No dejemos morir al hueso: interiorizándonos con los procesos óseos avasculares / Do not let the Bone Die: Interiorizing with the avascular Bone Processes

Resumen El objetivo de este artículo es realizar una revisión de las localizaciones, causas y hallazgos radiológicos específicos de los procesos avasculares óseos. Se define como isquemia ósea a las alteraciones ocasionadas por déficit de irrigación, llevando a la destrucción del hueso. Existen diferencias entre necrosis isquémica e infarto óseo, en base al sitio óseo de afectación. La etiología traumática es la más frecuente y suele ser unilateral. Los sitios de presentación más frecuentes son: cabeza femoral y humeral, rodilla y semilunar. La resonancia magnética (RM) es considerada la modalidad de imagen más sensible y específica, tanto para el diagnóstico temprano como estadificación y control. El conocimiento de las causas, características radiológicas y sus diferentes fases evitan el diagnóstico erróneo de otras etiologías, como las primarias o infecciosas, facilitando un correcto algoritmo terapéutico.

Abstract The aim of this article is to review the locations, causes and specific radiological findings of avascular bone processes. Bone ischemia is defined as the alterations caused by irrigation deficit, leading into the bone destruction. However, there are differences between ischemic necrosis and bone infarction, based on the bone site of involvement. The most frequent etiology is traumatic and is usually unilateral. The most frequent sites of presentation are: femoral and humeral head, knee, and lunate. Magnetic resonance imaging (MRI) is considered the most sensitive and specific imaging modality for early diagnosis, staging and control. The knowledge of the causes, radiological findings and their different phases avoid the erroneous diagnosis of other lesions, such as primary or infectious, facilitating a correct therapeutic algorithm.

Reduced Frequency of Bone Pain Crises in Patients with Sickle Cell Disease Given an Angiotensin-converting Enzyme Inhibitor / Reducción de la frecuencia de la crisis de dolor óseo en pacientes con la enfermedad de células falciformes mediante los inhibidores de la enzima convertidora de angiotensina

ABSTRACT Objective: To determine if sickle cell disease (SCD) bone pain crisis is mitigated by use of an angiotensin-converting enzyme inhibitor (ACEI), following a case report of ACEIs preventing bone pain crisis. Methods: Patients with SCD who attended the Haematology Clinic at Jos University Teaching Hospital, Nigeria, were assessed with a questionnaire, given 2.5 mg of ramipril and followed up monthly for three months. Frequencies of bone pain crises in the month preceding enrolment and three months following treatment as well as the cardiovascular status were evaluated. Results: Thirty-five patients with complete data were reported. Blood pressure remained stable, and cumulative frequency of bone pain crises fell. The relative risk reduction for bone pain was 56.2% at one month, 63.0% at two months and 13.0% at three months. Conclusion: Vaso-occlussion-induced hypoxia, triggering bone pain crises, produces angiotensin II from angiotensin and worsens vasoconstriction. Angiotensin-converting enzyme inhibitors block this process, reducing severity or preventing bone pain crises. With these observations and a stable blood pressure profile, we recommend wider use of ACEIs in patients with SCD to cut down on the need of opioid use with attendant addiction risk, as a way of improving their quality of life.

RESUMEN Objetivo: Determinar si la crisis de dolor por la enfermedad de células falciformes (ECF) es mitigada con el uso de los inhibidores de la enzima convertidora de angiotensina (IECA), luego de un informe que reporta que IECA previene la crisis del dolor óseo. Métodos: A pacientes con ECF que asistían a la Cínica de Hematología del Hospital Docente de la Universidad de Jos, Nigeria, se les evaluó con un cuestionario, se les suministró 2.5 mg de ramipril, y se les hizo un seguimiento mensual por tres meses. Se evaluaron las frecuencias de las crisis de dolor óseo en el mes anterior al alistamiento y tres meses después del tratamiento, así como el estado cardiovascular. Resultados: Se reportaron treinta y cinco pacientes con datos completos. La presión sanguínea se mantuvo estable, en tanto que se produjo un descenso de la frecuencia cumulativa de las crisis de dolor óseo. La reducción del riesgo relativo del dolor óseo fue de 56.2% en un mes, 63.0% en dos meses, y 13.0% en tres meses. Conclusión: La hipoxia inducida por vaso-oclusión, que desencadena la crisis de dolor óseo, produce angiotensina II a partir de la angiotensina y empeora la vasoconstricción. Los inhibidores de la enzima convertidora de angiotensina bloquean este proceso, reduciendo la severidad o previniendo las crisis del dolor óseo. Con estas observaciones y un perfil de presión arterial estable, recomendamos hacer un amplio uso de IECA en pacientes con ECF para reducir la necesidad del consumo de opioides y el consiguiente riesgo de adicción, como una manera de mejorar su calidad de vida.

