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Noonan syndrome is a multiple congenital anomaly syndrome, inherited in an autosomal dominant pattern. We studied 31 patients (18 males and 13 females) affected by this disorder regarding their clinical and genetic characteristics. The most frequent clinical findings were short stature (71%); craniofacial dysmorphisms, especially hypertelorism, ptosis, downslanting of the palpebral fissures; short or webbed neck (87%); cardiac anomalies (65%), and fetal pads in fingers and toes (70%). After studying the probands' first-degree relatives, we made the diagnosis of Noonan syndrome in more than one family member in three families. Therefore, the majority of our cases were sporadic.

Os achados radiográficos e os sinais e sintomas de pacientes com disfunções da articulação temporomandibular (ATM) foram estudados. Através da incidência transcranial para ATM, foram verificadas a presença de alterações degenerativas e a estimativa de excursão condilar. Pelo exame clínico, foram avaliados dados como queixa principal, padrão de ocorrência de dor, sinais de dor muscular e sinais articulares de disfunção. As alterações radiográficas mais freqüentes foram as degenerativas. As alterações degenerativas mais freqüentes foram, em ordem decrescente, facetamento, eburnação e osteofito. As alterações degenerativas foram mais freqüentes no côndilo do que na eminência articular. A estimativa de excursão condilar foi, em ordem decrescente, normo, hiper e hipoexcursão. Não foi observada relação entre achados radiográficos e sinais ou sintomas específicos

Intraventricular hemorrhage (IVH) is a severe complication in very low birth weight (VLBW) newborns (NB). With the purpose of studying the incidence of IVH, the associated risk factors, and the outcomes for these neonates, we studied all the VLBW infants born in our neonatal unit. Birth weight, gestational age, presence of perinatal asphyxia, mechanical ventilation, length of hospitalization, apnea crisis, hydrocephalus, and periventricular leukomalacia were analyzed. The diagnosis of IVH was based on ultrasound scan studies (Papile's classification) performed until the tenth day of life and repeated weekly in the presence of abnormalities. Sixty-seven/101 neonates were studied. The mortality rate was 30.6% (31/101) and the incidence of IVH was 29.8% (20/67) : 70% grade I, 20% grade III and 10% grade IV. The incidence of IVH in NB <1,000 g was 53.8% (p = 0.035) and for gestational age <30 weeks was 47.3% (p = 0.04), both considered risk factors for IVH. The length of hospitalization (p = 0.00015) and mechanical ventilation (p = 0.038) were longer in IHV NB. The IVH NB had a relative risk of 2.3 of developing apnea (p = 0.02), 3.7 of hydrocephalus (p = 0.0007), and 7.7 of periventricular leukomalacia (p < 0.00001). The authors emphasize the importance of knowing the risk factors related to IVH so as to introduce prevention schemes to reduce IVH and to improve outcomes of affected newborns.

Os microrganismos do gênero Candida são, em geral, comensais da cavidade bucal, mas, em determinados indivíduos e em situações específicas, podem transformar-se na forma parasitária, produzindo candidoses bucais. O objetivo deste trabalho foi analisar a presença das espécies do gênero Candida na saliva de indivíduos controle, comparando com pacientes que apresentavam diferentes fatores predisponentes. Foram avaliados 493 pacientes, divididos em 7 grupos: prótese total, prótese parcial removível, periodontite crônica de adulto, respiração bucal, aparelho ortodôntico fixo, aparelho ortodôntico removível e aparelho extrabucal. O grupo controle consistiu de 570 indivíduos que não apresentavam nenhum desses fatores, divididos em 2 grupos: infantil e adulto. Foram realizados o isolamento e a identificação das espécies do gênero Candida. Os resultados mostraram maior porcentagem de pacientes positivos para Candida nos grupos com fatores predisponentes em relação aos controles. Houve predominância de isolamento de C. albicans em todos os grupos, porém, os pacientes com fatores predisponentes apresentaram diversidade maior de espécies

