Analysis of 9 cases of drug induced hypersensitivity syndrome related hemophagocytic lymphohistiocytosis / 中华儿科杂志

Objective: To analyze the clinical features,treatment and prognosis of drug induced hypersensitivity syndrome related hemophagocytic lymphohistiocytosis (DIHS-HLH). Methods: This was a retrospective case study. Clinical characteristics, laboratory results, treatment and prognosis of 9 patients diagnosed with DIHS-HLH in Beijing Children's hospital between January 2020 and December 2022 were summarized. Kaplan-Meier survival analysis was used to calculate the overall survival rate. Results: Among all 9 cases, there were 6 males and 3 females, with the age ranged from 0.8 to 3.1 years. All patients had fever, rash, hepatomegaly and multiple lymph node enlargement. Other manifestations included splenomegaly (4 cases), pulmonary imaging abnormalities (6 cases), central nervous system symptoms (3 cases), and watery diarrhea (3 cases). Most patients showed high levels of soluble-CD25 (8 cases), hepatic dysfunction (7 cases) and hyperferritinemia (7 cases). Other laboratory abnormalities included hemophagocytosis in bone marrow (5 cases), hypofibrinogenemia (3 cases) and hypertriglyceridemia (2 cases). Ascending levels of interleukin (IL) 5, IL-8 and interferon-γ (IFN-γ) were detected in more than 6 patients. All patients received high dose intravenous immunoglobulin, corticosteroid and ruxolitinib, among which 4 patients were also treated with high dose methylprednisolone, 2 patients with etoposide and 2 patients with cyclosporin A. After following up for 0.2-38.6 months, 7 patients survived, and the 1-year overall survival rate was (78±14)%. Two patients who had no response to high dose immunoglobulin, methylprednisolone 2 mg/(kg·d) and ruxolitinib died. Watery diarrhea, increased levels of IL-5 and IL-8 and decreased IgM were more frequently in patients who did not survive. Conclusions: For children with fever, rash and a suspicious medication history, when complicated with hepatomegaly, impaired liver function and high levels of IL-5 and IL-8, DIHS-HLH should be considered. Once diagnosed with DIHS-HLH, suspicious drugs should be stopped immediately, and high dose intravenous immunoglobulin, corticosteroid and ruxolitinib could be used to control disease.

Neumonía por Pneumocystis jirovecii asociada a hemorragia alveolar difusa en un paciente con trasplante renal / Pneumocystis jirovecii pneumonia associated with diffuse alveolar hemorrhage in a renal transplant patient. Case report

La neumonía por Pneumocystis jirovecii es una enfermedad fúngica oportunista descrita principalmente en pacientes con VIH, sin embargo, tras la introducción de la TARV, ha incrementado su incidencia en pacientes con inmunosupresión no asociada a VIH, como neoplasias hematológicas y trasplantes de órganos sólidos. Presentamos el caso de un varón de 17 años, receptor de un trasplante renal, con inmunosupresión prolongada con corticoesteroides, con cuadro clínico de tos, disnea y fiebre. La TC mostró micronódulos pulmonares centrolobulillares y vidrio esmerilado. El LBA fue compatible con hemorragia alveolar difusa (HAD), con RPC positiva para P. jirovecii. Se descartaron otras infecciones y enfermedades autoinmunes. Recibió tratamiento con cotrimoxazol con buena evolución clínica y mejoría radiológica. Si bien las causas más frecuentes de HAD son etiologías autoinmunes como enfermedades reumatológicas o vasculitis, es prioritario descartar causas infecciosas, incluyendo P. jirovecii, ya que el tratamiento dirigido puede tener un impacto significativo en la mortalidad en este grupo de pacientes.

Pneumocystis jirovecii pneumonia is an opportunistic fungal infection, described mainly in HIV patients, however, after the introduction of ART, its presentation has increased in patients with non-HIV immunosuppression, such as hematological cancers, solid or hematopoietic stem cell transplantation. We report the case of a 17-year-old male, kidney transplant patient, with prolonged immunosuppression with corticoesteroids, with history of cough, dyspnea, and fever. Chest CT evidences centrilobular pulmonary micronodules with ground glass. BAL was performed compatible with diffuse alveolar hemorrhage, with positive PCR for P. jirovecii. Other infections and autoimmune disease were ruled out. He received treatment with cotrimoxazole with clinical improvement of the patient, and follow up chest CT at the end of treatment showed decrease of pulmonary infiltrates. Although the most frequent causes of DAH are autoimmune etiologies such as rheumatic diseases or vasculitis, it is a priority to rule out infectious causes, including P. jirovecii, since targeted treatment could have a significant impact on mortality outcomes in this group of patients.

