Jacob's Disease: case report / Doença de Jacob: relato de caso

ABSTRACT Jacob's disease or osteochondroma is a rare disease that presents as a pseudo-joint between the coronoid process of the mandible and the posterior surface of the zygomatic bone. The present case report is of an 11-year-old female patient with a maximum mouth opening of 2mm. When evaluating computed tomography with three-dimensional reconstruction, a mushroom-shaped increase in the coronoid process of the mandible on the left side was observed, intimately connected to the zygomatic bone (inferoposterior region of the body of the zygoma), which suggests a pseudoarticulation. Surgical removal of the tumor mass was performed under general anesthesia via intraoral access and is called coronoidectomy. Histopathological, the presence of hyaline cartilage was observed, a condition that is pathognomonic for Jacob's Disease. Jacob's disease case reports are still rare in the literature, with mushroom shape and histopathological confirmation due to the presence of hyaline cartilage.

RESUMO A Doença de Jacob ou osteocondroma é uma doença rara que se apresenta como uma pseudoarticulação entre o processo coronóide da mandíbula e a superfície posterior do osso zigomático. O presente relato de caso é de um paciente, do sexo feminino, com 11 anos de idade e com 2mm de abertura máxima de boca. Ao se avaliar exame de tomografia computadorizada com reconstrução tridimensional foi observado um aumento volumétrico no processo coronóide da mandíbula do lado esquerdo em formato de cogumelo, intimamente ligado ao osso zigomático (região inferoposterior do corpo do zigoma), o que sugere uma pseudoarticulação. A remoção cirúrgica da massa tumoral foi realizada sob anestesia geral por acesso intraoral e é denominada coronoidectomia. Histopatologicamente, observou-se presença de cartilagem hialina, condição é que patognomônica da Doença de Jacob. Ainda são raros os relatos de casos de Doença de Jacob na literatura, com formato de cogumelo e confirmação histopatológica devido à presença de cartilagem hialina.

Cartogenina associada à microperfuração do osso subcondral na reparação da cartilagem articular em equinos / Kartogenin associated to micro of the subchondral bone in the joint cartilage repair in horses

A reconstituição da cartilagem articular danificada por doenças de desenvolvimento, trauma e osteoartrite tem sido um grande desafio na veterinária. O objetivo do presente estudo foi avaliar o uso da cartogenina, uma molécula capaz de induzir diferenciação de células mesenquimais em condrócitos, associado ou não à técnica de microfratura na reparação de defeitos condrais induzidos na tróclea femoral de equinos. Foram utilizados seis equinos pesando em média (±DP) 342±1,58kg, com idade de 7,2±1,30 anos e escore corporal de 7,1±0,75, os quais foram submetidos à videoartroscopia para indução da lesão condral de 1cm2 na tróclea lateral do fêmur e à realização da técnica de microperfuração do osso subcondral de ambos os joelhos. Foram realizadas quatro aplicações semanais com 20µM de cartogenina intra-articular em um dos joelhos (grupo tratado) e solução de ringer com lactato na articulação contralateral (grupo controle). Os animais foram submetidos a avaliações física, radiográfica, ultrassonográfica, por um período de 60 dias. Não houve qualquer diferença estatística entre as articulações tratadas e as controle. A terapia com cartogenina, segundo protocolo utilizado, não produziu melhora clínica em lesões osteocondrais induzidas e tratadas com microperfurações na tróclea lateral do fêmur em equinos.(AU)

Articular cartilage reconstruction is still a challenge in Veterinary Medicine. The aim was to evaluate the therapeutic effects of kartogenin, a small molecule that promotes chondrocyte differentiation, in the repair of induced chondral defects pretreated with subchondral drilling. Six horses with a mean (± SD) weight of 342 ± 1.58Kg, aging 7.2 ± 1.30 years, and with a mean 7.1 ± 0.75 body score condition were used. In both stifles, a 1cm2 chondral defect was induced in the lateral femoral trochlea followed by treatment with subchondral drilling. Four intra-articular injections with kartogenin (20mM) were performed weekly in one stifle (treated group). The same procedure using saline solution was performed in the contralateral joint (control group). Clinical, radiographic, ultrasound evaluations were performed for a period of 60 days. No statistical differences were detected between groups in any of the studied variables. Although kartogenin has shown to improve articular cartilage repair in laboratory animal models, the same was not observed in this equine model. In conclusion, the kartogenin therapy, according to the used protocol, did not promote any clinical benefit in equine femoral trochlear defects pretreated with subcondral drilling.(AU)

