PEDIATRIC NEPHROLOGY 1.1 (AB) Flashcards

1
Q

Where is the glomerulus located?

A

Retroperitoneal

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2
Q

What structures are found in the outer layer (cortex) of the kidney?

A

Glomerulus

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3
Q

What structures are found in the inner layer (medulla) of the kidney?

A

Loops of Henle

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4
Q

How many nephrons are present at birth?

A

Approximately 1 million nephrons

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5
Q

When is nephron formation complete?

A

34-36 weeks gestation

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6
Q

Can new nephrons form after birth?

A

No

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7
Q

What are risk factors for reduced nephron number?

A

Low birth weight

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8
Q

What conditions can reduced nephron number lead to?

A

Primary hypertension

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9
Q

What is the role of mesangial cells and matrix?

A

They provide structural support for the glomerular capillary

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10
Q

What structure surrounds the glomerulus?

A

Bowman’s capsule

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11
Q

What is the best parameter to describe kidney function?

A

Glomerular filtration rate (GFR)

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12
Q

What types of molecules filter freely in the glomerulus?

A

Electrolytes

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13
Q

What types of molecules are retained in circulation by the glomerulus?

A

Albumin and globulins

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14
Q

What is the significance of albumin in urine?

A

It is almost always pathologic and indicates potential glomerular damage

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15
Q

What laboratory parameter is affected by hydration and nitrogen balance?

A

Blood Urea Nitrogen (BUN)

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16
Q

What is BUN a product of?

A

Protein breakdown

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17
Q

What is creatinine derived from?

A

Muscle metabolism

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18
Q

How is creatinine production described?

A

Steady and relatively constant

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19
Q

What happens to creatinine in renal insufficiency?

A

Serum creatinine rises because it is not excreted properly

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20
Q

What formula is used to estimate GFR in pediatric patients?

A

Schwartz formula

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21
Q

What is the Schwartz formula?

A

eGFR = 0.413 x height (cm) / serum creatinine (mg/dL)

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22
Q

What is the creatinine clearance for a 10-year-old male who is 144 cm tall with a creatinine of 0.56 mg/dL?

A

eGFR = 0.413 x 144 / 0.56 = 106.2 mL/min/1.73 m²

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23
Q

What does glomerular disease affect?

A

The glomerulus

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24
Q

What are potential causes of glomerular disease?

