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1.
BMC Surg ; 20(1): 176, 2020 Aug 05.
Article in English | MEDLINE | ID: mdl-32758203

ABSTRACT

PURPOSE: To determine if improvement in imaging reduces the non-resection rate (NRR) among patients with pancreatic ductal adenocarcinoma (PDAC). METHODS: From 2000 to 2019, 751 consecutive patients with PDAC were considered eligible for a intention-to-treat pancreatectomy and entered the operating room. In April 2011, our institution acquired a dual energy spectral computed tomography (CT) scanner and liver diffusion weighted magnetic resonance imaging (DW-MRI) was included in the imaging workup. We consequently considered 2 periods of inclusion: period #1 (February 2000-March 2011) and period #2 (April 2011-August 2019). RESULTS: All patients underwent a preoperative CT scan with a median delay to surgery of 18 days. Liver DW-MRI was performed among 407 patients (54%). Median delay between CT and surgery decreased (21 days to 16 days, P < .01), and liver DW-MRI was significantly most prescribed during period #2 (14% vs 75%, P < .01). According to the intraoperative findings, the overall NRR was 24.5%, and remained stable over the two periods (25% vs 24%, respectively). While vascular invasion, liver metastasis, and carcinomatosis rates remained stable, para-aortic lymph nodes invasion rate (0.4% vs 4.6%; P < 0.001) significantly increased over the 2 periods. The mean size of the bigger extra pancreatic tumor significantly decrease (7.9 mm vs 6.4 mm (P < .01), respectively) when the resection was not done. In multivariate analysis, CA 19-9 < 500 U/mL (P < .01), and liver DW-MRI prescription (P < .01) favoured the resection. CONCLUSIONS: Due to changes in our therapeutic strategies, the NRR did not decrease during two decades despite imaging improvement.


Subject(s)
Adenocarcinoma , Carcinoma, Pancreatic Ductal , Pancreatectomy/statistics & numerical data , Pancreatic Neoplasms , Adenocarcinoma/diagnostic imaging , Adenocarcinoma/surgery , Adult , Aged , Aged, 80 and over , Carcinoma, Pancreatic Ductal/diagnostic imaging , Carcinoma, Pancreatic Ductal/surgery , Diffusion Magnetic Resonance Imaging , Female , Humans , Male , Middle Aged , Pancreatic Neoplasms/diagnostic imaging , Pancreatic Neoplasms/surgery , Retrospective Studies
2.
Eur J Paediatr Dent ; 18(1): 23-26, 2017 Mar.
Article in English | MEDLINE | ID: mdl-28494598

ABSTRACT

AIM: Dental trauma is a frequent finding in people with special health care needs. The aim of this study was to determine the prevalence of dental trauma in a sample of Italian children and adolescents with special health care needs. MATERIALS AND METHODS: 556 medical and dental records of children and adolescents visited from January 2010 to March 2015 were examined. Information about medical diagnosis, gender, site and type of dental trauma (DT) were collected. According to age and reflecting the dentition stage, the sample was divided into 3 groups: subjects aged 0-5 years (group A, primary dentition), 6-11 years (group B, mixed dentition), 12-18 years (group C, permanent dentition). RESULTS: 113 individuals experienced a DT (prevalence 20.3%), with no difference in relation to gender. Individuals with cerebral palsy and autism showed the highest frequency of DT: 39.6% and 30.4%, respectively. The highest frequency of DT occurred both in group A (21.8%) and B (21.5%), which differed significantly from group C (9%). Avulsion was the most frequent type of DT in the primary dentition (24%) and enamel-dentin fracture without pulp exposure in the permanent dentition (60%). Upper central incisors were the most affected teeth. CONCLUSION: The prevalence of DT in a sample of Italian children and adolescents with special health care needs is high, especially in young individuals with cerebral palsy and autism. Preventive strategies for those patients should be developed in order to reduce the risk of DT.


