Download U03-3031

#428207110
•Patient developed acute and chronic renal
failure in 1999 associated with a renal stone.
History, and a diagnosis of chronic
pyelonephritis. She was started on peritoneal
dialysis.
•Laparoscopic left nephrectomy was
performed in June 2000 for retained stones.
S00-45788
Left kidney, fragmentary removal:
• End-stage kidney
– Chronic tubulointerstitial inflammation and
scarring, consistent with obstruction and/or
pyelonephritis.
– Marked parenchymal crystal deposits, probably
secondary oxalate deposition.
« The findings here are probably secondary
oxalosis on the basis of chronic renal
failure, although I concede that the degree
of crystal deposition is exceptional. I should
add that there are congenital metabolic
errors which usually lead to massive levels
of oxalate retention in tissues, but these
usually lead to end-stage renal disease
before the age of 20. »
• LRD kidney transplant was performed
October 25, 2000.
• Rising creatinine about 2 months later
lead to transplant biopsy
• Demonstration of crystals which lead to
investigations resulting in diagnosis of
primary oxaluria (type I)
• Creatinine continue to rise.
Renal biopsies after
st
1
Tx
Indication
Banff
Deposits
time of Tx
G0 I0 T0 V0 AH2
-
U00-14762 (~ 2  creat 120
weeks post-Tx)
G0 I0 T0 V0 AH2
Focal tubular
calcifications
U00-15830 (~ 1  creat 160
month post-Tx)
G0 I0 T0 V0 AH2
Acute tubular injury
with calcium oxalate
deposition
 creat 280
G0 I0 T0 V0 AH2
Extensive tubular
deposition of calcium
oxalate consistent with
oxalosis
U00-14101
U01-253 (~ 2
months postTx)
Scanned slide
• Combined liver/kidney transplant June 21,
2003 with initial serum creatinine of 80
µmol/L.
• First post transplant biopsy done when
creatinine rose to 120 µmol/L
Renal biopsies after
nd
2
Tx
Indication
Banff
Deposits
U03-9704
time of Tx
G0 I0 T0 V0 AH1
-
U03-12130
(~ 6 weeks
post-Tx)
 creat 120
with
proteinuria
N.S. findings
G0 CG0 I1 CI1 T0
CT1 V0 CV1 AH0
MM0
tubular calcium
phosphate deposition
U03-12130
• Ureteral stenosis diagnosed in September
2003 (stent ?)
• Second biopsy when 170 µmol/L (At time
of surgical incision of ureter post-stent
changes)
Renal biopsies after
nd
2
Tx
Indication
Banff
Deposits
U03-9704
time of Tx
G0 I0 T0 V0 AH1
-
U03-12130
(~ 6 weeks
post-Tx)
 creat 120
with
proteinuria
N.S. findings
G0 CG0 I1 CI1 T0
CT1 V0 CV1 AH0
MM0
tubular calcium
phosphate deposition
U03-15919
(~ 4 months
post-Tx)
creat 180
post stent
change
acute bacterial
interstitial nephritis
G0 CG0 I3 CI2 T1
CT2 AH2 MM0
extensive calcium
phosphate and
calcium oxalate
deposition
U03-15919
IF
• Cytoplasmic staining of plasma cells for
IgG, kappa, lambda
Diagnosis
Renal Biopsy:
• Acute bacterial interstitial nephritis with pus
casts probably on the basis of partial
ureteral obstruction by calcium oxalate
debris.
• Tubular deposition of calcific debris, both
calcium phosphate and calcium oxalate.
• Possible subclinical immune complex
glomerulonephritis.
• No evidence of rejection.
• (G0 CG0 I3 CI2 T1 CT2 V- CV- AH2
MM0)
April 2004
• Obstructed renal transplant secondary to
ischemic ureter ?
• Portions of transplant ureter (proximal
ureter) surgically removed + transplant
biopsy.
Renal biopsies after
nd
2
Tx
Indication
Banff
Deposits
U03-9704
time of Tx
G0 I0 T0 V0 AH1
-
U03-12130
(~ 6 weeks
post-Tx)
 creat 120
with
proteinuria
N.S. findings
G0 CG0 I1 CI1 T0
CT1 V0 CV1 AH0
MM0
tubular calcium
phosphate deposition
U03-15919
(~ 4 months
post-Tx)
creat 180
post stent
change
acute bacterial
interstitial nephritis
G0 CG0 I3 CI2 T1
CT2 AH2 MM0
extensive calcium
phosphate and
calcium oxalate
deposition
U04-6303
(~ 10 months
post-Tx)
ureteral
obstruction
acute rejection IA
G0 CG0 I3 CI2 T2
CT2V0 CV0 AH1
MM0
no crystals in kidney
but present in ureter
Diagnosis
Renal Biopsy:
Kidney allograft biopsy (10 months posttransplantation):
Active chronic tubulointerstitial nephritis
A) Changes consisted with chronic obstruction
B) Banff Score G0 CG0 I3 CI2 T2 CT2 V0 CV0
AH1 MM0
C) No deposits by EM
Proximal right ureter:
A) Focal granulation tissue consistent with ischemic
damage
B) Granulomatous reaction to crystalline material,
consistent with oxalate deposits
• Following her biopsy in April 2004 (with
imaging studies documenting ureteral
obstruction despite stent) at the time of
surgical ureteral repair, she returned home
and serum creatinine settled to the 130-140
mol/L range.
• On prednisone 5 mg daily, cellcept 500 mg
bid, tacrolimus 3 mg bid, and other meds.
• When serum creatinine rose to 160-170 in
late September 2006 and  200 in early
October 2006 with no change in
medications or acute medical illness,
concern lead to imaging studies (generally
normal) and renal transplant biopsy
(November 6).
• Subsequently serum creatinine seems to
have fallen 190  155.
U06-21176
#428207110
• Systemic oxalosis
• Liver-kidney transplant 3 yrs ago
• Base creat ~ 130
• Recent increase to 180-210 range
• ?rejection
• ?recurrent disease
IF
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IgG- Negative.
IgA- Negative.
IgM- Negative.
C3- Moderate vascular staining.
C1q- Negative.
Kappa- Negative.
Lambda- Negative.
Fibrinogen- Mild interstitial staining.
Albumin- Negative.
C4d- Negative.
C3
Fibrin
EM
• Will be ready in the coming weeks
Diagnosis
Renal Biopsy:
• Chronic pyelonephritis with medullary
calcium oxalate deposition related to
systemic oxalosis
• No evidence of rejection
• Banff scores:
– G0 CG1 I1 CI1 T0 CT1 V0 CV1 AH0 MM0