Ultrasound Elastography as a Non-Invasive Method to Monitor Liver Disease in Children with Short Bowel Syndrome: Updated Results

PurposeThe purpose of the study was to determine the accuracy of acoustic radiation force impulse (ARFI) ultrasound elastography in assessing the degree of liver disease in children with short bowel syndrome (SBS).MethodsA prospective observational cohort study of patients with SBS who underwent a liver biopsy and ARFI elastography was performed. Mean shear wave speed (SWS) and stage of fibrosis was evaluated using t-tests. Receiver operating characteristic curves (ROC) were generated and the area under the curves (AUC) estimated in order to assess the accuracy of SWS measurements to discriminate between stages of fibrosis.ResultsThirty-seven paired elastography and biopsy samples from 31 patients were included. The median age was 0.6 years, and 61% were male. There was a significant positive correlation between stage of fibrosis and mean SWS (β = 0.16 m/s increase per stage, p =<0.001). ROC analysis revealed that mean SWS had good accuracy for discriminating between mild liver fibrosis (F0-F1) and moderate to severe fibrosis (F2-F4) (AUC = 0.80, 95% CI 0.65-0.95). In addition, ROC analysis demonstrated that mean SWS can also accurately discriminate between mild to moderate fibrosis (F0-F2) and more severe fibrosis (F3-F4) (AUC = 0.84, 95% CI 0.71-0.96).ConclusionARFI elastography is an accurate, non-invasive method to monitor liver disease in children with SBS.Type of StudyRetrospective Cohort StudyLevel of EvidenceII     

The Efficacy of Acoustic Radiation Force Impulse Elastography for Predicting Clinical Outcomes in Living Donor Liver Transplant

PurposeAcoustic radiation force impulse (ARFI) elastography is widely used for evaluating liver fibrosis. Here we evaluated the efficacy of ARFI elastography for estimating graft quality and clinical outcomes in living donor liver transplant (LDLT).MethodsWe retrospectively evaluated the cases of 87 LDLT donors who preoperatively underwent ARFI elastography at Nagasaki University Hospital between August 2010 and June 2016. We analyzed whether the velocity of shear wave (Vs) obtained by ARFI elastography affected the regeneration rate of each donor's remnant liver and the 1-year survival rate of the recipients.ResultsThere were no significant correlations between Vs value and the donors' age. Only 1 donor (1.1%) showed significant fibrosis, F2 (portal fibrosis with few septa) in zero-biopsy. The 7 donors (8.0%), including 1 case, showed a high Vs value (> 1.33) that was equal to F2, although there was no abnormal pathologic finding except in 1 case. In those cases, the regeneration rate of the remnant liver after hepatectomy was significantly lower compared to other cases. The 1-year survival rate of the recipients paired with the high-Vs donors was also significantly poorer than that of the other cases (high-Vs: 57.1%, others: 84.2%, P = .04).ConclusionsARFI elastography might be an effective examination for the preoperative evaluation of the graft quality in LDLT.     

Co-morbidity of cystic fibrosis and celiac disease in Scandinavian cystic fibrosis patients

BackgroundThe co-morbidity of cystic fibrosis (CF) and celiac disease (CD) has been reported sporadically since the 1960s. To our knowledge, this is the first time a systematic screening is performed in a large cohort of CF patients.MethodsTransglutaminase-IgA (TGA), endomysium-IgA (EMA) and total IgA in serum were measured in 790 CF patients (48% females, 86% with pancreatic insufficiency). Six patients were diagnosed with CD prior to the study, all receiving a gluten-free diet.Patients with elevated TGA (> 50 Units/mL) and a positive EMA test were offered a gastroscopy obtaining mucosal biopsies from the duodenum.ResultsFour new cases of CD were diagnosed. Two additional patients had positive serological tests, but normal biopsies.In total, 10 cases of CD (1.2%, 1:83) indicate a prevalence rate about three times higher than the general prevalence of CD in Norway and Sweden. No CD patients were detected in the Danish CF cohort.Patients diagnosed with untreated CD reported symptoms typical of both CF and CD (poor weight gain, loose and/or fatty stools, fatigue, irritability, abdominal pain). They improved after introduction of a gluten-free diet.ConclusionsSystematic screening for CD in a Scandinavian cohort of CF patients revealed a higher prevalence of CD than in the general population. Clinical signs of CD are difficult to differentiate from CF with malabsorption, and patients may go undiagnosed for a long time. In a population where CD is common we recommend screening for CD in patients with CF.     

