Guidelines on sickle cell disease: primary stroke prevention in children and adolescents. Associação Brasileira de Hematologia, Hemoterapia e Terapia Celular Guidelines project: Associação Médica Brasileira - 2021

Loggetto, Sandra Regina; Veríssimo, Mônica Pinheiro de Almeida; Darrigo, Luiz Guilherme; Simões, Ricardo; Bernardo, Wanderley Marques; Braga, Josefina Aparecida Pellegrini

doi:10.1016/j.htct.2021.09.013

Cited by 8 publications

(2 citation statements)

References 43 publications

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“…Sickle cell disease (SCD) is a global burden, which is estimated to affect 14 million newborns by 2050 [ 1 ]. SCD is a group of autosomal recessive hemoglobinopathies that occur due to a genetic mutation of hemoglobin, whereby glutamic acid is substituted by valine in position 6 of the beta-globin chain leading to an abnormal hemoglobin known as hemoglobin S (HbS) [ 2 ]. Compared to normal hemoglobin, HbS has low solubility, and with low oxygenation, it results in the polymerization and formation of rigid sickle-shaped red blood cells.…”

Section: Introductionmentioning

confidence: 99%

Prevalence of Stroke in Individuals with Sickle Cell Disease Pre‐ and during Hydroxyurea Uses: A Descriptive Cross‐Sectional Study in Tanzania

Moshi,

Philipo,

Kileo

et al. 2024

Advances in Hematology

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Sickle cell disease (SCD) is an inherited blood disorder that leads to a variety of complications, including stroke. The use of hydroxyurea (HU) is reported to lessen the frequency and burden of stroke in SCD patients. However, less is known about the prevalence of stroke in SCD patients pre- and during the use of HU in sub-Saharan African (SSA) countries. Therefore, the study assessed stroke prevalence before and during uses of hydroxyurea among SCD patients in Tanzania. A hospital-based descriptive cross-sectional study was conducted at the sickle cell clinics in Dar es Salaam, Tanzania, from April 2023 to May 2023. A total of 228 participants were recruited, and data on demographic and clinical characteristics, HU use, and history of stroke were collected using a checklist from the respective patients’ medical records and verbal communication with the patients or caregivers. Data analysis was done using SPSS software version 25, and findings are summarized using frequency and percentages. Out of 228 enrolled SCD patients, 124 (54.4%) were females, 109 (47.8%) were aged between 6 and 12 years, 226 (99.1%) were not married, 181 (79.4%) had primary education, and 209 (95%) were unemployed. The prevalence of stroke pre-HU use was 28 (12.3%) and 6 (2.6%) after starting using HU. Out of 6 with stroke after starting using HU, 3 (50%) had a history of stroke pre-HU uses. The study showed that the prevalence of stroke among SCD patients is significantly reduced after HU use. The findings suggest the need for stakeholders to implement measures to ensure eligible SCD patients are kept on HU.

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Section: Introductionmentioning

confidence: 99%

Prevalence of Stroke in Individuals with Sickle Cell Disease Pre‐ and during Hydroxyurea Uses: A Descriptive Cross‐Sectional Study in Tanzania

Moshi,

Philipo,

Kileo

et al. 2024

Advances in Hematology

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“…For all children (aged two to 16 years) with SCD (HBSS/HBSβ0), transcranial Doppler (TCD) ultrasound should be performed regularly according to the guidelines as it helps early prediction and prevention of stroke [8]. However, TCD ultrasound screening is not available in many parts of the world, especially in developing countries [4].…”

Section: Introductionmentioning

confidence: 99%

Moyamoya Syndrome in Children With Sickle Cell Disease in Saudi Arabia: A Single-Center Experience

