Clinical, radiographic, biochemical and histological findings of florid cemento-osseous dysplasia and report of a case

A displasia cemento-óssea florida tem sido descrita como uma condição que afeta tipicamente os maxilares de mulheres negras de meia idade. Ela geralmente se manifesta como múltiplas massas radiopacas semelhantes ao cemento distribuídas nos maxilares. Esta condição também tem sido classificada por vários autores como cementoma gigantiforme, osteomielite esclerosante crônica, osteíte esclerosante e massas de cemento escleróticas. Os autores apresentam um caso de displasia cemento-óssea florida não complicada em uma mulher negra de 48 anos de idade. Múltiplas massas escleróticas com bordas radiolúcidas na mandíbula foram identificadas radiograficamente. Os achados histopatológicos revelaram formação de massas escleróticas densas calcificadas semelhantes ao cemento. Todos os aspectos clínicos, radiográficos, bioquímicos e histológicos foram sugestivos do diagnóstico de displasia cemento-óssea florida.

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.

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.

Displasia cemento-óssea florida: relato de caso

The florid cemento-osseous dysplasia is an asymptomatic lesion present in the fibro-osseous maxilla and mandible of uncertain etiology. It has higher expression in females, and patients melanoderm, middle-aged to elderly. This dysplasia is an asymptomatic condition that can be discovered when a radiograph is performed. A biopsy is contraindicated to avoid infection difficult to treat. We report the case of a white woman 52 years old, who searched the Clinic of Surgery and Traumatology Bucco-maxillofacial surgery, Faculty of Dentistry of Araçatuba with pain in the posterior portion of left mandible. After radiographic examination was diagnosed with florid cementoosseous dysplasia. Treatment was instituted clinical and radiographic.

Monostotic Fibrous Dysplasia of the Mandible

Fibrous dysplasia is a benign fibro-osseous disease that affects 1 or more bones. Deformities leading to aesthetic and functional disorders are observed in almost all cases. Plastic surgery is often recommended when the jaws are involved. Monostotic fibrous dysplasia of the mandible is an unusual manifestation of the disease that is usually benign, occurs in young individuals, and is managed by conservative curettage or debridement, such as surgical sculpting. The authors report a case of a 15-year-old patient with a large monostotic fibrous dysplasia located in the right mandible, which was treated by contouring bone. The lesion did not recur on follow-up for 4 years after the surgical procedure.

Tricuspid valve dysplasia and Ebstein's anomaly in dogs: case report

Dois casos de displasia da valva tricúspide são relatados neste trabalho. Os cães foram avaliados devido à fraqueza e presença de ascite. em ambos os casos, o exame ecocardiográfico mostrou insuficiência tricúspide e, em um deles, a inserção dos folhetos da valva tricúspide encontrava-se deslocada para baixo do ventrículo direito, caracterizando a anomalia de Ebstein. A terapia medicamentosa para insuficiência cardíaca congestiva foi iniciada, mas um dos animais veio a óbito subitamente alguns dias após o diagnóstico. O outro cão, apesar de inicialmente ter apresentado melhora significativa do quadro clínico, apresentou morte súbita. A necropsia dos animais revelou dilatação atrioventricular direita e folhetos tricúspides espessados. As características clínicas, métodos de diagnóstico e terapia medicamentosa são discutidas neste artigo.

Allosteric modulation of pyrophosphatase activity of rat osseous plate alkaline phosphatase by magnesium ions

