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应对β地中海贫血护理中的复杂性:来自资源有限环境的病例系列

Addressing complexities in β-thalassemia care: a case series from a resource-limited setting.

作者信息

Adhikari Pratik

机构信息

B.P. Koirala Institute of Health Sciences, Dharan, Nepal.

出版信息

Ann Med Surg (Lond). 2024 Aug 14;86(9):4979-4983. doi: 10.1097/MS9.0000000000002471. eCollection 2024 Sep.

Abstract

INTRODUCTION AND IMPORTANCE

β-thalassemia is a hereditary blood disorder with a global prevalence, presenting diagnostic and management challenges, particularly in regions with high consanguinity rates. Diagnostic methods include clinical assessments, genetic testing, and hemoglobin electrophoresis. Treatment typically involves transfusions and chelation therapy, with gene therapy showing promise. This case series emphasizes the need for tailored care strategies and global health initiatives to improve outcomes for β-thalassemia patients worldwide.

METHODS

This case series involves five patients from rural Nepal presenting various β-thalassemia manifestations. The cases highlight the challenges in diagnosis and management in resource-limited settings. Data were collected through clinical assessments, laboratory investigations, and follow-ups. Each patient's medical history, presentation, and treatment regimen were documented.

OUTCOMES

The cases underscore the importance of regular follow-ups, community engagement, and personalized treatment strategies tailored to genetic profiles. Key findings include the necessity for consistent transfusion schedules, iron overload monitoring, and managing complications associated with β-thalassemia. Enhanced education and healthcare collaboration were noted as critical for optimizing care and outcomes in resource-limited settings.

CONCLUSIONS

Managing β-thalassemia in resource-limited settings demands timely intervention, regular monitoring, and community involvement. Enhanced healthcare collaboration, access to advanced diagnostic tools, and tailored treatment strategies are paramount in addressing the unique challenges of β-thalassemia. These measures are essential for ensuring an improved quality of life for affected individuals in such regions.

摘要

引言与重要性

β地中海贫血是一种全球流行的遗传性血液疾病,带来了诊断和管理方面的挑战,尤其是在近亲结婚率高的地区。诊断方法包括临床评估、基因检测和血红蛋白电泳。治疗通常包括输血和螯合疗法,基因疗法显示出前景。本病例系列强调需要制定个性化的护理策略和全球健康倡议,以改善全球β地中海贫血患者的治疗效果。

方法

本病例系列涉及来自尼泊尔农村的五名表现出各种β地中海贫血症状的患者。这些病例突出了资源有限环境中诊断和管理的挑战。通过临床评估、实验室检查和随访收集数据。记录了每位患者的病史、症状表现和治疗方案。

结果

这些病例强调了定期随访、社区参与以及根据基因谱制定个性化治疗策略的重要性。主要发现包括持续输血计划、铁过载监测以及处理与β地中海贫血相关并发症的必要性。在资源有限的环境中,加强教育和医疗合作对于优化护理和治疗效果至关重要。

结论

在资源有限的环境中管理β地中海贫血需要及时干预、定期监测和社区参与。加强医疗合作、获得先进的诊断工具以及制定个性化治疗策略对于应对β地中海贫血的独特挑战至关重要。这些措施对于确保此类地区受影响个体的生活质量改善至关重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d650/11374262/ae4d325d3300/ms9-86-4979-g001.jpg

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