992 resultados para Síndrome de Prader-Willi
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Introduction: The HELLP syndrome is a severe complication of pregnant women with preeclampsia (PE), characterized by association of hemolysis, changes in liver enzymes and thrombocytopenia. Hemolysis, defined by the presence of microangiopathic hemolytic anemia, is one of the characteristics in this syndrome. However, as hemolysis occurs in a short time there is some difficulty in its laboratory diagnosis. Therefore, the search for a more sensitive and specific method for hemolysis determination may help in the early diagnosis of the HELLP syndrome. Objectives: a) To determine the plasma concentration of haptoglobin in normotensive pregnant women and in pregnant women with PE, classified into mild PE, severe PE and HELLP/partial HELLP syndrome; b) To compare the efficacy of haptoglobin plasma concentration and serum total bilirubin as criteria for hemolysis diagnosis in HELLP/partial HELLP syndrome. Methods: We conducted a cross-sectional analytical and comparative study involving 66 pregnant women diagnosed with PE, being 25 cases with mild PE, 28 with severe PE, and 13 with HELLP/partial HELLP syndrome. Twenty-one normotensive pregnant women were included for comparison of haptoglobin plasma concentration between the groups and to determine the normal values for pregnant women. The variables studied were: maternal age, gestational age, systolic and diastolic blood pressure, proteinuria, hematocrit and hemoglobin values, platelet count, serum total bilirubin, lactate dehydrogenase (LDH), glutamic oxaloacetic transaminase (AST) and glutamic-pyruvic transaminase (ALT), urea, creatinine and uric acid, and also plasma concentrations of haptoglobin. The results were analyzed by nonparametric tests, with a significance level of 5%. Results: The values of urea, uric acid, AST, ALT and LDH were significantly higher, while the number of platelets was lower in pregnant women with HELLP/partial HELLP syndrome compared to pregnant women with mild PE and ...
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The Combination Syndrome represents an important pathologic condition of the masticatory system that requires a complex rehabilitation treatment. The presence of flaccid mucosa is one of the most common features due to bone resorption of the pre-maxilla caused by anterior hyperfunction of the mandibular teeth on the maxillary complete denture, where the impact of oclusal loading in this region results in movement of the maxillary prosthesis into the ridge, resulting in increased resorption of the residual ridge, internal misfit and loss of the retention of the maxillary complete denture. The presence of flaccid mucosa, in such cases, leads to problems of support and stability of the maxillary complete denture, which can be treated by surgical reduction of the thickness of the tissue or using specific techniques of fabrication of prostheses. This article describes the oral rehabilitation of a patient with the Combination Syndrome where the surgical reduction of flaccid mucosa was not performed, using a modified technique of functional impression in two steps, with the objective of generating forces and less distortion in flaccid mucosa during functional impression.
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Hypohidrotic ectodermal dysplasia (HED) is a rare disease of genetic etiology. The most frequent form is of recessive linked to X-chromosome inheritance with affected male and female carriers. It can occur through autosomal mutations, of the gene EDA1 gene being responsible for the majority of the cases. It is characterized by the triad: hypohidrosis, oligodontia and hypotrichosis. We present two cases of patients with HED in which we observed characteristic signs of this syndrome: delicate skin, sparce hair, eyebrows and eyelashes, periorbital wrinkles, perioral and periorbital hyperpigmentation, prominent lips, in addition the patient in case 2 also present the depressed nasal bridge. We also found decreased salivary and lacrimal secretion and maxillary hypoplasia in both cases. At the oral examination in case 1 the upper right and left deciduous canines and lower right deciduous canine were present, and in case 2 the upper and lower (right and left) deciduous canines and two upper (one right and other left) permanent incisors were present with altered morphology, all of these dental elements were healthy. The early dental treatment of patients with HED, especially in the presence of oligodontia, as observed in our cases, is important not only to provide a better quality of life for these patients in the short term, but also an attempt to minimize the changes in facial growth to which these patients are subject.
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Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)
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Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)
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Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)
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The burning mouth syndrome (BMS) is a relevant pathology that has been defined as a chronic pain with difficult diagnosis and treatment. The prevalence of BMS is estimate to be 15% of the general population, being more common in the elderly and middle-aged. Characterized by a burning sensation in more than one area, more frequently on the two thirds of the tongue, on the anterior half of the hard palate and on the mucosa of the upper lip, the BMS is associated with clinical signs of normality and normal laboratory findings. For several studies the BMS etiology is controversial because it is multifactorial since it can be caused by local, neuropathic, psychological and/or systemic factors. These controversies make the diagnosis more difficult, especially if the dentist does not have knowledge about the syndrome. Thus, the aim of this paper is to present, by means of a literature review, the main features of BMS, indicating the procedures that may be useful for diagnosis, and also the available therapeutic modalities in order to increasing the knowledge of health professionals so that patients with BMS may benefit from appropriate treatment, support and professional confidence, always aiming to better quality of life.
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Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)
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Coordenação de Aperfeiçoamento de Pessoal de Nível Superior (CAPES)
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Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)
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To establish guidelines based on scientific evidence for the management of periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome. The Guideline was prepared from 5 clinical questions that were structured through PICO (Patient, Intervention or indicator, Comparison and Outcome), to search in key primary scientific information databases. After defining the potential studies to support the recommendations, these were graduated considering their strength of evidence and grade of recommendation. 806 articles were retrieved and evaluated by title and abstract; from these, 32 articles were selected to support the recommendations. 1. PFAPA is a diagnosis of exclusion established on clinical grounds, and one must suspect of this problem in children with recurrent and periodic febrile episodes of unknown origin, or with recurrent tonsillitis interspersed with asymptomatic periods, especially in children in good general condition and with preservation of weight and height development; 2. Laboratory findings are nonspecific. Additional tests do not reveal pathognomonic changes; 3. The evidence supporting an indication for surgical treatment (tonsillectomy with or without adenoidectomy), is based on two non-blinded randomized clinical trials with small numbers of patients; 4. The use of prednisone at the onset of fever in patients with PFAPA proved to be an effective strategy. There is still need for more qualified evidence to support its use in patients with PFAPA; 5. Despite promising results obtained in studies with IL-1ß inhibitors, such studies are limited to a few case reports.
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Pós-graduação em Saúde Coletiva - FMB
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The aim of this research was to verify the accuracy of the prediction trace. Records in 15 patients admitted for orthognathic surgical treatment were analysed. Predictive and postoperative positíons of maxilla were compared with linear measurements. Statistically significant difference between predicted and postoperative position were demonstrated, but the prediction trace revealed to be very useful to help in decision of direction of movements, easy to do at low cost
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Pós-graduação em Bases Gerais da Cirurgia - FMB
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Coordenação de Aperfeiçoamento de Pessoal de Nível Superior (CAPES)