Aim: Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED) is a rare, highly variable disease with significant oral health impacts. The aim of the study is to examine the existing evidence base surrounding the oral health impacts of APECED and consider the wider clinical significance of the condition relevant to the provision of pediatric dental care. Materials and Methods: The evidence surrounding APECED and the oral health impacts obtained from a review of multiple databases was qualitatively summarized in the form of a literature review. A case study was used to illustrate the relevance of literature in caring patients with APECED. Results: The literature describes a broad range of impacts from APECED, although it is characterized by chronic candidal infection, autoimmune hypoparathyroidism, and Addison’s disease. Oral manifestations of APECED appear frequently and can present early. Developmental defects of the teeth affect a large proportion of patients with APECED and together with candidiasis, characterize the key oral manifestations of the disease. An enhanced preventive and minimally invasive approach to oral health care is recommended for these patients in light of the complexities of their medical condition. Conclusion: Patients with APECED require careful multidisciplinary care to ensure that the optimal oral health outcomes are achieved.

Purpose: The purpose of this study is to twofold: 1) to determine the age when a child with spina bifida (SB) will most likely transition from caregiver clean intermittent catheterization (CIC) to self-CIC; and 2) identify factors associated with self-CIC in children older than that age. Methods: This is a retrospective, single-institution cohort study of individuals with SB. Data were collected prospectively as part of the National Spina Bifida Patient Registry. For Aim 1, we identified all individuals who perform self-CIC and who had a documented transition from caregiver-CIC. We then determined the age of transition to self-CIC. For Aim 2, we compared individuals over age 10 years (age cutoff determined by Aim 1) who use self-CIC to those who use caregiver-CIC to determine what variables were associated with self-CIC. Results: From our SB population, 206 individuals used self-CIC. Of these, 64 patients had documented ages of transitions from caregiver- to self-CIC. 46 (71.9%) and 56 (87.5%) patients had transitioned to self-CIC by 10 and 14 years, respectively. For Aim 2, we used age 10 as a cutoff, based on the findings from Aim 1, and found that 287/696 patients were ≥10 years and using CIC. Factors independently associated with lower likelihood of self-CIC were thoracic spinal lesions (odds ratio (OR) 0.45) and Medicaid insurance (OR 0.24). Conclusions: The ages at self-CIC transition vary, but most patients transition by age 10. Thoracic-level spinal lesions and Medicaid insurance are associated with lower odds of self-CIC.

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