<?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>0864-0289</journal-id>
<journal-title><![CDATA[Revista Cubana de Hematología, Inmunología y Hemoterapia]]></journal-title>
<abbrev-journal-title><![CDATA[Rev Cubana Hematol Inmunol Hemoter]]></abbrev-journal-title>
<issn>0864-0289</issn>
<publisher>
<publisher-name><![CDATA[Centro Nacional de Información de Ciencias MédicasEditorial Ciencias Médicas]]></publisher-name>
</publisher>
</journal-meta>
<article-meta>
<article-id>S0864-02892019000200002</article-id>
<title-group>
<article-title xml:lang="es"><![CDATA[Síndrome de Béguez-Steinbrinck-Higashi: un nuevo epónimo para el síndrome de Chediak-Higashi]]></article-title>
<article-title xml:lang="en"><![CDATA[Béguez-Steinbrinck-Higashi syndrome: a new eponymous for the Chediak-Higashi syndrome]]></article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Castro Castro]]></surname>
<given-names><![CDATA[Bernardo]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Ballester Santovenia]]></surname>
<given-names><![CDATA[José Manuel]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Peña Sánchez]]></surname>
<given-names><![CDATA[María Antonia]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Batista Duharte]]></surname>
<given-names><![CDATA[Alexander]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Robinson Rodríguez]]></surname>
<given-names><![CDATA[Rosa Julia]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
</contrib-group>
<aff id="Af1">
<institution><![CDATA[,Centro de Inmunoensayo, Sucursal Santiago de Cuba  ]]></institution>
<addr-line><![CDATA[ ]]></addr-line>
<country>Cuba</country>
</aff>
<aff id="Af2">
<institution><![CDATA[,Instituto de Hematología e Inmunología  ]]></institution>
<addr-line><![CDATA[La Habana ]]></addr-line>
<country>Cuba</country>
</aff>
<aff id="Af3">
<institution><![CDATA[,Universidad de Ciencias Médicas de Santiago de Cuba  ]]></institution>
<addr-line><![CDATA[ ]]></addr-line>
<country>Cuba</country>
</aff>
<aff id="Af4">
<institution><![CDATA[,Universidad de Estadual Paulista  ]]></institution>
<addr-line><![CDATA[São Paulo ]]></addr-line>
<country>Brasil</country>
</aff>
<pub-date pub-type="pub">
<day>00</day>
<month>06</month>
<year>2019</year>
</pub-date>
<pub-date pub-type="epub">
<day>00</day>
<month>06</month>
<year>2019</year>
</pub-date>
<volume>35</volume>
<numero>2</numero>
<copyright-statement/>
<copyright-year/>
<self-uri xlink:href="http://scielo.sld.cu/scielo.php?script=sci_arttext&amp;pid=S0864-02892019000200002&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.sld.cu/scielo.php?script=sci_abstract&amp;pid=S0864-02892019000200002&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.sld.cu/scielo.php?script=sci_pdf&amp;pid=S0864-02892019000200002&amp;lng=en&amp;nrm=iso"></self-uri><abstract abstract-type="short" xml:lang="es"><p><![CDATA[RESUMEN En diciembre de 1943, el Dr. Antonio María Béguez César detalló en el Boletín de la Sociedad Cubana de Pediatría los aspectos clínicos y hematológicos de una rara afección que padecieron tres niños de una familia santiaguera, quienesfallecieron en sus primeros años de vida. No había informes sobre hallazgos similares en la bibliografía médica, por lo cual se consideró la primera descripción de unaenfermedaddenominada por él comoneutropenia crónica maligna familiar con granulaciones atípicas de los leucocitos, que aún hoy suele divulgarse erróneamente como síndrome de Chediak-Higashi y no como síndrome de Béguez-Steinbrinck-Higashi. Esta enfermedad es una inmunodeficiencia primaria causada por mutaciones en el gen regulador de la función lisosomal, capaz de alterar la formación del fagolisosoma en el neutrófilo y determinar la presencia de gránulos secretores gigantes en su interior, asociadas a un predominio deinfecciones recurrentes generadas por bacterias piógenas. Aquí se realiza un recuento histórico del descubrimiento de esta entidad y se actualiza su fisiopatología.]]></p></abstract>
