Your SlideShare is downloading. ×
0
Cystinosis Tissue Repository Study of Atubular Glomeruli
Cystinosis Tissue Repository Study of Atubular Glomeruli
Cystinosis Tissue Repository Study of Atubular Glomeruli
Cystinosis Tissue Repository Study of Atubular Glomeruli
Cystinosis Tissue Repository Study of Atubular Glomeruli
Cystinosis Tissue Repository Study of Atubular Glomeruli
Cystinosis Tissue Repository Study of Atubular Glomeruli
Cystinosis Tissue Repository Study of Atubular Glomeruli
Cystinosis Tissue Repository Study of Atubular Glomeruli
Cystinosis Tissue Repository Study of Atubular Glomeruli
Cystinosis Tissue Repository Study of Atubular Glomeruli
Cystinosis Tissue Repository Study of Atubular Glomeruli
Cystinosis Tissue Repository Study of Atubular Glomeruli
Cystinosis Tissue Repository Study of Atubular Glomeruli
Cystinosis Tissue Repository Study of Atubular Glomeruli
Cystinosis Tissue Repository Study of Atubular Glomeruli
Cystinosis Tissue Repository Study of Atubular Glomeruli
Cystinosis Tissue Repository Study of Atubular Glomeruli
Cystinosis Tissue Repository Study of Atubular Glomeruli
Cystinosis Tissue Repository Study of Atubular Glomeruli
Upcoming SlideShare
Loading in...5
×

Thanks for flagging this SlideShare!

Oops! An error has occurred.

×
Saving this for later? Get the SlideShare app to save on your phone or tablet. Read anywhere, anytime – even offline.
Text the download link to your phone
Standard text messaging rates apply

Cystinosis Tissue Repository Study of Atubular Glomeruli

221

Published on

0 Comments
0 Likes
Statistics
Notes
  • Be the first to comment

  • Be the first to like this

No Downloads
Views
Total Views
221
On Slideshare
0
From Embeds
0
Number of Embeds
0
Actions
Shares
0
Downloads
2
Comments
0
Likes
0
Embeds 0
No embeds

Report content
Flagged as inappropriate Flag as inappropriate
Flag as inappropriate

Select your reason for flagging this presentation as inappropriate.

Cancel
No notes for slide
  • Darmady and Stranak Brit Med Bull 1957
  • Transcript

    • 1. Cystinosis Tissue Repository Study of Atubular Glomeruli Jess Thoene, M.D.
    • 2. Background <ul><li>The description of atubular glomeruli (ATG) in diabetic nephropathy , and the occurrence of the &quot;swan neck&quot; deformity in the proximal renal tubule in cystinosis prompted a pilot study in one cystinotic kidney to determine the occurrence of ATG in cystinotic kidney specimens.  CRN   funded a cystinosis tissue repository at the University of Michigan that has enabled us to obtain renal tissue post transplantation </li></ul>
    • 3. “ Swan-neck Deformity”
    • 4. Atubular Glomeruli and Glomerulotubular Junction Abnormalities in Diabetic Nephropathy BEHZAD NAJAFIAN, YOUNGKI KIM, JOHN T. CROSSON, and MICHAEL MAUER J Am Soc Nephrol 14: 908–917, 2003 Prior studies:
    • 5. Method <ul><li>Obtain renal tissue from cystinosis patients at time of renal transplantation </li></ul><ul><li>Cut 4 µ paraffin sections </li></ul><ul><li>Reconstruct glomeruli using sequential sections performed   by Patrick Walker and associates at Nephropath in Little Rock, Arkansas. </li></ul><ul><li>Determine the incidence of ATG </li></ul>
    • 6. Case Reports, ATG Study <ul><li>Case number one : This boy had a renal transplant at age 10 years having been diagnosed with nephropathic cystinosis at age 5 years. From the time of diagnosis until real transplantation he had been maintained on optimal doses of cysteamine and displayed excellent compliance. At transplantation the kidney was embedded in paraffin and sent for three-dimensional reconstruction analysis. Measurement of the cystine content showed 530 nmol/mg protein in the renal cortex and 740 nmol /mg protein in the medulla, consistent with previous such measurements in cystinotic renal tissue. </li></ul>
    • 7. Case Reports, ATG Study <ul><li>Case number two: No clinical information is available on this sample from kidneys removed at renal transplantation for ESRD due to cystinosis. The pathology report included extensive glomerulosclerosis, interstitial lymphocytic inflammation, and calcifications with tubular atrophy. </li></ul><ul><li>  </li></ul>
    • 8. Case Reports, ATG Study <ul><li>Case number three: This patient came to renal transplantation due to end-stage renal disease at age 24 years. He had been diagnosed at age 13 months , and at the time of transplantation was receiving Cystagon 2400 mg/day divided q6h. His creatinine had risen to 2.8 mg/dL at 23 years of age and he underwent a transplantation using a renal allograft at 24 years. </li></ul>
    • 9. Control Sections <ul><li>Tubule has a clear connection with Bowman’s Capsule </li></ul>
    • 10. Tubular Takeoff Point
    • 11. Tubular Takeoff Point
    • 12. Cystinotic Sections <ul><li>Few glomeruli show tubular connections </li></ul>
    • 13. Tubular Takeoff Point Next section No connection
    • 14. Tubular Takeoff Point Previous section No connection
    • 15. Tubular Takeoff Point
    • 16. Tubular Takeoff Point
    • 17. Tubular Takeoff Point
    • 18. Tubular Takeoff Point ALMOST…
    • 19. Frequency of ATG in Cystinosis Glomeruli with normal GTJ Glomeruli with atrophic GTJ Atubular glomeruli Cystinosis patients 1/106 (1%) 19/106 (18%) 86/106 (81%) Normal controls 68/71 (96%) 0/71 (0%) 3/71 (4%) p <0.0001 <0.0001 <0.0001
    • 20. Conclusions <ul><li>There is a large incidence of ATG in end stage renal disease in cystinosis </li></ul><ul><li>The finding is highly statistically significant </li></ul><ul><li>This finding may account for progressive renal failure in cystinosis </li></ul>

    ×