Epidermólise bolhosa congênita: uma revisão de literatura / Congenital bullous epidermolysis: a literature review

Introdução. A epidermólise bolhosa (EB) engloba patologias distintas na forma de apresentação, mas que compartilham 3 particularidades: hereditariedade, fragilidade mecânica e formação de bolhas. A doença pode ser dividida em EB simples, juncional, distrófica e Síndrome de Kindler (SK). A gravidade da doença está relacionada ao grau de formação das bolhas e ao tipo de alteração gênica, sendo muito variável entre os subtipos da EB. A prevalência e a incidência deste agravo varia ao redor do mundo, ficando entre 20 a 41,3 e entre 10 a 32 casos por milhão de habitantes, respectivamente. Deve-se suspeitar desse diagnóstico quando o paciente apresentar histórico de formação de bolhas ou erosões recorrentes com início na infância - principalmente se há resposta anormal a traumas - e tem casos semelhantes na família. O diagnóstico deve ser por meio da biópsia de pele com avaliação por imunofluorescência; biópsia para mapeamento eletrônico de transmissão e análise genética do indivíduo. Deve-se atentar ao fato de que todo órgão revestido por tecido epitelial tem chances de ser acometido nas formas de maior gravidade. A doença não tem cura e não apresenta tratamento específico que altere a sua história natural. No entanto, deve-se focar na prevenção de bolhas e das possíveis infecções e no reparo das complicações extracutâneas a fim de possibilitar melhor qualidade de vida ao paciente. Objetivos. O objetivo desta revisão é compreender melhor a EB congênita, bem como identificar  os microorganismos mais prevalentes nas infecções cutâneas secundárias, além de enfatizar sobre as indicações e sobre como é feito o tratamento antimicrobiano para a EB, baseando-se em evidências científicas. Metodologia. Feita pesquisa na base de dados do PubMed com as palavras-chave “epidermolysis”, “bullosa” e “antibiotic”, filtrando resultados dos últimos 10 anos que tenham essas palavras no “abstract”, obtendo 59 resultados. Resultados. A gentamicina, um antibiótico da classe dos aminoglicosídeos, é  utilizada para tratar infecções por bactérias gram-negativas. Como na epidermólise bolhosa as lesões de pele estão frequentemente colonizadas por bactérias, este poderia ser um tratamento utilizado. Além deste benefício, um estudo mostrou que a administração de gentamicina em pacientes com EB distrófica recessiva induziu produção de colágeno tipo VII na junção da derme-epiderme. Alguns estudos relatam melhora do aspecto das lesões de pele pela percepção dos pais, mas sem melhora no ganho de peso ou no curso da doença. Já um estudo retrospectivo analisou biópsias de pele de pacientes com EB encontrando um predomínio de colonização por Staphylococcus aureus nestas amostras, seguida da presença de Pseudomonas aeruginosa e Streptococcus pyogenes, além do perfil de susceptibilidade destes microrganismos. Conclusão. Antibióticos tópicos ou sistêmicos podem ser utilizados por curtos períodos, seguindo critérios estabelecidos, para evitar resistência bacteriana e sensibilização. O tratamento não pode depender apenas dos antimicrobianos. Cuidados gerais também são importantes, a exemplo da troca regular dos curativos, da higienização da pele, a fim de prevenir infecções e a morbimortalidade. Medidas não farmacológicas apresentam resultado significativo no manejo das lesões, sendo parte do manejo do paciente

CARCINOMA ADRENAL EM CRIANÇAS: ESTUDO LONGITUDINAL EM MINAS GERAIS, BRASIL

RESUMO Objetivo: Analisar as características clínicas, laboratoriais e histopatológicas e o percurso até o estabelecimento do diagnóstico e do tratamento de pacientes com carcinoma de suprarrenal (CSR). Métodos: Estudo retrospectivo com 13 pacientes tratados no serviço de oncologia pediátrica do Hospital das Clínicas da Universidade Federal de Minas Gerais (HC-UFMG) entre 2004 e 2015. Resultados: A idade ao diagnóstico variou de 1,0 a 14,8 anos (mediana: 2,0 anos). As manifestações de hipercortisolismo foram identificadas em todos os casos, e as de virilização, em todas as meninas. Todos os pacientes preencheram os critérios de Weiss para diagnóstico histopatológico de CSR. A imuno-histoquímica foi realizada em 61,5% dos casos. A maioria dos pacientes apresentou doença em estádio I (76,9%). Todos foram submetidos à ressecção tumoral total. Dois pacientes (estádios III e IV) receberam quimioterapia associada ao mitotano. O único óbito observado foi do paciente com doença em estádio IV. A probabilidade de sobrevida global para todo o grupo aos 5,0 anos foi de 92,3±7,4%. A mediana de tempo entre o início dos sintomas e o diagnóstico foi de 9,5 meses, e de 6,0 meses entre a primeira consulta e o início do tratamento. Conclusões: A baixa idade ao diagnóstico, o predomínio de casos com doença localizada e a ressecção tumoral completa - com apenas um caso de ruptura de cápsula tumoral - são possivelmente a explicação para a evolução favorável da população estudada. O longo percurso entre o início dos sintomas e o diagnóstico sugere a importância da capacitação dos pediatras para o reconhecimento precoce dos sinais e dos sintomas do CSR

