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Paediatrics > NEPHRITIS AND NEPHROTIC SYNDROME > Flashcards

NEPHRITIS AND NEPHROTIC SYNDROME Flashcards

1
Q

What are the characteristic features of acute nephritis?

A

Fluid retention (oedema, facial puffiness)

Hypertension

Haematuria

Proteinuria

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2
Q

What is the cause of the majority of acute nephritis in children?

A

Postinfectious - following a throat or skin infection with group A Beta haemolytic streptococci (eg strep pyogenes)

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3
Q

What are the non-streptococcal causes of acute nephritis in children?

A

HSP - Henoch-Schonlein purpura (see haematological cards)

IgA nephropathy

Systemic lupus erythematosus (SLE)

Mesangiocapillary glomerulonephritis

Haemolytic uraemic syndrome

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4
Q

What is the typical history of a child with acute nephritis?

A

Discoloured smoky urine

Facial swelling

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5
Q

What will be seen typically on examination of a child with acute nephritis?

A

High blood pressure

Oedema

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6
Q

What is the other name for IgA nephropathy?

A

Berger’s disease

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7
Q

What would typically be elicited in the history of someone with acute nephritis caused by IgA nephropathy?

A

Preceding respiratory tract infection

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8
Q

What is Henoch-Schonlein purpura (HSP)?

A

An immune mediated systemic vasculitis that affects many parts of the body including skin, joints, gut, and kidney. HSP can follow an upper respiratory tract infection.

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9
Q

Which group of patients is Henoch-Schonlein purpura (HSP) most likely to affect?

A

Boys

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10
Q

How does Henoch-Schonlein purpura (HSP) differ histologically from IgA nephropathy (Berger’s disease)?

A

It doesn’t

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11
Q

What are the skin changes associated with Henoch-Schonlein purpura (HSP)?

A

Purpuric rash seen over the extensor surfaces of the legs, arms and buttocks

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12
Q

What are the intestinal changes associated with Henoch-Schonlein purpura (HSP)?

A

Abdominal pain, vomiting and bleeding

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13
Q

What is the treatment for Henoch-Schonlein purpura (HSP)?

A

Children usually recover without need for treatment

Parents will need reassurance

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14
Q

What is the most common cause of acute renal failure in children?

A

Haemolytic uraemic syndrome

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15
Q

What normally causes haemolytic uraemic syndrome in children?

A

It is caused by Shiga-like toxin produced by E. coli O157:H7 which bind to GB3 receptors in the kidney. Children have more GB3 receptors than adults. The toxin results in red blood cell fragmentation (glomerular microangiopathic haemolytic anaemia) and thrombocytopenia.

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16
Q

Other than shiga-like toxin produced by E. coli, what are the causes of haemolytic uraemic syndrome (a lot of them not really seen in children)?

A

Tumours

Pregnancy

Ciclosporin

The Pill

Systemic lupus erythematosus

HIV

17
Q

What are the classic triad of haemolytic uraemic syndrome?

A

Haemolytic anaemia

Uremia (acute kidney failure)

Thrombocytopenia

18
Q

What are the clinical features of haemolytic uraemic syndrome?

A

History of diarrhoea

Abdominal pain

Confusion, fatigue, oedema

Nausea and vomiting

Haematuria

19
Q

What investigations would you do in a child who presents with a history of bloody diarrhoea, abdominal pain and haematuria suggestive of haemolytic uraemic syndrome? What would you expect to find to confirm your diagnosis?

A

FBC: anaemia, thrombocytopaenia, fragmented blood film

U&E: acute renal failure

Stool culture: E. coli

20
Q

How do we manage children with haemolytic uraemic syndrome?

A

Management is supportive (fluids, blood transfusion, dialysis) and 90% of children recover full renal function

Antibiotics are contraindicated as they induce expression and release of Shiga-like toxin.

The indications for plasma exchange in HUS are complicated. As a general rule plasma exchange is reserved for severe cases of HUS not associated with diarrhoea

21
Q

What investigations would you do for a child that presents with clinical features of acute nephritis?