Aplicação de ácido hialurônico e corticoide através de uma punção guiada por ultrassonografia no tratamento de disfunção da articulação temporomandibular: relato de caso / Hyaluronic and corticoid application through an ultrasound-guided puncture in the treatment of temporomandibular joint dysfunctions: case report / Aplicación de ácido hialurónico y corticoide a través de una punción guiada por ecografía en el tratamiento de la disfunción de la articulación temporomandibular: informe de un caso

Introdução: As Disfunções temporomandibulares (DTM) incluem desordens dos músculos da mastigação, das articulações temporomandibulares e da inervação local, frequentemente associadas a dor orofacial e que resultam em mioartropatias do Sistema Mastigatório. A tendência atual tende a começar com tratamento conservador e progredir a procedimentos mais invasivos na falha dos tratamentos iniciais. Relato de caso: O presente relato visa mostrar o resultado de uma técnica invasiva para o tratamento de uma DTM grave, com a aplicação do ácido hialurônico e de corticoide através de uma punção guiado por ultrassonografia. A paciente apresentava dor crônica e perda importante de peso devido a limitação da abertura da boca. A RM demonstrou disfunção das ATMs, com sinais de deslocamento parcial do disco direito anteromedialmente. Foi realizada a aplicação bilateral intra-articular de ácido hialurônico e de corticoide através de uma punção guiado por ultrassonografia. Considerações Finais: A associação destas classes na punção de ATMs ainda não está bem estabelecida havendo necessidade de estudos complementares para avaliar eficácia, como este relato de caso, que se mostrou favorável com grande melhora clínica da paciente... (AU)

Introduction: Temporomandibular dysfunctions (TMD) include disorders of the masticatory muscles, temporomandibular joints, and local innervation, often associated with orofacial pain and resulting in myoarthropathies of the masticatory system. The current trend tends to begin with conservative treatment and progress to more invasive procedures if the initial treatments fail. Case Report: The present report aims to show the result of an invasive technique for the treatment of a severe TMD, with the application of hyaluronic acid and corticoid through an ultrasound-guided puncture. The patient presented with chronic pain and significant weight loss due to limited mouth opening. MRI demonstrated TMJ dysfunction, with signs of partial anteromedial dislocation of the right disc. Bilateral intra-articular application of hyaluronic acid and corticoid was performed through an ultrasound guided puncture. Final considerations: The association of these classes in TMJ puncture is still not well established, and further studies are needed to evaluate efficacy, as in this case report, which proved favorable, with great clinical improvement for the patient... (AU)

Introducción: Los trastornos temporomandibulares (TTM) incluyen trastornos de los músculos masticatorios, de las articulaciones temporomandibulares y de la inervación local, a menudo asociados a dolor orofacial y que dan lugar a mioartropatías del sistema masticatorio. La tendencia actual es comenzar con un tratamiento conservador y progresar hacia procedimientos más invasivos al fracasar los tratamientos iniciales. Informe de un caso: El presente informe pretende mostrar el resultado de una técnica invasiva para el tratamiento de un TTM severo, con la aplicación de ácido hialurónico y corticoide a través de una punción guiada por ecografía. El paciente presentaba dolor crónico y una importante pérdida de peso debido a la limitación de la apertura bucal. La RMN demostró una disfunción de la ATM, con signos de dislocación parcial del disco derecho anteromedialmente. Se realizó la aplicación intraarticular bilateral de ácido hialurónico y corticoide mediante una punción guiada por ecografía. Consideraciones finales: La asociación de estas clases en la punción de la ATM aún no está bien establecida y se necesitan más estudios para evaluar la eficacia, como en el reporte de este caso, que resultó favorable con gran mejoría clínica del paciente... (AU)

Treatment and Management Experience of Idiopathic Granulomatous Mastitis in a Low-income Country / Experiência de tratamento e manejo da mastite granulomatosa idiopática em um país de baixa renda

Abstract Objective Reporting our experience of the management and treatment of Idiopathic granulomatous mastitis (IGM) in a low-income country by describing patients characteristics and therapy with emphasis on conservative surgical excision and postoperative care as the cornerstone of treatment. Methods A retrospective cohort of women with histopathological diagnosis of IGM from 2014 to 2018 at Instituto Nacional Materno Perinatal in Lima, Peru. Patients' characteristics, clinical presentation, treatment, management, postoperative care, and follow-up were analyzed. Results Thirty-eight patients with histopathological diagnosis of IGM were identified. Their average age was 35.9 years and 23 (60.5%) reported previous use of hormonal contraceptives. Nine (23.7%) patients had chronic mastitis with previous treatment. The time from the onset of symptoms to the first clinic consult was 5.1 months on average. Twenty-one (55.3%) patients had the lesion in the right breast, with a mean size of 6.9 cm. Conservative surgical excision was performed in all patients. Additionally, 86.8% required corticosteroids and 78.9% were treated with antibiotics. Complete remission was obtained at 141 days on average (range 44 to 292 days). Six (15.8%) women reported ipsilateral recurrence and 5 (13.2%), contralateral. The latency time was 25.5 months on average. Conclusion The conservative surgical treatment demonstrated and close follow-up made for a high cure rate, but with recurrence similar to that reported in the literature. Use of gloves is an alternative to manage post operative wounds in a low-income country. The most frequent adverse effect was breast surgical scar.

Treatment of Acute Spinal Cord Injuries: A Survey Among Iberolatinoamerican Spine Surgeons - Part 2 Timing to Surgery / Tratamento de lesões agudas da medula espinal: Uma pesquisa entre cirurgiões de coluna iberoamericanos - Parte 2: Momento da cirurgia

