Bone disease in primary hyperparathyroidism / Doença óssea no hiperparatiroidismo primário

Bone disease in severe primary hyperparathyroidism (PHPT) is described classically as osteitis fibrosa cystica (OFC). Bone pain, skeletal deformities and pathological fractures are features of OFC. Bone mineral density is usually extremely low in OFC, but it is reversible after surgical cure. The signs and symptoms of severe bone disease include bone pain, pathologic fractures, proximal muscle weakness with hyperreflexia. Bone involvement is typically characterized as salt-and-pepper appearance in the skull, bone erosions and bone resorption of the phalanges, brown tumors and cysts. In the radiography, diffuse demineralization is observed, along with pathological fractures, particularly in the long bones of the extremities. In severe, symptomatic PHPT, marked elevation of the serum calcium and PTH concentrations are seen and renal involvement is manifested by nephrolithiasis and nephrocalcinosis. A new technology, recently approved for clinical use in the United States and Europe, is likely to become more widely available because it is an adaptation of the lumbar spine DXA image. Trabecular bone score (TBS) is a gray-level textural analysis that provides an indirect index of trabecular microarchitecture. Newer technologies, such as high-resolution peripheral quantitative computed tomography (HR-pQCT), have provided further understanding of the microstructural skeletal features in PHPT.

A doença óssea no hiperparatiroidismo primário grave é representada pela osteíte fibrosa cística (OFC). Dor óssea, deformidades esqueléticas e fraturas patológicas são achados comuns na OFC. A densidade mineral óssea está, usualmente, extremamente diminuída na OFC, mas é reversível após a cura cirúrgica. Os sinais e sintomas da doença óssea grave incluem dor óssea, fraturas patológicas e fraqueza muscular proximal com hiper-reflexia. O comprometimento ósseo é tipicamente caracterizado pela aparência em “sal-e-pimenta” nos ossos do crânio, erosões ósseas e reabsorção das falanges, tumores marrons e cistos. Na radiografia, observam-se desmineralização difusa e fraturas patológicas especialmente nos ossos longos das extremidades. No hiperparatiroidismo primário (HPTP) sintomático grave, as concentrações séricas de cálcio e PTH estão usualmente bem elevadas e o comprometimento renal se caracteriza pela presença de urolitíase e nefrocalcinose. Uma nova tecnologia, recentemente aprovada para uso clínico nos Estados Unidos e na Europa, torna-se provável se difundir rapidamente, pois utiliza as imagens geradas pela densitometria DXA. O escore trabecular ósseo (TBS), obtido por meio da análise do nível da textura cinza das imagens dos corpos vertebrais, fornece informações indiretas sobre a microarquitetura trabecular. Novos métodos, como a tomografia de alta resolução quantitativa periférica computadorizada (HRpqCT), têm proporcionado conhecimentos adicionais sobre os achados da microarquitetura esquelética no HPTP.

Relación de la gingivitis crónica con algunas afecciones del sistema osteomioarticular según holograma del microsistema de dientes alemán / Relationship of chronic gingivitis with some conditions of the osteomyoarticular system according to hologram of the German dental microsystem

Se realizó un estudio descriptivo y transversal de 77 pacientes de ambos sexos y edades entre 20-45 años con alguna afección del sistema osteomioarticular, atendidos en la consulta estomatológica de la parroquia Leoncio Martínez de Caracas, Venezuela, desde enero hasta Junio de 2009, a fin de relacionar la gingivitis crónica con algunas afecciones de este sistema según holograma del microsistema de dientes alemán. En la casuística, el meridiano más afectado fue pulmón-intestino grueso; predominaron las dolencias del sistema osteomioarticular en el lado izquierdo y la mayoría de campos de interferencia con la gingivitis crónica estuvieron en la parte derecha de la cavidad bucal. Asimismo, se hallaron alteraciones en la columna vertebral de los afectados con gingivitis leve, moderada y avanzada

A descriptive and cross-sectional study was conducted in 77 patients of both sexes and ages between 20-45 years with some condition of the osteomyoarticular system, attended in the dental department of the parish Leoncio Martínez from Caracas, Venezuela, from January to June 2009, in order to relate chronic gingivitis to some conditions of this system according to hologram of the German dental microsystem. In the case material the most affected meridian was lung-large intestine; osteomyoarticular system conditions prevailed on the left and most of the interference fields with chronic gingivitis were on the right side of the oral cavity. Also, abnormalities in the spine of those patients with mild, moderate and severe gingivitis were found

Symptomatic Os Infranaviculare

The author observed a new accessory bone of the foot in the distal portion of navicular, which articulated with the medial cuneiform and the intermediate cuneiform, and named it os infranaviculare. A degenerative change was observed between the accessory bone and the navicular; this caused midfoot pain to the patient during weight-bearing. Thus, the patient was treated by excision of the accessory bone. The symptom was relieved at one-year postoperative.

