Hipocondroplasia en pediatría: reporte de un caso / Hypocondroplasya in pediatrics: case study / Hipocondroplasia em pediatia: relato de caso

La hipocondroplasia es una displasia esquelética caracterizada por baja estatura, constitución robusta, brazos y piernas desproporcionadamente cortos, manos y pies anchos y cortos, leve laxitud articular y macrocefalia. Los niños generalmente se presentan como pequeños, con velocidad de crecimiento disminuida, que conduce a una baja estatura y desproporción de las extremidades. La hipocondroplasia en la mayoría de los casos se hereda con carácter autosómico dominante, aunque se detectan numerosos casos esporádicos. El diagnóstico requiere una exhaustiva anamnesis y adecuada exploración física. Es importante valorar algunos indicadores de crecimiento como: peso para la edad, longitud/talla para la edad, relación entre peso y longitud/talla, velocidad de crecimiento, talla diana genética, medidas de segmentos corporales, entre otros. Las radiografías esqueléticas permiten diagnosticar la mayoría de las displasias óseas. Los estudios moleculares suelen ser la prueba de confirmación y se solicitan ante una sospecha diagnóstica. Es importante incluir las displasias óseas en el diagnóstico diferencial de la talla baja y tenerlas en cuenta ante cualquier caso de talla baja disarmónica con alteraciones fenotípicas. La hipocondroplasia en la actualidad, no es una indicación aprobada para tratamiento con hormona del crecimiento. Se presenta un caso clínico de una niña de 14 meses, con talla baja severa, desproporcionada, que presentó dificultades para llegar al diagnóstico definitivo de hipocondroplasia.

Hypochondroplasia is a skeletal dysplasia characterized by short height, robust build, disproportionately short arms and legs, short and broad hands and feet, mild joint laxity, and macrocephaly. Children generally show slow growth rate, which leads to short stature and limb disproportion. Hypochondroplasia is mostly inherited with an autosomal dominant character, although many sporadic cases have been detected. Diagnosis requires a thorough history and adequate physical examination. It is important to assess some growth indicators such as: weight for age, length/height for age, relationship between weight and length/height, growth speed, genetic target height, measurements of body segments, among others. Skeletal XRs can diagnose most bone dysplasias. Molecular studies are usually the confirmatory test and are requested when a diagnosis is suspected. It is important to include bone dysplasias in the differential diagnosis of short stature and to take them into account for any disharmonious short stature with phenotypic alterations. Hypochondroplasia is currently not an approved indication for growth hormone therapy. We present a clinical case of a 14-month-old girl, with a severe, disproportionate short stature, who presented difficulties in her definitive hypochondroplasia diagnosis.

A hipocondroplasia é uma displasia esquelética caracterizada por baixa estatura, constituição robusta, braços e pernas desproporcionalmente curtos, mãos e pés largos e curtos, frouxidão articular leve e macrocefalia. As crianças geralmente são pequenas, com diminuição da velocidade de crescimento, o que leva à baixa estatura e desproporção dos membros. A hipocondroplasia na maioria dos casos é herdada com caráter autossômico dominante, embora sejam detectados numerosos casos esporádicos. O diagnóstico requer uma história completa e um exame físico adequado. É importante avaliar alguns indicadores de crescimento como: peso para idade, comprimento/altura para idade, relação entre peso e comprimento/altura, taxa de crescimento, estatura alvo genético, medidas de segmentos corporais, entre outros. As radiografias esqueléticas permitem o diagnóstico da maioria das displasias ósseas. Os estudos moleculares são geralmente o teste de confirmação e são solicitados quando há suspeita de diagnóstico. É importante incluir as displasias ósseas no diagnóstico diferencial da baixa estatura e considerá-las em qualquer caso de baixa estatura desarmônica com alterações fenotípicas. A hipocondroplasia não é atualmente uma indicação aprovada para o tratamento com hormônio de crescimento. Apresenta-se o caso clínico de uma menina de 14 meses, com baixa estatura grave e desproporcional, que apresentou dificuldades em chegar ao diagnóstico definitivo de hipocondroplasia.

Bone Defects in Revision Total Knee Arthroplasty / Falhas ósseas nas revisões de artroplastia total do joelho

Abstract The increase in the number of revision total knee arthroplasty surgeries has been observed in recent years, worldwide, for several causes. In the United States, a 601% increase in the number of total knee arthroplasties, between 2005 and 2030, is estimated. Among the enormous challenges of this complex surgery, the adequate treatment of bone defects is essential to obtain satisfactory and lasting results. The adequate treatment of bone defects aims to build a stable and lasting support platform for the implantation of the definitive prosthetic components and, if possible, with the reconstruction of bone stock. Concomitantly, it allows the correct alignment of the prosthetic and limb components, as well as restoring the height of the joint interline and, thus, restoring the tension of soft parts and load distribution to the host bone, generating a joint reconstruction with good function, stable, and painless. There are several options for the management of these bone defects, among them: bone cement with or without reinforcement with screws, modular metallic augmentations, impacted bone graft, structural homologous graft and, more recently, metal metaphyseal cones, and metaphyseal sleeves. The objective of the present article was to gather classic information and innovations about the main aspects related to the treatment of bone defects during revision surgeries for total knee arthroplasty.

