Paediatric Renal Flashcards

1
Q

What is nephrotic syndrome?

A

Damage to the filtration barrier of the kidneys leading to heavy proteinuria, hypoalbuminaemia and oedema

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2
Q

What age is nephrotic syndrome most common in?

A

Ages 2-5

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3
Q

Describe the causes of nephrotic syndrome

A

Primary causes- occurs due to primary renal glomerular damage
- minimal change disease
- focal segmental glomerulosclerosis
- membranous nephropathy

Secondary causes: glomerular injury due to wider systemic injury (remember DDANI)
- Diabetes
- Drugs
- Amyloidosis
- HIV
- Henoch Schonlein purpura
- SLE

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4
Q

How can nephrotic syndrome be classified

A

Steroid sensitive or steroid resistant

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5
Q

Key features of nephrotic syndrome

A
  • proteinuria >3.5g/day
  • hypoalbuminaemia (<35g/l)
  • Oedema (peripheral or peri orbital)
  • hypercholesterolaemia
    May also have deranged liver profile, high BP and hypercoagulability
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6
Q

Why is there high cholesterol in nephrotic syndrome

A

Because the liver works in overdrive due to the low albumin

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7
Q

Why is there increased risk of infection in nephrotic syndrome?

A

Antibodies are lost through the filtration barrier

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8
Q

Presentation of nephrotic syndrome

A

Periorbital oedema
Frothy urine
Xanthelasma and xanthelomata
Fatigue
Scrotal, vulval, leg and ankle oedema
Ascites
Breathlessness
Infection- e.g peritonitis, septic arthritis

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9
Q

What might trigger nephrotic syndrome

A

A viral illlness

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10
Q

How is nephrotic syndrome diagnosed

A

Urine dipstick- heavy proteinuria
Urine protein: creatinine ratio
Plasma albumin
Gold- renal biopsy

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11
Q

Findings of renal biopsy for minimal change disease

A

Podocyte foot effacement (on electron microscopy)

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12
Q

Findings of renal biopsy for focal segmental glomerulosclerosis

A

Plaques of sclerosis (on light microscopy)

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13
Q

Treatment of nephrotic syndrome

A

High dose oral corticosteroids
If unresponsive then renal biopsy

May need additional drugs if steroid resistant:
- diuretics
- ACE inhibitors
- statins
- anticoagulants

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14
Q

Features that suggest nephrotic syndrome is steroid sensitive

A
  • age 1-10
  • no macroscopic haematuria
  • normal BP
  • normal complement levels
  • normal renal function
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15
Q

Complications of nephrotic syndrome

A

Hypovolaemia
Thrombosis
Infection

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16
Q

What is nephritic syndrome

A

An inflammatory process of the glomeruli (glomerulonephritis) leading to haematuria, variable proteinuria, renal impairment and hypertension

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17
Q

Most common causes of nephritic syndrome in children

A

Post-streptococcal glomerulonephritis and IgA nephropathy

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18
Q

Two main classifications of nephritic syndrome

A

Immune complex deposition in the glomeruli leading to an inflammatory response
Small vessel vasculitis

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19
Q

Causes of nephritic syndrome

A

Post- strep glomerulonephritis
IgA nephropathy
Systemic lupus erythematosus
Small vessel vasculitis (anti-GMB)

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20
Q

Presentation of nephritic syndrome

A

Haematuria (cola coloured urine)
Oliguria
Hypertension
Proteinuria (less than 3.5)
Oedema (peripheral or periorbital)
Shortness of breath (due to fluid overload)

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21
Q

Eplain post-streptococcal glomerulonephritis

A

Occurs 1-3 weeks after strep infection (e.g. tonsilitis with strep pyogenes)
Immune complexes to strep infection, antibodies and complement get stuck in the glomeruli and cause inflammation

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22
Q

How is pos strep glomerulonephritis confirmed

A

Evidence of a recent strep infection (either by culture or raised ASO and anti-DNAase)
Low complement levels

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23
Q

Explain rapidly progressing glomerulonephritis/ good pastures disease

A

It is a rare small vessel vasculitis where anti-GBM autoantibodies target collagen in the basement membranes leading to the deposition if IgG in the glomerular capillaries
Also targets the alveoli so may have haemoptysis

