RESUMO
BACKGROUND: To provide insight into the perspectives of children and young adults with transfusion-dependent thalassemia and sickle cell disease and their caregivers regarding the decision for hematopoietic stem cell transplantation (HSCT). PROCEDURE: A qualitative longitudinal multicenter study. Data collection consisted of 40 audio-recorded conversations between physicians and families and 77 interviews with patients and/or caregivers related to 27 unique cases, collected at different time points throughout the decision-making process. RESULTS: Conversations and interviews revealed "hoping for a normal life" as an overarching theme, consisting of four main topics: (i) "Building a frame of reference" refers to a process where patients or families try to obtain comprehensive information on HSCT and translate this to their situation to decide. (ii) "Balancing between loss and benefit" reports the process of considering the advantages and disadvantages of continuing with supportive care to treat their disease versus choosing HSCT. (iii) "Experiencing the impact of HSCT" describes the impactfull experience of the HSCT period by those who chose HSCT. (iv) "Balancing again" refers to reflecting on the decision made. CONCLUSIONS: The hope for a normal life guided the decision-making process, described as a constant balance between the impact of the disease and HSCT. A structured approach to explore patients' and caregivers' perspectives on HSCT decision-making is needed, where specifically discussing the impact of the disease and hope for a normal life need to be integrated in the process.
Assuntos
Anemia Falciforme , Transplante de Células-Tronco Hematopoéticas , Criança , Adulto Jovem , Humanos , Cuidadores , Pacientes , Anemia Falciforme/terapiaRESUMO
BACKGROUND: Sickle cell disease (SCD) is characterized by vaso-occlusive crises (VOCs) that impair the health-related quality of life (HRQoL). The aim of this study is to evaluate the impact of hospitalization for VOCs on HRQoL in children with SCD over time. METHODS: In this longitudinal cohort study, children aged 8-18 years diagnosed with SCD at the Amsterdam UMC were included between 2012 and 2021. HRQoL was annually measured as part of standard care using the Pediatric Quality of Life Inventory. The impact of hospitalization for VOC on HRQoL was evaluated using linear mixed models 3, 6, 9, and 12 months after hospitalization. The effect of frequency of hospitalization for VOC on HRQoL was evaluated over the last 12 months. RESULTS: In total, 94 children with SCD were included with a median age of 11.8 years (interquartile range [IQR]: 9-14). Thirty-seven patients (39%) had been hospitalized for a VOC. Hospitalization for VOC led to a decrease of 3.2-4.8 points in total HRQoL compared to patients without hospitalization, most pronounced 3 months after hospitalization. Recurrent admission for VOC in the last 12 months was associated with a decrease of 2.3 points in total HRQoL (p = .04). The most affected subscale was physical functioning. CONCLUSION: The adverse effects of hospitalization for VOC in children with SCD persist up to 12 months after hospitalization. After hospitalization for VOC, extra attention and support for its negative impact on HRQoL are recommended. This study also underlines the importance of systematically measuring HRQoL, allowing clinicians to intervene accordingly.
RESUMO
Delayed haemolytic transfusion reaction (DHTR) is a potentially life-threatening complication of red blood cell (RBC) transfusions in sickle cell disease (SCD) and is classically induced by reactivation of previously formed antibodies. Improved antigenic matching has reduced alloimmunization and may reduce DHTR risk. We conducted a retrospective cohort study to investigate the incidence rate of DHTR in SCD patients receiving extended matched units (ABO/RhDCcEe/K/Fya /Jkb /S). Occasional transfusion episodes (OTE) between 2011 and 2020 were reviewed for occurrence of DHTR symptoms using four screening criteria: decreased Hb, increased lactate dehydrogenase (LDH), pain, and dark urine. We included 205 patients who received a cumulative number of 580 transfusion episodes of 1866 RBC units. During follow-up, 10 DHTR events were observed. The incidence rate of DHTR was 13·8/1000 OTEs [95% confidence interval (CI): 7·37-22·2], with a cumulative incidence of 15·2% (95% CI: 8·4-24·0%) after 25 patients having received RBC units. One DHTR event was fatal (10%). Symptoms were misdiagnosed in four DHTR events (40%) as other acute SCD complications. Despite a lower incidence rate compared to most other studies, the incidence rate of DHTR in SCD remains high, in spite of extended matching of donor RBCs. Increased awareness of DHTR is of utmost importance to facilitate early diagnosis and, consequently, improve outcome.
