Institute of Pathobiochemistry and Physiological Chemistry, University of Münster, Münster, Germany.
Ehlers-Danlos Syndrome Type VI (EDS VI): problems of diagnosis and management
Article first published online: 2 JAN 2007
Volume 87, Issue 6, pages 708–710, June 1998
How to Cite
Heim, P., Raghunath, M., Meiss, L., Heise, U., Myllylä, R., Kohlschutter, A. and Steinmann, B. (1998), Ehlers-Danlos Syndrome Type VI (EDS VI): problems of diagnosis and management. Acta Paediatrica, 87: 708–710. doi: 10.1111/j.1651-2227.1998.tb01537.x
- Issue published online: 2 JAN 2007
- Article first published online: 2 JAN 2007
- Received May 20, 1997. Accepted Dec. 2, 1997
- Hydroxylysine deficiency;
- joint laxity;
- skin hyperextensibility;
- spinal fusion
Ehlers-Danlos Syndrome Type VI (EDS VI) is a rare autosomal recessively inherited connective tissue disorder, which poses several problems of diagnosis and management. We report on a patient who developed severe kyphoscoliosis long before the diagnosis was reached. We conclude that early biochemical diagnosis and a timely operative procedure by extensive posterior instrumentation is the basis for successful management of this disorder.