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24
Q

How does Henoch-Schonlein purpura present

A

Purpuric rash - non-blanching, usually begins on the legs and buttocks
Abdominal pain
Arthritis
Glomerulonephritis- haematuria, proteinuria, hypertension
Scrotal pain and swelling

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25
What is henoch schonlein purpura
It is a common autoimmune vasculitis affecting children
26
Epidemiology of henoch schonlein purpura
Most common vasculitis in children Peaks aged 2-10 Increased in winter (often preceded by URTI) Increased in men
27
Diagnostic criteria for HSP
Clinical diagnosis based on having purpura and at least one of the following: - abdominal pain - arthritis - renal involvement - IgA deposition on renal biopsy
28
How is HSP treated
Mainly self-limiting May use analgesia for abdominal and joint pain May use steroids if severe pain
29
At what age can enuresis be diagnosed
In children 5 years or older
30
What is primary nocturnal enuresis
Bed wetting when a child has never achieved continence
31
What is secondary nocturnal enuresis
Loss of continence in a child who has previously been dry for at least 6 months
32
Causes of nocturnal enuresis
Most commonly just a variation in normal development Others: - overactive bladder - fluid intake before bed - failure to wake due to deep sleep and underdeveloped bladder signals - psychological distress
33
Causes of secondary nocturnal enuresis
Constipation UTI Type 1 diabetes Maltreatment
34
diagnosis of nocturnal enuresis
2 week diary of fluid intake, toileting and bed wetting
35
First line treatment for nocturnal enuresis (after lifestyle changes(
Enuresis alarm
36
First line medication for nocturnal enuresis
Desmopressin
37
Other drug treatments for nocturnal enuresis (other than desmopressin)
Oxybutinin and imipramine
38
What is vesiocureteric reflux
A developmental anomaly of the vesicoureteric junction meaning that instead of entering the bladder at an angle the ureters enter directly and have a shortened/absent intramural course
39
Aetiology of vericoureteric reflux (3 causes)
Often familial - 30 to 50% occur in first degree relatives Can occur with bladder pathology (neuropathic bladder, urethral obstruction) Can occur temporarily after a UTI
40
What can occur as a consequence of severe vesicoureteric reflux
1. Intra-renal reflux 2. Pyelonephtritis 3. Renal scarring 4. Chronic kidney disease and hypertension
41
How does vesicoureteric reflux present
Recurrent UTIs
42
When should children be investigated for vesicoureteric reflux?
If they have recurrent UTIs or atypical UTIs
43
What are features suggesting atypical UTIs in children (6 features)
- seriously ill/ septic - poor urine flow - abdominal mass or bladder mass - raised creatinine - failure to respond to suitable Abx in 48 hours - infection with an atypical organism (non E.coli)
44
How can recurrent UTIs be classified in children (3 ways)
Two or more episodes of UTI with upper UTI (pyelonephritis) One episodes or more episodes UTI with upper UTI plus one or more episodes or UTI with lower UTI (cystitis) Three or more episodes of lower UTI
45
Presentation of UTI in infants
Fever Vomiting Lethargy Irritability Jaundice Poor feeding Septicaemia Offensive urine
46
Presentation of UTI in children
Dysuria Abdominal pain Fever and shivering Lethargy Anorexia Diarrhoea Bed wetting Darker, cloudy urine Haematuria
47
First line investigation for a UTI
Dipstick
48
What results on a urine dipstick suggest UTI
Nitrites and leukocyte esterase
49
Investigations for UTI in child
Dipstick Urine sample- microscopy and culture Further investigations may be done for abnormalities
50
How can urine samples been collected in infants
- clean catch (pot waiting when nappy is removed) - plastic adhesive bag applied to Perineum - cotton wool - urethral catheter - suprapubic aspiration (if very ill)
51
Most common causes of UTIs in children
E. Coli Proteus Pseudomonas q
52
Predisposing factors for UTIs in children
Incomplete bladder emptying- infrequent voiding, hurried micturition, obstruction due to constipation, neuropathic bladder Vesicoureteric reflux Poor hygiene
53
Who should have an USS after a UTI (3)
All children under 6 months with first UTI (within 6 weeks) Chidlren with recurrent UTIs (within 6 weeks) Children with atypical UTIs (during the illness )
54
How is vesicoureteric reflux diagnosed?
Micturating cystourethrogram (MCUG) A DMSA scan may also be used to look for scarring
55
Treatment of suspected UTI in child under 3 months
