{"id":2089,"date":"2009-12-30T18:14:12","date_gmt":"2009-12-30T23:14:12","guid":{"rendered":"http:\/\/insidesurgery.com\/?p=2089"},"modified":"2009-12-30T18:33:12","modified_gmt":"2009-12-30T23:33:12","slug":"dubin-johnson-syndrome","status":"publish","type":"post","link":"https:\/\/insidesurgery.com\/2009\/12\/dubin-johnson-syndrome\/","title":{"rendered":"Dubin Johnson Syndrome"},"content":{"rendered":"<p><strong>Pathophysiology<\/strong><\/p>\n<p><strong>1)<\/strong> decreased intracellular transport of conjugated bilirubin <strong>2)<\/strong> cause is defective hepatocellular secretion of bilirubin glucuronides into the canalicular lumen<\/p>\n<p><strong>Signs and Symptoms<\/strong><\/p>\n<p><strong>1)<\/strong> fairly benign clincially <strong>2)<\/strong> mild, episodic jaundice <strong>3)<\/strong> vague complaints of weakness and fatigue <strong>4)<\/strong> some cases with hepatomegaly <strong>5)<\/strong> 50% of patients have dark-colored urine <strong>6)<\/strong> often does not present until first pregnancy or use of oral contraceptives<\/p>\n<p><strong>Characteristic Test Findings<\/strong><\/p>\n<p><em>Laboratory<\/em> &#8211; <strong>1)<\/strong> chronically elevated, fluctuating levels of conjugated bilirubin (2-5 mg\/dL) <strong>2)<\/strong> bilirubinuria (bilirubin in the urine) <strong>3)<\/strong> total amount of serum coproporphyrins is normal, but the fraction of the type 1 isomer is increased from 25% to 80%<\/p>\n<p><strong>Histology\/Gross Pathology<\/strong><\/p>\n<p><strong>1)<\/strong> brownish pigment deposition in hepatocyte centrilobular zone and Kupffer cells <strong>2)<\/strong> grossly, the liver looks black<\/p>\n<p><strong>Biochemistry<\/strong><\/p>\n<p><strong>1)<\/strong> specific defect is in ATP-dependent transport mechanism of bilirubin glucuronides into the canaliculus <strong>2)<\/strong> caused by point mutation in multi-drug resistance-associated protein 2 (MRP2) <strong>3)<\/strong> defect also exists in transport of coproporphyrins<\/p>\n<p><strong>Inheritance\/Epidemiology<\/strong><\/p>\n<p>1) autosomal recessive 2) rare 3) tends to occur most commonly in groups with high rates of intermarriage such as Iranian Jews and remote island populations<\/p>\n<p><strong>Tips for USMLE<\/strong><\/p>\n<p><strong>1)<\/strong> study of the urinary coproporphyrin level is used to distinguish Dubin-Johnson from other conjugated hyperbilirubinemias <strong>2)<\/strong> in most liver diseases with jaundice, the total coproporphyrin is elevated with type 1 isomer no more than 65%<\/p>\n","protected":false},"excerpt":{"rendered":"<p>Pathophysiology 1) decreased intracellular transport of conjugated bilirubin 2) cause is defective hepatocellular secretion of bilirubin glucuronides into the canalicular lumen Signs and Symptoms 1) fairly benign clincially 2) mild, episodic jaundice 3) vague complaints of weakness and fatigue 4) some cases with hepatomegaly 5) 50% of patients have dark-colored urine 6) often does not [&hellip;]<\/p>\n","protected":false},"author":1,"featured_media":0,"comment_status":"open","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"_exactmetrics_skip_tracking":false,"_exactmetrics_sitenote_active":false,"_exactmetrics_sitenote_note":"","_exactmetrics_sitenote_category":0,"_genesis_hide_title":false,"_genesis_hide_breadcrumbs":false,"_genesis_hide_singular_image":false,"_genesis_hide_footer_widgets":false,"_genesis_custom_body_class":"","_genesis_custom_post_class":"","_genesis_layout":"","footnotes":""},"categories":[63],"tags":[728,729,726,725,727,730],"yoast_head":"<!