RESUMO
BACKGROUND: The condition known as 22q11 microdeletion syndrome has a broad phenotypic spectrum, with many affected individuals experiencing mild-to-moderate immunodeficiency. Currently, there are significant variations in live vaccine practices and immunological testing prior to live vaccine administration due to safety concerns and limited established guidelines. METHODS: Queensland Children's Hospital (QCH) Child Development Unit, offers a state-wide 22q11 microdeletion clinic. This is a retrospective single-centre review, capturing the majority of children with 22q11 microdeletion in Queensland, Australia. We describe the live vaccination status of 134 children, age 0 to 18 years under our care between 2000 and 2018, adverse events following immunisation (AEFI) and the proportion of children who received additional pneumococcal coverage. An immunological investigation pathway prior to live vaccine administration is proposed. RESULTS: Of the 134 children, 124 were eligible for live vaccinations as per the Australian National Immunisation Program: 82% had received dose one of measles, mumps and rubella (MMR) vaccine, 77% had completed MMR dose two and 66% had completed varicella immunisation. There were no AEFI notifications reported. Of the total sample of children, 18% received a fourth dose of conjugate pneumococcal vaccine (Prevenar 7 or 13) and 16% received a dose of Pneumovax 23 from 4 years of age. Immunology workup practices were demonstrated to vary widely prior to live vaccine administration. Most patients' immune profiles were consistent with mild-to-moderate immunodeficiency. CONCLUSION: We propose an immunological investigation and vaccination pathway with the aim of providing guidance and consistency to clinicians caring for children with 22q11 microdeletion.
RESUMO
BACKGROUND: The aim of this study is to review patient characteristics, injury patterns, and outcomes of trauma cases admitted to pediatric intensive care in Children's Health Queensland, Brisbane, Queensland, Australia. METHODS: Routinely recorded data collected prospectively from the Children's Health Queensland Trauma Service registry from November 2008 to October 2015 were reviewed. Demographic and clinical characteristics of trauma cases in children under 16 years of age are described, and their association with age and mortality analyzed. RESULTS: There were 542 cases of pediatric trauma identified and 66.4% were male. The overall mortality since January 2012 was 11.1%. The median injury severity score (ISS) was 11 (IQR = 9-22), 48.2% (n = 261) had an ISS > 12 and 41.7% (n = 226) patients had an ISS > 15. The most common injury patterns were isolated head injury (29.7%; n = 161) and multiple trauma (31.2%; n = 169). In 28.4% of cases (n = 154) surgery was required. The home was reported to be the most common place of injury (37.6%; n = 204). Children aged 0-4 years were least likely to survive their injury (15.3% mortality) compared with the 5-9 (5.6% mortality) and 10-15 (9.0% mortality) age groups. Higher mortality was associated with more severe injuries, abdomen/spine/thorax injuries, inflicted injuries, drowning and hanging. CONCLUSION: This description of major pediatric trauma cases admitted to pediatric intensive care in Children's Health Queensland, Australia, will inform future pediatric major trauma service requirements as it identifies injury patterns and profiles, injury severity, management and mortality across different age groups.
