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Brothers too hot to be healthy

Perspiring may be a social embarrassment, but don’t forget to thank your sweat glands.

Too few of them cause a disorder known as anhidrotic ectodermal dysplasia with immune deficiency. Medical journals claim only one in 2,50,000 individuals have it, but if you tell tribal mother Naguri Soy of West Singhbhum this number, you may get a hard stare.

According to doctors at Rajendra Institute of Medical Sciences (RIMS), Ranchi, where Naguri’s two boys, Rohit (5) and Mangal (3) are admitted since Monday evening, both the brothers seem to exhibit symptoms of this rare medical condition.

Their normal body temperature always high, they don’t have teeth and hair.

The boys, who were born in Dobrobasa village in Tantnagar block of West Singhbhum, have always had very high temperature.

Since their birth, Naguri has taken great pains to often immerse them in water, make them wear as little clothes as possible and keep them cool.

In normal people, heat escapes through vents such as sweat glands, natural body coolers.

But most persons with the disorder have little ability to sweat, medically called hypohidrosis.

Either they have fewer sweat glands than normal or those do not function properly. A complete inability to sweat, or anhidrosis, can lead to alarmingly hot skin, particularly in summer.

Hope dims in childhood for the severest cases, but in others, proper treatment can help sufferers live a life outside water buckets.

Arun Kumar Sharma, who is the head of the RIMS paediatrics department, told The Telegraph on Tuesday there was every possibility that both the boys from West Singhbhum had anhidrotic ectodermal dysplasia with immune deficiency.

To confirm it, he has asked the brothers to undergo skin biopsy.

Once the results come in, the disorder would be treated. “As far as I know, 7,000 cases have been reported across the world till date. Genetics are involved. While there is no cure, medication can alleviate the suffering,” the doctor said.

Naguri, meanwhile, does not understand anything except that her sons are “different” from others. “I don’t understand why my sons are different. We are simple folk. We don’t understand what doctors say,” said the young mother.

She added that her sons did not go to school to avoid wearing uniforms.

“They stay in underpants as putting on clothes will raise their body temperature further. Summer is very difficult for them,” Naguri said.

Sharma said this was not the first case in Jharkhand. “Way back in 1994, I had diagnosed one case in Hazaribagh. Once confirmation of the disorder in these two boys come, I will stress on the need to conduct research on the disorder, taking account of the family tree,” he said.