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OBJECTIVES: Congenital knee dislocation (CKD) is a rare condition, affecting 1 in 100 000 newborns. Its prenatal diagnosis is challenging and not well described in the literature, especially when it appears isolated and not as part of a complex malformation or syndromic pattern. The purpose of this study was to provide a comprehensive review of the available literature on the prenatal diagnosis and postnatal outcome of CKD and to summarize the current evidence on this topic. METHODS: A systematic review of the literature on the prenatal diagnosis of CKD was performed in PubMed, Scopus and EMBASE. A predefined combination of specific keywords was used, focusing on intrauterine manifestations, diagnostic methods, prenatal behavior, postnatal treatment and neonatal outcome as well as long-term outcome in terms of ambulation, motion and joint stability. The quality of studies was assessed using the National Institutes of Health tool for quality assessment of case series. A summary of results was carried out providing proportions and rates of diagnostic and prognostic features associated with this rare condition. RESULTS: In total, 20 cases were retrieved for analysis, of which 19 were obtained from the identified eligible studies (n = 16) and one was an unpublished case from our center. The median gestational age at prenatal diagnosis, which was made using ultrasound in most cases, was 20 weeks (range, 14-38 weeks). Bilaterality was observed in 11/20 (55%) cases. The condition was isolated in 7/20 (35%) cases and associated with other anomalies in 13/20 (65%) cases. An association was observed with oligohydramnios (4/20 (20%)), and an invasive procedure was performed in 13/20 (65%) cases, including 11 cases with an invasive procedure performed for diagnostic purposes. Genetic testing was normal in all isolated cases for which information was available (4/7), while a genetic syndrome was present in 10/13 (77%) non-isolated cases (Larsen, Noonan, Grebe, Desbuquois or Escobar syndrome). There were seven terminations of pregnancy, of which six were performed in cases with associated anomalies and one in an isolated case, 11 cases of postnatal survival, one case of intrauterine death and one of neonatal death. The fetal and neonatal deaths occurred in cases with associated anomalies or abnormal genetic findings. Postnatal treatment was mostly conservative, with only two reports (18% of the 11 surviving neonates) of surgical intervention, both in cases with associated anomalies. Postnatal follow-up was up to 1 year in most cases, and motor outlook appeared normal in all isolated cases. CONCLUSIONS: CKD is a rare fetal anomaly with a prenatal diagnosis achievable from the early second trimester, for which a favorable outcome can be expected when no associated anomalies are present. Prenatal diagnosis should include detailed ultrasound assessment and amniocentesis for extensive genetic studies, particularly in non-isolated cases. Early postnatal treatment achieves success in most cases without surgical intervention and leads to a normal motor outlook. © 2023 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology.
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Insuficiência Renal Crônica , Ultrassonografia Pré-Natal , Feminino , Humanos , Recém-Nascido , Gravidez , Feto , Testes Genéticos , Diagnóstico Pré-Natal/métodos , Estudos Retrospectivos , Ultrassonografia Pré-Natal/métodosRESUMO
This Letter presents the first observation on how a strong, 500 kG, externally applied B field increases the mode-two asymmetry in shock-heated inertial fusion implosions. Using a direct-drive implosion with polar illumination and imposed field, we observed that magnetization produces a significant increase in the implosion oblateness (a 2.5× larger P2 amplitude in x-ray self-emission images) compared with reference experiments with identical drive but with no field applied. The implosions produce strongly magnetized electrons (ω_{e}τ_{e}â«1) and ions (ω_{i}τ_{i}>1) that, as shown using simulations, restrict the cross field heat flow necessary for lateral distribution of the laser and shock heating from the implosion pole to the waist, causing the enhanced mode-two shape.
