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Exploring quality of life in individuals with a severe developmental and epileptic encephalopathy, CDKL5 Deficiency Disorder

CDKL5 Deficiency Disorder (CDD) is a rare genetic disorder caused by a mutation in the cyclin-dependent kinase-like 5 (CDKL5) gene. It is now considered to be a developmental and epileptic encephalopathy because of the early onset of seizures in association with severe global delay. Other features include cortical visual impairment, sleep and gastro-intestinal problems. Progress in clinical understanding, especially regarding the spectrum of functional ability, seizure patterns, and other comorbidities was initially slow but accelerated in 2012 with the establishment of the International CDKL5 Database (ICDD). Our aim was to use this data source to investigate quality of life (QOL) and associated factors in this disorder.

Disparities between Aboriginal and non-Aboriginal perinatal mortality rates in Western Australia from 1980 to 2015

This study aimed to examine the pattern of stillbirth and neonatal mortality rate disparities over time in Western Australia

Personalized transcriptomics reveals heterogeneous immunophenotypes in children with viral bronchiolitis

Dysregulated expression of IFN-dependent pathways after respiratory viral infections is a defining immunophenotypic feature of AVB-susceptible infants

Vagus nerve stimulation for the treatment of refractory epilepsy in the CDKL5 Deficiency Disorder

Our study suggests that vagus nerve stimulation is a generally safe and effective adjunct treatment for CDKL5-associated epilepsy

Distinguishing benign from pathologic TH2 immunity in atopic children

In addition to its role in blocking TH2 effector activation in the late-phase allergic response, IL-10 is a known IgG1 switch factor

Caregiver Perspective of Benefits and Side Effects of Anti-Seizure Medications in CDKL5 Deficiency Disorder from an International Database

CDKL5 deficiency disorder presents as a challenging condition with early-onset refractory seizures, severe developmental delays, and a range of other neurological symptoms. Our study aimed to explore the benefits and side effects of anti-seizure medications in managing seizures among individuals with CDKL5 deficiency disorder, drawing on data from the International CDKL5 Disorder Database.  

Protection against severe infant lower respiratory tract infections by immune training: Mechanistic studies

Results from recent clinical studies suggest potential efficacy of immune training (IT)-based approaches for protection against severe lower respiratory tract infections in infants, but underlying mechanisms are unclear.

Content Validation of Clinician-Reported Items for a Severity Measure for CDKL5 Deficiency Disorder

CDKL5 deficiency disorder (CDD) results in early-onset seizures and severe developmental impairments. A CDD clinical severity assessment (CCSA) was previously developed with clinician and parent-report items to capture information on a range of domains.

Mental Health & Wellbeing

Positive mental health, flourishing, and resilience to stress are not only critical to the overall wellbeing of a person but also their physical health, affecting both chronic and acute health conditions

Prenatal ambient heat exposure and neurodevelopment: A scoping review of human and animal research

Ambient heat exposure during pregnancy is associated with adverse outcomes, and adverse neurodevelopmental outcomes are an emerging concern. This scoping review synthesises human and animal evidence on the association between prenatal ambient heat exposure and poor neurodevelopmental outcomes.

Development and initial validation of the Communication Inventory Disability – Observer Reported (CID-OR): a measure of communication in CDKL5 deficiency disorder

CDKL5 Deficiency Disorder (CDD) is a rare neurodevelopmental disorder characterised by early onset seizures combined with complex healthcare needs and developmental impairment that influence functional domains including communication. Communication is a high priority domain for families but currently used measures demonstrate floor effects. 

Pharmacological targeting and characterization of Voltage-Gated Sodium Channels (VGSCs) expressed in the high-grade glioma microenvironment

High-grade glioma (HGG) cells reactivate neurodevelopmental programs regulated by ion channels to drive tumor progression. The activity of voltage-gated sodium channels (VGSCs) is fundamental to development, a target of blood-brain barrier (BBB)-permeable FDA-approved drugs, and aids tumor advancement in several cancers. However, the contribution of VGSC activity to HGG pathology remains unknown.

First Nations Health and Equity

Aboriginal health is everyone's business. The needs of Aboriginal and Torres Strait Islander families and kids is integrated into all relevant areas of our work. Improving the health and wellbeing of Aboriginal and Torres Strait Islander kids and families is an overarching priority for every team at The Kids.

Working Together Second Edition

This 2nd edition is intended for staff and students and all health practitioners working in areas that support Indigenous mental health and wellbeing.

Kulunga Aboriginal Unit

Facilitate research interest & opportunities that involve Aboriginal families & communities and build the capacity and development of Institute researchers

Start Stronger, Live Longer Resource Manual for Aboriginal Health Workers

This resource kit for Aboriginal health workers is an exciting milestone in the Rio Tinto Aboriginal Health partnership with The Kids Research Institute Australia

KAMS MOU

As part of the discussions with Kimberley Aboriginal Medical Service (KAMS) to establish the Broome site of the WAAHKN it has been agreed to establish...

CliniKids puts the ‘evidence’ in evidence-based practice

Autism researchers at The Kids have led the most comprehensive review of the evidence for autism intervention ever compiled