Complex visceral hyperalgesia in an adolescent with Pitt-Hopkins syndrome.

An early-adolescent boy with a background of Pitt-Hopkins syndrome was transferred to a tertiary paediatric hospital with symptoms of a functional large bowel obstruction. He required extensive surgical intervention including a transverse colectomy, drainage of an abdominal abscess, laparotomy and adhesionolysis, and insertion of a gastrostomy and jejunostomy. He had significant ongoing issues with visceral hyperalgesia that was refractory to a wide range of pharmacological treatments and required admission to the intensive care unit on multiple occasions, and consultations with international experts in Pitt-Hopkins syndrome and pain specialists. An individualised pain plan was created and adjusted over time, with eventual good effect, and he was transferred back to his regional hospital and subsequently discharged home.

mRNA or ChAd0x1 COVID-19 Vaccination of Adolescents Induces Robust Antibody and Cellular Responses With Continued Recognition of Omicron Following mRNA-1273.

Children and adolescents generally experience mild COVID-19. However, those with underlying physical health conditions are at a significantly increased risk of severe disease. Here, we present a comprehensive analysis of antibody and cellular responses in adolescents with severe neuro-disabilities who received COVID-19 vaccination with either ChAdOx1 (n=6) or an mRNA vaccine (mRNA-1273, n=8, BNT162b2, n=1). Strong immune responses were observed after vaccination and antibody levels and neutralisation titres were both higher after two doses. Both measures were also higher after mRNA vaccination and were further enhanced by prior natural infection where one vaccine dose was sufficient to generate peak antibody response. Robust T-cell responses were generated after dual vaccination and were also higher following mRNA vaccination. Early T-cells were characterised by a dominant effector-memory CD4+ T-cell population with a type-1 cytokine signature with additional production of IL-10. Antibody levels were well-maintained for at least 3 months after vaccination and 3 of 4 donors showed measurable neutralisation titres against the Omicron variant. T-cell responses also remained robust, with generation of a central/stem cell memory pool and showed strong reactivity against Omicron spike. These data demonstrate that COVID-19 vaccines display strong immunogenicity in adolescents and that dual vaccination, or single vaccination following prior infection, generate higher immune responses than seen after natural infection and develop activity against Omicron. Initial evidence suggests that mRNA vaccination elicits stronger immune responses than adenoviral delivery, although the latter is also higher than seen in adult populations. COVID-19 vaccines are therefore highly immunogenic in high-risk adolescents and dual vaccination might be able to provide relative protection against the Omicron variant that is currently globally dominant.

The effect of breaches of the psychological contract on the job satisfaction and wellbeing of doctors in Ireland: a quantitative study.

BACKGROUND: Medicine is one of the most popular college degrees at both undergraduate and postgraduate level. Despite this, morale and wellbeing in doctors at all levels internationally is reportedly low. Long hours and stressful working environments have been implicated as the cause of this. The psychological contract is the implicit expectations and mutual obligations held between an employee and employer. Breaches in this contract can lead to strong negative emotional responses. This study will examine the psychological contract of non-consultant doctors and gain further insight into their job satisfaction and wellbeing. It aims to ascertain the effect of breaches of the psychological contract on their job satisfaction and wellbeing. METHODS: This is a quantitative study performed using a questionnaire on a closed online forum. Job satisfaction, wellbeing and breaches of the psychological contract were measured using pre-existing and pre-validated scales. Statistical analysis was performed to determine the effect of breaches of the psychological contract on job satisfaction and wellbeing. RESULTS: This study ascertained that training and career development were the most important areas of the psychological contract for non-consultant doctors and training and organizational support the most important breaches. It found, overall, positive levels of job satisfaction and wellbeing. A statistically significant relationship between breaches of the psychological contract and job satisfaction and wellbeing was found. CONCLUSION: This study provides an insight into the psychological contract of non-consultant doctors in Ireland. By doing so, it identifies areas for change which may improve their future job satisfaction and wellbeing.

A case report of multiple cerebral abscess formation complicating serogroup B Neisseria meningitidis meningitis.