Caracterização de condrócitos articulares cultivados em sistema tridimensional in vitro / Characterization of articular chondrocytes cultured in three-dimensional system in vitro

A osteoartrite é uma doença degenerativa da cartilagem articular que afeta, principalmente, idosos a partir dos 60 anos de idade. Sua degeneração é lenta e progressiva, causando dor e decréscimo da qualidade de vida dos pacientes acometidos pela doença. Atualmente, o tratamento da doença é de caráter meramente paliativo, resultando em cirurgia para completa remoção da cápsula articular do joelho e a substituindo por uma prótese. Este estudo avaliou as características dos condrócitos encapsulados por um hidrogel de alginato de sódio, que simula o microambiente original dessas células. Avaliamos a viabilidade dos condrócitos cultivados por 28 dias nesse modelo de cultivo, bem como a presença de proteoglicanos durante análise histológica. Através de microscopia eletrônica de varredura, observamos diferença significativa no microambiente interno do hidrogel e na disposição dos condrócitos, quando cultivados por até 28 dias na gota de alginato de sódio. Analisamos, ainda, a capacidade de deformação da gota de alginato de sódio quando pressionada, à fim de observar o quanto ela suporta até se romper. Nossos dados indicam que o hidrogel de alginato de sódio é um modelo eficaz para o cultivo de condrócitos e nos permite estudar suas características morfohistológicas. Além do mais, observamos o quão promissor é o uso desse biomaterial para a bioengenharia de tecido

Osteoarthritis is a degenerative disease of the articular cartilage that affects mainly elder people from 60 years old. Its degeneration is slow and progressive, causing pain and decreasing quality of life of patients affected by the disease. Currently, the treatment of the disease is merely palliative resulting in surgery for complete removal of the knee articular capsule and substituting it for a prosthesis. This study evaluated the characteristics of chondrocytes encapsulated by a sodium alginate hydrogel, which simulates a unique microenvironment of these cells. We evaluated the viability of chondrocytes cultured for 28 days in this model, as well as the presence of proteoglycan for histological analysis. Through scanning electron microscopy, we observed significant differences in the internal microenvironment of the hydrogel and chondrocytes disposition when cultured for up to 28 days in sodium alginate drop. We also analyzed the deformability of sodium alginate bead when pressed in order to observe how much it supports until it breaks. Our data indicate that sodium alginate hydrogel is an effective model to culture chondrocytes and allows us to study its morpho-histological characteristics. Moreover, we observed that this biomaterial can be very promising for tissue bioengineering

Effects of hyperprolactinemia and ovariectomy on the tibial epiphyseal growth plate and bone formation in mice / Efeitos da hiperprolactinemia e ooforectomia sobre o disco epifisário da tíbia e formação óssea em camundongos fêmeas

PURPOSE: To evaluate the effects of ovariectomy and the hyperprolactinemia procedure in the tibial epiphyseal growth plate of female mice. METHODS: In this study, the epiphyseal growth plate of ovariectomized (OVX) and/or rendered hyperprolactinemic female mice by 50 days of treatment with 200 μg metoclopramide (M) was evaluated morphologically, morphometrically and immuno-histochemically. Forty female and adult mice were divided into four groups according to treatment: V group - animals treated with saline solution; H group - hyperprolactinemic animals; Ovx/V group - ovariectomized animals and treated with saline solution; Ovx/H group - hyperprolactinemic and ovariectomized animals. After the treatment period, the animals were sacrificed, tibia was removed and fixed in 10% buffered formalin and decalcified in 10% formic acid. The material was immersed in paraffin and subjected to histological processing in paraffin. The sections were stained with Masson's trichrome and immunohistochemistry was carried out for the pro-apoptotic protein BCL-2. The images for the morphological and morphometric study were analyzed with the imaging program AxioVision 4.8 (Carl-Zeiss(r), Germany). RESULTS: The combination of hyperprolactinemia and the ovariectomy procedure decreased the number of resting chondrocytes 1.5-fold, the number of proliferative chondrocytes 1.8-fold; the percentage of resting cartilage 2.4-fold and the percentage of trabecular bone 2.1-fold, compared with respective control animals. CONCLUSION: The procedure of ovariectomy combined with the metoclopramide-induced hyperprolactinemia in female mice has showed marked bone degeneration due to significant decrease of cell proliferation in the epiphyseal growth plate and bone formation. .

