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Royal College Exam > Nephrology > Flashcards

Nephrology Flashcards

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1
Q

Causes of glomerulonephritis associated with low C3 (immune complex deposition)

A
  • Lupus nephritis
  • post-strep nephritis
  • cryoglobulinemic GN
  • other immune complex GN (MPGN)
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2
Q

Causes of glomerulonephritis associated with anti-GBM autoantibodies

A

goodpastures, anti-GBM nephritis

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3
Q

most common cause of microscopic hematuria

A

benign familial hematuria (autosomal dominant) 63%

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4
Q

Alport’s disease

A

usually x-linked (85%) defect in COL4A5 gene, presents with microscopic hematuria, sensorineural hearing loss, ESRD by 4th decade (males)

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5
Q

gross hematuria presenting 1-3 days after a URTI

A

IgA nephropathy

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6
Q

gross hematuria presenting 7-21 days after a pharyngitis

A

post-strep GN

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7
Q

methods to measure proteinuria

A

protein:creatinine ratio (normal < 20 mg/mmol)
OR
24 hour protein collection (normal < 4 mg/m2/hr, nephrotic > 40 mg/m2/hr)
DO NOT DO URINANALYSIS

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8
Q

Hematuria: 4 tests on urinanalysis that would indicate a glomerular origin

A
  • colour = tea or cola-coloured
  • dysmorphic RBCs (acanthocytes)
  • RBC casts
  • proteinuria
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9
Q

Triad seen with post-strep GN

A

gross hematuria
generalized edema
hypertension

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10
Q

Long term complications of Fanconi syndrome

A

FTT, growth failure, intellectual impairment, ocular abnormalities (cataracts, glaucoma), enamel defects

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11
Q

Medical treatment of overactive bladder

A

treat constipation (PEG), anticholinergic (oxybutynin) or alpha-blockers (doxazosin)

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12
Q

Definition of nephrotic range proteinuria

A

> 40 mg/m2/hr

> 50 mg/kg/day

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13
Q

Most common cause of nephrotic syndrome

A

Minimal change disease

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14
Q

Nephrotic syndrome, finding on biopsy of glomerulus

A

effacement of podocyte foot processes

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15
Q

What organisms are patients with nephrotic syndrome at increased risk of?

A

Streptococcus pneumoniae and haemophilus influenza - due to loss of complement factor C3b, opsonins and immunoglobulins

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16
Q

What should you do before starting a patient with nephrotic syndrome on immunosuppressant medication?

A

PPD, give polyvalent pneumococcal vaccine (23-serotype and 7-valent conjugate vaccines)

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17
Q

Treatment of nephrotic syndrome

A

prednisone x 4-6 weeks

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18
Q

Most common cause of peritonitis in nephrotic syndrome

A

strep pneumoniae

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19
Q

Secondary infectious causes of nephrotic syndrome

A

malaria, schistosomiasis, hep B/C, HIV

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20
Q

Options for surgical repair of urinary reflux - 2 methods

A

Deflux (injection of stuff into ureter), reimplanation of ureter

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21
Q

Gross hematuria, nephrotic syndrome. what to worry about?

A

renal vein thrombosis

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22
Q

Five criteria of nephritis

A
  • hematuria (gross, red cell casts)
  • hypertension
  • azotemia (elevated urea, cr)
  • edema (fluid overload, high JVP)
  • proteinuria
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23
Q

Alports - extra-renal manifestations

A

eye - anterior lenticonus, recurrent corneal erosions, macular flecks

ear - deafness (bilateral)

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24
Q

BP tables depend on…

A

height
age
gender

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25
Q

What is cysteinuria?

A

Urinary loss of COLA amino acids (cysteine, ornithine, lyseine and arginine)

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26
Q

Diagnosis of cystinosis

A

leukocyte cysteine levels

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27
Q

Usual cause of renal vein thrombosis in infant

A

IDM

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28
Q

In gestation, when is formation of nephrons complete?

A

36-40 weeks GA

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29
Q

Approx how many nephrons are in the kidney?

A

1 million

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30
Q

What factors modify glomerular filtration?

A
  • glomerular hydrostatic pressure
  • glomerular oncotic pressure
  • rate of flow
  • permeability of glomerular capillary wall
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31
Q

Definition of hematuri

A

> = 5 RBCs/hpf

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32
Q

Causes of false positive for hematuria on dipstick

A 
oxidizing agents (ie. H2O2 used to clean perineum prior to obtaining sample)
betadine
alkaline urine (pH >9)
rhabdomyolysis
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33
Q

causes of false negative for hematuria on dipstick

A

presence of formalin in sample

high concentrations of ascorbic acid (as a result of high vit c intake)

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34
Q

what does a dipstick read as positive for blood?

