Perioperative Management and Preemptive ECMO Cannulation of a Parturient with Cystic Fibrosis Undergoing Cesarean Delivery

Physiologic changes of pregnancy and cystic fibrosis pathology provide a unique set of circumstances. Pulmonary disease accounts for over 90% of the morbidity and mortality of patients with cystic fibrosis. These abnormalities create anesthetic challenges due to multiple organ systems being affected including the respiratory, gastrointestinal, cardiovascular, and genitourinary tracts, where patients present with chronic respiratory failure, pancreatic insufficiency, poor nutrition, and cardiac manifestations. We present the perianesthetic management of a parturient with cystic fibrosis who successfully underwent preterm cesarean delivery under neuraxial anesthesia with preemptive bilateral femoral venous sheaths placed for potential extracorporeal membrane oxygenation (ECMO) initiation.

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Management of the Child With Cystic Fibrosis

Journal of Pharmacy Practice ◽

10.1177/089719009600900107 ◽

1996 ◽

Vol 9 (1) ◽

pp. 75-90

Author(s):

Jennifer H. Tran ◽

Karen M. Brennan

Keyword(s):

Cystic Fibrosis ◽

Gastrointestinal Tract ◽

Neonatal Period ◽

Pancreatic Insufficiency ◽

Childhood Illness ◽

Serum Concentrations ◽

Enzyme Replacement ◽

Organ Systems ◽

Made In

Cystic fibrosis is one of the most common lethal inherited diseases among the Caucasian population, with an incidence of 1 in 2,000. With the progress made in the management of the disease, a once-regarded childhood illness has now an improved survival rate of up to 30 years. It is a multifaceted disease affecting a number of organ systems primarily pulmonary and gastrointestinal tract, with the former leading to most of the mortality. Therefore, good pulmonary toilet, including daily chest physiotherapy and appropriate antibiotic treatment for acute pulmonary exacerbations, remains the cornerstone of therapy. Disease-specific pharmacokinetics seen in these patients require special dosing considerations specifically for antibiotics to ensure adequate serum concentrations. In addition, bronchodilators, steroids, and mucolytics also play a role. With respect to the gastrointestinal tract, pancreatic insufficiency occurs and requires enzyme replacement. Intestinal obstruction may occur as early as the neonatal period, as "meconium ileus," and recur throughout the patient's lifespan. More recent modalities including chloride-channel facilitators, antiproteases, and gene therapy may hold promise to further improve the survival and quality of life in these individuals. The pharmacists' role is vital, especially with the unique pharmacokinetic considerations specific to this population and the complexity of medications necessary for appropriate management of the disease. Copyright © 1996 by W.B. Saunders Company

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Toll like Receptor signalling by Prevotella histicola activates alternative NF-κB signalling in Cystic Fibrosis bronchial epithelial cells compared to P.aeruginosa

10.1101/2020.06.24.168682 ◽

2020 ◽

Author(s):

A. Bertelsen ◽

J.S. Elborn ◽

B.C. Schock

Keyword(s):

