{"id":2678,"date":"2010-02-10T20:48:02","date_gmt":"2010-02-11T01:48:02","guid":{"rendered":"http:\/\/insidesurgery.com\/?p=2678"},"modified":"2013-08-16T00:34:44","modified_gmt":"2013-08-16T04:34:44","slug":"peutzjeghers-syndrome","status":"publish","type":"post","link":"https:\/\/insidesurgery.com\/2010\/02\/peutzjeghers-syndrome\/","title":{"rendered":"Peutz-Jeghers Syndrome"},"content":{"rendered":"<p><strong>Pathophysiology<\/strong><\/p>\n<p><strong>1)<\/strong> inherited disorder marked by numerous GI polyps (usually in small bowel but occasionally from stomach to rectum) and mucocutaneous pigmentation <strong>2)<\/strong> heavy freckles on the face, hands, feet, and perineal areas usually fade at puberty but the buccal (mouth) pigmented spots do not <strong>3)<\/strong> although the polyps are considered benign, 2-3% of patients develop adenocarcinoma in the small bowel<\/p>\n<p><strong>Signs and Symptoms<\/strong><\/p>\n<p><strong>1)<\/strong> abdominal obstruction <strong>2)<\/strong> intussusception<\/p>\n<p><strong>Histology\/Gross Pathology<\/strong><\/p>\n<p>polyps are hamartomas with smooth muscle and intestinal glands<br \/>\n<strong><br \/>\nAssociated Conditions<\/strong><\/p>\n<p><em>increased risk of<\/em> &#8211; <strong>1)<\/strong> pancreatic cancer <strong>2)<\/strong> breast cancer <strong>3)<\/strong> ovarian cancer <strong>4)<\/strong> endometrial cancer <strong>5)<\/strong> testicular cancer<\/p>\n<p><strong>Biochemistry<\/strong><\/p>\n<p>affected gene codes for serine threonine kinase<\/p>\n<p><strong>Inheritance\/Epidemiology<\/strong><\/p>\n<p><strong>1)<\/strong> autosomal dominant <strong>2)<\/strong> affected gene is LKB1 on chromosome 19<\/p>\n<p><strong>Treatment<\/strong><\/p>\n<p>close surveillance for development of adenocarcinoma<\/p>\n<p><strong>Tips for USMLE<\/strong><\/p>\n<p><strong>1)<\/strong> if question mentions a 10 year-old boy with heavy freckles, including inside his mouth, and his freckles do not fade with age, think Peutz-Jeghers <strong>2)<\/strong> the other common disease with pigmented intraoral lesions is Addison&#8217;s disease<\/p>\n<p><strong>Related Posts<\/strong><\/p>\n<p><a href=\"http:\/\/insidesurgery.com\/2010\/02\/turcots-syndrome\/\">Turcot&#8217;s Syndrome<\/a><\/p>\n","protected":false},"excerpt":{"rendered":"<p>Pathophysiology 1) inherited disorder marked by numerous GI polyps (usually in small bowel but occasionally from stomach to rectum) and mucocutaneous pigmentation 2) heavy freckles on the face, hands, feet, and perineal areas usually fade at puberty but the buccal (mouth) pigmented spots do not 3) although the polyps are considered benign, 2-3% of patients [&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":[5151,5372,63],"tags":[1217,1218,1216,1220,1221,1214,1219,1215,1213],"yoast_head":"<!-- This site is optimized with the Yoast SEO plugin v22.6 - 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