A clinico-epidemiological study of thalassemia cases in India

Nitin Joseph, Siddharth Pai, Shreejita Sengupta, Suchaita Bharadwaj, Saksham Dhawan, Kanishk Khare

Research output: Contribution to journalArticle

Abstract

Introduction: Thalassemia is the most common single-gene disorder in India. Hence, care of these patients becomes a priority issue. Objectives: This study was done to assess the clinical presentations and management practices in thalassemia. Materials and Methods: Case sheets of patients with thalassemia admitted over the past 10 years from 2005 to 2014 were examined and recorded in a validated pro forma. Results: Of the total 183 cases, 179 (97.8%) were of beta thalassemia major, 3 (1.6%) of beta thalassemia intermediate, and 1 (0.6%) of beta thalassemia minor category. The median age at diagnosis was 1 year. Hardly, one-fourth of the cases were diagnosed in the first 6 months. Majority of cases were under-fives 58 (31.7%) and were males 116 (63.4%). Fever was the most common presenting symptom 34 (18.6%). Pallor 179 (97.8%) followed by hepatomegaly 172 (94%) were the most common signs. Bone deformities were reported in 13 (7.1%) cases. Among the under-fives, more than one-third were underweight and more than half were stunted. The mean posttransfusion value of hemoglobin after 1 year of transfusion among cases was 10 ± 1.6 g percent. Iron chelation therapy using desferrioxamine was given to 51 (27.9%) cases. The mean age of starting this therapy was 11.1 ± 8.2 years. Splenectomy was done in 4 cases, all of them being cases of beta thalassemia major. The mean age while performing splenectomy was 10.7 ± 4.8 years. Lenticular opacity was present among greater proportion of thalassemia cases on treatment with desferrioxamine (P = 0.022). Conclusion: Several complications were identified among thalassemia cases. A multidisciplinary care approach is therefore required for solving these problems.

LanguageEnglish
Pages236-241
Number of pages6
JournalJournal of Natural Science, Biology and Medicine
Volume9
Issue number2
DOIs
Publication statusPublished - 01-07-2018

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Deferoxamine
Thalassemia
beta-Thalassemia
Epidemiologic Studies
India
Opacity
Chelation
Bone
Hemoglobins
Iron
Genes
Splenectomy
Chelation Therapy
Pallor
Hepatomegaly
Thinness
Practice Management
Patient Care
Fever
Bone and Bones

All Science Journal Classification (ASJC) codes

  • Biochemistry, Genetics and Molecular Biology(all)

Cite this

Joseph, Nitin ; Pai, Siddharth ; Sengupta, Shreejita ; Bharadwaj, Suchaita ; Dhawan, Saksham ; Khare, Kanishk. / A clinico-epidemiological study of thalassemia cases in India. In: Journal of Natural Science, Biology and Medicine. 2018 ; Vol. 9, No. 2. pp. 236-241.
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abstract = "Introduction: Thalassemia is the most common single-gene disorder in India. Hence, care of these patients becomes a priority issue. Objectives: This study was done to assess the clinical presentations and management practices in thalassemia. Materials and Methods: Case sheets of patients with thalassemia admitted over the past 10 years from 2005 to 2014 were examined and recorded in a validated pro forma. Results: Of the total 183 cases, 179 (97.8{\%}) were of beta thalassemia major, 3 (1.6{\%}) of beta thalassemia intermediate, and 1 (0.6{\%}) of beta thalassemia minor category. The median age at diagnosis was 1 year. Hardly, one-fourth of the cases were diagnosed in the first 6 months. Majority of cases were under-fives 58 (31.7{\%}) and were males 116 (63.4{\%}). Fever was the most common presenting symptom 34 (18.6{\%}). Pallor 179 (97.8{\%}) followed by hepatomegaly 172 (94{\%}) were the most common signs. Bone deformities were reported in 13 (7.1{\%}) cases. Among the under-fives, more than one-third were underweight and more than half were stunted. The mean posttransfusion value of hemoglobin after 1 year of transfusion among cases was 10 ± 1.6 g percent. Iron chelation therapy using desferrioxamine was given to 51 (27.9{\%}) cases. The mean age of starting this therapy was 11.1 ± 8.2 years. Splenectomy was done in 4 cases, all of them being cases of beta thalassemia major. The mean age while performing splenectomy was 10.7 ± 4.8 years. Lenticular opacity was present among greater proportion of thalassemia cases on treatment with desferrioxamine (P = 0.022). Conclusion: Several complications were identified among thalassemia cases. A multidisciplinary care approach is therefore required for solving these problems.",
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A clinico-epidemiological study of thalassemia cases in India. / Joseph, Nitin; Pai, Siddharth; Sengupta, Shreejita; Bharadwaj, Suchaita; Dhawan, Saksham; Khare, Kanishk.

In: Journal of Natural Science, Biology and Medicine, Vol. 9, No. 2, 01.07.2018, p. 236-241.

Research output: Contribution to journalArticle

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AU - Pai, Siddharth

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AB - Introduction: Thalassemia is the most common single-gene disorder in India. Hence, care of these patients becomes a priority issue. Objectives: This study was done to assess the clinical presentations and management practices in thalassemia. Materials and Methods: Case sheets of patients with thalassemia admitted over the past 10 years from 2005 to 2014 were examined and recorded in a validated pro forma. Results: Of the total 183 cases, 179 (97.8%) were of beta thalassemia major, 3 (1.6%) of beta thalassemia intermediate, and 1 (0.6%) of beta thalassemia minor category. The median age at diagnosis was 1 year. Hardly, one-fourth of the cases were diagnosed in the first 6 months. Majority of cases were under-fives 58 (31.7%) and were males 116 (63.4%). Fever was the most common presenting symptom 34 (18.6%). Pallor 179 (97.8%) followed by hepatomegaly 172 (94%) were the most common signs. Bone deformities were reported in 13 (7.1%) cases. Among the under-fives, more than one-third were underweight and more than half were stunted. The mean posttransfusion value of hemoglobin after 1 year of transfusion among cases was 10 ± 1.6 g percent. Iron chelation therapy using desferrioxamine was given to 51 (27.9%) cases. The mean age of starting this therapy was 11.1 ± 8.2 years. Splenectomy was done in 4 cases, all of them being cases of beta thalassemia major. The mean age while performing splenectomy was 10.7 ± 4.8 years. Lenticular opacity was present among greater proportion of thalassemia cases on treatment with desferrioxamine (P = 0.022). Conclusion: Several complications were identified among thalassemia cases. A multidisciplinary care approach is therefore required for solving these problems.

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