Resumo O aumento do número de cirurgias de revisão de artroplastia total do joelho tem sido observado nos últimos anos, em todo o mundo, por diversas causas. Nos Estados Unidos, é estimado um aumento de 601% no número de artroplastias totais do joelho entre 2005 e 2030. Dentre os enormes desafios dessa cirurgia complexa, o adequado tratamento dos defeitos ósseos é essencial para a obtenção de resultados satisfatórios e duradouros. O adequado tratamento dos defeitos ósseos objetiva construir uma plataforma de suporte estável e duradoura para a implantação dos componentes protéticos definitivos e, se possível, com recomposição do estoque ósseo. Concomitantemente, possibilita o correto alinhamento dos componentes protéticos e do membro, assim como permite restabelecer a altura da interlinha articular e, dessa forma, restaurar a tensão de partes moles e distribuição de carga ao osso hospedeiro, gerando uma reconstrução articular com boa função, estável e indolor. Diversas são as opções para manejo dessas falhas ósseas, entre elas: cimento ósseo com ou sem reforço com parafusos, aumentos metálicos modulares, enxerto ósseo impactado, enxerto estrutural homólogo e, mais recentemente, cones metafisários de metal trabecular e sleeve metafisário. O objetivo do presente artigo foi reunir informações clássicas e inovações dos principais aspectos relativos ao tratamento das falhas ósseas durante as cirurgias de revisão de artroplastia total do joelho.

Absceso óseo en un paciente joven con artritis reumatoide, a propósito de un caso / Bone abscess in a young patient with rheumatoid arthritis, about a case

Introducción: la artritis reumatoide es una enfermedad inflamatoria sistémica y crónica que se caracteriza fundamentalmente por la presencia de dolor, inflamación, deformidad, discapacidad y disminución de la percepción de calidad de vida relacionada con la salud. El propio mecanismo etiopatogénico de la enfermedad y la acción de los fármacos utilizados en su control generan un estado de inmunosupresión que puede ocasionar la proliferación de bacterias y la aparición posterior de abscesos a distintos niveles. Objetivo: dar a conocer los elementos clínicos, de laboratorio e imagenológicos que permiten llegar al diagnóstico de un absceso óseo en un paciente con artritis reumatoide. Caso clínico: se presenta el caso de un paciente masculino, de 31 años de edad con diagnóstico de artritis reumatoide de 7 años de evolución que acude a consulta con manifestaciones clínicas, de laboratorio e imagenológicos que permiten llegar al diagnóstico de un absceso óseo. Conclusiones: los procesos infecciosos óseos constituyen un factor agravante de la calidad ósea, si los mismos se presentan en pacientes con enfermedades reumáticas como la artritis reumatoide, donde existen distintos niveles de afectación ósea, entonces se compromete más aún la salud y funcionamiento articular(AU)

Introduction: rheumatoid arthritis is a systemic and chronic inflammatory disease characterized mainly by the presence of pain, inflammation, deformity, disability and decreased perception of quality of life related to health. The own etiopathogenic mechanism of the disease and the action of the drugs used in its control generate a state of immunosuppression that can cause the proliferation of bacteria and the subsequent appearance of abscesses at different levels. Objective: to present the clinical, laboratory and imaging elements that allow us to reach the diagnosis of a bone abscess in a patient with rheumatoid arthritis. Clinical case: we present the case of a 31-year-old male patient with a 7-year history of rheumatoid arthritis who presented with clinical, laboratory and imaging findings that allow the diagnosis of a bone abscess. Conclusions: bone infectious processes are an aggravating factor of bone quality, if they occur in patients with rheumatic diseases such as rheumatoid arthritis, where there are different levels of bone involvement, then health and joint function are more compromised(AU)

Osteogénesis Imperfecta. A propósito de un caso tipo II / Osteogenesis Imperfecta. About a type II case

RESUMEN El síndrome de Osteogénesis Imperfecta (OI) tipo II está dentro del grupo de trastornos del tejido conectivo de origen genético-hereditario que se caracteriza por fragilidad ósea, fracturas múltiples, huesos largos anchos y acortados, además de una pobre mineralización ósea. Su frecuencia de aparición se calcula en aproximadamente 1: 55.000 nacidos vivos y es el resultado de mutaciones de dos genes que codifican las cadenas de colágeno tipo 1. El riesgo de recurrencia es alrededor de 6 % pero si ambos padres fueran heterocigotos, aumentaría a 10-25 %. También se han reportado casos esporádicos por mutación de novo. El diagnóstico se suele realizar por los hallazgos ecográficos en el segundo trimestre o en ecografías previas si los hallazgos son muy evidentes. Las pruebas invasivas son útiles sobretodo en casos de antecedentes familiares con formas leves de OI. En nuestro caso, encontramos durante la ecografía de las 20 semanas una notable hipomineralización de la calota fetal sospechada por hiporrefringencia de la misma, acortamiento de extremidades superiores e inferiores con múltiples fracturas óseas, arcos costales cortos, arqueados y una desproporción toraco-abdominal. En los casos en donde se prosigue con el embarazo más de 60% de los recién nacidos mueren el primer día de vida, el resto lo hace durante el primer mes y la sobrevivencia más allá de un año es rara. La principal causa de muerte postnatal suele ser por falla respiratoria.

SUMMARY Osteogenesis Imperfecta (OI) type II is within the group of connective tissue disorders hereditary genetic-origin characterized by bone fragility, multiple fractures, broad long bones and shortened, and a poor bone mineralization. Their frequency is estimated at approximately 1: 55,000 live births, and is the result of mutations of genes which encoding chains of type 1 collagen. The risk of recurrence is around 6% but if both parents were heterozygous, increase to 10-25%. There has also been reported sporadic cases with de novo mutation. The diagnosis is usually made by ultrasound findings in second trimester or previously if the findings are very obvious. Invasive tests are useful especially in cases of family history with mild forms of OI. In our case, we found during ultrasound 20 weeks a remarkable hypomineralization of fetal calvarial, shortening of upper and lower extremities with multiple bone fractures and short costal arches, arched and thoracoabdominal disproportion. In cases where continued pregnancy more than 60% of newborns die during the first day of life, 80% die in the first month and survival beyond one year is rare. Death can occur prenatally or postnatamente from respiratory failure.

