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Chronic cholestasis in a 20-year-old young man: A case of idiopathic adulthood ductopenia
Colestasis crónica en un joven de 20 años: un caso de la vida adulta ductopenia idiopática
Pilar Peniche-Moquela, José Luis Pérez-Hernándeza, Jacqueline Cordovab,
Corresponding author
a Unit of Gastroenterology, Mexico's General Hospital “Dr. Eduardo Liceaga”, O.D., Mexico
b Division of Transplantation, Mexico's General Hospital “Dr. Eduardo Liceaga”, O.D., Mexico
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    "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Idiopathic adulthood ductopenia &#40;IAD&#41; is a rare disease whose etiology is not known&#46; It is a chronic cholestatic liver condition which appears at an adult age&#59; in which there is loss of bile ducts in more than 50&#37; of portal tracts&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> Several conditions are associated with a decrease in the number of intrahepatic bile ducts of small and medium size&#46; Some result in development failure and others are disorders that lead to acquired ductopenia&#44; some of which are associated with destruction of the bile duct epithelium and for others there is no apparent inflammatory process&#46;<a class="elsevierStyleCrossRefs" href="#bib0010"><span class="elsevierStyleSup">2&#44;3</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">A male&#44; 20 years old&#44; previously healthy&#44; with no history of jaundice at birth or in childhood&#44; denies alcoholism&#44; smoking or other addictions&#44; no previous use of drugs or herbal products&#44; was referred to the Mexico&#39;s General Hospital with symptoms of 6 months of evolution characterized by fatigue&#44; weakness&#44; itching&#44; routine laboratory tests were performed finding persistent cholestasis&#46; Physical examination was normal&#44; with no stigmata of chronic liver disease&#46; The laboratory tests showed Total Bilirubin &#40;BT&#41; 0&#46;8<span class="elsevierStyleHsp" style=""></span>mg&#47;dL &#40;0&#46;3&#8211;0&#46;9<span class="elsevierStyleHsp" style=""></span>mg&#47;dL&#41;&#44; alkaline phosphatase &#40;ALP&#41; 618<span class="elsevierStyleHsp" style=""></span>U&#47;l &#40;44&#8211;147<span class="elsevierStyleHsp" style=""></span>U&#47;L&#41;&#44; Gamma glutamyltranspeptidase &#40;GGT&#41; 912<span class="elsevierStyleHsp" style=""></span>U&#47;l &#40;40&#8211;78<span class="elsevierStyleHsp" style=""></span>U&#47;L&#41;&#44; aspartate aminotransferase &#40;AST&#41; 213<span class="elsevierStyleHsp" style=""></span>U&#47;L &#40;8&#8211;40<span class="elsevierStyleHsp" style=""></span>U&#47;L&#41;&#44; alanine aminotransferase &#40;ALT&#41; 433<span class="elsevierStyleHsp" style=""></span>U&#47;L &#40;7&#8211;33<span class="elsevierStyleHsp" style=""></span>U&#47;L&#41;&#46; Viral Hepatitis Panel for B&#44; <span class="elsevierStyleSmallCaps">C</span> and HIV were performed and were negative&#46; Serologic tests for anti-nuclear&#44; anti-mitochondrial&#44; anti-smooth muscle&#44; anti-LKM1 antibodies were negative&#46; The ceruloplasmin and copper in 24-h urine were normal&#46; An abdominal ultrasound performed showed no abnormalities in liver morphology&#44; portal vein and hepatic permeable&#44; unaltered hepatic artery&#44; without dilatation of the intrahepatic or extrahepatic bile ducts&#46; The CPMRI was unaltered&#46; Percutaneous liver biopsy was performed reporting&#58; preserved liver architecture&#44; sparse chronic inflammatory infiltrate&#44; bile duct not observed in over 50&#37; of portal tracts observed &#40;see <a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">The patient was diagnosed with idiopathic adult ductopenia&#44; treatment was started with a dose of ursodeoxycholic acid &#40;13&#8211;15<span class="elsevierStyleHsp" style=""></span>mg&#47;kg&#47;day&#41; 250<span class="elsevierStyleHsp" style=""></span>mg every 12<span class="elsevierStyleHsp" style=""></span>h&#44; mild improvement in symptoms and liver function tests was observed after 6 months of treatment &#40;see <a class="elsevierStyleCrossRef" href="#tbl0005">Table 1</a>&#41;&#46;</p><elsevierMultimedia ident="tbl0005"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">Ductopenia describes the loss of septal and interlobular bile ducts for any reason with the possibility of the development of biliary cirrhosis or hepatic insufficiency&#46;<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> However&#44; there have been several clinical cases of non-progressive IAD&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> The most common causes of chronic cholestasis are primary biliary cirrhosis diseases &#40;PBC&#41; and primary sclerosing cholangitis &#40;PSC&#41;&#44; in 90&#37; of cases&#44; representing the IAD in only 1&#37; of cases&#46;<a