Mouthwashes used in patients with oral mucositis - A systematic review

The use of antineoplastic drugs causes different side effects, with oral and oropharyngeal mucositis being a frequent and serious adverse effect. Therefore, frequent attention has been given to a variety of rinses that have anti-inflammatory, antibacterial, healing activity, among others, which can be effective in the prevention and treatment of oral and oropharyngeal mucositis. Thus, this systematic review aims to verify, in the available scientific literature, evidence regarding the efficacy and safety of the clinical applicability of mouthwashes - chlorhexidine, benzydamine, allopurinol, and propolis - in the prevention and treatment of oral and oropharyngeal mucositis induced by chemotherapy and /or radiotherapy

Bloodstream infection in pediatric patients with febrile neutropenia induced by chemotherapy

Introduction: Febrile neutropenia (FN) is a serious complication of cancer chemotherapy. The present study aimed to identify risk factors for documented infection in pediatric patients with FN and cancer. Methods: This prospective cohort study included patients under 18 years from 2016 to 2018. Infection was defined according to the Centers for Disease Control and Prevention criteria. Results: A total of 172 febrile neutropenic episodes were evaluated. From univariate analysis, the risk factors were: female gender; monocyte count 90 mg/dl and hemoglobin 90mg/dl, fever onset and first blood culture with a positive result. The lowest probability of infection was related to first episode and to platelets > 50,000 at the onset of fever. Conclusion: A CRP > 90 at the onset of a febrile episode, platelets < 50,000, second episode or more, first fever episode during hospitalization and positive first blood culture were found to be associated with a higher risk of infection and they could be useful for the establishment of risk scores for infection in neutropenic children

Fluid overload: clinical outcomes in pediatric intensive care unit

Objective: The aim of this study was to analyze the effects of fluid overload related to mechanical ventilation, renal replacement therapy, and evolution to discharge or death in critically ill children. Methods: A retrospective study in a Pediatric Intensive Care Unit for two years. Patients who required invasive ventilatory support and vasopressor and/or inotropic medications were considered critically ill. Results: 70 patients were included. The mean age was 6.8 ± 6 years. There was a tolerable increase in fluid overload during hospitalization, with a median of 2.45% on the first day, 5.10% on the third day, and 8.39% on the tenth day. The median fluid overload on the third day among those patients in pressure support ventilation mode was 4.80% while the median of those who remained on controlled ventilation was 8.45% (p = 0.039). Statistical significance was observed in the correlations between fluid overload measurements on the first, third, and tenth days of hospitalization and the beginning of renal replacement therapy (p = 0.049) and between renal replacement therapy and death (p = 0.01). The median fluid overload was 7.50% in patients who died versus 4.90% in those who did not die on the third day of hospitalization (p = 0.064). There was no statistically significant association between death and the variables sex or age. Conclusions: The fluid overload on the third day of hospitalization proved to be a determinant for the clinical outcomes of weaning from mechanical ventilation, initiation of renal replacement therapy, discharge from the intensive care unit, or death among these children

Metástases pulmonares em crianças: estamos operando desnecessariamente?

RESUMO Objetivo: determinar, em pacientes pediátricos portadores de neoplasias malignas, as características de nódulos pulmonares identificados à tomografia computadorizada, capazes de diferenciar nódulos benignos de metástases. Métodos: estudo retrospectivo de pacientes submetidos a ressecções pulmonares de nódulos diagnosticados como metástases em um período de sete anos. Achados de tomografia e da cirurgia, assim como resultados dos exames anatomopatológicos foram comparados. Resultados: nove pacientes, submetidos a 11 intervenções cirúrgicas, foram estudados. Entre as variáveis estudadas, apenas o tamanho do nódulo, maior do que 12,5mm provou ser estatisticamente significante para predizer malignidade. Conclusão: esse estudo sugere que, entre as características tomográficas de nódulos pulmonares de crianças portadoras de neoplasias malignas, apenas o tamanho da lesão foi preditor de malignidade

Pulmonary metastases in children: are we operating unnecessarily?

