Scope of services provided to childhood cancer patients by the Brazilian Pediatric Palliative Care Network.

Introduction: Brazil is a developing and an Upper Middle Income, categorized by the World Bank. Therefore, it is a country that needs a special vision for children with oncological diseases who require Pediatric Palliative Care. This study aimed to understand the specificities of services that provide oncology services in comparison to those that do not provide oncological care. Methods: This is a descriptive, cross-sectional, and online survey study. A questionnaire was created by a multidisciplinary group of leaders from the Brazilian Pediatric Palliative Care Network and then the survey was distributed using a snowball strategy. Results: Of the 90 services that answered the questionnaire, 40 (44.4%) attended oncologic patients. The Southeast represented most of the services (57.57%), followed by the Northeast, with 18.89% (17 services), the South with 12.22% (11 services), and the Center West with 8.89% (8 services). No differences were observed in access to opioid prescriptions between the services. It was observed that those services that attended oncologic patients had a tendency to dedicate more time to Pediatric Palliative Care. Discussion: The distribution of services that cover oncology and those that do not, are similar in the different regions of Brazil. In Brazil, there are difficulties in accessing opioids in pediatrics: access to opioid prescriptions without differences revealed that even pediatric oncologists might have difficulty with this prescription, and this should improve. It is concluded that education in Pediatric Palliative Care is the key to improvements in the area.

Pediatric palliative care for metabolic diseases: 20-year epidemiological survey of outpatients at a Brazilian quaternary hospital.

The interface between pediatric palliative care (PPC) and inborn metabolic diseases (IMD) remains incipient, though these conditions fill the state of art of complex chronic diseases, eligible to this health approach. We analyzed the medical records of PPC clinic during the years 2001 to 2021 and the IMD outpatients. We established a parallel with the world scientific literature concerning the epidemiology of PPC and IMD. Among outpatients, 14% were diagnosed with IMD, which were referred to the PPC service earlier compared to Non-IMD cases. The Group 3 (complex molecules) was the most frequent (64.7%), following by Group 1 representing by small molecules (21.6%), the latter having a lower median age at diagnosis when compared to the former (0.7 vs. 5.2 years, p = 0.001). The sphingolipidoses were the pathologies most frequent in our cohort, in line with what was observed in the literature. There were no differences between IMD groups in terms of diagnosis and PPC referral age, however in Non-IMD conditions, the age of diagnosis were earlier than IMD. Nevertheless, IMD group showed lower age of referral to PPC. The IMD comprises large fraction of outpatients in the PPC setting, thus further studies are needed in this field.

Advantages of whole-exome sequencing over immunomapping in 67 Brazilian patients with epidermolysis bullosa.

BACKGROUND: Epidermolysis bullosa (EB) is characterized by skin fragility and blistering. In Brazil, the diagnosis is usually obtained through immunomapping, which involves a skin biopsy. Most recently, whole exome sequencing (WES) has become an important tool for the diagnosis of the subtypes of EB, providing information on prognosis as well as allowing appropriate genetic counseling for the families. OBJECTIVE: To compare the results of immunomapping and molecular analysis and to describe the characteristics of a Brazilian cohort of patients with EB. METHODS: Patients were submitted to clinical evaluation and WES using peripheral blood samples. WES results were compared to those obtained from immunomapping testing from skin biopsies. RESULTS: 67 patients from 60 families were classified: 47 patients with recessive dystrophic EB (DEB), 4 with dominant DEB, 15 with EB simplex (EBS), and 1 with junctional EB (JEB). Novel causative variants were: 10/60 (16%) in COL7A1 associated with recessive DEB and 3 other variants in dominant DEB; one homozygous variant in KRT5 and another homozygous variant in PLEC, both associated with EBS. Immunomapping was available for 59 of the 67 patients and the results were concordant with exome results in 37 (62%), discordant in 13 (22%), and inconclusive in 9 patients (15%). STUDY LIMITATIONS: Even though EB is a rare disease, for statistical purposes, the number of patients evaluated by this cohort can still be considered limited; other than that, there was a significant difference between the proportion of types of EB (only one case with JEB, against more than 50 with DEB), which unfortunately represents a selection bias. Also, for a small subset of families, segregation (usually through Sanger sequencing) was not an option, usually due to deceased or unknown parent status (mostly the father). CONCLUSION: Although immunomapping has been useful in services where molecular studies are not available, this invasive method may provide a misdiagnosis or an inconclusive result in about 1/3 of the patients. This study shows that WES is an effective method for the diagnosis and genetic counseling of EB patients.

