Twitter
Advertisement

Know everything about BENTA, the rare disease detected in a 7-month-old Bengaluru baby

Vijayadra's is first BENTA case that has been identified at an early stage in terms of age & severity. He can be saved by a blood stem cell transplant

Latest News
article-main
(Image Source: impactguru.com)
FacebookTwitterWhatsappLinkedin

A seven-month-old baby from Bengaluru is suffering from a serious and rare disease named BENTA. Worldwide only 14 children suffer from this disease. Actually this disease is a extremely rare primary immunodeficiency disorder. Vijayadra's case is the first case of BENTA in the world that has been identified at an early stage in terms of age and severity.

The toddler, Vijayadra's life can only be saved by a blood stem cell transplant, says DKMS BMST Foundation India. The Bengaluru-based blood stem cell registry is coordinating the search for a matching donor for the child. In India, only 0.04 per cent of the population is registered as potential donors especially due to unawareness and apprehensions people have around blood stem cell donation.

What is BENTA disease

The serious and rare disease named BENTA stands for B-cell expansion with NF-κB and T-cell anergy.

B-cell is a type of immune cell from the bone marrow and in this case the number is greater than normal.

NF-κB is a protein complex involved in gene expression, or the degree to which certain genes are turned on or off.

T-cell is a type of immune cell that matures in the thymus, a small organ located in the upper chest under the breastbone.

Anergy refers to a 'less-than-normal' (T cell) immune reaction to foreign substances.

The BENTA disease is characterised by high levels of certain immune cells starting in infancy.

This includes enlarged spleen, enlarged lymph nodes, immunodeficiency, and an increased risk of lymphoma, a type of cancer.

BENTA disease is diagnosed based on clinical and laboratory findings as well as genetic testing.

Children of a person who carries a 'CARD11 mutation' have a 50% chance of inheriting the mutation.

The main symptoms include spleen enlargement (splenomegalia) and frequent ear, sinus, and lung infections early in life.

Every person has two copies of the CARD 11 gene, one inherited from each parent.

Only one of the two copies of CARD 11 needs to be abnormal for a person to have BENTA disease. 

Children who do not inherit the mutation will not develop BENTA disease or pass on the mutation to their children.

People with BENTA disease must be closely monitored for any signs of a B-cell cancer.

Find your daily dose of news & explainers in your WhatsApp. Stay updated, Stay informed-  Follow DNA on WhatsApp.
Advertisement

Live tv

Advertisement
Advertisement