Nephrology Flashcards
1
Q
Glomerular disease causes what?
A
Nephrotic syndrome
Nephritic syndrome
2
Q
AKI causes what?
A
HUS
3
Q
How much blood is received through the kidneys?
A
Very high blood
25% CO / min
4
Q
GFR in neonates compared to adults
A
20-30ml/min/1.73m2
So when dealing with neonates there is already compromised system
5
Q
5 functions of the kidney
A
Waste handling (urea, creatinine) Water handling Salt balance (Na, K, Ca, P) Acid base control (bicarbonate) Endocrine (red cells, BP, bone health)
6
Q
What part of the glomerular filtration barrier is vulnerable to immune mediated injury?
A
The endothelial cell
7
Q
Features of the endothelial cell of the glomerular filtration barrier
A
Fenestrated
8
Q
Two proteins of the glomerular filtration membrane
A
Type IV collagen (COL4)
Laminin
9
Q
How is the glomerular filtration membrane synthesised?
A
From
- podocytes
- endothelial cells
10
Q
Functions of mesangial cells
A
Glomerular structural support
Embedded in GBM
Regulates blood flow of glomerular capilaries
11
Q
Presentation of glomerulopathy
A
Haematuria
Proteinuria
12
Q
Indications towards nephritic syndrome
A
Increasing haematuria
Intravascular overload
13
Q
Indications towards nephrotic syndrome
A
Increasing proteinuria
Intravascular depletion
14
Q
Pathology of acquired glomerulopathy
A
White component affected
- epithelial cell (podocyte) - Minimal change disease, FSGS, lupus
- Basement membrane - membranous glomerulopathy, MGPN, PIGN
- Endothelial cell - HUS, MPGN, lupus, infection associated glomerulonephritis (PIGN)
- mesangial cell - HSP, IgA nephropathy, lupus
15
Q
Is acquired glomerulopathy common?
A
Yes
16
Q
Is congenital glomerulopathy common?
A
No - it is rare
17
Q
Layers involves in congenital glomerulopathy
A
Podocyte skeletal integrity
Basement membrane proteins
Endothelial / microvascular integrity
18
Q
Presentation of nephrotic syndrome
A
3 - 4 days Nephrotic range proteinuria Frothy urine Blood in urine - not usually frank Hypoalbuminaemia Swollen face which is worse in the morning One eye closed in the morning Oedema - Periorbital - legs (pitting) - Ascites - small pleural effusions Well but pale Weight up from baseline BP can be raised Normal creatinine Protein creatinine ratio >250mg/mmol High levels of fat / lipids in the blood
19
Q
Pathology of nephrotic syndrome
A
Interaction between lymphocytes (T and B cells) and podocytes
Loss of size and charge barrier of the GFB
Oncotic (Osmotic) vs hydrostatic
Protein (osmotic) = magnet to water
20
Q
Can nephrotic syndromes have FH?
A
yes
21
Q
How do you test for proteinuria?
A
Dipstix
- gives concentration ; > 3+ usually abnormal
Protein creatinine ratio
- > 250 = nephrotic
24 hour urine collection (gold standard)
- normal < 60mg/m2/24hrs
- nephrotic = >1g/m2/24 hours in children
22
Q
Normal protein creatinine ratio
A
< 20mg/mmol
23
Q
Nephrotic range for protein creatinine ratio
A
> 250 mg/mmol
24
Q
What is the gold standard investigation for proteinuria?
A
24 hour urine collection
25
Treatment of nephrotic syndrome
Prednisolone for 8 weeks
26
S/Es of prednisolone
```
Behaviour (irritable, moody, dont sleep)
High BP
Facial change
Growth (only if recurrent courses)
GI distress
```
27
Other considerations when treating nephrotic syndrome
Varicella status and pneumococcal vaccination as both act as immunosuppressants
28
What indicates minimal change disease/
Response to steroids
29
If steroid resistance / dependent when treating nephrotic syndrome, what does this indicate?
maybe not dealing with minimal change disease
30
Presentation of minimal change disease
```
Resolving microscopic haematuria
Proteinuria
Oedema
Weight gain
Normal renal function
Normal BP
Atypical features
- suggestions of autoimmune disease
- abnormal renal function
- steroid resistance
Nephrotic syndromes
```
31
What age gets minimal change disease?