Extrusão ortodôntica de dentes condenados para reconstrução da arquitetura óssea tridimensional previamente à instalação de implantes osseointegrados ­ Análise crítica da literatura / Orthodontic extrusion of hopeless teeth for reconstruction of three-dimensional bone architeture prior to dental implant placement ­ a critical analysis of literature

Objetivo: o objetivo desta revisão sistemática foi investigar se a condição periodontal do defeito ao redor de dentes condenados à extração, por meio de extrusão ortodôntica para reconstrução da arquitetura óssea previamente à instalação de implantes, influencia a qualidade estética final. Material e métodos: estudos relatando extrusão ortodôntica para extração de dentes e posterior instalação de implantes, acompanhados por diferentes períodos após a instalação das próteses, foram considerados elegíveis. A busca por artigos, a extração de dados e a avaliação da estética foram realizadas por examinadores independentes. A condição periodontal inicial foi classificada em tipos, a partir das radiografias iniciais presentes nos estudos: I) sem perda óssea; II) até 75%; III) até 50%; IV) até 25%; ou V) menos de 25% de inserção remanescente. A fotografia final dos casos foi analisada de acordo com o índice de estética rosa (IER). Resultados: a busca no PubMed e Scopus resultou em 453 artigos, dois quais 394 foram excluídos pelo título e 41 por não atender aos critérios de inclusão, restando 18 artigos analisados. Não houve diferença estatisticamente significante no IER observado nos diferentes tipos de defeitos periodontais iniciais (Kruskal-Wallis, p=0,17). Conclusão: esses achados sugerem que a inserção residual existente previamente à extração de dentes por meio de extrusão ortodôntica não influencia a estética final do caso, devido à reconstrução dos tecidos gengival e ósseo, favorecendo o posicionamento ideal dos implantes.

Objective: the aim of this critical analysis of literature was to investigate if the periodontal status of defects surrounding hopeless teeth submitted to extraction by orthodontic extrusion prior to implant installation interfere at the final aesthetic outcome. Material and methods: studies describing this procedure followed by different periods after prosthesis installation were considered eligible. Search, data extraction and aesthetic evaluation were made by independent examiners. Inicial periodontal status was classified in types according inicial radiographs presented on studies: I) without bone loss; II) up to 75%; III) up to 50%; IV) up to 25%; or V) less than 25% remaining insertion. Cases' final photographs were evaluated according the pink aesthetics index (PAI). Results: the search in PubMed and Scopus resulted in 453 papers of which 394 were excluded by title and 41 for not fulfill the inclusion criteria, remaining 18 papers for analysis. No statistical significance difference on PAI was observed at different inicial periodontal defects (Kruskal-Wallis, p=0.17). Conclusion: these findings suggest that the residual insertion existent prior to tooth extraction through orthodontic extrusion do not influence the aesthetic outcome because of the reconstruction of the gingival and bone tissues, favoring an optimal site for implant positioning.

Repair of Bone Defects with Chitosan-Collagen Biomembrane and Scaffold Containing Calcium Aluminate Cement

Abstract Innovative biomaterials can provide a promising new direction for the treatment of bone defects, stimulating a proper repair process, with no damage to adjacent tissues. The purpose of this in vivo study was to evaluate the biocompatibility and the osteoinductive capacity of chitosan-collagen biomembrane and scaffold containing calcium aluminate cement. Eighteen New Zealand white rabbits (Oryctolagus cuniculus) were distributed according to the experimental times of analysis (7, 15 and 30 days). Four bone defects were created in the rabbits calvaria, which were individually filled with the biomembrane, scaffold, blood clot (negative control) and autologous bone (positive control). Histopathological analysis was performed using optical microscope at 32´, 64´, 125´ and 320´ magnifications. Cell response to inflammation and new bone tissue formation was quantified using a score system. The biomembrane group presented greater inflammatory response at 15 days, with significant difference to autologous bone group (p<0.05). There was no statistically significant difference for foreign body type reaction among groups (p>0.05). Concerning new bone formation, linear closure of the defect area was observed more evidently in the group with autologous bone. The scaffold group presented similar results compared with the autologous bone group at 30 days (p>0.05). Both tested biomaterials presented similar biocompatibility compared with the control groups. In addition, the biomembrane and scaffold presented similar osteoinductive capacity, stimulating bone repair process in the course of the experimental time intervals.

Resumo Biomateriais inovadores podem fornecer uma promissora nova direção para o tratamento de defeitos ósseos, estimulando um processo de reparo adequado, sem danos aos tecidos adjacentes. O objetivo deste estudo in vivo foi avaliar a biocompatibilidade e a capacidade osteoindutora de uma biomembrana e um scaffold compostos por colágeno e quitosana, contendo cimento de aluminato de cálcio. Dezoito coelhos (New Zealand White, Oryctolagus cuniculus) foram distribuídos de acordo com os períodos experimentais de análise (7, 15 e 30 dias). Quatro defeitos foram criados na calvaria dos coelhos, que foram individualmente preenchidos com a biomembrana, scaffold, coágulo (controle negativo) e osso autólogo (controle positivo). A avaliação histopatológica foi realizada em microscópio óptico em aumentos de 32´, 64´, 125´ e 320´. A resposta celular à inflamação e à formação de novo tecido ósseo foi quantificada utilizando um sistema de escore. O grupo da biomembrana apresentou maior resposta inflamatória no período de 15 dias, com diferença significativa para o grupo do osso autólogo (p<0,05). Não houve diferença estatística significante para a reação do tipo corpo estranho entre os grupos (p>0,05). Em relação à neoformação óssea, observou-se fechamento linear da área do defeito, que foi mais evidente no grupo em que se utilizou o osso autólogo. O grupo scaffold apresentou resultados semelhantes ao grupo do osso autólogo no período de 30 dias (p>0,05). Ambos os biomateriais testados apresentaram biocompatibilidade similar em comparação com os grupos controle. Além disso, a biomembrana e o scaffold apresentaram capacidade osteoindutora similar, estimulando o reparo ósseo ao longo dos intervalos de tempo experimentais.