Skeletal Manifestations of Hydatid Disease in Serbia: Demographic Distribution, Site Involvement, Radiological Findings, and Complications

Although Serbia is recognized as an endemic country for echinococcosis, no information about precise incidence in humans has been available. The aim of this study was to investigate the skeletal manifestations of hydatid disease in Serbia. This retrospective study was conducted by reviewing the medical database of Institute for Pathology (Faculty of Medicine in Belgrade), a reference institution for bone pathology in Serbia. We reported a total of 41 patients with bone cystic echinococcosis (CE) during the study period. The mean age of 41 patients was 40.9+/-18.8 years. In 39% of patients, the fracture line was the only visible radiological sign, followed by cyst and tumefaction. The spine was the most commonly involved skeletal site (55.8%), followed by the femur (18.6%), pelvis (13.9%), humerus (7.0%), rib (2.3%), and tibia (2.3%). Pain was the symptom in 41.5% of patients, while some patients demonstrated complications such as paraplegia (22.0%), pathologic fracture (48.8%), and scoliosis (9.8%). The pathological fracture most frequently affected the spine (75.0%) followed by the femur (20.0%) and tibia (5.0%). However, 19.5% of patients didn't develop any complication or symptom. In this study, we showed that bone CE is not uncommon in Serbian population. As reported in the literature, therapy of bone CE is controversial and its results are poor. In order to improve the therapy outcome, early diagnosis, before symptoms and complications occur, can be contributive.

Osteonecrosis maxilar por bifosfonatos: diagnóstico, prevención y tratamiento / Biphosphonates-induced osteonecrosis of the jaws: diagnosis, prevention and treatment

El empleo de los bifosfonatos es una medicación eficaz en el tratamiento de lesiones osteolíticas. Sin embargo, con el tiempo de su utilización, se observaron en algunos casos la aparición de osteonecrosis en los huesos maxilares. Ante esta patología agregado, se determinaron procedimientos para minimizar estos efectos secundarios.

Gingival fibromatosis with hemi-osseous hyperplasia of jaws, focal maxillary viral papillomatosis of gingiva, fissured tongue and congenitally missing anterior teeth: A case report and surgical management of a new syndrome.

Gingival fibromatosis is characterized by fibrotic enlargement of the gingiva that can occur as inherited or sporadic form. Inherited form can be an isolated trait or as a component of a syndrome. This article reports a 35 year old male patient affected by gingival fibromatosis associated with hemiosseous hyperplasia of mandible, maxilla, and zygoma on the right side, viral papillomatosis of maxillary anterior gingiva, fissured tongue and congenitally missing anterior teeth. The patient was subjected to phase I and phase II periodontal therapy. There was no evidence of recurrence of the enlargement after one year but the papillomatosis recurred. Gingival fibromatosis has been reported to be associated with various other abnormalities but not with those described in our case. This observation raises the possibility that the coexistence of these entities in our case may represent a new syndrome.

Etiopathogenesis of disk displacement of the temporomandibular joint: A review of the mechanisms.

Disk displacement of the temporomandibular joint (TMJ) is a clinically important condition, showing a high prevalence in both patient and non-patient populations. Despite its clinical importance, there is incomplete understanding of the etiopathogenic mechanisms leading to disk displacement. A number of possible risk factors have been identified. This article analyzes the etiopathogenesis from both the clinical and the biomechanical viewpoints and also reviews the literature on the association between disk displacement and the main risk factors (i.e., trauma, altered disk shape and/or dynamic properties, occlusal abnormalities, steepness of the articular eminence, hyperactivity of the lateral pterygoid muscle, joint hypermobility, etc.). According to our interpretation of available data, an impairment of joint lubrication may be a common finding in cases of disk displacement, thus suggesting the need for future studies addressing both local and systemic neuroendocrine aspects influencing the friction coefficient of the TMJ. A full comprehension of the etiopathogenesis of disk displacement is far from being achieved, and clinicians must take into account this consideration when treating patients with temporomandibular disorders.

