Graft-versus-host disease and other cutaneous manifestations in pediatric patients transplanted for Fanconi anemia.

OBJECTIVE: The aim of this study was to elaborate a specific protocol for the assessment and early identification of skin lesions in pediatric patients with Fanconi anemia undergoing hematopoietic stem cell transplantation. METHODS: This is a longitudinal, retrospective, and descriptive study. The medical records of 136 pediatric patients with Fanconi anemia who underwent hematopoietic stem cell transplantation between 2008 and 2018 at the Clinical Hospital of the Federal University of Paraná were reviewed. A specific protocol was created for data collection, which included age, sex, skin color, age at diagnosis of Fanconi anemia, transplantation data, family history of consanguinity, and pre- and post-transplant complications. In addition, the data included the presence of graft-versus-host disease of the skin and other organs, its classification, type of lesion, location, and also skin lesions not related to graft-versus-host disease. RESULTS: Among the skin manifestations in pre-transplant period, café-au-lait spots stood out (32.4%). At least one organ was affected by graft-versus-host disease in 55.1% of patients; the most common involvement being the mouth, followed by the skin. Rash and erythema were the most frequently observed cutaneous manifestations of graft-versus-host disease. CONCLUSION: A high prevalence of cutaneous manifestations of the disease was observed, as well as cutaneous manifestations of graft-versus-host disease. The protocol developed gathers relevant and standardized information for the follow-up of patients with Fanconi anemia undergoing hematopoietic stem cell transplantation, ensuring greater reliability of the information, and its implementation will allow the prospective evaluation of patients.

Graft-versus-host disease and other cutaneous manifestations in pediatric patients transplanted for Fanconi anemia / Doença do enxerto contra o hospedeiro e outras manifestações cutâneas em pacientes pediátricos transplantados por anemia de Fanconi

ABSTRACT Objective: The aim of this study was to elaborate a specific protocol for the assessment and early identification of skin lesions in pediatric patients with Fanconi anemia undergoing hematopoietic stem cell transplantation. Methods: This is a longitudinal, retrospective, and descriptive study. The medical records of 136 pediatric patients with Fanconi anemia who underwent hematopoietic stem cell transplantation between 2008 and 2018 at the Clinical Hospital of the Federal University of Paraná were reviewed. A specific protocol was created for data collection, which included age, sex, skin color, age at diagnosis of Fanconi anemia, transplantation data, family history of consanguinity, and pre- and post-transplant complications. In addition, the data included the presence of graft-versus-host disease of the skin and other organs, its classification, type of lesion, location, and also skin lesions not related to graft-versus-host disease. Results: Among the skin manifestations in pre-transplant period, café-au-lait spots stood out (32.4%). At least one organ was affected by graft-versus-host disease in 55.1% of patients; the most common involvement being the mouth, followed by the skin. Rash and erythema were the most frequently observed cutaneous manifestations of graft-versus-host disease. Conclusion: A high prevalence of cutaneous manifestations of the disease was observed, as well as cutaneous manifestations of graft-versus-host disease. The protocol developed gathers relevant and standardized information for the follow-up of patients with Fanconi anemia undergoing hematopoietic stem cell transplantation, ensuring greater reliability of the information, and its implementation will allow the prospective evaluation of patients.

