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Alpha 1-antitrypsin (AAT) belongs to the family of serpins (serine protease inhibitors). Loop sheet polymerization is the pathology behind serpinopathies which encompasses AAT, anti-thrombin III and neuroserpin deficiency. To the best of our knowledge, we report the first case of alpha 1-antitrypsin deficiency associated with hypogammaglobulinemia and recurrent pulmonary thrombosis without any concomitant use of drugs.

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Background : We conducted this national survey to quantitatively assess sleep medicine services in the Kingdom of Saudi Arabia (KSA) and to identify obstacles that specialists and hospitals face, precluding the establishment of this service. Materials and Methods: A self-administered questionnaire was designed to collect the following: General information regarding each hospital, information regarding sleep medicine facilities (SFs), the number of beds, the number of sleep studies performed and obstacles to the establishment of SFs. The questionnaire and a cover letter explaining the study objectives were mailed and distributed by respiratory care practitioners to 32 governmental hospitals and 18 private hospitals and medical centers in the KSA. Results: The survey identified 18 SFs in the KSA. The estimated per capita number of beds/year/100,000 people was 0.11 and the per capita polysomnography (PSG) rate was 18.0 PSG/year/100,000 people. The most important obstacles to the progress of sleep medicine in the KSA were a lack of trained sleep technologists and a lack of sleep medicine specialists. Conclusion: The sleep medicine services provided in the KSA have improved since the 2005 survey; however, these services are still below the level of service provided in developed countries. Organized efforts are needed to overcome the identified obstacles and challenges to the progress of sleep medicine in the KSA.

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Food-dependent exercise-induced anaphylaxis (FDEIA) is one of the severe allergic reactions in which symptoms develop only if exercise takes place within a few hours of eating a specific food. It is important to consider FDEIA in cases of unexplained anaphylaxis as reactions can occur several hours after ingesting the culprit food(s). We herein report the first two cases of FDEIA in the Middle East. The first one is induced by wheat, while the other by peanut. The pathophysiology, predisposing factors, diagnosis, and treatment of FDEIA are also summarized here.

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Aim: Rapid on-site evaluation (ROSE) is used widely during endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA). BIOEVALUATOR^® is a device used for determining whether the tissues obtained by EBUS-TBNA are appropriate for a pathological diagnosis. This study describes our experience with ROSE using BIOEVALUATOR^® during EBUS-TBNA for diagnosing pulmonary and mediastinal diseases. Materials And Methods: We retrospectively evaluated the results of 35 patients who underwent EBUS-TBNA with BIOEVALUATOR^® between December 2011 and February 2013. For the diagnosis, the tissue areas were appearing white and red through BIOEVALUATOR^® are considered to be appropriate and inappropriate, respectively. We examined their medical records to obtain information concerning the examination of BIOEVALUATOR^® results of the patient's materials (white/red), the diagnosis yield, site and size of lymph nodes and number of needle passes. Results: The median longest diameter of 40 lymph nodes (21 #7, 13 #4R, 4 #4L and 2 #11) from 35 patients was 27.9 (range 12.4-50.6) mm and the median number of needle passes was 2 (range 1-5). The definitive diagnosis was made by EBUS-TBNA in 28 of 35 patients, by thoracotomy in one patient and BIOEVALUATOR^® results were white and lymphocytes were seen in the rest six patients. The BIOEVALUATOR^® results of other patients without accurate diagnosis were left indefinitive. Finally, the six patients were judged as having benign lymphadenopathy because the lymph node size on computed tomography decreased or remained stable after for at least 8 months. Conclusions: Checking aspirated samples using BIOEVALUATOR^® appears useful for determining their adequacy for pathological diagnosis.