Pouchitis is the most frequent complication of ileal pouch-anal anastomosis for treatment of ulcerative colitis. There are several possible explanations. Among them, we focus on the one that considers pouchitis as an extracolonic manifestation of ulcerative colitis. The aim of this study was to investigate the association between pouchitis and extra-intestinal manifestations (EIM), which are frequent in these patients. Sixty patients underwent restorative proctocolectomy with an ileal J pouch (IPAA) from September 1984 to December 1998. Pouchitis was defined by clinical, endoscopic, and histologic criteria. The following extra-intestinal manifestations were studied: articular, cutaneous, hepatobiliary, ocular, genitourinary, and growth failure. Thirteen patients, of which 10 were female (76.9%), developed one or more episodes of pouchitis. Twelve patients of this group (92.3%) presented some kind of extra-intestinal manifestation, 4 pre-operatively (exclusively), 2 post-operatively (exclusively), and 6 both pre- and post-operatively (1.7 per patient). Twenty patients (42.7%) of the 47 without pouchitis did not present extra-intestinal manifestations; 10/35 (28.5%) of females had pouchitis, compared to 3/35 (12.0%) of men. Pouchitis was more frequent among females, though not statistically significant. EIM increases the risk of pouchitis. Pouchitis is related to EIM in 92.3 % of cases, corroborating the hypothesis that it could be an extracolonic manifestation of ulcerative colitis.

A fluoretação das águas de abastecimento público é considerada o método de prevenção de cáries mais efetivo em termos de abrangência coletiva. Além disso, trata-se de uma medida simples, econômica, segura e eficaz. Graças a essa medida, associada ao uso de dentifrícios fluoretados e aos programas preventivos implantados nos municípios, o país experimenta uma redução da prevalência de cárie. Este trabalho tem por objetivo avaliar a evolução das prevalências de cárie dentária nos anos de 1971 (início da fluoretação das águas de abastecimento público), 1977, 1980, 1992 e 1996, na cidade de Piracicaba, SP, Brasil. Comparando-se as prevalências de cárie em escolares de 7 a 12 anos, de ambos os sexos, nos anos de 1971 a 1996, verificou-se uma redução do índice CPOD de 79%. Foi observado que, no levantamento epidemiológico de 1996, o índice CPOD foi igual a 2,0 para os 12 anos de idade, superando as metas da OMS para o ano 2000. Verificou-se uma redução de cárie estatisticamente significante (no nível de 5%), ao se compararem as prevalências em escolares de Piracicaba nos últimos 25 anos de fluoretação das águas de abastecimento. Busca-se discutir os resultados desses levantamentos com os de outras localidades, bem como fazer uma análise em relação às recomendações da OMS para o ano 2000 e 2010.

Abdominal tumors that can grow through vascular lumen and spread to the right heart are rare. Although these tumors have different histologic aspects, they may cause similar abdominal and cardiac symptoms and are a serious risk factor for pulmonary embolism and sudden death when they reach the right atrium and tricuspid valve. The best treatment is radical surgical resection of the entire tumor using cardiopulmonary bypass with or without deep hypothermia and total circulatory arrest. We report the cases of two patients, the first with leiomyosarcoma of the inferior vena cava and the other with intravenous leiomyomatosis of the uterus that showed intravascular growth up to right atrium and ventricle, who underwent successful radical resection in a one-stage procedure with the use of cardiopulmonary bypass. We discuss the clinical and histologic aspects and imaging diagnosis and review the literature.

