Paediatric Nephrology Flashcards

1
Q

Breaking it Down

A

Haematuria/Proteinuria = Glomerular disease
glomerular disease = nephrotic/nephritic syndromes

Acute Kidney injury = Haemolytic uraemic syndrome

Chronic Kidney injury
Developmental anomalies - Reflux nephropathy

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2
Q

the nephron and GFR

A

Nephron receives 25% of CO/minute

GFR - neonate = 20-30ml/min/1.73m^2
by aged 2 this increases to around 90-120

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3
Q

5 kidney Functions - Do not change in adulthood

A

Waste - Urea and creatinine
Ion balance - Na/Cl/K
Acid base control - Bicarb
Endocrine - RAAS/PTH/activation of Vit D

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4
Q

Glomerulonephropathy

A

Blood and Protein in varying volumes will dictate: Clinical presentation and may suggest Dx

However:
damage to one area of GFB affects the other components too

HAEMATURIA = GLOMERULAR INJURY

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5
Q

Acquired glomerulonephroathy

A

M/C damaged = Epithelial cells (podocytes)
minimal Change, FSGN, Lupus

Basement Membrane - Membranous GN, MPGN, PIGN

Endothelial Cell - PIGN, HUS, MPGN, Lupus

Mesangium - IgA, HP, Lupus

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6
Q

Congenital is more rare

A

Podocyte Cytoskeleton

Basement membrane proteins

Endothelial/microvascular integrity

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7
Q

Definition of Nephrotic Syndrome

A

Nephrotic range proteinuria

Hypoalbuminaemia (normal is 36-44g/ - oedema at 25-30)

Oedema ( hypoalbuminaemia –> lack of oncotic pressure and vessels leak)

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8
Q

Typical Presentation of Nephrotic Syndrome

A

2.5 year old

Gastroenteritis 10 days ago
3-4 day hx of swollen face (worse in mornings) one eye closed in morning, swollen legs

No allergies
Great grandmother had a nephrectomy

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9
Q

Presentation of Nephropathy

A
Looks well but a bit pale
Periorbital oedema, 
pitting in legs, 
ascities, 
small pleural effusions
BP Normal or mildly raised
FROTHY URINE
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10
Q

Ix for Nephrotic Syndrome

A

FBC - Albumin Low Creatinine normal
Urine Dip - ++++ protein (24hr urine collection should be done + protein creatinine ratio)
BP
Renal function

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11
Q

Common Causes of Nephrotic Syndrome

A

Minimal Change GN

Typical features - 2-5yrs, Norm BP, Micro haematuria Normal Renal Function

Atypical features - Suggestions of autoimmune, Abnormal Renal Function, Steroid resistance

ONLY CONSIDER Bx IF ATYPICAL

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12
Q

Mx of nephrotic syndrome

A

Typically 8weeks prednisolone

S/E 
Varicella status
Pneumococcal 
Vaccination
CUSHINGS
BEHAVIOUR
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13
Q

Common Causes of Nephrotic Syndrome

A

Minimal Change GN

Typical features - 2-5yrs, Norm BP, Micro haematuria Normal Renal Function

Atypical features - Suggestions of autoimmune, Abnormal Renal Function, Steroid resistance

ONLY CONSIDER Bx IF ATYPICAL

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14
Q

Mx of nephrotic syndrome

A

Typically 8weeks prednisolone

S/E 
Varicella status
Pneumococcal 
Vaccination
CUSHINGS
BEHAVIOUR
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15
Q

Why would nephrotic syndrome be steroid resistant

A

Focal segmental GN - loss of podocytes and progressive inflammation with sclerosis

Congenital presentation also

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16
Q

Microscopic Haematuria Confirmation

A

If you investigate and there is more than tract on 2 or more separate occasions

(Macroscopic is easier as you will see the blood)

