Abstract Objective Advances in medicine have increased the life expectancy of pediatric patients with chronic illnesses, and challenges with the guided transition of adolescents and young adults from pediatric clinics to adult clinics have grown. The aim of this study was to better understand readiness and factors related to this transition process in Brazil. Method In this cross-sectional study of 308 patients aged from 16 to 21 years under follow-up in pediatric specialties, the degree of readiness for transition was assessed using the Transition Readiness Assessment Questionnaire (TRAQ) and its domains. Associations with demographic data, clinical data, socio-economic level, medication adherence, family functionality, and parental satisfaction with health care were evaluated. Results The median TRAQ score was 3.7 (3.2 - 4.2). Better readiness was associated with female patients, socio-economic class A-B, current active employment, higher level of education, not failing any school year, attending medical appointments alone, functional family, and a good knowledge of disease and medications. A low correlation was observed between TRAQ and age. TRAQ presented good internal consistency (alpha-Cronbach 0.86). In the multiple linear regression, TRAQ score showed a significant association with female gender, advanced age, socio-economic class A-B, better knowledge of disease and medications, and independence to attend appointments alone. Conclusion TRAQ instrument can guide healthcare professionals to identify specific areas of approach, in order to support adolescents with chronic disease to set goals for their own personal development and improve their readiness to enter into the adult healthcare system. In this study, some factors were related to better TRAQ scores.

Abstract Objective To determine the frequency of radiographic changes in the temporomandibular joint, in a representative population of patients with Juvenile Idiopathic Arthritis (JIA) and to compare with findings in healthy controls matched by sex and age. Patients and Methods One hundred and thirty-seven panoramic radiographies (PR) from JIA patients of a pediatric rheumatology outpatient clinic were prospectively evaluated and compared to 137 PR from healthy individuals. Results 102 (74.5%) JIA patients and 47 (34.3%) controls showed at least one radiological alteration (p < 0.001). The following radiographic alterations were more frequently observed in JIA patients than in controls: erosion (p < 0.001), altered condylar morphology (p < 0.001), disproportion between condylar process and the coronoid process (p < 0.001) and accentuated curve in the antegonial notch (p = 0.002). Twenty patients (14.6%) presented the four radiographic alterations simultaneously compared to only two controls (1.5%) (p < 0.001). Conclusion Due to the difference in the frequency of findings in the PR of patients and controls, we concluded that PR has value as a screening tool. In the presence of major changes in the mandible head in the PR of patients with a confirmed diagnosis of JIA, MRI should be considered to detect an active inflammatory process in this joint.

Introduction: Tuberous sclerosis complex is an autosomal dominant neurocutaneous disorder which is characterized by multisystem involvement, including the kidney. Since renal disease is one of the causes of morbidity and mortality, close management is essential. Subjects and Methods: In this retrospective observational,single-center study, authors analyse patients with a definite diagnosis of tuberous sclerosis complex who attended Pediatric Nephrology consultation between 1998 and 2019. Results: 20 patients were included. The median age at the time of the diagnosis was 3 years, mostly after seizures and the median age of the first Pediatric Nephrology consultation was 9 years. Seventeen patients had renal disease, the majority with angiomyolipomas and a smaller number with cysts. None had hypertension or history of acute kidney failure. Systemic mTOR inhibitors were used in three patients. Discussion: The present results were similar to those found in the literature regarding presentation features and kidney disease. The authors emphasized the importance of early diagnosis of renal involvement in order to reduce renal morbidity and mortality (especially when these patients reach adulthood) and highlighted the need for early guidance for pediatric nephrology consultation.

Abstract: Perinevic dermatosis neglecta is an underestimated skin condition usually affecting children and adolescents, characterized by the appearance of dirt-like brownish pigmentation around a preexisting nevus and often a matter of concern for parents. We describe the clinical and dermoscopic findings in a case of perinaevic dermatosis neglecta and discuss the possible etiology of this phenomenon.

