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印度镰状细胞病患儿的脑血液动力学:一项观察性队列研究。

Cerebral hemodynamics in children with sickle cell disease in India: An observational cohort study.

机构信息

Department of Neurology, Zydus Hospital, Ahmedabad, Gujarat, India.

Department of Neurology, Ramkrishna Care Hospitals, Raipur, Chhattisgarh, India.

出版信息

Medicine (Baltimore). 2022 Jul 8;101(27):e29882. doi: 10.1097/MD.0000000000029882.

Abstract

India has the second highest number of cases of sickle cell disease (SCD) and affects the most socioeconomically disadvantaged communities living in a horizontal belt from Gujarat to Odisha state. Despite high prevalence, information about cerebral hemodynamics among children with SCD in India remains scarcely described. We performed transcranial Doppler (TCD) to assess cerebral hemodynamics among Indian children with SCD and evaluated their association with clinical and hematological parameters. Children aged 3-18years, diagnosed with SCD living in Raipur in Chhattisgarh and Ahmedabad in Gujarat state were recruited. TCD was performed to obtain flow velocities from middle cerebral (MCA), intracranial internal carotid (ICA) and basilar artery. Associations were evaluated between timed-average-mean-maximum velocities (TAMMV) and various clinical and hematological parameters. Our prospective study included 62 consecutive children with known SCD. Mean ± SD age of the study population was 9.8 ± 3.9 years and 31 (50%) were male. Mean ± SD hemoglobin was 8.64 ± 1.34 Gm/dL while the mean HbSS ± SD was 70.25 ± 15.27%. While 6 (9.6%) children had suffered from stroke during previous 2 years, 7 (11%) demonstrated abnormal TAMMV. Higher HbSS level along with history of iron chelation therapy, blood transfusion and/or stroke showed a trend towards having higher TAMMV. Stroke and cerebral hemodynamic alterations are common among Indian children with SCD. Larger studies with detailed neuroimaging and genetic evaluations are needed for better understanding, characterization, risk stratification as well as optimization of the timing of blood transfusion to reduce physical disabilities among Indian children with SCD.

摘要

印度是镰状细胞病(SCD)病例数第二高的国家,受该病影响最大的是居住在古吉拉特邦到奥里萨邦的水平带内的社会经济地位最低的社区。尽管患病率很高,但有关印度 SCD 儿童脑血液动力学的信息仍鲜有描述。我们进行了经颅多普勒(TCD)检查,以评估印度 SCD 儿童的脑血液动力学,并评估其与临床和血液学参数的关系。在恰蒂斯加尔邦的赖布尔和古吉拉特邦的艾哈迈达巴德,我们招募了年龄在 3-18 岁之间、被诊断患有 SCD 的居住在那里的儿童。TCD 用于从大脑中动脉(MCA)、颅内颈内动脉(ICA)和基底动脉获得流速。评估了 TAMMV 与各种临床和血液学参数之间的相关性。我们的前瞻性研究包括 62 例连续患有已知 SCD 的儿童。研究人群的平均年龄为 9.8 ± 3.9 岁,其中 31 人(50%)为男性。平均血红蛋白为 8.64 ± 1.34 Gm/dL,而平均 HbSS ± SD 为 70.25 ± 15.27%。在过去的 2 年中,有 6 名(9.6%)儿童患有中风,有 7 名(11%)儿童出现异常 TAMMV。较高的 HbSS 水平以及铁螯合疗法、输血和/或中风史与较高的 TAMMV 呈趋势相关。中风和脑血流动力学改变在印度 SCD 儿童中很常见。需要更大规模的研究,包括详细的神经影像学和遗传学评估,以更好地了解、描述、分层风险以及优化输血时机,以减少印度 SCD 儿童的身体残疾。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f755/9259145/196e7b578703/medi-101-e29882-g001.jpg

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