Glomerulonephritis in children

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Glomerulonephritis in children

• Pavlyshyn H.A.

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Definition

• Acute glomerulonephritis is the inflammation of
  the glomeruli which causes the kidneys to
  malfunction
• It is also called Acute Nephritis,
  Glomerulonephritis and Post-Streptococcal
  Glomerulonephritis
• Predominantly affects children from ages 2 to 12
• Incubation period is 2 to 3 weeks

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Autoimmune Reactions
Some progress as either focal segmental
glomerulosclerosis or tubulointerstitial nephritis
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Possible Clinical Manifestations

• Proteinuria asymptomatic
• Haematuria asymptomatic
• Hypertension
• Nephrotic syndrome
• Nephritic syndrome
• Acute renal failure
• Rapidly progressive renal failure
• End stage renal failure

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Presentation

• Hematuria
• with Proteinuria
• with Dysmorphic rbcs
• with Rbc casts
• Oliguria
• Volume overload
• Hypertension

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Liquid Renal Biopsy
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Urine Sediment Analysis
G4 cell
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Other HP findings

• Neurological changes
• Pharyngitis
• URI / sinusitis
• Hemoptysis
• Rash
• Murmur
• Arthritis
• Edema

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Complement Abnormalities
Ab-Ag complexes
Classical pathway
C3 convertase
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Differential Diagnosis

• Hypocomplementemia
• PIGN
• MPGN
• SLE
• Cryoglobulinemia
• Bacterial Endocarditis
• Shunt nephritis

• Normal complement
• HUS
• IgAN
• HSP
• Alports / TBMD

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b-hemolytic Streptococci

• Most common organism in PIGN
• 20 children are asymptomatic carriers
• Nephritic factor
• Host susceptibility factors (HLA-DR)
• Treatment of prodromal illness doesnt prevent
  nephritis
• ASO titers are NOT helpful

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Post Infectious GN

• Pathogenesis
• Strep antigens trigger antibodies that
  cross-react to glomeruli
• Circulating immune complexes get filtered by
  glomerulus get stuck
• Immune complexes activate complement
• Diffuse generalized damage to glomeruli
• ? GFR due to inflammation, damage to BM
• ? RBF in proportion to GFR, so filtration
  fraction normal
• Tubular function is preserved
• Plasma renin and aldosterone are normal
• Presentation
• 7-14 days after pharyngitis
• 14-21 days after impetigo (upto 6 wks)
• Abrupt onset

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Manifestations of PIGN

• Edema 85
• HTN 60-80
• Gross hematuria 25-33
• CNS (i.e. Sz) 10
• Nephrotic syndrome rare
• ARF not uncommon
• C3 decreased
• C4 typically normal

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Management of PIGN

• Antibiotics do NOT prevent GN
• Sodium Fluid restriction
• Antihypertensives, diuretics for HTN
• Dialysis if necessary
• Prognosis usually excellent
• 0.5 mortality due to pulmonary edema or
  pneumonia
• lt1 progress to CKD stage 5
• Follow-up
• Gross hematuria resolves within 2 weeks
• Complement low for 6-8 weeks
• Proteinuria remains upto 6 months
• Hematuria remains upto 2 years

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Renal Biopsy
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Histopathology

• Diffuse all glomeruli
• Generalized all segments of glomeruli

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IgG Immunofluorescence
Starry Sky Pattern
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Electron microscopy - Normal
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Electron microscopy of PIGN

• Subepithelial immune deposits (humps)
• Mesangial, subendothelial, intramembranous
  deposits less common
• Effacement of foot processes

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Hemolytic Uremic Syndrome

• 2 cases/100,000 annually
• Peak incidence lt5yo (6/100,000)
• More common June-September
• Classification
• D diarrhea associated
• Strep pneumo
• Atypical HUS ADAM-TS13, C1q def

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Presentation of D HUS

• Prodromal acute gastroenteritis
• Shiga toxin producing E.coli O157H7
• Transmission from beef, veggies, direct
  person-to-person, and contaminated water all
  reported
• Incubation period 3-4 days
• Bloody diarrhea 2-3 days after cramping begins
• 50 with emesis, afebrile or low grade fever only
• Hemolytic anemia
• Thrombocytopenia
• ARF
• Begins 2-14 days after diarrhea
• CNS disease
• Overlap with ITP in 33 HUS cases
• Somnolence, confusion, seizures, coma

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Microangiopathic Hemolytic Anemia
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Henoch Schönlein Purupura
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Henoch Schönlein Purupura

• GI tract
• Cramping, vomiting, diarrhea
• Skin rash
• Lower extremities, buttocks
• Joint involvement
• HSP nephritis
• Incidence 20-50
• In 80, occurs within 4 weeks of rash GI upset
• In 15, occurs upto 1-3 months after rash GI
  upset

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Pathogenesis of Alports

• Abnormality of type IV collagen
• Disordered basement membrane
• Splitting of lamina densa of GBM

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Crescentic GN
Type Serology Primary Secondary
I Anti-GBM ANCA- Anti-GBM disease Goodpastures
II Anti-GBM- ANCA- idiopathic SLE, IgAN, MPGN
III Anti-GBM- ANCA Microscopic polyangiitis, Wegeners Drug-induced
IV Anti-GBM ANCA Anti-GBM disease Goodpastures
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Vasculitides

• C-ANCA

P-ANCA
Anti-proteinase 3 antibodies
Anti-myeloperoxidase antibodies
75 sensitive for Wegeners
66 sensitive for Microscopic polyangiitis
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Anti-GBM Disease
Silver stain
IgG immunofluorescence