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PURPOSE: Inferior vena cava (IVC) agenesis is an uncommon congenital vascular anomaly stemming from aberrant development during embryogenesis. It results from the failure of one or more of the supracardinal veins, subcardinal veins, vitelline veins or postcardinal veins to connect. The symptomatology resulting from this vascular malformation can be either absent or extremely rich and varied. METHODS: Thoracoabdominal-pelvic CT scan projections following iodine-based contrast product injection were analyzed and a three-dimensional model of vascularization constructed. RESULTS: Herein, an asymptomatic case of IVC agenesis with absence of the suprarenal and renal segments, with azygos continuation, presenting an accessory hepatorenal vein is reported. The presence of this type of accessory vein has never been described in the literature to date. The etiology of this case of IVC agenesis is explored in depth. We also analyzed the morphometric parameters of the IVC remnant segments and the azygos vein in order to quantify the dilatation of the collateral venous pathway overdeveloped to handle blood return. CONCLUSION: Using the findings from this case and those reported in the literature, we provide general recommendations that should be taken into account before managing a patient, symptomatic or asymptomatic, admitted to the hospital with IVC agenesis.
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Veia Ázigos , Veia Cava Inferior , Veia Ázigos/diagnóstico por imagem , Humanos , Fígado , Tomografia Computadorizada por Raios X , Veia Cava Inferior/diagnóstico por imagemRESUMO
INTRODUCTION: Given the high variability and fragility of the thoracic duct, good knowledge of its anatomy is essential for its repair or to prevent iatrogenic postoperative chylothorax. The objective of this study was to define a site where the thoracic duct is consistently found for its ligation. The second objective was to define an anatomically safe surgical pathway to prevent iatrogenic chylothorax in surgery for aortic arch anomalies with vascular ring, through better knowledge of the anatomical relationships of the thoracic duct. METHODS: Seventy adult formalin-fixed cadavers were dissected. The anatomical relationships of the thoracic duct were reported at the postero-inferior mediastinum, at levels T3 and T4. RESULTS: The thoracic duct was consistently situated between the left anterolateral border of the azygos vein and the right border of the aorta between levels T9 and T10, whether it was simple, double, or plexiform. It was located medially, anteromedially, or posteriorly to the left subclavian artery in 51%, 21%, and 28% of the cases, respectively, at the level of T3. At T4, it was posteromedial in 27% of the cases or had no direct relationship with the aortic arch. CONCLUSION: These results favor mass ligation of the thoracic duct at levels T9-T10 between the right border of the aorta and the azygos vein, eventually including the latter. To prevent iatrogenic postoperative chylothorax in aortic arch anomalies with vascular ring surgery, we recommend remaining strictly lateral to the left subclavian artery at the level of T3 to reach the aortic arch anomalies with vascular ring at T4.
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Ducto Torácico/anatomia & histologia , Idoso de 80 Anos ou mais , Variação Anatômica , Cadáver , Quilotórax/prevenção & controle , Feminino , Humanos , Doença Iatrogênica/prevenção & controle , Ligadura , Masculino , Ducto Torácico/cirurgiaRESUMO
BACKGROUND: There is no demonstrated benefit of high-tie versus low-tie vascular transections in low rectal cancer surgery. The aim of this study was to compare the effects of high tie and low tie of the inferior mesenteric artery on colonic length. METHODS: This study was conducted in a surgical anatomy research laboratory. Anatomical dissections were performed on 11 human cadavers. We performed full left colonic mobilization, section of the descending-sigmoid junction, and high and low ligation of the inferior mesenteric artery. Distance from the proximal colon limb to the lower edge of the pubis symphysis was recorded after each step of vascular division. Three measurements were successively performed: before vascular section, after inferior mesenteric artery ligation, and after inferior mesenteric artery and vein section. RESULTS: Before vascular section, the mean distance between colonic end and lower edge of the symphysis pubis was - 1.9 ± 3.5 cm. After combined artery and vein section, the mean distance was + 10.7 ± 4.6 cm for high tie and + 1.5 ± 3 cm for low tie. A limitation of this study is the use of embalmed anatomical specimens, rather than live patients, and the small number of specimens. This study also does not evaluate colon limb vascularization or the impact of proximal lymph node dissection on survival rates. CONCLUSIONS: High tie of the inferior mesenteric artery at its aortic origin allows a gain of extra length of about 9 cm over low tie.
