Belfast Telegraph

Early treatment key to helping 'giants' of mid-Ulster, insists leading professor

By Lynne Kelleher

One of the leading experts on gigantism is hoping early treatment can halt the excessive growth caused by the condition in some people in Northern Ireland.

One part of Mid Ulster was recently identified as a 'giant hotspot' by scientists studying a gene defect that can be traced back 2,500 years and that causes people to grow abnormally tall.

One in 150 people in Mid Ulster were found to carry the gene, called AIP, which is extremely rare outside Northern Ireland. It can result in too much growth hormone, which is produced and released by the pituitary gland, a pea-sized gland just below the brain.

In a new BBC documentary, Bones, Professor Marta Korbonits, who discovered the Irish giant gene, said the key to curbing the excessive growth was to identify carriers early.

"We cannot make people shorter. From that point of view, we cannot treat the giants", said the professor, an endocrinologist at Barts and the London School of Medicine. "We certainly can treat the disease. Gigantism is quite an awful disease with a huge number of complications."

It is hoped that increased awareness and screening will identify those at risk of passing on the gene to future generations, leading to earlier diagnosis and treatment.

Over-production of the growth hormone occurs as the result of a benign tumour in the gland, which can be removed through surgery. Medication can also be used to treat the condition, which has been narrowed down to 18 families.

Patients usually start to show symptoms between the ages of 10 and 20, but children can now be treated from as young as three.

Most carriers are unaffected, but around 20% are affected by the condition, which can be fatal if untreated.

Tyrone man Brendan Holland, who is 6ft 9ins tall, was only diagnosed with pituitary gigantism after he went to London aged 20.

"I was shocked when I heard the word 'giant' used, but I didn't consider myself that," Mr Holland said.

Bone Stories id on BBC Radio Four at 1.45pm on August 14

Belfast Telegraph


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