Behavioral characteristics of children with sickle cell disease

Conclusions: Behavioral impacts are highly prevalent in children with SCD. Individuals in socioeconomic classes C2 and D suffered more behavioral impacts than individuals in classes B2 and C1.RESUMO Objetivo: Avaliar aspectos sociodemogr áficos e clínicos de crianças com doença falciforme (DF) e suas características comportamentais. Métodos: Aplicação de entrevista sobre aspectos socioeconômicos e outras condições de saúde e do questionário de capacidades e dificuldades (SDQ) em pais de pacientes de quatro a dez anos com DF, em um ambulatório de referência. Dados clínicos foram obtidos dos prontuários médicos. Critérios de exclusão: uso de hidroxiureia, diagnóstico prévio de acidente vascular cerebral, encefalopatia crônica e/ou deficiência intelectual. Resultados: Analisados 45 pacientes (19 menina s e 26 meninos). Mediana de idade=7 anos. Diagnóstico da DF=26 hemoglobinopatia SC; 19 hemoglobinopatia SS. Classe econômica (SES): D=24,4%; C2=44,4%; C1=28,8%; B2=2,2%. Antecedentes clínicos: síndrome torácica aguda=40%; transfusões=66,7%; internações=82,2%. Achados SDQ=88,9% alteração cl ínica (subescala emocional=68,9%); pontuação total=alterada em 48,9%. Não foi possível estabelecer relação entre gra...
Source: Revista Paulista de Pediatria - Category: Pediatrics Source Type: research

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Authors: Shahid R Abstract Young adults are a key part of any society and the psychosocial and economic burden of any chronic or devastating disease in this particular group has significant consequences and impacts negatively on the development of the society as a whole. Although stroke in this age group is relatively rare, its consequences, in terms of lifelong dependence and disability, are substantial. Therefore, it is important to address this issue. In this retrospective epidemiological study, we analyzed the medical records of young patients (aged 18- 45 years) with a diagnosis of stroke during the study peri...
Source: Functional Neurology - Category: Neurology Tags: Funct Neurol Source Type: research
Conclusion: There is a wide heterogeneity of postoperative cerebral hemodynamic findings among TBI patients who underwent DC, including hemodynamic heterogeneity between their cerebral hemispheres. DC was proved to be effective for the treatment of cerebral oligoemia. Our data support the concept of heterogeneous nature of the pathophysiology of the TBI and suggest that DC as the sole treatment modality is insufficient. Introduction Decompressive craniectomy (DC) may effectively decrease intracranial pressure (ICP) and increase cerebral perfusion pressure (CPP) in traumatic brain injury (TBI) patients with refracto...
Source: Frontiers in Neurology - Category: Neurology Source Type: research
Conclusion: There is a wide heterogeneity of postoperative cerebral hemodynamic findings among TBI patients who underwent DC, including hemodynamic heterogeneity between their cerebral hemispheres. DC was proved to be effective for the treatment of cerebral oligoemia. Our data support the concept of heterogeneous nature of the pathophysiology of the TBI and suggest that DC as the sole treatment modality is insufficient. Introduction Decompressive craniectomy (DC) may effectively decrease intracranial pressure (ICP) and increase cerebral perfusion pressure (CPP) in traumatic brain injury (TBI) patients with refracto...
Source: Frontiers in Neurology - Category: Neurology Source Type: research
A Chinese scientist shocked the scientific community a couple of days ago with the announcement of having modified the very blueprint of life. If his claims are true, he tried to bestow two baby girls the ability to resist possible future infections with HIV. The outrage shows that humanity is not prepared to utilize the power of gene editing on embryos yet. We have no idea about the biological consequences, and we haven’t tackled the necessary legal and ethical issues. Genes to become toys of the “Gods”? Humanity has come a long way since Aldous Huxley pinned down how methods of genetic engineering, bio...