Advantages of whole-exome sequencing over immunomapping in 67 Brazilian patients with epidermolysis bullosa

Abstract Background Epidermolysis bullosa (EB) is characterized by skin fragility and blistering. In Brazil, the diagnosis is usually obtained through immunomapping, which involves a skin biopsy. Most recently, whole exome sequencing (WES) has become an important tool for the diagnosis of the subtypes of EB, providing information on prognosis as well as allowing appropriate genetic counseling for the families. Objective To compare the results of immunomapping and molecular analysis and to describe the characteristics of a Brazilian cohort of patients with EB. Methods Patients were submitted to clinical evaluation and WES using peripheral blood samples. WES results were compared to those obtained from immunomapping testing from skin biopsies. Results 67 patients from 60 families were classified: 47 patients with recessive dystrophic EB (DEB), 4 with dominant DEB, 15 with EB simplex (EBS), and 1 with junctional EB (JEB). Novel causative variants were: 10/60 (16%) in COL7A1 associated with recessive DEB and 3 other variants in dominant DEB; one homozygous variant in KRT5 and another homozygous variant in PLEC, both associated with EBS. Immunomapping was available for 59 of the 67 patients and the results were concordant with exome results in 37 (62%), discordant in 13 (22%), and inconclusive in 9 patients (15%). Study limitations Even though EB is a rare disease, for statistical purposes, the number of patients evaluated by this cohort can still be considered limited; other than that, there was a significant difference between the proportion of types of EB (only one case with JEB, against more than 50 with DEB), which unfortunately represents a selection bias. Also, for a small subset of families, segregation (usually through Sanger sequencing) was not an option, usually due to deceased or unknown parent status (mostly the father). Conclusion Although immunomapping has been useful in services where molecular studies are not available, this invasive method may provide a misdiagnosis or an inconclusive result in about 1/3 of the patients. This study shows that WES is an effective method for the diagnosis and genetic counseling of EB patients.

Patau and Edwards Syndromes in a University Hospital: beyond palliative care / Síndrome de Edwards e Patau em um hospital universitário: além do paliativo

ABSTRACT Objective: To describe the newborn population with Patau (T13) and Edwards Syndrome (T18) with congenital heart diseases that stayed in the Intensive Care Unit (ICU) of a quaternary care hospital complex, regarding surgical and non-surgical medical procedures, palliative care, and outcomes. Methods: Descriptive case series conducted from January/2014 to December/2018 through analysis of records of patients with positive karyotype for T13 or T18 who stayed in the ICU of a quaternary hospital. Descriptive statistics analysis was applied. Results: 33 records of eligible patients were identified: 27 with T18 (82%), and 6 T13 (18%); 64% female and 36% male. Eight were preterm infants with gestational age between 30-36 weeks (24%), and only 4 among the 33 infants had a birth weight >2500 g (12%). Four patients underwent heart surgery and one of them died. Intrahospital mortality was 83% for T13, and 59% for T18. The majority had other malformations and underwent other surgical procedures. Palliative care was offered to 54% of the patients. The median hospitalization time for T18 and T13 was 29 days (range: 2-304) and 25 days (13-58), respectively. Conclusions: Patients with T13 and T18 have high morbidity and mortality, and long hospital and ICU stays. Multicentric studies are needed to allow the analysis of important aspects for creating protocols that, seeking therapeutic proportionality, may bring better quality of life for patients and their families.