Pyrophosphatase activity of rat osseous plate alkaline phosphatase was studied at different concentrations of calcium and magnesium ions. with the aim of characterizing the modulation of enzyme activity by these metals. In the absence of metal ions, the enzyme hydrolysed pyrophosphate following Michaelian kinetics with a specific activity of 36.7 U/mg and K-0.5 = 88 mu M. In the presence of low concentrations (0.1 mM) of magnesium (or calcium) ions, the enzyme also exhibited Michaclian kinetics for the hydrolysis of pyrophosphate, but a significant increase in specific activity (123 U/mg) was observed. K-m values remained almost unchanged. Quite different behavior occurred in the presence of 2 mM magnesium (or calcium) ions. In addition to low-affinity sites (K-0.5 = 40 and 90 mu M, for magnesium and calcium, respectively), high-affinity sites were also observed with K-0.5 values 100-fold lower. The high-affinity sites observed in the presence of calcium ions represented about 10% of those observed for magnesium ions. This was correlated with the fact that only magnesium ions triggered conformational changes yielding a fully active enzyme. These results suggested that the enzyme could hydrolyse pyrophosphate, even at physiological concentrations (4 mu M), since magnesium concentrations are high enough to trigger conformational changes increasing the enzyme activity. A model, suggesting the involvement of magnesium ions in the hydrolysis of pyrophosphate by rat osseous plate alkaline phosphatase is proposed. (C) 1998 Published by Elsevier B.V. Ltd. All rights reserved.

Inorganic pyrophosphate-phosphohydrolytic activity associated with rat osseous plate alkaline phosphatase

Purified membrane-bound alkaline phosphatase from rat osseous plate hydrolyzed pyrophosphate in the presence of magnesium ions, with a specific activity of 92.7 U/mg. Optimal apparent pH for pyrophosphatase activity was 8.0 and it remained unchanged on increasing the pyrophosphate concentration. In the absence of magnesium ions the enzyme had a K-m = 88 mu M and V = 36.7 U/mg for pyrophosphate and no inhibition by excess substrate was observed. Pyrophosphatase activity was rapidly destroyed at temperatures above 40 degrees C, but magnesium ions apparently protected the enzyme against danaturation. Sodium metavanadate (Ki = 1.0 mM) was a competitive inhibitor of pyrophosphatase activity, while levamisole (Ki = 8.2 mM) and theophylline (Ki = 7.4 mM) were uncompetitive inhibitors. Magnesium ions (K-0.5 = 1.7 mu M) stimulated pyrophosphatase activity, while cobalt (Ki = 48.5 mu M) and zinc (Ki = 22.0 mu M) ions were non-competitive inhibitors. Manganese and calcium ions had no effect on pyrophosphatase activity. The M-w of the pyrophosphatase: protein was 130 kDa by gel filtration, but a value of 65 kDa was obtained by dissociative gel electrophoresis, suggesting that it was a dimer of apparently identical subunits. These results suggested that pyrophosphatase activity stems from the membrane-bound osseous plate alkaline phosphatase and not from a different protein.

Kinetic characterization of a membrane-specific ATPase from rat osseous plate and its possible significance on endochondral ossification

Treatment with phosphatidylinositol-specific phospholipase C of rat osseous plate membranes released up to 90-95% of alkaline phosphatase, but a specific ATPase activity (optimum pH = 7.5) remained bound to the membrane. The hydrolysis of ATP by this ATPase was negligible in the absence of magnesium or calcium ions. However, at millimolar concentrations of magnesium and calcium ions, the membrane-specific ATPase activity increased to about 560-600 U/mg, exhibiting two classes of ATP-hydrolysing sites, and site-site interactions. GTP, UTP, ITP, and CTP were also hydrolyzed by the membrane-specific ATPase. Oligomycin, ouabain, bafilomycin A(1), thapsigargin, omeprazole, ethacrynic acid and EDTA slightly affected membrane-specific ATPase activity while vanadate produced a 18% inhibition. The membrane-specific ATPase activity was insensitive to theophylline, but was inhibited 40% by levamisole. These data suggested that the membrane-specific ATPase activity present in osseous plate membranes, and alkaline phosphatase, were different proteins. (C) 1998 Elsevier B.V. B.V.