RESUMO Objetivo: Elaborar um protocolo específico para a avaliação e identificação precoces de lesões de pele em pacientes pediátricos com anemia falciforme submetidos ao transplante de células-tronco hematopoiéticas. Métodos: Estudo longitudinal, retrospectivo e descritivo. Foram revisados os prontuários dos pacientes pediátricos com anemia de Fanconi submetidos a transplante de células-tronco hematopoiéticas entre os anos de 2008 e 2018 no Hospital de Clínicas da Universidade Federal do Paraná, totalizando 136 pacientes. Foi criado um protocolo específico para a coleta de dados, que incluiu: idade, sexo, cor, idade ao diagnóstico da anemia de Fanconi, dados do transplante, história familiar de consanguinidade e complicações pré e pós-transplante. Além disso, foram verificados a presença de doença do enxerto contra o hospedeiro da pele e de outros órgãos, sua classificação, tipo de lesão, localização e, também, lesões de pele não relacionadas à doença. Resultados: Entre as manifestações de pele no período pré-transplante, destacaram-se as manchas café com leite (32,4%). Pelo menos um órgão foi afetado pela doença do enxerto contra o hospedeiro em 55,1% dos pacientes, sendo o acometimento mais comum o de boca, seguido pelo de pele. Exantema e eritema foram as manifestações cutâneas mais frequentemente observadas. Conclusões: Observou-se alta prevalência de manifestações cutâneas próprias da doença, bem como de manifestações cutâneas de doença do enxerto contra o hospedeiro. O protocolo elaborado reúne informações relevantes e padronizadas para o acompanhamento dos pacientes com anemia de Fanconi submetidos ao transplante, garantindo maior confiabilidade das informações, e sua implementação permitirá a avaliação prospectiva dos pacientes.

Cannabidiol for the treatment of refractory epilepsy in children: a critical review of the literature / Canabidiol para o tratamento de epilepsia refratária em crianças: uma revisão crítica da literatura

Abstract Objective: The aim of this current report was to present a critical review of the use of cannabidiol (CBD) in the treatment of refractory epilepsies in the pediatric population. Data source: Literature review was carried out in the Medline (PubMed), Cochrane, and Scientific Electronic Library Online (SciELO) databases with the descriptors "Cannabidiol" and "Epilepsy." The search was not limited by the date of publication, language, or study design. A total of 69 articles were included in the review. Data synthesis: The efficacy of CBD in treating epileptic seizures has been confirmed by randomized controlled trials for Lennox-Gastaut syndrome, Dravet syndrome, and tuberous sclerosis complex. The incidence of side effects reported in subjects of the studies is high. However, most studies indicate a good safety profile and tolerance to the drug, with most of the adverse effects being mild to moderate and transient. Conclusions: There is no consensus on the release of CBD as a therapeutic tool by the drug regulatory agencies worldwide. However, the use of CBD is promising since it has presented satisfactory results in crisis control in well-designed studies. In addition, this drug has a good safety and tolerance profile. However, further studies with a long follow-up period are needed to confirm its usefulness and the long-term safety in pediatric patients.

RESUMO Objetivo: Apresentar uma revisão crítica do uso de canabidiol no tratamento de epilepsias refratárias na população pediátrica. Fontes de dados: Revisão da literatura realizada nas bases de dados Medical Literature Analysis and Retrieval System Online (Medline/PubMed), Cochrane e Scientific Electronic Library Online (SciELO), com os descritores "cannabidiol" e "epilepsy". A busca não foi limitada por data de publicação, idioma ou desenho do estudo. Sessenta e nove artigos foram incluídos na revisão. Síntese dos dados: A eficácia do canabidiol no tratamento de crises epilépticas foi confirmada por ensaios clínicos randomizados para síndrome de Lennox-Gastaut, síndrome de Dravet e complexo de esclerose tuberosa. A incidência de efeitos colaterais relatados nos indivíduos dos estudos é alta. No entanto, a maioria dos estudos indica um bom perfil de segurança e tolerância ao medicamento, com efeitos adversos leves a moderados e transitórios. Conclusões: Não há consenso sobre a liberação do canabidiol como ferramenta terapêutica nas diversas agências reguladoras de medicamentos em todo o mundo. Entretanto, seu uso é promissor, uma vez que apresentou resultados satisfatórios no controle de crises em estudos bem delineados. Além disso, esse medicamento apresenta um bom perfil de segurança e tolerância. No entanto, mais estudos com longo período de acompanhamento são necessários para confirmar sua utilidade e sua segurança em longo prazo em pacientes pediátricos.

Cannabidiol for the treatment of refractory epilepsy in children: a critical review of the literature.

