Jervell-lange nielsen syndrome in a family with the long QT syndrome (LQTS)
Department of Pediatrics, Lokmanya Tilak Municipal Medical College & General Hospital, Sion, Mumbai, India Sir,
I read with interest the clinical brief entitled "Jervell-Lange Nielsen Syndrome in a Family with the Long QT Syndrome (LQTS)" by R.K.Mondal et al, published in the July 2006 issue of the Indian Journal of Pediatrics. I believe the authors have not been diligent in their search for cases reported from India since they claim that there has been no report of any family with Jervell Lange-Nielsen Syndrome prior to theirs.
The authors themselves have studied the families of three children with the long QT syndrome. The index cases in the first two families had profound sensorineural deafness - the classical Jervell Lange-Nielsen syndrome. There was a significant history of deafness and sudden deaths during infancy and early childhood in two siblings of the first child. Investigation revealed the long QT syndrome in three living members of his family and one of them a 12-year-old girl, had a history of recurrent syncope. No abnormality could be found in the parents of the second child, but two maternal uncles had a history of deafness but no syncope (they were not available for investigation)[1].
This study has been published in the February 2002 issue of this very journal(Shanbag Preeti)
I read with interest the clinical brief entitled "Jervell-Lange Nielsen Syndrome in a Family with the Long QT Syndrome (LQTS)" by R.K.Mondal et al, published in the July 2006 issue of the Indian Journal of Pediatrics. I believe the authors have not been diligent in their search for cases reported from India since they claim that there has been no report of any family with Jervell Lange-Nielsen Syndrome prior to theirs.
The authors themselves have studied the families of three children with the long QT syndrome. The index cases in the first two families had profound sensorineural deafness - the classical Jervell Lange-Nielsen syndrome. There was a significant history of deafness and sudden deaths during infancy and early childhood in two siblings of the first child. Investigation revealed the long QT syndrome in three living members of his family and one of them a 12-year-old girl, had a history of recurrent syncope. No abnormality could be found in the parents of the second child, but two maternal uncles had a history of deafness but no syncope (they were not available for investigation)[1].
This study has been published in the February 2002 issue of this very journal(Shanbag Preeti)