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1. Miastenia Gravis: La falla en la comunicación neuromuscular La Miastenia Gravis (MG) no es un problema del músculo en sí, sino de la transmisión de la señal nerviosa hacia él. Es una enfermedad autoinmune y fluctuante. ¿Dónde ocurre? En la membrana postsináptica de la unión neuromuscular. El nervio suelta la acetilcolina (el mensajero), pero el músculo no puede recibirla correctamente. Los culpables (Anticuerpos): El sistema inmune destruye los receptores. El documento menciona tres tipos: Anti-AChR (85%): Los más comunes. Anti-MuSK (10-15%): Suelen dar cuadros más severos, con mucha afectación de cara, cuello y músculos respiratorios. Anti-LRP4 (5%): Casos más leves. Nota clínica clave: Que los análisis salgan negativos (seronegativa) no descarta la enfermedad. Clínica (El día a día del paciente): La palabra clave aquí es fatigabilidad. El paciente se levanta bien, pero a medida que usa los músculos, se agotan. Ptosis y Diplopía: Párpado caído y visión doble (los músculos oculares son los primeros en afectarse). Crisis Miasténica: Es la complicación más grave. El diafragma se debilita tanto que el paciente entra en paro respiratorio. Diagnóstico: Además del laboratorio y la electromiografía (EMG), destaca la Prueba del Hielo (el frío mejora temporalmente la transmisión neuromuscular y levanta el párpado caído) y la TAC de Tórax. Esta última es vital porque la MG está súper ligada a alteraciones del timo (hiperplasia o timoma), que es donde se "entrenan" mal esos anticuerpos. 2. Distrofias Musculares: El daño estructural del músculo A diferencia de la Miastenia, aquí el problema sí es estructural y genético. Es una distrofinopatía. La proteína ausente (Distrofina): Imagina que la distrofina es el "amortiguador" o el pegamento que protege la membrana de la célula muscular cada vez que se contrae. Sin ella, el músculo se rompe con el uso y se destruye progresivamente. Genética: Ligada al cromosoma X (Xp21). Las mujeres suelen ser portadoras sanas y los hombres desarrollan la enfermedad. Duchenne vs. Becker: Duchenne: Es la forma grave. No hay nada de distrofina. Inicia en la infancia temprana (2-3 años) y el deterioro es rápido. Becker: Es más noble. Hay distrofina, pero es defectuosa o insuficiente. Inicia más tarde (adolescencia o adultez) y progresa lento. Signos Clínicos Típicos: Debilidad Progresiva NO Fluctuante: Aquí no hay días buenos o malos como en la Miastenia; el músculo se va perdiendo. Empieza en la cintura pélvica. Signo de Gowers: Como los músculos de la pelvis y piernas están débiles, el niño tiene que "escalar sobre sí mismo" usando las manos para poder ponerse de pie. Pseudohipertrofia de gemelos: Las pantorrillas se ven grandes y fuertes, pero es un engaño; el músculo destruido fue reemplazado por grasa y tejido conectivo. Marcha Anserina: Camina balanceando la cadera de un lado a otro (como un pato) por debilidad del glúteo medio. Tratamiento: Se usan corticoides para retrasar la pérdida de fuerza y proteger la función pulmonar/cardíaca el mayor tiempo posible. 3. Parálisis Cerebral (PC): Lesión en un cerebro en desarrollo La PC es una lesión fija (no va a empeorar ni a extenderse), pero ocurre en un cerebro inmaduro, lo que altera el desarrollo motor de por vida. Los detonantes principales: El documento apunta directo a la prematurez (< 36 semanas) y al bajo peso (< 2500g). Los vasos sanguíneos de un bebé prematuro son sumamente frágiles; ante cualquier cambio de presión o falta de oxígeno (hipoxia), se rompen o se infartan las zonas cercanas a los ventrículos cerebrales (vía piramidal), dejando secuelas motoras. Clasificación según la zona dañada: 1 Espástica (80-85%): Daño en la corteza cerebral / vía piramidal. Los músculos están rígidos, tensos y los reflejos exagerados. Dependiendo de qué lóbulo o zona afecte, puede asociarse a epilepsia o problemas cognitivos. 2 Discinética/Atetoide (10-15%): Daño en los núcleos basales. Produce movimientos involuntarios, descoordinados y cambios bruscos en el tono muscular (pasa de rígido a flácido). Se relaciona con posturas distónicas o movimientos de corea. 3 Atáxica (5%): Daño en el cerebelo. El principal problema es el equilibrio, la coordinación de movimientos y el tono muscular bajo (hipotonía), acompañado a veces de nistagmus (movimiento involuntario de los ojos). Cambios estructurales visibles en imagen (USG/RMN): Destaca la atrofia periventricular, que lesiona directamente las fibras que controlan el movimiento de las piernas y brazos, y la ventriculomegalia (dilatación de los ventrículos por pérdida de tejido cerebral circundante)
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Where is the main fuel strainer located in the aircraft fuel system? :: At the lowest point in the fuel system. If carburetor heat is applied on an aircraft with a fuel injection system, what happens? :: Carburetor heat is not installed. During idle mixture adjustments, what indicates the correct mixture has been achieved? :: Slight rise in RPM. What can cause an engine with a float-type carburetor to run rich at full throttle? :: Fuel level in the float bowl set too high. What happens if the idling jet becomes clogged in a float-type carburetor? :: The engine will not idle. What fuel/air mixture ratio normally produces maximum power in a reciprocating engine? :: 12:1. What results from excessively rich or lean idle mixtures? :: Incomplete combustion. What is the primary function of boost pumps in a fuel system? :: Provide a positive flow of fuel to the engine pump. What is the purpose of an engine-driven fuel pump bypass valve? :: Prevent a damaged or inoperative pump from blocking fuel flow from another pump. Where must a fuel strainer or filter be located? :: Between the tank outlet and the fuel metering device. Which is NOT a function of the carburetor venturi? :: Regulates the idle system. What corrective action should be taken if a carburetor leaks fuel from the discharge nozzle? :: Replace the needle valve