Abstract Objective The objective of the present study was to evaluate the current practice in terms of timing to surgery in acute spinal cord injury (ASCI) patients among spinal surgeons from Iberolatinoamerican countries. Methods A descriptive cross-sectional study design as a questionnaire was sent by an email for all members of the Sociedad Ibero Latinoamericana de Columna (SILACO, in the Spanish acronym) and associated societies. Results A total of 162 surgeons answered questions related to the timing for surgery. Sixty-eight (42.0%) considered that ASCI with complete neurology injury should be treated within 12 hours, 54(33.3%) performed early decompression within 24 hours, and 40 (24,7%) until the first 48 hours. Regarding ASCI with incomplete neurological injury, 115 (71.0%) would operate in the first 12 hours. There was a significant difference in the proportion of surgeons that would operate ASCI within ≤ 24 hours, regarding the type of injury (complete injury:122 versus incomplete injury:155; p<0.01). In the case of patients with central cord syndrome without radiological evidence of instability, 152 surgeons (93.8%) would perform surgical decompression: 1 (0.6%) in the first 12 hours, 63 (38.9%) in 24 hours, 4 (2.5%) in 48 hours, 66 (40.7%) in the initial hospital stay, and 18 (11.1%) after neurologic stabilization. Conclusion All inquired surgeons favour early decompression, with the majority performing surgery in the first 24 hours. Decompression is performed earlier in cases of incomplete than in complete injuries. In cases of central cord syndrome without radiological evidence of instability, there is a tendency towards early surgical decompression, but the timing is still extremely variable. Future studies are needed to identify the ideal timing for decompression of this subset of ASCI patients.

Resumo Objetivo O objetivo do presente estudo foi avaliar a prática atual em termos de momento de realização da cirurgia em pacientes com lesão medularaguda (LMA) entre cirurgiões de coluna de países ibero-americanos. Métodos Estudo transversal descritivo com base em um questionário enviado por correio eletrônico para todos os membros da Sociedad Ibero Latinoamericana de Columna (SILACO, na sigla em espanhol) e sociedades associadas. Resultados Um total de 162 cirurgiões responderam a perguntas relacionadas ao momento da cirurgia. Sessenta e oito (42,0%) consideraram que a LMA com lesão neurológica completa deve ser tratada em até 12 horas, 54 (33,3%) realizariam a descompressão precoce em até 24 horas e 40 (24,7%) fariam este procedimento nas primeiras 48 horas. Em relação à LMA com lesão neurológica incompleta, 115 (71,0%) operariam nas primeiras 12 horas. Houve diferença significativa na proporção de cirurgiões que fariam o tratamento cirúrgico da LMA em ≤ 24 horas quanto ao tipo de lesão (lesão completa [122] versus lesão incompleta [155]; p<0.01). Em pacientes com síndrome medular central sem evidência radiológica de instabilidade, 152 cirurgiões (93,8%) realizariam a descompressão cirúrgica: 1 (0,6%) nas primeiras 12 horas, 63 (38,9%) em 24 horas, 4 (2,5%) em 48 horas, 66 (40,7%) no internamento inicial e 18 (11,1%) após a estabilização neurológica. Conclusão Todos os cirurgiões participantes favoreceram a descompressão precoce; a grande maioria realizaria a cirurgia nas primeiras 24 horas. A descompressão é feita antes em casos de lesões incompletas do que em lesões completas. Nos casos de síndrome medular central sem evidência radiológica de instabilidade, há uma tendência à descompressão cirúrgica precoce, mas o momento de intervenção ainda é extremamente variável. Estudos futuros são necessários para identificar o momento ideal para descompressão neste subconjunto de pacientes com LMA.

Treatment of Acute Spinal Cord Injuries: A Survey Among Iberolatinoamerican Spine Surgeons - Part 1: Use of High-Dose Corticosteroids / Tratamento das lesões agudas da medula espinal: Uma pesquisa entre cirurgiões de coluna iberoamericanos - Parte 1: Uso de corticosteroides em altas doses

Abstract Objective The aim of the present study was to evaluate the current practice of using of methylprednisolone sodium succinate (MPSS) in acute spinal cord Injuries (ASCIs) among spine surgeons from Iberolatinoamerican countries. Methods A descriptive cross-sectional study design as a survey was conducted. A questionnaire composed of 2 sections, one on demographic data regarding the surgeons and MPSS administration, was sent by email to members of the Sociedad Ibero Latinoamericana de Columna (SILACO, in the Spanish acronym) and associated societies. Results A total of 182 surgeons participated in the study: 65.4% (119) orthopedic surgeons and 24.6% (63) neurosurgeons. Sixty-nine (37.9%) used MPSS in the initial management of ASCIs. There were no significant differences between countries (p = 0.451), specialty (p = 0.352), or surgeon seniority (p = 0.652) for the use of corticosteroids in the initial management of ASCIs. Forty-five (65.2%) respondents reported using an initial high-dose bolus (30 mg/Kg) followed by a perfusion (5.4 mg/ kg/h). Forty-six (66.7%) surgeons who used MPSS only prescribed it if the patients presented within 8 hours of the ASCI. Most of the surgeons (50.7% [35]) administered high-dose corticosteroids because of the conviction that it has clinal benefits and improves neurological recovery. Conclusion Results from the present survey show that MPSS use in ASCI is not widespread within spine surgeons and that the controversy regarding its use remains unresolved. This is probably due to the low level of evidence of the available data, to variations over the years, to inconsistencies in acute care protocols, and to health service pathways.

