Lesión crónica cerebral en la anemia falciforme y su relación con la calidad de vida / Chronic brain damage in sickle cell disease and its relation with quality of life

Antecedentes y objetivo: La anemia falciforme provoca una lesión orgánica progresiva. El objetivo de este trabajo es describir el rendimiento escolar de pacientes con anemia falciforme y los parámetros clínicos y de calidad de vida que pueden influir. La hipótesis es que si las alteraciones escolares se presentan sin otros datos objetivos, factores añadidos deben concurrir aparte de la propia enfermedad. Pacientes y métodos: Estudio transversal realizado en noviembre de 2015 considerando variables analíticas, complicaciones e imágenes neurorradiológicas de niños con anemia falciforme, y encuesta familiar sobre rendimiento escolar y calidad de vida. Resultados: Se incluyeron 60 pacientes. La mediana de edad fue de 6,8 años, y el 78% se diagnosticaron al nacimiento. El rendimiento escolar estaba alterado en el 51% y se relacionó con hipoxemia nocturna. El accidente cerebrovascular se presentó en el 6,7%. La ecografía doppler transcraneal fue patológica en el 4% y la resonancia magnética en el 16%. La calidad de vida arrojó resultados patológicos en todas las esferas y aumentó la proporción con valores bajos a mayor edad. El accidente cerebrovascular afectó la esfera física-social, y la neumopatía, la física-emocional. Conclusiones: El fracaso escolar como expresión de lesión crónica cerebral en la anemia falciforme afecta a la mitad de los pacientes y se relaciona con hipoxemia nocturna, aunque otros factores de confusión socioculturales pueden influir. La calidad de vida está alterada en la mayoría de los niños, independientemente del retraso escolar. La ausencia de una lesión orgánica objetiva en la neuroimagen o de parámetros de gravedad clínica no implican que la calidad de vida o la escolarización sean normales (AU)

Background and objective: Sickle cell anaemia causes progressive organ damage. The objective is to describe school performance of patients with sickle cell anaemia and their clinical parameters and quality of life that may have an influence. The hypothesis is that if school alterations occur without other objective data, additional factors must be present besides the disease itself. Patients and methods: Transversal study performed in November 2015 considering analytical variables, complications and neuroradiological images of children with sickle cell anaemia, and family survey on school performance and quality of life. Results: Median age was 6.8 years and 78% were diagnosed at birth. Sixty patients were included. School performance was altered in 51% of cases and was related to nocturnal hypoxemia. Acute stroke incidence was 6.7%. Transcranial ultrasound was abnormal in 4% of cases and magnetic resonance imaging in 16% of cases. Quality of life showed pathological findings in all areas and the low values increased proportionally in older ages. The stroke affected the physical and social sphere, and lung disease affected the physical and emotional spheres. Conclusions: Poor school performance affects half of the patients and it is related to nocturnal hypoxemia, although other socio-cultural factors may have an influence. Quality of life is affected in most of these cases independently of academic results. The absence of alterations in neuroimaging or the apparent lack of severe clinical parameters do not mean that quality of life and schooling are normal (AU)

Chronic brain damage in sickle cell disease and its relation with quality of life. / Lesión crónica cerebral en la anemia falciforme y su relación con la calidad de vida.

BACKGROUND AND OBJECTIVE: Sickle cell anaemia causes progressive organ damage. The objective is to describe school performance of patients with sickle cell anaemia and their clinical parameters and quality of life that may have an influence. The hypothesis is that if school alterations occur without other objective data, additional factors must be present besides the disease itself. PATIENTS AND METHODS: Transversal study performed in November 2015 considering analytical variables, complications and neuroradiological images of children with sickle cell anaemia, and family survey on school performance and quality of life. RESULTS: Median age was 6.8 years and 78% were diagnosed at birth. Sixty patients were included. School performance was altered in 51% of cases and was related to nocturnal hypoxemia. Acute stroke incidence was 6.7%. Transcranial ultrasound was abnormal in 4% of cases and magnetic resonance imaging in 16% of cases. Quality of life showed pathological findings in all areas and the low values increased proportionally in older ages. The stroke affected the physical and social sphere, and lung disease affected the physical and emotional spheres. CONCLUSIONS: Poor school performance affects half of the patients and it is related to nocturnal hypoxemia, although other socio-cultural factors may have an influence. Quality of life is affected in most of these cases independently of academic results. The absence of alterations in neuroimaging or the apparent lack of severe clinical parameters do not mean that quality of life and schooling are normal.

Neurocognition and quality of life after reinitiating antiretroviral therapy in children randomized to planned treatment interruption.

