Tumores indutores de osteomalácia: diagnóstico, caracterização tumoral e avaliação evolutiva em longo prazo de nove pacientes

INTRODUÇÃO: Tumores indutores de osteomalácia (TIOs) são raros, geralmente apresentam origem mesenquimal, têm produção excessiva de fosfatoninas sendo a mais comum o FGF23 (Fibroblast Growth Factor 23) que, em níveis elevados, provoca osteomalácia hipofosfatêmica. A cura dos TIOs envolve a remoção completa do tumor, o que torna essencial sua localização. OBJETIVOS: (1) caracterizar nove pacientes com TIO ao diagnóstico e avaliá-los evolutivamente em longo prazo; (2) avaliar a eficácia da cintilografia com Octreotida (Octreoscan®) e a da cintilografia de corpo inteiro com Mibi (MIBI) na detecção dos TIOs. MÉTODOS: O acompanhamento dos pacientes consistiu na avaliação clínica, na avaliação laboratorial com ênfase no metabolismo ósseo e na realização de exames de imagem para caracterização das deformidades esqueléticas. Para a localização dos TIOs, os pacientes foram submetidos a exames de Octreoscan®, MIBI, ressonância magnética (RM) e tomografia computadorizada (TC). RESULTADOS: O período de observação dos pacientes variou de dois a 25 anos. Ao diagnóstico, todos exibiam fraqueza muscular, dores ósseas e fraturas de fragilidade. Em relação à avaliação laboratorial, apresentavam: hipofosfatemia com taxa de reabsorção tubular de fosfato reduzida, fosfatase alcalina aumentada e níveis elevados de FGF23. O Octreoscan® permitiu a identificação dos TIOs nos nove pacientes e o MIBI possibilitou a localização dos TIOs em seis pacientes, sendo que ambos os exames foram concordantes entre si e com os exames topográficos (RM ou TC). Os achados histopatológicos das lesões dos nove pacientes confirmaram tratar-se de oito tumores mesenquimais fosfatúricos (PMTs) benignos e um PMT maligno. Após a primeira intervenção cirúrgica para a remoção dos TIOs, quatro pacientes encontram-se em remissão da doença e cinco evoluíram com persistência tumoral. Dos cinco, quatro foram reoperados e um aguarda nova cirurgia. Dos que foram reoperados, um paciente se mantém em remissão da doença, um foi a óbito por complicações clínicas, uma teve doença metastática e o último apresentou recidiva tumoral três anos após a segunda cirurgia. Deformidades ósseas graves foram observadas nos pacientes cujo diagnóstico e/ou tratamento clínico foram tardios. O tratamento da osteomalácia foi iniciado com fosfato e perdurou até a ressecção tumoral, tendo sido reintroduzido nos casos de persistência/recidiva tumoral. Quatro pacientes que fizerem uso regular desse medicamento por mais de seis anos evoluíram com hiperparatireoidismo terciário (HPT). CONCLUSÕES: O estudo revelou que tanto o Octreoscan® como o MIBI foram capazes de localizar os TIOs. Por isso, incentivamos a realização do MIBI nos locais onde o Octreoscan® não for disponível. Uma equipe experiente é indispensável para o sucesso cirúrgico visto que os tumores, embora benignos, costumam ser infiltrativos. Recomendamos o seguimento por tempo indeterminado em função do risco de recidiva tumoral. Assim como o FGF23, consideramos o fósforo um excelente marcador de remissão, persistência e recidiva dos TIOs. O diagnóstico e o tratamento precoce são fundamentais para a melhora dos sintomas podendo minimizar as deformidades esqueléticas e as sequelas ósseas. O uso prolongado do fosfato no tratamento da osteomalácia hipofosfatêmica foi associado ao desenvolvimento do HPT

El papel del Marqués de Vadillo como propagador de la vacuna en Soria (1801)

