{"id":9607,"date":"2022-01-19T15:16:41","date_gmt":"2022-01-19T15:16:41","guid":{"rendered":"https:\/\/medicalcriteria.com\/web\/?p=9607"},"modified":"2025-12-30T20:25:23","modified_gmt":"2025-12-30T20:25:23","slug":"wilson","status":"publish","type":"post","link":"https:\/\/medicalcriteria.com\/web\/es\/wilson\/","title":{"rendered":"Sistema de Puntuaci\u00f3n Diagn\u00f3stica para Enfermedad de Wilson"},"content":{"rendered":"<div class=\"7ca9444b8380138fe077da07353677a4\" data-index=\"1\" style=\"float: none; margin:0px 0 0px 0; text-align:center;\">\n<script async src=\"https:\/\/pagead2.googlesyndication.com\/pagead\/js\/adsbygoogle.js\"><\/script>\r\n<!-- MC 2019- Horizontal -->\r\n<ins class=\"adsbygoogle\"\r\n     style=\"display:block\"\r\n     data-ad-client=\"ca-pub-0127150553352455\"\r\n     data-ad-slot=\"3806776041\"\r\n     data-ad-format=\"auto\"\r\n     data-full-width-responsive=\"true\"><\/ins>\r\n<script>\r\n     (adsbygoogle = window.adsbygoogle || []).push({});\r\n<\/script>\n<\/div>\n<p>El consumo diet\u00e9tico normal y la absorci\u00f3n de cobre exceden las necesidades metab\u00f3licas, y la homeostasis de este elemento se mantiene exclusivamente por la excreci\u00f3n biliar de cobre. La enfermedad de Wilson es un trastorno hereditario en el que la excreci\u00f3n biliar defectuosa de cobre conduce a su acumulaci\u00f3n, particularmente en el h\u00edgado y el cerebro.<!--more--><\/p>\n<p><strong>Sistema de puntuaci\u00f3n desarrollado para la enfermedad de Wilson (Leipzig)<\/strong><\/p>\n<table style=\"border-collapse: collapse; width: 100%;\" border=\"1\">\n<tbody>\n<tr>\n<td style=\"width: 63.5741%;\"><strong>Presentaci\u00f3n cl\u00ednica y de laboratorio<\/strong><\/td>\n<td style=\"width: 36.4259%; text-align: center;\"><strong>Puntos<\/strong><\/td>\n<\/tr>\n<tr>\n<td style=\"width: 63.5741%;\">Anillos de Kayser\u2013Fleischer<\/p>\n<ul>\n<li>Presentes<\/li>\n<li>Ausentes<\/li>\n<\/ul>\n<\/td>\n<td style=\"width: 36.4259%; text-align: center;\">&nbsp;<\/p>\n<p>2<br \/>\n0<\/td>\n<\/tr>\n<tr>\n<td style=\"width: 63.5741%;\">S\u00edntomas neurol\u00f3gicos**<\/p>\n<ul>\n<li>Severos<\/li>\n<li>Leves<\/li>\n<li>Ausentes<\/li>\n<\/ul>\n<\/td>\n<td style=\"width: 36.4259%; text-align: center;\">&nbsp;<\/p>\n<p>2<br \/>\n1<br \/>\n0<\/td>\n<\/tr>\n<tr>\n<td style=\"width: 63.5741%;\">Ceruloplasmina s\u00e9rica<\/p>\n<ul>\n<li>Normal (&gt;0,2 g\/L)<\/li>\n<li>0,1-0,2 g\/L<\/li>\n<li>&lt;0,1 g\/L<\/li>\n<\/ul>\n<\/td>\n<td style=\"width: 36.4259%; text-align: center;\">&nbsp;<\/p>\n<p>0<br \/>\n1<br \/>\n2<\/td>\n<\/tr>\n<tr>\n<td style=\"width: 63.5741%;\">Anemia hemol\u00edtica Coombs-negativa<\/p>\n<ul>\n<li>Presente<\/li>\n<li>Ausente<\/li>\n<\/ul>\n<\/td>\n<td style=\"width: 36.4259%; text-align: center;\">&nbsp;<\/p>\n<p>1<br \/>\n0<\/td>\n<\/tr>\n<tr>\n<td style=\"width: 63.5741%;\">Cobre hep\u00e1tico (en ausencia de colestasis)<\/p>\n<ul>\n<li>&gt;5x LSN (&gt;4 \u03bcmol\/g)<\/li>\n<li>0,8-4 \u03bcmol\/g<\/li>\n<li>Normal (&lt;0,8 \u03bcmol\/g)<\/li>\n<li>Gr\u00e1nulos rodanina positivos*<\/li>\n<\/ul>\n<\/td>\n<td style=\"width: 36.4259%; text-align: center;\">&nbsp;<\/p>\n<p>2<br \/>\n1<br \/>\n-1<br \/>\n1<\/td>\n<\/tr>\n<tr>\n<td style=\"width: 63.5741%;\">Cobre urinario (en ausencia de hepatitis aguda)<\/p>\n<ul>\n<li>Normal<\/li>\n<li>1-2x LSN<\/li>\n<li>&gt;2x LSN<\/li>\n<li>Normal, pero &gt;5x LSN despu\u00e9s de D-penicilamina<\/li>\n<\/ul>\n<\/td>\n<td style=\"width: 36.4259%; text-align: center;\">&nbsp;<\/p>\n<p>0<br \/>\n1<br \/>\n2<br \/>\n2<\/td>\n<\/tr>\n<tr>\n<td style=\"width: 63.5741%;\">An\u00e1lisis de mutaci\u00f3n<\/p>\n<ul>\n<li>En ambos cromosomas detectados<\/li>\n<li>En 1 cromosoma