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Riancho" "autores" => array:3 [ 0 => array:4 [ "nombre" => "Álex" "apellidos" => "García Tellado" "email" => array:1 [ 0 => "agt1695@hotmail.com" ] "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] 1 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">*</span>" "identificador" => "cor0005" ] ] ] 1 => array:3 [ "nombre" => "Rosa" "apellidos" => "Herreras" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 2 => array:3 [ "nombre" => "José A." "apellidos" => "Riancho" "referencia" => array:3 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] 1 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] 2 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">c</span>" "identificador" => "aff0015" ] ] ] ] "afiliaciones" => array:3 [ 0 => array:3 [ "entidad" => "Servicio de Medicina Interna, Hospital Marqués de Valdecilla, Santander, Spain" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Instituto de Investigación Marqués de Valdecilla (IDIVAL), Universidad de Cantabria, Santander, Spain" "etiqueta" => "b" "identificador" => "aff0010" ] 2 => array:3 [ "entidad" => "Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER), Santander, Spain" "etiqueta" => "c" "identificador" => "aff0015" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Infección sistémica por <span class="elsevierStyleItalic">Campylobacter</span> en un paciente con agammaglobulinemia" ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">X-linked agammaglobulinaemia or Bruton’s disease is caused by the presence of a pathogenic variant in the Bruton’s Tyrosine Kinase (BTK) gene, which interferes with B-cell maturation and prevents the normal production of antibodies.<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> As a result, patients often have infections and, paradoxically, an increased incidence of autoimmune phenomena. Bruton’s disease is an ultra-rare disease with a prevalence of about one case per million population. It manifests in males, with a hemizygous mutation. Heterozygous female carriers are usually asymptomatic. Standard treatment is periodic infusions of immunoglobulins; allotransplantation is reserved for selected cases.<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">We report the case of a 26-year-old male diagnosed with X-linked agammaglobulinaemia, due to a hemizygous mutation of the <span class="elsevierStyleItalic">BTK</span> gene, under treatment with periodic infusions of intravenous immunoglobulins. In the last 8 weeks he reported persistent fever with peaks of up to 38.5 °C, sweating and weight loss, with no other accompanying symptoms or relevant signs on examination.</p><p id="par0015" class="elsevierStylePara elsevierViewall">Laboratory tests showed a moderate elevation of acute phase reactants, with a C-reactive protein of 12 mg/dl (NR: <0.4 mg/dl) and ESR of 120 mm/h (NR: 0–22 mm/h). Likewise, discrete elevation of alkaline phosphatase of 278 UI/l (NR: 44–147 UI/l), normochromic microcytic anaemia without associated nutritional deficit (haemoglobin: 9.3 g/dl; NR: 13.2–16.6 g/dl) and lymphopenia. Initial microbiological screening, which included several blood culture determinations, as well as serology for hepatotropic viruses (HCV, HBV, EBV, CMV) and HIV were negative. Respiratory virus smears (SARS-CoV-2, influenza A and B) and sputum culture were also negative. A thoracoabdominal CT scan revealed hepatosplenomegaly as the only notable finding, and a PET/CT scan showed faint activity in the subdiaphragmatic lymph nodes and bone marrow. For all these reasons, and with the suspicion of a lymphoproliferative or hemophagocytic syndrome type complication, a bone marrow biopsy was performed, which ruled out these processes. However, <span class="elsevierStyleItalic">Campylobacter jejuni</span> grew in the bone marrow culture. Intravenous treatment with imipenem was started, followed by oral doxycycline for a total of 30 days. The clinical course was favourable, with resolution of fever and other manifestations.</p><p id="par0020" class="elsevierStylePara elsevierViewall">In addition to the rarity of the underlying disease, this case is exceptional for several reasons. Firstly, it is a systemic infection by a microorganism, <span class="elsevierStyleItalic">Campylobacter,</span> which is a relatively common cause of digestive tract infections, usually in the form of gastroenteritis or colitis, with or without rectal bleeding, but without systemic manifestations. In fact, only a few isolated cases of systemic <span class="elsevierStyleItalic">Campylobacter</span> infection in patients with agammaglobulinaemia have previously been reported.