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1.

Background:

Osteoporosis occurs frequently in patients with chronic cholestatic liver diseases, yet data are scarce regarding the prevalence of osteoporosis and fragility fractures and their impact on Health-Related Quality of Life (HRQoL) in Primary Sclerosing Cholangitis (PSC).

Objectives:

We aimed to assess Bone Mineral Density (BMD), physical activity and incidence of fragility fractures in patients with PSC. We also sought associations between prior fractures and HRQoL.

Patients and Methods:

The study was performed on 33 patients (11 females, 22 males) aged 35.3 ± 13 years. HRQoL was assessed by Short Form (SF)-36, Primary Biliary Cirrhosis (PBC)-40 and PBC-27 questionnaires. BMD was measured by densitometry in the lumbar spine and hip. Physical activity was assessed by questionnaire.

Results:

In 32% of patients, BMD measured in the hip or spine was below 1.0 Standard Deviation. A history of fragility fractures (distal forearm and ribs) was reported in six patients (18%). In SF-36 assessment, patients with fractures had lower scores in the role functioning, general health and vitality domains and Physical Component Summary (PCS) than those without fractures. Prior fractures adjusted for gender and PSC duration were associated with lower PCS and Mental Component Summary (MCS) scores. Symptoms and fatigue (assessed by PBC) and prior fractures were inversely associated with MCS (P = 0.007).

Conclusions:

In middle-aged subjects with PSC, we found a high rate of non-vertebral fractures and a moderately decreased BMD in lumbar spine and hip. Fragility fractures had an impact on physical and mental aspects of HRQoL.  相似文献   

2.

Background

The clinical outcome and health-related quality of life (HRQoL) of living kidney donors is mostly not detrimental, but some donors experience impairment after donation. Gender-specific effects of living kidney donors was evaluated.

Methods

Clinical outcome was assessed in living kidney donors and HRQoL was obtained by self-reporting validated test systems as the Multidimensional Fatigue Inventory (MFI-20), the Short Form 36 (SF-36), and the Patient Health Questionnaire (PHQ-9).

Results

Two hundred and eleven (211) living renal donors were evaluated (female 62.2%). Response rate was 80.8%. In both genders, a decrease of renal function of 26% was observed after donation. De novo antihypertensives were introduced in 28.3% of women and 36.5% of men. HRQoL was comparable in female and male donors, except for mental HRQoL, which was lower in 51- to 60-year-old female donors, compared to age-matched male donors and to the female general population. Female donors aged 40–59?years demonstrated more fatigue than the age-matched general population. A low mental HRQoL (MCS; SF-36) was associated with higher values for fatigue (General Fatigue Score; MFI-20) in both genders. Multiple regression analysis detected the General Fatigue score of the MFI-20 questionnaire and depression identified by the PHQ-9 score as independent variables predicting MCS of the SF-36 in both genders. Lower age at time of donation contributed to a lower MCS in female donors.

Conclusions

Overall, HRQoL in living kidney donors exceeds that of the general population. Inferior mental health status and fatigue seem to be a problem, especially in middle-aged female donors, but not in all female donors. Psychological evaluation pre donation and psychological support post donation are required.
  相似文献   

3.

Aim

This study aims to assess the health-related quality of life (HRQoL) in a Dutch population of patients with primary biliary cholangitis (PBC) in relation to the prognosis and need for second line-therapy, based on both objective disease parameters and patients’ perspectives.

Methods

In this cross-sectional multicenter study, HRQoL was assessed by using the Dutch PBC-40 according to objective clinical parameters and patients’ perspectives on treatment and prognosis.

Results

In total, 178/269 (66%) patients responded; mean age 61.2 (SD 9.9) years and 165 (92.7%) women. The PBC-40 domain scores did not differ according to the GLOBE score response (p > 0.05 for all) or according to the POISE criteria (p > 0.05), except for the domain itch (p = 0.031). Patients who considered their survival to be impaired scored higher on all domains as compared to those expecting a normal prognosis (p < 0.05). Similarly, PBC-40 domain scores were higher among patients who considered that they were in need of additional therapy compared to those who did not (p < 0.05 for all, except for domain itch [p = 0.056]). However, 45/62 (72.6%) patients with a self-expected impaired prognosis had a GLOBE score indicative of a normal prognosis. Twenty-five of the 40 (62.5%) patients who believed they needed additional therapy were below POISE criteria.

