Aim: To evaluate compliance in children with acute lymphoblastic leukaemia (ALL). Methods: Compliance was assessed through specific interviews, annotations from medical charts, and erythrocytic determination of 6-mercaptopurine metabolites. Results: A total of 39 patients who had concluded maintenance phase of chemotherapy were included in the study. Mothers were responsible for delivering 6-MP in 87% of cases. Thirty five interviewees said that medical prescription was well understood and that the main reason for non-compliance was forgetfulness. Non-compliance was detected through interviews (33.3% of the cases), reports from medical charts (30.7%), and drug determination (16.6%); 53.8% of children were found to be non-compliant. Noncompliance was significantly associated with chronic undernourishment. Although not statistically significant, there was a trend for the group of non-compliant children to be associated with low per capita family income. No significant associations of non-compliance with age at diagnosis, gender, parents' schooling level, number of family members, power consumption, and medians of absolute leucocyte or neutrophil blood counts were detected. A short follow up period precluded valid analysis on outcome. In the non-compliant group (n = 21), seven children relapsed, contrasting with three relapses in the compliant group (n = 18). Conclusions: Results suggest that non-compliance is one of the mechanisms which underlies the adverse influence of socioeconomic factors on the outcome of children with ALL. Additional studies are necessary to confirm this hypothesis. Comprehensive approaches to the problem of non-compliance are urgently needed.A cute lymphoblastic leukaemia (ALL) is the most common malignancy in childhood. Nowadays, 70-80% of the recently diagnosed children in developed countries present a disease free survival of over five years, and most will eventually be cured.

OBJETIVOS: conhecer a percepção dos pais para o diagnóstico de leucemia em seus filhos. MÉTODOS: pesquisa qualitativa tendo como referencial a teoria das representações sociais ancorada na tradição da sociologia compreensiva. Utilizou-se entrevista semi estruturada para a coleta de dados. Foram entrevistados 20 pais de crianças e adolescentes com diagnóstico de leucemia linfóide aguda (LLA). Para análise dos dados empregou-se a técnica de "análise do conteúdo". As diretrizes de humanização da assistência e princípios bioéticos foram os eixos norteadores da análise dos dados. RESULTADOS: os pais recordaram-se claramente da progressão dos sintomas da doença até o diagnóstico. A palavra leucemia foi associada ao câncer e à morte. Dificuldades relacionadas à escuta dos sintomas, a particularidades clínicas da leucemia e ao encaminhamento no sistema de saúde foram os principais motivos para a demora do diagnóstico definitivo. Destacam-se as peregrinações por diferentes locais, perda da resolubilidade, gastos desnecessários, desgaste emocional e atraso nas ações de saúde. CONCLUSÕES: o câncer envolve valores culturais sobre a morte, cuja carga simbólica é maior no caso da criança. A busca do diagnóstico da leucemia pelos pais é exemplo emblemático do tortuoso caminho percorrido quando o mal que acomete a criança não se enquadra no rótulo das doenças "comuns".

The clinical manifestations of Langerhans cell histiocytosis vary widely, with a high relapse rate and low mortality rate.

Autoimmune hemolytic anemia is rare in children and adolescents. Although patients usually respond to corticosteroids and/or immunoglobulin, fatal cases can occur. Prognosis is worse in patients with chronic underlying diseases.

This study aimed at evaluating the use of immunophenotyping (IMP) in the identification of blast cells in the cerebrospinal fluid (CSF) of children and adolescents with acute lymphoblastic leukemia (ALL). Sixty-seven patients aged 18 years or younger were included. Fifty-five CSF samples were analyzed at initial diagnosis and 17 at the time of relapse. A cytological analysis (CA) was performed in all 72 samples, while IMP was done in 63. Blasts were identified in only three samples by CA, whereas all three samples were found negative by IMP, one of which had no isolation of nucleated cells after centrifugation. Among the samples analyzed by IMP, 11 showed a positive blast count, two of which had been inconclusive using CA. No equivalence was found between CA and IMP results (p = 0.55). CSF IMP positivity was not associated with other risk factors for ALL relapse. Among the 55 patients included at the time of diagnosis of ALL, eight relapsed during follow-up. Considering the cases of central nervous system (CNS) relapse, one of the patients belonged to the CSF IMP-positive group (11%) at diagnosis, and the other two cases, to the IMP-negative (5%) group. Detection of CSF blast cells using IMP was associated with a worse overall (p < 0.0001) and event-free survival (p < 0.0001). These results show that CSF IMP may be a useful additional method to conventional CA in the diagnosis of CNS involvement in ALL, and for the identification of high-risk subgroups that would benefit from an intensified therapy.

