Abstract: Provided are methods for treatment of Fabry disease in patients having HEK assay amenable mutations in ?-galactosidase A. Certain methods comprise administering migalastat or a salt thereof every other day, such as administering about 150 mg of migalastat hydrochloride every other day.

Abstract: A mechanical trigger device comprises a reset screw configured to re-assemble the mechanical trigger device. The resettable screw may be disposed in a slot in a housing of the mechanical trigger device and coupled to a firing pin disposed within the housing. The reset screw may be configured to translate the firing pin within the housing and re-engage a sear after use of the mechanical trigger device.

Abstract: A parachute assembly may comprise a canopy and one or more reefing lines. The one or more reefing lines may limit inflation of the canopy to attenuate impulse load or otherwise mitigate forces exerted by the parachute assembly on cargo or an occupant during deployment of the canopy. The one or more reefing lines may be broken in stages to selectively limit inflation of the canopy followed by selectively permitting inflation of the canopy in stages.

Abstract: A roll stabilization system for an aircraft ejection seat may be provided. An integral seat sequencer may activate an ejection sequence causing an aircraft ejection seat to be ejected from an aircraft. In various instances, particularly with lighter occupants of the aircraft ejection seat, the aircraft ejection seat may begin a rotational movement. To ameliorate this rotational movement, a roll correction rocket motor is installed on the aircraft ejection seat and selectively activated by the integral seat sequencer in the event that an undesired rotational movement is detected.

Abstract: An ejection seat may comprise a seatback, a headrest located at an upper end of the seatback, and a pitot tube rotatably coupled to the headrest. A pitot restraint assembly may be operably coupled to the pitot tube. The pitot restraint assembly may be configured to translate between a restrained state and a released state. The pitot tube may rotate from a stowed position to a deployed position in response to the pitot restraint assembly translating to the released state.

Abstract: An ejection seat may comprise a seatback, a headrest located at an upper end of the seatback, and a pitot tube rotatably coupled to the headrest. A pitot restraint assembly may be operably coupled to the pitot tube. The pitot restraint assembly may be configured to translate between a restrained state and a released state. The pitot tube may rotate from a stowed position to a deployed position in response to the pitot restraint assembly translating to the released state.

Abstract: An output device and/or an output device assembly configured to be initiated in response to different types of input. The disclosed output device may be generally configured so as to be able to receive different types of input in order to produce an output. For example, the two different types of input may be mechanical force and fluid pressure, and thus the disclosed output device may be able to receive either form of input and convert either input into the desired/predetermined output.

Abstract: An article of manufacture may include a tangible, non-transitory computer-readable storage medium having instructions stored thereon for controlling deployment of aircraft escape and ejection seat subsystems. The instructions, in response to execution by a first sequencer, cause the first sequencer to perform operations which may comprise receiving a power input, determining a seat location and a seat identity of a first ejection seat in which the first sequencer is installed, determining an ejection mode, sending a first deploy command to an escape path clearance subsystem, determining a deployment sequence for a seat rocket catapult subsystem and a plurality of ejection seat subsystems of the first ejection seat based on the seat location, the seat identity, and the ejection mode, sending a second deploy command to the seat rocket catapult subsystem, and sending a series of third deploy commands to the plurality of ejection seat subsystems.

Abstract: An article of manufacture may include a tangible, non-transitory computer-readable storage medium having instructions stored thereon for controlling deployment of aircraft escape and ejection seat subsystems. The instructions, in response to execution by a first sequencer, cause the first sequencer to perform operations which may comprise receiving a power input, determining a seat location and a seat identity of a first ejection seat in which the first sequencer is installed, determining an ejection mode, sending a first deploy command to an escape path clearance subsystem, determining a deployment sequence for a seat rocket catapult subsystem and a plurality of ejection seat subsystems of the first ejection seat based on the seat location, the seat identity, and the ejection mode, sending a second deploy command to the seat rocket catapult subsystem, and sending a series of third deploy commands to the plurality of ejection seat subsystems.

Abstract: A high energy catapult assembly may comprise an outer tube and an inner tube located within the outer tube. The outer tube may be configured to telescope relative to the inner tube. A propellant cartridge may be located in an inner tube chamber of the inner tube. An actuator may be configured to change an open volume of the inner tube chamber.

Abstract: A high energy catapult assembly may comprise an outer tube and an inner tube located within the outer tube. The outer tube may be configured to telescope relative to the inner tube. A propellant cartridge may be located in an inner tube chamber of the inner tube. An actuator may be configured to change an open volume of the inner tube chamber.

Abstract: A rocket catapult assembly for an ejection seat may comprise a motor assembly including a proximal end and a distal end, a first cartridge, and a second cartridge. The first cartridge and the second cartridge may be configured to provide a variable thrust based on an occupant's weight. The first cartridge may provide a thrust corresponding to a relatively light weight occupant, the second cartridge may provide a thrust corresponding to a relatively average weight occupant, and the first cartridge and second cartridge may provide a combined thrust corresponding to a relatively heavy weight occupant.

Abstract: An ejection seat for an aircraft is disclosed. In various embodiments, the ejection seat includes a seat frame; and a moveable headrest attached to the seat frame, the moveable headrest including a first piercer assembly configured for fracturing a canopy.

Abstract: An output device and/or an output device assembly configured to be initiated in response to different types of input. The disclosed output device may be generally configured so as to be able to receive different types of input in order to produce an output. For example, the two different types of input may be mechanical force and fluid pressure, and thus the disclosed output device may be able to receive either form of input and convert either input into the desired/predetermined output.

Abstract: A severance cutter may comprise a housing and a blade may be coupled to the housing. A restraint support may be configured to translate relative to the blade. A spring may be configured to bias a support opening defined by the restraint support away from the blade.

Abstract: A rocket catapult assembly for an ejection seat may comprise a drive motor, a metering tube, a first cartridge, and a second cartridge. The metering tube may include an outer wall having a gas pervious section and a gas impervious section. The drive motor may be configured to translate the metering tube and align the gas pervious section or gas impervious section with a first cartridge and a second cartridge to produce a desired thrust of the rocket catapult assembly.

Abstract: A rocket catapult assembly for an ejection seat may comprise a motor assembly including a proximal end and a distal end, an outlet tube coupled to the distal end, and a valve coupled to the outlet tube. The valve may be configured to vary an operating pressure based on an occupant's weight. The valve may be in a closed configuration, on open configuration, or a partially open configuration during ejection of the ejection seat.

Abstract: An adjustable leg restraint for an ejection seat may be configured to translate between an extended state and a nonextended state. The adjustable leg restraint may include a leg located proximate a side panel of the ejection seat. An extension flange may be coupled to the leg guard. The extension flange may be configured to translate the leg guard relative to the side panel of the ejection seat.

Abstract: A mechanical trigger device comprises a reset screw configured to re-assemble the mechanical trigger device. The resettable screw may be disposed in a slot in a housing of the mechanical trigger device and coupled to a firing pin disposed within the housing. The reset screw may be configured to translate the firing pin within the housing and re-engage a sear after use of the mechanical trigger device.

Abstract: A rocket catapult assembly for an ejection seat may comprise a drive motor, a metering tube, a first cartridge, and a second cartridge. The metering tube may include an outer wall having a gas pervious section and a gas impervious section. The drive motor may be configured to translate the metering tube and align the gas pervious section or gas impervious section with a first cartridge and a second cartridge to produce a desired thrust of the rocket catapult assembly.