Nephrotic Syndrome (Robbins 5th Ed., p. 948-956) or (Robbins 6th Ed., p. 952-961)
1. General:
a. Describe the clinicopathologic features of the nephrotic syndrome.
b. List the causes of nephrotic syndrome and distinguish them in terms of:1. Incidence in adults vs. children
2. Primary (e.g., minimal change) vs. secondary (diabetes mellitus) causes
2. Minimal Change Disease (MCD):
a. Describe the characteristic light, IF, and EM findings.
b. Describe the suggested mechanism for proteinuria. What happens to the negative charge of GBM?
c. What is the response to steroids?
3. Focal Segmental Glomerulosclerosis (FSGS):
a. Describe the characteristic light microscopic and EM findings.
b. How do patients with this pattern differ from MCD in terms of response to steroids, progression of renal disease, hematuria, hypertension, and selectivity of proteinuria?
c. Understand that FSGS may be idiopathic or be secondary, e.g., in HIV infection.
4. Membranous GN [also called membranous glomerulopathy (MGN)]:
a. Define membranous GN, and distinguish between primary and secondary forms.
b. What are the proposed immunologic mechanisms?
c. Describe the morphologic glomerular changes by light, IF and EM.
d. Discuss the salient clinical features (onset and course).
5. Membranoproliferative GN (MPGN):
a. What are the characteristic light microscopic features?
1. (Robbins 5th Ed., p. 948-956) or (Robbins 6th Ed., p. 952-961)
2. Images 1-4 relate to this scenario. Images 5-13 are related to other objectives.
A previously healthy 35-year-old man, complained of recent fatigue, and swelling of his feet and ankles. He was also noted to have puffy eyes as well. Physical exam revealed an afebrile man, with lower extremity edema, extending to the knee, periorbital edema, and a small amount of ascites. Laboratory features were as follows:
|
Creatinine |
0.8 mg/dL |
Urinalysis: |
protein - 4+ |
|
BUN |
18 mg/dL |
glucose - neg |
|
|
Albumin |
2.8 g/dL |
blood - neg |
|
|
Hematocrit |
40% |
bilirubin - neg |
|
|
Liver function tests |
normal |
Micro: rare RBCs, no WBCs, many oval fat bodies |
|
|
Triglycerides |
400 mg/dL |
||
|
Cholesterol |
375 mg/dL |
||
|
24 hr urine protein |
11.2 gm/24 hrs |
||
Drug history was negative. ANA, HIV, and hepatitis serology were negative.
The patient developed right flank pain the following day. Ultrasound revealed renal vein thrombosis. Renal biopsy was performed.