C syndrome with skeletal anomalies, mental retardation, eyelid chalazion, Bitot's spots and agenesis of the corpus callosum in an Egyptian child

We report a 2.5 year old female child, third in order of birth of healthy non consanguineous Egyptian parents with C syndrome. The patient had moderate mental retardation, trigonocephaly, protruding forehead, low anterior hair line, wide upslanted palpebral fissures, depressed nasal bridge, broad nose, high arched palate, microretrognathia, low set ears, short neck, scoliosis, hypertrichosis over the back, talipes equinovarus as well as interatrial septal defect. The patient had in addition chalazion in left lower eyelid as well as bilateral Bitot's spots most probably due to vitamin A deficiency. MRI brain revealed agenesis of the corpus callosum

Baraitser-winter syndrome: an additional Egyptian patient with skeletal anomalies, bilateral iris and choroid colobomas, retinal hypoplasia and hypoplastic scrotum

We report a 3.5 year old male child, second in order of birth of non consanguineous Egyptian parents with Baraitser-Winter syndrome [BRWS]. The patient had bilateral colobomas of the iris and choroid. Our patient had also retinal hypoplasia, which was not reported previously in this syndrome, bilateral congenital ptosis, hypertelorism, moderate mental retardation, short stature, short neck, hyperextensibility of the joints of the hands, talipes equinovarus, kyphoscoliosis and unilateral hypoplastic scrotum and testis

Células-tronco mononucleares associadas ao plasma rico em plaquetas na consolidação de falha óssea no cão. Estudo piloto / Mononuclear stem cells associated with platelet-rich plasma in the consolidation of bone defects in dogs. A pilot study

A padronização de técnicas e o teste da associação do Plasma Rico em Plaquetas (PRP) e das Células-Tronco Mononucleares (CTMs) na consolidação de falhas ósseas corticais, por meio de avaliação clínica, biomecânica, radiológica e histológica, é avaliada em um estudo piloto. Foram utilizados seis cães adultos, fêmeas, sem raça definida, pesando entre 5 e 10kg, separados por sorteio aleatório em seis tratamentos. Foi confeccionada uma falha elíptica de 1,0x0,4cm na cortical medial diafisária da tíbia direita de cada animal, sendo preenchida de acordo com o tratamento proposto. No cão I, a falha foi preenchida com solução fisiológica (SF); no II, com o PRP; no III, com a fração total das células mononucleares (FTCM); no IV, com a fração vascular estromal (FVE); no V, com o PRP associado à FTCM; no VI, com a associação PRP e FVE. Foram realizadas avaliações: clínicas, diariamente; dos graus de claudicação, semanalmente; radiológica e perimetria da coxa, antes, no pós-operatório imediato, aos 7, 14, 21 e 30 dias; biomecânica, antes do procedimento, aos 10, 20 e 30 dias; e biópsias, aos 15 e 30 dias. A FTCM obteve uma contagem e viabilidade média de 2,0x108cél. e 90%, respectivamente, enquanto a FVE obteve 3x106cél. e 50%. O PRP concentrou, em média, sete vezes o número inicial de plaquetas do sangue total, de 250.000 µl-1 plaquetas no sangue total para 1.750.000 µl-1 plaquetas no PRP. Obteve-se padronização adequada de técnicas, possibilitando o teste da associação entre as células-tronco mononucleares (CTMs) e o plasma rico em plaquetas (PRP), assim como seu uso isolado, no reparo de falhas ósseas corticais, indicando a possibilidade de a associação FTCM e PRP ser o melhor tratamento...

The standardization of techniques and tests of the association of Platelet Rich Plasma (PRP) and Mononuclear Stem Cells (MSCs) in the consolidation of cortical bone defects by clinical, biomechanical, radiological, and histological analysis is evaluated in a pilot study. Six adult female dogs of mixed breed, weighing between 5 and 10kg, separated by random draw in six treatments were used. An elliptical failure of 1.0 x0.4cm was done in the medial diaphyseal cortical of the right tibia of each animal, that was filled according with the proposed treatment. In dog I, the failure was filled with saline (S), in dog II with PRP, in dog III with total mononuclear cell fraction (TMCF), in dog IV with stromal vascular fraction (SVF), in dog V with association of PRP and TMCF, and in dog VI with an association of PRP and SVF. Daily clinical evaluation, weekly degrees of lameness, radiological and girth before, immediate postoperative, 7, 14, 21 and 30 days, biomechanics before the procedure, at 10, 20 and 30 days, and biopsies at 15 and 30 days were performed. The TMCF got a count and viability of 2,0x108cells and 90% respectively, while for SVF it was 3x106cells and 50%, respectively. The PRP concentrated on average seven times the original number of platelets from whole blood, platelets from whole blood 250.000 μl-1 to 1.750.000 μl-1 platelets in PRP. This afforded adequate standardization of techniques, enabling the test of association between mononuclear stem cells (MSCs) and platelet-rich plasma(PRP), as well as their separate use to repair cortical bone defects, indicating the possibility of the association between FTCM and PRP to be the best treatment...

Immunoexpression of PPAR-? and Osteocalcin Proteins for Bone Repair of Critical-Size Defects Treated with Fragmented Autogenous Abdominal Adipose Tissue Graft

Immunoexpression of PPAR-γ and osteocalcin proteins was evaluated for bone repair of critical-size defects (CSDs), created in rat calvaria (n=42) and treated with fragmented abdominal autogenous adipose tissue graft. Three groups (n=14) were formed: C (control - blood clot), AB (autogenous bone) and AT (fragmented adipose tissue). The groups were divided into subgroups (n=7) for euthanasia at 30 and 90 days. Histological and immunohistochemical analyses were performed. Data were subjected to descriptive statistics (mode). A complete bone closure was observed in Group AB 90 days after surgery. In Group C, repair was achieved by the formation of collagen fiber bundles oriented parallel to the wound surface at both post-surgery periods. In Group AT the type of healing was characterized by dense connective tissue containing collagen fiber bundles arranged amidst the remaining adipose tissue, with rare heterotopic bone formation associated with fibrosis and different types of tissue necrosis. Immunostaining of PPAR-γ was not observed in any specimen from Groups C and AB. In Group AT, the immunostaining of PPAR-γ was more evident 30 days after surgery. Immunostaining of osteocalcin was present in all groups and at both postoperative periods. The fragmented autogenous abdominal adipose tissue graft did not favor the repair of critical-size bone defects created surgically in rat calvaria as evidenced by the positive immunostaining of PPAR-γ protein and the negative immunostaining of osteocalcin in the osteoblast-like cells and bone matrix.