BACKGROUND: Invasive meningococcal disease (IMD) presenting with meningitis causes significant mortality and morbidity. Suppurative complications of serogroup B meningococcal sepsis are rare and necessitate urgent multidisciplinary management to mitigate long-term morbidity or mortality. CASE PRESENTATION: We present a rare case of invasive meningococcal disease in a 28-month old boy complicated by multiple abscess formation within a pre-existing antenatal left middle cerebral artery territory infarct. Past history was also notable for cerebral palsy with right hemiplegia, global developmental delay and West syndrome (infantile spasms). Two craniotomies were performed to achieve source control and prolonged antimicrobial therapy was necessary. The patient was successfully discharged following extensive multidisciplinary rehabilitation. CONCLUSIONS: Longstanding areas of encephalomalacia in the left MCA distribution may have facilitated the development of multiple meningococcal serogroup B abscess cavities in the posterior left frontal, left parietal and left temporal lobes following an initial period of cerebritis and meningitis. A combination of chronic cerebral hypoperfusion and some degree of pre-existing necrosis in these areas, may also have facilitated growth of Neisseria meningitidis, leading ultimately to extensive cerebral abscess formation following haematogenous seeding during meningococcemia. In this case report we review similar cases of cerebral abscess or subdural empyema complicating serogroup B meningococcal meningitis.

Gabapentin for the treatment of pain manifestations in children with severe neurological impairment: a single-centre retrospective review.

Pain, irritability and feeding intolerance are common symptoms affecting quality of life in children with severe neurological impairment (SNI). We performed a retrospective study to explore the use of gabapentinoid medications for symptom control in children with SNI. Patients attending the palliative care or gastroenterology department being treated with gabapentin for irritability, vomiting or pain of unknown origin were included. Information was gathered retrospectively from medical documentation. Irritability was reduced in 30 of the 42 patients included. Gabapentin was discontinued in 15 children, 12 of whom then received pregabalin. Three children had a good response to pregabalin, six a minimal improvement and three no improvement. These results support the use of gabapentinoids in this patient cohort.

Anorexia Nervosa Complicating Pediatric Crohn Disease-Case Report and Literature Review.

Crohn disease and anorexia nervosa share common symptoms of weight loss and reduced oral intake. The prevalence of both disorders has increased over time. Symptoms of Crohn disease and anorexia nervosa can mimic each other leading to a delayed diagnosis and requiring complex, multidisciplinary management. Here we present a case of a 15 year old girl with Crohn disease who subsequently developed anorexia nervosa, and review the published literature on the occurrence of both diagnoses.

Neonatal outcomes following elective caesarean delivery at term: a hospital-based cohort study.

OBJECTIVE: To assess neonatal outcomes following elective caesarean delivery (CD) at term (≥37 + 0 weeks gestation). METHODS: A retrospective cohort study was conducted in a single Irish maternity hospital. Elective CDs at term between August 2008 and July 2012 were reviewed. Outcome measures were admission to the neonatal intensive care unit (NICU), length of stay, respiratory complications, hypoglycaemia, jaundice, newborn sepsis and medical interventions. RESULTS: A total of 4242 women had an elective CD at term, accounting for approximately 15% of all term deliveries. Admission rate to the NICU at 37 weeks gestation was 21.8% versus 10% at 39 weeks (p for trend <0.0001). Similar trends of decreasing risk with later gestational age were noted for the other outcomes. An increased odds of admission to the NICU at 37 weeks [adjusted odds ratio (OR) 2.48 (95% CI 1.28, 4.79)] and at 38 weeks [OR 1.34, 95% CI 1.02, 1.77] compared to the reference of 39 weeks gestation was found. CONCLUSIONS: This study supports evidence that, with regard to neonatal outcome, 39 weeks gestational age is the optimal delivery time. Heightened awareness of the increased risk of neonatal morbidity, when delivery is performed electively before 39 weeks, is warranted among healthcare workers.

Mode of neonatal death in an Irish maternity centre.

UNLABELLED: Modes of neonatal dying vary among maternity centres, both within and between countries. There have been few reports concerning mode of dying from countries with low rates of termination of pregnancy, such as Ireland. We conducted a retrospective chart review of all neonatal deaths, between January 2010 and January 2013, within a single Irish maternity centre. The mode of dying was classified as one of (1) withholding life-sustaining treatment (LST), (2) withdrawal of LST in moribund infants, (3) withdrawal of LST for quality of life reasons or (4) death despite maximal intensive care treatment. There were a total of 64 deaths during the study period. Congenital abnormalities accounted for 47 % of deaths and prematurity for 41 % of deaths. Withholding LST was the most frequent mode of dying, occurring in 38 % of all deaths. A total of 12 % of neonatal deaths occurred despite maximal intensive care treatment. CONCLUSIONS: Congenital abnormalities were the most common cause of neonatal deaths. A high proportion followed LST being withheld, most likely a reflection of the low rates of medical termination in Ireland. Modes of dying in the neonatal period vary between maternity centres with culturally different backgrounds.