Zayed,

Al-Muhaimeed,

Al-Otaibi

et al. 2023

Cureus

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BackgroundSickle cell disease (SCD) is relatively common in Saudi Arabia. Its neurologic complications such as stroke and Moyamoya syndrome (MMS) can be severe and devastating. Such complications can be minimized by modern investigative tools such as transcranial Doppler (TCD) ultrasound, which is unavailable in many high-risk places. Our aim is to investigate the prevalence and characteristics of these complications in children with SCD in our center where TCD is not available. MethodsWe conducted a retrospective record review of children with SCD admitted to the pediatric ward and visited the pediatric hematology outpatient clinic of King Fahad Military Medical Complex, Dhahran, Saudi Arabia, from January 2010 to December 2021. The target population was children aged six months to 14 years with SCD and a history of stroke or transient ischemic attacks (TIAs). Their magnetic resonance imaging/magnetic resonance angiography (MRI/MRA) of the brain radiographic features were reviewed. A descriptive analysis was used to summarize the demographic characteristics and clinical features of patients with and without MMS. ResultsTwenty-six children (out of 385 with sickle cell anemia, originating mainly from the southwestern and eastern provinces of Saudi Arabia) experienced an overt stroke with an overall prevalence of 6.7%. All patients with stroke were originally from the Southwestern province. Their genotype was SS, and the median age at the onset of the first stroke was six years (IQR: 5.5). The main presenting symptoms were seizures (57.7%), motor weakness (42.3%), headache (15.3%), cranial nerve palsies (11.5%), cognitive deficit (7.6%), and dysphasia (3.8%). The majority of strokes were ischemic (92.3%). MMS was detected in 61.5% and was seen at the onset of the first stroke in all patients with this MRA abnormality. Seven children with moyamoya (43.8%) had recurrent strokes. ConclusionIn this study, the prevalence of overt stroke is 9% in children with SCD originating from the southwestern region of Saudi Arabia (26/286), and 61.5% of them (16/26) had MMS. It is absent in the children of Eastern origin (99 children). In places lacking TCD facilities, further studies are required to determine if MRA brain screenings of children with SCD may detect MMS before the onset of stroke and help start protective therapy.

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Pre‐operative transcranial Doppler ultrasound assessment of cerebral collateral circulation in children undergoing veno‐arterial extracorporeal membrane oxygenation or cardiac surgery

Reddan,

Malouf,

Venugopal

et al. 2024

J of Medical Radiation Sci

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Children requiring veno‐arterial extracorporeal membrane oxygenation (VA ECMO) or cardiac surgery often undergo cervical cannulation or carotid artery clamping, which can interrupt cerebral circulation. Inadequate collateral flow through the circle of Willis (CoW) may lead to cerebral ischaemia within the vascular territory and/or watershed regions. Pre‐cannulation survey of the CoW using transcranial Doppler (TCD) ultrasound may be performed to predict and plan neuroprotection. It is important to note in this article TCD refers to Duplex or colour coded trans‐cranial Doppler (TCCD) using radiology‐based machines, which is distinct from the more traditional trans‐cranial Doppler technique that does not incorporate a B‐mode image. This article describes our technique, in use since 2019, to guide surgical approaches and neuroprotective measures when an incomplete CoW is identified. High‐end radiology‐based ultrasound platforms and various transducers are used to assess brain morphology and haemorrhage through the anterior fontanelle in neonates. TCD is performed with the highest frequency transducer possible, utilising Doppler imaging to visualise cerebral arteries. Manual carotid compression can be used to functionally assess collateral flow when segments appear aplastic or hypoplastic. Potential pitfalls include mistaking the anterior choroidal artery for a hypoplastic posterior communicating artery (PCommA). Since implementing this protocol 5 years ago, no catastrophic infarcts related to cervical cannulation have occurred. This technique provides a practical solution for pre‐operative assessment of cerebral collateral circulation in children undergoing VA ECMO or cardiac surgery, allowing for consideration of neuroprotective measures and improving patient safety.

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Guidelines on sickle cell disease: primary stroke prevention in children and adolescents. Associação Brasileira de Hematologia, Hemoterapia e Terapia Celular Guidelines project: Associação Médica Brasileira - 2021

Cited by 8 publications

References 43 publications

Prevalence of Stroke in Individuals with Sickle Cell Disease Pre‐ and during Hydroxyurea Uses: A Descriptive Cross‐Sectional Study in Tanzania

Prevalence of Stroke in Individuals with Sickle Cell Disease Pre‐ and during Hydroxyurea Uses: A Descriptive Cross‐Sectional Study in Tanzania

Moyamoya Syndrome in Children With Sickle Cell Disease in Saudi Arabia: A Single-Center Experience

Pre‐operative transcranial Doppler ultrasound assessment of cerebral collateral circulation in children undergoing veno‐arterial extracorporeal membrane oxygenation or cardiac surgery

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