Narcolepsy in children and young people in Ireland: 2006-2017.

AIM: To describe the population of young people in Ireland diagnosed with narcolepsy with regards to vaccine exposure, symptomatology, investigation results and experience of medical treatment. METHOD: Retrospective review of medical records at the single tertiary referral centre for young people with narcolepsy in Ireland. RESULTS: Sixty-seven patients were diagnosed with narcolepsy between July 2006 and July 2017. Sixty-one (91%) of these developed symptoms after receiving the Pandemrix vaccine. The population was largely homogeneous with low hypocretin (87.5%), HLA DQB1∗0602 positivity (95%) and unremarkable findings on MRI Brain (100%). 77.6% experienced cataplexy; we also measured high levels of obesity, school non-attendance and psychosocial complexity. Symptoms often continued despite treatment, with multiple medications prescribed in 76.1% of patients. Prescription of sodium oxybate was associated with a significant reduction in BMI standard deviation scores at 6 months, with improved IOTF obesity scores seen at 36 month follow-up. CONCLUSIONS: This paper describes the experience of narcolepsy in children and young people in Ireland from 2006 - 2017 at the national tertiary referral centre. Narcolepsy in children and young people in Ireland carries a significant burden of illness, with impact on participation in education as well as physical and mental health. Symptoms can be refractory to medical treatment. Referral to tertiary centres for prompt treatment and multidisciplinary input is essential.

Chronic hemodialysis in children weighing less than 10 kg.

BACKGROUND: Hemodialysis (HD) in infants is usually used when peritoneal dialysis (PD) has failed. We describe our experience with HD, outlining the morbidity, complications, and outcomes for infants weighing less than 10 kg managed with HD for more than 6 months over a 10-year period. METHODS: A retrospective review of the clinical notes was conducted to collect demographic information, anthropometric data, dietary history, site and form of vascular access, details of HD prescription, complications, and outcomes. RESULTS: Nine patients weighing less than 10 kg were hemodialyzed for more than 6 months. Median age at commencement was 9 months. Median weight and height standard deviation score (SDS) at commencement of HD were -2.14 and -0.61, respectively, and at the end they were -1.56 and -1.61. Median energy intake was 96.6 kcal/kg/day and protein intake was 1.66 g/kg/day. Median number of line revisions was 0.32 line changes/patient year. Median central venous catheter (CVC) longevity was 13 months. Mean rate of line infection was 0.14/patient year. Median time on HD was 27 months. Median age at transplantation was 3.4 years. CONCLUSIONS: This case series shows that chronic HD is a viable management option in children <10 kg. Access issues can be minimized with good line care to maximize line longevity and minimize line infection rates.