OBJECTIVE: Understanding the effects of antiretroviral treatment (ART) interruption on neurocognition and quality of life (QoL) are important for managing unplanned interruptions and planned interruptions in HIV cure research. DESIGN: Children previously randomized to continuous (continuous ART, n = 41) vs. planned treatment interruption (PTI, n = 47) in the Pediatric European Network for Treatment of AIDS (PENTA) 11 study were enrolled. At study end, PTI children resumed ART. At 1 and 2 years following study end, children were assessed by the coding, symbol search and digit span subtests of Wechsler Intelligence Scale for Children (6-16 years old) or Wechsler Adult Intelligence Scale (≥17 years old) and by Pediatrics QoL questionnaires for physical and psychological QoL. Transformed scaled scores for neurocognition and mean standardized scores for QoL were compared between arms by t-test and Mann-Whitney U test, respectively. Scores indicating clinical concern were compared (<7 for neurocognition and <70 for QoL tests). RESULTS: Characteristics were similar between arms with a median age of 12.6 years, CD4⁺ of 830 cells/µl and HIV RNA of 1.7 log10copies/ml. The median cumulative ART exposure was 9.6 in continuous ART vs. 7.7 years in PTI (P = 0.02). PTI children had a median of 12 months off ART and had resumed ART for 25.2 months at time of first assessment. Neurocognitive scores were similar between arms for all tests. Physical and psychological QoL scores were no different. About 40% had low neurocognitive and QoL scores indicating clinical concern. CONCLUSION: No differences in information processing speed, sustained attention, short-term memory and QoL functioning were observed between children previously randomized to continuous ART vs. PTI in the PENTA 11 trial.

Disease disclosure, treatment adherence, and behavioural profile in a cohort of vertically acquired HIV-infected adolescents. NeuroCoRISpeS study.

UNLABELLED: Advances in care and antiretroviral treatment, improved life expectancy and quality of life in children with perinatally-acquired human immunodeficiency virus (HIV) infection. There is increasing interest in the chronic effects of growing up with HIV. The aim of this study was to assess the psychosocial, emotional and behavioural functioning in a cohort of perinatally-acquired HIV-infected adolescents. Data were obtained through semi-structured interviews and the Strengths and Difficulties Questionnaire (SDQ) for emotional and behavioural disorders screening. RESULTS: A total of 95 patients (58% women) were assessed with a median age of 15 years (11-19.1) and a median age at diagnosis of 1.7 years (0-12.2). The median CD4 count, at the inclusion, was 626 cells/mm(3) (132-998), with 34% (10-52%). Viral load was <50 copies/ml in 72% of patients. Eighty-one per cent knew their diagnosis and optimal adherence was achieved in 53%. Passive coping was reported in 58.4% of the adolescents. Only 7.7% of teenagers had a complete and adequate knowledge of their disease and only 18.2% had shared it with their friends. Six unwanted pregnancies occurred (11% of women). Most of them (90%) attended school but 60% had been held back one or more school years. Overall, SDQ scored a risk of behavioural and emotional problems in 24.5%. The report of behaviours associated with hyperactivity was high in 14.9% of the population and borderline in 18.1%. Adolescents with encephalopathy accounted for 44% of those whose total scores fell in either the abnormal and borderline ranges for emotional difficulties (p = .038). CONCLUSION: Perinatally-acquired HIV-infected adolescents showed significant psychosocial and behavioural health risks that should bring attention to prevention and health care programmes. An earlier disclosure to children could favour a better psychological adjustment and a better treatment adherence. Future studies are needed to assess the relationship between vertically acquired HIV-infection and hyperactivity.

Aspectos psicosociales en una cohorte de adolescentes con infección por el virus de la inmunodeficiencia humana por transmisión vertical. NeuroCoRISpeS / Psychosocial aspects in a cohort of vertically transmitted human immunodeficiency virus-infected adolescents

INTRODUCCIÓN: Los avances en el tratamiento antirretroviral han mejorado la esperanza de vida de niños con infección por VIH por transmisión vertical. Sin embargo, han aparecido nuevos retos. Planteamos este estudio con el objetivo de determinar los aspectos psicosociales y el conocimiento sobre su enfermedad en una cohorte de adolescentes con infección por VIH por transmisión vertical. MÉTODOS: Se incluyeron pacientes con infección por VIH por transmisión vertical con edades comprendidas entre 12-19 años. Los datos se obtuvieron mediante entrevista semiestructurada y el Strengths and Difficulties Questionnaire para cribado de trastornos emocionales y de conducta. RESULTADOS: Se evaluaron 96 pacientes (58% mujeres) con mediana de edad de 15 años (11-19,1) y mediana de edad del diagnóstico de 1,70 años (0-12,2). La mediana de CD4 en el momento del corte fue 626 céls/mm3 (132-998); el 72% de los pacientes presentaban una carga viral < 50 cop/ml. El 90% asistía al colegio; de ellos, el 60% había repetido algún curso. Conocían su diagnóstico el 81%. Solo el 30% conocía bien su enfermedad y el 18,2% había compartido el diagnóstico con sus amistades. Se detectaron 6 embarazos durante el periodo de estudio. El Strengths and Difficulties Questionnaire mostró riesgo de hiperactividad en el 33%. CONCLUSIÓN: Se objetivan dificultades psicosociales en un elevado porcentaje de pacientes (conocimiento de la enfermedad, relación con pares, fracaso escolar...) que podrían tener impacto en su incorporación a la vida adulta. Son necesarios más estudios para profundizar en el origen y evolución de las dificultades observadas, así como intervenir para prevenir y modificar esta situación