<abstract abstract-type="short" xml:lang="en"><p><![CDATA[ABSTRACT On December 1943, Dr. Antonio María Béguez César detailed in the Journal of the Cuban Pediatric Society the clinical and hematologic aspects of a rare disorder suffered by three children from a family in the locality, who expired during the first years of their lives in Santiago de Cuba. At that moment there was no report about similar findings in the medical literature, therefore it is considered the first description of a disease denominated by him as familial malignant chronic neutropenia with atypical granulations of leucocytes, misleadingly revealed as Chediak-Higashi syndrome instead of Béguez-Steinbrinck-Higashi syndrome. This disease consists of a primary immunodeficiency induced by mutations in the regulator gen of the lysosomal function, which is able to alter the formation of phagolysosoma in the neutrophil and determine the presence of giant secretor granules associated with the predominance of recurrent infections provoked by pyogen bacteria. Here, a brief history of it&#8217;s discovery as well as an updating of it&#8217;s physiopathology are carried out.]]></p></abstract>
<kwd-group>
<kwd lng="es"><![CDATA[Antonio María Béguez César]]></kwd>
<kwd lng="es"><![CDATA[neutropenia crónica]]></kwd>
<kwd lng="es"><![CDATA[granulaciones atípicas]]></kwd>
<kwd lng="es"><![CDATA[leucocitos]]></kwd>
<kwd lng="es"><![CDATA[neutrófilo]]></kwd>
<kwd lng="es"><![CDATA[bacteria piógena]]></kwd>
<kwd lng="es"><![CDATA[inmunodeficiencia primaria]]></kwd>
<kwd lng="es"><![CDATA[Cuba]]></kwd>
<kwd lng="en"><![CDATA[Antonio María Béguez César]]></kwd>
<kwd lng="en"><![CDATA[chronic neutropenia]]></kwd>
<kwd lng="en"><![CDATA[atypical granulations]]></kwd>
<kwd lng="en"><![CDATA[leucocytes]]></kwd>
<kwd lng="en"><![CDATA[neutrophil]]></kwd>
<kwd lng="en"><![CDATA[pyogen bacteria]]></kwd>
<kwd lng="en"><![CDATA[primary immunodeficiency]]></kwd>
<kwd lng="en"><![CDATA[Cuba]]></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[Béguez César]]></surname>
<given-names><![CDATA[AM]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Neutropenia crónica maligna familiar con granulaciones atípicas de los leucocitos]]></article-title>
<source><![CDATA[Bol Soc Cubana Pediatr]]></source>
<year>1943</year>
<volume>15</volume>
<numero>12</numero>
<issue>12</issue>
<page-range>900-22</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[Retureta]]></surname>
<given-names><![CDATA[AA]]></given-names>
</name>
<name>
<surname><![CDATA[Retureta]]></surname>
<given-names><![CDATA[GC]]></given-names>
</name>
<name>
<surname><![CDATA[Retureta]]></surname>
<given-names><![CDATA[JM]]></given-names>
</name>
<name>
<surname><![CDATA[Papazian]]></surname>
<given-names><![CDATA[O]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Beguez-Steinbrinck-Chédiak-Higashi Disease]]></article-title>
<source><![CDATA[International Pediatrics]]></source>
<year>1996</year>
<volume>11</volume>
<numero>2</numero>
<issue>2</issue>
<page-range>133-5</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[Peña Sánchez]]></surname>
<given-names><![CDATA[MA]]></given-names>
</name>
<name>
<surname><![CDATA[Miyares Peña]]></surname>
<given-names><![CDATA[MV]]></given-names>
</name>
<name>
<surname><![CDATA[Batista Duharte]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Paúltre Rivas]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
<name>
<surname><![CDATA[Béguez López]]></surname>
<given-names><![CDATA[V]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Antonio Béguez César y su descubrimiento de la neutropenia crónica maligna familiar con granulaciones atípicas de los leucocitos]]></article-title>
