<?xml version="1.0" encoding="ISO-8859-1"?><article xmlns:mml="http://www.w3.org/1998/Math/MathML" xmlns:xlink="http://www.w3.org/1999/xlink" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance">
<front>
<journal-meta>
<journal-id>1726-6718</journal-id>
<journal-title><![CDATA[Revista Cubana de Anestesiología y Reanimación]]></journal-title>
<abbrev-journal-title><![CDATA[Rev cuba anestesiol reanim]]></abbrev-journal-title>
<issn>1726-6718</issn>
<publisher>
<publisher-name><![CDATA[Editorial Ciencias Médicas]]></publisher-name>
</publisher>
</journal-meta>
<article-meta>
<article-id>S1726-67182022000200011</article-id>
<title-group>
<article-title xml:lang="es"><![CDATA[Manejo anestésico de una gestante con síndrome de Morquio]]></article-title>
<article-title xml:lang="en"><![CDATA[Anesthetic Management for a pregnant woman with Morquio syndrome]]></article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Valdés Torres]]></surname>
<given-names><![CDATA[Alejandro]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Veloz Gómez]]></surname>
<given-names><![CDATA[Irina]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[García Cid]]></surname>
<given-names><![CDATA[Shemanet]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[de la Paz]]></surname>
<given-names><![CDATA[María Elena]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[López]]></surname>
<given-names><![CDATA[Julio César]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
</contrib-group>
<aff id="Af1">
<institution><![CDATA[,Hospital Provincial General Docente &#8220;Roberto Rodríguez&#8221;  ]]></institution>
<addr-line><![CDATA[Morón Ciego de Ávila]]></addr-line>
<country>Cuba</country>
</aff>
<pub-date pub-type="pub">
<day>00</day>
<month>08</month>
<year>2022</year>
</pub-date>
<pub-date pub-type="epub">
<day>00</day>
<month>08</month>
<year>2022</year>
</pub-date>
<volume>21</volume>
<numero>2</numero>
<copyright-statement/>
<copyright-year/>
<self-uri xlink:href="http://scielo.sld.cu/scielo.php?script=sci_arttext&amp;pid=S1726-67182022000200011&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.sld.cu/scielo.php?script=sci_abstract&amp;pid=S1726-67182022000200011&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.sld.cu/scielo.php?script=sci_pdf&amp;pid=S1726-67182022000200011&amp;lng=en&amp;nrm=iso"></self-uri><abstract abstract-type="short" xml:lang="es"><p><![CDATA[RESUMEN  Introducción:  El síndrome de Morquio es una enfermedad hereditaria autosómica recesiva con distintos grados de afectación al metabolismo de los glúcidos, lo que genera incapacidad para romper los enlaces de las cadenas largas de glucosamiglicanos, esto provoca acumulación de mucopolisacáridos en distintos tejidos del cuerpo humano.  Objetivo:  Describir el manejo anestésico de una gestante con síndrome de Morquio.  Presentación del caso:  Gestante primigesta de 30 años de edad, de raza negra, de 103 cm de estatura y 33 Kg de peso. Acude a consulta preoperatoria por presentar embarazo a término, baja talla y se realizó interrupción del embarazo por vía alta. Se procede a la valoración preanestésica donde se recoge antecedentes de enfermedad genética e ingreso previo por presentar cifras elevadas de tensión arterial. La paciente padecía de alergia a la dipirona.  Conclusiones:  Los pacientes con mucopolisacaridosis tienen una alta incidencia de dificultad para la ventilación y la intubación endotraqueal asociada con insuficiencia cardiopulmonar. La afectación de la columna presenta dificultades adicionales para los anestesiólogos. Cualquier cirugía electiva requiere una evaluación preoperatoria de los factores de riesgo anestesiológicos y la disponibilidad de un espectro de equipos para el manejo de las vías respiratorias. La anestesia debe ser realizada por un equipo con experiencia en el manejo de la vía aérea.]]></p></abstract>