Resumo Objetivo: Traduzir para o português brasileiro e validar o Questionário de Avaliação do Preparo para a Transição em uma população de adolescentes e adultos jovens com doenças reumáticas crônicas. Este questionário avalia o preparo do paciente para realizar a transição do serviço de saúde pediátrico para a assistência ao adulto. Métodos: Seguimos a metodologia de quatro etapas para a tradução e validação de questionários genéricos que inclui tradução, retrotradução, teste piloto e validação clínica do instrumento final. Utilizamos Análise Fatorial Confirmatória e Coeficiente Alfa de Cronbach para testar a validade do instrumento e sua consistência interna. Resultados: Responderam ao questionário traduzido e adaptado 150 pacientes. A média de idade foi de 17,0 anos (DP = 2,2 anos, variação 14-21 anos). Tinham o diagnóstico de lúpus eritematoso sistêmico juvenil 71 pacientes (47,3%), 64 (42,7%) artrite idiopática juvenil e 15 (10%) dermatomiosite juvenil. Durante a análise fatorial confirmatória, a dimensão "Falando com a Equipe Médica" contendo duas questões teve que ser removida devido à presença de expressivo efeito teto. Todas as outras questões restantes contribuíram favoravelmente para aumentar a consistência interna do questionário, obteve-se um Coeficiente Alfa de Cronbach de 0,776. Conclusões: O Questionário de Avaliação do Preparo para a Transição na sua versão em português brasileiro pode ser validado em uma população de pacientes com doenças reumáticas crônicas em transição, com a exclusão de uma dimensão do questionário original. Por ser um questionário não específico para doenças reumáticas, poderá ser utilizado para avaliar o preparo para a transição de outros pacientes brasileiros com doenças crônicas.

Abstract Objective: To translate and validate the Brazilian Portuguese version of the Transition Readiness Assessment Questionnaire in a population of adolescents and young adults with chronic rheumatologic disorders. This questionnaire evaluates the patient's readiness for making the transition from the pediatric health service to adult care. Methods: The four-phase methodology for the translation and validation of generic questionnaires was followed, including translation, back-translation, pilot testing and clinical validation of the final tool. The confirmatory factor analysis was used for clinical validation and the Cronbach's alpha coefficient was used to assess the overall internal consistency of the final tool. Results: A total of 150 patients with a mean age of 17.0 years (SD = 2.2 years, range 14-21 years) were enrolled for the final tool validation. Of those, 71 patients had juvenile systemic lupus erythematosus (47.3%), 64 had juvenile idiopathic arthritis (42.7%), and 15 had juvenile dermatomyositis (10%). During the confirmatory factor analysis, the dimension "Talking with providers" consisting of two questions, was considered as not fitting the translated questionnaire due to a very high ceiling effect and was therefore excluded. All other translated items favorably contributed to the overall consistency of the final tool; removing that dimension did not result in a substantial increase in Cronbach's alpha, which was 0.776. Conclusions: The Brazilian Portuguese version of the Transition Readiness Assessment Questionnaire was validated in a population of transitional patients with chronic rheumatologic disorders, after one dimension from the original questionnaire was excluded. It is a non-specific disease questionnaire; thus, it can be used to evaluate the transition readiness of Brazilian patients with other chronic diseases.

Abstract We report the case of an 8-year-old child with subungual exostosis, whose diagnosis was suspected on the basis of dermoscopic findings and subsequently confirmed by X-ray and histopathology.

Resumen Sostenemos que, siendo el acto de comer racional y relacional, también debe ser un tema educativo que tiene que ver con la sociedad y el ambiente, la política y la salud, los gustos y las tendencias, así como los factores genéticos y epigenéticos. Esta hipótesis tiene que ver con una cierta teoría del acto humano y una antropología basada en las especulaciones filosóficas de MacIntyre y Aristóteles. En este sentido, argumentamos que las opciones alimenticias son un “híbrido de libertad”, racionalidad, y elementos inconscientes y ambientales, y se relacionan con las dimensiones espirituales y biológicas de los seres humanos. Finalmente, sugerimos que debemos cambiar los hábitos humanos para transformar la forma humana de actuar, ya que cada acto humano puede cambiar la esencia humana y viceversa.

