Bullous lesions following phototherapy in a newborn.

A male infant presented with progressive jaundice immediately after birth. Fecal acholia and choluria associated with extensive bullous skin lesions in his trunk, abdomen, and upper and lower limbs developed during phototherapy. Several diagnostic hypotheses were presented, including neonatal porphyria, hemochromatosis, Alagille syndrome, and neonatal lupus. A 24-hour urine sample for the dosage of urinary porphyrins was collected, showing high results (1823.6µg in 100mL). At 50 days of life, fluorescence spectroscopy using a Wood's lamp revealed simultaneous bright red fluorescence of urine-stained diapers and sample blood. A definitive diagnosis of congenital erythropoietic porphyria was made following identification of a mutation of the uroporphyrinogen synthetases III gene on genetic testing. The patient was subsequently maintained in a low light environment since then, resulting in improvement of the lesions. Congenital erythropoietic porphyria is a disease of the group of porphyrias that presents shortly after birth with blistering occurring in regions exposed to the sun or other ultraviolet light. Atrophic scars, mutilated fingers, and bright red fluorescence of the urine and teeth may also be observed. There is no specific treatment, and prophylaxis comprising a total avoidance of sunlight is generally recommended. A high degree of suspicion is required for diagnosis. An early diagnosis can lead to less damage. Here, we present the case of a newborn with congenital erythropoietic porphyria diagnosed after presenting with bullous lesions secondary to phototherapy.

Bullous lesions following phototherapy in a newborn

ABSTRACT A male infant presented with progressive jaundice immediately after birth. Fecal acholia and choluria associated with extensive bullous skin lesions in his trunk, abdomen, and upper and lower limbs developed during phototherapy. Several diagnostic hypotheses were presented, including neonatal porphyria, hemochromatosis, Alagille syndrome, and neonatal lupus. A 24-hour urine sample for the dosage of urinary porphyrins was collected, showing high results (1823.6µg in 100mL). At 50 days of life, fluorescence spectroscopy using a Wood's lamp revealed simultaneous bright red fluorescence of urine-stained diapers and sample blood. A definitive diagnosis of congenital erythropoietic porphyria was made following identification of a mutation of the uroporphyrinogen synthetases III gene on genetic testing. The patient was subsequently maintained in a low light environment since then, resulting in improvement of the lesions. Congenital erythropoietic porphyria is a disease of the group of porphyrias that presents shortly after birth with blistering occurring in regions exposed to the sun or other ultraviolet light. Atrophic scars, mutilated fingers, and bright red fluorescence of the urine and teeth may also be observed. There is no specific treatment, and prophylaxis comprising a total avoidance of sunlight is generally recommended. A high degree of suspicion is required for diagnosis. An early diagnosis can lead to less damage. Here, we present the case of a newborn with congenital erythropoietic porphyria diagnosed after presenting with bullous lesions secondary to phototherapy.

Authentic leadership among nursing professionals: knowledge and profile.

OBJECTIVES: to identify the knowledge of nursing professionals about leadership models and evaluate the authentic leadership profile among them. METHODS: analytical study, conducted between August and December 2015, involving 84 nursing professionals working in a public and tertiary hospital. We used two instruments: Sociodemographic Questionnaire with questions about leadership and the Authentic Leadership Questionnaire. RESULTS: both nurses and nursing technicians were unaware of authentic leadership. Both pointed to communication, planning, and organization as competencies of the leader (n = 58, 95%). Regarding the authentic leadership profile, we observed that the score was "high" among nurses and "low" among technicians. Holding a leadership position and professionally upgrading has positively influenced the highest-profile of authentic leadership. CONCLUSIONS: nurses demonstrated to know behavioral leadership, while nursing technicians showed knowledge about situational leadership. Nurses had a high score of authentic leadership behaviors, while nursing technicians had a low score, but we found no significant difference between them. Holding a leadership position and professionally upgrading has positively influenced the highest profile of authentic leadership.

