Hepatic and renal functions of paediatric patients with thalassaemia: a cross-sectional study from two large thalassaemia centres in Sri Lanka
Objectives Thalassaemia is a genetic disorder of haemoglobin synthesis characterised by life-long chronic anaemia. Although the endocrine and cardiac complications of thalassaemia are well-studied, hepatic and renal complications are understudied. This study aims to describe the hepatic and renal fu...
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BMJ Publishing Group
2025-02-01
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| Series: | BMJ Open |
| Online Access: | https://bmjopen.bmj.com/content/15/2/e089784.full |
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| author | Meranthi Fernando Anuja Premawardhena Sachith Mettananda Hashan Pathiraja Wasana Wijenayake Raveen Thennakoon Dayananda Bandara Roshitha De Silva |
| author_facet | Meranthi Fernando Anuja Premawardhena Sachith Mettananda Hashan Pathiraja Wasana Wijenayake Raveen Thennakoon Dayananda Bandara Roshitha De Silva |
| author_sort | Meranthi Fernando |
| collection | DOAJ |
| description | Objectives Thalassaemia is a genetic disorder of haemoglobin synthesis characterised by life-long chronic anaemia. Although the endocrine and cardiac complications of thalassaemia are well-studied, hepatic and renal complications are understudied. This study aims to describe the hepatic and renal functions and to understand their determinants among paediatric patients with β-thalassaemia.Design Cross-sectional study.Setting Two largest thalassaemia centres in Sri Lanka.Participants All haematologically confirmed patients with β-thalassaemia aged 1–16 years attending the study sites were recruited between 1 January and 31 March 2023. Data were collected by interviewing parents and patients, performing physical examinations and perusing clinical records.Results 72 children (girls 52.8%) were recruited. The mean age was 7.3 years (SD 3.8). A majority (44 (61.1%)) had β-thalassaemia major, while 22 (30.6%) had haemoglobin E β-thalassaemia. 55 children (76.4%) were transfusion dependent. Hepatomegaly was found in 47 (65.3%), while 28 (38.9%) had elevations of both alanine and aspartate transaminases. Haemoglobin E β-thalassaemia type (OR 13.6, 95% CI 2.0 to 92, p=0.008) and high ferritin above 1000 ng/mL (OR 6.2, 95% CI 1.0 to 38, p=0.047) were independent factors associated with high transaminases. 11 (15.5%) patients had an estimated glomerular filtration rate (eGFR) below 90 mL/min. The proportion of children with low eGFR was higher in β-thalassaemia major (23.3%), transfusion-dependent (18.5%) and deferasirox treatment (18.5%) groups.Conclusions Elevation of hepatic transaminases is common among children with thalassaemia, especially among the subset of patients with haemoglobin E β-thalassaemia and those with high ferritin. Milder reductions in eGFR are noted in some patients with transfusion-dependent β-thalassaemia major. |
| format | Article |
| id | doaj-art-8e56ff269a0c46f6962139fe801c6344 |
| institution | Kabale University |
| issn | 2044-6055 |
| language | English |
| publishDate | 2025-02-01 |
| publisher | BMJ Publishing Group |
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| series | BMJ Open |
| spelling | doaj-art-8e56ff269a0c46f6962139fe801c63442025-02-07T06:10:14ZengBMJ Publishing GroupBMJ Open2044-60552025-02-0115210.1136/bmjopen-2024-089784Hepatic and renal functions of paediatric patients with thalassaemia: a cross-sectional study from two large thalassaemia centres in Sri LankaMeranthi Fernando0Anuja Premawardhena1Sachith Mettananda2Hashan Pathiraja3Wasana Wijenayake4Raveen Thennakoon5Dayananda Bandara6Roshitha De Silva71 Colombo North Teaching Hospital, Ragama, Sri Lanka1 Colombo North Teaching Hospital, Ragama, Sri Lanka1 Colombo North Teaching Hospital, Ragama, Sri Lanka2 Department of Paediatrics, University of Kelaniya, Kelaniya, Sri Lanka1 Colombo North Teaching Hospital, Ragama, Sri Lanka2 Department of Paediatrics, University of Kelaniya, Kelaniya, Sri Lanka3 Provincial General Hospital, Kurunegala, Sri Lanka4 Department of Pathology, University of Kelaniya, Kelaniya, Sri LankaObjectives Thalassaemia is a genetic disorder of haemoglobin synthesis characterised by life-long chronic anaemia. Although the endocrine and cardiac complications of thalassaemia are well-studied, hepatic and renal complications are understudied. This study aims to describe the hepatic and renal functions and to understand their determinants among paediatric patients with β-thalassaemia.Design Cross-sectional study.Setting Two largest thalassaemia centres in Sri Lanka.Participants All haematologically confirmed patients with β-thalassaemia aged 1–16 years attending the study sites were recruited between 1 January and 31 March 2023. Data were collected by interviewing parents and patients, performing physical examinations and perusing clinical records.Results 72 children (girls 52.8%) were recruited. The mean age was 7.3 years (SD 3.8). A majority (44 (61.1%)) had β-thalassaemia major, while 22 (30.6%) had haemoglobin E β-thalassaemia. 55 children (76.4%) were transfusion dependent. Hepatomegaly was found in 47 (65.3%), while 28 (38.9%) had elevations of both alanine and aspartate transaminases. Haemoglobin E β-thalassaemia type (OR 13.6, 95% CI 2.0 to 92, p=0.008) and high ferritin above 1000 ng/mL (OR 6.2, 95% CI 1.0 to 38, p=0.047) were independent factors associated with high transaminases. 11 (15.5%) patients had an estimated glomerular filtration rate (eGFR) below 90 mL/min. The proportion of children with low eGFR was higher in β-thalassaemia major (23.3%), transfusion-dependent (18.5%) and deferasirox treatment (18.5%) groups.Conclusions Elevation of hepatic transaminases is common among children with thalassaemia, especially among the subset of patients with haemoglobin E β-thalassaemia and those with high ferritin. Milder reductions in eGFR are noted in some patients with transfusion-dependent β-thalassaemia major.https://bmjopen.bmj.com/content/15/2/e089784.full |
| spellingShingle | Meranthi Fernando Anuja Premawardhena Sachith Mettananda Hashan Pathiraja Wasana Wijenayake Raveen Thennakoon Dayananda Bandara Roshitha De Silva Hepatic and renal functions of paediatric patients with thalassaemia: a cross-sectional study from two large thalassaemia centres in Sri Lanka BMJ Open |
| title | Hepatic and renal functions of paediatric patients with thalassaemia: a cross-sectional study from two large thalassaemia centres in Sri Lanka |
| title_full | Hepatic and renal functions of paediatric patients with thalassaemia: a cross-sectional study from two large thalassaemia centres in Sri Lanka |
| title_fullStr | Hepatic and renal functions of paediatric patients with thalassaemia: a cross-sectional study from two large thalassaemia centres in Sri Lanka |
| title_full_unstemmed | Hepatic and renal functions of paediatric patients with thalassaemia: a cross-sectional study from two large thalassaemia centres in Sri Lanka |
| title_short | Hepatic and renal functions of paediatric patients with thalassaemia: a cross-sectional study from two large thalassaemia centres in Sri Lanka |
| title_sort | hepatic and renal functions of paediatric patients with thalassaemia a cross sectional study from two large thalassaemia centres in sri lanka |
| url | https://bmjopen.bmj.com/content/15/2/e089784.full |
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