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BackgroundIncorrect penicillin allergy labels result in the use of inappropriately broad-spectrum antibiotics. De-labelling inaccurate penicillin allergy promotes antimicrobial stewardship and optimises prescribing practices. The objectives were to evaluate paediatric clinicians’ knowledge and understanding of penicillin allergy and to identify barriers in tackling incorrect penicillin allergy labels.MethodsPaediatric clinicians from across the West Midlands of the UK were surveyed using an online, anonymised questionnaire between 1 August and 30 September 2021. Domains explored were (1) approach to penicillin allergy clinical vignettes, (2) knowledge of the impact of penicillin allergy labels, (3) frequency of allergy-focused history questions and (4) barriers in tackling incorrect penicillin allergy.ResultsResponses were received from 307 paediatric clinicians across 12 hospitals. Sixty-one per cent would not prescribe a penicillin-based antibiotic if a family history of penicillin allergy was reported. There was an overall deficit in taking an allergy-focused history with only 36.5% inquiring about diagnostic confirmation. Absence, or lack of awareness of a referral pathway for antibiotic allergy evaluation (58.6%) and unfamiliarity of the indications for oral provocation testing (55%) were the most common reported barriers to penicillin allergy de-labelling. Fifty-one per cent would rather ‘play it safe’ than explore penicillin allergy confirmation as it is felt that alternative treatments were readily available.ConclusionsThe deficiency in antibiotic allergy-focused history among paediatric clinicians highlights the need for better allergy education across all clinical grades. Pragmatic algorithms and clear referral pathways could address barriers faced by non-allergists in de-labelling incorrect penicillin allergy.

Peripheral intravenous cannulation (PIVC) in children is one of the most common procedures in acute care paediatrics. This can be difficult for experienced clinicians and daunting for those with limited paediatric experience. We aimed to evaluate the level of confidence of non-consultant hospital doctors (NCHDs) in PIVC and identify factors that impact on their level of confidence. This was a cross-sectional nationwide survey of NCHDs on the level of confidence in paediatric PIVC. Self-reported questionnaires explored clinical experience with PIVC, level of confidence, and potential factors impacting on confidence. Primary outcome was the level of confidence in PIVC. A survey response rate of 45% (202/450) was received from 12 Irish hospitals. Despite 89% (180/202) of NCHDs self-reporting average or above average success rates and 76% (155/202) having inserted ≥10 cannula within the preceding 3 months, self-reported confidence was low across the entire cohort. Only 48% (97/202) of NCHDs reported confidence scores >3 on 5-point scale and 37% (75/202) reported significant anxiety proceeding with PIVC. Level of confidence was increased with previous paediatric experience (p = 0.00004) and seniority of the doctor (p < 0.00001), whilst previous unsuccessful attempts by colleagues were strongly associated with decreased confidence (p = 0.009). Our study described the levels of confidence of NCHDs in PIVC. Although the experienced paediatric NCHDs are confident in PIVC, there still exists some degree of anxiety. These have implications for workforce educational planning and training to improve level of confidence which may lead to better patient care.

Background Wolf-Hirschhorn syndrome (WHS) is a rare genetic condition caused by partial deletion of short arm of chromosome 4. Clinical manifestations comprise but not limited to typical facie, mental retardation, growth delay, epilepsy, and congenital cardiac defects. Congenital heart defects occur in about 25–50% of cases. We describe a child with WHS presenting with a femoral fracture; he became apnoeic in the course of management resulting in a cascade of events. Case Report 4.5-year-old boy known to have WHS was admitted with left femoral fracture. The syndrome was an antenatal diagnosis. Other associations noted were severe growth delay (weight of 6 kg), epilepsy, global developmental delay, visual impairment, and tetralogy of Fallot (TOF). His TOF has been managed uneventfully on propranolol with acceptable oxygen saturation (Sp02) in the 70s. The spontaneous fracture required Gallows traction, which provoked distress. He developed hypoxic crisis requiring oxygen therapy to maintain acceptable SpO2. Subsequently, he went apnoeic and dusky needing bag and mask ventilation. Blood gas revealed severe metabolic acidosis, which improved after fluid bolus. Morphine dosing regimen for pain control was continued. Knee-chest position could not be attempted due to the fracture. Although he got treated for suspected sepsis due to the dilemma of events, he did have a hypercyanotic spell in hindsight. He was subsequently discharged with conservative management of the fracture. Conclusion To the best of our knowledge, this is the first reported case of hypercyanotic (Tet) spell in WHS. This rather uncommon event resulted in a significant learning point for medical and nursing staff.

