kid?

A

Diagnosis of Post-infective GN

Abx
Diuretic for fluid overload
Support renal function (e.g. restrict fluid, restrict Na, ACEI etc)

It will be self-limiting

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16
Q

IgA nephropathy is a commoner cause of nephritic syndrome in older kids/adults. What causes it?

A

Often comes 1-2days post or during an URTI (So much quicker than post-infective GN)

17
Q

How do you diagnose and treat IgA nephropathy?

A

Clinical diagnosis with biopsy to confirm

ACEI
If severe give Immunosuppression

18
Q

What is IgA Vasculitis / HSP?

A

Henoch-Sholein Purpura

It’s very similar to IgA nephropathy but comes with more systemic problems. You need Palpable purpura + 1 of

  • Renal involvement
  • Abdo pain
  • Arthritis
  • Renal biopsy showing IgA deposits

Again it comes 1-3days post viral URTI

19
Q

How do you treat HSP/IgA vasculitits?

A

Symptomatic treatments for joint/gut symptoms e.g. analgesia & NSAIDs

CCS may help gut

Immunosuppression if severe

ACEI for kidneys

Long term monitor for HTN & Proteinuria

20
Q

Which Glomerulonephritidis affect which layer of the Glomerular Filtration BArrier?

A

Podocytes = MCD & SLE

GMB = Membranous & Post-infective (PIGN)

Endothelial cells = PIGN, HUS, Membranoproliferative glomerulonephritis or SLE

mesangial = HSP, IgA nephropathy or SLE