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Sjogren-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 Sjogren-Larsson Syndrome in a 16 month-old male children who have dried thick skin, developmental delay, and spastic diplegia. He also has glistening spot on macula area with irregular high voltage slow wave (2-3Hz) on electroencephalography. From Biopsy finding, Lamella ictyosis with acanthosis and hyperkeratosis in epithelium is shown. He was much improved clinically for skin lesion and developmental status with etretinate supplement. We report the case with brief review of related literatures.
Acitretin ; Alcohol Dehydrogenase ; Biopsy ; Cerebral Palsy ; Child ; Electroencephalography ; Epithelium ; Etretinate ; Humans ; Infant ; Intellectual Disability ; Male ; Quadriplegia ; Sjogren-Larsson Syndrome* ; Skin ; Wills

9.A study of serologically confirmed measles.

Sang Mook CHOI ; Chang Soo OH ; Sang Chul PARK ; Chang Hwi KIM ; Sang Jhoo LEE

Journal of the Korean Pediatric Society 1992;35(4):508-514

10.Three Cases of Kawasaki Disease Accompanied with Rare Clinical Menifestation.

Min Yong OUM ; Sang Eun LEE ; Jae Ock PARK ; Chang Hwi KIM ; Sang Jhoo LEE

Journal of the Korean Pediatric Society 1989;32(9):1300-1308

1.A Case of Sirenomelia.

2.Two cases of meconium peritonitis.

3.Glucose-6 Phosphate Dehydrogenase Deficiency.

4.Three Cases of Insulin Dependent Diabetes Mellitus in Children.

5.A Case of Nonimmunologic Hydrops Fetalis Associated with Down Syndrome.

6.A Case of Congenital Adrenal Agenesis.

7.A Study on Lighting in school.

8.A Case of Sjogren-Larsson Syndrome.

9.A study of serologically confirmed measles.

10.Three Cases of Kawasaki Disease Accompanied with Rare Clinical Menifestation.

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