Effects of zoledronic acid therapy in fibrous dysplasia of bone: a single-center experience

ABSTRACT Objective: Fibrous dysplasia (FD) is a rare bone disorder that can involve any part of the skeleton, leading to bone pain, deformities, and fractures. Treatment with intravenous bisphosphonates has been used with variable results. Therefore, we aimed to evaluate the effects of zoledronic acid (ZA) therapy in patients with monostotic or polyostotic FD. Subjects and methods: The medical records of thirteen patients with FD evaluated between 2015 and 2020 were retrospectively analyzed. In the subgroup of patients treated with ZA (n = 7), data on pain relief, changes in bone turnover markers (BTMs), and adverse events following ZA infusions were retrieved. Moreover, radiological changes in response to treatment were recorded in patients who underwent radiological follow-up. Results: Of the patients, 5 (38%) presented with monostotic whereas 8 (62%) had polyostotic FD. Bone pain was a common finding (69%), and most patients (62%) exhibited elevated baseline BTMs. Partial or complete pain relief was reported in 6 of 7 patients treated with ZA. BTMs, especially C-telopeptide of type I collagen (CTX), significantly decreased after therapy (change rate: −61.8% [IQR −71, −60%]), and median CTX levels were significantly lower than at baseline (0.296 ng/mL [0.216, 0.298] vs. 0.742 ng/mL [0.549, 0.907], respectively; P = 0.04). No radiological improvement was observed in cases with radiological follow-up (n = 3). No serious adverse effects of ZA were reported. Conclusion: ZA treatment was well tolerated and provided beneficial effects in relieving bone pain and reducing BTMs, especially CTX. Our data reinforce the role of ZA in the treatment of FD-related bone pain.

Frequência de alterações espirométricas, aprisionamento aéreo e hiperinsuflação pulmonar em crianças e adolescentes com asma grave resistente à terapia: um estudo piloto / Frequency of spirometric changes, air trapping, and lung hyperinflation in children and adolescents with therapy-resistant severe asthma: a pilot study

Objetivos: avaliar a frequência de alterações espirométricas e pletismográficas em crianças e adolescentes com asma grave resistente à terapia (AGRT). Além disso, testaram-se possíveis associações entre esses desfechos. Métodos: trata-se de um estudo retrospectivo, no qual foram incluídas crianças e adolescentes (6-18 anos), com diagnóstico de AGRT, e que se encontravam em acompanhamento ambulatorial regular. Todos deveriam possuir informações antropométricas (peso, altura, índice de massa corporal), demográficas (idade, etnia e sexo), clínicas (teste cutâneo, teste de controle da asma, tabagismo familiar e medicações em uso) e de função pulmonar (espirometria e pletismografia corporal) registradas no banco de dados do serviço. Os testes de função pulmonar seguiram as recomendações das diretrizes nacionais e internacionais. Para fins estatísticos, utilizou-se análise descritiva e o teste de qui-quadrado de Pearson. Resultados: de um total de 15 pacientes com AGRT, 12 deles foram incluídos na amostra. A média de idade foi de 12,2 anos, com predomínio do sexo feminino (66,7%). Destes, 50,0% apresentaram a doença controlada, 83,3% foram considerados atópicos e 50,0% tinham histórico de tabagismo familiar. Em relação aos testes de função pulmonar (% do previsto), as médias dos parâmetros espirométricos e de plestismografia corporal encontraram-se dentro dos limites inferiores da normalidade. Apenas 16,7% da amostra apresentou espirometria alterada (130,0%) e 16,7% hiperinsuflação pulmonar (capacidade pulmonar total>120,0%). Houve frequência estatisticamente maior (p=0,045) de aprisionamento aéreo nos participantes com espirometria alterada, em comparação à espirometria normal. Contudo, não se observou diferença (p=0,341) em relação à hiperinsuflação pulmonar. Conclusões: os achados demonstraram pouco comprometimento espirométrico e dos volumes e das capacidades pulmonares em crianças e adolescentes com AGRT. Além disso, aqueles participantes com espirometria alterada obtiveram frequência maior de aprisionamento aéreo no exame de pletismografia corporal

