Nephrology Flashcards

1
Q

What is GFR as a neonate and when does it become similar to adults?

A

Neonate=20-30ml/min/1.73m2

Age 2yrs equals adult 90-120

(Kidney receives 25% of CO/min)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What are the functions of the kidney?

A
  1. Waste handling- urea and creatinine
  2. Water handling
  3. Salt balance-Na & calcium phosphate
  4. Acid base control-bicarb
  5. Endocrine-red cells/BP/bone health-erythropoietin, renin angiotensin aldosterone system, parathyroid hormone
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

How do those with glomerulopathy present?

A

Blood & protein in varying amounts dictate clinical presentation & suggests diagnosis

Injury to one part of the GFB affects the other components

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What does proteinuria signify?

A

Signifies glomerular injury

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Acquired glomerulopathy is common and what type depends on what components of GFB is affected. What are these types?

A

Minimal change disease=epithelial cell (podocyte)

PIGN=basement membrane

PIGN, HUS=Endothelial cell

HSP/IgA nephropathy=mesangial cell

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Congenital glomerulopathy is rare and the type depends on the layer involved, what are they?

A

Congenital nephrotic syndrome (proteins=podocin (AR), nephrin (AR))

Alport syndrome (AL)
Thin basement membrane disease (AD)

Complement regulatory proteins (MPGN)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

How much proteinuria is too much and how is this checked?

A
  1. Dipstix
    Give concentration
    ≥ 3+ usually abnormal
    False positives and negatives
  2. Protein Creatinine Ratio (practical)
    Early morning urine (best)
    normal: Pr:CR ratio <20mg/mmol
    Nephrotic range: >250mg/mmol
    >500mg/mmol - oedema
  3. 24hr urine collection (gold standard)
    normal <60mg/m²/24hrs
    Nephrotic range>1g/m²/24hrs
    Adults >3.5g/24hrs
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What is NephrOtic syndrome?

A
  • Nephrotic range proteinuria
  • Hypoalbuminemia
  • Oedema (increasing 3rd space fluid v)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

After what do children usually present with minimal change disease or steroid sensitive nephrotic syndrom?

A

Intercurrent illness such as gastroenteritis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What does a urine sodium <20 mean?

A

You are holding onto water

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What are the features of MCD (nephrotic syndrome)?

A
  • Age 1-10
  • Normal BP
  • No frank haematuria
  • Normal renal function

Atypical features:
- Suggestions of autoimmune disease
- Abnormal renal function
- Steroid resistance (failure to go into remission after 4 weeks of high dose oral steroids)
ONLY then consider a renal biopsy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

How is nephrotic syndrome treated?

A

If typical features=PREDNISOLONE 8 WEEKS

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What can be done about the infection risk that is increased from the use of high dose glucocorticoids?

A
  • Varicella status
  • Pneumococcal vax
  • Abx prophylaxis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What does steroid sensitivity predict?

A

Diagnosis and prognosis

90% steroid sensitive=MCD

Steroid resistance is more suggestive of focal segmental glomerulosclerosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What is the pathogenesis of Nephrotic syndrome?

A

Interaction between lymphocytes (T&B) and podocytes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What is the outcome of steroid sensitive nephrotic syndrome?

A

Remission
95% in 2-4 weeks

Relapse
80%

80% long term remission

Frequently relapsing=
Second line immunosuppression
- Steroid toxicity
- Steroid dependant and frequent relapses (>4/year)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

What are the causes of steroid resistant nephrotic syndrome?

A

Acquired:
Focal Segmental Glomeruloscerosis (FSGS)
- Podocyte loss
- Progressive inflammation and sclerosis
(50% of these children will
need renal replacement therapy in five years.)

Congenital:
Infant presentations
NPHS1 – nephrin
NPHS 2 – podocin
Podocyte loss

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

What is the investigation for microscopic haematuria?

A

Dipstix adequate

Investigate if >trace on 2 equations

Haemoglobinuria=stix +ve & microscopy negative

Associated proteinuria = glomerular disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

What can cause haematuria?

A

Systemic
- clotting disorders
Renal
- Glomerulonephritis
- Tumour
Wilm’s - nephroblastoma
- Cysts

Malignancies - sarcomas
Stones
UTI
Trauma
Urethritis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

How does Nephritic syndrome present?

