Analysis of salivary parameters of mucopolysaccharidosis individuals.

Patrícia Luciana Serra Nunes,Luiz Renato Paranhos,Elizabeth Soares Fernandes,Michelle Foigel Siqueira,Valentim Adelino Ricardo Barão,Cauane Blumenberg,Walter Luiz Siqueira,Rebeca Garcia Ferreira,Filipe Atahide Fonseca,Eduardo Buozi Moffa

doi:10.1590/1807-3107bor-2022.vol36.0011

Abstract

Mucopolysaccharidosis (MPS) is a heterogeneous group of rare, chronic, progressive and systemic inherited disorders resulting from deficiency or lack of lysosomal enzymes responsible for the degradation of glycosaminoglycans. Products of nitrosative stress have been previously detected in blood and urine samples of patients with MPS. However, it is unclear whether they are present in the saliva of MPS patients and also if they correlate with salivary parameters such as flow and pH. This study compared the salivary levels of NOX (NO2- + NO3-), nitrite (NO2-), protein (albumin), erythrocyte and leukocyte numbers, as well as the salivary flow rate and pH values of samples obtained from 10 MPS patients and 10 healthy subjects. MPS patients exhibited higher salivary levels of NOX and NO2- when compared to healthy subjects (p < 0.05). Albumin was only detected in six saliva samples of MPS patients and, erythrocytes and leukocytes were detected in 60% and 40% of the MPS patients, respectively. In addition, salivary flow rate and pH averages were statistically lower in this group when compared to healthy samples (p < 0.05). Overall, the data indicates that the salivary levels of NO products can be used in combination with other heath indicators to monitor MPS disorders.

Highlights

Mucopolysaccharidosis (MPS) is a heterogeneous group of rare, chronic, progressive and systemic inherited disorders resulting from deficiency or lack of lysosomal enzymes responsible for the degradation of glycosaminoglycans (GAGs)
MPS patients and control groups were comparable in regards to numbers of decayed permanent and deciduous teeth (Table 1), the data shows that 100% of the patients of the control group did not have any type of decayed teeth, for the MPS group 33% of patients presented at least one or more permanent/deciduous decayed teeth
Median and IIQR values observed for visible plaque index was higher in the MPS group compared with the control group (52.7 (17.5–97.9) versus 0; p< 0.001)

Summary

Introduction

Mucopolysaccharidosis (MPS) is a heterogeneous group of rare, chronic, progressive and systemic inherited disorders resulting from deficiency or lack of lysosomal enzymes responsible for the degradation of glycosaminoglycans (GAGs). In MPS, GAG oligosaccharide accumulation occurs within the lysosomes of cells, inducing cell and tissue damage and dysfunction.[1] MPS is classified into seven groups (I, II, III, IV, VI, VII and IX) according with the type of enzyme deficiency involved in the disorder. This study investigates the amounts of (NO2- + NO3-), nitrite (NO2-), protein (albumin), erythrocytes, leukocytes, pH and flow rate in saliva samples from MPS patients and healthy subjects. Possible correlations between these salivary parameters and the clinical signs of MPS such asvisible plaque, gingival bleeding on probing.

Methodology

Results

Discussion