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Y4R1 - Pediatrics I 👶 > L4: Nephrotic Syndrome > Flashcards

L4: Nephrotic Syndrome Flashcards

1
Q

Def of Nephrotic Syndrome

A
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2
Q

Classification of Nephrotic Syndrome

A

Clinical & Pathological

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3
Q

Secondary of Nephrotic Syndrome

A
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3
Q

Clinical Classification of Nephrotic Syndrome

A
  • NS is classified based upon whether or not there are signs of systemic disease into 1ry & 2ry
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4
Q

Primary Nephrotic Syndrome

A
  • 90% of childhood cases.
  • It refers to NS in the absence of an identifiable systemic disease.
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5
Q

Disorders Causing Nephrotic Syndrome

A
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6
Q

Disorders Causing Nephrotic Syndrome

  • Infections
A

HBV - HCV - HIV & Strept - syphilis & bilharziasis - malaria.

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7
Q

Disorders Causing Nephrotic Syndrome

  • Immune
A

SLE - anaphylactoid purpura.

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8
Q

Disorders Causing Nephrotic Syndrome

  • Iatrogenic
A

NSAIDs - penicillamine - gold - Heroin.

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9
Q

Disorders Causing Nephrotic Syndrome

  • Metabolic
A

DM - amyloidosis.

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10
Q

Disorders Causing Nephrotic Syndrome

  • Metals
A

Lead - mercury

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11
Q

Disorders Causing Nephrotic Syndrome

  • Others
A

Sickle cell anemia - alport syndrome - HUS - lymphoma.

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12
Q

Types of Nephrotic syndrome during the 1st year of life

A
  • Congenital NS: in 1st 3 months of life.
  • Infantie NS: in infants 1 year.
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13
Q

Etiology of Nephrotic syndrome during the 1st year of life

A
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14
Q

Pathological Classification of Nephrotic syndrome

A
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15
Q

Characters of Minimal change disease (MCD)

  • %
  • LM
  • IF
  • EM
  • Response to Steroids
  • Illustration
A
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16
Q

Characters of FSGS

  • %
  • LM
  • IF
  • EM
  • Response to Steroids
  • Illustration
A
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17
Q

Characters of DMP

  • %
  • LM
  • IF
  • EM
  • Response to Steroids
  • Illustration
A
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18
Q

Characters of Membranous Glumeronephritis

  • %
  • LM
  • IF
  • EM
  • Response to Steroids
  • Illustration
A
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19
Q

Characters of MPGN

  • %
  • LM
  • IF
  • EM
  • Response to Steroids
  • Illustration
A
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20
Q

Def of Idiopathic Nephrotic Syndrome

A
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21
Q

Incidence of Idiopathic Nephrotic Syndrome

A
  • It is the most common form of childhood NS.
  • Representing more than 90 percent of cases between 1 and 10 years of age and 50 percent after 10 years of age.
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22
Q

Pathogenesis of Idiopathic Nephrotic Syndrome

A
  • Mechanisms of glomerular injury
  • Mechanisms of edema
  • Mechanism of hypoalbuminemia
  • Mechanism of proteinuria
  • Mechanism of hyperlipidemia
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22
Q

Age in Idiopathic Nephrotic Syndrome

A

2:8 years

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23
Q

Sex in Idiopathic Nephrotic Syndrome

A

Male 2 : Female 1

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24
Q

Pathogenesis of Idiopathic Nephrotic Syndrome

  • Mechanism of Glomerular Injury
A
  • Circulating non-immune Factors
  • Circulating immune Factors
  • Mutations in podocyte or slit diaphragm proteins
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24
Q

Mechanism of Glomerular Injury in Nephrotic Syndrome

  • Circulating non-immune factors:
A
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25
Q

Mechanism of Glomerular Injury in Nephrotic Syndrome

  • Circulating immune factors:
A
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26
Q

Mechanism of Glomerular Injury in Nephrotic Syndrome

  • Mutations in podocyte or slit diaphragm proteins
A
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27
Q

The occurrence of prolonged remissions following measles, which down regulates CMI further endorses this hypothesis.

A

…

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28
Q

Abnormalities of T cell subsets and/or function have been variably reported in a number of patients with MCD.

