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Pediatrics > Renal > Flashcards

Renal Flashcards

1
Q

GFR approximates adult values at what age in children

A

3 yr old

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2
Q

Definition of Hematuria

A

presence of more than 5 RBCs/HPF in uncentrifuged urine

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3
Q

rbc result suggestive of underlying pathology in urinalysis

A

10-50 RBCs/uL

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4
Q

significant hematuria

A

> 50 RBCs/HPF

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5
Q

Scenario wherein hematuria becomes false-negative

A

use of formalin
high concentration of ascorbic acid

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6
Q

Scenario wherein hematuria becomes false-positive

A

Alkaline urine (pH >8)
Urine is contaminated with oxidizing agents such as H2O2 used for cleaning perineum

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7
Q

most common cause of GROSS hematuria

A

bacteral/viral UTI

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8
Q

this is considered when a child presents with unexplained perineal bruising and hematuria

A

child abuse

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9
Q

if a patient presents to you with asymptomatic isolated hematuria and normal evaluation, how often do you monitor?

A

every 3 months until hematuria resolves with BP

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10
Q

most common cause of chronic glomerular disease in children

A

Immunoglobulin A nephropathy/ Berger nephropathy

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11
Q

True or false: IgA nephropathy is commonly seen in female than in male?

A

False
IgA nephropathy is seen more often in male

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12
Q

how long does gross hematuria in IgA nephropathy presents after an upper respiratory or GI infection?

A

within 1-2 days
- this differentiates the latency period observed in post infectious glomerulonephritis which presents after 4-6 weeks

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13
Q

test to distinguish IgA nephropathy from postinfectious glomerulonephritis

A

Normal serum C3

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14
Q

poor prognostic indicators at prsenetation or follow up in IgA nephropathy

A

Persistent hypertension
Diminished renal function
Significant/increasing/prolonged proteinuria

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15
Q

how to diagnose IgA nephropathy

A

thru Renal biopsy

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16
Q

primary treatment of IgA nephropathy

A

appropriate blood pressure control - using ACEi or ARA
management of significant proteinuria - use of immunosuppresive therapy with corticosteroids

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17
Q

other term for heridetary nephritis

A

Alport Syndrome

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18
Q

type of protein mutated in Alport Syndrome

A

Type IV collagen
- major component of basement membrane

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19
Q

Presentation of all patients with Alport syndrome

A

Asymptomatic microscopic hematuria

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20
Q

An abnormality seen in Alport syndrome which is never congenital

A

Bilateral sensorineural hearing loss
- 90% of hemizygous males with X-linked AS
- 10% of heterozygous females with X-linked AS
- 67% with autosomal recessive AS

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21
Q

pathognomonic ophthalmic finding in Alport Syndrome

A

Anterior lenticonus

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22
Q

Features supportive of Alport syndrome (at least 2)

A

Macular flecks
Recurrent corneal erosions
GBM thickening and thinning
Sensorineural deafness

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23
Q

How frequent are Ocular abnormalities seen in X-linked Alport Syndrome?

A

30-40%

Ocular abnormalities include anterior lenticonus, macular flecks and corneal erosions

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24
Q

Presentation of Thin Basement Membrane Disease

A

Persistent Microscopic Hematuria
Isolated thinning of the GBM

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25
Q

This type of infection leads to postinfectious glomerulonephritis

A

Group A beta-hemolytic streptococcal infection

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26
Q

presentation of acute nephritic syndrome

A

gross hematuria
edema
hypertension
renal dysfunction

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27
Q

characteristic of immunofluorescence microscopy seen in Acute poststreptococcal glomerulonephritis

A

patter of “lumpy-bumpy” deposits of immunoglobulin and complement on the GBM and in mesangium

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28
Q

This type of protein has nephritogenic strains that cause APSGN

A

M proteins

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29
Q

Poststreptococcal GN is most common in what age group, and uncommon to which age group

A

common in children 5-12 years old
uncommon <3 yr old

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30
Q

onset of PSGN
after pharyngitis: _____
after pyoderma: _____

A

1-2 weeks after pharyngitis
3-6 weeks after pyoderma

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31
Q

What is the serum C3 level in APSGN?

A

Significantly reduced in >90% in the acute phase
- returns to normal 6-8 weeks after the onset

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32
Q

recommended antibiotic treatment for ASPGN

A

10 days of Penicillin
- limits the spread of nephritogenic organisms
- although this does not affect the natural history of APSGN

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33
Q

Best characterized and recognized causes of Membranous nephropathy in children

A

Chronic Hepatitis B infection
Congenital Syphilis

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34
Q

Membranoproliferative glomerulonephritis (MPGN) or mesangiocapillary glomerulonephritis is most commonly associated with what type of infection

A

Hepatitis B and C
Syphilis
Subacute bacterial endocarditis
Infected shunts

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35
Q

Known as the dense deposit disease

A

Type II MPGN

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36
Q

type of MPGN in which the C3 nephritic factor is present and appears not to be mdiated by immune complexes

