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KMID : 1144020160120020096
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Journal of Korean Association for Disability and Oral Health
2016 Volume.12 No. 2 p.96 ~ p.100
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DENTAL TREATMENT IN A PATIENT WITH METACHROMATIC LEUKODYSTROPHY UNDER GENERAL ANESTHESIA : A CASE REPORT
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Ryu Ji-Yeon
Shin Teo-Jeon
Hyun Hong-Keun
Kim Young-Jae
Kim Jung-Wook
Jang Ki-Taeg
Kim Chong-Chul
Lee Sang-Hoon
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Abstract
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Metachromatic leukodystrophy (MLD) is a progressive and degenerative neurological disease caused by a deficiency of the catabolic enzyme arylsulfatase A. Deficiency of arylsulfatase A results in accumulation of sulfatide in the white matter of the peripheral and central nervous system and it occurs demyelination as a result. The patient gradually goes through mental and motor failure. General symptoms of MLD include gait disturbance, mental deterioration, muscle rigidity and impaired swallowing. Inheritance of the disease is autosomal recessive.
We report a dental caries treatment of a 3-year old boy with MLD. The patient underwent hematopoietic stem cell transplantation (HSCT) to slow the progression of the disease. He was suffered from difficulties of mastication and swallowing from the degenerative neurological symptom. He was ingesting food by both oral feeding and tubal feeding after he took percutaneous endoscopic gastrostomy (PEG). The cause of multiple caries was mainly presumed as patient’s prolonged time of meal.
The treatment was performed under general anesthesia considering patient’s incompliance. Severely affected lower primary molars were treated with pulp treatment and restored with stainless steel crown. Others were restored with composite resin. There were no postoperative complications.
MLD is life threatening progressive disease and also has an impact on unfavorable condition for oral health. Routine home oral care and periodic professional dental care should be emphasized to the caregiver of patient considering the susceptibility of dental caries. Not only the medical care, but periodic dental office visit would benefit the quality of life of the patient.
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KEYWORD
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Metachromatic leukodystrophy, Dental treatment, General anesthesia
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