Challenges in the diagnosis and management of severe Pneumocystis jirovecii pneumonia in a non-HIV-infected patient - A case report

A 64-year-old woman was referred to our hospital due to progressive dypnoea for the past week, combined with fever and type 1 respiratory failure. White blood cell count and procalcitonin level were normal. The Chest X-ray showed bilateral disseminated pulmonary infiltrates. Within the next 24 hours the patient developed a severe ARDS. A first diagnostic work-up for typical and atypical pathogens as well as serological tests for CMV, RSV, HIV and HSV were negative. Analysis of a second bronchoalveolar lavage fluid revealed Pneumocystis jiroveci DNA. The patient was successfully treated with trimethoprim-sulfamethoxazole and off label use with caspofungin. The cause of the infection was a six week treatment with dexamethasone. The patient developed a toxic epidermal necrolysis during further course, but completely recovered. Pneumonia with Pneumocystis jirovecii must also be taken into account in non-HIV patients, whenever there are any indications that cellular immunity may be depressed

Core myopathies and risk of malignant hyperthermia

In this article, we analyze myopathies with cores, for which an association to malignant hyperthermia (MH) has been suggested. We discuss the clinical features, the underlying genetic defects, subsequent effects on cellular calcium metabolism, and in vitro muscle responses to MH triggers. We describe in detail central core disease, multiminicore disease, and nemaline rod myopathy. We categorize the diseases according to the affected proteins and discuss the risk for MH, which is high or theoretically possible when the calcium-conducting proteins are affected

European Neuromuscular Centre consensus statement on anaesthesia in patients with neuromuscular disorders

BACKGROUND Patients with neuromuscular conditions are at increased risk of suffering peri-operative complications related to anaesthesia. There is currently little specific anaesthetic guidance concerning these patients. Here we present the European Neuromuscular Centre (ENMC) consensus statement on anaesthesia in patients with neuromuscular disorders as formulated during the 259th ENMC workshop on Anaesthesia in neuromuscular disorders. METHODS International experts in the field of (paediatric) anaesthesia, neurology and genetics were invited to participate in the ENMC workshop. A literature search was conducted in PubMed and EMBASE whose main findings were disseminated to the participants and presented during the workshop. Depending on specific expertise, participants presented the existing evidence and their expert opinion concerning anaesthetic management in six specific groups of myopathies and neuromuscular junction disorders. The consensus statement was prepared according to the Appraisal of Guidelines for REsearch & Evaluation (AGREE II) reporting checklist. The level of evidence has been adapted according to the Scottish Intercollegiate Guidelines Network (SIGN) grading system. The final consensus statement was subjected to a modified Delphi process. RESULTS A set of general recommendations valid for the anaesthetic management of patients with neuromuscular disorders in general have been formulated. Specific recommendations were formulated for 1) neuromuscular junction disorders; 2) muscle channelopathies (non-dystrophic myotonia and periodic paralysis); 3) myotonic dystrophy (type 1 and 2); 4) muscular dystrophies; 5) congenital myopathies and congenital dystrophies and 6) mitochondrial and metabolic myopathies. CONCLUSION This ENMC consensus statement summarizes the most important considerations for planning and performing anaesthesia in patients with neuromuscular disorders

The European Neuromuscular Centre Consensus Statement on Anaesthesia in Patients with Neuromuscular Disorders.

BACKGROUND Patients with neuromuscular conditions are at increased risk of suffering peri-operative complications related to anaesthesia. There is currently little specific anaesthetic guidance concerning these patients. Here we present the European Neuromuscular Centre (ENMC) consensus statement on anaesthesia in patients with neuromuscular disorders as formulated during the 259th ENMC workshop on Anaesthesia in neuromuscular disorders. METHODS International experts in the field of (paediatric) anaesthesia, neurology and genetics were invited to participate in the ENMC workshop. A literature search was conducted in PubMed and EMBASE whose main findings were disseminated to the participants and presented during the workshop. Depending on specific expertise, participants presented the existing evidence and their expert opinion concerning anaesthetic management in six specific groups of myopathies and neuromuscular junction disorders. The consensus statement was prepared according to the Appraisal of Guidelines for REsearch & Evaluation (AGREE II) reporting checklist. The level of evidence has been adapted according to the Scottish Intercollegiate Guidelines Network (SIGN) grading system. The final consensus statement was subjected to a modified Delphi process. RESULTS A set of general recommendations valid for the anaesthetic management of patients with neuromuscular disorders in general have been formulated. Specific recommendations were formulated for 1) neuromuscular junction disorders; 2) muscle channelopathies (non-dystrophic myotonia and periodic paralysis); 3) myotonic dystrophy (type 1 and 2); 4) muscular dystrophies; 5) congenital myopathies and congenital dystrophies and 6) mitochondrial and metabolic myopathies. CONCLUSION This ENMC consensus statement summarizes the most important considerations for planning and performing anaesthesia in patients with neuromuscular disorders

Post-anaesthesia pulmonary complications after use of muscle relaxants (POPULAR): a multicentre, prospective observational study

Background Results from retrospective studies suggest that use of neuromuscular blocking agents during general anaesthesia might be linked to postoperative pulmonary complications. We therefore aimed to assess whether the use of neuromuscular blocking agents is associated with postoperative pulmonary complications. Methods We did a multicentre, prospective observational cohort study. Patients were recruited from 211 hospitals in 28 European countries. We included patients (aged ≥18 years) who received general anaesthesia for any in-hospital procedure except cardiac surgery. Patient characteristics, surgical and anaesthetic details, and chart review at discharge were prospectively collected over 2 weeks. Additionally, each patient underwent postoperative physical examination within 3 days of surgery to check for adverse pulmonary events. The study outcome was the incidence of postoperative pulmonary complications from the end of surgery up to postoperative day 28. Logistic regression analyses were adjusted for surgical factors and patients’ preoperative physical status, providing adjusted odds ratios (ORadj) and adjusted absolute risk reduction (ARRadj). This study is registered with ClinicalTrials.gov, number NCT01865513. Findings Between June 16, 2014, and April 29, 2015, data from 22803 patients were collected. The use of neuromuscular blocking agents was associated with an increased incidence of postoperative pulmonary complications in patients who had undergone general anaesthesia (1658 [7·6%] of 21694); ORadj 1·86, 95% CI 1·53–2·26; ARRadj –4·4%, 95% CI –5·5 to –3·2). Only 2·3% of high-risk surgical patients and those with adverse respiratory profiles were anaesthetised without neuromuscular blocking agents. The use of neuromuscular monitoring (ORadj 1·31, 95% CI 1·15–1·49; ARRadj –2·6%, 95% CI –3·9 to –1·4) and the administration of reversal agents (1·23, 1·07–1·41; –1·9%, –3·2 to –0·7) were not associated with a decreased risk of postoperative pulmonary complications. Neither the choice of sugammadex instead of neostigmine for reversal (ORadj 1·03, 95% CI 0·85–1·25; ARRadj –0·3%, 95% CI –2·4 to 1·5) nor extubation at a train-of-four ratio of 0·9 or more (1·03, 0·82–1·31; –0·4%, –3·5 to 2·2) was associated with better pulmonary outcomes. Interpretation We showed that the use of neuromuscular blocking drugs in general anaesthesia is associated with an increased risk of postoperative pulmonary complications. Anaesthetists must balance the potential benefits of neuromuscular blockade against the increased risk of postoperative pulmonary complications