Introductory Chapter: Introduction to the History, Pathology and Clinical Management of Sickle Cell Disease

Inusa, Baba; Casale, Maddalena; Ward, Nicholas

doi:10.5772/65648

Cited by 8 publications

(6 citation statements)

References 62 publications

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“…(8) In addition, clinically unapparent stroke can only be detected by magnetic resonance imaging and arteriography (MRI-MRA). (5, 9)…”

Section: Introductionmentioning

confidence: 99%

Hydroxyurea Therapy for Neurological and Cognitive Protection in Pediatric Sickle Cell Anemia in Uganda (BRAIN SAFE II): Protocol for a single-arm open label trial

Mboizi,

Nabaggala,

Munube

et al. 2024

Preprint

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Background: Children with sickle cell anemia (SCA) in Sub-Saharan Africa are at high risk of sickle cerebrovascular injury (SCVI). Hydroxyurea, a commonly used disease-modifying therapy, may prevent or decrease SCVI for reduced incident stroke, stroke risk and potentially cognitive dysfunction. We aim to test the impact of daily hydroxyurea therapy on these outcomes in Ugandan children with SCA. We hypothesize that hydroxyurea therapy over 36 months will prevent, stabilize or improve these complications of SCA. Methods: The BRAIN SAFE II study is an open-label, single-arm trial of daily hydroxyurea for 270 children with SCA (HbSS) in Uganda, ages 3-9 years. Following baseline assessments, participants began hydroxyurea therapy and clinically followed per local guidelines. Standard hydroxyurea dose is escalated to maximum tolerated dose (MTD). SCVI is assessed by cerebral arterial velocity using Doppler ultrasound, with cognitive function determined by formal neurocognitive testing (primary outcomes). Structural SCVI is assessed by magnetic resonance imaging (MRI) and angiography (MRA) in a sub-sample of 90 participants ages >5 years, along with biomarkers of anemia, inflammation and malnutrition (secondary outcomes). At trial midpoint (18 months) and completion (36 months), primary outcomes will be compared to participants baseline to determine hydroxyurea impact and relationships to secondary outcomes. Conclusion: This open-label, single-arm trial will examine the impact of hydroxyurea on preventing or ameliorating SCA SCVI in children, assessed by reducing incident stroke, stroke risk and neurocognitive dysfunction. Trial results will provide important insight into the role of hydroxyurea therapy on critical manifestations of SCVI in children with SCA.

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“…(8) In addition, clinically unapparent stroke can only be detected by magnetic resonance imaging and arteriography (MRI-MRA). (5, 9)…”

Section: Introductionmentioning

confidence: 99%

Hydroxyurea Therapy for Neurological and Cognitive Protection in Pediatric Sickle Cell Anemia in Uganda (BRAIN SAFE II): Protocol for a single-arm open label trial

Mboizi,

Nabaggala,

Munube

et al. 2024

Preprint

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“…This will lead to progressive vasculopathy and organ damage and will result in recurrent attacks of acute illness like acute painful episodes, acute chest syndrome (ACS), and stroke [ 4 ]. Furthermore, there is an increased risk of infections, acute splenic sequestration crises (ASSC), and chronic organ failure [ 4 , 5 ].…”

Section: Introductionmentioning

confidence: 99%

“…The improvements in the management of SCD have resulted in improved survival and decreased morbidity in these patients especially in developed countries; however, much less progress has been made in the developing world, including the Mediterranean region, where the disease is common, and is still considered an important cause of childhood mortality [ 5 ].…”

Section: Introductionmentioning

confidence: 99%

Emergency Department Utilization by Pediatric Patients With Sickle Cell Disease in Basrah, Iraq

Marroof,

Hassan

2024

Cureus

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Background Patients with sickle cell disease (SCD) often present in the Emergency Department (ED) with acute and debilitating pain and other SCD-related complications. Objectives The objective is to analyze the causes of ED visits of pediatric patients with SCD, assess the burden of ED admission due to SCD in relation to other pediatric diseases, the treatment given, and the outcomes. Methods A prospective analytical study was conducted on children and adolescents with SCD, 1-14 years old who had been admitted to the ED at Basrah Maternity and Children Hospital over a six-month period. Patient's sociodemographic and clinical data, drug history, length of ED stay, complications, outcome, and readmissions were recorded. Results A total of 422 patients with SCD were admitted to ED during the study period representing 4.10% of the total admitted cases; 276(65.40%) of them were recruited in this study, and their mean age was 7.84 ±3.47 years. The main cause for ED admission was pain (73.91%), followed by infection (10.14%) and hemolytic crisis (6.15%). The mean duration of stay at ED was 6.11±1.87 hours. All admitted SCD patients had received analgesia; non-steroidal anti-inflammatory drugs (NSAIDs) were the commonest (80.4%), followed by acetaminophen (39.5%), and opioid narcotic (18.5%). Readmission within 30-days was reported in 82(29.71%) patients and was associated with the number of ED visits/last year (B=0.151, P=0.023), length of stay at ED (B=0.140, P=0.034) and severe disease (B=0.253, P<0.001). Conclusions Acute painful episodes were the main cause of ED admission. Although most patients with pain did receive NSAIDs, only a small percentage of them did receive opioids. About one-third of patients have been readmitted within 30 days, and readmission was associated with the number of ED visits/last year, disease severity, and length of ED stay. These findings can help in establishing local guidelines for managing such patients in the ED especially pain management.

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“…In the United States, 1 in 600 African-Americans has been diagnosed with SCD [1,2], and it was reported that 1 in every 2000 births had SCD during a newborn screening programme in the United Kingdom [11,18]. This multisystem disorder results in the sickling of red blood cells, which can cause a range of complications such as micro-vascular occlusion, haemolysis and progressive organ failure [4,14,8].…”

Section: Introductionmentioning

confidence: 99%

Deep Learning for Automatic Spleen Length Measurement in Sickle Cell Disease Patients

Zhen

Puyol‐Antón

Jogeesvaran

et al. 2020

Preprint

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Sickle Cell Disease (SCD) is one of the most common genetic diseases in the world. Splenomegaly (abnormal enlargement of the spleen) is frequent among children with SCD. If left untreated, splenomegaly can be life-threatening. The current workflow to measure spleen size includes palpation, possibly followed by manual length measurement in 2D ultrasound imaging. However, this manual measurement is dependent on operator expertise and is subject to intra-and inter-observer variability. We investigate the use of deep learning to perform automatic estimation of spleen length from ultrasound images. We investigate two types of approach, one segmentation-based and one based on direct length estimation, and compare the results against measurements made by human experts. Our best model (segmentation-based) achieved a percentage length error of 7.42%, which is approaching the level of inter-observer variability (5.47%-6.34%). To the best of our knowledge, this is the first attempt to measure spleen size in a fully automated way from ultrasound images.

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Introductory Chapter: Introduction to the History, Pathology and Clinical Management of Sickle Cell Disease

Cited by 8 publications

References 62 publications

Hydroxyurea Therapy for Neurological and Cognitive Protection in Pediatric Sickle Cell Anemia in Uganda (BRAIN SAFE II): Protocol for a single-arm open label trial

Hydroxyurea Therapy for Neurological and Cognitive Protection in Pediatric Sickle Cell Anemia in Uganda (BRAIN SAFE II): Protocol for a single-arm open label trial

Emergency Department Utilization by Pediatric Patients With Sickle Cell Disease in Basrah, Iraq

Deep Learning for Automatic Spleen Length Measurement in Sickle Cell Disease Patients

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