RESUMO Objetivo: Descrever a população de recém-nascidos com síndrome de Patau (T13) e Edwards (T18) portadores de cardiopatias congênitas, que permaneceram em Unidades de Terapia Intensiva (UTI) de um complexo hospitalar quaternário, com relação a conduta cirúrgica ou não, cuidados paliativos e seus desfechos. Métodos: Série de casos de pacientes internados entre janeiro de 2014 a dezembro de 2018, com análise dos prontuários de portadores de T13 ou T18 que permaneceram internados em UTI que recebem neonatos nesse hospital quaternário. Utilizou-se análise estatística descritiva. Resultados: Foram identificados 33 prontuários para análise — 27 T18 (81,8%) e seis T13 (18,2%); 64% do sexo feminino e 36% do sexo masculino. Oito foram prematuros, nascidos com 30 a 36 semanas (24,2%), e apenas quatro nasceram com mais de 2500 g (12,1%). Quatro pacientes foram submetidos a cirurgia cardíaca e um deles foi a óbito. A mortalidade intra-hospitalar foi de 83% para T13 e 59% para T18. A maioria apresentava outras malformações e foi submetida a outras cirurgias. Cuidados paliativos foram oferecidos a 54% dos pacientes. A mediana do tempo de hospitalização para T18 e T13 foi respectivamente de 29 dias (variação: 2-304) e 25 dias (13-58). Conclusões: Pacientes com T13 e T18 cursam com alta morbimortalidade e longa permanência hospitalar em UTI. São necessários estudos multicêntricos para melhor análise de aspectos importantes para a criação de protocolos que, buscando proporcionalidade terapêutica, tragam melhor qualidade de vida para os pacientes e suas famílias.

Patau and Edwards Syndromes in a University Hospital: beyond palliative care.

OBJECTIVE: To describe the newborn population with Patau (T13) and Edwards Syndrome (T18) with congenital heart diseases that stayed in the Intensive Care Unit (ICU) of a quaternary care hospital complex, regarding surgical and non-surgical medical procedures, palliative care, and outcomes. METHODS: Descriptive case series conducted from January/2014 to December/2018 through analysis of records of patients with positive karyotype for T13 or T18 who stayed in the ICU of a quaternary hospital. Descriptive statistics analysis was applied. RESULTS: 33 records of eligible patients were identified: 27 with T18 (82%), and 6 T13 (18%); 64% female and 36% male. Eight were preterm infants with gestational age between 30-36 weeks (24%), and only 4 among the 33 infants had a birth weight >2500 g (12%). Four patients underwent heart surgery and one of them died. Intrahospital mortality was 83% for T13, and 59% for T18. The majority had other malformations and underwent other surgical procedures. Palliative care was offered to 54% of the patients. The median hospitalization time for T18 and T13 was 29 days (range: 2-304) and 25 days (13-58), respectively. CONCLUSIONS: Patients with T13 and T18 have high morbidity and mortality, and long hospital and ICU stays. Multicentric studies are needed to allow the analysis of important aspects for creating protocols that, seeking therapeutic proportionality, may bring better quality of life for patients and their families.

Exploring the Brazilian pediatric palliative care network: a quantitative analysis of a survey data.

OBJECTIVE: The aim of this study was to identify the characteristics of services in Brazil that compound the Brazilian Pediatric Palliative Care (PPC) Network. METHODS: An online survey was conducted among representatives from PPC services. A total of 90 services from Brazil completed the online survey and answered a questionnaire about the service's characterization, health professionals working in PPC, access to opioid prescription and education, and research in PPC. RESULTS: In total, 80 services (88.9%) were created after 2010, 52 (57.9%) were in the southeast region, 56 (62.2%) were in public hospitals, 63 (70%) had up to 100 beds, and 57 (63.3%) were at the tertiary level. Notably, 88 (97.8%) had a physician in the team and 68 (75.5%) dedicated part-time to PPC. Also, 33 (36.7%) revealed concern with the care of health professionals and 36 (40%) reported difficulty or no access to opioid prescription. Research studies were reported to be conducted in 29 (32.2%) services. CONCLUSIONS: This mapping points out to a concentration of PPC services in the southeast region, with part-time professional dedication, and the need to improve professionals' care. Difficulty in opioid access was reported. It is necessary to extend PPC participation to other Brazilian regions, increase time dedicated to PPC, improve professionals' care and improve access to opioid prescription.

