Nephritic & Nephrotic syndromes

sws42792's version from 2015-05-15 16:13

Nephritic and Nephrotic Syndromes

Question Answer
Minimal Change DiseaseMost common nephrotic synd. in children, assoc. w/ Hodgkin lymphoma (massive cytokine release by Reed-Sternberg cells), podocyte foot process effacement by cytokines (no immune complexes), excellent response to steroids
Focal Segmental Glomerulosclerosis (FSGS)Effacement of podocyte foot processes, no immune complexes, focal and segmental distribution, Hispanics & Af. americans, assoc w/ HIV, heroin, sickle cell, nephrotic
Membranous NephropathyThick glomerular BM, immune complex deposition - granular IF, subEPIthelial deposits, "spike and dome," assoc w/ Hep B and C, solid tumors, SLE, nephrotic syndrome
Membranoproliferative Glomerulonephritis type ISubENDOthelial deposits, 'tram-track' appearance, assoc. w/ HBV and HCV, thick glomerular BM, immune complex deposition - granular IF, nephritic and nephrotic
Membranoproliferative Glomerulonephritis type IIINTRAMEMBRANOUS deposits, C3 nephritic factor stabilizes C3 convertase, low serum C3, thick glomerular BM, immune complex deposition - granular IF, nephritic and nephrotic
Diabetes MellitusNephrotic, nonenzymatic glycosylation of vascular BM, hyaline arteriolosclerosis, efferent arteriole more affected, hyperfiltration leads to microalbuminuria, Kimmelsteil-Wilson nodules, ACEIs slow progression
Systemic AmyloidosisAmyloid deposits in mesangium, nephrotic syndrome, apple-green birefringence on Congo-red stain
Poststreptococcal Glomerulonephritis (PSGN)Nephritic, 2-3 wks after Grp A Strep infection, nephritogenic strains with M protein virulence factor, usually in children, subEPIthelial immune complex deposition
Rapidly Progressive GlomerulonephritisNephritis, crescents of fibrin and macrophages in Bowman's space, use IF to determine cause
IgA NephropathyNephritic, IgA immune complex deposition in mesangium, most common nephropathy worldwide, usually children following a mucosal infection (like gastroenteritis)
Alport SyndromeNephritic, inherited defect in type IV collagen, thinning and splitting of GBM, isolated hematuria, sensory hearing loss, ocular disturbances
Diffuse Proliferative GlomerulonephritisNephritic, subENDOthelial deposits, most common renal disease and cause of death in SLE, diffuse Ab-Ag complex deposits, "wire looping" of capillaries

IF Patterns in Rapidly Progressive Glomerulonephritis

Question Answer
LinearGoodpasture syndrome - anti basement membrane Ab (against BM collagen) in glomerular and alveolar BM, hematuria and hemoptysis
GranularPSGN, diffuse proliferative glomerulonephritis (most common type of renal disease in SLE, subendothelial Ab deposits)
Negative IF (pauci-immune)Wegener granulomatosis (c-ANCA), microscopic polyangiitis (p-ANCA), Churg-Strauss syndrome (p-ANCA w/ granulomas, eosinophilia, and asthma)

Lower Urinary Tract CA risk factors

Question Answer
Urothelial (Transitional Cell) CACigarette smoke, napthylamine, azo dyes, long-term cyclophosphamide use, phenacytin use
Squamous Cell CASquamous metaplasia caused by chronic cystitis (older woman), Schistosoma hematobium infection (young Middle-eastern male), long-standing nephrolithiasis
AdenocarcinomaUrachal remnant (dome of the bladder), cystitis glandularis (chronic inflamm. leads to columnar metaplasia), bladder extrophy

Immune Complex deposition patterns in Nephritic and Nephrotic syndromes

Question Answer
Membranous Nephropathysubepithelial "spike and dome"
Membranoproliferative GN Type Isubendothelial "tram-track"
Membranoproliferative GN Type IIintramembranous (possibly tram-track, but less than type I)
Poststreptococcal GNsubepithelial "humps" aka "lumpy-bumpy"
Goodpasture syndromeNo immune complexes, but linear IF from anti-BM Abs
Diffuse Proliferative GNsubendothelial
IgA Nephropathymesangium

Casts in urine

Question Answer
RBC castsGlomerulonephritis, malignant HTN, nephritic syndrome
WBC castsTubulointerstitial inflammation, acute pyelonephritis, transplant rejection
Fatty casts ("oval fat bodies")Nephrotic syndrome
Granular ("muddy brown") castsAcute tubular necrosis
Waxy castsEnd-stage renal disease/chronic renal failure
Hyaline castsNonspecific, can be a normal finding, often seen in concentrated urine samples

Renal tubular defects ("FABulous Glittering LiquidS")

Question Answer
Fanconi syndromegeneralized reabsorptive defect in PCT, increase excretion of amino acids, glucose, bicarb, phosphate, can result in metabolic acidosis
Bartter syndromereabsorptive defect in thick ascending loop of Henle, affects Na/K/Cl transporter, causes hypokalemia, metabolic alkalosis with hypercalciuria
Gitelman syndromereabsorptive defect of NaCl in DCT, less severe than Bartter syndrome, causes hypokalemia, hypomagnesemia, metabolic alkalosis, hypocalciuria
Liddle syndromegain of function mutation, increased Na reabsorption in collecting tubules (increased ENaC activity), causes HTN, hypokalemia, metabolic alkalosis, decreased aldosterone, treat w/ amiloride (K sparing diuretic)
Syndrome of apparent mineralocorticoid excesshereditary deficiency of 11B-hydroxysteroid dehydrogenase, which converts cortisol to cortisone before cortisol can act on mineralocorticoid receptors, causes HTN, hypokalemia, metabolic alkalosis, low serum aldosterone levels

Electrolyte decreases

Question Answer
Nanausea and malaise, stupor, coma, seizures
KU waves on ECG, flattened T waves, arrhythmias, muscle spasm
Catetany, seizures, QT prolongation
Mgtetany, torsades de pointes, hypokalemia
Phosphatebone loss, osteomalacia (adults), rickets (children)

Electrolyte increases

Question Answer
Nairritability, stupor, coma
Kwide QRS and peaked T waves on ECG, arrhythmias, muscle weakness
Castones, bones, groans, thrones, psychiatric overtones (renal stones, bone pain, abdominal pain, increased urinary freq, anxiety, altered mental status, but NOT necessarily calciuria)
Mgdecrease DTRs, lethargy, bradycardia, hypotension, cardiac arrest, hypocalcemia
Phosphaterenal stones, metastatic calcifications, hypocalcemia

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