<p></p><p>ABSTRACT Objective: to determine, in pediatric patients with malignant neoplasms, the characteristics of pulmonary nodules identified on computed tomography, as well as the possibility of differentiating benign lesions from metastases. Methods: we conducted a retrospective study of patients submitted to pulmonary resections of nodules diagnosed as metastases in a period of seven years. We compared computed tomography and surgery findings, as well as results of anatomopathological examinations. Results: we studied nine patients submitted to 11 surgical interventions. Among the studied variables, only nodule size greater than 12.5mm proved to be statistically significant to predict malignancy. Conclusion: among the tomographic characteristics of pulmonary nodules in children with malignant neoplasms, only the size of the lesion was a predictor of malignancy.</p><p></p

International consensus on mitotane treatment in pediatric patients with adrenal cortical tumors: indications, therapy, and management of adverse effects

Objective: Mitotane is an important cornerstone in the treatment of pediatric adrenal cortical tumors (pACC), but experience with the drug in the pediatric age group is still limited and current practice is not guided by robust evidence. Therefore, we have compiled international consensus statements from pACC experts on mitotane indications, therapy, and management of adverse effects. Methods: A Delphi method with 3 rounds of questionnaires within the pACC expert consortium of the international network groups European Network for the Study of Adrenal Tumors pediatric working group (ENSAT-PACT) and International Consortium of pediatric adrenocortical tumors (ICPACT) was used to create 21 final consensus statements. Results: We divided the statements into 4 groups: environment, indications, therapy, and adverse effects. We reached a clear consensus for mitotane treatment for advanced pACC with stages III and IV and with incomplete resection/tumor spillage. For stage II patients, mitotane is not generally indicated. The timing of initiating mitotane therapy depends on the clinical condition of the patient and the setting of the planned therapy. We recommend a starting dose of 50 mg/kg/d (1500 mg/m2/d) which can be increased up to 4000 mg/m2/d. Blood levels should range between 14 and 20 mg/L. Duration of mitotane treatment depends on the clinical risk profile and tolerability. Mitotane treatment causes adrenal insufficiency in virtually all patients requiring glucocorticoid replacement shortly after beginning. As the spectrum of adverse effects of mitotane is wide-ranging and can be life-threatening, frequent clinical and neurological examinations (every 2-4 weeks), along with evaluation and assessment of laboratory values, are required. Conclusions: The Delphi method enabled us to propose an expert consensus statement, which may guide clinicians, further adapted by local norms and the individual patient setting. In order to generate evidence, well-constructed studies should be the focus of future efforts

International consensus on mitotane treatment in pediatric patients with adrenal cortical tumors: indications, therapy, and management of adverse effects.

OBJECTIVE: Mitotane is an important cornerstone in the treatment of pediatric adrenal cortical tumors (pACC), but experience with the drug in the pediatric age group is still limited and current practice is not guided by robust evidence. Therefore, we have compiled international consensus statements from pACC experts on mitotane indications, therapy, and management of adverse effects. METHODS: A Delphi method with 3 rounds of questionnaires within the pACC expert consortium of the international network groups European Network for the Study of Adrenal Tumors pediatric working group (ENSAT-PACT) and International Consortium of pediatric adrenocortical tumors (ICPACT) was used to create 21 final consensus statements. RESULTS: We divided the statements into 4 groups: environment, indications, therapy, and adverse effects. We reached a clear consensus for mitotane treatment for advanced pACC with stages III and IV and with incomplete resection/tumor spillage. For stage II patients, mitotane is not generally indicated. The timing of initiating mitotane therapy depends on the clinical condition of the patient and the setting of the planned therapy. We recommend a starting dose of 50 mg/kg/d (1500 mg/m²/d) which can be increased up to 4000 mg/m2/d. Blood levels should range between 14 and 20 mg/L. Duration of mitotane treatment depends on the clinical risk profile and tolerability. Mitotane treatment causes adrenal insufficiency in virtually all patients requiring glucocorticoid replacement shortly after beginning. As the spectrum of adverse effects of mitotane is wide-ranging and can be life-threatening, frequent clinical and neurological examinations (every 2-4 weeks), along with evaluation and assessment of laboratory values, are required. CONCLUSIONS: The Delphi method enabled us to propose an expert consensus statement, which may guide clinicians, further adapted by local norms and the individual patient setting. In order to generate evidence, well-constructed studies should be the focus of future efforts