Half a century of continuous pacing: a living witness to the evolution of a technology.

To chart the development of pacing technology and its pitfalls we present the experience of a patient who has benefitted from it but also suffered as a result of it from its earliest days. A 53-year-old physician was referred to us with obstruction of the superior and inferior vena cava on a background of more than 50 years of continuous ventricular pacing and 24 previous pacemaker-related interventions. In a single surgical procedure, his existing pacing system and redundant leads were extracted, the superior vena cava was reconstructed, and a new biventricular pacing system with epicardial leads was implanted. Pacemakers can maintain life and preserve the quality of life for many decades. The quality of this therapy has improved due to advances in the technology and in techniques. Maintaining safe pacing in the very long term requires labour, patience, and ingenuity.

Preterm Infant Outcomes after Randomization to Initial Resuscitation with FiO2 0.21 or 1.0.

OBJECTIVE: To determine rates of death or neurodevelopmental impairment (NDI) at 2 years corrected age (primary outcome) in children <32 weeks' gestation randomized to initial resuscitation with a fraction of inspired oxygen (FiO2) value of 0.21 or 1.0. STUDY DESIGN: Blinded assessments were conducted at 2-3 years corrected age with the Bayley Scales of Infant and Toddler Development, Third Edition or the Ages and Stages Questionnaire by intention to treat. RESULTS: Of the 290 children enrolled, 40 could not be contacted and 10 failed to attend appointments. Among the 240 children for whom outcomes at age 2 years were available, 1 child had a lethal congenital anomaly, 1 child had consent for follow-up withdrawn, and 23 children died. The primary outcome, which was available in 238 (82%) of those randomized, occurred in 47 of the 117 (40%) children assigned to initial FiO2 0.21 and in 38 of the 121 (31%) assigned to initial FiO2 1.0 (OR, 1.47; 95% CI, 0.86-2.5; P = .16). No difference in NDI was found in 215 survivors randomized to FiO2 0.21 vs 1.0 (OR, 1.26; 95% CI, 0.70-2.28; P = .11). In post hoc exploratory analyses in the whole cohort, children with a 5-minute blood oxygen saturation (SpO2) <80% were more likely to die or to have NDI (OR, 1.85; 95% CI, 1.07-3.2; P = .03). CONCLUSIONS: Initial resuscitation of infants <32 weeks' gestation with initial FiO2 0.21 had no significant effect on death or NDI compared with initial FiO2 1.0. Further evaluation of optimum initial FiO2, including SpO2 targeting, in a large randomized controlled trial is needed. TRIAL REGISTRATION: Australian and New Zealand Clinical Trials Network Registry ACTRN 12610001059055 and the National Malaysian Research Registry NMRR-07-685-957.

Emergency department utilization by a jail population.

BACKGROUND: Incarcerated individuals represent a significant proportion of the US population and face unique healthcare challenges. Scarce articles have been published about emergency department (ED) care of these patients. We studied the ED visits from one urban jail to better describe this population. METHODS: A cohort study design was used, identifying patients who were sent to the ED from a city jail in 2015. Demographics, triage information, length of stay, number of studies, billing codes, diagnoses, and disposition data were collected. These were compared to the overall ED patient population in the same year. RESULTS: 868 ED visits by jail patients occurred, representing 1.3% of the ED census. Compared to the general population, incarcerated patients were younger (32.1 years vs. 44.0 years, p < .01), healthier based on Elixhauser comorbidity scores (0.71 vs. 0.98, p < .01), and had lower admission rates (11.29% vs. 21.54%, p < .01). An abnormal vital sign was noted in 25% of incarcerated patients. Laboratory (61% vs. 57%, p < .02) and radiologic (63% vs 45%, p < .001) testing was more frequent for inmates and length of stay was longer (271 vs. 225 min, p < .01). CONCLUSION: ED visits from jail were common, involving a relatively young and healthy population with a low incidence of abnormal vital signs and admission. Given the high costs associated with ED care and the medical resources available at some jails, further study should evaluate if increased jail medical capabilities could improve care and decrease costs by decreasing ED visits.

Clinicians in 25 countries prefer to use lower levels of oxygen to resuscitate preterm infants at birth.