Bone Loss During the Acute Stage Following Burn Injury: Is It Local or Systemic? / La pérdida ósea durante la etapa aguda después de una lesión por quemaduras, ¿es local o sistémica?

ABSTRACT Objective: To examine post-burn bone loss and determine whether it was local or diffuse. Methods: Thirty-six patients with burn injuries were investigated, and the total body surface area of the burns and their locations were recorded. The bone mineral densities of the lumbar 1-4 vertebrae, bilateral distal forearm, and bilateral proximal femur of the patients were recorded, and these were compared with the measurements of the non-burnt extremity. Results: No statistically significant correlations existed among the total body surface area of the burns, their severity and the z-scores. In addition, when comparing the z-scores of the burnt extremity with those of the non-burnt extremity, no statistically significant difference was found (p > 0.05). Conclusion: In this study, a remarkable decrease in bone mass occurred during the second month following the burn injuries. The post-burn bone loss could not be correlated with the severity of the burns, but these injuries caused systemic bone loss.

RESUMEN Objetivo: Examinar la pérdida ósea después de una quemadura y determinar si era local o difusa. Métodos: Se investigó a 36 pacientes con lesiones por quemaduras y se registró el área total de la superficie del cuerpo con quemaduras y sus ubicaciones. Las densidades minerales óseas de las vértebras lumbares 1-4, del antebrazo distal bilateral, y del fémur proximal bilateral de los pacientes, fueron registradas y comparadas con las mediciones de la extremidad sin quemaduras. Resultados: No existieron correlaciones estadísticamente significativas entre el área total de la superficie corporal de las quemaduras, su severidad y las puntuaciones z. Además, al comparar las puntuaciones z de la extremidad quemada con las de la extremidad no quemada, no se encontró ninguna diferencia estadísticamente significativa (p > 0.05). Conclusión: En este estudio, se observó la ocurrencia de una disminución notable de la masa ósea durante el segundo mes tras las lesiones de la quemadura. La pérdida ósea posterior a las quemaduras no se pudo correlacionar con la severidad de las quemaduras, pero estas lesiones por quemadura causaron pérdida sistémica del hueso.

Bone disease after transplantation: osteoporosis and fractures risk / Doença óssea pós-transplante: risco de osteoporose e fraturas

Organ transplantation is the gold standard therapy for several end-stage diseases. Bone loss is a common complication that occurs in transplant recipients. Osteoporosis and fragility fractures are serious complication, mainly in the first year post transplantation. Many factors contribute to the pathogenesis of bone disease following organ transplantation. This review address the mechanisms of bone loss including the contribution of the immunosuppressive agents as well as the specific features to bone loss after kidney, lung, liver, cardiac and bone marrow transplantation. Prevention and management of bone loss in the transplant recipient should be included in their post transplant follow-up in order to prevent fractures.

Transplantes de órgão é terapia padrão-ouro para várias doenças em estágio terminal. Perda óssea é uma complicação comum que ocorre em pacientes transplantados. Osteoporose e fraturas por fragilidade são complicações sérias, principalmente no primeiro ano pós-transplante. Muitos fatores podem contribuir para patogênese da doença óssea nesses pacientes. Esta revisão aborda os mecanismos de perda óssea incluindo o papel dos agentes imunossupressores, bem como os fatores específicos da perda óssea após rim, pulmão, fígado, coração e transplante de medula óssea. A prevenção e o tratamento da perda óssea nos pacientes transplantados devem ser realizados para evitar fraturas.

Distúrbio mineral ósseo e calcificação vascular em pacientes renais crônicos / Mineral and bone disorder and vascular calcification in patients with chronic kidney disease

Calcificações vasculares têm sido associadas aos distúrbios minerais e ósseos. As alterações nas concentrações séricas de cálcio e fosfato são fatores importantes implicados no processo da calcificação arterial na doença renal crônica. A patogênese da calcificação vascular é um mecanismo complexo e não completamente claro, podendo corresponder a um processo ativo de transformação celular e ossificação heterotópica. Além da hipercalcemia e hiperfosfatemia, estão envolvidos neste processo alterações no metabolismo de substâncias inibidoras e promotoras de calcificação como a fetuína A, osteopontina, osteoprotegerina e proteína de matriz gla. Para o diagnóstico da lesão arterial calcificada, estão disponíveis diversos métodos, um método de estimativa do risco cardiovascular baseado em radiografias simples de coluna lombar e outro método baseado em radiografias simples da pelve e das mãos. Apresentamos, a seguir, uma revisão abordando a relação entre calcificações vasculares e os distúrbios minerais.

