Fibrous Dysplasia / McCune Albright syndrome (FD/MAS) represents a wide spectrum of diseases due to somatic gain-of-function mutations of the GNAS gene. The mutation leads to overactivity in the target tissues and to a wide phenotype of clinical features that vary in severity and age of onset. The rarity of the disease and its variable presentation to multiple specialities often leads to misdiagnosis and inappropriate variability in investigations and treatments. To address this, our international consortium of clinicians, researchers, and patients’ advocates has developed pragmatic clinical guidelines for best clinical practice for the definition, diagnosis, staging, treatment and monitoring for FD/MAS to empower patients and support clinical teams in both general and specialised healthcare settings. With the lack of strong evidence to inform care, the guidelines were developed based on review of published literature, long-standing extensive experience of authors, input from other healthcare professionals involved in the care of FD/MAS patients and feedback from patients and patient groups across the globe. This has led to the formulation of a set of statements to inform healthcare professionals, patients, their families, carers and patient groups of the best practice of care. It is anticipated the implementation of these recommendations will lead to improvement in the care of patients with FD/MAS internationally. Electronic supplementary material The online version of this article (10.1186/s13023-019-1102-9) contains supplementary material, which is available to authorized users.

PBM is a safe, feasible, and effective treatment for children affected by chemotherapy-induced OM, as it accelerates mucosal recovery and reduces pain.

Objective.To propose multidisciplinary, consensus-based, standardization of operational terminology and method of assessment for temporomandibular joint (TMJ) involvement in juvenile idiopathic arthritis (JIA).Methods.Using a sequential expert group–defined terminology and methods-of-assessment approach by (1) establishment of task force, (2) item generation, (3) working group consensus, (4) external expert content validity testing, and (5) multidisciplinary group of experts final Delphi survey consensus.Results.Seven standardized operational terms were defined: TMJ arthritis, TMJ involvement, TMJ arthritis management, dentofacial deformity, TMJ deformity, TMJ symptoms, and TMJ dysfunction.Conclusion.Definition of 7 operational standardized terms provides an optimal platform for communication across healthcare providers involved in JIA-TMJ arthritis management.

Dental trauma is a frequent occurrence in children and adolescent and a correct diagnosis and treatment are essential for a favourable long-term prognosis. The present Guidelines aim to formulate evidence-based recommendations to assist dentists, paediatricians, surgeons, teachers, school and sport staff, parents in the prevention and first aid of dental trauma in children and to provide a careful assessment of the medico-legal implications, reviewing the first draft of the guidelines published in 2012. A multidisciplinary panel on the behalf of the Italian Ministry of Health and in collaboration with the WHO Collaborating Centre for Epidemiology and Community Dentistry of Milan, developed this document. The following four queries were postulated: 1) Which kind of precautions the health personnel, parents, sports and educational personnel must activate in order to prevent the dental trauma damage? 2) How an orofacial trauma in paediatric patients should be managed either in the Emergency Care Unit and/or in private dental office? 3) What criteria should be adopted by a dentist private practitioner to fill in a certificate in cases of dental and/or tempomandibular joint trauma occurring in children and adolescents? 4) What are the elements that should lead clinicians to suspect a non-accidental dental trauma? A systematic review and analysis of the scientific literature published in English, Italian and French from 2007 to 2017 regarding dental trauma in children and adolescents aged 0–18 years was performed, and about 100 papers were analysed and included. The following four domains were analysed and discussed: Dental Trauma Prevention Strategies and Health Education, First aid in orofacial and dental trauma, Certificate of the dental trauma, Oral and dental signs of child abuse and neglect. Twenty-eight recommendations were draw up and codified by the panel according to the Methodological handbook, produced by the Istituto Superiore di Sanità, in order to guide physicians in the prevention and first aid of dental trauma in children and adolescents. In addition, a careful assessment of the medico-legal implications is reported in this document.

