骨斑点症的X线分析(附6例报道及文献复习)

目的:对骨斑点症的临床特征、发病机理及鉴别诊断进行讨论,并探讨骨斑点症的X线特征,以提高对该病的影像学认识。方法:回顾性分析6例骨斑点症患者的临床及X线表现,其中3例患者属于同一家族,并对1例28岁患者随访1年,并复习相关文献。结果:病变好发部位为骨盆、跗骨、腕骨、指(趾)骨和长管状骨的骨骺及干骺端,影像表现为0.2~1.6 cm散在多发的大小不等的圆形或卵圆形致密阴影为特征,靠近关节面病灶较密集,大部分病灶位于松质骨内,病灶长轴与骨小梁走行相平行,密度均匀,边界清晰,相邻关节软骨骨质未见异常改变,其中4例病灶呈双侧对称性分布。1例随访病灶未见有明显变化。6例患者无任何症状,男女比为2:1,实验室相关检查均未见异常。结论:骨斑点症属于骨发育异常,是一种遗传性疾病,具有遗传学特点,X线检查是发现和诊断本病的主要依据,其X线表现具有特征性,熟悉其临床及X线特征有助于提高对该病的诊断。     

The skeleton of the eye: II. Overlap of the scleral ossicles of the domestic fowl

The scleral ossicles, a ring of overlapping membrane bones, lie just outside the corneal margin in the eyes of domestic fowl. Eighty percent of the bony rings contain 14 bones; less than 1% have 13 bones; 19% have 15 bones; about 1% have 16 bones. Each bone is foreshadowed during development by a transient papilliform thickening in the overlying conjunctival epithelium. These conjunctival papillae appear on the eighth day of incubation and disappear on the twelfth day, when the corresponding preosseous membranes begin to ossify. Observations, and experiments involving the removal of specific papillae early or late in their maturation (in order to delete, or to reduce the size of, individual bones), demonstrated the following constraints on the morphogenesis of the scleral ossicular ring. (1) The number of ossicles is a function, not only of the number of papillae, but also of the distance between adjacent papillae; when two papillae lie close together, a single ossicle may arise beneath the pair. (2) There are three regions in the ring: nasal, dorsal (in both of which the bones overlap in one direction) and temporal (in which the bones overlap in the opposite direction). (3) The determinants of the direction of overlap between adjacent bones are extrinsic to the ossicles themselves and are distributed throughout each region, rather than confined to the discrete locations within each region where overlap normally occurs. (4) The three places in the ring where these regions meet are characterized by the loss (in 13-membered rings) or the addition (in 15- and 16-membered rings) of papillae and of their corresponding bones, and by transitions in the direction of overlap between the bones.     

Correlations between rates of epiphyseal and diaphyseal elongation in the short bones of the hand

Serial annual radiographs of the hand have been used to analyze the rates of elongation of the epiphyses and diaphyses of the metacarpals and phalanges in children at ages from 3 to 13 years. The rates of elongation for many corresponding ephiphyses and diaphyses (i.e., of the same bone) are negatively correlated but to an extent that is not statistically significant for any particular bone. This tendency toward negative correlations is found for most of these bones although the correlation coefficients for most of the metacarpals are positive in each sex. Within rays, the correlation indices between the rates of elongation for corresponding epiphyses and diaphyses (i.e., of the same bone) have larger negative or smaller positive values than for those between either adjacent and non-corresponding or non-adjacent and non-corresponding epiphyses and diaphyses (i.e., not of the same bone but of either adjacent or non-adjacent bones). The communality indices for the ratio between the rates of epiphyseal and diaphyseal elongation in particular bones are more highly correlated in the girls than in the boys and within the rows than within the rays. Some implications of the tendency to negative correlations between the rates of elongation of corresponding epiphyses and diaphyses have been discussed.     