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Colectomia/métodos , Colo/cirurgia , Ligadura/métodos , Artéria Mesentérica Inferior/cirurgia , Neoplasias Retais/cirurgia , Idoso , Idoso de 80 Anos ou mais , Colo Sigmoide/cirurgia , Feminino , Humanos , Masculino , Neoplasias Retais/patologia , Resultado do TratamentoRESUMO
PURPOSE: Dissection provides direct experience in anatomy, which constitutes an essential discipline for medical students. For this purpose, we created a dissection guide for students in the 2nd-year of medical studies at the Grenoble University School of Medicine. The objective was to evaluate this tool of reverse pedagogy in terms of student satisfaction and educational interest. METHODS: Every 2nd-year student takes four sessions of limb dissection. To assist this dissection course, we developed a photographic guide launched in 2013. It includes an introduction presenting a methodology for dissection, followed by detailed protocols for each dissection area. Each step is illustrated with captioned photographs associated with a concise explanatory text. A questionnaire was then sent to 242 students to assess the impact of this tool and their overall satisfaction. RESULTS: Overall student satisfaction with this guidebook was rated 8.1 out of 10 with a 93.2% with significant improvement (p = 0.0137) and 78.7% of them declaring they had a better understanding of anatomy and mastery of the dissection techniques, respectively. In addition, students assessed the usefulness of the dissection guide at 3.6 out of 4 with the relevance of the content and presentation judged at 3.4 out of 4. Finally, the exam scores increased significantly with use of the guidebook (p < 0.0001). CONCLUSIONS: Students deemed the organization of this anatomy tutorial as highly satisfactory, and using the guidebook as a reference in dissection sessions allowed students to prepare for the dissection and improve their knowledge of anatomy, as demonstrated by improved exam scores.
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Anatomia/educação , Dissecação/educação , Educação de Graduação em Medicina/métodos , Ilustração Médica , Livros de Texto como Assunto , Anatomia/métodos , Cadáver , Estudos de Coortes , Currículo , Dissecação/métodos , Educação de Graduação em Medicina/estatística & dados numéricos , Avaliação Educacional/estatística & dados numéricos , Feminino , França , Humanos , Aprendizagem , Masculino , Satisfação Pessoal , Fotografação , Estudantes de Medicina/psicologia , Estudantes de Medicina/estatística & dados numéricos , Inquéritos e Questionários/estatística & dados numéricos , Universidades , Adulto JovemRESUMO
PURPOSE: Perfusion techniques on cadavers are heterogeneous and imperfect. The objective of this study was to improve the existing circulation model for surgical simulation on cadavers. METHODS: We used a three-step experimental approach. The first part of the experiment tested two variables: the type of circuit and the use of a heater for perfusion. The second approach evaluated two parameters: the injection fluid and the type of body conditioning (embalmed or freshly dead prepared using different washing techniques). The third one was an improvement on the best circulation obtained, which focused on the injection fluid. To compare the realism of these different techniques, we constructed a score with realism parameters: the volume of return flow, the presence of peripheral venous return and the perfusion of abdominal arteries. RESULTS: We found that the use of a heater seemed to improve the perfusion, while performing an arteriovenous bypass did not seem very effective. A correlation rate of 0.84 was found between the realism score and the injected fluid chosen. The best score (4/6) was found for a non-embalmed body with a low-pressure washing technique using a gelatin-based liquid at a concentration of 4 g/L for circulation. Scores obtained using embalmed bodies for both injection fluids for high-pressure washing or for 8-g/L gelatin injection fluid did not exceed 3/6. CONCLUSIONS: We showed that using a non-embalmed body with low-pressure washing and a 4-g/L gelatin-based fluid was the most effective technique for cadaver perfusion.
