O'Brien Nicole F, Moons Peter, Johnson Hunter, Tshimanga Taty, Musungufu Davin Ambitapio, Ekandji Robert Tandjeka, Mbaka Jean Pongo, Babatila Lydia Kuseyila, Mayindombe Ludovic, Giresse Buba, Mwanza Suzanna, Lupumpaula Clement, Chilima Janet Simanguwa, Nanyangwe Alice, Kabemba Peter, Kafula Lisa Nkole, Phiri Tusekile, June Sylvester, Gushu Montfort Bernard, Chagaluka George, Chunda-Liyoka Catherine M
Department of Pediatrics Division of Critical Care Medicine Nationwide Children's Hospital, The Ohio State University Columbus Ohio USA.
Department of Pediatrics and Child Health Kamuzu University of Health Sciences Blantyre Malawi.
EJHaem. 2023 Nov 29;5(1):3-10. doi: 10.1002/jha2.818. eCollection 2024 Feb.
The greatest burden of sickle cell anemia (SCA) globally occurs in sub-Saharan Africa, where significant morbidity and mortality occur secondary to SCA-induced vasculopathy and stroke. Transcranial Doppler ultrasound (TCD) can grade the severity of vasculopathy, with disease modifying therapy resulting in stroke reduction in high-risk children. However, TCD utilization for vasculopathy detection in African children with SCA remains understudied. The objective was to perform a prospective, observational study of TCD findings in a cohort of children with SCA from the Democratic Republic of the Congo, Zambia, and Malawi. A total of 770 children aged 2-17 years without prior stroke underwent screening TCD. A study was scored as low risk when the time-averaged maximum of the mean (TAMMX) in the middle cerebral artery or terminal internal carotid artery was <170 cm/s but >50 cm/s, conditional risk when 170-200 cm/s, and high risk when >200 cm/s. Low-risk studies were identified in 604 children (78%), conditional risk in 129 children (17%), and high risk in three children (0.4%). Additionally, 34 (4%) were scored as having an unknown risk study (TAMMX <50 cm/s). Over the course of 15 months of follow-up, 17 children (2.2%) developed new neurologic symptoms (six with low-risk studies, seven with conditional risk, and four with unknown risk). African children with SCA in this cohort had a low rate of high-risk TCD screening results, even in those who developed new neurologic symptoms. Stroke in this population may be multifactorial with vasculopathy representing only one determinant. The development of a sensitive stroke prediction bundle incorporating relevant elements may help to guide preventative therapies in high-risk children.
镰状细胞贫血（SCA）在全球的最大负担出现在撒哈拉以南非洲地区，在那里，SCA引发的血管病变和中风导致了严重的发病率和死亡率。经颅多普勒超声（TCD）可以对血管病变的严重程度进行分级，疾病改善疗法可降低高危儿童中风的发生率。然而，TCD在非洲SCA儿童血管病变检测中的应用仍未得到充分研究。目的是对来自刚果民主共和国、赞比亚和马拉维的一组SCA儿童的TCD检查结果进行前瞻性观察研究。共有770名2至17岁且未曾中风的儿童接受了TCD筛查。当中脑动脉或颈内动脉终末段的平均时间平均最大流速（TAMMX）<170 cm/s但>50 cm/s时，研究被评为低风险；当TAMMX为170 - 200 cm/s时为条件风险；当TAMMX>200 cm/s时为高风险。604名儿童（78%）的研究被确定为低风险，129名儿童（17%）为条件风险，3名儿童（0.4%）为高风险。此外，34名儿童（4%）的研究被评为风险未知（TAMMX<50 cm/s）。在15个月的随访过程中，17名儿童（2.2%）出现了新的神经症状（6名低风险研究儿童、7名条件风险儿童和4名风险未知儿童）。该队列中的非洲SCA儿童高危TCD筛查结果的发生率较低，即使是那些出现新神经症状的儿童也是如此。该人群中的中风可能是多因素的，血管病变只是其中一个决定因素。开发一个包含相关要素的敏感中风预测组合可能有助于指导高危儿童的预防性治疗。
