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1. Verfasser:	Cecilia Correa
Format:	Artículo científico
Sprache:	es
Veröffentlicht:	Instituto Mexicano del Seguro Social 2013
Schlagworte:	Medicina glucosilceramidasa Enfermedad de Gaucher
Online-Zugang:	https://www.redalyc.org/articulo.oa?id=457745489020
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Enfermedad neuropática de Gaucher tratada con terapia de reemplazo a largo plazo Cecilia Correa Medicina glucosilceramidasa Enfermedad de Gaucher La enfermedad de Gaucher (EG) es la más común entre las patologías hereditarias por depósito lisoso- mal. Se trata de un trastorno de tipo autosómico rece- sivo, en el que se registra catabolismo y depósito de cerebrósidos en los lisosomas celulares, debido a la ausencia de la enzima glucocerebrosidasa, que se encarga de su metabolismo. La EG muestra un patrón de presentación panétnico, aunque afecta particular- mente a la población de judíos Ashkenazi. Existen varias mutaciones reconocidas entre pacientes con EG y se reconoce además a los genotipos asocia- dos con afectación neurológica (L444P —la más fre- cuente en neuronopáticos— N188S, V394L y G377S). El depósito de cerebrósidos, como material lipídico, ejerce afectación multiorgánica. En años recientes, la terapia de reemplazo enzimático (TRE) ha sido efec- tiva para controlar inde fi nidamente a los pacientes con EG. Hoy en día, se cuestiona si la TRE puede detener la afectación neurológica a largo plazo. Este documento presenta los casos clínicos de dos niños mexicanos con EG, tratados con TRE en los hospi- tales generales de zona (HGZ) del Instituto Mexicano del Seguro Social (IMSS), ambos con mutaciones que caracterizan a la EG neuronopática tipo 3, cuya evo- lución se compara con manifestaciones neurológicas y sin estas. 2013 artículo científico 0443-5117 https://www.redalyc.org/articulo.oa?id=457745489020 es http://www.redalyc.org/revista.oa?id=4577 Revista Médica del Instituto Mexicano del Seguro Social application/pdf Instituto Mexicano del Seguro Social Revista Médica del Instituto Mexicano del Seguro Social (México) Num.3 Vol.51

if (filemtime($filename) + $maxlifetime < time()) { unlink($filename); $count++; } } return $count; } /** * A function that is called internally when session data is to be saved. * * @param string $sessId The current session ID * @param string $data The session data to write * * @return bool */ protected function saveSession($sessId, $data): bool { $sessFile = $this->path . '/sess_' . $sessId; if ($handle = fopen($sessFile, 'w')) { $return = false; // Lock the file for exclusive access to avoid issues with multiple // processes writing session simultaneously: if (flock($handle, LOCK_EX)) { $return = fwrite($handle, $data); // Make sure that there's no trailing data by truncating the file to // the correct length: ftruncate($handle, strlen($data)); fflush($handle); flock($handle, LOCK_UN); } fclose($handle); if ($return !== false) { return true; } } // If we got this far, something went wrong with the file output... // It is tempting to throw an exception here, but this code is called // outside of the context of exception handling, so all we can do is // echo a message.

// Anecdotal testing Today and Yesterday seems to indicate destroy() // is called by the garbage collector and everything is good. // Something to keep in mind though. return 0; } /** * Write function that is called when session data is to be saved. * * @param string $sessId The current session ID * @param string $data The session data to write * * @return bool */ public function write($sessId, $data): bool { if ($this->writesDisabled) { return true; } return $this->saveSession($sessId, $data); } /** * A function that is called internally when session data is to be saved. * * @param string $sessId The current session ID * @param string $data The session data to write * * @return bool */ abstract protected function saveSession($sessId, $data): bool; }

* * @return void */ public function writeClose() { // The assumption is that we're using PHP's ext/session. // session_write_close() will actually overwrite $_SESSION with an // empty array on completion -- which leads to a mismatch between what // is in the storage object and $_SESSION. To get around this, we // temporarily reset $_SESSION to an array, and then re-link it to // the storage object. // // Additionally, while you _can_ write to $_SESSION following a // session_write_close() operation, no changes made to it will be // flushed to the session handler. As such, we now mark the storage // object isImmutable. $storage = $this->getStorage(); if (! $storage->isImmutable()) { $_SESSION = $storage->toArray(true); session_write_close(); $storage->fromArray($_SESSION); $storage->markImmutable(); } } /** * Attempt to set the session name * * If the session has already been started, or if the name provided fails * validation, an exception will be raised. * * @param string $name * @return SessionManager * @throws Exception\InvalidArgumentException */ public function setName($name) { if ($this->sessionExists()) { throw new Exception\InvalidArgumentException( 'Cannot set session name after a session has already started'

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