2 - 5 y / o
32
What presentation in minimal change disease would make you consider a renal biopsy?
Atypical features
- suggestions of autoimmune disease
- abnormal renal function
- steroid resistance
33
Prognosis of Nephrotic syndrome
Remission 95% in 2 - 4 weeks
relapse 80%
- 50% frequent - 2 in 6 or 4 in a year
80% long term remission
34
If really frequent recurrences of nephrotic syndrome, what may be the problem?
Prednisolone toxicity
35
Acquired steroid resistant nephrotic syndrome condition and its pathology
Focal Segmental Glomeruloscerosis (FSG)
- podocyte loss
- progressive inflammation and sclerosis
36
What type of haematuria should always be investigated?
Macroscopic
37
When is microscopic haematuria investigated?
> trace on 2 occasions
38
Important causes of macroscopic haematuria
```
Glomerulonephritis
Post infect GN
IGA / HSP
UTI (dysuria)
Trauma
Stones (pain)
```
39
Important causes of microscopic haematuria
```
Glomerulonephritis
Post infect GN
IGA / HSP
UTI
Trauma
Stones / hypercalciuria
```
40
Symptoms of nephritic syndrome
```
Haematuria
Reduced GFR
oliguria
fluid overload
- raised JVP
- oedema
HTN
Worsening renal failure - rapidly progressive FN
```
41
What is nephritic syndrome an intrarenal cause of?
AKI
42
Causes of glomerulonephritis
```
Post infectious GN
HSP / IgA nephropathy
Membranoproliferative GN
Lupus nephritis
ANCA positive vasculitis
```
43
Investigations of nephritic syndrome
```
Renal USS
Chase the most likely diagnosis
- ASOT
- throat swabs
- complement tests
ANA, ANCA testing
Biopsy
- not needing when have a secure diagnosis
```
44
Age of onset of acute post infectious glomerulonephritis
2 - 5
45
Causes of acute post infectious glomerulonephritis
Usually group A strep
- beta haemolytic so completely haemolysis
Post throat infection 7-10 days
Post skin infection 2 - 4 weeks (impetigo common)
46
Investigations of acute post infectious glomerulonephritis
Bacterial culture
positive ASOT
Low C3 normalises
47
Prognosis of acute post infectious glomerulonephritis
Self limiting
good prognosis
can have haematuria for 1 - 2 years after but the function will improve
48
How does a e.g. Group A strep cause an AKI?
Nephrogenic antigens on the strep
Bind specific sites in the glomerulus
Also antibodies bind Ag forming circulating complexes with deposits in the kidney
Set up a humeral and cellular immune response
Activates alternative compliment pathway
---> AKI
49
Treatment of acute post infectious glomerulonephritis
Antibiotics for infection
Support renal functions
Overload / HTN
- diuretics
50
If post infectious glomerulonephritis is recurrent, what does this indicate?
It is not the right diagnosis
51
What is the most common glomerulonephritis?
IgA nephropathy
52
When does IgA nephropathy occur?
1 - 2 days after URTI
53
Who gets IgA nephropathy?
Older children
| Adults
54
Genetics of IgA nephropathy
Genetic predisposition
| - galactose deficient IgA
55
Presentation of IgA nephropathy
Recurrent macroscopic haematuria
+ / - chronic microscopic haematuria
Varying degree of proteinuria
56
Diagnosis of IgA nephropathy
Clinical picture
Negative autoimmune workup
Normal complement
Confirmation biopsy
57
Treatment of IgA nephropathy
Mild disease = ACEIs
| Moderate to severe = immunosuppression (KDIGO)
58
Prognosis of IgA nephropathy
25% ESRF by 10 years post diagnosis
| Outcome better in children
59
Age of onset of HSP IgA related vasculitis
5 - 15 years
60
Presentation of HSP IgA related vasculitis
```
Mandatory PALPABLE PURPURA
1 of
- abdominal pain
- renal involvement
- arthritis or arthralgia
- biopsy (IgA deposition)
```
61
What is the most common childhood vasculitis?
IgA vasculitis
62
What vessels does IgA vasculitis affect?
Small vessels
63
When does IgA vasculitis occur?