AIM: This study determined current international clinical practice and opinions regarding initial fractional inspired oxygen (FiO2 ) and pulse oximetry (SpO2 ) targets for delivery room resuscitation of preterm infants of less than 29 weeks of gestation. METHODS: An online survey was disseminated to neonatal clinicians via established professional clinical networks using a web-based survey programme between March 9 and June 30, 2015. RESULTS: Of the 630 responses from 25 countries, 60% were from neonatologists. The majority (77%) would target SpO2 between the 10th to 50th percentiles values for full-term infants. The median starting FiO2 was 0.3, with Japan using the highest (0.4) and the UK using the lowest (0.21). New Zealand targeted the highest SpO2 percentiles (median 50%). Most respondents agreed or did not disagree that a trial was required that compared the higher FiO2 of 0.6 (83%), targeting the 50th SpO2 percentile (60%), and the lower FiO2 of 0.21 (80%), targeting the 10th SpO2 percentile (78%). Most (65%) would join this trial. Many considered that evidence was lacking and further research was needed. CONCLUSION: Clinicians currently favour lower SpO2 targets for preterm resuscitation, despite acknowledging the lack of evidence for benefit or harm, and 65% would join a clinical trial.

Regional (spinal, epidural, caudal) versus general anaesthesia in preterm infants undergoing inguinal herniorrhaphy in early infancy.

BACKGROUND: With improvements in neonatal intensive care, more preterm infants are surviving the neonatal period and presenting for surgery in early infancy. Inguinal hernia is the most common condition requiring early surgery, appearing in 38% of infants whose birth weight is between 751 grams and 1000 grams. Approximately 20% to 30% of otherwise healthy preterm infants having general anaesthesia for inguinal hernia surgery at a postmature age have at least one apnoeic episode within the postoperative period. Research studies have failed to adequately distinguish the effects of apnoeic episodes from other complications of extreme preterm gestation on the risk of brain injury, or to investigate the potential impact of postoperative apnoea upon longer term neurodevelopment. In addition to episodes of apnoea, there are concerns that anaesthetic and sedative agents may have a direct toxic effect on the developing brain of preterm infants even after reaching postmature age. It is proposed that regional anaesthesia may reduce the risk of postoperative apnoea, avoid the risk of anaesthetic-related neurotoxicity and improve neurodevelopmental outcomes in preterm infants requiring surgery for inguinal hernia at a postmature age. OBJECTIVES: To determine if regional anaesthesia reduces postoperative apnoea, bradycardia, the use of assisted ventilation, and neurological impairment, in comparison to general anaesthesia, in preterm infants undergoing inguinal herniorrhaphy at a postmature age. SEARCH METHODS: The following databases and resources were searched: the Cochrane Central Register of Controlled Trials (CENTRAL, The Cochrane Library, 2015, Issue 2), MEDLINE (December 2002 to 25 February 2015), EMBASE (December 2002 to 25 February 2015), controlled-trials.com and clinicaltrials.gov, reference lists of published trials and abstracts published in Pediatric Research and Pediatric Anesthesia. SELECTION CRITERIA: Randomised and quasi-randomised controlled trials of regional (spinal, epidural, caudal) versus general anaesthesia, or combined regional and general anaesthesia, in former preterm infants undergoing inguinal herniorrhaphy in early infancy. DATA COLLECTION AND ANALYSIS: At least two of three review authors (LJ, JF, AL) independently extracted data and performed analyses. Authors were contacted to obtain missing data. The methodological quality of each study was assessed according to the criteria of the Cochrane Neonatal Review Group. Data were analysed using Review Manager 5. Meta-analyses were performed with calculation of risk ratios (RR) and risk difference (RD), along with their 95% confidence intervals (CI) where appropriate. MAIN RESULTS: Seven small trials comparing spinal with general anaesthesia in the repair of inguinal hernia were identified. Two trial reports are listed as 'Studies awaiting classification' due to insufficient information on which to base an eligibility assessment. There was no statistically significant difference in the risk of postoperative apnoea/bradycardia (typical RR 0.72, 95% CI 0.48 to 1.06; 4 studies, 138 infants), postoperative oxygen desaturation (typical RR 0.82, 95% CI 0.61 to 1.11; 2 studies, 48 infants), the use of postoperative analgesics (RR 0.42, 95% CI 0.15 to 1.18; 1 study, 44 infants), or postoperative respiratory support (typical RR 0.09, 95% CI 0.01 to1.64; 3 studies, 98 infants) between infants receiving spinal or general anaesthesia. When infants who had received preoperative sedatives were excluded, the meta-analysis supported a reduction in the risk of postoperative apnoea in the spinal anaesthesia group (typical RR 0.53, 95% CI 0.34 to 0.82; 4 studies, 129 infants). Infants with no history of apnoea in the preoperative period and receiving spinal anaesthesia (including a subset of infants who had received sedatives) had a reduced risk of postoperative apnoea and this reached statistical significance (typical RR 0.34, 95% CI 0.14 to 0.81; 4 studies, 134 infants). Infants receiving spinal rather than general anaesthesia had a statistically significant increased risk of anaesthetic agent failure (typical RR 7.83, 95% CI 1.51 to 40.58; 3 studies, 92 infants). Infants randomised to receive spinal anaesthesia had an increased risk of anaesthetic placement failure of borderline statistical significance (typical RR 7.38, 95% CI 0.98 to 55.52; typical RD 0.15, 95% CI 0.03 to 0.27; 3 studies, 90 infants). AUTHORS' CONCLUSIONS: There is moderate-quality evidence to suggest that the administration of spinal in preference to general anaesthesia without pre- or intraoperative sedative administration may reduce the risk of postoperative apnoea by up to 47% in preterm infants undergoing inguinal herniorrhaphy at a postmature age. For every four infants treated with spinal anaesthesia, one infant may be prevented from having an episode of postoperative apnoea (NNTB=4). In those infants without preoperative apnoea, there is low-quality evidence that spinal rather than general anaesthesia may reduce the risk of preoperative apnoea by up to 66%. There was no difference in the effect of spinal compared with general anaesthesia on the overall incidence of postoperative apnoea, bradycardia, oxygen desaturation, need for postoperative analgesics or respiratory support. Limitations on these results included varying use of sedative agents, or different anaesthetic agents, or combinations of these factors, in addition to trial quality aspects such as allocation concealment and inadequate blinding of intervention and outcome assessment. The meta-analyses may have inadequate power to detect a difference between groups for some outcomes, with estimates of effect based on a total population of fewer than 140 infants.The effect of newer, rapidly acting, quickly metabolised general anaesthetic agents on safety with regard to the risk of postoperative apnoea and neurotoxic exposure has not so far been established in randomised trials. There is potential for harm from postoperative apnoea and direct brain toxicity from general anaesthetic agents superimposed upon pre-existing altered brain development in infants born at very to extreme preterm gestation. This highlights the clear need for the examination of neurodevelopmental outcomes in the context of large randomised controlled trials of general, compared with spinal, anaesthesia, in former preterm infants undergoing surgery for inguinal hernia.There is a particular need to examine the impact of the choice of spinal over general anaesthesia on respiratory and neurological outcomes in high-risk infant subgroups with severe respiratory disease and previous brain injury.