<abstract abstract-type="short" xml:lang="en"><p><![CDATA[ABSTRACT  Introduction:  Morquio syndrome is an autosomal recessive hereditary disease that affects, to different extents, carbohydrate metabolism, which obstructs the ability to break bonds of long chains of glycosaminoglycans, causing mucopolysaccharides accumulation in different tissues of the human body.  Objective:  To describe the anesthetic management of a pregnant woman with Morquio syndrome.  Case presentation:  This is the case of a 30-year-old primigravid pregnant woman, of black skin, 103 cm of height and 33 kg of weight. She came for preoperative consultation because she was pregnant at term and had low body size; the pregnancy was terminated through the abdominal route. A preanesthetic assessment was performed, which permitted to observe a history of genetic disease and previous admission for high blood pressure. The patient was allergic to dipyrone.  Conclusions:  Among patients with mucopolysaccharidosis, there is a high incidence of difficulty for ventilation and endotracheal intubation associated with cardiopulmonary insufficiency. Spinal involvement represents additional difficulties for anesthesiologists. Any elective surgery requires preoperative assessment of anesthesiologic risk factors and the availability of a spectrum of airway management equipment. Anesthetic managment should be performed by a team experienced in airway management.]]></p></abstract>
<kwd-group>
<kwd lng="es"><![CDATA[mucopolisacaridosis IV]]></kwd>
<kwd lng="es"><![CDATA[anestesia general]]></kwd>
<kwd lng="es"><![CDATA[manejo de la vía aérea]]></kwd>
<kwd lng="en"><![CDATA[mucopolysaccharidosis IV]]></kwd>
<kwd lng="en"><![CDATA[general anesthesia]]></kwd>
<kwd lng="en"><![CDATA[airway management]]></kwd>
</kwd-group>
</article-meta>
</front><back>
<ref-list>
<ref id="B1">
<label>1</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Viviana]]></surname>
<given-names><![CDATA[Crespo Vallejo Dayssy]]></given-names>
</name>
<name>
<surname><![CDATA[Alexandra]]></surname>
<given-names><![CDATA[Rodríguez Pacheco Valeria]]></given-names>
</name>
<name>
<surname><![CDATA[Alejandra]]></surname>
<given-names><![CDATA[Ruiz Ayerve Mayra]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Síndrome de Morquio, una afección infrecuente]]></article-title>
<source><![CDATA[Rev Eug Esp]]></source>
<year>2021</year>
<volume>15</volume>
<numero>1</numero>
<issue>1</issue>
<page-range>66-72</page-range></nlm-citation>
</ref>
<ref id="B2">
<label>2</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Moretto]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Bosatra]]></surname>
<given-names><![CDATA[MG]]></given-names>
</name>
<name>
<surname><![CDATA[Marchesini]]></surname>
<given-names><![CDATA[L]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Anesthesiological risks in mucopolysaccharidoses]]></article-title>
<source><![CDATA[Ital J Pediatr]]></source>
<year>2018</year>
<volume>44</volume>
<page-range>116</page-range></nlm-citation>
</ref>
<ref id="B3">
<label>3</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Hung]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
<name>
<surname><![CDATA[Hernández]]></surname>
<given-names><![CDATA[G]]></given-names>
</name>
<name>
<surname><![CDATA[Briceño]]></surname>
<given-names><![CDATA[Y]]></given-names>
</name>
<name>
<surname><![CDATA[Silvestre]]></surname>
<given-names><![CDATA[R]]></given-names>
</name>
<name>
<surname><![CDATA[Barrios]]></surname>
<given-names><![CDATA[MC]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Síndrome de Morquio como causa infrecuente de talla baja desproporcionada: Enfoque fisiopatológico, diagnóstico y terapéutico. A propósito de un caso]]></article-title>
<source><![CDATA[Rev Vene Endocrinol Metab]]></source>
<year>2016</year>
<volume>14</volume>
<numero>3</numero>
<issue>3</issue>
<page-range>217-25</page-range></nlm-citation>
</ref>
<ref id="B4">
<label>4</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Tomatsu]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
<name>
<surname><![CDATA[Averill]]></surname>
<given-names><![CDATA[LW]]></given-names>
</name>
<name>
<surname><![CDATA[Sawamoto]]></surname>
<given-names><![CDATA[K]]></given-names>
</name>
<name>
<surname><![CDATA[Mackenzie]]></surname>
<given-names><![CDATA[WG]]></given-names>
</name>
<name>
<surname><![CDATA[Bober]]></surname>
<given-names><![CDATA[MB]]></given-names>
</name>
<name>
<surname><![CDATA[Pizarro]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Obstructive airway in Morquio A syndrome, the past, the present and the future]]></article-title>