class="elsevierStyleCrossRefs" href="#bib0025"><span class="elsevierStyleSup">5&#8211;7</span></a> In IAD there may be evidence of ductopenia in the biopsy&#44; but there are no clinical&#44; biochemical or serological findings indicating a specific cause&#46;<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> It affects young adults&#44; with a male predominance<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> with an average year of appearance at 30 for men and 26 for women&#46;<a class="elsevierStyleCrossRefs" href="#bib0025"><span class="elsevierStyleSup">5&#44;6</span></a> The current diagnostic criteria include biochemical cholestasis and histopathological evidence of ductopenia in the absence of primary sclerosing cholangitis or inflammatory bowel disease&#46;<a class="elsevierStyleCrossRefs" href="#bib0020"><span class="elsevierStyleSup">4&#44;8</span></a> The diagnosis must be made on the loss of septal or interlobular bile ducts in at least 50&#37; of portal tracts&#44; to be considered significant&#44; at least 20 or more portal tracts&#44; and the absence of granulomatous cholangitis or a florid duct lesion&#46;<a class="elsevierStyleCrossRefs" href="#bib0020"><span class="elsevierStyleSup">4&#44;9</span></a> The clinical course is variable&#44; some patients may be asymptomatic&#44; other patients may have episodes of jaundice and itching and with elevated serum liver enzymes&#46; Two types of IAD have been recognized&#44; each with different prognosis&#44; the patients who develop type 1 are asymptomatic or manifest symptoms of chronic cholestatic disease&#44; tend to have less destruction of bile ducts in liver biopsy specimens&#46; In contrast with type 2 where patients can have initial symptoms of decompensated biliary cirrhosis&#44; extensive destruction of intrahepatic bile ducts can be found&#44; and liver biopsy is often required&#46;<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">9</span></a> In a review of 25 patients with the diagnosis of IAD&#44; it was found that bilirubin levels could be up to 26<span class="elsevierStyleHsp" style=""></span>mg&#47;dL&#44; the ALP and GGT values ranged from 3 to 16 and 14 times the normal upper limit respectively&#44; and the levels of aminotransferases could vary from normal to an elevation of 10 times the upper limit&#46;<a class="elsevierStyleCrossRefs" href="#bib0020"><span class="elsevierStyleSup">4&#44;10</span></a> In about half the cases reported the IAD appears to have a benign course&#44; while the rest of the IAD were mortal or merited liver transplantation&#46;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a></p><p id="par0025" class="elsevierStylePara elsevierViewall">Although the cause has not been determined&#44; several hypotheses have been made&#44; such as&#44; that the destruction of the bile ducts is caused by an unknown virus or a toxic metabolite&#46; Host factors may contribute to susceptibility to developing the disease&#44; in a study by Garcia-Jimenez et al&#46; with genes HLA-DR&#44; no significant association between HLA-DRB1 alleles and disease was found&#44; but suggests that IAD may have immunogenetic basis in its development&#46;<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">9</span></a> The degree of ductopenia is variable&#44; when it is mild&#44; it may be overlooked during histopathology&#46;<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a></p><p id="par0030" class="elsevierStylePara elsevierViewall">The IAD is a rare disease&#44; with unknown pathogenesis and evolution&#46; The uncertainties about the etiology preclude a specific treatment&#59; in a non-aggressive disease &#40;type 1 IAD&#41; ursodeoxycholic acid &#40;UDCA&#41; can be used for treatment&#46; A beneficial effect has been suggested but it is unknown its effect on disease progression&#46;<a class="elsevierStyleCrossRefs" href="#bib0020"><span class="elsevierStyleSup">4&#44;6</span></a> Sometimes only liver transplantation offers a cure&#44; especially in cases where cholestatic symptoms are intractable like the severe itching or when there are symptoms of liver failure&#46;<a class="elsevierStyleCrossRefs" href="#bib0025"><span class="elsevierStyleSup">5&#44;6</span></a></p><p id="par0035" class="elsevierStylePara elsevierViewall">In conclusion&#44; it would be important to perform a research to identify possible causes and pathogenesis of the disease&#44; to predict the likely clinical course&#44; and to offer appropriate treatment according to the presentation of the IAD&#46;</p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Conflict of interest</span><p id="par0040" class="elsevierStylePara elsevierViewall">The authors have no conflict of interest to declare&#46;</p></span></span>"