Assuntos
Anemia Falciforme/complicações , Reação Transfusional/diagnóstico , Reação Transfusional/etiologia , Adolescente , Adulto , Anemia Hemolítica Autoimune/etiologia , Anemia Falciforme/diagnóstico , Anemia Falciforme/epidemiologia , Anemia Falciforme/terapia , Biomarcadores , Transfusão de Sangue , Criança , Gerenciamento Clínico , Suscetibilidade a Doenças , Índices de Eritrócitos , Feminino , Humanos , Incidência , Masculino , Modelos de Riscos Proporcionais , Estudos Retrospectivos , Fatores de Risco , Fatores de Tempo , Reação Transfusional/sangue , Reação Transfusional/epidemiologia , Adulto JovemRESUMO
Intravenous fluid therapy (IV-FT) is routinely used in the treatment of vaso-occlusive crises (VOCs), as dehydration possibly promotes and sustains erythrocyte sickling. Patients with sickle cell disease (SCD) are at risk of developing diastolic dysfunction and fluid overload due to IV-FT. However, data on the adverse effects of IV-FT for VOC is sparse. We aimed to evaluate the incidence and risk factors of fluid overload due to IV-FT in patients with SCD. Consecutive hospitalisations for VOC treated with IV-FT between September 2016 and September 2018 were retrospectively analysed. The median (interquartile range) age was 25·0 (18·3-33·8) years and 65% had a severe genotype (HbSS/HbSß0 -thal). Fluid overload occurred in 21% of 100 patients. Hospital stay was longer in patients with fluid overload (6·0 vs. 4·0 days, P = 0·037). A positive history of fluid overload (P = 0·017), lactate dehydrogenase level (P = 0·011), and top-up transfusion during admission (P = 0·005) were independently associated with fluid overload occurrence. IV-FT was not reduced in 86% of patients despite a previous history of fluid overload. Fluid overload is frequently encountered during IV-FT for VOC. IV-FT is often not adjusted despite a positive history of fluid overload or when top-up transfusion is indicated, emphasising the need for more awareness of this complication and a personalised approach.
Assuntos
Anemia Falciforme/terapia , Hidratação/efeitos adversos , Administração Intravenosa , Adolescente , Adulto , Anemia Falciforme/complicações , Feminino , Hidratação/métodos , Hospitalização , Humanos , Incidência , Masculino , Estudos Retrospectivos , Fatores de Risco , Adulto JovemRESUMO
In the Netherlands, between 1985 and 2007 secular changes in the health care of patients with sickle cell disease (SCD) have taken place, such as penicillin prophylaxis, vaccination programs and stroke prevention. We investigated the number and causes of death in a cohort of 298 SCD patients, established in 2007, before introduction of neonatal screening, to determine preventable deaths. All patients were diagnosed with SCD before the age of 18 (median age at diagnosis 5.1 y). Their vital status was determined up to January 2017. After a total follow-up period of 4565 patient years and a median time of follow-up of 15 years for all patients, 230 patients (77%) were still alive, 45 patients (15%) were lost to follow-up and a total of 23 patients (8%) had died. Estimated survival to 18 years was 92% with a global mortality rate of 0.48 deaths/100 patient years. Leading causes of death were infection (35%) followed by neurologic complications (22%) and death in the course of a painful episode (13%). Nine of the 20 known causes of death were preventable. These results strongly suggest the benefit of comprehensive care measures for patients with SCD in the Netherlands to further prevent morbidity and mortality.
