Familial malignant osteopetrosis in children: A case report

The clinical, radiological, pathological and laboratory findings of two brothers with autosomal recessive malignant osteopetrosis are presented. Our findings are similar to characteristics previously reported in the literature about patients with osteopetrosis. The 6-year-old male patient was pale and had petechiae on his arms and legs. He also had macrocephalia, splenomegaly, severe pancytopenia, genu valgus, hypocalcemia, amaurosis, cessation of physical development, generalized bone sclerosis and recurrent infections with a history of multiple incidences of acute otitis media. Generalized bone sclerosis and irregular sclerosis of the maxilla and mandible were seen on radiographs. The oral mucosa was apparently normal but permanent tooth eruption was delayed although there was early loss of deciduous teeth. The recommended treatment was blood transfusion and therapy with antibiotics when necessary; a bone marrow transplant was not possible due to lack of a compatible donor.

Imprint cytology of osteosarcoma of the jaw: A case report

Introduction. Osteosarcomas are highly malignant bone-forming neoplasms that account for about 20% of all sarcomas. In light of their aggressive behavior, early diagnosis is crucial for determining adequate treatment. Dental professionals may be the first to detect jaw osteosarcomas in their initial stages. The aim of this case report is to draw attention to the possibility of diagnosing this tumor based on clinical, radiographical and cytological characteristics before confirmation by histology. Case presentation. A 24-year-old Afro-Brazilian man presented with swelling and pain on the left side of the mandible in the region of the third molar (tooth 38). Radiography showed a poorly delimited intraosseous lesion with radiolucent and radiopaque areas. The cytological aspects were consistent with the diagnosis of osteosarcoma, which was confirmed by biopsy. Conclusion. Imprint cytology was found to be a reliable, rapid and easy complementary examination. An early diagnosis of osteosarcoma of the jaw is fundamental to the early determination of an adequate treatment. © 2009 Cabral et al; licensee BioMed Central Ltd.

Jaw musculature of the Meiglyptini (Aves: Piciformes: Picidae)

The Meiglyptini comprise eight species of woodpeckers grouped into three genera, two of which contain three species ( Meiglyptes and Mulleripicus), while one genus includes two species ( Hemicircus). The purpose of this study is to describe the mandibular apparatus found in six species of Meiglyptini and to compare them with each other and with other woodpecker species. The results reveal a number of structures that are worth mentioning: (i) the components of the external mandibular adductor system of Hemicircus concretus, particularly the M. adductor mandibulae externus caudalis medialis, are underdeveloped compared with the other investigated species; (ii) the muscles of the internal mandibular system are structurally different among Meiglyptes species and are less developed, both in size and in structure, in H. concretus; (iii) the M protractor quadrati is vestigial in Meiglyptes species; (iv) the muscles of the protractor system of the quadrate are relatively undeveloped in H. concretus; (v) most of the muscles of the pterygoideus system are structurally differentiated in Meiglyptes species. It appears that the complexity of the mandibular apparatus is associated with the type of food consumed, as the apparatus of the frugivorous species H. concretus is markedly different from that of the insectivorous species. © 2012 The Royal Swedish Academy of Sciences.

Jaw musculature of the Picini (Aves: Piciformes: Picidae)

The Picini tribe comprises 25 Old World woodpecker species grouped into seven genera that are widely distributed in Asia and include several representatives from the Eurasian region. Given the absence of detailed anatomical studies of Picini in the literature, the purposes of this study were to describe the jaw musculature of 14 species of Picini in detail and to compare the musculature patterns of these species. The results of this analysis indicate the following: (1) there is a clear association between the ventralis lateralis and dorsalis lateralis muscles through fleshy fibers that are connected in all species, (2) the jaw musculature of the genus Picus differs from that of other Picini genera in terms of the poor development of the protractor muscle system of the quadrate (M. protractor quadrati and M. protractor pterygoidei), (3) generally, the M. pseudotemporalis superficialis originates in the ventrocaudal region of the laterosphenoid (the lower part of the orbit), with the only noteworthy exception being an origin in the upper part of the orbit in Dinopium javanense, and (4) the protractor pterygoidei muscle is more developed in Blythipicus rubiginosus, Dinopium rafflesii, and D. javanense than in the other species. © 2012 Reginaldo José Donatelli.