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We propose a joint model to analyze the structure and intensity of the association between longitudinal measurements of an ordinal marker and time to a relevant event. The longitudinal process is defined in terms of a proportional-odds cumulative logit model. Time-to-event is modeled through a left-truncated proportional-hazards model, which incorporates information of the longitudinal marker as well as baseline covariates. Both longitudinal and survival processes are connected by means of a common vector of random effects. General inferences are discussed under the Bayesian approach and include the posterior distribution of the probabilities associated to each longitudinal category and the assessment of the impact of the baseline covariates and the longitudinal marker on the hazard function. The flexibility provided by the joint model makes possible to dynamically estimate individual event-free probabilities and predict future longitudinal marker values. The model is applied to the assessment of breast cancer risk in women attending a population-based screening program. The longitudinal ordinal marker is mammographic breast density measured with the Breast Imaging Reporting and Data System (BI-RADS) scale in biennial screening exams. © 2016 The Authors. Statistics in Medicine Published by John Wiley & Sons Ltd.
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Teorema de Bayes , Neoplasias da Mama/epidemiologia , Mama , Densidade da Mama , Feminino , Humanos , Medição de Risco/métodosRESUMO
OBJECTIVE: Assess reported risk of suicide attempts by patients with bipolar disorder (BD). METHOD: Systematic searching yielded 101 reports from 22 countries (79 937 subjects). We analyzed for risk (%) and incidence rates (%/year) of attempts, comparing sex and diagnostic types, including by meta-analysis. RESULTS: Attempt risk averaged 31.1% [CI: 27.9-34.3] of subjects, or 4.24 [3.78-4.70]%/year. In BD-I (43 studies) and BD-II subjects (30 studies), risks (29.9%, 31.4%) and incidence rates (4.01, 4.11%/year) were similar and not different by meta-analysis. Among women vs. men, risks (33.7% vs. 25.5%) and incidence (4.50 vs. 3.21%/year) were greater (also supported by meta-analysis: RR = 1.35 [CI: 1.25-1.45], P < 0.0001). Neither measure was related to reporting year, % women/study, or to onset or current age. Risks were greater with longer exposure, whereas incidence rates decreased with longer time at risk, possibly through 'dilution' by longer exposure. CONCLUSION: This systematic update of international experience underscores high risks of suicide attempts among patients with BD (BD-I = BD-II; women > men). Future studies should routinely include exposure times and incidence rates by diagnostic type and sex for those who attempt suicide or not.
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Transtorno Bipolar/diagnóstico , Tentativa de Suicídio/estatística & dados numéricos , Adulto , Transtorno Bipolar/psicologia , Feminino , Humanos , Incidência , Masculino , Pessoa de Meia-Idade , Fatores de Risco , Tentativa de Suicídio/psicologiaRESUMO
BACKGROUND: Previous studies have demonstrated significant variability in the processes of care and outcomes of chronic obstructive pulmonary disease (COPD) exacerbations. The AUDIPOC is a Spanish nationwide clinical audit that identified large between-hospital variations in care and clinical outcomes. Here, we test the hypothesis that these variations can be attributed to either patient characteristics, hospital characteristics and/or the so-called hospital-clustering effect, which indicates that patients with similar characteristics may experience different processes of care and outcomes depending on the hospital to which they are admitted. METHODS: A clinical audit of 5178 COPD patients consecutively admitted to 129 Spanish public hospitals was performed, with a 90-day follow-up. Multilevel regression analysis was conducted to model the probability of patients experiencing adverse outcomes. For each outcome, an empty model (with no independent variables) was fitted to assess the clustering effect, followed by a model adjusted for the patient- and hospital-level covariables. The hospital-clustering effect was estimated using the intracluster correlation coefficient (ICC); the cluster heterogeneity was estimated with the median odds ratio (MOR), and the coefficients of predictors were estimated with the odds ratio (OR). RESULTS: In the empty models, the ICC (MOR) for inpatient mortality and the follow-up mortality and readmission were 0.10 (1.80), 0.08 (1.65) and 0.01 (1.24), respectively. In the adjusted models, the variables that most represented the patients' clinical conditions and interventions were identified as outcome predictors and further reduced the hospital variations. By contrast, the resource factors were primarily unrelated with outcomes. CONCLUSIONS: This study demonstrates a noteworthy reduction in the observed crude between-hospital variation in outcomes after accounting for the hospital-cluster effect and the variables representing patient's clinical conditions. This emphasises the predictor importance of the patients' clinical conditions and interventions, and understates the impacts of hospital resources and organisational factors.
