(MHD) Lecture 2- Renal Path I

Question 1

What are the (3) characteristics which make up Nephritic Syndrome?

Answer 1

1. Hematuria w/ dysmorphic cells and RBC casts
2. Some degree of oliguria and azotemia
3. Hypertension

Question 2

Name the (5) types of Nephritic Syndromes

Answer 2

1. Membranoproliferative GN
2. Acute poststreptococcal GN (aka Postinfectious)
3. Rapidly Progessive GN (RPGN)
4. IgA nephropathy (Berger disease)
5. Alport Syndrome (Hereditary)

MARIA is Nephritic

Question 3

Describe the pathogenesis/epidemiology of Acute Postinfectious Glomerulonephritis (who does it affect? what specific disease is it associated with/when does it occur? what specifically occurs which causes nephritis?)

Answer 3

• Mostly children
• Occurs 1-4 weeks after Group A strep infection (pyogenes)
• Immune complexes form with IgG and M-proteins from strep. These deposit in the capillary wall and activate complement, attracting neutrophils and leading to structural damage (swiss cheese) which allows for hematuria

Question 4

Describe the “hump” phenomenon associated with Post-infectious glomerulonephritis

Answer 4

Immune complexes which have been formed from IgG and M-protein antigen are formed in-situ leading to large sub-epithelial deposit “humps” which are unique to PIGN!!!

Question 5

Key lab tests/observations for Acute Postinfectious Glomerulonephritis

What is its treatment?

Answer 5

1. Tea-color (smoky, coca-cola) urine
2. ASO (antisteptolysin O) INCREASE
3. Complement level DECREASE

Treatment: Supportive

Question 6

Describe the pathogenesis/epidemiology of IgA nephropathy (who does it affect? what disease is it associated with/when does it occur? what specifically occurs which causes nephritis?)

Answer 6

• Children and Young Adults
• Episode of gross hematuria 1-2 days after nonspecific upper respiratory tract infection (or GI/urinary)
• Hematuria for days w/ reccurrence every few months
• IgA immune complexes form which deposit in mesangium

Question 7

What is the main type of hereditary nephritis? What set of symptoms are associated with it?

Answer 7

• Alport Syndrome
• Associated with a Triad of symptoms
  
  • Nephritis
  • Nerve deafness
  • Various eye disorders/early cataracts

Question 8

How old are patients when they normally present with Alport Syndrome? When it progresses to renal failure? How does it affect male/females? What is the pathology associated wtih it?

Answer 8

• Age 5-20 y/o at presentation
• 20-50 y/o w/ overt renal failure
• It demonstrates X-linked inheritance so mostly men get it and women serves as carries (usually)
• There is a splitting of the lamina densa into “basket weave” which allows for flow of RBCs (seen on electron microscopy)