Cystic fibrosis anesthesia

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Lung transplantation is the only therapy for patients with end-stage lung disease. In advanced lung diseases such as cystic fibrosis (CF), life expectancy increases, and it is important to recognize extrapulmonary comorbidities. Cardiovascular involvement, including pulmonary hypertension, right-heart failure, and myocardial dysfunction, are manifest in the late stages of CF disease. Besides right-heart failure, left-heart dysfunction seems to be underestimated. Therefore, an optimal anesthesia and surgical management risk evaluation in this high-risk patient population is mandatory, especially concerning the perioperative use of mechanical circulatory support...

Intractable nausea can occur in numerous settings. We report on a 49-year-old woman with a past medical history of cystic fibrosis (CF) with chronic hypoxia, chronic nausea, complex infection history and frequent hospitalizations who was admitted to an academic medical center with a CF exacerbation. Her chronic nausea worsened with the use of antimicrobials, and she was unable to tolerate dopamine or serotonin antagonist antiemetics. Nausea persisted despite the use of benzodiazepines and antihistamines. She was given a one-time dose of fosaprepitant 150 mg intravenously (IV) with marked improvement of her nausea...

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OBJECTIVES: Severe hypotension and low systemic vascular resistance in the setting of adequate cardiac output, known as "vasoplegic syndrome" (VS), is a physiologic disturbance reported in 9% to 44% of cardiac surgery patients. Although this phenomenon is well-documented in cardiac surgery, there are few studies on its occurrence in lung transplantation. The goal of this study was to characterize the incidence of VS in lung transplantation, as well as identify associated risk factors and outcomes...

A 15-year-old boy attended Emergency Department with a complaint of difficulty in breathing due to dental infection. A pulmonologist was consulted regarding the severity of the cystic fibrosis. The patient was admitted and intravenous (IV) fluids and antibiotics were given. The infected mandibular right first permanent molar tooth # 30 was extracted under IV ketamine dissociative anesthesia in the hospital setting.

BACKGROUND: Procedural anxiety involves acute distress around medical procedures and may lead to avoidance or resistance behaviors that interfere with effective cystic fibrosis (CF) care and health outcomes. While individuals with CF commonly endure uncomfortable and/or distressing medical procedures, procedural anxiety among children and adolescents with CF has received little research attention. This study investigated the prevalence and correlates of procedural anxiety among individuals with CF aged 6-18 and their parents...

OBJECTIVE: Life expectancy for lung-transplant patients, especially those with cystic fibrosis (CF), is leading increasingly to more retransplantations. DESIGN: Retrospective monocentric cohort study. SETTING: Foch University Hospital, Suresnes, France. PARTICIPANTS: CF patients having had a primary double-lung transplantation (pLgTx) or a retransplantation (reLgTx) from 2012 to 2021. INTERVENTIONS: None...

The incidence of cystic lymphatic malformation (CL) in an extremity is very rare. CL can be a cause of lymphedema in a lower limb. The most effective treatment for CL is sclerotherapy or excision; however, these treatments have the potential to cause fibrosis and obliteration of ruptured lymphatic vessels, which impairs lymphatic drainage and increases the risk of lymphedema. Lymphaticovenular anastomosis (LVA) combined with sclerotherapy may be a minimally invasive treatment option for CL in a lower limb. In this report, we describe a patient with CL complicated by lymphedema in a lower extremity, who we treated using LVA and ethanol sclerotherapy with satisfactory results...

BACKGROUND: Several studies have demonstrated the utility of methylene blue (MB) to treat vasoplegic syndrome (VS), but some have cautioned against its routine use in lung transplantation with only two cases described in prominent literature. Cystic fibrosis patients commonly have chronic infections which predispose them to a systemic inflammatory syndrome-like vasoplegic response during lung transplantation. We present 13 cystic fibrosis patients who underwent lung transplantation and received MB for vasoplegic syndrome while on cardiopulmonary bypass, with or without inhaled pulmonary vasodilator therapy...