Background/aim High-flow nasal cannula oxygen (HFNCO) has becoming increasingly used as a respiratory support in children with bronchiolitis. We aimed to evaluate the clinical characteristics, course and outcome of infants on HFNCO since its introduction in a DGH. Methods This was a single-centre retrospective study of infants with clinical diagnosis of bronchiolitis on high-flow nasal cannula oxygen between September 2016 and January 2019. The clinical characteristics of these infants were evaluated. Their clinical course during therapy and outcome were analysed. Results Twenty-two infants with bronchiolitis received HFNCO therapy over the study period. The age of the infants ranged between 1 week and 11.9 months. Comorbidities included prematurity, chronic lung disease, cystic fibrosis and trisomy 21. The commonest indications for its use were severe respiratory distress and pCO2 greater than 7.5 kPa. Chest radiograph and blood gas analysis were performed in all patients prior to commencement. The median start and maximum flow rates were 1.55 (range: 0.8–3.7) and 1.75 (range: 1–3.7) L/kg/min respectively. The mean time of improvement in work of breathing and heart rate was 2.06 (SD. 0.81) hours. The median length of therapy was 3.2 (range: 0.25–9.4) days. Treatment failure was recorded in 27% of cases; with transfer to tertiary centre. At least a comorbidity was present in 83% of treatment failure cases. All but one of the treatment failures were intubated. There were no adverse events or mortality in the study group. Conclusions HFNCO has shown significant benefit in the management of moderately severe bronchiolitis since its introduction in this DGH. It can be safely applied in the emergency department or on the general paediatric wards. Infants with comorbidities presenting with bronchiolitis may require early escalation to HFNCO to reduce treatment failure.

Background/Aim Bronchiolitis is one of the commonest causes of hospital presentations during the winter period. It poses a great deal of strain on the health care staff and resources. A targeted assessment tool is thought to provide a prompt and optimal assessment to limit unwarranted investigations and hospital admissions. The acute assessment of children with a clinical diagnosis of bronchiolitis was initially audited with subsequent re-audit using the assessment pro forma. Methods Children admitted with bronchiolitis between November 2017 and January 2018 in one hospital site were randomly selected. Medical notes were reviewed to evaluate the clinical assessments and treatment(s) offered in comparison with NICE guidance. A pilot study was subsequently performed to assess the use of the new assessment pro forma by medical students and junior doctors. Results Thirty medical notes were randomly selected for the audit. History of apnoea was inquired in 7 of 20 infants aged 3 months and below. Oral intake was clearly documented in only 36.7% (11/30). Neonatal events were clearly documented in 96.7% (29/30). Documentation of exposure to passive smoking occurred in 23.3% (7/30). Only 50% had documented assessment of hydration status and femoral pulses. Blood tests were requested in 13 infants with clinical indications documented in 7. Sixty-five percent (11/17) had no documented clinical indication for chest radiograph. Inappropriate treatments (bronchodilators, steroids, antibiotics) were initiated in 30% of infants. The pilot study involved 15 volunteers (medical students and junior doctors) on the use of the assessment pro forma in the 2 hospital sites. Analyses of the pro forma demonstrated 100% compliance in all parameters re-audited. Conclusion The new pro forma has demonstrated its usefulness in targeted assessment of children with suspected bronchiolitis. Well-focused history and examination will safely stratify children to be managed in the community or hospital. The feedbacks received from its use were excellent.

Aims/Objectives/BackgroundUnnecessary blood testing in the paediatric emergency department (PED) is a potential starting point for diagnostic dilemma, anxiety to families and increased healthcare costs. We hypothesized that a significant number of blood tests are performed instinctively rather than clinically indicated. This stimulated a quality improvement initiative to enlighten trainees on the utility of blood tests while aiming to enhance clinical decision making.Methods/DesignChildren presenting to the acute care team in a tertiary PED who had blood tests over a 2-week period in April 2019 were enrolled. Blood tests requested were interpreted in line with presenting features and clinical impression. Following implementation of changes (posters, QI champions, educational sessions), a repeat analysis was done over a 2-week period in October 2019.Results/ConclusionsOne hundred and one children in the first cycle were enrolled. Blood testing analysis revealed that 70%, 47%, and 32% had liver bloods, bone profile, and clotting testing done, respectively. Over half of these blood tests had no clear clinical indication. The yield of the tests performed without clinical indication was 0%. Case vignettes were attempted by trainees and ANPs to evaluate their attitudes to blood test requests. Number of requested blood tests not clinically indicated was lower than anticipated; probably explained by self-thought processes. Blood tests performed on 100 children in the second cycle demonstrated a significant reduction in tests done without clear clinical indication. Liver bloods, clotting screen, bone profile tests were performed on 40%, 24% and 27% respectively in the second cycle.The utility of blood tests may be an under recognised subject in paediatric training which needs addressing. This project typified positive impact of culture change via QI champions and educational sessions. Implementation of such changes is sustainable with an estimated savings of at least £500/month.