Source: The Medical Futurist - Category: Information Technology Authors: Tags: Bioethics Future of Medicine Genomics designer babies designer baby Gene gene editing genes Genome genome sequencing Health Healthcare healthcare system Innovation technology Source Type: blogs
Conclusion: Overall, home based primary care seems to be a promising alternative for pts with SCD. It had a significant impact on patient quality of care and may improve prescription adherence, but more data are needed to determine if it has an effect on healthcare utilization for pts with SCD.DisclosuresMoore: Ohio State University College of Medicine: Research Funding. Desai: FDA: Research Funding; Pfizer: Research Funding; University of Pittsburgh: Research Funding; Selexy/Novartis: Research Funding; NIH: Research Funding; Ironwood: Other: Adjudication Committee.
Source: Blood - Category: Hematology Authors: Tags: 901. Health Services Research-Non-Malignant Conditions: Poster III Source Type: research
Conclusions: The high severity scores among this cohort supports the validity of employing a medical history checklist as a measure of disease severity, reflecting the cumulative end organ damage due to SCD and near universal need for daily pain medication among patients requiring inpatient admission for VOC. Two-thirds of patients were categorized as having high disease severity, driven primarily by daily pain medication use prior to hospitalization and pulmonary complications, consistent with a known subgroup of adults with SCD-related complications who are more likely to have lower health-related quality of life and acc...
Source: Blood - Category: Hematology Authors: Tags: 903. Outcomes Research-Non-Malignant Hematology: Poster II Source Type: research
Conclusions: The risk for cerebral infarction and/or vasculopathy progression after initiation of treatment with either chronic transfusion, HSCT or EDAS is still a major concern. Our data suggest HSCT and surgical revascularization with chronic transfusion provide the greatest benefit in reducing stroke risk and HSCT reduces risk for progression of a severe vasculopathy. Additional, large population studies are needed to clarify the risk.DisclosuresMajumdar: NIMHD: Research Funding. Campbell: Functional Fluitics: Membership on an entity's Board of Directors or advisory committees; Global Blood Therapeutics: Membership on ...
Source: Blood - Category: Hematology Authors: Tags: 114. Hemoglobinopathies, Excluding Thalassemia-Clinical: Poster I Source Type: research
ré C Abstract Cerebral vasculopathy is a common and severe complication of sickle cell disease in children. The pathophysiology consists of progressive damage to the basal intracranial arteries and cerebral microcirculation, while chronic anemia worsens exposure to cerebral hypoxia. It results in stroke and subclinical or poorly symptomatic ischemic lesions. Many clinical, biological, and radiological risk factors have been identified. The prevention strategy through systematic transcranial Doppler screening of large-vessel vasculopathy has revolutionized the management of this disease and has greatly decre...
Source: Archives de Pediatrie - Category: Pediatrics Authors: Tags: Arch Pediatr Source Type: research
Authors: DeBaun MR, King AA Abstract Since 1998, the National Institutes of Health has funded 5 randomized controlled trials (RCTs) for primary and secondary prevention of strokes in children with sickle cell anemia (SCA). In a systematic fashion, these trials have significantly advanced the care of children with SCA. In the absence of an RCT, clinicians are often compelled to make decisions at the bedside, based on experience, observational studies, and principles of hematology. We will provide an initial example that describes how a team-based, learning collaborative developed a multisite standard care protocol with a low budget (
Source: Hematology ASH Education Program - Category: Hematology Tags: Hematology Am Soc Hematol Educ Program Source Type: research
Conclusions:Our case series illustrates the following clinical pearls: a) SCD-related CFES is vastly under-diagnosed, as it closely mimics sepsis, vasculitis or hypoxic ischemic encephalopathy; b) The diagnosis is confirmed with SWI that demonstrates pathognomonic microbleeds in the splenium and subcortical location; c) SCD-related CFES carries considerable morbidity and mortality.Disclosure: Dr. Ramachandiran has nothing to disclose. Dr. Raniga has nothing to disclose. Dr. Al Kindi has nothing to disclose. Dr. Dennison has nothing to disclose. Dr. Al Farsi has nothing to disclose. Dr. Al Busaidi has nothing to disclose. D...
Source: Neurology - Category: Neurology Authors: Tags: Stroke in the Young Source Type: research
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