A

hemoglobin
RBCs
myoglobin

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35
Q

What can differentiate glomerular vs non-glomerular hematuria on urinanalysis?

A

presence of RBC casts and dysmorphic RBCs = glomerular origin!

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36
Q

what condition should you think of with isolate microscopic hematuria (on repeated samples)

A

hypercalciuria (test by checking calcium:creatinine ratio)

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37
Q

most common form of primary GN

A

IgA nephropathy

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38
Q

Pathology characteristics of IgA nephropathy

A

mesangial proliferation, crescents/sclerosis, IgA deposition + C3

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39
Q

IgA nephropathy can present as….

A

gross hematuria
microscopic hematuria
nephritic
nephrotic

40
Q

What is the C3 in IgA nephropathy?

A

NORMAL

41
Q

What can decrease the rate of progression of IgA nephropathy in adults

A

fish oil (omega 3)

42
Q

what proportion of IgA nephropathy patients progress to have significant renal disease?

A

30%

43
Q

Alports - pathology

A

mesangial proliferation

lipid-laden cells (foam cells)

44
Q

How do you work-up / diagnose alports?

A

Fam hx
Audiogram
Ophtho - presence of anterior lenticonus is pathognomonic!

45
Q

Risk of ESRD in Alports

A

High! 75% of males (x-linked hemizygotes) and homozygotes (AR form) develop ESRD by age 30

Tx: control hypertension, ACEi

46
Q

Recurrent episodes of hematuria with occasional gross hematuria post-URTI

Family members w/ same sxs

No hearing loss

What syndrome?

A

Thin basement membrane disease!

AKA benign familial hematuria

47
Q

post-strep GN

clinical situations and latency period

A

strep pharyngitis: 5-21 days

pyoderma/impetigo: 3-6 weeks

48
Q

natural hx of post-strep GN

A

acute phase resolves in 6-8 weeks, can have persistent microscopic hematuria x 1-2 yrs

49
Q

Lab tests for post-strep GN

A

decreased C3
normal C4
low CH50
confirmation of strep diagnosis (positive throat cx, rising ASOT or anti-DNase B, streptozyme screen)

50
Q

Types of GN with a normal C3

A 
Alports
IgA nephropathy
ANCA-positive GN
Goodpastures
Anti-GBM
51
Q

Types of GN with a LOW C3

A 
post-strep
post-infectious
shunt nephritis
MPGN
cryoglobulinemia
SLE
bacterial endocarditis
52
Q

Common causes of membranous nephropathy in children

A

SLE
Hep B
Congenital syphilis

53
Q

Most common cause of membranous nephropathy world wide

A

malaria

54
Q

What can cause membranoproliferative GN?

A

Think of infections!

Hep B/C, syphilis, endocarditis

55
Q

If someone has a low C3 GN which doesn’t improve after 2 months, what conditions should you think of?

A

SLE
MPGN
cryoglobulinemia

56
Q

When should you biopsy a patient with SLE and evidence of renal involvement?

A

always!
no correlation between SLE disease activity and severity of renal involvement. the extent of disease will be used to guide therapy

57
Q

How do you treat lupus nephritis?

A

prednisone x 4-6 mos (or azathioprine as steroid-sparing agent)

if very severe, can tx with cyclophosphamide

58
Q

which class of lupus nephritis has the highest risk of progression to ESRD?

A

Class IV lupus nephritis

59
Q

Henoch Schonlein Purpura

Clinical features

A

palpable purpura
abdominal pain
arthritis

50% develop nephritis

60
Q

How should you monitor for nephritis in patients with HSP?

A

Urinanalysis weekly during period of active disease, then monthly x 6 mos

If anything abnormal - refer to nephro!

61
Q

Which types of GNs are associated with crescenteric / rapidly progressive GN?