Visão geral da doença óssea na doença renal crônica (DRC) e nova classificação / Bone disease in chronic kidney disease (CKD): general view and new classification

Os distúrbios na homeostase do cálcio, do fósforo, do calcitriol e do paratormônio ocorrem precocemente nos pacientes com doença renal crônica (DRC) e desempenham papel fundamental na fisiopatologia das doenças ósseas que acometem esses pacientes. Essa síndrome antes conhecida pelo nome“osteodistrofia renal” (ODR) foi modificada pelo KDIGO (Kidney Disease: Improving Global Outcomes), recebendo uma denominação mais ampla que reúne as alterações clinicas, bioquímicas e ósseas, além das calcificações extra-ósseas, freqüentemente observadas na doença renal crônica. Essa síndromerecebeu o nome “distúrbio mineral e ósseo da doença renal crônica” (DMO-DRC) e o termo ODR ficou reservado para as alterações na histologia ósseaavaliadas por biópsia.

Chronic kidney disease (CKD) is accompanied by disturbances in calcium, phosphate, calcitriol, and parathyroid hormone (PTH) homeostasis that play animportant role in the pathophysiology of renal bone disease and are an important cause of morbidity. These disturbances have traditionally been termed renalosteodystrophy and classified based on bone biopsy. “Kidney Disease: Improving Global Outcomes” (KDIGO) recommended that the term renal osteodystrophy should be used exclusively to define alterations in bone histology associated with CKD, and proposed a new term to describe the syndrome of biochemical,bone and extra-skeletal calcification abnormalities that occur in these patients. The new term is CKD-MBD (CKD-Mineral and Bone Disorder).

Modalidade de diálise e o controle do hiperparatireoidismo secundário / Modality of dialysis and the management of secondary hyperparathyroidism

A redução dos níveis de vitamina D e a retenção de fósforo na doença renal crônica estimulam a proliferação celular da paratireóide, enquanto a liberaçãoaguda de PTH pela glândula é mais dependente do nível de cálcio ionizado no líquido extracelular. Os balanços de fósforo e de cálcio nos pacientes emdiálise assim como a influência que exercem sobre a função da paratireóide e a remodelação óssea são modificáveis por alguns medicamentos como osquelantes de fósforo e a vitamina D e, também, pela prescrição da diálise. Em especial, a concentração de cálcio no dialisato, tanto na hemodiálise quantona diálise peritoneal, pode ter um papel importante no manuseio do hiperparatireoidismo secundário.

Low vitamin D levels and phosphorus retention stimulate parathyroid cell proliferation in chronic kidney disease, whereas acute glandular PTH release is mainly dependent on ionized calcium concentration in the extracellular fluid. Phosphorus and calcium balance in patients on dialysis and their effects on parathyroid function and bone turnover can be influenced by medications such as phosphate binders and vitamin D as well as by dialysis prescription. Of note, the calcium concentration in dialysis solution, on either hemodialysis or peritoneal dialysis can have a special role in the management of renal osteodystrophy.

Doença óssea em glomerulopatia primária / Bone disease in primary glomerulopathy

O objetivo deste estudo foi analisar o metabolismo ósseo de pacientes com proteinúria glomerular sem uso prévio de drogas que afetassem esse metabolismo. Dezessete pacientes foram estudados com biópsia óssea para análise histomorfométrica e fragmentos ósseos foram obtidos para cultura de célula (n=13) na qual nós avaliamos proliferação de osteoblasto. A comparação dos achados histomorfométricos a controles de literatura demonstrou uma diminuição da remodelação óssea e comprometimento de sua microarquitetura. Corroborando com esse resultado houve diminuição da proliferação dos osteoblastos dos pacientes quando comparados a controles (n=5) doadores de órgãos. Análise bioquímica revelou correlação negativa da 25(OH)D3 com a proteinúria e positiva com a proliferação dos osteoblastos em cultura / The objective of this study was to analyze bone metabolism in proteinuria glomerular patients not having previously used drugs affecting bone metabolism. Seventeen patients were studied with histomorphometric analysis of bone biopsies and bone fragments were obtained for cell culture (n = 13), in which we evaluated osteoblastic proliferation. Comparing patients to controls of literature indicate reduced bone remodeling and altered bone microarchitecture. In corroboration, mean osteoblast proliferation was lower in patient samples when compared with those for normal osteoblasts obtained from age-matched, gender-matched donor organs (n = 5). Concentrations of 25-hydroxyvitamin-D3 correlated negatively with proteinuria and positively with osteoblast proliferation in culture...