INTRODUCTION: Thanks to advances in antiretroviral treatment, children with HIV infections through vertical transmission have improved their life expectancy. However, new challenges have emerged. We propose this study in order to determine the psychosocial aspects and knowledge of infections in a cohort of adolescents with vertically transmitted HIV infections. METHODS: Patients with vertically-acquired HIV infection between 12 and 19 years old were included. Data were obtained through semi-structured interviews and a Strengths and Difficulties Questionnaire for emotional and behavioral disorders screening. RESULTS: We evaluated 96 patients (58% females) with a median age of 15 years (11-19.1) and a median age at diagnosis of 1.70 years (0-12.2). The median CD4 count was 626 cells/mm3 (132-998), and the viral load was < 50 cp/ml in 72% of patients. Among them, 90% attended school and 60% repeated at least one course. Although 81% of them knew of their diagnosis, only 30% understood their disease, with 18.2% having discussed it with friends. Six unwanted pregnancies occurred during the study period. Strengths and Difficulties Questionnaire showed hyperactivity risk in 33%. CONCLUSION: A high percentage of adolescents show difficulties in several areas (disease knowledge, peer relationship, school failure...) that can have an impact on their adult lives. Further studies are needed to evaluate their origin and development in depth, as well as interventions to modify this situation

[Psychosocial aspects in a cohort of vertically transmitted human immunodeficiency virus-infected adolescents]. / Aspectos psicosociales en una cohorte de adolescentes con infección por el virus de la inmunodeficiencia humana por transmisión vertical. NeuroCoRISpeS.

INTRODUCTION: Thanks to advances in antiretroviral treatment, children with HIV infections through vertical transmission have improved their life expectancy. However, new challenges have emerged. We propose this study in order to determine the psychosocial aspects and knowledge of infections in a cohort of adolescents with vertically transmitted HIV infections. METHODS: Patients with vertically-acquired HIV infection between 12 and 19 years old were included. Data were obtained through semi-structured interviews and a Strengths and Difficulties Questionnaire for emotional and behavioral disorders screening. RESULTS: We evaluated 96 patients (58% females) with a median age of 15 years (11-19.1) and a median age at diagnosis of 1.70 years (0-12.2). The median CD4 count was 626cells/mm(3) (132-998), and the viral load was<50cp/ml in 72% of patients. Among them, 90% attended school and 60% repeated at least one course. Although 81% of them knew of their diagnosis, only 30% understood their disease, with 18.2% having discussed it with friends. Six unwanted pregnancies occurred during the study period. Strengths and Difficulties Questionnaire showed hyperactivity risk in 33%. CONCLUSION: A high percentage of adolescents show difficulties in several areas (disease knowledge, peer relationship, school failure...) that can have an impact on their adult lives. Further studies are needed to evaluate their origin and development in depth, as well as interventions to modify this situation.

Supervivencia de cáncer infantil. Efectos en el tiempo. Un estudio cualitativo / Childhood cancer survivors. Psychosocial long -term effects. A cualitative study

La supervivencia en cáncer infantil creció dramáticamente en las últimas décadas. Paralelamente a estos cambios epidemiológicos apareció una nueva población de estudio en el campo de la Psico-oncología: los supervivientes. Más allá de los distintos abordajes hay un elemento siempre presente que constata todo aquél que trabaja con esta población: El tratamiento deja marcas. El concepto de marcas, huellas, permite incluir las distintas líneas de investigación que se han llevado a cabo en esta población: secuelas post traumáticas, déficit, desajustes, por un lado y crecimiento post traumático, resiliencia por otro. El objetivo general del estudio es conocer los significados personales que tomó la experiencia para los niños y sus padres y explorar la relación entre edad al diagnóstico, tipo de comunicación y estilo de afrontamiento familiar con el tipo de huella que se encuentra en el superviviente y con la existencia o no de marcas y de recuerdos. Al ser un estudio muy amplio, en esta comunicación nos centraremos en las marcas, los significados personales y la influencia de la edad al diagnóstico respecto del recuerdo. También en la importancia de la interacción padres-niño en el proceso de atravesar la experiencia y otorgarle significado. A partir del presente trabajo se observa que el rol de los padres, la forma en que han tramitado la experiencia y cómo la han transmitido es decisivo en el resultado que experimentan los niños. La edad el diagnóstico parece ser una variable clave a la hora de determinar los efectos (AU)

