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.

Neuromyelitis Optica Spectrum Disorder Associated with Cryptogenic Organizing Pneumonia in a Young Patient.

Neuromyelitis optica spectrum disorder (NMOSD) is associated with other autoimmune disorders and probably with cryptogenic organizing pneumonia (COP) as well. Here we present the case of a 14-year-old girl presenting with typical NMOSD together with radiological evidence of COP. Our case is unique as the previous two reports of this association were in elderly patients. LEARNING POINTS: There probably is an association between neuromyelitis optica spectrum disorder (NMOSD) and cryptogenic organizing pneumonia (COP).In contrast to previously published reports describing NMOSD and COP in two elderly patients, our case report highlights the fact that this disease combination can be present in young patients as well.

Outcomes of a Newly Established Electromagnetic Navigation Bronchoscopy Service in the Middle East.

Aims: Electromagnetic Navigation Bronchoscopy (EMNB) is a useful tool for the bronchoscopist to target peripheral pulmonary lesions. It has a well-established efficacy and safety profile; however, there are no studies describing its utility in a Middle Eastern population. In this paper, we describe the efficacy and safety outcomes of a newly established EMNB service. Background: The diagnosis of peripheral pulmonary lesions presents a significant challenge to the bronchoscopist, especially in the era of increased thoracic imaging with computerized tomography (CT) scans. EMNB is a relatively novel technique that utilizes an image-guided localization system akin to Global Positioning Satellite (GPS) technology, offering the bronchoscopist an accurate navigational pathway to sample peripheral pulmonary targets. Objective: We present our initial experience of performing EMNB and report our diagnostic and safety outcomes with EMN bronchoscopy. Methods: We conducted a retrospective review of the medical notes of all patients booked for EMNB from May 2015 to December 2019 at our tertiary care center using the electronic medical record system. Results and Discussion: Fifty-five patients were scheduled for EMNB, and 47 patients (24 males, 23 females) had EMNB-guided sampling between May 2015 and December 2019. The median age of the patients was 61 years (IQR 49.5-74.3). A bronchus sign was present on the CT chest in 29 (61.7%) cases. Thirty-one (66%) patients had positive EMNB guided samples. There was a weak correlation between the lesion size and the positive EMNB guided sampling (r: 0.34). Twenty-one of 29 (72%) patients with positive bronchus signs had positive EMNB guided samples, compared to 10/18 (56%) patients without bronchus signs; however, the difference was not statistically significant (p-value 0.335). When the presence or absence of rapid onsite examination of cytopathological specimens (ROSE) was compared during the procedure, a trend favoring the presence of ROSE could be seen, but this was statistically non-significant (p-value 0.078). In this series, one patient with pre-existing triple vessel coronary artery disease developed an inferior wall ST-segment elevation myocardial infarction (STEMI), likely secondary to spasm. This patient recovered completely and was discharged from the hospital. Conclusion: This study demonstrates that EMNB can be safely performed in a Middle Eastern population with results similar to those reported in major international studies. The highest diagnostic yield was in patients with a bronchus sign on a CT scan, and combining EMNB with ROSE can increase the chances of having a positive diagnostic bronchoscopy. However, patients and physicians need to be aware of the need to follow up with the patients with negative biopsies to ensure that false negatives are not missed.

Invasive Mediastinal Mucormycosis with Pulmonary and Cardiac Involvement in an Adult with Chronic Granulomatous Disease: Case Report and Review of the Literature.

Mucormycosis is a rare fungal infection that often causes rhinocerebral disease. However, there have been rare cases of mediastinal involvement. These patients remain a therapeutic challenge and mortality in this group is very high. We report a case of mediastinal mucormycosis with invasion of the heart and right lung in a patient with chronic granulomatous disease (CGD) and also review the available literature on mediastinal mucormycosis. LEARNING POINTS: Mucormycosis is a very rare cause of mediastinal mass, and has a high risk of mortality.Early recognition and treatment will likely increase the patient's chances of survival.Chronic granulomatous disease (CGD) is associated with an increased risk of fungal infections and should be considered for itraconazole prophylaxis.

