Journal club is a long-standing pedagogy within clinical practice and education. While journal clubs throughout the world traditionally follow an established format, new approaches have emerged in recent times, including learner-centred and digital approaches. Key factors to journal club success include an awareness of the learning goals of the target audience, judicious article selection and emphasis on promoting the engagement of participant learners. This article reviews the role that journal club plays in modern clinical education and considers how to optimise its benefit for contemporary learners.

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In utero exposure to maternal antibodies targeting the fetal acetylcholine receptor isoform (fAChR) can impair fetal movement, leading to arthrogryposis multiplex congenita (AMC). fAChR antibodies have also been implicated in apparently rare, milder myopathic presentations termed fetal acetylcholine receptor inactivation syndrome (FARIS). The full spectrum associated with fAChR antibodies is still poorly understood. Moreover, since some mothers have no myasthenic symptoms, the condition is likely underreported, resulting in failure to implement effective preventive strategies. Here we report clinical and immunological data from a multicenter cohort (n = 46 cases) associated with maternal fAChR antibodies, 29 novel and 17 previously reported with novel follow-up data. Remarkably, in 50% of mothers there was no previously established MG diagnosis. All mothers (n = 30) had AChR antibodies, and where tested, against the fAChR (binding to fAChR was often much greater than that to the adAChR). Offspring death occurred in 11/46 (23.9%) cases, mainly antenatally due to termination of pregnancy prompted by severe AMC (7/46, 15.2%) or, during early infancy mainly from respiratory failure (4/46, 8.7%). Weakness, contractures, bulbar and respiratory involvement were prominent early in life, but improved gradually over time. Facial (25/34; 73.5%) and variable peripheral weakness (14/32; 43.8%), velopharyngeal insufficiency (18/24; 75%) and feeding difficulties (16/36; 44.4%) were the most common sequelae in long-term survivors. Other unexpected features included hearing loss (12/32; 37.5%), diaphragmatic paresis (5/35; 14.3%), CNS involvement (7/40; 17.5%) and pyloric stenosis (3/37; 8.1%). Oral salbutamol used empirically in 16/37 (43.2%) offspring resulted in symptom improvement in 13/16 (81.3%). Combining our series with all previously published cases, we identified 21/85 mothers treated with variable combinations of immunotherapies (corticosteroids/IVIG/PLEX) during pregnancy either for maternal MG symptom control (12/21 cases) or for fetal protection (9/21 cases). Compared to untreated pregnancies (64/85), maternal treatment resulted in a significant reduction in offspring deaths (P < 0.05) and other complications, with treatment approaches involving IVIG/PLEX administered early in pregnancy most effective. We conclude that presentations due to in utero exposure to maternal (fetal) AChR antibodies are more common than currently recognised and may mimic a wide range of neuromuscular disorders. Considering the wide clinical spectrum and likely diversity of underlying mechanisms, we propose Fetal Acetylcholine Receptor Antibody-related Disorder (FARAD) as the most accurate term for these presentations. FARAD is vitally important to recognise, to institute appropriate management strategies for affected offspring and to improve outcomes in future pregnancies. Oral salbutamol is a symptomatic treatment option in survivors.

Review ante- and post-mortem diagnoses and assign a Goldman error classification and establish autopsy rates. A retrospective analysis of autopsies performed on patients who died in paediatric intensive care unit (PICU) between November 13, 2012, and October 31, 2018. We reviewed medical and autopsy data of all patients, and Goldman classification of discrepancy between ante- and post-mortem diagnoses was assigned. Tertiary PICU. All patients that died in PICU within the designated timeframe. Goldman error classification assignment. Three hundred ninety-six deaths occurred in PICU from 8329 (4.75%) admissions. Ninety-nine (25%) had an autopsy, 75 required by the coroner. All were included in the study. Fifty-three were male and 46 females. Fifty-three patients were transfers from external hospitals, 46 from our centre. Forty-one were neonates, 32 were < 1 year of age, and 26 were > 1 year of age. Median length of stay was 3 days. Eighteen were post-cardiac surgery, and three post-cardiac catheter procedure. Major diagnostic errors (class I/II) were identified in 14 (14.1%): 2 (2%) were class I and 12 (12.1%) were class II errors. Class III and IV errors occurred in 28 (28.2%) patients. Complete concordance (class V) occurred in 57 (57.5%) cases. Conclusion: The autopsy rate and the diagnostic discrepancy rate within our PICU are comparable to those previously reported. Our findings show the continuing value of autopsy in determining cause of death and providing greater diagnostic clarity. Given their value, post-mortem examinations, where indicated, should be considered part of a physician’s duty of care to families and future patients. What is Known: • Major diagnostic discrepancies (class I/II) in PICU have been reported at 20.2%. (10) • PICU autopsy rates have varied from 36 to 67% since 1994 with most recently reported rates in 2018 being 36%. (6-9) What is New: • We report an Irish PICU major diagnostic discrepancy (class I/II) rates of 14.1% contributing further to reported discrepancy rates in PICU literature to date. • This study contributes the Irish PICU post-mortem rate in a tertiary centre which was 25% over an almost 6-year period.

Patient×is a 3 year old male who had neonatal hypoglycaemia and confirmed homozygosity for the GSD Type IIIa gene. Patient×was commenced on the KD at 8 months of age when progression of his GSD resulted in severe cardiomyopathy. The following outlines the transition onto the KD and clinical findings between 8-22 months Method Standard dietetic treatment was provided from birth to maintain euglycaemia along with placement of a percutaneous endoscopic gastrostomy. Continuous 24 hour feeding was required due to unsuccessful bolus feeding. A modified KD incorporating MCT fat -which was has been noted to further aid ketosis was commenced at 8 months with a ketogenic ratio of 0.5:1 building to 1:1 within 2 weeks. Ketones and blood sugar levels were closely monitored with a threshold of 2.6 mmol/L of glucose and ketones >1 mmol/L before hypoglycaemia intervention was required. Results Prior to the KD, there was a high glucose infusion rate (GIR) of 9.75 mg glucose/kg/minute. After initiation of the KD, the GIR reduced initially to 5.8 mg glucose/kg/ minute, with a gradual increase of MCT fat from 6% to 28% and the GIR further reduced to 2.73 mg glucose/kg/minute. There were no episodes of hypoglycaemia and ketones ranged from 1-2.9 mmol/L. Echocardiographs showed a significant improvement in cardiac function with a cardiac output reduction of 137 to 39 mmHg. Discussion The KD was trialled as an alternative treatment. It resulted in the reduced intake of carbohydrate and the subsequent reduction of glycogen build-up within cardiac muscle. Ketones were used as an alternative fuel source and euglycaemia was maintained