A

…

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29
Q

Pathogenesis of Idiopathic Nephrotic Syndrome

  • Mechanism of Edema
A
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30
Q

Mechanism of Edema in Nephrotic Syndrome

  • Arterial Underfiling
A
  • A reduction in plasma oncotic pressure induced by hypoalbuminemia would seem to favor the movement of fluid out of the vascular space into the interstitium and produce arterial underfilling.
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31
Q

Mechanism of Edema in Nephrotic Syndrome

  • Sodium retention
A
  • Sodium retention is due to increased reabsorption in the collecting tubules mainly due to increased activity of the Na-K-ATPase pump in the cortical collecting tubule and relative resistance to atrial natriuretic peptide.
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32
Q

Pathogenesis of Nephrotic Syndrome

  • Mechanism of hypoalbuminemia
A
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33
Q

Pathogenesis of Nephrotic Syndrome

  • Mechanism of Proteinurea
A
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34
Q

The filtration of macromolecules across the glomerular capillary wall is normally restricted by two mechanisms:

A
  • charge-selectivity
  • size-selectivity
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35
Q

The pores between feet processes are closed by a thin membrane called the …..

A

slit diaphragm.

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36
Q

In comparison: circulating IgG is predominantly neutral or cationic, and its filtration is not limited by charge.

A

…

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37
Q
A

…

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37
Q

The glomerular capillary wall is size-selective; having functional pores of an approximate radius of 40 to 45 A (the radius of albumin is roughly 36 A).

A

…

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38
Q

Pathogenesis of Idiopathic Nephrotic Syndrome

  • Mechanism of Hyperlipidemia
A
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39
Q

CP of Nephrotic Syndrome

A
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40
Q

CP of Nephrotic Syndrome

  • Edema
A
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41
Q

What is the major Presenting Symptom of Nephrotic Syndrome?

A

Edema

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42
Q

Edema in Nephrotic Syndrome

  • Manifestation
A

It becomes clinically detectable when fluid retention exceeds 3-5% of body weight.

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43
Q

Edema in Nephrotic Syndrome

  • Sites
A
  • Periorbital edema frequently misdiagnosed as allergy is often the initial symptom.
  • Edema is gravity dependent, localized to the lower extremities in the upright position, and to the dorsal part of the body in reclining position.
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44
Q

Edema in Nephrotic Syndrome

  • Characters
A
  • This edema is soft and pitting, keeping the marks of clothes or finger pressure.
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45
Q

Edema in Nephrotic Syndrome

  • Severity
A
  • Anasarca may develop with ascites, and pleural and pericardial effusions. (If anasarca occurred can’t be AGN)
  • Edema of the scrotum and penis, or labia, may be seen.
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46
Q

CP of Nephrotic Syndrome

  • Abdominal Pain
A
  • It may be related to rapid formation of ascites or concomitant hypovolemia.
  • It is occasionally due to a complication such as peritonitis, thrombosis or rarely, pancreatitis.
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47
Q

CP of Nephrotic Syndrome

  • HTN
A
  • Mild in 95% of cases.
  • Blood pressure is usually normal but sometimes elevated.
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48
Q

CP of Nephrotic Syndrome

  • Macroscopic Hematuria
A

It is observed in 3% of cases.

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49
Q

CP of Nephrotic Syndrome

  • Complications
A

The disease may also be revealed by a complication

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50
Q

CP of Nephrotic Syndrome

  • Asymptomatic
A

NS is occasionally discovered during a routine urine analysis.

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51
Q

Urine Analysis in Nephrotic Syndrome

  • Volume
  • Color
  • Casts
  • Specific Gravity
A
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51
Q

Investigations for Nephrotic Syndrome

  • Urine Analysis
A
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51
Q

Investigations for Nephrotic Syndrome

A
  • Urine & Blood
52
Q

Urine Analysis in Nephrotic Syndrome

  • Proteinuria
53
Q

Qualitative Evaluation of Proteinuria in Nehrotic Syndrome

54
Q

Quantitative Evaluation of Proteinuria in Nehrotic Syndrome

55
Q

What is the screening test for Proteinuria in Nephrotic Syndrome?

A

Using urine dipstick

55
Q

Urine Analysis in Nephrotic Syndrome

  • Urine Protein Selectivity
56
Q

Investigations for Nephrotic Syndrome

  • Blood Tests
56
Q

What is the Confirmatory test for Proteinuria in Nephrotic Syndrome?

A
  • Using 24-hour urine protein
  • Using Spot urine protein / creatinine ratio
57
Q

Blood Tests for Nephrotic Syndrome

  • CBC
  • PTN
  • Lipids
  • C3
  • Ck
58
Q

Pathological Dx of Nephrotic Syndrome

59
Q

Etiological Dx of Nephrotic Syndrome

60
Q

When is Renal Bx Indicated in children in Nephrotic Syndrome?

