{"id":175,"date":"2018-04-23T11:51:48","date_gmt":"2018-04-23T11:51:48","guid":{"rendered":"http:\/\/edren.org\/ren\/?page_id=175"},"modified":"2024-12-04T15:22:30","modified_gmt":"2024-12-04T15:22:30","slug":"pkd","status":"publish","type":"page","link":"https:\/\/edren.org\/ren\/handbook\/unithdbk\/individual-kidney-diseases\/pkd\/","title":{"rendered":"PKD"},"content":{"rendered":"<h3><strong><span style=\"color: #993300;\">PKD and the use of Tolvaptan<\/span><\/strong><\/h3>\n<p>This page is mainly aimed at staff. We have additional information for patients and others at <a href=\"http:\/\/edren.org\/ren\/edren-info\/polycystic-kidney-disease-pkd\/got-pkd\/\"><strong>edren.org\/gotpkd<\/strong><\/a>, and other <a href=\"http:\/\/edren.org\/ren\/handbook\/unithdbk\/pkd\/#Further_info\">further info<\/a>.\u00a0 We also have a page on <a href=\"https:\/\/edren.org\/ren\/handbook\/unithdbk\/ckd\/renal-cysts-how-to-evaluate\/\">how to evaluate patients<\/a> who are found to have kidney cysts (which may be simple, malignant or due to an inherited cystic disease).<\/p>\n<h3><strong><span style=\"color: #993300;\">Tolvaptan<\/span><\/strong><\/h3>\n<p>Tolvaptan is an ADH receptor antagonist. It has been shown to reduce rate of cyst growth and GFR\u00a0decline in PKD, and since late 2015 is licensed for use in the UK and other countries for patients with PKD\u00a0who are at high risk of progression. Our criteria for this, and other pathway details, are subject to change.<\/p>\n<p>Assessing risk of progression is based primarily on:<\/p>\n<ul style=\"list-style-type: disc;\">\n<li><strong><span style=\"color: #993300;\">GFR\u00a0decline<\/span><\/strong> &#8211; though this is a late sign, it often occurs over a decade or more.\u00a0 Where you&#8217;re sure that PKD is the cause (very big kidneys with no other explanation)\u00a0the criteria are (eGFR\u00a0in ml\/min\/1.73m2)\n<ul style=\"list-style-type: disc;\">\n<li>A fall in eGFR\u00a0of 5 or more over 6-12 months (confirmed by more than one test, ensuring no other explanation)<\/li>\n<li>A fall in eGFR of 2.5 or more per year over a longer time period<\/li>\n<\/ul>\n<\/li>\n<li><strong><span style=\"color: #993300;\">Kidney volume<\/span><\/strong> normalized for height (HtTKV), matched to age. Bigger kidneys in younger people are more likely to lead to loss of kidney function. <a href=\"#Protocol\">Estimating kidney volume<\/a> is described below. With this criterion it is hoped to identify and treat patients at high risk before their GFR falls. \u00a0But it poses the question of how to identify and screen all who may be in this group.<\/li>\n<\/ul>\n<ul>\n<li><strong><span style=\"color: #993300;\">Other factors<\/span><\/strong>, including genetic information, family history of early ESRF, early onset of symptoms or hypertension, easily palpable kidneys in young people, are useful in drawing attention to the need to estimate GFR and kidney size.