Conclusion

The HRQoL of patients with PBC was impaired in terms of nonfavorable disease status according to the expectations of patients, but not according to objective disease parameters. Substantial discrepancies between patients’ perspectives and objective parameters were observed, which highlights the need for better patient guidance among patient with PBC.  相似文献   

4.
Background and AimsPrimary biliary cholangitis (PBC) is a chronic liver disease that negatively affects the health-related quality of life (HRQoL) of patients. Furthermore, the HRQoL of Chinese patients has been neglected for a long time. The present study aimed to assess the HRQoL of Chinese patients with PBC and explore the clinical variables correlating to the improvement of itch and fatigue.MethodsThis was an observational, cross-sectional study. The PBC-40 and itch numerical rating scales were used to evaluate the symptoms and HRQoL of patients.ResultsA total of 383 patients were recruited, and 86.4% were female, with a median age of 55 years (range: 49–63 years). We found that females had significantly higher scores than males in symptoms (p=0.033) and cognitive domains (p=0.021), and the fatigue domain was higher in elderly patients (p=0.007). Meanwhile, patients whose body mass index was <18.5 had the highest scores in the symptoms (p=0.009), fatigue (p=0.010), and cognitive (p=0.019) domains. Age at participation (odds ratio [OR]=1.068, p=0.015) and albumin level at 12 months after ursodeoxycholic acid treatment (OR=208.807, p=0.025) were independent factors that affected the improvement of the itch and fatigue domains, respectively.ConclusionsThe HRQoL of Chinese patients with PBC was significantly impaired depending on sex, age, and body mass index. Age and albumin level were significantly associated with the improvement of itch and fatigue, respectively. Therefore, treatment and support aimed at these two factors can be provided to improve the HRQoL of patients.  相似文献   

5.

Background

Cardiovascular patients are likely to have an impaired health-related quality of life (HRQoL) due to functional and psycho-social limitations. The main objective of this study was to assess the distribution of HRQoL scores in coronary heart disease (CHD) patients across 22 European countries and to identify factors associated with the variation between patients.

Methods

Data from the EUROASPIRE III survey (European Action on Secondary and Primary Prevention by Intervention to Reduce Events), on 8734 patients, were used. Patients with a diagnosis of CHD (coronary artery bypass graft (CABG), percutaneous coronary intervention (PCI), acute myocardial infarction (AMI) or myocardial ischemia) were interviewed and examined at least 6 months after their acute coronary event. Quality of life of each patient was measured using 2 standardized questionnaires: the EuroQoL-5D (EQ-5D) and the 12-item short-form health survey (SF-12v2).

Results

HRQoL values differed significantly across countries. Lower HRQoL estimates were found in women, older patients, less educated patients, patients with myocardial infarction or ischemia as recruiting diagnosis, patients with a history of stroke and patients who suffered from a recurring CHD event. In addition, HRQoL was significantly associated with current smoking, central obesity, lack of exercise and inappropriate HbA1c control in patients with diabetes. Furthermore the number of risk factors is inversely associated with HRQoL.

Conclusion

Overall, a large heterogeneity was observed in HRQoL values between countries and patient groups. There seems to be a significant association between quality of life and patient characteristics with lifestyle risk factors as important determinants of HRQoL.  相似文献   

6.
A significant proportion of patients with primary biliary cirrhosis (PBC) suffer from severe fatigue. The aim of this study was to characterize patterns of daytime sleep in patients with PBC (using both objective and subjective assessment approaches) and to study the association between sleep abnormality and fatigue severity. Fatigue severity was assessed in 48 female subjects with PBC (using a disease-specific quality of life instrument (the PBC-40) and a generic fatigue measure (Fatigue Impact Scale [FIS]) as well as 48 case-matched normal controls. All participants also completed the Pittsburgh Sleep Quality Index (PSQI) and the Epworth Sleepiness Scale (ESS, which assesses daytime hypersomnolence). Objective sleep assessment was performed using accelerometry over 7 days. Global sleep quality assessed by the PSQI was significantly lower in the PBC group compared to controls (P < .0001). ESS scores were significantly higher in patients with PBC than controls (P = .0001), suggesting significantly greater daytime somnolence in the patients with PBC. Objective sleep assessment confirmed that subjects with PBC were sleeping on average almost twice as long as controls during the daytime. Both degree of daytime somnolence (ESS) and actual daytime sleep activity (accelerometry) correlated strongly with fatigue severity in the patient group (r2 = 0.5, P < .0001 and r2 = 0.2, P < .01, respectively). In conclusion, Sleep abnormality, in the form of excessive daytime somnolence, is present in a significant proportion of patients with PBC, with the degree of daytime somnolence correlating strongly with the degree of fatigue. Existing agents effective at reducing daytime somnolence (such as modafinil) hold potential for the treatment of fatigue in PBC.  相似文献   

7.

Objective

The assessment of health-related quality-of-life (HRQoL) in rheumatoid arthritis (RA) is becoming increasingly common in both research and clinical practice. One of the most widely used tools for measuring HRQoL is the Medical Outcomes Study 36-item Short-Form Health Survey (SF-36). We conducted a systematic review examining the impact of RA on HRQoL, measured through the SF-36.

Methods

MEDLINE and Embase were searched for observational studies reporting mean and standard deviation scores for each domain of the SF-36 in adult RA patients. Studies were reviewed in accordance with PRISMA guidelines, and a random-effects meta-analysis was performed.

Results

In total, 31 studies were eligible for inclusion in the meta-analysis, including 22,335 patients. Meta-analyses found that pooled mean HRQoL score for the SF-36 physical component summary was 34.1 (95% CI: 22.0–46.1) and mental component summary was 45.6 (95% CI: 30.3–60.8). Increased age was associated with reduced physical function and physical component summary (PCS) scores but improved mental health and mental component summary (MCS) scores. Female gender was associated with improved scores on role physical, bodily pain and PCS but reduced mental health and MCS scores. Longer disease duration was associated with improved MCS. Patients with RA have a substantially reduced HRQoL in comparison to both other physical illnesses and in comparison to normative datasets from UK and USA populations.

Conclusions

RA has a substantial impact on HRQoL. This supports recent NICE guidelines stipulating that RA patients should be regularly assessed for the impact their disease has on HRQoL and appropriate management provided.  相似文献   

8.

Background

Health-related quality of life (HRQoL) values shortly after pacemaker (PM) implantation for bradycardia have been established, however little is known about long-term HRQoL.

Methods

Using the generic SF-36 and the PM specific Aquarel questionnaire, HRQoL was repeatedly measured during a 7.5 year follow-up period in 881 bradycardia PM recipients included in the large scale nationwide Dutch FOLLOWPACE study. HRQoL over time, corrected for age, gender, diabetes, hypertension, heart failure, cardiovascular disease and AV-synchrony, was assessed with a linear mixed model.

Results

Increased scores both on overall SF-36 and on all SF-36 subscales were observed shortly after implantation. Although scores on SF-36 gradually declined over time, scores remained improved over the measured pre-implantation values. Also, scores for almost all subscales remained increased throughout the 7.5 year observation period, except for physical functioning which showed a gradual decline several years after the initial rise.Additionally, higher scores on all Aquarel scales were observed after implantation. Scores on the arrhythmias and chest discomfort subscales improved and remained stable throughout follow-up (FU), whereas the dyspnea at exertion subscale showed a gradual decline during FU to reach pre-implantation values at 5 years.

Conclusions

Increased HRQoL is observed not only shortly after PM implantation, but also after long-term FU.

Clinical Trial Registration

ClinicalTrials.gov Identifier: NCT00135174; http://www.clinicaltrials.gov/ct2/show/NCT00135174.  相似文献   

9.
The true impact of fatigue in primary biliary cirrhosis: a population study   总被引:8,自引:0,他引:8  
BACKGROUND & AIMS: Patient surveys suggest that fatigue is a common problem in primary biliary cirrhosis (PBC). The actual extent of the problems caused by fatigue in PBC has yet to be determined as previous studies addressing this question have tended to use selected patient subgroups and subjective or non-quantitative fatigue assessment tools. Here, we have attempted to more accurately assess the extent of fatigue in PBC, and the specificity of the symptom for this disease, by the application of an objective measure of fatigue impact (the fatigue impact score [FIS]) to a geographically based patient cohort, age- and sex-matched normal controls, and chronic liver disease controls. METHODS: Postal completion of the FIS and linked symptom assessment tools. RESULTS: Median FIS was significantly higher in patients (n = 136) than community controls (40 [0-138] vs. 28 [0-156]; P < 0.0001) and chronic liver disease controls (n = 38) (20.5 [0-145]; P < 0.05). Fatigue scores in the 11 patients who had undergone liver transplantation (median 3.5 years previously) were the same as those in non-transplanted patients with advanced disease. CONCLUSIONS: Fatigue is a significant and specific problem in PBC. It is not, however, universal and affects fewer patients than has previously been thought to be the case based on data from selected patient cohorts. This definition of the "normal range" for fatigue in PBC will assist in future studies of etiology and therapy.  相似文献   

10.

