High morbid-mortability and reduced level of osteoporosis diagnosis among elderly people who had hip fractures in São Paulo City

OBJECTIVE: To know the morbid-mortality following an osteoporotic hip fracture in elderly patients living in São Paulo. PATIENTS AND METHODS: This study evaluated prospectively all patient over 60 years admitted in 2 school-hospitals in the city of São Paulo in a following 6-month period due to a osteoporotic proximal femur fracture. All of them filled up the Health Assessment Questionnaire (HAQ) and had their chart reviewed. After 6 months they were re-interviewed. Linear regression analysis was utilized to determine the factors related to functional ability. RESULTS: 56 patients were included (mean age 80.7 ± 7.9 years old, 80.4% females). After the 6-month follow up the mortality rate was 23.2%. Only 30% of the patients returned to their previous activities, and 11.6% became totally dependent. Factors related to worse functional ability after fracture were HAQ before fracture, institutionalization after fracture and age (r² 0.482). The diagnosis of osteoporosis was informed only by 13.9% of them, and just 11.6% received any treatment for that. CONCLUSION: Our results showed the great impact of these fractures on mortality and in the functional ability of these patients. Nevertheless, many of our physicians do not inform the patients about the diagnosis of osteoporosis and, consequently, the treatment of this condition is jeopardized.As fraturas osteoporóticas de fêmur proximal trazem graves conseqüências quanto à morbimortalidade e à qualidade de vida, mas desconhece-se este impacto no Brasil. OBJETIVO: Conhecer a morbimortalidade decorrente deste tipo de fraturas em idosos na cidade de São Paulo. MÉTODOS: Foram incluídos todos os pacientes com mais de 60 anos internados por fraturas de fêmur proximal durante seis meses, em dois hospitais de São Paulo. Os pacientes preencheram o questionário de capacidade funcional (HAQ), tiveram seu prontuário examinado e foram reavaliados após seis meses. Utilizou-se a análise de regressão linear para determinar os fatores relacionados à capacidade funcional. RESULTADOS: Cinqüenta e seis pacientes foram incluídos no estudo (80,7 ± 7,9 anos; 80,4% mulheres). A mortalidade em seis meses foi de 23,2%. Apenas 30% retornaram plenamente às suas atividades prévias e 11,6% tornaram-se completamente dependentes. Os fatores que mais bem conseguiram prever pior capacidade funcional após a fratura foram HAQ pré-fratura, institucionalização pós-fratura e idade (r² 0,482). Somente 13,9% receberam o diagnóstico de osteoporose e 11,6% iniciaram algum tratamento. CONCLUSÕES: Os resultados do presente estudo demonstram o impacto deste tipo de fraturas sobre a mortalidade e a capacidade funcional. Entretanto, a falha médica no diagnóstico e na orientação de tratamento da osteoporose permanece elevada.Universidade Federal de São Paulo (UNIFESP) Escola Paulista de MedicinaSanta Casa de Misericórdia de São Paulo Departamento de OrtopediaUNIFESP-EPM EPMUNIFESP, EPM, EPMSciEL

Loss-of-function mutations in UDP-Glucose 6-Dehydrogenase cause recessive developmental epileptic encephalopathy

Developmental epileptic encephalopathies are devastating disorders characterized by intractable epileptic seizures and developmental delay. Here, we report an allelic series of germline recessive mutations in UGDH in 36 cases from 25 families presenting with epileptic encephalopathy with developmental delay and hypotonia. UGDH encodes an oxidoreductase that converts UDP-glucose to UDP-glucuronic acid, a key component of specific proteoglycans and glycolipids. Consistent with being loss-of-function alleles, we show using patients’ primary fibroblasts and biochemical assays, that these mutations either impair UGDH stability, oligomerization, or enzymatic activity. In vitro, patient-derived cerebral organoids are smaller with a reduced number of proliferating neuronal progenitors while mutant ugdh zebrafish do not phenocopy the human disease. Our study defines UGDH as a key player for the production of extracellular matrix components that are essential for human brain development. Based on the incidence of variants observed, UGDH mutations are likely to be a frequent cause of recessive epileptic encephalopathy

