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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
Treamtnet of PIGN
Abx Supportive of renal Function Treat overload.Hypertension - Diuretics Condition is mostly self limiting and should not come back
26
IgA nephropathy
M/C glomerulonephritis 1-2 days after URTI usually in older children and adults
27
Dx of IgA
Clinical Dx based on... Recurrent macroscopic haematuria +/- microscopic varying degrees of proteinuria Clinical picture negative autoimmune workup normal compliment --> confirm with Biopsy
28
Pathogenesis
Circulating IgA1 --> Abs made against IgA1 --> immune complexes develop and circulate --> complexes develop in situ in kidneys --> immune response results in injury
29
Henoch-Schonlein Purpura
Age of onset - 5-15 years
30
Dx of HSP
``` MANDATORY - palpable purpura + one of the 4 1. Abdo pain 2. Renal Involvement 3. Arthritis or arthralgia 4. Bx for IgA deposition ```
31
IgA vasculitis
Most common child hood vasculitis --> small vessel | IgA nephropathy/IgA vasculitis with nephritis (HSP)
32
What triggers IgA vasculitis
1-3 days post Viral Infection (70% URTI) Streptococcus, Drugs Duration - 4-6 weeks Causes mesangial Cell injury
33
Treatment of IgA vasculitis
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
Small Summary so far
NEPHRITIC SYNDROME PIGN/HSP/IgA increasing haematuria Intravascular overload Hypertension NEPHROTIC SYNDROME FSGN/minimal Change Increasing proteinuria Intravascular depletion
35
Acute kidney injury
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
Some Feature of AKI
Anuria/Oliguria <0.5ml/Kg/hr for 8 hours Hypertension with fluid oveload Rapid rise in PLASMA CREATININE (1.5 x age reference)
37
Interpreting AKI severity
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
AKI Management
The 3 Ms of prevention Monitor - urine output, PEWS, BP, Weight Maintain - good hydration Minimise - Drugs
39
Causes of AKI
Intra-renal Problems: Glomerular disease (HUD, GN) Tubular injury (acute tubular necrosis) - Hypoperfusion/drugs Interstitial nephritis - NSAIDs, autoimmune Post renal = Obstructive
40
Haemolytic uraemic syndrome
Typically post diarrhoea (enterohaemotthagic E. coli - EHEC) - Verotoxin Producing/ Shigatoxin (VTEC/STEC) other causes may involve - pneumococcal infection/drugs
41
Presentation of HUS
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
HUS Triad
Microangipathic haemolytic anaemia Thrombocytopaenia AKI Renal Transplant required in 30-40% of cases
43
DDx for HUS
ITP
44
3Ms of HUS
Monitor - 5 key kidney functions Be aware of other organs Maintain - IV normal saline and fluid - renal replacement therapy Minimise - NO ANTIBIOTICS
45
Long term Consequences of AKI
Blood pressure Proteinuria monitoring Evolution to CKD
46
Urinary Tract infection
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
possible organisms causing UTI in children
E. Coli Klebsiella Proteus (stones) Step Faecalus
48
Worry about Scarring -Triad
UTI VUR Vulnerable Kidney Scarring is found in the middle of it all
49
VUR = Vesicoureteric reflux - Graded 1-5
``` 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
Screening for scarring
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
Renal Investigation
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
Treatments of UTI
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
Factors that may affect outcome of CKD
``` Late referral HYPERTENSION PROTEINURIA high protein intake poor bone health Acidosis Recurrent UTIs ```

Paediatrics (19 decks)

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