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Doença Pulmonar Obstrutiva Crônica/mortalidade , Idoso , Auditoria Clínica , Feminino , Mortalidade Hospitalar , Hospitais Públicos/estatística & dados numéricos , Humanos , Masculino , Pessoa de Meia-Idade , Razão de Chances , Readmissão do Paciente/estatística & dados numéricos , Prognóstico , Espanha/epidemiologiaRESUMO
OBJECTIVE: Mouse articular cartilage (AC) is mostly assessed by histopathology and its mechanics is poorly characterised. In this study: (1) we developed non-destructive imaging for quantitative assessment of AC morphology and (2) evaluated the mechanical implications of AC structural changes. METHODS: Knee joints obtained from naïve mice and from mice with osteoarthritis (OA) induced by destabilization of medial meniscus (DMM) for 4 and 12 weeks, were imaged by phosphotungstic acid (PTA) contrast enhanced micro-computed tomography (PTA-CT) and scored by conventional histopathology. Our software (Matlab) automatically segmented tibial AC, drew two regions centred on each tibial condyle and evaluated the volumes included. A finite element (FE) model of the whole mouse joint was implemented to evaluate AC mechanics. RESULTS: Our method achieved rapid, automated analysis of mouse AC (structural parameters in <10 h from knee dissection) and was able to localise AC loss in the central region of the medial tibial condyle. AC thickness decreased by 15% at 4 weeks and 25% at 12 weeks post DMM surgery, whereas histopathology scores were significantly increased only at 12 weeks. FE simulations estimated that AC thinning at early-stages in the DMM model (4 weeks) increases contact pressures (+39%) and Tresca stresses (+43%) in AC. CONCLUSION: PTA-CT imaging is a fast and simple method to assess OA in murine models. Once applied more extensively to confirm its robustness, our approach will be useful for rapidly phenotyping genetically modified mice used for OA research and to improve the current understanding of mouse cartilage mechanics.
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Cartilagem Articular/diagnóstico por imagem , Articulação do Joelho/diagnóstico por imagem , Osteoartrite do Joelho/diagnóstico por imagem , Tíbia/diagnóstico por imagem , Animais , Fenômenos Biomecânicos , Cartilagem Articular/patologia , Cartilagem Articular/fisiopatologia , Meios de Contraste , Modelos Animais de Doenças , Diagnóstico Precoce , Imageamento Tridimensional , Articulação do Joelho/patologia , Articulação do Joelho/fisiopatologia , Meniscos Tibiais/cirurgia , Camundongos , Osteoartrite do Joelho/patologia , Osteoartrite do Joelho/fisiopatologia , Ácido Fosfotúngstico , Tíbia/patologia , Tíbia/fisiopatologia , Microtomografia por Raio-XRESUMO
There are few reports in the literature of hepatitis as a manifestation of Parvovirus B19 infection. We describe a case of Parvovirus B19 associated acute cholestatic hepatitis diagnosed based on a positive serologic test (IgM) and molecular detection of parvovirus B19 DNA in peripheral blood. Parvovirus B19 infection should be considered in the differential diagnosis of patient presenting with acute hepatitis of unknown etiology.