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          "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Evolution of Liver function test profiles &#40;LFT&#41;&#44; at the beginning of treatment and during follow-up at 3&#44; 6 and 12 months&#46;</p>"
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      "titulo" => "References"
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        0 => array:2 [
          "identificador" => "bibs0005"
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            0 => array:3 [
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                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Idiopathic biliary ductopenia in adults without symptoms of liver disease"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:4 [
                            0 => "A&#46; Moreno"
                            1 => "V&#46; Carre&#241;o"
                            2 => "A&#46; Cano"
                            3 => "C&#46; Gonz&#225;lez"
                          ]
                        ]
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                    ]
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                    0 => array:2 [
                      "doi" => "10.1056/NEJM199703203361204"
                      "Revista" => array:6 [
                        "tituloSerie" => "NEJM"
                        "fecha" => "1997"
                        "volumen" => "336"
                        "paginaInicial" => "835"
                        "paginaFinal" => "838"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/9062092"
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                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Primary biliary cirrhosis and other ductopenic diseases"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:1 [
                            0 => "D&#46;B&#46; Alastair"
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                  "host" => array:1 [
                    0 => array:1 [
                      "Revista" => array:6 [
                        "tituloSerie" => "Clin Liver Dis"
                        "fecha" => "2002"
                        "volumen" => "6"
                        "paginaInicial" => "363"
                        "paginaFinal" => "380"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/12122861"
                            "web" => "Medline"
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              "referencia" => array:1 [
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                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "The variant forms of cholestatic diseases involving small bile ducts in adults"
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                        0 => array:2 [
                          "etal" => false
                          "autores" => array:1 [
                            0 => "O&#46; Chazouill&#232;res"
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                    0 => array:1 [
                      "Revista" => array:5 [
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                        "paginaFinal" => "18"
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                    0 => array:2 [
                      "titulo" => "Idiopathic adulthood ductopenia&#58; an update"
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                        0 => array:2 [
                          "etal" => false
                          "autores" => array:1 [
                            0 => "J&#46; Ludwing"
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                      "doi" => "10.1016/S0025-6196(11)64473-X"
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                        "tituloSerie" => "Mayo Clin Proc"
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                          "etal" => false
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                      "titulo" => "A case of idiopathic adulthood ductopenia"
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                          "etal" => true
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                            3 => "H&#46;B&#46; Chae"
                            4 => "S&#46;J&#46; Yoon"
                            5 => "R&#46; Sung"
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                    0 => array:1 [
                      "Revista" => array:5 [
                        "tituloSerie" => "Korean J Intern Med"
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                            3 => "B&#46; de Cuenca"
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                            4 => "G&#46; Mazzella"
                            5 => "A&#46; Roda"
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ISSN: 02105705
Original language: English
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es en pt

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