Transcranial Doppler scanning and the assessment of stroke risk in children with haemoglobin sickle cell disease

Deane, Colin; Goss, David; O’Driscoll, Sandra; Mellor, Sarah; Pohl, Keith; Dick, Moira; Height, Sue; Rees, David C.

doi:10.1136/adc.2007.125799

Cited by 23 publications

(21 citation statements)

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“…1 It is less severe than sickle cell anemia (HbSS), with half the prevalence of acute pain, 2 and life expectancy up to 20 years longer. 3 However, serious complications occur: people with HbSC disease have a 100 times higher risk of stroke compared to the general population, 4 and an increased risk of proliferative retinopathy, with up to 70% of adults affected. 5 As with all types of sickle cell disease, there is marked, unexplained clinical variability: about 50% patients report no episodes of pain per year, whereas a minority suffer severe pain more than six times per year.…”

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confidence: 99%

The clinical significance of K-Cl cotransport activity in red cells of patients with HbSC disease

et al. 2015

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confidence: 99%

The clinical significance of K-Cl cotransport activity in red cells of patients with HbSC disease

et al. 2015

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confidence: 99%

“…Even though comprehensive medical care has been shown to decrease health care resource utilization and to improve quality of life [3] for patients with SCD, in the United States only 36% of children with SCD perform at least one hematology visit per year for comprehensive care [4] and the rate of missed appointments is around 45% [5]. Moreover, although medical advances have resulted in availability of improved treatments for children with SCD, less than 50% are enrolled in Transcranial Doppler (TCD) screening programs for stroke prevention both in Europe [6][7] and the United States [8][9].In many countries SCD affects people belonging to minority communities, such as African Americans in the United States or African immigrants in Europe [10]. Immigrants present cultural, social, and financial barriers in accessing the health system and these factors have an effect on the management of chronic illnesses [11][12][13].…”

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Comprehensive care for sickle cell disease immigrant patients: A reproducible model achieving high adherence to minimum standards of care

Colombatti

Montanaro

Guasti

et al. 2012

Pediatric Blood & Cancer

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INTRODUCTIONSickle Cell Disease (SCD) is the most common genetic disease worldwide. In the past decades advances in basic research and clinical investigations have produced a marked decrease in morbidity and mortality during early childhood. Newborn screening, prophylactic penicillin, effective vaccinations, and stroke prevention have largely contributed to these results [1][2]. Even though comprehensive medical care has been shown to decrease health care resource utilization and to improve quality of life [3] for patients with SCD, in the United States only 36% of children with SCD perform at least one hematology visit per year for comprehensive care [4] and the rate of missed appointments is around 45% [5]. Moreover, although medical advances have resulted in availability of improved treatments for children with SCD, less than 50% are enrolled in Transcranial Doppler (TCD) screening programs for stroke prevention both in Europe [6][7] and the United States [8][9].In many countries SCD affects people belonging to minority communities, such as African Americans in the United States or African immigrants in Europe [10]. Immigrants present cultural, social, and financial barriers in accessing the health system and these factors have an effect on the management of chronic illnesses [11][12][13].New models of comprehensive care need to be developed in order to ensure that all patients with SCD receive high quality care and benefit from the advances in clinical research, overcoming patient-related and health system-related barriers to specialized health care [14].Italy does not have a hemoglobinopathy newborn screening program and for many years pediatric hematology services have focused primarily on thalassemia. SCD has emerged as an important health condition in the last decade due to immigration, mainly from Africa and Albania, and the number of affected children is steadily increasing [15]. The majority of patients are diagnosed with SCD in the Emergency Room during acute events or because referred to a specialized center by the pediatric general practitioner, by friends or relatives. SCD is considered a rare disease and, according to the legislation of European countries, if a patient is registered as having a rare disease by a specialized reference center, patients and families can apply for disability benefits, care is free, and drugs are provided almost for free, with only a minimum contribution (prescription token). Children usually refer to their pediatric general practitioner for routine primary care, to local hospitals for emergencies and to tertiary care reference centers for specialized care.The Clinic of Pediatric Hematology-Oncology of the Azienda Ospedaliera-Università di Padova began assisting patients with SCD in 2003.From 2003 to 2005, several patient-related and health-operator related barriers to care had been noted. Patient-related barriers in seeking and accepting care regarded mainly language difficulties, socioeconomic factors (no permit of stay, precarious living conditions, low income, dif...