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Colestase/diagnóstico , Hepatite/diagnóstico , Infecções por Parvoviridae/diagnóstico , Doença Aguda , Adolescente , Colestase/virologia , DNA Viral/sangue , Diagnóstico Diferencial , Feminino , Hepatite/virologia , Humanos , Imunoglobulina M/sangue , Parvovirus B19 Humano/isolamento & purificaçãoRESUMO
INTRODUCTION: Research has demonstrated that patients with insomnia are at an increased risk of experiencing suicidal ideation and/or making a suicide attempt. OBJECTIVES: To evaluate the relation between insomnia and suicidal behaviour. AIMS: To examine factors associated with a diagnosis of insomnia in patients admitted to an Emergency Department (ED) and assessed by the psychiatrist in charge. METHODS: Participants were 843 patients consecutively admitted to the ED of Sant'Andrea Hospital in Rome, between January 2010 and December 2011. All patients admitted were referred to a psychiatrist. A clinical interview based on the Mini International Neuropsychiatric Interview (MINI) and a semi-structured interview was conducted. Patients were asked about 'ongoing' suicidal ideation or plans for suicide. RESULTS: Forty-eight percent of patients received a diagnosis of bipolar disorder (BD), major depressive disorder (MDD) or an anxiety disorder; whereas, 17.1% were diagnosed with Schizophrenia or other non-affective psychosis. Patients with insomnia (compared to patients without insomnia) more frequently had a diagnosis of BD (23.9% vs. 12.4%) or MDD (13.3% vs. 9.5%; p < 0.001). Moreover, patients with insomnia less frequently had attempted suicide in the past 24 h (5.3% vs. 9.5%; p < 0.05) as compared with other patients, but those patients with insomnia who attempted suicide more frequently used a violent method (64.3% vs. 23.6%; p < 0.01) compared to other suicide attempters. CONCLUSIONS: Our results do not support an association between insomnia and suicidal behaviour. However, suicide attempters with insomnia more frequently used violent methods, and this phenomenon should be taken into serious consideration by clinicians.
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Distúrbios do Início e da Manutenção do Sono/complicações , Tentativa de Suicídio/psicologia , Transtorno Bipolar/complicações , Transtorno Depressivo Maior/complicações , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Cidade de Roma , Esquizofrenia/complicações , Ideação Suicida , Tentativa de Suicídio/estatística & dados numéricosRESUMO
Borderline personality disorder (BPD) is a severe psychiatric condition that affects up to 2.7% of the population and is highly linked to functional impairment and suicide. Despite its severity, there is a lack of knowledge about its pathophysiology. Studies show genetic influence and childhood violence as factors that may contribute to the development of BPD; however, the involvement of neuroinflammation in BPD remains poorly investigated. This article aimed to explore the pathophysiology of BPD according to the levels of brain-derived neurotrophic factor (BDNF), inflammatory cytokines, and oxidative stress substances that exacerbate neuronal damage. Few articles have been published on this theme. They show that patients with BPD have a lower level of BDNF and a higher level of tumor necrosis factor (TNF)-α and interleukin (IL)-6 in peripheral blood, associated with increased plasma levels of oxidative stress markers, such as malondialdehyde and 8-hydroxy-2-deoxyguanosine. Therefore, more research on the topic is needed, mainly with a pre-clinical and clinical focus.
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Transtorno da Personalidade Borderline , Humanos , Criança , Transtorno da Personalidade Borderline/epidemiologia , Transtorno da Personalidade Borderline/genética , Transtorno da Personalidade Borderline/psicologia , Fator Neurotrófico Derivado do Encéfalo/genética , Interleucina-6 , Fator de Necrose Tumoral alfaRESUMO
Introduction: In an era in which patients are becoming more and more demanding and in which there are many ways to satisfy their needs, modern implantology must consider the correct management of soft tissues during treatment planning, aiming for both functional and aesthetic rehabilitation while creating a prosthetic construction that is in harmony not only with the natural dentition of the patient but also with their face. The patient who came to our notice had a rehabilitative prosthetic implant on the left central incisor area, which did not satisfy any functional or aesthetic parameter. Furthermore, he presented an altered passive eruption in the contralateral hemiarch. Materials and Methods: The prosthetic crown was removed, the tissues were studied, and the team decided to proceed with customizing a provisional restoration that would cause the soft tissues to descend. A surgical periodontal procedure was then performed to solve the altered passive eruption condition that was also compromising the aesthetics. In conclusion, a permanent prosthetic crown was fixed into place. Discussion. Using a periodontal approach that was both surgical and prosthetic, the patient was rehabilitated correctly regaining both functions and aesthetics. It is of fundamental importance that each step in the procedure is carefully programmed; otherwise, the risk of making mistakes increases and solving the problems becomes less simple or less immediate. In order to do this, one must bear in mind that certain clinical cases can apparently concern just one tooth, yet the mouth must be considered as a whole, both functionally and aesthetically. To perform an optimal implantology, the clinician should be an expert in periodontology so that they can plan and, should it be necessary, perform all the therapeutical options (surgical and nonsurgical) that can lead to the best possible result. Conclusions: The resolution of this complex clinical case has been documented in order to share useful advice for the resolution of analogous cases. We strongly advise that each proposed procedure be planned meticulously and that the periodontological aspect of the case never be separated from the prosthetic or the implantological aspects since the integration of the periodontal tissues is of vital importance for both the functional and the aesthetic results.