1 - 3 days post trigger
64
Triggers of IgA vasculitis
Viral UTI in 70%
Streptococcus
Drugs
65
Duration of symptoms of IgA vasculitis
4 - 6 weeks
1 / 3rd relapse
- skin and kidney relapse
66
Pathology of IgA vasculitis
Nephritis - mesangial cell injury
67
Treatment of IgA vasculitis
```
Symptomatic - joints, gut
Glucocorticoid therapy (not for renal disease, helps GI disease)
Immunosuppression
```
68
Long term prognosis of IgA vasculitis and any screenings that are carried out
Very good
| Hypertension and proteinuria screening
69
Definition of AKI
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
70
Causes of AKI
Perfusion problem
- drugs
- renal artery stenosis
Intrinsic
- Glomerular disease (HUS, glomerulonephritis)
- Tubular injury (acute tubular necrosis as a consequence of Hypoperfusion or drugs)
- interstitial nephritis (NSAIDs, autoimmune)
Post renal
- obstructive
71
Presentation of AKI
HUS
Anuria / oliguria (<0.5ml/kg/hr) for 8 hours
HTN with fluid overload
Rapid rise in plasma creatinine >1.5x age
72
AKI warning score
AKI 1; measured creatinine >1.5-2x reference creatinine/ULRI
AKI 2; measured creatinine 2-3x reference creatinine/ULRI
AKI 3; serum creatinine >3x reference creatinine/ULRI
73
Management of AKI (the 3Ms)
```
Monitor
- urine output
- PEWS
- BP
- weight
Maintain
- good hydration
Minimise
- further injury
- particular with drugs
```
74
What does PEWS stand for?
Paediatric Early warning score
75
Long term consequences of AKI
BP
Proteinuria (has to be monitored)
Evolution to CKD
76
When does HUS usually occur?
Post diarrhoea
- Entero-haemorrhagic E Coli
Either verotixin producing E coli or Shiga toxin
77
Causes of HUS
Post diarrhoea
pneumococcal infection
drugs e.g. chemotherapeutic drugs
Congenital or acquired autoimmune processes
78
How do toxins from bacteria cause HUS?
Toxins cross enterocytes and bind to white cells on the endothelium, causing endothelial damage calling an hypethrombic state causing haemolysis
79
Triad of HUS
1. microangiopathic haemolytic anaemia
2. thrombocytopenia
3. acute renal failure / AKI
80
Presentation of HUS
```
E coli 0157;H7 serotype
Bloody diarrhoea
Neurological involvement
- reduced consciousness
- seizures
renal failure
```
81
Where do you get E Coli 0157;H7?
Cattle
| Sheep
82
Period of risk of HUS after diarrhoea, and how many get HUS?
Up to 14 days after onset of diarrhoea
| 15 % develop HUS
83
Management of HUS (3 Ms)
```
Monitor (the 5 kidney functions)
- fluid balance
- electrolytes
- acidosis
- HTN
- aware of other organs
Maintain
- IV normal saline and fluid
- RRT
minimise
- no antibiotics
```
84
What does CAKUT stand for?
Congenital anomalies of the kidney and urinary tract
85
Presentation of CAKUT
Reflux nephropathy
Dysplasia
Obstructive uropathy
86
Hereditary chronic kidney diseases
Cystic kidney disease
| Cystinosis
87
What can CAKUT be associated to?
Turner
Trisomy 21
Bracio-oto-renal
Prune belly syndrome
88
Presentation of CKD
Variable depending on which function is altered
- bladder dysfunction
- salt wasting (low P, K high)
- renal tubular acidosis
- waste handling
- water handling
- endocrine
89
What is needed to diagnose a UTI
Clinical signs PLUS
| Bacterial culture midstream urine
90
Most common presenting symptoms of neonates with a UTI
```
Fever
vomiting
lethargy
Irritability
Poor feeding
Failure to thrive
```
91
Most common presenting symptoms of pre verbal children with a UTI
Fever
abdo / loin pain / tenderness
vomiting
poor feeding
92
Most common presenting symptoms of verbal children with a UTI
```
Frequency
Dysuria
Dysfunctional voiding
Changes to continence
Abdominal / loin pain / tenderness
```
93
Septic shock secondary to UTI is more common in who?