Aims There is very limited understanding of the role of Type 1 Interferon (IFN) in human anti-viral immunity. However, IFNs are known to contribute to the activation of innate and adaptive immune responses. Human Interferon-alpha/beta receptor 2 (IFNAR2) deficiency causes fatal susceptibility to live viral vaccines, revealing a vital but narrow nonredundant role for IFN-alpha/beta in viral immunity.1 We describe 2 cases of IFNAR2 deficiency. Sanger sequencing identified homozygous single nucleotide deletion c.A311del in IFNAR2 gene in both cases. Methods The cases are siblings; the older one having died of fatal encephalitis before the younger one was born. Clinical and laboratory data were reviewed. Results Case 1: A 14-month-old previously healthy boy presented acutely unwell with a preceding history of MMR vaccination. He deteriorated rapidly and subsequently died from haemophagocytic lymphohistiocytosis complicating vaccine-strain mumps and rubella encephalitis. Post-mortem systemic and brain samples demonstrated clear evidence of sustained replication of vaccine viruses and HHV-6. In retrospect, he had no problems with common viral infections during infancy. Case 2: Younger sister of the first case was diagnosed IFNAR2 deficient at about 6 weeks of age. She had uneventful neonatal and infancy periods. She has been on prophylactic aciclovir since post neonatal period. Subcutaneous immunoglobulin was commenced after her third childhood vaccine (while avoiding all live vaccines). She was admitted at 9 months of age for respiratory tract infection which completely resolved. Parents are heterozygous for this mutation. Wider family members were screened; all were homozygous wild-type at this position implying neither affected nor carriers of the disease. Conclusion These are the first documented cases of IFNAR2 deficiency worldwide. Perhaps, this rather novel primary immunodeficiency will broaden the knowledge of the role of IFN-alpha/beta in human antiviral immunity. Reference . Duncan CJ, Mohamad SMB, Young DF, Skelton AJ, Leahy TR, et al. Human IFNAR2 deficiency: Lessons for antiviral immunity. Sci Transl Med2015;7:307ra154.

Aim Stroke is an acute neurologic deficit that results from an ischaemic or haemorrhagic cerebral vascular event, persists for at least 24 hours, and results from a disturbance of the cerebral circulation. Childhood stroke is not common, but leads to significant morbidity and mortality. It is among the top ten causes of death in childhood. Stroke mimics are much commoner which make the diagnosis more challenging. We describe an intriguing case for which a wide array of tests has not revealed the precise aetiology. Methods The clinical, laboratory and radiological data were reviewed. Results A 3-year-old girl, only child of non-consanguineous Polish parents presented with marked altered level of consciousness preceded by upper respiratory tract infection. Examination revealed non-reactive pupils without focal neurological signs. Electroencephalography demonstrated diffuse encephalopathy. She was managed for probable encephalitis. There was clinical deterioration evident by headaches, an episode of focal seizure with subsequent right sided hemiparesis. Serial magnetic resonance image (MRI) and angiography (MRA) of the brain revealed diffuse abnormality of intracranial vessels with attenuated calibre of intracranial vessels and bilateral watershed infarcts. There was visual loss without evidence of optic neuritis. Metabolic, coagulation, autoimmune, infection, extracranial imaging and available genetic screen have been normal so far. Fluctuating blood pressure has remained a challenge in her management. She is being managed on immunosuppressive, antiepileptic, antihypertensive and antithrombotic agents. Multidisciplinary team follow-up is ongoing. Follow-up brain MRI and MRA (at five months) confirmed stable bilateral infarcts and vessels appearance. Conclusion Cerebral arteriopathy has been recognised as a major risk factor for arterial ischaemic stroke in children. It may develop in a previously healthy child without any known risk factors. However, recent upper respiratory tract infection has been shown to be a significant predictor of underlying arteriopathy.1 The risk of recurrence in this case remains uncertain. Reference . Amlie-Lefond C, Bernard TJ, Sebire G, Friedman NR, Heyer GL, et al. Predictors of cerebral arteriopathy in children with arterial ischaemic stroke: Results of the international paediatric stroke study. Circulation2009;119(10):1417–23.

