Paeds: Proteinuria

Question 1

Transient versus persistant proteinuria

Answer 1

May occur during febrile illnesses or after exercise and does not require investigation.
Does nto exceed 0.15g/24hr

Question 2

If persistent next step?

Answer 2

Should be quantified by measuring the urine protein/creatinine ratio in an early morning sample (protein should not exceed 20mg/mmol of creatinine)

Question 3

Causes – non-pathological proteinuria

Answer 3

Transient
Fever
Exercise
UTI
Orthostatic proteinuria

Question 4

Causes: pathological

Answer 4

Glomerular abnormalities
-	Minimal change disease
-	Glomerulonephritis
-	Abnormal glomerular BM (familial nephritides)
Increased glomerular filtration pressure
Reduced renal mass
Hypertension
Tubular proteinuria – tubular interstitial nephritis
CKD

Question 5

Orthostatic proteinuria

Answer 5

Protein only found when child is upright e.g. during the day
Diagnosed: measuring the urine protein/creatinine ratio in a series of early morning urine specimens
Prognosis: excellent and further investigations are not necessary.

Question 6

Nephrotic Syndrome:

Description

Answer 6

Heavy proteinuria results in a low plasma albumin and oedema.

Question 7

Nephrotic Syndrome:

Cause

Answer 7

Unknown, but a few cases are a secondary to systemic diseases such as henoch-Schonlein purpura and other vasculitides e.g. SLE, infections (e.g. malaria) or allergens (e.g. bee sting)

Question 8

Nephrotic Syndrome:

Clinical signs of the nephrotic syndrome are:

Answer 8

• Periorbital oedema (particularly on waking), the earliest sign
• Scrotal or vulval, leg and ankle oedema
• Ascites
  Breathlessness due to pleural effusions and abdominal distension

Question 9

Nephrotic Syndrome:

Initial investigations

Answer 9

• Urine protein – dipstick
• FBC and ESR
• U+E, creatinine and albumin
• Complement levels – C3, C4
• Antistreptolysin O or anti-DNase B titres and throat swab
• Urine microscopy and culture
• Urinary sodium concentration
• Hepatitis B and C screen
  Malaria screen if travel abroad

Question 10

Nephrotic Syndrome: Diagnosis

Answer 10

Heavy proteinuria and low plasma albumin

Question 11

Nephrotic Syndrome: Types

Answer 11

• Steroid-nephrotic syndrome

Steroid-resistant syndrome

Question 12

Steroid-sensitive nephrotic syndrome Description

Answer 12

In 85-90% of children with nephrotic syndrome, the proteinuria resolves with corticosteroid therapy (steroid-sensitive nephrotic syndrome). These children do not progress to renal failurev

Question 13

Epi

Steroid-sensitive nephrotic syndrome

Answer 13

Commoner in boys vs. girls
Asian children than in Caucasians
Weak association with atopy

Question 14

Precipitated sometime

Steroid-sensitive nephrotic syndrome

Answer 14

Resp infections

Question 15

Features suggesting steroid-sensitive nephrotic syndrome

Steroid-sensitive nephrotic syndrome

Answer 15

• Age between 1 and 10 years
• No macroscopic haematuria
• Normal blood pressure
• Normal complement levels
  Normal renal function

Question 16

Management

Steroid-sensitive nephrotic syndrome

Answer 16

• Oral corticosteroids (60mg/m2 per day of prednisolone), unless atypical features.
  After 4 weeks dose reduced to 40mg/m2 on alternate days for 4 weeks and then stopped

Question 17

Median time for urine to be free of protein

Steroid-sensitive nephrotic syndrome

Answer 17

11 days

Question 18

New evidence shows

Steroid-sensitive nephrotic syndrome

Answer 18

Extending the initial course of steroids, by gradually tapering the alternate day part of the course, leads to a marked reduction in the proportion of children who develop relapsing or steroid-dependeant course.