A

Urine dip - positive for blood and protein

Microscopy of urine - reveals red cells and casts

U&Es

Abdominal USS can exclude other causes of haematuria eg cysts

Throat swab - Group A strep

Anti-DNAase B - positive in Group A strep

Complement C3 levels - decreased in mesangiocapillary GN and post-strep GN

Biopsy if severe - histology

22
Q

How do we manage children with acute nephritis?

A

Control of fluid and electrolyte balance

Diuretics and antihypertensives as required

Treat underlying cause

No evidence that treatment of the preceding infection prevents renal complications.

23
Q

What is the prognosis for postinfectious acute nephritis in children?

A

Usually very good. Rarely, a rapidly progressive glomerulonephritis with renal failure occurs, especially with nephritis from other causes.

24
Q

What is the classic triad of nephrotic syndrome in children?

A

Proteinuria - loss of more than 1 g per day in children

Oedema

Hypoalbuminaemia - serum albumin typically less than 25g/L

25
Q

What are the other features of nephrotic syndrome in children (aside from proteinuria, hypoalbuminaemia and oedema)?

A

Hyperlipidaemia/Hypercholesteraemia - liver respond to loss of protein

Hypercoagulability - loss of antithrombin III

Predisposition to infection

26
Q

What is the most common cause of nephrotic syndrome in children?

A

Minimal change disease (80%)

27
Q

What are the two types of minimal change disease?

A

Steroid sensitive (90%)

Steroid resistant (10%)

28
Q

What is the peak incidence of nephrotic syndrome in children?

A

2 to 5 years old

29
Q

What are the clinical features of nephrotic syndrome in children?

A

Oedema:

  • Facial puffiness
  • Leg and foot swelling
  • Gross scrotal oedema
  • Ascites
  • Pleural effusions
30
Q

What baseline investigations should be carried out in a child that presents with signs and symptoms consistent with nephrotic syndrome?

A

U&Es

Urine microscopy - check for blood

C3 and C4 levels - usually normal

Anti-DNAase B - should be negative

Hepatitis serology - non-minimal change nephrotic syndrome is associated with hep B

31
Q

What features from the history and baseline investigations in a child with nephrotic syndrome should prompt biopsy to rule out rarer causes?

A

Age: less than 1 or more than 12 years old

Macroscopic haematuria

Low C3

Failure to respond to steroid therapy

Hypertension

Renal failure

32
Q

How do we manage a child with features of classic nephrotic syndrome?

A

4 weeks of oral prednisolone 60 mg/m^2/day with a gradually reducing regimen

Fluid balance must be closely monitored with daily weighing and salt restriction

Prophylactic penicillin is given in the acute phase to prevent secondary infection

Pneumococcal vaccination

33
Q

What is the definition of steroid resistant minimal change disease?

A

No remission (ie continued proteinuria) within 4 weeks

34
Q

What are the serious complications of nephrotic syndrome in children?

A

Hypovolaemia

Thrombosis

Secondary infection

Hyperlipidaemia

35
Q

What is the relapse rate of nephrotic syndrome in children?

A

70%

36
Q

What additional immunosuppressants might be given to a child with recurrent nephrotic syndrome?

A

Ciclosporin

Cyclophosphamide

Levamisole

37
Q

What histological patterns are typically associated with a nephritic picture (haematuria/hypertension)?

A

Rapidly progressive glomerulonephritis (aka crescentic glomerulonephritis)

IgA nephropathy

38
Q

What histological patterns are typically associated with a mixed nephritic/nephrotic presentation?

A

Diffuse proliferative glomerulonephritis

Membranoproliferative glomerulonephritis (mesangiocapillary)

39
Q

What histological patterns are typically associated with a nephrotic picture?

A

Minimal change disease

Membranous glomerulonephritis

Focal segmental glomerulosclerosis

Paediatrics (56 decks)

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