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TAS Revision > Nephrology > Flashcards

Nephrology Flashcards

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1
Q

What are the 4 primary causes of Nephrotic Syndrome?

A

Minimal change disease - most common cause in children
Focal segmental glomerulosclerosis (FSGS)
Membranous Nephropathy
Membranoproliferative Glomerulonephritis

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2
Q

Between which age does minimal change disease tend to present?

A

2-6 years

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3
Q

What is the pathophysiology of minimal change disease?

A

Minimal change disease is a disease of T-cells which produce cytokines which destroy the epithelial layer (causes flattening of the podocytes which causes leakage of albumin and protein)

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4
Q

Which cancer is associated with minimal change disease?

A

Hodgkins Lymphoma

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5
Q

What happens to complement factors in minimal change disease?

A

Normal C3 and C4

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6
Q

What are the indications for biopsy in minimal change disease?

A

<1 year or >10 years
Not responsive to steroids (no response to steroids after 6 weeks of Tx w/ high dose steroids)

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7
Q

What are the two main complications of minimal change disease?

A
  • Spontaneous Bacterial Peritonitis (due to strep. pneumoniae, e.coli or group B strep)
  • Ascities
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8
Q

What percentage of children will outgrow minimal change disease?

A

80%

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9
Q

Which nephrotic condition should you consider if minimal change disease is not respondent to steroids?

A

FSGS (NB: FSGS can progress to end stage renal disease)

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10
Q

Which conditions are commonly associated with FSGS?

A

Heroin use
HIV
Sickle cell anaemia

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11
Q

Which two conditions cause effacement of the foot processes of the epithelial layer?

A

Minimal change disease
Focal segmental glomerulosclerosis

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12
Q

Which conditions are associated with membranous nephropathy?

A

SLE (most common GN in SLE)
Solid tumours
Gold
Penicillamine
Infections (Hep B, syphillis, malaria)

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13
Q

What percentage of children with membranous nephropathy progress to end stage renal disease?

A

25%

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14
Q

Which nephrotic condition is associated with low C3 levels?

A

Membranoproliferative GN

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15
Q

Which condition is associated with tram/ train track appearance on H&E staining?

A

Membranoproliferative GN

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16
Q

What is tram track appearance of H&E staining?

A

Generalised increase in mesangial cells and matrix, capillary walls appear thickened and contain regions of duplication and splitting

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17
Q

What is the characteristics of Minimal change disease/ FSGS?

A

Proteinuria
Hypoalbuminaemia
Hyperlipidaemia
Hypercoagulable state
Normal C3

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18
Q

“Spike and dome” appearance is associated with which nephrotic condition?

A

Membranous nephropathy

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19
Q

What are the diagnostic indicators in Lupus Nephritis?

A
  • Positive ANA and anti-dsDNA antibodies
  • Low C3 and C4
  • Definitive diagnosis is by renal biopsy
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20
Q

When does congenital nephrotic syndrome present?

A

Between birth and 3 months of age

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21
Q

What are the associations with congenital nephrotic syndrome?

A
  • Severe intractable oedema
  • Iron deficiency and vitamin D deficiency
  • Hypothyroidism
  • Frequent infections due to loss of IgG
  • Clots due to loss of antithrombin III
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22
Q

When does screening for microalbuminuria occur in children with diabetes?

A

Type 1 diabetes - 5 years after diagnosis
Type 2 diabetes - at the time of diagnosis

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23
Q

How long does it take for eGFR to reach adult levels?

24
Q

Creatinine levels reflect that of the mother for how long after birth?

