1/6. Endoscopic repair of bronchial dehiscence after lung transplantation.We report a case of bronchial dehiscence after right single lung transplantation and describe a novel means of management: bronchoscopic closure of the defect with alpha-cyanoacrylate glue.- - - - - - - - - - ranking = 1keywords = alpha (Clic here for more details about this article) |
2/6. MVarallo: a new M(Like) alpha 1-antitrypsin-deficient allele.A 73-year-old never-smoker woman with chronic bronchitis, increasing dyspnoea, and airflow limitation with a FEV1 of 49% of predicted value had low serum level of alpha-1-antitrypsin (69 mg/dL, normal range 150-350). isoelectric focusing showed an Mlike pattern. Direct sequencing showed, in the second exon, a particular dna alteration localized between codon 41 and codon 51: a region of 30 base pairs (bp) was completely deleted and substituted by a 22-bp sequence. The resulting loss of 8 bp yields, in the second exon, a 70-71 stop codon. This new Mlike variant was denominated MVarallo from the site where it was discovered.- - - - - - - - - - ranking = 2960.8177735043keywords = antitrypsin, alpha (Clic here for more details about this article) |
3/6. dexmedetomidine as a sole sedating agent with local anesthesia in a high-risk patient for axillofemoral bypass graft: a case report.The alpha2-agonist dexmedetomidine is indicated for sedation of patients receiving mechanical ventilation in the intensive care unit. It has additional off-label uses for coadministration with local, regional, and general anesthesia. This report describes the use of dexmedetomidine as a sole sedating agent in conjunction with local anesthesia for major vascular surgery. A pubmed literature search produced no previous report of the use of dexmedetomidine as a sole sedating agent used in conjunction with local anesthesia. The anxiolytic, hypnotic-sedative, anesthetic-sparing, and analgesic actions of the drug along with the lack of significant respiratory depressant effects are described. The patient required no airway management with the exception of supplemental mask oxygen. He tolerated the procedure well and was discharged without sequelae on the third postoperative day. dexmedetomidine should be used judiciously, and understanding the potential adverse effects and how to treat them is of paramount importance. However, with vigilant intraoperative monitoring of blood pressure, heart rate, and level of consciousness, it can be administered safely, thus lessening the anesthetic requirements and possibly improving the surgical outcome of the high-risk patient. This report describes the indications, dosing, off-label uses, pharmacodynamics, pharmacokinetics, and common adverse effects of dexmedetomidine.- - - - - - - - - - ranking = 1keywords = alpha (Clic here for more details about this article) |
4/6. Nonbacterial thrombotic endocarditis after lung transplantation.bacteria-free verrucae has been recognized as a condition associated with several clinical conditions such as bone marrow transplantation, malignant tumors, autoimmune disorders, and acquired immunodeficiency syndrome, but it has not been reported in relation to lung transplantation. We report the case of a patient who underwent bilateral lung transplant and died 3 days later. Histologic examination revealed, among other lesions, the presence of nonbacterial thrombotic endocarditis in the right atrium and mitral and tricuspid valves that was not present in the preoperative echocardiographic studies. Even with transesophageal echocardiography, a reliable detection of vegetations may not be possible. Hypoxigenic pulmonary states developed in the course of lung transplant could be the factor that triggers the interaction between the coagulation system, platelets, and endothelial cells that induce the formation of bacteria-free verrucae.- - - - - - - - - - ranking = 1.3951941971741keywords = deficiency (Clic here for more details about this article) |
5/6. Alpha1-antitrypsin deficiency: incidence and implications.Alpha1-antitrypsin (AAT) deficiency is an underrecognized inherited disorder with pulmonary and hepatic implications for both adults and neonates. Clinical expressions of AAT deficiency are seen in the lung, liver, and the skin, with considerable variability in the severity of clinical disease. AAT deficiency accounts for nearly 3% of all cases of chronic obstructive pulmonary disease and is responsible for early-onset emphysema in nonsmokers. Ten to twenty percent of affected neonates develop significant liver disease. panniculitis, a rare skin complication of AAT deficiency, is characterized by acute inflammatory infiltrate and fat necrosis. While we concentrate on the pulmonary aspect of AAT deficiency, we have included discussion of liver disease and panniculitis. critical care and advanced practice nurses will benefit from gaining a better understanding of the causes, pathophysiology, diagnosis, and treatment of this disorder.- - - - - - - - - - ranking = 16224.583650567keywords = antitrypsin deficiency, antitrypsin, deficiency (Clic here for more details about this article) |
6/6. Genetic and biophysical basis for bupivacaine-induced ST segment elevation and VT/VF. anesthesia unmasked brugada syndrome.BACKGROUND: brugada syndrome is an inherited disease associated with sudden cardiac death. The electrocardiographic pattern associated with brugada syndrome has been linked to the use of sodium channel blockers, including antiarrhythmics, trycyclics and anesthetics. OBJECTIVE: We report a case of bupivacaine-induced brugada syndrome, in which we investigated the genetic, biophysical and path physiological mechanism involved. methods AND RESULTS: The patient developed a Brugada-like electrocardiographic pattern twice under the influence of bupivacaine. The first occurrence was accompanied by ventricular tachycardia (VT) which subsided after withdrawal of the anesthetic. The VT was also observed during co-administration of diltiazem and isosorbide-5-mononitrate, agents thought to facilitate ST segment elevation in the brugada syndrome. Genetic analysis revealed a missense mutation in the alpha subunit of the cardiac sodium channel, SCN5A. Biophysical analysis by whole-cell patch-clamping revealed a reduction in sodium current as a result of the mutation. The study of bupivacaine in the wedge model revealed use-dependent changes in conduction, heterogeneous loss of the action potential dome in RV epicardium and phase 2 re-entry when the preparations were pretreated with low concentrations of the calcium channel blocker verapamil. CONCLUSION: Our findings indicate that bupivacaine may induce the electrocardiographic and arrhythmic manifestations of the brugada syndrome in silent carriers of SCN5A mutations. The data have important implications in the management of patients who develop ST segment elevation when under the influence of anesthetics such as bupivacaine.- - - - - - - - - - ranking = 1keywords = alpha (Clic here for more details about this article) |