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Cadáver , Treinamento com Simulação de Alta Fidelidade/métodos , Perfusão/métodos , Procedimentos Cirúrgicos Operatórios/educação , Corantes/administração & dosagem , Embalsamamento , Estudos de Viabilidade , Gelatina/administração & dosagem , Humanos , Soluções Hipertônicas/administração & dosagem , Azul de Metileno/administração & dosagem , PressãoRESUMO
PURPOSE: The objective of this study was to identify the most appropriate cadaver perfusion techniques for surgical training through a systematic review with a description of the protocols used. METHODS: The search strategy included PubMed and reference tracking. Studies were identified by searching the electronic Medline databases. The search concepts included perfusion, cadavers and simulation training, and the protocol used is reported. This resulted in a qualitative review of 12 articles out of 250 articles consulted. We collected all the important data from these 12 articles. RESULTS: Regarding the characteristics of the studies and the declotting or perfusion techniques, the results were heterogeneous. Indeed, in several studies, a good deal of information was unclear or insufficiently precise, making it unfeasible to summarize the data. The methods used were not sufficiently explicit and detailed. However, a majority of the fresh cadavers used tap water for declotting. Perfusion, type of fluid, number of pumps, pressure, pulsatility, and arterial or venous approaches differed greatly. Only two studies fulfilled five of our six realism criteria for surgical simulation. CONCLUSIONS: This systematic review provided an overview of all the different cadaver perfusion techniques. It could be used to establish a reference method of a simulation model.
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Cadáver , Preservação de Órgãos/métodos , Perfusão/métodos , Especialidades Cirúrgicas/educação , Dissecação , Humanos , Soluções para Preservação de Órgãos , Treinamento por SimulaçãoRESUMO
PURPOSE: The pain involved in the herniated discs could be generated by some mobility of the nerve roots during straight leg raising (SLR). SLR produces some movement of nerves, but the magnitude of this displacement needs to be thorough, that is why we have investigated lumbo-sacral nerve root displacement in the spinal canal during the passive straight leg raise (SLR). METHODS: Fourteen cadavers underwent laminectomy to mark the nerve roots of L2-S1 with lead balls. X-rays were taken during different movements imposed on the body: bilateral hip extension, left SLR then right and bilateral SLR. By superimposing these images two by two, the displacement of the nerve roots is quantified numerically during the various SLR maneuvers with respect to the reference position corresponding to the bilateral hip extension. RESULTS: The median range of the different nerve root movements ranged from 0.10 to 0.51 cm (p < 0.05 except for the L2 root) when the left SLR is applied, from 0.26 to 0.48 cm (p < 0.05) with the right SLR and from 0.30 to 0.65 cm (p < 0.05) with a bilateral SLR. No statistically significant relationship was found between age and movement value. CONCLUSIONS: The lumbo-sacral nerve roots in the spinal canal region move statistically significantly in response to the clinically applied SLR test, except for L2 root during the left SLR. This movement is symmetric and greater when a bilateral SLR is applied. These anatomical results are correlated with those observed empirically in clinical practice.
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Degeneração do Disco Intervertebral/fisiopatologia , Deslocamento do Disco Intervertebral/fisiopatologia , Perna (Membro)/inervação , Perna (Membro)/fisiopatologia , Região Lombossacral/inervação , Movimento/fisiologia , Raízes Nervosas Espinhais/anatomia & histologia , Raízes Nervosas Espinhais/fisiopatologia , Idoso , Cadáver , Feminino , Humanos , Laminectomia , Masculino , Medição da DorRESUMO
PURPOSE: The anatomy of gubernaculum testis (GT) is often discussed; however, the postnatal anatomy of the GT or scrotal ligament (SL) is rarely described. Hence, we performed an anatomical and histological study to analyze histologically the structures between testis and scrotum. METHODS: We performed anatomical dissections on 25 human fresh cadavers' testes. Each testis was removed with its envelopes and macroscopically analyzed. Then samples were included for histological study. Finally, they were analyzed under microscope, looking for attachments between testis, epididymis and scrotal envelopes. RESULTS: The absence of proximal and distal attachment was found in 56.0% of cases. Looking at the proximal attachment of the SL, the main one found is the epididymal attachment (28.0%), whereas no cases of testis attachment was found. Distally, there are more variations with scrotal attachment (12%) and cremaster attachment (12.0%). We found a significant prevalence of multiple adherences in 16.0% of cases too. Finally, in 15 cases (57.7%) an attachment is present between testis and epididymis, as it is commonly described. CONCLUSIONS: In the majority of cases there is no attachment of the lower pole of the testis and epididymis and these structures remain free. So it seems that the SL disappears with aging. Moreover, there is not only one kind of ligamentous attachment, but a high variability of attachments at the lower pole of the testiculo-epididymal structure. When it exists, this structure is never a real ligament and it seems more appropriate to use the term "attachments".