-- This site is optimized with the Yoast SEO plugin v22.5 - https:\/\/yoast.com\/wordpress\/plugins\/seo\/ -->\n<title>Dubin Johnson Syndrome - InsideSurgery Medical Information Blog<\/title>\n<meta name=\"robots\" content=\"index, follow, max-snippet:-1, max-image-preview:large, max-video-preview:-1\" \/>\n<link rel=\"canonical\" href=\"https:\/\/insidesurgery.com\/2009\/12\/dubin-johnson-syndrome\/\" \/>\n<meta property=\"og:locale\" content=\"en_US\" \/>\n<meta property=\"og:type\" content=\"article\" \/>\n<meta property=\"og:title\" content=\"Dubin Johnson Syndrome - InsideSurgery Medical Information Blog\" \/>\n<meta property=\"og:description\" content=\"Pathophysiology 1) decreased intracellular transport of conjugated bilirubin 2) cause is defective hepatocellular secretion of bilirubin glucuronides into the canalicular lumen Signs and Symptoms 1) fairly benign clincially 2) mild, episodic jaundice 3) vague complaints of weakness and fatigue 4) some cases with hepatomegaly 5) 50% of patients have dark-colored urine 6) often does not [&hellip;]\" \/>\n<meta property=\"og:url\" content=\"https:\/\/insidesurgery.com\/2009\/12\/dubin-johnson-syndrome\/\" \/>\n<meta property=\"og:site_name\" content=\"InsideSurgery Medical Information Blog\" \/>\n<meta property=\"article:published_time\" content=\"2009-12-30T23:14:12+00:00\" \/>\n<meta property=\"article:modified_time\" content=\"2009-12-30T23:33:12+00:00\" \/>\n<meta name=\"author\" content=\"Editor\" \/>\n<meta name=\"twitter:label1\" content=\"Written by\" \/>\n\t<meta name=\"twitter:data1\" content=\"Editor\" \/>\n\t<meta name=\"twitter:label2\" content=\"Est. reading time\" \/>\n\t<meta name=\"twitter:data2\" content=\"1 minute\" \/>\n<script type=\"application\/ld+json\" class=\"yoast-schema-graph\">{\"@context\":\"https:\/\/schema.org\",\"@graph\":[{\"@type\":\"WebPage\",\"@id\":\"https:\/\/insidesurgery.com\/2009\/12\/dubin-johnson-syndrome\/\",\"url\":\"https:\/\/insidesurgery.com\/2009\/12\/dubin-johnson-syndrome\/\",\"name\":\"Dubin Johnson Syndrome - InsideSurgery Medical Information Blog\",\"isPartOf\":{\"@id\":\"https:\/\/insidesurgery.com\/#website\"},\"datePublished\":\"2009-12-30T23:14:12+00:00\",\"dateModified\":\"2009-12-30T23:33:12+00:00\",\"author\":{\"@id\":\"https:\/\/insidesurgery.com\/#\/schema\/person\/80c1e5c67f8a2c348e38e202270e8594\"},\"breadcrumb\":{\"@id\":\"https:\/\/insidesurgery.com\/2009\/12\/dubin-johnson-syndrome\/#breadcrumb\"},\"inLanguage\":\"en-US\",\"potentialAction\":[{\"@type\":\"ReadAction\",\"target\":[\"https:\/\/insidesurgery.com\/2009\/12\/dubin-johnson-syndrome\/\"]}]},{\"@type\":\"BreadcrumbList\",\"@id\":\"https:\/\/insidesurgery.com\/2009\/12\/dubin-johnson-syndrome\/#breadcrumb\",\"itemListElement\":[{\"@type\":\"ListItem\",\"position\":1,\"name\":\"Home\",\"item\":\"https:\/\/insidesurgery.com\/\"},{\"@type\":\"ListItem\",\"position\":2,\"name\":\"Dubin Johnson Syndrome\"}]},{\"@type\":\"WebSite\",\"@id\":\"https:\/\/insidesurgery.com\/#website\",\"url\":\"https:\/\/insidesurgery.com\/\",\"name\":\"InsideSurgery Medical Information Blog\",\"description\":\"Details of surgical procedures, pathophysiology, signs and symptoms, and treatment of medical diseases,medical and surgical eponyms, and surgeons and surgery in the news\",\"potentialAction\":[{\"@type\":\"SearchAction\",\"target\":{\"@type\":\"EntryPoint\",\"urlTemplate\":\"https:\/\/insidesurgery.com\/?s={search_term_string}\"},\"query-input\":\"required name=search_term_string\"}],\"inLanguage\":\"en-US\"},{\"@type\":\"Person\",\"@id\":\"https:\/\/insidesurgery.com\/#\/schema\/person\/80c1e5c67f8a2c348e38e202270e8594\",\"name\":\"Editor\",\"image\":{\"@type\":\"ImageObject\",\"inLanguage\":\"en-US\",\"@id\":\"https:\/\/insidesurgery.com\/#\/schema\/person\/image\/\",\"url\":\"https:\/\/secure.gravatar.com\/avatar\/41c3c845307fcd3eff88632d6ccaf656?s=96&d=mm&r=g\",\"contentUrl\":\"https:\/\/secure.gravatar.com\/avatar\/41c3c845307fcd3eff88632d6ccaf656?s=96&d=mm&r=g\",\"caption\":\"Editor\"}}]}<\/script>\n<!