Nephritic Syndrome Flashcards

1
Q

What is nephritic syndrome? What is it characterized by? Wha does biopsy reveal? How is the damage caused?

A

Glomerular disrders characterized by glomerualr inflammation and bleeding. 1. Limited proteinuria (

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2
Q

What causes poststreptococcal glomerulonephritis (PSGN)? What does it look like on H&E? What does it look ilke on EM and IF? How is it treated? What can some adults progress to?

A

Nephritic syndrome that arises after group A beta-hemolytic streptococcal infection of teh skin (impetigo) or pharynx. Hypercellular, inflamed glomeruli on H&E. MEdiated by immune complex deposition (granular IF); subepithelial ‘humps’ on EM. Treatment is supportive. Some adults (25%) develop rapidly progressive glomerulonephritis (RPGN).

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3
Q

A patient presents 2 weeks after an infection. He has a cola colored urine, oliguris, hypertension and periorbital edema. What was he infected with?

A

Poststreptococcal glomerulonephritis (PSGN)

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4
Q

What is the histological hallmark of rapidly progressing glomerulonephritis?

A

Crescents in Bowmanspace of glomeruli on H&E stain. Crescent are comprised of fibrin and macrphages.

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5
Q

What is the most common nephropathy worldwide? When and how does it present? What is seen on IF? What can it progress to?

A

IgA Nephropathy (Berger Disease) which is IgA immune comple deposition in mesangium of glomeruli. Presents during childhood as episodic gross or microscopi hematuria with RBC casts, usually following mucosal infections. IgA immune complex deposition in the mesangium is seen on IF. May slowly progress to renal failure

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6
Q

What is Alport Syndrome? What is its inheritance pattern? What does it result in and how does it present?

A

Inherited defect in type IV collagen, most commonly X-linked. Results in thinning and splitting of teh glomerular basement membrane. Presents as isolated hematuria, sensory hearing loss and ocular disturbances.

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7
Q

What is the immunofluorescence pattern of Poststreptococcal glomerulonephritis (PSGN) or diffuse proliferaive glomerulonephrtis? Where is the antibody deposited? Where is diffuse proliferative glomerulonephritis?

A

Granular (immune complex deposition). Usually sub-endothelial. Most common type of renal disease in SLE.

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8
Q

What is the immunofluorescence pattern of Poststreptococcal glomerulonephritis (PSGN) or diffuse proliferaive glomerulonephrtis? Where is the antibody deposited? Where is diffuse proliferative glomerulonephritis?

A

Granular (immune complex deposition). Usually sub-endothelial. Most common type of renal disease in SLE.

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9
Q

What is the immunofluorescence pattern of Goodpasture syndrome? What is the antibody formed against? How does it present?

A

Linear (anti-basement membrane antibody). Antibody against collagen in glomerular and alveolar basement membranes. Presents as hematuria and hemoptysis, clasically in young, adult males.

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10
Q

What is the immunofluorescence pattern of Wegener granulomatosis, microscopic polyangitis and Churg-Strauss syndrome? What is the antibody formed against?

A

Negatice IF (pauci-immune). Wegener granulomatosis is associated with c-ANCA. Microscopic polyangitis and Churg-Strauss syndrome are associated with p-ANCA.

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11
Q

Microscopic polyangitis and Churg-Strauss syndrome are associated with p-ANCA. How do you differentiate between them?

A

Churg-Strauss has granulomatous inflammation, eosinophilia and asthma

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