Assuntos
Anemia Falciforme/mortalidade , Causas de Morte/tendências , Adolescente , Adulto , Anemia Falciforme/epidemiologia , Anemia Falciforme/etiologia , Criança , Pré-Escolar , Feminino , Seguimentos , Humanos , Lactente , Recém-Nascido , Masculino , Pessoa de Meia-Idade , Países Baixos/epidemiologia , Prognóstico , Estudos Prospectivos , Estudos Retrospectivos , Taxa de Sobrevida , Fatores de Tempo , Adulto JovemAssuntos
Anemia Falciforme , Angiografia por Ressonância Magnética , Ultrassonografia Doppler Transcraniana , Humanos , Anemia Falciforme/terapia , Estudos Retrospectivos , Criança , Feminino , Masculino , Ultrassonografia Doppler Transcraniana/métodos , Angiografia por Ressonância Magnética/métodos , Adolescente , Transfusão de Sangue/métodos , Suspensão de Tratamento , Pré-EscolarAssuntos
Anemia Falciforme , Triagem Neonatal , Humanos , Anemia Falciforme/mortalidade , Anemia Falciforme/epidemiologia , Anemia Falciforme/diagnóstico , Anemia Falciforme/complicações , Recém-Nascido , Países Baixos/epidemiologia , Seguimentos , Feminino , Masculino , Criança , Pré-Escolar , Lactente , AdolescenteRESUMO
Sickle cell disease (SCD) is complicated by silent cerebral infarcts, visible as white matter hyperintensities (WMHs) on magnetic resonance imaging (MRI). Both local vaso-occlusion, elicited by endothelial dysfunction, and insufficiency of cerebral blood flow (CBF) have been proposed to be involved in the aetiology. We performed an explorative study to investigate the associations between WMHs and markers of endothelial dysfunction and CBF by quantifying WMH volume on 3.0 Tesla MRI. We included 40 children with HbSS or HbSß(0) thalassaemia, with a mean age of 12.1 ± 2.6 years. Boys demonstrated an increased risk for WMHs (odds ratio 4.5, 95% confidence interval 1.2-17.4), unrelated to glucose-6-phosphate dehydrogenase deficiency. In patients with WMHs, lower fetal haemoglobin (HbF) was associated with a larger WMH volume (regression coefficient = -0.62, R2 = 0.5, P = 0.04). Lower ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) levels were associated with lower CBF in the white matter (regression coefficient = 0.07, R2 = 0.15, P = 0.03), suggesting that endothelial dysfunction could potentially hamper CBF. The findings of our explorative study suggest that a high level of HbF may be protective for WMHs and that endothelial dysfunction may contribute to the development of WMHs by reducing CBF.
Assuntos
Anemia Falciforme/complicações , Infarto Cerebral/etiologia , Substância Branca/patologia , Adolescente , Anemia Falciforme/sangue , Coagulação Sanguínea/fisiologia , Infarto Cerebral/sangue , Infarto Cerebral/patologia , Circulação Cerebrovascular/fisiologia , Criança , Endotélio Vascular/fisiopatologia , Feminino , Hemoglobina Fetal/metabolismo , Humanos , Imageamento por Ressonância Magnética/métodos , Masculino , Estudos Prospectivos , Fatores de Risco , Fatores SexuaisAssuntos
Acetilcisteína/uso terapêutico , Anemia Falciforme/patologia , Dor/prevenção & controle , Acetilcisteína/efeitos adversos , Acetilcisteína/farmacologia , Adolescente , Adulto , Anemia Falciforme/tratamento farmacológico , Antioxidantes , Criança , Feminino , Humanos , Masculino , Adesão à Medicação , Resultado do Tratamento , Adulto JovemRESUMO
PURPOSE: To evaluate the applicability of arterial spin labeling (ASL) cerebral blood flow (CBF) measurements in children with sickle cell disease (SCD). MATERIALS AND METHODS: We included 12 patients and five controls. Conventional magnetic resonance imaging (MRI) (T2, fluid attenuated inversion recovery [FLAIR], and MR angiography) was performed to diagnose silent infarcts, vasculopathy, or leukoencephalopathy. Pseudo-continuous ASL was performed to measure CBF using two postlabeling delays to identify transit-time effects. Perfusion estimates were corrected for hematocrit and blood velocity in the labeling plane and compared to phase-contrast MR. CBF asymmetries between the flow maps of the left and right internal carotid arteries were tested for significance using paired t-tests. Significant asymmetries were expressed in terms of an asymmetry ratio (AR = absolute difference/mean). An AR >10% was considered clinically relevant. RESULTS: Mean CBF was higher in patients than in controls. Agreement between CBF and flow improved after applying hematocrit and velocity corrections. At a 2100 msec postlabeling delay one patient had a clinically relevant asymmetry. No association was observed between CBF asymmetries and silent infarcts. CONCLUSION: Care must be taken in the interpretation of ASL-CBF measurements in SCD patients. A long postlabeling delay with blood velocity correction anticipates overestimation of CBF asymmetries.