Humerus Varus in a Patient with Pseudohypoparathyroidism

A 7-yr-old girl presented with progressive shortening of the right upper arm and limitation of shoulder motion. Pseudohypoparathyroidism associated with Albright's hereditary osteodystrophy was diagnosed by biochemical, hormonal and radiographic studies. Her condition was complicated by severe humerus varus on the right side. Proximal humeral valgization osteotomy and concomitant humeral lengthening resulted in an improvement of the shoulder joint motion and activity in daily life.

Doença vestibular periférica decorrente de osteoartropatia temporoioídea em um eqüino / Peripheral vestibular disease associated with temporohyoid osteoarthropathy in a horse

Um eqüino com 22 anos de idade apresentou síndrome vestibular periférica associada à paralisia de nervo facial esquerdo devido à osteoartropatia temporoioídea. O exame endoscópico das bolsas guturais mostrou alteraçäo de contorno da bula timpânica esquerda e aumento de volume da extremidade proximal do osso estiloióide do mesmo lado.

Giant cell reparative granuloma of the base of the skull presenting as a parapharyngeal mass.

Giant cell reparative granuloma is an infrequent non-tumoral lesion affecting the maxillary and mandibular bones and only rarely, the cranial bones. We report a case of giant cell reparative granuloma of the base of the skull in a 12-year-old female who presented with a parapharyngeal mass. Computerized Tomography (CT) showed the intracranial extension and destruction of surrounding bones.

Triángulo de Codman vs. signos de malignidad. Presentación de 2 casos / Codman triangle versus malignancy signs. Report of 2 cases

Las reacciones periostáticas son comunes en las lesiones óseas. A pesar de la facilidad con que se las reconoce radiográficamente, existen considerables aprehensiones infundadas sobre su diagnóstico. El triángulo de Codman es una imagen descrita por primera vez en 1914 por Ribbert, quien la atribuyó a una elevación perióstica causada por una masa de expansión. Considerado desde tiempo atrás como una manifestación de osteopatía maligna, en la actualidad se admite que puede deberse a cualquier bulto que levante el periostio, sea benigno o maligno. Se presentan 2 casos en los cuales aparece en las radiografías, desde las etapas iniciales, reacción perióstica tipo triángulo de Codman cuyo diagnóstico de anatomía patológica corresponde a lesiones tumorales benignas

Bone paracoccidioidomycosis in the HIV positive patient [Letter]

AIDS patients are vulnerable to infection by opportunistic microbes, including various fungi such as Pneumocystis carinii, Cryptococcus neoformans, Histoplasma capsulatum, Candida albicans and many others. However, the association of AIDS and infection with Paracoccidioides brasiliensis has been rarely recorded. We report a case of an HIV-positive patient with bone infections by this fungus with a clinical form not previously published. This clinical presentation included primarily a massive bone lesion, but it did not included the lymphatic and disseminated disease described in HIV-positive patients. The patient responded well to medical and surgical treatment. We suggest that patients with moderate, rather than severe, immunosuppression may have forms of paracoccidioidomycosis with a patho;ogic process intermediate to those sen in the immunologically normal host and the full AIDS syndrome.

Transplante de peroné vascularizado. Reconstrucción de pérdidas óseas segmentarias mayores de 6 cm. Informe de 11 casos / Microvascular fibular graft transplantation. Reconstruction for segmental bone loss greater than six centimeters: report of 11 cases

