653 resultados para spondylothoracic dysplasia


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A síndrome de Goldenhar é uma anomalia congênita rara, de etiologia ainda desconhecida e caracterizada por uma tríade clássica de alterações oculares, auriculares e vertebrais. FORMA DE ESTUDO: Estudo de série. MATERIAL E MÉTODO: Este trabalho foi realizado com 30 indivíduos portadores da síndrome de Goldenhar regularmente matriculados no HRAC, de ambos os gêneros, com faixa etária variando de 8 a 34 anos de idade. OBJETIVO: foi caracterizar o perfil audiológico dos indivíduos portadores dessa síndrome, garantindo assim um melhor tratamento e orientação para os mesmos, assim como também estabelecer a freqüência do comprometimento auditivo contralateral nos indivíduos com o clássico envolvimento unilateral. A avaliação audiológica do estudo constou de ATL, timpanometria, EOA-T e BERA. RESULTADO: de acordo com os resultados concluímos que 34% (=10) dos indivíduos apresentaram como característica de seu perfil audiológico perda auditiva do tipo sensório-neural mista com grau variando de moderado a profundo (7 uni e 3 bilaterais); 13% (n=4) apresentaram perda do tipo condutiva (bilateralmente) com grau de leve a severo e 3% (n=1) apresentaram ou perda do tipo sensório-neural profundo unilateral. Encontramos 40% (n=12) com audição normal bilateralmente e em 10% (n=3) não foi possível estabelecer a característica do perfil audiológico por utilizarmos como avaliação apenas o BERA como pesquisa de limiar eletrofisiológico. Dos doze (12) indivíduos com malformação de OE unilateral encontramos apenas dois (02) com comprometimento auditivo na orelha contralateral, sendo um do tipo misto de grau severo e um condutivo de grau moderado. Com relação a variável sexo encontramos predominância maior da síndrome de Goldenhar no gênero feminino (57%) do que no masculino (43%), mas considerado estatisticamente sem significância, assim como também o lado anatomicamente afetado, que foi predominantemente o direito.

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Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq)

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The prostate of the female gerbil (Meriones unguiculatus) is similar to the human female prostate (Skene gland) and, despite its reduced size, it is functional and shows secretory activity. However, virtually nothing is known about its physiological regulation. This study was thus undertaken to evaluate the behavior of the gerbil female prostate in a hyperandrogenic condition. Adult females received subcutaneous injections of testosterone cypionate (1 mg/kg body weight every 48 h) up to 21 days. Circulating levels of testosterone and estradiol were monitored, and the prostate and ovaries subjected to structural and immunocytochemical analyses. The treatment resulted in sustained high levels of circulating testosterone, and caused a transient increase in estradiol. There was an increase in epithelial cell proliferation accompanied by significant reorganization of the epithelium and an apparent reduction in secretory activity, followed by a progressive increase in luminal volume density and accumulation of secretory products. Immunocytochemistry identified the expression of androgen receptor and a prostate-specific antigen (PSA)-related antigen in prostatic epithelial cells. A circulating PSA-related antigen was also found, and its concentration showed strong negative correlation with circulating estrogen. Epithelial dysplasia was detected in the prostate of treated females. Analysis of the ovaries showed the occurrence of a polycystic condition and stromal cell hyperplasia. The results indicate that testosterone has a stimulatory effect on the female prostate, inducing epithelial cell proliferation, differentiation, secretory activity, and dysplasia. The results also suggest that prostatic growth and activity, polycystic ovaries, and ovarian stromal cell hyperplasia are related to a hyperandrogenic condition in females.

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Abnormalities in any component of the cell cycle regulatory machine may result in oral. cancer, and markers of cell proliferation have been used to determine the prognosis of tumor progression. The aim of this study was to determine whether silver-stained nucleolar organizer region (AgNOR) and Ki-67 measurements could improve the assessment of growth rates in oral lesions. Eighty-three oral biopsies were studied, 20 of which were classified as fibrous inflammatory hyperplasia (FIH), 40 as leukoplakia (LKP) and 23 as oral. squamous cell carcinoma (OSCC). Within the LKP group, 22 out of 29 biopsies were diagnosed as non-dysplastic leukoplakia (LK) and 18 as dysplastic teukoptakia (DLK), presenting discrete, moderate and severe dysplasia. Ki-67 immunotabeting of the lesions increased steadily in the following order: FIH, DLK, LK and OSCC, indicating that Ki-67 is a good marker for predicting the protiferative fraction among benign, premalignant and malignant oral lesions. The median values of AgNOR parameters indicate that the morphometric index gives better results regarding the proliferative rate than the numerical one. A series of linear regressions between AgNOR parameters and Ki-67 showed positive associations. We conclude that a combination of Ki-67 and morphometric AgNOR analyses could be used as an aid in the determination of the protiferative status of oral epithelial. cells in oral cancer. (C) 2007 Elsevier GmbH. All rights reserved.

