<?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>1688-1249</journal-id>
<journal-title><![CDATA[Archivos de Pediatría del Uruguay]]></journal-title>
<abbrev-journal-title><![CDATA[Arch. Pediatr. Urug.]]></abbrev-journal-title>
<issn>1688-1249</issn>
<publisher>
<publisher-name><![CDATA[Sociedad Uruguaya de Pediatría]]></publisher-name>
</publisher>
</journal-meta>
<article-meta>
<article-id>S1688-12492024000301315</article-id>
<article-id pub-id-type="doi">10.31134/ap.95.2.14</article-id>
<title-group>
<article-title xml:lang="es"><![CDATA[Hemosiderosis pulmonar secundaria a poliangeitis microscópica. A propósito de un caso]]></article-title>
<article-title xml:lang="en"><![CDATA[Pulmonary hemosiderosis secondary to microscopic polyangiitis. A clinical case study]]></article-title>
<article-title xml:lang="pt"><![CDATA[Hemossiderose pulmonar secundária a poliangeíte microscópica. Relato de caso]]></article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Rijo]]></surname>
<given-names><![CDATA[Sofía]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Cedrés]]></surname>
<given-names><![CDATA[Liliana]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Notejane]]></surname>
<given-names><![CDATA[Martín]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Cantirán]]></surname>
<given-names><![CDATA[Valentina]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Pinchak]]></surname>
<given-names><![CDATA[Catalina]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Moreira]]></surname>
<given-names><![CDATA[Isabel]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Cameto]]></surname>
<given-names><![CDATA[Juan]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Rébori]]></surname>
<given-names><![CDATA[Anabella]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[García]]></surname>
<given-names><![CDATA[Loreley]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
</contrib-group>
<aff id="Af1">
<institution><![CDATA[,UDELAR Facultad de Medicina ]]></institution>
<addr-line><![CDATA[ ]]></addr-line>
<country>Uruguay</country>
</aff>
<aff id="Af2">
<institution><![CDATA[,UDELAR Facultad de Medicina Unidad Académica Pediatría B]]></institution>
<addr-line><![CDATA[ ]]></addr-line>
<country>Uruguay</country>
</aff>
<aff id="Af3">
<institution><![CDATA[,UDELAR Facultad de Medicina Unidad Académica Pediatría B]]></institution>
<addr-line><![CDATA[ ]]></addr-line>
<country>Uruguay</country>
</aff>
<aff id="Af4">
<institution><![CDATA[,UDELAR Facultad de Medicina ]]></institution>
<addr-line><![CDATA[ ]]></addr-line>
<country>Uruguay</country>
</aff>
<aff id="Af5">
<institution><![CDATA[,UDELAR Facultad de Medicina Unidad Académica Pediatría B]]></institution>
<addr-line><![CDATA[ ]]></addr-line>
<country>Uruguay</country>
</aff>
<aff id="Af6">
<institution><![CDATA[,ASSE HP. CHPR Unidad de Neumología Pediátrica]]></institution>
<addr-line><![CDATA[ ]]></addr-line>
</aff>
<aff id="Af7">
<institution><![CDATA[,ASSE HP. CHPR Unidad de Reumatología Pediátrica]]></institution>
<addr-line><![CDATA[ ]]></addr-line>
</aff>
<aff id="Af8">
<institution><![CDATA[,ASSE HP. CHPR Unidad de Nefrología Pediátrica]]></institution>
<addr-line><![CDATA[ ]]></addr-line>
</aff>
<aff id="Af9">
<institution><![CDATA[,UDELAR Facultad de Medicina Unidad Académica Pediatría B]]></institution>
<addr-line><![CDATA[ ]]></addr-line>
<country>Uruguay</country>
</aff>
<pub-date pub-type="pub">
<day>00</day>
<month>00</month>
<year>2024</year>
</pub-date>
<pub-date pub-type="epub">
<day>00</day>
<month>00</month>
<year>2024</year>
</pub-date>
<volume>95</volume>
<numero>2</numero>
<copyright-statement/>
<copyright-year/>
<self-uri xlink:href="http://www.scielo.edu.uy/scielo.php?script=sci_arttext&amp;pid=S1688-12492024000301315&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://www.scielo.edu.uy/scielo.php?script=sci_abstract&amp;pid=S1688-12492024000301315&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://www.scielo.edu.uy/scielo.php?script=sci_pdf&amp;pid=S1688-12492024000301315&amp;lng=en&amp;nrm=iso"></self-uri><abstract abstract-type="short" xml:lang="es"><p><![CDATA[resumen está disponible en el texto completo]]></p></abstract>
<abstract abstract-type="short" xml:lang="en"><p><![CDATA[Summary:  Introduction:  pulmonary hemosiderosis (PH) is a rare, chronic disease characterized by alveolar hemorrhage and accumulation of hemosiderin in alveolar macrophages. It can occur as a primary lung disease or secondary to vasculitis and heart disease.  Objectives:  to describe the diagnostic and therapeutic approach used on a girl with PH secondary to microscopic polyangiitis.  