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paediatrics > Nephrology > Flashcards

Nephrology Flashcards

1
Q

how much blood does the nephron receive?

A

25% of cardiac output per minute

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2
Q

how does GFR differ between neonates and children?

A

neonates: 20-30ml/minute

2 years old: 90-120ml/minute (same as adults)

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3
Q

what are the 5 functions of the kidneys?

A
  • waste handling
  • water handling
  • salt balance
  • acid base control
  • endocrine (RBC/BP/Bone health)
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4
Q

what are the components of the glomerular filtration barrier?

A

endothelial cells: fenestrated (vulnerable to injury)
GBM (laminin and collage IV)
podocytes (podocin and nephrin)
mesangial cells

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5
Q

how do patients with a glomerulopathy usually present?

A

blood and/or protein in the urine

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6
Q

what does proteinuria signify?

A

glomerular injury

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7
Q

what leans towards nephritic syndrome?

A
  • increasing haematuria

- intravascular overload

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8
Q

what leans towards nephrotic syndrome

A
  • increasing proteinuria

- intravascular depletion

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9
Q

what are causes of acquired glomerulopathy

A 
minimal change disease (podocyes)
post infectious glomerulonephritis (endothelial cells, GBM)
haemolytic uraemic syndrome (endothelial cells)
HSP (mesangial cells)
IgA nephropathy (mesangial cells)
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10
Q

what are causes of congenital glomerulaopathy

A

podocyte cytoskeletal integrity
basement membrane proteins
endothelial cells/microvascular integrity

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11
Q

what is the definition of nephrotic syndrome?

A

nephrotic range proteinuria
hypoalbuminaemia
oedema

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12
Q

why does oedema occur in nephrotic syndrome?

A

starling’s forces

  • osmotic vs hydrostatic
  • protein is a magnet to water
  • leakage of protein into 3rd space leads to osmotic force
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13
Q

how can proteinuria be tested for?

A

dipstix
- 3 or above= abnormal

protein creatinine ratio
- PR:CR >250mg/mmol= nephrotic range)

24 hour urine collection
- >1g/m^2/24 hours= nephrotic range

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14
Q

how is nephrotic syndrome diagnosed?

A

oedema

proteinuria

  • urine dipstix: protein 3+, blood 2+ (not frank)
  • protein creatinine ratio: 1200mg/mmol
  • urine Na 10mmol/l

bloods

  • albumin low
  • normal creatinine
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15
Q

what are the typical features of minimal change disease?

A

age: 1 - 10
blood pressure: normal
renal function: normal
frank haematuria: none

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16
Q

what type of nephrotic syndrome is most common in children

A

minimal change disease

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17
Q

when should renal biopsy be considered in minimal changed disease?

A

if atypical features present

  • suggestions of autoimmune disease
  • steroid resistance
  • abnormal renal function
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18
Q

what is the treatment for nephrotic syndrome?

A
  • typical features: 8 week course of prednisolone

- second line: immunosupression

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19
Q

what are the possible side effects of high dose glucocorticoids?

A
  • behaviour change
  • mood lability
  • sleep disturbance
  • susceptibility to infection
  • growth disturbance
  • hypertension
  • GI distress due to increased acid
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20
Q

what is the pathogenesis of minimal change disease?

A

interaction between lymphocytes (T and B cells) and podocytes

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21
Q

what are the possible outcomes of minimal change disease?

A

95% remission within 2-4 weeks
80% relapse
80% reach long term remission

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22
Q

what are the causes of steroid resistant nephrotic syndrome?

A

acquired: focal segmental glomerulosclerosis
congenital: podocyte loss (NPHS1 – nephrin, NPHS 2 – podocin)

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23
Q

What is the approach to haematuria?

A

macroscopic/frank: investigate

microscopic: dipstick

associated proteinuria = glomerular disease

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24
Q

what can cause haematuria?

A

systemic: clotting disorders
renal: glomerulonephritis, tumours, cysts
malignancies: sarcomas
stones
UTI
trauma
urethritis

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25
Q

how does nephritic syndrome present?

A

haematuria and proteinuria

reduced GFR

  • oliguria
  • fluid overload (raised JVP and oedema)
  • hypertension
  • worsening renal failure
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26
Q

what type of AKI can nephritic syndrome cause?

A

intrarenal AKI

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27
Q

give examples of causes of glomerulonephritis.

