scholarly journals Liver Dysfunction may be Associated with QOL in Adults with CF

2019 ◽  
Vol 2 (1) ◽  
Author(s):  
Megana Rao, BS ◽  
Regina Weber, BS ◽  
Naga Chalasani, MD

Background: More individuals with cystic fibrosis (CF) are living into their adulthood due to significant advances in medical care. The adult CF clinic at Indiana University cares for one of the largest adult populations with CF. Liver disease is common in children and adults with CF. However, the characteristics and consequences of CF liver disease (CFLD) in adults are not well understood. In this ongoing, IRB approved study, we systematically characterized the liver manifestations and their health related quality of life in adults with well characterized CF. Methods: Individual patient demographics and clinical data were collected for study participants. Participants completed two quality of life study questionnaires: Chronic Liver Disease Questionnaire (CLDQ) and Revised Cystic Fibrosis Questionnaire (CFQ-R). 10mL of blood was collected for future research. Finally, participants underwent a bedside transient elastography via FibroScan®. Results: There were 50 patients, with a mean age of 30.9 years, enrolled in the study. Pancreatic insufficiency was the most common co-morbidity, as it affected 90% of patients. The mean AST was 23.55 units/L, mean ALT was 25.20 units/L, and mean alkaline phosphatase level was 117.73 units/L. From the FibroScan®, the median liver stiffness measurement (LSM) was 4.65 kPa and the mean controlled attenuation parameter (CAP) was 219.52 dB/M. The mean CLDQ score was 5.343. Conclusion: This ongoing study reveals that increased alkaline phosphatase and CAP scores are associated with a poor QOL. More data is needed to further understand the pathophysiology behind CFLD. Use of noninvasive imaging, noninvasive markers, and the CLDQ may aid in early identification of adult CFLD.    

2019 ◽  
Vol 6 (1) ◽  
pp. e06-e06
Author(s):  
Rohola Shirzadi ◽  
Safoura Navaei ◽  
Mohammadreza Modaresi ◽  
Farzad Masiha

Introduction: Cystic fibrosis (CF) is a serious genetic life-shortening disease. Quality of life (QoL) measurement related to CF children is a relatively new field of research, which includes the patient’s perspective in research and clinical practice. Objectives: This study aimed to evaluate the QoL in children with CF and its association with FEV1 (forced expiratory volume in 1 second). Patients and Methods: This cross-sectional study was carried out on 7-14 years old children with CF attending children’s medical center from March 2017 to March 2018. Throat swab cultures and spirometry evaluation was performed for all patients. FEV1 was determined and the 6-min walk test (6MWT) was conducted. The patient’s psychological status was assessed using the Persian version of pediatric QoL inventory. ANOVA, t test, and chisquare tests were used for data analysis. Results: Seventy-six subjects with the mean age of 10.49±3.18 years were studied since 59% of them (n=44) were boys. The mean total QoL was 65.34±17.73. Patients with lower pulmonary function had a lower QoL. There was a significant association between FEV1 and school and emotional functioning (P=0.005 and P=0.002, respectively). A significant association was found between SPO2 (peripheral capillary oxygen saturation) reduction after 6MWT and FEV1 decline (P=0.001). Additionally, a significant association was detected between FEV1 and the distance walked during 6MWT (P=0.030). Conclusion: Regarding the association between pulmonary function and QoL in CF patients and lower QoL score in our study, the importance of assessing pulmonary function in these patients should not be neglected.


Antioxidants ◽  
2020 ◽  
Vol 9 (11) ◽  
pp. 1125
Author(s):  
Diego Martinez-Urbistondo ◽  
Rafael Suarez del Villar ◽  
Josepmaria Argemí ◽  
Lidia Daimiel ◽  
Omar Ramos-López ◽  
...  

