Journal of the Korean Pediatric Society 1994;37(12):1757-1761.
Published online December 15, 1994.
A Case of Sjören-Larsson Syndrome
Kil Joon Lee, Jong Bock Kim, Dong Hwan Lee, Sang Man Shin, Sang Jhoo Lee
Department of Pediatrics, SoonChungHang University, College of Medicine, Seoul, Korea
Sjören-Larsson Syndrome 1례
이길준, 김종복, 이동환, 신상만, 이상주
순천향대학교 의과대학 소아과학교실
Abstract
Sjören-Larsson Syndrome is a rare disorder chracterized by congenital icthyosis, spastic diplegia or tetraplegia and mental retardation. The inheritance is autosomal recessive, due to deficiency of alcohol dehydrogenase activity of fatty acid matabolism. We have experienced a case of Sjören-Larsson Syndrome in a 16 month-old male children who have dried thick skin, developmental delay, and spastic diplegia. He was much improved clinically for skin lesion and developental status with etretinate supplement. We report the case with brief review of related literatures.
Key Words: Sjö, ren-Larsson Syndrome


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