A

SLE
HSP (if severe)
ANCA positive granulomatosis with polyangiitis (formerly, Wegeners)

62
Q

Clinical features of Goodpasture’s disease

A

pulmonary hemorrhage
glomerulonephritis

(due to antibodies against GBM)

63
Q

HUS - triad

A

“RAT”
renal failure
anemia (MAHA)
thrombocytopenia

64
Q

TTP - features

A 
"FAT RN"
fever
anemia (MAHA)
thrombocytopenia
renal failure
neurologic sxs
65
Q

Diagnostic criteria of HUS

A

An acute illness with HUS features (renal failure, MAHA, thrombocytopenia) occurring within 3 wks after an episode of acute or bloody diarrhea

66
Q

Common bacterial etiologies of diarrhea in HUS

A

Vero-toxin producing e.coli (0157:H7)

Shiga-toxin producing Shigella

67
Q

Common foods contaminated with e.coli 0157:H7

A

unpasteurized milk
undercooked meat (beef)
apple cider

68
Q

Genetic forms of HUS are due to…

A

Deficiencies in
ADAMTS13
Complement factor H, I or B

69
Q

Intestinal complications of HUS

A 
severe inflammatory colitis
ischemic enteritis
bowel perforation
intussusception
pancreatitis
70
Q

Should you give platelets in HUS?

A

NO! They are immediately consumed, may worsen clinical course

71
Q

Should you give RBCs in HUS?

A

Maybe!
If you suspect pneumococcus-associated HUS, then you should wash the RBCs before transfusion (IgM on cell surface can worsen course)

72
Q

Treatment of HUS

A

Supportive care (NO PLATELETS)

Plex if ADAMTS13 or factor H deficient

Eculizumab for atypical HUS

73
Q

Nephrotic syndrome characteristics

A

Nephrotic range proteinura
Hypoalbuminemia
Edema
Hyperlipidemia

74
Q

Definition of nephrotic range proteinuria

A

> 40 mg/m2/hr on a 24 hr collection

75
Q

Genetic disorders causing nephrotic syndrome

A

Finnish-type congenital nephrotic syndrome

Denys-Drash

Alport syndrome

Nail-patella syndrome

76
Q

Causes of idiopathic nephrotic syndrome

A

Minimal change disease

FSGS

Membranous nephropathy

77
Q

Hallmark pathologic finding of idiopathic nephrotic syndrome

A

Effacement of podocyte foot processes

78
Q

Why is nephrotic syndrome a hypercoagulable state?

A

Vascular stasis (due to 3rd spacing, low intravascular volume)

Increase in clotting factor production (due to increased hepatic protein synthesis)

Loss of anti-coagulants via kidneys

79
Q

Immunofluorescence in FSGS

A

IgM and C3 deposits

80
Q

Natural history of FSGS

A

does not respond well to steroids, often leads to ESRD

81
Q

What should you do before starting tx for nephrotic syndrome?

A

PPD

Pneumococcal vaccine

82
Q

Tx for nephrotic syndrome

A

Prednisone DAILY x 4-6 weeks

83
Q

relapse rate of nephrotic syndrome (if given 4-6 weeks of initial steroids at diagnosis)

A

30-40%

84
Q

what is steroid dependent nephrotic syndrome?

A

kids that relapse while steroids are being weaned (on alternate day schedule) OR within 28 days of completing steroid course

85
Q

definition of frequent relapser in nephrotic syndrome?

A

relapse >= 4x in 12 mos

86
Q

definition of steroid resistant nephrotic syndrome

A

fail to respond to 8 weeks of steroid tx (usually FSGS)

87
Q

how can you treat steroid-dependent and frequently relapsing nephrotic syndrome?

A

cyclophosphamide

monitor for neutropenia

88
Q

how can you treat steroid resistant nephrotic syndrome?

A

cyclosporine, tacrolimus

89
Q

side effects of cyclosporine

A

nephrotoxicity
hirsutism
gingival hypertrophy
PRES

90
Q

Bugs causing SBP in nephrotic syndrome

A

strep pneumo

e.coli

91
Q

Clinical features of Denys-Drash

A

early-onset nephrotic syndrome

wilms tumour

ambiguous genitalia

progressive renal insufficiency

92
Q

Child with suspected proteinuria - how do you test?

A

First void urine protein to creatinine ratio (abnormal > 20)

or 24 hr urine collection (> 40 mg/m2/hr = nephrotic)

93
Q

Causes of transient proteinuria

A 
fever
dehydration
exercise
cold
stress
seizures
94
Q

what is orthostatic proteinuria?

A

Increased urinary protein excretion when in the upright position

95
Q

how do you diagnose orthostatic proteinuria?

A

first void spot protein:creatinine ratio x 3 demonstrating absence of proteinuria

(other evaluations showing + proteinuria)

96
Q

definition of hypertension

A

SBP or DBP >= 95th %ile on 3 occasions

97
Q

when should you start measuring BP in kids? how often?

A

at every visit in children > 3 yrs

should measure at EVERY visit regardless of age if prem, NICU, CHD, UTIs, kidney anomaly, malignancy, BMT, etc

Royal College Exam (32 decks)

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