O estudo das imagens do dente em tratamento endodôntico e das suas estruturas anexas favorece a identificação precoce das alterações capazes de comprometer o prognóstico de sucesso. A perfeita informação radiográfica e a correta interpretação comprovadamente orientam a conduta terapêutica, estreitando a relação entre a cura e a patologia. Assim sendo, e devido à importância da fidelidade desse procedimento, os autores apresentam um dispositivo que, acoplado a qualquer posicionador comercializado, possibilita a tomada de imagens semelhantes sob o aspecto das suas dimensões. A importância desse fato surgiu da necessidade do confronto das imagens obtidas entre as sessões do tratamento. Os estudos clínicos indicaram que o posicionador personalizado obtido através de moldagem com material pesado favoreceu sensivelmente as análises comparativas das áreas da superfície radicular externa acometidas de reabsorção cemento-dentinária, como seqüela de trauma dental, bem como as regiões portadoras de rarefação óssea periapical no decorrer da avaliação da neoformação.

Primary biliary cirrhosis (PBC) is a cholestatic liver disease, which is characterized by a chronic inflammatory destruction of intrahepatic bile ducts. It is a rare disorder whose precise etiology is still to be elucidated. Even though the liver is the principal target of PBC, other organ systems also might be affected. Muscular involvement has rarely been described in this disease, and in the majority of cases, muscular weakness has been interpreted as polymyositis. We report the case of a 48-year-old woman suffering from classic PBC, in association with a myopathy whose histological features are distinct from the cases reported before. We also performed a MEDLINE research for PBC and concomitant muscular diseases.

Foi avaliado o poder antimicrobiano dos cimentos Endomethasone, AH26, Sealer 26, Sealer 26 acrescido de 5% de hexametilenotetramina, Sealer 26 acrescido de 10% de hexametilenotetramina, Sealapex e pasta aquosa de hidróxido de cálcio. Foram utilizados o método de difusão radial em placas ágar escavadas e cepas puras de microorganismos, sendo que as leituras foram efetuadas após 24 e 48 horas de incubação em aerobiose e microaerofilia. Os resultados mostraram que o Endomethasone apresentou os maiores halos de inibição do crescimento bacteriano e que o acréscimo de hexametilenotetramina acarretou um aumento nos halos proporcionados pelo Sealer 26. O cimento Sealapex e a pasta de hidróxido de cálcio não inibiram os microorganismos testados

Although oportunistic fungal infections occur commonly in immunocompromised hosts, mycetoma has never been reported in association with HIV infection. The authors present a case that to their knowledge is the first reported case of mycetoma associated with HIV infection. Diagnosis was confirmed by direct examination of grains and histologic examination. Precise identification of the agent, an actinomycete, was not possible. The unusual site of infection may probably be related to the use of contaminated needless and sirynges for HIV drug injection.

Com o objetivo de verificar a imagem do cirurgião-dentista, os autores desenvolveram um estudo qualitativo na cidade de Belo Horizonte, no ano de 1994. Foram realizadas 80 entrevistas, com um roteiro aberto e uma amostra de conveniência, em duas etapas. A 1ª etapa era composta de 40 pessoas na faixa etária de 20-30 anos (10 homens e 10 mulheres com até o 1º grau completo e 10 homens e 10 mulheres cursando, no mínimo, o 3º grau) e a 2ª etapa, composta de 40 pessoas na faixa etária acima de 50 anos (10 homens e 10 mulheres com até o 1º grau completo e 10 homens e 10 mulheres cursando, no mínimo, o 3º grau). Os dados colhidos foram analisados segundo a Técnica de Análise de Conteúdo. O medo e a dor estavam fortemente associados à imagem do dentista, sendo relacionados ao instrumental e ao tratamento. Em alguns relatos de pessoas de 20-30 anos, o cirurgião-dentista apresentou uma imagem negativa, aparecendo como um carrasco, um castigo; em contrapartida, em outros relatos, ele assumiu características positivas, um profissional da saúde. As pessoas acima de 50 anos destacaram uma evolução do profissional em aspectos técnicos e de relacionamento. Para todas as categorias analisadas, os discursos se assemelham independentemente do sexo e grau de instrução