Vascular calcifications has been associated with bone and mineral disorders. The alterations in the serum level of calcium concentrations and phosphate are importants factors implicated in the arterial calcification in chronic kidney disease. The pathogenesis of vascular calcification is a complex mechanism and not completely clear, being able to correspond to an active process of cellular transformation and heterotopic ossification. Beyond the hypercalcemia and hyperphosphatemia, they are involved in this process changes in the metabolism of inhibitors and promoters of calcification such as fetuin A, osteopontin, osteoprotegerin, and matrix gla protein. For the diagnosis of the calcified arterial injury are available several complementary methods, a method of estimate of the cardiovascular risk based on plain radiographs of the lumbar column and another method based on simple x-rays of the pelvis and hands. Below, we will present a review approching the link between vascular calcifications and mineral disorders.

Novedades en enfermedades colestásicas / Latest advances in cholestatic diseases

Cholestatic liver diseases arise from impaired hepatobiliary production and excretion of bile. They have some common clinical manifestations and pathogenic features, but at the same time differences that require a special approach. Intrahepatic cholestasis of pregnancy and drug induced liver cholestasis are the most common reversible intrahepatic cholestasis. Primary biliary cirrhosis and primary sclerosing cholangitis are chronic cholestatic diseases. This review will be focused on these two types, in particular their clinical and therapeutic management and complications. While in recent years there has not been much change in the basic clinical approach of these diseases, every day we receive more information from both the basic and clinical science studies, which has enabled to develop new therapeutic lines and reject others that have not confirmed effectiveness.

Las enfermedades colestásicas se caracterizan por la disminución de la formación o excreción del flujo de bilis. Ellas tienen ciertas manifestaciones clínicas y mecanismos patogénicos comunes, pero a su vez diferencias que requieren un enfrentamiento no siempre similar. La colestasia intrahepática del embarazo(CIE) y la colestasia secundaria a fármacos son las más frecuentes dentro de las colestasias intrahepáticas reversibles. La cirrosis biliar primaria (CBP) y la colangitis esclerosante primaria (CEP), son las enfermedades colestásicas crónicas en que centramos esta revisión, en particular en los aspectos clínicos y de manejo terapéutico tanto de éstas, como de sus complicaciones. Si bien en los últimos años no ha habido un cambio significativo en el manejo fundamental de estas enfermedades, cada vez tenemos más información tanto en el área de ciencias básicas como en aspectos clínicos, lo que ha permitido ir desarrollando nuevas líneas terapéuticas y descartando otras que no han confirmado efectividad.

Bone disease in thyrotoxicosis.

Thyrotoxicosis, a clinical syndrome characterized by manifestations of excess thyroid hormone, is one of the commonly-recognised conditions of the thyroid gland. Thyrotoxicosis causes acceleration of bone remodelling and though it is one of the known risk factors for osteoporosis, the metabolic effects of thyroxine on bone are not well discussed. Studies show that thyroid hormones have effects on bone, both in vitro and in vivo. Treatment of thyrotoxicosis leads to reversal of bone loss and metabolic alterations, and decreases the fracture risk. There are limited studies in India as to whether these changes are fully reversible. In this review we discuss about the effects of thyrotoxicosis (endogenous and exogenous) on bone and mineral metabolism, effects of subclinical thyrotoxicosis on bone and mineral metabolism and effects of various forms of treatment in improving the bone mineral density in thyrotoxicosis.

Cushing's syndrome: aftermath of the cure

Cushing's syndrome (CS) is a chronic and systemic disease caused by endogenous or exogenous hypercortisolism, associated with an increase of mortality rate due to the clinical consequences of glucocorticoid excess, especially cardiovascular diseases. After cure, usually obtained by the surgical removal of the tumor responsible for the disease, the normalization of cortisol secretion is not constantly followed by the recovery of the clinical complications developed during the active disease, and it is often followed by the development of novel clinical manifestations induced by the fall of cortisol levels. These evidences were mostly documented in patients with pituitary-dependent CS, after surgical resection of the pituitary tumor. Indeed, despite an improvement of the mortality rate, metabolic syndrome and the consequent cardiovascular risk have been found to partially persist after disease remission, strictly correlated to the insulin resistance. Skeletal diseases, mainly osteoporosis, improve after normalization of cortisol levels but require a long period of time or the use of specific treatment, mainly bisphosphonates, to reach the normalization of bone mass. A relevant improvement or resolution of mental disturbances has been described in patients cured from CS, although in several cases, cognitive decline persisted and psychological or psychiatric improvement was erratic, delayed, or incomplete. On the other hand, development or exacerbation of autoimmune disorders, mainly thyroid autoimmune diseases, was documented in predisposed patients with CS after disease remission. The totality of these complications persisting or occurring after successful treatment contribute to the impairment of quality of life registered in patients with CS after disease cure.