A

Type II

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37
Q

Most common in the second decade of life

A

Membranoproliferative glomerulonephritis

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38
Q

natural history of most forms of CGN

A

rapid loss of the renal function

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39
Q

this has histopathologic finding of epithelial crescents involving 50% or more glomeruli

A

CGN

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40
Q

presentation of nephrotic syndrome

A

proteinuria
edema
hypoalbuminemia

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41
Q

classic histopathologic lesion of HIV-associated nephropathy

A

focal segmental glomerulosclerosis

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42
Q

most important cause of morbidity and mortality in.SLE

A

Lupus nephritis

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43
Q

diagnostic/characteristic of Lupus Nephritis

A

granular deposition of all Ig isotypes (IgG, IgM, IgA) and complements (C3, C4, C1q) in the glomerular mesangium and capillary walls

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44
Q

SLE is common in what age group

A

adolescent, usually females (5:1 ratio)

45
Q

remains the cornerstone of therapy in children with SLE

A

Immunosuppression

46
Q

Initial therapy of patients with SLE

A

Prednisone at 1-2 mg/kg/day, followede by slow steroid taper over 4-6 months beginning 4-6 weeks after achieving serologic remission

47
Q

what is meant by clinical remission in lupus nephritis-SLE?

A

normalization of renal function and proteinuria

48
Q

alternative of cyclophosphamide in treatment of lupus nephritis

A

Mycophenolate mofetil or Azathioprine at 1.5-2 mg/kg single dose OD

49
Q

major side effects of mycophenolate mofetil

A

Diarrhea
Leukopenia
Teratogenicity

50
Q

risk factors for progression to end-stage renal disease in lupus nephritis

A

Diffuse Class IV lupus nephritis
Poor renal function at presentation or
persistent nephrotic-range proteinuria

51
Q

known as IgA vasculitis

A

Henoch-Schönlein Purpura Nephritis

52
Q

most common small-vessel vasculitis in children

A

HSP

53
Q

Enumerate the s/sx of HSP/classic tetrad of HSP

A

Palpable purpuric rash
Arthritis/arthralgia
Abdominal pain
Evidence of renal disease

54
Q

indications for a kidney biopsy in HSP nephritis

A

Significant proteinuria (urine protein >1g/day or urine protein/creatinine ratio >1.0)
Significant Hypertension
Elevated serum creatinine

55
Q

does mild HSP nephritis need treatment?

A

no because it resolves spontaneously

56
Q

S/Sx of Goodpasture Disease

A

Pulmonary Hemorrhage
Rapidly progressive glomerulonephritis
Elevated Anti GBM antibody titers

57
Q

In goodpasture disease, what is the pathognomonic findings in immunofluorescence microscopy?

A

continuous linear deposition of IgG along the GBM

58
Q

Most common form of thrombotic microangiopathy (TMA) in children

A

Hemolytic-Uremic Syndrome

59
Q

This causes the most common form of HUS

A

Shiga toxin-producing Escherichia coli (STEC)

60
Q

STEC-HUS is commonly transmitted via?

A

undercooked meat or unpasteurized (raw) milk and apple cider

61
Q

A major feature characteristic of genetic forms of HUS

A

absence of a preceding diarrheal prodrome

62
Q

Diagnosis of HUS

A

Combination of microangiopathic hemolytic anemia with schistocytes
Thrombocytopenia
Kidney involvement

++ with the presence of a diarrheal prodrome or pneumococal infection

63
Q

Why are platelets not be administered in HUS?

A

because they are rapidly consumed by the active coagulation and theoretically can worsen the clinical course

64
Q

FDA approved for the treatment of atypical HUS

A

Eculizumab

65
Q

most common cause of intrinstic acute kidney injury (AKI)

A

Acute Tubular Necrosis

66
Q

Nephrotic range proteinuria

A

> 40 mg/m2/hr

67
Q

ratio of urine-protein creatinine which is suggestive of nephrotic-range proteinuria

A

greater than 2

68
Q

most common cause of persistent proteinuria in school-age children and adolescents

A

Orthostatic (Postural) proteinuria

69
Q

This confirms the diagnosis of orthostatic proteinuria

A

absence of proteinuria (dipstick negative/trace, normal UPCR) on the first morning urine sample for 3 consecutive days

70
Q

possible causes of Orthostatic proteinuria

A
  • altered renal hemodynamics
  • partial left renal vein obstruction in the upright lordotic position
  • increased BMI
71
Q

this is the predominant cell of injury in most glomerular diseases characterized by heavy proteinuria

A

podocyte

72
Q

triad of clinical findings associated with nephrotic syndrome

A

Hypoalbuminemia (<2.5 g/dL)
Edema
Hyperlipidemia (cholesterol >200 mg/dL)

73
Q

standard therapy for nephrotic syndrome

A

glucocorticoid therapy

74
Q

why are children with nephrotic syndrome susceptible to infections?

A

because of hypoglobulinemia, defects in the complement cascade — resulting from urinary losses of IgG, complement factors (C3, C5) leading to impaired opsonization of microorganisms

75
Q

what particular type of infection is children with NS susceptible with?

