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Nelson - Nephrology > Nephrotic Syndrome > Flashcards

Nephrotic Syndrome Flashcards

1
Q

Nephrotic-range proteinuria is defined as

A

> 3.5g/24hr or UPCr >2g/g

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2
Q

Triad of clinical findings associated with nephrotic syndrome arise from

A

Large urinary losses of protein

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3
Q

Triad of clinical findings associated with nephrotic syndrome

A

1) Hypoalbuminemia (less than 2.5g/dL) 2) Edema 3) Hyperlipidemia (cholesterol >200mg/dL)

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4
Q

Without treatment, nephrotic syndrome is associated with a high risk of death, most commonly from

A

Infections

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5
Q

___% of children with nephrotic syndrome respond to corticosteroid therapy

A

80

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6
Q

MC glomerular lesion associated with idiopathic nephrotic syndrome

A

Minimal change disease

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7
Q

Underlying abnormality in nephrotic syndrome

A

Increased permeability of the glomerular capillary wall

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8
Q

A highly differentiated epithelial cell located on the outside of the glomerular capillary loop

A

Podocyte

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9
Q

Functions of the podocyte

A

1) Structural support of the capillary loop 2) Major component of the glomerular filtration barrier to proteins 3) Involved in the synthesis and repair of the GBM

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10
Q

MC presenting symptom of children with nephrotic syndrome

A

Edema

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11
Q

Postulates that nephrotic syndrome is associated with primary sodium retention, with subsequent volume expansion and leakage of excess fluid into the interstitium

A

Overfill hypothesis

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12
Q

Postulates that sodium and water retention therefore occur as a consequence of intravascular volume depletion.

A

Underfill hypothesis

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13
Q

Goal of nephrotic syndrome therapy

A

Gradual reduction of edema with 1) Judicious use of diuretics 2) Sodium restriction 3) Cautious use of albumin infusions

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14
Q

Why are children with nephrotic syndrome especially susceptible to infections

A

1) Hypoglobulinemia as a result of the urinary losses of immunoglobulin (Ig) G 2) Impaired opsonization from urinary loss of complement factors (predominantly C3 and C5)

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15
Q

Children with nephrotic syndrome are at significantly increased risk for infection with what kind of bacteria

A

Encapsulated, particularly pneumococcal

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16
Q

Most frequent cause of peritonitis

A

Pneumococcus

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17
Q

Peritoneal leukocyte counts of ____ are highly suggestive of SBP

A

> 250

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18
Q

Nephrotic syndrome us a hypercoagulable state resulting from

A

1) Vascular stasis from hemoconcentration and intravascular depletion 2) Increased platelet number and aggregability 3) Changes in coagulation factor levels (increased production of fibrinogen, urinary losses of antithrombotic factors ATIII and protein S)

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19
Q

Clinical risk of hypercoagulable state in children is

A

2-5%, low compared to adults

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20
Q

Primary Renal Disease That Manifest as Idiopathic Nephrotic Syndrome: Most commonly presents as nephrotic syndrome

A

MCNS

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21
Q

Primary Renal Disease That Manifest as Idiopathic Nephrotic Syndrome: Least hematuria

A

MCNS

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22
Q

Primary Renal Disease That Manifest as Idiopathic Nephrotic Syndrome: Asymptomatic proteinuria

A

MPGN

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23
Q

Primary Renal Disease That Manifest as Idiopathic Nephrotic Syndrome: Most hypertensive

A

MPGN

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24
Q

Primary Renal Disease That Manifest as Idiopathic Nephrotic Syndrome: Does not progress to renal failure

A

MCNS

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25
Q

Primary Renal Disease That Manifest as Idiopathic Nephrotic Syndrome: Associated with HIV and heroin use

A

FSGS

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26
Q

Primary Renal Disease That Manifest as Idiopathic Nephrotic Syndrome: Associated with sickle cell disease

A

FSGS

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27
Q

Primary Renal Disease That Manifest as Idiopathic Nephrotic Syndrome: Associated with RVT

A

MN

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28
Q

Primary Renal Disease That Manifest as Idiopathic Nephrotic Syndrome: Associated with partial lipodystrophy

A

MPGN II

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29
Q

Primary Renal Disease That Manifest as Idiopathic Nephrotic Syndrome: Associated with reflux nephropathy

A

FSGS

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30
Q

Primary Renal Disease That Manifest as Idiopathic Nephrotic Syndrome: Low complement levels

A

MPGN I

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31
Q

Primary Renal Disease That Manifest as Idiopathic Nephrotic Syndrome: Steroid resistant

A

MN, MPGN

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32
Q

Approx ___% of children with nephrotic syndrome have idiopathic nephrotic syndrome

