Sputum and Plasma Neutrophil Elastase in Stable Adult Patients With Cystic Fibrosis in Relation to Chronic Pseudomonas Aeruginosa Colonization.

Background and purpose Neutrophil elastase (NE) has been implicated in the pathogenesis of airway inflammation in cystic fibrosis (CF) patients and it impairs defenses against Pseudomonas aeruginosa (PA) infection or colonization. Sputum NE may act as a biomarker of neutrophilic inflammation in CF patients. This study aimed to determine sputum and plasma total NE levels in clinically stable adult CF patients and control subjects, and their correlation to PA colonization and lung functions. Methods This is a cross-sectional study. Total NE was measured on spontaneously expectorated sputum and plasma obtained from 21 CF patients, aged 18-40 years, during routine visits to the adult CF clinic. This was compared to plasma obtained from 22 matching healthy controls. The levels of NE were measured by the magnetic bead-based multiplex assay. Results Sputum and plasma NE levels had a significant positive correlation (Pearson r=0.533, P=0.013) with PA colonization. Sixteen CF patients (76.2%) were chronically colonized with PA. Both median sputum and plasma NE were found to be higher in CF patients with PA as compared with non-PA patients, even though this difference was statistically insignificant. Sputum and plasma NE levels did not correlate with the percentage predicted forced expiratory volume in one second (FEV1), the forced vital capacity (FVC), and FEV1/FVC and no association with PA. Conclusion The findings suggest that clinically stable adult CF patients colonized with PA may have higher NE levels in both plasma and sputum as compared to non-PA CF patients and probably total NE does not influence lung functions.

An Unusual and Unexpected Result of Diagnostic Sampling for COVID-19.

Coronavirus disease 2019 (COVID-19) is an infectious disease caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). It has spread globally, resulting in an ongoing pandemic. Real-time reverse transcription-polymerase chain reaction (rRT-PCR) from a viral swab is diagnostic. The most common site to take this swab is from the nasopharyngeal area; however, patients with tracheostomies represent a major challenge as they have two sources for colonization and possible infection including the nose and the trachea. We present the case of a patient who had a COVID-19 diagnostic swab through his tracheostomy, when unfortunately the swab broke, resulting in a bronchial foreign body.

CT score and correlation with lung function and microbiology of adult patients with cystic fibrosis with predominant I1234V genotype in Qatar.

Background: Computed tomography (CT) features of cystic fibrosis (CF) lung disease can be objectively quantified using current CT scoring systems to assess the extent and severity of the disease. The aims of this study were to calculate the Santamaria CT scores in adult patients with CF with the predominant CFTR I1234V genotype, determine its reliability, and correlate these parameters with lung function, microbial colonization, compliance to treatment, and exacerbations. Methodology: This retrospective observational study was conducted on adult patients with CF who were regularly followed up in the adult CF service at Qatar via CT scans that were taken not during an acute exacerbation. CT scans were scored using the Santamaria scoring system. Corresponding spirometry, microbiological data of sputum culture, and relevant clinical data were correlated with individual CT scores. Results: Only 23 of the 31 patients underwent CT when not in an acute exacerbation and were included in the study analysis. A total of 20 (87%) patients had the I1234V genotype. There was good agreement between the two radiologists on the Santamaria CT scores with an intraclass correlation coefficient (ICC) value of 0.991. Bronchiectasis was the most consistent finding, followed by interlobular and intralobular septal thickening. Patients with poor lung function and frequent exacerbations had significantly higher CT scores (p = 0.015). The CT scores of patients colonized with Pseudomonas aeruginosa were higher but nonsignificant (p = 0.20). The mean CT scores were significantly higher in patients who were noncompliant to regular treatment than in those who were compliant (p = 0.012). Conclusion: Santamaria CT scores comprise a reliable scoring system for adult patients with CF and can be used to determine the extent and severity of lung disease. P. aeruginosa colonization causes more structural lung damage than other common colonizing organisms. Noncompliance to treatment has a significant impact on the increasing severity of CF lung disease.

