Interobserver Variance in Clinical Scoring for Cystic Fibrosis

Lewiston, Norman J.; Moss, Richard B.; Hindi, Robert; Rubinstein, Steven; Sullivan, Margaret M.

doi:10.1378/chest.91.6.878

Cited by 22 publications

(11 citation statements)

References 7 publications

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“…The height is generally less frequently compromised than the body weight, so its deficit is indicative of a serious and persistent malnutrition. Lung function was assessed by the quantity of air exhaled in one second during a forced breath (FEV1), expressed as a percentage of the theoretical value [12]. As a tool for assessing spirometry was used the Sensor Medics Vmax229.…”

Section: Methodsmentioning

confidence: 99%

Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Allelic Variants Relate to Shifts in Faecal Microbiota of Cystic Fibrosis Patients

et al. 2013

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IntroductionIn this study we investigated the effects of the Cystic Fibrosis Transmembrane conductance Regulator (CFTR) gene variants on the composition of faecal microbiota, in patients affected by Cystic Fibrosis (CF). CFTR mutations (F508del is the most common) lead to a decreased secretion of chloride/water, and to mucus sticky secretions, in pancreas, respiratory and gastrointestinal tracts. Intestinal manifestations are underestimated in CF, leading to ileum meconium at birth, or small bowel bacterial overgrowth in adult age.MethodsThirty-six CF patients, fasting and under no-antibiotic treatment, were CFTR genotyped on both alleles. Faecal samples were subjected to molecular microbial profiling through Temporal Temperature Gradient Electrophoresis and species-specific PCR. Ecological parameters and multivariate algorithms were employed to find out if CFTR variants could be related to the microbiota structure.ResultsPatients were classified by two different criteria: 1) presence/absence of F508del mutation; 2) disease severity in heterozygous and homozygous F508del patients. We found that homozygous-F508del and severe CF patients exhibited an enhanced dysbiotic faecal microbiota composition, even within the CF cohort itself, with higher biodiversity and evenness. We also found, by species-specific PCR, that potentially harmful species (Escherichia coli and Eubacterium biforme) were abundant in homozygous-F508del and severe CF patients, while beneficial species (Faecalibacterium prausnitzii, Bifidobacterium spp., and Eubacterium limosum) were reduced.ConclusionsThis is the first report that establishes a link among CFTR variants and shifts in faecal microbiota, opening the way to studies that perceive CF as a ‘systemic disease’, linking the lung and the gut in a joined axis.

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Section: Methodsmentioning

confidence: 99%

Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Allelic Variants Relate to Shifts in Faecal Microbiota of Cystic Fibrosis Patients

et al. 2013

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“…A perfect score of 100 points means a normal asymptomatic individual. The system is subjective, but it has recently been demonstrated to have "acceptable" interobserver variance (46). The score has not been tested for prognostic value.…”

Section: Patients' Age In Yearsmentioning

confidence: 99%

Serum Mucin-Associated Antigen Levels of Cystic Fibrosis Patients Are Related to Their Ages and Clinical Statuses

et al. 1989

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“…In addition, several studies showed good correlation between different observers using this scoring system. 3,4 On the other hand, it is imperative to promptly and repeatedly monitor pulmonary function to detect early evidence of airway obstruction. Pulmonary function tests have an additional value beyond SK score and are an objective marker of the course of the disease, since alterations are identified earlier than in chest films 5 and clinical picture.…”

Section: Introductionmentioning

confidence: 99%

Assessing correlations between spirometry and Shwachman‐Kulczycki score in children and adolescents

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Reis

et al. 2003

Pediatric Pulmonology

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A retrospective observational study was carried out to analyze the correlations between formal pulmonary function tests and the Shwachman-Kulczycki (SK) score. Forty-six Brazilian cystic fibrosis (CF) patients, clinically stable, aged 7-19 years, were included. Clinical and radiological findings of the SK score system and spirometry variables were assessed by independent observers in a blinded manner. The strongest correlation (r = 0.75; 95% CI, 0.59-0.85; P < 0.001) was found for forced expiratory volume in 1 sec (FEV1). Despite some peculiarities of our patients, the results are in agreement with studies undertaken in industrialized countries, showing good correlation between FEV1 and SK score and its usefulness in managing CF. Accordingly, in countries where pulmonary function testing is not available, the SK score remains a valuable parameter on which to base treatment results.

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Interobserver Variance in Clinical Scoring for Cystic Fibrosis

Cited by 22 publications

References 7 publications

Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Allelic Variants Relate to Shifts in Faecal Microbiota of Cystic Fibrosis Patients

Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Allelic Variants Relate to Shifts in Faecal Microbiota of Cystic Fibrosis Patients

Serum Mucin-Associated Antigen Levels of Cystic Fibrosis Patients Are Related to Their Ages and Clinical Statuses

Assessing correlations between spirometry and Shwachman‐Kulczycki score in children and adolescents

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