Nephrology and genitourinary

Question 1

Aetiology of acute nephritis

Answer 1

Caused by increased glomerular cellularity, restricting glomerular blood flow and therefore leads to decreased filtration

Post-infectious - eg. streptococcus

Vasculitis:
HSP
SLE
Wegener Granulomatosis
Microscopic Polyarteritis

IgA nephropathy
Goodpasture syndrome (rare..)

Question 2

Clinical features of acute nephritis

Answer 2

Oliguria
Hypertension - may lead to seizure
Oedema - characteristacally around eyes

Haematuria and proteinuria

Question 3

Management of acute nephritis

Answer 3

Monitor water and electrolyte balance.

Administer diuretics if necessary

If there is rapidly progressive glomerulonephritis (rare, but can occur with any acute nephritis, except post-streptococcal) which can cause irreversible renal failrure over weeks or months
-may need renal biopsy and treatment with immunosuppression and plasma exchange

Question 4

Prognosis of acute nephritis

Answer 4

0.5% mortality

5% long-term abnormalities

Question 5

Features of Henoch-Schönlein Purpura

Answer 5

HSP

Rash - purpura -
buttocks, extensor surfaces of legs, arms and ankles
Arthralgia (knees and ankles)
Periarticular oedema (joint swelling - knees and ankles)

Abdo pain - haematemesis and melaena
Glomerulonephritis - microscopic/macroscopic haematuria (80%)

Often there is fever

Question 6

in which age group does HSP occur

Answer 6

3-10yrs

2x as common in boys

Often preceded by an URTI

Question 7

Long term complications of HSP and follow up

Answer 7

With severe proteinuria, there may be nephrotic syndrome.

All children with renal involvement will be followed up for a year to detec those with persisting urinary abnormalities (5-10%).
HTN and declining renal function may develop after an interval of several years

Question 8

Risk factors for progressive renal disease in HSP

Answer 8

Renal biopsy will determine whether treatment is necessary, but:

Heavy proteinuria
Oedema
HTN
Deteriorating renal function

Question 9

Management plan for HSP. Prognosis

Answer 9

Same as acute nephritis.

Long-term follow up for severe renal involvement

<1% of HSP patients will progress to end-stage renal failure, but prognosis is worse in older children

Follow up in 6 months if normal urinalysis

Question 10

What are the commonest causes of acute glomerulonephritis in childhood?

Answer 10

Post-streptococcal/infection nephritis
Familial nephritis
Vasculitis
SLE

Question 11

What is post-streptococcal nephritis

Answer 11

usually follows a streptococcal sore throat or skin infection

Diagnosed by evidence of recent streptococcal infection (culture, raised ASO/anti-DNAse B titres)
Low C3 complement

Good longterm prognosis

Question 12

What is Familial nephritis

Answer 12

Commones is Alport syndrome - X-linked recessive

Progresses to end-stage renal failure by early adult life in males

Nerve deafness
Ocular defects

Mother may have haematuria

Question 13

Characteristic symptoms of vasculitis

Answer 13

(HSP, polyarteritis nodosa, microscopic polyarteritis, Wegener granulomatosis)

Fever
Malaise
Rash
Weight loss
Arthropathy

Renal involvement may be severe and rapidly progressing

Question 14

Treatment of vasculitis

Answer 14

steroids
plasma exchange
IV cyclophosphamide (for months)

Question 15

Epidemiology of SLE

Answer 15

Mainly presents in adolescent girls and young women

Commoner in Asians and Afro-caribbeans

Question 16

What is SLE

Answer 16

Presence of multiple autoantibodies
Antibodies to double-stranded DNA (C3 and C4 may be low)

Haematuria and Proteinuria are indicators for renal biopsy (determines intensity of immunosuppression)

Question 17

Investigations in patients presenting with acute glomerulonephritis

Answer 17

Electrolytes and creatinine (renal function)

FBC - infection, anaemia

Urinalysis (infection, protein, blood)

Urine culture

Complement levels

ASO titre

Anti-DNAse B

Serum IgA measurement

Renal biopsy is the single most effective diagnosis

Renal ultrasonography usually performed to exclude other causes

Question 18

What is enough to diagnose post-streptococcal glomerulonephritis without the need for renal biopsy

Answer 18

Fitting history
Low C3
Postive ASO
Anti-DNAse B

Question 19

What histopathology does IgA nephropathy share with HSP?