<\/li>\n<\/ul>\n<h3><strong><span style=\"color: #993300;\">Protocol<\/span><\/strong><\/h3>\n<p><a id=\"Protocol\"><\/a>March 2016 (additional detail in protocol document at foot of page)<\/p>\n<p style=\"text-align: center;\"><a href=\"http:\/\/edren.org\/ren\/wp-content\/uploads\/2018\/10\/PKD_algorithm_d.gif\"><img loading=\"lazy\" decoding=\"async\" class=\"alignnone size-full wp-image-1903\" src=\"http:\/\/edren.org\/ren\/wp-content\/uploads\/2018\/10\/PKD_algorithm_d.gif\" alt=\"\" width=\"710\" height=\"838\" \/><\/a><\/p>\n<p style=\"text-align: center;\">* see above or below:\u00a0 <a href=\"#Protocol\">Other risk factors<\/a> &#8211; <a href=\"#Volume\">imaging uncertainty<\/a> &#8211; tolvaptan exclusion criteria<\/p>\n<p>Note that several criteria cited here are experimental, including ultrasound sizes to request MRI, and re-scan intervals. Progress will be monitored and recommendations modified accordingly. Equally it may be justifiable to vary these criteria in particular circumstances.<\/p>\n<h3><strong><span style=\"color: #993300;\">Estimating kidney volume<\/span><\/strong><\/h3>\n<p>Kidney volume can be a useful predictor of risk, IF kidneys are reasonably evenly enlarged by many small to medium sized cysts* and there are not other important factors operating.\u00a0 You need measurements of subject height as well as kidney dimensions by MRI\u00a0or CT &#8211; enter these into the online TKV calculator (we use <a href=\"https:\/\/www.mayo.edu\/research\/documents\/pkd-center-adpkd-classification\/doc-20094754\">this one<\/a> from the Mayo Clinic PKD center). Mayo 1C, 1D and 1E are deemed higher risk of losing kidney function as per the study by <a href=\"https:\/\/journals.lww.com\/jasn\/abstract\/2015\/01000\/imaging_classification_of_autosomal_dominant.19.aspx\">Izabal et al<\/a>.<\/p>\n<p><a href=\"https:\/\/edren.org\/ren\/wp-content\/uploads\/2024\/12\/HtTKV-1.png\"><img loading=\"lazy\" decoding=\"async\" class=\"alignnone wp-image-6569\" src=\"https:\/\/edren.org\/ren\/wp-content\/uploads\/2024\/12\/HtTKV-1-300x119.png\" alt=\"\" width=\"654\" height=\"260\" srcset=\"https:\/\/edren.org\/ren\/wp-content\/uploads\/2024\/12\/HtTKV-1-300x119.png 300w, https:\/\/edren.org\/ren\/wp-content\/uploads\/2024\/12\/HtTKV-1-1024x406.png 1024w, https:\/\/edren.org\/ren\/wp-content\/uploads\/2024\/12\/HtTKV-1-768x305.png 768w, https:\/\/edren.org\/ren\/wp-content\/uploads\/2024\/12\/HtTKV-1-150x59.png 150w, https:\/\/edren.org\/ren\/wp-content\/uploads\/2024\/12\/HtTKV-1.png 1029w\" sizes=\"auto, (max-width: 654px) 100vw, 654px\" \/><\/a><\/p>\n<p>Click on image for better resolution.<\/p>\n<p>* Kidneys should be diffusely enlarged by many cysts &#8216;where all cysts contribute similarly to TKV&#8217; &#8211; in other words, a few giant cysts shouldn&#8217;t make a substantial component, and there shouldn&#8217;t be substantial areas without cysts, or atrophied. \u00a0But it is important to note that even when conditions are optimal, estimations of risk made using these techniques are likely to give imprecise estimates of risk for individuals. Actual progression may be much shorter or much longer, depending on cofactors that we may only partially understand, or currently be unaware of.<\/p>\n<h3><strong><span style=\"color: #993300;\">Exclusions to taking tolvaptan<\/span><\/strong><\/h3>\n<ul style=\"list-style-type: disc;\">\n<li>GFR &lt;30ml\/min\/1.73m2 is an exclusion to <em>commencing<\/em> therapy in current EMA, NICE, SMC guidance. \u00a0(In England only, NICE guidance also states that eGFR&gt;90 is an exclusion). However if decline rate is very slow, you could still usefully lengthen time to ESRF.