<source><![CDATA[MEDISAN]]></source>
<year>2007</year>
<volume>11</volume>
<numero>3</numero>
<issue>3</issue>
</nlm-citation>
</ref>
<ref id="B4">
<label>4</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Steinbrinck]]></surname>
<given-names><![CDATA[W]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Über eine neue Granulatons anomalie der Leukocyten]]></article-title>
<source><![CDATA[Dtsch Arch klin Med (Leipzig)]]></source>
<year>1948</year>
<volume>193</volume>
<page-range>577-81</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[Higashi]]></surname>
<given-names><![CDATA[O]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Congenital gigantism of peroxidase granules: the first case ever reported of qualitative abnormity of peroxidase]]></article-title>
<source><![CDATA[Tohoku J Exp Med]]></source>
<year>1954</year>
<volume>59</volume>
<numero>3</numero>
<issue>3</issue>
<page-range>315-32</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[Chédiak]]></surname>
<given-names><![CDATA[MM]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Nouvelle anomalie leucocytaire de caractere constitutionnel et familial]]></article-title>
<source><![CDATA[Rev Hematol]]></source>
<year>1952</year>
<volume>7</volume>
<page-range>362-7</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[Sato]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Chédiak and Higashi's disease: probable identity of a new leucocytal anomaly (Chédiak) and congenital gigantism of peroxidase granules (Higashi)]]></article-title>
<source><![CDATA[Tohoku J Exp Med]]></source>
<year>1955</year>
<volume>61</volume>
<numero>2-3</numero>
<issue>2-3</issue>
<page-range>201-10</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[Sánchez Avalos]]></surname>
<given-names><![CDATA[JC]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Historia del Grupo Cooperativo Latinoamericano de Hemostasia y Trombosis]]></article-title>
<source><![CDATA[Acta Bioquím Clín Latinoam]]></source>
<year>2006</year>
<volume>40</volume>
<numero>3</numero>
<issue>3</issue>
<page-range>295-8</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[Peña Sánchez]]></surname>
<given-names><![CDATA[MA]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[A la memoria del Dr. Antonio María Béguez César (1895-1975): descubridor de la neutropenia crónica maligna familiar con granulaciones atípicas de los leucocitos]]></article-title>
<source><![CDATA[MEDISAN]]></source>
<year>2016</year>
<volume>20</volume>
<numero>1</numero>
<issue>1</issue>
<page-range>109-17</page-range></nlm-citation>
</ref>
<ref id="B10">
<label>10</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Endi Felfli]]></surname>
<given-names><![CDATA[FJ]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Elegía al Dr. Antonio María Béguez César]]></article-title>
<source><![CDATA[MEDISAN]]></source>
<year>2011</year>
<volume>15</volume>
<numero>5</numero>
<issue>5</issue>
<page-range>715-7</page-range></nlm-citation>
</ref>
<ref id="B11">
<label>11</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Peña Sánchez]]></surname>
<given-names><![CDATA[M A]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Dr. Antonio María Béguez César (1895-1975): del plagio a la verdad histórica]]></article-title>
<source><![CDATA[MEDISAN]]></source>
<year>2014</year>
<volume>18</volume>
<numero>5</numero>
<issue>5</issue>
<page-range>733-47</page-range></nlm-citation>
</ref>
<ref id="B12">
<label>12</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Gómez-Lobo]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Las intenciones de Heródoto]]></article-title>
<source><![CDATA[Estudios Públicos]]></source>
<year>1995</year>
<volume>59</volume>
<page-range>1-15</page-range></nlm-citation>
</ref>
<ref id="B13">