Kidney tissue elastography and interstitial fibrosis observed in kidney biopsy

IntroductionKidney interstitial fibrosis is an important risk factor for the progression of chronic kidney disease. Kidney elastography is a noninvasive imaging modality that might be used to assess tissue fibrosis. In this study, we aimed to investigate the relationship between tissue stiffness detected in kidney elastography and interstitial fibrosis observed in kidney biopsy.Materials and methodsPatients who were hospitalized in a tertiary care university hospital with a kidney biopsy indication were included in this study. In all patients, the transverse and sagittal elastography measurements were made using a sonoelastography device before the biopsy. The total histological score was calculated.ResultsFifty-seven native kidney patients with proteinuria were included in the study. Patients were divided into two groups according to the presence (n = 6) and absence of fibrosis (n = 51) as detected by kidney biopsy. A significant correlation was found between the presence of fibrosis detected by biopsy and elastography outcomes (p = .046, r = .192). A significant correlation was found between the urea and creatinine levels and transverse elastography measurements (p = .036, r = .240). No correlation was observed between the transverse elastography measurements and total histological score consisting of glomerular, vascular, and tubular scores (r = .006, p = .967)ConclusionThe findings of our study suggest a significant relationship between the elastography measurements and interstitial fibrosis. Because of the high negative predictive value (91%), we suggest that elastography should mainly be used as an exclusion test for the presence of fibrosis. We also believe that elastography may be useful to evaluate the fibrosis status in kidney diseases.     

Prevalence of Helicobacter pylori infection in patients with cystic fibrosis

IntroductionHelicobacter pylori (H. pylori) is one of the most common bacterial infections worldwide. The prevalence of Hp infection in cystic fibrosis (CF) is unclear. Thus, the aim of our study was to determine the prevalence of H. pylori infection in CF patients and to correlate H. pylori presence with CF expression.Material and methodsThe presence of H. pylori infection was assessed using a breath test with isotope-labeled urea in CF 79 patients compared to 302 healthy control subjects (HS).ResultsFifteen (19.0%) CF patients were H. pylori positive. No statistical differences in the basic clinical parameters or in their distribution were documented. No clinical factor was an independent risk factor of H. pylori infection. The corrected prevalence of H. pylori infection in pediatric CF patients and HS was 14.4% and 9.8%, respectively.ConclusionThe prevalence of H. pylori infection in CF patients is not different from that in healthy subjects.     

The fungal airway microbiome in cystic fibrosis and non-cystic fibrosis bronchiectasis

BackgroundThe prevalence of fungal disease in cystic fibrosis (CF) and non-CF bronchiectasis is increasing and the clinical spectrum is widening. Poor sensitivity and a lack of standard diagnostic criteria renders interpretation of culture results challenging. In order to develop effective management strategies, a more accurate and comprehensive understanding of the airways fungal microbiome is required. The study aimed to use DNA sequences from sputum to assess the load and diversity of fungi in adults with CF and non-CF bronchiectasis.MethodsNext generation sequencing of the ITS2 region was used to examine fungal community composition (n = 176) by disease and underlying clinical subgroups including allergic bronchopulmonary aspergillosis, chronic necrotizing pulmonary aspergillosis, non-tuberculous mycobacteria, and fungal bronchitis. Patients with no known active fungal disease were included as disease controls.ResultsITS2 sequencing greatly increased the detection of fungi from sputum. In patients with CF fungal diversity was lower, while burden was higher than those with non-CF bronchiectasis. The most common operational taxonomic unit (OTU) in patients with CF was Candida parapsilosis (20.4%), whereas in non-CF bronchiectasis sputum Candida albicans (21.8%) was most common. CF patients with overt fungal bronchitis were dominated by Aspergillus spp., Exophiala spp., Candida parapsilosis or Scedosporium spp.ConclusionThis study provides a framework to more accurately characterize the extended spectrum of fungal airways diseases in adult suppurative lung diseases.     

