BPSU study - autoimmune Addison’s disease in children

Surveillance of autoimmune Addison's disease commenced in July 2011 and concluded in July 2012. The study was led by Dr Hima Bindu Avatapalle, winner of the Sir Peter Tizard Bursary 2009-10. A paper has yet to be published.

Lead investigator

Dr Hima Bindu Avatapalle
Manchester Children’s Hospital
Manchester M13 9WL
Email: bindu.avatapalle@cmft.nhs.uk

About the study

Overview

The most common cause of severe adrenal insufficiency in children, now known as Addison’s disease, is autoimmune. In this condition, the body’s own immune system attacks the adrenal glands and destroys them.

People suffering from Addison's disease are also at increased risk of other organ-specific autoimmune diseases, e.g. of the thyroid gland.

If untreated the condition is life-threatening and unfortunately it is not always spotted early enough. Though it is now more than 150 years since it was first described, the disease remains under-diagnosed, leading to unnecessary morbidity and mortality.

Autoimmune Addison’s disease in children is an uncommon but potentially lethal condition.

We intend to undertake a 13-month study to evaluate this rare condition with important clinical relevance. The current incidence of autoimmune Addison’s disease in UK (under 16 yrs) will be identified. Clinical presentation and diagnostic strategies will be analysed and reported in scientific literature. Variations in emergency management will be highlighted (for instance provision of emergency injectable treatment, steroid card and ambulance cover).

You can download the protocol card, including references, below.

Case definition

Any child from birth up to but not including 16 years of age with newly presenting suspected or proven auto immune Addison’s disease should be reported on the BPSU orange card.

The diagnosis of autoimmune Addison’s disease can be made following clinical presentation of adrenal insufficiency or Addisonian crisis.

Reporting instructions

Please report any child seen for the first time in the UK or Ireland during the study period who satisfies the case definition regardless of country of birth.

If the diagnosis is awaiting confirmation, (adrenal cortical antibodies not done or awaited), the child should still be reported.

Duration

July 2011 - July 2012 (13 months).

Funding

Sir Peter Tizard Bursary.

Approval

This study has been approved by West London REC (Ref: 11/LO/0581) and has been granted Section 251 NIGB permission under reference: ECC 6-02 (FT4/BPSU)/2011.