Assuntos
Anemia Falciforme/patologia , Anemia Falciforme/fisiopatologia , Circulação Cerebrovascular , Transtornos Cerebrovasculares/patologia , Transtornos Cerebrovasculares/fisiopatologia , Angiografia por Ressonância Magnética/métodos , Adolescente , Anemia Falciforme/complicações , Velocidade do Fluxo Sanguíneo , Transtornos Cerebrovasculares/etiologia , Criança , Feminino , Humanos , Masculino , Reprodutibilidade dos Testes , Sensibilidade e Especificidade , Marcadores de Spin , Adulto JovemRESUMO
This study analyzed the mortality and causes of death in sickle cell disease patients in the Netherlands, to provide a baseline for monitoring the effect of the recently introduced neonatal screening programme and to indicate areas of improvement in the care for these patients. All children (<18 years) diagnosed with sickle cell disease in a tertiary hospital from 1985 to 2007 were included. Vital status was determined up to March 2008. A total of 298 children were included: 189 (63%) patients had HbSS, 17 (6%) HbSß(0) thalassaemia, 72 (24%) HbSC and 20 (7%) HbSß(+) thalassaemia. Twelve patients (4%) died during a total follow-up of 3896 patient years. All known deaths were sickle cell disease-related. Meningitis/sepsis (n=4; 33%), stroke (n=3; 25%) and death during a visit to the country of origin (n=3; 25%) were the most common causes of death. The overall mortality rate was 0·27 deaths/100 patient years [95% confidence interval (CI): 0·15-0·43]. The estimated survival at the age of 18 years was 97·3% (95% CI: 95-99%). This report confirms that the burden of mortality in sickle cell disease is increasingly shifting to adults. It is recommended that compliance to antibiotic prophylaxis, thorough counselling and support for patients travelling abroad and specialized peri-operative care should receive continuous attention.
Assuntos
Anemia Falciforme/mortalidade , Adolescente , Anemia Falciforme/complicações , Anemia Falciforme/diagnóstico , Causas de Morte , Criança , Pré-Escolar , Feminino , Seguimentos , Humanos , Lactente , Recém-Nascido , Masculino , Meningites Bacterianas/complicações , Meningites Bacterianas/mortalidade , Triagem Neonatal , Países Baixos/epidemiologia , Infecções Oportunistas/complicações , Infecções Oportunistas/mortalidade , Acidente Vascular Cerebral/etiologia , Acidente Vascular Cerebral/mortalidadeRESUMO
BACKGROUND: Although neurocognitive deficits in children with sickle cell disease (SCD) have been well documented, the etiology of these deficits has not been completely clarified. The aim of this study was to investigate the association of laboratory markers of disease severity and radiological parameters with neurocognitive functioning in children with SCD. DESIGN AND METHODS: Participants were 37 children with SCD ((HbSS or HbS-ß(0)-thalassemia) aged 6-18 years. All participants underwent extensive neurocognitive assessment. Further data (TCD values, laboratory test results, and MRI data) were obtained from medical charts. Associations were analyzed by hierarchical regression analysis. RESULTS: Hemoglobin was associated with a decrease in verbal short-term memory. There was no association between TCD velocities and neurocognitive functioning, when controlled for age. Children with silent infarcts did not differ from children with normal MRI in neurocognitive functioning. Children with right-left asymmetries in cerebral blood flow as measured by continuous arterial spin labelling (CASL) MRI had better sustained attention than children without asymmetries. CONCLUSIONS: Neurocognitive deficits are associated with the severity of anemia, indicating reduced oxygen delivery to the brain as an etiological mechanism. This implies that children with SCD and normal MRIs may still suffer from neurocognitive impairments, possibly affecting their academic development and full participation in society.