Hospital admission and IV antibiotics
56
Treatment of suspected upper UTI in child >3 months
Start oral antibiotics (initially cefalexin or in sensitive co-amoxiclav)
57
Treatment of children over 3 months with lower UTI
Oral antibiotics (trimethoprim or nitrofuratoin)
58
When is a DMSA scan used after a UTI?
After the acute infection but within 4-6 months Used for all children under 3 with atypical UTI and for those with recurrent UTI
59
What does the eGFR need to be for nitrofuratoin to be used?
Above 45
60
What is haemolytic uraemic syndrome?
A triad of microangiopathic haemolytic anaemia, thrombocytopenia and acute kidney injury
61
What is the most common cause of haemolytic uraemic syndrome
Gastrointestinal infection with shiga toxin secreting E.coli - mainly acquired through contact with farm animals or eating uncooked beef
62
Pathophysiology of haemolytic uraemic syndrome
The toxin from E.coli enters the GI mucosa and localises to the endothelial cells of the kidneys where it causes intravascular thrombogenesis This activates the clotting cascade in which platelets are consumed (thrombocytopenia). There is damage to the RBC as they circulate through the partially occluded microcirculation leading to haemolytic anaemia
63
Presentation of haemolytic uraemic syndrome
- E.coli infection causing bloody diarrhoea - 5 days later: - decreased urine output (AKI) - haematuria - abdominal pain - lethargy - confusion - bruising
64
Diagnosis of haemolytic uraemic syndrome
- FBC: anaemia, thrombocytopenia - Blood film- shows fragmented RBC (schistocytes and helmet cells) - U and E shows AKI (raised creatinine) - Stool culture - E.coli infection, PCR for shiga toxin - LDH increased due to RBC destruction
65
Treatment of haemolytic uraemic syndrome
1st line- supportive fluids (IV isotonic crystalloids) May need: - antihypertensives - blood transfusions - Haemodialysis Plasma exchange may be used in severe cases Eculizumab ( a monoclonal ab) may be used
66
What is hypospadias
A congenital defect where the urethral meats is abnormally located on the penis (usually it will be on the under side of the penis rather than the tip)
67
Three key features of hypospadias
- ventral opening of the urethral meatus - ventral curvature of the penis or chordee - dorsal hooded foreskin
68
How common is hypospadias
1 in 200
69
How is hypospadias treated
May perform surgery however not mandatory
70
What procedure must children with hypospadias not undergo and why?
Circumcision due to preservation of tissue for reconstruction
71
What is phimosis
Tight foreskin which cannot be retracted properly from the head of the penis
72
What is true phimosis
A scar occurs on the foreskin which prevents it from retracting
73
How may phimosis present ?
Ballooning of the foreskin during urination Inability to retract the foreskin Discomfort and pain
74
How is phimosis treated
Topical steroids for 4-6 weeks Circumcision if not successful
75
How long after URTI would post strep glomerulonephritis present?
7-14 days
76
Which cause of nephrotic syndrome presents with podocyte foot effacement on electron microscopy
Minimal change disease
77
How soon after URTI does IgA nephropathy present?
1-2 days
78
Which cause of nephrotic syndrome is associated with malignancy
Membranous nephropathy
79
Most common cause of nephrotic syndrome in those who are HIV positive
Focal segmental glomerulosclerosis
80
What medication may be needed as prophylaxis in nephrotic syndrome
Low molecular weight heparin - enoxaparin
81
What is present in 10% of patients with hypospadias
Cryptorchidism
82
Most common cause of nephrotic syndrome in children
Minimal change disease
83
What is cryptorchidism
Undescended testes
84
What parameter should be monitored frequently in HSP
Blood pressure
85
where should children under 3 months with a UTI be treated
in hospital
86
what follow up test should patients with HSP have for a year
urine dipstick to check for remaining haematuria or proteinuria
87
88
Best method of urine collection in infant
Urine collection pad
89
Which children should have an USS during a UTI ?
- any child with an atypical infection - those under 6 months with recurrent UTIs
90
which children should have a USS within 6 weeks of a UTI?
- those under 6 months whose UTI responded well to antibiotics - over 6 months with recurrent UTIs
91
which children should be referred for a MSUG for UTI?
those under 6 months with recurrent or atypical UTIs
92
Which children should be referred for a DMSA scan following a UTI
those <3 years with atypical UTIs any child with recurrent UTIs
93