Resumo Objetivo O objetivo do presente estudo foi avaliar a prática atual de uso do succinato sódico de metilprednisolona (MPSS, na sigla em inglês) nas lesões agudas da medula espinal (LAMEs) entre cirurgiões de coluna de países ibero-americanos. Métodos Um estudo transversal descritivo foi realizado. O questionário continha duas seções, uma sobre os dados demográficos dos cirurgiões e acerca da administração de MPSS, e foi enviado por correio eletrônico aos membros da Sociedad Ibero Latinoamericana de Columna (SILACO, na sigla em espanhol) e sociedades associadas. Resultados No total, 182 cirurgiões participaram do estudo: 65,4% (119) eram cirurgiões ortopédicos e 24,6% (63), neurocirurgiões. Sessenta e nove (37,9%) usaram MPSS no tratamento inicial da LAME. Não houve diferenças significativas entre países (p = 0,451), especialidades (p = 0,352) ou senioridade do cirurgião (p =0,652) em relação ao uso de corticosteroides no tratamento inicial da LAME. Destes, 45 (65,2%) relataram a administração de um bolus de alta dose (30 mg/kg) seguido por perfusão (5,4 mg/kg/h). Quarenta e seis (66,7%) dos cirurgiões que usam MPSS apenas o prescrevem a pacientes tratados nas primeiras 8 horas após a LAME. A maioria dos cirurgiões (50,7% [35]) administrou corticosteroides em alta dose devido à convicção de seus benefícios clínicos e melhora da recuperação neurológica. Conclusão Os resultados do presente questionário mostram que o uso de MPSS na LAME não está disseminado entre os cirurgiões de coluna e que a controvérsia sobre sua administração ainda não foi resolvida. É provável que isto se deva ao baixo nível de evidência dos dados existentes, a variações ao longo dos anos, a inconsistências nos protocolos terapêuticos agudo e a diferentes sistemas de saúde.

Efetividade das intervenções para prevenção de displasia broncopulmonar em recém-nascidos prematuros sob ventilação mecânica invasiva: overview de revisões sistemáticas / Effectiveness of interventions for the prevention of bronchopulmonary dysplasia in premature newborns under invasive mechanical ventilation: overview of systematic reviews

Contextualização: A displasia broncopulmonar é uma das principais causas de enfermidade respiratória crônica na infância, levando a hospitalizações frequentes e prolongadas e com altos índices de mortalidade, alterações do crescimento pôndero-estatural e desenvolvimento neuropsicomotor. Tamanho impacto justifica o grande investimento nas pesquisas para identificar suas causas e buscar alternativas para prevenção e tratamento. Objetivos: Avaliar a efetividade das intervenções para prevenção de displasia broncopulmonar em recém-nascidos prematuros com ventilação mecânica invasiva. Métodos: Trata-se de overview de revisões sistemáticas realizadas pela Colaboração Cochrane. Procedeu-se à busca na Cochrane Library (2022), utilizando os termos "neonatal prematurity" e "bronchopulmonary dysplasia". Foram incluídos todos os ensaios clínicos randomizados. O desfecho primário de análise foi a redução de morbimortalidade. Resultados: A estratégia de busca recuperou um total de 47 revisões sistemáticas. Oito foram incluídas, totalizando 94 ensaios clínicos randomizados e 10.511 participantes. Discussão: Os estudos demonstram efetividade de corticosteroides, mas é necessário cautela na dosagem e no momento correto para sua administração. O uso de surfactante sintético pode trazer benefícios respiratórios, mas requer novos estudos. Não se justifica o uso de pentoxifilina. Conclusão: A displasia broncopulmonar tornou-se um grande desafio para o neonatologista e as revisões sistemáticas Cochrane sugerem que a corticoterapia pode ser efetiva na prevenção dessa condição, embora novos estudos sejam recomendados para estabelecer dosagem ideal e melhor momento para a terapêutica.

COVID-19 related multisystem inflammatory syndrome in children (MIS-C): A case series from Ethiopia

Background: One in hventy ofpeople qffected by the ongoing COVID-19 pandemic have been children and adolescents. A unique complication in this age group is the Multi-inflammatory syndrome associated Il'ith COVID-19 (MS-C). We report a single-center case series ofchildren diagnosed with MS-Cfrom Addis Ababa, Ethiopia. Case descriptions This case series describes the clinical presentation and treatment outcomes offour male patients presenting at a mean age of3 years and 11 months. Allfulfilled the World Health Organization case definition criteria for the Multi-inflammatomy syndrome associated 'Vith COVID-19. All "'ere not eligible for vaccinations against severe acute respiratory syndrome coronavirus 2 (SARS CoV-2) at the time oftheir diagnosis. They were treated with varying combinations of intravenous immunoglobulin, aspirin, and corticosteroids, and all recovered upon completion oftheirfollow-up period. Conclusion: Cases of Multi-inflammatomy syndrome associated with COVID-19 are often misdiagnosed. This case series highlights when to consider such a diagnosis and its therapeutic options

Características clínicas, tratamiento y evolución de una serie de niños con síndrome inflamatorio multisistémico asociado a SARS-CoV-2 hospitalizados en dos centros de salud / Clinical characteristics, treatment and evolution of a series of children with multisystemic inflammatory syndrome associated with SARS-CoV-2 hospitalized in two health centers / Características clínicas, tratamento e evolução de uma série de crianças com síndrome inflamatória multissistêmica associada ao SARS-CoV-2 internadas em dois centros de saúde

