Updated consensus guidelines on the management of Phelan-McDermid syndrome.

Phelan-McDermid syndrome (PMS) is a genetic condition caused by SHANK3 haploinsufficiency and characterized by a wide range of neurodevelopmental and systemic manifestations. The first practice parameters for assessment and monitoring in individuals with PMS were published in 2014; recently, knowledge about PMS has grown significantly based on data from longitudinal phenotyping studies and large-scale genotype-phenotype investigations. The objective of these updated clinical management guidelines was to: (1) reflect the latest in knowledge in PMS and (2) provide guidance for clinicians, researchers, and the general community. A taskforce was established with clinical experts in PMS and representatives from the parent community. Experts joined subgroups based on their areas of specialty, including genetics, neurology, neurodevelopment, gastroenterology, primary care, physiatry, nephrology, endocrinology, cardiology, gynecology, and dentistry. Taskforce members convened regularly between 2021 and 2022 and produced specialty-specific guidelines based on iterative feedback and discussion. Taskforce leaders then established consensus within their respective specialty group and harmonized the guidelines. The knowledge gained over the past decade allows for improved guidelines to assess and monitor individuals with PMS. Since there is limited evidence specific to PMS, intervention mostly follows general guidelines for treating individuals with developmental disorders. Significant evidence has been amassed to guide the management of comorbid neuropsychiatric conditions in PMS, albeit mainly from caregiver report and the experience of clinical experts. These updated consensus guidelines on the management of PMS represent an advance for the field and will improve care in the community. Several areas for future research are also highlighted and will contribute to subsequent updates with more refined and specific recommendations as new knowledge accumulates.

Child Life Interventions for Pediatric Dental Patients: A Pilot Study.

Purpose: The purpose of this pilot study was to assess whether child life intervention can be an effective alternative to pharmacologic behavior management in uncooperative pediatric dental patients. Methods: Thirty uncooperative four- to eight-year-old patients with no history of a negative invasive dental experience were randomly assigned into two groups: experimental (E) and control (C). Group E was given two 30- minute child life interventions (CLIs) by a certified child life specialist. Group C did not receive CLIs. Both groups then had an invasive restorative dental appointment, which was video recorded, edited, and viewed to assess behavior via the Houpt scale. Results: Group E demonstrated overall better cooperation for the appointment (Group C equals 3.63, and group E equals 4.07.) Conclusions: Child life interventions may be considered an adjunct to other behavior guidance techniques, but further investigations should be conducted to evaluate the effectiveness of CLIs on behavior in the dental setting.

Dental Impacts on Health-related Quality of Life of Children with Orofacial Clefts.

PURPOSE: Children with orofacial clefts experience risks for psychosocial and behavioral problems, making it important to evaluate for negative impacts on health-related quality of life. The purpose of this study was to evaluate health-related quality of life (HRQOL) and surgical history to determine if these measures correlated with caries and/or behavior during dental treatment. METHODS: Data for this retrospective study were obtained for children encountered between March 2011 and August 2013. Inclusion criteria were four- to 10-year-olds with a non-syndromic orofacial cleft diagnosis. Parent-reported HRQOL data were obtained for 79 children and child-reported data for a subgroup of 23. Pediatric Quality Of Life Inventory (PedsQL) scores, surgeries, and demographics were collected from craniofacial team medical charts. Decayed, missing, and filled teeth (dmft) and behavior (Frankl) scores were collected from dental records. RESULTS: Significant correlations were found between total parent-reported sample PedsQL scores and dmft scores, and between the subgroup's PedsQL scores and Frankl scores. CONCLUSIONS: For children with orofacial clefts, higher caries was associated with lower health-related quality of life in the total parent-reported sample. Negative behavior was associated with lower HRQOL in eight- to 10-year-olds, who also more accurately reported psychosocial risks for negative behavior than parents by proxy. Surgeries were not associated with caries or behavior.

Relative value of oral health in well-child care: a survey of pediatricians in Texas and Ohio.

PURPOSE: The purpose of this study was to assess knowledge, penetration, and support of the American Academy of Pediatrics' (AAP) 2003 oral health policy statement among pediatricians and compare the perceived relative value of oral health among health topics. METHODS: A 33-question survey was sent to 1,320 randomly selected pediatricians in Texas and Ohio. The survey queried practice patterns, perceived importance of oral health, and familiarity/implementation of the AAP policy. RESULTS: The return rate was 30 percent, with a 23 percent useable response rate; 51 percent of the pediatricians had read the policy, and 74 percent were implementing some component. Overall, 40 percent referred one-year-old infants to a dentist and 29 percent believed it was important. Parental acceptance was identified to be the most common barrier for dental referral by one year of age. Oral health anticipatory guidance ranked fifth at the six- and 12-month office visits but ranked second at the 24-month visit. CONCLUSION: While three out of four pediatricians were implementing some portion of the AAP's 2003 oral health policy, referral rates and perceived importance of oral health remained low among pediatricians.

