1. UNUSUAL CASES OF RENAL FAILURE
WITH CRYSTAL NEPHROPATHY.
Kiran Sathe
Fellow- Pediatric Nephrology ( IPNA, ISPN )
Bai Jerbai Wadia Hospital For children

2.  4 months old female child
 Oliguric renal failure with convulsions and
respiratory distress following acute
gastroenteritis
 No response to fluid and furosemide
challenge
 Referred for further care

3.  IIIrd degree consanguinity
 Previous sibling (male) death at 3 mo –
convulsions.
 4 kg/ 53 cm ,B wt 2.5 kg
 Pale,dehydrated,acidotic breathing
 No renal lump, rash, chest- clear

4. Initial Investigations
Hb 5.4
TC 12400
plt 3.5 lac
Retic
count
0.5%
BUN 110
creat 7.2
Na/ K/Cl 13/5.2/105
AG 25
pH 7.15
HCo3 6.5
 Urine-alb 2+, 60-80
RBC,10-12 PC
 FeNa-67.8
 Urine alb/creat ratio 7.2
 USG- RK-5.1 * 1.5 cm
 LK-4.7 * 1.8 cm
 nephrocalcinosis
 Doppler-N

5. USG- Nephrocalcinosis

6. Possible D.Ds:
 Drug induced- Furosemide,Vit D
 Metabolic causes-
 Distal RTA,Bartter’s
syndrome,Hyperoxaluria.

7.  Peritoneal Dialysis instituted for 3 days
 Uremia improved
 Packed cells transfusion
 CAPD- in view of prolonged oliguric renal
failure

8. Renal Biopsy- LM, polarised

9. Oxalosis, Kidney
 Normal glomeruli
 All tubules distended with oxalate crystals,
few surounded by giant cells
 Interstitium- focally fibrotic
 Vesseles-unremarkable
 IF- negative

10. Case 2
 Dr Kiran Sathe.
 Dr Kumud Mehta.
 Jaslok Hospital & Research Centre.

11.  6 yr old muslim girl presented with-
 colicky abdominal pain
 severe anemia
 Edema
 Breathlessness
 Short Stature Ht 98 cm, wt 14 kg
 Firm Hepatosplenomegaly

12. Family h/o
 Consanguinity 3 rd degree
 Elder sibling-female
 Died at 7 months with renal failure
 USG- nephrocalcinosis
 H/o cousin- died at 15th day of life with renal
failure requiring peritoneal dialysis.

13.  Chronic Kidney Disease stage 5
 (End stage renal disease)
 Extensively investigated in infancy in view of
significant family h/o
 Diagnosed as Hyperoxaluria.

14. age Hb creat Ca/p/
Alp
Hco3 Urine
alb
Urin
e
RBC
Urine
Pus
cells
Urine
Cal/
creat
Urine
oxala
te
USG
5 mo 11.5 0.4 9.7/
4.9/
342
19 2+ 5-6 5-6 0.9
12 mo 9.8 0.6 8.1/
2.9/
543
10.8 2+ 4-5 15-20 neph
rocal
cinosi
s
24 mo 8.5 0.7 17 20-
25
8-10 36mg
/d
32 mo 7.1 2.6 7.4/
6.2/
217
20 82mg
/d
neph
rocal
cinosi
s
60 mo 8.0 5.0 21 Normal 24 hr urine
oxalate excretion
<2 mg/kg
<45 mg/1.73 m②
72 mo 7.2 8.7 9.4/
10.4
21 2+

15. Family counseled about the poor prognosis
and the treatment options.
Renal supportive meds
Oral Pyridoxine
Further deterioration
Severe cardiac dysfunction and pulmonary
edema.

16. Primary hyperoxaluria

17. questions
 1. what are the possible clinical presentations for
hyperoxaluria and oxalosis?
 2.When do we suspect it?
 3. organ systems which are affected and the
pathogenesis?
 4. how do we diagnose it?
 5.Treatment options available?
 6.Ideal time to consider-
 Liver transplant
 Combined kidney- liver transplant