Abstract We argue that, given that the act of eating is rational and relational, it should also be an educational issue dealing with society and environment, politics and health, tastes and trends, as well as genetic and epigenetic factors. This hypothesis arises from a particular theory of the human act and an anthropological approach based on the philosophical speculations of MacIntyre and Aristotle. We argue that eating choices are “hybrids of freedom,” rationality, and unconscious and environmental elements. Finally, we suggest that people have to change their habits in order to transform the human way of acting, since every human act can change the human essence and vice versa.

Resumo Sostenemos que, siendo el acto de comer racional y relacional, también debe ser un tema educativo que tiene que ver con la sociedad y el ambiente, la política y la salud, los gustos y las tendencias, así como los factores genéticos y epigenéticos. Esta hipótesis tiene que ver con una cierta teoría del acto humano y una antropología basada en las especulaciones filosóficas de MacIntyre y Aristóteles. En este sentido, argumentamos que las opciones alimenticias son un “híbrido de libertad”, racionalidad, y elementos inconscientes y ambientales, y se relacionan con las dimensiones espirituales y biológicas de los seres humanos. Finalmente, sugerimos que debemos cambiar los hábitos humanos para transformar la forma humana de actuar, ya que cada acto humano puede cambiar la esencia humana y viceversa.

Resumo Objetivos: Avaliar a vasculite digital (VD) clínica como uma manifestação inicial do lúpus eritematoso sistêmico de início na infância (LESi) em uma grande população. Métodos: Estudo transversal multicêntrico que incluiu 852 pacientes com LESi (critérios do ACR), acompanhados em dez centros de reumatologia pediátrica do Estado de São Paulo. Resultados: Observou-se VD em 25/852 (3%) pacientes com LESi. Diagnosticaram-se hemorragia periungueal em 12 (48%), infarto periungueal em sete (28%), úlcera de ponta de dígito em quatro (16%), nódulos dolorosos em um (4%) e gangrena em um (4%). Um desfecho ruim, com reabsorção digital, ocorreu em cinco (20%) pacientes. A comparação entre pacientes com e sem VD revelou maior frequência de erupção malar (80% vs. 53%, p = 0,008), erupção discoide (16% vs. 4%, p = 0,017), fotossensibilidade (76% vs. 45% p = 0,002) e outras vasculites cutâneas (80% vs. 19%, p < 0,0001), enquanto a frequência de características constitucionais totais (32% vs. 61%, p = 0,003), febre (32% vs. 56% p = 0,020) e hepatomegalia (4% vs. 23%, p = 0,026) foram menores nesses pacientes. A frequência do gênero feminino, o envolvimento grave de múltiplos órgãos, perfil de autoanticorpos e baixo complemento foram semelhantes nos dois grupos (p > 0,05). A mediana no Sledai-2 K, exclusive o descritor de VD, foi significativamente menor nos pacientes com VD em comparação com aqueles sem essa manifestação [10 (0 a 28) vs. 14 (0 a 58), p = 0,004]. Não foram observadas vasculite visceral nem morte nessa coorte de pacientes com LESi. A frequência de uso de ciclofosfamida (0% vs. 18%, p = 0,014) foi significativamente menor no grupo VD. Conclusão: Este grande estudo multicêntrico identificou a VD clínica como uma rara manifestação inicial do LESi ativo, associada a doença multissistêmica leve, apesar da ocorrência de reabsorção digital em alguns desses pacientes.