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Epididimo/anatomia & histologia , Gubernáculo/anatomia & histologia , Ligamentos/anatomia & histologia , Escroto/anatomia & histologia , Testículo/anatomia & histologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Variação Anatômica , Cadáver , Técnicas Histológicas , Humanos , Masculino , Pessoa de Meia-IdadeRESUMO
PURPOSE: Classically, the round ligament of the uterus (RLU) attaches distally in the ipsilateral labia majora. This attachment has rarely been described in adults. That is why we have performed an anatomical study focused on this distal ending. PATIENTS: We performed in 2015 the cadaveric dissection of 19 RLU. METHODS: In all cases, the RLU was individualized on its entire length from its uterine origin to the inguinal canal. Then this canal was open from its internal orifice to its external orifice. We described the distal attachment of the RLU according four areas: before the internal inguinal ring, after the external inguinal ring, under the pubic bone and in labia majora. RESULTS: We found 3 types of distal attachments with first an attachment after the external inguinal ring in more than half of cases (52.6%). Then, before the internal inguinal ring (26.3%) and under the pubic bone (22.1%). No RLU was found inlabia majora. However, the proximal attachment seems constant at the antero-superior face of uterus, near the tubo-uterine junction like its pelvic path under the broad ligament. CONCLUSION: In adult, the RLU is a structure, which begins at the cranio-ventral part of the uterine bottom near the tubo-uterine junction. Then it passes under the broad ligament and reaches the inguinal canal, that it crosses in more half of cases. However, 3 distal attachment areas have been identified but never in the labia majora. Indeed, some anatomical information available in anatomical treaties seems not correct and should be amended.
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Ligamento Redondo do Útero/anatomia & histologia , Vulva/anatomia & histologia , Cadáver , Dissecação , Feminino , Humanos , Canal Inguinal/anatomia & histologiaRESUMO
PURPOSE: To improve the knowledge of the morphometry and the surrounding anatomical structures of the intersigmoid fossa and to determine possible surgical applications. METHOD: Forty eight adult cadavers (29 female and 19 male; mean age 83 years) underwent dissection in the Laboratoire d'Anatomie des Alpes Francaises. Two injections in the right carotid resulted in a total body concentration of formalin of 1.3 %. The study parameters were the dimensions of the intersigmoid fossa orifice and the fossa's relationship to surrounding structures. Data were recorded and analyzed using Microsoft Office Excel (MS Cerp). A Pearson coefficient r was used to examine the correlation between the length of colon and the ISF volume. RESULTS: The intersigmoid fossa was present in 75 % of cases (n = 36). The average dimensions for the transverse diameter, longitudinal diameter, and the depth were, respectively, 20.5 ± 0.2, 20.3 ± 0.13, and 26.8 ± 0.2 mm. The primary and secondary roots bordering this fossa measured on average 59.1 ± 0.1 and 48.3 ± 0.13 mm. In 13.9 % of cases (n = 5), the maximum depth was >40 mm and in 16.7 % of cases (n = 6), one of the diameters of the orifice entry of the fossa was >40 mm. The ureter and external iliac artery were the most frequently encountered structures during the dissection of the fundus of the intersigmoid fossa. CONCLUSION: The intersigmoid fossa remains present in most of the reported dissections of cadavers. It constitutes an essential landmark in the surgery of the sigmoid colon due to its deep structural relationship with the left ureter and external iliac artery.