Exploring the Brazilian pediatric palliative care network: a quantitative analysis of a survey data / Explorando a rede brasileira de cuidados paliativos pediátricos: uma análise quantitativa de levantamento de dados

Abstract Objective: The aim of this study was to identify the characteristics of services in Brazil that compound the Brazilian Pediatric Palliative Care (PPC) Network. Methods: An online survey was conducted among representatives from PPC services. A total of 90 services from Brazil completed the online survey and answered a questionnaire about the service's characterization, health professionals working in PPC, access to opioid prescription and education, and research in PPC. Results: In total, 80 services (88.9%) were created after 2010, 52 (57.9%) were in the southeast region, 56 (62.2%) were in public hospitals, 63 (70%) had up to 100 beds, and 57 (63.3%) were at the tertiary level. Notably, 88 (97.8%) had a physician in the team and 68 (75.5%) dedicated part-time to PPC. Also, 33 (36.7%) revealed concern with the care of health professionals and 36 (40%) reported difficulty or no access to opioid prescription. Research studies were reported to be conducted in 29 (32.2%) services. Conclusions: This mapping points out to a concentration of PPC services in the southeast region, with part-time professional dedication, and the need to improve professionals' care. Difficulty in opioid access was reported. It is necessary to extend PPC participation to other Brazilian regions, increase time dedicated to PPC, improve professionals' care and improve access to opioid prescription.

RESUMO Objetivo: Identificar as características dos serviços que compõem a Rede Brasileira de Cuidados Paliativos Pediátricos (CPP). Métodos: Estudo do tipo survey online entre representantes de serviços de CPP. O total de 90 serviços do Brasil participaram da pesquisa e responderam a um questionário sobre a caracterização do serviço, os profissionais de saúde que nele atuam, o acesso à prescrição de opioides e a educação e pesquisa em CPP. Resultados: Oitenta serviços (88,9) foram criados após 2010, 52 (57,9%) estão na Região Sudeste, 56 (62,2%) em hospitais públicos, 63 (70%) têm até cem leitos e 57 (63,3%) estão no nível terciário. Oitenta e oito serviços (97,8%) tinham um médico na equipe e, em 68 (75,5%), os profissionais dedicavam parte de seu tempo aos CPP. Trinta e três serviços (36,7%) revelaram preocupação com o atendimento aos profissionais de saúde. Trinta e seis (40%) relataram dificuldade ou nenhum acesso à prescrição de opioides. Foi reportada a realização de pesquisas em 29 (32,2%). Conclusões: Este mapeamento aponta para a concentração dos serviços de CPP na Região Sudeste, com dedicação parcial dos profissionais, e para a necessidade de cuidar deles. Foi relatada dificuldade no acesso aos opioides. É necessário estender a participação na rede de CPP para outras regiões do Brasil, aumentar o tempo dedicado aos CPP pelos profissionais e cuidar destes, além de melhorar o acesso à prescrição de opioides.

Clinical management of fever in children in Brazil: practical recommendations from an expert panel.

The objective of this study was to answer several questions related to the assessment and treatment of fever, as well as other controversies that exist during its management in pediatric patients. First, an advisory board with medical experts was conducted to discuss the clinical journey of these patients, considering the main challenges and possible solutions. After this discussion, a non-systematic literature review was performed, between November 2019 and January 2020, to collect the most relevant evidence available in the scientific databases MEDLINE, Lilacs, and SciELO. A narrative review was carried out based on scientific evidence and on extensive experience of experts in clinical practice. The experts developed a set of recommendations and clarifications about the assessment of the severity of fever in pediatrics, the need for treatment and the choice of the most appropriate antipyretic. The most common controversies in the management of fever in pediatric patients were also addressed, such as alternating antipyretics, persistent fever, and dose equivalence. In primary management of pediatric patients, fever should be seen as a relevant symptom that requires treatment with antipyretics in potentially more complex or severe cases, when it causes discomfort to children or is associated with infectious diseases.