El método desarrollado por Edward Jenner para prevenir la viruela en 1798 se difundió con extraordinaria rapidez por Europa. Las primeras vacunaciones en diciembre de 1800 marcan en España el inicio de una práctica que fue promovida por la iniciativa particular de médicos y cirujanos en distintos núcleos del país. Ignacio María Ruiz de Luzuriaga, secretario de la Real Academia de Medicina de Madrid, fue una figura central en la propagación del método al establecer una red espontánea de corresponsales a los que remitía vacuna e información sobre cómo utilizarla. Esta correspondencia, conservada en un legajo de la Academia con el título de Papeles de la vacuna, muestra la relación mantenida no solo con otros facultativos, sino también con miembros de la nobleza y burguesía peninsular que contribuyeron a impulsar la práctica. Este estudio analiza las cartas remitidas por Francisco González de Castejón, Marqués de Vadillo, interesado inicialmente por preservar de la viruela a su hija, y que junto al médico Josef Ubis propagó la vacuna en la provincia de Soria y algunas localidades de Navarra entre los meses de agosto y diciembre de 1801.

Phagocytosis of Photoreceptor Outer Segments by Transplanted Human Neural Stem Cells as a Neuroprotective Mechanism in Retinal Degeneration

Purpose. Transplantation of human central nervous system stem cells (HuCNS-SC) into the subretinal space of Royal College of Surgeons (RCS) rats preserves photoreceptors and visual function. To explore possible mechanism(s) of action underlying this neuroprotective effect, we performed a detailed morphologic and ultrastructure analysis of HuCNS-SC transplanted retinas. Methods. The HuCNS-SC were transplanted into the subretinal space of RCS rats. Histologic examination of the transplanted retinas was performed by light and electron microscopy. Areas of the retina adjacent to HuCNS-SC graft (treated regions) were analyzed and compared to control sections obtained from the same retina, but distant from the transplant site (untreated regions). Results. The HuCNS-SC were detected as a layer of STEM 121 immunopositive cells in the subretinal space. In treated regions, preserved photoreceptor nuclei, as well as inner and outer segments were identified readily. In contrast, classic signs of degeneration were observed in the untreated regions. Interestingly, detailed ultrastructure analysis revealed a striking preservation of the photoreceptor–bipolar–horizontal cell synaptic contacts in the outer plexiform layer (OPL) of treated areas, in stark contrast with untreated areas. Finally, the presence of phagosomes and vesicles exhibiting the lamellar structure of outer segments also was detected within the cytosol of HuCNS-SC, indicating that these cells have phagocytic capacity in vivo. Conclusions. This study reveals the novel finding that preservation of specialized synaptic contacts between photoreceptors and second order neurons, as well as phagocytosis of photoreceptor outer segments, are potential mechanism(s) of HuCNS-SC transplantation, mediating functional rescue in retinal degeneration.

Anotaciones a la biografía de Ignacio María Ruiz de Luzuriaga (1763–1822), el inicio de la vacunación contra la viruela en España

Ignacio María Ruiz de Luzuriaga (1763–1822) tuvo un papel destacado durante el inicio de la vacunación antivariólica en España. Desde su posición como secretario de la Real Academia de Medicina, Luzuriaga fomentó una red epistolar con médicos, cirujanos, aristócratas y burgueses de clara mentalidad ilustrada, a los que proporcionaba vacuna y consejos sobre ella y de los que recibía información sobre los progresos de la vacunación. Fue un intento fallido de formar un Comité Central de la Vacuna como existía en otros países europeos. El texto revisa la figura de Luzuriaga aportando detalles no conocidos sobre su trayectoria vital.