25
ADH primarily acts at which site?
Collecting ducts
26
Renin is produced at which site?
The juxtaglomerular apparatus
27
What is the typical presentation of Alpert's Syndrome?
Sensorineural hearing loss Microscopic heamaturia Progresses to severe renal disease
28
What are the characteristics of medullary sponge kidneys?
Presents in middle age Recurrent nephrolithiasis Urinary tract infections
29
What is the commonest cause of hypertension in neurofibromatosis type 1?
Renal artery stenosis or phaeochromocytoma
30
What are the main complications of nephrotic syndrome?
Infections - including spontaneous bacterial peritonitis Thrombosis - e.g. DVT, PE, renal vein thrombosis Hypertension
31
Which investigation assesses for reflux and the presence of posterior urethral valves?
MCUG
32
Which investigation assesses for scarring of the kidneys?
DMSA
33
Bartter syndrome can be associated with which neonatal complication?
Polyhydramnios
34
Features of Gitelmann syndrome?
Hypokalaemia + hypomagnesaemia + hypocalciuria Normal blood pressure
35
What is the pathophysiology of Gitelman syndrome?
Autosomal recessive condition resulting in a defect in the sodium chloride transporter in the distal collecting tubules
36
Features of cystinosis?
Hypokalaemia + hypophosphataemia + a metabolic acidosis with a high urinary pH
37
Which glomerulonephritis has normal complement?
Primary GN: - FSGS - IgA nephropathy Secondary GN: - HSP
38
Which glomerulonephritis has low complement levels?
Primary GN: - Acute post-streptococcal GN (low C3, normal C4) Membranoproliferative GN: - Low C3, low C4 Secondary GN: - Lupus nephritis (low C3, low C4)
39
Which medication is indicated in genetic HUS due to complement dysregulation?
Eculizumab
40
HUS is a triad of which three conditions?
Microangiopathic haemolytic anaemia Thrombocytopenia Acute kidney injury
41
What are the predictive factors for a poor prognosis in HUS?
- Non Shiga toxin HUS - Prolonged oliguria and anuria - Severe hypertension - Involvement in medium sized arteries - Severity of CNS symptoms - Peristent consumption of clotting factors - Age >5 years
42
Which receptor is mutated in Bartter's syndrome?
Na+ K+ Cl- co-transporter in the thick ascending limb of the loop of henle
43
Features of Bartters syndrome?
Metabolic alkalosis (due to K+ exchange for H+ in the distal convulsed tubules) Low Na, K+ and Chloride Hyperplasia of the juxtaglomerular apparatus Elevated renin and aldosterone
44
What are the investigations of choice in vesico-ureteric reflux?
MCUG - assess degree of reflux DMSA - assess degree of scarring Video urodynamic studies - clarify type and severity of VUR
45
The presence of bilateral hydronephrosis and oligohydramnios on antenatal screening should raise suspicion of?
VUR
46
Outline the grading of VUR?
Grade I: Reflux into a non-dilated ureter only Grade II: Reflux into the pelvis and calyces with no dilatation Grade III: Reflux into the pelvis and calyces with mild dilatation Grade IV: Reflux into the pelvis and calyces with moderate ureteral tortuosity Grade V: Ureter/ renal pelvis/ calyces is grossly distended with loss of papillary impressions
47
Normal complement - purely renal involvement?
IgA nephropathy Anti-GBM disease Anti-ANCA disease
48
Normal complement with systemic involvement (and renal involvement)?
HSP Alports syndrome
49
Low C3 with purely renal involvement?
Post streptococcal GN Mesangioproliferative GN C3 glomerulonephropathy
50
Low C3 with systemic involvement?
SLE Subacute bacterial endocarditis
51
What is Goodpasture's Disease a triad of?
GN + Pulmonary haemorrhage + anti-GBM antibody formation
52
What findings occur in post-streptococcal GN?
Low C3 Low CH50
53
What are the three heritable renal cystic diseases?
Cysts associated with multiple malformations Infantile (autosomal recessive) polycystic disease Adult (autosomal dominant) polycystic disease
54
Commonest chromosomal defect in polycystic disease 1?
Chromosome 16
55
Nephrotic syndrome with partial lipodystrophy is most associated with...?
Mesangiocapillary nephritis with C3 nephritic factor
56
What triad of symptoms could represent a renal vein thrombosis?
Macroscopic haematuria Thrombocytopenia Enlarged kidneys (unilateral/ bilateral) - may present with deranged creatinine/ urea
57
Which PKD is associated with hepatic involvement?
Autosomal recessive

TAS Revision (14 decks)

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