Objective

To multidimensionally assess fatigue in rheumatoid arthritis (RA) and to evaluate the impact of fatigue on health‐related quality of life (HRQOL).

Methods

The study was conducted in 1999 among 490 RA patients with varying disease duration. Fatigue was measured with the Multidimensional Fatigue Inventory (MFI‐20) and HRQOL with a validated Dutch version of the RAND 36‐Item Health Survey. We evaluated the impact of fatigue on HRQOL by multiple linear regression analyses taking into account RA‐related pain and depressive symptoms.

Results

Different aspects of fatigue selectively explained different dimensions of HRQOL. The MFI‐20 was entered last to the linear regression models, resulting in an additional increase of explained variance of 1% (mental health) to 14% (vitality).

Conclusion

The multidimensional portrayal of RA‐related fatigue can be used to develop intervention strategies targeted to specific aspects of fatigue. Fatigue, supplementary to RA‐related pain and depressive symptoms, appears to be a feasible and treatable target in the clinical management of RA to increase HRQOL.
  相似文献   

11.
Jacoby A  Rannard A  Buck D  Bhala N  Newton JL  James OF  Jones DE 《Gut》2005,54(11):1622-1629
BACKGROUND AND AIMS: Study of health related quality of life (HRQOL) and the factors responsible for its impairment in primary biliary cirrhosis (PBC) has, to date, been limited. There is increasing need for a HRQOL questionnaire which is specific to PBC. The aim of this study was to develop, validate, and evaluate a patient based PBC specific HRQOL measure.Subjects and METHODS: A pool of potential questions was derived from thematic analysis of indepth interviews carried out with 30 PBC patients selected to represent demographically the PBC patient population as a whole. This pool was systematically reduced, pretested, and cross validated with other HRQOL measures in national surveys involving a total of 900 PBC patients, to produce a quality of life profile measure, the PBC-40, consisting of 40 questions distributed across six domains. The PBC-40 was then evaluated in a blinded comparison with other HRQOL measures in a further cohort of 40 PBC patients. RESULTS: The six domains of PBC-40 relate to fatigue, emotional, social, and cognitive function, general symptoms, and itch. The highest mean domain score was seen for fatigue and the lowest for itch. The measure has been fully validated for use in PBC and shown to be scientifically sound. PBC patient satisfaction, measured in terms of the extent to which a questionnaire addresses the problems that they experience, was significantly higher for the PBC-40 than for other HRQOL measures. CONCLUSION: The PBC-40 is a short easy to complete measure which is acceptable to PBC patients and has significantly greater relevance to their problems than other frequently used HRQOL measures. Its scientific soundness, shown in extensive testing, makes it a valuable instrument for future use in clinical and research settings.  相似文献   

12.

Background/Aims

Many patients with inflammatory bowel disease (IBD) often complain of fatigue. To date, only a few studies in Western countries have focused on fatigue related to IBD, and fatigue has never been specifically studied in Asian IBD patients. The aim of the present study was to investigate the fatigue level and fatigue-related factors among Korean IBD patients.

Methods

Patients in remission or with mild to moderate IBD were included. Fatigue was assessed using the Functional Assessment of Chronic Illness Therapy-Fatigue and the Brief Fatigue Inventory. Corresponding healthy controls (HCs) also completed both fatigue questionnaires.

Results

Sixty patients with Crohn disease and 68 patients with ulcerative colitis (UC) were eligible for analysis. The comparison group consisted of 92 HCs. Compared with the HCs, both IBD groups were associated with greater levels of fatigue (p<0.001). Factors influencing the fatigue score in UC patients included anemia and a high erythrocyte sedimentation rate (ESR).