<a class="elsevierStyleCrossRefs" href="#bib0010"><span class="elsevierStyleSup">2–4</span></a> On the other hand, it is remarkable that the micro-organism was isolated from the bone marrow, while other samples, including blood cultures, were negative.</p><p id="par0025" class="elsevierStylePara elsevierViewall">In short, the case highlights the need to be alert to the possibility that patients with immunodeficiencies, despite receiving replacement therapy, may develop serious infections with atypical manifestations, either in unusual sites or disseminated by microorganisms that typically cause benign processes.</p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Ethical considerations</span><p id="par0030" class="elsevierStylePara elsevierViewall">Informed consent was obtained from the patient for publication of the case.</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Funding</span><p id="par0035" class="elsevierStylePara elsevierViewall">No funding has been received.</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Conflict of interest</span><p id="par0040" class="elsevierStylePara elsevierViewall">The authors declare that they have no conflicts of interest.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:4 [ 0 => array:2 [ "identificador" => "sec0005" "titulo" => "Ethical considerations" ] 1 => array:2 [ "identificador" => "sec0010" "titulo" => "Funding" ] 2 => array:2 [ "identificador" => "sec0015" "titulo" => "Conflict of interest" ] 3 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:4 [ 0 => array:3 [ "identificador" => "bib0005" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "An international survey of allogeneic hematopoietic cell transplantation for X-linked agammaglobulinemia" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "A. Nishimura" 1 => "R. Uppuluri" 2 => "R. Raj" 3 => "V.V. Swaminathan" 4 => "Y. Cheng" 5 => "R.F. Abu-Arja" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1007/s10875-023-01551-2" "Revista" => array:6 [ "tituloSerie" => "J Clin Immunol" "fecha" => "2023" "volumen" => "43" "paginaInicial" => "1827" "paginaFinal" => "1839" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/37454339" "web" => "Medline" ] ] ] ] ] ] ] ] 1 => array:3 [ "identificador" => "bib0010" "etiqueta" => "2" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Recurrent <span class="elsevierStyleItalic">Campylobacter jejuni</span> bacteremia in a patient with hypogammaglobulinemia: a case report" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:6 [ 0 => "Y. Kim" 1 => "J.A. Shin" 2 => "S.B. Han" 3 => "B. Cho" 4 => "D.C. Jeong" 5 => "J.H. Kang" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1097/MD.0000000000007238" "Revista" => array:6 [ "tituloSerie" => "Medicine (Baltimore)" "fecha" => "2017" "volumen" => "96" "paginaInicial" => "e7238" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/28640123" "web" => "Medline" ] ] "itemHostRev" => array:3 [ "pii" => "S0016510720341857" "estado" => "S300" "issn" => "00165107" ] ] ] ] ] ] ] 2 => array:3 [ "identificador" => "bib0015" "etiqueta" => "3" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Relapsing <span class="elsevierStyleItalic">Campylobacter jejuni</span> systemic infections in a child with X-linked agammaglobulinemia" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "P. Ariganello" 1 => "G. Angelino" 2 => "A. Scarselli" 3 => "I. Salfa" 4 => "M. della Corte" 5 => "A. de Matteis" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1155/2013/735108" "Revista" => array:6 [ "tituloSerie" => "Case Rep Pediatr" "fecha" => "2013" "volumen" => "2013" "paginaInicial" => "735108" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/23862092" "web" => "Medline" ] ] "itemHostRev" => array:3 [ "pii" => "S0016510719316657" "estado" => "S300" "issn" => "00165107" ] ] ] ] ] ] ] 3 => array:3 [ "identificador" => "bib0020" "etiqueta" => "4" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "X-linked agammaglobulinemia: infection frequency and infection-related mortality in the USIDNET Registry" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "D. O’Toole" 1 => "D. Groth" 2 => "H. Wright" 3 => "F.A. Bonilla" 4 => "R.L. Fuleihan" 5 => "C. 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Journal Information
Vol. 163. Issue 1.
Pages 49-50 (July 2024)
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