OBJETIVO: Avaliar os efeitos do procedimento de ooforectomia e da hiperprolactinemia no disco epifisário da tíbia de camundongos fêmeas. MÉTODOS: Neste estudo, o disco epifisário de camundongos fêmeas ovariectomizadas (OVX) e/ou com hiperprolactinemia induzida por tratamento com 200 μg de metoclopramida por 50 dias (M) foi avaliado morfologicamente, morfometricamente e imunohistoquimicamente. Quarenta camundongos fêmeas e adultas foram divididas em quatro grupos, segundo o tratamento: Grupo V - animais tratados com solução salina; Grupo H - animais hiperprolactinêmicos; Grupo Ovx/V - animais ooforectomizados e tratados com o solução salina; Grupo Ovx/H - animais ooforectomizados e hiperprolactinêmicos. Após o período de tratamento, os animais foram sacrificados, as tíbias removidas e fixadas em formalina tamponada a 10% e descalcificadas em ácido fórmico a 10%. O material foi emblocado em parafina e submetido a processamento histológico em parafina. Os cortes foram corados pelo tricrômico de Masson e foi feita a imunohistoquímica para a proteína pró-apoptótica BCL-2. As imagens para o estudo morfológico e morfométrico foram analisadas com o programa de imagem AxioVision 4.8 (Carl-Zeiss(r), Alemanha). RESULTADOS: A combinação da hiperprolactinemia e do procedimento de ovariectomia levou à redução do número de condrócitos de repouso em 1,5 vezes; o número de condrócitos proliferativos em 1,8; a percentagem de cartilagem de repouso em 2,4, e a percentagem de osso trabecular em 2,1 vezes, em comparação com os respectivos animais controles. CONCLUSÃO: O procedimento de ooforectomia combinado com a condição de hiperprolactinemia induzida pela metoclopramida em camundongos fêmeas evidenciou degeneração óssea ...

The Importance of a Conchal Bowl Element in the Fabrication of a Three-Dimensional Framework in Total Auricular Reconstruction

BACKGROUND: To construct a sophisticated three-dimensional framework, numerous modifications have been reported in the literature. However, most surgeons have paid little attention to the anatomical configuration of the concha and more to its deepness and hollowness, leading to unsatisfactory outcomes. METHODS: For a configuration of the concha that is definitely anatomical, the author further developed and employed the conchal bowl element, which has been used by several surgeons although the results have not been published elsewhere. The author constructed the conchal bowl element in one of three patterns according to the amount of available cartilages: one block, two-pieces, or a cymba bowl element only. A total of 20 patients underwent auricular reconstruction using a costal cartilage framework between 2009 and 2012. The 8 earliest reconstructions were performed without a conchal bowl element and the latter 12 with a conchal bowl element. The patients were followed up for more than 1 year. The aesthetic results were scored by evaluating characteristics involving the stability of the crus helicis, the conchal definition, and the smoothness of the helical curve. RESULTS: The ears reconstructed early without a conchal bowl element showed a shallow and one or two incompletely separated concha with an obliterated cymba conchal space. They also did not have a realistic or smooth curve of the helix because of an unstable crus helicis. However, ears reconstructed later with the concha bowl element showed a definite crus helicis, deep cymba conchal space, and smooth helical curve. CONCLUSIONS: The construction of the conchal bowl element is simple, not time-consuming procedure. It is suggested that the conchal bowl element must be constructed and attached to the main framework for natural configuration of the reconstructed ear.

Aspectos moleculares dos esteroides sexuais sobre a cartilagem e os ossos / Molecular features of sexual steroids on cartilage and bone

No Brasil, o crescimento dos casos registrados de doenças degenerativas das cartilagens articulares por ano é de 20%, o que representa, anualmente, que mais de 200 mil brasileiros desenvolvem doenças degenerativas das articulações e, com repercussões negativas sobre a massa óssea. Este trabalho mostra evidências que a produção hormonal de esteroides sexuais (estrogênios, progestagênios e androgênios) têm influência na qualidade da cartilagem, bem como na massa óssea. Portanto, o objetivo dessa revisão foi o de analisar os dados da literatura sobre a ação molecular e gênica dos esteroides sexuais na fisiologia da cartilagem hialina e do osso, bem como a interferência da osteoartrite na qualidade dessas estruturas.