Demographic and clinical patterns of severe asthma in the Middle East.

BACKGROUND: Severe asthma is a major burden on health-economic resources; hence, knowing the epidemiology of these patients is important in planning and provision of asthma care. In addition, identifying and managing the comorbidities helps improve symptoms and reduce associated morbidity and mortality. OBJECTIVES: Epidemiology of difficult asthma has not been well studied in the Middle East, so in this study, we present the demographic and clinical characteristics of severe asthma in the United Arab Emirates (UAE). METHODS: We retrospectively reviewed the notes of severe asthma patients attending three tertiary care hospitals between May 2015 and December 2019. Data on baseline demographics, asthma characteristics, treatment, and comorbidities were collected. RESULTS: We reviewed the notes of 458 patients (271 females and 187 males) that fulfilled the 2019 Global Initiative for Asthma guidelines for the diagnosis of severe asthma. The mean age was 47.7 (standard deviation 17.2) years. Males had significantly higher asthma control test scores (17.9 vs. 16, P = 0.01) and mean blood eosinophils (0.401 vs. 0.294, P <0.01) than females. The most common comorbidity observed was allergic rhinitis (52.2%) followed by gastroesophageal reflux disease (27.1%). In total, 109 (23.8%) patients were on biological therapies with most patients being on omalizumab and dupilumab (29 and 18 patients, respectively). Most patients were nonsmokers (97.2%), and majority were of TH2-high phenotype (75.7%). CONCLUSIONS: In this first report of severe asthma characteristics in the UAE, we found a pattern of female preponderance and most patients having a Th2-high phenotype. The findings are likely to help optimize asthma care in the region in the era of biologic therapies.

Correlation between Reduced FEF25-75% and a Positive Methacholine Challenge Test in Adults with Nonobstructive Baseline Spirometry.

OBJECTIVE: To clarify whether in adults with a nonobstructive spirometry a reduced FEF25-75% is associated with a positive methacholine challenge test (MCT). METHODS: Data was collected for all the patients who had a MCT done between April 2014 and January 2020 but had nonobstructive baseline spirometry. Logistic regression was utilized to estimate the log odds of a positive methacholine test as a function of FEF25-75% and also for age, gender, BMI, FEV1, and FEV1/FVC. RESULTS: Out of 496 patients, 187 (38%) had a positive MCT. Baseline characteristics in two groups were similar except that patients with positive MCT were younger (32 ± 11.57 vs. 38 ± 13.25 years, respectively, p < 0.001). Mean FEF25-75% was lower in MCT positive (3.12 ± 0.99 L/s) vs. MCT negative (3.39 ± 0.97 L/s) patients, p = 0.003. Logistic regression results suggest that MCT outcome is inversely related to FEF25-75%, age, and gender. Specifically, as FEF25-75% percentage of predicted value increases, the log odds of a positive MCT decrease (odds ratio (OR) = 0.90, 95% confidence intervals (CI) = 0.84-0.96, p = 0.002). Also, as age increases, the log odds of a positive MCT decrease (OR = 0.95, 95%CI = 0.94-0.97, p < 0.001). CONCLUSIONS: Reduced FEF25-75% in adults with nonobstructive spirometry can predict a positive response to MCT in younger patients. However, this relationship becomes weaker with increasing age.

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.

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.

Usefulness of Clinical Prediction Rules, D-dimer, and Arterial Blood Gas Analysis to Predict Pulmonary Embolism in Cancer Patients.