Clinical management of fever in children in Brazil: practical recommendations from an expert panel

ABSTRACT The objective of this study was to answer several questions related to the assessment and treatment of fever, as well as other controversies that exist during its management in pediatric patients. First, an advisory board with medical experts was conducted to discuss the clinical journey of these patients, considering the main challenges and possible solutions. After this discussion, a non-systematic literature review was performed, between November 2019 and January 2020, to collect the most relevant evidence available in the scientific databases MEDLINE, Lilacs, and SciELO. A narrative review was carried out based on scientific evidence and on extensive experience of experts in clinical practice. The experts developed a set of recommendations and clarifications about the assessment of the severity of fever in pediatrics, the need for treatment and the choice of the most appropriate antipyretic. The most common controversies in the management of fever in pediatric patients were also addressed, such as alternating antipyretics, persistent fever, and dose equivalence. In primary management of pediatric patients, fever should be seen as a relevant symptom that requires treatment with antipyretics in potentially more complex or severe cases, when it causes discomfort to children or is associated with infectious diseases.

Peculiarities of neuropathic pain in children / Peculiaridades da dor neuropática na criança

ABSTRACT BACKGROUND AND OBJECTIVES: In spite of its importance, neuropathic pain is underestimated and undertreated in childhood. Although many conditions causing neuropathic pain in adults are uncommon during childhood, most therapeutic strategies effective for adults were extrapolated for the pediatric population. This study aimed at bringing to light peculiarities of neuropathic pain in children, which shall contribute for its better recognition in childhood and adolescence. CONTENTS: Pubmed, Scielo and Lilacs databases were queried to identify studies and literature reviews on neuropathic pain during childhood and adolescence. CONCLUSION: Neuropathic pain treatment in childhood and adolescence should be multimodal, including pharmacological treatment, rehabilitation and psychological support. Rehabilitation, physiotherapy, occupational therapy and integrative therapies should be part of the integral treatment in childhood and adolescence and vary in a case-by-case basis.

RESUMO JUSTIFICATIVA E OBJETIVOS: Apesar de sua importância a dor neuropática é subestimada e subtratada na infância. Embora muitas das condições que causem dor neuropática no adulto sejam raras na infância; grande parte das estratégias terapêuticas que demonstraram ser eficazes em adultos foi extrapolada para uso na população pediátrica. O objetivo deste artigo foi trazer à luz peculiaridades da dor neuropática na criança, o que contribuirá para seu melhor reconhecimento na infância e adolescência. CONTEÚDO: Foram realizadas buscas nos bancos de dados Pubmed, Scielo e Lilacs, identificando-se estudos e revisões da literatura sobre dor neuropática na infância e adolescência. CONCLUSÃO: O tratamento da dor neuropática na infância e adolescência deve ser multimodal, incluindo tratamento farmacológico, reabilitação e suporte psicológico. Reabilitação, fisioterapia, terapia ocupacional e terapias integrativas devem fazer parte do tratamento integral na infância e adolescência, e variam conforme as necessidades em cada caso.

Air pollution and children's health: sickle cell disease.

The hallmarks of sickle cell disease are anemia and vasculopathy. The aim of this study was to assess the association between air pollution and children's emergency room visits of sickle cell patients. We adopted a case-crossover design. Daily counts of children's and adolescents' sickle cell disease emergency room visits from the pediatric emergency unit in São Paulo, Brazil, were evaluated from September 1999 to December 2004, matching by temperature, humidity and controlling for day of the week. Interquartile range increases of the four-day moving averages of PM10, NO2, SO2, CO, and O3 were associated with increases of 18.9% (95%CI: 11.2-26.5), 19% (95%CI: 8.3-29.6), 14.4% (95%CI: 6.5-22.4), 16,5% (95%CI: 8.9-24.0), and 9.8% (95%CI: 1.1-18.6) in total sickle cell emergency room visits, respectively. When the analyses were stratified by pain, PM10 was found to be 40.3% higher than in sickle cell patients without pain symptoms. Exposure to air pollution can affect the cardiovascular health of children and may promote a significant health burden in a sensitive group.