A

90

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33
Q

Approx 85% of total cases of nephrotic syndrome in children is ___ in pathology

A

MCNS

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34
Q

___% of children with MCD respond to corticosteroid therapy

A

85

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35
Q

FSGS: LM findings

A

Mesangial cell proliferation and segmental scarring

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36
Q

FSGS: IF findings

A

Positive for IgM and C3

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37
Q

FSGS: EM findings

A

Segmental scarring of the glomerular tuft with obliteration of the glomerular capillary lumen

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38
Q

Only ___% of patients with FSGS respond to prednisone

A

20

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39
Q

Idiopathic nephrotic syndrome is more common in what gender

A

Males

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40
Q

Idiopathic nephrotic syndrome appears between the ages of

A

2 and 6 years

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41
Q

MC cause of idiopathic nephrotic syndrome in children less than 6 y/o

A

MCNS

42
Q

MC cause of idiopathic nephrotic syndrome in adolescents

A

FSGS

43
Q

Nephrotic syndrome can initially be misdiagnosed as ___ because of the periorbital swelling that decreases throughout the day

A

Allergic disorder

44
Q

Important features of minimal change idiopathic nephrotic syndrome

A

Absence of hypertension and gross hematuria

45
Q

Diagnosis of nephrotic syndrome is confirmed by

A

1) Urinalysis with first morning urine protein:creatinine ratio 2) Serum electrolytes, BUN, Crea, albumin, cholesterol

46
Q

Evaluation to rule out secondary forms of nephrotic syndrome (children >/10 yr)

A

C3, ANA, dsDNA, Hepatitis B and C, HIV

47
Q

Evaluation for children >/12 yr with idiopathic nephrotic syndrome, who are less likely to have MCNS

A

Kidney biopsy

48
Q

Nephrotic syndrome: UA protein

A

3+ or 4+

49
Q

Microscopic hematuria is present in ___% of children with nephrotic syndrome

A

20%

50
Q

Nephrotic syndrome: Spot urine protein:crea ratio

A

> 2

51
Q

Nephrotic syndrome: Serum albumin level

A

Less than 2.5g/dL

52
Q

Nephrotic syndrome: Serum complement

A

Normal

53
Q

Idiopathic nephrotic syndrome is less likely to be MCNS if it occurs at what ages

A

Less than 1 year or >12 years old

54
Q

Mainstay of therapy of MCNS

A

Corticosteroids

55
Q

Response to steroids is defined as

A

Attainment of remission within the initial 4 weeks of corticosteroid therapy

56
Q

MCNS: Remission consists of

A

UPCr <0.2 or <1+ protein on dipstick for 3 consecutive days

57
Q

MCNS: Vast majority of children who respond to prednisone therapy do so within

A

First 5 weeks of treatment

58
Q

Nephrotic syndrome: Indication for admission

A

Severe symptomatic edema (Large pleural eff, ascites, or severe genital edema)

59
Q

Sodium restriction

A

<1500mg/day

60
Q

Management of hyponatremia in children with nephrotic syndrome

A

Fluid restriction

61
Q

Management of swollen scrotum in nephrotic syndrome

A

Elevated with pillows to enhance fluid removal by gravity

62
Q

Indications for administration of IV albumin in children with nephrotic syndrome

A

Severe generalized edema with evidence of intravascular volume depletion (hemoconcentration, hypotension, tachycardia, etc)

63
Q

Nephrotic syndrome: Albumin is administered at what dose

A

0.5-1.0g/kg) followed by furosemide (1-2mkdose IV)

64
Q

Potential complications of parenteral albumin therapy

A

1) Volume overload 2) Hypertension 3) Heart failure 4) Pulmonary edema

65
Q

Nephrotic syndrome: Management of dyslipidemia

A

1) Limit dietary fat intake to <30% of calories 2) Limit saturated fat intake to <10% of calories 3) Limit dietary cholesterol intake to <300mg/day

66
Q

Nephrotic syndrome: Empiric antibiotic therapy should be broad enough to cover

A

1) Pneumococcus 2) G- bacteria (3rd gen cephalosporin is a common choice)

67
Q

Nephrotic syndrome: Management of thromboembolism

A

1) Imaging studies to confirm presence of a clot 2) Studies to delineate a specific underlying hypercoagulable state 3) Heparin, LMWH, or Warfarin

68
Q

Nephrotic syndrome: Relapse is defined as

A

1) UPCr >2 or 2) >/3+ protein on urine dipstick testing for 3 consecutive days

69
Q

Nephrotic syndrome: Relapses are common, especially in younger children and are often triggered by