Serum zinc concentration in cystic fibrosis patients with CFTR I1234V mutation associated with pancreatic sufficiency.

OBJECTIVE: To determine serum zinc (Zn) level among cystic fibrosis (CF) patients with homozygous CFTR I1234V mutation associated with pancreatic sufficiency (PS). METHODS: A cross-sectional study was conducted including both pediatric and adult CF patients. Data on age, weight, height, body mass index (BMI), BMI Z-score, FEV1, and chronic Pseudomonas aeruginosa infection were collected. Serum Zn, albumin, and total proteins were measured and analyzed. RESULTS: Forty-five CF patients with homozygous CFTR I1234V mutation belonging to a large Arab kindred tribe and eight CF patients with other mutations associated with pancreatic insufficiency (PI). Patient's age ranged from 2 to 49 years with a mean age of 15.1 ± 9.1 years and mean plasma Zn of 0.78 ± 0.15 mcg/mL. Seven (13.2%) patients with CFTR I1234V and PS had low Zn levels (<0.6 mcg/mL). Mean age among Zn deficient group was significantly older. The mean FEV1 in the deficient group was found to be insignificant low. Persistent P. aeruginosa colonization was more prevalent in Zn deficient group. BMI Z-scosre of CF patients were positively correlated with Zn levels. Forty-five healthy subjects belonging to the same Arab tribe were selected in order to assess their Zn levels and their mean plasma Zn of 0.84 ± 0.11 mcg/mL (range 0.65-1.1 mcg/mL) with mean age 20.4 ± 10.1 years (range 6-40 years). CONCLUSION: These findings suggest that Zn deficiency can occur in CF patients with PS. The association of Zn levels and the frequency of P. aeruginosa isolated in CF patients need further investigation.

Bone Mineral Density in Cystic Fibrosis Patients with the CFTR I1234V Mutation in a Large Kindred Family Is Associated with Pancreatic Sufficiency.

Objectives. To study bone mineral density (BMD) in cystic fibrosis (CF) children and adults with the CFTR I1234V mutation associated with pancreatic sufficiency. Methods. Lumbar spine, total hip, and whole-body mineral density were measured by dual-energy radiographic absorptiometry (DEXA) scan. Z score was used for those less than 21 years and T score was used for those 21 years or older. Results. Twenty-one CF patients were younger than 21 years and 5 CF patients were 21 years or older. Mean age was 17.29 ± 4.95 years, ranging from 10 to 33 years. The mean BMD Z scores for patients younger than 21 years were -0.69 ± 0.96 (lumbar spine = L1-L4), -0.48 ± 0.92 (total hip), and -0.38 ± 0.86 (total body). The mean T scores for patients 21 years or older were 0.14 ± 0.7 (L1-L4), 0.38 ± 1 (total hip), and 0.52 ± 1.03 (total body). BMD reduction less than -1 was found in 7 (26.9%) CF patients. Vitamin D deficiency in 20 CF patients (76.9%) tended to be lower in CF patients with low BMD. BMD was significantly correlated with FEV1; however, no significant association was observed with P. aeruginosa colonization. Conclusion. BMD reduction does occur in patients with mild CFTR mutation associated with pancreatic sufficiency.

Molecular identification and susceptibility pattern of clinical Nocardia species: Emergence of Nocardia crassostreae as an agent of invasive nocardiosis.