Assuntos
Anemia Falciforme/complicações , Encéfalo/irrigação sanguínea , Transtornos Cognitivos/etiologia , Transtornos da Memória/etiologia , Adolescente , Biomarcadores/sangue , Velocidade do Fluxo Sanguíneo , Encéfalo/patologia , Criança , Feminino , Humanos , Imageamento por Ressonância Magnética , Masculino , Memória de Curto Prazo , Países Baixos , Análise de Regressão , Índice de Gravidade de Doença , Ultrassonografia Doppler TranscranianaRESUMO
BACKGROUND: Sickle cell disease (SCD) can lead to profound cerebral damage, associated with neurocognitive deficits. The aim of the current study was to evaluate a broad range of neurocognitive functions in children with SCD compared to a SES-matched control group, in order to gain more insight into the specific deficits of these patients. METHODS: Forty-one children with homozygous SCD (HbSS or HbS-ß0-thalassemia) and 38 controls were assessed on a comprehensive set of well-defined and validated measures of neurocognitive functioning. Besides general intelligence, we evaluated executive functioning extensively (including response inhibition, sustained attention, planning, visuo-spatial working memory, and verbal working memory) as well as visuo-motor functioning. RESULTS: SCD was clearly associated with lower IQ scores. More than one in three children with SCD had a Full-scale IQ below 75. Furthermore, children with SCD showed deficits in visuo-motor functioning. Some evidence was found for executive dysfunction: Children with SCD displayed poor visuo-spatial working memory, as well as subtle deficits in sustained attention and planning. No significant differences were found between children with SCD and controls in terms of response inhibition and verbal working memory. CONCLUSIONS: Children with SCD are at increased risk of lower intelligence, visuo-motor impairments, and executive dysfunction. These neurocognitive deficits may underlie high rates of scholastic impairments in these children. The present findings further illuminate the importance of regular neurocognitive evaluations and future neurocognitive rehabilitation programs for children with SCD.
Assuntos
Anemia Falciforme/complicações , Transtornos Cognitivos/etiologia , Doenças do Sistema Nervoso/etiologia , Adolescente , Anemia Falciforme/fisiopatologia , Atenção , Estudos de Casos e Controles , Criança , Transtornos Cognitivos/fisiopatologia , Feminino , Hemoglobina Falciforme/genética , Humanos , Inteligência , Masculino , Testes Neuropsicológicos , PrognósticoRESUMO
BACKGROUND: Infants who are born in The Netherlands receive oral vitamin K to prevent bleeding due to a vitamin K deficiency. However the incidence of such bleedings are higher compared to other European countries. Therefore, the Dutch Health Council advised in 2017 to change this guideline from oral to intramuscular administration. CASE DESCRIPTION: A 2 months old girl presented with a fatal intracranial hemorrhage. A day before she developed a hematoma on her foot and orbit. Despite daily oral vitamin K, blood results revealed a severe vitamin K deficiency-related bleeding. Postmortem liver biopsy and genetic studies showed cholestasis as the most likely cause of malabsorption of fat soluble vitamins due to a heterozygous pathogenic variant in the ABCB11 gene, which could possibly be transient. CONCLUSION: Our case illustrates the importance of revising the national guideline for vitamin K prophylaxis to intramuscular administration, according to the recommendation of the Dutch Health Council.
