Analysis of salivary parameters of mucopolysaccharidosis individuals

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), 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 NO X and NO 2 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.

Current developments and opportunities of pluripotent stem cells-based therapies for salivary gland hypofunction

“…Saliva flow in children with Down syndrome is 36% lower than in healthy sibling pairs (Areias et al, 2012). In addition to these, a number of other genetic disorders have been reported to be associated with SGH or insufficient salivation, including cystic fibrosis (da Silva Modesto et al, 2015), familial amyloid polyneuropathy (Johansson et al, 1992), Fanconi anemia (Mattioli et al, 2010), Gosher's disease (Dayan et al, 2003), Papillon-Lefèvre syndrome (Lundgren et al, 1996), hemochromatosis (Rao et al, 2021), mucopolysaccharides (Nunes et al, 2022), and neurofibromatosis type 1 (Cunha et al, 2015).…”