A
  • post Infectious GN
  • HSP / IgA nephropathy
  • membranoproliferative GN
  • lupus Nephritis
  • ANCA positive vasculitis
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28
Q

what is usually the cause of post-infectious GN?

A

group A beta-haemolytic streptococcus

  • throat: 7 - 10 days
  • skin: 2 - 4 weeks
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29
Q

what is the pathogenesis of post-infectious GN?

A
  1. antigen mimicry

2. Ab-Ag complexes

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30
Q

how is post-infectious GN diagnosed?

A
  • bacterial culture
  • positive ASOT
  • low C3 normalises
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31
Q

what is the prognosis of post-infectious GN?

A

good prognosis with no recurrence

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32
Q

how is post-infectious GN treated?

A
  • antibiotics for group A strep
  • support renal functions
  • overload / hypertension (give diuretics)
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33
Q

what is the most common GN worldwide?

A

IgA nephropathy

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34
Q

who does IgA nephropathy usually affect?

A

older children and adults

1-2 day after an URTI

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35
Q

how does IgA nephropathy present clinically?

A

recurrent macroscopic haematuria
+/- chronic microscopic haematuria
varying degree of proteinuria

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36
Q

how is IgA nephropathy diagnosed?

A

clinical picture

confirmation biopsy: mesangial IgA, IgG and C3 deposits

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37
Q

how is IgA nephropathy treated?

A

mild disease: proteinuria with ACEI

moderate - severe disease: immunosuppression (KDIGO)

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38
Q

what is the outcome of IgA nephropathy?

A

variable

  • 25% in ESRF by 10 years
  • outcome better in children
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39
Q

how is a clinical diagnosis of HNS made?

A

age: 5 - 15 yrs

mandatory palpable purpura plus 1 of:

  • abdominal pain
  • renal involvement
  • arthritis or arthralgia
  • biopsy (IgA deposition)
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40
Q

what is the most common childhood vasculitis?

A

IgA vasculitis

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41
Q

describe IgA vasculitis

A

most common vasculitis

very similar to IgA nephropathy
- mesangial cell injury

small vessel vasculitis - arterioles, capillaries, venules

occurs 1 - 3 days post trigger: viral URTI, strep, drugs

symptoms: 4 - 6 wks (1/3 relapse)
treatment: symptomatic, glucocorticoid therapy, immunosuppression, long term hypertension and proteinuria screening

42
Q

What is AKI?

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

43
Q

What are the clinical features of AKI?

A
  • Anuria/oliguria (<0.5ml/kg/hr)
  • Hypertension with fluid overload
  • Rapid rise in plasma creatinine
44
Q

How is AKI defined?

A

Serum creatinine: > 1.5x age specific reference creatinine (or previous baseline if known)

Urine output<0.5 ml/kg for > 8hours

45
Q

What is AKI 1?

A

Measured creatinine >1.5-2x reference creatinine/ULRI

46
Q

What is AKI 2?

A

Measured creatinine 2-3x reference creatinine/ULRI

47
Q

What is AKI 3?

A

Serum creatinine >3x reference creatinine/ULRI

48
Q

How is AKI managed?

A

Monitor
-Urine output, PEWs, BP, weight

Maintain
-Good hydration

Minimise
-Drugs

49
Q

What causes pre-renal AKI?

A

Perfusion problem

50
Q

What are the intrinsic causes of AKI?

A

Glomerular disease

  • HUS
  • Glomerulonephritis

Tubular injury: Acute tubular necrosis (ATN)

  • Consequence of hypoperfusion
  • Drugs

Interstitial nephritis
-NSAID, autoimmune

51
Q

What causes post-renal AKI?

A

Obstruction

52
Q

What causes atypical HUS?

A
  • Autoimmune process

- Can be congenital or acquired

53
Q

What can cause HUS?

A

Typical: post-diarrhoea
-Entero-haemorrhagice E. coli (vertoxin producing E.coli or Shiga toxin

Other causes
-Pneumococcal infection, drugs

54
Q

What serotype of E.coli is responsible for HUS?

A

E.coli O157

55
Q

What is the period of risk of HUS with E coli O157 infection?

A
  • Up to 14 days after the onset of diarrhoea

- 15% develop HUS

56
Q

What is blood diarrhoea in children?

A

Medical emergency

57
Q

What organs are vulnerable in shiga toxin dissemination?

A
  • Kidneys
  • Brain
  • Lungs
  • Pancreas
  • Adrenals
  • Heart
58
Q

What is the triad of HUS?

