Paciente con Síndrome Lesch-Nyhan atendido en el Departamento de Estomatología Pediátrica del Hospital Infantil de Tamaulipas. Reporte de un caso.

Abstract

The Lesch-Nyhan syndrome is a disease of hereditary origin. It is only presented in men and is characterized by the total deficiency of the hipoxantine guanine phosphoribosyl transferase enzyme. This enzyme does not allow the conversion of hipoxantine to inosine, so the uric acid level reaches levels higher than normal causing the uric acid to crosse the haemato encephalic barrier in the newborn due to its immaturity, resulting the disorders observed in this syndrome such as mental delay and behavior disorders, being the aggressiveness the main characteristic. This case describes a 3-year-old male patient diagnosed with Lesch-Nyhan syndrome, he attended the Odontopediatrics Department of the Children Hospital in Tamaulipas due to constant lesions self inflicted by bites in tongue, lips, cheeks and fingers. Extractions of the infantile teeth were indicated in the operating room and the problem was eliminated, but it was present again with the eruption of the permanent teeth. The case is described in detail in this article.