61
Q

Nephrotic sydrome not fulfilling criteria of Finnish type

62
Q

Complications of Nephrotic Syndrome

A
  • Infections
  • Thromboembolism
  • Hypovolemia
  • Renal Insufficiency
  • Corticosteroids SE
63
Q

Most Common Complications of Nephrotic Syndrome

A

Infections

64
Q

Infections in Nephrotic Syndrome

  • Types
A

Bacterial & Viral

65
Q

Bacterial Infections in Nephrotic Syndrome

  • most Common Site
A
  • The most common infection is peritonitis.
  • Patients may also develop meningitis, pneumonia, UTI and cellulitis.
66
Q

Bacterial Infections in Nephrotic Syndrome

  • Organism
A
  • Streptococcus pneumoniae “most common”
  • Others: E. coli, streptococcus B, Haemophilus influenzae & other G-ve organisms
67
Q

Bacterial Infections in Nephrotic Syndrome

  • Predisposing Factors
67
Q

Cofactor of Cb of the alternative pathway of complement which has an important role in opsonization of encapsulated bacteria as Streptococcus pneumoniae.

68
Q

Viral Infections in Nephrotic Syndrome

  • RF
A
  • Viral infections may be observed in patients receiving steroids or immunosuppressive agents.
68
Q

Viral Infections in Nephrotic Syndrome

  • Organisms
A

Chickenpox is often observed in these young children and may be life threatening.

69
Q

Complication of Nephrotic Syndrome

  • Hypovolemia
70
Q

Hypovolemia in Nephrotic Syndrome

  • When does it happen?
A

This complication is observed typically early during a relapse or in acute phase of NS.

71
Q

Hypovolemia in Nephrotic Syndrome

  • Predisposing Factors
A

Sepsis, diarrhea or diuretics may precipitate hypovolemia.

72
Q

Hypovolemia in Nephrotic Syndrome

  • Complaint
73
Q

Complications in Nephrotic Syndrome

  • Thromboembolism
74
Q

Most Dangerous Complication in Nephrotic Syndrome

A

Thromboembolism

75
Q

Thromboembolism in Nephrotic Syndrome

  • Incidence
A
  • 2-3%
  • However, this may underestimate the true incidence since many thromboembolic events are asymptomatic.
76
Q

Thromboembolism in Nephrotic Syndrome

  • RF
77
Q

Thromboembolism in Nephrotic Syndrome

  • Sites
A
  • Both arterial and venous thromboses have been reported.
  • Most common: pulmonary artery, renal vein, deep leg veins, inferior vena cava, and femoral/iliac artery.
  • Others: cerebral and meningeal arteries, mesenteric and hepatic veins.
78
Q

Complications in Nephrotic Syndrome

  • Renal Insufficiency
79
Q

Complications in Nephrotic Syndrome

  • Corticosteroids SE
80
Q

TTT Aspects of Nephrotic Syndrome

A
  • General
  • Specific
81
Q

General TTT of Nephrotic Syndrome

  • Diet
82
Q

General TTT of Nephrotic Syndrome

  • Bed Rest
A

Noooo “cause thrombosis”

83
Q

General TTT of Nephrotic Syndrome

  • Edema
84
Q

General TTT of Nephrotic Syndrome

  • Hypovolemia
85
Q

General TTT of Nephrotic Syndrome

  • Prevention of Complications
86
Q

Prevention of Complications in Nephrotic Syndrome

  • Infections
A
  • S pneumonia: Oral penicillin + Pneumococcal vaccine
  • Varicella: Acyclovir + Varicella vaccine
87
Q

Prevention of Complications in Nephrotic Syndrome

  • Thromboembolism
88
Q

Prevention of Complications in Nephrotic Syndrome

  • Hyperlipidemia
A

It is reasonable to consider a lipid lowering regimen in children with a persistent NS

89
Q

Specific TTT of Nephrotic Syndrome

  • DOC
A
  • Oral steroids: Corner stone
  • The commonly used preparation is prednisone.
90
Q

Specific TTT of Nephrotic Syndrome

  • TTT of First Episode
91
Q

Indications of Empiric TTT of Nephrotic Syndrome with Corticosteroids

92
Q

TTT of First Episode in Nephrotic Syndrome

  • Induction of Steroid Dose
92
Q

TTT of First Episode in Nephrotic Syndrome

  • Maintenaince of Steroid Dose
93
Q

TTT of First Episode in Nephrotic Syndrome

  • Monitoring
A

Once a patient responds to steroid therapy, monitoring for proteinuria is required to detect relapses early.

94
Q

Def of Relapse in Nephrotic Syndrome

A

Appearance of significant proteinuria (>40 mg/h/ m2 or >50 mg/kg/day or Albustix +++) for 3 consecutive days after having been in remission.

