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Original Research Article: Clinical Profile and Pattern of Hemoglobinopathies and Thalassemias Among Children Admitted in a Tertiary Care Hospital, Assam, India
| Content Provider | Semantic Scholar |
|---|---|
| Author | Kalita, Dulal Kalita, P. Kumar, D. Arun |
| Copyright Year | 2019 |
| Abstract | Introduction: Inherited hemoglobin disorders ( hemoglobinpathies and Thalassemias) constitute an important cause of mobidity and mortality in children. They impose a heavy burden on the affected families and the health sector. This is a public health problem in Assam and North Eastern states. Data pertaining to the pattern of the thalassemias and hemoglobinopathies are lacking in our state .Hence, this study was undertaken to know the clinical profile and pattern of hemoglobinpathies and thalassemias cases admitted in pediatrics department. Methods: The present study was conducted in the department of Pediatrics , Tezpur Medical College and hospital,Tezpur,Assam over a period of one year from January 2017 to December 2017 . Total 75 children of inherited hemoglobin disorders (chronic hemolytic anemia) were included in this study. This was a retrospective study. We collected the bed head tickets (medical record files) of all children from medical record department of the hospital. Relevant data of the cases were collected , analyzed and interpreted accordingly. Results: The present study included total 75 children of thalassemia and hemoglobinopathies admitted during the study period. Total number of patients admitted in the pediatric ward, during the study period were 1883. Out of 75 studied cases , 58.6% patients were male and 41.3% were female. In the present study, most common hemoglobin disorder was HbE trait (HbAS) 26.6% followed by Beta thalassemia trait 21.3% , Sickle cell trait (18.7%) , E beta thalassemia (9.3%) , HbE disease 8% and sickle cell disease (Hb SS ) 8% respectively. The present study also showed that all cases of beta thalassemia major , E beta thal and Sickle cell diseases had presented with pallor , generalised weakness and hepatosplenomegaly., It was also seen that all the beta thalassemia major, E beta thalassemia and Sickle cell diseases patients required blood transfusions. It was observed that 46.6% of cases were malnourished/stunted and 29.3% children had skeletal changes. Conclusion: The present study showed the pattern of thalassemias and hemoglobinopathies prevalent in Assam. We found that HbE trait/ HbE disease,Beta thalssemia major/trait,sickle cell anemia/trait , compound HbE beta thalassemia were prevalent in Assam and impose a big burden to the affected families as well as society. Further , many children remain undiagnosed at the community level due to lack of knowledge , poverty and lack of laboratory facility at tea garden and rural hospital. We know that this inherited hemoglobin disorder is a major cause of morbidity and mortality in children. Hence, mass education , genetic counselling, premarital counseling, neonatal screening and antenatal diagnosis are the mainstay for prevention and control of the disease. Further, Early diagnosis by HPLC , adequate management of the cases with blood transfusion, chelation therapy, nutritional supplementation and splenectomy will decrease the associated complications and improve the quality of life. |
| File Format | PDF HTM / HTML |
| Alternate Webpage(s) | http://iosrjournals.org/iosr-jdms/papers/Vol18-issue9/Series-9/B1809090509.pdf |
| Language | English |
| Access Restriction | Open |
| Content Type | Text |
| Resource Type | Article |