<label>13</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Pereira]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[La peripecia de Béguez César]]></article-title>
<source><![CDATA[Cuba Internacional]]></source>
<year>1973</year>
<volume>5</volume>
<numero>44</numero>
<issue>44</issue>
<page-range>14-9</page-range></nlm-citation>
</ref>
<ref id="B14">
<label>14</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Singh]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Bryan]]></surname>
<given-names><![CDATA[MM]]></given-names>
</name>
<name>
<surname><![CDATA[Roney]]></surname>
<given-names><![CDATA[JC]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[A clinical report of Chédiak-Higashi syndrome in infancy with a novel genotype from the Indian subcontinent]]></article-title>
<source><![CDATA[Internat J Dermatol]]></source>
<year>2016</year>
<volume>55</volume>
<numero>3</numero>
<issue>3</issue>
<page-range>317-21</page-range></nlm-citation>
</ref>
<ref id="B15">
<label>15</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Lozano]]></surname>
<given-names><![CDATA[ML]]></given-names>
</name>
<name>
<surname><![CDATA[Rivera]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
<name>
<surname><![CDATA[Sánchez-Guiu]]></surname>
<given-names><![CDATA[I]]></given-names>
</name>
<name>
<surname><![CDATA[Vicente]]></surname>
<given-names><![CDATA[V]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Towards the targeted management of Chédiak-Higashi syndrome]]></article-title>
<source><![CDATA[Orphanet J Rare Dis]]></source>
<year>2014</year>
<volume>9</volume>
<page-range>132</page-range></nlm-citation>
</ref>
<ref id="B16">
<label>16</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Ortuño]]></surname>
<given-names><![CDATA[FJ]]></given-names>
</name>
<name>
<surname><![CDATA[Fuster]]></surname>
<given-names><![CDATA[JL]]></given-names>
</name>
<name>
<surname><![CDATA[Jeréz]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Síndrome de Chédiak-Higashi]]></article-title>
<source><![CDATA[Med Clin (Barc)]]></source>
<year>2010</year>
<volume>135</volume>
<numero>11</numero>
<issue>11</issue>
<page-range>512-8</page-range></nlm-citation>
</ref>
<ref id="B17">
<label>17</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Introne]]></surname>
<given-names><![CDATA[WJ]]></given-names>
</name>
<name>
<surname><![CDATA[Westbroek]]></surname>
<given-names><![CDATA[W]]></given-names>
</name>
<name>
<surname><![CDATA[Cullinane]]></surname>
<given-names><![CDATA[AR]]></given-names>
</name>
<name>
<surname><![CDATA[Groden]]></surname>
<given-names><![CDATA[CA]]></given-names>
</name>
<name>
<surname><![CDATA[Bhambhani]]></surname>
<given-names><![CDATA[V]]></given-names>
</name>
<name>
<surname><![CDATA[Golas]]></surname>
<given-names><![CDATA[GA]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Neurologic involvement in patients with atypical Chédiak-Higashi disease]]></article-title>
<source><![CDATA[Neurology]]></source>
<year>2016</year>
<volume>86</volume>
<numero>14</numero>
<issue>14</issue>
<page-range>1320- 8</page-range></nlm-citation>
</ref>
<ref id="B18">
<label>18</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Rudramurthy]]></surname>
<given-names><![CDATA[P]]></given-names>
</name>
<name>
<surname><![CDATA[Lokanatha]]></surname>
<given-names><![CDATA[H]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Chédiak-higashi syndrome: A case series from Karnataka]]></article-title>
<source><![CDATA[Indian J Dermatol]]></source>
<year>2015</year>
<volume>60</volume>
<numero>5</numero>
<issue>5</issue>
<page-range>524</page-range></nlm-citation>
</ref>
<ref id="B19">
<label>19</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Jaiswal]]></surname>
<given-names><![CDATA[P]]></given-names>
</name>
<name>
<surname><![CDATA[Yadav]]></surname>
<given-names><![CDATA[YK]]></given-names>
</name>
<name>