The Acoustic Radiation Force Impulse Elastography Evaluation of Liver Fibrosis in Posttransplantation Dysfunction of Living Donor Liver Transplantation

Background

The acoustic radiation force impulse elastography (ARFI) is a new technology of elastography integrated into B-mode ultrasonography. It has been a reliable method to evaluate liver fibrosis of chronic liver disease in recent years, but less applied in the posttransplantation liver. The aim of the study was to evaluate liver fibrosis by the ARFI with correlation of pathological stages in living donor liver transplantation (LDLT).

Materials and Methods

From August 2010 to August 2012, there were 57 LDLT patients with liver biopsy (LB) due to posttransplantation dysfunction; all patients also received posttransplantation ARFI liver stiffness measurement (LSM) after transplantation for liver fibrosis staging. The ARFI elastography was performed using a Siemens Acuson S2000 ultrasound system with 4V1 transducers (Acusion, Siemens Medical Systems Co. Ltd. Erlangen, Germany). The ARFI LSM value was presented by shear wave velocity (SWV, m/s). The fibrosis staging as F0 to F4 was in accordance with the Metavir scoring system.

Results

A total of 57 patients had both posttransplantation LB and effective ARFI fibrosis staging for correlation. The ARFI LSM value increased with severity of liver fibrosis and had significant linear correlation with the results of histological fibrosis staging. The ARFI LSM sensitivities (Se), specificities (Sp), and cutoff values based on receiver-operator characteristic curve were F0: 0.75 m/s (Se: 93.8%, Sp: 4%), F1: 1.06 m/s (Se: 95.5%, Sp: 25.7%), F2: 1.81 m/s (Se: 50%, Sp: 83.6%) and F3: 2.33 m/s (Se: 100%, Sp: 92.9%). Predictive value of ARFI LSM reported a significant difference between early fibrosis stage (F0–F1) and advanced fibrosis stage (F ≧ 2) (P < .05).

Conclusion

In this study, ARFI demonstrated a strong linear correlation and severity of liver fibrosis with LB pathologic staging. ARFI can be an alternative and compensatory method for frequent LB in the posttransplantation liver.     

Utility and Limitations of Transient Elastography to Monitor Hepatic Steatosis,Hepatic Fibrosis,and Methotrexate-Associated Hepatic Disease in Psoriasis: A Systematic Review

ObjectivePsoriasis is associated with hepatic steatosis, fibrosis, and methotrexate-associated liver injury. There is a need for reliable methods to monitor liver disease in psoriasis. Transient elastography (TE) is a validated non-invasive method for assessing hepatic steatosis and fibrosis. Psoriasis-specific TE studies have been limited until recently. Here, we review the utility and limitations of TE to detect and monitor liver disease in the context of psoriasis.MethodsA comprehensive search using OVID, PubMed, and gray literature was conducted (2005–November 2019) to identify studies of TE use in psoriasis for assessment of hepatic steatosis and fibrosis.ResultsFifteen studies met inclusion criteria. A total of 1,536 patients with psoriasis or psoriatic arthritis were represented. TE-detected liver fibrosis is associated with age, diabetes, obesity, and severity of psoriasis. TE successfully evaluates hepatic steatosis and fibrosis. Elastography has a high negative predictive value and specificity in the context of methotrexate-associated liver fibrosis in psoriasis; however, reported associations between abnormal elastography results and cumulative methotrexate dose varied significantly despite methotrexate’s association with hepatotoxicity and fibrosis. The presence of central adiposity is associated with increased TE failure rate.LimitationThe TE studies included in this review date from 2007 to 2019, which could contribute to publication bias, as the technique of TE has improved over this time period.ConclusionTE is a useful and non-invasive modality to detect hepatic steatosis and fibrosis in psoriasis. Dermatologists might consider TE in psoriatic patients and concomitant risk factors for fibrosis with the understanding that failure rates may be higher in patients with central adiposity.     

