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一名镰状细胞病的耶和华见证会儿童出现明显升高的经颅多普勒速度值的无血管理:一家三级中心的经验-病例报告。

Bloodless management of significantly elevated transcranial Doppler velocity value in a Jehovah's witness child with sickle cell disease: A tertiary centre experience-A case report.

机构信息

Department of Paediatrics, Faculty of Medicine, Nnamdi Azikiwe University, Nnewi Campus, Anambra State, Nigeria.

Department of Haematology and Blood Transfusion, Faculty of Basic Clinical Sciences, Nnamdi Azikiwe University, Nnewi Campus, Anambra State, Nigeria.

出版信息

J Natl Med Assoc. 2024 Jun;116(3):247-251. doi: 10.1016/j.jnma.2024.01.016. Epub 2024 Feb 2.

Abstract

BACKGROUND

Effective management of complications in sickle cell disease (SCD), such as stroke prevention, often necessitates the use of blood transfusions. However, individuals who adhere to the religious tenets of Jehovah's Witnesses strictly abstain from accepting blood transfusions, thereby presenting a formidable challenge in clinical decision-making.

CASE REPORT

This is a case of a 3 year old child Jehovah's Witness who was found to have significantly elevated transcranial Doppler (TCD) velocity values between 193 and 203 cm/s, following routine screening. This was an otherwise clinically stable child, whose mother was diligently ensuring he had adequate medical care. Ideally, a prophylactic exchange blood transfusion program would have been commenced immediately but was not done due to due to the lack of consent from the caregiver. Patient was initially on hydroxyurea at 15 mg/kg and self medicating on omega 3 supplements and astymin syrup. Further elevation of TCD velocity upto 242 cm/s after a repeat testing, necessitated graduated increase of the dosage of hydroxyurea to 35 mg/kg to optimize its therapeutic effect, and discontinuation of omega 3 fatty acids and replacement of astymin with folic acid, vitamin C and B complex. Following these adjustments, the TCD dropped to below 190 cm/s reducing the risk of stroke in the child.

CONCLUSION

This case report demonstrates the successful implementation of a bloodless management strategy for stroke prevention in a Jehovah's Witness child with SCD. This study contributes to the existing literature by providing valuable insights and practical guidance for healthcare providers facing similar ethical and medical dilemmas.

摘要

背景

镰状细胞病(SCD)并发症的有效管理,如预防中风,通常需要输血。然而,遵守耶和华见证人宗教教义的人严格拒绝接受输血,这在临床决策中带来了巨大的挑战。

病例报告

这是一名 3 岁的耶和华见证人儿童的病例,在常规筛查中发现其经颅多普勒(TCD)速度值显著升高,介于 193 至 203cm/s 之间。这是一个临床情况稳定的孩子,他的母亲一直在努力确保他得到充分的医疗护理。理想情况下,应立即开始预防性换血输血计划,但由于护理人员不同意,因此未进行。患者最初接受 15mg/kg 的羟基脲治疗,并自行服用欧米伽 3 补充剂和 astymin 糖浆。重复测试后 TCD 速度进一步升高至 242cm/s,需要逐渐增加羟基脲的剂量至 35mg/kg,以优化其治疗效果,并停用欧米伽 3 脂肪酸,用叶酸、维生素 C 和 B 复合物代替 astymin。进行这些调整后,TCD 降至 190cm/s 以下,降低了儿童中风的风险。

结论

本病例报告展示了在一名 SCD 耶和华见证人儿童中成功实施无血管理策略预防中风的情况。本研究通过为面临类似伦理和医疗困境的医疗保健提供者提供有价值的见解和实用指导,为现有文献做出了贡献。

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