Conclusions

Greater levels of fatigue were detected in Korean IBD patients compared with HCs. Anemia and ESR were determinants of fatigue in UC patients. Physicians need to be aware of fatigue as one of the important symptoms of IBD to better understand the impact of fatigue on health-related quality of life.  相似文献   

13.
BACKGROUND AND AIMS: Fatigue is considered to be a specific manifestation of primary biliary cirrhosis (PBC). Recent reports have, however, questioned these findings. Considering the high rate of comorbidities in PBC patients and the fact that fatigue is a multifactorial symptom, we hypothesized that it might also be due to nonhepatic causes. Our aim was to evaluate fatigue in PBC patients and its relationship with comorbidities and depression. METHODS: We enroled 49 Italian PBC patients (44 women; mean age: 58.9 years, range: 21-73 years) and 30 matched healthy controls, who completed the Fatigue Impact Scale (FIS), Modified FIS (MFIS), Fatigue Severity Score (FSS) and Rand Medical Outcomes Study Depression Screener. Comorbidities and several clinical and biochemical data were investigated. Linear regression, analysis of variance and post-hoc analysis were applied. RESULTS: Fatigue was higher in patients than in controls (FIS: 33 vs. 24; MFIS: 24 vs. 14; FSS: 3.3 vs. 1.9). Physical domain was significantly different in all the three questionnaires (FIS: P=0.05; MFIS: P=0.002; FSS: P=0.0002). Comorbidities (38% of patients) were independently associated with higher fatigue scores (FIS: 45; MFIS: 32; FSS: 3.3). Depressed patients (30%) were more fatigued, even if not always significantly (FIS: 43; MFIS: 29; FSS: 3.5), than controls and patients with no depression. Patients without comorbidities or depression (51%) did not have higher fatigue than controls (FIS: 20; MFIS: 17; FSS: 2.4). CONCLUSIONS: Fatigue in patients with PBC was higher, but not always significantly, than in healthy controls. Comorbidities and depression might have played a role in its pathogenesis. Our data arouse doubts about the specificity of fatigue in PBC and the pathogenetic role of liver impairment.  相似文献   

14.

Objective

To develop a conceptual definition of quality of life (QoL) with a left ventricular assist device (LVAD).

Background

Conceptual and operational definitions of QoL with an LVAD are lacking.

Methods

A grounded theory method was used. Adult, outpatient LVAD recipients (n = 11) participated twice in individual or paired interviews.

Results

A conceptual definition of QoL while living with an LVAD was established as: “Being well enough to do and enjoy day-to-day activities that are important to me.” Participants described 5 important life domains consistent with QoL literature: physical, emotional, social, cognitive, and spiritual/meaning. However, participants identified unique concerns not addressed by generic or heart failure disease specific measures typically used in the LVAD population.

Conclusion

Existing generic and heart-failure specific QoL measures are not adequate for understanding QoL among LVAD patients. Cognition and spiritual/meaning domains were significant; these need inclusion for comprehensive QoL assessment in the LVAD population.  相似文献   

15.
Background: Fatigue was recently suggested to predict an increased risk of mortality in a primary biliary cirrhosis (PBC) cohort during follow‐up. Aims: To analyse the impact of fatigue on prognosis in PBC. Methods: Patients with PBC who had earlier completed the fatigue impact scale (FIS) were identified. Prognosis in terms of death and liver transplantation (Tx) was determined. Results: FIS values at baseline were analysed from 208 patients (192 females; median age 59 years (interquartile range 51–67), median follow‐up of 5 years. Overall, 181 patients were alive at follow‐up, 22 (12%) died and five (2.4%) underwent transplantation. FIS at baseline was 28 (12–47) and FIS at follow‐up was 25 (8–64) (P<0.001; r=0.69). Among survivors, FIS at baseline was 27 (12–43), 36 (12–72) in those who died (P=0.059) and 99 (41–102) in those who underwent transplantation (P=0.0008). FIS at baseline was 44 (12–88) in patients with death and/or Tx vs. 27 (12–43) in survivors (P=0.003). Age [hazard ratio (HR) 1.1 (confidence interval (CI) 1.0–1.2)] and aspartate aminotransferase [HR 2.0 (CI 1.3–3.0)] were independently associated with decreased survival on multivariate analysis. FIS scores over 40 [HR 9.6 (CI 2.3–39.7)] and bilirubin [HR 4.8 (CI 2.8–8.2)] were independently associated with a poor outcome in patients who underwent Tx or had a liver‐related death. Conclusions: Fatigue seems to change little over time in PBC. Fatigue levels were higher at baseline in those who died or underwent Tx. High fatigue levels seem to be a predictor of risk of liver‐related mortality and need for transplantation over time but not a predictor of non‐liver‐related mortality.  相似文献   

