Etiology and clinical characteristics of a non-cystic fibrosis bronchiectasis cohort in a middle eastern population.

BACKGROUND: Bronchiectasis is a widely prevalent airway disease characterized by airway dilatation and recurrent infections, that can lead to respiratory failure in severe cases. The etiology of bronchiectasis varies geographically, but there is a lack of published data examining its etiology specifically within the Middle Eastern population. METHODS: We conducted a retrospective analysis of our bronchiectasis patient registry, extracting clinical and demographic characteristics from electronic medical records. Quantitative variables were presented as the median and interquartile range (IQR), while categorical variables were expressed as numbers and percentages. Statistical comparisons for continuous characteristics were performed using the t-test, and significance was determined by a p-value less than 0.05. RESULTS: In total we analysed 260 records (63% female, 37% male), with median age of 58 years (interquartile range (IQR) 38-71), Body Mass Index (BMI) 25.8(IQR 22-30), forced expiratory volume in the first second (FEV1) %predicted 65 (IQR 43-79) and FEV1/forced vital capacity (FVC) 0.76 (0.67-0.86). Sixty-five cases (25%) were post-infectious in aetiology (excluding post-TB - n:27 10.4%). Forty-eight (18.5%) patients were labelled idiopathic, while Primary Ciliary Dyskinesia (PCD) accounted for 23 (8.8%) cases. Pseudomonas aeruginosa was the most common colonizing organism (32.7%), followed by Haemophilus influenzae (9.2%) and Methicillin-Sensitive Staphylococcus aureus(6.9%). At the time of review, 11 patients had died (median age, FEV %predicted, and bronchiectasis severity index (BSI) 59 years, 38% and 15.5 respectively), all due to respiratory failure, and as expected, all were classed severe on BSI. The BSI score was available for 109 patients, of which 31(28%) were classed mild, 29(27%) were moderate, and 49 (45%) were classed severe. The median BSI score was 8 (IQR 4-11). On dividing the patients according to obstructive vs. restrictive spirometry, we found that patients with FEV1/FVC < 0.70 had significantly higher BSI (10.1 vs. 6.9, p-value < 0.001) and that 8 out of the 11 deceased patients had FEV1/FVC < 70%. CONCLUSIONS: In our study, post-infectious, idiopathic, and PCD were identified as the most common etiologies of bronchiectasis. Additionally, patients with obstructive spirometry appeared to have a worse prognosis compared to those with restrictive spirometry.

Classic Cystic Fibrosis Presentation in Two Middle Eastern Siblings with a rare CFTR mutation (c.80G>T).

Cystic fibrosis (CF) is a common autosomal recessive disorder which is mainly found in Caucasians but has also been reported in Asian populations. CF is primarily caused by mutations in the CFTR gene which regulates the transport of chloride ions across the cell membrane. We describe the cases of two siblings with CF diagnosed with the rare missense mutation c.80G>T, which has only been referenced once in the literature and shows a possible association with classical form of CF. LEARNING POINTS: c.80G>T is a very rare CFTR missense mutation which has not been known to be a disease-causing alteration.The mutation causes an amino acid switch from glycine to valine at position 27 in exon 2, resulting in the production of defective CFTR protein.In the homozygous state, c.80G>T seems to be associated with the classic CF phenotype.

Thoracic surgery in United Arab Emirates.

The United Arab Emirates (UAE) has undergone a significant change in its population and economy in the last decades and in parallel its healthcare system has evolved rapidly to provide advanced, innovative and world-leading care. At the forefront of this revolution in healthcare is the development of a multidisciplinary multimodality thoracic service provision, offered at quaternary referral hospitals amalgamating academics, training, research and innovation. Previously, thoracic service care was limited to single providers at various public and private hospitals, usually performing lower complexity cases. Most complex thoracic cases were repatriated outside the UAE. This practice was replaced with the opening of Cleveland Clinic Abu Dhabi (CCAD), in 2015, which created a multidisciplinary thoracic program. This included the start of a mini-invasive surgical and lung transplantation program. Since that time other public and private hospitals have emerged providing care in a similar model. The impact of these programs has been a decreased transfer of patients abroad for treatment. Under the umbrella of the Emirates Thoracic Society (ETS) a platform for greater collaboration aimed at improving patient care, potential research and physician education has been created. Direct links have been established with world-leading Thoracic surgery and Respiratory Medicine Centers facilitating this development and offering support and guidance. This article charts these changes in thoracic care in the recent past, present, and delineates plans for the future in the UAE.

The Journey for Lung Cancer Screening where we Stand Today.