OBJECTIVES: Pulmonary embolism (PE) is seven times more common in cancer patients than non-cancer patients. Since the existing clinical prediction rules (CPRs) were validated predominantly in a non-cancer population, we decided to look at the utility of arterial blood gas (ABG) analysis and D-dimer in predicting PE in cancer patients. METHODS: Electronic medical records were reviewed between December 2005 and November 2010. A total of 177 computed tomography pulmonary angiograms (CTPAs) were performed. We selected 104 individuals based on completeness of laboratory and clinical data. Patients were divided into two groups, CTPA positive (patients with PE) and CTPA negative (PE excluded). Wells score, Geneva score, and modified Geneva score were calculated for each patient. Primary outcomes of interest were the sensitivities, specificities, positive, and negative predictive values for all three CPRs. RESULTS: Of the total of 104 individuals who had CTPAs, 33 (31.7%) were positive for PE and 71 (68.3%) were negative. There was no difference in basic demographics between the two groups. Laboratory parameters were compared and partial pressure of oxygen was significantly lower in patients with PE (68.1 mmHg vs. 71 mmHg, p = 0.030). Clinical prediction rules showed good sensitivities (88-100%) and negative predictive values (93-100%). An alveolar-arterial (A-a) gradient > 20 had 100% sensitivity and negative predictive values. CONCLUSIONS: CPRs and a low A-a gradient were useful in excluding PE in cancer patients. There is a need for prospective trials to validate these results.

An unusual presentation of endobronchial Hodgkin's lymphoma.

Endobronchial involvement of Hodgkin's lymphoma is a rare presentation of the disease. However, it needs to be considered in patients presenting with non-resolving pneumonia in the setting of Hodgkin's disease. In such cases, clinicians need to ensure adequate and multiple biopsies as patients may have co-existent pulmonary infection and disease involvement. A 16 years old patient reported with a history of relapsed Hodgkin's disease, with bilateral pulmonary infiltrates, that failed to resolve after empiric antibiotic therapy. Positron Emission Tomography (PET) scan was performed demonstrating equivocal uptake in left upper lobe. Bronchoscopy revealed a necrotic endobronchial mass. Initial biopsy only revealed necrotic inflammatory debris, however, since the patient continued to lose weight, repeat biopsy was performed which demonstrated CD 30 positive Hodgkin's lymphoma cells.

Use of microdebrider bronchoscopy for the treatment of endobronchial leiomyoma.

AIMS: Pulmonary leiomyomas are rare benign tumors that may cause symptoms when they spread endobronchially. Traditionally they were managed surgically or through interventional bronchoscopy with the aid of thermal modalities to assist in debulking of tumor. We report the novel use of microdebrider bronchoscopy to debulk an endobronchial leiomyoma in a symptomatic patient. METHOD/RESULTS: The microdebrider successfully débrided the endobronchial leiomyoma. CONCLUSION: This modality can be successfully employed when oxygenation is an issue, preventing use of thermal modalities. In addition, use of microdebrider not only reduced procedure time but also decreased the risk of airway fire and damage to adjacent normal tissue in our patient.

Does malaria during pregnancy affect the newborn?

OBJECTIVE: To investigate the effect of malarial infection during pregnancy on the newborn. METHODS: A retrospective cohort study was conducted at The Aga Khan University Hospital (AKUH), Karachi, using in-patient hospital records over an 11-year period from 1988 to 1999. The incidence of preterm delivery, low birth weight (LBW) and intrauterine growth retardation (IUGR) in 29 pregnant women with malaria, was compared with that in 66 selected pregnant women without malaria, who delivered at the AKUH during the same time period. RESULTS: Pregnant women with malaria had a 3.1 times greater risk of preterm labor (p=0.14). They were more likely to be anaemic compared to women without malaria (RR=2.9, 95% CI=1.6-5.4) and had a significantly lower mean haemoglobin level (p=0.0001). Maternal malaria was significantly associated with LBW babies (p=0.001). The mean birth weight of infants born to pregnant women with malaria was 461 g less (p=0.0005). No significant association was, however, found between malarial infection during pregnancy and IUGR (p=0.33). CONCLUSION: Malarial infection during pregnancy is associated with poor maternal and fetal outcome. It is significantly associated with maternal anaemia and LBW infants. Appropriate measures must, therefore, be taken to prevent malaria during pregnancy, especially in endemic areas.