Posterior fossa choroidplexus papilloma in the pediatric population: case series and literature review / Papiloma de plexo coroide na fossa posterior na população pediátrica: série de casos e revisão da literatura

Abstract Choroid plexus papillomas (CPPs) are rare benign neoplasms which are particularly uncommon in the posterior fossa in children. We herein present a case series of five patients treated at a tertiary care hospital. A comprehensive literature review was also carried out. The patients treated at the tertiary care hospital were aged between 4 and 16 years. Gross total resection (GTR) was initially achieved in two patients. All patients showed clinical improvement. Moreover, 27 articles published between 1975 and 2021 were selected for the literature review, totaling 46 patients; with the 5 patients previously described, the total sample was composed of 51 cases, With a mean age was 8.2 years. The lesions were located either in the fourth ventricle (65.3%) or the cerebellopontine angle (34.7%). Hydrocephalus was present preoperatively in 66.7% of the patients, and a permanent shunt was required in 31.6% of the cases. The GTR procedure was feasible in 64.5%, and 93.8% showed clinical improvement. For CPPs, GTR is the gold standard treatment and should be attempted whenever feasible, especially because the role of the adjuvant treatment remains controversial. Neuromonitoring is a valuable tool to achieve maximal safe resection. Hydrocephalus is common and must be recognized and promptly treated. Most patients will need a permanent shunt. Though there is still controversy on its efficacy, endoscopic third ventriculostomy is a safe procedure, and was the authors' first choice to treat hydrocephalus.

Resumo Os papilomas do plexo coroide (PPCs) são neoplasias benignas raras e, na população pediátrica, são particularmente incomuns na fossa posterior. Apresentamos uma série de casos de cinco pacientes atendidos em um hospital terciário. Além disso, foi realizada uma ampla revisão da literatura. Os pacientes atendidos no hospital terciário tinham entre 4 e 16 anos. Ressecção macroscópica total (RMT) foi inicialmente realizada em dois pacientes. Todos os pacientes apresentaram melhora clínica. Além disso, 27 artigos publicados entre 1975 e 2021 foram selecionados para a revisão da literatura, totalizando 46 pacientes. Somados à série de casos atuais, encontramos 51 pacientes, com média de idade de 8,2 anos. As lesões localizavam-se no quarto ventrículo (65,3%) ou no ângulo pontocerebelar (34,7%). Hidrocefalia estava presente no pré-operatório em 66,7% dos pacientes, e derivação ventricular permanente foi necessária em 31,6% dos casos. A RMT foi possível em 64,5%, e 93,8% tiveram melhora clínica. Para os CPPs, a RMT é o tratamento padrão-ouro e deve ser tentado sempre que possível, especialmente porque ainda existem controvérsias quanto ao papel do tratamento adjuvante. A neuromonitorização é uma ferramenta importante para se atingir a máxima ressecção segura. A hidrocefalia é comumente vista nesses pacientes e deve ser identificada e tratada. A maioria dos pacientes irá precisar de uma derivação permanente. Apesar de persistirem controvérsias sobre sua eficácia, a terceiro-ventriculostomia endoscópica foi a primeira escolha para tratar a hidrocefalia na experiência dos autores e é uma opção segura.

Clinical evolution of a severe asthmatics group in the use of immunobiological therapy in a Brazilian Public Hospital

SUMMARY OBJECTIVE: A small portion of the asthmatic population (3.6%) has severe asthma (SA), presenting high morbimortality rates and demanding more financial resources than other asthmatic populations. The use of immunobiological therapy is an effective tool in controlling symptoms, decreasing the number of exacerbations, and reducing the use of systemic corticosteroids in these patients. In Brazil, epidemiological data regarding this asthmatic population using immunobiologicals and their evolution are scarce. METHODS: This is an observational, analytical, cross-sectional, and retrospective study. The sample consisted of adult patients with SA in follow-up at the pulmonology service of the Complexo Hospital de Clínicas of the Federal University of Paraná, from January 2011 to August 2019. The analyzed variables were as follows: the number of exacerbations that required hospitalization in the previous year, forced expiratory volume in one second (FEV1), and asthma control test (ACT) scores before and after the start of immunobiological therapy. RESULTS: We studied 20 patients with SA using omalizumab or mepolizumab. We observed an increase in the mean ACT score of 4.8 points, a nonsignificant reduction in the number of exacerbations that required hospitalization, and a slight improvement in the FEV1. Regarding the patients using chronic systemic corticosteroid therapy, 14.2% (n=1) of patients had the medication discontinued and 57% (n=4) of patients had the dose reduced by half. CONCLUSION: The use of omalizumab and mepolizumab as additional therapy in SA provided a significant improvement in the ACT and allowed the dose reduction of systemic corticosteroids, without significant improvement in FEV1 and in the frequency of severe exacerbations.

Pain measurement as part of primary healthcare of adult patients with sickle cell disease

OBJECTIVE: The aim of this exploratory, cross-sectional study was to evaluate pain in sickle cell disease patients and aspects related to primary healthcare. METHODS: Data were obtained through home interviews. The assessment instruments (body diagram, Numerical Pain Scale, McGill Pain Questionnaire) collected information on the underlying disease and on pain. Data were analyzed using the Statistical Package for Social Sciences program for Windows. Associations between the subgroups of sickle cell disease patients (hemoglobin SS, hemoglobin SC, sickle β-thalassemia and others) and pain were analyzed using contingency tables and non-parametric tests of association (classic chi-square, Fisher's and Kruskal-Wallis) with a level of 5% (p-value < 0.05) being set for the rejection of the null hypothesis. RESULTS: Forty-seven over 18-year-old patients with sickle cell disease were evaluated. Most were black (78.7%) and female (59.6%) and the mean age was 30.1 years. The average number of bouts of pain annually was 7.02; pain was predominantly reported by individuals with sickle cell anemia (hemoglobin SS). The intensity of pain (Numeric Pain Scale) was 5.5 and the quantitative index (McGill) was 35.9. This study also shows that patients presented a high frequency of moderately painful crises in their own homes. CONCLUSION: According to these facts, it is essential that pain related to sickle cell disease is properly identified, quantified, characterized ...

Revascularização do miocárdio em paciente com situs inversus totalis / Myocardial revascularization in a patient with situs inversus totalis

Relatamos o caso de um paciente com situs inversus totalis associado a coronariopatia obstrutiva em artérias descendente anterior e posterior, coronária direita, primeiro ramo diagonal e ramo marginal esquerdo, quadro condizente com a intervenção de revascularização do miocárdio. Esse procedimento não é freqüente na literatura médica, sendo encontrado apenas um relato na literatura brasileira. A revascularização do miocárdio foi realizada com a artéria mamária interna direita para a artéria descendente anterior, e uma ponte de safena para coronária direita, marginal esquerda, primeiro ramo diagonal e descendente posterior. A cirurgia foi realizada com o auxílio de circulação extracorpórea.

We report the case of a patient with dextrocardia and situs inversus totalis associated with obstructive coronariopathy in the anterior and posterior descending arteries, right coronary artery, first diagonal branch and left marginal branch. The patient underwent coronary artery bypass grafting surgery. This surgery has been rarely reported in literature and we found only one similar case in the national medical literature. The myocardial revascularization was carried out with the right mammary artery for the anterior descending artery. The saphenous vein anastomosed the aorta to the right coronary artery, left marginal branch, fist diagonal branch and posterior descending artery. The surgery was performed with extracorporeal circulation.