Método:trata-se de uma pesquisa de abordagem qualitativa, descritiva e exploratória. Os dados foram coletados por meio de entrevistas e a análise dos dados ocorreu por categorização temática. Resultados: observou-se que os profissionais apresentaram dificuldades no trabalho realizado junto à oncologia pediátrica devido a sua proximidade com sentimentos de dor, morte e sofrimento. Os profissionais manifestaram sentimentos variados em relação ao cuidar que foram do pesar ao não considerar diferenças em relação a outros pacientes pediátricos. Conclusão: existe a necessidade de apoio psicológico da instituição e de capacitação para melhor enfrentamento dos sentimentos que essa doença acarreta ao profissional e para a qualificação do cuidado junto às crianças e suas famílias. Descritores: Criança hospitalizada; Relações profissional-paciente; Pediatria; Oncologia. ABSTRACT: Aim: to analyze the experience of a nursing staff in the care of children with cancer in a Pediatric Unit of a University Hospital in southern Brazil. Method: it is a qualitative, descriptive and exploratory research. Data were collected through interviews and the analysis was carried out through thematic categorization. Results: it was observed that the professionals had difficulties in Pediatric Oncology work due to the approximation with feelings of pain, suffering and death. The professionals expressed various feelings in relation to the care, which ranged from sorrow to not considering differences in relation to other pediatric patients. Conclusion: it is necessary to provide the staff with psychological support and training programs so that they can better tackle with the feelings that the disease causes on professionals and to qualify the care of the children and their families. Descriptors: Child, hospitalized; Professional-patient relations; Pediatrics; Medical oncology. RESUMEN: Objetivo: analizar la vivencia del equipo de enfermería en el cuidado al niño con cáncer, en una unidad pediátrica de un hospital universitario del sur de Brasil. Metodo: estudio cualitativo, descriptivo y exploratorio. Los datos fueron recolectados por medio de entrevistas. El análisis de datos se realizó por categorización temática. Resultados: se observó que los profesionales presentaron dificultades en el trabajo junto

Background Morquio B disease (MBD) is a distinct GLB1‐related dysostosis multiplex presenting a mild phenocopy of GALNS‐related Morquio A disease. Previously reported cases from European countries carry the W273L variant on at least one GLB1 allele and exhibit a pure skeletal phenotype (pure MBD). Only a minority of MBD cases have been described with additional neuronopathic findings (MBD plus). Objectives and Methods With the aim to further describe patterns of MBD‐related dysostosis multiplex, we analyzed clinical, biochemical, and genetic features in 17 cases with GLB1‐related dysostosis multiplex living and diagnosed in Brazil. Results About 14 of the 17 individuals had three or more skeletal findings characteristic of Morquio syndrome. Two had no additional neuronopathic features (pure MBD) and 12 exhibited additional neuronopathic features (MBD plus). Three of the 17 cases had mild dysostosis without distinct features of MBD. Seven of the 12 MBD plus patients had signs of spinal cord compression (SCC), as a result of progressive spinal vertebral dysostosis. There was an age‐dependent increase in the number of skeletal findings and in the severity of growth impairment. GLB1 mutation analysis was completed in 10 of the 14 MBD patients. T500A occurred in compound heterozygosity in 8 of the 19 alleles. Conclusion Our study extends the phenotypic spectrum of GLB1‐related conditions by describing a cohort of patients with MBD and GM1‐gangliosidosis (MBD plus). Targeting the progressive nature of the skeletal manifestations in the development of new therapies for GLB1‐related conditions is warranted.