Background: Lung cancer remains a leading cause of cancer mortality worldwide with many patients presenting with advanced disease. Objective: We reviewed the available literature for lung cancer screening using low dose computed tomography (LDCT). We reviewed the National Lung Screening Trial (NLST), Early Lung Cancer Action Program (ELCAP) and the (Nederlands-Leuvens Longkanker Screenings Onderzoek (NELSON) trials. We also look at different lung cancer risk prediction models that may aid in identifying target populations and also discuss the cost-effectiveness of LDCT screening in different groups of smokers and ex-smokers. Lastly, we discuss recent guideline changes that have occurred in line with new and emerging evidence on lung cancer screening. Conclusion: LDCT has been shown reduce lung cancer mortality in certain groups of current and former smokers and should be considered to help in the early diagnosis of lung cancer.

Genetic and Clinical Demographics of Adult Cystic Fibrosis Patients in a Middle Eastern Population.

OBJECTIVE: Cystic fibrosis (CF) is the commonest life-limiting inherited illness in the Caucasian population but is uncommon in the Middle East, and so the genotypes and clinical course of disease in this population is not well known. MATERIAL AND METHODS: In this retrospective observational study, we collected and reviewed the data on CF mutations, body mass index (BMI), lung function, microbiology, and the demographics in adult CF patients in the United Arab Emirates (UAE). RESULTS: Data was reviewed for 39 adult CF patients. The median age of adult CF patients presenting to our clinic was 25 years (interquartile range (IQR) 22-31), the median BMI was 19 (IQR 17-22), and the median percentage predicted forced expiratory volume at 1 second (FEV1) was 49.5% (IQR 38.5-62.5). S549R was the commonest mutation (n = 11, 28%) followed by ∆F508 (n = 9, 23%). Only 5 (13%) out of 39 patients were heterozygote for CF mutations which reflects the high level of consanguinity in the region. Twelve (30%) patients were diagnosed after the age of 16, and in total, 19 (48%) were diagnosed after the age of 10. Thirty-two (82%) of patients are pseudomonas colonized, and 31% had 3 or more exacerbations in the last 12 months. CONCLUSION: The CF mutation patterns in the UAE are different from western populations with low ∆F508 prevalence, with the presence of rare mutations more specific to this region and a high rate of homozygosity. Late diagnosis, high pseudomonas colonization rate, and exacerbation frequency remain a problem in this region and lead to poor long-term outcomes.

Complications of surgical and percutaneous tracheostomies, and factors leading to decannulation success in a unique Middle Eastern population.

INTRODUCTION: Surgical and percutaneous tracheostomy remains a commonly performed procedure in the intensive care unit (ICU). Given the unique patient population in the Middle East we decided to perform a review of the procedures performed in our hospital over a two-year period. METHODS: Single centre, retrospective observational study. All tracheostomies performed between January 2016 and January 2018 were included in the study. The primary outcome was the rate of tracheostomy complications. Multivariate logistic regression analysis was used to identify the independent factors associated with complications and decannulations. RESULTS: One hundred sixty-four patients were included in the study. Percutaneous tracheostomy was performed in 99 patients (60.4%). Complications occurred in thirty-eight patients (23%). Higher Left ventricular ejection fraction (OR = 0.94, 95%CI: [0.898-0.985]) and percutaneous tracheostomy (OR = 0.107, 95%CI: [0.029-0.401]) were associated with lower complications. Good Eastern Cooperative Oncology Group (ECOG) performance status (OR = 4.1, 95%CI: [1.3-13.3]) and downsized tracheostomy tube (OR = 6.5, 95%CI: [2.0-21.0]) were associated with successful decannulations. Successful decannulation was associated with lower hospital mortality when compated to those who could not be decannulated (3.2% vs 33.3% p < 0.0001). CONCLUSION: In our older population with high comorbidities, percutaneous tracheostomies were associated with less complications than surgical tracheostomies. Patients with poor premorbid functional status and those who could not have their tracheostomy tube sucessfuly downsized were less likely to be decannulated, and had a higher mortality. This data enables physicians to inform the families of the added risks involved with tracheostomy in this patient group.

Complications following symptom-limited thoracentesis using suction.