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Colo Sigmoide/anatomia & histologia , Hérnia Abdominal/cirurgia , Idoso , Idoso de 80 Anos ou mais , Cadáver , Dissecação , Feminino , Humanos , Masculino , Pessoa de Meia-IdadeRESUMO
PURPOSE: The feasibility of coronary artery bypass grafting using an internal thoracic artery (ITA) depends on the length of the graft with respect to the optimal route to reach the coronary target. The goal of this study was to assess the gain in length afforded by skeletonization and to evaluate the lengths of different pathways of the ITAs to the left coronary arteries. METHODS: The left and right ITAs were dissected out from 20 specimens and measured before and after skeletonization. Distance between the origin of the right ITA and the base of the left atrial appendage, corresponding to the proximal circumflex artery, was measured for both the transverse pericardial sinus and preaortic routes. RESULTS: Skeletonization gave a significant gain of length for both ITAs. Analysis showed no significant correlation between the ITA length and the height, weight, and BMI of specimens. We found no association between the length of the sternum and the length of skeletonized RITA or LITA. The anterior route of the skeletonized right ITA was shorter than the transverse pericardial sinus route in 18 cases. The average length to the circumflex artery territory was 132.8 ± 23.5 mm in front of the aorta and 150.5 ± 18.8 through the transverse pericardial sinus with a gain of length of 17.7 mm (p < 0.0001). CONCLUSION: Skeletonization gave significant gains in length of both ITAs. The preaortic route for the skeletonized right ITA toward the circumflex territory was shorter than the transverse pericardial sinus route in 90 % of cases.
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Ponte de Artéria Coronária , Vasos Coronários/anatomia & histologia , Artéria Torácica Interna/anatomia & histologia , Idoso , Idoso de 80 Anos ou mais , Cadáver , Dissecação , Feminino , Humanos , MasculinoRESUMO
BACKGROUND: Distant metastases of differentiated thyroid cancers are exceptionally localized in the soft tissues and the pelvic cavity. This last event complicates diagnosis, especially in female patients because of false positive nuclear imaging and unrelated iodine uptakes. False positive due to body secretions, urinary and fecal iodine retention and iodine uptake by non-thyroid tissues or ectopic thyroid tissue and bone metastasis of differentiated thyroid carcinoma. METHODS: We present two cases of metastasectomy, guided by a peroperative gamma probe and intraoperative frozen sections of the specimens. In females patients because of potentially false positive nuclear imaging and iodine uptakes unrelated to pathology, it is very difficult to identify a pelvic metastasis. Our experience of two cases underlines these pitfalls and the possibilities to avoid them: the use of 131 Iodine SPECT/CT whole body scan, peroperative gamma probe and intraoperative frozen sections of specimen. CONCLUSION: Preoperative localization needs a precise 3D imaging and the surgical treatment must be guided by a peroperative gamma probe and frozen sections.
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Carcinoma Papilar, Variante Folicular/diagnóstico por imagem , Cuidados Intraoperatórios , Radioisótopos do Iodo , Imagem Multimodal , Neoplasias Musculares/diagnóstico por imagem , Neoplasias Ovarianas/diagnóstico por imagem , Tomografia por Emissão de Pósitrons , Neoplasias da Glândula Tireoide/diagnóstico por imagem , Tomografia Computadorizada por Raios X , Adulto , Carcinoma Papilar, Variante Folicular/secundário , Carcinoma Papilar, Variante Folicular/cirurgia , Diagnóstico Diferencial , Feminino , Humanos , Achados Incidentais , Imagem Multimodal/métodos , Neoplasias Musculares/secundário , Neoplasias Musculares/cirurgia , Neoplasias Ovarianas/secundário , Neoplasias Ovarianas/cirurgia , Diafragma da Pelve/diagnóstico por imagem , Diafragma da Pelve/patologia , Neoplasias da Glândula Tireoide/patologia , Neoplasias da Glândula Tireoide/cirurgia , Resultado do TratamentoRESUMO
The aim of this study was to test whether an omental pouch can be used as an alternative site for islet implantation in diabetic monkeys. Here we report the successful engraftment of islets in diabetic cynomolgus monkeys when loaded on a synthetic biodegradable scaffold and placed in an omental pouch. One autologous and five allogeneic diabetic monkey transplants under the cover of steroid-free immune suppression (SFIS) were undertaken. Fasting blood glucose (FBG) and C-peptide (CP), exogenous insulin requirements (EIR), intravenous glucose tolerance test (IVGTT), A1C and histopathology were used to assess islet engraftment and survival. All animals achieved CP levels > 1.0 ng/mL following transplant, a 66-92% posttransplant decrease in EIR and reduced A1C. Following graft removal, CP became negative and histopathological analysis of the explanted grafts demonstrated well-granulated and well-vascularized, insulin-positive islets, surrounded by T-cell subsets and macrophages. Compared to intrahepatic allogeneic islet transplants (n = 20), there was a delayed engraftment for omental pouch recipients but similar levels of CP production were ultimately achieved, with a broad range of IEQ/kg transplanted in both sites. Our results suggest this extrahepatic transplantation site has potential as an alternative site for clinical islet cell transplantation.