<surname><![CDATA[Bhasker]]></surname>
<given-names><![CDATA[N]]></given-names>
</name>
<name>
<surname><![CDATA[Kushwaha]]></surname>
<given-names><![CDATA[R]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Accelerated phase of Chédiak-Higashi syndrome at initial presentation: a case report of an uncommon occurrence in a rare disorder]]></article-title>
<source><![CDATA[J Clin Diagn Res]]></source>
<year>2015</year>
<volume>9</volume>
<numero>12</numero>
<issue>12</issue>
<page-range>ED13-4</page-range></nlm-citation>
</ref>
<ref id="B20">
<label>20</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Singh]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Bryan]]></surname>
<given-names><![CDATA[MM]]></given-names>
</name>
<name>
<surname><![CDATA[Roney]]></surname>
<given-names><![CDATA[JC]]></given-names>
</name>
<name>
<surname><![CDATA[Cullinane]]></surname>
<given-names><![CDATA[AR]]></given-names>
</name>
<name>
<surname><![CDATA[Gahl]]></surname>
<given-names><![CDATA[WA]]></given-names>
</name>
<name>
<surname><![CDATA[Khurana]]></surname>
<given-names><![CDATA[N]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Medical Genetics. A clinical report of Chédiak-Higashi syndrome in infancy with a novel genotype from the Indian subcontinent]]></article-title>
<source><![CDATA[Int J Dermatol]]></source>
<year>2016</year>
<volume>55</volume>
<numero>3</numero>
<issue>3</issue>
<page-range>317-21</page-range></nlm-citation>
</ref>
<ref id="B21">
<label>21</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Shravani]]></surname>
<given-names><![CDATA[MR]]></given-names>
</name>
<name>
<surname><![CDATA[Murali]]></surname>
<given-names><![CDATA[BH]]></given-names>
</name>
<name>
<surname><![CDATA[Chandrakala]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Chédiak Higashi syndrome presenting in accelerated phase a case report and literature review]]></article-title>
<source><![CDATA[Int J Contemp Pediatr]]></source>
<year>2017</year>
<volume>4</volume>
<numero>4</numero>
<issue>4</issue>
<page-range>1537-40</page-range></nlm-citation>
</ref>
<ref id="B22">
<label>22</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Maaloul]]></surname>
<given-names><![CDATA[I]]></given-names>
</name>
<name>
<surname><![CDATA[Talmoudi]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
<name>
<surname><![CDATA[Chabchoub]]></surname>
<given-names><![CDATA[I]]></given-names>
</name>
<name>
<surname><![CDATA[Ayadi]]></surname>
<given-names><![CDATA[L]]></given-names>
</name>
<name>
<surname><![CDATA[Kamoun]]></surname>
<given-names><![CDATA[TH]]></given-names>
</name>
<name>
<surname><![CDATA[Boudawara]]></surname>
<given-names><![CDATA[T]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Chédiak-Higashi syndrome presenting in accelerated phase: A case report andliterature review]]></article-title>
<source><![CDATA[Hematol Oncol Stem Cell Ther]]></source>
<year>2016</year>
<volume>9</volume>
<numero>2</numero>
<issue>2</issue>
<page-range>71-5</page-range></nlm-citation>
</ref>
<ref id="B23">
<label>23</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Ho]]></surname>
<given-names><![CDATA[MC]]></given-names>
</name>
<name>
<surname><![CDATA[Hsieh]]></surname>
<given-names><![CDATA[YT]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Mixed hyperpigmentation and hypopigmentation of iris and choroid in Chédiak-Higashi syndrome]]></article-title>
<source><![CDATA[J AAPOS]]></source>
<year>2013</year>
<volume>17</volume>
<numero>5</numero>
<issue>5</issue>
<page-range>558-60</page-range></nlm-citation>
</ref>
<ref id="B24">
<label>24</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Al-Tamemi]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
<name>
<surname><![CDATA[Al-Zadjali]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
<name>
<surname><![CDATA[Al-Ghafri]]></surname>