A imunoexpressão das proteínas PPAR-γ e osteocalcina foi avaliada para o reparo ósseo de defeitos de tamanho crítico (DTC) criados em calvária de ratos (n = 42) e tratados com enxerto de tecido adiposo autógeno abdominal macerado. Foram formados três grupos (n=14): C (controlo - coágulo de sangue), AB (osso autógeno) e AT (tecido adiposo fragmentado). Estes grupos foram divididos em subgrupos (n = 7) para a eutanásia em 30 e 90 dias. Foram realizadas análises histológicas e imuno-histoquímico. Os dados foram submetidos à análise estatística descritiva (moda). Um fechamento ósseo completo foi observada no grupo AB 90 dias após a cirurgia. No grupo C a reparação foi alcançada através da formação de feixes de fibras de colágeno orientadas paralelamente à superfície da ferida nos dois períodos pós-operatórios. No Grupo AT, o tipo de cicatrização predominante foi caracterizada pela presença de tecido conjuntivo denso contendo feixes de colágeno de fibras dispostas em meio ao tecido adiposo restante, com raras formações ósseas heterotópicas associada à fibrose e diferentes tipos de necrose tecidual. A imunomarcação do PPAR- γ não foi observada em nenhum espécime dos grupos C e AB. No grupo AT a imunomarcação do PPAR-γ foi mais evidente aos 30 dias de pós-operátorio. A imunomarcação da osteocalcina estava presente em todos os grupos e nos dois períodos de pós-operatório O enxerto autógeno abdominal fragmentado tecido adiposo não favoreceu a reparação de defeitos ósseos de tamanho crítico criados cirurgicamente em calvária de ratos como evidenciado pela imunomarcação positiva da proteína PPAR-γ e a imunomarcação negativa de osteocalcina nos osteoblastos e matriz óssea.

prevalence of vitamin d deficiency in ancient egyptian population from baharia oasis, the greco roman period

Vitamin D deficiency is considered to be the most common nutritional deficiency andalso one of the most common undiagnosed medical conditions in the world. Vitamin D is naturallypresent only in minor amounts in most foods; the great majority is synthesized by the action ofultraviolet light on chemical precursors in the skin.The manifestation of vitamin D deficiency in sub adults is referred to as rickets, and in adults,osteomalacia . Rickets and osteomalacia are the sub adult and adult expressions of a disease in whichthe underlying problem is a failure to mineralize bone protein [osteoid]. The most common cause ofthis disease is a physiological deficiency in vitamin D. The associated problems include deformedbones. This study aimed to investigate the skeletal remains of ancient Egyptiansfrom Baharia Oasis population for lesions indicative of vitamin D deficiency [rickets andosteomalacia]. The material consisted of 1075 commingled bones [38 sub adults and 1037 adults]. They were recovered from Baharia oasis. The results showed that, there was no evidence of rickets in sub adult group. The prevalenceof osteomalacia in adult Baharia populations was 7.4% ; all were adult males.This result could indicate that this population was subjected to sunlight all over the year and their dietwas rich of calcium and phosphorus. These few cases that were found may be due to mechanical stress during wine andtextile production

Immediate implant placement and temporization in the aesthetic zone in the presence of horizontal and vertical bone defect. A case report

A 52-year-old male patient presented with an endodontically failed right maxillary central incisor. The patient expressed high esthetic demands: "I can't walk away without a tooth, and I can't support any removable appliance". The clinical and radiographic examination revealed according to Elian et al. [2007] a type 2 defect site requiring guided bone regeneration procedure prior to implant placement, moreover the presence of diastema contraindicated any bonded provisional prosthetic solution. After tooth extraction, a localized alveolar ridge deficiency did not prevail the immediate placement of a tapered 5x 13mm diameter dental implant [Full Osseotite tapered implant, Biomet 3i, Palm Beach Garden, Florida] with bane regeneration using xenograft granules of Endobon [Biomet 3i, Palm beach Garden, Florida]. Simultaneously, a temporary platform switching abutment was screw-retained on top of the implant allowing for a four months healing period. Later, the implant demonstrated excellent conditions for impression taking and the final restoration was screw-retained 5 months following implant placement. The immediate implant placement is an ideal treatment option for the proper candidate. Several factors must be taken into consideration to allow clinical success and attain patient's expectations. This case report describes the successful replacement and temporization of a failed maxillary central incisor with a Biomet 3i implant after immediate placement and a bucco-lingual augmentation of the resorbed alveolar ridge

Avaliação dos efeitos do laser Er, Cr: YSGG em defeitos ósseos críticos e no tratamento de doença periodontal induzida em ratos submetidos a inalação de fumaça de cigarro / Evaluation of the effects of Er, Cr: YSGG laser in critical bone defects and treatment of periodontal disease induced in rats subjected to inhalation of cigarette smoke