Copaiba oil effect on experimental jaw defect in Wistar rats

PURPOSE: To evaluate the effects of copaiba oil on jaw defects repair in Wistar rats treated with bioglass or adipose tissue. METHODS: A jaw defect was randomly created in forty-two rats and filled with bioglass or adipose tissue. The two groups (Gbio and Gcell) were subdivided in three subgroups with seven animals each according to gavage administration: control (distillated water), oil (copaiba oil) and melox (meloxicam). Euthanasia was performed after forty post-operative days. The bone formation was analyzed regarding the histological aspects. RESULTS: The osteoclasts activity was observed only in four subgroups (p=0.78). Regarding the osteoblasts presence, it was very similar between the subgroups, the difference was due to Gcell-melox (p=0.009) that presented less osteoblastic activity. The inflammatory cells were more evident in Gcell-melox subgroup, however, there was no difference in comparison with the other subgroups (p=0.52). Bone formation was observed in all subgroups, just two animals showed no bone formation even after 40 days. More than 50% of bone matrix mineralization was observed in 56% (23 animals) of the analyzed areas. The bone matrix mineralization was not different between subgroups (p=0.60). CONCLUSIONS: The subgroups that received copaiba oil showed bone repair, although not statistically significant in comparison to subgroups treated whit meloxicam or controls. Copaiba oil administered by gavage had no effect on bone repair in this experimental model.

Patient Education and Self-Care for the Management of Jaw Pain upon Awakening: A Randomized Controlled Clinical Trial Comparing the Effectiveness of Adding Pharmacologic Treatment with Cyclobenzaprine or Tizanidine

Aims: To compare the effectiveness of adding cyclobenzaprine, tizanidine, or placebo to patient education and a self-care management program for patients with myofascial pain and specifically presenting with jaw pain upon awakening. Methods: Forty-five patients with a diagnosis of myofascial pain based on the guidelines of the American Academy of Orofacial Pain participated in this 3-week study. The subjects were randomly assigned into one of three groups: placebo group, TZA group (tizanidine 4 mg), or CYC group (cyclobenzaprine 10 mg). Patients were evaluated for changes in pain intensity, frequency, and duration by using the modified Severity Symptoms Index and changes in sleep quality with the use of the Pittsburgh Sleep Quality Index. Data were analyzed by ANOVA and post-hoc or nonparametric statistical tests as appropriate. Results: All three groups had a reduction in pain symptoms and improvement of sleep quality based on a comparison of pretreatment and treatment scores. However, no significant differences among the groups were observed at the posttreatment evaluation. Conclusion: The use of tizanidine or cyclobenzaprine in addition to self-care management and patient education was not more effective than placebo for the management of patients with myofascial jaw pain upon awakening.

Relationship Between Jaw Apparatus, Feeding Habit, and Food Source in Oriental Woodpeckers

Associations among feeding habit, beak type, and food source in birds have been widely studied and are well known to exist. The relationship between feeding habit and jaw apparatus in birds has not attracted attention from ornithologists, perhaps because of the complexity of the skeletal morphology of the feeding system of birds. The goal of this study was to compare the jaw apparatus and foraging strategies of various Oriental species of the Picidae (Meiglyptini and Picini tribes) using a morphofunctional analysis of the skeletal structure of the jaw apparatus. This study showed that there are at least three types of jaw apparatus in these woodpeckers, as follows: 1) robust, developed, and complex; 2) complexity and development intermediate, as observed in Meiglyptes tristis and Dinopium spp., whose main foraging method involves gleaning, probing, and tapping; and 3) poorly developed, as observed in Picus miniaceus and Hemicircus concretus. The success of woodpeckers as a natural group is due not only to their feeding diversity, but also their ability to explore a wide range of different resources, as appropriate to their jaw apparatus.

Fetal malformation in a marmoset (Callithrix jacchus): case report

Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)

Congenital bone malformation in a rainbow boa Epicrates cenchria crassus - case report

Bone malformation in snakes stems from inbreeding or temperature variations during gestation or incubation. Differential diagnosis are osteoarthropathy due to nutritional, metabolic and infectious diseases. For an accurate diagnosis of these changes, a radiographic exam is essential because it provides a general view of the skeleton and detects morphological abnormalities. This study aimed to report the clinical care of a rainbow boa (Epicrates cenchria crassus) with congenital kyphosis and changes in scales, from a snake pit with controlled temperature and humidity since its gestation. Multiple dorsal deviations of the column without the presence of osteoarthrosis were seen upon radiographic examination. Studies aiming to understand the cases of congenital malformation in snakes should be performed to prevent the birth of animals with this type of change.

