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Renal Stones - Is liver the culprit?
Presenter:
Shivani Deswal - Apollo Hospital, New Delhi

Moderator:

Panelists:

John ...
Renal stones - is liver the culprit?
Dr Shivani Deswal
Apollo Centre for Advanced Pediatrics
13 year old Sudanese boy presented with
Passage of small stones in urine - 6 years
Generalized swelling and anuria - 12 ho...
No history of
hematuria / dysuria / UTI
polyuria / polydipsia
flank pain
diarrhea
excessive vitamin intake
bone pains / fr...
Diagnosed as ESKD 1 year back
On hemodialysis 3 times a week
Non consanguineous marriage
3rd in birth order
2 siblings alive and healthy
No family h/o renal stones
On examination
Height 145 cm and weight 29 Kg (BMI-13.8)
Pale
B.P-180/100 mm Hg
Anasarca
No icterus
P/A- liver 2 cm BCM, s...
Investigations
Urine- Ph-6
calcium oxalates++
pus cells, RBCs nil
24 hr urinary oxalate - 180 mg/24h/1.73 m2
(>100 mg /24 ...
Blood urea- 70mg/dl
S.creatinine-3.5 mg/dl
GFR- 17 ml/min/1.73 m2
LFT
TSB-0.6 mg/dl
SGOT/SGPT-18/15 IU
ALP-220 IU
INR-1.1
...
Plasma oxalate level - 81.9 mmol/L (normal <1.8)
Bone Marrow biopsy - oxalate crystals +
Whole-gene sequencing of the AGXT...
Hemodialysis done (SLED) 10 hours a day
Antihypertensives
LR Simultaneous Liver - Kidney Transplant
Hemodialysis done 12 hours prior to surgery
Kidney Donor - Sister , 27 years , B+ive
Right open donor nephrectomy
Liver Do...
Post transplant
POD 2
Bile leak in drain
Rexploration - Duct to duct anastomosis was
taken down and Hepatoco-jeujonostomy
...
Renal biopsy
Pale bluish-white radially arranged laminated crystals
were seen
Interstitium showed fibrosis
Extensive intra...
Primary hyperoxaluria type-1 (PH1)
Autosomal recessive disorder
Deficiency of the hepatic enzyme alanine- glyoxylate - ami...
Calcium oxalate stones
Transplantation for primary oxalosis
Preemptive liver transplant
Slowly declining GFR - 40-60 ml/min/1.73 m 2
Optimal timi...
LK transplant
Simultaneous
Sequential
When not to transplant

Severe systemic oxalosis with cardiac disease
Indications for Liver transplantation
Normal Liver – extra hepatic disease
Crigler- Najjar Type I
Organic acidemias
Urea c...
LK transplant
Primary hyperoxaluria type 1
Autosomal recessive polycystic kidney disease
Liver disease with an occasional ...
Learning from each other
Take home message
Renal stones is liver the culprit
Renal stones is liver the culprit
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Renal stones is liver the culprit

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Transcript of "Renal stones is liver the culprit"

  1. 1. Renal Stones - Is liver the culprit? Presenter: Shivani Deswal - Apollo Hospital, New Delhi Moderator: Panelists: John Matthai – Pediatric Gastroenterologist, PSG Inst. of Medl Sciences, Coimbatore Winita Hardikar – Pediatric Hepatologist, Royal Children's Hospital, Melbourne, Australia Kumud Mehta – Pediatric Nephrologist, Jaslok Hospital, Mumbai Anupam Sibal – Pediatric Gastroenterologist, Apollo Hospital, New Delhi
  2. 2. Renal stones - is liver the culprit? Dr Shivani Deswal Apollo Centre for Advanced Pediatrics
  3. 3. 13 year old Sudanese boy presented with Passage of small stones in urine - 6 years Generalized swelling and anuria - 12 hours
  4. 4. No history of hematuria / dysuria / UTI polyuria / polydipsia flank pain diarrhea excessive vitamin intake bone pains / fractures
  5. 5. Diagnosed as ESKD 1 year back On hemodialysis 3 times a week
  6. 6. Non consanguineous marriage 3rd in birth order 2 siblings alive and healthy No family h/o renal stones
  7. 7. On examination Height 145 cm and weight 29 Kg (BMI-13.8) Pale B.P-180/100 mm Hg Anasarca No icterus P/A- liver 2 cm BCM, span-10cm No e/o encephalopathy
  8. 8. Investigations Urine- Ph-6 calcium oxalates++ pus cells, RBCs nil 24 hr urinary oxalate - 180 mg/24h/1.73 m2 (>100 mg /24 h per 1.73 m2) Urinary oxalate/creatinine - 51 (<70-82) Spot calcium/creatinine - 0.12 (<0.2) 24hr urine calcium - 60mg/day (<4mg/kg/day)
  9. 9. Blood urea- 70mg/dl S.creatinine-3.5 mg/dl GFR- 17 ml/min/1.73 m2 LFT TSB-0.6 mg/dl SGOT/SGPT-18/15 IU ALP-220 IU INR-1.1 S.PTH - 51.8 pg/ml (11-54 pg/ml) Vitamin D (25 OH) - 32 ng /ml (10-55 ng/ml)
  10. 10. Plasma oxalate level - 81.9 mmol/L (normal <1.8) Bone Marrow biopsy - oxalate crystals + Whole-gene sequencing of the AGXT gene s/o Primary Hyperoxaluria Type 1
  11. 11. Hemodialysis done (SLED) 10 hours a day Antihypertensives LR Simultaneous Liver - Kidney Transplant
  12. 12. Hemodialysis done 12 hours prior to surgery Kidney Donor - Sister , 27 years , B+ive Right open donor nephrectomy Liver Donor - Sister, 27 years, B+ive Left lobe graft
  13. 13. Post transplant POD 2 Bile leak in drain Rexploration - Duct to duct anastomosis was taken down and Hepatoco-jeujonostomy done Urine routine - PH-7,RBCs-35-40,Oxalate crystals 1-2 /hpf
  14. 14. Renal biopsy Pale bluish-white radially arranged laminated crystals were seen Interstitium showed fibrosis Extensive intraluminal and interstitial deposits of oxalate crystals
  15. 15. Primary hyperoxaluria type-1 (PH1) Autosomal recessive disorder Deficiency of the hepatic enzyme alanine- glyoxylate - aminotransferase which catalyzes the conversion of glyoxylate to glycine Increased oxalate from liver, excreted by kidneys Ca oxalate deposits, stones, nephrocalcinosis, renal failure Also bones, vessels, heart Haemodialysis provides insufficient oxalate clearance
  16. 16. Calcium oxalate stones
  17. 17. Transplantation for primary oxalosis Preemptive liver transplant Slowly declining GFR - 40-60 ml/min/1.73 m 2 Optimal timing debatable
  18. 18. LK transplant Simultaneous Sequential
  19. 19. When not to transplant Severe systemic oxalosis with cardiac disease
  20. 20. Indications for Liver transplantation Normal Liver – extra hepatic disease Crigler- Najjar Type I Organic acidemias Urea cycle defects Protein C deficiency Familial hypercholesterolemia
  21. 21. LK transplant Primary hyperoxaluria type 1 Autosomal recessive polycystic kidney disease Liver disease with an occasional kidney failure Atypical hemolytic uremic syndrome Methylmalonic acidemia
  22. 22. Learning from each other
  23. 23. Take home message

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