Transition From Pediatric to Adult Rheumatology Care: An Exploratory Study From Latin America.

BACKGROUND/PURPOSE: Adequate transition from pediatric to adult care is associated with better adherence to treatment and better outcomes in pediatric patients with chronic diseases. There are little data on transition programs, outcomes, use of transition guidelines, and available tools in pediatric rheumatology centers from Latin America (LATAM). In this study, we described the characteristics of transition programs from 3 pediatric rheumatology centers. We also introduced results of the first survey examining the transition experience in countries from LATAM. METHODS: The experience and implementation process of transition programs from 3 pediatric rheumatology centers were described. A survey based on a questionnaire created by Chira et al (J Rheumatol. 2014;41:768-779) from the Childhood Arthritis and Rheumatology Research Alliance was also administrated to pediatric rheumatology centers from LATAM. RESULTS: A total of 49 (68%) pediatric rheumatologists answered the survey. Most centers do not have an official and written transition program and reported a need for more tools and resources in their services to facilitate the transition experience. CONCLUSIONS: Transition guidelines culturally tailored to developing countries are needed in LATAM.

Most Fractures Treated Nonoperatively in Individuals With Fibrodysplasia Ossificans Progressiva Heal With a Paucity of Flareups, Heterotopic Ossification, and Loss of Mobility.

BACKGROUND: Fibrodysplasia ossificans progressiva (FOP) is an ultrarare genetic disorder with episodic and progressive heterotopic ossification. Tissue trauma is a major risk factor for flareups, heterotopic ossification (HO), and loss of mobility in patients with FOP. The International Clinical Council on FOP generally recommends avoiding surgery in patients with FOP unless the situation is life-threatening, because soft tissue injury can trigger an FOP flareup. Surprisingly little is known about flareups, HO formation, and loss of mobility after fractures of the normotopic (occurring in the normal place, distinct from heterotopic) skeleton when treated nonoperatively in patients with FOP. QUESTIONS/PURPOSES: (1) What proportion of fractures had radiographic evidence of union (defined as radiographic evidence of healing at 6 weeks) or nonunion (defined as the radiographic absence of a bridging callus at 3 years after the fracture)? (2) What proportion of patients had clinical symptoms of an FOP flareup because of the fracture (defined by increased pain or swelling at the fracture site within several days after closed immobilization)? (3) What proportion of patients with fractures had radiographic evidence of HO? (4) What proportion of patients lost movement after a fracture? METHODS: We retrospectively identified 36 patients with FOP from five continents who sustained 48 fractures of the normotopic skeleton from January 2001 to February 2021, who were treated nonoperatively, and who were followed for a minimum of 18 months after the fracture and for as long as 20 years, depending on when they sustained their fracture during the study period. Five patients (seven fractures) were excluded from the analysis to minimize cotreatment bias because these patients were enrolled in palovarotene clinical trials (NCT02190747 and NCT03312634) at the time of their fractures. Thus, we analyzed 31 patients (13 male, 18 female, median age 22 years, range 5 to 57 years) who sustained 41 fractures of the normotopic skeleton that were treated nonoperatively. Patients were analyzed at a median follow-up of 6 years (range 18 months to 20 years), and none was lost to follow-up. Clinical records for each patient were reviewed by the referring physician-author and the following data for each fracture were recorded: biological sex, ACVR1 gene pathogenic variant, age at the time of fracture, fracture mechanism, fracture location, initial treatment modality, prednisone use at the time of the fracture as indicated in the FOP Treatment Guidelines for flare prevention (2 mg/kg once daily for 4 days), patient-reported flareups (episodic inflammatory lesions of muscle and deep soft connective tissue characterized variably by swelling, escalating pain, stiffness, and immobility) after the fracture, follow-up radiographs of the fracture if available, HO formation (yes or no) as a result of the fracture determined at a minimum of 6 weeks after the fracture, and patient-reported loss of motion at least 6 months after and as long as 20 years after the fracture. Postfracture radiographs were available in 76% (31 of 41) of fractures in 25 patients and were independently reviewed by the referring physician-author and senior author for radiographic criteria of fracture healing and HO. RESULTS: Radiographic healing was noted in 97% (30 of 31) of fractures at 6 weeks after the incident fracture. Painless nonunion was noted in one patient who sustained a displaced patellar fracture and HO. In seven percent (three of 41) of fractures, patients reported increased pain or swelling at or near the fracture site within several days after fracture immobilization that likely indicated a site-specific FOP flareup. The same three patients reported a residual loss of motion 1 year after the fracture compared with their prefracture status. HO developed in 10% (three of 31) of the fractures for which follow-up radiographs were available. Patient-reported loss of motion occurred in 10% (four of 41) of fractures. Two of the four patients reported noticeable loss of motion and the other two patients reported that the joint was completely immobile (ankylosis). CONCLUSION: Most fractures treated nonoperatively in individuals with FOP healed with few flareups, little or no HO, and preservation of mobility, suggesting an uncoupling of fracture repair and HO, which are two inflammation-induced processes of endochondral ossification. These findings underscore the importance of considering nonoperative treatment for fractures in individuals with FOP. Physicians who treat fractures in patients with FOP should consult with a member of the International Clinical Council listed in the FOP Treatment Guidelines ( https://www.iccfop.org ). LEVEL OF EVIDENCE: Level IV, therapeutic study.