The assessment of liver fibrosis has gained importance since the progression of non-alcoholic fatty liver disease (NAFLD). Indeed, the description of the association between undetected liver fibrosis and lifestyle in terms of antioxidant habits, comorbidity and quality of life (QoL) domains may help in the characterization of subjects with NAFLD. A cross-sectional evaluation of (n = 116) consecutive patients from an Internal Medicine ambulatory evaluation was performed. Demographic data, lifestyle, co-morbidity, QoL (according to the SF-36 index) and analytical values to calculate the oxidative related Fibrosis-4 (FIB-4) index were recorded. The association between FIB-4 and co-morbidity, antioxidant habits in QoL was assessed in univariate analysis (p < 0.05) and confirmed in multivariable analysis for 4 of the 8 SF-36 categories: Physical QoL, Physical role, Social QoL and General QoL, as well as in the Physical summary of SF-36 (p < 0.05). Finally, interactions were assessed between co-morbidity, FIB-4 and antioxidant habits showed in the prediction of mean SF-36 (p < 0.01). Liver fibrosis assessed by the oxidative surrogate index FIB-4 is associated with the interaction between antioxidant lifestyle, co-morbidity and physical, social and general aspects of QoL in apparent liver disease-free individuals, generating a proof of concept for health empowerment and personalized medicine.


2020 ◽  
Author(s):  
Nemat Bilan ◽  
Effat Marefat ◽  
Leila Nikniaz ◽  
Mahdieh Abbasalizad Farhangi ◽  
Zeinab Nikniaz

Abstract Background: There is no clinical trial that assesses the effect synbiotic supplementation on HRQOL in CF children. Considering the importance of HRQOL as an essential primary outcome and determinant of therapeutic benefit in chronic diseases like cystic fibrosis, the present clinical trial aimed to determine the efficacy of synbiotic supplementation on HRQOL in children with CF.Methods: In the present double-blind randomized clinical trial, 40 CF children were randomly allocated to the two groups. The intervention group was supplemented with synbiotics supplements and the patients in the placebo group received maltodextrin for six months. Demographic data and information about antibiotic use were recorded using the questionnaire. The health-related quality of life was assessed using the Persian version of quality of life inventory questionnaires. Paired t-test and ANCOVA were used for statistical analysis. Results: Totally, 36 participants completed the trial. The mean score of HRQOL was 76.34±17.33. There were no significant differences between synbiotic and placebo groups regarding baseline demographic and quality of life characteristics. Compared with baseline values, the mean total score and subscores of quality of life did not change significantly after synbiotic and placebo supplementation (p>0.05). Moreover, the results of ANCOVA showed that there were no significant differences between the two groups regarding the post-trial value of HRQOL total score and subscores. Conclusion: According to results, six-month supplementation with synbiotic did not have a significant effect on the HRQOL in children with CF. However, further studies with larger sample sizes and using more disease-specific questionnaires are needed for a more precise conclusion. The protocol of the study was registered at Iranian registry clinical trials (Registration code: IRCT2017011732004N1; Registration date: 2017-02-14).


2021 ◽  
Vol 15 (11) ◽  
pp. 3467-3469
Author(s):  
Niaz Muhammad ◽  
Kashif Rafi ◽  
Hassan Nadeem ◽  
Muhammad Omer Farooq ◽  
Asmat Ullah ◽  
...  

Aim: To determine the quality of life score in patients with chronic liver disease using CLO questionnaire. Study Design: Cross sectional study Place and Duration: Department of Gastroenterology, Shaikh Zayed Hospital Lahore during from 14-June-2016to 15-December-2016. Methodology: 180 consecutive patients of chronic liver disease were selected after taking Informed consent. The diagnosis was confirmed according to the data of clinical, biochemical examinations and the results of percutaneous liver biopsy data in some selected cases. All patients were subjected to exam, LFT's, RFT's and CBC, ultrasound abdomen, esophagogastroscopy which were enrolled in this study. Anti HCV, HBsAg, ferritin, Cerruloplasmin and Autoimmune profile, was done to establish the etiology. Severity of liver disease was estimated by Child-Pugh Score (Annexed). The chronic liver disease questionnaire (CLDQ) was applied as the instrument for measuring quality of life. Results: The mean age of the patients was 35.28±7.26 years. Majority of the patients were male as there frequency was 142 (78.9%). 127 (70.60 %) cases were found to be having a child pugh grade c severity of disease. Mean quality of life score was 16.3±5.88. Mean quality of life score in males was 16.21±6.21 while in female was 16.63±4.48. Similarly the mean quality of life score in child pugh class B was 13.77±4.58 and in child pugh class C was 17.35±6.05. Conclusion: It is concluded that the quality of life was bad in the patients who had child pugh class C as compared to those who had class B. Keywords: Quality of Life, Chronic Liver Disease, Cirrhosis


2015 ◽  
Vol 29 (3) ◽  
pp. 139-144 ◽  
Author(s):  
Matthew D Sadler ◽  
Pam Crotty ◽  
Linda Fatovich ◽  
Stephanie Wilson ◽  
Harvey R Rabin ◽  
...  