Systemic lupus erythematosus (SLE) is an autoimmune disease of unknown aetiology, which can affect any organ system. Tuberculosis (TB) is a common infection in SLE because of immune dysregulation associated with the latter. We report a case of an adolescent female who presented with a year’s history of polyarticular arthralgia and fever. Physical examination revealed a large left effusion that needed drainage. Investigations revealed a combined diagnosis of SLE and TB. Management comprised quadruple anti-TB therapy and SLE treatment. She made a steady recovery and has maintained a stable state from the lupus perspective.

Intraventricular haemorrhage (IVH) is characterized by bleeding of the immature subependymal germinal matrix in preterm infants, but the pathogenesis is multifactorial. IVH and posthaemorrhagic hydrocephalus (PHH) are common causes of neonatal morbidity and mortality among preterm infants. We describe a preterm male infant who was born clinically stillbirth; became moderately severe encephalopathic. He had bilateral IVH (III right and IV left) with consequent PHH. His incredible outcome following a stormy perinatal period appears intriguing. Long-term follow-up is needed to evaluate the severity of deficits as he matures. Whether therapeutic cooling would have made a difference or not is debatable.

Background/aim The use of oral rehydration therapy (ORT) remains limited despite international guidelines for diarrhoeal disease management. This study was done to assess the perceptions and practices of ORT among caregivers in the management of diarrhoeal disease. Materials and methods A descriptive cross-sectional study was conducted in the Paediatric Department of Cork University Hospital, Ireland. The study population consisted of parents/guardians of children attending the Paediatric Day Ward or Paediatric Outpatient Department. A questionnaire consisting of 30 questions was originally designed by the first author to include all the study variables, and was given to the participants. Results Out of 402 respondents, 76.6% (n=308) could describe diarrhoea correctly and perceived it as a serious illness. 81.3% (n=327) of participants knew about ORT but only 27.2% (n=89) agreed it was an ideal first line of management of diarrhoeal disease. Diarrhoeal episodes in the preceding 12 months were reported predominantly in children under the age of 5 years (p Conclusion Poor uptake of ORT appears to be due to caregivers’ negative perceptions of ORT. This emphasises the need for correct and adequate education.

Aim Non accidental injury (NAI) is defined as any abuse inflicted on a person or knowingly not prevented by a care giver.The injury is not consistent with the account of its occurrence. We describe an unusual case that presented with symptoms strongly suggestive of immunodeficiency with significant diagnostic dilemma. Methods Clinical data, laboratory and radiological records of our case were reviewed. Results A 15 month old girl presented with alopecia, recurrent infections comprising left dacryocystitis with subsequent preseptal cellulitis, left maxilla zygomatic abscess and left suppurative otitis media. She also had several episodes of swelling and bruising in various body parts. There was an initial strong suspicion of an underlying immunodeficiency or autoimmune disorder. She was reviewed extensively by multiple disciplines with a battery of blood investigations which was not in keeping with either. Chest radiograph was normal but surprisingly revealed fractures of both proximal humeral metaphyses beyond the field of focus. Further radiological investigations including skeletal surveys showed multiple healing fractures without evident osteopenia. She made an excellent recovery evident on 5 months follow-up. She is well known to child protection services. Conclusion The diagnosis of NAI is complex; this will ultimately depend on evidence collected from all agencies involved in the care of the child.1 The thorough investigation of children with suspected NAI is critical to assure their safety.

Background: Urinary tract infection (UTI) is a common reason for referral to the emergency department (ED) especially in unwell infants. Upper UTIs are particularly at risk of significant complications later in life. Rapid dipstick urinalysis and microscopy are often used in unwell children as a screening tool to guide early diagnosis and treatment. This study aims to evaluate the sensitivity of dipstick urinalysis and microscopy in the diagnosis of UTI. Methods: A retrospective review of children aged 16 years and below with positive urine culture (UC) over a 3-year period was done. The results of urine dipstick and microscopy were compared with the positive UC and sensitivities calculated. Results: Dipstick urinalysis and microscopy of 262 children were studied. Female-to -male ratio of 1.8:1. Median age was 0.79 (range: 0.02–15.95) years. The sensitivity of nitrite, blood, and leukocyte esterase (LE) were 0.54, 0.74, and 0.86 (95% confidence interval [CI] = 0.46–0.62, 0.68–0.80, and 0.82–0.91), respectively. The sensitivity of pyuria of ≥100 cells/mm3 was 0.92 (95% CI = 0.89–0.95). The presence of any of the 3 dipstick parameters increased the sensitivity to 0.97 (95% CI = 0.95–0.99). The lowest sensitivity 0.49 (95% CI = 0.40–0.58) was found with combined positive LE and nitrite. There was a significant comparison between positive LE dipstick test and pyuria (P = 0.000004). Conclusions: Dipstick urinalysis may not be reliable in ruling out UTI in children. However, considering both positive dipstick and pyuria will be more useful in making the diagnosis.