Authentic leadership among nursing professionals: knowledge and profile / Liderazgo auténtico entre profesionales de enfermería: conocimiento y perfil / Liderança autêntica entre profissionais de enfermagem: conhecimento e perfil

ABSTRACT Objectives: to identify the knowledge of nursing professionals about leadership models and evaluate the authentic leadership profile among them. Methods: analytical study, conducted between August and December 2015, involving 84 nursing professionals working in a public and tertiary hospital. We used two instruments: Sociodemographic Questionnaire with questions about leadership and the Authentic Leadership Questionnaire. Results: both nurses and nursing technicians were unaware of authentic leadership. Both pointed to communication, planning, and organization as competencies of the leader (n = 58, 95%). Regarding the authentic leadership profile, we observed that the score was "high" among nurses and "low" among technicians. Holding a leadership position and professionally upgrading has positively influenced the highest-profile of authentic leadership. Conclusions: nurses demonstrated to know behavioral leadership, while nursing technicians showed knowledge about situational leadership. Nurses had a high score of authentic leadership behaviors, while nursing technicians had a low score, but we found no significant difference between them. Holding a leadership position and professionally upgrading has positively influenced the highest profile of authentic leadership.

RESUMEN Objetivos: identificar el conocimiento de los profesionales de enfermería sobre los modelos de liderazgo y evaluar el perfil de liderazgo auténtico entre ellos. Métodos: estudio analítico, realizado entre agosto y diciembre de 2015, que involucró 84 profesionales de enfermería actuantes en un hospital público y terciario. Se ha utilizado dos instrumentos: Cuestionario Sociodemográfico con cuestiones sobre liderazgo y el Authentic Leadership Questionnaire. Resultados: tanto enfermeros cuanto técnicos de enfermería desconocían el liderazgo auténtico. Ambos apuntaron la comunicación, el planeamiento y la organización como competencias del líder (n = 58, 95%). Referente al perfil de liderazgo auténtico, se observó que el escore ha sido "alto" entre enfermeros y "bajo" entre los técnicos. Ejercer cargo de liderazgo y actualizarse influenció positivamente el mayor perfil de liderazgo auténtico. Conclusiones: los enfermeros demostraron conocer el liderazgo del comportamiento, mientras los técnicos de enfermería mostraron conocimiento sobre el liderazgo situacional. Los enfermeros presentaron escore alto de comportamientos de liderazgo auténtico, a la vez que los técnicos de enfermería presentaron escore bajo, sin embargo, no se evidenció diferencia significante entre ellos. Ejercer cargo de liderazgo y actualizarse influenció positivamente el comportamiento de liderazgo auténtico.

RESUMO Objetivos: identificar o conhecimento dos profissionais de enfermagem sobre modelos de liderança e avaliar o perfil de liderança autêntica entre eles. Métodos: estudo analítico, realizado entre agosto e dezembro de 2015, que envolveu 84 profissionais de enfermagem atuantes em um hospital público e terciário. Utilizaram-se dois instrumentos: Questionário Sociodemográfico com questões sobre liderança e o Authentic Leadership Questionnaire. Resultados: tanto enfermeiros quanto técnicos de enfermagem desconheciam a liderança autêntica. Ambos apontaram a comunicação, o planejamento e a organização como competências do líder (n = 58, 95%). Referente ao perfil de liderança autêntica, observou-se que o escore foi "alto" entre enfermeiros e "baixo" entre os técnicos. Exercer cargo de liderança e atualizar-se influenciou positivamente o maior perfil de liderança autêntica. Conclusões: os enfermeiros demonstraram conhecer a liderança comportamental, enquanto os técnicos de enfermagem mostraram conhecimento sobre a liderança situacional. Os enfermeiros apresentaram escore alto de comportamentos de liderança autêntica, ao passo que os técnicos de enfermagem apresentaram escore baixo, contudo, não se evidenciou diferença significante entre eles. Exercer cargo de liderança e atualizar-se influenciou positivamente o comportamento de liderança autêntica.