O objetivo deste trabalho foi avaliar as influências do laser Er,Cr:YSGG na criação e reparo de defeitos críticos e no tratamento de doença periodontal em ratos submetidos a inalação de fumaça de cigarro. Utilizou-se 180 ratos, divididos em 6 grupos: Fumante Raspagem (FRAR) - inalação de fumaça de cigarro (IFC), indução da doença periodontal (IDP), e tratamento com raspagem e alisamento radicular (RAR); Fumante Laser (FL) - IFC, IDP e irradiação com o laser de Er,Cr:YSGG (Laser); Fumante Raspagem + Laser (FRAR+L) - IFC, IDP e tratamento com RAR+Laser; Raspagem (RAR) ­ IDP e RAR; Laser (L) ­ IDP e Laser; Raspagem + Laser (RAR+L) ­ IDP e RAR+ Laser. Os resultados histométricos mostraram que em todos os grupos avaliados houve tendência a formação óssea ao longo dos períodos, no entanto essa diferença foi significante apenas no grupo FRAR, comparando os períodos de 15 e 30 dias (p<0,05). A comparação intergrupos mostrou que no período de 15 dias, grupo FL a porcentagem de área óssea foi maior no grupo FL em relação ao grupo FRAR. Nenhuma diferença significante foi observada nos grupos não expostos a inalação de fumaça de cigarro. Na reação por PCR-tempo real, os grupos L e RAR+L mostraram maior expressão de VEGF aos 30 dias (p<0,05) comparados aos 15 dias (p<0,01). O grupo FRAR mostrou menor expressão de VEGF aos 30 dias (p<0,05) em relação aos 15 dias. Para criação dos defeitos, 100 ratos foram divididos em 4 grupos: GT: criação do defeito com trefina; GL: defeito criado com o laser; GFT: IFC e criação do defeito com trefina; GFL: IFC e criação de defeito com o laser. Histometricamente, o defeito foi menor no grupo trefina aos 30 e 60 dias em relação ao período inicial (p<0,05). No grupo laser essa diminuição ocorreu aos 15, 30 e 60 dias comparados ao período inicial (p<0,05, p<0,01, p<0,001) e aos 30 e 60 dias em relação aos 7 dias (p<0,05 e p<0,01). Nos animais expostos a inalação de fumaça de cigarro, houve maior reparo no grupo laser comparado ao trefina no período de 60 dias (p<0,001), dentro do grupo trefina houve diminuição no comprimento do defeito aos 30 dias comparado aos 7 dias (p<0.05) e no grupo laser essa diminuição foi observada nos períodos de 15 (p<0.05), 30 (p<0.05) e 60 dias (p<0.01) em relação aos períodos de 0 e 7 dias. Concluiu-se que o laser de Er,Cr:YSGG associado ou não a raspagem e alisamento radicular (RAR) promoveu condições favoráveis ao reparo tecidual e quando utilizado na criação dos defeitos críticos em calota, este laser proporcionou cortes precisos sem causar danos térmicos aos tecidos subjacentes, favorecendo o reparo na região, independente da exposição a fumaça de cigarro

The aim of this study was to evaluate the influence of the Er, Cr: YSGG laser in criticalsize defects and in the treatment of periodontal disease in rats subjected to cigarette smoke inhalation. It was used 180 rats divided into 6 groups: Cigarette Smoke scaling (CSRP) - cigarette smoke inhalation (CSI), induction of periodontal disease (IPD) and scaling and root planing (SRP); Cigarette Smoke Laser (CL) ­ CSI, IPD and Er,Cr:YSGG laser irradiation; Cigarette Smoke SRP+Laser (CSRP+L) ­ CSI, IPD and RAR + Laser; Scaling and root planning (SRP) IPD, SRP; Laser (L) ­ IPD and Laser; Scaling and root planning + Laser (SRP+L) ­ IPD and SRP followed by Laser. The histometric results showed that in all groups analyzed it was observed a tendency to bone formation over the periods, however this difference was only significant in the CSRP comparing 15 and 30 days (p<0,05). The intergroup comparison showed that the 15-day period, the percentage of bone area was greater in the FL group compared to FRAR group. No difference was observed in the groups not exposed to cigarette smoke inhalation. In reaction by real-time PCR, L and SRP + L groups showed higher expression of VEGF at 30 days (p <0.05) compared to 15 days (p <0.01). The FRAR group showed lower expression of VEGF at 30 days (p<0.05) compared to 15 days. For creation of the defects, 100 rats were divided into 4 groups: GT: creation of the defect with trephine; GL: defect created with the laser; GFT: IFC and creation of defect with trephine; GFL: IFC and creating defect with the laser. The results showed that histometrically, the defect was lower in the trephine group at 30 and 60 days compared to the initial period (p <0.05). In the laser group this decrease occurred at 15, 30 and 60 days compared to the initial period (p <0.05, p <0.01, p <0.001) and at 30 and 60 days compared to 7 days (p <0, 05 and p <0.01). In animals exposed to cigarette smoke inhalation, there was a higher repair in the laser group compared to trephine in 60 days (p <0.001). Intragroupu analysis showed that in the trephine group there was a decrease in the length of the defect at day 30 compared to day 7 (p <0.05). This decrease in the laser group was observed at 15 (p <0.05), 30 (p <0.05), and 60 days (p <0, 01) compared to 0 and 7 days. It was concluded that the Er, Cr: YSGG laser with or without scaling and root planing (SRP) promoted favorable conditions for tissue repair and when used in the critical size defects creation, this laser provided precise cuts without causing thermal damage to surrounded tissues, favoring the repair in the region, regardless of exposure to cigarette smoke inhalation

Do the Clinical Effects of Enamel Matrix Derivatives in Infrabony Defects Decrease Overtime? A Systematic Review and Meta-Analysis

Previous systematic reviews have demonstrated better results with enamel matrix derivative proteins (EMDP) as compared with open flap debridement (OFD) for the management of infrabony periodontal defects (IPD). The aim of this study was to determine whether these differences vary according to the follow-up and quality of the studies. Cochrane Central Register of Controlled Trials, Medline/PubMed, Lilacs, Embase and Web of Science electronic databases were searched up to August 2013 for randomized clinical trials.Eligible outcomes were changes in probing depth (PD), clinical attachment level (CAL),gingival recession (GR) and bone changes (BC). Studies with follow-up of 12 months showed differences of 0.97 mm (CI95% 0.52 - 1.43) and 1.19 mm (CI95% 0.77 - 1.60) for PD and CAL, respectively, favorable for EMDP. Studies with follow-up ≥ 24 months presented advantages of 1.11 mm (CI95% 0.74 -1.48) for CAL and 0.83 mm (CI95% 0.19 -1.48) for PD,with use of EMDP. Considering the quality of studies, those with low risk of bias showed lower difference between groups, presenting 0.8 mm (CI95% 0.24-1.36) for CAL, favorable for EMDP and without differences for PS (0.51 mm, CI95% -0.21 - 1.23). In conclusion, follow-up time (< or > 2 years) and the risk of bias influence the results of treatment with EMDP in IPD.

