METHODS: GBS patients were consecutively recruited and the results were compared to age- and gender-matched healthy controls. A series of autonomic function tests including computation-dependent tests (power spectrum analysis of HRV and BRS at rest) and challenge maneuvers (deep breathing, eyeball compression, active standing, the Valsalva maneuver, sustained handgrip, and the cold pressor test) were performed.
RESULTS: Ten GBS patients (six men; mean age = 40.1 ± 13.9 years) and ten gender- and age-matched healthy controls were recruited. The mean GBS functional grading scale at disease plateau was 3.4 ± 1.0. No patients required intensive care unit admission or mechanical ventilation. Low-frequency HRV (p = 0.027), high-frequency HRV (p = 0.008), and the total power spectral density of HRV (p = 0.015) were significantly reduced in patients compared to controls. The mean up slope (p = 0.034), down slope (p = 0.011), and total slope (p = 0.024) BRS were significantly lower in GBS patients. The diastolic rise in blood pressure in the cold pressor test was significantly lower in GBS patients compared to controls (p = 0.008).
INTERPRETATION: Computation-dependent tests (HRV and BRS) were more useful for detecting autonomic dysfunction in GBS patients, whereas the cold pressor test was the only reliable challenge test, making it useful as a bedside measure of autonomic function in GBS patients.
METHODS: Children undergoing FB were prospectively enrolled. Their FB was digitally recorded and assessed (two clinicians blinded to each other and clinical history) for six features: secretion amount (six-point scale), secretion color (BronkoTest, 0-8), mucosal oedema (0-3), ridging (0-3), erythema (0-3), and pallor (0-3) based on pre-determined criteria. We correlated (Spearman's rho) each feature with bronchoalveolar lavage (BAL) neutrophil percentage (neutrophil%). BScore was then derived using models with combinations of the six features that best related to airway BAL neutrophil%. The various models of BScore were plotted against BAL neutrophil% using receiver operating characteristic (ROC) curves.
RESULTS: We analyzed 142 out of 150 children enrolled. Eight children were excluded for unavailability of BAL cytology or FB recordings. Chronic/recurrent cough was the commonest indication for FB (75%). The median age was 3 years (IQR, 1.5-5.3 years). Secretion amount (r = 0.42) and color (r = 0.46), mucosal oedema (r = 0.42), and erythema (r = 0.30) significantly correlated with BAL neutrophil%, P 10%).
CONCLUSION: This prospective study has developed the first validated bronchitis scoring tool in children based on bronchoscopic visual inspection of airways. Further validation in other cohorts is however required.
METHODS: The original PCPI-S was reviewed and adapted for cultural suitability by an expert committee to ensure conceptual and item equivalence. The instrument was subsequently translated into the local Malay language using the forward-backward translation by two independent native speakers, and modified following pre-tests involving cognitive debriefing interviews. The psychometric properties of the corresponding instrument were determined by assessing the internal consistency, test-retest reliability, and correlation of the instrument, while the underlying structure was analysed using exploratory factor analysis.
RESULTS: Review by expert committee found items applicable to local context. Pre-tests on the translated instrument found multiple domains and questions were misinterpreted. Many translations were heavily influenced by culture, context, and language discrepancies. Results of the subsequent pilot study found mean scores for all items ranged from 2.92 to 4.39. Notable ceiling effects were found. Internal consistency was high (Cronbach's alpha > 0.9). Exploratory factor analysis found formation of 11 components as opposed to the original 17 constructs.
CONCLUSION: The results of this study provide evidence regarding the reliability and underlying structure of the PCPI-S instrument with regard to primary care practice. Culture, context, language and local practice heavily influenced the adaptation as well as interpretation of the underlying structure and should be given emphasis when translating person-centred into practice.
Methods: A total of 42 patients with congenital heart defects, as confirmed by echocardiography, were recruited. Genetic molecular analysis using a fluorescence in situ hybridization (FISH) technique was conducted as part of routine 22q11.2DS screening, followed by multiplex ligation-dependent probe amplification (MLPA), which serves as a confirmatory test.
Results: Two of the 42 CHD cases (4.76%) indicated the presence of 22q11.2DS, and interestingly, both cases have conotruncal heart defects. In terms of concordance of techniques used, MLPA is superior since it can detect deletions within the 22q11.2 locus and outside of the typically deleted region (TDR) as well as duplications.
Conclusion: The incidence of 22q11.2DS among patients with CHD in the east coast of Malaysia is 0.047. MLPA is a scalable and affordable alternative molecular diagnostic method in the screening of 22q11.2DS and can be routinely applied for the diagnosis of deletion syndromes.
AIMS: Our primary objective was to evaluate the incidence of post-ESD esophageal stricture with the application of carboxymethyl cellulose (CMC) sheets. Secondary objectives were to determine the number of sessions of endoscopic balloon dilatation (EBD) required to resolve post-ESD strictures and the incidence rate of peri-operative adverse events.
METHODS: This was a pilot, single-center, prospective study. Seven patients who had high risks of developing post-ESD esophageal stricture were enrolled into our study. CMC sheets were applied to the mucosal defects immediately after the completion of ESD. Patients were monitored and reviewed after ESD to detect any adverse events.
RESULTS: The incidence rate of post-operative stricture was 57 % (4/7 patients). Among patients who required EBD, the number of sessions performed was 2.8 ± 2.2. No serious post-operative adverse events were reported.
CONCLUSION: The use of CMC sheets appears to be a safe and effective prophylactic treatment for esophageal stricture following extensive ESD.