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A brief history of MECP2 duplication syndrome: 20-years of clinical understandingMECP2 duplication syndrome (MDS) is a rare, X-linked, neurodevelopmental disorder caused by a duplication of the methyl-CpG-binding protein 2 (MECP2) gene-a gene in which loss-of-function mutations lead to Rett syndrome (RTT). MDS has an estimated live birth prevalence in males of 1/150,000.
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A Review of Cardiac Surgical Procedures and Their Outcomes for Paediatric Rheumatic Heart Disease in Western AustraliaSurgical intervention is an important treatment modality for advanced rheumatic heart disease (RHD). This study aimed to describe patient characteristics and outcomes from cardiac surgery for RHD in patients referred to the only tertiary paediatric hospital in Western Australia.
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Immunogenicity, reactogenicity, and IgE-mediated immune responses of a mixed whole-cell and acellular pertussis vaccine schedule in Australian infants: A randomised, double-blind, noninferiority trialIn many countries, infant vaccination with acellular pertussis (aP) vaccines has replaced use of more reactogenic whole-cell pertussis (wP) vaccines. Based on immunological and epidemiological evidence, we hypothesised that substituting the first aP dose in the routine vaccination schedule with wP vaccine might protect against IgE-mediated food allergy. We aimed to compare reactogenicity, immunogenicity, and IgE-mediated responses of a mixed wP/aP primary schedule versus the standard aP-only schedule.
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Klebsiella aerogenes Adhesion Behaviour during Biofilm Formation on MonaziteThe adsorption behaviour of micro-organisms during the initial attachment stage of biofilm formation affects subsequent stages. The available area for attachment and the chemophysical properties of a surface affect microbial attachment performance.
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Immunogenicity and Safety of a 2 + 1 DTPa Priming Schedule in Australian Infants and the Impact of Maternally Derived Antibodies on Pertussis Antibody Responses up to 4 Years of AgeWe assessed the impact of maternally derived pertussis antibodies on infant responses to a 2 + 1 vaccine schedule (6 weeks, 12 weeks, and 12 months). Infants with baseline antibodies showed lower IgG responses following the primary vaccination series, but this did not impair booster responses at 4 years of age.
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Nirsevimab immunisation of infants and respiratory syncytial virus (RSV)-associated hospitalisations, Western Australia, 2024: a population-based analysisChristopher Peter Hannah Blyth Richmond Moore MBBS (Hons) DCH FRACP FRCPA PhD MBBS MRCP(UK) FRACP OAM BSc (Hons) GradDipClinEpi PhD Centre Head,
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PCV10 elicits Protein D IgG responses in Papua New Guinean children but has no impact on NTHi carriage in the first two years of lifeNasopharyngeal colonisation with nontypeable Haemophilus influenzae (NTHi) is associated with development of infections including pneumonia and otitis media. The 10-valent pneumococcal conjugate vaccine (PCV10) uses NTHi Protein D (PD) as a carrier. Papua New Guinean children have exceptionally early and dense NTHi carriage, and high rates of NTHi-associated disease.
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Optimising a 6-plex tetanus-diphtheria-pertussis fluorescent bead-based immunoassaySmall volume assays are required for large-scale research studies and in particular paediatric trials, where multiple measures are required from a single sample. Fluorescent bead-based technology (Bioplex/Luminex) allows high through-put and simultaneous quantification of multiple analytes in a single test. This technology uses sets of microspheres, each with a unique spectral address that can be coated with a different antigen of interest.
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Impaired Cytokine Responses to Live Staphylococcus epidermidis in Preterm Infants Precede Gram-positive, Late-onset SepsisLate-onset sepsis (LOS) with Staphylococcus epidermidis is common in preterm infants, but the immunological mechanisms underlying heightened susceptibility are poorly understood. Our aim is to characterize the ontogeny of cytokine responses to live S. epidermidis in preterm infants with and without subsequent Gram-positive LOS.
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Pertussis immunisation in infancy and atopic outcomes: A protocol for a population-based cohort study using linked administrative dataThe burden of IgE-mediated food allergy in Australian born children is reported to be among the highest globally. This illness shares risk factors and frequently coexists with asthma, one of the most common noncommunicable diseases of childhood.