-- \/ Yoast SEO plugin. -->","yoast_head_json":{"title":"Dubin Johnson Syndrome - InsideSurgery Medical Information Blog","robots":{"index":"index","follow":"follow","max-snippet":"max-snippet:-1","max-image-preview":"max-image-preview:large","max-video-preview":"max-video-preview:-1"},"canonical":"https:\/\/insidesurgery.com\/2009\/12\/dubin-johnson-syndrome\/","og_locale":"en_US","og_type":"article","og_title":"Dubin Johnson Syndrome - InsideSurgery Medical Information Blog","og_description":"Pathophysiology 1) decreased intracellular transport of conjugated bilirubin 2) cause is defective hepatocellular secretion of bilirubin glucuronides into the canalicular lumen Signs and Symptoms 1) fairly benign clincially 2) mild, episodic jaundice 3) vague complaints of weakness and fatigue 4) some cases with hepatomegaly 5) 50% of patients have dark-colored urine 6) often does not [&hellip;]","og_url":"https:\/\/insidesurgery.com\/2009\/12\/dubin-johnson-syndrome\/","og_site_name":"InsideSurgery Medical Information Blog","article_published_time":"2009-12-30T23:14:12+00:00","article_modified_time":"2009-12-30T23:33:12+00:00","author":"Editor","twitter_misc":{"Written by":"Editor","Est. reading time":"1 minute"},"schema":{"@context":"https:\/\/schema.org","@graph":[{"@type":"WebPage","@id":"https:\/\/insidesurgery.com\/2009\/12\/dubin-johnson-syndrome\/","url":"https:\/\/insidesurgery.com\/2009\/12\/dubin-johnson-syndrome\/","name":"Dubin Johnson Syndrome - InsideSurgery Medical Information Blog","isPartOf":{"@id":"https:\/\/insidesurgery.com\/#website"},"datePublished":"2009-12-30T23:14:12+00:00","dateModified":"2009-12-30T23:33:12+00:00","author":{"@id":"https:\/\/insidesurgery.com\/#\/schema\/person\/80c1e5c67f8a2c348e38e202270e8594"},"breadcrumb":{"@id":"https:\/\/insidesurgery.com\/2009\/12\/dubin-johnson-syndrome\/#breadcrumb"},"inLanguage":"en-US","potentialAction":[{"@type":"ReadAction","target":["https:\/\/insidesurgery.com\/2009\/12\/dubin-johnson-syndrome\/"]}]},{"@type":"BreadcrumbList","@id":"https:\/\/insidesurgery.com\/2009\/12\/dubin-johnson-syndrome\/#breadcrumb","itemListElement":[{"@type":"ListItem","position":1,"name":"Home","item":"https:\/\/insidesurgery.com\/"},{"@type":"ListItem","position":2,"name":"Dubin Johnson Syndrome"}]},{"@type":"WebSite","@id":"https:\/\/insidesurgery.com\/#website","url":"https:\/\/insidesurgery.com\/","name":"InsideSurgery Medical Information Blog","description":"Details of surgical procedures, pathophysiology, signs and symptoms, and treatment of medical diseases,medical and surgical eponyms, and surgeons and surgery in the news","potentialAction":[{"@type":"SearchAction","target":{"@type":"EntryPoint","urlTemplate":"https:\/\/insidesurgery.com\/?s={search_term_string}"},"query-input":"required name=search_term_string"}],"inLanguage":"en-US"},{"@type":"Person","@id":"https:\/\/insidesurgery.com\/#\/schema\/person\/80c1e5c67f8a2c348e38e202270e8594","name":"Editor","image":{"@type":"ImageObject","inLanguage":"en-US","@id":"https:\/\/insidesurgery.com\/#\/schema\/person\/image\/","url":"https:\/\/secure.gravatar.com\/avatar\/41c3c845307fcd3eff88632d6ccaf656?s=96&d=mm&r=g","contentUrl":"https:\/\/secure.gravatar.com\/avatar\/41c3c845307fcd3eff88632d6ccaf656?s=96&d=mm&r=g","caption":"Editor"}}]}},"_links":{"self":[{"href":"https:\/\/insidesurgery.com\/wp-json\/wp\/v2\/posts\/2089"}],"collection":[{"href":"https:\/\/insidesurgery.com\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/insidesurgery.com\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/insidesurgery.com\/wp-json\/wp\/v2\/users\/1"}],"replies":[{"embeddable":true,"href":"https:\/\/insidesurgery.com\/wp-json\/wp\/v2\/comments?post=2089"}],"version-history":[{"count":0,"href":"https:\/\/insidesurgery.com\/wp-json\/wp\/v2\/posts\/2089\/revisions"}],"wp:attachment":[{"href":"https:\/\/insidesurgery.com\/wp-json\/wp\/v2\/media?parent=2089"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/insidesurgery.com\/wp-json\/wp\/v2\/categories?post=2089"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/insidesurgery.com\/wp-json\/wp\/v2\/tags?post=2089"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}