<source><![CDATA[Mol Genet Metab]]></source>
<year>2016</year>
<volume>117</volume>
<numero>2</numero>
<issue>2</issue>
<page-range>150-6</page-range></nlm-citation>
</ref>
<ref id="B5">
<label>5</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Frawley]]></surname>
<given-names><![CDATA[G]]></given-names>
</name>
<name>
<surname><![CDATA[Fuenzalida]]></surname>
<given-names><![CDATA[D]]></given-names>
</name>
<name>
<surname><![CDATA[Donath]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
<name>
<surname><![CDATA[Yaplito Lee]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
<name>
<surname><![CDATA[Peters]]></surname>
<given-names><![CDATA[H]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[A retrospective audit of anesthetic techniques and complications in children with mucopolysaccharidoses]]></article-title>
<source><![CDATA[Paediatr Anaesth]]></source>
<year>2012</year>
<volume>22</volume>
<numero>8</numero>
<issue>8</issue>
<page-range>737-44</page-range></nlm-citation>
</ref>
<ref id="B6">
<label>6</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Lucio Medina]]></surname>
<given-names><![CDATA[IM]]></given-names>
</name>
<name>
<surname><![CDATA[Rincón Sánchez]]></surname>
<given-names><![CDATA[O]]></given-names>
</name>
<name>
<surname><![CDATA[Pizaña Vázquez]]></surname>
<given-names><![CDATA[SA]]></given-names>
</name>
<name>
<surname><![CDATA[Peña García]]></surname>
<given-names><![CDATA[I]]></given-names>
</name>
<name>
<surname><![CDATA[López García]]></surname>
<given-names><![CDATA[R]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Síndrome de Morquio en cesárea bajo anestesia general: reporte de un caso]]></article-title>
<source><![CDATA[Anest. Méx]]></source>
<year>2019</year>
<volume>31</volume>
<numero>1</numero>
<issue>1</issue>
<page-range>56-61</page-range></nlm-citation>
</ref>
<ref id="B7">
<label>7</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Theroux]]></surname>
<given-names><![CDATA[MC]]></given-names>
</name>
<name>
<surname><![CDATA[Nerker]]></surname>
<given-names><![CDATA[T]]></given-names>
</name>
<name>
<surname><![CDATA[Ditro]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
<name>
<surname><![CDATA[Mackenzie]]></surname>
<given-names><![CDATA[WG]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Anesthetic care and perioperative complications of children with Morquio syndrome]]></article-title>
<source><![CDATA[Padiatr Anaesth]]></source>
<year>2012</year>
<volume>22</volume>
<numero>9</numero>
<issue>9</issue>
<page-range>901-7</page-range></nlm-citation>
</ref>
<ref id="B8">
<label>8</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Berger]]></surname>
<given-names><![CDATA[KI]]></given-names>
</name>
<name>
<surname><![CDATA[Fagondes]]></surname>
<given-names><![CDATA[SC]]></given-names>
</name>
<name>
<surname><![CDATA[Giugliani]]></surname>
<given-names><![CDATA[R]]></given-names>
</name>
<name>
<surname><![CDATA[Hardy]]></surname>
<given-names><![CDATA[KA]]></given-names>
</name>
<name>
<surname><![CDATA[Lee]]></surname>
<given-names><![CDATA[KS]]></given-names>
</name>
<name>
<surname><![CDATA[McArdle]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Respiratory and sleep disorders in mucopolysaccharidosis]]></article-title>
<source><![CDATA[J Inherit Metab Dis]]></source>
<year>2013</year>
<volume>36</volume>
<numero>2</numero>
<issue>2</issue>
<page-range>201-10</page-range></nlm-citation>
</ref>
<ref id="B9">
<label>9</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Clark]]></surname>
<given-names><![CDATA[BM]]></given-names>
</name>
<name>
<surname><![CDATA[Sprung]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
<name>
<surname><![CDATA[Weingarten]]></surname>
<given-names><![CDATA[TN]]></given-names>
</name>
<name>
<surname><![CDATA[Warner]]></surname>
<given-names><![CDATA[ME]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Anesthesia for patients with mucopolysaccharidoses: Comprehensive review of the literature with emphasis on airway management]]></article-title>
<source><![CDATA[Bosn J Basic Med Sci]]></source>
<year>2018</year>
<volume>18</volume>
<numero>1</numero>
<issue>1</issue>
<page-range>1-7</page-range></nlm-citation>
</ref>
</ref-list>
</back>
</article>
