Postoperative chronic pain in arthroplasty papillon-lefèvre syndrome: 17-year dental follow-up: case report / Síndrome de papillon-lefèvre: seguimiento dental de 17 años: reporte de caso

Introduction: The present report describes the case of a 12-year-old patient with 17-year follow-up who was previously diagnosed with Papillon-Lefèvre Syndrome (PLS), which is a rare autosomal recessive irregularity in the cathepsin C gene (CTSC) characterized by palmoplantar hyperkeratosis and premature loss of primary and permanent teeth. Case Report: A specific mutation in the c.203 T > G gene inducing loss of function leading to PLS was detected, as was a mutation in the HLA-DRB1*11 allele, which is associated with this syndrome. There is no consanguinity of the parents, and the siblings are entirely healthy. Early identification of the main characteristics of this syndrome is imperative. Accurate diagnosis by genetic analysis allows differential diagnoses and timely comprehensive dental treatment. Conclusions: Additionally, it allows consultation with a dermatologist to maintain or improve the quality of life of patients with this condition due to progressive worsening and severity of the main physical manifestations. Keywords: Papillon-Lefevre Disease; Keratoderma, Palmo-plantar; Cathepsin C; Periodontitis; Skin Diseases, Genetic; Case reports

Introducción: El presente reporte describe el caso de un paciente de 12 años de edad con 17 años de seguimiento a quien previamente se le diagnosticó Síndrome de Papillon-Lefèvre (PLS), el cual es una rara irregularidad autosómica recesiva en el gen de la catepsina C (CTSC) caracterizada por hiperqueratosis palmoplantar y pérdida prematura de dientes primarios y permanentes. Reporte de Caso: Se detectó una mutación específica en el gen c.203 T > G que induce pérdida de función que conduce a PLS, así como una mutación en el alelo HLA-DRB1*11, que se asocia a este síndrome. No presenta consanguinidad de los padres, padres y hermanos totalmente sanos. La identificación temprana de las principales características de este síndrome es imperativa. El diagnóstico certero por análisis genético permite diagnósticos diferenciales y tratamientos odontológicos integrales oportunos. Conclusiones: Adicionalmente, permite la consulta con un dermatólogo para mantener o mejorar la calidad de vida de los pacientes con esta condición debido al progresivo empeoramiento y severidad de las principales manifestaciones físicas.

Root morphology of lateral incisors adjacent to impacted maxillary canines: A cone-beam computed tomography retrospective cross-sectional study.

INTRODUCTION: To compare the root morphology of the maxillary lateral incisors (MLIs) adjacent to an impacted (IMC) versus non-impacted maxillary canine (non-IMC). MATERIALS AND METHODS: This cross-sectional study included cone beam computed tomography (CBCT) images derived from 154 subjects (with unilateral/bilateral impacted maxillary canines or without canine impaction). Each MLI was considered as a unit of analysis. Thus, the study group included 122 MLIs adjacent to impacted maxillary canine (IMCG) and the control group included 122 MLIs adjacent to a non-impacted maxillary canine (CG). Groups were matched by age, sex and evaluation side. Root length, root dilaceration angle and root convergence angle were evaluated in both the coronal and sagittal planes. Comparisons between groups were made with Student's t-test and the chi-square test with Bonferroni correction (P<0.008, P<0.01, respectively). RESULTS: The root measurements of the MLIs showed some significant differences between the groups. The coronal root length was 0.52mm shorter but not highly significant (P=0.032), the coronal root dilaceration angle was 7.51° smaller (P<0.001), and the coronal root convergence angle was 1.28° greater (P<0.001) in the IMCG compared to the CG. The orientation of root dilaceration in the MLIs showed significant differences between the groups: it was mostly distopalatal in the GC (87.5%), to a lesser extent in the IMCG (58.9%) and also mesiopalatal in the IMCG (22.3%), (P=0.001). CONCLUSIONS: The root morphology of MLIs adjacent to an IMC showed a tendency of shorter length, smaller root dilaceration and greater root convergence in the coronal section than MLIs adjacent to a non-IMC.