Cell lineage in mammalian craniofacial mesenchyme

We have analysed the contributions of neural crest and mesoderm to mammalian craniofacial mesenchyme and its derivatives by cell lineage tracing experiments in mouse embryos, using the permanent genetic markers Wnt1-cre for neural crest and Mesp1-cre for mesoderm, combined with the Rosa26 reporter. At the end of neural crest cell migration (E9.5) the two patterns are reciprocal, with a mutual boundary just posterior to the eye. Mesodermal cells expressing endothelial markers (angioblasts) are found not to respect this boundary; they are associated with the migrating neural crest from the 5-somite stage, and by E9.5 they form a pre-endothelial meshwork throughout the cranial mesenchyme. Mesodermal cells of the myogenic lineage also migrate with neural crest cells, as the branchial arches form. By E17.5 the neural crest-mesoderm boundary in the subectodermal mesenchyme becomes out of register with that of the underlying skeletogenic layer, which is between the frontal and parietal bones. At E13.5 the primordia of these bones lie basolateral to the brain, extending towards the vertex of the skull during the following 4-5 days. We used DiI labelling of the bone primordia in ex-utero E13.5 embryos to distinguish between two possibilities for the origin of the frontal and parietal bones: (1) recruitment from adjacent connective tissue or (2) proliferation of the original primordia. The results clearly demonstrated that the bone primordia extend vertically by intrinsic growth, without detectable recruitment of adjacent mesenchymal cells.     

Load transfer analysis of the distal radius from in-vivo high-resolution CT-imaging.

Prevention of osteoporotic bone fractures requires accurate diagnostic methods to detect the increase in bone fragility at an early stage of osteoporosis. However, today's bone fracture risk prediction, primarily based on bone density measurement, is not sufficiently precise. There is increasing evidence that, in addition to bone density, also the bone microarchitecture and its mechanical loading conditions are important factors determining the fracture risk. Recently, it has been shown that new high-resolution imaging techniques in combination with new computer modeling techniques based on the finite-element (FE) method can account for these additional factors. These techniques might provide information that is more relevant for the prediction of bone fracture risk. So far, however, these new imaged-based FE techniques have not been feasible in-vivo. The objectives of this study were to quantify the load transfer through the trabecular network in a distal radius using a computer model based on in-vivo high-resolution images and to determine if common regions of fractures can be explained as a result of high tissue loading in these regions. The left distal radius and the two adjacent carpal bones of a healthy volunteer were imaged using a high-resolution three-dimensional CT system providing an isotropic resolution of 165 microm. The bone representation was converted into a FE-model that was used to calculate stresses and strains in the trabecular network. The two carpal bones were loaded using different load ratios (for each load case 1000 N in total) representing impact forces on the hand either in near-neutral position or ulnar/radial deviation. The load transfer through the trabecular network of the radius was characterized by the tissue strain energy density (SED) distribution for all load cases. It was found that the distribution of the tissue loading depends on the ratio of the forces acting on the carpal bones. For all load cases the higher SED values (on average: 0.02 +/- 0.08 (S.D.) N mm(-2)) are found in a 10 mm region adjacent to the articular surface which corresponds well with the region where Colles- or Chauffeur-fractures occur. We expect that, eventually, this new approach can lead to a better prediction of the fracture risk than methods based on bone density alone since it accounts for the bone microstructure as well as its loading conditions.     

Communalities for rates of diaphyseal elongation of short bones of the hand of children with Down syndrome

Communality indices for rates of elongation of diaphyses of short bones of the hand were computed from serial data for children with Down syndrome, 7 to 14 years of age. Communalities were larger for adjacent than for nonadjacent bones and also larger for bones grouped in rows rather than rays of the hand. This pattern is similar to that reported for normal children. Communality indices for rates of diaphyseal elongation for girls with Down syndrome were lower than those of boys with Down syndrome and normal children.     