Influence of Estrogen Deficiency on Bone Around Osseointegrated Dental Implants: An Experimental Study in the Rat Jaw Model

Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)

Effects of Maternal Pb2+ poisoning during pregnancy on the development of the jaw (Meckel´s Cartilage) of rat fetus

The purpose of this study was to investigate the effects of maternal lead poisoning during pregnancy on the development of the jaw (Meckel's cartilage) of rat fetuses by histologic and morphometric methods. Pregnant rats received a single intraperitoneal injection of 2.5 mg of lead acetate/100g body weight on the 10th day of pregnancy. Meckel's cartilage of fetuses of the lead-treated group showed smaller volume density and size of the lacunae, as well as modification of the lacunae shape. Moreover, the number density of lacunae and the volume density of the matrix increased significantly in the Meckel's cartilage in treated group fetuses. The results suggest that lead poisoning during the period of organogenesis can induce disturbances in the development and differentiation of the fetal stomatognathic system. Reducing the consumption of alcoholic beberages and smoking cessation by women in childbearing age, along with a strict policy of control of the environmental lead exposure can bring great benefits to the future generations of children.

Time series analysis of jaw muscle contraction and tissue deformation during mastication in miniature pigs

Masticatory muscle contraction causes both jaw movement and tissue deformation during function. Natural chewing data from 25 adult miniature pigs were studied by means of time series analysis. The data set included simultaneous recordings of electromyography (EMG) from bilateral masseter (MA), zygomaticomandibularis (ZM) and lateral pterygoid muscles, bone surface strains from the left squamosal bone (SQ), condylar neck (CD) and mandibular corpus (MD), and linear deformation of the capsule of the jaw joint measured bilaterally using differential variable reluctance transducers. Pairwise comparisons were examined by calculating the cross-correlation functions. Jaw-adductor muscle activity of MA and ZM was found to be highly cross-correlated with CD and SQ strains and weakly with MD strain. No muscle’s activity was strongly linked to capsular deformation of the jaw joint, nor were bone strains and capsular deformation tightly linked. Homologous muscle pairs showed the greatest synchronization of signals, but the signals themselves were not significantly more correlated than those of non-homologous muscle pairs. These results suggested that bone strains and capsular deformation are driven by different mechanical regimes. Muscle contraction and ensuing reaction forces are probably responsible for bone strains, whereas capsular deformation is more likely a product of movement.

Laryngeal malformation in Richieri-Costa Pereira syndrome: New findings

Laryngeal structural anomalies were described in 13 cases of Richieri-Costa Pereira syndrome, and four previously reported cases were reviewed. The 17 individuals examined had the typical laryngeal anomalies and vocal disorders previously described. The new findings are the laryngeal microweb observed in three cases and arytenoid anteriorization movement observed in 14 cases. (C) 2012 Wiley Periodicals, Inc.

Feelings of women regarding end-of-life decision making after ultrasound diagnosis of a lethal fetal malformation

Objective: this study investigated the feelings of women regarding end-of-life decision making after ultrasound diagnosis of a lethal fetal malformation. The aim of this study was to present the decision making process of women that chose for pregnancy termination and to present selected speeches of women about their feelings. Design: open psychological interviews conducted by a psychologist immediately after the diagnosis of fetal malformation by ultrasound. Analysis of the results was performed through a content analysis technique. Setting: the study was carried out at a public university hospital in Brazil. Participants: 249 pregnant women who had received the diagnosis of a severe lethal fetal malformation. Findings: fetal anencephaly was the most frequent anomaly detected in 135 cases (54.3%). Termination of pregnancy was decided by 172 (69.1%) patients and legally authorised by the judiciary (66%). The reason for asking for termination was to reduce suffering in all of them. In the 77 women who chose not to terminate pregnancy (30.9%), the reasons were related to feelings of guilt (74%). Key conclusions: the results support the importance of psychological counselling for couples when lethal fetal malformation is diagnosed. The act of reviewing moral and cultural values and elements of the unconscious provides assurance in the decision-making process and mitigates the risk of emotional trauma and guilt that can continue long after the pregnancy is terminated. (C) 2011 Elsevier Ltd. All rights reserved.

