{"id":625,"date":"2020-02-14T07:30:31","date_gmt":"2020-02-14T07:30:31","guid":{"rendered":"https:\/\/meddists.com\/learn\/?page_id=625"},"modified":"2020-02-14T07:32:32","modified_gmt":"2020-02-14T07:32:32","slug":"pagets-disease","status":"publish","type":"page","link":"https:\/\/meddists.com\/learn\/pre-clinical\/pathology\/bone-pathology\/pagets-disease\/","title":{"rendered":"Paget&#8217;s disease"},"content":{"rendered":"<span class=\"block-heading\" id=\"header_1\">\n<h4 class=\"wp-block-heading\" class=\"wp-block-heading\" class=\"title_collection title1\">Definition<\/h4>\n<\/span><span class=\"block-content\" id=\"contents_1\">\n\n\n<p class=\"wp-block-paragraph\">Disease of the bones involving bone breakdown followed by a disordered pattern of excessive bone growth.<\/p>\n\n\n<\/span><span class=\"block-heading\" id=\"header_2\">\n<h4 class=\"wp-block-heading\" class=\"wp-block-heading\" class=\"title_collection title1\">Characteristics<\/h4>\n<\/span><span class=\"block-content\" id=\"contents_2\">\n\n\n<ul class=\"wp-block-list\"><li>Also known as <strong>osteitis deformans<\/strong><\/li><li>Late-onset&nbsp;(late adulthood)<\/li><li>Initially, the bone breaks down, with excessive reconstruction later on<\/li><li>Most cases involve multiple bones (<strong>polyostotic<\/strong>), while single bone involvement (<strong>monostotic<\/strong>) is more rare\n<ul><li>Does not involve the entire skeleton<\/li><\/ul>\n<\/li><li>The typical facial involvement at the late stage is known as &#8220;lion face&#8221; (leontiasis ossea) due to the facial bone deformities (maxilla, mandible)<\/li><li>An increase of&nbsp;NF-\u03baB activity stimulates osteoclast activity<\/li><li>The cause is thought to be related to a viral infection (measles)<\/li><li>More common in Caucasians<\/li><\/ul>\n\n\n<\/span><span class=\"block-heading\" id=\"header_3\">\n<h4 class=\"wp-block-heading\" class=\"wp-block-heading\" class=\"title_collection title1\">Phases<\/h4>\n<\/span><span class=\"block-content\" id=\"contents_3\">\n\n\n<p class=\"wp-block-paragraph\">The disease progression consists of 3 consecutive phases:<\/p>\n\n\n\n<ol class=\"wp-block-list\"><li><strong>Osteolytic phase<\/strong> &#8212; Osteoclasts activate without control and break down the bone\n<ul><li>Histologic sections show giant-cell osteoclasts (100 or more nuclei)<\/li><\/ul>\n<\/li><li><strong>Mixed-phase<\/strong> &#8212; Osteoblasts activate in an attempt to reconstruct the bone\n<ul><li>They do so excessively and in a rush<\/li><\/ul>\n<\/li><li><strong>Osteosclerotic phase<\/strong> &#8212; Osteoclast &#8220;burnout&#8221;, osteoblast activity&nbsp;continues for some time afterward<\/li><\/ol>\n\n\n<\/span><span class=\"block-heading\" id=\"header_4\">\n<h4 class=\"wp-block-heading\" class=\"wp-block-heading\" class=\"title_collection title1\">Appearance and symptoms<\/h4>\n<\/span><span class=\"block-content\" id=\"contents_4\">\n\n\n<p class=\"wp-block-paragraph\">The bone will appear <strong>large<\/strong>, <strong>heavy<\/strong> and <strong>deformed<\/strong>, but more <strong>fragile<\/strong>.