Experiencia del proceso de transición de adolescentes con enfermedades crónicas desde la atención pediátrica a la atención de adultos en un hospital general / Experience with the transition process of adolescents with chronic diseases from pediatric to adult care in a general hospital

Introducción. Es clave para la atención óptima de la salud la continuidad del cuidado al pasar de pediatría a la medicina del adulto. Objetivo. Describir la experiencia del proceso de transición de pacientes adolescentes conenfermedades crónicas desde la atención enpediatría a la atención de adultos en un hospital general. Población y métodos. Estudio de cortetransversal de pacientes entre 16 y 24 años con antecedente de trasplante hepático, trasplante renal, enfermedades endocrinas, metabólicas, reumatológicas y mielomeningocele atendidos en un hospital general universitario de tercer nivel entre 2015 y 2019, durante el proceso de transición. Se evaluaron el proceso de atención y el éxito de la transición. Se utilizó el cuestionario de evaluación de preparación para la transición (Transition Readiness Assessment QuestionnaireTRAQ, por su sigla en inglés). Resultados. Se incluyeron 372 pacientes. Las especialidades de atención más frecuentesfueron clínica de mielomeningocele, equipo de trasplante renal y de trasplante hepático. El 37 % participó del proceso de transición. La media de seguimiento por pediatría hasta el inicio de la transición fue de 9 años. La media de edad de comienzo de la transición fue 19 años y la media de edad de finalización, 21 años. La estrategia de transición más frecuente fue clínica conjunta en el 96 %. La mediana del TRAQ ordinal fue de 4; de estos, el 32 % ya había consultado a adultos. El 32,7 % cumplió con una transición exitosa. Conclusiones. La continuidad del cuidadodurante la transición es un proceso que llevó casi dos años y en más de un tercio de los pacientes se realizó en forma exitosa.

Introduction. The continuity of care from pediatrics to adult medicine is key to optimal health care. Objective. To describe the experience of the transition process of adolescent patients with chronic diseases from pediatric to adult care in a general hospital. Population and methods. Cross-sectional study of patients aged 16­24 years with a history of liver transplantation, kidney transplantation, endocrine, metabolic, rheumatic diseases, and myelomeningocele seen at a tertiary care teaching general hospital between 2015 and 2019 during the transition process. The process of health care and transition success were assessed. The Transition Readiness Assessment Questionnaire (TRAQ) was used. Results. A total of 372 patients were included. The myelomeningocele clinic, the kidney transplant and the liver transplant teams were the most common specialties. Thirty-seven percent of participants were involved in the transition process. The mean duration of follow-up by pediatrics until transition initiation was 9 years. The mean age at the beginning of transition was 19 years, and the mean age at the end, 21 years. The joint clinic transition strategy was the most frequent, used in 96% of cases. The median value of the ordinal TRAQ was 4; of these, 32% had already seen adult care physicians. A successful transition was achieved by 32.7%. Conclusions. The continuity of care during transition is a process that took almost 2 years; more than one third of the patients had a successful transition.

Experience with the transition process of adolescents with chronic diseases from pediatric to adult care in a general hospital. / Experiencia del proceso de transición de adolescentes con enfermedades crónicas desde la atención pediátrica a la atención de adultos en un hospital general.

INTRODUCTION: The continuity of care from pediatrics to adult medicine is key to optimal health care. OBJECTIVE: To describe the experience of the transition process of adolescent patients with chronic diseases from pediatric to adult care in a general hospital. POPULATION AND METHODS: Cross-sectional study of patients aged 16-24 years with a history of liver transplantation, kidney transplantation, endocrine, metabolic, rheumatic diseases, and myelomeningocele seen at a tertiary care teaching general hospital between 2015 and 2019 during the transition process. The process of health care and transition success were assessed. The Transition Readiness Assessment Questionnaire (TRAQ) was used. RESULTS: A total of 372 patients were included. The myelomeningocele clinic, the kidney transplant and the liver transplant teams were the most common specialties. Thirty-seven percent of participants were involved in the transition process. The mean duration of follow-up by pediatrics until transition initiation was 9 years. The mean age at the beginning of transition was 19 years, and the mean age at the end, 21 years. The joint clinic transition strategy was the most frequent, used in 96% of cases. The median value of the ordinal TRAQ was 4; of these, 32% had already seen adult care physicians. A successful transition was achieved by 32.7%. CONCLUSIONS: The continuity of care during transition is a process that took almost 2 years; more than one third of the patients had a successful transition.