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This study was performed to compare the orbital and peri-orbital morphological variations in Apert syndrome patients with different cranial vault suture synostosis, so as to provide an anatomic basis for individualized surgical planning. Computed tomography scans of 57 unoperated Apert syndrome patients and 59 controls were subgrouped as follows: type I, bilateral coronal synostosis; type II, pansynostosis; type III, perpendicular combinations of cranial vault suture synostoses. Orbit bony cavity volume was significantly reduced in type I and type II, by 19% (P < 0.001) and 24% (P < 0.001), respectively. However, the reduction of orbital cavity volume in type III did not reach statistical significance. Globe volume projection beyond the orbital rim, however, increased by 76% (P < 0.001) in type III, versus an increase of 54% (P < 0.001) in type I and 53% (P < 0.001) in type II, due to different ethmoid and sphenoid bone malformations. Maxillary bone volume was only significantly reduced in type I bicoronal synostosis (by 24%, P = 0.048). Both type I and type II developed relatively less zygoma and sphenoid bone volume. Different cranial vault suture synostoses have varied influence on peri-orbital development in Apert syndrome. Instead of mitigating the abnormalities resulting from bicoronal synostosis in type I, additional midline suture synostosis worsens the exorbitism due to a more misshaped ethmoid.
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Acrocefalossindactilia , Craniossinostoses , Acrocefalossindactilia/diagnóstico por imagem , Suturas Cranianas/diagnóstico por imagem , Craniossinostoses/diagnóstico por imagem , Craniossinostoses/cirurgia , Humanos , Lactente , Órbita/diagnóstico por imagem , Osso Esfenoide/diagnóstico por imagemRESUMO
BACKGROUND: Most plastic surgeons have encountered complications related to venous thromboembolism in their profession. However, considerable deficits in risk assessment and prophylaxis have been described. As outpatient procedures are increasing, so are concerns with regard to patient safety. The presented study investigates the global distribution pattern of patient care for mammaplasties and compares international common practices of perioperative safety measures to prevent thromboembolic events. METHODS: A questionnaire was sent to over 5000 plastic surgeons in 77 countries. The survey inquired about surgeon demographics, standard perioperative safety measures, type of thrombosis prophylaxis, and international patterns of patient care. The results were evaluated and correlated with the evidence-based literature. RESULTS: A total of 1431 surveys were gathered (response rate: 29%). The gathered data show international disparity with regard to inpatient vs. outpatient care. Mammaplasties in the USA are being performed as same-day surgeries in > 80% of cases, while globally inpatient and outpatient procedures are distributed evenly. Also, we found no international consensus with regard to safety measures to prevent thromboembolic events. Geographical regions showed differences with regard to type, dosage, timing, and duration of thrombosis prophylaxis. CONCLUSIONS: International practice patterns remain incoherent. This highlights the need for coherent and detailed global guidelines for plastic surgical interventions. High-quality studies are needed in order to establish evidence-based, standardized, and universally applicable practice guidelines.
