NEPHROLOGY SUMMARY Flashcards
What is the most common chronic glomerular disease in children?
Immunoglobulin A Nephropathy (Berger Nephropathy)
What immunoglobulin predominates in mesangial deposits in IgA nephropathy?
IgA
What symptoms are typically seen in IgA nephropathy?
Hematuria, Hypertension, Oliguria
What often precedes IgA nephropathy by 1-2 weeks?
Upper respiratory tract infection (URTI)
What is the status of serum C3 in IgA nephropathy?
Normal
What is the gold standard for diagnosing IgA nephropathy?
Renal biopsy with immunological staining
What are the key biopsy findings in IgA nephropathy?
Focal and segmental mesangial proliferation, increased mesangial matrix, crescent formation, sclerosis
What is the general treatment strategy for IgA nephropathy in children?
Blood pressure control (non-aggressive), dialysis only if required
What genetic mutation causes Alport Syndrome?
COL4A5 (Type IV collagen) mutation
What are the main clinical features of Alport Syndrome?
Microscopic hematuria, progressive proteinuria, bilateral sensorineural hearing loss, ocular abnormalities
What is the inheritance pattern of Alport Syndrome?
X-linked
What ocular abnormality is associated with Alport Syndrome?
Anterior lenticonus
What is the expected progression of Alport Syndrome?
ESRD before 30 years old
What are the key diagnostic criteria for Alport Syndrome?
Hematuria + 2 of: macular flecks, corneal erosions, GBM changes, hearing loss
What electron microscopy findings are seen in Alport Syndrome?
Diffuse thickening, thinning, splitting, layering of GBM and tubular basement membrane
What is the treatment for Alport Syndrome?
No specific therapy, ACE inhibitors/ARBs to slow progression, manage hypertension, anemia, electrolyte imbalances
What is the main finding in Thin Basement Membrane Disease?
Persistent microscopic hematuria
What is the electron microscopy finding in Thin Basement Membrane Disease?
Isolated thinning of GBM
What triggers hematuria in Thin Basement Membrane Disease?
Respiratory tract infections (RTI)
What is the management for Thin Basement Membrane Disease?
Monitor for hypertension, progressive proteinuria, renal insufficiency
What infection precedes Acute Post-Streptococcal Glomerulonephritis (APSGN)?
Throat or skin infection by Group A Beta-hemolytic streptococcus
What is the serum C3 level in APSGN?
Low (normalizes within 6 weeks)
What are the clinical features of APSGN?
Hematuria, oliguria (edema), hypertension
What is the timeline for symptoms after a throat infection in APSGN?
1-2 weeks
What is the timeline for symptoms after a skin infection in APSGN?
3-4 weeks
What are the biopsy findings in APSGN?
Enlarged, bloodless glomeruli, mesangial proliferation, neutrophil infiltration
What are the electron microscopy findings in APSGN?
Electron-dense deposits on epithelial side of GBM
What is the treatment for APSGN?
Blood pressure control, diuresis for edema, 10 days Penicillin for residual infection, monitor C3 and hematuria
What is the most common presentation of Membranoproliferative Glomerulonephritis (MPGN)?
Nephritic syndrome
What are the biopsy findings in Type I MPGN?
Lobular glomeruli, capillary wall thickening, immune complex deposits
What are the biopsy findings in Type II MPGN?
Dense deposit disease, C3 deposits without Ig, positive C3 nephritic factor
What is the serum C3 level in MPGN?
Persistent low C3
What is the prognosis of MPGN?
50% progress to ESRD in 10 years, 90% lose renal function in 20 years
What is the key feature of Rapidly Progressive Glomerulonephritis (RPGN)?
Crescentic glomerulonephritis with rapid progression to ESRD
What are the 3 types of primary RPGN?
Type I: Anti-GBM, Type II: Immune complex-mediated, Type III: Pauci-immune (ANCA+)
What systemic diseases can cause secondary RPGN?
MPGN, IgA nephropathy, HSP nephritis, PSGN, lupus, PAN, hypersensitivity angiitis
What is the treatment for RPGN?
High-dose steroids, cyclophosphamide, plasmapheresis
What are the triad findings in Hemolytic Uremic Syndrome (HUS)?
Microangiopathic hemolytic anemia, thrombocytopenia, renal insufficiency
What triggers HUS?
E. coli, genetic factors, drugs
What are the common symptoms of HUS in children?
Sudden irritability, weakness, lethargy, oliguria 5-10 days after GI or respiratory symptoms
What confirms HUS diagnosis?
Microangiopathic changes, anemia, renal injury; classified as probable (no clear diarrhea) or confirmed (with bloody diarrhea)
What is the treatment for HUS?
Supportive care, avoid platelets and antibiotics at onset
What causes Renal Vein Thrombosis in neonates and infants?
Asphyxia, dehydration, sepsis, maternal diabetes
What causes Renal Vein Thrombosis in older children?
Cyanotic heart disease, nephrotic syndrome, contrast exposure
What are the clinical features of Renal Vein Thrombosis?
Sudden hematuria, unilateral/bilateral flank pain, hypertension, oliguria
What imaging confirms Renal Vein Thrombosis?
Renal enlargement on ultrasound, Doppler confirms thrombosis
What is the treatment for Renal Vein Thrombosis?
Supportive care, fluids/electrolyte correction, surgery, heparin
What genetic mutation causes Autosomal Recessive Polycystic Kidney Disease (ARPKD)?
PKHD1
What protein does PKHD1 encode?
Fibrocystin
What are the key clinical features of ARPKD?
Bilateral flank masses, oligohydramnios, pulmonary hypoplasia, respiratory distress, Porter facies, hypertension, hyponatremia, hepatic fibrosis
What imaging findings are seen in ARPKD?
Enlarged, hyperechogenic kidneys with poor corticomedullary distinction
What is the prognosis for ARPKD?
Poor for severe neonatal cases, 50% develop ESRD in first decade