Introducción: en marzo del 2021 se registró el pico de incidencia de COVID-19 en Uruguay y un aumento de la infección en pediatría. Objetivo: describir las características clínicas, el tratamiento y la evolución de una serie de menores de 15 años con SIM-Ped S hospitalizados en dos centros de salud. Metodología: estudio descriptivo, retrospectivo, de los niños hospitalizados entre el 1/3 y el 31/6 de 2021 que cumplieron los criterios diagnósticos de SIM-Ped de la OMS. Se analizan variables clínicas, paraclínicas, tratamiento y evolución. Resultados: se incluyeron 12 niños, mediana de edad 7 años (22 meses-10 años). Se presentaron complicación posinfecciosas en 8 y en el curso de la infección en 4. Las manifestaciones fueron: fiebre (media 6 días, rango 3-10), digestivas 10 y mucocutáneas 7. Se presentaron como enfermedad Kawasaki símil 5 y como shock 2. La infección por SARS CoV-2 se confirmó por PCR en 6, serología 4 y test antigénico 2. Recibieron tratamiento en cuidados moderados 8 e intensivos 4: inmunoglobulina 9, corticoides 11, heparina 7 y ácido acetilsalicílico 7. Presentaron dilatación de arterias coronarias 2, alteraciones valvulares 2, disminución de la FEVI 2 y derrame pericárdico 2. Todos evolucionaron favorablemente. Conclusiones: en estos centros, los primeros casos de SIMS-Ped S coincidieron con el pico de incidencia de COVID-19 en el país. Predominaron las formas postinfecciosas en escolares con manifestaciones digestivas. Este estudio puede contribuir al reconocimiento de esta entidad y adecuar los algoritmos nacionales de manejo.

Introduction: in March 2021, there was a peak incidence of COVID-19 and an increase in pediatric infections in Uruguay. Objective: describe the clinical characteristics, treatment and evolution of a group of children under 15 years of age with SIM-Ped S hospitalized in two health centers. Methodology: descriptive, retrospective study of children hospitalized between 3/1 and 6/31 of 2021 who met the WHO diagnostic criteria for SIM-Ped. Clinical and paraclinical variables, as well as treatment and evolution were analyzed. Results: 12 children were included, median age 7 years (22 months-10 years). Eight of them showed post-infectious complications and 4 of them had complications during the course of the infection. The manifestations were: fever (mean 6 days, range 3-10), digestive symptoms 10 and mucocutaneous 7. Five of them presented a Kawasaki-like disease and 2 of them shock. SARS CoV-2 infection was confirmed by PCR in 6 cases, serology in 4 and antigenic test in 2. Eight of them received treatment in moderate care and 4 of them in intensive care: immunoglobulin 9, corticosteroids 11, heparin 7 and acetylsalicylic acid 7. Two of them presented dilated arteries coronary , valvular alterations 2, decreased LVEF 2 and pericardial effusion 2. All progressed favorably. Conclusions: in these centers, the first cases of SIMS-Ped S coincided with the peak incidence of COVID-19 in the country. Post-infectious forms predominated in schoolchildren who showed digestive manifestations. This study may contribute to the recognition of this entity and to the adaptation of national management algorithms.

Introdução: em março de 2021, foi registrado no Uruguai um pico de incidência da COVID-19 e um aumento dos casos da infecção pediátrica. Objetivo: descrever as características clínicas, tratamento e evolução de uma série de crianças menores de 15 anos com SIM-Ped S internadas em dois centros de saúde. Metodologia: estudo descritivo, retrospectivo, de crianças internadas entre 1/3 e 31/6 de 2021 que preencheram os critérios diagnósticos da OMS para o SIM-Ped. Foram analisadas variáveis clínicas e para-clinicas, tratamento e evolução. Resultados: foram incluídas 12 crianças, com idade média de 7 anos (22 meses-10 anos). Oito delas apresentaram complicações pós-infecciosas e 4 delas durante o curso da infecção. As manifestações foram: febre (média de 6 dias, intervalo 3-10), digestivas 10 e mucocutânea 7. Cinco delas apresentaram doença de Kawasaki-like e 2 delas sofreram Shock. A infecção por SARS CoV-2 foi confirmada por PCR em 6, sorologia em 4 e teste antigênico em 2. Oito delas receberam tratamento em cuidados moderados e 4 delas em cuidados intensivos: imunoglobulina 9, corticosteroides 11, heparina 7 e ácido acetilsalicílico 7. Duas delas apresentaram artérias coronárias dilatadas 2, alterações valvares 2, diminuição da FEVE 2 e derrame pericárdico 2. Todas evoluíram favoravelmente. Conclusões: nesses centros, os primeiros casos de SIMS-Ped S coincidiram com um pico de incidência de COVID-19 no país. As formas pós-infecciosas predominaram em escolares com manifestações digestivas. Este estudo pode contribuir para o reconhecimento desta entidade e adaptar algoritmos nacionais de gestão.

Corticoesteroide oral vs. intrarticular en el tratamiento de la capsulitis adhesiva / Oral versus intra-articular corticosteroid in the treatment of adhesive capsulitis

Introducción: La capsulitis adhesiva es una enfermedad que se caracteriza por el engrosamiento de la cápsula articular del hombro, lo que se traduce clínicamente en dolor y una pérdida progresiva de la movilidad. El tratamiento conservador es la primera opción. En este estudio, se evaluó el uso de corticoesteroides articulares para el manejo de este cuadro. Materiales y Métodos: Se llevó a cabo un estudio retrospectivo entre 2015 y 2020. Se evaluaron los registros de consulta externa de pacientes con diagnóstico de capsulitis adhesiva u hombro congelado, que recibieron tratamiento con corticoesteroides por vía oral o articular. Resultados: Se analizó a 19 pacientes, 8 recibieron tratamiento por vía oral y 11, por vía articular. Hubo una mejoría importante en ambos grupos, pero los pacientes que recibieron corticoesteroides articulares comunicaron una mejoría a largo plazo. Conclusiones: La administración de corticoesteroides tanto por vía oral como articular para tratar la capsulitis adhesiva fue eficaz; sin embargo, a largo plazo, los corticoesteroides articulares resultaron más eficaces. Nivel de Evidencia: III

