Naso-ethmoidal phosphaturic mesenchymal tumor: a rare tumor site for an uncommon paraneoplastic syndrome

Mesenchymal Phosphaturic Tumors (MPTs) are the most common cause of an uncommon paraneoplastic syndrome known as Tumor Induced Osteomalacia (TIO). They typically occur in soft tissues and bone and in less than 5% in the head and neck region where the naso-ethmoidal is rarely involved. The presentation of the case includes also the analysis of the expression by RT-PCR of two “phosphatonins” that are known to be involved in the development of the syndrome. For the rarity of MPTs in the head and neck, otolaryngologists and maxillofacial surgeons might not be familiar with these tumors and with TIO whose knowledge is mandatory for the appropriate clinical work-up and treatment of the affected patients

Desmoplastic Melanoma: Report of 5 Cases

Background. The clinical presentation of desmoplastic melanoma is often challenging. We report the experience of the Melanoma Unit of Spedali Civili University Hospital of Brescia, Italy. Method. Study subjects were drawn from 1770 patients with histologica confirmed melanoma. Within this group, desmoplastic melanoma developed in 5 patients. For each diagnosed melanoma, histological characteristics, treatment, and outcomes were evaluated. Results. Of the 5 patients described in this study, 2 were males and 3 females. The average age was 62.4 years ranging from 56 to 68 years. Breslow thickness ranged from 2.1 to 12 mm with a mean thickness of 5.8 mm. Primary treatment of 5 patients included a wide local excision of their primary lesions. Conclusions. Desmoplastic melanoma is a rare neoplasm which clinically may mimic other tumours or cutaneous infiltrate of uncertain significance. The diagnosis is hiastopathological and radical resection is necessary

The Mediating Role of Physical Inactivity on the Relationship between Inflammation and Artery Thickness in Prepubertal Adolescents

Objective: To analyze the relationship between inflammatory markers and the lipid profile, blood flow, and artery structure in prepubertal adolescents stratified according to sports practice.Study design The sample was composed of 120 adolescents (57 boys and 63 girls) with a mean age of 11.7 +/- 0.7 years (ranging from 11 to 13 years). Intima-media thickness (IMT) and blood flow were measured with ultrasonography. The lipid profile and high-sensitivity C-reactive protein were measured after the subjects had fasted for 12 hours overnight. Trunk fatness was measured by dual-energy x-ray absorptiometry. Organized sports participation was analyzed as a categorical variable. Biological maturation was determined via the age at peak height velocity. Results: In the adjusted model, high-sensitivity C-reactive protein was significantly related to high-density lipoprotein-cholesterol (beta = -5.797 [-11.500 to -0.093]), femoral IMT (beta = 0.062 [0.008-0.116]), and the sum of femoral and carotid IMT (beta = 1.107 [0.223-1.919]), but only in the group without sports participation. Slopes of the crude linear regression were greater in the group without sports participation for femoral IMT (t = 2.621; P = .009) and the sum of femoral and carotid IMT (t = 2.876; P = .004) when compared with the group with sports participation. Conclusion: Independent of body fatness and biological maturation, inflammatory status was related to artery IMT and dyslipidemia in prepubertal adolescents, modulated by sport participation.Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq

Description of hospital pharmacy management practice

Health institutions, particularly hospitals, are characterized as complex structures that need managers with a global view of the institution and its relations with the external environment. The hospital pharmacy is a strategic unit, which cooperates with the institutional management and integrates the multiprofessional team in the process related to the acquisition, provision and control of essential inputs for the inpatient care process. The objective in this study is to demonstrate the applicability, in the context of hospital-based health, of a performance measuring system at the pharmacy. Method: A descriptive and longitudinal study was undertaken on the evolution of the key indicator Absence Rate of Standardized Drugs in inpatient care between March 2004 and December 2013. This indicator was employed to monitor the impact of changes the Pharmacy Division has been implementing, as the first step of the pharmaceutical care cycle in the model of the process-based managed approach at a public university hospital. Qualitative data collection methods were used, including observation and documentary analysis, as well as quantitative data collection. Results: After the application of the model, one point of change in the key performance indicator was detected in the tenth month, when the process-based management model was implemented at the pharmacy. Conclusions: The process-based management approach was effective for the hospital pharmacy. The premise adopted is that the administrative changes (interferences), focused on the improvement of the processes and the selection and monitoring of indicators, influence the processes, reducing the variability and improving the qualityAs instituições de saúde, especialmente hospitais, são caracterizadas como estruturas complexas que precisam de administradores com uma visão global da instituição e de suas relações com o ambiente externo. A farmácia hospitalar é uma unidade estratégica, que colabora com a administração institucional e integra a equipe multiprofissional no processo que tange a aquisição, provisão e controle de insumos essenciais para o processo do atendimento do paciente internado. Este estudo tem como objetivo demonstrar a aplicabilidade, no contexto da saúde hospitalar, de um sistema de medição de desempenho da farmácia. Método: Trata-se de um estudo descritivo, longitudinal, sobre a evolução do indicador chave Taxa de Falta de Medicamentos Padronizados na assistência do paciente internado, no período de março de 2004 a dezembro de 2013. Esse indicador foi empregado para monitoramento do impacto de mudanças que a Divisão de Farmácia vem implementando, como primeira etapa do ciclo de assistência farmacêutica dentro do modelo da abordagem de gestão por processos, em um hospital público universitário. Foram usados métodos de coleta de dados qualitativos, incluindo a observação e análise documental, bem como coleta de dados quantitativos. Resultados: Após a aplicação do modelo, um ponto de mudança no indicador chave de desempenho, foi detectado no 10º mês, quando o modelo de gestão baseado em processo foi implementado na farmácia. Conclusões: A abordagem de gestão baseada em processos foi eficaz para a farmácia hospitalar. A premissa adotada é que as mudanças administrativas (interferências), com foco na melhoria dos processos e seleção e acompanhamento de indicadores, têm influencia sobre os processos, reduzindo a variabilidade e melhoria da qualidad