A

Genetic

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25
What causes glomerulonephritis?
Immunologic injury to the glomerulus
26
What is the definition of hematuria?
Presence of more than 5 RBC/HPF
27
What are benign causes of transient hematuria?
Fever
28
How many RBC/uL suggest underlying pathology?
10-50 RBC/uL
29
What level of RBC/HPF is considered significant hematuria?
More than 50 RBC/HPF
30
What substances can cause false positive hematuria?
Ascorbic acid >2000 mg/day
31
What are common causes of upper urinary tract disease?
IgA nephropathy
32
What are multisystem diseases that can cause hematuria?
SLE nephritis
33
What are examples of tubulointerstitial diseases?
Pyelonephritis
34
What vascular disorders can cause hematuria?
Arterial/venous thrombosis
35
What anatomic disorders can cause hematuria?
Hydronephrosis
36
What lower urinary tract diseases can cause hematuria?
Cystitis
37
What condition was formerly known as Munchausen syndrome by proxy?
Factitious syndrome by proxy
38
Which glomerulonephritides present with hypocomplementemia?
Postinfectious GN
39
What percentage of children with acute or chronic hematuria is associated with systemic illness?
0.1
40
What is the most common chronic glomerular disease in children?
Immunoglobulin A Nephropathy (Berger Nephropathy)
41
Where are IgA deposits found in Berger nephropathy?
Mesangial glomerular deposits
42
How is Berger nephropathy diagnosed?
Renal biopsy with immunologic staining
43
What is the triad presentation of nephropathy/nephritis?
Hematuria
44
What condition presents with the same abnormalities as Henoch-Schönlein purpura nephritis?
Immunoglobulin A Nephropathy (Berger Nephropathy)
45
What is the typical preceding event 1-2 weeks before the onset of IgA nephropathy?
Upper Respiratory Tract Infection (URTI)
46
What is the serum C3 level in IgA nephropathy?
Normal
47
What is the main treatment goal for all nephritis?
Appropriate blood pressure control
48
When is dialysis indicated for nephritis in children?
Only if absolutely required
49
What gene mutation is responsible for 85% of Alport Syndrome cases?
COL4A5 gene
50
What type of collagen is affected in Alport Syndrome?
Type IV collagen
51
What is the hallmark microscopic hematuria finding in Alport Syndrome?
Persistent asymptomatic microscopic hematuria
52
In Alport Syndrome
when does proteinuria typically worsen in boys?
53
What type of hearing loss occurs in Alport Syndrome?
Bilateral sensorineural hearing loss (non-congenital)
54
What is the pathognomonic ocular abnormality in Alport Syndrome?
Anterior lenticonus
55
What are key diagnostic steps for Alport Syndrome?
Family history
56
What are the microscopy findings in Alport Syndrome?
Mesangial proliferation
57
What is seen on electron microscopy in Alport Syndrome?
Diffuse thickening
58
What medications can slow the progression of Alport Syndrome?
ACE inhibitors and ARBs
59
At what age does ESRD typically occur in Alport Syndrome?
Before 30 years old
60
What condition is characterized by persistent microscopic hematuria and isolated GBM thinning?
Thin Basement Membrane Disease
61
What triggers hematuria in Thin Basement Membrane Disease?
Respiratory tract infections
62
What are the key monitoring parameters in Thin Basement Membrane Disease?
Hypertension
63
What type of infection often precedes Acute Post-Streptococcal Glomerulonephritis (APSGN)?
Throat (1-2 weeks) or skin (3-4 weeks) infection
64
What pathogen causes Acute Post-Streptococcal Glomerulonephritis?
Group A beta-hemolytic streptococci
65
What is the age range most commonly affected by APSGN?
5-12 years old
66
What type of immune reaction causes APSGN?
Immune complex mediated
67
What happens to serum C3 in APSGN?
Low
68
What is the triad presentation of APSGN?
Hematuria
69
What causes oliguria and edema in APSGN?
Inflammation leading to fluid retention
70
What is the first-line medication for hypertension in APSGN?
Furosemide (loop diuretics)
71
What dietary restriction helps manage APSGN?
Sodium restriction
72
What antibiotic is given for 10 days to treat residual streptococcal infection in APSGN?
Penicillin
73
How long should microscopic hematuria be monitored in APSGN?
12-24 months
74
How soon should C3 normalize after APSGN onset?
Within 6 weeks
75
What is the histologic hallmark of Membranoproliferative Glomerulonephritis (MPGN) Type I?
Lobular pattern and capillary wall thickening
76
What is the characteristic finding in Type II MPGN (Dense Deposit Disease)?
C3 immunofluorescence without Ig
77
What autoantibody is found in Type II MPGN?
C3 nephritic factor
78
How is MPGN diagnosed?
Renal biopsy and persistently low C3
79
What is the prognosis of MPGN 10 years after onset?
50% progress to ESRD
80
What is the prognosis of MPGN 20 years after onset?
90% progress to ESRD
81
What is another name for Rapidly Progressive Glomerulonephritis (RPGN)?
Crescentic glomerulonephritis
82
What is the defining feature of RPGN?
Rapid progression to ESRD
83
What is the biopsy hallmark of RPGN?
Crescents overlying the glomerulus
84
What is the classification of Primary RPGN Type I?
Anti-GBM antibody disease (e.g.
85
What is the classification of Primary RPGN Type II?
Immune complex mediated
86
What is the classification of Primary RPGN Type III?
Pauci-immune (ANCA-positive)
87
What systemic diseases can cause Secondary RPGN?
SLE
88
What are the main diagnostic tools for RPGN?
Clinical evaluation
89
What is the main treatment for RPGN?
High-dose steroids and cyclophosphamide
90
What adjunct treatment can be used in severe RPGN cases?
Plasmapheresis