Air pollution and children's health: sickle cell disease / Poluição do ar e a saúde das crianças: a doença falciforme / Contaminación atmosférica y salud de los niños: la enfermedad de células falciformes

The hallmarks of sickle cell disease are anemia and vasculopathy. The aim of this study was to assess the association between air pollution and children's emergency room visits of sickle cell patients. We adopted a case-crossover design. Daily counts of children's and adolescents' sickle cell disease emergency room visits from the pediatric emergency unit in São Paulo, Brazil, were evaluated from September 1999 to December 2004, matching by temperature, humidity and controlling for day of the week. Interquartile range increases of the four-day moving averages of PM10, NO2, SO2, CO, and O3 were associated with increases of 18.9% (95%CI: 11.2-26.5), 19% (95%CI: 8.3-29.6), 14.4% (95%CI: 6.5-22.4), 16,5% (95%CI: 8.9-24.0), and 9.8% (95%CI: 1.1-18.6) in total sickle cell emergency room visits, respectively. When the analyses were stratified by pain, PM10 was found to be 40.3% higher than in sickle cell patients without pain symptoms. Exposure to air pollution can affect the cardiovascular health of children and may promote a significant health burden in a sensitive group.

O objetivo deste estudo foi avaliar a associação entre a poluição do ar e atendimentos de emergência pediátrica de pacientes portadores de anemia falciforme. Adotamos um estudo de case-crossover. Visitas de crianças e adolescentes portadores de anemia falciforme ao pronto-socorro pediátrico, em São Paulo, Brasil, foram avaliadas a partir de setembro de 1999 até dezembro de 2004, controlando a temperatura, umidade e dia da semana. Variações interquartis das médias móveis de quatro dias de PM10, NO2, SO2, CO e O3 foram associadas com aumentos de 18,9% (IC95%: 11,2-26,5), 19% (IC95%: 8,3-29,6), 14,4% (IC95%: 6,5-22,4), 16,5% (IC95%: 8,9-24,0) e 9,8% (IC95%: 1,1-18,6) nos atendimentos totais, respectivamente. Quando as análises foram estratificadas por dor, verificou-se que PM10 apresentou correlação 40,3% maior do que a observada em pacientes falciformes sem sintomas de dor. A exposição à poluição do ar pode afetar a saúde cardiovascular de crianças e promover um fardo significativo para a saúde em um grupo suscetível, como o de pacientes com anemia falciforme.

El objetivo de este estudio fue evaluar la asociación entre la contaminación atmosférica y las visitas de emergencia de los pacientes pediátricos con anemia de células falciformes. Hemos adoptado un estudio de casos cruzados. Visitas de niños y adolescentes con anemia de células falciformes en urgencias pediátricas, en São Paulo, Brasil, se evaluaron entre 1999 y 2004 con un control de la temperatura, humedad y día de la semana. El rango intercuartil de los promedios móviles de 4 días de PM10, NO2, SO2, CO y O3 se asociaron con un aumento del 18,9% (IC95%: 11,2-26,5), 19,0% (IC95%: 8,3-29,6), 14,4% (IC95%: 6,5-22,4), 16,5% (IC95%: 8,9-24,0), y 9,8% (IC95%: 1,1-18,6) en el total de visitas, respectivamente. Cuando los análisis se estratificaron por el dolor, se encontró que el PM10 mostró una correlación un 40,3% mayor que la observada en pacientes sin síntomas de dolor. La exposición a la contaminación del aire puede afectar la salud cardiovascular de los niños y puede promover una importante carga para la salud en pacientes con anemia de células falciformes.

Living with pain: the experience of children and adolescents in palliative care.