Introducción. Es clave para la atención óptima de la salud la continuidad del cuidado al pasar de pediatría a la medicina del adulto. OBJETIVO: Describir la experiencia del proceso de transición de pacientes adolescentes con enfermedades crónicas desde la atención en pediatría a la atención de adultos en un hospital general. Población y métodos. Estudio de corte transversal de pacientes entre 16 y 24 años con antecedente de trasplante hepático, trasplante renal, enfermedades endocrinas, metabólicas, reumatológicas y mielomeningocele atendidos en un hospital general universitario de tercer nivel entre 2015 y 2019, durante el proceso de transición. Se evaluaron el proceso de atención y el éxito de la transición. Se utilizó el cuestionario de evaluación de preparación para la transición (Transition Readiness Assessment Questionnaire, TRAQ, por su sigla en inglés). RESULTADOS: Se incluyeron 372 pacientes. Las especialidades de atención más frecuentes fueron clínica de mielomeningocele, equipo de trasplante renal y de trasplante hepático. El 37 % participó del proceso de transición. La media de seguimiento por pediatría hasta el inicio de la transición fue de 9 años. La media de edad de comienzo de la transición fue 19 años y la media de edad de finalización, 21 años. La estrategia de transición más frecuente fue clínica conjunta en el 96 %. La mediana del TRAQ ordinal fue de 4; de estos, el 32 % ya había consultado a adultos. El 32,7 % cumplió con una transición exitosa. CONCLUSIONES: La continuidad del cuidado durante la transición es un proceso que llevó casi dos años y en más de un tercio de los pacientes se realizó en forma exitosa.

Gene Therapy for Fibrodysplasia Ossificans Progressiva: Feasibility and Obstacles.

Fibrodysplasia ossificans progressiva (FOP) is a rare and devastating genetic disease, in which soft connective tissue is converted into heterotopic bone through an endochondral ossification process. Patients succumb early as they gradually become trapped in a second skeleton of heterotopic bone. Although the underlying genetic defect is long known, the inherent complexity of the disease has hindered the discovery of effective preventions and treatments. New developments in the gene therapy field have motivated its consideration as an attractive therapeutic option for FOP. However, the immune system's role in FOP activation and the as-yet unknown primary causative cell, are crucial issues which must be taken into account in the therapy design. While gene therapy offers a potential therapeutic solution, more knowledge about FOP is needed to enable its optimal and safe application.

Relatos sobre COVID-19 de profesionales de la salud en un hospital / Stories about covid-19 from health professionals in a hospital

Introducción: la pandemia de COVID-19 impone a los profesionales de la salud: altas exigencias y modificaciones en el modo de vincularse con pacientes, pares y familiares. Estos cambios implican consecuencias emocionales tales como el incremento del nivel de estrés y síntomas de ansiedad y de depresión. Objetivo: describir un proyecto interdisciplinario creado bajo el modelo de la Medicina Narrativa dirigido a habilitar el relato de la experiencia de profesionales de la salud pertenecientes a un hospital general privado de alta complejidad de la ciudad de Buenos Aires durante la pandemia de COVID-19. Metodología: se creó una lista de correo y se convocó a profesionales del hospital a realizar una producción escrita, oral o gráfica que represente su experiencia durante la pandemia. Luego, el material recibido se envió a la misma lista de distribución con frecuencia semanal. En cada correo se renovó la invitación a narrar lo vivido o comentar los relatos de otros. Resultados: en el transcurso de siete semanas se recibieron diez producciones individuales: ocho textos, un audio y un gráfico. Los principales temas tratados pudieron agruparse en tres ejes: sala COVID, comunidad y telemedicina. Los autores fueron profesionales de Medicina, Enfermería, Psicología y de Puericultura. Conclusión: desarrollamos un proyecto bajo el modelo de la Medicina Narrativa que permitió a profesionales narrar su experiencia durante la pandemia de COVID-19, habilitando la posibilidad de poner en palabras lo vivido, reflexionar sobre modelos de actuación y elaborar el desgaste emocional generado por el contacto permanente con el dolor y el sufrimiento. (AU)

Introduction: the COVID-19 pandemic imposes high demands on health professionals and changes in the way they relate to patients, peers and family members. These changes involve emotional consequences such as increased stress levels, symptoms of anxiety and depression. Objective: to describe an interdisciplinary project created under the Narrative Medicine model aimed at enabling the reporting of the experience of health professionals belonging to a highly complex private general hospital in the city of Buenos Aires during the COVID-19 pandemic. Methodology: a mailing list was created and hospital professionals were invited to make a written, oral or graphic production that represents their experience during the pandemic. Then, the received material was sent to the same distribution list on a weekly basis. In each email, the invitation to narrate what was experienced or to comment on the stories of others was renewed. Results: over the course of seven weeks, ten individual productions were received: eight texts, one audio and one graphic. The main topics discussed could be grouped into three axes: COVID room, community and telemedicine. The authors were professionals from Medicine, Nursing, Psychology and Childcare. Conclusion: we developed a project under the Narrative Medicine model that allowed professionals to tell their experience during the covid 19 pandemic, enabling the possibility of putting into words what was experienced, reflecting on models of action and elaborating on the emotional exhaustion generated by permanent contact with pain and suffering. (AU)

Spatial patterns of heterotopic ossification in fibrodysplasia ossificans progressiva correlate with anatomic temperature gradients.