A

URTI or GI infections

70
Q

Nephrotic syndrome: How are relapses treated

A

Usually in a similar manner to the initial episode except that daily prednisone courses are shortened

71
Q

Nephrotic syndrome: Steroid resistance is defined as

A

Failure to achieve remission after 8 weeks of corticosteroid therapy

72
Q

SRNS further eval includes

A

1) Kidney biopsy 2) KFT 3) Quantitation of urine protein excretion on top of urine dipstick testing

73
Q

SRNS is usually caused by

A

FSGS in 80% of cases, MCNS, or MPGN

74
Q

SRNS, specifically FSGS, is associated with a ___% risk for end-stage kidney disease within 5 years of diagnosis if patients do not achieve a partial or complete remission

A

50

75
Q

Reduces the number of relapses in children with frequently relapsing and steroid-dependent nephrotic syndrome

A

Cyclophosphamide therapy

76
Q

Potential side effects of Cyclophosphamide therapy

A

1) Neutropenia 2) Disseminated varicella 3) Hemorrhagic cystitis 4) Alopecia 5) Sterility 6) Increased risk of future malignancy

77
Q

During cyclophosphamide therapy, what blood parameter should be monitored weekly?

A

WBC count, if below 5,000/mm3, withhold drug

78
Q

Cumulative threshold of Cyclophosphamide above which oligospermia or azoospermia occurs in boys is

A

> 250mg/kg

79
Q

Recommended as initial therapy for children with steroid-resistant nephrotic syndrome

A

Calcineurin inhibitors (cyclosporine or tacrolimus)

80
Q

Side effects of calcineurin inhibitors

A

1) Hypertension 2) Nephrotoxicity 3) Hirsutism 4) Gingival hyperplasia

81
Q

An antihelmintic agent with immunomodulating effects that has been shown to reduce the risk of relapse in comparison to prednisone

A

Levamisole

82
Q

Rituximab is a chimeric monoclonal antibody against ___

A

CD20

83
Q

___ and ___ may be helpful as adjunct therapy to reduce proteinuria in steroid-resistant patients

A

Angiotensin-converting enzyme inhibitors and angiotensin II receptor blockers

84
Q

Live vaccination should be deferred until prednisone dose is

A

Either below 1mkday or 2mkday on alternate days

85
Q

T/F Live virus vaccines are contraindicated in children receiving corticosteroid- sparing agents such as cyclophosphamide or cyclosporine

A

T

86
Q

Guideline for children on immunosuppresives following close contact with varicella infection

A

VZ immune globulin

87
Q

T/F Most children with steroid-responsive nephrotic syndrome have repeated relapses

A

T

88
Q

T/F Relapses in children with steroid-responsive NS decrease in frequency as the child grows older

A

T

89
Q

Children with nephrotic syndrome with these characteristics are likely to follow an infrequently relapsing course

A

Children who respond rapidly to steroids and those who have no relapses during the first 6 mo after diagnosis

90
Q

Infectious agents associated with nephrotic syndrome

A

1) Malaria 2) Schistosomiasis 3) Hepatitis B 4) Hepatitis C 5) Filaria 6) Leprosy 7) HIV

91
Q

Proposed mechanism of nephrotic syndrome in lymphoma

A

Lymphoma produces a lymphokine that increases permeability of the glomerular capillary wall

92
Q

Drugs associated with Membranous glomerulopathy

A

Penicillamine, captopril, gold, nonsteroidal antiinflammatory drugs, mercury compounds

93
Q

Drugs associated with MCNS

A

Probenecid, ethosuximide, methimazole, lithium

94
Q

Drugs associated with Proliferative GN

A

Procainamide, chlorpropamide, phenytoin, trimethadione, paramethadione

95
Q

Congenital nephrotic syndrome is defined as

A

Nephrotic syndrome manifesting at birth or within the 1st 3 mo of life

96
Q

Caused by mutations in the WT1 gene, which results in abnormal podocyte function

A

Denys-Drash syndrome

97
Q

Manifestations of Denys-Drash syndrome

A

1) Early-onset nephrotic syndrome 2) Progressive renal insufficiency 3) Ambiguous genitalia 4) Wilms tumor

98
Q

Syndrome caused by mutation in LAMB2 gene, leading to abnormalities in β2-laminin, a critical component of glomerular and ocular basement membranes.

A

Pierson syndrome

99
Q

Congenital nephrotic syndrome presenting with bilateral microcoria (fixed narrowing of the pupil)

A

Pierson syndrome

100
Q

T/F Secondary congenital nephrotic syndrome can resolve with treatment of the underlying cause

A

T

101
Q

Definitive treatment of congenital nephrotic syndrome

A

Renal transplantation

Nelson - Nephrology (13 decks)

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