A qualitative study was conducted with semi-structured interviews with the aim of understanding the experience of children and adolescents under palliative care when managing pain daily and how they describe the intensity, quality and location of pain. We used Piaget's theory of cognitive development as a theoretical framework and oral history as a methodological framework. We found four themes: describing pain; seeking a life closer to normality, despite pain and disease; using a variety of alternatives for pain control; and living with damaged physical appearance. Although pain is a limiting factor in the lives of children and adolescents, we found that they faced their daily pain and still had a life beyond pain and illness. In addition, we highlight the relevance of nurses' understanding that effective management of pain in children is essential for a normal life and less suffering.

Living with pain: the experience of children and adolescents in palliative care / Vivir con dolor: la experiencia de los niños y adolescentes en los cuidados paliativos / Vivenciando a dor: a experiência de crianças e adolescentes em cuidados paliativos

A qualitative study was conducted with semi-structured interviews with the aim of understanding the experience of children and adolescents under palliative care when managing pain daily and how they describe the intensity, quality and location of pain. We used Piaget’s theory of cognitive development as a theoretical framework and oral history as a methodological framework. We found four themes: describing pain; seeking a life closer to normality, despite pain and disease; using a variety of alternatives for pain control; and living with damaged physical appearance. Although pain is a limiting factor in the lives of children and adolescents, we found that they faced their daily pain and still had a life beyond pain and illness. In addition, we highlight the relevance of nurses’ understanding that effective management of pain in children is essential for a normal life and less suffering.

El estudio tuvo como objetivo conocer la experiencia de niños y adolescentes en cuidados paliativos en el manejo diario del dolor. La investigación cualitativa fue realizada con entrevistas semi-estructuradas con seis niños entre siete y 17 años. La teoría del Desarrollo cognitivo de Piaget fue utilizada como Marco teórico y la Historia Oral como Referencial Metodológico. Cuatro temas fueron encontrados: describiendo el dolor; buscando una vida más próxima de la normalidad, a pesar del dolor y la enfermedad; utilizando varias alternativas para el control del dolor y viviendo la autoimagen perjudicada. A pesar del dolor ser un agente limitante en la vida de los niños y adolescentes, verificamos que enfrentaban el dolor diariamente y, que así mismo, tenían vida además del dolor y la enfermedad. Adicionamos, aun, la importancia de los enfermeros comprender que el eficaz manejo del dolor es esencial para una vida mas próxima de la normalidad, reduciendo su sufrimiento.

Objetivo Conhecer como as crianças e adolescentes em cuidados paliativos manejam a dor em seu cotidiano e como a descrevem em intensidade e qualidade. Método Pesquisa qualitativa realizada com entrevistas semiestruturadas com seis crianças entre 6 e 17 anos. A teoria do desenvolvimento cognitivo de Piaget foi utilizada como marco teórico e a história oral como referencial metodológico. Resultados Foram encontrados quatro temas: Descrevendo a dor; Buscando uma vida mais próxima da normalidade, apesar da dor e da doença; Utilizando várias alternativas para o controle da dor; e Vivenciando a autoimagem prejudicada. Conclusão Apesar de a dor ser um agente limitante na vida de crianças e adolescentes, constatamos que enfrentavam a dor diariamente e, mesmo assim, tinham vida além da dor e da doença. Acrescentamos, ainda, a importância de os enfermeiros compreenderem que o manejo eficaz da dor é essencial para uma vida mais próxima da normalidade, reduzindo seu sofrimento.

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Estudo da influência de fatores ambientais sobre o desencadeamento de crise álgica em crianças e adolescentes portadores de anemia falciforme na cidade de São Paulo / Study of the influence of environmental factors in the development of pain crisis in children and adolescents with sickle cell disease in the city of São Paulo

