<?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>1812-9528</journal-id>
<journal-title><![CDATA[Memorias del Instituto de Investigaciones en Ciencias de la Salud]]></journal-title>
<abbrev-journal-title><![CDATA[Mem. Inst. Investig. Cienc. Salud]]></abbrev-journal-title>
<issn>1812-9528</issn>
<publisher>
<publisher-name><![CDATA[Instituto de Investigaciones en Ciencias de la Salud]]></publisher-name>
</publisher>
</journal-meta>
<article-meta>
<article-id>S1812-95282022000300142</article-id>
<article-id pub-id-type="doi">10.18004/mem.iics/1812-9528/2022.020.03.142</article-id>
<title-group>
<article-title xml:lang="es"><![CDATA[Fibrosis pulmonar familiar en el síndrome de Hermansky-Pudlak]]></article-title>
<article-title xml:lang="en"><![CDATA[Family pulmonary fibrosis in Hermansky-Pudlak syndrome]]></article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Pérez-Bejarano]]></surname>
<given-names><![CDATA[Domingo]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
</contrib-group>
<aff id="Af1">
<institution><![CDATA[,Hospital General de Luque Servicio de Neumología Área Consultorio Externo]]></institution>
<addr-line><![CDATA[Luque ]]></addr-line>
<country>Paraguay</country>
</aff>
<pub-date pub-type="pub">
<day>00</day>
<month>12</month>
<year>2022</year>
</pub-date>
<pub-date pub-type="epub">
<day>00</day>
<month>12</month>
<year>2022</year>
</pub-date>
<volume>20</volume>
<numero>3</numero>
<fpage>142</fpage>
<lpage>147</lpage>
<copyright-statement/>
<copyright-year/>
<self-uri xlink:href="http://scielo.iics.una.py/scielo.php?script=sci_arttext&amp;pid=S1812-95282022000300142&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.iics.una.py/scielo.php?script=sci_abstract&amp;pid=S1812-95282022000300142&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.iics.una.py/scielo.php?script=sci_pdf&amp;pid=S1812-95282022000300142&amp;lng=en&amp;nrm=iso"></self-uri><abstract abstract-type="short" xml:lang="es"><p><![CDATA[RESUMEN La fibrosis pulmonar familiar en el contexto del síndrome de Hermansky-Pudlak es una entidad nosológica poco frecuente y cuyo único enfoque terapéutico definitivo es el trasplante pulmonar en los estadios avanzados. Describimos algunos aspectos clínicos diagnósticos y terapéuticos que configuran el manejo de las enfermedades huérfanas en países como el nuestro.]]></p></abstract>
<abstract abstract-type="short" xml:lang="en"><p><![CDATA[ABSTRACT Family pulmonary fibrosis in the context of Hermansky-Pudlak syndrome is a rare nosological entity whose only definitive therapeutic approach is lung transplantation in the advanced stages. We describe some diagnostic and therapeutic clinical aspects that shape the management of orphan diseases in countries like ours.]]></p></abstract>
<kwd-group>
<kwd lng="es"><![CDATA[Fibrosis pulmonar familiar]]></kwd>
<kwd lng="es"><![CDATA[síndrome de Hermansky-Pudlak]]></kwd>
<kwd lng="es"><![CDATA[neumonía intersticial idiopática]]></kwd>
<kwd lng="es"><![CDATA[albinismo oculo-cutáneo]]></kwd>
<kwd lng="en"><![CDATA[Family pulmonary fibrosis]]></kwd>
<kwd lng="en"><![CDATA[Hermansky-Pudlak syndrome]]></kwd>
<kwd lng="en"><![CDATA[interstitial idiopathic pneumonia]]></kwd>
<kwd lng="en"><![CDATA[oculocutaneous albinism]]></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[Hortense]]></surname>
<given-names><![CDATA[AB]]></given-names>
</name>
<name>
<surname><![CDATA[Santos]]></surname>
<given-names><![CDATA[MK]]></given-names>
</name>
<name>
<surname><![CDATA[Wada]]></surname>
<given-names><![CDATA[D]]></given-names>
</name>
<name>
<surname><![CDATA[Fabro]]></surname>
<given-names><![CDATA[AT]]></given-names>
</name>
<name>
<surname><![CDATA[Lima]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Rodrigues]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Familial pulmonary fibrosis: a heterogeneous spectrum of presentations]]></article-title>
<source><![CDATA[J Bras Pneumol.]]></source>
<year>2019</year>
<volume>45</volume>
<numero>5</numero>
<issue>5</issue>
</nlm-citation>
</ref>
<ref id="B2">
<label>2</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Aburto]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Aguirre]]></surname>
<given-names><![CDATA[U]]></given-names>
</name>
<name>
<surname><![CDATA[Iciar-Arrizubieta]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Pérez Izquierdo]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
<name>
<surname><![CDATA[Bronte]]></surname>