Uno de los problemas más difíciles de resolver a los que se enfrenta el ortopedista es la reconstrucción de grandes pérdidas óseas segmentarias secundarias a traumatismos, malformaciones congénitas, infecciones óseas o a resecciones en bloque por tumores óseos. La reconstrucción con aportes osteogénicos convencionales es impredecible en defectos mayores de 6 cm. El transplante microvascular de hueso vascularizado ha probado ser una técnica efectiva, ya que no tiene que existir sustitución celular, sino que se integra consolidando en sus extremos como una fractura. En este trabajo se presentan 11 casos con pérdidas óseas segmentarias mayores de 6 cm, tratados con transplante de peroné microvascular en el Instituto Nacional de Ortopedia durante el periodo comprendido de marzo de 1993 a julio de 1995. De los 11 pacientes, seis correspondieron al sexo masculino (54.5 por ciento). Las edades oscilaron entre cinco y 33 años (promedio de 19.9 años). La etiología fue multifactorial: pseudoartrosis postraumática en cinco casos, osteomielitis hematógena en uno, pseudoartrosis congénita de tibia en dos, resecciones en bloque por tumores óseos en dos y pérdida segmentaria debido a herida por proyectil de arma de fuego en un caso. Diferentes huesos largos fueron afectados: radio (tres casos), tibia (tres), húmero (dos) y fémur (dos); uno de los pacientes presentó afección de cúbito y radio. El tiempo de evolución en los casos crónicos osciló de seis meses a 13 años (promedio de 4.2 años); sólo un paciente fue tratado en estado agudo. Nueve sujetos ya habían sido intervenidos quirúrgicamante en varias ocasiones. Las pérdidas óseas variaron desde 8 hasta 26 cm (promedio de 13.4 cm). La longitud del injerto fue de 9 a 30 cm (promedio de 17.5 cm). El tipo de osteosínteis que utilizamos varió en cada caso en particular; en seis casos se utilizó clavo centromedular más inmovilización externa con fibra de vidrio o yeso, en cuatro se empleó fijador externo y en un caso tornillos de cortical. Se utilizó isla de piel en nueve pacientes. Los tiempos quirúrgicos variaron de 6 a 12 horas (promedio de 7.57 horas). En todos los casos se presentó hipertrofia del peroné. Los tiempos de consolidación oscilaron entre nueve y 16 semanas. Las complicaciones que se presentaron no difieren de las consignadas en otras series. Los resultados obtenidos son alentadores, ya que en todos los casos existió integración del peroné transplantado con tiempos de consolidación similares a los de...

Las pérdidas óseas infectadas. Tratamiento con clavo Colchero y fijador externo / Infected bone loss. Treatment by colchero's intramedullary interlocked rod and external fixator

Se presentan 68 casos de pérdidas óseas en tibia y fémur, originadas por diafisectomía como tratamiento en infecciones, 53 como tratamiento previo de infección con diafisectomía en otros hospitales, persistiendo la sepsis y otros 15 tratados por nosotros a los cuales tuvimos que hacer diafisectomía por denudación completa de periostio y necrosis (únicos casos en que hacemos resección ósea) y posteriormente, "escarificaciones" para control de las sepsis. Fueron 52 hombres y 16 mujeres. Edad promedio de 35 años. Tibia 38 casos, fémur 28 y resección de dos rodillas. Osteosíntesis inmediata al control de la infección: 10 casos con clavo Colchero y pernos (pérdidas pequeñas); 58 casos con clavo Colchero, pernos y fijador externo, con clavos de Steinmann dentro de los orificios del clavo y en algunos casos en hueso. En pérdidas óseas pequeñas injerto esponjoso autólogo y en grandes el mismo, mas peroné libre vascularizado. Heridas cerradas por sutura secundaria, colgajos libres vascularizados o rotados, injertos de piel y epitelización. Inicio de reahabilitación al primer día postoperatorio, marcha con apoyo total con promedio 15 días (3-30). Consolidación en 66 pacientes. Tres casos de abandono: en uno reactivación de la sepsis y consolidación; en dos, aflojamiento de fijador con pseudoartrosis. El retiro de fijadores en todos los enfermos se efectuó en la consolidación grado dos, dejando clavo y pernos

Linfomas primarios de hueso: reporte de 3 casos en Costa Rica / Primary bone lymphoma: report of 3 cases in Costa Rica

Los linfomas óseos primarios son raros; en este trabajo se presentan tres casos encontrados en 25 años en los archivos de Anatomía Patológica del Hospital México, San José, Costa Rica. Dos pacientes eran mujeres y uno varón, con edades de 31, 36 y 52 años . Todos los casos ameritaron tratamiento combinado de cobalto y quimioterapia eventualmente, a pesar de que los estudios iniciales determinaron que la neoplasia estaba localizada (estadío IEA). Los tres casos fueron clasificados histológicamente como linfomas de células grandes no hendidas, según la Formulación de Trabajo para Uso Clínico (1), con inmunofenotipo B demostrado por análisis inmunohistoquímicos. Dos pacientes están vivos y uno falleció con linfoma, con un seguimiento promedio de 48 meses