Ventilator-dependent premature infants are often treated with dexamethasone. Several trials showed that steroids while improve pulmonary compliance and facilitate extubation, some treated infants may have adverse effects, such as alterations of growth curves. We conducted this retrospective study to evaluate the effects of steroids on mechanical ventilation, oxygen therapy, hospital length stay and mortality, in ventilator-dependent infants with bronchopulmonary dysplasia (BPD) (defined as the need of oxygen supplementation at 28 days of life). Twenty-six newborns with BPD were evaluated during 9 -- 42 days postpartum (mean = 31 days) and were divided into two groups: Group I - 14 newborns that did not receive dexamethasone, and Group II - 12 newborns that received dexamethasone at 14 --21 days of life. Dexamethasone was given at a dose of 0.25 mg per kilogram of body weight twice daily intravenously for 3 days, after which the dose was tapered. RESULTS: There were no statistically significant differences in the mean length of mechanical ventilation (Group I - 37 days, Group II - 35 days); oxygen supplementation (Group I - 16 days, Group II - 29 days); hospital stay (Group I - 72 days, Group II - 113 days); mortality (Group I - 35.7%, Group II - 41.6%). At birth, Group II was lighter (BW: Group I - 1154 grams ± 302, Group II - 791 grams ± 165; p < 0.05) and smaller (height: Group I - 37.22 cm ± 3.3, Group II - 33.5 ± 2.4; p< 0.05) than Group I. At 40 weeks, there were no statistically significant differences between groups in relation to anthropometric measurements. CONCLUSIONS: The use of corticosteroids in bronchopulmonary dysplasic infants may influence the somatic growth during its use. However, after its suspension, a recovery seems to occur, suggesting that its influence could be transitory.

The purpose of this study was to determine, through the shear bond strength of in vitro tests, that the type of glass-ionomer cements (conventional or hybrid) and dentin depth (superficial or deep) are factors that may influence the adhesion of these materials to the dentin structure. Specimens of two conventional glass-ionomer cements (Vidrion R® - SS White and Chelon Fil®- Espe) and a hybrid-glass ionomer cement (Vitremer® - 3M) were separated in groups and prepared for the shear bond strength test. The results submitted to statistical analysis were (all values are in MPa): Group I - Vidrion R - superficial dentin 1.97 (<FONT FACE="Symbol">±</FONT> 0.56); deep dentin 3.15 (<FONT FACE="Symbol">±</FONT> 1.51); Group II - Chelon Fil - superficial dentin 2.43 (<FONT FACE="Symbol">±</FONT> 1.43); deep dentin 3.21 (<FONT FACE="Symbol">±</FONT> 0.89); and Group III - Vitremer - superficial dentin 7.04 (<FONT FACE="Symbol">±</FONT> 2.04); deep dentin 10.30 (<FONT FACE="Symbol">±</FONT> 1.99). There were significant differences between dentin depth and type of materials

Cylindrical specimens of bone measuring 15 mm in diameter were obtained from the lateral cortical layer of 10 pairs of femurs and tibias. A central hole 3.2 mm in diameter was drilled in each specimen. The hole was tapped, and a 4.5 mm cortical bone screw was inserted from the outer surface. The montage was submitted to push-out testing up to a complete strip of the bone threads. The cortical thickness and rupture load were measured, and the shear stress was calculated. The results were grouped according to the bone segment from which the specimen was obtained. The results showed that bone cortex screw holding power is dependent on the bone site. Additionally, the diaphyseal cortical bone tissue is both quantitatively and qualitatively more resistant to screw extraction than the metaphyseal tissue.