Introduction: Adhesive capsulitis is a condition defined by thickening of the shoulder joint capsule, which clinically translates into discomfort and progressive loss of range of motion, with conservative therapy being the initial option. In this study, we evaluate the use of intra-articular corticosteroid injections for the treatment of this condition. Materials and Methods: A retrospective analysis was conducted between 2015 and 2020, assessing the outpatient records of patients diagnosed with adhesive capsulitis or frozen shoulder and treated with corticosteroids either orally or intra-articularly. Results: A total of 19 patients were analyzed, 8 received oral treatment and 11 received intra-articular injection, with both groups showing considerable improvement, with the difference that the patients in the articular corticosteroid group reported long-term improvement. Conclusion: Corticosteroids have been found to be effective in the treatment of adhesive capsulitis both orally and by intra-articular injection; however, intra-articular corticosteroids have proved to be more effective in the long term. Level of Evidence: III

Actualización del consenso chileno SOCHINEP-SER para el manejo de las sibilancias recurrentes del preescolar / Update of the chilean consensus SOCHINEP-SER for the management of recurrent wheezing in preschool children

Las sibilancias recurrentes del preescolar son un problema prevalente. 50% de todos los niños tiene al menos un episodio de sibilancias en los primeros 6 años. Sin embargo, solo 4 % de los menores de 4 años tiene diagnóstico de asma. Por este motivo es fundamental realizar una adecuada anamnesis y examen físico tendientes a descartar causas secundarias, lo que debe ser complementado con exámenes de laboratorio de acuerdo con la orientación clínica. En la actualidad se recomienda indicar tratamiento de mantención con corticoides inhalados en aquellos niños que tengan episodios repetidos de obstrucción bronquial y que tengan una alta probabilidad de respuesta favorable a esta terapia. Se ha demostrado que aquellos pacientes que tienen un recuento de eosinófilos en sangre > 300 células por mm3 o aquellos que presentan una prueba cutánea positiva o IgE específicas positivas para alérgenos inhalados, responderán adecuadamente al tratamiento con esteroides inhalados.

Recurrent wheezing in preschoolers has a high prevalence. 50% of all children have at least one wheezing episode in the first six years of life. However, only 4% of children under four years of age are diagnosed with asthma. Therefore, it is essential to carry out an adequate medical history and physical examination to rule out secondary causes, which must be complemented with laboratory tests in accordance with clinical guidance. It is recommended to indicate maintenance treatment with inhaled corticosteroids to those children who have repeated episodes of wheezing and who have a high probability of a good response to this therapy. It has been demonstrated that those patients who have blood eosinophil count > 300 cells per mm3 or those who have a positive skin test or positive specific IgE for inhaled allergens will have a good response to inhaled corticosteroids.

Eritema multiforme, una rara presentación de exantema en población pediátrica: a propósito de un caso / Erythema multiforme, a rare occurrence of exanthema in the pediatric population: on the subject of a case

El Eritema multiforme (EM) o eritema polimorfo es una enfermedad aguda de la piel de naturaleza inmunológica con o sin compromiso de mucosas, que puede comportarse como crónica recurrente. Se presenta con lesiones cutáneas en diana distintivas, a menudo acompañado de úlceras o bullas en mucosas (oral, genital u ocular). Entre sus formas clínicas se distingue: una forma menor caracterizado por un síndrome cutáneo leve y su forma mayor que se manifiesta como una afectación cutánea con daño mucoso marcado. Entre sus principales diagnósticos diferenciales se encuentran el Síndrome de Stevens-Johnson (SSJ) y Síndrome de Lyell (Necrólisis epidérmica tóxica (NET)). Tiene una incidencia estimada < 1%, siendo su forma mayor levemente más frecuente que su forma menor (0.8-6 por millón/año). Puede darse a cualquier edad, presentando un peak de incidencia entre los 20 y 30 años, predominando ligeramente el sexo masculino con una proporción 3:2, sin predilección racial. Su presentación en edad pediátrica es rara, más aún en la primera infancia. En esta población es más frecuente el EM menor recurrente. En el presente texto se reporta un caso de EM en población pediátrica como una rara forma de presentación exantemática, abordado en el Servicio de Pediatría del Complejo Asistencial Dr Victor Rios Ruiz (CAVRR)en la ciudad de Los Ángeles, Chile en el presente año.

Erythema multiforme (EM) also known as polymorph erythema is an acute skin disease of immunological nature with or without mucous membrane involvement, which may behave as chronic recurrent. It presents with distinctive targets like skin lesions, often together with ulcers or bullae in mucous membranes (oral, genital or ocular). Among its clinical forms are: a minor form characterized by a mild skin syndrome and its major form that manifests as a skin disease with marked mucosal damage. Among its main differential diagnoses are Stevens-Johnson Syndrome (SJS) and Lyell Syndrome (Toxic Epidermal Necrolysis (TEC)). It has an estimated incidence < 1%, with its major form being slightly more frequent than its minor form (0. 8-6 per million/year). It can occur at any age, presenting a peak incidence at the age between 20 and 30 years, with a slight predominance of males with a 3:2 ratio, without racial predilection. Its presentation in pediatric age is rare, even more so in early childhood. Minor recurrent EM is more common in this population. This paper reports a case of EM in the pediatric population as a rare form of exanthematic presentation, addressed at the Department of Pediatrics of the Complejo Asistencial Victor Rios Ruiz (CAVRR) in the city of Los Angeles, Chile this year.