<given-names><![CDATA[F]]></given-names>
</name>
<name>
<surname><![CDATA[Dennison]]></surname>
<given-names><![CDATA[D]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Chédiak-Higashi Syndrome: novel mutation of theCHS1/LYST gene in 3 Omani patients]]></article-title>
<source><![CDATA[J Pediatr Hematol Oncol]]></source>
<year>2014</year>
<volume>36</volume>
<numero>4</numero>
<issue>4</issue>
<page-range>e248-50</page-range></nlm-citation>
</ref>
<ref id="B25">
<label>25</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Morrone]]></surname>
<given-names><![CDATA[K]]></given-names>
</name>
<name>
<surname><![CDATA[Wang]]></surname>
<given-names><![CDATA[Y]]></given-names>
</name>
<name>
<surname><![CDATA[Huizing]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Sutton]]></surname>
<given-names><![CDATA[E]]></given-names>
</name>
<name>
<surname><![CDATA[White]]></surname>
<given-names><![CDATA[JG]]></given-names>
</name>
<name>
<surname><![CDATA[Gahl]]></surname>
<given-names><![CDATA[WA]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Two Novel Mutations Identified in an African-American Child with Chédiak-Higashi Syndrome]]></article-title>
<source><![CDATA[Case Rep Med]]></source>
<year>2010</year>
<page-range>4</page-range></nlm-citation>
</ref>
<ref id="B26">
<label>26</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Leliefeld]]></surname>
<given-names><![CDATA[PHC]]></given-names>
</name>
<name>
<surname><![CDATA[Wessels]]></surname>
<given-names><![CDATA[CM]]></given-names>
</name>
<name>
<surname><![CDATA[Leenen]]></surname>
<given-names><![CDATA[LPH]]></given-names>
</name>
<name>
<surname><![CDATA[Koenderman]]></surname>
<given-names><![CDATA[L]]></given-names>
</name>
<name>
<surname><![CDATA[Pilay]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[The role of neutrophils in immune dysfunction during severe inflammation]]></article-title>
<source><![CDATA[Critical care]]></source>
<year>2016</year>
<volume>20</volume>
<numero>73</numero>
<issue>73</issue>
</nlm-citation>
</ref>
<ref id="B27">
<label>27</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Palaniyandi]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
<name>
<surname><![CDATA[Pasupathy]]></surname>
<given-names><![CDATA[U]]></given-names>
</name>
<name>
<surname><![CDATA[Ravichandran]]></surname>
<given-names><![CDATA[L]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Chédiak-Higashi syndrome presenting in the accelerated phase]]></article-title>
<source><![CDATA[South African Journal]]></source>
<year>2017</year>
<volume>11</volume>
<numero>2</numero>
<issue>2</issue>
</nlm-citation>
</ref>
<ref id="B28">
<label>28</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Kishore]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Marwah]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
<name>
<surname><![CDATA[Kumar]]></surname>
<given-names><![CDATA[V]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Accelerated phase of Chédiak Higashi Syndrome: An Unusual case of pancytopenia]]></article-title>
<source><![CDATA[An Pathol Lab Med]]></source>
<year>2016</year>
<volume>3</volume>
<numero>3</numero>
<issue>3</issue>
</nlm-citation>
</ref>
<ref id="B29">
<label>29</label><nlm-citation citation-type="book">
<person-group person-group-type="author">
<name>
<surname><![CDATA[JustizVaillant]]></surname>
<given-names><![CDATA[AA]]></given-names>
</name>
<name>
<surname><![CDATA[Zito]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Neutropenia]]></article-title>
<source><![CDATA[Stat Pearls]]></source>
<year>2018</year>
<page-range>s-Disp</page-range><publisher-loc><![CDATA[Treasure Island(FL) ]]></publisher-loc>
<publisher-name><![CDATA[Stat Pearls Publishing]]></publisher-name>
</nlm-citation>
</ref>
<ref id="B30">