A

Clinical diagnosis describes glomerulonephritis

Haematuria and proteinuria
Reduced GFR (creatinine)
- Oliguria
- Fluid overload (Raised JVP, oedema)
- Hypertension
- Worsening renal failure = Rapidly Progressive GN

Intrarenal cause of Acute kidney injury (AKI)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

What does raised creatinine indicate?

A

Abnormal renal function

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Acquired glomerulopathy: which components can be affected and if they are what condition do they cause?

A

Epithelial cell (podocyte) = MCD, FSGS, Lupus

Basement Membrane = Membranous glomerulopathy, MPGN, PIGN

Endothelial cell = post infectious glomerulonephritis (PIGN), Haemolytic Uraemic Syndrome, Membranoproliferative Glomerulonephritis (MPGN), Lupus, ANCA vasculitis

Mesangial cell = HSP / IgA nephropathy, Lupus

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

For nephritic syndrome for example how is it investigated?

A

Radiology
Renal USS – normal

Chase most likely diagnosis
- ASOT - Raised 1200
- Positive throat swab group A strep

Immunology workup
- Complement C3 low, normal C4
- Autoimmune diseases (ANA, ANCA negative)

Biopsy
Not required at present

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

What is acute post infectious glomerulonephritis and what causes it?

A

Age of onset-usually 3-5years

Cause:
- Usually group A strep
- Beta haemolytic
- Site (throat 7-10 days, skin 2-4weeks)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Acute post infectious glomerulonephritis is SELF LIMITING: How is it diagnosed?
- Bacterial culture - positive ASOT - low C3 normalises Remember differential diagnosis (IgA / Lupus / MPGN) ASOT – Anti streptolysin O titre
26
What is the treatment of Acute post infectious glomerulonephritis?
- Abx - Support renal functions (electrolyte/acid base) - Overload/HT-Diuretics NOT RECURRENT
27
What is the commonest glomerulonephritis and how does it present?
Most common glomerulonephritis 1-2 days after URTI Usually older children and adults Clinically: Recurrent macroscopic haematuria +/-Chronic microscopic haematuria Varying degree of proteinuria Clinical diagnosis
28
How is IgA nephropathy diagnosed?
Clinical picture - Negative autoimmune workup and normal compliment Confirmation biopsy (IgA deposits within the mesangial cells)
29
What is the treatment for IgA nephropathy: mild & severe?
Mild=proteinuria with ACEI Moderate to severe=Immunosuppression (KDIGO)
30
What is the age of onset of HSP IgA related vasculitis?
Age of onset=5-15 years
31
How is HSP IgA related vasculitis clinically diagnosed?
Mandatory palpable purpura + one of 4 1. Abdominal pain 2. Renal involvement 3. Arthritis or arthralgia 4. Biopsy -IgA depostition
32
IgA vasculitis is the most common childhood vasculitis affecting what vessels?
Small vessel vasculitis - Overlaps with IgA nephropathy - Non granulomatous HSP
33
What is IgA vasculitis triggered by and what is the duration of symptoms?
1-3 days post trigger - Viral URTI - Strep, drugs 4-6 weeks duration and a 1/3 relapse` (Nephritis-mesangial cell injury)
34
How is IgA vasculitis treated?
Symptomatic-Joints, gut Glucocorticoid therapy - Not helpful in mild nephritis - May help with gastrointestinal involvement Immmunosuppresion - Trial in moderate to severe renal disease Long term=Hypertension and proteinuria screening
35
What is AKI and what are the clinical signs of it?
“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” Anuria/oliguria (<0.5ml/kg/hr) Hypertension with fluid overload Rapid rise in plasma creatinine
36
How is AKI diagnosed?
Serum creatinine: > 1.5x age specific reference creatinine (or previous baseline if known) Urine output<0.5 ml/kg for > 8hours
37
What are the AKI warning score criteria?
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
38
What are the 5 key kidney functions and what is associated with them in assessment?