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Background: Anesthesia for bronchoscopy presents unique challenges, as constant stimulus due to bronchoscope needs to be obtunded using drugs with a minimal post-procedure residual effect. Remifentanil for maintenance is an ideal choice, but optimal doses are yet to be determined. Materials and Methods: Bronchoscopic procedures were prospectively evaluated for 4 months studying the frequency of complications and anesthesia techniques. Anesthesia was maintained on remifentanil/propofol infusion avoiding neuromuscular blockers. Laryngeal mask airway was used for the controlled ventilation (with high oxygen concentration) that also served as a conduit for bronchoscope insertions. Anesthesiologists were blinded to the study (avoiding performance bias) and the Pulmonologist was blinded to the anesthesia technique (to document unbiased procedural satisfaction scores). Procedures were divided into 2 groups based on the dose of remifentanil used for maintenance: Group-H (high dose −0.26 to 0.5 μg/kg/min and Group-NH (non-high dose ≤0.25 μg/kg/min). Results: Observed 75 procedures were divided into Group-H (42) and Group-NH (33). Number of statistical difference was found in demography, procedural profile, hemodynamic parameters and total phenylephrine used. Chi-square test showed Group-NH had significantly higher frequency of laryngospasm (P = 0.047) and coughing (P = 0.002). The likelihood ratio of patient coughing and developing laryngospasm in Group-NH was found to be 4.56 and 10.97 times respectively. Minimum pulse-oximeter saturation was statistically higher in Group-H (98.80% vs. 96.50% P = 0.009). Pulmonologist satisfaction scores were significantly better in Group-H. Conclusions: High dose of remifentanil infusion is associated with a lower incidence of coughing and laryngospasms during bronchoscopy. Simultaneously, it improves Pulmonologist's satisfaction and procedural conditions.

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Rationale: Near-fatal asthma (NFA) has not been well studied in Saudi Arabia. We evaluated NFA risk factors in asthmatics admitted to a tertiary-care hospital and described NFA management and outcomes. Materials and Methods: This was a retrospective study of NFA patients admitted to an ICU in Riyadh (2006-2010). NFA was defined as a severe asthma attack requiring intubation. To evaluate NFA risk factors, randomly selected patients admitted to the ward for asthma exacerbation were used as controls. Collected data included demographics, information on prior asthma control and various NFA treatments and outcomes. Results: Thirty NFA cases were admitted to the ICU in the five-year period. Compared to controls (N = 120), NFA patients were younger (37.5 ± 19.9 vs. 50.3 ± 23.1 years, P = 0.004) and predominantly males (70.0% vs. 41.7%, P = 0.005) and used less inhaled steroids/long-acting ß2-agonists combination (13.6% vs. 38.7% P = 0.024. Most (73.3%) NFA cases presented in the cool months (October-March). On multivariate analysis, age (odds ratio [OR] 0.96; 95% confidence interval [CI], 0.92-0.99, P = 0.015) and the number of ED visits in the preceding year (OR, 1.25; 95% CI, 1.00-1.55) were associated with NFA. Rescue NFA management included ketamine (50%) and theophylline (19%) infusions. NFA outcomes included: neuromyopathy (23%), mechanical ventilation duration = 6.4 ± 4.7 days, tracheostomy (13%) and mortality (0%). Neuromuscular blockade duration was associated with neuromyopathy (OR, 3.16 per one day increment; 95% CI, 1.27-7.83). Conclusions: In our study, NFA risk factors were younger age and higher number of ED visits. NFA had significant morbidity. Reducing neuromuscular blockade duration during ventilator management may decrease neuromyopathy risk.