BACKGROUND: Thoracentesis using suction is perceived to have increased risk of complications, including pneumothorax and re-expansion pulmonary oedema (REPO). Current guidelines recommend limiting drainage to 1.5 L to avoid REPO. Our purpose was to examine the incidence of complications with symptom-limited drainage of pleural fluid using suction and identify risk factors for REPO. METHODS: A retrospective cohort study of all adult patients who underwent symptom-limited thoracentesis using suction at our institution between January 1, 2004 and August 31, 2018 was performed, and a total of 10 344 thoracenteses were included. RESULTS: Pleural fluid ≥1.5 L was removed in 19% of the procedures. Thoracentesis was stopped due to chest discomfort (39%), complete drainage of fluid (37%) and persistent cough (13%). Pneumothorax based on chest radiography was detected in 3.98%, but only 0.28% required intervention. The incidence of REPO was 0.08%. The incidence of REPO increased with Eastern Cooperative Oncology Group performance status (ECOG PS) ≥3 compounded with ≥1.5 L (0.04-0.54%; 95% CI 0.13-2.06 L). Thoracentesis in those with ipsilateral mediastinal shift did not increase complications, but less fluid was removed (p<0.01). CONCLUSIONS: Symptom-limited thoracentesis using suction is safe even with large volumes. Pneumothorax requiring intervention and REPO are both rare. There were no increased procedural complications in those with ipsilateral mediastinal shift. REPO increased with poor ECOG PS and drainage ≥1.5 L. Symptom-limited drainage using suction without pleural manometry is safe.

Managing malignant pleural effusion.

Malignant pleural effusion can be managed in different ways, including clinical observation, thoracentesis, placement of an indwelling pleural catheter, and chemical pleurodesis. The optimal strategy depends on a variety of clinical factors. This article uses cases to illustrate the rationale for determining the best approach in different situations.

Quality gaps and comparative effectiveness in lung cancer staging: the impact of test sequencing on outcomes.

BACKGROUND: Evidence-based guidelines recommend mediastinal sampling as the first invasive test in patients with suspected lung cancer and mediastinal adenopathy. The goal of this study was to assess practice patterns and outcomes of diagnostic strategies in this patient population. METHODS: We conducted a retrospective analysis of all patients in 2009 who had mediastinal adenopathy without distant metastatic disease to determine whether guideline-consistent care was delivered. Guideline-consistent care was defined as mediastinal lymph node sampling being performed as part of the first invasive procedure. RESULTS: One hundred thirty-seven patients were included. Guideline-consistent care was provided in 30 cases (22%). Patients receiving guideline-consistent care had fewer invasive tests than patients with guideline-inconsistent care (1.3 ± 0.5 tests/patient vs 2.3 ± 0.5 tests/patient, respectively; P < .0001) and fewer complications (0 of 30, 0% vs 18 of 108, 17%; P = .01). Most of the complications (16 of 18) were related to CT image-guided needle biopsy. Endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) was sufficient to guide treatment decisions without any other invasive tests in 88 patients (64%). Although not all the complications and costs due to CT image-guided biopsies could have been avoided, roughly two-thirds could have been eliminated by just changing the testing sequence. CONCLUSION: Quality gaps in lung cancer staging in patients with mediastinal adenopathy are common and lead to unnecessary testing and increased complications. In patients with suspected lung cancer without distant metastatic disease with mediastinal adenopathy, EBUS-TBNA should be the first test.

Randomized clinical trial of endobronchial ultrasound needle biopsy with and without aspiration.

BACKGROUND: Endobronchial ultrasound (EBUS)-guided transbronchial needle aspiration (EBUS-TBNA) is performed with a dedicated 22- or 21-gauge needle while suction is applied. Fine-needle sampling without suction (capillary sampling) has been studied for endoscopic ultrasound and for biopsies at various body sites and has resulted in similar diagnostic yield and fewer traumatic samples. However, the role of EBUS-guided transbronchial needle capillary sampling (EBUS-TBNCS) is still to be determined. METHODS: Adults with suspicious hilar or mediastinal lymph nodes (LNs) were included in a single-blinded, prospective, randomized trial comparing EBUS-TBNA and EBUS-TBNCS. The primary end point was the concordance rate between the two techniques in terms of adequacy and diagnosis of cytologic samples. The secondary end point was the concordance rate between the two techniques in terms of quality of samples. RESULTS: A total of 115 patients and 192 LNs were studied. Concordance between EBUS-TBNA and EBUS-TBNCS was high, with no significant difference in adequacy (88% vs 88%, respectively [P ± .858]; concordance rate, 83.9% [95% CI, 77.9-88.8]); diagnosis (36% vs 34%, respectively [P ± .289]; concordance rate, 95.8% [95% CI, 92-92.8]); diagnosis of malignancy (28% vs 26%, respectively [P ± .125]; concordance rate, 97.9% [95% CI, 94.8-99.4]); or sample quality (concordance rate, 83.3% [95% CI, 73.3-88.3]). Concordance between EBUS-TBNA and EBUS-TBNCS was high irrespective of LN size (≤ 1 cm vs > 1 cm). CONCLUSIONS: Regardless of LN size, no differences in adequacy, diagnosis, or quality were found between samples obtained using EBUS-TBNA and those obtained using EBUS-TBNCS. There is no evidence of any benefit derived from the practice of applying suction to EBUS-guided biopsies. TRIAL REGISTRY: ClinicalTrials.gov; No.: NCT00886847; URL: www.clinicaltrials.gov

Iron status and chronic kidney disease predict restless legs syndrome in an older hospital population.

