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Autosomal Dominant for Adult onset Medullary Cystic Disease
#1 Cause of ESRD in children (nephronopthisis)
Gross Look
Medullary Cystic Kidney Disease
Autosomal Dominant
Renal Fibrosis and Failure
Cysts within Medullary Collecting Ducts Only
Small Kidneys
Calcium Stones
Amyloidosis
Etiologies
Primary
Multiple Myeloma
Secondary
Tuberculosis (TB)
Rheumatoid Arthritis
Pathology
Congo Red Stain
Apple Green Birefringence
Complications
Nephrotic Syndrome
2 mins
Multiple Myeloma Signs and Symptoms
Signs and Symptoms
Increased Susceptibility to Infection
Back/Bone Pain
Punched Out Lytic Bone Lesions
Hypercalcemia
Primary Amyloidosis
Renal Insufficiency
Anemia
2 mins
Multiple Myeloma Pathophysiology
Pathophysiology
Monoclonal Plasma Cell Cancer
Clock Face Chromatin
Diagnosis
M Spike on Protein Electrophoresis
Increased IgG and IgA
Fried Egg Appearance (BM Biopsy)
Rouleaux Formation
Stacked RBC's
Bence Jones Protein
2 mins
Nephritic Syndrome
Inflammatory
Hematuria
RBC casts
Increased Nitrogen in blood
Oliguria
Hypertension
Proteinuria < 3.5 grams per day
2 mins
Nephrotic Syndrome
Signs and Symptoms
Massive Proteinuria > 3.5g per Day
Edema
Increased risk of infection
Thromboembolism
Hyperlipidemia
Fatty casts
1 min
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
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
Goodpasture's
Smooth/Linear IgG & C3
Anti GBM AB
Sx: Hemoptysis
Sx: Hematuria
Sx: Young men
Edema & Crackles in Lungs
RPGN: Dysmorphic RBC's, Red Casts
AB against Alpha 3 chain of Type IV collagen
Alveolar Hemorrhage: Causes High CO2 Diffusing Capacity in the lungs
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
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
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
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