Subject(s)
Disabled Children , Tooth Injuries/epidemiology , Adolescent , Child , Child, Preschool , Cross-Sectional Studies , Female , Humans , Infant , Italy/epidemiology , Male , Prevalence , Retrospective Studies , Risk Factors
3.
Orthod Craniofac Res ; 19(1): 46-53, 2016 Feb.
Article in English | MEDLINE | ID: mdl-26428540

ABSTRACT

OBJECTIVES: Literature is poor of data about the occlusion in children affected by neurofibromatosis type 1 (NF1). This case-control study investigated the occlusal traits in a group of children with NF1. SETTING AND SAMPLE POPULATION: A hundred and fifteen children with NF1 were enrolled; non-NF1 controls were sequentially selected among subjects referred to the Pediatric Dentistry Department. MATERIAL AND METHODS: All patients underwent a clinical dental examination and a panoramic radiography. The following orthodontic variables were considered: molar relationship, overjet, overbite, cross-bite, scissor bite, and crowding/spacing. RESULTS: Class III molar relationship resulted significantly (p = 0.01) more common in children with NF1 than in the control group as well as the unilateral posterior cross-bite (p = 0.0017). Forty-three children with NF1 (37.3%) showed radiographic abnormalities; in one case, a plexiform neurofibroma was detected. CONCLUSIONS: An early orthodontic evaluation might be planned in the management of children with NF1 to prevent or decrease the need for extensive orthodontic interventions.


Subject(s)
Neurofibromatosis 1 , Case-Control Studies , Child , Humans , Malocclusion , Overbite
4.
Apoptosis ; 20(1): 10-28, 2015 Jan.
Article in English | MEDLINE | ID: mdl-25351440

ABSTRACT

Alzheimer's disease (AD) and cancer proceed via one or more common molecular mechanisms: a metabolic shift from oxidative phosphorylation to glycolysis-corresponding to the activation of the Warburg effect-occurs in both diseases. The findings reported in this paper demonstrate that, in the early phase of apoptosis, glucose metabolism is enhanced, i.e. key proteins which internalize and metabolize glucose-glucose transporter, hexokinase and phosphofructokinase-are up-regulated, in concomitance with a parallel decrease in oxygen consumption by mitochondria and increase of L-lactate accumulation. Reversal of the glycolytic phenotype occurs in the presence of dichloroacetate, inhibitor of the pyruvate dehydrogenase kinase enzyme, which speeds up apoptosis of cerebellar granule cells, reawakening mitochondria and then modulating glycolytic enzymes. Loss of the adaptive advantage afforded by aerobic glycolysis, which occurs in the late phase of apoptosis, exacerbates the pathological processes underlying neurodegeneration, leading inevitably the cell to death. In conclusion, the data propose that both aerobic, i.e. Warburg effect, essentially due to the protective numbness of mitochondria, and anaerobic glycolysis, rather due to the mitochondrial impairment, characterize the entire time frame of apoptosis, from the early to the late phase, which mimics the development of AD.


Subject(s)
Glycolysis , Mitochondria/metabolism , Neurons/metabolism , Up-Regulation , Animals , Apoptosis/drug effects , Cerebellum/cytology , Cerebellum/metabolism , Dichloroacetic Acid/pharmacology , Glucose Transport Proteins, Facilitative/metabolism , Hexokinase/metabolism , Hippocampus/cytology , Hippocampus/metabolism , Humans , Neurons/ultrastructure , Oxygen/metabolism , Phosphofructokinases/metabolism , Primary Cell Culture , Rats, Wistar
5.
Sleep Breath ; 19(4): 1213-20, 2015 Dec.
Article in English | MEDLINE | ID: mdl-25682270