<given-names><![CDATA[O]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Checking Siblings of Patients with Idiopathic Pulmonary Fibrosis as a Scheme for Early Disease Detection]]></article-title>
<source><![CDATA[Ann Am Thorac Soc]]></source>
<year>2021</year>
<volume>18</volume>
<numero>1</numero>
<issue>1</issue>
<page-range>172-4</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[García-Sancho]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
<name>
<surname><![CDATA[Buendía-Roldán]]></surname>
<given-names><![CDATA[I]]></given-names>
</name>
<name>
<surname><![CDATA[Fernández-Plata]]></surname>
<given-names><![CDATA[MR]]></given-names>
</name>
<name>
<surname><![CDATA[Navarro]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
<name>
<surname><![CDATA[Pérez-Padilla]]></surname>
<given-names><![CDATA[R]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Familial pulmonary fibrosis is the strongest risk factor for idiopathic pulmonary fibrosis]]></article-title>
<source><![CDATA[Respir Med.]]></source>
<year>2011</year>
<volume>105</volume>
<numero>12</numero>
<issue>12</issue>
<page-range>1902-7</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[van Batenburg]]></surname>
<given-names><![CDATA[AA]]></given-names>
</name>
<name>
<surname><![CDATA[van Oosterhout]]></surname>
<given-names><![CDATA[MFM]]></given-names>
</name>
<name>
<surname><![CDATA[Knoppert]]></surname>
<given-names><![CDATA[SN]]></given-names>
</name>
<name>
<surname><![CDATA[Kazemier]]></surname>
<given-names><![CDATA[K]]></given-names>
</name>
<name>
<surname><![CDATA[Van der Vis]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
<name>
<surname><![CDATA[Grtter]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[The Extent of Inflammatory Cell Infiltrate and Fibrosis in Lungs of Telomere- and Surfacta nt-Related Familial Pulmonary Fibrosis]]></article-title>
<source><![CDATA[Front. Med.]]></source>
<year>2021</year>
<volume>8</volume>
<page-range>736485</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[Steele]]></surname>
<given-names><![CDATA[MP]]></given-names>
</name>
<name>
<surname><![CDATA[Speer]]></surname>
<given-names><![CDATA[MC]]></given-names>
</name>
<name>
<surname><![CDATA[Loyd]]></surname>
<given-names><![CDATA[JE]]></given-names>
</name>
<name>
<surname><![CDATA[Brown]]></surname>
<given-names><![CDATA[K]]></given-names>
</name>
<name>
<surname><![CDATA[Herron]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Slifer]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Clinical and pathologic features of familial interstitial pneumonia]]></article-title>
<source><![CDATA[Am J Respir Crit Care Med.]]></source>
<year>2005</year>
<volume>172</volume>
<numero>9</numero>
<issue>9</issue>
<page-range>1146-52</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[Vicary]]></surname>
<given-names><![CDATA[GW]]></given-names>
</name>
<name>
<surname><![CDATA[Vergne]]></surname>
<given-names><![CDATA[Y]]></given-names>
</name>
<name>
<surname><![CDATA[Santiago-Cornier]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Young]]></surname>
<given-names><![CDATA[L]]></given-names>
</name>
<name>
<surname><![CDATA[Roman]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Pulmonary Fibrosis in Hermansky-Pudlak Syndrome]]></article-title>
<source><![CDATA[Ann Am Thorac Soc]]></source>
<year>2016</year>
<volume>13</volume>
<numero>10</numero>
<issue>10</issue>
<page-range>1839-46</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[Velázquez-Díaz]]></surname>
<given-names><![CDATA[P]]></given-names>
</name>
<name>
<surname><![CDATA[Nakajima]]></surname>
<given-names><![CDATA[E]]></given-names>
</name>
<name>
<surname><![CDATA[Sorkhdini]]></surname>
<given-names><![CDATA[P]]></given-names>
</name>
<name>
<surname><![CDATA[Hernández]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Eberle]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Yang]]></surname>
<given-names><![CDATA[D]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Hermansky-Pudlak Syndrome and Lung Disease: Pathogenesisand Therapeutics]]></article-title>
<source><![CDATA[Front. Pharmacol.]]></source>
<year>2021</year>
<volume>12</volume>
<page-range>644671</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[Berber]]></surname>
<given-names><![CDATA[I]]></given-names>
</name>
<name>
<surname><![CDATA[Erkurt]]></surname>
<given-names><![CDATA[MA]]></given-names>
</name>
<name>
<surname><![CDATA[Kuku]]></surname>