Infants
94
How can a urine specimen be obtained in children?
```
Normal social cleanliness - water
Clean catch urine or mid stream urine
Collection pads or urine bags (easily contaminated)
Sick infants
- catheter samples
- suprapubic aspiration (USS)
```
95
Suggestive tests of UTI
```
Dipstix
- leucocyte esterase activity, nitries
Microscopy
- pyuria > 10 WBC per cubic mm
- bacteria
Culture > 10 5 colony forming units
- E coli
```
96
Why worry about UTIs?
UTI, VUR and vulnerable kidneys can lead to scarring
97
Treatment of UTI
lower tract
- 3 days oral Ax
upper tract / pyelonephritis
- antibiotics 7 - 10 days (oral if systemically well)
98
Prevention of UTI
Hygiene
Constipation
Voiding dysfunction
99
Indications to use imaging to investigate the renal tract
```
Upper tract symptoms
- temp > 38C
- vomiting
- systemically unwell etc
Younger
- younger you are = higher risk of renal dysplasia
```
100
Renal imaging used
USS - first line
DMSA
Micturating cytourethrogram
MAG 3 scan
101
Factors affecting the progression of CKD
```
late referral
HTN
Proteinuria
High intake of protein, phosphate and salt
Bone health
- PTH
- P
- Vitamin D
Acidosis
Recurrent UTIs
```
102
What is used to investigate BP?
Doppler (gold standard < 5s)
Sphigmanomter
oscillometry
103
Factors affecting BP
Sex
age
height
104
Management of CKD
```
Nutrition / protein intake
- low K diet
- avoid hypercalcaemia
- reduce P in diet
Give / restrict fluids
Bicard if needed for acid base control
Support for anaemia
ACEIS
Bone health
Bladder dysfunction
```
105
Treatment for metabolic bone disease
Low phosphate diet
Phosphate binders
Active Vit D
If ongoing poor growth - growth hormone
106
Why is metabolic bone disease involved with the kidneys?
The kidneys pee out phosphate
High phosphate - increases PTH
High PTH is associated with CVS in adults and IHD
107
Types of cystic renal disease
```
Simple
Developmental
- dysplasia
- multicystic dysplastic
Genetic
- autosomal recessive (ARPKD)
- autosomal dominant (ADPKD)
- syndromic
Acquired
- cancer
```
108
Pathology of multicystic dysplastic kidney (MCDK)
Non functioning kidney
ureteric atresia
Hypertrophy of normal collateral kidney - usually unilateral (75%)
109
Pathology of ARPKD and potters sequences
Antenatally large bright kidneys
110
Presentation of ARPKD and potters sequence
```
Oligohydramnios
Severe respiratory distress
- pulmonary hyperplasia
- nephromegaly mass effect
Potters sequence
- decreased amniotic fluid
- pulmonary hyperplasia
- foetal compression of faces, contracture
- bilateral renal agenesis (absent uteteric bud)
- AR polycystic kidney disease
Loss of function
```
111
What is Renal cysts and diabetes (RCAD)?
```
autosomal dominant glomerulocystic kidney disease
early onset DM (MODY)
US = cortical cysts
Genetic heterogenecity
= HNF1B mutations
```
112
What is Alport syndrome?
Glomerular basement membrane disease
113
Pathology of Alport syndrome
Collagen 4 abnormalities
| X linked dominant inheritance
114
Presentation of Alport syndrome
```
Haematuria - micro and macroscopic
Proteinuria
HTN
deafness - high tone and senori neural loss
Renal failure in early adult life (20-30 yrs)
eye changes
- lenticonous
- macular changes in retina
```
115
Investigation for renal scarring in a child with VUR
Radionucleotide scan using dimercaptosuccinic acid (DMSA)
116
What does VUR look like on a micturating cystourethrogram?
Gross dilatation of the ureter, pelvis and calyces with ureteral tortuosity
117
Features of an atypical UTI in children
```
Seriously ill
Poor urine flow
Abdominal or bladder mass
Raised creatinine
Septicaemia
Failure to respond to treatment with suitable antibiotics within 48 hours
Infection with non E coli organisms
```
118
Investigation to diagnose VUR
Micturating cystourethrogram