ABSTRACT

PURPOSE: Evaluation of the effects of rapid maxillary expansion and mandibular advancement using Propulsor Universal Light appliance on the upper airways in Marfan's syndrome children through home sleep studies, Epworth Sleepiness Scale questionnaire, and cephalometric analysis of the upper airways on lateral radiographs. METHODS: The study sample consisted of 30 children with Marfan's syndrome, and the control group consisted of 30 untreated and matched children. For Marfan subjects, data were taken at different time points compared to treatment: at T0 (before treatment), T1 (after rapid maxillary expansion), and T2 (after mandibular advancement). For control subjects, data were taken at similar intervals, at three follow-up visits: at T0 (as a starting screening tool), T1 (after approximately 2 years), and T2 (in proximity of the peak skeletal growth). RESULTS: Apnea-hypopnea and oxygen desaturations were significantly higher in the study group at T0 and T1 compared with control children. At T2, the values were not significant (p value 0.442 for both apnea-hypopnea index (AHI) and oxygen desaturation index (ODI)). Horizontal airway dimensions were significantly reduced, and vertical airway values were significantly increased in Marfan's syndrome at T0 and T1 but not at T2 (p values at T2: 0.071 for Phw1-Psp, 0.106 for Phw1-Psp', 0.101 for Phw2-Tb, 0.559 for UAL in male and 0.560 for UAL in female). CONCLUSIONS: Early rapid maxillary expansion and mandibular advancement using Propulsor Universal Light appliance significantly improved airway patency of Marfan's syndrome children and are strongly encouraged as a routine treatment for both correction of class II malocclusions and prevention of obstructive sleep apnea.


Subject(s)
Cephalometry , Mandibular Advancement/methods , Marfan Syndrome/physiopathology , Marfan Syndrome/surgery , Orthodontic Appliances , Palatal Expansion Technique/instrumentation , Polysomnography , Pulmonary Ventilation/physiology , Sleep Apnea, Obstructive/physiopathology , Sleep Apnea, Obstructive/surgery , Case-Control Studies , Child , Female , Follow-Up Studies , Humans , Male , Mandibular Advancement/instrumentation , Orthodontic Appliance Design
6.
Eur J Paediatr Dent ; 15(3): 303-8, 2014 Sep.
Article in English | MEDLINE | ID: mdl-25306149

ABSTRACT

AIM: The aim of this study was to evaluate the presence and degree of depressive symptoms in mothers of disabled children and to assess the correlation between maternal major depression risk and son/daughter oral health. MATERIALS AND METHODS: A prospective study was conducted in 51 disabled children and their 51 mothers. In children dmft/DMFT values, food and/or sugar- sweetened consumption levels and daily tooth brushing frequency were evaluated. Depressive maternal symptoms were measured by EDPS questionnaire: the questionnaire scores were converted into positive predictive values (PPV) that represented the risk of falling into major depression. A regression analysis was performed on the variables (statical significance was set at p value ≤ 0.05). RESULTS: Children (8.68 ± 3.98 years old) average dmft/DMFT was 2.7. Fifty three percent of the mothers (38.37 ± 6.04 years) were at risk for depression (PPV > 60%), while depressive symptoms were already present in 25% of the subjects (PPV=100%). DISCUSSION AND CONCLUSION: Mothers of disabled children are more likely to fall into major depression compared to mothers of healthy children. For each mother-child couple the correlation between different variables was evaluated: there was a statistically significant correlation between children's dmft/DMFT values and mothers' depression risk. The risk of maternal depression was statistically correlated to prevalence of caries and sugar consumption in children.


Subject(s)
Depression/psychology , Disabled Children , Mothers/psychology , Oral Health , Adolescent , Adult , Child , Child, Preschool , DMF Index , Depressive Disorder, Major/psychology , Dietary Sucrose/administration & dosage , Educational Status , Employment , Feeding Behavior , Female , Humans , Male , Middle Aged , Mothers/education , Predictive Value of Tests , Prospective Studies , Risk Assessment , Toothbrushing/statistics & numerical data
7.
Eur J Paediatr Dent ; 15(1): 45-50, 2014 Mar.
Article in English | MEDLINE | ID: mdl-24745592

ABSTRACT

AIM: To investigate with a reliable method the oral features in Italian patients in remission from cancer, highlighting the relationship with age at cancer therapy and to compare the data with healthy controls. MATERIALS AND METHODS: Twenty five childhood cancer survivors treated under the age of 10 years with chemotherapy w/wo Haemopoietic Stem Cell Transplantation and/or head-neck Radiotherapy, in remission from cancer for at least 3 years, were examined for dental caries and enamel defects. To assess dental age and dental abnormalities a panoramic radiograph was taken. Patients were grouped according to age at cancer therapy (<3 years: subgroup Y; 3.1-5 years: subgroup M; >5 years: subgroup O). A control group of 26 healthy children was included. RESULTS: There was not a statistically significant difference in caries prevalence between the two groups. A statistically significant difference between the two groups was found for enamel defects, dental abnormalities and dental age. The chi-squared test revealed a relationship between age at therapy and specific dental abnormalities. CONCLUSION: This study shows that cancer therapy may increase the risk of development of enamel defects and dental abnormalities, especially in children treated under the age of 3 years.


