Pubertal Development in Girls with Beta Thalassaemia and Assessment of the Adequacy of Chelation Therapy: A Quasi-experimental Study
Introduction: Beta thalassaemia is the most prevalent hereditary autosomal disorder, significantly impacting endocrine function during pubertal development. The pathology is rooted in the excessive deposition of iron in vital organs. If left untreated, this condition leads to serious morbidity and m...
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JCDR Research and Publications Private Limited
2024-03-01
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| Series: | Journal of Clinical and Diagnostic Research |
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| Online Access: | https://www.jcdr.net/articles/PDF/19165/64430_CE[Ra1]_F(SL)_Ref_PAT(SL)_QC(SHK_IS))_PF1(RI_DK_OM)_PFA(RI_KM)_PN(KM).pdf |
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| author | Rupali Modak Amitava Pal Shuvendu Das Dilip Kumar Biswas |
| author_facet | Rupali Modak Amitava Pal Shuvendu Das Dilip Kumar Biswas |
| author_sort | Rupali Modak |
| collection | DOAJ |
| description | Introduction: Beta thalassaemia is the most prevalent hereditary autosomal disorder, significantly impacting endocrine function during pubertal development. The pathology is rooted in the excessive deposition of iron in vital organs. If left untreated, this condition leads to serious morbidity and mortality. Hypogonadism stands as the most common endocrine complication.
Aim: To observe pubertal development in girls with thalassaemia and evaluate the adequacy of chelation therapy in such patients by measuring levels of Follicle Stimulating Hormone (FSH), Luteinising Hormone (LH), oestrogen, and serum ferritin.
Materials and Methods: A quasi-experimental study was conducted at the Thalassaemia Clinic at the Department of Obstetrics and Gynaecology, Burdwan Medical College, Purba Bardhaman, West Bengal, India involving 300 diagnosed Beta Thalassaemia Major (BTM) (case) patients aged 13 to 17 years over a period of one and a half years (January 2020 to June 2021). The girls in the study groups were sequentially enrolled from the Outpatient Department of the Thalassaemia Clinic after meeting inclusion and exclusion criteria. Demographic data, anthropometric measurements, and Sexual Maturity Rating (SMR) were recorded. Hormonal assays of serum FSH, LH, oestrogen, and serum ferritin levels were conducted. Thalassaemic girls with serum ferritin levels exceeding 500 ng/mL received chelation therapy. Serum ferritin levels were measured at two-month intervals for up to six months, with reassessment of serum FSH, LH, oestrogen, and ferritin levels at the end of the six-month period. Statistical analysis was conducted using Statistical Package for Social Sciences (SPSS) version 27.0, Microsoft excel spreadsheet, and Epi Info 7.
Results: A total of 190 (63.3%) of the thalassaemic girls were aged 13 to 15 years, with a mean Body Mass Index (BMI) of 22.5000±2.6100 kg/m2, and 74 of them (38.94%) experienced menarche in this age group. Out of 300 cases, 269 thalassaemic girls (89.6%) received chelation therapy, and 89 cases (33.09%) reached menarche after chelation therapy. A significant (p<0.0001) increase in mean LH and FSH levels was observed after chelation therapy. The mean ferritin level decreased from 3168.85 ng/mL to 2227.24 ng/mL following chelation therapy.