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Diabetes Mellitus Experimental/cirurgia , Sobrevivência de Enxerto , Transplante das Ilhotas Pancreáticas , Omento , Animais , Macaca fascicularis , EstreptozocinaRESUMO
Lung transplantation (LT) is a recognized procedure for selected patients with end-stage respiratory failure. We performed 123 LT, including 32 single lung, 84 double lung, and 7 heart-lung transplantations in 48 patients with chronic obstructive pulmonary disease (COPD), 13 patients with pulmonary hypertension (PH), 33 with cystic fibrosis (CF), and 29 with interstitial lung disease (ILD) between July 1990 and January 2008. Survival was compared for periods before and after December 2001. The mean age of patients was 44.4 years (range 16-66.5 years); 84 (69%) were men. Before LT, 1 second forced expiratory volume was 28.7% +/- 18.1% and PaCO(2) = 6.3 kPa. Fifty-five patients were on noninvasive ventilation. Cold ischemia time was 320 +/- 91 minutes. Cardiopulmonary bypass (CPB) was used in 77 patients (64%). There were 18 early surgical reinterventions, 8 extracorporeal membrane oxygenations, and 38 bronchial stent insertions among 206 at-risk bronchial sutures. Crude survivals were 69%, 58%, 41%, and 18% at 1, 2, 5, and 10 years, respectively. Comparing before (n = 70 with 15 CF) vs after December 2001 (n = 53 with 17 CF), survivals were 63% vs 78%, 51% vs 71%, and 33% vs 60% at 1, 2, and 5 years, respectively (P = .01) and for CF patients, 52% vs 100%, 52% vs 94%, and 25% vs 94% at 1, 2, and 5 years, respectively (P = .005). There was significant improvement in survival before and after 2001 in 123 LT and particularly among CF patients. Improvement in survival after LT may be related to the sum of numerous changes in our practice since December 2001, including the use of pulmonary rehabilitation pre-LT, extracellular pneumoplegia, statins, macrolides for chronic rejection, monitoring of Epstein-Barr blood load, changes in maintenance immunosuppressants, as well as position movement up the coordinator nurse and learning curve.
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Sobrevivência de Enxerto/fisiologia , Transplante de Pulmão/fisiologia , Fibrose Cística/cirurgia , Feminino , Transplante de Coração-Pulmão/mortalidade , Transplante de Coração-Pulmão/fisiologia , Humanos , Hipertensão Pulmonar/cirurgia , Pneumopatias/cirurgia , Transplante de Pulmão/mortalidade , Masculino , Doença Pulmonar Obstrutiva Crônica/cirurgia , Estudos Retrospectivos , Análise de Sobrevida , SobreviventesRESUMO
AIM: To provide an overview of cadaver models for cardiac arrest and to identify the most appropriate cadaver model to improve cardiopulmonary resuscitation through a systematic review. METHODS: The search strategy included PubMed, Embase, Current contents, Pascal, OpenSIGLE and reference tracking. The search concepts included "heart arrest", "cardiopulmonary resuscitation" and "cadavers". All studies, published until February 2019, in English or French, on research or simulation in the field of cardiac arrest and using cadaver models were eligible for inclusion. RESULTS: Overall, 29 articles out of the 244 articles located were selected. The characteristics of the studies and the cadaver models were heterogenous. Indeed, 31% of the studies lacked a proper description of the model used and its specificities. Fresh cadavers were used in 55% of the studies and chest compressions were performed in 90%. This model was appreciated for its realism in terms of mechanical properties and tissue conservation. Thiel-embalmed cadavers also showed promising results concerning lung and chest compliance. The lack of circulation stood out as the strongest limitation of all types of human cadaver models. CONCLUSION: Four types of cadaver models are used in cardiac arrest research. The great heterogeneity of these models coupled with unequal quality in reporting makes comparisons between studies difficult. There is a need for uniform reporting and standardisation of human cadaver models in cardiac arrest research.