Aid decision algorithms to estimate the risk in congenital heart surgery

Background and objective: In this paper, we have tested the suitability of using different artificial intelligence-based algorithms for decision support when classifying the risk of congenital heart surgery. In this sense, classification of those surgical risks provides enormous benefits as the a priori estimation of surgical outcomes depending on either the type of disease or the type of repair, and other elements that influence the final result. This preventive estimation may help to avoid future complications, or even death. Methods: We have evaluated four machine learning algorithms to achieve our objective: multilayer perceptron, self-organizing map, radial basis function networks and decision trees. The architectures implemented have the aim of classifying among three types of surgical risk: low complexity, medium complexity and high complexity. Results: Accuracy outcomes achieved range between 80% and 99%, being the multilayer perceptron method the one that offered a higher hit ratio. Conclusions: According to the results, it is feasible to develop a clinical decision support system using the evaluated algorithms. Such system would help cardiology specialists, paediatricians and surgeons to forecast the level of risk related to a congenital heart disease surgery.

Pharmacopea de la Armada (1760), un libro singular para uso de los sanitarios navales del siglo XVIII

La Pharmacopea de la Armada, obra de Leandro de Vega, publicada en 1760 para uso de médicos y cirujanos de los buques y hospitales de la marina española a lo largo del siglo XVIII, está considerada como la primera farmacopea naval española. Su autor la define con un «catálogo de medicamentos pertenecientes a las enfermedades médicas», en definitiva un nomenclátor de fórmulas para la preparación de los medicamentos de mayor utilidad para los navegantes de la época, tanto de uso interno como externo. En el presente artículo se analiza en profundidad esta obra, reconocida su relevancia de fuente primaria, situándola en su contexto histórico, detallando el contenido de sus tratados, así como dando noticia biográfica de su autor e informando de la posición profesional y misión de sus destinatarios.

Cases copied by James Lloyd from Mr. Steed, apothecary in Guy's Hospital, London, 1751-1787 (inclusive)

Contains medical cases copied by James Lloyd (1728-1810), primarily between 1751 and 1754, from Mr. Steed, an apothecary at Guy's Hospital in London, England. The volume has additional medical cases dating from 1780 to 1787. Lloyd transcribed the names, ages, and symptoms of the patients, as well as the medicines and medical care delivered to them. The volume is divided into chapters based on the type of case, which included vision loss; fluor albus, or leucorrhoea; diabetes; and dysentery. There is also a letter pasted into the volume addressed to Dr. Brigham of the Boston Medical Library Association from Lloyd's great-grandson, dated 4 November 1887.

A Virtual Joystick as a Novel Interaction Modality for Surgeon-Computer Interaction During Computer-Assisted Surgical Procedures

Interacting with a computer system in the operating room (OR) can be a frustrating experience for a surgeon, who currently has to verbally delegate to an assistant every computer interaction task. This indirect mode of interaction is time consuming, error prone and can lead to poor usability of OR computer systems. This thesis describes the design and evaluation of a joystick-like device that allows direct surgeon control of the computer in the OR. The device was tested extensively in comparison to a mouse and delegated dictation with seven surgeons, eleven residents, and five graduate students. The device contains no electronic parts, is easy to use, is unobtrusive, has no physical connection to the computer and makes use of an existing tool in the OR. We performed a user study to determine its effectiveness in allowing a user to perform all the tasks they would be expected to perform on an OR computer system during a computer-assisted surgery. Dictation was found to be superior to the joystick in qualitative measures, but the joystick was preferred over dictation in user satisfaction responses. The mouse outperformed both joystick and dictation, but it is not a readily accepted modality in the OR.