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17
Q

Haemaglobinuria

A

Stix positive + Microscopy Negative

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18
Q

Persistent haematuria + proteinuria =

A

Glomerular disease

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19
Q

causes of Microscopic haematuria

A

Glomerulonephritis: Post infective, IgA/HSP, MPGN, Lupus, ANCA positive vasculitis

UTI/Trauma/Stones

Benign recurrent haematuria (family Hx

Alports syndrome
Thin basement membrane

HUS

Nephrotic Syndrome
MCGN
FSGN

Wilm’s tumour
Sickle cell
Exercise
Clotting abnormalities

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20
Q

Causes of Macroscopic haematuria

A

Glomerulonephritis: Post infective, MPGN, Lupus, IgA/HSP

UTI
Trauma
Stones

Benign recurrent (FHx)
Alports

HUS

Wilm’ tumour

Clotting abnormalities

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21
Q

What is nephritic Syndrome (clinical Diagnosis)

A

Describes glomerulonephritis

Haematuria and Proteinuria
Reduce GFR - Oliguria, Fluid overload (raised JVP, oedema), Hypertension, Worsening renal failure= Rapidly progressing GN)

Intrarenal Cause of acute kidney injury (AKI)

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22
Q

Causes of GlomeruloNEPHRITIS

A
PIGN
HSP/IgA
MPGN
 Lupus
ANCA positive vasculitis
23
Q

Acute post infective glomerulonephritis

A

Usually caused by a Group A strep
Beta Haemolytic

Most commonly in the throat or on the skin

24
Q

Dx of PIGN

A

Bacterial Culture (e.g. throat swab)
Positive ASOT - anti-streptolysin O Titre (Ab against strep)
Low C3 that normalises
Bx not required at this moment

Remember possible DDx - IgA/Lupus/MCGN

25
Q

Treamtnet of PIGN

A

Abx
Supportive of renal Function
Treat overload.Hypertension - Diuretics

Condition is mostly self limiting and should not come back

26
Q

IgA nephropathy

A

M/C glomerulonephritis
1-2 days after URTI
usually in older children and adults

27
Q

Dx of IgA

A

Clinical Dx based on…
Recurrent macroscopic haematuria
+/- microscopic
varying degrees of proteinuria

Clinical picture
negative autoimmune workup
normal compliment –> confirm with Biopsy

28
Q

Pathogenesis

A

Circulating IgA1 –> Abs made against IgA1 –> immune complexes develop and circulate –> complexes develop in situ in kidneys –> immune response results in injury

29
Q

Henoch-Schonlein Purpura

A

Age of onset - 5-15 years

30
Q

Dx of HSP

A
MANDATORY - palpable purpura 
\+ one of the 4
1. Abdo pain
2. Renal Involvement 
3. Arthritis or arthralgia
4. Bx for IgA deposition
31
Q

IgA vasculitis

A

Most common child hood vasculitis –> small vessel

IgA nephropathy/IgA vasculitis with nephritis (HSP)

32
Q

What triggers IgA vasculitis

A

1-3 days post Viral Infection (70% URTI)
Streptococcus, Drugs

Duration - 4-6 weeks

Causes mesangial Cell injury

33
Q

Treatment of IgA vasculitis

A

Symptoms - Joints and Gut

Glucocorticoid therapy - Not helpful with renal disease but may help GI situation

Immunosuppression - Trial in Mod to Severe renal dysfunction

Long term hypertension and Proteinuria screening

34
Q

Small Summary so far

A

NEPHRITIC SYNDROME
PIGN/HSP/IgA

increasing haematuria
Intravascular overload
Hypertension

NEPHROTIC SYNDROME
FSGN/minimal Change

Increasing proteinuria
Intravascular depletion

35
Q

Acute kidney injury

A

Abrupt loss of kidney function, resulting in the retention of urea and other nitrogenous waste products and in the dysregulation of extracellular volume and electrolytes