Revisões sistemáticas prévias tem demonstrado melhores resultados com proteínas derivadas da matriz de esmalte (PDME) em comparação a retalho de espessura total (RET) para o manejo de defeitos periodontais infraósseos (DPI). O objetivo desse estudo foi determinar se essas diferenças variam de acordo com o tempo de acompanhamento e com a qualidade dos estudos. As bases de dados Cochrane Central Register of Controlled Trials, MEDLINE (PubMed), Lilacs, Embase e Web of Science foram pesquisadas sem limitação de tempo ate agosto de 2013 para ensaios clínicos randomizados. Os desfechos elegíveis foram alterações na profundidade de sondagem (PS), nível de inserção clinica (NIC), recessão gengival (RG) e alterações ósseas (AO). Resultados: Estudos com acompanhamento de ate 12 meses mostraram diferenças de 0.97 mm (CI95% 0.52 – 1.43) e 1.19 mm (CI95% 0.77 – 1.60) para PS e NIC, favoráveis a PDME, respectivamente. Estudos com acompanhamento ≥24 meses demonstraram vantagens de 1.11 mm (CI95% 0.74 -1.48) para NIC e 0.83 mm (CI95% 0.19 -1.48) para PS, com o uso de PDME. Considerando a qualidade dos estudos, publicações com baixo risco de viés exibiram menores diferenças entre os grupos apresentando 0.8 mm (CI95% 0.24-1.36) para o NIC, sem diferenças para PS (0.51 mm, CI95% -0.21 – 1.23). Pode-se concluir que o tempo de acompanhamento (< ou > 2anos) e o risco de viés são variáveis que influenciam nos resultados do tratamento com PDME em DPIO.

Alteraciones morfológicas de las vellosidades placentarias asociadas a malformaciones fetales múltiples del sistema esquelético / Morphological changes in placental villi associated with multiple malformations of the skeletal system

Describir y cuantificar alteraciones morfológicas en vellosidades placentarias de embarazadas cuyo feto desarrolló malformaciones esqueléticas múltiples. MÉTODOS: se analizaron cuatro placentas de abortos terapéuticos a las 13, 16, 20 y 38 semanas de gestación. Estas se compararon con placentas normales a la misma edad de gestación de abortos electivos por indicación médico legal. Tinción de hematoxilinaeosina se aplicó a 10 láminas de 5 regiones de cada placenta utilizando un protocolo con 4 variables cuantitativas: madurez, cambios fibrinoides, edema y fibrosis estromal y una variable cualitativa: trombosis. Los resultados cuantitativos se analizaron utilizando el análisis de varianza (ANAVAR) según arreglo completamente aleatorizado y el test de Tukey. Para la variable cualitativa se aplicó la prueba de tendencia para datos correlacionados. Se empleó el software statistix 8.0 y SAS 9.0 para Windows. RESULTADOS: existen diferencias significativas (p<0,05) entre las placentas asociadas a malformaciones múltiples del sistema esquelético y las placentas control en relación a las variables cuantitativas. No se encontraron diferencias significativas (p>0,05) en relación a la variable cualitativa. CONCLUSIONES: la población de vellosidades placentarias asociadas a malformaciones múltiples del sistema esquelético presentó un alto porcentaje de alteraciones indicando que la barrera placentaria está dañada afectando el intercambio de gases, nutrientes y metabolitos durante el desarrollo del feto...

To describe and quantify morphological changes in placental villi in pregnancies with multiple fetal malformations of the skeletal system. METHODS: four placentas from fetuses of gestational ages 13, 16, 20 and 38 weeks, aborted for therapeutic reasons were examined. Normal placentas of the same gestational age, from cases where legal elective abortion had been recommended on medical grounds, were taken as the control. The hematoxilineosin stain was applied to ten slides in five regions of each placenta using a protocol with four quantitative variables: maturity of villi, fibrinoid changes, edema and stromal fibrosis and one qualitative variable: thrombosis. The quantitative results were analyzed using ANOVA in a randomized manner and the Tukey test was applied; for the qualitative variable the trend test for correlated data was used. The software used was Statistix 8.0 and SAS 9.0 for Windows. RESULTS: there were significant differences (p<0.05) between the placentas associated with multiple malformations of the skeletal system and control placentas in terms of the quantitative variables. No significant differences were found (p>0.05) in relation to the qualitative variable. CONCLUSIONS: the population of placental villi associated with multiple malformations of the skeletal system exhibited a high percentage of changes which is an indication that the placenta is damaged, thereby affecting the exchange of gases, nutrients and metabolites during the development of the fetus...