Assuntos
Colestase , Sangramento por Deficiência de Vitamina K , Feminino , Hemorragia , Humanos , Lactente , Recém-Nascido , Hemorragias Intracranianas , Vitamina K , Sangramento por Deficiência de Vitamina K/tratamento farmacológico , Sangramento por Deficiência de Vitamina K/prevenção & controleRESUMO
There is no instrument to measure severity of sickle cell disease (SCD) in pediatric patients that is generally accepted. The aim of this study was to develop and validate a severity index for SCD in children. We developed an index consisting of 12 items and tested its validity of the index using data from 92 children. We tested whether different scores were obtained for patients classified by severity both subjectively and objectively by a partially validated existing index. Furthermore, we tested whether the index could differentiate patients classified according to genotype or the number of α-gene deletions and evaluated whether the score on the index was correlated with the average number and days of hospitalizations/year, age and a risk of death score. We explored the effect of three different weighting systems (Score A, B, and C) to summarize these items. All weightings demonstrated a significant difference between the scores of mild, moderate, and severely affected patients, as classified by a subjective rating or with an existing index (P < 0.01). The index clearly differentiated patients by genotype (P < 0.01) or α-gene deletions (P < 0.01). The correlation with hospitalization was moderate. Age and the risk of death score were weakly associated with the pediatric severity index for SCD. This is the first pediatric SCD severity index that was developed and validated using modern clinimetric methodology. The validity and reliability of this index should be further evaluated in a prospective study including a larger cohort, preferably diagnosed at birth.
Assuntos
Anemia Falciforme/classificação , Índice de Gravidade de Doença , Adolescente , Fatores Etários , Anemia Falciforme/sangue , Anemia Falciforme/complicações , Anemia Falciforme/genética , Anemia Falciforme/mortalidade , Biomarcadores , Criança , Pré-Escolar , Genótipo , Hospitalização/estatística & dados numéricos , Humanos , Incidência , Tempo de Internação/estatística & dados numéricos , Osteonecrose/epidemiologia , Osteonecrose/etiologia , Dor/epidemiologia , Dor/etiologia , Infecções Pneumocócicas/epidemiologia , Infecções Pneumocócicas/etiologia , Risco , Traço Falciforme/genética , Trombose/epidemiologia , Trombose/etiologia , Talassemia beta/complicações , Talassemia beta/genéticaRESUMO
BACKGROUND: Low health-related quality of life (HRQoL) of children with sickle cell disease (SCD) may be associated with consequences of the disease, or with the low socio-economic status (SES) of this patient population. The aim of this study was to investigate the HRQoL of children with SCD, controlling for SES by comparing them to healthy siblings (matched for age and gender), and to a Dutch norm population. METHODS: The HRQoL of 40 children with homozygous SCD and 36 healthy siblings was evaluated by the KIDSCREEN-52. This self-report questionnaire assesses ten domains of HRQoL. Differences between children with SCD and healthy siblings were analyzed using linear mixed models. One-sample t-tests were used to analyze differences with the Dutch norm population. Furthermore, the proportion of children with SCD with impaired HRQoL was evaluated. RESULTS: In general, the HRQoL of children with SCD appeared comparable to the HRQoL of healthy siblings, while children with SCD had worse HRQoL than the Dutch norm population on five domains (Physical Well-being, Moods & Emotions, Autonomy, Parent Relation, and Financial Resources). Healthy siblings had worse HRQoL than the Dutch norm population on three domains (Moods & Emotions, Parent Relation, and Financial Resources). More than one in three children with SCD and healthy siblings had impaired HRQoL on several domains. CONCLUSION: These findings imply that reduced HRQoL in children with SCD is mainly related to the low SES of this patient population, with the exception of disease specific effects on the physical and autonomy domain. We conclude that children with SCD are especially vulnerable compared to other patient populations, and have special health care needs.
