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Path Block 2
Nephrotic Syndrome
Signs and Symptoms
Massive Proteinuria > 3.5g per Day
Edema
Increased risk of infection
Thromboembolism
Hyperlipidemia
Fatty casts
1 min
Minimal Change Disease
Pathophysiology
Nephrotic
Most Common in Children
May be Triggered by Recent Infection or Immune Stimulus
Diagnosis
On Electron Microscopy (EM) See Foot Process (Podocyte) Effacement
On Light Microscopy (LM) See Normal Glomeruli
Loss of Negative Charge
Selective Loss of Albumin
Treatment
Responds to Corticosteroids
2 mins
Focal Segmental Glomerulosclerosis
Most Common Cause of Nephrotic Syndrome in Hispanic People and African Americans
Associated with Sickle Cell Disease and HIV
Associated Interferon Therapy and Heroin Abuse
Histopathology
LM Segmental Sclerosis and Hyalinosis
EM Focal Damage of Visceral Epithelial Cells
IF Negative
Signs & Symptoms
Microscopic Hematuria
Nonselective Proteinuria
Considerations
Poor Prognosis
2 mins
Membranous Glomerulonephritis
Characteristics
Nephrotic
Diagnosis
LM Thickening of Capillary and Glomerular Basement Membrane (GBM)
EM Spike and Dome Appearance with Subepithelial Deposits
IF Granular
Primary
Phospholipase A2 Receptor Autoantibodies
Secondary
NSAIDS
Gold
Solid Tumors
Hepatitis B Virus (HBV)
Lupus
3 mins
Membranoproliferative Glomerulonephritis
Nephritic-Nephrotic Syndrome
Type I
Subendothelial Immune Complexes
Hepatitis C Virus
Granular IF
Tram Tracks Caused by Splitting of the GBM
Ingrowth of Mesangium
Type II
Dense Deposit Disease
C3 Nephritic Factor
2 mins
Amyloidosis
Etiologies
Primary
Multiple Myeloma
Secondary
Tuberculosis (TB)
Rheumatoid Arthritis
Pathology
Congo Red Stain
Apple Green Birefringence
Complications
Nephrotic Syndrome
2 mins
Diabetic Glomerulonephropathy
Pathophysiology
Nonenzymatic Glycosylation of Glomerular Basement Membrane
Nonenzymatic Glycosylation of Arterioles
Hyperfiltration
Increased GFR
Histological Findings
Hyaline Arteriolosclerosis
Mesangial Expansion
Type IV Collagen Deposition
Kimmelstiel Wilson Lesions
Presentation
Nephrotic
2 mins
Nephritic Syndrome
Inflammatory
Hematuria
RBC casts
Increased Nitrogen in blood
Oliguria
Hypertension
Proteinuria < 3.5 grams per day
2 mins
Alport Syndrome
Pathophysiology
Nephritic
X-linked
Mutation in Type IV Collagen
Diagnosis
Split Basement Membrane
Basket Weave Appearance
Signs and Symptoms
Ocular Disorders
Deafness
2 mins
Berger's Disease (IgA Nephropathy)
Nephritic
IgA Nephropathy
Immune Complex Deposit in Mesangium
Increased Synthesis of IgA
Most Common Cause of Nephritic Syndrome
Overlapping Features with Henoch–Schönlein Purpura
Presents with Pharyngitis, URI or Acute Gastroenteritis
1 min
Post Streptococcal Glomerulonephritis
Pathophysiology
Nephritic
Children
Signs and Symptoms
Edema
Cola Colored Urine
Follows Group A Streptococcal pharyngitis or Skin Infection
Diagnosis
Neutrophils
Immunofluorescence (IF) Granular
Lumpy Bumpy Appearance on Immunofluorescence (IF)
Subepithelial Immune Complex Humps
2 mins
Rapidly Progressive Glomerulonephritis
Pathogenesis
Nephritic Syndrome
Crescent Shape
Crescents Consist of Fibrin and C3b
With Parietal Cells, Monocytes, and Macrophages
IMMUNOFLUORESCENCE
Linear Immunofluorescence
Negative Immunofluorescence
Granular Immunofluorescence
Etiology
Goodpasture Syndrome
Granulomatosis with Polyangiitis (Formerly Wegener's)
Microscopic Polyangiitis
Prognosis
Poor Prognosis
3 mins
Anti-glomerular Basement Membrane Antibodies
Glomerulonephritis
Goodpasture's Disease
Linear immunofluorescence
Hemoptysis
Hematuria
1 min
Casts in Urine (Differential Diagnosis)
Characteristics
RBC Casts
Glomerular Disease
WBC Casts
Acute Interstitial Nephritis
Acute Pyelonephritis
Fatty Casts
Maltese Cross Sign
Nephrotic Syndrome
Granular Muddy Brown Casts
Acute Tubular Necrosis (ATN)
Waxy Casts
End Stage Renal Disease (ESRD) (Chronic Renal Failure)
Hyaline Casts
Non-Specific
2 mins
Enterohemorrhagic E. Coli (EHEC)
Characteristics
O157:H7
Non-Sorbitol Fermenting
Hamburger Meat
Produces Shiga-like Toxin
Gram-negative
Lysogeny
Inactivates 60S Ribosome
Disease
Bloody Diarrhea
Hemolytic Uremic Syndrome (HUS)
Hemolytic Anemia
Acute Renal Failure
Thrombocytopenia
2 mins
Shigella
Characteristics
Gram-Negative
Bacilli
Non Lactose Fermenting
White on Macconkey Agar
Oxidase Negative
Non H2S Producing
Day Care Centers
High Virulence
Replicates Intracellularly
Actin Rocket
Signs and Symptoms
Bloody Diarrhea
Shiga Toxin
Inhibits 60S Ribosome
Hemolytic Uremic Syndrome (HUS)
Hemolytic Anemia
Thrombocytopenia
Kidney Failure
3 mins
Hemolytic Uremic Syndrome (HUS)
Children
Pathophysiology
Preceded by Acute Diarrheal Illness
Endothelial Damage
Classic Triad of Symptoms
Microangiopathic Hemolytic Anemia (MAHA)
Thrombocytopenia
Renal Failure
Labs
Helmet cells
LDH
Treatment
Dialysis
Supportive
2 mins
Thrombotic Thrombocytopenic Purpura (TTP)
Inhibition or deficiency of ADAMTS 13
vWF Multimers are Not Degraded
Microthrombi (and Emboli)
Symptoms
FAT RN Pentad
Fever
Anemia
Thrombocytopenia
Renal Dysfunction
Neurologic Abnormalities (Altered Mental status)
Treatment
Plasmapheresis
Corticosteroids
Splenectomy
2 mins
Autosomal Dominant Polycystic Kidney Disease
Cause
PKD1 Mutation
Symptoms
Bilateral Enlarged Cystic Kidneys
Flank Pain
Hematuria
Hypertension
Associated With
Berry Aneurysms
Subarachnoid Hemorrhage
Mitral Valve Prolapse (MVP)
Hepatic Cyst
Transplant Rejection
Hyperacute
Occurs within Minutes
Antibody Mediated Due to Preformed Antidonor Antibodies
Acute
Occurs within Weeks
Cytotoxic T Lymphocytes Cause Cell-mediated or Antibody-mediated Response
Chronic
Occurs Months to Years Later
Antibody and T Cell-mediated Vascular Damage
2 mins
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