BACKGROUND: Liver disease is the third leading cause of mortality in patients with cystic fibrosis (CF). However, detection of CF-associated liver disease (CFLD) is challenging.OBJECTIVE: To evaluate the diagnostic performance of noninvasive methods for the detection of CFLD with a focus on transient elastography (TE).METHODS: Patients at the Adult CF Clinic of Calgary and Southern Alberta (n=127) underwent liver stiffness measurement (LSM) by TE using the FibroScan (FS, Ecosens, France) M probe; aspartate amino-transferase to platelet ratio index (APRI) and FibroTest (FT) scores were also calculated. The diagnostic performance of these tools for the detection of CFLD (defined as two or more the following criteria: abnormal liver biochemistry, hepatomegaly or sonographic abnormalities other than steatosis) were compared using the area under ROC curves.RESULTS: Forty-seven percent of the cohort was male. The median age was 27 years (interquartile range [IQR] 22 to 37 years) and body mass index 21 kg/m2(IQR 19 kg/m2to 23 kg/m2); 25% of patients were on ursodeoxycholic acid and 12% had undergone lung transplantation. The prevalence of CFLD was 14% (n=18). FS was successful in all patients; one (0.8%) patient had poorly reliable results (IQR/M >30% and LSM ≥7.1kPa). Compared with patients without CFLD (n=109), individuals with CFLD had higher median LSM according to FS (3.9 kPa [IQR 3.4 to 4.9 kPa] versus 6.4 kPa [IQR 4.4 to 8.0 kPa]), APRI (0.24 [IQR 0.17 to 0.31] versus 0.50 [IQR 0.22 to 1.18]) and FT scores (0.08 [IQR 0.05 to 1.5] versus 0.18 [IQR 0.11 to 0.35]; all P<0.05). Area under ROC curve for FS, APRI and FT for the detection of CFLD were 0.78 (95% CI 0.65 to 0.92), 0.72 (95% CI 0.56 to 0.87) and 0.76 (95% CI 0.62 to 0.90) (P not significant). At a threshold of >5.2 kPa, the sensitivity, specificity, positive and negative predictive values of LSM according to FS for detecting CFLD were 67%, 83%, 40% and 94%, respectively.CONCLUSIONS: FS, APRI and FT were useful noninvasive methods for detecting CFLD in adults.


2020 ◽  
Author(s):  
Nemat Bilan ◽  
Effat Marefat ◽  
Leila Nikniaz ◽  
Mahdieh Abbasalizad Farhangi ◽  
Zeinab Nikniaz

Abstract Background There is no clinical trial that assesses the effect synbiotic supplementation on HRQOL in CF children. Considering the importance of HRQOL as an essential primary outcome and determinant of therapeutic benefit in chronic diseases like cystic fibrosis, the present clinical trial aimed to determine the efficacy of synbiotic supplementation on HRQOL in children with CF.Methods In the present double-blind randomized clinical trial, 40 CF children were randomly allocated to the two groups. The intervention group was supplemented with synbiotics supplements and the patients in the placebo group received maltodextrin for six months. Demographic data and information about antibiotic use were recorded using the questionnaire. The health-related quality of life was assessed using the Persian version of quality of life inventory questionnaires. Paired t-test and ANCOVA were used for statistical analysis.Results Totally, 36 participants completed the trial. The mean score of HRQOL was 76.34 ± 17.33. There were no significant differences between synbiotic and placebo groups regarding baseline demographic and quality of life characteristics. Compared with baseline values, the mean total score and subscores of quality of life did not change significantly after probiotic and placebo supplementation (p > 0.05). Moreover, the results of ANCOVA showed that there were no significant differences between the two groups regarding the post-trial value of HRQOL total score and subscores.Conclusion According to results, six-month supplementation with synbiotic did not have a significant effect on the HEQOL in children with CF. However, further studies with larger sample sizes and using more disease-specific questionnaires are needed for a more precise conclusion.