Sinonasal mucosal melanoma: Molecular profile and therapeutic implications from a series of 32 cases

BACKGROUND: Primary sinonasal mucosal melanomas are aggressive tumors with a poor clinical control by current treatments, raising the urgent need of novel strategies. METHODS: By fluorescence in situ hybridization (FISH), direct sequencing, and immunohistochemistry, we investigate the spectrum of molecular abnormalities in a cohort of 32 cases of primary sinonasal mucosal melanomas. RESULTS: We found that all primary sinonasal mucosal melanomas lack BRAF V600E mutation; in addition, they are characterized by somatic mutations of NRAS (22%) and KIT (12.5%), together with amplification of RREB1 (100%) and loss of MYB (76%). The large majority of cases showed KIT protein expression (96.9%). Among tumor suppressor genes, primary sinonasal mucosal melanomas showed loss of PTEN (48.1%) and p16/INK4a (55.2%). All tested cases showed expression of pAkt and pErk, suggesting a combined activation of PI3K/Akt and RAS-mitogen-activated protein kinase (MAPK) pathways. CONCLUSIONS: This molecular fingerprint strongly argues against the clinical efficacy of BRAF-inhibitors, but could candidate primary sinonasal mucosal melanomas to therapeutic strategies targeting RAS and KIT mutations or inhibiting PI3K-Akt-mTOR pathway

Expectativas da equipe da unidade de terapia intensiva pediátrica quanto à atuação do farmacêutico clínico

Objetivo: O objetivo do estudo foi conhecer as expectativas da equipe da unidade de terapia intensiva pediátrica quanto às atividades a serem realizadas pelo farmacêutico clínico a fim de planejar seu trabalho junto com a equipe multidisciplinar. Desenho do estudo: Estudo transversal, envolvendo a equipe multidisciplinar da unidade de terapia intensiva pediátrica de um hospital universitário terciário. Método: Um questionário autopreenchível contendo perguntas sobre as expectativas da equipe da unidade de terapia intensiva pediátrica em relação ao papel do farmacêutico clínico na equipe multidisciplinar foi aplicado aos membros da equipe, utilizando a escala de resposta do tipo Likert. Resultados: Os auxiliares/ técnicos de enfermagem e fisioterapeutas mostraram maior expectativa em relação a questões relacionadas com a administração de medicamentos; para enfermeiros, a expectativa sobre o desempenho do farmacêutico clínico focou-se em questões relacionadas à prescrição de medicações, como a avaliação da antibioticoterapia, enquanto os médicos consideraram mais importantes informações sobre a terapia medicamentosa, como a notificação de alergias relacionadas às drogas, informações sobre o aprazamento de medicamentos e avaliação da administração de drogas por sonda gástrica ou enteral. Conclusões: As expectativas da equipe variaram de acordo com as necessidades e a rotina de cada categoria profissional. Os resultados deste estudo têm contribuído para a implantação do serviço de farmácia clínica em uma unidade de terapia intensiva pediátrica brasileiraObjective: We aimed to know the expectations of a pediatric intensive care unit staff for the activities to be undertaken by the clinical pharmacist, in order to plan her work along with the multidisciplinary team. Study design: A cross-sectional study, involving the multidisciplinary team of the pediatric intensive care unit of a tertiary-care university hospital. Method: A self-completion questionnaire containing questions regarding expectations of the pediatric intensive care unit staff for the role of the clinical pharmacist in the multidisciplinary team was applied to the team members, using a Likert-type scale response. Results: The nursing assistants/technicians and physiotherapists showed higher expectation for issues related to administration of medications; for nurses, the expectation for the clinical pharmacist performance focused on issues related to prescription drugs, such as evaluation of antibiotic therapy, while physicians considered most important information on drug therapy, as notification of allergies related to drugs, information on period of validity of medicines and evaluation of drug administration by gastric or enteral tubes. Conclusions: Team expectations varied according to the needs and routine of each professional category. The results of this study have contributed to implementation of the clinical pharmacy service in a Brazilian pediatric intensive care uni