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Reanimação Cardiopulmonar/métodos , Parada Cardíaca/terapia , Massagem Cardíaca/métodos , Cadáver , HumanosRESUMO
The prevalence and characteristics of patients operated for adrenal adenoma (Conn syndrome) as well as their post-operative arterial pressure evolution are varying through literature. Our aim was to report the Grenoble University Hospital experience. From 1993 to 2005, 24 patients (mean age = 46 +/-11 years) presented the biological criteria of primary hyperaldosteronism and benefited from adrenalectomy with confirmation of adrenal adenoma. All had an uncontrolled hypertension, refractory in 42% of cases, with a hypokaliemia (mean = 2.65 +/- 0.47 mmol/l). All adenomas measured more than 10 mm in scanner imaging. After a mean post-operative follow-up of 46 +/- 43 months, 70% of them were normotensive, with (45%) or without (25%) anti-hypertensive therapy. the post-operative kaliemia was normal in all cases. Only 25% had post-operative hormonal dosages for control. Post-operative spontaneous normotensive patients had, at the diagnosis of adrenal adenoma, a more recent and non-refractory hypertension, with a lower number of antihypertensive drugs, a better response to spirinolactone and higher aldosterone plasmatic levels. Two lessons can be taken from this study: 1) Whether 70% of patients operated for adrenal adenoma are normotensive (with or without treatement) post-operatively, only 25% are definitely cured after 4 years. Factors associated to a post-operative cure highlight the interest of an ealy diagnosis. 2) There is probably an underdiagnosis of adrenal adenoma (Conn syndrome) because neither adenomas with normokaliemia, nor adenomas <10 mm in scanner imaging have ever been diagnosed or at least, sent to surgery.
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Neoplasias do Córtex Suprarrenal/diagnóstico , Neoplasias do Córtex Suprarrenal/cirurgia , Adenoma Adrenocortical/diagnóstico , Adenoma Adrenocortical/cirurgia , Neoplasias do Córtex Suprarrenal/complicações , Adrenalectomia , Adenoma Adrenocortical/complicações , Adulto , Pressão Sanguínea , Feminino , Seguimentos , Humanos , Hiperaldosteronismo/etiologia , Masculino , Pessoa de Meia-Idade , Prognóstico , Estudos RetrospectivosRESUMO
We present an in vivo and in vitro study of congenital adrenal hyperplasia in a patient with 11beta-hydroxylase deficiency. Sequencing of the CYP11B1 gene showed two new base substitutions, a conservative 954 G-->C transversion at the last base of exon 5 (T318T), and a IVS8 + 4A-->G transition in intron 8. In addition, two polymorphisms were found in exons 1 and 2. The genetically female patient was raised as a male because of severe pseudohermaphroditism. Glucocorticoid-suppressive treatment encountered difficulties in equilibration and compliance, resulting in uncontrolled hypertension with pronounced hypertrophic cardiomyopathy. At 42 yr of age the occurrence of central retinal vein occlusion with permanent loss of left eye vision led to the decision to perform bilateral laparoscopic adrenalectomy. Surgery was followed by normalization of blood pressure and good compliance with glucocorticoid and androgen substitutive therapies. In vitro, adrenal cells in culture and isolated mitochondria showed extremely low 11beta-hydroxylase activity. Analysis of adrenal CYP11B1 messenger ribonucleic acid (mRNA) by RT-PCR and sequencing showed the expression of a shorter mRNA that lacked exon 8 and did not contain either the exon 5 mutation or the exon 1 and 2 polymorphisms. This suggested that one CYP11B1 allele carried the intron 8 mutation, responsible for skipping exon 8. The other allele carried the exon 5 mutation, and its mRNA was not detectable. Western blot analysis showed weak expression of a shorter CYP11B immunoreactive band of 43 kDa, consistent with truncation of exon 8. Thus, bilateral adrenalectomy in this patient allowed effective treatment of severe hypertension and helped in understanding the mechanisms and physiopathological consequences of two novel mutations of CYP11B1.
Assuntos
Hiperplasia Suprarrenal Congênita/genética , Hiperplasia Suprarrenal Congênita/cirurgia , Adrenalectomia , Processamento Alternativo , Hipertensão/etiologia , Mutação , Esteroide 11-beta-Hidroxilase/genética , Glândulas Suprarrenais/patologia , Hiperplasia Suprarrenal Congênita/patologia , Hormônio Adrenocorticotrópico/sangue , Adulto , Sequência de Bases , Transtornos do Desenvolvimento Sexual/diagnóstico , Transtornos do Desenvolvimento Sexual/etiologia , Éxons , Feminino , Glucocorticoides/sangue , Humanos , Hipertensão/genética , Laparoscopia , Mineralocorticoides/sangue , Renina/sangue , Reação em Cadeia da Polimerase Via Transcriptase ReversaRESUMO
We studied a patient with food-induced, ACTH-independent, Cushing's syndrome and a unilateral adrenocortical adenoma. In vivo cortisol secretion was stimulated by mixed, glucidic, lipidic, or proteic meals. Plasma ACTH levels were undetectable, but iv injection of ACTH stimulated cortisol secretion. Unilateral adrenalectomy was followed by hypocortisolism with loss of steroidogenic responses to both food and ACTH. In vitro, cortisol secretion by isolated tumor cells was stimulated by the gut hormone gastric inhibitory polypeptide (GIP) and ACTH, but not by another gut hormone, glucagon-like peptide-1 (GLP-1). Both peptides stimulated the production of cAMP but not of inositol 1,4,5-trisphosphate. In quiescent cells, GIP and ACTH stimulated [3H]thymidine incorporation and p42-p44 mitogen-activated protein kinase activity. GIP receptor messenger ribonucleic acid (RNA), assessed by RT-PCR, was highly expressed in the tumor, whereas it was undetectable in the adjacent hypotrophic adrenal tissue, in two adrenal tumors responsible for food-independent Cushing's syndrome, and in two hyperplastic adrenals associated with ACTH hypersecretion. In situ hybridization demonstrated that expression of GIP receptor RNA was confined to the adrenocortical tumor cells. Low levels of ACTH receptor messenger RNA were also detectable in the tumor. We conclude that abnormal expression of the GIP receptor allows adrenocortical cells to respond to food intake with an increase in cAMP that may participate in the stimulation of both cortisol secretion and proliferation of the tumor cells.
Assuntos
Adenoma/complicações , Neoplasias do Córtex Suprarrenal/complicações , Síndrome de Cushing/etiologia , Polipeptídeo Inibidor Gástrico/farmacologia , Adenoma/metabolismo , Adenoma/cirurgia , Neoplasias do Córtex Suprarrenal/metabolismo , Neoplasias do Córtex Suprarrenal/cirurgia , Adrenalectomia , Hormônio Adrenocorticotrópico/farmacologia , Adulto , Proteínas Quinases Dependentes de Cálcio-Calmodulina/metabolismo , DNA/biossíntese , Feminino , Expressão Gênica , Glucagon/farmacologia , Peptídeo 1 Semelhante ao Glucagon , Humanos , Hidrocortisona/sangue , Hidrocortisona/metabolismo , Fragmentos de Peptídeos/farmacologia , Reação em Cadeia da Polimerase , Precursores de Proteínas/farmacologia , RNA Mensageiro/análise , Receptores da Corticotropina/genética , Receptores dos Hormônios Gastrointestinais/genética , Células Tumorais CultivadasRESUMO
Gorham's disease is a rare disorder characterized by a proliferation of thin-walled lymphatic vessels (lymphangiectasia) resulting in an osteolysis. A chylothorax is present in about one-fifth of the patients and carries a poor prognosis. In this circumstance, surgery including thoracic duct ligation, pleurodesis, and excision of involved tissue is probably the treatment of choice. It is facilitated by a precise radiological assessment using a computed tomographic scanning coupled with a lymphography. We report such a case.
Assuntos
Quilotórax/complicações , Osteólise Essencial/complicações , Quilotórax/diagnóstico por imagem , Quilotórax/cirurgia , Feminino , Humanos , Pessoa de Meia-Idade , Radiografia Torácica , Tomografia Computadorizada por Raios XRESUMO
We report a case of mediastinal liposarcoma resected by thoracoscopy. Despite the precautionary measures, chest wall implantations occurred rapidly at the port's sites in the chest wall and led to death within 24 months. We conclude that thoracoscopy is not a good approach for resection of anterior mediastinal masses in view of their possible malignant character.