Abstract Objectives: To assess clinical digital vasculitis (DV) as an initial manifestation of childhood-onset systemic lupus erythematosus (cSLE) within a large population. Methods: Multicenter cross-sectional study including 852 cSLE patients (ACR criteria) followed in ten Pediatric Rheumatology centers in São Paulo State, Brazil. Results: DV was observed in 25/852 (3%) cSLE patients. Periungual hemorrhage was diagnosed in 12 (48%), periungual infarction in 7 (28%), tip finger ulceration in 4 (16%), painful nodules in 1 (4%) and gangrene in 1 (4%). A poor outcome, with digital resorption, occurred in 5 (20%). Comparison of patients with and without DV revealed higher frequency of malar rash (80% vs. 53%, p = 0.008), discoid rash (16% vs. 4%, p = 0.017), photosensitivity (76% vs. 45%, p = 0.002) and other cutaneous vasculitides (80% vs. 19%, p < 0.0001), whereas the frequency of overall constitutional features (32% vs. 61%, p = 0.003), fever (32% vs. 56%, p = 0.020) and hepatomegaly (4% vs. 23%, p = 0.026) were lower in these patients. Frequency of female gender, severe multi-organ involvement, autoantibodies profile and low complement were alike in both groups (p > 0.05). SLEDAI-2K median, DV descriptor excluded, was significantly lower in patients with DV compared to those without this manifestation [10 (0-28) vs. 14 (0-58), p = 0.004]. Visceral vasculitis or death were not observed in this cSLE cohort. The frequency of cyclophosphamide use (0% vs. 18%, p = 0.014) was significantly lower in the DV group. Conclusion: Our large multicenter study identified clinical DV as one of the rare initial manifestation of active cSLE associated with a mild multisystemic disease, in spite of digital resorption in some of these patients.

Os linfangiomas quísticos pancreáticos são lesões benignas raras com origem em vasos linfáticos, correspondendo a menos de 0,2% da totalidade de quistos pancreáticos. Na maioria são assintomáticos sendo a sua descoberta incidental. Tradicionalmente o seu diagnóstico era cirúrgico, com completa ressecção de todo o tecido tumoral para prevenir recorrência. Actualmente, o desenvolvimento da ecoendoscopia (EUS) permitiu identificar estes quistos combinando as suas características ultrasonográficas, aparência macroscópica do fluido aspirado, e avaliação bioquímica e citológica da amostra. Os autores descrevem dois casos de linfangiomas quísticos pancreáticos diagnosticados por EUS, permitindo uma abordagem conservadora. Estes demonstram que os linfangiomas quísticos pancreáticos devem ser considerados no diagnóstico diferencial de lesões quísticas pancreáticas e que a EUS é importante no seu reconhecimento.

Pancreatic cystic lymphangiomas are rare benign lesions that arise from lymphatic vessels, accounting for less than 0.2% of all pancreatic cysts. Typically it is asymptomatic and discovery occurs during imaging exams for non-pancreatic disease. In the past, a definite diagnosis was made through surgery, with complete resection of all tumoral tissue to prevent recurrence. Nowadays, the development of endoscopic ultrasound (EUS) made it possible to identify these cysts combining morphologic ultrasound features, macroscopic aspirated fluid appearance, biochemical and cytological evaluation of the sample. We report two cases of cystic pancreatic lymphangioma diagnosed through EUS, allowing conservative management without surgery. These cases show that cystic pancreatic lymphangioma should be considered in the differential diagnosis of cystic pancreatic lesions and that EUS is an important tool for their recognition.

As perturbações de ansiedade são altamente prevalentes entre crianças em idade escolar. Estas perturbações tendem a seguir um curso crónico e interferem significativamente no funcionamento diário dos jovens. O objetivo principal deste estudo foi examinar as propriedades psicométricas da versão Portuguesa da Escala de Interferência da Ansiedade na Vida da Criança - versões para Pais e Crianças (CALIS-P e CALIS-C). Participaram 132 crianças, com idades entre os 7 e 12 anos, com diagnóstico principal de ansiedade, e seus respetivos pais. A análise fatorial da CALIS-P revelou três fatores que coincidem com as subescalas hipotetizadas: Interferência Em Casa, Fora de Casa e na Vida da Família. Para a CALIS-C, a análise fatorial revelou dois fatores que dizem respeito à Interferência nas Relações Próximas e no Desempenho, que não correspondem às subescalas da versão original. Os estudos psicométricos mostraram uma boa consistência interna para as várias subescalas da CALIS e boas qualidades na validade convergente e divergente. Foi observada ainda uma correlação significativa entre os resultados da CALIS e outra medida de interferência. Estes resultados sugerem que a versão portuguesa da CALIS é uma medida confiável e válida para a avaliação do impacto da ansiedade na criança e no funcionamento familiar.