2019 ◽  
Vol 10 (1) ◽  
pp. 91-98 ◽  
Author(s):  
Maryam Hassanzad ◽  
Kourosh Fakhimi Derakhshan ◽  
Hosseinali Ghaffaripour ◽  
Ali Safavi Naeini ◽  
Habib Emami ◽  
...  

AbstractObjectiveSinusitis is a common complaint in children with cystic fibrosis. However, the actual prevalence of chronic rhinosinusitis and its effect on the quality of life of children have not been well considered. Therefore, the objective of this study was to determine the effect of sinonasal quality of life in children with cystic fibrosis.Materials and methodsThis study was a diagnostic study performed on 80 children with cystic fibrosis ranging from 2 to 20 years old, who were referred to the cystic fibrosis clinic of Masih Daneshvari Hospital from 2017–2018. The questionnaires used in this study were chronic rhinosinusitis screening questionnaire based on the European task force and the evaluation of the sinonasal quality of life was based on the SN-5 survey.ResultsOf the 80 patients with fibrosis from 2 to 20 years old who were recruited in the study, 41 patients were female (51.3%) and 39 were male (48.8%). In 61 cases (76.3%), there was no chronic rhinosinusitis and 19 cases (23.8%) had chronic rhinosinusitis. The mean SN-5 score in 19 patients with chronic rhinosinusitis was 3.4105 and the mean score of patients without rhinosinusitis was 1.8426, with a P-value of 0.000. The mean SN-5 score was significant between the two groups. In patients with nasal congestion, there was a significant difference in quality of life factors such as sinus infection, nasal obstruction, and allergy symptoms (P<0.001). In patients with facial pain, there was a significant difference in quality of life factors such as sinus infection, nasal obstruction, allergic symptoms, and physical activity limitation (P <0.001). There was also a significant difference in the quality of life factors such as sinus infection, nasal obstruction, and allergy symptoms in patients with postnasal drip (P <0.001).ConclusionIn children with cystic fibrosis, the quality of life of sinonasal has a significant relationship with absence of chronic rhinosinusitis. This study showed that children with chronic renosinusitis have significantly lower quality of sinonasal life than children with chronic rhinosinusitis. The results demonstrated that quality of life scores in sinus infections, nasal obstruction, and allergy symptoms were significantly higher in patients with chronic rhinosinusitis than in those without chronic rhinosinusitis. The findings of this study are important for improving children’s health related quality of life, as it leads to promoting communication between the patient and the health care provider, identifying overlooked problems, monitoring the progress of the disease and the burden of treatment, and promoting interventions in the daily life of patients.


2019 ◽  
Vol 3 (Supplement_1) ◽  
pp. S511-S511
Author(s):  
Tsuann Kuo ◽  
Hei-Yin Ng ◽  
Der-Yan Han

Abstract A rapidly increasing older population and changing family structures in Taiwan have resulted in a significant shift in caregiving resources; there are now more older caregivers, and their psychosocial well-being and quality of life may be affected as a result of their new responsibilities. This study used secondary data from the 2018 Survey of Elderly Mental Health (N=1,113) to examine the psychosocial status, resilience, and depression of caregivers who were 65 years old and older. The results showed that the typical older caregiver was 74.15 years old, likely to be married and living with a spouse (63.6%), retired (57.4%), and had a monthly income less than 30,000 NT (30.7%). Compared with caregivers under 65 years of age, older caregivers had higher co-morbidity and lower quality of life as well as greater resilience and higher levels of depression. Both positive emotions (p&lt;0.05) and self-assessed health (p&lt;0.001) were found to be significant variables affecting depression and resilience. And negative emotions (p&lt;0.001) also influenced depression. This study was the first in the nation to tackle issues related to aging caregivers that can identify policy, research, and practice implications to improve the general health and lives of older caregivers. Future research can use the results to explore opportunities for older caregivers that will minimize negative emotions in order to build resilience and combat depression.


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