hyperinsulism vs diabetes

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Treatment Breakthrough for Rare Disease Linked to Diabetes

16-Mar-2011

University of Manchester scientists have led an international team to

discover new treatments for a rare and potentially lethal childhood disease

that is the clinical opposite of diabetes mellitus.

Congenital hyperinsulinism (CHI) is a condition where the body's pancreas

produces too much insulin - rather than too little as in diabetes - so

understanding the disease has led to breakthroughs in diabetes treatment.

This latest study, published in the journal Diabetes today (Wednesday,

3-16-2011), was carried out with clinical colleagues at hospitals throughout

Europe and at the two referral centres for hyperinsulinism in the UK, the

Royal Manchester Children's Hospital and Great Ormond Street Hospital,

London.

" In healthy insulin-producing cells of the pancreas, a small group of

proteins act as switches and regulate how much insulin is released, " said Dr

Cosgrove, who led the research with Professor Mark Dunne in

Manchester's Faculty of Life Sciences.

" When these proteins fail to function the cells can either release too

little insulin - resulting in diabetes mellitus, or too much insulin -

leading to congenital hyperinsulinism. "

She continued: " CHI causes dangerously low blood sugar levels which can lead

to convulsions and brain damage if not treated promptly. It is a complex

condition caused by gene defects that keep the insulin-producing cells

switched on when they should be switched off.

" Our group was the first to show how these gene defects led to uncontrolled

insulin release in patients a number of years ago. Now we have taken the

cells from patients following surgery and proven that, in some cases, it is

possible to correct defects in the rogue cells. "

Current drug treatments for CHI often fail in the most severe forms of the

disease and the patient has to have some, or most, of their pancreas

removed. The Manchester researchers discovered that treating cells under

specially modified conditions helped to recover the function of the internal

switches that control insulin release. Through these experiments the team

have provided the first evidence that the outcomes of gene defects can be

reversed in human insulin-producing cells.

One of the drugs used in their studies is currently in clinical trials to

treat patients with cystic fibrosis but has not been tested in patients with

CHI. The team hope that their findings will pave the way for new or similar

drugs to be used in clinical trials for hyperinsulinism.

" Although our results are really encouraging this is not a magic bullet for

the treatment of this devastating condition, but it does offer real hope

that in the future we may be able to use new drugs which can reverse the

cellular defects, " added Dr Cosgrove.

The clinical service at Royal Manchester Children's Hospital now includes

state-of-the-art facilities for imaging the pancreas to detect

hyperinsulinism. The clinical and academic teams work closely together

within NorCHI (Northern Congenital Hyperinsulinism in Infancy service) to

learn more about causes and treatments for this disease.

Source: University of Manchester

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