Sinonasal mucosal melanoma: Molecular profile and therapeutic implications from a series of 32 cases

BACKGROUND: Primary sinonasal mucosal melanomas are aggressive tumors with a poor clinical control by current treatments, raising the urgent need of novel strategies. METHODS: By fluorescence in situ hybridization (FISH), direct sequencing, and immunohistochemistry, we investigate the spectrum of molecular abnormalities in a cohort of 32 cases of primary sinonasal mucosal melanomas. RESULTS: We found that all primary sinonasal mucosal melanomas lack BRAF V600E mutation; in addition, they are characterized by somatic mutations of NRAS (22%) and KIT (12.5%), together with amplification of RREB1 (100%) and loss of MYB (76%). The large majority of cases showed KIT protein expression (96.9%). Among tumor suppressor genes, primary sinonasal mucosal melanomas showed loss of PTEN (48.1%) and p16/INK4a (55.2%). All tested cases showed expression of pAkt and pErk, suggesting a combined activation of PI3K/Akt and RAS-mitogen-activated protein kinase (MAPK) pathways. CONCLUSIONS: This molecular fingerprint strongly argues against the clinical efficacy of BRAF-inhibitors, but could candidate primary sinonasal mucosal melanomas to therapeutic strategies targeting RAS and KIT mutations or inhibiting PI3K-Akt-mTOR pathway

Sentinel lymph node surgery after neoadjuvant chemotherapy in patients with T2 to T4, N0 and N1 breast cancer

Abstract Background Histological status of axillary lymph nodes is an important prognostic factor in patients receiving surgery for breast cancer (BC). Sentinel lymph node (SLN) biopsy (B) has rapidly replaced axillary lymph node dissection (ALND), and is now the standard of care for axillary staging in patients with clinically node-negative (N0) operable BC. The aim of this study is to compare pretreatment lymphoscintigraphy with a post primary systemic treatment (PST) scan in order to reduce the false-negative rates for SLNB. Methods In this single-institution study we considered 170 consecutive T2-4 N0-1 M0 BC patients treated with anthracycline-based PST. At the time of incisional biopsy, we performed sentinel lymphatic mapping. After PST, all patients repeated lymphoscintigraphy with the same methodology. During definitive surgery we performed further sentinel lymphatic mapping, SLNB and ALND. Results The SLN was removed in 158/170 patients giving an identification rate of 92.9% (95% confidence interval (CI) = 88.0–96.3%) and a false-negative rate of 14.0% (95% CI = 6.3–25.8%). SLNB revealed a sensitivity of 86.0% (95% CI = 74.2–93.7%), an accuracy of 94.9% (95% CI = 90.3–97.8%) and a negative predictive value of 92.7% (95% CI = 86.1–96.8%). Conclusion Identification rate, sensitivity and accuracy are in accordance with other studies on SLNB after PST, even after clinically negative node conversion following PST. This study confirms that diagnostic biopsy and neoadjuvant chemotherapy maintain breast lymphatic drainage unaltered

Splenic marginal zone lymphoma: a prognostic model for clinical use.

The Integruppo Italiano Linfomi (IIL) carried out a study to assess the outcomes of splenic marginal zone lymphoma and to identify prognostic factors in 309 patients. The 5-year cause-specific survival (CSS) rate was 76%. In univariate analysis, the parameters predictive of shorter CSS were hemoglobin levels below 12 g/dL (P < .001), albumin levels below 3.5 g/dL (P = .001), International Prognostic Index (IPI) scores of 2 to 3 (P < .001), lactate dehydrogenase (LDH) levels above normal (P < .001), age older than 60 years (P = .01), platelet counts below 100 000/μL (P = .04), HbsAg-positivity (P = .01), and no splenectomy at diagnosis (P = .006). Values that maintained a negative influence on CSS in multivariate analysis were hemoglobin level less than 12 g/dL, LDH level greater than normal, and albumin level less than 3.5 g/dL. Using these 3 variables, we grouped patients into 3 prognostic categories: low-risk group (41%) with no adverse factors, intermediate-risk group (34%) with one adverse factor, and high-risk group (25%) with 2 or 3 adverse factors. The 5-year CSS rate was 88% for the low-risk group, 73% for the intermediate-risk group, and 50% for the high-risk group. The cause-specific mortality rate (x 1000 person-years) was 20 for the low-risk group, 47 for the intermediate-risk group, and 174 for the high-risk group. This latter group accounted for 54% of all lymphoma-related deaths. In conclusion, with the use of readily available factors, this prognostic index may be an effective tool for evaluating the need for treatment and the intensity of therapy in an individual patient. © 2006 by The American Society of Hematology