Characteristics of dental clinics in US children's hospitals.

PURPOSE: This study's purpose was to describe the workforce, patient, and service characteristics of dental clinics affiliated with US children's hospitals belonging to the National Association of Children's Hospital and Related Institutions (NACHRI). METHODS: A 2-stage survey mechanism using ad hoc questionnaires sought responses from hospital administrators and dental clinic administrators. Questionnaires asked about: (1) clinic purpose; (2) workforce; (3) patient population; (4) dental services provided; (5) community professional relations; and (5) relationships with medical services. RESULTS: Of the 222 NACHRI-affiliated hospitals, 87 reported comprehensive dental clinics (CDCs) and 64 (74%) of CDCs provided data. Provision of tertiary medical services was significantly related to presence of a CDC. Most CDCs were clustered east of the Mississippi River. Size, workload, and patient characteristics were variable across CDCs. Most were not profitable. Medical diagnosis was the primary criterion for eligibility, with all but 1 clinic treating special needs children. Most clinics (74%) had dental residencies. Over 75% reported providing dental care prior to major medical care (cardiac, oncology, transplantation), but follow-up care was variable. CONCLUSIONS: Many children's hospitals reported comprehensive dental clinics, but the characteristics were highly variable, suggesting this element of the pediatric oral health care safety net may be fragile.

Measuring persistent temporomandibular joint nociception in rats and two mice strains.

Temporomandibular joint (TMJ) pain has been reported to last for prolonged periods in humans. In rodents a variety of methods have been used to measure TMJ nociception, but for most of these methods the period of measurement has been minutes to a couple of hours. In addition, most measurement protocols required restraint or training of the animal. Previous studies from our laboratory demonstrated that feeding behavior, particularly meal duration, was an indicator of TMJ nociception in unrestrained and untrained male and female Sprague-Dawley rats for up to two days. In this study, we first found that injection of complete Freund's adjuvant (CFA) into the TMJ of rats significantly lengthened meal duration for 19 days and also decreased meal frequency for 42 days. Interestingly, the meal duration varied significantly from day to day within the 19 day period. TMJ interleukin-1 beta (IL-1 beta) and calcitonin gene-related peptide (CGRP) were significantly elevated in the TMJ tissues of CFA-injected animals and the level of these markers was attenuated as the meal duration decreased with time. Control animals injected with saline into the TMJ or CFA into the knee did not show a significant lengthening in meal duration but did show a decrease in meal frequency. In a second study, DBA/1LacJ mice given TMJ CFA injections showed a significantly lengthened meal duration on four of the seven days measured using end-of-the meal definition of 5 or 10 min. No other meal pattern changed significantly. Two days post-CFA injection, the DBA/1LacJ mice showed significantly elevated interleukin-6 (IL-6), but not elevated IL-1 beta. Seven days post-injection, both IL-6 and IL-1 beta were significantly elevated. No change in CGRP was detected. In this study C57Bl/6 mice also received TMJ CFA injections, but they did not show a lengthening in any meal pattern or significant increases in IL-1 beta, IL-6 or CGRP. Our data show, for the first time, that meal duration can be used to measure CFA-induced nociception in the TMJ over the course of several weeks in unrestrained rats and for up to seven days in the DBA/1LacJ mouse strain. In addition, C57Bl/6 mice are resistant to CFA-induced TMJ nociception at the same dose used in the DBA/1LacJ mice.

Preventive resin restorations: practice and billing patterns of pediatric dentists.

PURPOSE: The purpose of this study was to determine practice patterns of pediatric dentists for preventive resin restorations (PRRs) and if they believe a code should be added to the American Dental Association's current dental terminology (CDT) for the PRR. METHODS: A 16-question survey sent to 475 pediatric dentists randomly selected from the American Academy of Pediatric Dentistry database, addressed demographics, treatment planning, techniques in preparation and restoration, billing practices, and perceptions about the need for a CDT code for PRRs. RESULTS: Two hundred thirty-eight (50%) surveys were returned, revealing that 72% of respondents perform PRRs and 64% feel that a PRR code should be added to the CDT Fifty-two percent believe not having a CDT code could cause dentists to perform more invasive dentistry to comply with billing requirements that Class I restorations be in dentin. PRRs are commonly treatment planned for deep pits and fissures with questionable decoy not entering dentin. Up to 50% of respondents could be erroneously billing for PRRs. CONCLUSIONS: Most pediatric dentists perform preventive resin restorations in their office and believe that a code for the procedure needs to be added to the current dental terminology.

Caries risk assessment practices among Texas pediatric dentists.