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Introduction: Readmission in the intensive care unit (ICU) is a significant morbidity index, which has been related to poor patient outcomes Aim: To identify the preoperative and intraoperative risk factors for readmission in the cardiac surgery ICU. Methods: We conducted a retrospective cohort study of 595 consecutive patients who were admitted to the cardiac surgery ICU of a tertiary hospital of Athens - Greece during the one-year period (September 2011-September 2012). Data collection was carried out, retrospectively, by the use of a short questionnaire and based on the review of medical and nursing patient records at December 2012. Results: The incidence of ICU readmission was 3.7% (22/595). Respiratory disorders were the main reason for readmission (45.4%). Readmitted patients had a significantly higher in-hospital mortality compared to those requiring no readmission (P < 0.001). Multivariate analysis revealed that female gender [for males odds ratio (OR) 0.37, 95% confidence interval (CI) 0.15-0.89], high logistic EuroSCORE (OR 1.02, 95% CI 1.00-1.04), prolonged cardiopulmonary (CPB) duration (OR 1.01, 95% CI 1.00-1.02) and preoperative renal failure (OR 1.02, 95% CI 1.00-1.05) were the independent risk factors for readmission to the cardiac surgery ICU. Conclusions: One intraoperative and three preoperative variables are associated strongly with higher probability for ICU readmission. Shorter CPB duration could contribute to lower ICU readmission incidence. In addition, the early identification of high risk patients for readmission in the cardiac surgery ICU could encourage both the more efficient healthcare planning and resources allocation.

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Objectives: The objective of this study is to determine the outcome of pulmonary embolism (PE) and the clinico-radiological predictors of mortality in a university hospital setting. Methods: A Prospective observational study conducted at King Khalid University Hospital, Riyadh Saudi Arabia between January 2009 and 2012. A total of 105 consecutive patients (49.9 ± 18.7 years) with PE diagnosed by computed tomography pulmonary angiography were followed until death or hospital discharge. Results: Overall in hospital mortality rate was 8.6%, which is lower than other international reports. Two-thirds of patients developed PE during the hospitalization. The most common risk factors were surgery (35.2%), obesity (34.3%) and immobility (30.5%). The localization of the embolus was central in 32.4%, lobar in 19% and distal in 48.6%. A total of 26 patients (25%) had evidence of right ventricular strain and 14 (13.3%) were hypotensive. Multivariate analysis revealed that heart failure (Beta = −0.53, P < 0.001), palpitation (Beta = −0.24, P = 0.014) and high respiratory rate (Beta = −0.211, P < 0.036) were significant predictors of mortality. There was no significant difference in the localization of the embolus or obstruction score between survivors and non-survivors. Conclusion: The outcome of PE is improving; however, it remains an important risk factor for mortality in hospitalized patients. Congestive heart failure, tachypnea and tachycardia at presentation were associated with higher mortality. These factors need to be considered for risk stratification and management decisions of PE patients. Radiological quantification of clot burden was not a predictor of death.

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Pulmonary rehabilitation (PR) is an integral component of the comprehensive management plan of patients with chronic lung diseases by addressing their functional and psychological deficits. PR is generally recommended to symptomatic patients with chronic lung diseases who develop shortness of breath on their own pace at level ground while receiving optimum therapy. From a regional perspective, this review covers the description of a PR program, its establishment and outcome assessment.

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Background: Secondary spontaneous pneumothorax is difficult to treat and has been thought to have high morbidity and mortality rate due to the underlying diseases and presence of comorbidities in the patients. However, early surgical intervention will be beneficial if it is tolerable by the patient. In the surgical approach for treating pneumothorax, video-assisted thoracoscopic surgery (VATS) may reduce the postoperative drainage period and hospital stay compared with open thoracotomy. Materials and Methods: A retrospective review of the clinical data of 40 patients with secondary spontaneous pneumothorax who underwent open thoracotomy (n = 20) or two-port VATS (n = 20) between January 2008 and December 2012 was performed. Results: Postoperative drainage period of open thoracotomy group and two-port VATS group was 9.85 ± 5.28 and 6.75 ± 2.45, respectively, with a significant inter-group difference. Postoperative hospital stay was 11.8 ± 5.12 in the open thoracotomy group and 8.25 ± 2.88 in the two-port VATS group, with a significant inter-group difference. Recurrence rate and postoperative complication rate were not significant between the two groups. Conclusion: In selected patients with secondary spontaneous pneumothorax treated with surgical approach, two-port VATS resulted in shorter postoperative drainage period and hospital stay compared with open thoracotomy.

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