Molecular analysis of the PROP1 and HESX1 genes in patients with septo-optic dysplasia and/or pituitary hormone deficiency

OBJETIVO: O presente estudo teve como objetivo avaliar os genes PROP1 e HESX1 em um grupo de pacientes com displasia septo-óptica (DSO) e deficiência hormonal hipofisária (combinada - DHHC; ou deficiência isolada de GH - DGH). Onze pacientes com apresentação clínica e bioquímica consistente com DHHC, DGH ou DSO foram avaliados. SUBJECTS and METHODS: em todos os pacientes, o gene HESX1 foi analisado pelo sequenciamento direto e, nos casos de DHHC, o gene PROP1 foi também sequenciado. RESULTADOS: Um polimorfismo no gene HESX1 (1772 A > G; N125S) foi identificado em um paciente com DSO. Foram encontrados três pacientes portadores da variação alélica 27 T > C; A9A e 59 A > G; N20S no éxon 1 do gene PROP1. Mutações no gene PROP1 e HESX1 não foram identificadas nesses pacientes com DGH, DHHC e DSO esporádicos. CONCLUSÃO: Alterações genéticas em um ou diversos outros genes ou mecanismos não genéticos devem estar implicados nesse processo patogênico.

Conductive hearing loss in children with bronchopulmonary dysplasia: A longitudinal follow-up study in children aged between 6 and 24 months

Aims: To determine the occurrence of isolated and recurrent episodes of conductive hearing loss (CHL) during the first two years of life in very low birth weight (VLBW) infants with and without bronchopulmonary dysplasia (BPD).Study design, subjects and outcome measures: In a longitudinal clinical study. 187 children were evaluated at 6, 9, 12,15 18 and 24 months of age by visual reinforcement audiometry, tympanometry and auditory brain response system.Results: of the children with BPD, 54.5% presented with episodes of CHL, as opposed to 34.7% of the children without BPD. This difference was found to be statistically significant. The recurrent or persistent episodes were more frequent among children with BPD (25.7%) than among those without BPD (8.3%). The independent variables that contributed to this finding were small for gestational age and a 5 min Apgar score.Conclusions: Recurrent CHL episodes are more frequent among VLBW infants with BPD than among VLBW infants without BPD. (C) 2010 Elsevier B.V. All rights reserved.

Collagen dysplasia (cutaneous asthenia) in a cat

Hereditary collagen dysplasias comprise a complex group of connective-tissue disorders that result in the reduced tensile strength of affected tissues. These processes are called cutaneous asthenia in the skin of dogs and cats. We report here the case of a crossbred male cat, aged 6 months, that presented with two skin wounds in the region of the right thorax and right iliac tuberosity. The skin of these regions and of the animal's dorsum was hyperextensible, smooth to the touch, and easily torn with minor trauma. Microscopic examination of skin samples revealed reduced dermal connective tissue consisting of shortened and fragmented collagen fibers. Normal fibers were intermingled with altered fibers. Ultrastructural changes in collagen fibers included disorientation of fibrils within the same bundle, marked spacing differences, and variation in the diameter of transverse sections. The fibrils maintained the transverse striations characteristic of normal collagen.

Acetabular deepening in the treatment of severe canine hip dysplasia

Uma técnica para restituir a anatomia acetabular com aprofundamento da cavidade acetabular e reconstrução do ligamento da cabeça do fêmur e da cápsula articular, foi testada em nove cães, de raças de grande porte, portadores de displasia coxofemoral grave com subluxação acentuada ou luxação. O procedimento cirúrgico foi constituído de duas fases. Inicialmente, foi realizada a pectineotomia bilateral em todos os cães. A segunda intervenção nos mesmos cães incluiu abordagem e aprofundamento do acetábulo, reconstrução do ligamento da cabeça do fêmur e da cápsula. em geral, 30 dias após a cirurgia, os cães apoiavam o membro operado para se locomover. Com exceção de dois cães, todos os outros recuperaram a função locomotora do membro pélvico dentro de 60-90 dias. É concluído que a técnica de acetabuloplastia é uma boa alternativa para o tratamento da displasia coxofemoral grave.

Alveolar osseous defect in rat for cell therapy. Preliminary report

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