Subject(s)
Head and Neck Neoplasms/therapy , Neoadjuvant Therapy , Survivors , Tooth Diseases/etiology , Adolescent , Age Determination by Teeth , Age Factors , Anodontia/etiology , Antineoplastic Agents/therapeutic use , Case-Control Studies , Child , DMF Index , Dental Caries/etiology , Dental Enamel/abnormalities , Female , Follow-Up Studies , Head and Neck Neoplasms/radiotherapy , Hematopoietic Stem Cell Transplantation , Humans , Italy , Male , Odontogenesis/physiology , Oral Health , Pilot Projects , Radiography, Panoramic , Tooth Abnormalities/etiology , Tooth Root/abnormalities , Young Adult
8.
Eur J Paediatr Dent ; 13(2): 159-60, 2012 Jun.
Article in English | MEDLINE | ID: mdl-22762182

ABSTRACT

BACKGROUND: The 47,XXY syndrome, or Klinefelter syndrome, though it is a rare occurrence, it is the most common sex choromosome disorder affecting male subjects. This syndrome is underdiagnosed and seldomly before puberty. In this case, diagnosis was made before birth, through chorion villus sampling. CASE REPORT: A 16 month-old Italian male with 47 XXY syndrome showed the absence of primary teeth, with a delay of about 8-10 months, whereas during the first 15 months of life the auxological development has been normal both in weight and height (about 50th percentile). We assumed that this delay may be linked with Klinefelter syndrome, as sexual chromosomes play an important role in the dental development.


Subject(s)
Klinefelter Syndrome/physiopathology , Tooth Eruption/physiology , Tooth, Deciduous/physiopathology , Body Height , Body Weight , Follow-Up Studies , Humans , Infant , Male
9.
Eur J Paediatr Dent ; 13(2): 147-50, 2012 Jun.
Article in English | MEDLINE | ID: mdl-22762179

ABSTRACT

AIM: This was to validate a taste test on healthy school children. A cross-sectional study was designed to perform a standardised clinical evaluation of the gustatory sensitivity. MATERIALS AND METHODS: Forty (18 males and 22 females) children were selected. Inclusion criteria were age between 5 and 12 years, absence of systemic disease and no antibiotic treatment in the last six months. The taste assessment tests were performed following a standardised protocol, repeated at two different intervals: a) tested at time 0; b) tested after 20-30 days. Tests were performed using a pipette with the sample solution: sucrose, sodium chloride, citric acid and quinine hydrochloride at different concentrations. The examiner reported in a grid the flavour perceived by the subject. A placebo (tap water) was administered in between the flavours RESULTS: The majority of the subjects detected the bitter taste at the lowest concentration (mean=1.83). The sour taste was detected with the second concentration (mean=2.56). The sweet solution was detected with the most diluted concentration (mean=1.56). The salty taste has a threshold of 2 (mean=2.04), which means that the majority of subjects detected the salty solution with the second concentration. Regarding the perceived intensity, it increases with the increasing concentrations and it reaches maximum values that are inversely proportional to the threshold, corresponding to the second concentration. Moreover, no statistically significant gender differences were detected regarding the threshold values or the perceived intensity. CONCLUSION: The proposed test allows for a controlled, reliable and standardised evaluation of the gustatory modality.