Emergency Department Point-of-care Ultrasound Identification of Suspected Lemierre's Syndrome: A Case Report.

INTRODUCTION: Lemierre's syndrome is septic thrombophlebitis of the internal jugular vein, most commonly associated with head and neck infections. While central catheters are associated with venous thromboembolism and catheter-associated bacterial infections, cases of Lemierre's syndrome caused by central catheters are extraordinarily rare. CASE REPORT: We detail a case of Lemierre's syndrome resulting from a peripherally inserted central catheter in a pregnant female patient. Diagnosis of this rare and potentially life-threatening disease process was expedited using point-of-care ultrasound. CONCLUSION: Diagnosis of rare but potentially life- or limb-threatening pathologies is paramount to the successful practice of emergency medicine. Identifying these rare disease processes requires a high index of suspicion and a work-up strategy that includes consideration of medical history in combination with lab and imaging findings to determine appropriate interventions.

A Retrospective Application of the Arbon and Hartman Models to the Union Cycliste International Mountain Bike World Cup.

INTRODUCTION: Outdoor activities have accelerated in the past several years. The authors were tasked with providing medical care for the Union Cycliste International (UCI) mountain biking World Cup in Snowshoe, West Virginia (USA) in September 2021. The Hartman and Arbon models were designed to predict patient presentation and hospital transport rates as well as needed medical resources at urban mass-gathering events. However, there is a lack of standardized methods to predict injury, illness, and insult severity at rural mass gatherings. STUDY OBJECTIVE: This study aimed to determine whether the Arbon model would predict, within 10%, the number of patient presentations to be expected and to determine if the event classification provided by the Hartman model would adequately predict resources needed during the event. METHODS: Race data were collected from UCI event officials and injury data were collected from participants at time of presentation for medical care. Predicted presentation and transport rates were calculated using the Arbon model, which was then compared to the actual observed presentation rates. Furthermore, the event classification provided by the Hartman model was compared to the resources utilized during the event. RESULTS: During the event, 34 patients presented for medical care and eight patients required some level of transport to a medical facility. The Arbon predictive model for the 2021 event yielded 30.3 expected patient presentations. There were 34 total patient presentations during the 2021 race, approximately 11% more than predicted. The Hartman model yielded a score of four. Based on this score, this race would be classified as an "intermediate" event, requiring multiple Advanced Life Support (ALS) and Basic Life Support (BLS) personnel and transport units. CONCLUSION: The Arbon model provided a predicted patient presentation rate within reasonable error to allow for effective pre-event planning and resource allocation with only a four patient presentation difference from the actual data. While the Arbon model under-predicted patient presentations, the Hartman model under-estimated resources needed due to the high-risk nature of downhill cycling. The events staffed required physician skills and air medical services to safely care for patients. Further evaluation of rural events will be needed to determine if there is a generalized need for physician presence at smaller events with inherently risky activities, or if this recurring cycling event is an outlier.

Lessons on Mass Gatherings Learned From the 2019 Union Cycliste Internationale Mountain Bike World Cup.

The Union Cycliste Internationale (UCI) Mountain Bike World Cup in 2019 provided unique challenges for effective prehospital care. While on-site medical care has demonstrated improved outcomes along with reduced emergency department and emergency medical services (EMS) utilization, this aspect has not been well documented in the literature with respect to rural mass gathering events (MGEs). Conducted at a large mass gathering event in a geographically isolated area, this study aimed to assess the medical needs at this specific event and will hopefully assist in future coordination of similar events. All patients who were treated at the event clinic were included in the analysis. Primary investigators collected and recorded data while providing care. We believe the on-site clinic was successful in reducing barriers to healthcare by improving access, streamlining the treatment process, and optimizing resource utilization. This benefit extended to race participants, support staff, spectators, and the local EMS system.

Targeted Oxygen in the Resuscitation of Preterm Infants, a Randomized Clinical Trial.

BACKGROUND AND OBJECTIVES: Lower concentrations of oxygen (O2) (≤30%) are recommended for preterm resuscitation to avoid oxidative injury and cerebral ischemia. Effects on long-term outcomes are uncertain. We aimed to determine the effects of using room air (RA) or 100% O2 on the combined risk of death and disability at 2 years in infants <32 weeks' gestation. METHODS: A randomized, unmasked study designed to determine major disability and death at 2 years in infants <32 weeks' gestation after delivery room resuscitation was initiated with either RA or 100% O2 and which were adjusted to target pulse oximetry of 65% to 95% at 5 minutes and 85% to 95% until NICU admission. RESULTS: Of 6291 eligible patients, 292 were recruited and 287 (mean gestation: 28.9 weeks) were included in the analysis (RA: n = 144; 100% O2: n = 143). Recruitment ceased in June 2014, per the recommendations of the Data and Safety Monitoring Committee owing to loss of equipoise for the use of 100% O2. In non-prespecified analyses, infants <28 weeks who received RA resuscitation had higher hospital mortality (RA: 10 of 46 [22%]; than those given 100% O2: 3 of 54 [6%]; risk ratio: 3.9 [95% confidence interval: 1.1-13.4]; P = .01). Respiratory failure was the most common cause of death (n = 13). CONCLUSIONS: Using RA to initiate resuscitation was associated with an increased risk of death in infants <28 weeks' gestation. This study was not a prespecified analysis, and it was underpowered to address this post hoc hypothesis reliably. Additional data are needed.

Recurrent de novo BICD2 mutation associated with arthrogryposis multiplex congenita and bilateral perisylvian polymicrogyria.