Involvement of the temporomandibular joint (TMJ) is common in juvenile idiopathic arthritis (JIA). TMJ arthritis can lead to orofacial symptoms, orofacial dysfunction, and dentofacial deformity with negative impact on quality of life. Management involves interdisciplinary collaboration. No current recommendations exist to guide clinical management. We undertook this study to develop consensus-based interdisciplinary recommendations for management of orofacial manifestations of JIA, and to create a future research agenda related to management of TMJ arthritis in children with JIA. Recommendations were developed using online surveying of relevant stakeholders, systematic literature review, evidence-informed generation of recommendations during 2 consensus meetings, and Delphi study iterations involving external experts. The process included disciplines involved in the care of orofacial manifestations of JIA: pediatric rheumatology, radiology, orthodontics, oral and maxillofacial surgery, orofacial pain specialists, and pediatric dentistry. Recommendations were accepted if agreement was >80% during a final Delphi study. Three overarching management principles and 12 recommendations for interdisciplinary management of orofacial manifestations of JIA were outlined. The 12 recommendations pertained to diagnosis (n = 4), treatment of TMJ arthritis (active TMJ inflammation) (n = 2), treatment of TMJ dysfunction and symptoms (n = 3), treatment of arthritis-related dentofacial deformity (n = 2), and other aspects related to JIA (n = 1). Additionally, a future interdisciplinary research agenda was developed. These are the first interdisciplinary recommendations to guide clinical management of TMJ JIA. The 3 overarching principles and 12 recommendations fill an important gap in current clinical practice. They emphasize the importance of an interdisciplinary approach to diagnosis and management of orofacial manifestations of JIA.

Oral mucositis (OM) is a debilitating and serious side effect in patients undergoing hematopoietic stem cell transplantation (HSCT) and chemotherapy (CT). Laser therapy is becoming a promising treatment option in these patients, avoiding the necessity of enteral/parenteral nutrition. The aim of this study was to evaluate the efficacy of laser therapy in patients affected by oral mucositis induced by chemotherapy and HSCT. Sixteen onco-hematological pediatric patients receiving chemotherapy and hematopoietic stem cell transplantation, affected by oral mucositis, were enrolled in this study. They were divided in two randomized groups: the laser group and the placebo-control group. Patients in the laser group were treated with HPLT (970 ± 15 nm, 3.2 W (50%), 35-6000 Hz, 240 s) for four consecutive days, once a day; and placebo group underwent sham treatment. The assessment of mucositis was recorded through WHO Oral Mucositis Grading Objective Scale, and pain was evaluated through Visual Analogue Scale (VAS). Patients were monitored and evaluated 3, 7, and 11 days after the first day of laser therapy. Once OM was diagnosed, the patients had mucositis grading assessments before laser or sham application at day 3, 7, and 11 after first application. All patients of laser group demonstrated improvement in pain sensation from day 3 after first application of laser (p < 0.05), ulcerations reduced their dimensions and erythema disappeared. The patients of placebo group had improvement from day 7. In laser group, all mucositis were fully resolved from day 7 (p < 0.05). Oral mucositis negatively impacts on nutritional intake, oral hygiene, and quality of life. Laser therapy appears to be a safe and innovative approach in the management of oral mucositis. In this preliminary study, HPLT encourages to consider laser therapy as a part of onco-hematological protocol, providing to decrease pain and duration of OM induced by CT and HSCT. Further researches will be needed, especially randomized, controlled clinical trials with a large number of enrolled patients and a long term of follow-up to confirm the efficacy of laser therapy in prevention and control of OM in onco-hematological pediatric patients.

Standard, professional oral hygiene procedures and non-surgical periodontal therapy including antimicrobials were associated with a marked improvement of clinical and patient-related outcomes in pediatric cases of PCG.

It is generally accepted that salivary components are important for dental health, but to date no clear correlation has been found between one or more of these components and the outcome of dental caries. The identification of salivary factors preventing, favoring or signaling dental caries might help to control the disease. In the present study, western blotting analysis of whole saliva from 20 healthy caries-free children showed the presence of the soluble form of CD14, a bacterial pattern-recognition receptor for many bacterial components that is involved in the innate immune response. The identity of the protein was confirmed through N-terminal sequencing by Edman degradation, and by partial sequencing with mass spectrometry of tryptic peptides. Conversely, CD14 was completely absent in the saliva of 20 age-matched patients affected by two to eight carious lesions, but appeared in their saliva a few weeks after dental restoration. These results suggest that the absence of salivary soluble CD14 could represent an useful index of caries activity, and might be used to detect early carious lesions not visible by oral inspection.