In Brazil, the increase in the reported cases of degenerative diseases of articular cartilage is 20% per year, meaning that 200,000 Brazilians develop degenerative joint diseases every year, which have a negative impact on bone mass. This study shows evidence that hormone production of sexual steroids (estrogens, progestogens, and androgens) have an influence on cartilage quality, as well as on bone mass. Therefore, this review aimed to analyze literature data on the molecular and genic action of sexual steroids on hyaline cartilage and bone physiology, as well as osteoarthritis interference on the quality of these structures.

Unusual nodular lesion of the tongue: clinical report of chondroid metaplasia / Lesão incomum de língua: relato clínico de metaplasia condoide

OBJETIVOS: Revisar os conceitos de metaplasia condroide e relatar um caso incomum desta rara condição que se apresentou como uma massa nodular na borda lateral da língua em um paciente adolescente. DISCUSSÃO E CONCLUSÃO: Metaplasia é a conversão, na vida pós-natal, de um tipo de célula para outra. Pode ser causada por algum estímulo anormal. A origem de cartilagem em tecidos moles bucais é desconhecida, mas várias hipóteses foram postuladas. Embora a etiopatogênese seja incerta, sugeriu-se que a metaplasia condroide ocorre como uma reação defensiva e originada de células mesenquimais miltipotentes. A metaplasia condroide é uma alteração benigna que ocorre como resposta à irritação crônica física ou química. A diferenciação entre coristoma cartilaginoso e metaplasia condroide pode ser difícil e, muitas vezes, uma questão de conceito. Considerando os achados do presente caso, a condição foi diagnosticada como metaplasia condroide de língua.

OBJECTIVES: To review the concepts of chondroid metaplasia and to report an unusual case of this rare condition, presenting as a nodular soft tissue mass in the lateral border of the tongue in an adolescent. DISCUSSION AND CONCLUSION: Metaplasia means the conversion, in postnatal life, of one cell type to another. It could be caused by some abnormal stimuli. The origin of cartilage in the oral soft tissues is unknown but several hypotheses have been postulated. Although the etiopathogenesis is unclear, it has been suggested that chondroid metaplasia may occur as a defensive reaction and it is originated from multipotential mesenchymal cells. Chondroid metaplasia is a benign change that occurs as a response to chronic physical or chemical irritation. The differentiation between cartilaginous choristoma and chondroid metaplasia could be very difficult and sometimes a matter of concept. Considering the findings of the current case, the lesion was diagnosed as chondroid metaplasia of the tongue.

Tissue Engineered Cartilage Formation using Human Hyaline Chondrocytes and Elastic Chondrocytes

The purpose of this study was to evaluate the influence of different types of PLGA scaffolds on the formation of human auricular and septal cartilages. The scaffolds were formed in tubular shape from 110,000 g/mol PLGA (poly lactic glycolic acid) and 220,000 g/mol one. Elastic cartilage was taken from the ear of a patient aged under 20 years old and hyaline cartilage from the nasal septum. The chondrocytes cells were then isolated by Klausburn method. After second passages, the chondrocytes were seeded on the PLGA scaffolds followed by in vitro culture for one week. The cells-PLGA scaffold complex was implanted at the back of nude mouses for 8 weeks. The tissue engineered cartilages were separated from nude mouse and examined histologically after staining with the Hematoxylin Eosin and Verhoeff. The formation of extracellular matrix and the porosity of the scaffolds were examined by scanning electron microscopy. The pores were well formed and uniformly distributed in both 110,000 g/mol and 220,000 g/mol PLGA scaffolds. The extracellular matrix was formed better in 110,000 g/mol PLGA compared to 220,000 g/mol one. And hyaline cartilage was proliferated better in vitro culture than elastic cartilage. After 8 weeks in vivo culture, cartilage was well formed with 110,000 g/mol PLGA, however lumen was collapsed. In contrast with 220,000 g/mol PLGA scaffold, neocartilage was formed in minimal amount while the architecture of scaffold was well preserved. Elastic cartilage seems to be better than hyaline one in terms of neocartilage formation. From the analysis after Verhoeff staining the cartilages, the neocartilage from elastic cartilage was proved to be elastic cartilage. In summary, there was no significant difference between elastic cartilage and hyaline cartilage in their morphologies, proliferation rates and the degree of cartilage formation since they were tissue engineered, however marked difference was found in neocartilage formation and preservation of scaffold architecture between 110,000 g/mol PLGA scaffold and 220,000 one. From the present findings, it is concluded that the influence of scaffold materials is significantly higher than that of different types of cells on the formation of new tissues.