16.
BACKGROUND: Fatigue has been reported to be common in patients with primary biliary cirrhosis (PBC). Limited data exist on comparison with fatigue in the general population and comparison with patients with other chronic gastrointestinal disorders are lacking. METHOD: We enrolled 96 patients with PBC (87 females); mean age 63 (range 34-65) who completed the Fatigue Impact Scale (FIS). In comparison we included matched controls from the general population, patients with organic (OGD) and functional GI disorders (FGD). Liver function test and the latest liver biopsy were analysed and correlated with fatigue scores. RESULTS: The mean duration of PBC was 7.4 years, the mean bilirubin 13 micromol/l. Twelve per cent of patients had cirrhosis, 29% were in stage I on Ludwig's histology score and 30% and 29% were in stages II and III, respectively. The PBC patients had a median FIS total score of 29 in comparison with 38 in GP controls (P<0.05). Patients with OGD and FGD had more severe fatigue (FIS total score 67 and 59 (P<0.01 compared with PBC)). Fatigue in the PBC patients did not correlate with liver tests and histology stage. CONCLUSION: PBC patients had less severe fatigue measured with the FIS than controls from the GP and patients with OGD and FGD. This study also confirms results of other studies showing no correlation with fatigue in PBC and liver disease parameters. These results argues strongly against fatigue as a specific symptom in PBC.  相似文献   

17.
Fatigue impairs the quality of life of primary biliary cirrhosis (PBC) patients. In this study, we explored the psychological factors and coping strategies in fatigued PBC patients. Patients participated in a semi-structured interview examining thoughts regarding the impact of fatigue and coping strategies. All completed the disease-specific quality-of-life tool, PBC-40, the Penn State Worry Questionnaire (PSWQ) (degree of habitual worry) and Hospital Anxiety and Depression Scale (HADS) (current anxiety and depression). PBC patients were allocated into high (>38, n=10) and low-fatigue (<38, n=14) groups. No differences were seen between high-fatigue and low-fatigue groups regarding age, marital status, employment status, PBC stage, years with diagnosis and years experiencing fatigue. High-fatigue participants were significantly more anxious (P=0.008), more depressed (P<0.001), and more likely to worry (<0.05). High-fatigue participants had more frequent thoughts about the impact of fatigue (P<0.005) and lower self-efficacy scores (P<0.001). In conclusion, PBC patients can experience profound distress associated with fatigue. PBC patients with high levels of fatigue seem to be more vulnerable to emotional distress, more likely to perceive that their quality of life has been negatively affected and are less confident to engage in everyday activities compared with those with low levels of fatigue.  相似文献   

18.

Objectives

To explore disparities between non-Hispanic Blacks and non-Hispanic Whites presenting to the emergency department (ED) with potential acute coronary syndrome (ACS).

Background

Individuals with fewer resources have worse health outcomes and these individuals are disproportionately those of color.

Methods

This prospective study enrolled 663 patients in four EDs. Clinical presentation, treatment, and patient-reported outcome variables were measured at baseline, 1, and 6 months.

Results

Blacks with confirmed ACS were younger; had lower income; less education; more risk factors; more symptoms, and longer prehospital delay at presentation compared to Whites. Blacks experiencing palpitations, unusual fatigue, and chest pain were more than 3 times as likely as Whites to have ACS confirmed. Blacks with ACS had more clinic visits and more symptoms 1 month following discharge.

Conclusions

Significant racial disparities remain in clinical presentation and outcomes for Blacks compared to Whites presenting to the ED with symptoms suggestive of ACS.  相似文献   

19.

Background

Measures of health related quality of life (HRQoL) in patients with α1-antitrypsin deficiency (AATD) can help to determine the impact of the disease and provide an important insight into the intervention outcomes. There is few data regarding this issue in the literature. The aim of this study is to assess the relationship between HRQoL and gender, functional parameters and history of hospitalizations in patients with AATD.

Methods

This is a cross-sectional study of 26 patients with severe AATD recruited in the pulmonology outpatient clinic at a tertiary care medical center. Social-demographic, clinical and functional parameters were recorded and HRQoL was assessed with the Portuguese version of the medical outcome study short form-36 (SF-36) self-administered questionnaire.

Results

Older patients, females and patients with at least one hospitalization in the previous year due to respiratory disease had statistical lower scores in some dimensions of the SF-36 questionnaire. Superior FEV1 and higher distance mark in the 6-min walking test distance influenced positively several dimensions of the questionnaire. Higher scores in the mMRC scale influenced negatively the HRQoL.

Conclusions

These data suggests that older and female patients with AATD have worse HRQoL. Hospitalizations and functional markers of respiratory disease progression influenced negatively the HRQoL, suggesting that the SF-36 questionnaire could be useful as an outcome for AATD patients with lung involvement.  相似文献   

20.
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