Construction of an educational video on postoperative care for cheiloplasty and palatoplasty / Elaboración de un video educativo sobre los cuidados post-operatorio de las cirugías de queiloplastia y palatoplastia / Construção de um vídeo educativo sobre os cuidados pós-operatórios de queiloplastia e palatoplastia

ABSTRACT Objective: to describe the process for building up an educational video on the postoperative cares for primary cheiloplasty and palatoplasty surgeries. Method: a five-step technology elaborated development study (analysis and planning, modeling, implementation, evaluation and distribution) conducted in a public institution specialized in treating cleft lip and palate. Results: the evaluation was carried out by six judges regarding content criticism and criteria of familiarity, plausibility and linguistic clarity. Concordance percentage was 98%, which obtained approval and consent from most of the judges participating in the study. The feature adopted after the judges' analysis was qualified as a facilitator of the information needed to train caregivers' skills in the specific postoperative condition, and an additional in procedures related to basic health care in the hospital system. The video was completed with 11 minutes and 50 seconds. Conclusion: the educational video proved to be efficient in its constitution and applicability for preparing parents and other children caregivers who live with the need to learn about the postoperative care of cheiloplasty and palatoplasty surgeries.

RESUMEN Objetivo: describir el proceso para elaborar un video educativo sobre los cuidados post-operatorio de las cirugías primarias de queiloplastia y palatoplastia. Método: estudio de desarrollo de tecnología elaborado en cinco etapas (análisis y planificación, modelado, evaluación y distribución), realizado en una institución pública especializada en el tratamiento de fisuras labiopalatinas. Resultados: la evaluación fue llevada a cabo por seis jueces en lo referente a la crítica del contenido y de los criterios de familiaridad, viabilidad y claridad lingüística. El porcentaje de concordancia fue del 98%, valor que obtuvo la aprobación y concordancia de la mayoría de los jueces que participó del estudio. El recurso adoptado después del análisis de los jueces se calificó como facilitador de las informaciones necesarias para capacitar a los cuidadores en cuanto a las habilidades de la condición post-operatoria específica, y es un adicional en los procedimientos relacionados con la atención básica de la salud en el sistema hospitalario. La duración final del video fue de 11 minutos con 50 segundos. Conclusión: el video educativo demostró ser eficiente en su constitución y aplicabilidad para preparar a padres y demás cuidadores de niños que necesitan interiorizarse sobre los cuidados posoperatorios de las cirugías de queiloplastia y palatoplastia.

RESUMO Objetivo: descrever o processo de construção de um vídeo educativo sobre os cuidados pós-operatórios das cirurgias primárias de queiloplastia e palatoplastia. Método: estudo de desenvolvimento de tecnologia elaborado em cinco etapas (análise e planejamento, modelagem, implementação, avaliação e distribuição) e realizado em uma instituição pública especializada no tratamento de fissuras labiopalatinas. Resultados: a avaliação foi realizada por seis juízes, quanto à crítica de conteúdo e critérios de familiaridade, plausibilidade e clareza linguística. O percentual de concordância foi de 98%, o qual obteve aprovação e concordância da maioria dos juízes participantes do estudo. O recurso adotado após a análise dos juízes foi qualificado como facilitador das informações necessárias para o treino de habilidades de cuidadores na condição pós-operatória específica, e um adicional nos procedimentos relacionados à atenção básica de saúde no sistema hospitalar. O vídeo foi finalizado com 11 minutos e 50 segundos. Conclusão: o vídeo educativo mostrou-se eficiente na sua constituição e aplicabilidade para preparação de pais e demais cuidadores de crianças que vivenciam a necessidade de aprendizagem sobre os cuidados pós-operatórios de cirurgias de queiloplastia e palatoplastia.

Relapse after Le Fort I surgery in oral cleft patients: a 2-year follow-up using digitized and 3D models.