Progressive heterotopic ossification (HO) is a hallmark of fibrodysplasia ossificans progressiva (FOP); however, this tissue transformation is not random. Rather, we noticed that HO in FOP progresses in well-defined but inexplicable spatial and temporal patterns that correlate precisely with infrared thermographs of the human body. FOP is caused by gain-of-function mutations in Activin A receptor type I (ACVR1/ALK2), a bone morphogenetic protein (BMP) type I receptor kinase. As with all enzymes, the activity of ACVR1 is temperature-dependent. We hypothesized that connective tissue progenitor cells that express the common heterozygous ACVR1R206H mutation (FOP CTPCs) exhibit a dysregulated temperature response compared to control CTPCs and that the temperature of FOP CTPCs that initiate and sustain HO at various anatomic sites determines, in part, the anatomic distribution of HO in FOP. We compared BMP pathway signaling at a range of physiologic temperatures in primary CTPCs isolated from FOP patients (n = 3) and unaffected controls (n = 3) and found that BMP pathway signaling and resultant chondrogenesis were amplified in FOP CTPCs compared to control CTPCs (p < 0.05). We conclude that the anatomic distribution of HO in FOP may be due, in part, to a dyregulated temperature response in FOP CTPCs that reflect anatomic location. While the association of temperature gradients with spatial patterns of HO in FOP does not demonstrate causality, our findings provide a paradigm for the physiologic basis of the anatomic distribution of HO in FOP and unveil a novel therapeutic target that might be exploited for this disabling condition.

Calidad de vida de los hermanos de pacientes con enfermedad crónica / Quality of life among siblings of patients with chronic conditions

Introducción. La calidad de vida relacionada con la salud (CVRS) es una medida de resultado de salud. Evalúa el impacto subjetivo y global de las enfermedades en la vida cotidiana. Brinda información multidimensional sobre el bienestar físico, relación familiar y sus pares. Los estudios de CVRS de hermanos son limitados.Objetivo. Comparar CVRS de los hermanos de pacientes pediátricos con patologías reumáticas crónicas, trasplante renal o hepático con la de niños sanos con hermanos sin enfermedades crónicas.Resultados. Se compararon hermanos de niños con trasplante renal (n: 65), trasplante hepático (n: 35) y patologías reumáticas crónicas (n: 36) con el grupo control de niños sanos (n: 51). El grupo total de hermanos tuvieron puntuación más baja, estadísticamente significativa, en las dimensiones bienestar físico, amigos-apoyo social y recursos económicos. Los hermanos de trasplante renal tuvieron baja puntuación en las dimensiones de bienestar físico (p < 0,02; tamaño del efecto ­TE­: 0,66) y recursos económicos (p < 0,01; TE: 0,66). Los hermanos de trasplante hepático percibieron menor bienestar físico (p = 0,04), tenían menos amigos y apoyo social (p < 0,01), dificultades en el entorno escolar (p < 0,02) y recursos económicos (p < 0,01). Los hermanos de patologías reumáticas crónicas tuvieron menor bienestar físico (p < 0,05; TE: 0,44) y apoyo social-amigos (p < 0,01; TE: 0,58).Conclusión. La CVRS de niños/as sanos de hermanos con patologías crónicas es menor en bienestar físico, amigos-apoyo social y recursos económicos comparada con el grupo de niños sanos.

Introduction. Health-related quality of life (HRQoL) is a measure of health outcomes. It assesses the subjective and overall impact of diseases on daily life. It also provides multidimensional data about physical well-being, family and peers relations. HRQoL studies on siblings are limited.Objective. To compare HRQoL among siblings of pediatric patients with chronic rheumatic diseases, kidney or liver transplant and healthy children whose siblings had no chronic conditions.Results. The siblings of children with kidney transplant (n: 65), liver transplant (n: 35), and chronic rheumatic diseases (n: 36) were compared to the healthy children group (n: 51). The total siblings group had a lower, statistically significant score in the physical well-being, social support and peers, and financial resources dimensions. The siblings of kidney transplant patients had a low score in the physical well-being (p < 0.02; effect size [ES]: 0.66) and financial resources (p < 0.01; ES: 0.66) dimensions. The siblings of liver transplant patients perceived a lower physical well-being (p = 0.04), less social support and peers(p < 0.01), and difficulties in relation to school environment (p < 0.02) and financial resources (p < 0.01). The siblings of those with chronic rheumatic diseases had a lower score in the physical well-being (p < 0.05; ES: 0.44) and social support and peers (p < 0.01; ES: 0.58) dimensions.Conclusion. HRQoL among healthy children whose siblings have a chronic disease was lower in the physical well-being, social support and peers, and financial resources dimensions compared to the healthy children group.

