Nephrotic nephritic syndroms usmle notes
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www.brain101.info 1 NEPHROTIC and NEPHRITIC SYNDROMES Disease Most Frequent Clinical Presentation Pathogenesis Light Microscope F.M. (Fluorescence Microscope) E.M. (Electron Microscope) Age Group Affected Treatment and Outcome Minimal Change Disease (Lipoid Nephrosis) • Selective proteinuria (Albumin) • Loss of foot processes • Loss of GBM polyanionic sites • Appearance of villi on epithelial cells Normal Lipid in tubules F.M. = negative E.M. = loss of foot processes, lipid vacuoles #1 cause of Nephrotic Syndrome in children, esp. boys younger than 6 yrs. old. Responds well to corticosteroids. No progression into chronic renal failure Focal Segmental Glomerular Sclerosis • Non-selective proteinuria • Hypertension • Microscopic hematuria • Idiopathic • Lower renal mass (in obese) • 2 causes: heroin use, HIV • Focal and segmental sclerosis • Hyalinosis • Adhesions to Bowman's Capsule • Hypercellular mesangium • Thick B.M. F.M. = IgM, C3 E.M. = Loss of foot processes, detachment of epithelium from B.M. Majority occur in older children. Also occurs in adults. Does not respond to corticosteroids. Leads to renal failure. Membranous Nephropathy (Glomerulonephritis) • Persistent proteinuria • Idiopathic • 2 causes: carcinomas, SLE, hepatitis, Diabetes Mellitus, thyroiditis, drugs. • Glomeruli are enlarged yet normocellular • No cellular proliferation • B.M. Thickening F.M. = "Spike and Dome." Granular IgG, C3 E.M. = Subepithelial immune deposits in B.M., thickened B.M. #1 cause of Nephrotic syndrome in adults Benefit of corticosteroids is unknown.
Transcript of Nephrotic nephritic syndroms usmle notes
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NEPHROTIC and NEPHRITIC SYNDROMES
Disease Most FrequentClinical Presentation
Pathogenesis Light Microscope F.M.(FluorescenceMicroscope)
E.M. (ElectronMicroscope)
Age Group Affected Treatment and
Outcome
Minimal Change Disease
(Lipoid Nephrosis)
• Selectiveproteinuria (Albumin)
• Loss of footprocesses• Loss of GBMpolyanionic sites• Appearance of villion epithelial cells
Normal
Lipid in tubules
F.M. = negative
E.M. = loss of footprocesses, lipidvacuoles
#1 cause of NephroticSyndrome in children,esp. boys younger than6 yrs. old.
Responds well tocorticosteroids.
No progressioninto chronic renalfailure
Focal SegmentalGlomerular Sclerosis
• Non-selectiveproteinuria• Hypertension• Microscopichematuria
• Idiopathic• Lower renal mass(in obese)• 2 causes: heroin use,HIV
• Focal andsegmental sclerosis• Hyalinosis• Adhesions toBowman's Capsule• Hypercellularmesangium• Thick B.M.
F.M. = IgM, C3
E.M. = Loss offoot processes,detachment ofepithelium fromB.M.
Majority occur in olderchildren. Also occursin adults.
Does not respondtocorticosteroids.Leads to renalfailure.
MembranousNephropathy
(Glomerulonephritis)
• Persistentproteinuria
• Idiopathic• 2 causes:carcinomas, SLE,hepatitis, DiabetesMellitus, thyroiditis,drugs.
• Glomeruli areenlarged yetnormocellular• No cellularproliferation• B.M.Thickening
F.M. = "Spike andDome." GranularIgG, C3
E.M. =Subepithelialimmune depositsin B.M., thickenedB.M.
#1 cause of Nephroticsyndrome in adults
Benefit ofcorticosteroids isunknown.
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Diabetic Nephropathy
(DiabeticGlomerulosclerosis)
• Proteinuria• No hematuria
• Diabeticmicroangiopathy• Thickened B.M.• Massive mesangialgrowth• "KimmelstielWilson" nodularglomerulosclerosis• Diffuseglomerulosclerosis
• Kimmelstiel-WilsonNodules arepathognomonic.• Massive mesangialhypercellularity.
F.M. = negative
E.M. = massivemesangial growth,thickened B.M.