Factors associated with the inappropriate use of topical Corticosteroids among out-patient dermatology patients: A cross-sectional study

@#Topical corticosteroids (TC) are among the most commonly prescribed topical agents and are used to treat various dermatoses. This study aimed to determine the prevalence, factors associated and reasons patients inappropriately use TC. Incidence of AE and risk of the development of AE due to inappropriate TC. Out-patient dermatology patients in a tertiary hospital in the Philippines were screened for TC use and interviewed using a questionnaire. Descriptive analysis and logistic regression to determine odds ratios were done. Out of 801 patients recruited, 260 (32%) used TC. Among those who used TC, 147 (56.53%) inappropriately used TC. Among the factors associated with inappropriate use of TC were: 1) Diagnosis of dermatitis as primary dermatosis (OR = 2.82, 95% CI 0.497, 1.276), 2) Lack of awareness of the FDA advisory (OR = 2.1, 95% CI 1.245, 3.601), 3) Lack of knowledge that TC cannot be applied for prolonged periods (OR = 5.5, 95% CI 3.201, 9.334), and 4) Lack of knowledge that TC use can result into AE (OR = 4.5, 95% CI 2.637, 7.657). Relatives and friends as source of information (OR=437, 95% CI 25.997, >1000), procurement (OR=60, 95% CI 3.081, >1000) and instruction (OR=337, 95% CI 19.827, >1000) were highly associated with inappropriate use. Twenty-two percent of those who inappropriately used TC self-medicated upon recommendation by family and friends. Hypopigmentation was the most common AE. There is a 3.8 times (OR= 3.8, 95% CI: 1.918, 7.662) likelihood for a person who has inappropriately used TC to have an AE as compared to a person who has appropriately used TC. There is a need to educate the general public regarding proper TC use. Patient encounters at the clinic may be a good opportunity to reinforce guidelines on the use of TC.

Prevalence of oral candidiasis in asthma and COPD patients using inhaled corticosteroids in the Philippine General Hospital Department of out-patient services

Background and Objectives@#Oral candidiasis (OC) is a well-known local side effect of inhaled corticosteroid (ICS) therapy in patients with asthma and chronic obstructive pulmonary disease (COPD). This study aimed to determine the prevalence of OC and its association with ICS-related factors in out-patient asthma and COPD patients of the Departments of Pulmonology and Pediatric Pulmonology of the Philippine General Hospital (PGH).@*Methods@#This is a cross-sectional study conducted from October 2019 to January 2020. Data was collected through a two-part questionnaire accomplished by doctors and patients with asthma or COPD. Results. A total of 67 patients were included in the study. Oral candidiasis was observed in 4 (5.97%) ICS users, and the prevalence was 1.65% to 14.59% (95% CI, SE: 0.028946).@*Conclusion@#This study determined the prevalence of oral candidiasis in asthma and COPD patients and its association with ICS-related factors, including the dosage, medication, device, and duration of therapy. The prevalence of OC in ICS users in PGH cannot be interpreted as high or low due to the small number of respondents, but is consistent with OC prevalence found in related literature. Increased prevalence was observed in adult females with asthma under low dose ICS therapy with Fluticasone/Salmeterol DPI for more than a year. There was no statistically significant correlation among OC prevalence, age, sex, and components of ICS-therapy including dosage, medication, device, frequency, and duration of therapy. A large-scale study is recommended for more accurate assessment of OC prevalence in the population and to determine statistically significant associations among the factors. It is also recommended to quantifiably measure patient compliance, inhalation technique and instruction, and its association to OC prevalence. Findings may be used to strengthen patient education, preventive measures, and disease management to facilitate improved compliance and effective treatment outcomes.

Clinical characteristics and related factors analysis of adrenal crisis occurred in children with primary nephrotic syndrome / 中华儿科杂志

Objective: To investigate the clinical characteristics and related factors of corticosteroid induced adrenal crisis (AC) in children with primary nephrotic syndrome (NS). Methods: Case control study. The case group included 7 children aged 1 to 18 years with NS combined with AC hospitalized in Peking University First Hospital from January 2016 to May 2021 (AC group). According to the ratio of case group: control group 1: 4, 28 children aged 1 to 18 years who were diagnosed with NS without AC during the same period were matched as controls (non-AC group). Clinical data were collected. The clinical characteristics of AC were described. The clinical parameters were compared between the 2 groups by t test, Mann-Whitney U test or Fisher's test. Receiver operating characteristic (ROC) curve was used to analyze the cutoff values of clinical parameters for prediction of AC. Results: The AC group included 4 boys and 3 girls aged 6.9 (4.6, 10.8) years. The non-AC group included 20 boys and 8 girls aged 5.2 (3.3, 8.4) years. All AC events occurred during the relapse of NS with infection. Seven children had gastrointestinal symptoms such as nausea, vomiting and abdominal pain. Six children had poor mental state or impaired consciousness. No significant differences in NS course, corticosteroid treatment course, corticosteroid type, steroid dosage, steroid medication interval, the proportion of gastroenteritis and fever existed between the two groups (all P>0.05). Compared with the non-AC group, the duration from the onset of the relapse of NS until hospitalization in the AC group was significantly shorter (0.2 (0.1, 0.6) vs. 1.0 (0.4, 5.0) month,U=25.50, P=0.005). The 24 h urinary total protein (UTP) level was significantly higher in the AC group (193 (135, 429) vs. 81 (17, 200) mg/kg, U=27.00,P=0.036) than the non-AC group. The serum albumin level in the AC group was significantly lower((13.1±2.1) vs. (24.5±8.7) g/L,t=-6.22,P<0.001) than the non-AC group. There were significantly higher total white blood cell counts ((26±9)×109 vs. (11±5)×109/L,t=4.26,P=0.004), percentage of neutrophils (0.71±0.08 vs. 0.60±0.19,t=2.56,P=0.017) and the proportion of children with C reactive protein level≥8 mg/L (3/7 vs. 0,P=0.005) in the AC group than in the non-AC group. ROC curve analysis showed that the cutoff value of 24 h UTP was 122 mg/(kg·d) with a sensitivity of 100.0% and specificity of 70.4%. The cutoff value of serum albumin was 17.0 g/L with a sensitivity of 100.0% and specificity of 82.1%. Conclusions: Gastrointestinal symptoms and poor mental state were prominent manifestations of AC in children with NS. High 24 h UTP level, low serum albumin level, high peripheral white blood cell counts, high neutrophils percentage, and high C-reactive protein level during the early stage of NS relapse may be related to the occurrence of AC in children with NS.