Aproximación al currículum oculto a través de la medicina narrativa en la formación de estudiantes de Medicina / Approach to the hidden curriculum through narrative medicine in the training of medical students

Introducción. Currículum oculto son aquellos contenidos aprendidos que no pertenecen al currículum formal. La medicina narrativa permite conocer las experiencias de profesionales de la salud. Aproximarse al currículum oculto permitiría modificar o reforzar instancias de aprendizaje no formal.Objetivo. Analizar producciones narrativas de estudiantes de Medicina para identificar contenidos del currículum oculto.Población y métodos. Estudio de corte transversal con estudiantes de la materia Pediatría durante 2017.Resultados. Los 14 estudiantes de una cursada realizaron 50 narraciones. El 53 % correspondieron al contexto internación; el 17 %, a atención ambulatoria; el 17 %, a la central de emergencias, y el 13 %, a neonatología. Los principales contenidos fueron comunicación médico-paciente, inclusión de la familia en la consulta pediátrica, empatía, enfermedad crónica en los niños y comunicación de malas noticias.Conclusiones. Las producciones narrativas permitieron aproximarse al currículum oculto a través de reflexiones sobre el accionar profesion

Introduction. Hidden curricula are those contents learned that do not belong to the formal curriculum. Narrative medicine allows knowledge of health professionals ́ experiences. Approaching to the hidden curriculum can allow modifying or reinforcing instances of the non-formal learning.Objective. Analyze narrative medical students ́ productions to identify hidden curriculum contents.Population and methods. A cross-sectional study was conducted with Pediatric students during 2017.Results. All the students of the course (14) participated and performed 50 narrative productions: 53 % corresponded to the hospitalization context, 17 % to ambulatory care, 17 % to the emergency center and 13 % to neonatology.The main topics described were doctor-patient communication, importance of family inclusio in the pediatric consultation, empathy, impact of chronic disease in children and communication of bad news.Conclusions. Narrative productions allowed an approximation to the hidden curriculum through reflections on professional action

Quality of life among siblings of patients with chronic conditions. / Calidad de vida de los hermanos de pacientes con enfermedad crónica.

INTRODUCTION: Health-related quality of life (HRQoL) is a measure of health outcomes. It assesses the subjective and overall impact of diseases on daily life. It also provides multidimensional data about physical wellbeing, family and peers relations. HRQoL studies on siblings are limited. OBJECTIVE: To compare HRQoL among siblings of pediatric patients with chronic rheumatic diseases, kidney or liver transplant and healthy children whose siblings had no chronic conditions. RESULTS: The siblings of children with kidney transplant (n: 65), liver transplant (n: 35), and chronic rheumatic diseases (n: 36) were compared to the healthy children group (n: 51). The total siblings group had a lower, statistically significant score in the physical well-being, social support and peers, and financial resources dimensions. The siblings of kidney transplant patients had a low score in the physical wellbeing (p < 0.02; effect size [ES]: 0.66) andfinancial resources (p < 0.01; ES: 0.66) dimensions. The siblings of liver transplant patients perceived a lower physical well-being (p = 0.04), less social support and peers (p < 0.01), and difficulties in relation to school environment (p < 0.02) and financial resources (p <0.01). The siblings of those with chronic rheumatic diseases had a lower score in the physical well-being (p < 0.05; ES: 0.44) and social support and peers (p <0.01; ES: 0.58) dimensions. CONCLUSION: HRQoL among healthy children whose siblings have a chronic disease was lower in the physical well-being, social support and peers, and financial resources dimensions compared to the healthy children group.

Introducción. La calidad de vida relacionada con la salud (CVRS) es una medida de resultado de salud. Evalúa el impacto subjetivo y global de las enfermedades en la vida cotidiana. Brinda información multidimensional sobre el bienestar físico, relación familiar y sus pares. Los estudios de CVRS de hermanos son limitados. Objetivo. Comparar CVRS de los hermanos de pacientes pediátricos con patologías reumáticas crónicas, trasplante renal o hepático con la de niños sanos con hermanos sin enfermedades crónicas. Resultados. Se compararon hermanos de niños con trasplante renal (n: 65), trasplante hepático (n: 35) y patologías reumáticas crónicas (n: 36) con el grupo control de niños sanos (n: 51). El grupo total de hermanos tuvieron puntuación más baj a, estadísticamente significativa, enlas dimensiones bienestar físico, amigos-apoyo social y recursos económicos. Los hermanos de trasplante renal tuvieron baja puntuación en las dimensiones de bienestar físico (p < 0,02; tamaño del efecto -TE-: 0,66) y recursos económicos (p < 0,01; TE: 0,66). Los hermanos de trasplante hepático percibieron menor bienestar físico (p = 0,04), tenían menos amigos y apoyo social (p < 0,01), dificultades en el entorno escolar (p < 0,02) y recursos económicos (p < 0,01). Los hermanos de patologías reumáticas crónicas tuvieron menor bienestar físico (p < 0,05; TE: 0,44) y apoyo social-amigos (p < 0,01; TE: 0,58). Conclusión. La CVRS de niños/as sanos de hermanos con patologías crónicas es menor en bienestar físico, amigos-apoyo social y recursos económicos comparada con el grupo de niños sanos.