A síndrome de Cushing (SC) é uma desordem sistêmica crônica causada por hipercortisolismo endógeno ou exógeno, associada a um aumento da taxa de mortalidade devido às conseqüências clínicas do excesso de glicocorticóides, especialmente a doença cardiovascular. Após a cura, usualmente obtida pela remoção cirúrgica do tumor responsável pela desordem, a normalização da secreção de cortisol não é sistematicamente seguida da recuperação das complicações clínicas desenvolvidas durante a fase ativa da doença, e é freqüentemente seguida pelo surgimento de novas manifestações clínicas induzidas pela queda dos níveis de cortisol. Estas evidências foram, na sua maioria, documentadas em pacientes com SC de origem hipofisária, após a ressecção cirúrgica do tumor na hipófise. Na verdade, a despeito de uma melhoria na taxa de mortalidade, a síndrome metabólica e seu conseqüente risco cardiovascular têm se mostrado parcialmente persistentes após a remissão da doença, em estrita relação com a resistência à insulina. Anormalidades esqueléticas, especialmente a osteoporose, melhoram após a normalização dos níveis de cortisol, mas requerem um longo tempo ou o uso de tratamento específico, principalmente bisfosfonatos, para se obter a normalização da massa óssea. Uma melhora significativa ou mesmo resolução dos distúrbios mentais têm sido descritos em pacientes curados da SC, embora em vários casos o declínio cognitivo persista e a melhora psicológica ou psiquiátrica tenham sido erráticas, demoradas ou incompletas. Por outro lado, o desenvolvimento ou exacerbação de processos autoimunes, em especial as doenças autoimunes da tiróide, foram documentadas em pacientes predispostos com SC, após a remissão da doença. A totalidade dessas complicações, persistentes ou ocorrendo após o tratamento bem sucedido, contribuem para um prejuízo da qualidade de vida registrado em pacientes com SC após a cura da doença.

Pathoecology and paleodiet in Postclassic: Historic Maya from northern coastal Belize

This paper examines the synergism among diet, disease, and ecology at two related coastal Maya sites in Belize (Marco Gonzalez and San Pedro) for the Postclassic and Historic periods (1350-1650 AD), which immediately follow the Classic period collapse. Stable carbon- and nitrogen-isotope ratios in collagen and stable carbon-isotope ratios in structural carbonate were analysed for bones from 65 humans and a wide variety of faunal species. There are no apparent differences in whole diets or degree of carnivory between individuals with lesions indicative of anemia and those without, but those with lesions appear to have consumed significantly more C4 foods and protein from lower trophic levels. Non-specific infection (periostitis) and vitamin C deficiency (scurvy) are also present in high frequencies and appear to co-occur with lesions indicative of anemia, particularly in childhood. Individuals with scurvy also appear to have consumed significantly more C4 foods than normal individuals. Spondyloarthropathy is common in adults. These findings are discussed in light of: (1) the debate on how anemia versus scurvy are manifest and diagnosed, (2) Spanish ethnohistoric descriptions of the poor state of Maya health at the time of contact, and (3) the Osteological Paradox. We suggest that although this coastal environment exacerbated morbidity because of possible parasitic infection, the inhabitants were probably able to survive physiological stresses better than either their inland contemporaries or their modern counterparts.

Análise imaginológica dos osteomas / Osteomas imaging evaluation

Osteomas são tumores osteoblásticos benignos, compostos de osso maduro, compacto ou medular. Possuem etiologia aparentemente desconhecida, apesar de existirem teorias para explicar sua aparição. São essencialmente restritos ao esqueleto craniofacial e raramente encontrados em outros ossos, sendo, geralmente, diagnosticados ao acaso durante tomadas radiográficas de rotina nessa região. Seu crescimento lento e com ausência de sintomatologia dolorosa dificulta ainda mais o diagnóstico precoce. Existem variações entre autores quanto à forma de abordagem e a técnica utilizada para intervenção, contudo, a indicação de tratamento é a remoção cirúrgica. Este trabalho tem como finalidade fazer uma breve revista de literatura e demonstrar algumas imagens de diversos osteomas