<given-names><![CDATA[I]]></given-names>
</name>
<name>
<surname><![CDATA[Kaya]]></surname>
<given-names><![CDATA[E]]></given-names>
</name>
<name>
<surname><![CDATA[Koroglu]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Nizam]]></surname>
<given-names><![CDATA[I]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Hermansky-pudlak syndrome: a case report]]></article-title>
<source><![CDATA[Case Rep Hematol.]]></source>
<year>2014</year>
<page-range>249195</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[Dell&#8217;Angelica]]></surname>
<given-names><![CDATA[EC]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[The building BLOC(k)s of lysosomes and related organelles]]></article-title>
<source><![CDATA[Curr.Opin. Cell Biol.]]></source>
<year>2004</year>
<volume>16</volume>
<page-range>458-64</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[Huizing]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Anikster]]></surname>
<given-names><![CDATA[Y]]></given-names>
</name>
<name>
<surname><![CDATA[Gahl]]></surname>
<given-names><![CDATA[WA]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Hermansky-Pudlak syndrome and related disorders of organelle formation]]></article-title>
<source><![CDATA[Traffic]]></source>
<year>2004</year>
<volume>1</volume>
<page-range>823-35</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[Yokoyama]]></surname>
<given-names><![CDATA[T]]></given-names>
</name>
<name>
<surname><![CDATA[Gochuico]]></surname>
<given-names><![CDATA[BR]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Hermansky-Pudlak syndrome pulmonary fibrosis: a rare inherited interstitial lung disease]]></article-title>
<source><![CDATA[Eur Respir Rev]]></source>
<year>2021</year>
<volume>30</volume>
<page-range>200193</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[Krauss]]></surname>
<given-names><![CDATA[E]]></given-names>
</name>
<name>
<surname><![CDATA[Gehrken]]></surname>
<given-names><![CDATA[G]]></given-names>
</name>
<name>
<surname><![CDATA[Drakopanagiotakis]]></surname>
<given-names><![CDATA[F]]></given-names>
</name>
<name>
<surname><![CDATA[Tello]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
<name>
<surname><![CDATA[Dartsch]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
<name>
<surname><![CDATA[Mauer]]></surname>
<given-names><![CDATA[O]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Clinical characteristics of patients with familial idiopathic pulmonary fibrosis (f-IPF)]]></article-title>
<source><![CDATA[BMC Pulm Med]]></source>
<year>2019</year>
<volume>19</volume>
<page-range>130</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[Brantly]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Avila]]></surname>
<given-names><![CDATA[NA]]></given-names>
</name>
<name>
<surname><![CDATA[Shotelersuk]]></surname>
<given-names><![CDATA[V]]></given-names>
</name>
<name>
<surname><![CDATA[Lucero]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
<name>
<surname><![CDATA[Huizin]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Gahl]]></surname>
<given-names><![CDATA[W]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Pulmonary function and high-resolution CT findings in patients with an inherited form of pulmonary fibrosis, Hermansky-Pudlak syndrome, due to mutations in HPS-1]]></article-title>
<source><![CDATA[Chest]]></source>
<year>2000</year>
<volume>117</volume>
<page-range>129-36</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[Gahl]]></surname>
<given-names><![CDATA[WA]]></given-names>
</name>
<name>
<surname><![CDATA[Brantly]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Troendle]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
<name>
<surname><![CDATA[Avila]]></surname>
<given-names><![CDATA[N]]></given-names>
</name>
<name>
<surname><![CDATA[Padua]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[al]]></surname>
<given-names><![CDATA[et]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Effect of pirfenidone on the pulmonary fibrosis of Hermansky-Pudlak syndrome]]></article-title>
<source><![CDATA[Mol Gen Metabol]]></source>
<year>2002</year>
<volume>76</volume>
<page-range>234-42</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[Avila]]></surname>
<given-names><![CDATA[NA]]></given-names>
</name>
<name>
<surname><![CDATA[Brantly]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Premkumar]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Huizing]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Dwyer]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Gahl]]></surname>
