PMID- 27242065 OWN - NLM STAT- MEDLINE DCOM- 20170510 LR - 20191210 IS - 1469-8749 (Electronic) IS - 0012-1622 (Linking) VI - 58 IP - 11 DP - 2016 Nov TI - Intravenous immunoglobulin in paediatric neurology: safety, adherence to guidelines, and long-term outcome. PG - 1180-1192 LID - 10.1111/dmcn.13159 [doi] AB - AIM: Intravenous immunoglobulin (IVIG) is an expensive therapy used in immunodeficiency and autoimmune disorders. Increasing demands and consequent shortages result in a need for usage to conform to guidelines. METHOD: We retrospectively evaluated IVIG use for neuroimmunological indications and adherence to existing guidelines in a major Australian paediatric hospital between 2000 and 2014. RESULTS: One-hundred and ninety-six children (96 male, 100 female; mean age at disease onset 6y 5mo [range 3mo-15y 10mo], mean age at first IVIG dose 7y 2mo [range 3mo-16y 5mo]) received IVIG for neuroimmunological indications during the study period (28.1% had Guillain-Barre syndrome), representing 15.5% of all hospital indications. In total, 1669 IVIG courses were administered (total 57 221g, median 78g/patient, range 12-5748g). The highest median numbers of courses were in chronic inflammatory demyelinating polyneuropathies, opsoclonus-myoclonus ataxia syndrome, suspected immune-mediated epilepsies, and Rasmussen's encephalitis. Adverse reactions occurred in 25.5% of patients, but these were mostly minor. Outcome at follow-up was best in anti-N-methyl-d-aspartate receptor (anti-NMDAR) encephalitis, Guillain-Barre syndrome, and myasthenia gravis, and worst in Rasmussen's encephalitis and epilepsies. The total cost of IVIG was US$2 595 907 (median $3538/patient, range $544-260 766). Of patients receiving IVIG, 45.4% to 57.1% were given the therapy for 'weak' indications or indications 'not listed' in international guidelines. Some entities commonly treated with IVIG in current practice, such as anti-NMDAR encephalitis and transverse myelitis, are not listed in most guidelines. INTERPRETATION: Our study demonstrates that IVIG is generally well tolerated but expensive, and discloses discrepancies between guidelines and clinical practice in paediatric neurology, suggesting both the need for greater adherence to current recommendations, and for recommendations to be updated to accommodate emerging indications. CI - (c) 2016 Mac Keith Press. FAU - Nosadini, Margherita AU - Nosadini M AD - Neuroimmunology Group, Institute for Neuroscience and Muscle Research, Kids Research Institute, Children's Hospital at Westmead, University of Sydney, Sydney, NSW, Australia. AD - Paediatric Neurology Unit, Department of Paediatrics, University of Padua, Padua, Italy. FAU - Mohammad, Shekeeb S AU - Mohammad SS AD - Neuroimmunology Group, Institute for Neuroscience and Muscle Research, Kids Research Institute, Children's Hospital at Westmead, University of Sydney, Sydney, NSW, Australia. FAU - Suppiej, Agnese AU - Suppiej A AD - Paediatric Neurology Unit, Department of Paediatrics, University of Padua, Padua, Italy. FAU - Sartori, Stefano AU - Sartori S AD - Paediatric Neurology Unit, Department of Paediatrics, University of Padua, Padua, Italy. FAU - Dale, Russell C AU - Dale RC AD - Neuroimmunology Group, Institute for Neuroscience and Muscle Research, Kids Research Institute, Children's Hospital at Westmead, University of Sydney, Sydney, NSW, Australia. russell.dale@health.nsw.gov.au. CN - IVIG in Neurology Study Group AD - Neuroimmunology Group, Institute for Neuroscience and Muscle Research, Kids Research Institute, Children's Hospital at Westmead, University of Sydney, Sydney, NSW, Australia. LA - eng PT - Journal Article DEP - 20160531 PL - England TA - Dev Med Child Neurol JT - Developmental medicine and child neurology JID - 0006761 RN - 0 (Immunoglobulins, Intravenous) RN - 0 (Immunologic Factors) SB - IM CIN - Dev Med Child Neurol. 2016 Nov;58(11):1105-1106. PMID: 27358086 MH - Adolescent MH - Australia MH - Autoimmune Diseases of the Nervous System/*drug therapy MH - Child MH - Child, Preschool MH - Female MH - Follow-Up Studies MH - Guideline Adherence/*standards/statistics & numerical data MH - Hospitals, Pediatric/statistics & numerical data MH - Humans MH - Immunoglobulins, Intravenous/administration & dosage/adverse effects/economics/*therapeutic use MH - Immunologic Factors/administration & dosage/adverse effects/economics/*therapeutic use MH - Infant MH - Male MH - Neurology/*standards/statistics & numerical data MH - Outcome Assessment, Health Care/statistics & numerical data MH - Pediatrics/*standards/statistics & numerical data MH - Retrospective Studies FIR - Barclay, Peter IR - Barclay P FIR - Koh, Yvonne IR - Koh Y FIR - Teo, Juliana IR - Teo J FIR - Tantsis, Esther M IR - Tantsis EM FIR - Ramanathan, Sudarshini IR - Ramanathan S FIR - Webster, Richard IR - Webster R EDAT- 2016/06/01 06:00 MHDA- 2017/05/11 06:00 CRDT- 2016/06/01 06:00 PHST- 2016/04/09 00:00 [accepted] PHST- 2016/06/01 06:00 [pubmed] PHST- 2017/05/11 06:00 [medline] PHST- 2016/06/01 06:00 [entrez] AID - 10.1111/dmcn.13159 [doi] PST - ppublish SO - Dev Med Child Neurol. 2016 Nov;58(11):1180-1192. doi: 10.1111/dmcn.13159. Epub 2016 May 31.