Waste handling Water handling Salt balance Acid base control Endocrine red cells / blood pressure / bone health Waste – urea / creatinine Salt – sodium / potassium / calcium drops / phosphate rise Acid base – bicarbonate Endocrine – Renin aldosterone angiotensin system / PTH / activation of Vitamin D (Vit D = sterole) / Erythropoietin
39
How is AKI managed (think 3 Ms)?
PREVENTION 3Ms: Monitor=PEWS(BP), Urine Output, weight Maintain=good hydration / electrolytes / acid - base Minimise=Drugs
40
What are the possible causes of AKI?
Pre-renal-Perfusion problem Renal Post renal-Obstructive uropathies
41
What are the general intrinsic renal problems that can cause AKI?
Glomerular disease - HUS - Glomerulonephritis Tubular injury - acute tubular necrosis (ATN) (Consequence of hypoperfusion, Drugs) Interstitial nephritis - NSAID, autoimmune
42
What is the definition of haemolytic uraemic syndrome (parameters)?
HAEMOLYSIS - Packed cell volume <30% - Hb level <10g/dl & fragmented erythrocytes on blood film THROMBOCYTOPENIA Platelet count <150x10⁹/l AKI - Serum creatinine greater than the age-related range (>97th pc) - GFR <80 mls/min/1.73m² Proteinuria
43
What causes typical HUS to present?
Typical HUS - post diarrhoea Entero-Haemorrhagic E.coli (EHEC) - Verotoxin producing E.coli – VTEC or Shiga toxin (STEC) Other causes=pneumococcal infection, drugs
44
What causes atypical HUS?
Defect (overactivation) in alternative compliment pathway - autoimmune, drugs, hereditary
45
How does HUS preset?
E coli O157:H7 serotype Period of risk of HUS - Up to 14 days after onset of diarrhoea - 15% develop HUS Bloody diarrhoea is a medical emergency in children
46
What triad presents in HUS?
Microangiopathic haemolytic anaemia Thrombocytopenia Acute Kidney Injury / Acute Renal Failure
47
How is bloody diarrhoea and HUS managed?
Prevention of oliguric HUS = intravascular volume expansion
48
What are the long term consequences of AKI?
- BP - Proteinuria monitoring - Evolution of CKD
49
How is HUS managed (same as all AKI)?
3Ms Monitor 5 kidney functions Fluid balance - hypertension Electrolytes (monitor for hyperkalaemia) Acidosis (excess urea) Waste Hormones - hypertension Aware of other organs Maintain IV normal saline and fluid Renal replacement therapy Minimise No antibiotics / NSAIDS
50
What are some chronic kidney disease causes ?
Congenital Anomalies of the Kidney and Urinary Tract (CAKUT) - 55% of paediatric CKD - Reflux nephropathy - Dysplasia - Obstructive Uropathy (example - posterior urethral valves) Hereditary conditions - Cystic kidney disease (in adult is Autosomal dominant polycystic kidney disease) - Cystinosis Glomerulonephritis
51
CAKUT may not be isolated what should you be thinking of if it is not?
Think Syndromic Turner Trisomy 21 Branchio-oto-renal Prune Belly syndrome
52
What is prune belly syndrome?
Prune-Belly syndrome, also known as Eagle-Barrett syndrome, is a rare disorder characterized by partial or complete absence of the stomach (abdominal) muscles, failure of both testes to descend into the scrotum (bilateral cryptorchidism), and/or urinary tract malformations.
53
At what GFR do you start to find signs and symptoms?
<60
54
Symptoms vary in CKD depending on what function of the kidney is affected?
Uraemic – loss of appetite, weight loss, itch Water – polyuria Salt / acid base – lethargy Endocrine – lethargy, reduced effort tolerance, anaemia, HT, hypotensive if very polyuric Bladder dysfunction - neuropathic bladders such as spina bifida
55
How is a UTI diagnosed?
Clinical signs PLUS - Bacteria culture from midstream urine - Any growth on suprapubic aspiration or catheter
56
What does a fever indicate in UTI often?
Pyelonephritis
57
What are the difference in in symptoms for younger and older age for a UTI?
Younger age more systemic symptoms Older the age more lower tract symptoms Also symptoms depends whether it is upper or lower tract
58
How is a urine specimen obtained?