Subject(s)
Taste Threshold/physiology , Child , Child, Preschool , Citric Acid/administration & dosage , Cross-Sectional Studies , Female , Follow-Up Studies , Humans , Male , Quinine/administration & dosage , Sodium Chloride/administration & dosage , Sucrose/administration & dosage , Water
10.
Eur J Paediatr Dent ; 23(2): 116-120, 2022 Jun.
Article in English | MEDLINE | ID: mdl-35722847

ABSTRACT

AIM: The aetiology of Molar Incisor Hypomineralisation (MIH) is currently unclear. Over time, several aetiological hypotheses have come forward, including pre- and perinatal medical problems and postnatal illness. The aim of this case-control study is the identification of possible predisposing factors involved in MIH aetiology. METHODS: Methods Study Design: By hypothesising the probability of at least one predisposing factor present 2.5 times more in MIH cases than in controls, with an estimated prevalence of MIH patients requiring therapy equal to 30%, at a unilateral alpha level of 5% and a power of 80%, 63 couples of subjects are needed with an allocation ratio of 1:1; individual matching for age and gender was carried out. After clinical examination, 78 children with MIH (EAPD criteria) were recruited (mean age 9.36 years). An anamnestic form filled-in by a parent was used to collect data on possible predisposing factors including demographic characteristics, pregnancy, birth, childhood medical illness and medications' intake. STATISTICS: One-tail McNemar chi square test was used to evaluate the significance of the association between predisposing factor and MIH; odds ratio and 95% confidence intervals were computed. CONCLUSION: A multifactorial aetiology may be advocated for MIH development; in particular, further investigations are required to confirm and clarify the role of genetic factors.


Subject(s)
Dental Enamel Hypoplasia , Molar , Case-Control Studies , Causality , Child , Dental Enamel Hypoplasia/epidemiology , Dental Enamel Hypoplasia/etiology , Female , Humans , Incisor , Pregnancy , Prevalence
11.
Eur J Paediatr Dent ; 12(4): 256-60, 2011 Dec.
Article in English | MEDLINE | ID: mdl-22185252

ABSTRACT

AIM: The scientific literature is poor of data concerning NF1-related oral manifestations in children. The purpose of this study was to investigate the oral findings in a group of children with NF1. MATERIALS AND METHODS: This study was designed as a clinical research. A total of 50 children affected by NF1 were enrolled. Non-NF1 controls were selected among subjects referred to the Paediatric Dentistry Department of the Dental Clinic at the University of Brescia, Italy. The craniofacial and all the dental examinations were carried out. The following parameters were investigated: dental caries, dental abnormalities, periodontal status, oral and perioral neurofibromas, orthodontic features, mandible and temporomandibular joint abnormalities. Statistics Unpaired Student's t test was calculated for Gaussian distributed variables; Fisher's test for non-Gaussian distributed binomial variables was used. The values inferior to p <0.05 (threshold) were considered significant for the study. RESULTS: Children with NF1 showed poorer oral hygiene conditions, more frequent Class III dental malocclusions and severe alterations of the mandible and the temporal mandibular joint, if compared with the control group. CONCLUSION: NF1 related-oral manifestations are evidenced also during childhood, even if some of the best known, like the neurofibromas, are not yet developed. It is important to keep a long-term follow-up to monitor the possible development of other oral lesions.


Subject(s)
Jaw Abnormalities/etiology , Malocclusion, Angle Class III/etiology , Neurofibromatosis 1/complications , Tooth Abnormalities/etiology , Case-Control Studies , Child , DMF Index , Female , Humans , Male , Mandible/abnormalities , Oral Hygiene Index , Periodontal Index
12.
Eur J Paediatr Dent ; 22(3): 243-247, 2021 Sep.
Article in English | MEDLINE | ID: mdl-34544255

ABSTRACT

AIM: Autism Spectrum Disorder (ASD) is characterised by communication deficits and repetitive unusual behaviours. The behaviour guidance of these children represents a challenge for the dental team. The aim of the study was to evaluate the oral health status in a group of Italian children with ASD. MATERIALS AND METHODS: Study Design: Sixty-four Italian children with ASD and 64 controls were included. Data were collected by means of questionnaires and clinical examinations. RESULTS: Dental trauma (p=0.007), bruxism (p=0.001) and biting objects habit (p=0.021) were more frequent in the study group; fluoride exposure was lower (p=0.001) (chi-square test). The mean plaque index was 1.48 ±0.75 in the study group and 0.81±0.56 in the control group (p=0.001; Mann-Whitney U test); the mean dmft/DMFT was 3.00 ±1.2 and 2.3 ±1.8 in the study group and 1.8 ±1.1 and 1.0 ±1.1 in the control group (p<0.001; Mann-Whitney U test). Anterior open bite was more frequent in the study group (p=0.013; Chi-square test). No significant differences were found for enamel defects, molar relationship, posterior crossbite and deep bite. Significantly more children with ASD showed a negative behaviour (80% vs 35%: p =0.001; Chi-square test). CONCLUSION Children with ASD have a poorer oral health status than healthy children. The early establishment of a home dental hygiene should be encouraged.