<label>30</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Sônego]]></surname>
<given-names><![CDATA[F]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Paradoxical roles of the neutrophil in sepsis: protective and deleterious]]></article-title>
<source><![CDATA[Front Immunol]]></source>
<year>2016</year>
<volume>7</volume>
<page-range>155</page-range></nlm-citation>
</ref>
<ref id="B31">
<label>31</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Söderberg]]></surname>
<given-names><![CDATA[D]]></given-names>
</name>
<name>
<surname><![CDATA[Segelmark]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Neutrophil Extracelular Traps in ANCA-Associated Vasculitis]]></article-title>
<source><![CDATA[Front Immunol]]></source>
<year>2016</year>
<volume>7</volume>
<page-range>256</page-range></nlm-citation>
</ref>
<ref id="B32">
<label>32</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Dabrowska]]></surname>
<given-names><![CDATA[D]]></given-names>
</name>
<name>
<surname><![CDATA[Jablonska]]></surname>
<given-names><![CDATA[E]]></given-names>
</name>
<name>
<surname><![CDATA[Garley]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Ratajczak-wrona]]></surname>
<given-names><![CDATA[W]]></given-names>
</name>
<name>
<surname><![CDATA[Iwaniuk]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[New aspects of the biology of Neutrophil Extracellular Traps]]></article-title>
<source><![CDATA[Scand J Immunol]]></source>
<year>2016</year>
<volume>84</volume>
<page-range>317-22</page-range></nlm-citation>
</ref>
<ref id="B33">
<label>33</label><nlm-citation citation-type="book">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Holland]]></surname>
<given-names><![CDATA[SM]]></given-names>
</name>
<name>
<surname><![CDATA[Bellanti]]></surname>
<given-names><![CDATA[JA]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Immune Deficiency Disorders]]></article-title>
<person-group person-group-type="editor">
<name>
<surname><![CDATA[Joseph]]></surname>
<given-names><![CDATA[ABellanti]]></given-names>
</name>
</person-group>
<source><![CDATA[Immunology IV Clinical implications in Health and disease]]></source>
<year>2012</year>
<page-range>559-637</page-range><publisher-loc><![CDATA[Maryland ]]></publisher-loc>
<publisher-name><![CDATA[I Care Press Bethesda]]></publisher-name>
</nlm-citation>
</ref>
<ref id="B34">
<label>34</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Gil-Krzewska]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Wood]]></surname>
<given-names><![CDATA[SM]]></given-names>
</name>
<name>
<surname><![CDATA[Murakami]]></surname>
<given-names><![CDATA[Y]]></given-names>
</name>
<name>
<surname><![CDATA[Nguyen]]></surname>
<given-names><![CDATA[V]]></given-names>
</name>
<name>
<surname><![CDATA[Chiang]]></surname>
<given-names><![CDATA[SC]]></given-names>
</name>
<name>
<surname><![CDATA[Cullinane]]></surname>
<given-names><![CDATA[AR]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Chédiak-Higashi syndrome: Lysosomal trafficking regulator domains regulate exocytosis of lytic granules but not cytokine secretion by natural killer cells]]></article-title>
<source><![CDATA[J Allergy Clin Immunol]]></source>
<year>2016</year>
<volume>137</volume>
<numero>4</numero>
<issue>4</issue>
<page-range>1165-77</page-range></nlm-citation>
</ref>
<ref id="B35">
<label>35</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Jin]]></surname>
<given-names><![CDATA[Y]]></given-names>
</name>
<name>
<surname><![CDATA[Zhang]]></surname>
<given-names><![CDATA[L]]></given-names>
</name>
<name>
<surname><![CDATA[Wang]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
<name>
<surname><![CDATA[Chen]]></surname>
<given-names><![CDATA[F]]></given-names>
</name>
<name>
<surname><![CDATA[Gu]]></surname>
<given-names><![CDATA[Y]]></given-names>
</name>
<name>
<surname><![CDATA[Hong]]></surname>
