Cleidocranial dysplasia: importance of radiographic images in diagnosis of the condition.

Cleidocranial dysplasia (CCD) is a rare syndrome usually caused by an autosomal dominant gene, although 40% of cases of CCD appear spontaneously with no apparent genetic cause. This condition is characterized by several cranial malformations and underdevelopment, absence of the clavicles, and multiple supernumerary and impacted permanent teeth. The diagnosis of this condition is usually based on the presence of the main features (supernumerary teeth, partial or total absence of one or both the clavicles, and bony malformations) and on clinical and familial evidence. The bony and dental features of CCD may be visualized on radiographic images of the face and skull. Here, we present a familial case of CCD and discuss the importance of dental radiographs in diagnosis of the condition.

Florid cemento-osseous dysplasia: A report of three cases

Florid cemento-osseous dysplasia (FCOD) is a non-neoplastic condition of the jaws that Is not associated with inflammation of the pulp or periodontal tissue. This article reports on three cases that were diagnosed as FCOD, demonstrating the importance of both clinical and radiographic diagnosis and the clinical management of these lesions.

Immunohistochemical expression of PCNA, p53, bax and bcl-2 in oral lichen planus and epithelial dysplasia.

The potential for malignant transformation of oral lichen planus is still controversial. The expression of proteins related to cell proliferation and apoptosis in oral lichen planus and epithelial dysplasia was analyzed to evaluate the true potential for malignant transformation of this disease. Twenty-four cases of each lesion were subjected to the streptoavidin-biotin technique for identifying the immunohistochemical expression of PCNA, p53, bax, and bcl-2 proteins. Of the 24 cases of oral lichen planus, 14 (58.33%) were positive for PCNA, 10 (41.67%) for p53, 4 (16.67%) for bcl-2 and 12 (50%) for bax, whereas of the 24 cases of epithelial dysplasia, 20 (83.33%) were positive for PCNA, 10 (41.67%) for p53, 6 (25%) for bcl-2, and 20 (83.33%) for bax. Chi-squared test showed no statistically significant differences between the expression of p53 and bcl-2 in oral lichen planus and epithelial dysplasia, regardless of the grade (P > 0.05). However, the expression of PCNA and bax was significantly increased in epithelial dysplasia (P < 0.05). The results of this study showed that alterations in expression of these proteins are observed in oral lichen planus and epithelial dysplasia, suggesting the potential for malignant transformation in both lesions.

Radiographic study of patients with ectodermal dysplasia and partial

Aim: To investigate the association between gender and type of cleft with hypodontia and to verify if the presence of the cleft interferes with hypodontia of one or more type of teeth in patients with ectodermal dysplasia attending the Hospital for Rehabilitation of Craniofacial Anomalies (HRCA), University of Sγo Paulo (USP). Materials and Methods: Panoramic radiographs of 54 patients of both genders (29 males, 25 females), from the files of the oral radiology sector of HRCA/USP, presenting with ectodermal dysplasia and cleft lip and/or palate were evaluated by radiographic observation of hypodontia of one or more types of teeth. Statistical Analysis: We performed descriptive statistics and statistical analysis by Fisher test. Results: Hypodontia was observed in 50% of females and 50.88% of males; 50% for cleft palate and 50.59% for complete cleft lip and palate. Only 22.22% of patients with cleft palate and 6.67% with complete cleft lip and palate presented with hypodontia of one type of teeth, whereas 77.78% of cases with cleft palate and 93.33% with complete cleft lip and palate displayed hypodontia of more than one type of teeth. Conclusion: Based on the present methodology, there were no statistically significant differences in hypodontia of one or more types of teeth between genders or types of cleft.

Protective effect of gamma-tocopherol-enriched diet on N-methyl-N-nitrosourea-induced epithelial dysplasia in rat ventral prostate

Despite recent advances in understanding the biological basis ofprostate cancer (PCa), the management of this disease remains a challenge. Chemoprotective agents have been usedto protect against or eradicateprostatemalignancies. Here, we investigated the protective effect of -tocopherol on N-methyl-N-nitrosourea (MNU)-induced epithelial dysplasia in the rat ventral prostate (VP). Thirty-two male Wistar rats were divided into four groups (n=8): control (CT): healthy control animals fed a standard diet; control+-tocopherol (CT+T): healthy control animals without intervention fed a -tocopherol-enriched diet (20mg/kg); N-methyl-N-nitrosourea (MNU): rats that received a single dose of MNU (30mg/kg) plus testosterone propionate (100mg/kg) and were fed a standard diet; and MNU+-tocopherol (MNU+T): rats that received the same treatment of MNU plus testosterone and were fed with a -tocopherol-enriched diet (20mg/kg). After 4months, the VPs were excised to evaluate morphology, cell proliferation and apoptosis, as well as cyclooxygenase-2 (Cox-2), glutathione-S-transferase-pi (GST-pi) and androgen receptor (AR) protein expression, and matrix metalloproteinase-9 (MMP-9) activity. An increase in the incidence of epithelial dysplasias, such as stratified epithelial hyperplasia and squamous metaplasia, in the MNU group was accompanied by augmented cell proliferation, GST-pi and Cox-2 immunoexpression and pro-MMP-9 activity. Stromal thickening and inflammatory foci were also observed. The administration of a -tocopherol-enriched diet significantly attenuated the adverse effects of MNU in the VP. The incidence of epithelial dysplasia decreased, along with the cell proliferation index, GST-pi and Cox-2 immunoexpression. The gelatinolytic activity of pro-MMP-9 returned to the levels observed for the CT group. These results suggest that -tocopherol acts as a protective agent against MNU-induced prostatic disorders in the rat ventral prostate.