Increased prevalence of colonic adenomas in patients with cystic fibrosis

BackgroundCystic fibrosis (CF) is the most common lethal genetic illness in the Caucasian population. Studies have shown that CF patients are at an elevated risk of developing colon cancer. Colonic adenomas are the precursors of colon cancer. This study aims to determine the prevalence of adenomas in patients with cystic fibrosis.MethodsAll patients were recruited prospectively at The Ottawa Hospital Cystic Fibrosis Clinic from 2010 through 2015. Baseline demographic and cystic fibrosis disease characteristics were collected from the clinic's CF patient database. Upon presentation at the endoscopy unit, and after a brief history and physical exam, a colonoscopy was performed. Polyps were resected if detected and sent to the pathology department for characterization. Findings were compared with a control group (pairing each CF patient with 5 age and sex-matched controls) of near-average risk patients who underwent a colonoscopy at the same center.ResultsOf the 33 patients that provided informed consent to participate in the study, 30 patients underwent colonoscopy and 13/30 (43.3%) were found to have colonic adenomas compared to 7 (4.7%) of the 150 control patients. The relative risk ratio for adenoma detection in a CF patient as compared to a matched control patient was 9.29 (95% CI 4.04–21.31), p < 0.01.ConclusionsColonic adenomas are more prevalent in CF patients compared to the general population. This study suggests the need for additional research to support recently published screening guidelines for CF patients.     

The Utility of Assessing Liver Allograft Fibrosis and Steatosis Post–Liver Transplantation Using Transient Elastography With Controlled Attenuation Parameter

AimAllograft steatosis is an emerging concern after liver transplantation (LT). The use of transient elastography (TE) with controlled attenuation parameter (CAP) may facilitate early detection of and intervention for allograft steatosis. This study aimed to evaluate the prevalence and risk factors of allograft steatosis using TE and CAP.MethodsThe presence of steatosis and severe steatosis were defined by CAP ≥222 and ≥290 dB/m, respectively. Demographics and clinical characteristics were compared between patients with and without severe steatosis. Regression analyses were performed to determine factors associated with severe steatosis.ResultsOf 150 patients, 105 (70%) had steatosis while 40% of these had severe steatosis. Thirty-four (81.0%) patients with severe steatosis had normal alanine transaminase at the time of TE. In multivariable analyses, age at LT (odds ratio [OR] 1.05, 95% confidence interval [CI] 1.01-1.08), post-LT obesity (OR 5.34, 95% CI 1.53-18.65), and alcoholic liver disease (OR 12.86, 95% CI 2.24-73.74) were significant predictors of severe steatosis. Five patients underwent liver biopsies as a result of advance fibrosis seen on TE and were later diagnosed with chronic allograft rejection. Two of these patients had normal liver chemistries, and the remaining 3 had mild elevation of alkaline phosphatase.ConclusionSteatosis was present in 70% of patients who underwent TE after LT. Advanced age at LT, post-LT obesity, and alcoholic liver disease were significant predictors for severe steatosis. The majority of patients with severe steatosis had normal liver enzymes. TE should be considered as a screening modality for allograft steatosis and fibrosis even when liver chemistries are normal.     

The prevalence,clinical status and genotype of cystic fibrosis patients living in Cuba using national registry data

BackgroundWe aimed to establish a national cystic fibrosis (CF) registry for Cuba, a developing country.MethodsRegional centres that deliver care for all CF patients provided information for a national database.FindingsThe prevalence of CF in Cuba is 26.3 cases per 1,000,000 population. The median age at diagnosis is 2 years, and the median age of the total population was 15 years. Of those aged 16 years or older, the prevalence of Pseudomonas aeruginosa infection was 46%, the prevalence of Staphylococcus aureus infection was 36%, and 80% of individuals were receiving oral azithromycin. The commonest gene mutation was F508del which was observed in 50% of patients.InterpretationThese data demonstrate that it is possible to establish a national CF registry in a developing country such as Cuba. This provides baseline data to permit evaluation of health care delivery enable the spread of good clinical practice nationally.     

