Nephrology Flashcards

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?

A

1-2 years

24
Q

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

A

48 hours

25
Q

ADH primarily acts at which site?

A

Collecting ducts

26
Q

Renin is produced at which site?

A

The juxtaglomerular apparatus

27
Q

What is the typical presentation of Alpert’s Syndrome?

A

Sensorineural hearing loss
Microscopic heamaturia
Progresses to severe renal disease

28
Q

What are the characteristics of medullary sponge kidneys?

A

Presents in middle age
Recurrent nephrolithiasis
Urinary tract infections

29
Q

What is the commonest cause of hypertension in neurofibromatosis type 1?

A

Renal artery stenosis or phaeochromocytoma

30
Q

What are the main complications of nephrotic syndrome?

A

Infections - including spontaneous bacterial peritonitis
Thrombosis - e.g. DVT, PE, renal vein thrombosis
Hypertension

31
Q

Which investigation assesses for reflux and the presence of posterior urethral valves?

A

MCUG

32
Q

Which investigation assesses for scarring of the kidneys?

A

DMSA

33
Q

Bartter syndrome can be associated with which neonatal complication?

A

Polyhydramnios

34
Q

Features of Gitelmann syndrome?

A

Hypokalaemia + hypomagnesaemia + hypocalciuria
Normal blood pressure

35
Q

What is the pathophysiology of Gitelman syndrome?

A

Autosomal recessive condition resulting in a defect in the sodium chloride transporter in the distal collecting tubules

36
Q

Features of cystinosis?

A

Hypokalaemia + hypophosphataemia + a metabolic acidosis with a high urinary pH

37
Q

Which glomerulonephritis has normal complement?

A

Primary GN:
- FSGS
- IgA nephropathy
Secondary GN:
- HSP

38
Q

Which glomerulonephritis has low complement levels?

A

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
Q

Which medication is indicated in genetic HUS due to complement dysregulation?

A

Eculizumab

40
Q

HUS is a triad of which three conditions?

A

Microangiopathic haemolytic anaemia
Thrombocytopenia
Acute kidney injury

41
Q

What are the predictive factors for a poor prognosis in HUS?

A
  • 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
Q

Which receptor is mutated in Bartter’s syndrome?

A

Na+ K+ Cl- co-transporter in the thick ascending limb of the loop of henle

43
Q

Features of Bartters syndrome?

A

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
Q

What are the investigations of choice in vesico-ureteric reflux?

A

MCUG - assess degree of reflux
DMSA - assess degree of scarring
Video urodynamic studies - clarify type and severity of VUR

45
Q

The presence of bilateral hydronephrosis and oligohydramnios on antenatal screening should raise suspicion of?

A

VUR

46
Q

Outline the grading of VUR?

A

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
Q

Normal complement - purely renal involvement?

A

IgA nephropathy
Anti-GBM disease
Anti-ANCA disease

48
Q

Normal complement with systemic involvement (and renal involvement)?

A

HSP
Alports syndrome

49
Q

Low C3 with purely renal involvement?

A

Post streptococcal GN
Mesangioproliferative GN
C3 glomerulonephropathy

50
Q

Low C3 with systemic involvement?

A

SLE
Subacute bacterial endocarditis

51
Q

What is Goodpasture’s Disease a triad of?

A

GN + Pulmonary haemorrhage + anti-GBM antibody formation

52
Q

What findings occur in post-streptococcal GN?

A

Low C3
Low CH50

53
Q

What are the three heritable renal cystic diseases?

A

Cysts associated with multiple malformations
Infantile (autosomal recessive) polycystic disease
Adult (autosomal dominant) polycystic disease

54
Q

Commonest chromosomal defect in polycystic disease 1?

A

Chromosome 16

55
Q

Nephrotic syndrome with partial lipodystrophy is most associated with…?

A

Mesangiocapillary nephritis with C3 nephritic factor

56
Q

What triad of symptoms could represent a renal vein thrombosis?

A

Macroscopic haematuria
Thrombocytopenia
Enlarged kidneys (unilateral/ bilateral) - may present with deranged creatinine/ urea

57
Q

Which PKD is associated with hepatic involvement?

A

Autosomal recessive