As childhood cancer survival rates had a dramatic growth in the last two decades, a new population of study emerged: childhood cancer survivors. There have been great interest in understanding the psychosocial sequelae and identifying needed areas of intervention. Although working with different approaches and frameworks, every scientist working in the field agrees that cancer treatments leaves marks, imprints, traces of the experience. The concept of traces allows to include different lines of study: post-traumatic stress, psychosocial sequelae, deficits and maladjustment on one hand and post-traumatic growth, resilience on the other. The objective of the study was to explore parents and children personal meanings about the experience and analyze the relationship of age at diagnosis, communication style and coping style in the family with type of trace in the adolescent and presence or absence of memories. This communication focuses of the influence of parent survivor interaction and age at diagnosis in the meaning making process and elaboration of the experience. Parents role, their personal meanings and the way the transmit the experience determine children outcome. Age at diagnoses seems to be essential to have own memories and experience post traumatic growth (AU)

Initial validation of the Argentinean Spanish version of the PedsQL 4.0 Generic Core Scales in children and adolescents with chronic diseases: acceptability and comprehensibility in low-income settings.

BACKGROUND: To validate the Argentinean Spanish version of the PedsQL 4.0 Generic Core Scales in Argentinean children and adolescents with chronic conditions and to assess the impact of socio-demographic characteristics on the instrument's comprehensibility and acceptability. Reliability, and known-groups, and convergent validity were tested. METHODS: Consecutive sample of 287 children with chronic conditions and 105 healthy children, ages 2-18, and their parents. Chronically ill children were: (1) attending outpatient clinics and (2) had one of the following diagnoses: stem cell transplant, chronic obstructive pulmonary disease, HIV/AIDS, cancer, end stage renal disease, complex congenital cardiopathy. Patients and adult proxies completed the PedsQL 4.0 and an overall health status assessment. Physicians were asked to rate degree of health status impairment. RESULTS: The PedsQL 4.0 was feasible (only 9 children, all 5 to 7 year-olds, could not complete the instrument), easy to administer, completed without, or with minimal, help by most children and parents, and required a brief administration time (average 5-6 minutes). People living below the poverty line and/or low literacy needed more help to complete the instrument. Cronbach Alpha's internal consistency values for the total and subscale scores exceeded 0.70 for self-reports of children over 8 years-old and parent-reports of children over 5 years of age. Reliability of proxy-reports of 2-4 year-olds was low but improved when school items were excluded. Internal consistency for 5-7 year-olds was low (alpha range = 0.28-0.76). Construct validity was good. Child self-report and parent proxy-report PedsQL 4.0 scores were moderately but significantly correlated (rho = 0.39, p < 0.0001) and both significantly correlated with physician's assessment of health impairment and with child self-reported overall health status. The PedsQL 4.0 discriminated between healthy and chronically ill children (72.72 and 66.87, for healthy and ill children, respectively, p = 0.01), between different chronic health conditions, and children from lower socioeconomic status. CONCLUSION: Results suggest that the Argentinean Spanish PedsQL 4.0 is suitable for research purposes in the public health setting for children over 8 years old and parents of children over 5 years old. People with low income and low literacy need help to complete the instrument. Steps to expand the use of the Argentinean Spanish PedsQL 4.0 include an alternative approach to scoring for the 2-4 year-olds, further understanding of how to increase reliability for the 5-7 year-olds self-report, and confirmation of other aspects of validity.

Valid informed consent and participative decision-making in children with cancer and their parents: a report of the SIOP Working Committee on psychosocial issues in pediatric oncology.

This is the tenth official document of the SIOP Working Committee on psychosocial issues in pediatric oncology, instituted in 1991. It is addressed to the pediatric oncology community. Children clearly have a right to participate in medical decisions regarding their own treatment, based on the developmental level of the child. The objective of these guidelines is to encourage physicians to share with the child developmentally relevant medical information specific to that particular child's health status, in the context of the child's own culture, so that he or she can actively participate in the decision-making process regarding his or her own health. These guidelines, geared toward this objective, discuss the child's right to medical information, the parents' legal responsibility for but not exclusive right over the child's health, and ways of encouraging the younger child's active participation in his or her own health care at an age-appropriate level of understanding. For adolescents, there should be a full and legally mandated power to make their own decisions regarding medical treatment.