<\/li>\n<li>&lt;18 years old<\/li>\n<li>Pregnancy<\/li>\n<li>Requirement for diuretics is a relative contraindication; use caution<\/li>\n<li>Not able to drink freely<\/li>\n<li>High risk of AKI\u00a0from other etiologies. ACEi therapy is not a contraindication in healthy patient who can follow sick day rules<\/li>\n<li>Liver disease<\/li>\n<li>Not willing to comply with monitoring.<\/li>\n<\/ul>\n<h3><strong><span style=\"color: #993300;\">Other aspects of management<\/span><\/strong><\/h3>\n<p>We recommend adding a statin in high-risk patients. Atorvastatin 20mg daily is the NICE-recommended therapy for CKD. Other issues are considered in the short Edinburgh protocol that is downloadable at the foot of this page.<\/p>\n<h3><strong><span style=\"color: #993300;\">Further info<\/span><\/strong><\/h3>\n<ul style=\"list-style-type: disc;\">\n<li><a href=\"http:\/\/edren.org\/ren\/edren-info\/polycystic-kidney-disease-pkd\/got-pkd\/\">Got PKD?<\/a>\u00a0&#8211; landing page for patients, GPs and others seeking info<\/li>\n<li><a href=\"http:\/\/edren.org\/ren\/edren-info\/polycystic-kidney-disease-pkd\/\">PKD<\/a> \u2013 advanced info for patients \u2013 including about Tolvaptan (Edren Info)<\/li>\n<li><a href=\"http:\/\/www.edrep.org\/pages\/textbook\/inheritedcongenital.php\">Inherited kidney diseases<\/a> \u2013 very concise for students (Edren textbook)<\/li>\n<li><a href=\"http:\/\/radiopaedia.org\/articles\/autosomal-dominant-polycystic-kidney-disease-1\">PKD on Radiopaedia<\/a> \u2013 excellent images, good background info, plus links to browse<\/li>\n<li><a href=\"http:\/\/www.edrep.org\/clinic\/2016\/02\/a-patient-with-big-kidneys\/\">A patient with big kidneys<\/a> (Virtual Renal Clinic) &#8211; take this simple test case<\/li>\n<\/ul>\n<p>&nbsp;<\/p>\n","protected":false},"excerpt":{"rendered":"<p>PKD and the use of Tolvaptan This page is mainly aimed at staff. We have additional information for patients and others at edren.org\/gotpkd, and other further info.\u00a0 We also have a page on how to evaluate patients who are found to have kidney cysts (which may be simple, malignant or\u2026<\/p>\n<p> <a class=\"continue-reading-link\" href=\"https:\/\/edren.org\/ren\/handbook\/unithdbk\/individual-kidney-diseases\/pkd\/\"><span>Continue reading<\/span><i class=\"crycon-right-dir\"><\/i><\/a> <\/p>\n","protected":false},"author":2,"featured_media":0,"parent":5392,"menu_order":0,"comment_status":"closed","ping_status":"closed","template":"","meta":{"_lmt_disableupdate":"no","_lmt_disable":"","footnotes":""},"class_list":["post-175","page","type-page","status-publish","hentry"],"_links":{"self":[{"href":"https:\/\/edren.org\/ren\/wp-json\/wp\/v2\/pages\/175","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/edren.org\/ren\/wp-json\/wp\/v2\/pages"}],"about":[{"href":"https:\/\/edren.org\/ren\/wp-json\/wp\/v2\/types\/page"}],"author":[{"embeddable":true,"href":"https:\/\/edren.org\/ren\/wp-json\/wp\/v2\/users\/2"}],"replies":[{"embeddable":true,"href":"https:\/\/edren.org\/ren\/wp-json\/wp\/v2\/comments?post=175"}],"version-history":[{"count":30,"href":"https:\/\/edren.org\/ren\/wp-json\/wp\/v2\/pages\/175\/revisions"}],"predecessor-version":[{"id":6572,"href":"https:\/\/edren.org\/ren\/wp-json\/wp\/v2\/pages\/175\/revisions\/6572"}],"up":[{"embeddable":true,"href":"https:\/\/edren.org\/ren\/wp-json\/wp\/v2\/pages\/5392"}],"wp:attachment":[{"href":"https:\/\/edren.org\/ren\/wp-json\/wp\/v2\/media?parent=175"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}