<given-names><![CDATA[E]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Novel Compound Heterozygous Lysosomal Trafficking Regulator Gene Mutations Associated with Autosomal Recessive Chédiak-Higashi Syndrome]]></article-title>
<source><![CDATA[Sci Rep]]></source>
<year>2017</year>
<volume>7</volume>
<numero>41308</numero>
<issue>41308</issue>
</nlm-citation>
</ref>
<ref id="B36">
<label>36</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Sánchez-Guiu]]></surname>
<given-names><![CDATA[I]]></given-names>
</name>
<name>
<surname><![CDATA[Anton]]></surname>
<given-names><![CDATA[AI]]></given-names>
</name>
<name>
<surname><![CDATA[García-Barbera]]></surname>
<given-names><![CDATA[N]]></given-names>
</name>
<name>
<surname><![CDATA[Navarro-Fernández]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
<name>
<surname><![CDATA[Martínez]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
<name>
<surname><![CDATA[Fuster]]></surname>
<given-names><![CDATA[JL]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Chédiak-Higashi syndrome: description of two novel homozygous missense mutations causing divergent clinical phenotype]]></article-title>
<source><![CDATA[Eur J Haematol]]></source>
<year>2013</year>
<volume>92</volume>
<page-range>49-58</page-range></nlm-citation>
</ref>
<ref id="B37">
<label>37</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Wu]]></surname>
<given-names><![CDATA[XL]]></given-names>
</name>
<name>
<surname><![CDATA[Zhao]]></surname>
<given-names><![CDATA[XQ]]></given-names>
</name>
<name>
<surname><![CDATA[Zhang]]></surname>
<given-names><![CDATA[BX]]></given-names>
</name>
<name>
<surname><![CDATA[Xuan]]></surname>
<given-names><![CDATA[F]]></given-names>
</name>
<name>
<surname><![CDATA[Goo]]></surname>
<given-names><![CDATA[HM]]></given-names>
</name>
<name>
<surname><![CDATA[Ma]]></surname>
<given-names><![CDATA[FT]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[A Novel frameshift of Chediak-Higashi syndrome and treatment in the accelerated phase]]></article-title>
<source><![CDATA[Braz Biol Res]]></source>
<year>2017</year>
<volume>50</volume>
<numero>4</numero>
<issue>4</issue>
</nlm-citation>
</ref>
<ref id="B38">
<label>38</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Lehky]]></surname>
<given-names><![CDATA[TJ]]></given-names>
</name>
<name>
<surname><![CDATA[Groden]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
<name>
<surname><![CDATA[Lear]]></surname>
<given-names><![CDATA[B]]></given-names>
</name>
<name>
<surname><![CDATA[Toro]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
<name>
<surname><![CDATA[Introne]]></surname>
<given-names><![CDATA[WJ]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Peripheral nervous system (PNS) manifestations of Chediak-Higashi Disease (CHD)]]></article-title>
<source><![CDATA[Muscle Nerve]]></source>
<year>2017</year>
<volume>55</volume>
<numero>3</numero>
<issue>3</issue>
<page-range>359-65</page-range></nlm-citation>
</ref>
<ref id="B39">
<label>39</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Machín García]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
<name>
<surname><![CDATA[Svarch]]></surname>
<given-names><![CDATA[E]]></given-names>
</name>
<name>
<surname><![CDATA[González Otero]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Menéndez Veitía]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Hernández Cabezas]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Serrano Mirabal]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Síndrome Beguez-Chédiak-Higashi. Comunicación de un nuevo caso en Cuba]]></article-title>
<source><![CDATA[Rev Cubana Hematol Inmunol Hemoter]]></source>
<year>2011</year>
<volume>27</volume>
<numero>4</numero>
<issue>4</issue>
<page-range>435-44</page-range></nlm-citation>
</ref>
</ref-list>
</back>
</article>