<\/p>\n\n\n\n<ul class=\"wp-block-list\"><li>Depending on the location of involvement, it may cause pain, movement restriction, and displacement of nearby soft tissue\n<ul><li>The pain is caused by microfractures or compression of spinal nerves<\/li><li>If it appears inside the skull base (<strong>platybasia<\/strong>) it may cause compression of the posterior fossa<\/li><\/ul>\n<\/li><li>The fragile bone tends to fracture more easily and damages nearby tissues (vertebral fracture -&gt; spinal cord injury)<\/li><\/ul>\n\n\n<\/span><span class=\"block-heading\" id=\"header_5\">\n<h4 class=\"wp-block-heading\" class=\"wp-block-heading\" class=\"title_collection title1\">Complications<\/h4>\n<\/span><span class=\"block-content\" id=\"contents_5\">\n\n\n<ul class=\"wp-block-list\"><li>Increased risk for tumor development &#8212; <strong>secondary osteosarcomas<\/strong><\/li><\/ul>\n\n\n<\/span><span class=\"block-heading\" id=\"header_6\">\n<h4 class=\"wp-block-heading\" class=\"wp-block-heading\" class=\"title_collection title1\">Treatment<\/h4>\n<\/span><span class=\"block-content\" id=\"contents_6\">\n\n\n<p class=\"wp-block-paragraph\">Although Padget&#8217;s disease is usually benign, treatments include administration of&nbsp;<strong>calcitonin<\/strong> and&nbsp;<strong>bisphosphonates&nbsp;<\/strong>to suppress&nbsp;osteoclast activity.<\/p>\n\n\n\n<p class=\"wp-block-paragraph\">In this topic you should also learn the <a href=\"https:\/\/meddists.com\/course\/pathology\/lessons\/hyperparathyroidism-3\/\">hyperparathyroidism chapter<\/a><\/p>\n<\/span><div id=\"the_titles\" style=\"display:none;\"><h4 class=\"wp-block-heading\" class=\"wp-block-heading\">Definition<\/h4><h4 class=\"wp-block-heading\" class=\"wp-block-heading\">Characteristics<\/h4><h4 class=\"wp-block-heading\" class=\"wp-block-heading\">Phases<\/h4><h4 class=\"wp-block-heading\" class=\"wp-block-heading\">Appearance and symptoms<\/h4><h4 class=\"wp-block-heading\" class=\"wp-block-heading\">Complications<\/h4><h4 class=\"wp-block-heading\" class=\"wp-block-heading\">Treatment<\/h4><\/div>","protected":false},"excerpt":{"rendered":"<p>Definition Disease of the bones involving bone breakdown followed by a disordered pattern of excessive bone growth. Characteristics Also known as osteitis deformans Late-onset&nbsp;(late adulthood) Initially, the bone breaks down, with excessive reconstruction later on Most cases involve multiple bones (polyostotic), while single bone involvement (monostotic) is more rare Does not involve the entire skeleton [&hellip;]<\/p>\n","protected":false},"author":1,"featured_media":0,"parent":6596,"menu_order":0,"comment_status":"closed","ping_status":"closed","template":"","meta":{"footnotes":""},"class_list":["post-625","page","type-page","status-publish","hentry"],"yoast_head":"<!-- This site is optimized with the Yoast SEO plugin v28.0 - https:\/\/yoast.com\/product\/yoast-seo-wordpress\/ -->\n<title>Paget&#039;s disease &#8211; Meddists<\/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:\/\/meddists.com\/learn\/pre-clinical\/pathology\/bone-pathology\/pagets-disease\/\" \/>\n<meta name=\"twitter:label1\" content=\"Est. reading time\" \/>\n\t<meta name=\"twitter:data1\" content=\"1 minute\" \/>\n<script type=\"application\/ld+json\" class=\"yoast-schema-graph\">{\"@context\":\"https:\\\/\\\/schema.org\",\"@graph\":[{\"@type\":\"WebPage\",\"@id\":\"https:\\\/\\\/meddists.com\\\/learn\\\/pre-clinical\\\/pathology\\\/bone-pathology\\\/pagets-disease\\\/\",\"url\":\"https:\\\/\\\/meddists.com\\\/learn\\\/pre-clinical\\\/pathology\\\/bone-pathology\\\/pagets-disease\\\/\",\"name\":\"Paget's disease &#8211; 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