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Mamoplastia , Trombose , Tromboembolia Venosa , Anticoagulantes , Feminino , Humanos , Mamoplastia/métodos , Assistência ao Paciente , Trombose/tratamento farmacológico , Tromboembolia Venosa/etiologia , Tromboembolia Venosa/prevenção & controleRESUMO
The use of bladder antimuscarinics is very common in the elderly. However, recent population-based studies that assessed the use of anticholinergics or bladder antimuscarinics showed an increased risk of dementia when these drugs were used for a prolonged period. Several of these population-based studies included patients who used solifenacin, which is a bladder antimuscarinic released in 2005 with the prospect of being a more selective antimuscarinic for M3 receptors (M3R), which could make it a safer drug when trying to avoid unwanted effects of older bladder antimuscarinics such as oxybutynin, especially with regard to changes in cognition. Since the various bladder antimuscarinics have distinct pharmacological characteristics, such as in the ability to penetrate the blood-brain barrier, in selectivity for muscarinic receptors, and in brain efflux mechanisms, their effects on the central nervous system (CNS) may vary. Solifenacin was the drug selected in this review, which aims to describe the results of several articles published in recent years reporting the effects of solifenacin on cognition or the risk of dementia development. Although preclinical studies show that solifenacin can also act on brain M1 receptors (M1R), short-term clinical studies have shown it to be safe for cognition. However, there are no long-term randomized studies that prove the safety of this drug for the CNS. Thus, until the safety of solifenacin has been established by long-term studies, it seems advisable to avoid prolonged use of this drug in elderly patients.
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Disfunção Cognitiva , Demência , Bexiga Urinária Hiperativa , Idoso , Disfunção Cognitiva/induzido quimicamente , Demência/induzido quimicamente , Humanos , Succinato de Solifenacina/efeitos adversos , Bexiga Urinária , Bexiga Urinária Hiperativa/tratamento farmacológicoRESUMO
BACKGROUND AND OBJECTIVES: Despite the improvement of the surgical technique and several experiences reported in literature about prosthetic incisional hernioplasty, the prevalence rate of recurrence and of the classic complications has not changed over the years. We analyze our caseload, establishing some technical cornerstones in order to reduce their occurrence. PATIENTS AND METHODS: 283 patients underwent incisional hernioplasty in our Department of Surgery in the decade 1999-2008. They were retrospectively divided into four groups (A-D) according to the surgical technique adopted for a comparative analysis: A, 37 primary direct closure; B, 207 Rives-Stoppa procedures; C, 9 Chevrel procedures; D, 30 intraperitoneal repairs. The outcomes were considered in terms of postoperative surgical complications. RESULTS: In total, we observed 11 cases of hernia recurrence (3.9%), 13 cases of infections (4.6%), 7 cases of seroma/hematoma (2.4%) and one case of acute respiratory insufficiency. DISCUSSION: The Rives-Stoppa procedure is, among all those practised, the treatment of choice in incisional hernioplasty. Thanks to the introduction of some simple modifications to this technique and preventing the postoperative infections, we obtained excellent results in terms of recurrence rate (only 1 case on 207 patients, 0.48%) and morbidity.
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Hérnia Ventral/cirurgia , Laparoscopia/métodos , Complicações Pós-Operatórias/epidemiologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Complicações Pós-Operatórias/etiologia , Desenho de Prótese , Implantação de Prótese , Recidiva , Estudos Retrospectivos , Telas CirúrgicasRESUMO
Brain tissue is a heterogeneous material, constituted by a soft matrix filled with cerebrospinal fluid. The interactions between, and the complexity of each of these components are responsible for the non-linear rate-dependent behaviour that characterises what is one of the most complex tissue in nature. Here, we investigate the influence of the cutting rate on the fracture properties of brain, through wire cutting experiments. We also present a computational model for the rate-dependent behaviour of fracture propagation in soft materials, which comprises the effects of fluid interaction through a poro-hyperelastic formulation. The method is developed in the framework of finite strain continuum mechanics, implemented in a commercial finite element code, and applied to the case of an edge-crack remotely loaded by a controlled displacement. Experimental and numerical results both show a toughening effect with increasing rates, which is linked to the energy dissipated by the fluid-solid interactions in the region surrounding the crack tip.