Results suggest that comprehensive approaches to the problem of non-compliance are urgently needed.

ObjectiveTo evaluate the approach to palliative care for hematological oncology patients in the pediatric ward of a tertiary hospital.MethodsThis was a retrospective, descriptive study of 29 hematological oncology patients who died between 2009 and 2011. Data regarding the approach and prevalence of pain, prevalence of other symptoms, multidisciplinary team participation, communication between staff and family and limited invasive therapy were collected from the medical records.ResultsTwenty-seven (93.1%) patients displayed disease progression unresponsive to curative treatment. The median age at death was ten years old. Pain was the most prevalent symptom with all patients who reported pain receiving analgesic medications. The majority took weak (55.2%) and/or strong (65.5%) opioids. The patients were followed by pediatricians and a pediatric hematologist/oncologist. Participation of other professionals was also documented: 86.2% were followed by social services and 69% by psychologists, among others. There were explicit descriptions of limitation of invasive therapy in the medical records of 26 patients who died with disease progression. All these decisions were shared with the families.ConclusionAlthough the hospital where this study was conducted does not have a specialized team in pediatric palliative care, it meets all the requirements for developing a specific program. The importance of approaching pain and other prevalent symptoms in children with cancer involving a comprehensive multidisciplinary team is evident. Discussions were had with most of the families on limiting invasive therapy, but no record of a well-defined and coordinated treatment plan for palliative care was found.

ResumoA expansão tecnológica das últimas décadas modificou o perfil dos pacientes na pediatria, tornando cada vez mais frequente a assistência a crianças com doenças crônicas e ameaçadoras à vida. Cuidados paliativos envolvem a assistência ativa e total prestada a essas crianças, nas dimensões de seu corpo, mente e espírito, bem como o suporte oferecido a sua família. O presente estudo revisa aspectos relevantes na abordagem paliativa: a prevalência e abordagem dos sintomas, a participação de equipe multiprofissional, a comunicação com a família, os cuidados ao final da vida e a limitação de terapêuticas invasivas. A despeito do reconhecimento da importância da medicina paliativa, nossas universidades ainda priorizam a medicina curativa. Instituir esse tema no processo de formação do médico é uma necessidade real e absoluta, visando proporcionar vida e morte dignas aos nossos pacientes. Palavras-chave: Cuidados paliativos. Dor. Assistência. Criança. ResumenLos cuidados paliativos en pediatría: una revisión La expansión tecnológica de las últimas décadas ha cambiado el perfil de los pacientes de pediatría, volviendo cada vez más frecuente la asistencia a los niños con enfermedades crónicas y potencialmente mortales. Cuidados paliativos involucran la asistencia activa y total prestada a estos niños y niñas, en el ámbito de su cuerpo, mente y espíritu, así como el apoyo ofrecido a toda su familia. Este estudio proporciona un análisis de los aspectos relevantes en el enfoque paliativo: la prevalencia y abordaje de los síntomas, la participación de un equipo multidisciplinario, la comunicación con la familia, la atención al final de la vida y la limitación de las terapias invasivas. A pesar del reconocimiento de la importancia de la medicina paliativa, nuestras universidades siguen dando prioridad a la medicina curativa. Establecer ese tema en el proceso de la educación médica es una necesidad real y absoluta, con el objetivo de proporcionar una vida y una muerte dignas a nuestros pacientes. Palabras-clave: Cuidados paliativos. Dolor. Atención. Niños. Abstract Palliative care in pediatrics: a reviewThe technological expansion in recent decades changed the profile of the patients in pediatrics, with assistance to children with chronic and life-threatening conditions more frequent each time. Palliative care involves an active and full assistance provided to these children, in the context of their body, mind and spirit as well as the support offered to their entire family. This study provides a review of relevant aspects in palliative approach: the prevalence and approach of symptoms, the participation of a multidisciplinary team, communication with family, care at end of life and limitation of invasive therapies. Despite recognition of the importance of palliative medicine, our universities still give priority to curative medicine. Introducing this theme in the process of medical education is a real and absolute need, aiming to provide dignified life and death to our patients.