RESUMEN
X-Linked Hypophosphatemia (XLH) is the most common cause of inherited hypophosphatemic rickets. Dental involvement, including spontaneous abscesses and/or fistulae, is an important part of the disease and has not been completely defined, especially in cohorts from developing countries. To describe oral health status in a cohort of Chilean patients with XLH and explore its correlation with biochemical presentation and treatment, we conducted a cross-sectional observational study of patients with PHEX mutation-confirmed XLH. All patients had an oral clinical exam, radiographic evaluation; clinical and biochemical data were obtained to determine their association with oral features. Twenty-six patients were included, 77% adults and 23% children. Most adults (89%) had past or current dental pulp pathology (abscesses and/or fistulae). Pulpal chamber enlargement and radiolucent apical lesions were common radiological features (94 and 74%, respectively). In children, abscess and/or fistulae were also common (33%). Caries index, which was determined by dmft/DMFT, was higher than the Chilean national average. Early and long-term therapy with phosphate and activated vitamin D was associated with lower carious index and attachment loss. XLH patients frequently present with high pulpal involvement and carious index. Conventional therapy was associated with lower carious index and attachment loss. These data highlight the importance of early and periodical dental care in order to prevent dental damage and assure a good quality of oral health for XLH patients.
Asunto(s)
Raquitismo Hipofosfatémico Familiar , Hipofosfatemia , Adulto , Niño , Estudios Transversales , Raquitismo Hipofosfatémico Familiar/complicaciones , Raquitismo Hipofosfatémico Familiar/epidemiología , Raquitismo Hipofosfatémico Familiar/genética , Factor-23 de Crecimiento de Fibroblastos , Humanos , Mutación , Salud Bucal , Endopeptidasa Neutra Reguladora de Fosfato PHEX/genética , FosfatosRESUMEN
Presenta un estudio retrospectivo sobre enfermedades pulmonares en el servicio de Neumología del Hospital "Abel Gilbert Pontón" en el año de 1996, en el que fueron tratados 47 casos; 5 de ellos corresponden a broncoquiectasia. El tratamiento se estableció de acuerdo al diagnóstico de la broncoquiestasia mediante la evolución clínica y estudios paraclínicos. En este estudio se clasificaron los casos de acuerdo a el sexo y efectos secundarios, como el diagnóstico diferencial de las enfermedades pulmonares.