A new indicator of the oral hygiene habits of disabled persons: relevance of the carer's personal appearance and interest in oral health.

OBJECTIVE: To investigate whether there is a relationship between the oral hygiene habits of individuals with severe disability the carer's personal appearance and interest in oral health. PATIENTS AND METHODS: The study group was formed of 60 disabled persons and their respective carers who came for the first time to consultation in the Special-Needs Dentistry Unit of the University of Santiago de Compostela, Spain. All the carers answered a standardised questionnaire of 28 questions divided into four sections: disabled individual's demographic data, disabled individual's general medical details, social aspects of the carer (personal appearance of the carer and interest in oral health), and disabled individual's oral hygiene habits. The personal appearance of the carers and their interest in the disabled individual's oral health were evaluated using independent scales designed specifically for the study, with five binary items in each scale. RESULTS: The carer's personal appearance and interest in the disabled individual's oral health showed a statistically significant relationship with the individual's oral hygiene habits, particularly with respect to the frequency and duration of toothbrushing, need for physical restraint during toothbrushing, use of a manual toothbrush and use of toothpaste. CONCLUSIONS: The carer's personal appearance and interest in the disabled individual's oral health are good indicators of the oral hygiene habits of an individual with severe disability. Consideration should be given to the inclusion of these aspects as a complementary element of the dental record.

A novel mutation in the OFD1 (Cxorf5) gene may contribute to oral phenotype in patients with oral-facial-digital syndrome type 1.

BACKGROUND: Oral-facial-digital syndrome (OFDS) type 1 (OFD1) is an X-linked dominant condition associated with embryonic male lethality. It almost always affects the oral cavity, face, and digits. It is considered to be a ciliopathy caused by mutations in the OFD1 gene. A variety of mutations have been described, and a genotype-phenotype correlation has been suggested. OBJECTIVE AND METHODS: The proband was an 8-year-old Spanish girl with suspected OFD1. We extended the pedigree to three proband's generations, performing a thorough physical examination and screening for OFD1 mutations in nine individuals. RESULTS: The proband, her mother, and her sister showed oral findings consistent with OFD1. Ultrasound evaluation revealed the existence of renal cysts only in the proband's mother. The rest of the family (all male) had no relevant morphological abnormalities. A single-base deletion in exon 16 of OFD1 (c.2183delG) leading to a frameshift was detected in the proband, her mother, and her sister. CONCLUSION: Because all three women had a similar oral phenotype, this new mutation might be involved in the development of the OFD1 oral manifestations. In cases of OFDS, physical examination (including the oral cavity and renal function) and genetic screening of the probands and their relatives are mandatory.

Case report of segmental odontomaxillary dysplasia with cutaneous manifestations.

BACKGROUND: Segmental odontomaxillary dysplasia is an uncommon nonhereditary growth disorder that affects the maxilla, gums and ipsilateral dentition. The disorder is diagnosed mainly based on dental (over-retention of primary teeth, dental agenesis and diastemas) and bone findings (bone sclerosis, irregular trabeculation of immature bone and reduced maxillary sinus). This paper provides a case report. CASE REPORT: A 5-year-old child with skin manifestations including hypertrichosis, facial erythema and pigmented nevus was diagnosed with type II segmental odontomaxillary dysplasia based on clinical, radiographic and histopathological analysis. CONCLUSION: The skin findings can help with the suspicion of segmental odontomaxillary dysplasia, although the definitive diagnosis is typically established by a paediatric dentist based on clinical and radiological findings.

Oral bisphosphonate-related osteonecrosis of the jaws in dental implant patients: a case series.

Bisphosphonates have been associated with a serious adverse reaction known as bisphosphonate-related osteonecrosis of the jaws (BRONJ). The aim of this study was to describe its clinical characteristics in patients with dental implants who were taking bisphosphonates orally. We made a retrospective multicentre study in 3 hospitals in Galicia, Spain. The medical records and clinical and radiological follow-up of the oral cavity were reviewed for those patients given bisphosphonates and diagnosed with BRONJ after the placement of dental implants within the previous 3 years. The series comprised 9 white patients (mean age 66 years). The bisphosphonates were alendronate (n=6), ibandronate (n=2), and risedronate (n=1), and the most common indication was osteoporosis (n=7). The mean interval between the initiation of treatment and the onset of BRONJ lesions was 60 months. Most of the lesions were located around the mandibular implants (n=8). The mean interval between placement of dental implants and the onset of BRONJ was 34 (range 1-96) months. After treatment 7/9 patients recovered completely. The prevalence of BRONJ secondary to treatment with bisphosphonates taken orally after placement of dental implants may be higher than expected in a particular geographical region, but to date specific risk factors have not been identified. Clinical characteristics and the outcomes of treatment of lesions are similar to those seen in patients with BRONJ that is unrelated to placement of dental implants.

Is oral bisphosphonate-related osteonecrosis of the jaw an endemic condition?

Since the introduction of bisphosphonates to treat diseases that affect bone remodelling, there has been an increasing number of cases of bisphosphonate (BP)-related osteonecrosis of the jaw (BRONJ). Epidemiological data regarding BRONJ vary widely between studies, and a number of potential methodological biases have been detected. In some small preliminary studies, single nucleotide polymorphisms associated with an increased risk of BRONJ among cancer patients have been identified. However, genetic susceptibility to oral BP-related BRONJ has not previously been discussed. We suggest that epidemiological variability could be related to the existence of a susceptibility factor particularly prevalent in the population of a well-defined geographical region. To support our hypothesis we performed a search for published case series, only including those with at least 10 patients detected in a single city or a delimited geographical region; this showed that 55% of reports came from Mediterranean countries such as Italy, Israel, Spain and France. The finding does not appear to be conditioned by publication bias. Furthermore, conditions such as classic Kaposi sarcoma and beta-thalassaemia, though not exclusive to the Mediterranean region, also have a high prevalence in that area. We speculate that some of the patients included in the selected US and Australian series may be of Spanish or Italian descent. With an ageing population, the prevalence of osteoporosis will increase, and the number of cases of oral BP-related BRONJ may rise exponentially. Identification of risk groups with susceptibility to BRONJ will arise caution when prescribing BPs and will allow new preventive and therapeutic strategies to be developed.

Osseointegrated dental implants in patients with intellectual disability: a pilot study.

PURPOSE: The objective of this study was to investigate if intellectual disability (ID) itself constitutes an absolute contraindication to oral rehabilitation with osseointegrated implants, or if adequately selected patients can benefit from this dental treatment technique. METHOD: We report a series of eight patients with non-syndromic ID and no associated neuromuscular disorders, craniofacial abnormalities, or serious systemic complications, in whom oral rehabilitation was performed using implant-supported prostheses, with a follow-up of one to three years. RESULTS: A total of 18 titanium implants were inserted and nine implant-supported prostheses were constructed. Follow-up examination showed that although the majority of implants presented a degree of peri-implant mucositis, all were osseointegrated and the prostheses were functional. CONCLUSIONS: Although there is very little literature on this subject, the results of this pilot study allow us to suggest that osseointegrated oral implants could constitute a therapeutic option for patients with ID. The success of oral rehabilitation depends fundamentally on appropriate patient selection. IMPLICATIONS FOR REHABILITATION: • Apart from the obvious difficulties related to eating and communication, edentulism is of great cosmetic importance in patients with ID in terms of social acceptance. • It has been suggested that patients with ID do not receive the same level of dental treatment as the general population. • The results of this pilot study allow us to suggest that osseointegrated oral implants could constitute a therapeutic option for patients with ID.

Correlation between dental maturation and chronological age in patients with cerebral palsy, mental retardation, and Down syndrome.

Determining a child's chronological age and stage of maturation is particularly important in fields such as paediatrics, orthopaedics, and orthodontics, as well as in forensic and anthropological studies. Some systemic conditions can cause abnormal physiological maturation, and skeletal maturation is usually more delayed than dental maturation. The aim of this study was to determine dental age in a group of patients with the most prevalent congenital or perinatally occurring physical and mental disabilities. The study group comprised 155 white Spanish children aged 3-17 years (35 with cerebral palsy, 83 with mental retardation and no associated syndromes or systemic conditions, and 37 with Down syndrome). The dental maturation indices described by Nolla and Demirjian were used to generate regression lines for the dental age of individuals in a control group (688 white Spanish children aged 3-17 years) and the formulae were then used to determine the dental age of patients in the study group. No significant differences were found between dental and chronological age in boys with cerebral palsy, mental retardation, or Down syndrome. In contrast, dental age (calculated from the linear regression model that included values for the Demirjian index) was significantly delayed compared with chronological age in girls with cerebral palsy or Down syndrome.