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“…Contudo, estudos têm demonstrado que taxas menores, de aproximadamente 128 cm/s, também apontam para maior risco e para a necessidade de realizar exames por imagem mais aprofundados em tais pacientes 71 .…”

Section: Métodos De Diagnóstico Por Imagemunclassified

Stroke in children

Filho¹,

Carvalho²

2009

J Pediatr (Rio J)

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Objectives: To summarize 1) the definitions and epidemiological features of stroke in children; 2) the main risk factors that can lead to stroke in pediatrics and neonatology; 3) the main pathophysiological features involved in the genesis of brain injury in stroke; 4) the clinical manifestations and imaging diagnosis; and 5) the latest recommendations concerning the support measures, treatment, and prophylaxis of stroke in children.Sources: A review of the literature published in PubMed, EMBASE, and SciELO databases using the search terms stroke, pediatrics, and neonatology was performed, including relevant references from the chosen texts. Summary of the findings:Stroke in children are rare conditions, with incidence rates among two and eight per 100,000 children up to 14 years, and most cases show an underlying disease such as heart diseases, prothrombotic conditions, sickle-cell disease, and vascular malformations. There are no specific guidelines currently in place for the treatment of stroke in children, although central elements include support treatment, monitoring, and anticoagulation as secondary prevention in certain cases. Prognosis depends on the extent of brain damage and the underlying disease but recurrence rates are high in most cases. Conclusions:Early diagnosis of stroke in children is very important and pediatricians should be aware of the lack of specificity of the symptoms to avoid late sequelae and improve life quality.J Pediatr (Rio J). 2009;85(6):469-479: Stroke, pediatrics, neonatology, intensive care, anticoagulants. ResumoObjetivos: Listar 1) as definições e características epidemiológicas de acidentes vasculares encefálicos (AVE) em crianças; 2) os principais fatores de risco para a ocorrência de AVE em pediatria e neonatologia; 3) as principais características fisiopatológicas para a ocorrência de lesão cerebral no AVE; 4) as manifestações clínicas e o diagnóstico por imagem; e 5) as recomendações atualizadas para as medidas de suporte, tratamento e profilaxia dos AVE em pediatria. Fontes dos dados:Realizou-se revisão da literatura nas bases de dados PubMed, EMBASE e SciELO utilizando-se como palavras-chave stroke, pediatrics e neonatology, além da utilização de referências bibliográficas importantes dos textos escolhidos. Síntese dos dados:A ocorrência de AVE em pediatria é incomum, com a incidência variando entre duas e oito por 100.000 crianças abaixo de 14 anos, tendo a maioria como denominador comum a ocorrência de doenças de base como cardiopatias congênitas, anemia falciforme e malformações vasculares. Não há padronização nas recomendações de tratamento para o AVE em crianças, embora as medidas fundamentais incluam monitoração, tratamento de suporte e anticoagulação em casos selecionados. O prognóstico depende da extensão de área cerebral acometida e da doença de base, mas as taxas de recorrência são altas na maioria dos casos. Conclusões:O diagnóstico precoce dos AVE em pediatria é fundamental, e é importante que os pediatras estejam atentos à falta de especificidade...

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Transcranial Doppler scanning and the assessment of stroke risk in children with haemoglobin sickle cell disease

Abstract: Further studies are needed to assess stroke risk in HbSC disease, but we suggest that TCD measurements are potentially useful in this condition, and that readings greater than 128 cm/s are abnormally high and warrant further investigation.

Cited by 23 publications

References 12 publications

The clinical significance of K-Cl cotransport activity in red cells of patients with HbSC disease

The clinical significance of K-Cl cotransport activity in red cells of patients with HbSC disease

Comprehensive care for sickle cell disease immigrant patients: A reproducible model achieving high adherence to minimum standards of care

Stroke in children

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