Probable destructive meningioma in an archaeological adult male skull from Alaska

Several intracranial pathological conditions can affect the bones of the skull. The most common cause of these conditions is tumor, but infection and other diseases are also known to affect the bones of the skull. Distinguishing between the various causes of intracranial skeletal pathology in archaeological human remains is usually a challenging exercise, and a specific diagnosis will often be impossible. Meningiomas are tumors that arise in arachnoid tissues embedded in the outer layer of the dura. Because of this association, they occur almost exclusively in the skull and vertebral column. Usually meningiomas are slow-growing tumors that do not metastasize to other organs and tissues of the body. However, rare cases can be malignant and, even when meningiomas are benign, their presence and growth can adversely affect the nervous and vascular supply to other tissues in the skull and vertebral column. Their effect on adjacent bone tissue varies from stimulating bone-forming lesions to causing highly destructive lesions. A few examples of meningioma have been described in the paleopathological literature. Most of these cases are bone-stimulating meningiomas. The case presented here is a probable example of a highly destructive meningioma of the skull base, with unilateral extension into the left side of the cranium. This case is compared with a modern clinical case of destructive intracranial meningioma that was documented both radiographically and pathologically. Destructive meningiomas can be confused with other pathological conditions, including benign and malignant tumors. Criteria for differentiating the diagnostic options are reviewed.     

The sutural pattern of skull-roof bones in Lower Permian Discosauriscus austriacus from Moravia

Sutures between ornamented bones of Discosauriscus austriacus are mostly simple, but there are also more complicated, rarely serrated, sutures between some bones. In small individuals, the sutures are simple, but the same sutures also occur in the largest specimens. The character of the sutures and the incomplete ossification of bones around the pineal foramen indicate the larva type of organization of Discosauriscus The fenestra between premaxillaries and nasals appears to be absent. In the majority of specimens, a squamosal-intertemporal sutural contact is present, althought it is sometimes reduced and in a few cases interrupted by a postorbital and supratemporal contact Therefore the character 'intertemporal-squamosal suture present or absent' cannot be used in this rigorous sense for testing the relationships of early tetrapods. The configuration of the suture between both parietals in osteolepiforms, Discosauriscus , and various early amphibians and reptiles indicates that the bones enclosing the pineal foramen in osteolepifonns are frontals. *** D iscosauriscus . Seymouriamorpha, Lower Permian tetrapod, skull exoskeleton, sutures.     

Potentialities of temporal bone computed tomography in the identification of causes of hypoacusis of postinflammatory genesis

Temporal bone computed tomography (CT) was used to examine 64 patients with impaired hearing due to inflammatory diseases of the middle year. In 21 patients, the pathological process was bilateral. A total of 85 series of temporal bone CT scans were analyzed. The patients' age ranged from 2 to 66 years. CT verified adhesive otitis media in 62 cases, otosclerosis in 7, local malformation of the auditory ossicles and/or the labyrinthine fenestrae in 11. No CT changes were revealed in 5 cases. The CT symptoms of adhesive otitis media were identified. These included soft tissue bands and/or soft tissue-density portions that fix the auditory ossicles or block the niches of the labyrinthine fenestrae (31 temporal bones); sclerosis or ossification of the ligaments and tendons of the middle ear (7 temporal bones); calcification foci in the tympanic cavity (9 cases); osteosclerotic changes in the epitympanus (2 cases); cicatricial changes in the tympanic membrane (24 cases); destructive changes in the auditory ossicles (19 temporal bones). There has been evidence that CT may be used for the differential diagnosis of adhesive otitis media from otosclerosis and congenital malformations of the structures of the middle ear.     

Biomechanics of the rostrum and the role of facial sutures

The rostrum is a large diameter, thin-walled tubular structure that receives loads from the teeth. The rostrum can be conceptualized both as a rigid structure and as an assemblage of several bones that interface at sutures. Using miniature pigs, we measured in vivo strains in rostral bones and sutures to gain a better understanding of how the rostrum behaves biomechanically. Strains in the premaxillary and nasal bones were low but the adjacent maxillary-premaxillary, internasal, and intermaxillary suture strains were larger by an order of magnitude. While this finding emphasizes the composite nature of the rostrum, we also found evidence in the maxillary and nasal bones for rigid structural behavior. Namely, maxillary strain is consistent with a short beam model under shear deformation from molar loading. Strain in the nasal bones is only partially supported by a long beam model; rather, a complex pattern of dorsal bending of the rostrum from incisor contact and lateral compression is suggested. Torsion of the maxilla is ruled out due to the bilateral occlusion of pigs and the similar working and balancing side strains, although it may be important in mammals with a unilateral bite. Torsional loading does appear important in the premaxillae, which demonstrate working and balancing side changes in strain orientation. These differences are attributed to asymmetrical incisor contact occurring at the end of the power stroke.     