Aims: to assess the frequency of spirometric and plethysmographic changes in children and adolescents with severe therapy-resistant asthma (SRTA). In addition, possible associations between these outcome were tested. Methods: this is a retrospective study. Children and adolescents (6-18 years old), diagnosed with SRTA and who were in regular outpatient follow-up were included. Everyone should have anthropometric (weight, height, body mass index), demographic (age, ethnicity and gender), clinical (skin test, asthma control test, family smoking and medications in use) and pulmonary function (spirometry and body plethysmography) recorded in the service's database. Pulmonary function tests followed the recommendations of national and international guidelines. For statistical purposes, descriptive analysis and Pearson's chi-square test were used. Results: from a total of 15 patients with SRTA, 12 of them were included in the sample. The average age was 12.2 years, with a predominance of females (66.7%). Of these, 50.0% had the disease under control, 83.3% were considered atopic, and 50.0% had a family history of smoking. Regarding the pulmonary function tests (% of predicted), the means of spirometric parameters and body plestismography were within the lower limits of normality. Only 16.7% of the sample had altered spirometry (<5th percentile), 25.0% air trapping (residual volume>130.0%) and 16.7% pulmonary hyperinflation (total lung capacity>120.0%). There was a statistically higher frequency (p=0.045) of air trapping in participants with altered spirometry, compared to normal spirometry. However, there was no difference (p=0.341) in relation to pulmonary hyperinflation. Conclusions: the findings demonstrated little impairment of spirometry and lung volumes and capacities in children and adolescents with AGRT. In addition, those participants with altered spirometry had a higher frequency of air trapping in the body plethysmography exam.

Aprepitant plus palonosetron for the prevention of postoperative nausea and vomiting after breast cancer surgery: a double blind, randomized trial

OBJECTIVES: To evaluate the addition of a fourth antiemetic intervention in patients at high risk for postoperative nausea and vomiting (PONV). METHODS: High-risk patients (Apfel score 3 or 4) scheduled for unilateral mastectomy were randomly allocated in one of two groups, oral aprepitant (oral aprepitant 80 mg, intravenous dexamethasone 8 mg, and palonosetron 0.075 mg) and oral placebo (oral placebo, intravenous dexamethasone 4 mg, and palonosetron 0.075 mg). Patients and caregivers were blinded to the group assignments. The primary efficacy endpoints included the incidence of nausea and vomiting, and the secondary endpoints included use of rescue antiemetics during a 48-hour postoperative period. ClinicalTrials.gov: NCT02431286. RESULTS: One hundred patients were enrolled in this study and 91 were analyzed, 48 in group A and 43 in group P. No patient presented with nausea or vomiting in the first 2 hours after surgery. From the 2nd to the 6th hour, the incidence of PONV was 8.33% in group A and 9.30% in group P. In the first 24 hours, the incidence of PONV was 27.08% in the group A and 20.93% in group P. From the 24th to the 48th hour, the incidence of PONV was 8.33% in group A and 13.95% in group P. There were no statistically significant differences in PONV between groups. CONCLUSION: The addition of aprepitant as a third antiemetic resulted in no significant reduction in the incidence of PONV in this population. However, the incidence of PONV was reduced in relation to the general population.

Dieta hiperproteica e função renal: discutindo seus efeitos em adultos normais / High-protein diet and kidney disease: a discussion on effects in regular adults

OBJETIVOS: O objetivo deste estudo é revisar e atualizar a literatura sobre os efeitos de uma dieta hiperprotéica em comparação com uma dieta hipoprotéica na função renal de indivíduos adultos previamente hígidos. MÉTODOS: Foram utilizadas as bases digitais Pubmed/Medline e Lilacs durante o mês de maio de 2018. Os termos utilizados foram "Diet, High-protein AND Kidney Disease", ambos Mesh Terms, sendo pesquisados artigos em língua portuguesa e em língua inglesa. Os critérios de inclusão utilizados selecionaram adultos saudáveis em uso de dieta hiperproteica, com função renal preservada ou minimamente alterada. Estudos que não preenchessem esses critérios, bem como artigos que abordaram grupos de neonatos, crianças e adolescentes, não foram selecionados. RESULTADOS: Foram selecionados 11 estudos. Analisamos os efeitos da ingestão aumentada de proteínas na Taxa de Filtração Glomerular (TGF), da pressão arterial e do metabolismo do cálcio e da ureia. O conteúdo das revisões dos estudos conflagra um aumento dos níveis de vasopressina, combinado à elevação da Taxa de Filtração Glomerular, da excreção de cálcio na urina e dos níveis séricos de ureia. Não foram encontradas evidências significativas quanto a risco de lesões renais. CONCLUSÕES: De acordo essa revisão, não foram encontradas evidências de que uma dieta com alto teor protéico possa acarretar prejuízo na função renal a curto ou médio prazo, em indivíduos normais.

AIMS: This study aims to review and atualize the literature about the effects on renal function comparing a high-protein diet and a low-protein diet in healthy adults. METHODS: Pubmed/Medline and Lilacs were the digital databases in which the search was performed during May 2018. The Mesh terms used here were "Diet, High-protein AND Kidney Disease" and the articles chosen were either in portuguese or english language. There were only included studies related to hiperproteic diets in healthy human adults with null or negative outcomes towards renal functionality. Studies that did not meet this criteria, such as articles that approached newborn, children or teenagers, were not picked up. RESULTS: We picked 11 studies. We analyzed the effects of high-protein diets in the Glomerular Filtration Rate (GFR), blood pressure and in urea and calcium metabolism. The review content of the studies could display an increase of vasopressin levels, as well as Glomerular Filtration Rate, excretion of calcium and serum level of urea. There were not identified significant evidence about risk of renal lesions. CONCLUSIONS: According to this review, any evidence that a high-protein diet is capable of causing renal impairment at short and medium term in healthy individuals could not be found.

Improving idiophatic orthostatic hypotension in a patient with multiple system atrophy (Shy-Drager syndrome) with cardiac pacemaker implantation: case report / Melhora da hipotensão ortostática em um paciente com atrofia de múltiplos sistemas (syndrome de Shy-Drager) com implantação de marca-passo cardíaco: relato de caso

Orthostatic hypotension is a frequent symptom in patients with multiple system atrophy and it has an important impact on their quality of life. We report a case of idiopathic orthostatic hypotension, in a patient with multiple system atrophy (Shy-Drager syndrome), treated with pacemaker implantation resulting in a substantial improvement in the quality of his life.

Hipotensão ortostática é um sintoma freqüente em pacientes com atrofia de múltiplos sistemas e tem importante impacto na sua qualidade de vida. Relatamos um paciente com hipotensão ortostática idiopática e atrofia de múltiplos sistemas (Síndrome de Shy-Drager), tratado com implantação de marca-passo resultando numa evidente melhora na qualidade de vida.

Percepção médica quanto aos protocolos clínicos / Physician perception of clinical protocols

Os protocolos clínicos são condutas e procedimentos desenvolvidos com suporte em evidências atualizadas e consistentes, que objetivam promover uma melhor prática da medicina. Esses protocolos vêm auxiliar o médico em decisões sobre a melhor e mais apropriada conduta em situações clínicas específicas, permitindo resolução mais rápida e eficiente das enfermidades, gerando melhor qualidade de vida aos pacientes. Neste sentido, os guidelines buscam aumento da precisão diagnóstica, qualidade da assistência médica, dos serviços de saúde e controle de custos. Em estudo, verificou-se a percepção médica em relação ao uso de protocolos, no tocante às vantagens e limitações da prática, principais beneficiados, influência nos custos e possível diferença entre os protocolos adotados nos sistemas de saúde. Aplicou-se questionário contendo nove perguntas, respondidas individualmente e voluntariamente por 80 médicos. Constatou-se que 98,75% dos médicos tem conhecimento do uso de protocolos; 95% concordam com seu uso, sendo que 83,75% dos médicos o fazem parcialmente. Os entrevistados reconheceram as limitações de sua aplicabilidade. Dentre eles, 38,75% consideram que o julgamento clínico é mais importante, e 72,5% avaliam que os protocolos trazem benefícios, sendo que 77,5% reconhecem a diminuição nos custos. Concluiu-se que, apesar de haver limitações na aplicabilidade deste recurso, o conhecimento e uso dos protocolos por parte dos médicos já são significativos, sendo, portanto, instrumentos de auxílio em um contexto, no qual a experiência clínica deve ser integrada à informação científica, de forma crítica e racional, objetivando melhorar a qualidade da assistência médica. Desta forma, os protocolos devem ser amplamente divulgados, constantemente atualizados e adequados à realidade de cada paciente.

Clinical protocols are evidence-based sets of procedures and approaches aiming to improve medical practice and inform clinical decision-taking. With a body of information about better and more appropriate approaches to specific clinical situations, clinical protocols allow for quicker and more efficient responses to disease to be made, while providing patients with better quality of life. Guidelines seek to increase diagnostic accuracy, medical care quality, health services quality and cost control. The study investigated the following items regarding medical protocols: advantages and limitations as seen by physicians; their main beneficiaries; economic impact; and possible differences among adopted protocols. A nine-question questionnaire was answered individually and voluntarily by 80 physicians. As a result it was observed that 98.75% were aware of protocol use; 95% agreed with the use of protocols, of whom 83.75% did it partially; those interviewed recognized the limitations of protocol applicability; 38.75% considered clinical judgment to be paramount; 72.5% believed the protocols to be beneficial; 77.5% recognized cost reduction with their use. In spite of limitations to their applicability, knowledge about and use of clinical protocols by physicians are already significant. Because clinical protocols are helpful tools to improve medical care, in a context in which clinical experience must be critically and rationally integrated with scientific information, they must be widely spread, constantly updated and adapted to each patient`s reality.