Medical Curriculum Change at Queen's-affiliated Medical Colleges: 1881 – 1910

This study explores the curriculum at Queen’s-affiliated medical colleges, specifically The Royal College of Physicians and Surgeons, Kingston, the Kingston Women’s Medical College, and Queen’s Medical College, from 1881 to 1910, using the textbooks prescribed by these institutions as primary sources. The central question encompasses what factors primarily motivated the curriculum at Queen’s-affiliated medical colleges to change. Within the historiographical scholarship on Queen’s College, this question has not yet been addressed and, to my knowledge, this is the first medical education history to specifically address textbooks as part of a medical school curriculum. During this period, these institutions experienced reorganizational shifts, such as the reunification of Queen’s Medical College with The Royal College of Physicians and Surgeons, Kingston, as well as the introduction and subsequent exclusion of female students. Within this context, this study examines how the forces of scientific innovation and co-education impacted the curriculum during the period under study, as measured by textbook change, specifically in the courses of obstetrics and gynaecology, the theory and practice of medicine, and surgery. To what degree was curriculum in these courses responsive to scientific inventions and discoveries, changing therapeutic practices, and possible gender biases? From 1881 to 1910, innovations such as x-ray and anaesthesia became commonplace within medical practice. Some technologies gained acceptance in the curriculum, while others fell out of favour. This study tracks these scientific discoveries through the textbooks used at Queen’s-affiliated medical colleges in order to demonstrate how the evolving nature of medicine was represented in the curriculum. To address how gender influenced the curriculum, textbooks from the Kingston Women’s Medical College and The Royal College of Physicians and Surgeons, Kingston, were compared. For two out of the three examined courses, it was found that sections of textbooks discussing various topics at the Kingston Women’s Medical College contained significantly more detail than their corresponding sections within The Royal College’s textbooks. It was speculated that the instructors preferred to teach their female students through textbooks, rather than lectures.

Hiperparatiroidismo primário em doentes jovens

Trabalho Final do Curso de Mestrado Integrado em Medicina, Faculdade de Medicina, Universidade de Lisboa, 2014

Caractérisation des unités de soins aigus chirurgicaux au sein des départements de chirurgie générale au Canada

Introduction : The acute care surgery (ACS) units are dedicated to the prompt management of surgical emergencies. It is a systemic way of organizing on-call services to diminish conflict between urgent care and elective obligations. The aim of this study was to define the characteristics of an ACS unit and to find common criteria in units with reported good functioning. Methods : As of July 1st 2014, 22 Canadian hospitals reported having an ACS unit. A survey with questions about the organization of the ACS units, the population it serves, the number of emergencies and trauma cases treated per year, and the satisfaction about the implementation of this ACS unit was sent to those hospitals. Results : The survey’s response rate was 73%. The majority of hospitals were tertiary or quaternary centers, served a population of more than 200 000 and had their ACS unit for more than three years. The median number of surgeons participating in an ACS unit was 8.5 and the majority were doing seven day rotations. The median number of operating room days was 2.5 per week. Most ACS units (85%) had an estimated annual volume of more than 2500 emergency consultations (including both trauma and non-trauma) and 80% operated over 1000 cases per year. Nearly all the respondents (94%) were satisfied with the implementation of the ACS unit in their hospital. Conclusion : Most surgeons felt that the implementation of an ACS unit resulted in positive outcomes. However, there should be a sizeable catchment population and number of surgical emergencies to justify the resulting financial and human resources.

Type A aortic dissection model to improve endovascular research and technologies.

OBJECTIVE Type A aortic dissection is a life-threatening disease requiring immediate surgical treatment. With emerging catheter-based technologies, endovascular stent-graft implantation to treat aneurysms and dissections has become a standardized procedure. However, endovascular treatment of the ascending aorta remains challenging. Thus we designed an ascending aortic dissection model to allow simulation of endovascular treatment. METHODS Five formalin-fixed human aortas were prepared. The ascending aorta was opened semicircularly in the middle portion and the medial layer was separated from the intima. The intimal tube was readapted using running monofilament sutures. The preparations were assessed by 128-slice computed tomography. A bare-metal stent was implanted for thoracic endovascular aortic repair in 4 of the aortic dissection models. RESULTS Separation of the intimal and medial layer of the aorta was considered to be sufficient because computed tomography showed a clear image of the dissection membrane in each aorta. The dissection was located 3.9 ± 1.4 cm proximally from the aortic annulus, with a length of 4.6 ± 0.9 cm. Before stent implantation, the mean distance from the intimal flap to the aortic wall was measured as 0.63 ± 0.163 cm in the ascending aorta. After stent implantation, this distance decreased to 0.26 ± 0.12 cm. CONCLUSION This model of aortic dissection of the ascending human aorta was reproducible with a comparable pathological and morphological appearance. The technique and model can be used to evaluate new stent-graft technologies to treat type A dissection and facilitate training for surgeons.

