Development of an inflammatory bowel disease (IBD) Patient-Reported Experience Measure (PREM): A patient-led consensus work and 'think aloud' study for a quality improvement programme.

BACKGROUND: Patient-Reported Experience Measures (PREMs) are key in improving healthcare quality, but no PREM exists for inflammatory bowel disease (IBD). This study aimed to co-produce a PREM with IBD service users for IBD service evaluation and quality improvement programme. METHODS: A pool of 75 items was drawn from published survey instruments covering interactions with services and aspects of living with IBD. In Stage 1, during two workshops, eight expert service users reduced candidate items through a ranked-choice voting exercise and suggested further items. During Stage 2, 18 previously uninvolved people with IBD assessed the face and content validity of the candidate items in 'Think Aloud' interviews. During two final workshops (Stage 3), the expert service users removed, modified and added items based on the interview findings to produce a final version of the PREM. RESULTS: Stage 1 generated a draft working PREM mapped to the following four domains: Patient-Centred Care; Quality; Accessibility; Communication and Involvement. The PREM included a set of nine items created by the expert group which shifted the emphasis from 'self-management' to 'living with IBD'. Stage 2 interviews showed that comprehension of the PREM was very good, although there were concerns about the wording, IBD-relevance and ambiguity of some items. During the final two workshops in Stage 3, the expert service users removed 7 items, modified 15 items and added seven new ones based on the interview findings, resulting in a 38-item PREM. CONCLUSIONS: This study demonstrates how extensive service user involvement can inform PREM development. PATIENT OR PUBLIC CONTRIBUTION: Patients were involved as active members of the research team and as research participants to co-produce and validate a PREM for IBD services. In Stage 1, eight expert service users ('the expert group') reduced candidate items for the PREM through a voting exercise and suggested new items. During Stage 2, 18 previously uninvolved people with IBD (the 'think aloud' participants) assessed the validity of the candidate items in 'Think Aloud' interviews as research participants. In Stage 3, the expert group removed, changed and added items based on the interview findings to produce a final version of the 38-item PREM. This study shows how service user involvement can meaningfully inform PREM development.

Ten-Year Analysis of Bacterial Colonisation and Outcomes of Major Burn Patients with a Focus on Pseudomonas aeruginosa.

A retrospective descriptive study included patients admitted with severe burns over the course of 10 years (2008-2018). Across all patients, there were 39 different species of bacteria, with 23 species being Gram-negative and 16 being Gram-positive bacteria, with also five different species of fungi cultured. Pseudomonas aeruginosa was the most commonly isolated organism, with 57.45% of patients having a positive culture. There was a significant difference in the number of P. aeruginosa isolated from patients that acquired their burns at work, in a garden, inside a vehicle, in a garage or in a public place. In patients that were positive for P. aeruginosa, the number of operations was higher (2.4) and the length of stay was significantly increased (80.1 days). Patients that suffered from substance abuse demonstrated significantly higher numbers of isolated P. aeruginosa (14.8%). Patients that suffered from both mental health illness and substance abuse demonstrated significantly higher numbers of P. aeruginosa isolated (18.5%). In the P. aeruginosa-negative group, there were significantly fewer patients that had been involved in a clothing fire. Furthermore, in the P. aeruginosa-negative patient cohort, the mortality rate was significantly higher (p = 0.002). Since the incidence of P. aeruginosa was also associated with a decreased mortality rate, it may be that patients admitted to hospital for shorter periods of time were less likely to be colonised with P. aeruginosa. This study demonstrates novel factors that may increase the incidence of P. aeruginosa isolated from burn patients.

Photodynamic antimicrobial chemotherapy coupled with the use of the photosensitizers methylene blue and temoporfin as a potential novel treatment for Staphylococcus aureus in burn infections.

Photodynamic antimicrobial chemotherapy (PACT) is a novel alternative antimicrobial therapy that elicits a broad mechanism of action and therefore has a low probability of generating resistance. Such properties make PACT ideally suited for utilization in localized applications such as burn wounds. The aim of this study was to determine the antimicrobial activity of MB and temoporfin against both a S. aureus isolate and a P. aeruginosa isolate in light (640 nm) and dark conditions at a range of time points (0-20 min). A Staphylococcus aureus isolate and a Pseudomonas aeruginosa isolate were treated in vitro with methylene blue (MB) and temoporfin under different conditions following exposure to light at 640 nm and in no-light (dark) conditions. Bacterial cell viability [colony-forming units (c.f.u.) ml-1] was then calculated. Against P. aeruginosa , when MB was used as the photosensitizer, no phototoxic effect was observed in either light or dark conditions. After treatment with temoporfin, a reduction of less than one log (7.00×107 c.f.u. ml-1) was observed in the light after 20 min of exposure. However, temoporfin completely eradicated S. aureus in both light and dark conditions after 1 min (where a seven log reduction in c.f.u. ml-1 was observed). Methylene blue resulted in a loss of S. aureus viability, with a two log reduction in bacterial viability (c.f.u. ml-1) reported in both light and dark conditions after 20 min exposure time. Temoporfin demonstrated greater antimicrobial efficacy than MB against both the S. aureus and P. aeruginosa isolates tested. At 12.5 µM temoporfin resulted in complete eradication of S. aureus . In light of this study, further research into the validity of PACT, coupled with the photosensitizers (such as temoporfin), should be conducted in order to potentially develop alternative antimicrobial treatment regimes for burn wounds.

The association of blood pressure variability with adverse outcomes in a primary care chronic kidney disease cohort.

BACKGROUND: Hypertension is common in individuals with chronic kidney disease and both conditions are associated with adverse outcomes including cardiovascular morbidity. Therefore, it is clinically important to identify methods of risk prediction in individuals with chronic kidney disease. Blood pressure variability has recently emerged as a predictor of cardiovascular events and mortality in the general population, with growing evidence indicating that it may play a similar role in individuals with chronic kidney disease. However, there have been no large studies assessing blood pressure variability in individuals with chronic kidney disease in primary care, where the majority of these patients are managed. METHOD: Using a retrospective observational study design, we analyzed routinely collected blood pressure readings from 16 999 individuals in The Leicester and County Chronic Kidney Disease cohort. Standard deviation, coefficient of variation and average real variability of SBP were used to calculate blood pressure variability. RESULTS: During a median follow-up of 5.0 (IQR 3.3--5.0) years, 2053 (12.1%) patients had cardiovascular events, death occurred in 5021 (29.6%) individuals and 156 (0.9%) individuals had endstage kidney disease events. In adjusted models, standard deviation and coefficient of variation were associated with cardiovascular events, all-cause mortality and endstage kidney disease. Average real variability was associated with all-cause mortality and cardiovascular events, but not endstage kidney disease. CONCLUSION: Blood pressure variability may be an accessible, routinely collected, noninvasive measure for stratifying the risk of adverse events in individuals with chronic kidney disease in a primary care setting.

CMV pneumonitis in a patient with Crohn's disease taking azathioprine.

This case report discusses the rare presentation of cytomegalovirus (CMV) pneumonitis in a young patient with moderately severe Crohn's disease managed with low dose azathioprine. CMV pneumonitis was initially suspected on CT chest images and confirmed by PCR for CMV. She was treated with intravenous ganciclovir and later stepped down to oral valganciclovir. Although this patient had a prolonged and complicated hospital admission, a good clinical outcome was achieved. CMV infection was raised as an early differential and antiviral treatment was started without delay. This case study, therefore, makes the case for increased awareness of the possibility of, and recognition of CMV pneumonitis among healthcare professionals as a way of preventing significant morbidity and mortality. It also raises awareness of checking for slow metabolisers of azathioprine before initiation to look for individuals who may be at increased risk of azathioprine's adverse effects.

Expectations About Satiety and Thirst Are Modified by Acute Motivational State.

Prior research has shown that consumers have clear and measurable expectations about the likely effects of food and drink items on their appetite and thirst, which are acquired with experience and influenced by a product's taste and texture. What is unclear is whether expression of these expectations also varies with current appetitive state. It is possible that current appetite could increase or decrease the relevance of these expectations for future food choice and magnify a product's expected impact on appetite. To test this, we contrasted expectations about satiety and thirst for four products consumed 2 h after an appetite manipulation at breakfast, achieved through ad libitum access to low-energy drinks only (hunger condition), cereal only but no drinks (thirst condition) or both foods and drinks (sated condition). The test products were two soups and two drinks, with a thicker and thinner version of each product type to act as positive control to ensure sensitivity in detecting differences in expectations. For satiety, the predicted differences between products were seen: soups and thicker products were expected to be more filling and to suppress subsequent hunger more than drinks and thinner products, but these differences were more pronounced in the hunger than thirsty or sated conditions. Being thirsty also enhanced expectations of how much drinks would appease immediate thirst. Overall the data show that expectations were adjusted subtly by a person's current appetitive state, suggesting that we have mechanisms that highlight the most important features of a product at the time when it may be most beneficial to the consumer.

Additive effects of sensory-enhanced satiety and memory for recent eating on appetite.

The sensory characteristics of a product have been shown to interact with actual nutrient content to generate satiety. Separately, cued recall of recent eating has also been shown to reduce food intake. Here we explore for the first time how these two effects interact, with the hypothesis that sensory enhancement of satiety might be mediated by more vivid memory of the earlier consumed item. On each of two test sessions, 119 women volunteers consumed a control drink (lemonade) on one morning and then one of two test drinks on the next day 30 min before an ad libitum lunch. The test drinks were equicaloric but one was noticeably thicker and creamier, and expected to generate stronger satiety. Just prior to the test lunch, participants were asked to recall either the test drink (test recall) or the drink from the previous day (control recall). Overall, lunch intake was significantly lower after the thicker and creamier (enhanced sensory ES) than thinner (low sensory: LS) test drink (p < 0.001, η2 = 0.11) regardless of recall condition (p = 0.65, η2 < 0.01), but was significantly lower after the test than control recall condition (p < 0.001, η2 = 0.14). Rated hunger was lower after consuming the ES than LS drink both immediately after consumption (p < 0.001, η2 = 0.11) and prior to the test lunch (p = 0.007, η2 = 0.06), while rated hunger just before lunch tended to be lower after recalling the test than control drink (p = 0.052, η2 = 0.03) regardless of the sensory characteristics (p = 0.27, η2 = 0.01). Overall these data further demonstrate the power of 'sensory-enhanced satiety' and cued recall of earlier eating as methods to reduce acute food intake, but suggest these effects operate independently.

Satiety Impact of Different Potato Products Compared to Pasta Control.

OBJECTIVE: A variety of potato dishes are regularly consumed worldwide, but the satiety value of these foods is not well established. The primary objective of this study was to compare the satiating effects of 4 equi-energy meals containing different potato preparations with an equi-energy pasta control meal. METHODS: This study used a randomized crossover design to assess the impact of 4 equi-energy potato-based meals (fried French fries, baked potato, mashed potato, or potato wedges) on subjective satiety sensations (visual analogue scale [VAS] ratings) and subsequent energy intake (ad libitum meal [kcal]), compared to a control pasta-based meal. Thirty-three healthy nonobese men and women participated in the study. RESULTS: VAS ratings indicated that the meal containing fried french fries was perceived to be substantially more satiating than the equi-energy pasta control meal, with all other potato-based meals not differing overall from control. All test meals had a comparable effect on energy intake at a later ad libitum meal. CONCLUSIONS: Consumers reported higher levels of satiety following a meal where the principal carbohydrate source was fried french fries, compared to when they had consumed an energy-matched meal containing carbohydrate in the form of pasta. All other potato preparations had similar effects on satiety as pasta. It is concluded that participants perceived a meal with fried french fries as providing greater satiety than a pasta control meal.

A mid-morning snack of almonds generates satiety and appropriate adjustment of subsequent food intake in healthy women.

PURPOSE: To assess the effect of consuming a mid-morning almond snack (28 and 42 g) tested against a negative control of no almonds on acute satiety responses. METHOD: On three test days, 32 healthy females consumed a standard breakfast followed by 0, 28 or 42 g of almonds as a mid-morning snack and then ad libitum meals at lunch and dinner. The effect of the almond snacks on satiety was assessed by measuring energy intake (kcal) at the two ad libitum meals and subjective appetite ratings (visual analogue scales) throughout the test days. RESULTS: Intake at lunch and dinner significantly decreased in a dose-dependent manner in response to the almond snacks. Overall, a similar amount of energy was consumed on all three test days indicating that participants compensated for the 173 and 259 kcals consumed as almonds on the 28 and 42 g test days, respectively. Subjective appetite ratings in the interval between the mid-morning snack and lunch were consistent with dose-dependent enhanced satiety following the almond snacks. However, in the interval between lunch and dinner, appetite ratings were not dependent on the mid-morning snack. CONCLUSION: Almonds might be a healthy snack option since their acute satiating effects are likely to result in no net increase in energy consumed over a day.

Fluid or fuel? The context of consuming a beverage is important for satiety.

Energy-containing beverages have a weak effect on satiety, limited by their fluid characteristics and perhaps because they are not considered 'food'. This study investigated whether the context of consuming a beverage can influence the satiating power of its nutrients. Eighty participants consumed a lower- (LE, 75 kcal) and higher-energy (HE, 272 kcal) version of a beverage (covertly manipulated within-groups) on two test days, in one of four beverage contexts (between-groups): thin versions of the test-drinks were consumed as a thirst-quenching drink (n = 20), a filling snack (n = 20), or without additional information (n = 20). A fourth group consumed subtly thicker versions of the beverages without additional information (n = 20). Lunch intake 60 minutes later depended on the beverage context and energy content (p = 0.030): participants who consumed the thin beverages without additional information ate a similar amount of lunch after the LE and HE versions (LE = 475 kcal, HE = 464 kcal; p = 0.690) as did those participants who believed the beverages were designed to quench-thirst (LE = 442 kcal, HE = 402 kcal; p = 0.213), despite consuming an additional 197 kcal in the HE beverage. Consuming the beverage as a filling snack led participants to consume less at lunch after the HE beverage compared to the LE version (LE = 506 kcal, HE = 437 kcal; p = 0.025). This effect was also seen when the beverages were subtly thicker, with participants in this group displaying the largest response to the beverage's energy content, consuming less at lunch after the HE version (LE = 552 kcal, HE = 415 kcal; p<0.001). These data indicate that beliefs about the consequences of consuming a beverage can affect the impact of its nutrients on appetite regulation and provide further evidence that a beverage's sensory characteristics can limit its satiating power.

Effects of repeated consumption on sensory-enhanced satiety.

Previous research has suggested that sensory characteristics of a drink modify the acute satiating effects of its nutrients, with enhanced satiety being evident when a high-energy drink was thicker and tasted creamier. The present study tested whether this modulation of satiety by sensory context was altered by repeated consumption. Participants (n 48) consumed one of four drinks mid-morning on seven non-consecutive days, with satiety responses being measured pre-exposure (day 1), post-exposure (day 6) and at a 1-month follow-up. The drinks combined two levels of energy (lower energy (LE), 326 kJ and higher energy, 1163 kJ) with two levels of satiety-predictive sensory characteristics (low sensory (LS) or enhanced sensory). Test lunch intake 90 min after drink consumption depended on both the energy content and sensory characteristics of the drink before exposure, but on the energy content alone after exposure and at the follow-up. The largest change was an increase in test meal intake over time in the LE/LS condition. The effects on intake were reflected in appetite ratings, with rated hunger and expected filling affected by sensory characteristics and energy content pre-exposure, but were largely determined by energy content post-exposure and at the follow-up. In contrast, a measure of expected satiety reflected sensory characteristics regardless of energy content on all the three test days. Overall, these data suggest that some aspects of the sensory modulation of satiety are changed by repeated consumption, with covert energy becoming more effective in suppressing appetite over time, but also suggest that these behavioural changes are not readily translated into expectations of satiety.

Does modifying the thick texture and creamy flavour of a drink change portion size selection and intake?

Previous research indicates that a drink's sensory characteristics can influence appetite regulation. Enhancing the thick and creamy sensory characteristics of a drink generated expectations of satiety and improved its actual satiating effects. Expectations about food also play an important role in decisions about intake, in which case enhancing the thick and creamy characteristics of a drink might also result in smaller portion size selection. In the current study forty-eight participants (24 female) completed four test days where they came into the laboratory for a fixed-portion breakfast, returning two hours later for a mid-morning drink, which they could serve themselves and consume as much as they liked. Over the test days, participants consumed an iso-energetic drink in four sensory contexts: thin and low-creamy; thin and high-creamy; thick and low-creamy; thick and high-creamy. Results indicated that participants consumed less of the thick drinks, but that this was only true of the female participants; male participants consumed the same amount of the four drinks regardless of sensory context. The addition of creamy flavour did not affect intake but the thicker drinks were associated with an increase in perceived creaminess. Despite differences in intake, hunger and fullness ratings did not differ across male and female participants and were not affected by the drinks sensory characteristics. The vast majority of participants consumed all of the drink they served themselves indicating that differences in intake reflected portion size decisions. These findings suggest women will select smaller portions of a drink when its sensory characteristics indicate that it will be satiating.

Satiety-relevant sensory qualities enhance the satiating effects of mixed carbohydrate-protein preloads.

BACKGROUND: Orosensory cues such as food texture and flavor have been shown to play a role in satiation, but their role in satiety remains less clear. OBJECTIVE: The objective was to determine whether satiety-relevant orosensory cues enhance the satiating effects of energy in the context of beverage preloads. DESIGN: The effects of 6 drink preloads that combined 2 amounts of energy [high energy (HE): 279 kcal; low energy (LE): 78 kcal] and 3 satiety-relevant sensory contexts [low sensory (LS), medium sensory (MS), and high sensory (HS)] on subsequent appetite and test meal intake were assessed in 36 healthy nonobese volunteers. RESULTS: The ability of the preloads to modify appetite 30 min after consumption depended on both energy content and sensory context (P-interaction < 0.05), with hunger significantly being lower after consumption of the HE than after the LE preload in the HS context (P < 0.001), tending to be lower in the MS context (P = 0.08), but not different in the LS context. Food intake at lunch was lower after the HE than after the LE preloads (effect of energy P < 0.001), but this effect depended on sensory context (P < 0.005). The degree to which reduced test meal intake compensated for the added energy in the HE preloads was 88% in the HS context, which was significantly greater than in the MS (47%) and LS (18%) contexts. CONCLUSION: Small changes in the sensory characteristics of drinks altered the degree to which added energy was satiating, which implies that nutrients become more satiating when they are predicted by relevant sensory cues such as thickness and creaminess. This trial was registered at http://www.controlled-trials.com as ISRCTN36258511.

Facilitating or undermining? The effect of reward on food acceptance. A narrative review.

Using rewards in child feeding is commonplace and viewed as effective by parents, although some express concern about using 'bribery'. Psychological and economic theorists emphasize the beneficial effects of rewards in enhancing performance, although, there is evidence that the offer of rewards undermines intrinsic motivation and decreases enjoyment of the rewarded task. In the food domain, results have been mixed, but this may be explained, at least partly in terms of the measured outcome (liking vs intake) and the initial level of motivation towards the target foods (liked vs disliked). Where intake is the outcome, rewards have had broadly positive effects, but when it is liking, rewards can have negative effects if the target food is already liked. Another issue concerns the type of reward offered. While offering food as a reward appear to be universally negative, there is evidence to suggest that non-food tangible rewards (e.g., stickers), or non-tangible rewards (praise) can be highly effective in encouraging children to taste new or less liked foods sufficiently often to benefit from the 'mere exposure' effect. We suggest that the judicious use of rewards may facilitate children's acceptance of healthy foods.

Individual differences in satiety response to carbohydrate and fat. Predictions from the Three Factor Eating Questionnaire (TFEQ).

A high carbohydrate/low fat diet is recommended for reduced risk of overeating, but it is not clear whether satiety responses to carbohydrate and fat differ between individuals. The objective of this study was to determine how women with either a low or a high tendency to overeat (TFEQ-disinhibition) and with either a low or a high tendency to restrict intake (TFEQ-restraint) respond to fat and carbohydrate consumed at breakfast. Sixty-four healthy-weight women preselected for high and low TFEQ scores of restraint and disinhibition were enrolled for the study. They came to the laboratory on two separate days to consume breakfast; on one day the principle energy source was fat and on the other day carbohydrate. Satiety responses were determined by ratings of hunger and fullness for 2 h after breakfast, and intake at a mid-morning snack test. Those scoring high on the TFEQ-disinhibition scale consumed more energy at the snack test than those with low TFEQ-disinhibition, but this was only following the high carbohydrate breakfast. The TFEQ-restraint scale did not independently predict food intake or interact with the TFEQ-disinhibition related overeating. In normal-weight females the tendency to overeat may be related to insensitivity to the satiating effects of carbohydrate.

Eating for pleasure or profit: the effect of incentives on children's enjoyment of vegetables.

Parents commonly use rewards to encourage children to eat healthfully, but this practice remains controversial because rewards are suspected of undermining children's intrinsic motivation. A cluster-randomized trial examined children's acceptance of a disliked vegetable over 12 daily taste exposures. These exposures were paired with a tangible reward, a social reward, or no reward, and the findings were compared with the results from a no-treatment control condition. Liking and intake of the vegetable were assessed in a free-choice consumption task at preintervention, postintervention, 1 month after intervention, and 3 months after intervention. Liking increased more in the three intervention conditions than in the control condition, and there were no significant differences between the intervention conditions. These effects were maintained at follow-up. Children in both reward conditions increased consumption, and these effects were maintained for 3 months; however, the effects of exposure with no reward became nonsignificant by 3 months. These results indicate that external rewards do not necessarily produce negative effects and may be useful in promoting healthful eating.

A2B adenosine receptors regulate the mucus clearance component of the lung's innate defense system.

Adenosine (ADO) signaling is altered in both asthma and chronic obstructive pulmonary disease, and the A(2B) adenosine receptor (A(2B)-R) may drive pulmonary inflammation. Accordingly, it has been proposed that specific inhibition of the A(2B)-R could treat inflammatory lung diseases. However, stimulation of the cystic fibrosis transmembrane conductance regulator (CFTR) by ADO may be crucial in permitting the superficial epithelium to maintain airway surface liquid (ASL) volume, which is required to ensure hydrated and clearable mucus. Our goal was to determine which ADO receptor (ADO-R) underlies ASL volume regulation in bronchial epithelia. We used PCR techniques to determine ADO-R expression in bronchial epithelia and used nasal potential difference measurements, Ussing chambers studies, and XZ-confocal microscopy to look at Cl- secretion and ASL volume regulation. The A(2B)-R was the most highly expressed ADO-R in donor specimens of human bronchial epithelia, and inhibition of ADO-R in vivo prevented activation of CFTR. A(2B)-R was the only ADO-R detected in cultured human bronchial epithelial cells and inhibition of this receptor with specific A(2B)-R antagonists resulted in ASL height collapse and a failure to effect ASL height homeostasis. Removal of ADO with ADO deaminase and replacement with 5'N-ethylcarboxamide adenosine resulted in dose-dependent changes in ASL height, and suggested that the cell surface (ADO) may be in excess of 1 microM, which is sufficient to activate A(2B)-R. A(2B)-R are required for ASL volume homeostasis in human airways, and therapies directed at inhibiting A(2B)-R may lead to a cystic fibrosis-like phenotype with depleted ASL volume and mucus stasis.

