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1.
P R Health Sci J ; 42(4): 332-334, 2023 Dec.
Article En | MEDLINE | ID: mdl-38104293

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.


Chondroitinsulfatases , Mucopolysaccharidosis IV , Male , Humans , Child , Female , Child, Preschool , Mucopolysaccharidosis IV/genetics , Mucopolysaccharidosis IV/therapy , Chondroitinsulfatases/genetics , Chondroitinsulfatases/metabolism , Delivery of Health Care
2.
J Indian Soc Pedod Prev Dent ; 41(4): 297-301, 2023 Oct 01.
Article En | MEDLINE | ID: mdl-38235815

BACKGROUND: The tongue coating is a grayish-white deposit that quickly reflects the state of health or disease of the human body. Quantitative light-induced fluorescence (QLF) is a novel digital imaging system that objectively quantifies tongue coating. AIMS: The present study aims to evaluate the correlation between the visual assessment of tongue coating and tongue coating by analysis of QLF-digital (QLF-D) images. SETTINGS AND DESIGN: This was an in vivo explorative study. MATERIALS AND METHODS: Fifty children aged 11-13 years with clinically visible tongue coating were selected for the study. Tongue coating was assessed clinically by the Tongue Coating Index (Shimizu et al., 2007) and digitally by QLF-D Biluminator™ 2, C3 software. STATISTICAL ANALYSIS: Data obtained were subjected to statistical analysis using SPSS 23.0 software. Spearman's rho correlation test was done, and P < 0.05 was considered statistically significant. RESULTS: A statistically significant correlation was found between the visual assessment scoring and the QLF image analysis for the evaluation of tongue coating. CONCLUSION: The Digital QLF tongue imaging system was found to be reliable due to its correlation with the clinical score and objective nature.


Dental Caries , Quantitative Light-Induced Fluorescence , Child , Humans , Fluorescence , Tongue/diagnostic imaging
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