Cornelia-de Lange syndrome in an Egyptian infant with unusual bone deformities

We report a 4 month old female infant with the typical features of Cornelia-de Lange syndrome. What was striking in our patient was the presence of skeletal anomalies not reported previously. These included arachriodactly of both fingers and toes, flexion of thumbs at metacarpoph alengeal joints, bilateral short big toes, angulation of the lower part of the bones of right forearm and both legs with multiple skin folds. Also biochemical and X-ray evidence of rickets was detected mostly due to malnutrition and failure to thrive. The patient died at the age of 5 months with bron chopneumonia and gastroenteritis

Tomografia computadorizada da matriz óssea mineralizada heteróloga fragmentada e metilmetacrilato na reparação de falhas ósseas segmentares produzidas em tíbia de coelhos / Computed tomography to evaluate the association of fragmented heterolog cortical bone and methylmethacrylate to repare segmental bone defect produced in tibia of rabbits

Foi realizada falha segmentar de 6mm na região metafisária medial da tíbia de 12 coelhos, seguida de preenchimento desta por matriz óssea mineralizada heteróloga fragmentada conservada em glicerina (98%) e metilmetacrilato autoclavado, bem como avaliação por meio da tomografia computadorizada de feixe cônico (cone beam) aos 30, 60 e 90 dias. Houve incorporação gradativa do implante no leito receptor em relação ao tempo em 100% dos casos, o que mostra ser este biologicamente compatível, ao promover reparação da falha óssea, sem sinais de infecção, migração e/ou rejeição, caracterizando-se, assim, como nova opção de substituto ósseo para preenchimento de falhas ósseas.

A 6mm segmental defect was performed on the metaphyseal region of the tibia of 12 rabbits and the autoclaved fragmented heterolog cortical bone conserved in glycerin (98%) and methylmethacrylate was used as a bone graft for the reconstruction. The graft was placed in the receptor bed and its integration was evaluated by computed tomography after 30, 60 and 90 days. There was gradual bone graft incorporation in the receptor bed during the time in 100% of the cases. Fragmented cortical bone heterograft and methylmethacrylate was biologically compatible and promotes bone defect reparation without signs of infection, migration and or rejection, featuring a new option of osseous substitute to fill in bone defects.

Osteomielite de punho em paciente com paracoccidioidomicose disseminada: uma rara apresentação / Osteomyelitis of the wrist in a patient with disseminated paracoccidioidomycosis: a rare presentation

Paracoccidioidomicose é a micose sistêmica endêmica mais frequente no Brasil. No início, o paciente não desenvolve sintomas. Com a progressão da doença, o indivíduo pode apresentar envolvimento disseminado, sendo que o acometimento ósseo é extremamente raro. O objetivo deste artigo é avaliar as alterações ósseas encontradas em estudos de imagem em um paciente com osteomielite de punho decorrente de paracoccidioidomicose disseminada.

Paraccocidioidomycosis is the most frequently found endemic systemic mycosis in Brazil. No symptoms are observed in the early phases of the disease. As the disease progresses, the patient may present disseminated involvement, but bone involvement is extremely rare. The present report is aimed at evaluating bone changes found on imaging studies in a patient with osteomyelitis of the wrist as a result of disseminated paracoccidioidomycosis.

Comparison of the palatal connective tissue graft as a membrane with collagen membrane in combination with Bio-Oss and PRGF for the treatment of intrabony defects: a randomized clinical trial

This randomized clinical trial was aimed at comparing the outcomes of palatal connective tissue+ Bio-Oss + PRGF versuscollagen membrane + Bio-Oss+ PRGFin the treatment of intrabny defects. Fifteen patients affected by chronic periodontitis were enrolled. Each patient had at least two intrabonydefect [>/= 3mm]. They were randomly assigned into two groups: patients treated i] with collagen membrane+PRGF+Bio-oss [control group] and ii] with palatal connective tissue as membrane + PRGF+Bio-Oss [experimental group]. Clinical and intrasurgical examinations included probing depth[PD], clinical attachment level[CAL],gingival recession[GR],defect fill[DF], alveolar crest level[AC] and defect resolution[DR] were measured at baseline and after 6 months with re-entry surgery. Statistical analysis was performed usingTwo-way Repeated Measure ANOVA and Wilcoxon. After 6 months, all of the evaluated clinical parameters showed statistically significant changes from baseline within each group [p< 0/05]. The test group showed a significantly smaller amount of gingival recession as compared with control group.[0/8mm versus 1/7mm respectively; P<0/05] But there were not any statistically differences in other clinical parameters between the test and control groups[P>0/05]: pocket depth reduction [3/7mm versus 3/5mm], clinical attachment gain [1/8mm versus 1/6mm], alveolar crest loss [1/8mm versus 1/7mm], defect fill[2/3mm versus 2/2mm] and defect resolution [4/1mm versus3/9mm]. According to the results obtained from this study it can be suggested that clinical effects of application of palatal connective tissue as a membrane and collagen membrane, in combination with Bio-Oss and PRGF in treatment of vertical bone defects, did not have any statistical significant differences except for gingival recession that was statistically smaller in connective tissue group

Maffucci’s Syndrome: report of a case with oral manifestation / Síndrome de Maffucci: relato de caso com manifestação oral

Introduction: Maffucci’s Syndrome is a rare nonhereditary mesodermal dysplasia consisting of multiple haemangioma of the soft tissue and enchondromas, mostly affecting phalanges and long bones. The syndrome can also be associated with a variety of other benign and malignant tumors. Case report: Here we report a case of Maffucci’s Syndrome and haemangioma of lip and palate which is rare in this syndrome. This case report describes an 18 year old boy with multiple nodular soft tissue swellings involving the anterior hard palate and lower labial mucosa and bony abnormalities (enchondromas) involving the lower limb.

Introdução: A Síndrome de Maffucci é uma displasia mesodermal não hereditária rara. Consiste de múltiploshemangiomas de tecido mole e encondromas, a maioria afetando as falanges e os ossos longos. A síndromepode também estar associada a uma variedade de tumores benignos e malignos. Relato de caso: O presente trabalho apresenta um caso de Síndrome de Maffucci e hemangioma de lábio e palato, o que é raro nessa síndrome, descrevendo o caso de um garoto de 18 anos de idade com inchaços nodulares múltiplos de tecido mole envolvendo o palato duro anterior e a mucosa labial inferior, além de anomalias ósseas (encondromas)acometendo um membro inferior.