[Approach to the hidden curriculum through narrative medicine in the training of medical students]. / Aproximación al currículum oculto a través de la medicina narrativa en la formación de estudiantes de Medicina.

INTRODUCTION: Hidden curricula are those contents learned that do not belong to the formal curriculum. Narrative medicine allows knowledge of health professionals' experiences. Approaching to the hidden curriculum can allow modifying or reinforcing instances of the nonformal learning. OBJECTIVE: Analyze narrative medical students' productions to identify hidden curriculum contents. POPULATION AND METHODS: A cross-sectional study was conducted with Pediatric students during 2017. RESULTS: All the students of the course (14) participated and performed 50 narrative productions: 53 % corresponded to the hospitalization context, 17 % to ambulatory care, 17 % to the emergency center and 13 % to neonatology. The main topics described were doctor-patient communication, importance of family inclusion in the pediatric consultation, empathy, impact of chronic disease in children and communication of bad news. CONCLUSIONS: Narrative productions allowed an approximation to the hidden curriculum through reflections on professional actions.

Introducción. Currículum oculto son aquellos contenidos aprendidos que no pertenecen al currículum formal. La medicina narrativa permite conocer las experiencias de profesionales de la salud. Aproximarse al currículum oculto permitiría modificar o reforzar instancias de aprendizaje no formal. Objetivo. Analizar producciones narrativas de estudiantes de Medicina para identificar contenidos del currículum oculto. Población y métodos. Estudio de corte transversal con estudiantes de la materia Pediatría durante 2017. Resultados. Los 14 estudiantes de una cursada realizaron 50 narraciones. El 53 % correspondieron al contexto internación; el 17%, a atención ambulatoria; el 17 %, a la central de emergencias, y el 13 %, a neonatología. Los principales contenidos fueron comunicación médico-paciente, inclusión de la familia en la consulta pediátrica, empatia, enfermedad crónica en los niños y comunicación de malas noticias. Conclusiones. Las producciones narrativas permitieron aproximarse al currículum oculto a través de reflexiones sobre el accionar profesional.

International physician survey on management of FOP: a modified Delphi study.

Fibrodysplasia ossificans progressiva (FOP), a disabling disorder of progressive heterotopic ossification (HEO), is caused by heterozygous gain-of- function mutations in Activin receptor A, type I (ACVR1, also known as ALK2), a bone morphogenetic protein (BMP) type I receptor. Presently, symptomatic management is possible, but no definitive treatments are available. Although extensive guidelines for symptomatic management are widely used, regional preferences exist. In order to understand if there was worldwide consensus among clinicians treating FOP patients, an expert panel of physicians directly involved in FOP patient care was convened. Using a modified Delphi method, broad international consensus was reached on four main topics: diagnosis, prevention of flare-ups, patient and family-centered care and general clinical management issues. This study of physician preferences provides a basis for standardization of clinical management for FOP.

Adaptación transcultural del cuestionario de transición a la atención médica del adulto al castellano argentino / Cross-cultural adaptation of the Transition Readiness Assessment Questionnaire to Argentinian Spanish

En Argentina, no había un instrumento adaptado y validado de preparación para la transición. El objetivo fue describir el proceso de adaptación transcultural del Transition Readiness Assessment Questionnaire 5.0 al español argentino. Para ello, se contactó a los autores del cuestionario. Etapa 1: dos traductores lo tradujeron al castellano. Etapa 2: a partir de esas versiones, se consensuó la versión 1. Etapa 3: se realizaron dos retrotraducciones. Etapas 4 y 5: se compararon ambas versiones en inglés y se adaptó la versión en castellano para asegurar la equivalencia con la original. Se agregaron los subtítulos de cada dominio y se obtuvo la versión 2. Etapa 6: se realizó una prueba de campo para determinar si el vocabulario era culturalmente adecuado. Se obtuvo la versión 3. En la última etapa (etapa 7), se probó el cuestionario en dos hospitales. Finalmente, se obtuvo la traducción y adaptación del Transition Readiness Assessment Questionnaire 5.0 para la población argentina.

