[et_pb_section fullwidth=”on” specialty=”off”][et_pb_fullwidth_slider admin_label=”Fullwidth Slider” show_arrows=”on” show_pagination=”on” auto=”off” parallax=”off” parallax_method=”off” module_id=”interior”][et_pb_slide background_image=”https:\/\/depts.washington.edu\/cfrtc\/wp-content\/uploads\/2014\/12\/microscope11.jpg” background_color=”#ffffff” alignment=”center” background_layout=”dark” \/][\/et_pb_fullwidth_slider][\/et_pb_section][et_pb_section][et_pb_row][et_pb_column type=”1_4″][et_pb_sidebar admin_label=”Pilots Sidebar” orientation=”left” area=”et_pb_widget_area_7″ background_layout=”light” \/][\/et_pb_column][et_pb_column type=”3_4″][et_pb_text admin_label=”Text” background_layout=”light” text_orientation=”left”]<\/p>\n

Testing association between epithelial chloride transport and measures of clinical improvement<\/h1>\n

P.I.:<\/strong>\u00a0Dave Nichols, MD
Associate Professor,
Pediatrics<\/p>\n

Jason Debley, MD, MPH
Associate Professor,
Pediatrics<\/p>\n

Drug therapies to enhance CFTR function (\u201cmodulators\u201d) are an important therapeutic step in CF care and research. Highly effective modulator therapy is increasingly available to people with CF but individual responses to these medications can be highly variable. Furthermore, many patients have rare, poor characterized mutations and their response to modulator drugs cannot be predicted. In vitro assessment of improved ion transport after exposure to modulator compounds holds promise to help address these questions related to heterogeneity or unpredictability of clinical response. Subject cohorts in Ireland with G551D or R117H mutations have and continue to be carefully phenotyped and characterized after initiation of ivacaftor. We propose to obtain and test the in vitro response to ivacaftor in primary nasal airway epithelial cells. We will then test for association between the in vitro improvements in ion transport and clinical outcomes measured in these subjects.<\/p>\n

\u00a0<\/p>\n

[\/et_pb_text][\/et_pb_column][\/et_pb_row][\/et_pb_section]<\/p>\n","protected":false},"excerpt":{"rendered":"

Pilot 12: Identification of Biomarkers for Cystic Fibrosis Associated Nephrotoxicity<\/p>\n

P.I.: \tNeal Paragas, PhD
\n\tResearch Assistant Professor,
\n\tMedicine (Nephrology)<\/p>\n","protected":false},"author":1,"featured_media":0,"comment_status":"closed","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"_et_pb_use_builder":"on","_et_pb_old_content":"

P.I.: Steven Kahn, MB, ChB<\/a>
Professor of Medicine
Metabolism, Endocrinology and Nutrition<\/p>

Kristina Utzschneider, MD<\/a>
Associate Professor of Medicine
Metabolism, Endocrinology and Nutrition<\/p>

With the increased life expectancy of patients with cystic fibrosis (CF), other co-morbidities have become apparent in these patients. One of these is abnormal glucose metabolism, where CF-related diabetes (CFRD) is common. More recently, another abnormality of glucose metabolism has been recognized; namely late hypoglycemia following oral glucose loading. In this study, we propose to test the hypothesis that the post-glucose load hypoglycemia observed in patients with CF results from a deficient counterregulatory hormone response and\/or an insulin response that is exaggerated and delayed. This increased insulin response could be the result of an exaggerated incretin hormone response or altered gastric emptying. To address this hypothesis, we will perform a 3-hour oral glucose tolerance test during which we will measure counterregulatory, islet and incretin hormone responses and determine the rate of gastric emptying using acetaminophen. To determine whether patients with CF and late hypoglycemia also have episodes of hypoglycemia during daily living that includes mixed meals, we will use a continuous glucose monitoring system (CGMS) to examine 24-hour glucose profiles for 3 days. All these measures will be compared between patients with CF who develop late hypoglycemia, CF patients who do not develop hypoglycemia, and age and body mass index-matched healthy controls. The findings from this study will provide important new information regarding the mechanism(s) responsible for the late hypoglycemia observed in patients with CF and the data could be used as the basis for future grant applications. The ultimate goal is to gain insight into the condition of late hypoglycemia in order to better manage patients with CF.<\/p>","_et_gb_content_width":"","_monsterinsights_skip_tracking":false,"_monsterinsights_sitenote_active":false,"_monsterinsights_sitenote_note":"","_monsterinsights_sitenote_category":0,"footnotes":"","_links_to":"","_links_to_target":""},"categories":[12],"tags":[],"_links":{"self":[{"href":"https:\/\/depts.washington.edu\/cfrtc\/wp-json\/wp\/v2\/posts\/1327"}],"collection":[{"href":"https:\/\/depts.washington.edu\/cfrtc\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/depts.washington.edu\/cfrtc\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/depts.washington.edu\/cfrtc\/wp-json\/wp\/v2\/users\/1"}],"replies":[{"embeddable":true,"href":"https:\/\/depts.washington.edu\/cfrtc\/wp-json\/wp\/v2\/comments?post=1327"}],"version-history":[{"count":1,"href":"https:\/\/depts.washington.edu\/cfrtc\/wp-json\/wp\/v2\/posts\/1327\/revisions"}],"predecessor-version":[{"id":1328,"href":"https:\/\/depts.washington.edu\/cfrtc\/wp-json\/wp\/v2\/posts\/1327\/revisions\/1328"}],"wp:attachment":[{"href":"https:\/\/depts.washington.edu\/cfrtc\/wp-json\/wp\/v2\/media?parent=1327"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/depts.washington.edu\/cfrtc\/wp-json\/wp\/v2\/categories?post=1327"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/depts.washington.edu\/cfrtc\/wp-json\/wp\/v2\/tags?post=1327"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}