OBJECTIVE: The aim of this current report was to present a critical review of the use of cannabidiol (CBD) in the treatment of refractory epilepsies in the pediatric population. DATA SOURCE: Literature review was carried out in the Medline (PubMed), Cochrane, and Scientific Electronic Library Online (SciELO) databases with the descriptors "Cannabidiol" and "Epilepsy." The search was not limited by the date of publication, language, or study design. A total of 69 articles were included in the review. DATA SYNTHESIS: The efficacy of CBD in treating epileptic seizures has been confirmed by randomized controlled trials for Lennox-Gastaut syndrome, Dravet syndrome, and tuberous sclerosis complex. The incidence of side effects reported in subjects of the studies is high. However, most studies indicate a good safety profile and tolerance to the drug, with most of the adverse effects being mild to moderate and transient. CONCLUSIONS: There is no consensus on the release of CBD as a therapeutic tool by the drug regulatory agencies worldwide. However, the use of CBD is promising since it has presented satisfactory results in crisis control in well-designed studies. In addition, this drug has a good safety and tolerance profile. However, further studies with a long follow-up period are needed to confirm its usefulness and the long-term safety in pediatric patients.

Cutaneous disseminated sporotrichosis in immunocompetent patient: Case report and literature review.

Sporotrichosis is a global occurring implantation (subcutaneous) mycosis, caused by Sporothrix species, usually affecting the skin and the lymphatic vessels, from where it can disseminate. Nowadays, the vast majority of sporotrichosis infections in Brazil are caused by zoonotic transmission of S. brasiliensis which is the main etiologic agent. We report a cutaneous disseminated case of this disease, observed in an immunocompetent farmer living in southern Brazil, the Sporothrix schenckii-infection was successfully treated with itraconazole.

Use of sildenafil and L-arginine in an experimental rat model for the prevention of neonatal necrotizing enterocolitis.

Necrotizing enterocolitis (NEC) has a 45% mortality in neonatal intensive care units. This paper aimed to evaluate the isolated and combined effects of sildenafil and L-arginine in the prevention of necrotizing enterocolitis. Neonatal rats were fed formula milk and submitted to hypoxia under a 100% N2 atmosphere for 70 s. Then, animals were subjected to hypothermia (4 °C for 10 min), twice a day for 3 days. Forty neonatal rats were divided into five groups: negative control-not submitted to the protocol (n = 5), sildenafil group-NEC protocol (n = 9), L-arginine group-NEC protocol (n = 9), L-arginine and sildenafil group-NEC protocol (n = 9) and positive control-NEC protocol and intraperitoneal saline solution (n = 8). Jejunum and terminal ileus were removed for histopathologic and immunohistochemical Ki-67 analysis. Kruskal-Wallis test was used to analyze mortality, survival, body weight, intestinal injury score and Ki-67 proliferation index. All animals submitted to the protocol developed enterocolitis. Mortality rate was higher in group that received only L-arginine (p = 0.0293). The Ki-67 analysis showed a higher proliferative index in groups that received interventional drugs (p = 0.017). In conclusion, sildenafil and L-arginine were not effective to reduce intestinal injury.

FEMORAL HERNIA: UNCOMMON, BUT ASSOCIATED WITH POTENTIALLY SEVERE COMPLICATIONS.