DiabeticsProgresses to renalfailure
Reoidosisnal AmylSubendothelial andmesangial amyloiddeposits
• Amyloid depositsare initially mesangial,producing mesangialwidening withouthypercellularity.• Later, the amyloidobliterates the lumen• PAS(-)• Congo-Red (+)
F.M. = negative
E.M. =characteristic criss-cross fibrillaryproteins.
Any age groupSevere amyloidinfiltration leads torenal failure
Alport Syndrome
(Hereditary Nephritis)
• Recurrenthematuria before age20• Hypertension• Deafness andocular problems
Structural defect inCollagen IV leads toleaky basementmembranes.
Looks normal F.M. = negative
E.M. = glomerularB.M. splitting
Symptoms appearbefore age 20
Progresses torenal failure
Benign FamilialHematuria
(Thin B.M. Disease)
• Recurrenthematuria• Most frequentcause ofasymptomatichematuria.
Reduced thickness ofglomerular B.M.
Looks normal
F.M. = negative
E.M. = reducedglomerular B.M.thickness
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Acute (Post-Streptococcal)Glomerulonephritis
• Acute nephritis• Abrupt oliguria,hematuria, facialedema, hypertension.
• Immune-complexmediated (Type-IIIhypersensitivity)• Occurs afterStreptococcalpharyngitis orHepatitis-B• High ASO-titer, lowC3
• Glomerularhypercellularity• Increase inendothelial cells,mesangial cells,and PMN's.• No increase inepithelial cells.• No B.M.thickening
F.M. = "lumpy-bumpy" granulardeposits of IgGand C3
E.M. =Subepithelial (notsubendothelial)"humps,"otherwise normalappearing B.M.
Common renal diseasein childhood
Return to normalin 8 weeks.
Completerecovery withouttreatment(especially inkids) within 3years.
SLE Nephropathy
Degree of kidneyinvolvementcorrelates withprognosis in SLE.
Anti ds-DNAantibodies.
• WHO I: Normal• WHO II:Increasedmesangial matrix• WHO III: Focalproliferation• WHO IV:Diffuseproliferation,worst.• WHO V:Identical toMembranousNephropathy
F.M. = IgM, IgG +C3• Type-I:Granularappearance• Type-II:Pseudo-linearappearance
IgA Nephropathy(Berger's Disease):Most commonprimaryglomerulonephritis
Circulating IgA +fibronectin (due tochronic liver disease)
• Mesangial cellproliferation
F.M. = Granularappearance, IgG +C3
E.M. = Mesangialdeposits
Young men 15-30Focal SegmentalGlomerulonephritis
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Henoch-SchonleinPurpura
Same as above, plussystemic disease:purpura ofextremities, arthritis,colicky abdominalpain.
• Mesangial cellproliferation, moreserious than above.
F.M. = Granularappearance, IgG +C3
E.M. = Mesangialdeposits
Children
Endocarditis S. Aureus• Subepithelialimmune deposits
F.M. = Granularappearance, IgG + C3
Kidney diseaseresolves wheninfection is cured.
Rapidly ProgressiveCrescenticGlomerulonephritis
• Wegener's: kidney +upper respiratory tract.• Anuria• Oliguria
• Inflamedglomerular capillaries• ANCA (+)
• Cells accumulate inBowman's Capsule• Fibrin trapped inglomeruli• Epithelial cellproliferation• Macrophage, PMNinfiltrates
F.M. = Pauci-immune. Irregular
E.M. = wrinkling,discontinuity ofB.M.
Must be treated or itwill go to renal failurewithin weeks.
Goodpasture Syndrome
(Anti-BM AntibodyDisease)
Lung (hemoptysis) +kidneys (hematuria)
Anti-B.M. antibodies,against Type-IVcollagen
Similar to Crescenticglomerulonephritis, asabove.
F.M. = Linearpattern, IgG + C3
E.M. = Noimmune complexdeposits
Males 25-30
Responds toimmunosuppressivetherapy andplasmapheresis
MembranoproliferativeGlomerulonephritis
(MesangiocapillaryGlomerulonephritis)
• B.M. thickening andcellular proliferation• Mesangialexpansion makesglomerular B.M.appear as though itwere in two layers
E.M. = "Tram-track" appearance,resulting fromdouble-layerappearance ofglomerular B.M.