BACKGROUND: Iron deficiency is important in the pathogenesis of restless legs syndrome (RLS), and serum ferritin measurement, using a cutoff of 45-50ng/ml, is widely recommended as the optimal screening test for iron deficiency in RLS. Serum ferritin often increases with inflammation, and a higher cutoff may be better in those with acute and chronic inflammatory conditions, including those with chronic kidney disease (CKD). METHODS: The relationships between RLS and potential secondary causes were examined in hospital patients aged 50years or more. Diagnosis of RLS was based on a clinician interview. RESULTS: Of 301 patients, 55 (18.3%) had RLS. Ferritin levels less than 40ng/ml and between 40 and 69ng/ml and Stage 4 CKD (estimated glomerular filtration rate [eGFR] between 15 and 29ml/min and not on dialysis) were associated with significantly higher odds for RLS in univariate and multivariate analyses. CONCLUSION: Iron deficiency and chronic kidney disease are the strongest predictors of RLS in older hospital patients. Ferritin less than 70ng/ml is the best cutoff for identifying possible iron deficiency in RLS patients with inflammatory conditions. Independent of iron status, RLS is strongly associated with chronic kidney disease that is not severe enough to require dialysis, and the results of this study suggest that eGFR values and stages of CKD should be reported in future studies of RLS.

Management of malignant pleural effusions.

Malignant pleural effusions are a common clinical problem in patients with primary thoracic malignancy and metastatic malignancy to the thorax. Symptoms can be debilitating and can impair tolerance of anticancer therapy. This article presents a comprehensive review of pharmaceutical and nonpharmaceutical approaches to the management of malignant pleural effusion, and a novel algorithm for management based on patients' performance status.

Initial evaluation of the nonsmall cell lung cancer patient: diagnosis and staging.

PURPOSE OF REVIEW: The initial diagnosis and staging of nonsmall cell lung cancer patients is complex and involves multiple technologies. This review evaluates the recent literature and integrates it into a systematic method for evaluating patients. RECENT FINDINGS: The goal of the initial diagnosis and staging of nonsmall cell lung cancer is to provide sufficient information to allow definitive treatment. Initial steps should include a history and physical, basic laboratory tests, pulmonary functions, and PET-computed tomography (CT) imaging. If there is evidence of metastatic disease, then biopsy of the most advanced lesion is warranted. If there is no evidence of metastatic disease, the evaluation should focus on evaluation of the mediastinal lymph nodes. If there is evidence of nodal involvement by PET-CT, then endobronchial ultrasound-guided transbronchial needle aspiration is warranted. If there is no evidence of nodal involvement on PET-CT, then either surgery or CT-guided fine needle aspiration is warranted. Other factors that should be kept in mind when selecting a diagnostic strategy include whether or not the patient is a surgical candidate, the impact of comorbidities, the type of cancer, the need for predictive biomarker analysis, and the range of possible treatment options. SUMMARY: Integrating new technologies such as PET-CT and endobronchial ultrasound into the initial evaluation of patients can save unnecessary diagnostic procedures and lead to more rapid and accurate staging.

Mediastinal Infection After Ultrasound-guided Needle Aspiration.

We describe 2 cases of mediastinal abscess developing after endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) in patients with thoracic malignancies. The first case was that of a 68-year-old male patient with a history of colon cancer presenting with new mediastinal adenopathy. EBUS-TBNA confirmed metastatic colon adenocarcinoma; however, he developed fever and atrial fibrillation 32 days after the procedure and was diagnosed with mediastinal abscess. The patient was successfully treated with antibiotics. The second case was that of a 66-year-old previously healthy male who presented for evaluation of cough. Imaging showed a right paratracheal mass and he underwent EBUS-TBNA sampling, which showed malignant cells. Eight days after the procedure the patient developed fever and was diagnosed with mediastinal abscess. The second patient was treated with antibiotics and several debridements; however, he was eventually transitioned to hospice care because of a continued spread of his cancer. Although rare, mediastinal infection is a complication of endoscopic mediastinal needle aspiration.