WISKOTT-ALDRICH SYNDROME WITH DENTAL PROBLEMS : CASE REPORT

Lee, Yeon-Joo; Hyun, Hong-Keun; Jang, Chul-Ho; Kim, Yeong-Jae; Kim, Jung-Wook; Jang, Ki-Taek; Kim, Chong-Chul; Hahn, Se-Hyun; Lee, Sang-Hoon

Journal of the Korean Academy of Pediatric Dentistry 2007;34(3):468-472.
Published online August 31, 2007.

WISKOTT-ALDRICH SYNDROME WITH DENTAL PROBLEMS : CASE REPORT

Yeon-Joo Lee, Hong-Keun Hyun, Chul-Ho Jang, Yeong-Jae Kim, Jung-Wook Kim, Ki-Taek Jang, Chong-Chul Kim, Se-Hyun Hahn, Sang-Hoon Lee

Department of Pediatric Dentistry, College of Dentistry and Dental Research Institute, Seoul National University

Wiskott-Aldrich 증후군 환아의 증례보고

이연주, 현홍근, 장철호, 김영재, 김정욱, 장기택, 김종철, 한세현, 이상훈

서울대학교 치과대학 소아치과학교실 및 치학연구소

Correspondence: Sang-Hoon Lee, Tel: 02-2072-2680, Email: musso@snu.ac.kr

Abstract

The Wiskott-Aldrich Syndrome (WAS) is an inherited immunodeficiency caused by a variety of mutations in the gene encoding the WAS protein (WASp). First described in 1937 by Wiskott, the incidence of WAS has so far been estimated at 4 in 106 live births. The Wiskott-Aldrich Syndrome is an X-linked condition characterized by 1) an increased tendency to bleed caused by a reduced number of platelets, 2) recurrent bacterial, viral and fungal infections, and 3) eczema of the skin. The purpose of this report is to present cases highlighting the clinical features of the syndrome and the required considerations in the treatment of patients. The report consists of two particular cases: a 2-year-11-month-old boy seen for a routine oral examination prior to his bone marrow transplantation and a 2-year-6-month-old boy with herpes gingivostomatitis and teeth discoloration.

Key Words: Wiskott-Aldrich syndrome, Thrombocytopenia, Infection, Discoloration, Dental consideration