The sutureless aortic valve at 1 year: A large multicenter cohort study

OBJECTIVE Sutureless aortic valve replacement (AVR) offers an alternative to standard AVR in aortic stenosis. This prospective, single-arm study aimed to demonstrate safety and effectiveness of a bovine pericardial sutureless aortic valve at 1 year. METHODS From February 2010 to September 2013, 658 patients (mean age 78.3 ± 5.6 years; 40.0% octogenarian; 64.4% female; mean Society of Thoracic Surgeons score 7.2 ± 7.4) underwent sutureless AVR in 25 European centers. Concomitant cardiac procedures were performed in 29.5% and minimally invasive cardiac surgery in 33.3%. RESULTS One-year site-reported event rates were 8.1% for all-cause mortality, 4.5% for cardiac mortality, 3.0% for stroke, 1.9% for valve-related reoperation, 1.4% for endocarditis, and 0.6% for major paravalvular leak. No valve thrombosis, migration, or structural valve deterioration occurred. New York Heart Association class improved at least 1 level in 77.5% and remained stable (70.4% New York Heart Association class I or II at 1 year). Mean effective orifice area was 1.5 ± 0.4 cm(2); pressure gradient was 9.2 ± 5.0 mm Hg. Left ventricular mass decreased from 138.5 g/m(2) before surgery to 115.3 g/m(2) at 1 year (P < .001). Echocardiographic core laboratory findings confirmed that paravalvular leak was rare and remained stable during follow-up. CONCLUSIONS The Perceval sutureless valve resulted in low 1-year event rates in intermediate-risk patients undergoing AVR. New York Heart Association class improved in more than three-quarters of patients and remained stable. These data support the safety and efficacy to 1 year of the Perceval sutureless valve in this intermediate-risk population.

Which Anesthesia Regimen Is Best to Reduce Morbidity and Mortality in Lung Surgery? A Multicenter Randomized Controlled Trial.

BACKGROUND One-lung ventilation during thoracic surgery is associated with hypoxia-reoxygenation injury in the deflated and subsequently reventilated lung. Numerous studies have reported volatile anesthesia-induced attenuation of inflammatory responses in such scenarios. If the effect also extends to clinical outcome is yet undetermined. We hypothesized that volatile anesthesia is superior to intravenous anesthesia regarding postoperative complications. METHODS Five centers in Switzerland participated in the randomized controlled trial. Patients scheduled for lung surgery with one-lung ventilation were randomly assigned to one of two parallel arms to receive either propofol or desflurane as general anesthetic. Patients and surgeons were blinded to group allocation. Time to occurrence of the first major complication according to the Clavien-Dindo score was defined as primary (during hospitalization) or secondary (6-month follow-up) endpoint. Cox regression models were used with adjustment for prestratification variables and age. RESULTS Of 767 screened patients, 460 were randomized and analyzed (n = 230 for each arm). Demographics, disease and intraoperative characteristics were comparable in both groups. Incidence of major complications during hospitalization was 16.5% in the propofol and 13.0% in the desflurane groups (hazard ratio for desflurane vs. propofol, 0.75; 95% CI, 0.46 to 1.22; P = 0.24). Incidence of major complications within 6 months from surgery was 40.4% in the propofol and 39.6% in the desflurane groups (hazard ratio for desflurane vs. propofol, 0.95; 95% CI, 0.71 to 1.28; P = 0.71). CONCLUSIONS This is the first multicenter randomized controlled trial addressing the effect of volatile versus intravenous anesthetics on major complications after lung surgery. No difference between the two anesthesia regimens was evident.

Railway surgeon.

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