Cystic Fibrosis ◽

Epithelial Cells ◽

Bacterial Infections ◽

Bacterial Species ◽

Multiple Organ ◽

Bronchial Epithelial Cells ◽

Toll Like Receptor ◽

Bronchial Epithelial ◽

Hek 293 ◽

Organ Systems

AbstractCystic Fibrosis (CF), caused by mutations affecting the CFTR gene, is characterised by viscid secretions in multiple organ systems. CF airways contain thick mucus, creating a gradient of hypoxia, which promotes the establishment of polymicrobial infection. Such inflammation predisposes to further infection, a self-perpetuating cycle in mediated by NF-κB. Anaerobic Gram-negative Prevotella spp. are found in sputum from healthy volunteers and CF patients and in CF lungs correlate with reduced levels of inflammation. Prevotella histicola (P.histicola) can suppress murine lung inflammation, however, no studies have examined the role of P.histicola in modulating infection and inflammation in the CF airways. We investigated innate immune signalling and NF-kB activation in CF epithelial cells CFBE41o-in response to clinical stains of P.histicola and Pseudomonas aeruginosa (P.aeruginosa). Toll-Like Receptor (TLR) expressing HEK-293 cells and siRNA assays for TLRs and IKKa were used to confirm signalling pathways.We show that P.histicola infection activated the alternative NF-kB signalling pathway in CF bronchial epithelial cells inducing HIF-1α protein. TLR5 signalling was responsible for the induction of the alternative NF-kB pathway through phosphorylation of IKKα. The induction of transcription factor HIF-1α was inversely associated with the induction of the alternative NF-kB pathway and knockdown of IKKα partially restored canonical NF-kB activation in response to P.histicola.This study demonstrates that different bacterial species in the respiratory microbiome can contribute differently to inflammation, either by activating inflammatory cascades (P.aeruginosa) or by muting the inflammatory response by modulating similar or related pathways (P.histicola). Further work is required to assess the complex interactions of the lung microbiome in response to mixed bacterial infections and their effects in people with CF.

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Respiratory failure: Classification, Epidemiology and Etiology Review

Journal La Medihealtico ◽

10.37899/journallamedihealtico.v1i2.96 ◽

2020 ◽

Vol 1 (2) ◽

pp. 15-20

Author(s):

Vergel Ernest

Keyword(s):

Respiratory Failure ◽

Acute Respiratory Failure ◽

Mortality Rates ◽

Multiple Organ ◽

Oxygen Intake ◽

Multiple Organ Dysfunction ◽

Central Nervous System Depression ◽

Organ Systems ◽

Failure Classification ◽

Carbon Dioxide Release

Respiratory failure is a detailed syndrome of the respiratory system failing to perform the function of gas exchange, oxygen intake, and carbon dioxide release. Acute respiratory failure is the most common cause of organ failure in intensive care units (ICUs) with high mortality rates. The classification can be reviewed from two categories of acute respiratory failure and chronic respiratory failure. Acute respiratory failure is often found with the failure of other vital organs. Death is caused by multiple organ dysfunction syndrome (MODS). Etiology can be seen in two categories, namely Central nervous system Depression, Impaired ventilation, Impaired equilibrium perfusion ventilation (V/Q Mismatch), Trauma, Pleural effusion, hemothorax, and pneumothorax, Acute pulmonary disease. Respiratory failure, like failure in other organ systems, can be identified based on clinical features or laboratory tests.

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Cystic fibrosis presenting with hypokalemia and metabolic alkalosis in a previously healthy adolescent.

Journal of the American Society of Nephrology ◽

10.1681/asn.v82352 ◽

1997 ◽

Vol 8 (2) ◽

pp. 352-355

Author(s):

C M Bates ◽

M Baum ◽

R Quigley

Keyword(s):

Cystic Fibrosis ◽

Metabolic Alkalosis ◽

Extracellular Fluid ◽

Multiple Organ ◽

Healthy Adolescent ◽

Volume Contraction ◽

Warm Weather ◽

Organ Systems ◽

Cystic Fibrosis Transmembrane ◽

Sweat Sodium

Cystic fibrosis (CF) is an exocrine disease affecting multiple organ systems. Patients with CF usually present with respiratory or gastrointestinal abnormalities. This study presents a case of a previously healthy 17-yr-old man who was diagnosed with CF after presenting with metabolic alkalosis and hypokalemia. The defect associated with CF is in the cystic fibrosis transmembrane regulator (CFTR), which acts primarily as a chloride channel. Partially functional CFTR may be associated with less severe pulmonary and gastrointestinal manifestations, as in the case presented. Dysfunctional CFTR in the sweat ducts of CF patients are responsible for excessive chloride and sodium losses, especially in warm weather. Hypokalemia seen with heat stress is secondary to sweat as well as renal potassium wasting. Metabolic alkalosis is maintained by the excessive sweat sodium chloride losses which leads to extracellular fluid (ECF) volume contraction and chloride depletion. Generation of alkalosis may be related to dysfunctional CFTR in the kidney, but is most likely secondary to hypokalemia with ECF volume contraction. Finally, one must consider CF when confronted with hypokalemia and alkalosis in a previously healthy patient.

