Despite significant advancements made in life expectancy over the past century, cystic fibrosis remains a life-threatening genetic disease that affects the gastrointestinal tract, and it has significant impact on the nutrition status of those with the disease. Nutrition management includes a high-calorie/high-fat diet, pancreatic enzyme replacement therapy, vitamin and mineral replacement, and enteral support as needed. As patients are living longer, clinicians may encounter patients with cystic fibrosis in obstetrician offices, endocrine clinics, or hospital settings, owing to lung transplantation or for treatment for distal intestinal obstruction syndrome.

Considerable variation exists in the utilization of ventilator support among infants of closely related gestational age. In addition, a number of medical risk factors influence the need for, and length of, assisted ventilation. These models explain much of the variance in LOV among preterm infants but explain substantially less among term infants.neonatal intensive care, assisted ventilation, Score for Neonatal Acute Physiology, resource consumption, prematurity.

Rationale. Cystic fibrosis related diabetes (CFRD) is the most common comorbidity in patients with CF. In spite of increased screening, diagnosis, and treatment of CFRD, the mortality rate in patients with CFRD still far exceeds the mortality rate in those without CFRD. Guidelines suggest that screening for CFRD be performed annually using the 2-hour 75-gram oral glucose tolerance test (OGTT). Adherence to recommended screening has been poor, with only approximately one-quarter of adults with CF undergoing OGTT in 2014. Use of continuous glucose monitoring (CGM) for diagnosis may become an alternative. Objectives. Our objective was to determine whether abnormal CGM predicts subsequent development of CFRD, lung function, and body mass index (BMI) decline and increased rate of CF pulmonary exacerbations in adults with CF. Methods. In a prospective single center pilot trial from September 2009 to September 2010, 21 adult patients due for routine OGTT were recruited to complete simultaneous 3-day CGM and 2-hour 75 gram OGTT. Subsequently, clinical information was reviewed from 2008 to 2015. Conclusions. There was a moderate correlation between interpreted results of 2-hour OGTT and CGM (p = 0.03); CGM indicated a greater level of glucose impairment than OGTT. Glucose >200 mg/dL by CGM predicted development of CFRD (p = 0.0002).

No abstract

No abstract

IntroductionTherapeutic advances have markedly increased life expectancy for those with cystic fibrosis (CF), resulting in a median predicted survival over 50 years. Consequently, people with CF (pwCF) are living through their reproductive years and the rate of pregnancy is rapidly rising. Despite the increased relevance of this topic, multicentre studies investigating the association between maternal health and choices made during pregnancy on maternal and fetal outcomes do not exist. Furthermore, there are very limited data on the outcomes following CF transmembrane conductance regulator (CFTR) modulator use during pregnancy and lactation.Methods and analysisMaternal and Fetal Outcomes in the Era of Modulators (MAYFLOWERS) is a prospective, multicentre observational clinical trial which will enrol approximately 285 pregnant pwCF including those who are modulator ineligible and those who choose to continue or discontinue CFTR modulator therapy during pregnancy and lactation. The primary aim of this 35-month study is to assess whether lung function changes during pregnancy differ based on the continued use of modulators or other factors such as pre-existing comorbid conditions. Secondary objectives include evaluation of pregnancy related and obstetrical complications and changes in mental health.Ethics and disseminationThe design of this study required special consideration of study burden on pregnant and lactating people with chronic illness in the setting of a substantial number of unanswered questions under these conditions. MAYFLOWERS is the first prospective clinical trial examining pregnancy in CF; the outcomes will guide providers on pregnancy management in pwCF and others with chronic respiratory disease.

Background: Obtaining informed consent is an important responsibility of all doctors and is a major component of their day-to-day practice. However, little is known regarding practising doctors' understanding of consent in relation to medical law. Aims: To gain insights into current doctors' understanding of the legal requisites that underpin the consent of patients to medical procedures in Australia. Methods: A cross-sectional survey of Western Australian medical practitioners was conducted. A 15-question online questionnaire (SurveyMonkey, USA) was developed and distributed to Western Australia medical practitioners via social media, hospitalbased Junior doctor society pages and through the email accounts of practitioners registered with MDA National -a large medical defence organisation. Doctors were questioned on their understanding of medicolegal responsibilities, informed consent practice and knowledge of a historically significant Australian medicolegal case (Rogers v Whitaker, 1992). Results: A total of 172 responses was received during the survey period. The respondents came from various levels of seniority and from a variety of subspecialist areas. The survey demonstrated that among the respondents, the understanding of their medicolegal responsibilities around the issues of informed consent was deficient. Only 31% of respondents were aware that it is a court of law that defines the reasonable standard of care in relation to obtaining informed consent. Less than half of the respondents (48%) were aware of the High Court of Australia's definition by which the standard of reasonable care is defined. Conclusion:The results from our survey suggest that there is a requirement to enhance the education of medical practitioners to meet the medicolegal requirements and optimise consent.

Background Physicians often lack the skills and confidence needed to have difficult conversations with patients and their families. Patients and families who have experienced these conversations can provide valuable insight for resident physicians. Objective We developed a communication skills workshop for pediatrics residents using parents and a team of social workers, nurses, chaplains, and physician facilitators in role-playing exercises. Methods From 2007 to 2016, half-day ''difficult conversation'' workshops were held annually for postgraduate year 1 (PGY-1) and PGY-2 residents that included an interprofessional team and parents of children with life-threatening diagnoses. Questionnaires assessed residents' prior training, effectiveness of the sessions, and narrative feedback on the impact of this approach. Parents and team members were surveyed on the effectiveness of the training and the value of parent involvement. Results Median self-reported confidence levels for incoming PGY-1 residents following the workshop rose from 2 to 4 on a 5point Likert scale (99% response rate [128 of 129 surveyed], P , .001). The majority of PGY-2 residents (91%, 115 of 126) reported the workshop increased their confidence in engaging in difficult conversations (91% response rate [126 of 139]). Parents and clinical care team members agreed that parents would likely be preferable to standardized actors for these types of role-playing exercises (84% response rate [37 of 44]). Conclusions Involving patients' parents and an interprofessional team in role-playing scenarios was a well-received method for teaching residents how to engage in difficult conversations with patients and families, and improved their self-reported confidence when having these conversations.