Comparison of Metal Concentrations in Bones of Long-Living Mammals

The aim of this study was to compare zinc, copper, lead, cadmium, and mercury concentrations in the bones of long-living mammals—humans (Homo sapiens) and Canidae (dogs Canis familiaris and foxes Vulpes vulpes) from northwestern Poland and to determine the usefulness of Canidae as bioindicators of environmental exposure to metals in humans. Zinc concentrations in cartilage with adjacent compact bone and in spongy bone were highest in foxes (～120 mg/kg dry weight (dw)) and lowest in dogs (80 mg/kg dw). Copper concentrations in cartilage with adjacent compact bone were greatest in foxes (1.17 mg/kg dw) and smallest in humans (～0.8 mg/kg dw), while in spongy bone they were greatest in dogs (0.76 mg/kg dw) and lowest in foxes (0.45 mg/kg dw). Lead concentrations in both analyzed materials were highest in dogs (>3 mg/kg dw) and lowest in humans (>0.6 mg/kg dw). Cadmium concentration, also in both the analyzed materials, were highest in foxes (>0.15 mg/kg dw) and lowest in humans (>0.04 mg/kg dw). Mercury concentration in bones was low and did not exceed 0.004 mg/kg dw in all the examined species. The concentrations of essential metals in the bones of the examined long-living mammals were similar. The different concentrations of toxic metals were due to environmental factors. As bone tissues are used in the assessment of the long-term effects of environmental exposure to heavy metals on the human body, ecotoxicological studies on the bones of domesticated and wild long-living mammals, including Canidae, may constitute a significant supplement to this research.     

In situ solubilization of phosphorus‐bearing raw materials by Bacillus megaterium

This paper presents the results of in situ studies on solubilization of different phosphorus‐bearing raw materials by application of natural ability to produce organic acids by Bacillus megaterium. Poultry bones as well as fish bones were used as renewable sources of phosphates. Morocco phosphorite was used as nonrenewable sources of phosphates. Glass columns, filled with the soil mixed with different doses (1, 5, and 10%) of mentioned sources of phosphorus, were used as a medium for solubilization. It was found that the amount of released phosphorus (determined in the eluent and expressed as P₂O₅) significantly increased in the cases of columns where B. megaterium was used, when compared with the control group (without microflora). Higher doses of phosphorus‐bearing material used in the experiment influenced in the release of higher amount of phosphorus. The highest effectiveness of solubilization was found in the case of poultry bones. The experiment was repeated for poultry bones but with the supplementation of nutrients. It was found that the delivery of nutrients had a strong effect on the increase of effectiveness of solubilization. Two times higher amount of phosphorus (express as P₂O₅) was released from the hydroxyapatite structure of bones. It was confirmed that poultry bones could serve as a source of phosphates in microbial solubilization performed in in situ.     

Determination of adult stature from metatarsal length

The results of a study to determine the value of foot bones in reconstructing stature are presented. The data consist of length measurements taken on all ten metatarsals as well as on cadaver length from a sample of 130 adults of documented race, sex, stature, and, in most cases, age. Significant correlation coefficients (.58-.89) are shown between known stature and foot bone lengths. Simple and multiple regression equations computed from the length of each of these bones result in standard errors of estimated stature ranging from 40-76 mm. These errors are larger than those for stature calculated from complete long bones, but are approximately the same magnitude for stature calculated from metacarpals and fragmentary long bones. Given that metatarsals are more likely to be preserved unbroken than are long bones and given the ease with which they are accurately measured, the formulae presented here should prove useful in the study of historic and even prehistoric populations.     