Growth retardation and reduced growth hormone secretion in cystic fibrosis. Clinical observations from three CF centers

ObjectiveGrowth delay in cystic fibrosis is frequent and is usually the result of several interacting causes. It most often derives from severe respiratory impairment and severe malabsorption. There are however patients whose clinical condition is not severe enough to be held accountable for this phenomenon. We aimed at describing patients who showed growth delay, who were not affected by severe pulmonary disease or malabsorption and who, when tested, showed a reduced GH secretion after stimulation with conventional agents. We noticed a disproportionately large prevalence of growth hormone (GH) release deficit (GHRD) in pediatric cystic fibrosis (CF) patients.Patients and methodsWe examined all patients under our care in the period 2006–11, who were older than 5 and younger than 16 years old. We focussed on those who fell below the 3rd height percentile, or whose growth during the previous 18 months faltered by > 2 SD, and who did not present clinical conditions that could reasonably explain their failure to thrive. These patients were subjected to standard GH provocative tests.ResultsOut of 285 who matched the age criterion, 33 patients also matched the height percentile criterion. While 15/33 suffered clinical conditions that could reasonably explain their failure to thrive, 18/33 underwent GH release provocative tests and 12/18 showed a release deficit.ConclusionsWe conclude that impaired GH secretion is more frequent among CF patients compared to the prevalence of GH deficiency in the general population and that GH release impairment may be an independent cause of growth delay in CF. Our findings are in agreement with recent studies that have described low GH levels in CF piglets and in neonates with CF [1].     

Use of noninvasive scores for advanced liver fibrosis can guide the need for hepatic biopsy during bariatric procedures

BackgroundPatients with obesity are at increased risk for nonalcoholic fatty liver disease (NAFLD). The effectiveness of noninvasive screening tests for ruling out advanced fibrosis (stage 3–4) is unknown.ObjectivesTo determine the prevalence of advanced fibrosis in patients undergoing routine liver biopsy during bariatric surgery and assess the effectiveness of existing noninvasive risk calculators.SettingAcademic medical center in the United States.MethodsRoutine liver biopsies were obtained during first-time bariatric surgery (January 2001–December 2017). Patient demographic characteristics, co-morbidities, and preoperative laboratory values were compiled. Sensitivity, specificity, negative predictive value (NPV), and positive predictive value (PPV) were compared between 3 noninvasive risk calculators for advanced fibrosis: the fibrosis-4 index, NAFLD fibrosis score, and aminotransferase-to-platelet ratio index (APRI).ResultsAmong 2465 patients, the prevalence of advanced fibrosis (stage 3–4) was 3.4%. The mean age was 45.5 years, and the mean body mass index was 46.8. The sensitivity of noninvasive risk calculators ranged from 85% (NAFLD fibrosis score) to 24% (APRI). The NAFLD fibrosis score performed best in screening out advanced fibrosis, with an NPV of 99%. The PPV ranged from 9% to 65%. In this study cohort, the use of the NALFD fibrosis score correctly ruled out advanced fibrosis in 893 (36%) patients, with 13 false negatives.ConclusionsThe prevalence of advanced fibrosis in individuals undergoing routine first-time bariatric procedures is 3.4%. Use of the NALFD fibrosis score can rule out advanced fibrosis in one-third of this population, and guide surgical decision-making.     

Alveolar inflammation in cystic fibrosis

BackgroundIn infected lungs of the cystic fibrosis (CF) patients, opportunistic pathogens and mutated cystic fibrosis transmembrane conductance regulator protein (CFTR) contribute to chronic airway inflammation that is characterized by neutrophil/macrophage infiltration, cytokine release and ceramide accumulation. We sought to investigate CF lung inflammation in the alveoli.MethodsLung tissue from 14 CF patients and four healthy individuals was analyzed for numbers of effector cells, elastin and collagen concentrations, inflammatory markers and density of Pseudomonas aeruginosa. Additionally, desmosine and isodesmosine concentrations were determined in 52 urine specimens from CF patients to estimate the burden of elastase activities in respiratory secretions.ResultsElastin concentration was significantly decreased and collagen significantly increased in CF alveolar tissues as compared to age-matched, healthy individuals. Elastin split products were significantly increased in urine samples from patients with CF and correlated inversely with age, indicating local tissue remodelling due to elastin degradation by unopposed proteolytic enzymes. Alveolar inflammation was also characterized by a significant cell infiltration of neutrophils, macrophages and T cells, extensive nuclear factor-κB and insulin-like growth factor-1 activation in various cell types and increased intercellular adhesion molecule-1 expression, and increased numbers of myofibroblasts. Additionally, ceramide accumulated in type II alveolar epithelial cells, lacking CFTR. P. aeruginosa organisms were rarely present in inflamed alveoli.ConclusionsChronic inflammation and remodeling is present in alveolar tissues of the CF lung and needs to be addressed by anti-inflammatory therapies.     

Development and evaluation of an educational website for adults with cystic fibrosis

BackgroundThe high prevalence of internet use among adults with cystic fibrosis (CF) provides an opportunity for healthcare professionals to use the internet as a tool for patient education. The Toronto Adult Cystic Fibrosis website was developed, implemented, and evaluated (www.torontoadultcf.com).MethodsThe website structure and content were developed following a needs assessment questionnaire. The website was evaluated with a satisfaction survey and through examination of compliance to American Medical Association (AMA)'s guidelines for health information sites.ResultsUsers showed a high prevalence of internet usage (98.7%), described a need for more information about CF and provided content suggestions. A satisfaction survey revealed that CF patients compose the highest proportion of site users (57.5%), and users perceive the site as useful, with a mean score of 4.3 on a five-point satisfaction scale. Key areas for improvement have been identified. Website compliance with AMA guidelines was excellent at 80%.ConclusionsThe Toronto Adult CF website provides needed information for our patients. Future directions include ongoing improvement of the website as a patient resource and assessment of the ability of the website to improve patient knowledge and outcomes.     

Sinonasal manifestations of cystic fibrosis: A correlation between genotype and phenotype?

BackgroundPatients with Cystic Fibrosis are prone to develop sinonasal disease. Studies in genotype–phenotype correlations for sinonasal disease are scarce and inconclusive.MethodsIn this observational study several aspects of sinonasal disease were investigated in 104 adult patients with CF. In each patient a disease specific quality of life questionnaire (RSOM-31), nasal endoscopy and a CT scan of the paranasal sinuses were performed. Patients were divided into two groups, class I–III mutations and class IV–V mutations, based on their CFTR mutations.ResultsThe prevalence of rhinosinusitis in adult patients with CF was 63% and the prevalence of nasal polyps 25%. Patients with class I–III mutations had significantly smaller frontal sinuses, sphenoid sinuses, more opacification in the sinonasal area and more often osteitis/neoosteogenesis of the maxillary sinus wall compared to patients with class IV and V mutations.ConclusionThese data suggest more severe sinonasal disease in patients with class I–III mutations compared to patients with class IV–V mutations.     

Characteristics of electrolyte imbalance and pseudo-bartter syndrome in hospitalized cystic fibrosis children and adolescents

IntroductionPseudo-Bartter syndrome (PBS) is a rare manifestation of Cystic fibrosis (CF) and can often be the initial presentation in these patients, however, due to significantly overlapping symptoms it is often misdiagnosed as simple dehydration or Bartter syndrome. The objective of our study was to highlight the key features of PBS and electrolyte imbalance in CF patients helping in early and prompt diagnosis.MethodWe performed a retrospective study from January 2015 to December 2019 at the Aga Khan University Hospital (AKUH), Pakistan. CF patients aged from 1–18 years, admitted at AKUH were enrolled and their laboratory data and individual charts were reviewed. Patients were categorized into three groups based on their serum electrolyte profile and their clinical findings were compared.ResultWe enrolled 72 CF patients, out of which 42 (58%) were categorized into the Normal Electrolyte (NE) group, 19 (26%) into the Electrolyte Imbalance (EI) group and 11 (15%) in the PBS group. Out of 11 cases, 6 (54.54%) patients in PBS group presented with features consistent with PBS leading to CF diagnosis labeled as “early presenters”. Mean age of patients in the PBS group was 3.81± 0.86 years and their age at diagnosis were significantly lower as compared to other groups. Gastrointestinal disturbances including diarrhea, vomiting and constipation were more common in the EI and PBS groups. Polyuria was most common in the PBS (72%) group. Length of hospital stay showed no significant difference.ConclusionPseudo-Bartter syndrome can be a presenting feature of cystic fibrosis. Electrolyte imbalance should be anticipated in hospitalized CF children and adolescent.     

Exhaled molecular profiles in the assessment of cystic fibrosis and primary ciliary dyskinesia

BackgroundEarly diagnosis and monitoring of disease activity are essential in cystic fibrosis (CF) and primary ciliary dyskinesia (PCD). We aimed to establish exhaled molecular profiles as the first step in assessing the potential of breath analysis.MethodsExhaled breath was analyzed by electronic nose in 25 children with CF, 25 with PCD and 23 controls. Principle component reduction and canonical discriminant analysis were used to construct internally cross-validated ROC curves.ResultsCF and PCD patients had significantly different breath profiles when compared to healthy controls (CF: sensitivity 84%, specificity 65%; PCD: sensitivity 88%, specificity 52%) and from each other (sensitivity 84%, specificity 60%). Patients with and without exacerbations had significantly different breath profiles (CF: sensitivity 89%, specificity 56%; PCD: sensitivity 100%, specificity 90%).ConclusionExhaled molecular profiles significantly differ between patients with CF, PCD and controls. The eNose may have potential in disease monitoring based on the influence of exacerbations on the VOC-profile.     

Attitudes of pain and opioids prescription practices in U.S. cystic fibrosis centers

BackgroundThe high incidence and prevalence of chronic pain in patients with cystic fibrosis (CF) is well documented. However, there is limited data on chronic pain management in this population.MethodsWe designed a questionnaire examining care team members’ views on the prevalence and characteristics of pain, pain management, and opioid use. The questionnaire was distributed to accredited programs throughout the US via a CF Foundation (CFF) email list-serve.ResultsResponses came from 52 adult core or affiliated centers (Adult Responders – AR), 36 pediatric core or affiliated centers (Pediatric Responders – PR), and 9 were from combined programs. AR perceive more patients having chronic pain compared to PR. Furthermore, 40% of the AR said that > 50% of those with chronic pain also have comorbid depression or anxiety. 61% of PR ranked sinus/headache symptoms as the most common while AR ranked chest wall as the most frequent site (58%). While most centers (83%) report that pain management in patients with CF is a very important or important issue, 50% of AR feel uncomfortable or only slightly comfortable in prescribing opioids. 44% report that CF providers are currently responsible for this task.ConclusionsChronic pain is common in adult patients with CF and management presents a formidable challenge to providers. The development of guidelines and/or collaboration with pain specialists will likely benefit both patients and providers.     

Variation in lung function and nutritional decline in cystic fibrosis by genotype: An analysis of the Canadian cystic fibrosis registry

BackgroundThis study aimed to improve our understanding of the natural history of cystic fibrosis (CF) by comparing lung function and body mass index z-score (zBMI) between patients with different genotypes and identify a genotype with outcomes most comparable to homozygous ΔF508 patients.MethodsData was obtained from the Canadian CF Registry between January 1st 2007-January 1st 2016. Patients were categorized into one of five groups based on their genotype. A mixed-effects model was conducted with adjustments for age, sex, age of diagnosis, and baseline clinical measures.ResultsAmong 2612 patients, those with non-mild forms of CF, and particularly adult patients with the same functional allele classification were found to have a lung function trajectory comparable to individuals with the homozygous ∆F508 genotype (annual change in percent predicted forced expiratory volume in 1 s of −0.83, 95% CI: −0.93, −0.73). The rates of zBMI change over the study period were not significantly different between the genotype groups.ConclusionThis population-based study of Canadian CF patients provides adjusted rates of lung function decline and zBMI over ten years. The finding that there are different genotypes with comparable rates of lung function decline to patients of the homozygous ∆F508 group supports the use of multiple comparison groups to assess the long-term efficacy of emerging CF therapies.