Normal social cleanliness - water Clean catch urine or midstream urine (if continent) ?? collection pads, urine bags (good at excluding infection but not the best for diagnosing because of the risk of contamination of normal gram-negative bacteria found in the perineum and skin) Sick infants = catheter samples or suprapubic aspiration (USS) Acutely unwell - do not delay treatment
59
How is a UTI diagnosed?
Suggestive tests - Dipstix Leucocyte esterase activity, nitrites unreliable < 2 yrs of age Microscopy Pyuria Bacturia GOLD STANDARD:Culture > 105 Colony forming units/ml - Gram negative bacteria - E.coli
60
Why are UTIs a worry?
In the context of VESICO-URETERIC REFLUX may cause a kidney injury Grades 1-5 High grade/bilateral (??renal dysplasia)
61
The combination of a vulnerable kidney, UTI & VUR can result in?
SCARRING (Papilla shape – concave associated with intra renal reflux and found in the renal poles the most common place for renal scaring)
62
In UTI what are the principle things we are looking for and what guidelines are there on who to investigate?
Principles: Congenital vs. acquired - Screening for children at risk of progressive scaring (Reflux nephropathy) & Capture those with renal dysplasia - Urological abnormalities - Unstable bladder-Voiding dysfunction Guidelines who to investigate: Upper tract symptoms Younger Recurrent
63
What investigations are done in a worrying renal case of UTI?
USS within 6 weeks-structure DMSA (isotope scan) Scaring / function Micturating cysto-urethrogram (GOLD STANDARD FOR REFLUX) MAG 3 scan-dynamic
64
What is the treatment for UTI?
Lower tract 3 days oral antibiotic Upper tract / pyelonephritis antibiotics for 7-10 days - Oral if systemically well Role of prophylaxis ?? (done if have abnormal urinary tract) Prevention-Fluids, hygiene, constipation Manage voiding dysfunction
65
What are key factors that affect the progression of CKD?
- HT - Proteinuria High intake of protein, phosphate and salt Bone health - PTH - Phosphate - Vitamin D Acidosis Recurrent UTIs
66
What is the gold standard for assessing BP?
Sphigmanomter - Doppler (<5yrs) Oscillomerty Technique Bladder size - NEVER use cuff too small White coat effect-24 hour Ambulatory Blood Pressure Monitors
67
What factors affect BP?
Sex, Age & Height specific
68
What is the definition of HT in paediatrics?
3 occasions Hypertension: ≥95th percentile Borderline ≥90 but <95th pc
69
How is CKD managed?
Depends on what function is affected: Waste – urea – nutrition / protein intake / minimise weight loss (catabolism) Water – polyuria or oliguria Salt – salt loosing / high potassium - low potassium diet / avoid hypercalcaemia / reduce phosphate in diet Acid base – bicarbonate loss Anaemia-EPO Relax bladder function-Oxybutynin?
70
How does metabolic bone disease occur?
Kidneys wee out phosphate High phosphate ----- increase PTH Kidneys activate Vitamin D3 (High PTH causes metabolic bone disease and cardiovascular disease) (Most potent driver for PTH is Hypocalcaemia)
71
How is metabolic bone disease treated?
Treatment Principles - Low phosphate diet - Phosphate binders - Active Vitamin D If ongoing poor growth=Growth hormone
72
What is the CV risk from CKD?
Accelerated atherosclerosis Traditional risk factors PLUS Anaemia / metabolic bone disease (PTH)
73
What are results of and predictors of ongoing kidney injury?
Proteinuria and HT
74
What conditions are important from a paediatric perspective in terms of proteinuria/haematuria, AKI & CKD?
Proteinuria / Haematuria - Glomerular disease Nephrotic Syndrome Nephritic Syndrome Acute Kidney Injury Haemolytic Uraemic Syndrome Chronic Kidney Disease - Developmental anomalies (CAKUT) - Reflux nephropathy
75
How much of your kidney function do you need to lose before your creatinine starts to become abnormal?
40%
76
What is BP ideally controlled using?
ACEIs
77
In UTIs what should be considered?
Reflux nephropathy-reduce recurrent injury Monitor for Upper tract symptoms/Younger/Recurrent UTIs