95
Q

Patients who respond to steroid have one of the following courses:

96
Q

Non relapsers (36%)

A

No relapses within the six-month period.

97
Q

Infrequent relapsers (18%)

A

A single relapse within the six-month period.

98
Q

Frequent relapsers

A
  • ≥ 2 relapses within the six-months of end of therapy or
  • ≥ 4 relapses within 1 year of end of therapy.
99
Q

Steroid dependence

A

2 consecutive relapses during steroid therapy or within 14 days after cessation of therapy.

100
Q

Steroid resistance

A

Failure to induce remission in 4 weeks using conventional doses of corticosteroid

101
Q

Late non-responders (3%)

A

steroid resistance in a patient who had previously responded to corticosteroid therapy.

102
Q

TTT of Relapses in Nephrotic Syndome

103
Q

Treatment of frequent relapsing/ steroid dependent NS:

104
Q

Steroid-sparing agents should be considered in children …….

A
  • Who have significant steroid toxicity and those requiring prednisone at doses exceeding 1 mg/kg on alternate days to maintain remission.
105
Q

Treatment of steroid resistant NS (SRNS)

  • First Step
A
  • A renal biopsy and screening for genetic disorders should be performed in this setting, as the underlying pathology or a detection of a genetic disorder may affect therapeutic choices.
  • Mutations in the genes that encode for podocyte proteins (NPHS1, NPHS2 and WT1) are detected in 20% of children with non-familial SRNS.
106
Q

Treatment of steroid resistant NS (SRNS)

  • Due to Genetic Disorder
107
Q

Treatment of steroid resistant NS (SRNS)

  • Not Due to Gentic Disorder
A
  • ACEI and ARB are used.
  • Immunosuppressive therapy depends on the underlying pathology.
108
Q

What are Examples of Steroid Sparing Agents?

A
  • Levamisole
  • Cyclophosphamide
  • Cyclosporine
  • Myco-phenolate mofetil
109
Q

Dose of Levamisole

A

2.5 mg/kg on alternate days for 6-31 months.

110
Q

Dose of Cyclophosphamide

A

2 - 3 mg/kg/day for 8 - 12 weeks Oral

111
Q

SE of Levamisole

A

Rare like:
Leukopenia - Vasculitic rash - Liver toxicity.

112
Q

SE of Cyclophosphamide

A
  • Bone marrow suppression
  • Alopecia
  • Infection
  • Hemorrhagic cystitis
  • Gonadal toxicity
113
Q

The development of gonadal toxicity resulting in infertility generally requires a total dose > 200 to 300 mg/kg which exceeds the recommended cumulative dose {168 mg/kg}.

114
Q

SE of Cyclosporine (CsA)

A
  • Prolonged TTT with CA exposes patients to nephrotoxicity.
  • It mandates careful monitoring of renal functions and renal biopsy to assess for evidence of CA induced vasculopathy
114
Q

Dose of Cyclosporine (CsA)

A

4 - 5 mg/kg (100 - 150 mg/m2) for 1 - 3 days

115
Q

Dose of Myco-phenolate mofetil

A

1200 mg/m2 daily for at least 6 months

116
Q

SE of Myco-phenolate mofetil

A

Abdominal pain - Diarrhea - Hematological abnormalities.

117
Q

Prognosis of Nephrotic Syndrome

118
Q

Compare between Nephrotic & Nephritic Syndrome in terms of

  • Strept inf
119
Q

Compare between Nephrotic & Nephritic Syndrome in terms of

  • Edema
120
Q

Compare between Nephrotic & Nephritic Syndrome in terms of

  • Oliguria
121
Q

Compare between Nephrotic & Nephritic Syndrome in terms of

  • HTN
122
Q

Compare between Nephrotic & Nephritic Syndrome in terms of

  • Hematuria
123
Q

Compare between Nephrotic & Nephritic Syndrome in terms of

  • Serum Albumin
124
Q

Compare between Nephrotic & Nephritic Syndrome in terms of

  • Serum Cholesterol
125
Q

Compare between Nephrotic & Nephritic Syndrome in terms of

  • C3 & C4
126
Q

Compare between Nephrotic & Nephritic Syndrome in terms of

  • ASO
127
Q

Compare between Nephrotic & Nephritic Syndrome in terms of

  • Proyeinuria
128
Q

Compare between Nephrotic & Nephritic Syndrome in terms of

  • Casts
129
Q

Compare between Nephrotic & Nephritic Syndrome in terms of

  • Renal Function

Y4R1 - Pediatrics I 👶 (49 decks)

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