A evolução dos preparos de classe II com a introdução de novos materiais e a conscientização da importância da preservação de estrutura dental sadia são abordadas nos preparos do tipo Almqvist, Roggenkamp e túnel, em que se observa a necessidade de menor desgaste da estrutura dentária, com aumento da resistência à fratura, melhor estética e melhor retenção

METHOD: Eighty patients were prospectively randomized for precolonoscopic cleansing either with 750 ml of 10% mannitol (Group M) or 180 ml of a sodium phosphate preparation (Group NaP). Laboratory examinations before and after preparation on all patients included hemoglobin, hematocrit, sodium, potassium, phosphorous, calcium and serum osmolarity. A questionnaire was used to assess undesirable side effects and patient tolerance to the solution. The quality of preparation was assessed by the endoscopist who was unaware of the solution employed. RESULTS: Statistically significant changes were verified in serum sodium, phosphorous, potassium and calcium between the two groups, but no clinical symptoms were observed. There were no significant differences in the frequency of side effects studied. Six of the eight patients in Group NaP who had taken mannitol for a previous colonoscopy claimed better acceptance of the sodium phosphate solution. The endoscopic-blinded trial reported excellent or good bowel preparation in 85% prepared with sodium phosphate versus 82.5% for mannitol (p=0.37). CONCLUSIONS: Quality of preparation and frequency of side effects was similar in the two solutions. The smaller volume of sodium phosphate necessary for preparation seems to be related to its favorable acceptance. Nevertheless, the retention of sodium and phosphate ions contraindicates the use of sodium phosphate in patients with renal failure, cirrhosis, ascites, and heart failure.

Os autores testaram a resistência ao cisalhamento de dentes submetidos a duas técnicas de clareamento. Foram utilizados 50 incisivos centrais superiores, divididos em 5 grupos, a saber: controle (só abertura coronária), clareamento sem calor, clareamento catalisado por calor controlado, clareamento catalisado por calor controlado e restauração com resina composta de última geração e dentes hígidos. Após o teste de cisalhamento, constatou-se que, nos dentes submetidos ao clareamento dental com perborato de sódio/Peridrol com ou sem aplicações de calor controlado, apesar de estes apresentarem leve diminuição da resistência ao cisalhamento, esta não é estatisticamente significante quando comparada com a dos dentes em que se executa o acesso endodôntico sem clareamento. A restauração do dente após o clareamento dental com adesivo dentinário de 4ª geração e resina fotopolimerizada aumenta a resistência do elemento dental clareado. Não existem diferenças estatísticas de resistência ao cisalhamento entre dentes clareados sem o uso de calor e aqueles em que o calor foi empregado de forma controlada, como preconizado neste estudo. Dentes hígidos apresentam resistência maior do que os dentes clareados com calor, sem calor e aqueles não clareados, com significância estatística no nível de 5%. Já com os dentes clareados e restaurados, os dentes hígidos não mostraram diferenças estatísticas significantes

The size of gastroesophageal varices is one of the most important factors leading to hemorrhage related to portal hypertension. An endoscopic evaluation of the size of gastroesophageal varices before and after different operations for portal hypertension was performed in 73 patients with schistosomiasis, as part of a randomized trial: proximal splenorenal shunt (PSS n=24), distal splenorenal shunt (DSS n=24), and esophagogastric devascularization with splenectomy (EGDS n=25). The endoscopic evaluation was performed before and up to 10 years after the operations. Variceal size was graded according to Palmer's classification: grade 1 -- up to 3 mm, grade 2 -- from 3 to 6 mm, grade 3 -- greater than 6 mm, and were analyzed in four anatomical locations: inferior, middle or superior third of the esophagus, and proximal stomach. The total number of points in the pre-operative grading minus the number of points in the post-operative grading gave a differential grading, allowing statistical comparison among the surgical groups. Good results, in terms of disappearance or decrease of variceal size, were observed more frequently after PSS than after DSS or EGDS - 95.8%, 83.3%, and 72%, respectively. When differential grading was analyzed, a statistically significant difference was observed between PSS and EGDS, but not between proximal and distal splenorenal shunts. In conclusion, shunt surgeries were more efficient than devascularization in diminishing variceal size.