This retrospective observational study aimed to evaluate and identify the relapse rate after orthognathic surgery for maxillary advancement (Le Fort I maxillary osteotomy) in oral cleft patients through digitized cephalograms and 3D dental models, following 2 years. Lateral cephalograms and dental casts of 17 individuals, enrolled in Orthodontics Department in Hospital of Rehabilitation of Craniofacial Anomalies, were carried out. The digital cephalometric tracings were evaluated in: T1-before surgery, T2-immediate after surgery, T3-6-month to 1-year after surgery. The dental study casts were digitized and evaluated in: F1-before surgery; F2-3-month to 1-year after surgery; F3-1 to 2 years after surgery. The analyses of the dental arches were performed directly on the scanned images. A single examiner previously trained and calibrated performed all the assessments. Repeated measures ANOVA was applied to study the variables and compare the periods, followed by Tukey test to evaluate the statistically significant differences, with level of significance of 5%. The digital cephalogram results showed that the vertical movement statistically differed from T2 to T3 (p = 0.002). The right and left premolar relationship in digitized models revealed that at F2 the individuals exhibited » Class II and Class I, in 29.4 and 23.5% of the cases, respectively; and at F3, Class I, 58.8 and 70.6% of the cases, respectively. The cephalometry showed the relapse in the vertical movement after orthognathic surgery for maxillary advancement, but no relapse in the other evaluated parameters.

Vídeo educativo: estratégia de treinamento para cuidadores de crianças com fissura labiopalatina / Educational video: a training strategy for caregivers of children with cleft lip and palate

Resumo Objetivo Verificar o conhecimento adquirido por cuidadores informais de crianças com fissura labiopalatina sobre os cuidados pós-operatórios de queiloplastia e palatoplastia por meio da utilização de um vídeo educativo. Métodos Ensaio clínico randomizado, realizado com 80 cuidadores de crianças com fissura labiopalatina, dispostos em dois grupos: experimental (G1) e controle (G2). A coleta de dados ocorreu durante o período de hospitalização da criança, em três etapas, utilizando um questionário estruturado e um vídeo educativo construído e validado para o presente estudo. Resultados Foi evidenciada melhora na aquisição de conhecimento sobre os cuidados pós-operatórios em ambos os grupos (p<0,001), porém, foi maior o G1 em comparação a G2. Conclusão O vídeo educativo mostrou-se eficaz no treinamento de cuidadores de crianças com fissura labiopalatina em situação pós-operatória de queiloplastia e palatoplastia.

Abstract Objective To check the knowledge informal caregivers of children with cleft lip and palate acquire about the postoperative care of cheiloplasty and palatoplasty through the use of an educational video. Methods Randomized clinical trial conducted with 80 caregivers of children with cleft lip and palate, arranged into two groups: experimental (G1) and control (G2). Data collection occurred during the child’s hospital stay, in three stages, using a structured questionnaire and an educational video developed and validated for this study. Results There was evidence of improvement in the acquisition of knowledge about postoperative care in both groups (p <0.001), but it was higher in G1 compared to G2. Conclusion The educational video was shown to be effective to train caregivers of children with cleft lip and palate after cheiloplasty and palatoplasty.

Adesão ao tratamento de pacientes com fatores de risco cardiovascular em ambulatório da Zona Sul de São Paulo / Adherence to the treatment of patients with cardiovascular risk factorsat the ambulatory of South Zone of São Paulo

JUSTIFICATIVA E OBJETIVOS: As doenças cardiovascularessão a causa isolada de morte mais comum no mundo desenvolvidoe no Brasil. Desta forma, é necessário intervir em seusfatores de risco com uma modificação do estilo de vida e adesãoao tratamento. No entanto, estima-se que aproximadamente85% dos pacientes não são aderentes em algum momento desua doença. A proposta deste estudo consiste em identificar osprincipais motivos para essa não adesão nos pacientes do Ambulatóriode Reabilitação do Centro Universitário São Camilo.MÉTODOS: Foi realizado um estudo observacional através deum questionário que aborda a presença de fatores de risco cardiovasculare motivos desencadeantes para uma dificuldade naadesão ao tratamento. Participaram 100 indivíduos, sendo que44 homens e 56 mulheres, com idade média de 55,48 anos. Foinotada uma taxa de 82% de sedentarismo, 50% de hipertensos,22% de diabéticos, 31% de dislipidêmicos, 9% de fumantes,42% de obesos, 6% sem fator de risco e 12% com histórico dedoença cardiovascular. RESULTADOS: No final, 15% dos pacienteseram aderentes ao tratamento proposto, com uma maioraderência ao tratamento da hipertensão arterial e uma menor aprática de exercícios físicos, sendo a mudança de estilo de vida,a dificuldade mais citada. CONCLUSÕES: Este estudo reafirmaa baixa aderência dos pacientes ao tratamento das doençascardiovasculares, especialmente na mudança da rotina, de modo que o profissional da área da saúde tem papel fundamental noincremento da adesão, ao orientar corretamente o doente, tirandosuas dúvidas e estreitando a relação médico-paciente.(AU)

