一例先前未确诊的镰状细胞病患者因热力消耗导致休克和死亡。

Q2 Medicine Medico-Legal Journal Pub Date : 2025-03-01 Epub Date: 2022-09-22 DOI:10.1177/00258172221114567
Jagadish Rao Padubidri, Amanda Christina Pinto, Jyoti R Kini, Hema Kini, Matthew Antony Manoj, Tanya Singh
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引用次数: 0

摘要

镰状细胞病(SCD)是一种常染色体隐性遗传病,其特征是血红蛋白(HbS)发生变异。HbS 在低氧条件下聚合成针状长纤维,导致红细胞形成镰刀形红细胞。随着镰状红细胞的反复出现,红细胞会不可逆转地镰状化并滞留在血液循环中,从而导致血管闭塞性危象。患者是一名 25 岁的女性,之前未确诊为 SCD,表现为高烧、脾脏肿大,因热力消耗诱发镰状细胞危象、多器官功能衰竭和休克而死亡。
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Heat exertion precipitating shock and death in a case of previously undiagnosed sickle-cell disease.

Sickle cell disease (SCD) is an autosomal recessive genetic condition characterized by the presence of a mutated form of haemoglobin (HbS). HbS polymerises into long needle-like fibres under low oxygen conditions, leading to the erythrocytes forming sickle shaped red blood cells. With repeated sickling, the red blood cells become irreversibly sickled and trapped within the circulation, and this leads to vaso-occlusive crisis. The patient, a 25-year-old female, previously undiagnosed with SCD, presented with high grade fever, splenomegaly and succumbed due to heat exertion precipitating sickling crisis, multiorgan failure and shock.

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来源期刊
Medico-Legal Journal
Medico-Legal Journal Medicine-Medicine (all)
CiteScore
3.00
自引率
0.00%
发文量
63
期刊介绍: The Medico-Legal journal is the official journal of Medico-Legal Society. The Royal Society of Medicine Press is delighted to announce the launch in July 2009 of the Medico-Legal journal, on behalf of the Medico-Legal Society. The Medico-Legal Journal provides an official record of the proceedings of the Medico-Legal Society, and is dedicated to promoting Medico-Legal knowledge in all its aspects. As well as providing a record of activity in the Society, the journal includes a unique collection of contributions and speeches from eminent speakers at society events.
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