Objetivo: Esse estudo foi desenvolvido para avaliar o impacto dos poluentes do ar na morbidade nas crianças e adolescentes portadoras de anemia falciforme. Métodos: Foram utilizadas as associações entre as concentrações diárias entre as concentrações dos poluentes do ar (PM10, SO2, NO2, CO, e O3) e os atendimentos no Pronto Socorro de anemia falciforme (CID 10 - D57) no período de setembro de 1999 até dezembro de 2004. Foi utilizado o desenho de case - crossover com dias de exposição de referencia escolhidos utilizando-se uma abordagem estratificada por tempo onde as exposições no dia índice foram comparadas a exposições com dias do mesmo mês com o mesmo valor de temperatura como o caso índice, controlando-se para o dia da semana. Estação do ano, clima e covariantes variáveis lentas foram controladas por pareamento. Também foram adotados modelos onde as exposições do caso índice foram comparadas às exposições para o mesmo dia da semana ou a cada três dias. Os efeitos estimados também foram estratificados para as duas causas principais de atendimento no pronto socorro de pacientes falciformes, dor e infecção respiratória. Resultados: As variações de interquartile aumentaram nas médias móveis de PM10 (25.9 mg/m3), NO2 (64.8 mg/m3), SO2 (8.1 mg/m3), CO (1.2 ppm), e O3 (57.23 mg/m3) foram associadas com aumento de 19.2% (95% CI: 11.2 - 27.8), 15.6% (95% CI: 6.3 - 25.8), 14.5% (95% CI: 6.6 - 23.0), 15,1% (95% CI: 7.7 -23.1), e 10% (95% CI: 1,3 - 18,6) nos atendimentos totais dos pacientes falciformes no pronto socorro, respectivamente. Na análise estratificada para dor, que é a manifestação cardinal dos processos de vaso-oclusão, os aumentos nos atendimentos dos pacientes falciformes com dor (23.1%, 95%CI: 11.0 - 36.5) devido a um aumento na variação interquartile da média móvel de 3 dias do PM10 foi 43% maior que nos pacientes falciformes sem dor. Conclusão: esse estudo mostra que o efeito da poluição do ar na saúde da população pediátrica não se restringe...

Objective: This study was developed to assess the impact of air pollutants on sickle cell morbidity in children and adolescents. Methods we examined the associations between daily air pollutants concentrations (PM10, SO2, NO2, CO, and O3) and sickle cell (ICD10th revision: D57) emergency room visits, from September 1999 to December 2004. We applied a case-crossover design with referent exposure days chosen using the time-stratified approach such that exposures on the case day were compared to exposures on days of the same month with the same value of temperature as the case day, controlling for day of the week. Season, weather, and slowly varying covariates were controlled for by matching. We also adopted models where exposures on the case day were compared to exposures on the same day of the week or on every third day. Effects estimates were also stratified by the two main causes of sickle cell emergency room visits, pain and respiratory infections. Results: interquartile range increases of the 3-day moving averages of PM10 (25.9 mg/m3), NO2 (64.8 mg/m3), SO2 (8.1 mg/m3), CO (1.2 ppm), and O3 (57.23 mg/m3) were associated with increases of 19.2% (95% CI: 11.2 - 27.8), 15.6% (95% CI: 6.3 - 25.8), 14.5% (95% CI: 6.6 - 23.0), 15,1% (95% CI: 7.7 - 23.1), and 10% (95% CI: 1,3 - 18,6) in total sickle cell emergency room visits, respectively. When the analyses were stratified by pain, the main clinical manifestation of vase-occlusion process, increases in visits of sickle cell patients with pain (23.1%, 95%CI:11.0 -36.5) due to an interquartile range increase of the 3-day moving averages of PM10 were 43% higher than in sickle cell patients without pain. Conclusions: this study found that the effects of air pollutants on children's health are not limited to respiratory diseases. Also, among sickle cell patients, the main manifestation of adverse effect was associated with inflammatory process of vases, reproducing results already observed among healthy...

Cuidados paliativos em pediatria / Palliative Care in Pediatrics

As causas de morte na faixa etária pediátrica são substancialmente diferentes das causa de óbito da população adulta, e as necessidades das crianças e suas famílias merecem uma atenção especial com a criação de protocolos clínicos específicos para a faixa etária. Os cuidados paliativos em pediatria são necessários para uma gama de patologias as quais diferem das doenças dos adultos e, que em muitos casos são raras e de caráter familiar. O diagnóstico influencia no tipo de cuidado que a criança e a família necessitarão.