Caffeine deprivation state modulates expression of acquired liking for caffeine-paired flavours.

Previous studies found that caffeine consumers acquired a liking for the flavour of novel caffeinated drinks when these drinks were consumed repeatedly in a caffeine-deprived, but not nondeprived, state. Expression of this acquired liking appeared acutely sensitive to current caffeine deprivation state, but the use of between-subjects designs confounded interpretation of those studies. The present study evaluated these findings further using a within-subject design, with one flavour paired with caffeine (CS + ) and the second with the absence of caffeine (CS-). During four CS + and four CS- training days, 32 moderate caffeine consumers alternatively consumed a novel flavoured drink a CS + paired with caffeine and a CS- flavour paired with placebo. Participants evaluated both drinks before and after training in two motivational states: caffeine deprived and nondeprived. As predicted, pleasantness ratings for the caffeine-paired flavour increased overall. However, this acquired liking was only significant when tested in a caffeine-deprived state. These data are consistent with a conditioned-flavour preference model and imply that expression of acquired liking for a novel caffeinated flavour depends on the need for the effects of caffeine at the time when the drink is evaluated.

Liquid movement across the surface epithelium of large airways.

The cystic fibrosis transmembrane conductance regulator CFTR gene is found on chromosome 7 [Kerem, B., Rommens, J.M., Buchanan, J.A., Markiewicz, D., Cox, T.K., Chakravarti, A., Buchwald, M., Tsui, L.C., 1989. Identification of the cystic fibrosis gene: genetic analysis. Science 245, 1073-1080; Riordan, J.R., Rommens, J.M., Kerem, B., Alon, N., Rozmahel, R., Grzelczak, Z., Zielenski, J., Lok, S., Plavsic, N., Chou, J.L., et al., 1989. Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA. Science 245, 1066-1073] and encodes for a 1480 amino acid protein which is present in the plasma membrane of epithelial cells [Anderson, M.P., Sheppard, D.N., Berger, H.A., Welsh, M.J., 1992. Chloride channels in the apical membrane of normal and cystic fibrosis airway and intestinal epithelia. Am. J. Physiol. 263, L1-L14]. This protein appears to have many functions, but a unifying theme is that it acts as a protein kinase C- and cyclic AMP-regulated Cl(-) channel [Winpenny, J.P., McAlroy, H.L., Gray, M.A., Argent, B.E., 1995. Protein kinase C regulates the magnitude and stability of CFTR currents in pancreatic duct cells. Am. J. Physiol. 268, C823-C828; Jia, Y., Mathews, C.J., Hanrahan, J.W., 1997. Phosphorylation by protein kinase C is required for acute activation of cystic fibrosis transmembrane conductance regulator by protein kinase A. J. Biol. Chem. 272, 4978-4984]. In the superficial epithelium of the conducting airways, CFTR is involved in Cl(-) secretion [Boucher, R.C., 2003. Regulation of airway surface liquid volume by human airway epithelia. Pflugers Arch. 445, 495-498] and also acts as a regulator of the epithelial Na(+) channel (ENaC) and hence Na(+) absorption [Boucher, R.C., Stutts, M.J., Knowles, M.R., Cantley, L., Gatzy, J.T., 1986. Na(+) transport in cystic fibrosis respiratory epithelia. Abnormal basal rate and response to adenylate cyclase activation. J. Clin. Invest. 78, 1245-1252; Stutts, M.J., Canessa, C.M., Olsen, J.C., Hamrick, M., Cohn, J.A., Rossier, B.C., Boucher, R.C., 1995. CFTR as a cAMP-dependent regulator of sodium channels. Science 269, 847-850]. In this chapter, we will discuss the regulation of these two ion channels, and how they can influence liquid movement across the superficial airway epithelium.