NEPHROLOGY SUMMARY Flashcards

1
Q

What is the most common chronic glomerular disease in children?

A

Immunoglobulin A Nephropathy (Berger Nephropathy)

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2
Q

What immunoglobulin predominates in mesangial deposits in IgA nephropathy?

A

IgA

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3
Q

What symptoms are typically seen in IgA nephropathy?

A

Hematuria, Hypertension, Oliguria

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4
Q

What often precedes IgA nephropathy by 1-2 weeks?

A

Upper respiratory tract infection (URTI)

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5
Q

What is the status of serum C3 in IgA nephropathy?

A

Normal

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6
Q

What is the gold standard for diagnosing IgA nephropathy?

A

Renal biopsy with immunological staining

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7
Q

What are the key biopsy findings in IgA nephropathy?

A

Focal and segmental mesangial proliferation, increased mesangial matrix, crescent formation, sclerosis

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8
Q

What is the general treatment strategy for IgA nephropathy in children?

A

Blood pressure control (non-aggressive), dialysis only if required

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9
Q

What genetic mutation causes Alport Syndrome?

A

COL4A5 (Type IV collagen) mutation

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10
Q

What are the main clinical features of Alport Syndrome?

A

Microscopic hematuria, progressive proteinuria, bilateral sensorineural hearing loss, ocular abnormalities

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11
Q

What is the inheritance pattern of Alport Syndrome?

A

X-linked

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12
Q

What ocular abnormality is associated with Alport Syndrome?

A

Anterior lenticonus

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13
Q

What is the expected progression of Alport Syndrome?

A

ESRD before 30 years old

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14
Q

What are the key diagnostic criteria for Alport Syndrome?

A

Hematuria + 2 of: macular flecks, corneal erosions, GBM changes, hearing loss

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15
Q

What electron microscopy findings are seen in Alport Syndrome?

A

Diffuse thickening, thinning, splitting, layering of GBM and tubular basement membrane

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16
Q

What is the treatment for Alport Syndrome?

A

No specific therapy, ACE inhibitors/ARBs to slow progression, manage hypertension, anemia, electrolyte imbalances

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17
Q

What is the main finding in Thin Basement Membrane Disease?

A

Persistent microscopic hematuria

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18
Q

What is the electron microscopy finding in Thin Basement Membrane Disease?

A

Isolated thinning of GBM

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19
Q

What triggers hematuria in Thin Basement Membrane Disease?

A

Respiratory tract infections (RTI)

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20
Q

What is the management for Thin Basement Membrane Disease?

A

Monitor for hypertension, progressive proteinuria, renal insufficiency

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21
Q

What infection precedes Acute Post-Streptococcal Glomerulonephritis (APSGN)?

A

Throat or skin infection by Group A Beta-hemolytic streptococcus

22
Q

What is the serum C3 level in APSGN?

A

Low (normalizes within 6 weeks)

23
Q

What are the clinical features of APSGN?

A

Hematuria, oliguria (edema), hypertension

24
Q

What is the timeline for symptoms after a throat infection in APSGN?

25
What is the timeline for symptoms after a skin infection in APSGN?
3-4 weeks
26
What are the biopsy findings in APSGN?
Enlarged, bloodless glomeruli, mesangial proliferation, neutrophil infiltration
27
What are the electron microscopy findings in APSGN?
Electron-dense deposits on epithelial side of GBM
28
What is the treatment for APSGN?
Blood pressure control, diuresis for edema, 10 days Penicillin for residual infection, monitor C3 and hematuria
29
What is the most common presentation of Membranoproliferative Glomerulonephritis (MPGN)?
Nephritic syndrome
30
What are the biopsy findings in Type I MPGN?
Lobular glomeruli, capillary wall thickening, immune complex deposits
31
What are the biopsy findings in Type II MPGN?
Dense deposit disease, C3 deposits without Ig, positive C3 nephritic factor
32
What is the serum C3 level in MPGN?
Persistent low C3
33
What is the prognosis of MPGN?
50% progress to ESRD in 10 years, 90% lose renal function in 20 years
34
What is the key feature of Rapidly Progressive Glomerulonephritis (RPGN)?
Crescentic glomerulonephritis with rapid progression to ESRD
35
What are the 3 types of primary RPGN?
Type I: Anti-GBM, Type II: Immune complex-mediated, Type III: Pauci-immune (ANCA+)
36
What systemic diseases can cause secondary RPGN?
MPGN, IgA nephropathy, HSP nephritis, PSGN, lupus, PAN, hypersensitivity angiitis
37
What is the treatment for RPGN?
High-dose steroids, cyclophosphamide, plasmapheresis
38
What are the triad findings in Hemolytic Uremic Syndrome (HUS)?
Microangiopathic hemolytic anemia, thrombocytopenia, renal insufficiency
39
What triggers HUS?
E. coli, genetic factors, drugs
40
What are the common symptoms of HUS in children?
Sudden irritability, weakness, lethargy, oliguria 5-10 days after GI or respiratory symptoms
41
What confirms HUS diagnosis?
Microangiopathic changes, anemia, renal injury; classified as probable (no clear diarrhea) or confirmed (with bloody diarrhea)
42
What is the treatment for HUS?
Supportive care, avoid platelets and antibiotics at onset
43
What causes Renal Vein Thrombosis in neonates and infants?
Asphyxia, dehydration, sepsis, maternal diabetes
44
What causes Renal Vein Thrombosis in older children?
Cyanotic heart disease, nephrotic syndrome, contrast exposure
45
What are the clinical features of Renal Vein Thrombosis?
Sudden hematuria, unilateral/bilateral flank pain, hypertension, oliguria
46
What imaging confirms Renal Vein Thrombosis?
Renal enlargement on ultrasound, Doppler confirms thrombosis
47
What is the treatment for Renal Vein Thrombosis?
Supportive care, fluids/electrolyte correction, surgery, heparin
48
What genetic mutation causes Autosomal Recessive Polycystic Kidney Disease (ARPKD)?
PKHD1
49
What protein does PKHD1 encode?
Fibrocystin
50
What are the key clinical features of ARPKD?
Bilateral flank masses, oligohydramnios, pulmonary hypoplasia, respiratory distress, Porter facies, hypertension, hyponatremia, hepatic fibrosis
51
What imaging findings are seen in ARPKD?
Enlarged, hyperechogenic kidneys with poor corticomedullary distinction
52
What is the prognosis for ARPKD?
Poor for severe neonatal cases, 50% develop ESRD in first decade