detectado<\/li>\n<li>Sin mutaciones detectadas<\/li>\n<\/ul>\n<\/td>\n<td style=\"width: 36.4259%; text-align: center;\">&nbsp;<\/p>\n<p>4<br \/>\n1<br \/>\n0<\/td>\n<\/tr>\n<tr>\n<td style=\"width: 100%;\" colspan=\"2\">PUNTUACI\u00d3N TOTAL Evaluaci\u00f3n:<\/p>\n<ul>\n<li>4 o m\u00e1s Diagn\u00f3stico establecido<\/li>\n<li>3 Diagn\u00f3stico posible, se necesitan m\u00e1s pruebas<\/li>\n<li>2 o menos Diagn\u00f3stico muy poco probable<\/li>\n<\/ul>\n<\/td>\n<\/tr>\n<\/tbody>\n<\/table>\n<p>*Si no hay cobre hep\u00e1tico cuantitativo disponible, **o anomal\u00edas t\u00edpicas en la resonancia magn\u00e9tica cerebral. KF, Kayser\u2013Fleischer; LSN, l\u00edmite superior del normal.<\/p>\n<p>&nbsp;<\/p>\n<p><strong>Bibliograf\u00eda:<\/strong><\/p>\n<ol>\n<li>European Association for Study of Liver. EASL Clinical Practice Guidelines: Wilson&#8217;s disease. J Hepatol. 2012 Mar;56(3):671-85. <a href=\"https:\/\/pubmed.ncbi.nlm.nih.gov\/22340672\/\" target=\"_blank\" rel=\"noopener\">[Medline]<\/a><\/li>\n<li>Kasztelan-Szczerbinska B, Cichoz-Lach H. Wilson&#8217;s Disease: An Update on the Diagnostic Workup and Management. J Clin Med. 2021 Oct 30;10(21):5097. <a href=\"https:\/\/pubmed.ncbi.nlm.nih.gov\/34768617\/\" target=\"_blank\" rel=\"noopener\">[Medline]<\/a><\/li>\n<\/ol>\n<p>&nbsp;<\/p>\n<p>Creado Ene 17, 2022.<\/p>\n\n<div style=\"font-size: 0px; height: 0px; line-height: 0px; margin: 0; padding: 0; clear: both;\"><\/div>","protected":false},"excerpt":{"rendered":"<p>El consumo diet\u00e9tico normal y la absorci\u00f3n de cobre exceden las necesidades metab\u00f3licas, y la homeostasis de este elemento se mantiene exclusivamente por la excreci\u00f3n biliar de cobre. La enfermedad de Wilson es un trastorno hereditario en el que la excreci\u00f3n biliar defectuosa de cobre conduce a su acumulaci\u00f3n, particularmente en el h\u00edgado y el [&hellip;]<\/p>\n","protected":false},"author":2,"featured_media":0,"comment_status":"closed","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"_lmt_disableupdate":"no","_lmt_disable":"no","_exactmetrics_skip_tracking":false,"_exactmetrics_sitenote_active":false,"_exactmetrics_sitenote_note":"","_exactmetrics_sitenote_category":0,"footnotes":""},"categories":[53],"tags":[14,2466,51,50,445,794,494,507,646],"class_list":["post-9607","post","type-post","status-publish","format-standard","hentry","category-gastroenterology","tag-diagnostic","tag-diagnostica","tag-disease","tag-enfermedad","tag-puntuacion","tag-scoring","tag-sistema","tag-system","tag-wilson"],"modified_by":"Guillermo Firman","_links":{"self":[{"href":"https:\/\/medicalcriteria.com\/web\/es\/wp-json\/wp\/v2\/posts\/9607","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/medicalcriteria.com\/web\/es\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/medicalcriteria.com\/web\/es\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/medicalcriteria.com\/web\/es\/wp-json\/wp\/v2\/users\/2"}],"replies":[{"embeddable":true,"href":"https:\/\/medicalcriteria.com\/web\/es\/wp-json\/wp\/v2\/comments?post=9607"}],"version-history":[{"count":12,"href":"https:\/\/medicalcriteria.com\/web\/es\/wp-json\/wp\/v2\/posts\/9607\/revisions"}],"predecessor-version":[{"id":9624,"href":"https:\/\/medicalcriteria.com\/web\/es\/wp-json\/wp\/v2\/posts\/9607\/revisions\/9624"}],"wp:attachment":[{"href":"https:\/\/medicalcriteria.com\/web\/es\/wp-json\/wp\/v2\/media?parent=9607"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/medicalcriteria.com\/web\/es\/wp-json\/wp\/v2\/categories?post=9607"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/medicalcriteria.com\/web\/es\/wp-json\/wp\/v2\/tags?post=9607"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}