Question 19

Renal histology in steroid-

Steroid-sensitive nephrotic syndrome

Answer 19

Normal on light microscopy but fusion of the specialised epithelial cells that invest the glomerular capillaries (podocytes) is seen on electron microscopy—MINIMAL CHANGE DISEASE

Question 20

With nephrotic syndrome is susceptible to several serious complications at presentation or relapse:

Steroid-sensitive nephrotic syndrome

Answer 20

• Hypovolaemia
• Thrombosis
• Infection
  Hypercholesterolaemia

Question 21

Prognosis

Steroid-sensitive nephrotic syndrome

Answer 21

• Relapses are identified by parents on urine testing
• Side-effects of corticosteroid therapy may be reduced by an alternate-day regimen
• Potentially steroid sparing agents
  1/3 resolves directly, 1/3 infreqeunt relapses, 1/3 freqeunt relapses – steroid-dependant

Question 22

Steroid sparing agents

Steroid-sensitive nephrotic syndrome

Answer 22

immunomodulator levamisole, alkylating agents (e,g, cyclophosphamide), calcineurin inhibitors such as tacrolimus and ciclosporin A and the ummunosuppressant mycophenolate mofetil

Question 23

Hypovolaemia

Steroid-sensitive nephrotic syndrome

Answer 23

• Initial phase of oedema formation the intravascular compartment may become volume depleted

Question 24

Hypovolaemia symptoms

Steroid-sensitive nephrotic syndrome

Answer 24

Abdominal pain and may feel faint

Question 25

Indications of hypovolaemia Steroid-sensitive nephrotic syndrome

Answer 25

Low urinary sodium (

Question 26

Management if indications present Steroid-sensitive nephrotic syndrome

Answer 26

Urgent reatment with intravenous albumin as the child is at risk of vascular thrombosis and shock.

Question 27

Thrombosis: Processes Steroid-sensitive nephrotic syndrome

Answer 27

Urinary losses of antithrombin, thrombocytosis which may be exacerbated by steroid therapy, increased synthesis of clotting factors and increased blood viscosity from the raised haematocrit, predisposes to thrombosis.

Question 28

Steroid-sensitive nephrotic syndrome: thombosis complications

Answer 28

May affect the brain, limbs and splanchnic circulation

Question 29

Steroid-sensitive nephrotic syndrome: Hypercholesterolemia: | description

Answer 29

Correlates inversely with the serum albumin, but the cause of the hyperlipidaemia is not fully understood.

Question 30

Steroid-resistant nephrotic syndrome: Management of the oedema is by

Answer 30

- Referral to a paediatric nephrologist. | - - diuretic therapy, salt restriction, ACE inhibitors and sometimes NSAIDS, which may reduce proteinuria

Question 31

Steroid-resistant nephrotic syndrome: | Cause

Answer 31

Focal segmental glomerulosclerosis Mesangiocapillary glomerulonephritis (membranoproliferative glomerulonephritis Membranous nephropathy

Question 32

Focal segmental glomerulosclerosis Specific features Prognosis

Answer 32

Most common 30% progress to end-stage renal failure in 5 years Respond to cyclophosphamide, ciclosporin, tacrolimus or rituximab Recurrence post-transplant is common

Question 33

Mesangiocapillary glomerulonephritis (membranoproliferative glomerulonephritis Specific features Prognosis

Answer 33

- More common in older children Haematuria and low complement level present - Decline in renal function over many years

Question 34

Membranous nephropathy Specific features Prognosis

Answer 34

Associated with hepatitis B May precede SLE Most remit spontaneously within 5 years

Question 35

Congenital Nephrotic syndrome: Presence

Answer 35

- First 3 months of life. | It is rare

Question 36

Congenital Nephrotic syndrome: Aetiology

Answer 36

- Commonest kind is recessively inherited and the gene frequency is particularly high in Finns UK more common in consanguineous families.

Question 37

Congenital Nephrotic syndrome: Associated with

Answer 37

High mortality due to complication of hypoalbuminaemia rather than renal failure

Question 38

Congenital Nephrotic syndrome: | Outcome

Answer 38

Hypoalbuminaemia may be so severe that a unilateral nephrectomy may be necessary for its control, followed by dialysis for renal failure, which is continued until the child is large and fit enough for renal transplantation.