Rare diabetic syndrome found in Kashmir valley
Patients Suffer From Blindness, Deafness Before Death
Srinagar, Mar 23: A new form of diabetes has emerged in the Valley. Known as Wolfram Syndrome, it has been found in seven persons in Kashmir out of whom two have died.
Wolfram Syndrome is also known as Diabetes Insipidus Diabetes Mellitus Optic Atrophy and Deafness (DIDMOAD). It is relatively rare inherited neurodegenerative disorder first evident in childhood as an association of juvenile-onset diabetes mellitus.
The affected person shows progressive visual handicap leading to blindness due to degeneration of main nerve of the eye (optic atrophy). This is followed by progressively worsening deafness and increased formation of urine due to lack of hormone called vasopressin (diabetes insipidus).
The basic defect being genetic, the disorder causes neuro-degeneration in the body and death usually occurs at younger age. The affected persons don’t cross 50 years. Two of the persons among seven affected have died at age 35 and 18.
Doctors say intra-family marriages prevalent among Muslim families in the Valley could also lead to this disease if the family has genetic defect.
“Over the past five years, seven cases of Wolfram Syndrome were detected among people who have got diabetes at young age. But the diabetes found in these patients was different from the routine type-1 or 2 diabetes mellitus,” says Dr Muhammad Ashraf Ganai, Associate Professor in the Department of Endocrinology, Sher-e-Kashmir Institute of Medical Sciences, who is the lead author of the research which has been accepted and published by DIABETIC MEDICINE- official journal of British Diabetes Association.
“We got clue by seeing very brittle type of diabetes with visual disturbance due to optic nerve involvement. These people were treated as type-1 diabetes mellitus. After detailed evaluation of families of affected persons including genetic analysis in collaboration of culprit gene WFS-1, the disease was confirmed. This is the largest series from Asia and only one case was documented from South India,” says Dr. Ashraf.
There is no treatment available for the disorder except controlling blood-glucose and giving medication for decreasing urination.
“At present only help we can do to the family is to screen or pick up the members having gene defect and counseling them about the risks of intra-family marriage,” says Dr. Ashraf.
Doctors advise that if any couple is consanguineous (married in close relations) then they should go for pre-natal genetic testing, followed by medical termination of affected pregnancies.
They say Wolfram Syndrome is one of the autosomal recessive disorders, where marriages within family increase the disorder. The child who gets both defective gene copies from either parent gets the disease.
“Since Kashmiris marry within families like other Muslim populations of the world the disorder exists among them. However, in the present series only two persons out of seven had come from consanguineous parents indicating high prevalence of gene defect in our population,” says Dr Ashraf.
The gene defect can occur in the families of affected persons and can get converted into a full blown disease in a child who is born to parents having the gene defect.
Seeing the importance of the disorder, a review article was invited on the subject in the journal of Pediatric Endocrinology and Metabolism.
Lastupdate on : Fri, 23 Mar 2012 21:30:00 Makkah time
Lastupdate on : Fri, 23 Mar 2012 18:30:00 GMT
Lastupdate on : Sat, 24 Mar 2012 00:00:00 IST
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