Dental involvement in congenital chloride diarrhea

Dental involvement in a case of congenital chloride diarrhea (CCD) was studied using light microscopy of decalcified and undecalcified sections, microradiography and TEM. Mixed enamel lesions were found, involving gross hypoplasia at the cuspal level and hypocalcification occurring all over enamel. The hypocalcification resulted in the immature aspect of the prismatic enamel, porosities, amorphous areas, prominent incremental lines and round-ended crystallites. A marked hypocalcification suggests a temporary break of amelogenesis likely related to acute episodes of the disease. Globular calcifications were associated with enamel formation. Dentinal hypocalcification was conspicuous and diffuse all over dentine. A globular mineralization pattern was revealed by the tretracycline (TC) stain which was well preserved in decalcified sections, thus indicating that TC uptake is related to the organic matrix. It is unlikely that TC alone is responsible for the hypocalcified areas which extended widely beyond the TC-stained bands. It is suggested that TC uptake only adds to dental defects induced by the metabolic troubles of CCD.