The management of lung transplant patients has continued to evolve in recent years. The year 2021 was marked by the publication of the International Consensus Recommendations for Anesthetic and Intensive Care Management of Lung Transplantation. There have been major changes in lung transplant programs over the last few years. This review will summarize the knowledge in anesthesia management of lung transplantation with the most recent data. It will highlight the following aspects which concern anesthesiologists more specifically: (1) impact of COVID-19, (2) future of transplantation for cystic fibrosis patients, (3) hemostasis management, (4) extracorporeal membrane oxygenation management, (5) early prediction of primary graft dysfunction, and (6) pain management...

Clearance of secretions remains a challenge in ventilated patients. Despite high-frequency percussive ventilation (HFPV) showing benefits in patients with cystic fibrosis and neuromuscular disorders, very little is known about its effects on other patient categories. Therefore, we designed a physiological pilot study investigating the effects on lung aeration and gas exchange of short HFPV cycles in tracheostomized patients undergoing mechanical ventilation. Electrical impedance tomography (EIT) was recorded at baseline (T0) by a belt wrapped around the patient's chest, followed by the HFPV cycle lasting 10 min...

BACKGROUND: The Brody II score uses chest CT to guide therapeutic changes in children with cystic fibrosis; however, patients and providers are often reticent to undergo chest CT given concerns about radiation. OBJECTIVE: We sought to determine the ability of a reduced-dose photon-counting detector (PCD) chest CT protocol to reproducibly display pulmonary disease severity using the Brody II score for children with cystic fibrosis (CF) scanned at radiation doses similar to those of a chest radiograph...

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Cystic fibrosis-related diabetes (CFRD) is a common complication of cystic fibrosis (CF), and restoring metabolic control in these patients may improve their management after lung transplantation. In this multicenter, prospective, phase 1-2 trial, we evaluate the feasibility and metabolic efficacy of combined pancreatic islet-lung transplantation from a single donor in patients with CFRD, terminal respiratory failure, and poorly controlled diabetes. Islets were infused via the portal vein under local anesthesia, 1 week after lung transplantation...

Cystic fibrosis (CF) is the most common fatal genetic disease in North America. While CF is more common among Whites, it is increasingly being recognized in other races and ethnicities. Although there is no cure, life expectancy has steadily improved, with the median survival exceeding 46 years in the United States. There are now more adults than children with CF in the United States. CF is caused by mutations in a gene that encodes the cystic fibrosis transmembrane conductance regulator (CFTR) protein, expressed in many epithelial cells...

Cystic fibrosis is a multi-systemic disease of impaired sodium and chloride transport across epithelial surfaces. Cystic fibrosis is one of the most common autosomal recessive diseases among Caucasian children. However, recent epidemiologic studies suggest that the disease in Hispanic, African American, and Asian American populations may be more common than previously recognized. The phenotypic expression is characterized by the constellation of pulmonary, pancreatic, hepatobiliary, and gastrointestinal dysfunction...

INTRODUCTION: When performing computerized tomography chest imaging in children, obtaining high quality, motion-free images is important in the accurate diagnosis of underlying pathology. General anesthesia is associated with the development of atelectasis, which can impair accurate diagnosis by obscuring or altering the appearance of the lung parenchyma or airways. Recruitment maneuvers, performed by anesthesiologists, can be used to effectively re-expand atelectatic lung. METHODS: The computerized tomography chest imaging in 44 children aged between 2 months and 7 years, undergoing serial imaging for monitoring of cystic fibrosis, were reviewed and graded for atelectasis...

Lung transplantation is the last resort for end-stage lung disease treatment. Due to increased survival, lung recipients present an increased likelihood to be submitted to anesthesia and surgery. This case report describes a 23-year-old female patient with history of lung transplantation for cystic fibrosis, with multiple complications, and chronic kidney disease, and who underwent kidney transplantation under general anesthesia. Understanding the pathophysiology and changes related to immunosuppressive therapy is essential to anesthetic technique planning and safety, and for perioperative management...

No abstract text is available yet for this article.