Anxiety disorders are highly prevalent among school-aged children. These disorders become chronic in a substantial proportion of youths and cause significant interference with daily functioning. The purpose of the current investigation was to examine the psychometric properties of the Portuguese version of the Child Anxiety Life Interference Scale - Parent and Child versions (CALIS-P and CALIS-C). The sample consisted of 132 children between the ages of 7 and 12 with a main diagnosis of anxiety disorder and their parents. A factor analysis of the CALIS-P yielded three factors that agreed with the hypothesised subscales of At Home, Outside Home and Parent Life interference. For the CALIS-C, a factor analysis yielded two factors corresponding to Close Relationships and Performance interference that did not match the subscales of the original version. The internal consistency of the various CALIS subscales was good. Finally, evidence was found for both convergent and divergent validity. The CALIS scores were also significantly correlated with another measure of interference. The results provide initial support that the Portuguese version of the CALIS is a reliable and valid measure for the assessment of the impact of anxiety on child and family functioning.

El eje de la reflexión bioética es la formación personal del médico, centrada en un correcto planteamiento de la relación médico-paciente. De aquí que el punto crucial de la formación médica sea la relación entre conocimiento, comprensión, interpretación y comunicación. Si está correctamente planteada, permite superar el dualismo que contrapone la objetividad de los datos científicos a la subjetividad de las vivencias, tanto del médico como del paciente. Para crecer en esta actitud, donde están implicados, por un lado, el conocimiento profesional específico del médico, y por otro, su dimensión humana de compartir con otro y de compasión, se hace necesaria una honda formación cultural, donde las ciencias humanas, como la filosofía y la literatura, aporten aquel conocimiento del homo patiens indispensable para hacerse cargo de su cuidado.

The core axis of bioethical reflection is the personal molding of the physician as focused on the right approach of the doctor-patient relationship. That is why the crucial point of medical training is the relation between knowledge, understanding and communication. Where properly set out, it allows us to overcome the kind of dualism that counters scientific data objectivity with the subjectivity of life experiences of both the doctor and the patient. In order to grow in this disposition of mind in which on the one hand are involved the specific professional knowledge of the physicians and their human dimension of generosity and compassion on the other, a deep cultural education becomes necessary, where human sciences such as philosophy and literature contribute their knowledge of the homo patiens, which is undoubtedly indispensable to take care of the sick.

A terceira versão do Diagnostic and Statistic Manual of Mental Disorders (DSM III), publicado pela American Psychiatric Association em 1980, implicou uma mudança de paradigma no conhecimento psiquiátrico vigente, tanto nos Estados Unidos quanto no contexto internacional em geral. Apresentamos um pequeno histórico dessa transformação e procuramos expor e discutir a nova arquitetura do manual e sua lógica classificatória. Construímos nossa argumentação em torno do destino reservado às antigas “neuroses” e aos novos “transtornos sexuais”.

The third edition of the Diagnostic and Statistic Manual of Mental Disorders (DSM III), published by the American Psychiatric Association in 1980, brought about a paradigmatic change in psychiatric knowledge in the United States, as well as in other countries around the world. In this article we present a short history of this change, and discuss the new architecture of the manual and its logic of classification. Our argument focuses around the way the concepts of “neurosis” and “sexual disorders” were dealt with in the latest version of the manual.

La tercera versión del Diagnostic and Statistic Manual of Mental Disorders (DSM III), publicado por la American Psychiatric Association en 1980, implicó un cambio de paradigma en el conocimiento psiquiátrico vigente, tanto en los Estados Unidos como en el contexto internacional en general. Presentamos un pequeño histórico de esa transformación y buscamos exponer y discutir la nueva arquitectura del manual y su lógica clasificatoria. Construimos nuestra argumentación alrededor del destino reservado a las antiguas “neurosis” y a los nuevos “trastornos sexuales”.

La troisième version du Diagnostic and Statistic Manual of Mental Disorders (DSM III), publié par l’ American Psychiatric Association en 1980 a entraîné un changement de canon dans le savoir psychiatrique, non seulement aux États Unis, mais aussi dans les autres pays du monde. Nous présentons l’histoire résumeé de cette transformation, en analisant le changement de l’architecture et de la logique sous-jacente du manuel. Notre argumentation est centrée sur le nouveau statut des névroses et des “troubles sexuels”.