BACKGROUND: Nocardia species are rare, opportunistic organisms that cause disease in both immunocompetent and immunocompromised individuals. OBJECTIVE: To investigate the clinical presentations of various Nocardia infections based on the 16S ribosomal RNA gene of the isolate, as well as related risk factors and susceptibility patterns to antimicrobial agents. METHODS: Thirteen patients with a diagnosis of nocardiosis were included in the present study. Seven Nocardia species were identified by 16S ribosomal RNA. Susceptibility testing was performed using six antimicrobial agents. RESULTS: Five patients were immunocompromised, and eight were immunocompetent with predisposing factors including cystic fibrosis, tuberculosis and ophthalmic infections. Nocardia caused pulmonary infections in eight patients (61.5%), invasive systemic infections in three patients (23%) and local (ophthalmic) infections in two patients (15.4%). In the patients with pulmonary disease, nocardiosis was caused by six species (Nocardia cyriacigeorgica, Nocardia otitidiscaviarum, Nocardia farcinica, Nocardia carnea, Nocardia testacea and Nocardia asiatica). The seventh species identified in the present study was Nocardia crassostreae. DISCUSSION: N crassostreae is a multidrug-resistant organism that was reported to be an emerging human pathogen causing invasive nocardiosis in a patient with non-Hodgkin's lymphoma. N farcinica was isolated from blood in a patient with breast cancer. None of the Nocardia isolates were resistant to linezolid. One N otitidiscaviarum isolate was a multidrug-resistant organism. All patients in the present study were treated with the appropriate antibiotics and their condition resolved without further sequelae. CONCLUSIONS: The present study is the first report on N crassostreae as a human pathogen. The detection of multidrug-resistant species necessitate molecular identification and susceptibility testing, and should be performed for all Nocardia infections. Nocardiosis manifests various clinical features depending on the Nocardia species and underlying conditions.

HISTORIQUE: Les espèces de Nocardia sont des organismes opportunistes rares qui sont pathogènes à la fois chez les personnes immunocompétentes et immunodéprimées. OBJECTIF: Explorer la présentation clinique de diverses infections à Nocardia d'après le gène d'ARN ribosomique 16S de l'isolat, ainsi que les facteurs de risque connexes et les profils de susceptibilité aux antimicrobiens. MÉTHODOLOGIE: Treize patients ayant un diagnostic de nocardiose ont participé à la présente étude. Les chercheurs ont repéré sept espèces de Nocardia au moyen de l'ARN ribosomique 16S. Ils ont effectué les tests de susceptibilité à six antimicrobiens. RÉSULTATS: Cinq patients étaient immunodéprimés et huit étaient immunocompétents, mais présentaient des facteurs de prédisposition, y compris la fibrose kystique, la tuberculose et des infections ophtalmiques. La Nocardia a provoqué des infections pulmonaires chez huit patients (61,5 %), des infections systémiques invasives chez trois patients (23 %) et des infections locales (ophtalmiques) chez deux patients (15,4 %). Chez les patients atteints d'une maladie pulmonaire, la nocardiose était attribuable à six espèces (Nocardia cyriacigeorgica, Nocardia otitidiscaviarum, Nocardia farcinica, Nocardia carnea, Nocardia testacea et Nocardia asiatica). La septième espèce observée dans la présente étude était la Nocardia crassostreae. EXPOSÉ: La N crassostreae, un organisme multirésistant considéré comme un agent anthropopathogène émergent, était responsable d'une nocardiose invasive chez un patient atteint d'un lymphome non hodgkinien. Les chercheurs ont isolé le N farcinica dans le sang d'un patient atteint d'un cancer du sein. Aucun des isolats de Nocardia n'était résistant à la linézolide. Un isolat de N otitidiscaviarum était multirésistant. Tous les patients participant à la présente étude ont reçu un traitement aux antibiotiques pertinent et se sont rétablis sans autres séquelles. CONCLUSION: La première étude est la première à faire état de la N crassostreae comme agent anthropopathogène. Pour déceler les espèces multirésistantes, il faut procéder à une identification moléculaire et à un test de susceptibilité, des mesures qu'il faudrait prendre à l'égard de toutes les infections à Nocardia. La nocardiose s'associe à diverses caractéristiques cliniques, selon l'espèce de Nocardia et les maladies sousjacentes.