Answer 19

Episodes of macroscopic haematuria

commonly associated with URTI

Same histological findings and management

Question 20

Aetiology of nephrotic syndrome

Answer 20

Can be secondary to systemic disease:

HSP
vasculitides
SLE
Infections (malaria)
Allergens (eg. bee sting)

Question 21

Clinical signs of nephrotic syndrome

Answer 21

Periorbital oedema
Particularly on waking
Scrotal or vulcal, leg, ankle oedema
Ascites

Breathlessness - due to pleural effusions and abdominal distension
Cloudy/frothy urine

Question 22

Define nephrotic syndrome

Answer 22

Proteinuria > 200mg protein/mmol creatinine

Serum albumin (hypoalbuminaemia) <2.5g/dl
Oedema that begins in the face
Hyperlipidaemia

Question 23

Name the commonest types of nephrotic syndrome in childhood

Answer 23

Steroid sensitive nephrotic syndrome

Steroid resistant nephrotic syndrome

Congenital nephrotic syndrome

Question 24

Incidence of nephrotic syndrome

Answer 24

1 in 50000 per year

Median age of onset is 2.5 yrs

boys > girls

Question 25

How much of nephrotic syndrome is responsive to steroid therapy?

Answer 25

85-90%
It is the commonest type
These children do NOT progress to renal failure

Question 26

What may precipitate steroid-sensitive nephrotic syndrome

Answer 26

URTI

associated with atopy

Question 27

Features that suggest steroid-sensitive nephrotic syndrome

Answer 27

Age 1-10 years
NO macroscopic haematuria
Normal blood pressure
Normal complement levels
Normal renal function

Question 28

Complications of nephrotic syndrome

Answer 28

Hypovolaemia
Thrombosis (due to urinary loss of antithrombin and raised haematocrit)
Infection (especially pneumococcus)
Hypercholesterolaemia

Question 29

Indicators for hypovolaemia in nephrotic shock

Answer 29

Abdo pain
Feel faint

Peripheral vasoconstriction
Low urinary sodium (due to retention)

High packed cell volume of RBCs

Requires urgent treatment with IV albumin to prevent vascular thrombosis and shock

Question 30

Management of steroid-sensitive nephrotic syndrome

Answer 30

Oral corticosteroids (60mg/m2 per day of prednisolone)

If no atypical features, reduce the dose the dose after 4 weeks.
This should happen over 4 weeks.

Median time for urine free of protein is 11 days

Question 31

Prognosis of steroid sensitive nephrotic syndrome

Answer 31

1/3 resolve directly
1/3 will relapse infrequently
1/3 will relapse to steroid-dependent

Relapse identified by parent on urine testing

Question 32

What are causes of steroid-resistant nephrotic syndrome

Answer 32

Focal segmental glomerulosclerosis (most common)

Mesangiocapillary glomerulonephritis (haematuria and low complement level)

Membranous nephropathy (associated with Hep B)

Question 33

Management of oedema in nephrotic syndrome

Answer 33

Diuretic therapy
Salt restriction
ACE inhibitors
NSAIDs may reduce proteinuria

Question 34

What is congenital nephrotic syndrome

Answer 34

Presents in first 3 months of life

High mortality due to hypoalbuminaemia - unilateral nephrectomy may be needed, followed by dialysis for renal failure (until fit enough for transplant)

Question 35

Initial management of nephrotic syndrome

Answer 35

Fluid management: (difficult because there is fluid overload with possible hypovolaemic shock)
Fluid restriction
Diuretics
Albumin infusions

Treat underlying disease with Prednisolone and other immunosuppressants

Management of complications:
Infections
Thrombosis
Side effects of treatment
Renal failure

Question 36

What are the atypical features of nephrotic syndrome

Answer 36

No response to 4-8 weeks of corticosteroid therapy

Age <12 months or >12 years
HTN
Haematuria
Low C3 complement
Renal failure

Question 37

What should be done in atypical presentation of renal failure

Answer 37

Renal biopsy

-renal histology