Riabilitazione protesica ed implanto-protesica di pazienti in età evolutiva affetti da Displasia Ectodermica

Introduction. Ectodermal Dysplasias are a heterogeneous group of inherited disorders characterized by dysplasia of tissues of ectodermal origin (hair, nails, teeth, skins and glands). Clinically, it may be divided into two broad categories: the X-linked hypoidrotic form and the hidrotic form. Hypohidrotic Ectodermal Dysplasia (H.E.D) is characterized by the triad oligo-anodontia, hypotricosis, hypo-anhydrosis (Christ-Siemens-Tourane syndrome). The incidence of HED is about 1/100,000. Mutation in the actodysplasin-A (EDA) and ectodysplasin-A receptor (EDAR) genes are responsible for X-linked and autosomal HED. The clinical features include sparse, fine hair, missing or conical-shaped teeth, decreased sweat and mucous glands, hypoplastic skin, and heat intolerance with exercise or increased ambient temperature. Complete or partial anodontia and malformation of teeth are the most frequent dental findings. Incisors and canines are often conical-shaped while primarily second molars, if present, are mostly affected by taurodontism. Treatment is supportive and includes protection from heat exposure, early prosthetic rehabilitation, skin, hair ear, nose and nail care, and genetic counseling for family planning. The diagnosis of HED in the neonatal and early infancy period may be difficult since sparse hair and absent teeth are normal finding at this age. In childhood the diagnosis is more easily made on the basis of history and clinical examination. Dental abnormalities are the most common complaint. Prosthetic rehabilitation has been recommended as an essential part of the management of HED because is important from functional, esthetic, and psychological standpoint. A team approach that includes input from a pediatric dentist, an orthodontist, a prosthodontist, and an oral and maxillofacial surgeon is necessary for a successful outcome. Conventional prosthodontic rehabilitation in young patient is often difficult because of the anatomical abnormalities of existing teeth and alveolar ridges. The conical shaped teeth and “knife-edge” alveolar ridges result in poor retention and instability of dentures. Moreover, denture must permit jaws expansion and a correct pattern of growth. Materials and Methods. Complete removable dentures were provided to allow for normal physiological development and a corrected masticatory function. Initial maxillary and mandibular impressions were made with smallest stock trays and irreversible hydrocolloid and then final impressions ware made with light-bodied polysulfide rubber base impression material. A base of autopolymerizing resin was constructed and a wax rim was added to the base. The patient’s vertical dimension of occlusion was established by assessing phonetic and esthetic criteria. Preliminary occlusal relations were recorded, and the mandibular cast was mounted on the articulator. Acrylic resin teeth specific for children dentures were selected and mounted. The dentures were tried in and, after proper adjustments, were inserted. The patients were monitored clinically every month to fit prostheses. Cephalometric radiographs were taken every 6 month with the prostheses in place in order to evaluate correct pattern of growth. Cephalometric measurements were realized and used to evaluate the effect of rehabilitation on craniofacial growth. Cephalometric measurements of sound patients were compared with ED patients. After two month expander screws (three-way screw in the upper denture and two-way the lower one)were inserted in each denture in order to permit the expansion of the denture and the jaws growth. Where conical teeth were present, composite crown were realized and luted to improve the esthetic and phonesis. In order to improve retention the placement of endosseous implants was carried out. TC 3D Accuitomo was performed and a resin model of mandibular bone of the patient was realized. At the age of 11 years two implants were inserted into anterior mandible in a child with anodontia. Despite a remarkable multi-dimensional atrophy of the mandibular alveolar process, the insertion of two tapered screw implants (SAMO Smiler, diameter 3.8, length 10 mm). After a submerged healing period of two-three month, the implants were exposed. Implants were connected with an expansion guide that permits mandibular growth and prosthetic retention. The amount of mandibular growth was also evaluate dusing the expansion guide. Results. Early oral rehabilitation improve oral function, phonesis and esthetic, reducing social impairment. Treated patients showed normal cephalometric measurement. Early rehabilitation is able to prevent the prognatissm of the mandibula . The number of teeth was significantly related to several changes in craniofacial morphology. Discussion. In the present study the 5,3% of ED patients showed hypodontia, the l’89,4% di oligodontia, and the 5,3% di anodontia. The cephalometric analysis supports that ED patients showed midface hypoplasia. ED groups showed an increased pogonion to nasion measurement than sound patients, indicative of class III tendency. The present study demonstrated that number of teeth was significantly correlated with deviation of cephalometric measurements from normality. Oligoanodontia is responsible for changing of cephalometric measuraments also on sagittal plane with a class III tendency. Maxillary jaw showed a retrused position related to the presence of hypodontia.