Cross-cultural adaptation and validation of the CFAbd-Score for gastrointestinal symptoms in patients with cystic fibrosis.

OBJECTIVE: Translating and cross-culturally adapting the CFAbd-Score, Cystic Fibrosis (CF) Abdominal Score, to use in Brazilian spoken Portuguese. The CFAbd-Score is a questionnaire for assessing CF-related abdominal symptoms and their influence on the quality of life (QoL). It comprises 28 questions on five domains: abdominal pain, bowel movements, eating and appetite, gastroesophageal reflux symptoms, and the impact of gastrointestinal (GI) symptoms on QoL. METHOD: Cross-cultural adaptation included assessment of conceptual and item equivalence, semantic, operational, and measurement equivalence. Content validity was assessed. The validation and psychometric analysis phase included 97 people with CF (pwCF), median age:14.58y (IQR 9/19), and 105 healthy individuals, 15.10y (IQR 9/20). Exploratory factor analysis (FA) identified retained factors. Internal consistency of the extracted domains was evaluated using Cronbach's α, and the Kaiser-Meyer-Olkin test (KMO) was used to check the sample adequacy. Bartlett's test tested the null hypothesis that the correlation matrix is an identity matrix. RESULTS: All items were considered relevant to the construct and good semantic equivalence of the version was recognized. FA showed the appropriate weight of all items and good internal consistency, with Cronbach's alpha 0.89. Bartlett's test significance level (p < 0.001) and KMO coefficient of 0.72 indicated good adequacy for structure. Internal consistency coefficients (Cronbach's alpha) were good for abdominal pain: 0.84; abdominal bloating: 0.73; flatulence: 0.76; heartburn: 0.81, and low for reflux: 0.54. CONCLUSION: The CFAbd-Score was adapted to the Brazilian spoken Portuguese and demonstrated content and semantic equivalence. The final version showed appropriate validity, and internal consistency, preserving the psychometric properties of the original version.

Prevalence of constipation in cystic fibrosis patients: a systematic review of observational studies / Prevalência de constipação em pacientes com fibrose cística: uma revisão sistemática de estudos observacionais

Abstract Objective: To systematically revise the literature in search of data about the prevalence of constipation in patients with cystic fibrosis according to the publications in this field, which partly refer to guidelines defined in 2010 by the European Society for Pediatric Gastroenterology, Hepatology, and Nutrition. Sources: Systematic review selecting articles based on the Preferred Reporting Items for Systematic Reviews and Meta-Analyses, including Cystic Fibrosis patients of all ages. Sources of information were selected to identify the articles without period limitation: CADTH - Canadian Agency for Drugs and Technologies in Health, CINAHL Complete, Clinical Trials US NIH, Cochrane Library, Embase, MEDLINE via Ovid, Scopus, Web Of Science, PubMed, SciELO, MEDLINE and LILACS , Health Systems Evidence, PDQ Evidence, CRD Canadian Agency for Drugs and Technologies in Health, INAHTA - International Network of Agencies for Health Technology Assessment, and PEDro. Findings: The prevalence of constipation was reported in eight observational studies. Only two studies assessed the frequency of constipation as a primary objective; in the others, constipation was quoted along with the prevalence of the spectrum of gastrointestinal manifestations. Altogether, the publications included 2,018 patients, the reported prevalence varied from 10% to 57%. Only two of the six articles published after 2010 followed the definition recommended by the European Society. Conclusions: Constipation is a frequent but still insufficiently assessed complaint of Cystic Fibrosis patients. The use of diverse diagnostic criteria restricts comparison and epidemiological conclusions, future studies should compulsorily apply the European Society for Pediatric Gastroenterology, Hepatology, and Nutrition definition.

Resumo Objetivo Revisar sistematicamente a literatura em busca de dados sobre a prevalência de constipação em pacientes com fibrose cística (FC), de acordo com as publicações nesse campo, que se referem parcialmente às diretrizes definidas pela European Society for Paediatric Gastroenterology Hepatology and Nutrition (ESPGHAN, 2010). Fontes de dados Revisão sistemática, selecionaram-se artigos com base no Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA), incluindo todos os pacientes com FC de todas as faixas etárias. As fontes de informação foram selecionadas para identificar os artigos sem limitação de período para a pesquisa: CADTH (Canadian Agency for Drugs and Technologies in Health), CINAHL Complete, Clinical Trials US NIH, Cochrane Library, Embase, Medline via Ovid, Scopus, Web of Science, PubMed, SciELO, Medline e Lilacs por meio da Biblioteca Virtual em Saúde (BVS), Health Systems Evidence, PDQ Evidence, CRD (Canadian Agency for Drugs and Technologies in Health), INAHTA (International Network of Agencies for Health Technology Assessment) e PEDRO. Achados A prevalência de constipação em pacientes com FC foi relatada em oito estudos observacionais. Apenas dois estudos avaliaram a frequência de constipação como objetivo primário; nos outros, a constipação foi citada juntamente com a prevalência do espectro de manifestações gastrointestinais. No total, as publicações incluíram 2.018 pacientes e a prevalência relatada variou amplamente, de 10 a 57%. Apenas dois dos seis artigos publicados após 2010 seguiram a definição recomendada pela ESPGHAN. Conclusões A constipação é uma queixa frequente, mas ainda insuficientemente avaliada, dos pacientes com FC. O uso de diversos critérios diagnósticos restringe as comparações e declarações epidemiológicas, de modo que futuros estudos deveriam aplicar a definição ESPGHAN de maneira compulsória.

