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FOLLOW UP IN THALASSEMIA CLINIC

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Presentation on theme: "FOLLOW UP IN THALASSEMIA CLINIC"— Presentation transcript:

1 FOLLOW UP IN THALASSEMIA CLINIC
Presented by: Dr. Afsana Jahan (PGT) Dr. Aditi Baruah (MD); Dr. P. Bishwanath (MD) Department of Paediatrics Assam Medical College & Hospital, Dibrugarh

2 Introduction Thalassemia is the commonest chronic haematological disease needing repeated blood transfusion. North-East specially Assam is a reservoir of abnormal haemoglobins & also thalassemia because of ethnic diversity of the inhabitants. Present thalassemia clinic was established in April’2005, AMCH, Dibrugarh where regular follow-up of thalassemia pateints are done.

3 Aim of the Study To study the follow-up cases of haemo- globinopathy with special reference to thalassemia in thalassemia clinic

4 Materials & Methods Place of Study : Thalassemia clinic, AMCH
Period of Study : May’2005 to Oct’2007 Study universe : 50 diagnosed and registered cases of Haemoglobinopathy Selection criteria : Cases of Haemoglobinopathy diagnosed by HPLC.

5 Age & Sex distribution at registration

6 Distribution of haemoglobinopathies

7 Caste & Community distribution

8 Showing status of spleen
SPLEEN SIZE β THALASSEMIA MAJOR HbE – β THALASSEMIA Not palpable 1 3 < 2cm 2 2 – 7cm 11 >7cm 5 Splenectomized 4 6

9 Photograph showing facial features and heptosplenomegaly

10 Complications of Haemoglobinopathy

11 PHOTOGRAPH SHOWING BONY CHANGES IN SKULL X-RAY

12 Effect of chelation on Serum Ferritin
No. of patients Drug and dose mg/kg/day Length of study S. Ferritin level (μg/L) Initial Final 16 Deferiprone 50 – 100 1yr 3261 1420 4 Desferoxamine 35 – 55 3190 1392 3 Combined 9months 5261 1276

13 Effect of use of filter on transfusion reaction

14 Hb E trait - β – thalassemia trait
Parental HPLC study DIAGNOSIS CASE NO. MOTHER FATHER β – thalassemia major 49 β – thalassemia trait Hb E - thalassemia 1 Hb E trait 2 Not done 3 Hb E trait - β – thalassemia trait 6 7 8 13 40 48 Hb A A

15 Chromatogram

16 Problems in Thalassemia clinic
Family study and convincing parents and relatives for necessity of the test. Irregular follow-up visits. Irregular blood transfusion Lack of voluntary blood donation. Cost of filters and chelation therapy limits their use. Cost of different laboratory tests.

17 Need of the HOUR! Public awareness. Education of health professional.
Antenatal diagnosis. Emphasis on conventional treatment in particular chelation in view of its high efficacy. Cure of the disease with increasing possibility of bone marrow transplantation and gene therapy. Concrete support for achieving high standard for management of thalassemia in this part of Country.

18 Conclusion Thalassemia clinic is playing an important role in making thalassemia a preventable, manageable and curable disease in prospect of high incidence in this region.

19 THANK YOU & wish you all a very HAPPY NEW YEAR


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