Assuntos
Anemia Falciforme , Qualidade de Vida , Irmãos , Classe Social , Adolescente , Anemia Falciforme/economia , Estudos de Casos e Controles , Criança , Feminino , Humanos , Masculino , Países Baixos , Pais/educação , Psicometria/métodos , Inquéritos e QuestionáriosRESUMO
BACKGROUND AND PURPOSE: Cerebral infarction is an important complication of sickle cell disease (SCD) and occurs in one third of the patients with SCD. The risk of infarction is commonly attributed to the hyperemia that is associated with anemia and reduces the cerebral vascular reserve. We measured regional cerebral blood flow (rCBF) by continuous arterial spin labeling MRI, which is a noninvasive method that does not require ionizing radiation. The purpose of this study was to examine rCBF in children with SCD and compare it with rCBF in healthy children. METHODS: rCBF was measured at 3-T continuous arterial spin labeling MRI in 24 neurological normal patients with SCD and in 12 healthy children matched for ethnicity and age (mean age in both groups 13 years). rCBF was calculated for 6 vascular territories (left and right anterior, middle and posterior cerebral artery). Asymmetry in rCBF was evaluated by measuring differences in flow between left and right hemispheres. The definition of asymmetry (>11.7 mL/100 g/min) was based on a repeatability study performed in 6 healthy adults. RESULTS: The rCBF was of similar magnitude in patients with SCD and control subjects in the frontal, middle, and posterior territories. The majority of patients with SCD (58%) demonstrated a left-right asymmetry of rCBF in one or more vascular territories, whereas none of the control subjects did. CONCLUSIONS: In contrast to previous studies, we found no difference in cerebral blood flow between patients and control subjects. We did observe an asymmetry in rCBF in the majority of patients with SCD that was not present in healthy control subjects.
Assuntos
Anemia Falciforme/diagnóstico , Anemia Falciforme/fisiopatologia , Circulação Cerebrovascular/fisiologia , Adolescente , Envelhecimento/fisiologia , Angiografia Cerebral , Criança , Feminino , Humanos , Processamento de Imagem Assistida por Computador , Angiografia por Ressonância Magnética , Masculino , Marcadores de Spin , Talassemia/fisiopatologia , Adulto JovemRESUMO
BACKGROUND: Behavioral and emotional problems in children with sickle cell disease (SCD) may be related to disease factors, or to socio-demographic factors. The aim of this study was to investigate the prevalence of behavioral and emotional problems in children with SCD living in a Western European country, compared to healthy siblings (who were comparable in age, gender, ethnicity, and socio-economic status-SES), and to a Dutch norm population. METHODS: The Child Behavior Checklist (CBCL), Teacher Report Form (TRF) and Disruptive Behavior Disorders rating scale (DBD) were distributed among caregivers and teachers of 119 children with SCD aged 6-18 years and among caregivers and teachers of 38 healthy siblings. RESULTS: Questionnaires were returned by caregivers and/or teachers of 106 children with SCD and 37 healthy siblings. According to caregivers and teachers, children with SCD had more severe internalizing problems than healthy siblings and the norm population. According to teachers, subgroups of both children with SCD and healthy siblings had more severe externalizing problems than the norm population. Children with SCD had more difficulties than healthy siblings in terms of school functioning, showed less competent social behavior and tended to have more attention deficits. CONCLUSIONS: Children with SCD are at increased risk of developing internalizing problems as a result of their disease. Subgroups of children with SCD are at increased risk of developing severe externalizing problems, which may either be related to socio-demographic factors, or to disease factors, such as neurocognitive deficits associated with cerebral infarction.
Assuntos
Sintomas Afetivos/epidemiologia , Anemia Falciforme/psicologia , Transtornos do Comportamento Infantil/epidemiologia , Emigrantes e Imigrantes/psicologia , Irmãos/psicologia , Adolescente , Sintomas Afetivos/etiologia , Cuidadores , Criança , Transtornos do Comportamento Infantil/etiologia , Docentes , Feminino , Genótipo , Doença da Hemoglobina SC/psicologia , Humanos , Deficiências da Aprendizagem/epidemiologia , Deficiências da Aprendizagem/etiologia , Masculino , Países Baixos/epidemiologia , Pais , Índice de Gravidade de Doença , Traço Falciforme/complicações , Traço Falciforme/psicologia , Comportamento Social , Fatores Socioeconômicos , Inquéritos e Questionários , Talassemia beta/complicações , Talassemia beta/psicologiaRESUMO
Several studies have reported remission of immune thrombocytopenic purpura ( ITP) after eradication of a coexistent Helicobacter pylori infection in adults. Data in children are limited. Here we report the results of a prospective study of Helicobacter pylori determination and eradication in children with chronic ITP in the Netherlands.