Prevalence of constipation in cystic fibrosis patients: a systematic review of observational studies.

OBJECTIVE: To systematically revise the literature in search of data about the prevalence of constipation in patients with cystic fibrosis according to the publications in this field, which partly refer to guidelines defined in 2010 by the European Society for Pediatric Gastroenterology, Hepatology, and Nutrition. SOURCES: Systematic review selecting articles based on the Preferred Reporting Items for Systematic Reviews and Meta-Analyses, including Cystic Fibrosis patients of all ages. Sources of information were selected to identify the articles without period limitation: CADTH - Canadian Agency for Drugs and Technologies in Health, CINAHL Complete, Clinical Trials US NIH, Cochrane Library, Embase, MEDLINE via Ovid, Scopus, Web Of Science, PubMed, SciELO, MEDLINE and LILACS , Health Systems Evidence, PDQ Evidence, CRD Canadian Agency for Drugs and Technologies in Health, INAHTA - International Network of Agencies for Health Technology Assessment, and PEDro. FINDINGS: The prevalence of constipation was reported in eight observational studies. Only two studies assessed the frequency of constipation as a primary objective; in the others, constipation was quoted along with the prevalence of the spectrum of gastrointestinal manifestations. Altogether, the publications included 2,018 patients, the reported prevalence varied from 10% to 57%. Only two of the six articles published after 2010 followed the definition recommended by the European Society. CONCLUSIONS: Constipation is a frequent but still insufficiently assessed complaint of Cystic Fibrosis patients. The use of diverse diagnostic criteria restricts comparison and epidemiological conclusions, future studies should compulsorily apply the European Society for Pediatric Gastroenterology, Hepatology, and Nutrition definition.

Antibody response against Pseudomonas aeruginosa and its relationship with immune mediators in the upper and lower airways of cystic fibrosis patients.

BACKGROUND: The upper airways (UAW) are a niche and a reservoir of Pseudomonas aeruginosa strains that cause chronic infection of the lower airways (LAW) in cystic fibrosis (CF). Here, we assessed the role of anti-P. aeruginosa immunoglobulin A (IgA) and IgG antibodies in upper and lower airway infections in cystic fibrosis patients. METHODS: Nasal lavage fluid and induced sputum samples of 40 CF patients were microbiologically cultured. We searched for correlations between anti-P. aeruginosa IgA and IgG levels, measured by enzyme-linked immunosorbent assay (optical density), and unspecific immune mediators in both specimens. RESULTS: Anti-P. aeruginosa IgA (median optical density: 0.953 vs 0.298) and IgG (0.120 vs 0.059) were significantly higher in nasal lavage than in sputum, but not significantly different between patients with and without chronic P. aeruginosa infection in UAW. Matrix metallopeptidase-9 (MMP-9) in nasal lavage and neutrophil elastase (NE) in sputum were predictors of IgA in nasal lavage and IgA in sputum, respectively. IgA was a predictor of myeloperoxidase (MPO) in nasal lavage. Tissue inhibitor of metalloproteinases-1 (TIMP-1) was a predictor of IgG in sputum. IgG, TIMP-1, and NE in sputum were predictors of IgG in nasal lavage. CONCLUSION: The anti-P. aeruginosa IgA response was more prominent in CF patients' UAW, indicating a lower degree of inflammatory responses. Proteases may play a role in the anti-P. aeruginosa humoral response in the upper and LAW, and anti-P. aeruginosa IgG may be involved in the crosstalk between upper and lower airways in cystic fibrosis patients.

Caregiver Burden Due to Pulmonary Exacerbations in Patients with Cystic Fibrosis.

OBJECTIVE: To describe the poorly understood burden of pulmonary exacerbations experienced by primary caregivers of children (aged 2-17 years) with cystic fibrosis (CF), who frequently require prolonged hospitalizations for treatment of pulmonary exacerbations with intravenous (IV) antibiotics. STUDY DESIGN: In this prospective observational study, 88 caregivers in Germany, Ireland, the United Kingdom, and the US completed a survey during pulmonary exacerbation-related hospitalizations (T1) and after return to a "well state" of health (T2). The impact of pulmonary exacerbations on caregiver-reported productivity, mental/physical health, and social/family/emotional functioning was quantified. RESULTS: Primary caregivers of children with CF reported significantly increased burden during pulmonary exacerbations, as measured by the 12-item Short-Form Health Survey mental health component and the Work Productivity and Activity Impairment: Specific Health Problem absenteeism, presenteeism, work productivity loss, and activity impairment component scores. Compared to the "well state," during pulmonary exacerbations-related hospitalization caregivers reported lower physical health scores on the Child Health Questionnaire-Parent Form 28. Quality-of-life scores on the Caregiver Quality of Life Cystic Fibrosis scale and total support score on the Multidimensional Scale of Perceived Social Support did not differ significantly between T1 and T2. More caregivers reported a negative impact on family/social/emotional functioning during pulmonary exacerbations than during the "well state." CONCLUSIONS: Pulmonary exacerbations necessitating hospitalization impose a significant burden on primary caregivers of children with CF. Preventing pulmonary exacerbations may substantially reduce this burden.