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The effect of ascorbic acid deficiency was determined in Piaractus mesopotamicus Holmberg, 1887, fish (pacu) under laboratory conditions. A total of 120 fish with an average body weight of 8.64 +/- 1.62 g and measuring 6.15 +/- 0.33 cm in length at the beginning of the experiment were fed diets containing 0, 50, 100 or 200 mg palmitate-coated ascorbic acid/kg dry ration for a period of 24 weeks with measurements every 4 weeks. The experiment was conducted in 20 fiber-cement aquaria of 81-liter capacity. Each aquarium was supplied with dechlorinated water at a flow rate of 1 l/min. Water temperature was measured daily and pH, dissolved oxygen, alkalinity and water conductivity were determined weekly. A fully randomized experimental design was utilized, with 5 replicates of each treatment and 6 fish per aquarium. Ascorbic acid-supplemented fish presented significantly increased growth when compared to unsupplemented fish. Furthermore, unsupplemented fish presented a higher incidence of hyperplasia, hypertrophy and dysplasia of the bone cartilage of gill filaments. The gill lamellae of unsupplemented fish had twisted cartilage and an inflammatory infiltrate at the ends. Anorexia and increased handling stress were also observed in fish fed the unsupplemented diet. The present study suggests that 50 mg ascorbic acid/kg dry ration is sufficient to improve development of pacu fingerlings but the optimum level under aquarium conditions, determined by regression analysis, is 139 mg ascorbic acid/kg dry ration.

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Background: Canine hip dysplasia (HD) is characterized by hip joint laxity and subluxation. It is the most common cause of osteoarthritis in dogs, especially in larger breeds. Its management includes nutritional supplements, nonsteroidal anti-inflammatory drugs, physical therapy, acupuncture or surgical procedures. Implantation of gold beads in acupuncture points and trigger points around a joint has been used in the treatment of osteoarthritis in dogs for at least 30 years. Gold bead implants(GBI) acts as continuous acupuncture stimulation and trigger point treatment in canine HD with long lasting results. Electrophysiological investigations of trigger points reveal dysfunctional muscle spindles which indicate that the electrical activity of active loci arises from extrafusal motor endplates.Case: This is a report on the use of acupuncture and GBI for bilateral HD in a nine year old female German Shepherd. The patient has a HD non-responsive to anti-inflammatory drugs and was unable to stand up or walk by its own. Radiographs showed marked dysplasia, significant subluxation with the femoral head partly out of a shallow acetabulum and massive secondary arthritic bone changes, mainly on the right side. The animal was submitted to eight acupuncture sessions with seven days interval. After the first acupuncture session the use of NSAID was interrupted. After eight weeks the dog was considered rehabilitated and underwent GBI in acupoints and trigger points as maintenance treatment. During the one-year follow-up period the improvement remained unchanged with no need of analgesics.Discussion: It has been suggested that acupuncture or GBI can treat the chronic pain resulting from osteoarthritis induced by HD. According to AP theory, GBI is permanent and long-lasting acupoint stimulation. Moreover, the method is inexpensive, quick and easy to perform, with no postoperative pain or need of exercise restriction. Although gold is extremely corrosion-resistant, the surface of the gold implants stimulates a reaction from the immune system causing an oxidative liberation of gold ions with anti-inflammatory actions. It is well known that gold ions are effective inhibitors of the respiratory burst of neutrophils and monocytes and the proliferation of lymphocytes. These findings suggest that gold implantation, on a local scale, mimics the anti-inflammatory and pain-relieving effect of drugs with chemically bound gold ions. The relatively slow speed of the process results in a limited liberation of gold ions securing that they are taken up almost exclusively by cells close to the implant. The nine year old female German shepherd had a positive response to acupuncture with pain relieve and locomotor rehabilitation. For the nine year old female German shepherd previous acupuncture sessions to GBI resulted in no post-implant worsening period. Indeed, the association acupuncture/GBI does not have the anti-inflammatory drugs undesirable effects and brings long lasting results. In conclusion, GBI therefore should be considered for canine HD when conservative or medical treatments fail to give the desired effect.

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We report on a Brazilian child with typical manifestations of the FG syndrome. Pigmentary dysplasia, metacarpal fusion and peculiar anatomopathological findings are additional undescribed signs.

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A syndrome--incontinentia pigmenti--of probable genetic etiology, is discussed and a new case report presented. The syndrome presents systemic and dental manifestations, which are separate although similar to those found in other disease entities, i.e., congenital syphilis, hereditary ectodermal dysplasia.

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Albright's syndrome is characterized by the presence of polyostotic fibrous dysplasia, endocrinopathies and brown spots on the skin. In the present article the authors describe a case occurring in a 20-year-old female patient, who is currently being followed radiographically after a mandibular bone biopsy.