THE RETINAL TARGETS OF CENTRIFUGAL NEURONS AND THE RETINAL NEURONS PROJECTING TO THE ACCESSORY OPTIC-SYSTEM

In birds, neurons of the isthmo-optic nucleus (ION), as well as ''ectopic'' neurons, send axons to the retina, where they synapse on cells in the inner nuclear layer (INL). Previous work has shown that centrifugal axons can be divided into two anatomically distinct types depending on their mode of termination: either ''convergent'' or ''divergent'' (Ramon y Cajal, 1889; Maturana and Frenk, 1965). We show that cytochrome-oxidase histochemistry specifically labels ''convergent'' centrifugal axons and target neurons which appear to be amacrine cells, as well as three ''types'' of ganglion cells: two types found in the INL (displaced ganglion cells) and one in the ganglion cell layer. Labeled target amacrine cells have distinct darkly labeled ''nests'' of boutons enveloping the somas, are associated with labeled centrifugal fibers, and are confined to central retina. Lesions of the isthmo-optic tract abolish the cytochrome-oxidase labeling in the centrifugal axons and in the target amacrine cells but not in the ganglion cells. Cytochromeoxidase-labeled ganglion cells in the INL are large; one type is oval and similar to the classical displaced ganglion cells of Dogiel, which have been reported to receive centrifugal input; the other type is rounder. Rhodamine beads injected into the accessory optic system results in retrograde label in both types of cells, showing that two distinct types of displaced ganglion cells project to the accessory optic system in chickens. The ganglion cells in the ganglion cell layer that label for cytochrome oxidase also project to the accessory optic system. These have proximal dendrites that ramify in the outer inner plexiform layer. Neither the target amacrine cells nor either of the displaced ganglion cells are immunoreactive for the inhibitory transmitter gamma aminobutyric acid. At least some of the target amacrine cells may, however, be cholinoceptive: we found that the antibody to the alpha-7 subunit of the nicotinic ACh receptor labels a population of cells in the INL that are similar in location, size, and the presence of labeled bouton-like structures to those we find labeled with cytochrome oxidase. This antibody also labels neurons in the ION proper but not ectopic cells. In conclusion, it appears that cytochrome oxidase may be a marker for ''convergent'' centrifugal axons and at least one of their target cells in the INL.

Sonographic and radiographic findings in congenital renal dysplasia in a female Lhasa apso

An 8-month-old Lhasa Apso female dog was referred to the Veterinary Hospital, FMVZ, Unesp, Botucatu Campus, with suspected congenital renal disorder. Ultrasound images revealed higher renal echogenicity, loss of corticomedullary demarcation and diverticular mineralization on the right kidney. The mucosal lining of the stomach was hyperechoic and radiographic examination demonstrated mineralization of wall and folds, which is consistent with uremic gastropathy.

Frontonasal dysplasia: clinical evaluation on audiological and brainstem electrophysiological profiles

Frontonasal dysplasia (FND) is a rare malformative complex affecting the frontal portion of the face, the eyes and the nose; it may occur singly or associated with other clinical signs. No systematic studies describing hearing in this condition were found. AIM: To evaluate hearing sensitivity and sound stimulus conduction from cochlea to brainstem in patients with clinical signs of FND. METHODS: 21 patients with isolated or syndromic FND were submitted to a clinical (otological/vestibular antecedents and otoscopy) and instrumental (pure tone and speech audiometry, tympanometry and brainstem auditory evoked response) hearing evaluation. DESIGN: A clinical, cross-sectional observational prospective study. RESULTS: Hearing thresholds were normal in 15 (70%) patients, abnormal in 5 (25%), mostly with conductive hearing loss; one patient did not cooperate with testing. The tympanometric curve was type A in 30 (72%) ears, type C in 5 (12%), type As in 4 (9%) and type B in 3 (7%). The auditory brainstem response (ABR) showed no abnormalities. CONCLUSION: Patients with FND showed no abnormalities in the auditory system from cochlea to brainstem in this study. Mild conductive hearing loss found in some is probably related to cleft palate. Further evaluation of hearing pathways at higher levels is recommended.

Orthognathic Surgery in Patients With Cleidocranial Dysplasia

The aim of this study was to report the orthodontic-surgical approach of a 21-year-old female patient diagnosed with cleidocranial dysplasia. An orthognathic surgery was performed in the maxilla and mandible during the same procedure to correct an existing dentofacial deformity (class III malocclusion). In addition, malar prostheses were used to correct midface deficiency. After surgical intervention, orthodontic treatment continued in order to promote stability, function, and aesthetics. Cases of cleidocranial dysplasia treated with the defined criteria can bring aesthetic and functional benefits to the patient.

Geometrical parameter analysis of a high-sensitivity fiber optic angular displacement sensor

Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq)