Nephrology Practice Board Question – FSGS Answers

As I’ve been studying I’ve been trying to make up questions of information that I found to be important or interesting (at least to me). These questions and those to follow are a random grouping of  multiple choice, true/false, or guess what I’m thinking.
1.) Collapsing FSGS is associated with what disease/drugs?
1.) HIV
2.) Parvovirus B19 infection
3.) Interferon therapy
4.) Pamidronate toxicity
2.) Which monosaccharide sugar has been shown to normalize the permeability factor in FSGS?
A. Fructose
B. Sucrose
C. Galactose
D. Glucose
It has a high affinity for the cardiotrophin-like cytokine 1 permeability factor (aka the FSGS PF or FSPF). I has be shown to improve the permeability factor in several patient with refractory FSGS in (case studies).
3.) Secondary forms of FSGS occur via what mechanism?
They are mediated by adaptive structural-functional responses due to reduction in te number of functioning nephrons (wheter acquired or congenital). There is increased hemodynamic stress on the remaining nephrons and lead to glomerular hypertrophy and perihilar lesions of segmental sclerosis and hyalinosis.
4.) In children with FSGS as the cause of ESRD a transplant from a living related donor gave a better allograft survival than from a cadaveric donor.
True, especially in African American children with FSGS where there is no difference in long term allograft survival vs non-FSGS allograft recipents.
5.) Mutations to APOL1 are protective against what infectious parasite?
A. Plasmodium falciparum
B. Trypanosoma brucei
C. Giardia lamdia
D. Toxoplasma gondii
6.) Pseudocrescents (hyperplastic visceral epithelial cells swollen and crowded together) are associated with which variant of FSGS?
A. Collpasing
B. Perihilar
C. Tip
D. Cellular
7.) Secondary forms of FSGS associated with hyperfiltration such as remnant kidney and obesity-related glomerulopathy typically have lower levels of proteinuria. True/False?
TRUE
8.) The structure in the picture is associated with which diseases?

 
 
 
 
 
 
 
Interferon therapy, HIV, and Lupus. This is a picture of a tubuloreticular body.
 
9.) What IF pattern is often seen in FSGS?
A. IgM
B. IgA
C. C3
D. C4d
E. A and C
F. A and B
G. B and D
10.) What is the first-line treatment of FSGS in patients with nephrotic syndrome?
A. Pulse methylprednisolone for 3 days
B. Prednisone 1mg/kg/day for 6-8 weeks with subsequent tapering
C. Cyclophosphamide 0.5mg/m2/day IV for 3 months
D. No immunosuppression just conservative therapy
11.) What medications/drugs are associated with secondary FSGS?
Heroin
Lithium
Pamidronate
Sirolimus
Interferon (alpha,beta, or gamma)
12.) What risk factors are best prognosticators for progression to ESRD in FSGS at biopsy?
A. Reduced GFR
B. Greater degree of proteinuria
C. Greater degree of intersitial fibrosis.
D. All of the above
-Adrian