<given-names><![CDATA[W]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Hermansky-Pudlak syndrome: radiography and CT of the chest compared with pulmonary function tests and genetic studies]]></article-title>
<source><![CDATA[AJR Am J Roentgenol]]></source>
<year>2002</year>
<volume>179</volume>
<page-range>887-92</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[Hengst]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Naehrlich]]></surname>
<given-names><![CDATA[L]]></given-names>
</name>
<name>
<surname><![CDATA[Mahavadi]]></surname>
<given-names><![CDATA[P]]></given-names>
</name>
<name>
<surname><![CDATA[Grosse-On]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
<name>
<surname><![CDATA[Terheggen]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
<name>
<surname><![CDATA[Skanke]]></surname>
<given-names><![CDATA[L]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Hermansky-Pudlak syndrome type 2 manifests with fibrosing lung disease early in childhood]]></article-title>
<source><![CDATA[Orphanet J Rare Dis]]></source>
<year>2018</year>
<volume>13</volume>
<page-range>42</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[Berkmen]]></surname>
<given-names><![CDATA[Y]]></given-names>
</name>
<name>
<surname><![CDATA[Dsouza]]></surname>
<given-names><![CDATA[B]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Case 124: Hermansky-Pudlak Syndrome]]></article-title>
<source><![CDATA[Radiology]]></source>
<year>2007</year>
<volume>245</volume>
<page-range>595-9</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[King Jr]]></surname>
<given-names><![CDATA[TE]]></given-names>
</name>
<name>
<surname><![CDATA[Pardo]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Selman]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Idiopathic pulmonary fibrosis]]></article-title>
<source><![CDATA[Lancet]]></source>
<year>2011</year>
<volume>378</volume>
<page-range>1949-61</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[Osanai]]></surname>
<given-names><![CDATA[K]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Rab38 Mutation and the Lung Phenotype]]></article-title>
<source><![CDATA[Int. J. Mol. Sci.]]></source>
<year>2018</year>
<volume>19</volume>
<page-range>2203</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[Nakatani]]></surname>
<given-names><![CDATA[Y]]></given-names>
</name>
<name>
<surname><![CDATA[Nakamura]]></surname>
<given-names><![CDATA[N]]></given-names>
</name>
<name>
<surname><![CDATA[Sano]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
<name>
<surname><![CDATA[Inayama]]></surname>
<given-names><![CDATA[Y]]></given-names>
</name>
<name>
<surname><![CDATA[Kawano]]></surname>
<given-names><![CDATA[N]]></given-names>
</name>
<name>
<surname><![CDATA[Kamanaka]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Interstitial pneumonia in Hermansky-Pudlak syndrome: significance of florid foamy swelling/degeneration (giant lamellar body degeneration) of type-2 pneumocytes]]></article-title>
<source><![CDATA[Virchows Arch]]></source>
<year>2000</year>
<volume>437</volume>
<page-range>304-13</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[O&#8217;Brien]]></surname>
<given-names><![CDATA[KJ]]></given-names>
</name>
<name>
<surname><![CDATA[Introne]]></surname>
<given-names><![CDATA[WJ]]></given-names>
</name>
<name>
<surname><![CDATA[Akal]]></surname>
<given-names><![CDATA[O]]></given-names>
</name>
<name>
<surname><![CDATA[Akal]]></surname>
<given-names><![CDATA[T]]></given-names>
</name>
<name>
<surname><![CDATA[Barbu]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Prolonged treatment with open-label pirfenidone in Hermansky- Pudlak syndrome pulmonary fibrosis]]></article-title>
<source><![CDATA[Mol. Genet. Metab.]]></source>
<year>2018</year>
<volume>125</volume>
<page-range>168-73</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[Meyer]]></surname>
<given-names><![CDATA[KC]]></given-names>
</name>
<name>
<surname><![CDATA[Decker]]></surname>
<given-names><![CDATA[CA]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Role of pirfenidone in the management of pulmonary fibrosis]]></article-title>
<source><![CDATA[Ther Clin Risk Manag]]></source>
<year>2017</year>
<numero>13</numero>
<issue>13</issue>
<page-range>427-37</page-range></nlm-citation>
</ref>
</ref-list>
</back>
</article>