Conclusion: Pubertal development failure is common in beta thalassaemic girls. Intervention in the form of adequate chelation therapy in girls with high serum ferritin levels yielded favourable outcomes, as evident from serum gonadotropin and oestrogen levels. |
| first_indexed | 2024-04-25T01:13:23Z |
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| id | doaj.art-2f32455b153e428ebd686808b3d7605e |
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| publishDate | 2024-03-01 |
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| series | Journal of Clinical and Diagnostic Research |
| spelling | doaj.art-2f32455b153e428ebd686808b3d7605e2024-03-09T11:52:48ZengJCDR Research and Publications Private LimitedJournal of Clinical and Diagnostic Research2249-782X0973-709X2024-03-011803050910.7860/JCDR/2024/64430.19165Pubertal Development in Girls with Beta Thalassaemia and Assessment of the Adequacy of Chelation Therapy: A Quasi-experimental StudyRupali Modak0Amitava Pal1Shuvendu Das2Dilip Kumar Biswas3Assistant Professor, Department of Obstetrics and Gynaecology, RG Kar Medical College, Kolkata, West Bengal, India.Professor, Department of Obstetrics and Gynaecology, MJN Medical College, Coochbehar, West Bengal, India.Junior Resident, Department of Obstetrics and Gynaecology, Burdwan Medical College, Burdwan, West Bengal, India.Associate Professor, Department of Obstetrics and Gynaecology, Burdwan Medical College, Burdwan, West Bengal, India.Introduction: Beta thalassaemia is the most prevalent hereditary autosomal disorder, significantly impacting endocrine function during pubertal development. The pathology is rooted in the excessive deposition of iron in vital organs. If left untreated, this condition leads to serious morbidity and mortality. Hypogonadism stands as the most common endocrine complication. Aim: To observe pubertal development in girls with thalassaemia and evaluate the adequacy of chelation therapy in such patients by measuring levels of Follicle Stimulating Hormone (FSH), Luteinising Hormone (LH), oestrogen, and serum ferritin. Materials and Methods: A quasi-experimental study was conducted at the Thalassaemia Clinic at the Department of Obstetrics and Gynaecology, Burdwan Medical College, Purba Bardhaman, West Bengal, India involving 300 diagnosed Beta Thalassaemia Major (BTM) (case) patients aged 13 to 17 years over a period of one and a half years (January 2020 to June 2021). The girls in the study groups were sequentially enrolled from the Outpatient Department of the Thalassaemia Clinic after meeting inclusion and exclusion criteria. Demographic data, anthropometric measurements, and Sexual Maturity Rating (SMR) were recorded. Hormonal assays of serum FSH, LH, oestrogen, and serum ferritin levels were conducted. Thalassaemic girls with serum ferritin levels exceeding 500 ng/mL received chelation therapy. Serum ferritin levels were measured at two-month intervals for up to six months, with reassessment of serum FSH, LH, oestrogen, and ferritin levels at the end of the six-month period. Statistical analysis was conducted using Statistical Package for Social Sciences (SPSS) version 27.0, Microsoft excel spreadsheet, and Epi Info 7. Results: A total of 190 (63.3%) of the thalassaemic girls were aged 13 to 15 years, with a mean Body Mass Index (BMI) of 22.5000±2.6100 kg/m2, and 74 of them (38.94%) experienced menarche in this age group. Out of 300 cases, 269 thalassaemic girls (89.6%) received chelation therapy, and 89 cases (33.09%) reached menarche after chelation therapy. A significant (p<0.0001) increase in mean LH and FSH levels was observed after chelation therapy. The mean ferritin level decreased from 3168.85 ng/mL to 2227.24 ng/mL following chelation therapy. Conclusion: Pubertal development failure is common in beta thalassaemic girls. Intervention in the form of adequate chelation therapy in girls with high serum ferritin levels yielded favourable outcomes, as evident from serum gonadotropin and oestrogen levels.https://www.jcdr.net/articles/PDF/19165/64430_CE[Ra1]_F(SL)_Ref_PAT(SL)_QC(SHK_IS))_PF1(RI_DK_OM)_PFA(RI_KM)_PN(KM).pdfbeta thalassaemic patientsgonadotropinhormonal levelshypogonadismpubertysexual maturity rating |
| spellingShingle | Rupali Modak Amitava Pal Shuvendu Das Dilip Kumar Biswas Pubertal Development in Girls with Beta Thalassaemia and Assessment of the Adequacy of Chelation Therapy: A Quasi-experimental Study Journal of Clinical and Diagnostic Research beta thalassaemic patients gonadotropin hormonal levels hypogonadism puberty sexual maturity rating |
| title | Pubertal Development in Girls with Beta Thalassaemia and Assessment of the Adequacy of Chelation Therapy: A Quasi-experimental Study |
| title_full | Pubertal Development in Girls with Beta Thalassaemia and Assessment of the Adequacy of Chelation Therapy: A Quasi-experimental Study |
| title_fullStr | Pubertal Development in Girls with Beta Thalassaemia and Assessment of the Adequacy of Chelation Therapy: A Quasi-experimental Study |
| title_full_unstemmed | Pubertal Development in Girls with Beta Thalassaemia and Assessment of the Adequacy of Chelation Therapy: A Quasi-experimental Study |
| title_short | Pubertal Development in Girls with Beta Thalassaemia and Assessment of the Adequacy of Chelation Therapy: A Quasi-experimental Study |
| title_sort | pubertal development in girls with beta thalassaemia and assessment of the adequacy of chelation therapy a quasi experimental study |
| topic | beta thalassaemic patients gonadotropin hormonal levels hypogonadism puberty sexual maturity rating |
| url | https://www.jcdr.net/articles/PDF/19165/64430_CE[Ra1]_F(SL)_Ref_PAT(SL)_QC(SHK_IS))_PF1(RI_DK_OM)_PFA(RI_KM)_PN(KM).pdf |
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