Assessment of health related quality of life in polish patients with primary biliary cirrhosis

Raszeja‐Wyszomirska, Joanna; Krawczyk, Marek; Rigopoulou, Eirini I.; Kostrzewa, Konrad; Norman, Gary L.; Bogdanos, Dimitrios P.; Milkiewicz, Piotr

doi:10.1016/j.clinre.2015.10.006

Cited by 43 publications

(49 citation statements)

References 43 publications

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“…The neuroimaging abnormalities suggest that the brain changes seen in PBC occur early in the pathological process, even before significant liver damage has occurred [24,25]. Cognitive impairment in PBC patients was previously reported by groups of Raszeja-Wyszomirska et al [26] and Newton et al [27] as the symptom independent of liver disease severity and associated with poorer performance on objective cognitive testing. Cognitive impairment was, in turn, associated with structural brain lesions and autonomic dysfunction, which might predict risk of cognitive decline in the study of Newton et al [27] Brain perfusion scintigraphy provides dynamic, functional measures of brain function based on regional cerebral blood flow and tracer accumulation in brain grey matter compared with the static, structural data provided by conventional computerized axial tomography or magnetic resonance imaging (MRI).…”

Section: Discussionmentioning

confidence: 58%

Brain Perfusion Scintigraphy in Evaluation of Pathogenesis of Fatigue in Patients with Primary Biliary Cholangitis (PBC) – A Pilot Study

Raszeja‐Wyszomirska¹,

Zorga²,

Kotulski³

et al. 2017

IJCEMS

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Primary biliary cholangitis (PBC) is an autoimmune cholestatic liver disease characterized by a breakdown of immune tolerance to mitochondrial and nuclear antigens, causing injury to the biliary epithelial cells. Fatigue is the commonest reported symptom in PBC and has a negative impact on patients' perceived quality of life, often through social isolation. It is unrelated to the severity of liver disease and appears unresponsive to current therapies, including ursodeoxycholic acid and transplantation. Fatigue in PBC is complex, with numerous associated peripheral and central nervous system (CNS) features. Initially, cholestasis causes degenerative CNS change affecting areas of the brain regulating autonomic dysfunction and sleep, and these changes lead directly to some manifestations of fatigue and the associated cognitive impairment. The aim of the study was to examine global cerebral blood flow with brain perfusion scintigraphy SPECT in well-defined group of PBC Caucasian patients with verbally reported fatigue. Twenty consecutive PBC female patients (median age 58.9, ranges 38-80; 4 cirrhotic) with mean duration of the disease 3.3 years, were prospectively enrolled into the study. Fatigue Impact Scale (FIS) questionnaire was administered to every patients at the moment of brain examination. Brain perfusion scintigraphy SPECT was performed after intravenous injection of 760-800 MBq of technetium 99m labeled exametazime ( 99m Tc -HMPAO). Then patients were examined using double head gamma camera system, and data were analyzed with dedicated nuclear medicine software. In analyzed cohort the median FIS score was 70.5 points (ranges 21-160), which was higher than previously reported. There were no correlation between age of patients at the SPECT/FIS examination, duration of the disease, the presence of liver cirrhosis, Mayo Risk Score, and FIS domains: Cognitive, Physical and Social, as well as with brain blood flow. However, positive correlation between Cognitive dimension of FIS measure and right frontal lobe perfusion impairment assessed with SPECT technic (p<0.05) was found. The results of this study showed that right lobe perfusion impairment might impact brain function in PBC. Cognitive dimension was stage-independent symptom in analyzed cohort, and cognitive impairment might be, in turn, associated with functional brain lesion.

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Section: Discussionmentioning

confidence: 58%

Brain Perfusion Scintigraphy in Evaluation of Pathogenesis of Fatigue in Patients with Primary Biliary Cholangitis (PBC) – A Pilot Study

Raszeja‐Wyszomirska¹,

Zorga²,

Kotulski³

et al. 2017

IJCEMS

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“…Our study sample had similar demographic and clinical characteristics to other PBC cohorts [12, 13], with the exception that only 20% of our cohort had biopsy proven cirrhosis (histological stage 4), less than the number seen in a recent Polish cohort [13]. …”

Section: Discussionmentioning

confidence: 70%

“…To meet this necessity, Jacoby et al [11], created the first disease specific quality of life scale for PBC, the PBC-40, which was evaluated and found to have appropriate validity and reliability. The PBC-40 has since been cross-culturally adapted and validated into different languages [12, 13]; alongside the creation of the shorter PBC-27 [12]. …”

Section: Introductionmentioning

confidence: 99%

Translation and validation of the Serbian primary biliary cholangitis-40 questionnaire

