The Effect of Multiple Sulfatase Deficiency (MSD) on Dental Development: Can We Use the Teeth as an Early Diagnostic Tool?

ABSTRACT:

Background

Multiple sulfatase deficiency (MSD) is a rare autosomal recessive inborn error of metabolism due to reduced catalytic activity of the different sulfatase. Affected individuals show neurologic deterioration with mental retardation, skeletal anomalies, organomegaly, and skin changes as in X-linked ichthyosis. The only organ that was not examined in MSD patients is the dentition.

Objectives

To evaluate the effect of the metabolic error on dental development in a patient with the intermediate severe late-infantile form of MSD (S155P).

Methods

Histological and chemical study were performed on three deciduous and five permanent teeth from MSD patient and pair-matched normal patients.

Results

Tooth germ size and enamel thickness were reduced in both deciduo

SUBMITTER: Zilberman U 

PROVIDER: S-EPMC5110438 | biostudies-literature | 2016

REPOSITORIES: biostudies-literature