A
  • Microangiopathic haemolytic anaemia
  • Thrombocytopenia
  • Acute renal failure / AKI
59
Q

How is HUS managed?

A

Monitor (5 kidney functions)

  • Fluid balance, electrolytes, acidosis
  • Hypertension
  • Aware of other organs

Maintain

  • IV normal saline and fluid
  • Renal replacement therapy

Minimise
-No antibiotics

60
Q

What are the potential long term consequences of AKI?

A
  • Blood pressure
  • Proteinuria monitoring
  • Evolution to CKD
61
Q

What can cause paediatric CKD?

A

Congenital Anomalies of the Kidney and Urinary Tract (CAKUT) 55%

  • Reflux nephropathy
  • Dysplasia
  • Obstructive Uropathy (example - posterior urethral valves)

Hereditary conditions 17%

  • Cystic kidney disease
  • Cystinosis (most common inherited tubular disease)

Glomerulonephritis 10%

62
Q

What syndromes may be associated with CAKUT?

A
  • Turner
  • Trisomy 21
  • Branchio-oto-renal
  • Prune Belly syndrome
63
Q

What are the stages of CKD?

A
  • Normal: GFR 90-120
  • CKD 2: GFR 60-89
  • CKD 3: GFR 30-59
  • CKD 4: GFR 15-29
  • CKD 5: ESRD
64
Q

What is presentation of CKD dependent on?

A

Which kidney functions are affect

65
Q

How can CKD present?

A

Asymptomatic

Abnormalities in

  • Waste handling
  • Water handling
  • Salt balance
  • Acid base control
  • Endocrine functions

Bladder dysfunction

Itch

66
Q

How does NICE define UTI?

A

Clinical signs PLUS

  • Bacteria culture from midstream urine
  • Any growth on suprapubic aspiration or catheter
67
Q

How do neonates present with UTI?

A
  • Fever
  • Vomiting
  • Lethargy
  • Irritability
  • Poor feeding
  • Failure to thrive
68
Q

How do pre-verbal children present with UTIs?

A
  • Fever
  • Abdominal pain
  • Abdominal/loin tenderness
  • Vomiting
  • Poor feeding
  • Lethargy
  • Irritability
69
Q

How doe verbal children present with UTI?

A
  • Frequency
  • Dysuria
  • Dysfunctional voiding
  • Changes to continence
  • Abdominal/loin pain or tenderness
  • Fever
  • Malaise
  • Vomiting
70
Q

How can urine samples be obtained in children?

A
  • Normal social cleanliness - water
  • Clean catch urine or midstream urine
  • ?? collection pads, urine bags (contamination risks)
  • Sick infants via catheter samples or suprapubic aspiration (USS)
  • Acutely unwell - do not delay treatment to obtain sample
71
Q

How is UTI diagnosed?

A

Suggestive tests

  • Dipstix: Leucocyte esterase activity, nitrites but unreliable < 2 yrs of age
  • Microscopy: Pyuria >10 WBC per cubic mm and bacturia

Culture > 105 Colony forming units
-E.coli

72
Q

Why do we worry about UTIs?

A
  • UTI on top of vulnerable kidney with VUR can lead to scarring
  • Scarring predisposes to future problems
73
Q

How is VUR graded?

A

Unilateral/bilateral

  • Grade1: Ureter only
  • Grade2: Ureter, pelvis and calyces
  • Grade3: Dilatation of ureter
  • Grade4: Moderate dilatation of ureter, pelvis, tortuous ureter and obliteration of fornices
  • Grade5: Gross dilatation/tortuosity, no papillary impression in calyces
74
Q

What are the principles of investigating the renal tract?

A

Screening for children at risk of progressive scaring
-Reflux nephropathy

Capture those with renal dysplasia

Urological abnormalities / unstable bladder
-Voiding dysfunction

75
Q

Who should be investigated with UTI?

A
  • Upper tract symptoms
  • Younger <6 months
  • Recurrent
  • Septic presentation
76
Q

What investigations are used in complex UTI?

A

Ultrasound
-Structure

DMSA (isotope scan)
-Scaring/function

Micturating cystourethrogram MAG 3 scan
-Dynamic

77
Q

How are UTIs treated?

A

Lower tract
-3 days oral antibiotic

Upper tract / pyelonephritis
-Antibiotics for 7-10 days (Oral if systemically well_
-Prophylaxis is falling out of favour unless anatomical abnormality or high grade VUR
Prevention

78
Q

How can UTIs be prevented

A
  • Fluids, hygiene, constipation treatment

- Voiding dysfunction

79
Q

What factors affect progression of CKD?