Predictors of obstructive sleep apnea in snorers.

BACKGROUND: Snoring is a commong problem that poses a high risk for obstructive sleep apnea (OSA). We studied the contribution of risk factors for OSA in snorers for full-night polysomnography (PSG). METHODS: A questionnaire was administered to subjects referred for PSG in the period from April 2002 to March 2005. RESULTS: There were 191 (84%) snorers identified by 227 PSG studies. They had a mean age of 48.1+/-9.8 years, (age range, 23-73 years) and 78.5% were males. OSA as indicated by a respiratory disturbance index (RDI) of >5 events/hour was seen in 126 (66%) subjects. In males, 72.7% had OSA, with a mean RDI of 43.0+/-26 events/hour, versus 41.5% [corrected] with OSA in females with a mean RDI of 27.8+/-26.5 events/hour (P<0.001). The OSA group had a higher mean Epworth Sleepiness Scale (ESS) (P<0.001), a larger mean neck circumference (P<0.01), an increased mean age (P<0.050), and more witnessed apneas (P<0.001) but not choking (P=0.096). The mean increase in body mass index was linked to OSA only in females (P<0.05) but not in the overall study (P=0.507). Multivariate analysis showed that ESS, male gender, and a history of witnessed apneas were associated with OSA, while controlling for obesity, large neck circumference, age, and history of choking. CONCLUSION: In screening snorers for PSG, male gender, ESS and a history of witnessed apneas were the most important predictors of OSA, but other factors should be considered in referring snorers for PSG. In males, obesity did not contribute to the risk of OSA in our study population.

Indications, diagnostic yields and complications of transbronchial biopsy over 5 years in the State of Qatar.

OBJECTIVES: To review the indications, diagnostic yields and complications of transbronchial biopsy (TBB) in a tertiary hospital in the State of Qatar. METHODS: A retrospective review of our records revealed 1006 adult flexible fibre optic bronchoscopies (FFB) at Hamad General Hospital, State of Qatar between January 1999 and December 2003. A total of 85 (8.4%) TBB were performed, but complete data were available for 71/85 (83.5%), which were reviewed for indications, diagnostic yields and complications. RESULTS: Adequate samples were obtained in 58/71 TBBs (81.7%), while 13/71 TBBs (18.3%) yielded bronchial mucosa. The main indications in 16/71 (22.5%) TBBs for radiographic localized pulmonary disease were to rule out tuberculosis (TB) in 13 cases, and malignancy in 3 cases. Tuberculosis was verified in 3 (23%) of the 13 cases with localized disease. Fifty-five out of 71 (77.5%) TBBs were performed for radiographic diffuse pulmonary disease: 16/55 (29%) for miliary shadows, while 39/55 (70.9%) were carried out for reticular/reticulonodular infiltrates. Histopathology showed granulomatous lesions consistent with TB in 10/16 (62.5%) cases of miliary shadow. In the other pattern of diffuse disease, the histopathological diagnosis were obtained in 25/39 (64%) cases. It showed non-specific pulmonary fibrosis in 13 cases, sarcoidosis in 4 cases, connective tissue disease associated interstitial fibrosis in 4 cases, bronchiolitis obliterans organizing pneumonia (BOOP) in one case, eosinophilic pneumonia in one case, amiodarone toxicity in one case and lymphangitis carcinomatosis in one case. The main complications were minor bleeding <50 cc in 17 cases (23.9%), pneumothorax in 7 cases (9.8%)) and one case had sepsis. CONCLUSION: Our experience substantiates previous reports of the value and safety of transbronchial biopsy in the rapid diagnosis of smear-negative miliary TB. In diffuse lung diseases of a non-infectious nature, other than sarcoidosis, lymphangitis carcinomatosis and few other conditions, a pathological diagnosis are much less likely to be reliably made on small pieces of tissue such as those provided by TBB.