Loss-of-function mutations in UDP-Glucose 6-Dehydrogenase cause recessive developmental epileptic encephalopathy

AbstractDevelopmental epileptic encephalopathies are devastating disorders characterized by intractable epileptic seizures and developmental delay. Here, we report an allelic series of germline recessive mutations in UGDH in 36 cases from 25 families presenting with epileptic encephalopathy with developmental delay and hypotonia. UGDH encodes an oxidoreductase that converts UDP-glucose to UDP-glucuronic acid, a key component of specific proteoglycans and glycolipids. Consistent with being loss-of-function alleles, we show using patients’ primary fibroblasts and biochemical assays, that these mutations either impair UGDH stability, oligomerization, or enzymatic activity. In vitro, patient-derived cerebral organoids are smaller with a reduced number of proliferating neuronal progenitors while mutant ugdh zebrafish do not phenocopy the human disease. Our study defines UGDH as a key player for the production of extracellular matrix components that are essential for human brain development. Based on the incidence of variants observed, UGDH mutations are likely to be a frequent cause of recessive epileptic encephalopathy.</jats:p

IGBT Tail Current Reduction by Current Injection Technique

In this paper, experiments where current was injected into an IGBT (Insulated Gated Bipolar Transistor) during its turn-off transient were carried out. Results showed a significant reduction in the IGBT’s collector tail current which could increase the operating frequency for an application. These experiment includes a switching transient test circuit to simulate the collector tail currents, and the implementation of a current injection circuit (CIC) and a non-invasive current injection circuit (NICIC) into the experiments to reduce the collector tail current

High Power Switching Devices: Past, Present and Future

Switching devices are key components in any power electronic circuit or system as they control and limit the flow of power from the source to the load. Their power level requirements (current & voltage) and switching frequency are continually increasing in the power electronic industry, and this demands larger and faster switching devices. This paper will focus on the development of high power switching devices and will present an up to date perspective of switching device technology and materials. The most important material has been and still is silicon (Si) for solid-state semiconductor devices. It dominates the world market at present, particularly in its crystalline form. However, silicon power device operation is generally limited to relatively low frequency and temperature. Silicon Carbide, Gallium Nitride and Diamond offer the potential to overcome the frequency, temperature and power management limitations of silicon. A large number of new concepts and materials are still in the research stage. At present, Silicon Carbide is considered to have the best trade-off between material properties and commercial maturity. Multilayer Silicon Carbide (SiC) power semiconductor devices being in development are promising devices for the near future, but long term reliability, crystal degradation and forward voltage drift problems need to be solved before commercialisation

Electronic Circuits for Switching-time Reduction of Bipolar Semiconductor Devices

Bipolar semiconductor devices are often used as switches in very high power electronic circuits and systems. They have replaced the old conventional gas filled tubes and vacuum devices in many applications. This is mainly due to the fact that solid-state devices are more efficient, smaller in size, cheaper and more reliable. In addition, solid-state devices are considered environmental friendly, since they do not contain nasty gases and toxic materials used in old devices. The power level requirements and switching frequency are continually increasing in the power electronic industry, and this demands larger and faster switching devices. As a result, both bipolar and unipolar semiconductor devices have undergone continued improvement in current and voltage ratings, and switching speed. The main advantage of bipolar devices is their low conduction losses due to conductivity modulation, but their main disadvantage is the high switching losses which is due to minority carrier injection. The Insulated Gate Bipolar Transistor (IGBT) combines the advantages of both unipolar and bipolar devices. It has a simple gate drive circuit like that of the MOSFET, with high current and low saturation voltage capability of bipolar transistor. The main problem remains with the relatively long tail turn-off current. To reduce the turn-off time of the IGBT and other bipolar devices, different lifetime control techniques and structural changes have been developed and used. Details of these and new techniques developed by using auxiliary electronic circuits for reducing the turn-off time and increasing the switching speed of bipolar semiconductor devices are presented in this paper

Enhancing the Efficacy of Stem Cell Therapy with Glycosaminoglycans

10.1016/j.stemcr.2019.12.003Stem Cell Reports141105-12