In Argentina, there was not an adapted and validated instrument to evaluate readiness for the transition to adult health care. The purpose of this study was to describe the process of cross-cultural adaptation of the Transition Readiness Assessment Questionnaire 5.0 to Argentinian Spanish. The authors of the instrument were contacted to this effect. Stage 1: two translators performed the translation to Spanish. Stage 2: based on these two versions, version 1 was agreed upon. Stage 3: two back-translations were performed. Stages 4 and 5: both back-translated versions were compared, and the Spanish version was adapted to ensure correspondence with the original. Subheads were added in each domain, and version2 was obtained. Stage 6: the questionnaire was field tested to ensure the cultural adequacy of the vocabulary, thus obtaining version 3. In the last stage (stage 7), the instrument was tested in two hospitals. Finally, the cross-cultural adaptation of the Transition Readiness Assessment Questionnaire 5.0 for the Argentinian population was obtained.

Cross-cultural adaptation of the Transition Readiness Assessment Questionnaire to Argentinian Spanish. / Adaptación transcultural del cuestionario de transición a la atención médica del adulto al castellano argentino.

In Argentina, there was not an adapted and validated instrument to evaluate readiness for the transition to adult health care. The purpose of this study was to describe the process of cross-cultural adaptation of the Transition Readiness Assessment Questionnaire 5.0 to Argentinian Spanish. The authors of the instrument were contacted to this effect. Stage 1: two translators performed the translation to Spanish. Stage 2: based on these two versions, version 1 was agreed upon. Stage 3: two back-translations were performed. Stages 4 and 5: both back-translated versions were compared, and the Spanish version was adapted to ensure correspondence with the original. Subheads were added in each domain, and version 2 was obtained. Stage 6: the questionnaire was field tested to ensure the cultural adequacy of the vocabulary, thus obtaining version 3. In the last stage (stage 7), the instrument was tested in two hospitals. Finally, the cross-cultural adaptation of the Transition Readiness Assessment Questionnaire 5.0 for the Argentinian population was obtained.

En Argentina, no había un instrumento adaptado y validado de preparación para la transición. El objetivo fue describir el proceso de adaptación transcultural del Transition Readiness Assessment Questionnaire 5.0 al español argentino. Para ello, se contactó a los autores del cuestionario. Etapa 1: dos traductores lo tradujeron al castellano. Etapa 2: a partir de esas versiones, se consensuó la versión 1. Etapa 3: se realizaron dos retrotraducciones. Etapas 4 y 5: se compararon ambas versiones en inglés y se adaptó la versión en castellano para asegurar la equivalencia con la original. Se agregaron los subtítulos de cada dominio y se obtuvo la versión 2. Etapa 6: se realizó una prueba de campo para determinar si el vocabulario era culturalmente adecuado. Se obtuvo la versión 3. En la última etapa (etapa 7), se probó el cuestionario en dos hospitales. Finalmente, se obtuvo la traducción y adaptación del Transition Readiness Assessment Questionnaire 5.0 para la población argentina.

[Transition in health care, from pediatrics to adult care]. / Transición en la atención médica, de la pediatría a la medicina del adulto.

The number of adolescent patients with chronic diseases and special heath needs are increasing, and they are reaching adulthood. Sometimes the passage to the adult health care is abrupt, depending upon the chronological age reached or because of an acute health problem that requires hospitalization. In order to facilitate the transition process, preparation of the child, the family and the health professionals involved is needed, as well as the coordination between the pediatric group and the adult team that will be incharge of the patient. This review shows the obstacles to this process and the recommended implementation steps required to a successful transition. It also describes the main aspects of a program that we implemented at the Hospital Italiano de Buenos Aires in conjunction with some departments of adult health care high lighting the main steps to follow before and during program´s implementation.

Estimación de la prevalencia de dolormusculoesquelético en niños atendidosen un hospital general de comunidad / Estimated prevalence of musculoskeletal pain in children, who attend a community hospital

El dolor musculoesquelético esfrecuente en la infancia. La prevalencia se estima entre 5 y 19 por ciento en niños sanos.Objetivos. Estimar la prevalencia de dolor musculoesquelético y describir las características epidemiológicasen niños de 3 a 12 años.Población, material y métodos. Estudio observacional de corte transversal. Se realizó encuesta autoadministrada en la sala de espera. Se excluyeron niños con enfermedad crónica.Resultados. Se entregaron 240 encuestas y se completaron 229. La mediana de edad de los niños fue 6 (3-12) años y de las madres 36 (25-50)años. El 54 por ciento eran niñas. El 21, 4 por ciento y el 19,2 por ciento refirieron dolor en el último año y en los últimos 3 meses, respectivamente. Los factores asociados con dolor en el último año fueron: edad y antecedentede dolor en un familiar durante la infancia y actualmente. No se encontraron diferencias con el sexo del paciente, edad materna, familiamonoparental, tener hermanos ni indicadores de estrés familiar en el último año.En 66 por ciento el dolor fue localizado en miembros inferiores, 52 por ciento a nivel muscular, 38 por ciento articular y33 por ciento bilateral. El 40 por ciento presentó dolor de minutos de duración, el 55 por ciento manifestó dolor, al menos, una vez al mes; el 51 por ciento aparecía en relación con el ejercicio y el 16 por ciento refirió limitación de la actividad.El 63 por ciento consultó a un pediatra y el 8 por ciento a un traumatólogo.Al comparar las características de dolor en menores y mayores de 6 años, los últimos presentaron dolor más de una vez por mes y posterior al ejercicio.Conclusiones. La prevalencia de dolor musculoesquelético fue 21,4 por ciento. Se asoció con la edad y antecedentes de dolor en familiar. Fue referido a miembros inferiores, predominantemente en músculo, más de una vez por mes y relacionado con el ejercicio.(AU)