A

infectiopn with encapsulated bacteria/ pneumococcal disease

76
Q

90% of children with NS have this type of NS

A

Idiopathic nephrotic syndrome

77
Q

85% of total cases of nephrotic syndrome in children

A

Minimal change nephrotic syndorme

78
Q

most common cause of end-stage renal disease in adolescents

A

Focal segmental glomerulosclerosis

79
Q

MCNS most commonly appears in what age group

A

2 - 6 yr

80
Q

mainstay therapy for MCNS

A

Corticosteroids

81
Q

definition of response in the treatment of MCNS

A

attaintment of remission (UPCR <0.2 or <1+ protein on urine dipstick testing for 3 consecutive days) within the initial 4 weeks of corticosteroid therapy

82
Q

Dyslipidemia in children with NS should be managed with

A

low-fat diet

83
Q

Definition of relapse in nephrotic syndrome

A

UPCR of >2 or >=3+ protein on urine dipstick testing for 3 consecutive days

84
Q

how many percentage is the risk for ESKD for children with steroid resistant nephrotic syndrome

A

50% risk within 5 years of diagnosis

85
Q

recommended initial theraoy for children with steroid-resistant nephrotic syndrome

A

Calcineurin inhibitors such as cyclosporine or tacrolimus

86
Q

Vaccines to be given in Nephrotic syndrome

A
  • Pneumococcal vaccine (13 valent conjugant, 23 valent polysaccharide)
  • Influenza
    > defer live vaccination until prednisone dose <1 mg/kg daily or 2 mg/kg on alternate days
87
Q

treatment period for Nephrotic syndrome

A

at least 12 week of steroid treatment

  • single daily dose of 60 mg/m2/day or 2 mkday, max of 60 mg OD for 4-6 weeks followed by alternate day prednisone for 8 weeks to 5 months
88
Q

This is caused by mutations in the WT1 gene, resulting in abnormal podocyte function

A

Denys-Drash Syndrome

89
Q

Mainstream therapy for congenital nephrotic syndrome

A

-Albumin and diuretic infusion
- High amount of protein (3-4 g/kg), lipids
- High caloric intake
- Vitamin and thyroid hormone replacement

90
Q

Definitive treatment of congenital nephrotic syndrome

A

Renal transplantation

91
Q

Enumerate the 4 main types of RTA

A

Type I: Classic Distal
Type II: Proximal RTA
Type III: Combined
Type IV: Hyperkalemic RTA

92
Q

rare X linked disorder characterized by congenital cataracts, mental retardation and Fanconi syndrome

A

Lowe Syndrome

93
Q

presentation of Fanconi Syndrome

A

-low molecular weight proteinuria
- glycosuria
- phosphaturia
- aminoaciduria
- PROXIMAL RTA

94
Q

Electrolyte imbalance seen in distal Type I RTA

A

Hypokalemia
Hypercalciuria

95
Q

distinguishing features of distal RTA

A

Nephrocalcinosis
Hypercalciuria

96
Q

differentiate urine pH of proximal and distal RTA

A

pH <5.5 in proximal RTA and pH >6.0 in distal RTA

97
Q

Formula of serum anion gap and urine anion gap

A

Serum: [Na] - [Cl + HCO3]
Urine: [Na + K ] - Cl

98
Q

Mainstay of therapy in all forms of RTA

A

bicarbonate replacement

99
Q

defined as an abrupt loss of kidney function leading to a rapid decline in the glomerular filitrate rate (GFR), accumulation of waste prodcuts such as BUN and creatinine, dysregulation of extracellular volume and electrolyte homeostasis

A

Acute Kidney Injury

100
Q

UO of 0.5 cc/kg/hr for 6-12 hour: What stage in the KDIGO staging of AKI

A

Stage I

101
Q

anuria for >12 hour: what stage in the KDIGO staging of AKI

A

Stage III

102
Q

Definition of AKI

A

Increase in serum crea by >0.3 mg/dL from baseline within 48 hour or
increase in serum crea to >1.5x baseline within the prior 7 days or
Urine volume <0.5 cc/kg/hr for 6 hr

103
Q

Indications for dialysis in AKI

A

-Anuria/oliguria
-Vol overload with evidence of hypertension and/or pulmonary edema refractory to diuretic therapy
-Persistent hyperkalemia
-Severe metabolic acidosis unresponsive to medical management
-Uremia (encephalopathy, pericarditis, neuropathy)
-Calcium:phosphorus imbalance

104
Q

Leading causes of death in childhood onset ESRD

A

Infectious diseases
Cardiovascular cause

105
Q

Criteria for definition of CKD

A

Either of the ff:
1. Kidney damage > 3 months, defined as structural or functional abnormalities of the kidney, with or without decreased eGFR
— abnormalities in the composition of the blood or urine
— abnormalities in imaging tests
— abnormalities on kidney biopsy

  1. GFR <60 for 3 months, with/without other signs of kidney damage
106
Q

primary determinant of CKD progression

A

Progression of tubulointerstitial fibrosis

107
Q

gold standard of BP evaluation

A

Ambulatory Blood Pressure monitoring over 24 hours

108
Q

cause of hypertension in patients with CKD

A

Volume overload or excessive production of renin

Pediatrics (17 decks)

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