Amparo Pérez1*, Clara Serna1, Antonio José Ortiz1 and Marcelo Bonecker2
1Department of Dentistry, Hospital Morales Meseguer, Spain
2Department of Pediatric Dentistry, Universidad de Sao Paulo, Spain
Niemann Pick disease is an autosomal recessive neurodegenerative disorder produced by an accumulation of intracellular cholesterol in lysosomes and endosomes. It manifests in middleto- late childhood and provokes ataxia, dysarthria, dysphagia (making oral nutrition difficult), hypotonia, spasticity, seizures and developmental abnormalities, which means that maintaining oral hygiene is difficult. A six-year-old girl diagnosed with Niemann Pick type C, currently treated with 2-hydroxypropyl-beta-cyclodextrin-intrathecal. Her oral health has been managed for 4 years applying the caries management by risk assessment (CAMBRA) preventive protocol. Through oral hygiene maintenance at home (good brushing technique, and 0.12% chlorhexidine mouthwash) and three-monthly plaque check-ups and fluoride varnish applications, the child has remained free of caries. From the outset, treatment has included psychological management with the help of the patient’s mother. The use of preventative protocols makes it possible to manage the oral health of children with Niemann Pick disease. The help and participation of the family are essential to the oral health and psychological management of children with this disease.
Pérez A, Serna C, Ortiz AJ, Bonecker M. Importance of Early Dental Care in a Child with Niemann-Pick Disease, Type C. Ann Clin Case Rep. 2018; 3: 1493.