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Cystic fibrosis and caseload midwifery

British Journal of Midwifery ◽

10.12968/bjom.2021.29.12.712 ◽

2021 ◽

Vol 29 (12) ◽

pp. 712-717

Author(s):

Sophie Borges

Keyword(s):

Cystic Fibrosis ◽

Multiple Organ ◽

Holistic Care ◽

Health And Wellbeing ◽

Caseload Midwifery ◽

Organ Systems ◽

Psychosocial Implications ◽

Small Team ◽

Improve Health ◽

Made In

Midwives must provide woman-centred, holistic care for the diverse UK childbearing population. As the NHS moves to adopt the recommendations made in the ‘Better Births’ report, is there an argument to invest in protected caseload midwifery contacts for women with cystic fibrosis? Caseload midwifery refers to a continuity model where a small team of midwives provide care throughout the antenatal, intrapartum and postnatal continuum. Cystic fibrosis affects multiple organ systems and requires specialist medical management during pregnancy. Living with cystic fibrosis has many psychosocial implications and pregnancy presents additional challenges. Health and wellbeing outcomes are improved when individuals are treated holistically in the non-pregnant population; therefore, during pregnancy, birth and postnatally, caseload midwifery may provide a legitimate intervention to improve health outcomes in pregnant women with cystic fibrosis.

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Preoperative Evaluation of the Vascular Patient

10.2310/vasc.3005 ◽

2015 ◽

Author(s):

Issam Koleilat ◽

Christopher G. Carsten

Keyword(s):

Perioperative Management ◽

Elective Surgery ◽

Coronary Revascularization ◽

Risk Index ◽

Preoperative Evaluation ◽

Heart Association ◽

Antiplatelet Agents ◽

Cardiac Risk ◽

Multiple Organ ◽

Organ Systems

Almost as critical as an operation itself is the preparation of the patient. Although this often includes psychosocial concerns such as expectations of recovery, inpatient stay, and other patient-centered issues, the discussion prior to surgery should not be limited to these factors. A medical assessment of the patient’s fitness and physiologic preparedness for the planned procedure must be performed by the surgeon and the resultant findings and plan reviewed with the patient. Although vascular disease affects multiple organ systems requiring a thorough general preoperative patient assessment, the focus of preoperative risk reduction strategies center on cardiac outcomes. Therefore, this review focuses on cardiac-related interventions with added coverage of preoperative strategies regarding diabetes, pulmonary and renal risk assessment, and infection reduction. Lastly, the perioperative management of anticoagulation/antiplatelet medications and cerebrovascular disease are discussed Techniques and treatments to optimize patients for surgery are integrated into the respective sections, allowing for a primer to guide this critical phase in a patient’s journey through surgery. Tables outline the Revised Cardiac Risk Index, assessment of functional capacity from patient self-reported activities, optimal delay in elective surgery after percutaneous coronary revascularization according to the 2014 American College of Cardiology/American Heart Association clinical practice guidelines, Respiratory Failure Risk Index, Szilagyi classification of vascular surgical site infection, and recommendations regarding perioperative management of anticoagulants and antiplatelet agents. A suggested algorithm for preoperative cardiac workup and the Cockcroff-Gault equation are provided. This review contains 2 figures, 6 tables, and 115 references.

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Cystic fibrosis - general review on sinonasal complications and case report

Romanian Journal of Rhinology ◽

10.1515/rjr-2017-0004 ◽

2017 ◽

Vol 7 (25) ◽

pp. 33-37

Author(s):

Claudiu Manea ◽

Alina Diaconescu

Keyword(s):

Quality Of Life ◽

Cystic Fibrosis ◽

Case Report ◽

Life Expectancy ◽

Multiple Organ ◽

Lower Airway ◽

General Review ◽

Organ Systems ◽

Sinonasal Disease

Abstract An irreversible disease, cystic fibrosis (CF), is responsible for affecting multiple organ systems containing epithelia. It is well known that the sinonasal disease caused by CF has consequences for the incidence of the lower airway exacerbations, as well as affecting the quality of life of those patients. This review provides an update by evaluating the available literature regarding pathogenesis, management and treatment of cystic fibrosis patients. To gain a better view of the disease and obtain a higher life expectancy, further studies are needed.

