This case is presented to arouse the awareness of the medical staff and nurses to this phenomenon, which can mimic an intrinsic respiratory effort in patients allegedly diagnosed with brain death. Along with this case report, we review the English language publications for similar cases.
IntroductionDyskeratosis congenita is a rare genodermatosis, characterized by a triad of reticular skin pigmentation, nail dystrophy and leukoplakia of mucous membranes. It is also associated with a variety of non-cutaneous abnormalities such as bone marrow failure, malignancy and pulmonary complications. Among its wide range of clinical manifestations, fatal pneumothorax has rarely been reported.Case presentationWe report the case of a 31-year-old Lebanese woman with dyskeratosis congenita who succumbed to devastating bilateral pneumothoraces.ConclusionCareful surveillance of patients with dyskeratosis congenita is required as incipient respiratory failure due to pneumothorax may be successfully treated if detected at an early stage.
BackgroundMetabolic alkalosis is common in patients with respiratory failure and may delay weaning in mechanically ventilated patients. Carbonic anhydrase inhibitors block renal bicarbonate reabsorption, and thus reverse metabolic alkalosis. The objective of this systematic review is to assess the benefits and harms of carbonic anhydrase inhibitor therapy in patients with respiratory failure and metabolic alkalosis.MethodsWe searched the following electronic sources from inception to August 2017: the Cochrane Central Register of Controlled Trials (CENTRAL), MEDLINE, EMBASE, and SCOPUS. Randomized clinical trials were included if they assessed at least one of the following outcomes: mortality, duration of hospital stay, duration of mechanical ventilation, adverse events, and blood gas parameters. Teams of two review authors worked in an independent and duplicate manner to select eligible trials, extract data, and assess risk of bias of the included trials. We used meta-analysis to synthesize statistical data and then assessed the certainty of evidence using the GRADE methodology.ResultsSix eligible studies were identified with a total of 564 participants. The synthesized data did not exclude a reduction or an increase in mortality (risk ratio (RR) 0.94, 95% confidence interval (CI) 0.57 to 1.56) or in duration of hospital stay (mean difference (MD) 0.42 days, 95% CI −4.82 to 5.66) with the use of carbonic anhydrase inhibitors. Carbonic anhydrase inhibitor therapy resulted in a decrease in the duration of mechanical ventilation of 27 h (95% CI −50 to −4). Also, it resulted in an increase in PaO2 (MD 11.37 mmHg, 95% CI 4.18 to 18.56) and a decrease in PaCO2 (MD −4.98 mmHg, 95% CI −9.66, −0.3), serum bicarbonate (MD −5.03 meq/L, 95% CI −6.52 to −3.54), and pH (MD −0.04, 95% CI −0.07 to −0.01). There was an increased risk of adverse events in the carbonic anhydrase inhibitor group (RR 1.71, 95% CI 0.98 to 2.99). Certainty of evidence was judged to be low for most outcomes.ConclusionIn patients with respiratory failure and metabolic alkalosis, carbonic anhydrase inhibitor therapy may have favorable effects on blood gas parameters. In mechanically ventilated patients, carbonic anhydrase inhibitor therapy may decrease the duration of mechanical ventilation. A major limitation of this finding was that only two trials assessed this clinically important outcome.Electronic supplementary materialThe online version of this article (10.1186/s13054-018-2207-6) contains supplementary material, which is available to authorized users.
The historic perspective that used to define chronic obstructive pulmonary disease has changed. As reviewed in this article, it is based on a better understanding of the underlying inflammatory airflow obstruction and a multidimensional classification, which mostly targets a subgroup called 'frequent exacerbators'. Clinical and radioimaging predictors are the stamina for an aggressive therapeutic approach. A simplified explanation of the updated Global Initiative for Obstructive Lung Disease guidelines will ease the burden of treatment selection.
Asthma has classically been categorized into several phenotypes to address the complexities of this disease. However, phenotypes only cover the clinically relevant aspects of the disease, but do not address the relationship between the disease and its etiology and pathophysiology. This led to the development of the term "endotypes" which links key pathogenic mechanisms with asthma phenotypes, and ultimately leads to better selection of treatment resulting in improved response. Although the exact pathogenesis of asthma is still under investigation, targeted-therapy based on asthma phenotypes and endotypes has shown some success, and the future appears promising for patients suffering from severe asthma since treatment is being tailored according to individual biology. We review in this manuscript the best evidence and updates currently available in the classification and treatment recommendations for severe asthma.
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