BACKGROUND: Although the laparoscopic access is becoming the preferable treatment for femoral hernia, there are only few studies on this important subject. AIM: To assess the outcomes of the totally extraperitoneal laparoscopic (TEP) access in the treatment of femoral hernia. METHODS: Data of 62 patients with femoral hernia who underwent herniorrhaphy were retrospectively reviewed. The diagnosis of femoral hernia was established by clinical and/or imaging exams in 55 patients and by laparoscopic findings in seven. RESULTS: There were 55 (88.7%) females and 7 (11.3%) males, with female to male ratio of 8:1. The mean age was of 58.9±15.9 years, ranging from 22 to 92 years. Most patients (n=53; 85.5%) had single hernia and the remaining (n=9; 14.5%) bilateral, making a total of 71 hernias operated. Prior lower abdominal operations were recorded in 21 (33.9%) patients. Conversion to laparoscopic transabdominal preperitoneal procedure was performed in four (6.5%). Open herniorrhaphy was needed in two (3.2%), one with spontaneous enterocutaneous fistula in the groin region (Richter's hernia) and the another with incidental perforation of the adjacent small bowel that occurred during dissection of hernia sac. There was no mortality. CONCLUSION: Femoral hernia is uncommon, and it may be associated with potentially severe complications. Most femoral hernias may be successfully treated with totally extraperitoneal laparoscopic access, with low conversion and complication rates.

Femoral hernia: uncommon, but associated with potentially severe complications / Hérnia femoral: incomum, mas associada a complicações potencialmente graves

ABSTRACT Background: Although the laparoscopic access is becoming the preferable treatment for femoral hernia, there are only few studies on this important subject. Aim: To assess the outcomes of the totally extraperitoneal laparoscopic (TEP) access in the treatment of femoral hernia. Methods: Data of 62 patients with femoral hernia who underwent herniorrhaphy were retrospectively reviewed. The diagnosis of femoral hernia was established by clinical and/or imaging exams in 55 patients and by laparoscopic findings in seven. Results: There were 55 (88.7%) females and 7 (11.3%) males, with female to male ratio of 8:1. The mean age was of 58.9±15.9 years, ranging from 22 to 92 years. Most patients (n=53; 85.5%) had single hernia and the remaining (n=9; 14.5%) bilateral, making a total of 71 hernias operated. Prior lower abdominal operations were recorded in 21 (33.9%) patients. Conversion to laparoscopic transabdominal preperitoneal procedure was performed in four (6.5%). Open herniorrhaphy was needed in two (3.2%), one with spontaneous enterocutaneous fistula in the groin region (Richter's hernia) and the another with incidental perforation of the adjacent small bowel that occurred during dissection of hernia sac. There was no mortality. Conclusion: Femoral hernia is uncommon, and it may be associated with potentially severe complications. Most femoral hernias may be successfully treated with totally extraperitoneal laparoscopic access, with low conversion and complication rates.

RESUMO Racional: Embora o acesso laparoscópico esteja se tornando o tratamento preferencial para a hérnia femoral, poucos são os estudos sobre esse importante assunto. Objetivo: Avaliar os resultados do acesso laparoscópico totalmente extraperitoneal no tratamento da hérnia femoral. Métodos: Os dados de 62 pacientes com hérnia femoral que foram submetidos a herniorrafia foram revisados ​​retrospectivamente. O diagnóstico foi estabelecido por exames clínicos e/ou de imagem em 55 pacientes e por achados laparoscópicos em sete. Resultados: Havia 55 (88,7%) mulheres e 7 (11,3%) homens, com proporção feminino/masculino de 8: 1. A média de idade foi de 58,9±15,9 anos (22-92). A maioria (n=53, 85,5%) apresentava hérnia única e o restante (n=9, 14,5%) bilaterais, perfazendo um total de 71 hérnias femorais operadas. Operações prévias no abdome inferior foram registradas em 21 (33,9%) pacientes. A conversão para procedimento pré-peritoneal transabdominal laparoscópico foi realizada em quatro (6,5%). Herniorrafia aberta foi necessária em dois pacientes (3,2%), um com fístula enterocutânea espontânea na região da virilha (hérnia de Richter) e o outro com perfuração incidental do intestino delgado adjacente que ocorreu durante a dissecção do saco herniário. Não houve mortalidade. Conclusão: A hérnia femoral é incomum e pode estar associada a complicações potencialmente graves. A maioria das hérnias femorais pode ser tratada com sucesso através do acesso laparoscópico totalmente extraperitoneal, com baixas taxas de conversão e complicações.