Zec

Popović

Matovic³

et al. 2017

PLoS ONE

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Background and aimsTo translate into Serbian and validate the Primary Biliary Cholangitis–40 (PBC-40) and PBC-27 questionnaires.Materials and methodsNinety-four consecutive outpatients with the diagnosis of PBC from three departments across two tertiary care institutions in Belgrade were enrolled from February to October 2016. Standard methodology for cultural adaption of healthcare related quality of life questionnaires was used, and included: a forward translation, backward translation and a pilot test of the Serbian PBC-40 on five patients who gave suggestions and comments. For evaluation of the questionnaires, acceptance was shown by the proportion of missing items and the internal consistency was assessed using Cronbach’s α coefficient. The PBC-40 was self-administered under the supervision of an experienced hepatologist. The PBC-27 is a shorter version of the PBC-40.ResultsA total of 92 (97.9%) of the patients were females. The mean age was 59.26 ± 1.05 years and the average length of disease was 60.45 ± 48.314 months. The average PBC-40 score was 85.62 ± 30.46. The total time needed to complete the questionnaire ranged from 7 to 16 minutes. The proportion of missing items was 5.45% (205/3760). Cronbach’s α for the entire scale was 0.93. Reliability for all of the domains of the PBC-40 was above 0.70, except for the domain “Symptoms” (α = 0.52). Overall reliability of the PBC-27 was α = 0.90. Domains “Dryness”, “Symptoms” and “Fatigue” demonstrated reliability below α = 0.70.ConclusionsThe Serbian PBC-40 is a valid and reasonably adequate for use in Serbian PBC patients. The PBC-40 is preferred over the PBC-27.

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“…We have recently published that patients with PBC have impaired HRQoL, irrespectively of whether they are cirrhotic or not [18]; thus an association between the analyzed polymorphisms and HRQoL measures depicted by the PBC-40 or PBC-27 questionnaires would be rather unlikely; this appears to be the case, as our analysis failed to identify significant associations.…”

Section: Discussionmentioning

confidence: 73%

“…Clinical and laboratory features of analyzed subjects are summarized in Table 1. Health-related quality of life (HRQoL) was assessed with disease-specific questionnaires (PBC-40 and PBC-27) [18]. PBC-specific AMA were tested by ELISA (Inova Diagnostics, San Diego, CA, USA).…”

Section: Methodsmentioning

confidence: 99%

Polymorphisms of IL12RB2 May Affect the Natural History of Primary Biliary Cholangitis: A Single Centre Study

Wasik

Norman

Rigopoulou

et al. 2017

Journal of Immunology Research

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Background. Recent GWAS in primary biliary cholangitis (PBC) showed strong associations with SNPs located within interleukin-12 receptor (IL12R) beta-2 (IL12RB2) gene. Aims. We assessed whether genetic variation of IL12RB2 is associated with laboratory and clinical features of PBC. Methods. Genomic DNA was isolated from 306 patients with PBC and 258 age/gender-matched controls. PBC-specific anti-mitochondrial antibodies (AMA) were tested in all subjects by ELISA. Two SNPs, rs3790567 and rs6679356, of IL12RB2 were genotyped using the MGB-TaqMan SNP assay. Results. Despite comparable age at diagnosis of cirrhotic and noncirrhotic PBC patients, allele A of rs3790567 and allele C of rs6679356 were overrepresented in the former rather than the latter group (p = 0.0009 and p = 0.002, resp.). The risk of cirrhosis at presentation increased when allele A and allele C coexisted. AMA-M2 titres were significantly higher in AA homozygotes of rs3790567 compared to GG homozygotes (132 ± 54 versus 103 ± 62, p = 0.02) and in rs6679356 when C allele was present (p = 0.038). There were no other significant associations between IL12RB2 polymorphisms and laboratory or clinical features. Conclusion. In this first study analyzing phenotypic features of PBC carriers of the IL12RB2 polymorphisms, we found that carriers are more frequently cirrhotic at diagnosis and have significantly higher titres of AMA.

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Assessment of health related quality of life in polish patients with primary biliary cirrhosis

Cited by 43 publications

References 43 publications

Brain Perfusion Scintigraphy in Evaluation of Pathogenesis of Fatigue in Patients with Primary Biliary Cholangitis (PBC) – A Pilot Study

Brain Perfusion Scintigraphy in Evaluation of Pathogenesis of Fatigue in Patients with Primary Biliary Cholangitis (PBC) – A Pilot Study

Translation and validation of the Serbian primary biliary cholangitis-40 questionnaire

Polymorphisms of IL12RB2 May Affect the Natural History of Primary Biliary Cholangitis: A Single Centre Study

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