36
Q

Some Feature of AKI

A

Anuria/Oliguria <0.5ml/Kg/hr for 8 hours
Hypertension with fluid oveload
Rapid rise in PLASMA CREATININE (1.5 x age reference)

37
Q

Interpreting AKI severity

A

AKI 1 - Creatinine 1.5- 2 x reference range

AKI 2 - Creatinine 2-3 x reference range

AKI 3 - Creating > 3 x reference range

38
Q

AKI Management

A

The 3 Ms of prevention

Monitor - urine output, PEWS, BP, Weight

Maintain - good hydration

Minimise - Drugs

39
Q

Causes of AKI

A

Intra-renal Problems: Glomerular disease (HUD, GN)

Tubular injury (acute tubular necrosis) - Hypoperfusion/drugs

Interstitial nephritis - NSAIDs, autoimmune

Post renal = Obstructive

40
Q

Haemolytic uraemic syndrome

A

Typically post diarrhoea (enterohaemotthagic E. coli - EHEC) - Verotoxin Producing/ Shigatoxin (VTEC/STEC)

other causes may involve - pneumococcal infection/drugs

41
Q

Presentation of HUS

A

E. Coli 0157:H7 serotype
Period of HUS risk - up to 14 days post diarrhoea

BLOODY DIARRHOEA IS A MEDICAL EMERGENCY IN CHILDREN (blood = Thrombotic)

child will develop Diarrhoea, pain fever and possibly vomiting - blood will come 1-3 days later

42
Q

HUS Triad

A

Microangipathic haemolytic anaemia
Thrombocytopaenia
AKI

Renal Transplant required in 30-40% of cases

43
Q

DDx for HUS

A

ITP

44
Q

3Ms of HUS

A

Monitor - 5 key kidney functions
Be aware of other organs

Maintain - IV normal saline and fluid - renal replacement therapy

Minimise - NO ANTIBIOTICS

45
Q

Long term Consequences of AKI

A

Blood pressure
Proteinuria monitoring
Evolution to CKD

46
Q

Urinary Tract infection

A

Making the Dx

MSSU/Clean Catch? consider catheterisation

Suggestive Tests
Urine Dip - Leucocyte esterase activity - Unreliable <2

Microscopy - Pyuria > 10 wBC per cubic mm

Culture >10^5 colony forming units - E. Coli

47
Q

possible organisms causing UTI in children

A

E. Coli
Klebsiella
Proteus (stones)
Step Faecalus

48
Q

Worry about Scarring -Triad

A

UTI
VUR
Vulnerable Kidney

Scarring is found in the middle of it all

49
Q

VUR = Vesicoureteric reflux - Graded 1-5

A
1 - ureter only 
2 - ureter, pelvis, Calyces
3 - dialation of ureter
4 - Moderate/advancing dilation of ureter (involves other structures/may be torturous)
5 - Gross Dilation/Tortuosity
50
Q

Screening for scarring

A

Imaging of the renal tract

Screening children at risk of scarring - Reflux nephropathy

Capture those with renal dysplasia
Urological abnormalities/unstable bladder - Voiding dysfunction

Guidlines on who to investigate
- Upper tract symptoms
Younger
Recurrent

51
Q

Renal Investigation

A

Ultrasound - Structure

DMSA (isotope scan) - Scarring and function
Good hydration required

MAG 3 scan - Dynamic flow/obstruction
Good hydration
Requires to be very still

52
Q

Treatments of UTI

A

Lower tract - 3 days oral Abx - Trimethoprime, Co-amoxiclav, Caphalosporin

Upper tract/Pyelonephritis- Abx for 7-10 days - oral if systemically well

IV - 3rd generation Cephalosporin or Co-amoxiclav, IV Aminoglycosides effective (good renal excretion)

53
Q

Factors that may affect outcome of CKD

A
Late referral 
HYPERTENSION
PROTEINURIA
high protein intake
poor bone health 
Acidosis
Recurrent UTIs