Emerging workstreams

EATC4Children continue to work on the development of emerging workstreams, including a focus on Behcet's syndrome, Juvenile Dermatomyositis (JDM) and our Childhood renal inflammatory programme.

Behcet’s syndrome

Behçet’s syndrome is a rare, multi-system inflammatory condition characterised by recurrent oral ulceration, genital ulceration, eye and skin involvement. There is currently very little data on incidence and prevalence of Behçet’s syndrome in children and young people, particularly within the UK and ROI population.

BPSU Behçet’s study.

The purpose of this study is to establish the current UK and ROI incidence and prevalence of Behçet’s syndrome and to describe the burden of disease in children under 16 years of age in the UK. This study has completed follow up (CI: C. Pain).

Molecular mechanisms of bronchial inflammation

Does inhalation therapy enhance bronchial inflammation in viral bronchiolitis?

Small airway inflammation (bronchiolitis) is the most common cause of hospital admission in the first year of life. Currently, there are no specific treatments for this condition other than management based on supportive measures that can include inhalation treatment. This is thought to induce cough as a clearing mechanism for mucus that would otherwise obstruct airways, but its effectiveness as a treatment for bronchiolitis is currently unknown. This study will help to understand mechanisms of airway inflammation during virus infection, and whether inhalation treatment helps or hinders these mechanisms (CIs: C. Hedrich and P. McNamara).

Systemic inflammation in Cystic Fibrosis

Cystic fibrosis (CF) is a heritable and devastating multisystem disease, particularly involving the lungs and gastrointestinal tract. Tissue inflammation is a central contributor to disease expression and organ damage in CF, but the exact mechanisms triggering inflammation remain unclear.

Since a subset of CF patients develop joint pain and/or inflammation (arthritis), and some patients even develop symptoms of vasculitis (blood vessel inflammation), uncontrolled activation of immune cells appears likely to be involved in the pathophysiology of CF and its sequelae. In this project, we investigate molecular mechanisms contributing to bronchial and systemic inflammation (CIs: C. Hedrich and P. McNamara).

Juvenile dermatomyositis (JDM)

Juvenile dermatomyositis (JDM) is an autoimmune, inflammatory disease of the muscle, skin, and blood vessels. It is a rare condition that affects approximately 3 million children in the UK each year and girls are twice as likely to be affected as boys.

Juvenile Dermatomyositis National (UK and Ireland) Cohort Biomarker Study and Repository for Idiopathic Inflammatory Myopathies

This UK network is collecting data and samples from JDM cases to be used to used questions about JDM (Investigator L. McCann).

Development of an internationally agreed minimal dataset for Juvenile Dermatomyositis (JDM).

The JDM minimal dataset study aims to get international agreement on a core set of information (data) that clinicians and researchers need to collect on all patients with JDM (CI: L. McCann).

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