Aims: Non accidental injury (NAI) is defined as any abuse inflicted on a person or knowingly not prevented by a care giver. The injury is not consistent with the account of its occurrence. We describe an unusual case that presented with symptoms suggesting immunodeficiency with significant initial diagnostic dilemma. Methods: Clinical data, laboratory and radiological records of our case were reviewed. Results: A 15 month old girl presented with alopecia, recurrent infections comprising left dacryocystitis with subsequent preseptal cellulitis, left maxilla zygomatic abscess and left suppurative otitis media occurring within the preceding 3 months. She also had several episodes of swelling and bruising in various body parts. There was an initial strong suspicion of an underlying immunodeficiency or autoimmune disorder. She was reviewed extensively by multiple disciplines with a battery of blood investigations which was not in keeping with either. Chest radiograph was normal but surprisingly revealed fractures of both proximal humeral metaphyses beyond the field of focus. Further radiological investigations including skeletal surveys showed multiple healing fractures without evident osteopenia. These events in retrospect were closely linked to the period of care by a close care giver. She made an excellent recovery evident on 5 months follow up. She is well known to child protection services. Conclusion: The diagnosis of NAI is complex; this will ultimately depend upon evidence collected from all agencies involved in the care of the child. The thorough investigation of children with suspected NAI is critical to assure their safety.

Background: The use of oral rehydration therapy (ORT) remains limited despite international guidelines for diarrhoeal disease management. This study was done to assess the perception and practice of ORT among caregivers in the management of diarrhoeal disease. Methods: A descriptive cross-sectional study was conducted in the Paediatric Department of Cork University Hospital, Ireland. The study population consisted of parents/guardians of children attending the Paediatric Day Ward or Paediatric Outpatient Department. A questionnaire consisting of 30 questions was originally designed by the first author to include all the study variables, and was given to the participants. Results: Out of 402 respondents, 76.6% (n=308) could describe diarrhoea correctly and perceived it as a serious illness. 81.3% (n=327) of participants knew about ORT but only 27.2% (n=89) agreed it was an ideal first line of management of diarrhoeal disease. Diarrhoeal episodes in the preceding 12 months were reported predominantly in children under the age of 5 years (p

Introduction: Anorexia nervosa is a commonly encountered cause of severe underweight in pediatric settings. It is an eating disorder characterized by the inability to maintain a minimally normal weight, a devastating fear of weight gain, relentless dietary habits that prevent weight gain, and a disturbance in the way in which the body image is perceived. Hypernatremia is an unusual electrolyte imbalance in anorexia nervosa. Case Report: This is a case of a 12-year-old male admitted with progressive weight loss following restrictive food intake for the preceding one year. He presented with severe hypernatremia. Correction of severe hypernatremia was successful after four to five days. This correction was slow but gradually achieved. There was no altered sensorium throughout admission which remains the worst nightmare of any managing team. Feeding protocol as per Junior MARSIPAN guidelines was adhered to. He made an excellent recovery and was discharged after 24 days of admission with a weight gain of 6.1 kg. Conclusion: Anorexia nervosa is commonly encountered in pediatric settings and can cause potentially life-threatening physical and psychological complications. Hypernatremia is an uncommon metabolic abnormality; slow correction averts untoward side effects.

Medium-chain acyl-Coenzyme A dehydrogenase deficiency (MCADD) is a disorder of fatty acid β oxidation inherited in an autosomal recessive manner. The enzyme is useful in hepatic ketogenesis, a major source of energy once hepatic glycogen stores become depleted during prolonged fasting. It is a cause of hypoketotic hypoglycaemia in a previously well child. MCADD is not part of newborn screening in Ireland; children are likely to be missed if routine hypoglycaemic screen is not instituted when blood glucose level is below 2.6 mmol/L. This is a case of an otherwise healthy 23-month-old baby girl who presented with severe hypoglycaemia with some initial diagnostic dilemma.