2. Case 2 A 23-year old Indian young man Homozygous Beta-Thalassemia Major Diagnosed at the age of 6 months. On regular transfusion every 4 weeks since that time. First visit to Thalassemia centre at the age of 8 years old.

3. Findings on 1 st presentation: Liver: 8cm Spleen: 9 cm. Hb: 6.2 g/dl. S. Ferritin: 1122 ng/l Hepatitis and HIV markers are negative

4. Recent Clinical and laboratory Evaluation Normal growth Normal pubertal development Pre-transfusion Hb maintained at 10g/dl HIV, HCV and HBV markers are negative Ethyroid Euglycemic Normal parathyroid function Normal cardiac function (EF 67%)

5. Chelation history: Started Desfral chelation therapy at the age of 2 years (June 1987) with good compliance ( s. ferritin 767) Patients wanted to be shifted to Exjade

6. What do next? A-Continue Desferal B-Shift to Exjade C-Shift to Deferiprone D-Shift to Deferiprone / Desferal

7. Underwent baseline investigation in preparation to shift to Exjade.

8. Baseline investigation FBC Acceptable S creatinine 0.8mg/dl Creatinine clearance 152.9 ml/min 24 hr Urinary protein <150 mg/ml Urea electrolyte normal Liver function test normal Normal cardiac function EF 67%

9. With this baseline investigations would you start exjade ? A-YES B-NO C-Need more investigations FBC Acceptable S creatinine 0.8mg/dl Creatinine clearance 152.9 ml/min 24 hr Urinary protein <150 mg/ml Urea electrolyte normal Liver function test normal Normal cardiac function EF 67%

10. Exjade was started in November 2006 at a dose of 1000mg (20mg/kg).

12. What to do next? A-Continue Exjade same dose and observe renal function B-Continue Exjade at a lower dose and observe renal function C-Stop Exjade and return to Desferal D-Stop all chelation therapy and evaluate renal function.

13. We stopped all chelation therapy and reevaluated renal function. Repeat Creatinine clearance Ultrasound KUB Nephrology consultation

14. Ultrasound KUB (15/102008) Left kidney Small & contracted (7.1 X 2.6cm) Suggesting Chronic contracted kidney? Congenital small left kidney? Right Kidney normal size and shape with decreased Corticomedullary differentiation Suggesting Renal parenchyma diseases or changes of nephritis Imaging

15. Nephrology opinion that these changes are longstanding and have existed before starting Exjade. Needs further evaluation for a cause?

18. Follow up after stop of Exjade Creatinine level decrease to 1 mg/dl (Jan2009) Creatinine Clearance increased to 84 ml/min (oct 2008).

19. We would like to recommend using creatinine clearance to monitor renal function in patients with starting S. creatinine high normal. All chelation should be with held including desferal if there is evidence of renal injury.