Clinical case:  2 years of age, female. Down syndrome (DS), corrected atrioventricular septal defect. Severe bronchiolitis at 22 months, later recurrent wheezing. One month prior to admission, severe iron deficiency anemia requiring transfusion. Four days prior, respiratory symptoms and progressive fatigue occur in apyrexia. Physical examination: reactive, fair general condition, non-mucosal skin pallor. Drawings, wheezing and bilateral subcrackles. Systolic murmur 2/6. We carried out oxygen therapy, bronchodilators and corticosteroids. Radiography: bilateral and diffuse interstitial infiltrate, without cardiomegaly. Hemogram: mild, microcytic, hypochromic anemia. Iron metabolism confirms iron deficiency. Urine: transient microhematuria. Normal kidney function. Fiberoptic bronchoscopy with bronchioalveolar lavage (LAB): macrophages with brownish granules suggestive of hemosiderin. Etiological studies: Antibodies (Ab): positive antinuclear (1/80), negative anticardiolipins and lupus inhibitor. Negative anti-glomerular basement membrane (GBM) Ab. Positive anti-neutrophil cytoplasmic Ab (ANCA), P-ANCA pattern, positive anti-myeloperoxidase Ab (MPO), negative anti-proteinase 3 Ab (PR3). After the presentation of microscopic polyangiitis, we started administering methylprednisolone, then oral corticosteroids and immunosuppressants. Clinical improvement, without relapses, oxygen was suspended, microhematuria did not recur, she continued receiving mycophenolate during follow-up.  Discussion: severe iron deficiency anemia with hemodynamic repercussions in a child with DS led to HP. The LAB confirmed it. The absence of renal involvement with transient microhematuria and negative anti-GBM antibodies ruled out Goodpasture syndrome. Positive ANCA Abs with the detected pattern indicated microscopic polyangiitis. The prognosis is variable, usually worse in cases with relapses, corticosteroid dependence or lack of complete recovery. Individualized, interdisciplinary long-term follow-up is suggested.]]></p></abstract>
<abstract abstract-type="short" xml:lang="pt"><p><![CDATA[Resumo:  Introdução: a hemossiderose pulmonar (HP) é uma doença crônica rara, caracterizada por hemorragia alveolar e acúmulo de hemossiderina nos macrófagos alveolares. Pode ocorrer como doença pulmonar primária ou secundária a vasculite e doenças cardíacas.  Objetivos:  descrever a abordagem diagnóstica e terapêutica de uma menina com HP secundária a poliangeíte microscópica.  Caso clínico: 2 anos, sexo feminino. Síndrome de Down (SD), comunicação interatrioventricular corrigida. Bronquiolite grave aos 22 meses, posteriormente sibilância recorrente. Um mês antes da internação, anemia ferropriva grave necessitando de transfusão. Quatro dias antes, ocorrem sintomas respiratórios e fadiga progressiva na apirexia. Exame físico: reativo, estado geral razoável, palidez cutânea não mucosa. Desenhos, sibilos e subcrepitações bilaterais. Sopro sistólico 2/6. São realizados: oxigenoterapia, broncodilatadores e corticosteroides. Radiografia: infiltrado intersticial bilateral e difuso, sem cardiomegalia. Hemograma: anemia leve, microcítica e hipocrômica. O metabolismo do ferro confirma a deficiência de ferro. Urina: microhematúria transitória. Função renal normal. Broncoscopia com fibra ótica com lavagem bronquioalveolar (BAL): macrófagos com grânulos acastanhados sugestivos de hemossiderina. Estudos etiológicos: Anticorpos (Ab): antinuclear positivo (1/80), anticardiolipinas e inibidor lúpico negativos. Membrana basal anti-glomerular negativa (GBM) Ab. Ab citoplasmático antineutrófilo positivo (ANCA), padrão P-ANCA, Ab antimieloperoxidase positivo (MPO), Ab antiproteinase 3 negativo (PR3). Com apresentação de poliangeíte microscópica, inicia-se metilprednisolona, seguida de corticosteroides orais e imunossupressores. Melhora clínica, sem recidivas, suspensão de oxigênio, não houve recidiva de microhematúria, continuou com micofenolato para acompanhamento.  Discussão:  anemia ferropriva grave com repercussão hemodinâmica em criança com SD levou à PH. O LAB confirmou isso. A ausência de envolvimento renal com microhematúria transitória e anticorpos anti-GBM negativos descartaram a síndrome de Goodpasture. ANCA Abs positivo com o padrão detectado indicou poliangiite microscópica. O prognóstico é variável, sendo pior nos casos com recidivas, dependência de corticosteroides ou falta de recuperação completa. Sugere-se acompanhamento individualizado e interdisciplinar em longo prazo.]]></p></abstract>
<kwd-group>
<kwd lng="es"><![CDATA[Hemosiderosis Pulmonar]]></kwd>
<kwd lng="es"><![CDATA[Poliangitis Microscópica]]></kwd>
<kwd lng="es"><![CDATA[Vasculitis Asociadas a]]></kwd>
<kwd lng="es"><![CDATA[Anticuerpos Citoplasmático]]></kwd>
<kwd lng="es"><![CDATA[Antineutrofilos]]></kwd>
<kwd lng="es"><![CDATA[Niño]]></kwd>
<kwd lng="en"><![CDATA[Pulmonary Hemosiderosis]]></kwd>
<kwd lng="en"><![CDATA[Microscopic Polyangiitis]]></kwd>
<kwd lng="en"><![CDATA[Vasculitis Associated with]]></kwd>
<kwd lng="en"><![CDATA[Antineutrophil Cytoplasmic]]></kwd>
<kwd lng="en"><![CDATA[Antibodies]]></kwd>
<kwd lng="en"><![CDATA[Child]]></kwd>
<kwd lng="pt"><![CDATA[Hemossiderose Pulmonar]]></kwd>
<kwd lng="pt"><![CDATA[Poliangiite Microscópica]]></kwd>
<kwd lng="pt"><![CDATA[Vasculite Associada a]]></kwd>
<kwd lng="pt"><![CDATA[Anticorpos Citoplasmáticos Antineutrófilos]]></kwd>
<kwd lng="pt"><![CDATA[Criança]]></kwd>
</kwd-group>
</article-meta>
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