BACKGROUND AND OBJECTIVES: Cardiovascular diseasesare the most common single cause of death in the developedworld and in Brazil. In this way, it?s necessary a interventionon their risk factors with changes in life style and adherence totreatment. However it?s estimated that around 85% of patientsmay not adhere to it at some point in their disease. The purpose ofthis study is to identify the main reasons for this non-adherencein patients attending the Rehabilitation Ambulatory of CentroUniversitário São Camilo. METHODS: An observational studywas carried out through a questionnaire that addresses thepresence of cardiovascular risk factors and reasons for triggeringa difficulty in adherence to the treatment. 100 people attended,of which 44 males and 56 females with age of 55,48 years.A rate of 82% of sedentary was noted, 50% hypertensive,22% diabetic, 31% dyslipidemic, 9% smokers, 42% obese,6% without risk factor and 12% with cardiovascular disease.RESULTS: Overall, 15% of the patients were adhering to theproposed treatment, with a greater adherence to the treatmentof hypertension and a lower to the practice of physical exercises,and that the difficult to adhere to the treatment, the most citedwere the ones. CONCLUSIONS: This study reaffirms the lowadherence of patients to the cardiovascular diseases treatment mainly on regarding life style changes, thus the health careprofessional has a fundamental role in the increase of adherencein order to correctly guide the ill patient, clarifying their doubtsand building a doctor-patient relationship.(AU)

Nursing workload in specialized Semi-intensive Therapy unit: workforce size criteria / Carga de trabajo de enfermería en unidad de cuidados semi-intensivos especializada: criterios para dimensionamiento de personal / Carga de trabalho de enfermagem em Unidade de Terapia Semi-intensiva especializada: critérios para dimensionamento de pessoal

Abstract OBJECTIVE To assess the nursing workload (NW) in Semi-intensive Therapy Unit, specialized in the care of children with Craniofacial anomalies and associated syndromes; to compare the amount of workforce required according to the Nursing Activities Score (NAS) and the COFEN Resolution 293/04. METHOD Cross-sectional study, whose sample was composed of 72 patients. Nursing workload was assessed through retrospective application of the NAS. RESULTS the NAS mean was 49.5%. Nursing workload for the last day of hospitalization was lower in patients being discharged to home (p<0.001) and higher on the first compared to last day of hospitalization (p< 0.001). The number of professionals required according to NAS was superior to the COFEN Resolution 293/04, being 17 and 14, respectively. CONCLUSION the nursing workload corresponded to approximately 50% of the working time of nursing professional and was influenced by day and outcome of hospitalization. The amount of professionals was greater than that determined by the existing legislation.

Resumen OBJETIVO Evaluar la carga de trabajo de enfermería (CTE) en Unidad de Cuidados Semi-Intensivos, especializada en la atención a niños con anomalías craneofaciales y síndromes asociados; comparar el cuantitativo de personal requerido según el Nursing Activities Score (NAS) y la Resolución COFEN-293/04. MÉTODO Estudio transversal, cuya muestra fue de 72 pacientes. La carga de trabajo de enfermería fue evaluada por medio de la aplicación retrospectiva del NAS. RESULTADOS El promedio del NAS fue del 49,5%. La carga de trabajo de enfermería referente al último día de estancia hospitalaria fue menor en pacientes que recibieron alta para el domicilio (p<0,001) y mayor el primer día de la estancia en comparación con el último (p<0,001). El cuantitativo de profesionales requerido según el NAS fue superior al de la Resolución COFEN-293/04, siendo 17 y 14, respectivamente. CONCLUSIÓN La carga de trabajo de enfermería correspondió a un 50% del tiempo de trabajo del profesional de enfermería y fue influenciada por el día y resultado de la hospitalización. El cuantitativo de profesionales fue superior al determinado por la legislación existente.