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OBJECTIVE: To evaluate the discrepancy index between the clinical and histological diagnosis and the prevalence of epithelial dysplasia and carcinoma in 45 patients with potentially malignant epithelial oral lesions (PMEL). PATIENTS AND METHODS: We submitted 45 patients with PMEL to clinical examination and obtained a biopsy from each. The results of histological diagnosis were compared to the clinical diagnosis. RESULTS: Clinical diagnosis showed that the most common PMEL was leukoplakia followed by lichen planus and by actinic cheilitis associated with leukoplakia. The most common site was the buccal mucosa. Histological diagnosis revealed that 46.7% of the PMEL were lichen planus. The discrepancy index between clinical and histological diagnosis was 24.4%. The higher discrepancy index occurred among leukoplakias. The prevalence of epithelial dysplasia and carcinoma was 17.8%. CONCLUSIONS: We conclude that all PMEL should be submitted to a microscopic analysis because the discrepancy between clinical and histological diagnosis was present in a quarter of these lesions. Otherwise, the epithelial dysplasia and carcinoma were more frequent in the leukoplakias.

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The expression of uroplakins, the tissue-specific and differentiation- dependent membrane proteins of the urothelium, was analyzed immunohistochemically in N butyl-N-(4-hydroxybutyl)nitrosamine (BBN)-treated rats and mice during bladder carcinogenesis. Male Fischer 344 rats were treated with 0.05% BBN in the drinking water for 10 wk and were cuthanatized at week 20 of the experiment. BBN was administered to male B6D2F1 mice; it was either provided at a rate of 0.05% in the drinking water (for 26 wk) or 5 mg BBN was administered by intragastric gavage twice weekly for 10 wk, followed by 20 wk without treatment. In rats, BBN-induced, noninvasive, low grade, papillary, transitional cell carcinoma (TCC) showed decreased uroplakin-staining of cells lining the lumen but showed increased expression in some nonluminal cells. In mice, nonpapillary, high-grade dysplasia, carcinoma in situ, and invasive carcinoma were induced. There was a marked decrease in the number of uroplakin-positive cells lining the lumen and in nonluminal cells. This occurred in normal-appearing urothelium in BBN-treated mice and in dysplasic urothelium, in carcinoma in situ, and in invasive TCC. The percentage of uroplakin-positive nonluminal cells was higher in control mice than in rats, but it was lower in the mouse than in the rat after BBN treatment. Uroplakin expression was disorderly and focal in BBN-treated urothelium in both species. These results indicate that BBN treatment changed the expression of uroplakins during bladder carcinogenesis, with differences in rats and mice being related to degree of tumor differentiation.

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The aim of this literature review is to discuss the use of dental implants in growing patients and the influence of maxillary and mandibular skeletal and dental growth on the stability of those implants. It is recommended to wait for the completion of dental and skeletal growth, except for severe cases of ectodermal dysplasia.

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Albright hereditary osteodystrophy is a hereditary metabolic disorder of dominant autosomal etiology that is commonly characterized by short stature, round face, small metacarpus and metatarsus, mental retardation, osteoporosis, subcutaneous calcification, variable hypocalcemia, and hyperphosphatemia. In this study, we report a clinical case of a 17-year-old woman with Albright hereditary osteodystrophy, and we discuss her clinical, radiographic, and laboratory test characteristics together with the oral manifestations, and we correlate them with the characteristics found in the literature. We also discuss the odontological management of treatment of related periodontal disease and planning for corrections of related malocclusions.

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We describe affected individuals in three generations of a family and another sporadic case, all Brazilian patients, with a combination of signs that diagnose the BCD syndrome. In addition to the cardinal signs, the sporadic case has hypothyroidism and imperforate anus, which was observed previously in one patient. The broadened phenotype and the possibility of involvement of p63 and IRF6 genes in this condition are discussed. © 2003 Wiley-Liss, Inc.

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The prostate is an accessory gland of the mammal reproductive system with great volume and high functional importance. Many works infer that, in addition to the androgenic ones, the estrogen can be associated with benign prostatic hyperplasia and prostatic cancer, but no conclusive evidence exists on the role of estrogen in normal prostatic and neoplastic tissue. The objective of this work was to evaluate the effects of chronic administration of estradiol benzoate on the lateral prostate of guinea pigs in the pre-pubescent, pubescent, post-pubescent and adult phases, with emphasis on the modifications provoked by this hormone on the glandular epithelium. The analyses of the estradiol-treated and control groups were investigated using histological procedures and transmission electron microscopy. The histopathological analysis of the lateral prostate in the treated group revealed areas where epithelial dysplasia was observed, assuming at some places a pattern of epithelial stratification characteristic of prostatic intraepithelial neoplasia. After ultrastructural analysis, the following were observed: enlargement of the internal membranes, heterogeneity in the cellular types, hypertrophy of the basal cells and apparent decrease of cytoplasmic organelles in some cells of the prostatic intraepithelial neoplasia. Still, a loss of cellular polarity was observed, along with nuclei of various forms, sizes and heights - as well as irregular chromatin distribution patterns. Such alterations were found mainly in pubescent, post-pubescent and adult animals subject to the chronic administration of estradiol. These findings reinforce the already existent data in understanding the role of estrogen in the etiology of prostatic diseases.