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Encéfalo , Análise de Elementos Finitos , GéisRESUMO
BACKGROUND: The S-Index assessed by means of electronic devices is a measure of Inspiratory Muscle Strength (IMS) that highly correlates with the maximal inspiratory pressure (MIP). The variables involved when using regression models for the prediction of IMS/MIP depend on both the sample characteristics and the device or protocol used. In light of the scarce information on the influence of physical activity (PA) on IMS in healthy older adults (OA), together with the incorporation of new assessment devices, the objectives of this research are: 1) to determine which factors influence the IMS in a group of trained OA, using a portable electronic device; and 2) to propose a regression model to improve its assessment and training. METHODS: One hundred and fifty-nine individuals were assessed for body composition, lung capacity, IMS, and PA. A total of 92 individuals (72.73 ± 4.99 years) were considered for the final sample. RESULTS: Using age, sex, and weight as control variables, forced expiratory volume in the first second (FEV1) is the variable which is most likely to be included in the model (80%), without physical fitness appearing to be decisive. In the absence of spirometric variables, cardiorespiratory fitness (6MWT) takes on this role in a predictive model (16%). CONCLUSIONS: This is the first study proposing IMS predictive formulas considering spirometry and/or physical fitness results for a Spanish, healthy, and trained OAs population. A predictive formula including also the spirometric variables (mainly the FEV1) might better predict the inspiratory muscle strength. In addition, physical and respiratory functions confirm to be different, so it is necessary for the inspiratory muscles to be trained in a specific way.
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Exercício Físico/fisiologia , Força Muscular/fisiologia , Músculos Respiratórios/fisiologia , Idoso , Idoso de 80 Anos ou mais , Composição Corporal , Aptidão Cardiorrespiratória/fisiologia , Feminino , Nível de Saúde , Humanos , Masculino , Testes de Função Respiratória , Espanha , Dispositivos Eletrônicos VestíveisRESUMO
Tracheotomy in infancy helps patients with Pfeiffer syndrome to survive by preventing respiratory crisis, but difficulty in decannulation may consequently be a challenge. This study has investigated the regional abnormalities of the nasopharyngeal airway in children with Pfeiffer syndrome to provide an anatomical basis for the surgical treatment and decannulation of the upper airway. Seventy-two preoperative computed tomograms (CT) (Pfeiffer syndrome n=30; control n=42) were included. The airway volume, cross-sectional area, and cephalometrics were measured using Materialise software. Patients with Pfeiffer syndrome developed a 50% (p<0.001) reduction of nasal airway volume, and a 44% (p=0.003) restriction in pharyngeal airway volume. In patients with Pfeiffer syndrome the cross-sectional area at the choana was only half that of the controls (p<0.001). The posterior width of the nasal airway in patients with Pfeiffer syndrome was shortened by 13% (p=0.003), and the height reduced by 21% (p<0.001). The cross-sectional areas at the condylion and gonion levels, which indicate the calibre of the pharyngeal airway at the entrance and midsection, were reduced by 67% (p<0.001) and 47% (p<0.001), respectively, when compared with the controls. The volume of the nasal airway in patients with Pfeiffer syndrome was significantly restricted in length, height, and width, and by choanal stenosis in all cases in this cohort. The reduced anteroposterior length of the nasal airway contributed to the shortened maxilla more than the anteroposterior position. The limited height and width of the nasal pathway was the result of a hypoplastic sphenoid. Restricted mediolateral and anteroposterior dimensions were evident across the entire course of the pharyngeal airway. Mediolateral maxillary expansion in addition to maxillomandibular advancement is therefore likely to benefit these patients.
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Acrocefalossindactilia , Acrocefalossindactilia/diagnóstico por imagem , Cefalometria , Criança , Tomografia Computadorizada de Feixe Cônico , Humanos , Maxila , Técnica de Expansão Palatina , Faringe/diagnóstico por imagemRESUMO
The severity of obstructive respiratory difficulty varies among affected Crouzon syndrome patients. The aim of this study was to investigate the correlation between the restricted airway volume in Crouzon syndrome and the associated type of cranial vault suture synostosis. Computed tomography scans of 68 unoperated Crouzon syndrome patients and 89 control subjects were subgrouped into four types: type I, bilateral coronal synostosis; type II, sagittal synostosis; type III, pansynostosis; type IV, perpendicular combinations of synostoses. Measurements were made using Mimics software. Of type I Crouzon patients, 42% had a restricted nasal airway (P=0.002), while the pharyngeal airway volume was not significantly reduced. Type II Crouzon patients grew normal segmental airway volumes. Crouzon patients of type III developed simultaneously reduced nasal and pharyngeal airway volumes in infancy, by 38% (P=0.034) and 51% (P=0.014), respectively. However, the nasal airway achieved a normal volume by 2 years of age without any intervention, while the pharyngeal airway remained significantly reduced up to 6 years of age, by 42% (P=0.013), compared to controls. Type IV Crouzon patients developed a reduced nasal airway volume (32%, P=0.048) and a non-significant restricted pharyngeal airway (18%, P=0.325). Airway compromise in Crouzon syndrome is variable when associated with different craniosynostosis fusion patterns. Type II (sagittal synostosis) Crouzon patients grew a normal nasopharyngeal airway volume. Those with types I (bicoronal synostosis) and IV (perpendicular synostoses) had significantly restricted nasal airways and a tendency towards a reduced pharyngeal volume. Type III (pansynostosis) Crouzon infants had the worst restriction of both airways, although there was some improvement with age.
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Disostose Craniofacial , Craniossinostoses , Suturas Cranianas/diagnóstico por imagem , Disostose Craniofacial/diagnóstico por imagem , Disostose Craniofacial/cirurgia , Craniossinostoses/diagnóstico por imagem , Craniossinostoses/cirurgia , Ossos Faciais , Humanos , Lactente , Suturas , Tomografia Computadorizada por Raios XRESUMO
The altered orbital morphology of patients with Crouzon syndrome could have an impact on the planning of treatment in diverse populations, in spite of the confounding influences of different cranial suture synostosis. This study attempted to explore the differences in orbital characteristics between Asian, Caucasian patients with Crouzon syndrome, associated pansynostosis. Eighty-six preoperative computed tomograms (CT) were included (Asian Crouzon syndrome: n=10; Asian controls: n=24; Caucasian Crouzon syndrome: n=19; Caucasian controls: n=33) and measured using Mimics software (Materialise). Unique cephalometric measurements related to orbital morphology and position were designed. Crouzon syndrome and race both have interactive effects on protrusion of the globe (p=0.009) and medial horizontal angle (p=0.012) in the assessment of orbital morphology. They also interact in the width of the ethmoid sinus (p=0.009) and influence bilateral orbital relations. The anteroposterior orbital roof in Caucasian patients with Crouzon syndrome was shortened by 4.09mm (p=0.002) compared with Caucasian controls. However, in Asian patients this dimension developed normally. The anteroposterior orbital floor was significantly reduced to a similar extent in both Asian and Caucasian Crouzon patients (both p<0.001). The visual axes in Caucasian patients with Crouzon showed more inferior rotation, by 4.38° (p=0.031) than they did in Caucasian controls, but did not achieve a statistically significant difference in other comparisons. The effect of Crouzon syndrome on orbital malformation and placement is influenced by race, especially structures related to the ethmoid sinus. Asian patients need greater infraorbital advancement for better correction of orbital proptosis and aesthetic benefits, but may require less fronto-orbital advancement than Caucasian patients.
Assuntos
Disostose Craniofacial , Craniossinostoses , Cefalometria , Disostose Craniofacial/diagnóstico por imagem , Estética Dentária , Humanos , Órbita/diagnóstico por imagem , Tomografia Computadorizada por Raios XRESUMO
The ongoing debate about the nature of coupling between climate and tectonics in mountain ranges derives, in part, from an imperfect understanding of how topography, climate, erosion, and rock uplift are interrelated. Here, we demonstrate that erosion rate is nonlinearly related to fluvial relief with a proportionality set by mean annual rainfall. These relationships can be quantified for tectonically active landscapes, and calculations based on them enable estimation of erosion where observations are lacking. Tests of the predictive power of this relationship in the Himalaya, where erosion is well constrained, affirm the value of our approach. Our model allows estimation of erosion rates in fluvial landscapes using readily available datasets, and the underlying relationship between erosion and rainfall offers the promise of a deeper understanding of how climate and tectonic evolution affect erosion and topography in space and time and of the potential influence of climate on tectonics.