Keywords
Mucopolysaccharidosis Type IV
Syndrome
Graft Vs Host Disease
Dental Caries
Dental Enamel
Morquio Syndrome
Glycosaminoglycans.
Syndrome
Graft Vs Host Disease
Dental Caries
Dental Enamel
Morquio Syndrome
Glycosaminoglycans.
How to Cite
HP, C., Harjai, G., Doddawad, V. G., & S, M. (2023). Comprehensive Preventive and Therapeutic Oral Health Care: A Case Report of Mucopolysaccharidosis Type IV A in a Pediatric Patient. Puerto Rico Health Sciences Journal, 42(4), 332–334. Retrieved from https://prhsj.rcm.upr.edu/index.php/prhsj/article/view/3043
Abstract
Mucopolysaccharidosis (MPS) is a metabolic disorder resulting from a deficiency of lysosomal enzymes. It is an autosomal recessive disorder with similar incidences in men and women. Mucopolysaccharidosis type IV A is caused by a deficiency of N-acetylgalactosamine-6-sulfatase, which deficiency is, in turn, caused by alterations in the GALNS gene. It is marked by a short stature, a pigeon chest, frontal bossing, kyphosis, and a flat nasal bridge. Intraorally, macroglossia, hypodontia, dentinogenesis imperfecta, a broad mouth, and an anterior open bite are some of the common features. The present paper reports on a case of MPS in a 5-year-old male patient, along with providing a review of the literature and insight into the oral manifestations related to MPS IV A, also called Morquio A syndrome, and its dental treatment. It aims to highlight the clinical recommendations for oral health care in such cases during different phases of MPS IV A treatment.
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