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Previous Volume 336:1562-1567 May 29, 1997 Number 22 Next

	Since this article has no abstract, we have provided an extract of the first 100 words of the full text and any section headings.

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In 1957, Jervell and Lange-Nielsen reported a syndrome of congenital sensory deafness associated with a prolonged QT interval in four children of a Norwegian family.¹ The affected children had multiple syncopal episodes, and three died suddenly at the ages of four, five, and nine years. Since 1957, other examples of the long-QT syndrome associated with deafness (the Jervell and Lange-Nielsen syndrome) have been described.^2,3,4 In all cases, the apparent mode of inheritance was autosomal recessive. This syndrome is rare (estimated incidence, 1.6 to 6 cases per million).² Affected persons are susceptible to recurrent syncope, and they have a high incidence . . . [Full Text of this Article]

Methods

Ascertainment and Phenotyping of the Kindred

Linkage Analysis

Mutation Analysis

DNA-Sequence Analysis

Results

Phenotypic Characteristics

Linkage Analysis

Mutation Analysis

Discussion

Source Information

From the Eccles Institute of Human Genetics (I.S., D.L.A., M.T.K.), the Cardiology Division (K.W.T., G.M.V., M.T.K.), and the Howard Hughes Medical Institute (D.L.A., M.T.K.), University of Utah; and the Department of Medicine (K.W.T., G.M.V.), Latter-Day Saints Hospital — all in Salt Lake City.

Address reprint requests to Dr. Keating at the Howard Hughes Medical Institute, Suite 5100 EIHG, University of Utah, Salt Lake City, UT 84112.

References

Related Letters:

Molecular Basis of the Long-QT Syndrome
Bitner-Glindzicz M., Tyson J., Jamieson R., Keating M. T.
Extract | Full Text  
N Engl J Med 1997; 337:1011-1013, Oct 2, 1997. Correspondence

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