PURPOSE: The purpose of this study was to determine current caries risk assessment (CRA) practices of Texas pediatric dentists. METHODS: A 20-question survey was sent to all 204 active members of the Texas Academy of Pediatric Dentistry. The mailing list was obtained from the Texas Academy of Pediatric Dentistry roster. RESULTS: The response rate was 62% (127/204). Eighty-three percent of respondents reported that parents are receptive to education about caries risk factors. Socioeconomic status was unrelated to receptivity. Ninety-three percent of respondents reported that they are actively involved in CRA. Thirty-eight percent of those responding reported performing CRAs on greater than 76% of their patients; 9% reported no CRA use. Forty percent of respondents reported that they assess caries risk but do not document the caries risk status. Seventy percent of respondents reported a desire for more CRA education. No significant differences were found between: (1) year o fgraduation; (2) practice type; or (3) payer sources received and CRA practices. CONCLUSIONS: A need exists for more comprehensive caries risk assessment practices and increased documentation of caries risk status by Texas pediatric dentists. The continuing education of dental professionals in caries risk assessment is essential.

The critical incident technique: a useful tool for conducting qualitative research.

The critical incident technique (CIT) is a well-established qualitative research tool used in many areas of the health sciences, including nursing, medicine, and dentistry, and their respective education systems. It is a flexible set of principles that can be modified and adapted to meet the specific situation at hand. By gathering factual reports made by observers, researchers can build a picture of the situation under study. The CIT maximizes the positive and minimizes the negative attributes of anecdotes, effectively turning anecdotes into data. In this, the first of two companion articles, the origins and current state of the CIT and its potential applications in dentistry and dental education are described.

The critical incident technique and pediatric dentistry: a worked example.

Evaluating dental students' experiences in pediatric dentistry may help dental educators better prepare graduates to treat the children in their communities. This qualitative investigation aimed to collect and analyze data using the critical incident technique (CIT). Sixty dental students at one southwestern dental school participated in 103 recorded interviews. They described 150 positive and 134 negative experiences related to the pediatric dentistry clinic. Analysis of the data resulted in the identification of four key factors related to their experiences: 1) the instructor; 2) the patient; 3) the learning process; and 4) the learning environment. The contribution made by the patient to dental students' education has not been previously addressed. The CIT is a useful data collection and analysis technique that provides rich, useful data and has many potential uses in dental education.

Capsaicin sensitive neurons role in the inflamed TMJ acute nociceptive response of female and male rats.

Computerized meal pattern analysis, and more specifically meal duration, has recently been used as a non-invasive biological marker of nociception in the temporomandibular joint (TMJ). Cells responsible for the nociceptive response in the inflamed TMJ may include capsaicin (CAP) sensitive neurons. To test the role of CAP sensitive neurons in acute nociceptive responses first, male and female rats were treated neonatally with vehicle or CAP, an agent known to destroy a majority of C fibers. Second, after 56 days the rats were divided into four groups: neonatal vehicle-injected and treated with and without complete Freund's adjuvant (CFA). Treatment groups included neonatal non-CAP vehicle treated and TMJ not-injected (CON); vehicle treated and TMJ CFA injected (CFA); CAP-treated and not-injected (CAP); and CAP-treated and CFA injected (CAP+CFA). Meal patterns were analyzed for two days after injection. CFA-injection in non-CAP-treated rats lengthened meal duration on the first and second day after treatment in the males, but only on the first day in the females. CAP treatment in male and female rats prevented significant lengthening of meal duration induced by CFA. CAP treatment attenuated the CFA-induced increase in calcitonin gene-related peptide expression in the trigeminal ganglia similarly in males and females. The data suggests CAP-sensitive neurons are responsible, in part, for transmission of acute nociceptive responses associated with CFA administration and suggest gender can affect nociception in the inflamed TMJ region.

Pharmacologic behavior management of pediatric dental patients diagnosed with attention deficit disorder/attention deficit hyperactivity disorder.

PURPOSE: The purpose of this study was to conduct a survey of Texas pediatric dentists to determine: (1) the percentage of patients they treat with attention deficit disorder (ADD)/attention deficit hyperactivity disorder (ADHD); (2) the behavior management techniques that are utilized to treat their patients who suffer from ADD/ADHD; and (3) the relative success rates of these techniques in their practices. METHODS: A 17-question, single-answer, multiple choice survey was mailed to 343 Texas pediatric dentists. The mailing list was obtained from American Academy of Pediatric Dentistry and Texas Academy of Pediatric Dentistry member rosters. One mailing was sent, including a self-addressed stomped envelope, for returned responses. RESULTS: A 54% response rate (186 surveys) revealed that nitrous oxide was the most frequently used pharmacologic behavior management technique; however, demerol/promethazine/nitrous oxide was rated as effective most often for treating ADD/ADHD patients. CONCLUSIONS: Practitioners believe the incidence of attention deficit disorder/attention deficit hyperactivity disorder is increasing, and they are familiar with the medications used to treat the conditions. Texas pediatric dentists are using a variety of sedation techniques and are interested in developing guidelines for sedation of these patients.