and seat. How is the float level commonly adjusted in a float-type carburetor? :: Bend tab or add/remove shims under the needle valve. When should engine idle speed and mixture settings be adjusted? :: With the engine warmed up and operating. What is the function of the economizer system in a float-type carburetor? :: Supplies additional fuel above cruising power. When are fuel boost pumps operated? :: To provide a positive flow of fuel to the engine. On a carburetor without automatic mixture control, what happens to the mixture as altitude increases? :: It becomes richer. Where is the engine fuel shutoff valve usually located? :: Aft of the firewall. What is true regarding proper throttle rigging? :: The throttle stop on the carburetor must contact before the cockpit stop. What decreases reciprocating engine power at all altitudes? :: Increased humidity. When should float carburetors normally be overhauled? :: At engine overhaul. Why should fuel lines avoid sharp curves and steep rises/falls? :: To prevent vapor lock. How is float level measured in a float-type carburetor? :: From the fuel level to the parting surface of the carburetor. Why are fuel lines kept away from heat and sharp bends? :: To reduce vapor lock. At idle speed, where is fuel discharged in a float-type carburetor? :: From the idle discharge nozzle. What carburetor component limits maximum airflow at full throttle? :: Venturi. When an electric primer is used, fuel pressure is supplied by what? :: Boost pump. Why does an aircraft carburetor have a mixture control? :: To prevent the mixture from becoming too rich at high altitudes. What must all aircraft fuel systems include? :: A positive means of shutting off fuel to all engines. What additional effect does a carburetor air scoop provide? :: Increases incoming air pressure by ram effect. What happens when carburetor heat is applied? :: The mixture becomes richer. What does an increase in RPM or manifold pressure after applying carburetor heat indicate? :: Carburetor ice was forming. If an engine runs rough at high power and smooths out when leaned, what is the probable cause? :: Excessively rich mixture. What is the function of the venturi in a carburetor? :: Creates a vacuum to draw fuel into the airstream. What can result from an excessively rich fuel mixture? :: Carbon buildup on spark plugs. What is the primary purpose of the mixture control lever? :: Adjust fuel flow for changes in altitude. What typically happens to engine performance when carburetor heat is applied? :: Engine RPM decreases. What device prevents or eliminates carburetor icing? :: Carburetor heat. What condition is most likely to cause carburetor icing? :: High humidity and moderate temperatures. What is the main disadvantage of a float-type carburetor compared to fuel injection? :: Increased risk of carburetor icing. What is the most common fuel metering device used in small aircraft engines? :: Float-type carburetor. What is the primary function of a carburetor? :: Mix fuel and air in the correct ratio for combustion. What is the primary function of an aircraft induction system? :: Deliver air to the engine for combustion. What are light aircraft engines usually equipped with? :: Carburetor or fuel injection system. What does a typical induction system on a naturally aspirated engine consist of? :: All of these. What does a turbocharger use to increase manifold pressure? :: Exhaust gases. How does altitude affect a normally aspirated engine? :: Decreases power output. What is a disadvantage of a carbureted induction system? :: Higher risk of icing. How does an alternate air system function? :: Allows the engine to draw unfiltered air if the main filter is blocked. What is a common method for checking induction leaks on a carbureted engine? :: Look for blue stains near the induction manifold and use a soapy water solution. On small aircraft engines, how may fuel vaporization be increased? :: By circulating the fuel-air mixture through passages in the oil sump. What additional effect can a carburetor airscoop provide? :: Increase the pressure of incoming air by ram effect. What is true regarding volumetric efficiency? :: Supercharging can increase volumetric efficiency above 100%. What fluid is commonly used for reciprocating engine induction system deicing? :: Alcohol. What is the system called when the fuel-air mixture flows through passages in the oil sump? :: Hot Spot Induction. What is the most satisfactory extinguishing agent for a tailpipe or intake fire? :: Carbon dioxide. What effect does applying carburetor heat have during engine operation? :: Decreases the weight of the fuel-air charge. If carburetor heat is applied and no icing is present, what happens? :: The mixture becomes richer. What happens in some aircraft if the induction air filter becomes blocked? :: The system automatically allows warm, unfiltered air into the engine. What should an operator do if an induction fire starts during engine starting? :: Continue cranking the engine. What effect does carburetor heat have on the mixture? :: The mixture becomes richer. What does an increase in RPM or manifold pressure after applying carburetor heat indicate? :: Ice was forming in the carburetor. In what position should the carburetor heat control be during engine starting? :: Cold or Off. Is carburetor heated air filtered? :: No. What can result from using carburetor heat when it is not needed? :: Decrease in power and possible detonation. What part of an aircraft will usually accumulate ice first in flight? :: Carburetor. How may carburetor icing be eliminated? :: Alcohol spray and heated induction air. What is the most common method of preventing carburetor icing? :: Preheating the intake air. Where would the carburetor air heat valve be located in a fuel injection system? :: None is required. What are the two most common types of aircraft induction air filters? :: Dry paper filters and wetted mesh filters
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