Successfully reported this slideshow.
Cystinosis Tissue Repository Study of Atubular Glomeruli Jess Thoene, M.D.
Background <ul><li>The description of atubular glomeruli (ATG) in diabetic nephropathy , and the occurrence of the &quot;s...
“ Swan-neck Deformity”
Atubular Glomeruli and Glomerulotubular Junction Abnormalities in Diabetic Nephropathy BEHZAD NAJAFIAN, YOUNGKI KIM, JOHN ...
Method <ul><li>Obtain renal tissue from cystinosis patients at time of renal transplantation </li></ul><ul><li>Cut 4 µ par...
Case Reports, ATG Study <ul><li>Case number one : This boy had a renal transplant at age 10 years having been diagnosed wi...
Case Reports, ATG Study <ul><li>Case number two:  No clinical information is available on this sample from  kidneys remove...
Case Reports, ATG Study <ul><li>Case number three:  This patient came to renal transplantation due to end-stage renal dise...
Control Sections <ul><li>Tubule has a clear connection with Bowman’s Capsule </li></ul>
Tubular Takeoff Point
Tubular Takeoff Point
Cystinotic Sections <ul><li>Few glomeruli show tubular connections </li></ul>
Tubular Takeoff Point Next section No connection
Tubular Takeoff Point Previous section No connection
Tubular Takeoff Point
Tubular Takeoff Point
Tubular Takeoff Point
Tubular Takeoff Point ALMOST…
Frequency of ATG in Cystinosis Glomeruli with normal GTJ Glomeruli with atrophic GTJ Atubular glomeruli Cystinosis patient...
Conclusions <ul><li>There is a large incidence of ATG in end stage renal disease in cystinosis </li></ul><ul><li>The findi...
Upcoming SlideShare
Loading in …5
×

Cystinosis Tissue Repository Study of Atubular Glomeruli

530 views

Published on

  • Be the first to comment

  • Be the first to like this

Cystinosis Tissue Repository Study of Atubular Glomeruli

  1. 1. Cystinosis Tissue Repository Study of Atubular Glomeruli Jess Thoene, M.D.
  2. 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. 3. “ Swan-neck Deformity”
  4. 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. 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. 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. 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. 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. 9. Control Sections <ul><li>Tubule has a clear connection with Bowman’s Capsule </li></ul>
  10. 10. Tubular Takeoff Point
  11. 11. Tubular Takeoff Point
  12. 12. Cystinotic Sections <ul><li>Few glomeruli show tubular connections </li></ul>
  13. 13. Tubular Takeoff Point Next section No connection
  14. 14. Tubular Takeoff Point Previous section No connection
  15. 15. Tubular Takeoff Point
  16. 16. Tubular Takeoff Point
  17. 17. Tubular Takeoff Point
  18. 18. Tubular Takeoff Point ALMOST…
  19. 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. 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>

×