Estimación de la prevalencia de dolormusculoesquelético en niños atendidosen un hospital general de comunidad / Estimated prevalence of musculoskeletal pain in children, who attend a community hospital

El dolor musculoesquelético esfrecuente en la infancia. La prevalencia se estima entre 5 y 19 por ciento en niños sanos.Objetivos. Estimar la prevalencia de dolor musculoesquelético y describir las características epidemiológicasen niños de 3 a 12 años.Población, material y métodos. Estudio observacional de corte transversal. Se realizó encuesta autoadministrada en la sala de espera. Se excluyeron niños con enfermedad crónica.Resultados. Se entregaron 240 encuestas y se completaron 229. La mediana de edad de los niños fue 6 (3-12) años y de las madres 36 (25-50)años. El 54 por ciento eran niñas. El 21, 4 por ciento y el 19,2 por ciento refirieron dolor en el último año y en los últimos 3 meses, respectivamente. Los factores asociados con dolor en el último año fueron: edad y antecedentede dolor en un familiar durante la infancia y actualmente. No se encontraron diferencias con el sexo del paciente, edad materna, familiamonoparental, tener hermanos ni indicadores de estrés familiar en el último año.En 66 por ciento el dolor fue localizado en miembros inferiores, 52 por ciento a nivel muscular, 38 por ciento articular y33 por ciento bilateral. El 40 por ciento presentó dolor de minutos de duración, el 55 por ciento manifestó dolor, al menos, una vez al mes; el 51 por ciento aparecía en relación con el ejercicio y el 16 por ciento refirió limitación de la actividad.El 63 por ciento consultó a un pediatra y el 8 por ciento a un traumatólogo.Al comparar las características de dolor en menores y mayores de 6 años, los últimos presentaron dolor más de una vez por mes y posterior al ejercicio.Conclusiones. La prevalencia de dolor musculoesquelético fue 21,4 por ciento. Se asoció con la edad y antecedentes de dolor en familiar. Fue referido a miembros inferiores, predominantemente en músculo, más de una vez por mes y relacionado con el ejercicio.

[Estimated prevalence of musculoskeletal pain in children, who attend a community hospital]. / Estimación de la prevalencia de dolor musculoesquelético en niños atendidos en un hospital general de comunidad.

INTRODUCTION: Musculoskeletal pain is a common symptom in childhood. It is estimated that 5 to 19% of healthy children experience musculoskeletal pain. AIMS: To estimate the prevalence of musculoskeletal pain and to describe epidemiological characteristics in children 3 to 12 years of age, who attend a community hospital. POPULATION, MATERIAL AND METHODS: This is an observational transversal study. A self-administered questionnaire was completed in the waiting room of the Pediatric ambulatory area. Children with chronic diseases were excluded. Statistical tests used included chi(2) test, Mc Nemar and a logistic regression model analysis. RESULTS: A total of 240 questionnaires were distributed and 229 were returned. Median age of children was 6 years and 36 (25-50) years in their mothers. Fifty four percent were girls. Pain was present in 21.4% in the last year, and 19.2% in the past 3 months, both groups were concordant (Mc Nemar test not significant). Associated factors with pain in the last year were: age and family history of current pain and during childhood (logistic regression model). No relation was found with sex of the child, mother age, mono-parental family, siblings or stress family factors in the last year. The musculoskeletal pain was localized in: lower limbs (66%), muscles (52%), joints (38%) and was bilateral in 33%. In 40% of children the pain duration was transient (minutes), 55% developed pain, at least, once a month and in 51% was related to exercise. Limitation of daily living activities due to pain was seen in 16%. The pain resolved spontaneously in 50%. Pediatricians were consulted in 63% of the cases and 8% consulted in Orthopedics. When comparing the pain pattern in 2 groups of children (< and > 6 years), we found that older children tended to present pain more than once a month and related to exercise. CONCLUSIONS: The prevalence of musculoskeletal pain was 21.4%. Pain was associated to child age and family history of musculoskeletal pain. Pain was referred to lower limbs (mainly muscles), periodic (more than once a month) and related to exercise.