Displasia cleidocraniana: relato de caso clínico / Cleidocranial dysplasi: case report

Paciente de 24 anos, sexo feminino, portadora de sinais característicos da Displasia Cleidocraniana foi submetida a exames clínico e radiográfico, os quais evidenciaram anormalidades na aparência física e constituição esquelética da mesma, confirmando a suspeita diagnóstica. O tratamento proposto foi excisão cirúrgica de numerosos dentes inclusos, para viabilizar posterior reabilitação protética. A peça cirúrgica foi encaminhada para exame histopatológico, para avaliar a presença de lesão cística e possíveis distúrbios na constituição morfológica dos dentes removidos. Diante da raridade desta síndrome, este artigo objetiva relatar os aspectos gerais da doença e a terapêutica utilizada

Compromiso óseo en histiocitosis de celulas de Langerhans en el niño: estudio radiológico simple: presentación clínica y diagnóstico radiológico / Bone compromised in childrens with Langerhans cell histiocytosis: clinical report and radiological diagnosis

Introducción. La Histiocitosis de células de Langerhans (HCL) es una patología poco frecuente, con diferentes manifestaciones radio-lógicas. Su causa es desconocida y se caracteriza por una proliferación de las células de Langerhans. Sus manifestaciones varían desde lesiones óseas aisladas hasta el compromiso sistémico. Muchas veces el primer estudio diagnóstico corresponde a la radiografía simple de la zona afectada y sus hallazgos pueden ser difíciles de interpretar. El diagnóstico diferencial debe incluir fundamen-talmente osteomielitis, sarcoma de Ewing y linfoma. Objetivos: Mostrar las manifestaciones radiológicas de la HCL, que permiten al radiólogo sospechar el diagnóstico. Material y métodos: Análisis retrospectivo de las manifestaciones clínicas y radiológicas en los casos de HCL confirmados con histología, que forman parte del Registro Nacional de Tumores Oseos (RENATO) y que fueron incorporados a éste entre los años 1959 y 1999. Resultados: Se obtuvo un total de 59 pacientes, 34 (58 por ciento) varones y 25 (42 por ciento) niñas en quienes el estudio histológico fue compatible con el diagnóstico de HCL. El rango de edad varió desde 0.8 hasta 17 años (promedio 6.1 años). Se tuvo acceso a las historias clínicas en 56 de los casos. El tiempo promedio de duración de los síntomas fue de 5,5 meses, y el motivo de consulta más frecuente fue dolor del sitio afectado (n= 50). Del total, 48 (81 por ciento) presentaron lesión ósea única y 11 (19 por ciento) dos o más lesiones. En 35 pacientes hubo compromiso de huesos largos y en 26, del esqueleto axial. El patrón radiológico predominante fue el de una lesión osteolítica pura (45 pacientes). En 13 pacientes se observó un patrón permeativo y 3 se presentaron con una vértebra plana. En 30 había reacción perióstica y 29 tenían aumento de volumen de las partes blandas vecinas. Conclusiones: El patrón de presentación radiológica de la HCL es variado, sin embargo su diagnóstico debe ser sospechado por el médico rad...

Frequency of hypercalcemia in patients of multiple myeloma in Karachi

To determine the frequency of hypercalcemia and skeletal abnormalities in multiple myeloma patients. Cross-sectional study. Study was conducted from January 1999 to July 2004 at the Aga Khan University Hospital [AKUH], a tertiary care hospital in Karachi, Pakistan. Medical records of all the diagnosed cases of multiple myeloma at their presentation to hospital were reviewed. The frequency of hypercalcemia and skeletal lesions was calculated. Independent sample test and chi-square test as appropriate, was applied to calculate the difference in parameters between normocalcemic and hypercalcemic patients. Sixty-seven percent patients were males and 33% were females. The mean age was 61 +/- 11 years. Of the 105 study patients, 51.2% had hypercalcemia [S.Ca >2.64 mmol/l]. Radiological survey showed different levels of skeletal involvement in 89.8% of patients. It was also found that 94.3% of hypercalcemic patients had skeletal lesions, out of which 20% had advanced [stage 3] bone lesions. Also noteworthy was the fact that 84.6% of normocalcemic patients had mild /moderate [stage 1 and 2] skeletal lesions. There was higher frequency of hypercalcemia in multiple myeloma patients in this series. Skeletal lesions were present in significant number of these patients. It is suggested that patients with multiple myeloma should be checked for serum corrected calcium and radiological surveys be included in routine workup for early detection and prevention of hypercalcemia and skeletal lesions