The correlation between FCER2 gene polymorphism and the efficacy of inhaled corticosteroids in patients with chronic rhinosinusitis / 临床耳鼻咽喉头颈外科杂志

Objective:To investigate the correlation between FCER2（2206A>G） gene polymorphism and the efficacy of inhaled corticosteroids（ICS） in patients with chronic rhinosinusitis（CRS）. Methods:A total of 208 CRS patients were routinely treated with functional endonasal sinus surgery and postoperative ICS. DNA extraction, PCR amplification and gene sequencing were performed to observe the FCER2（2206A>G） gene polymorphism and calculate the allele frequency. The visual analog scale（VAS） score, Lund-Kennedy score, and computed tomography（CT） Lund-Mackay score were determined 6 months after surgery among patients with different genotypes. Moreover, the polymorphism frequency was compared among different subgroups（chronic rhinosinusitis with nasal polyps versus chronic rhinosinusitis without nasal polyps, eosinophilic chronic rhinosinusitis versus non-eosinophilic chronic rhinosinusitis）. Results:There were FCER2（2206A>G） gene polymorphism in patients with CRS, and the phenotypes included 3 genotypes, AA, AG and GG, with distribution frequencies of 68（32.7%）, 116（55.8%） and 24（11.5%） cases, respectively. No significant differences were found in age, VAS score, nasal endoscopic Lund-Kennedy score and CT imaging Lund-Mackay score among patients with CRS of each genotype before surgery. In patients with the AA genotype, the changes in VAS score（5.74±1.10）, Lund Kennedy score（5.92 ± 1.14）, and CT imaging Lund-Mackay score（13.26±4.26） were significantly higher than in patients with the AG（4.37±0.86, 5.37±1.24, 10.82±3.77） and GG（4.26±0.80, 5.18±1.56, 10.10±3.53） genotype（P<0.05）. However, there were no marked difference between patients with the AG genotype and those with the GG genotype（P>0.05）. Compared with patients with non-eosinophilic sinusitis, Among them, the differences between the GG genotype and AG /AA genes were more significant in eosinophilic sinusitis compared to non-eosinophilic sinusitis（P<0.01）. Conclusion:The FCER2（2206A>G） gene in patients with CRS has genetic polymorphism and is associated with the recovery of CRS patients after surgery, individual corticosteroid sensitivity, and subgroup variability.

Comparison of Cyclosporine A and Cyclosporine A Combined with Corticosteroid in the Treatment of Acquired Pure Red Cell Aplasia / 中国实验血液学杂志

OBJECTIVE@#To evaluate the efficacy, safety and relapse of cyclosporine A (CsA) and CsA combined with corticosteroid (CS) as the frontline therapy for patients with newly diagnosed acquired pure red cell aplasia (aPRCA).@*METHODS@#The clinical features, treatment responses, relapses and clinical outcomes of patients with newly diagnosed aPRCA in Peking Union Medical College Hospital (PUMCH) from January 2015 to May 2020 were analyzed retrospectively. All the enrolled patients had been treated with either CsA or CsA+CS for at least 6 months and had been followed up for at least 12 months, with complete clinical data and consent forms.@*RESULTS@#96 patients including 72 treated with CsA and 24 treated with CsA+CS were enrolled. With comparable baseline characteristics and follow-up periods, patients treated with CsA or with CsA+CS had similar overall response rates (ORRs) and complete response rates (CRRs) at the 3rd, 6th and 12th month and at the end of follow-up (P>0.05). Meanwhile, no significant difference was found between the two groups in the optimal ORR, optimal CRR, time to response or time to complete response. CsA+CS and CsA groups had similar adverse event (AE) rates, but CsA+CS group had higher CS-related infection rate (P <0.05). One patient in CsA+CS group died of multiple infections. As for the relapse, the two groups had compatible relapse rates at different time points, time to relapse, overall relapse rate and relapse-free survival (P>0.05). CsA exposure time, rather than different therapy regimens, was the only influence factor for either ORR or relapse rate (P <0.05).@*CONCLUSION@#CsA monotherapy has similar efficacy, AE rate and relapse rate as compared with CsA+CS for patients with newly diagnosed aPRCA, and shows less CS-related AEs such as infection.