Subject(s)
Autism Spectrum Disorder , Dental Caries , Malocclusion , Autism Spectrum Disorder/complications , Autism Spectrum Disorder/epidemiology , Child , Cross-Sectional Studies , Humans , Italy/epidemiology , Oral Health
13.
Eur J Paediatr Dent ; 22(4): 309-313, 2021 Dec.
Article in English | MEDLINE | ID: mdl-35034460

ABSTRACT

AIM: Down syndrome is the most common form of aneuploidia compatible with a long survival. The affected subjects are more susceptible to severe early-onset periodontal disease and show a lower risk to develop dental caries than the non-affected population. This study investigated the prevalence of periodontal pathogens in the subgingival plaque of deciduous teeth in children with Down syndrome without signs of periodontal breakdown. METHODS: Thirty children suffering from Down syndrome and 46 matched healthy subjects were studied. A total of 228 subgingival plaque samples from deciduous teeth were separately collected and evaluated by polymerase chain reaction assays. CONCLUSION: In absence of periodontal impairment, Down syndrome children display a clear presence of periodontal pathogens already in the deciduous dentition. The hypothesis of an intrinsic predisposing condition is here supported.


Subject(s)
Dental Caries , Dental Plaque , Down Syndrome , Case-Control Studies , Child , Down Syndrome/complications , Humans , Tooth, Deciduous
14.
Eur J Paediatr Dent ; 11(1): 39-43, 2010 Mar.
Article in English | MEDLINE | ID: mdl-20359281

ABSTRACT

AIM: Cleidocranial dysplasia (CCD) is a rare disorder that is inherited as an autosomal genetic trait. It is characterised by defective ossification, delayed bone and tooth development, stomatognathic and craniofacial abnormalities and it is caused by mutations in the RUNX2 gene that is responsible for osteoblast differentiation. CCD is a dental disease that needs complex rehabilitation and the assistance of several specialists. In most cases, this disease precipitates towards the end of childhood with the progressive morbidity of the deciduous dentition, thus leading to edentulism at a young age and giving patients an older appearance. Several therapeutic approaches have been proposed within literature. The aim of this paper is to revise the literature on the proposed therapeutic approaches for the functional and aesthetical rehabilitation of the typical defects caused by CCD in the cephalic region, and to identify the most effective therapy currently available. CONCLUSIONS: The most effective therapeutic approach for the early treatment of the orthodontic and orthopaedic diseases in CCD patients would start with an orthopaedic treatment of the upper jaw followed by orthodontic and surgical treatments. When growth is completed a reintervention for maxillofacial surgery and the orthodontic treatment may be required. Finally, if some teeth are missing. function and aesthetics can be achieved replacing them with prosthesis.


Subject(s)
Cleidocranial Dysplasia/complications , Mouth Rehabilitation , Tooth Abnormalities/etiology , Tooth Diseases/etiology , Dental Prosthesis Design , Humans , Orthodontics, Corrective , Orthognathic Surgical Procedures , Tooth Abnormalities/therapy , Tooth Diseases/therapy
15.
Eur J Paediatr Dent ; 11(3): 146-8, 2010 Sep.
Article in English | MEDLINE | ID: mdl-21080756

ABSTRACT

BACKGROUND: Aicardi Syndrome is an X-linked autosomal recessive neurodegenerative encephalopathy. The diagnostic triad is composed by infantile spasms, agenesis of corpus callosum and chorioretinal lacunae. Additional common findings are: microencephaly, spasticity and severe mental delay. It affects only females because of early embryonic lethality in males. A significant number of females with Aicardi syndrome are of normal birth and develop normally until three months of age when infantile seizures begin. Psychomotor retardation is usually severe, neuromotor retardation is commonly present with lack of motor and language skills. Literature reports only few information about the dentofacial features of the syndrome. CASE REPORT: We present the case of a patient, by describing the dental and facial characteristics with focus on dental prevention in order to avoid dental pain and the risks connected to general anaesthesia, and ultimately for improving the quality of life.


