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
Q

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

26
Q

What are the biopsy findings in APSGN?

A

Enlarged, bloodless glomeruli, mesangial proliferation, neutrophil infiltration

27
Q

What are the electron microscopy findings in APSGN?

A

Electron-dense deposits on epithelial side of GBM

28
Q

What is the treatment for APSGN?

A

Blood pressure control, diuresis for edema, 10 days Penicillin for residual infection, monitor C3 and hematuria

29
Q

What is the most common presentation of Membranoproliferative Glomerulonephritis (MPGN)?

A

Nephritic syndrome

30
Q

What are the biopsy findings in Type I MPGN?

A

Lobular glomeruli, capillary wall thickening, immune complex deposits

31
Q

What are the biopsy findings in Type II MPGN?

A

Dense deposit disease, C3 deposits without Ig, positive C3 nephritic factor

32
Q

What is the serum C3 level in MPGN?

A

Persistent low C3

33
Q

What is the prognosis of MPGN?

A

50% progress to ESRD in 10 years, 90% lose renal function in 20 years

34
Q

What is the key feature of Rapidly Progressive Glomerulonephritis (RPGN)?

A

Crescentic glomerulonephritis with rapid progression to ESRD

35
Q

What are the 3 types of primary RPGN?

A

Type I: Anti-GBM, Type II: Immune complex-mediated, Type III: Pauci-immune (ANCA+)

36
Q

What systemic diseases can cause secondary RPGN?

A

MPGN, IgA nephropathy, HSP nephritis, PSGN, lupus, PAN, hypersensitivity angiitis

37
Q

What is the treatment for RPGN?

A

High-dose steroids, cyclophosphamide, plasmapheresis

38
Q

What are the triad findings in Hemolytic Uremic Syndrome (HUS)?

A

Microangiopathic hemolytic anemia, thrombocytopenia, renal insufficiency

39
Q

What triggers HUS?

A

E. coli, genetic factors, drugs

40
Q

What are the common symptoms of HUS in children?

A

Sudden irritability, weakness, lethargy, oliguria 5-10 days after GI or respiratory symptoms

41
Q

What confirms HUS diagnosis?

A

Microangiopathic changes, anemia, renal injury; classified as probable (no clear diarrhea) or confirmed (with bloody diarrhea)

42
Q

What is the treatment for HUS?

A

Supportive care, avoid platelets and antibiotics at onset

43
Q

What causes Renal Vein Thrombosis in neonates and infants?

A

Asphyxia, dehydration, sepsis, maternal diabetes

44
Q

What causes Renal Vein Thrombosis in older children?

A

Cyanotic heart disease, nephrotic syndrome, contrast exposure

45
Q

What are the clinical features of Renal Vein Thrombosis?

A

Sudden hematuria, unilateral/bilateral flank pain, hypertension, oliguria

46
Q

What imaging confirms Renal Vein Thrombosis?

A

Renal enlargement on ultrasound, Doppler confirms thrombosis

47
Q

What is the treatment for Renal Vein Thrombosis?

A

Supportive care, fluids/electrolyte correction, surgery, heparin

48
Q

What genetic mutation causes Autosomal Recessive Polycystic Kidney Disease (ARPKD)?

49
Q

What protein does PKHD1 encode?

A

Fibrocystin

50
Q

What are the key clinical features of ARPKD?

A

Bilateral flank masses, oligohydramnios, pulmonary hypoplasia, respiratory distress, Porter facies, hypertension, hyponatremia, hepatic fibrosis

51
Q

What imaging findings are seen in ARPKD?

A

Enlarged, hyperechogenic kidneys with poor corticomedullary distinction

52
Q

What is the prognosis for ARPKD?

A

Poor for severe neonatal cases, 50% develop ESRD in first decade