Autosomal dominantly inherited mutations of BICD2 are associated with congenital-onset spinal muscular atrophy characterised by lower limb predominance. A few cases have also showed upper motor neuron pathology, including presenting with features resembling hereditary spastic paraplegia. The age-of-onset for the published families is usually at birth but also included cases with childhood- and adult-onset disease. In this report we described two isolated probands that presented in utero with features associated with reduced fetal movements. Both cases were diagnosed at birth with arthrogryposis multiplex congenita (AMC) and hypotonia. Other variable features included congenital fractures, hip dislocation, micrognathia, respiratory insufficiency, microcephaly and bilateral perisylvian polymicrogyria. Patient 1 is 4 years of age and stable, but shows significant motor developmental delay and delayed speech. Patient 2 passed away at 7 weeks of age. Through next generation sequencing we identified the same missense substitution in BICD2 (p.Arg694Cys) in both probands. Sanger sequencing showed that in both cases the mutation arose de novo. The in utero onset in both cases suggests that the p.Arg694Cys substitution may have a more deleterious effect on BICD2 function than previously described mutations. Our results broaden the phenotypes associated with BICD2 mutations to include AMC and cortical malformations and therefore to a similar phenotypic spectrum to that associated with its binding partner DYNC1H1.

Herpes simplex virus colitis in a neonate.

Involvement of the gastrointestinal tract in neonates with congenital herpes simplex virus (HSV) infection is rarely described. We report a case of a newborn with disseminated HSV infection associated with profuse hematochezia and late sigmoid colon perforation. Histologic examination showed patchy areas of ulceration with multinucleated giant cells and HSV nucleic acid was detected by polymerase chain reaction in colonic tissue. No clinically apparent episodes of recurrent colitis occurred in the first year of life.

Paternal uniparental isodisomy for chromosome 14 with mosaicism for a supernumerary marker chromosome 14.

Uniparental disomy (UPD) describes the inheritance of two homologous chromosomes from a single parent. Disease phenotypes associated with UPD and chromosomal imprinting, rather than with mutations, include Beckwith-Wiedemann syndrome (paternal UPD11p), Angelman syndrome (paternal UPD15), Prader-Willi syndrome (maternal UPD15), and transient neonatal diabetes (paternal UPD6). Here we report on the first case of paternal uniparental isodisomy of chromosome 14 with a mosaicism for a supernumerary marker chromosome 14. The patient demonstrated a small thorax with a 'coat hanger' shape of the ribs, kyphoscoliosis, hypoplasia of the maxilla and mandible, a broad nasal bridge with anteverted nares, contractures of the wrists with ulnar deviation bilaterally, diastasis recti, and marked muscle hypotonia. Vertical skin creases under the chin and stippled epiphyses of the humeri were features not previously described in patients with paternal UPD14. This case illustrates that as with the finding of an isochromosome, a supernumerary marker chromosome can be an important clue to the presence of UPD14.

Neonatal resuscitation educational experience of staff in New South Wales and Australian Capital Territory hospitals.

OBJECTIVE: There is international recognition that health personnel involved in deliveries should be adequately trained in neonatal resuscitation. A survey was carried out in New South Wales (NSW) and the Australian Capital Territory (ACT) to ascertain the type, frequency and availability of training in neonatal resuscitation to staff who may need to resuscitate an infant at birth. The survey included a self-perception rating of confidence and competence in neonatal resuscitation. METHODS: Questionnaires were sent to 117 hospitals carrying out deliveries in NSW and ACT. Questionnaires were distributed to staff members who may be present at a delivery in a designated 24-h period. RESULTS: In total, 1457 questionnaires from 101 hospitals were returned and analysed. Overall response rate was 86.3% with 96.1% able to be assigned to tertiary, urban non-tertiary or rural areas. Eighty-five per cent of tertiary respondents had a training programme available to them compared with 59% of urban non-tertiary staff and 31% of rural practitioners. Approximately one-third of respondents in rural and urban non-tertiary units had either never been trained or had training more than 2 years before the survey. In rural areas more than 25% staff were not confident in their resuscitation skills and only 9% felt very competent. CONCLUSION: Three-quarters of all births in NSW and ACT take place in rural or urban non-tertiary hospitals where one-third of health personnel are inadequately trained in neonatal resuscitation and many do not feel confident in their skills. Effective neonatal resuscitation training for these areas is urgently required.