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Case Study: Cystic Fibrosis in the Newborn

Neonatal Network The Journal of Neonatal Nursing ◽

10.1891/0730-0832.37.3.164 ◽

2018 ◽

Vol 37 (3) ◽

pp. 164-168

Author(s):

Paulina A. Saravia ◽

Cheryl Riley

Keyword(s):

Cystic Fibrosis ◽

Family Support ◽

Clinical Course ◽

Genetic Disorders ◽

Pancreatic Insufficiency ◽

Interdisciplinary Team ◽

Pulmonary Complications ◽

Effective Management ◽

Organ Systems

Cystic fibrosis (CF) is considered one of the most commonly occurring fatal genetic disorders. This disorder is associated with pancreatic insufficiency and pulmonary complications. However, at birth the initial complications are associated with bowel obstruction. Cystic fibrosis management warrants an interdisciplinary team because this disorder affects various organ systems. Effective management of the newborn with CF assists in improving the child’s overall prognosis. Family support is critical throughout the prenatal and postnatal periods. The case presented reviews a child born with suspected CF and the clinical course within the NICU.

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Mycoplasma Pneumoniae Associated with Stevens Johnson Syndrome

Anaesthesia and Intensive Care ◽

10.1177/0310057x0703500317 ◽

2007 ◽

Vol 35 (3) ◽

pp. 414-417 ◽

Cited By ~ 24

Author(s):

J. M. Mulvey ◽

A. Padowitz ◽

M. Lindley-Jones ◽

R. Nickels

Keyword(s):

Respiratory Failure ◽

Mycoplasma Pneumoniae ◽

Multiple Organ ◽

Stevens Johnson Syndrome ◽

Skin Reactions ◽

Genital Ulceration ◽

Johnson Syndrome ◽

Organ Systems ◽

Prolonged Ventilation ◽

Tract Involvement

We describe a case of Mycoplasma pneumoniae chest infection associated with Stevens Johnson syndrome. The patient had extensive epidermal bullous vesicles, oropharyngeal and genital ulceration and required prolonged ventilation due to respiratory failure. Mycoplasma pneumoniae infections are often asymptomatic but can involve multiple organ systems. Respiratory tract involvement is generally benign though 3 to 10% of patients develop clinical pneumonia. Secondary skin reactions are common (20 to 25%), although few patients infected develop Stevens Johnson syndrome. It has been suggested that Mycoplasma pneumoniae may be the most common infectious cause of Stevens Johnson syndrome.

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Acute respiratory failure as primary manifestation of antineutrophil cytoplasmic antibodies-associated vasculitis

Clinics and Practice ◽

10.4081/cp.2014.653 ◽

2014 ◽

Vol 4 (2) ◽

Author(s):

Evdokia Sourla ◽

Vasilis Bagalas ◽

Helias Tsioulis ◽

Asimina Paspala ◽

Sofia Akritidou ◽

...

Keyword(s):

Respiratory Failure ◽

Acute Respiratory Failure ◽

Distress Syndrome ◽

Clinical Presentation ◽

Ventilatory Support ◽

Multiple Organ ◽

Alveolar Hemorrhage ◽

Antineutrophil Cytoplasmic Antibodies ◽

Organ Systems ◽

Hypoxic Respiratory Failure

The systemic vasculitides are multifocal diseases characterized by the presence of blood vessel inflammation in multiple organ systems. Their clinical presentation is variable extending from self-limited illness to critical complications including diffuse alveolar hemorrhage and glomerulonephritis. Alveolar hemorrhage is a lifethreatening manifestation of pulmonary vasculitis that can rapidly progress into acute respiratory failure requiring ventilatory support. We present the case of a 74-year-old patient admitted to the Intensive Care Unit with severe hypoxic respiratory failure and diffuse alveolar infiltrates in chest imaging that was later diagnosed as antineutrophil cytoplasmic antibodies-associated vasculitis. The report highlights the importance of differentiate between alveolar hemorrhage and acute respiratory distress syndrome of other etiology because alveolar hemorrhage is reversible with prompt initiation of treatment.

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