A
  • Late referral
  • Hypertension
  • Proteinuria
  • High intake of protein, phosphate and salt
  • Bone health
  • Acidosis
  • Recurrent UTIs
80
Q

What is proteinuria a sign of in CKD?

A

Renal injury and causes ongoing renal injury

81
Q

What system is used to classificy CKD and prognosis?

A

KDIGO

82
Q

How is BP measured in children?

A

Doppler
-Children under 5

Sphigmanomter

Oscillomerty

White coat effect use 24 hour Ambulatory Blood Pressure Monitoring
-Need to be about 120cm (5/6 years of age to tolerate)

83
Q

What factors affect blood pressure?

A
  • Sex
  • Age
  • Height
84
Q

How is hypertension defined in a child?

A

95th centile or higher

85
Q

What is considered borderline in terms of hypertension in children?

A

Between the 90th and 95th centiles

86
Q

Why does bone disease occur in kidney disease?

A
  • Kidneys excrete phosphate.
  • Damage leads to high levels of phosphate and therefore high levels of PTH
  • PTH aims to increase serum calcium and so drives calcium out of the bones
  • Kidney unable to activate vitamin D. Activated vitamin D would normally suppress PTH
87
Q

How is metabolic bone disease treated?

A
  • Low phosphate diet
  • Phosphate binders
  • Active Vitamin D

If ongoing poor growth then growth hormone (if normalised bone biochemistry)

88
Q

Why is there increase cardiovascular risk in kidney disease?

A

Accelerated atherosclerosis

  • Traditional risk factors
  • Anaemia and metabolic bone disease (high PTH levels)
89
Q

How can cystic renal disease be defined?

A

Simple

Developmental

  • Dysplasia
  • Multicystic dysplastic

Genetic

  • Autosomal Recessive (ARPKD)
  • Autosomal Dominant (ADPKD)
  • Syndromic (Various forms of Juvenile Familial Nephronophthisis (JFN))

Acquired
-Cancer

90
Q

What is the incidence of developmental multicystic kidney?

A

1 in 2,000-4,000 (usually sporadic)

91
Q

How does developmental multicystic kidney present?

A

Non-functioning kidney
Ureteric atresia
Hypertrophy of the normal contralateral kidney

92
Q

What is Potter sequence?

A
  • Decreased amniotic fluid
  • Pulmonary hypoplasia
  • Fetal compression of faces, contracture
  • Bilateral renal agenesis (absent ureteric bud)
  • AR polycystic kidney disease (truncating mutation)
93
Q

How do babies with autosomal recessive polycystic kidney disease present?

A
  • Antenatally large bright kidneys
  • Oligohydramnios
  • Severe respiratory distress (pulmonary hypoplasia, nephromegaly mass effect)
94
Q

What are the target organs in ARPKD?

A
  • Renal collecting duct

- Hepatic ductal plate

95
Q

What are the target organs in ADPKD?

A
  • All nephron segments
  • Liver
  • Pancreas
  • Brain
96
Q

What are the target organs in nephronophthisis?

A
  • Tubular (medullary)cysts
  • Retina
  • Liver
  • Brain
  • Bones
97
Q

What are the clinical features of ciliopathy syndromes?

A
  • Renal cysts on US
  • Retinal pigment increases
  • Cystic tubular dilatation on renal biopsy
  • Cerebellar vermis aplasia (molar tooth sign)
98
Q

What is RCAD?

A

Renal cysts and diabetes

-Autosomal dominant glomerulocystic kidney disease

99
Q

How does RCAD present?

A
  • US - cortical cysts
  • Early onset diabetes mellitus (MODY)
  • Genetic heterogeneity (HNF1β mutations)
100
Q

What is Alport syndrome?

A
  • Glomerular Basement Membrane disease
  • Collagen 4 abnormalities
  • X linked dominant inheritance (COL4A5 gene on the X chromosome)
  • Less common AR and AD inheritance
101
Q

What is the clinical presentation of Alport syndrome?

A

Renal

  • Haematuria - microscopic and macroscopic
  • Proteinuria
  • Hypertension

Deafness - high tone sensori-neural loss

Renal failure in early adult life
-Age 20-30 years

Eye changes

  • Lenticonus
  • Macular changes in retina

paediatrics (37 decks)

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