Subject(s)
Aicardi Syndrome/complications , Open Bite/etiology , Tooth Abnormalities/etiology , Anticonvulsants/adverse effects , Candidiasis, Oral/complications , Child, Preschool , Dental Caries/complications , Facies , Female , Gingival Overgrowth/chemically induced , Humans
16.
Minerva Stomatol ; 59(3): 117-27, 2010 Mar.
Article in English, Italian | MEDLINE | ID: mdl-20357738

ABSTRACT

Cleidocranial dysplasia (CCD) is a rare disorder which is inherited as an autosomal genetic trait. It is characterized by defective ossification, delayed bone and tooth development, stomatognathic and craniofacial abnormalities, and it is caused by mutations in the RUNX2 gene that is responsible for osteoblast differentiation. The purpose of this review is to collect and analyze data in the literature on orofacial typical manifestations of the syndrome and to present knowledge of the eziopatogenics mechanisms of the CCD. Clinical, genetic, aetiopathogenetic studies on this syndrome were compliled through a systematic approach using Medline. This review reports the cranio-facial features and dental characteristics of the CCD on the basis of all data in the literature. This review pays particular attention on the eziopatogenics mechanisms of CCD and summarises the results of the most recent studies. Access to detailed review of the etiopathogenic mechanisms of CCD is a fundamental support for clinicians as it can allow to make an informed assessment regarding the most effective choice of therapy. The review shows how an interdisciplinary approach is necessary for an appropriate treatment since CCD patients suffer from a skeletal third class, transverse deficiency of the maxilla, supernumerary permanent teeth and deficient eruption of impacted permanent teeth.


Subject(s)
Abnormalities, Multiple , Cleidocranial Dysplasia/complications , Cleidocranial Dysplasia/etiology , Craniofacial Abnormalities/complications , Stomatognathic System Abnormalities/complications , Humans
17.
Minerva Stomatol ; 59(3): 129-37, 2010 Mar.
Article in English, Italian | MEDLINE | ID: mdl-20357739

ABSTRACT

Combined methylmalonic aciduria and homocystinuria (MMA-HC) is a rare metabolic disease characterized by an inborn defect in B12 vitamin metabolism. This case report concerns an 11-year-old patient with MMA-HC, which developed during the neonatal period. The patient shows some of the facial features that were already reported in the literature (high forehead, large floppy, low-set ears, flat philtrum and hypotonia of perioral and masticatory muscles) but no dolichocephalic skull nor long face. The patient also shows signs that had not been previously described: epicanthal folds, broad nasal bridge, long and flat philtrum, amimic expression and, particularly, a postural alteration (the head is rotated and bent towards the left shoulder, which is lower than the right one). Such alteration can be attributed to visual impairment and is responsible for breaking muscular and skeletal balance in the frontal plane, thus causing the horizontal planes of both maxillary bones to converge towards the right--as highlighted by the cephalometric analysis of the Teleradio-graph of the skull in Posteroanterior projection according to Ricketts. As for the patient's teeth, eruption times are normal, but there are anomalies of shape (chisel-like central incisors). As far as dental caries is concerned, the patient's DMFT is 4 (D=4, M=0, F=0). This clinical case highlights the need for dental prevention programs suggested by the pediatrician once the pathology is diagnosed.


Subject(s)
Abnormalities, Multiple , Craniofacial Abnormalities/complications , Face/abnormalities , Homocystinuria/complications , Metabolism, Inborn Errors/complications , Metabolism, Inborn Errors/urine , Methylmalonic Acid/urine , Tooth Abnormalities/complications , Vitamin B 12 Deficiency/complications , Child , Female , Humans , Phenotype
18.
Eur Arch Paediatr Dent ; 21(5): 617-622, 2020 Oct.
Article in English | MEDLINE | ID: mdl-31898299