Resumo OBJETIVO Avaliar a carga de trabalho de enfermagem (CTE) em Unidade de Terapia Semi-intensiva, especializada no atendimento de crianças com anomalias craniofaciais e síndromes associadas; comparar o quantitativo de pessoal requerido segundo o Nursing Activities Score (NAS) e a Resolução COFEN-293/04. MÉTODO Estudo transversal, cuja amostra foi de 72 pacientes. A carga de trabalho de enfermagem foi avaliada por meio da aplicação retrospectiva do NAS. RESULTADOS A média do NAS foi de 49,5%. A carga de trabalho de enfermagem referente ao último dia de internação foi menor em pacientes que receberam alta para o domicílio (p<0,001) e maior no primeiro dia de internação em comparação ao último (p<0,001). O quantitativo de profissionais requerido segundo o NAS foi superior ao da Resolução COFEN-293/04, sendo 17 e 14, respectivamente. CONCLUSÃO A carga de trabalho de enfermagem correspondeu a aproximadamente 50% do tempo de trabalho do profissional de enfermagem e foi influenciada pelo dia e desfecho da internação. O quantitativo de profissionais foi superior ao determinado pela legislação existente.

[Nursing workload in specialized Semi-intensive Therapy unit: workforce size criteria]. / Carga de trabalho de enfermagem em unidade de Terapia Semi-intensiva especializada: critérios para dimensionamento de pessoal.

OBJECTIVE: To assess the nursing workload (NW) in Semi-intensive Therapy Unit, specialized in the care of children with Craniofacial anomalies and associated syndromes; to compare the amount of workforce required according to the Nursing Activities Score (NAS) and the COFEN Resolution 293/04. METHOD: Cross-sectional study, whose sample was composed of 72 patients. Nursing workload was assessed through retrospective application of the NAS. RESULTS: the NAS mean was 49.5%. Nursing workload for the last day of hospitalization was lower in patients being discharged to home (p<0.001) and higher on the first compared to last day of hospitalization (p< 0.001). The number of professionals required according to NAS was superior to the COFEN Resolution 293/04, being 17 and 14, respectively. CONCLUSION: the nursing workload corresponded to approximately 50% of the working time of nursing professional and was influenced by day and outcome of hospitalization. The amount of professionals was greater than that determined by the existing legislation.

Increase in age is associated with worse outcomes in alveolar bone grafting in patients with bilateral complete cleft palate.

This prospective study aimed at evaluating the surgical outcomes of alveolar bone grafting (ABG) in subjects with bilateral cleft lip and palate treated at the Hospital for Rehabilitation of Craniofacial Anomalies, University of São Paulo, Bauru, Brazil, by means of cone-beam computed tomography. Twenty-five patients with bilateral complete cleft lip and palate, resulting in 50 clefts, were analyzed. Subjects were divided into 2 groups according to the dentition status at the time of surgery: (1) SABG group: subjects with mixed dentition operated on before or immediately after eruption of the permanent canine (10-13 years); (2) TABG group: subjects with permanent dentition (15-23 years). Cone-beam computed tomography analysis was performed in the buccal, intermediate, and palatal views, 2 and 6 to 12 months postoperatively. In the SABG group, 96% of the grafts were classified as successful, and no failure cases were observed. In the TABG group, successful cases decreased to 65%, and failures were seen in 27% of the cleft sites. In both postoperative periods, significantly better outcomes (lower mean scores) were observed for the SABG group in all the cone-beam computed tomography views (P < 0.05). Results show that the timing of surgery is an important factor in determining the outcomes of ABG in patients with bilateral cleft lip and palate, with increasing age being associated with the worse outcomes.