ABSTRACT

PURPOSE: Self-induced soft-tissue injuries (SSI) are reported as local anesthesia complications, particularly in children. The purpose of the study was to evaluate the frequency of SSI following dental anesthesia in children with and without intellectual disability. METHODS: 241 children receiving dental treatments with local anesthesia were divided into 2 groups: A, children without intellectual disability (159 individuals, 299 injections); B, children with intellectual disability (82 individuals, 165 injections). Each group was divided into subgroups according to age, injection technique and dental treatment. Two days after the dental procedure, a phone survey was conducted to determine the presence of SSI. RESULTS: The frequency of SSI in group B was 19%, with no differences in relation to gender and age. In group A the frequency of SSI was significantly lower (9%; p = 0.002; Chi-square test); the children in the ≤ 6 years-old subgroup experienced a higher frequency of SSI (p = 0.002). The lower arch was at major risk of SSI in both groups (p = 0.002). According to a multilevel approach group (p = 0.001) and injection technique (p = 0.0001) significantly influenced SSI; no influence of dental treatment is evidenced. CONCLUSIONS: SSI are common complications of local anesthesia in young children and individuals with intellectual disability.


Subject(s)
Anesthesia, Dental/adverse effects , Intellectual Disability/complications , Anesthesia, Local/adverse effects , Child , Child, Preschool , Humans , Injections , Prospective Studies
19.
Eur J Paediatr Dent ; 21(2): 153-156, 2020 Jun.
Article in English | MEDLINE | ID: mdl-32567948

ABSTRACT

AIM: To evaluate the effect of audiovisual distraction on the dental chairside behaviour of children with Down syndrome (DS) during dental restorations and its influence on the operator stress and the duration of the appointment. MATERIALS AND METHODS: Study design: This randomised controlled trial included 48 children with DS requiring dental restorations. The study group was treated while wearing video eyeglasses, the control group with conventional behaviour management techniques. The child behaviour was evaluated using the revised Face, Leg, Activity, Cry, Consolability scale (r-FLACC) and the Frankl scale. The operator stress was evaluated using a VAS scale and the duration of the appointment was recorded. RESULTS: In the study group 64% of the children refused to wear the video eyeglasses during the whole duration of the dental treatment, the median r-FLACC score was significantly higher (p= 0.01552; Mann Whitney U test) and significantly more children showed a negative behaviour (68%vs 30%: p =0.011; Chi-square test). CONCLUSION: Audiovisual distraction using video eyeglasses is not useful in managing the dental chairside behaviour of children with DS.


Subject(s)
Anesthesia, Dental , Down Syndrome , Child , Dental Anxiety , Dental Care , Eyeglasses , Humans
20.
Minerva Stomatol ; 58(10): 539-46, 2009 Oct.
Article in English, Italian | MEDLINE | ID: mdl-19893477

ABSTRACT

The craniosynostoses represent a group of pathologies characterised by the premature fusion (during prenatal or perinatal stages or early infancy) of one or more of the cranial sutures. Its genetic origins are not completely clear although mutations in the genes that code for fibroblast growth factor receptors have been described; depending upon the gene involved, the type of mutation and the embryological period in which the mutation itself occurs, a type of craniosynostosis arises that may involve one or more cranial sutures. The premature fusion results in a lack of growth in the regions corresponding to the cranial vaults, with compensatory growth occurring in other regions and the consequent deformation of the skull. Here, a case report is presented of a Caucasian male child affected by craniostenosis of the sagittal suture with a orthodontic-orthopedic diagnosed ogival palate and insufficient space for the eruption of the superior canines. The patient was treated by fitting a rapid palatal protractor, an orthopedic device that acts upon the median palatine suture of the palate increasing the transverse diameter of the upper jaw and causes the widening and lowering of the floor of the nasal cavity. The results obtained were equivalent to those obtained in subjects with no past medical history of craniosynostosis. This clinical study demonstrated that the premature fusion of the mid-sagittal suture of the skull, that characterises sagittal craniosynostoses, can occur without the involvement of the median suture of the palate, rendering expansion possible by means of orthopedic treatment.


Subject(s)
Craniosynostoses/complications , Palatal Expansion Technique , Palate, Hard/abnormalities , Palate, Hard/surgery , Child , Humans , Male , Time Factors
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