Radiographic features of experimentalCandida arthritis in rats

Sprague-Dawley rats were inoculated intravenously (i.v.) withCandida albicans, and limb joints showing signs ofCandida-induced arthritis were subjected to radiographic and histologic examination. New bone formation and bone resorption were morbidly enhanced in bones sampled from the arthritic joints. Sparsely distributed needle-shaped calcified deposits began to be formed on bony surfaces in parallel with the onset of joint swelling. The calcified deposits gradually became denser and then covered the bony surfaces almost entirely, giving rise to an exostosis-like profile. In addition to the new bone formation, bone resorption was also observed in regions adjacent to the sites of new bone formation, and punched-out bone lesions were produced. Eventually, severe deformation of joint bones due to new bone formation and bone resorption was evident. Reflecting these unusual radiographic changes, abundant osteoblasts and osteoclasts were demonstrated histologically in the bones. On the basis of these results, possible mechanisms for the induction of arthritis byCandida infection are discussed.     

Calcaneal measurement in estimation of stature of South African blacks

Stature (height) is an important factor in establishing the identity of a person in the living as well as in the skeletonized state. When stature is estimated from the bones of the limbs, regression equations, which estimate the ratios of the lengths of bones to the height of the individual, are generated. The majority of bones that were used previously were the long bones. The calcaneus was used for estimating stature only in American whites and blacks (Holland [1995] Am. J. Phys. Anthropol. 96:315-320). The regression equations that he generated were found to be useful for stature estimation in these population groups. Since the calcaneus has not been used for the same purpose in South Africa, the aim of this study was to derive regression equations that will allow this bone to be used for stature estimation in South African blacks. In total, 116 complete skeletons (60 males and 56 females) were selected from the Raymond A. Dart Collection of Human Skeletons, School of Anatomical Sciences, University of the Witwatersrand (Johannesburg, South Africa). The skeletal heights of these sets of skeletons were calculated using the anatomical method of Fully ([1956] Ann. Med. Leg. 35:266-273). Nine parameters of the calcaneus were measured and matched against skeletal heights, using univariate and multivariate regression methods. Regression equations were obtained for estimation of the stature of the South African black population from the calcaneus. The standard error of estimate that was obtained with univariate regression analysis was higher than the corresponding values using multivariate regression analysis. In both cases, the standard errors of estimate compared well with the values obtained for fragmentary long bones by previous authors.     

Automatic generation of accurate subject-specific bone finite element models to be used in clinical studies

Most of the finite element models of bones used in orthopaedic biomechanics research are based on generic anatomies. However, in many cases it would be useful to generate from CT data a separate finite element model for each subject of a study group. In a recent study a hexahedral mesh generator based on a grid projection algorithm was found very effective in terms of accuracy and automation. However, so far the use of this method has been documented only on data collected in vitro and only for long bones. The present study was aimed at verifying if this method represents a procedure for the generation of finite element models of human bones from data collected in vivo, robust, accurate, automatic and general enough to be used in clinical studies. Robustness, automation and numerical accuracy of the proposed method were assessed on five femoral CT data sets of patients affected by various pathologies. The generality of the method was verified by processing a femur, an ileum, a phalanx, a proximal femur reconstruction, and the micro-CT of a small sample of spongy bone. The method was found robust enough to cope with the variability of the five femurs, producing meshes with a numerical accuracy and a computational weight comparable to those found in vitro. Even when the method was used to process the other bones the levels of mesh conditioning remained within acceptable limits. Thus, it may be concluded that the method presents a generality sufficient to cope with almost any orthopaedic application.     

Optimum strengths for bones liable to fatigue and accidental fracture

Limb bones are liable to fail by fatigue, due to stresses imposed repeatedly in activities such as running. They may also be broken by larger stresses which occur occasionally in accidents. Too weak a bone will probably fail but too strong a bone is unduly heavy. A mathematical model predicts optimum strengths for bones subject to the hazards both of fatigue and of accidents. When accidents are mild and predictable, fatigue is the more important hazard. When they are large and unpredictable they are the more important hazard and stronger bones are generally preferred, but in extreme cases it may become advantageous to dispense with the bone. There is a restricted range of circumstances in which fatigue and accidents are both important hazards. The strengths of many limb bones seem surprisingly low, in the light of the theory and of current knowledge of the fatigue properties of bone.     

Radiodiagnosis of pelvic injuries

The paper deals with an algorithm for the radiodiagnosis of pelvic injuries. The examination of victims with pelvic injuries allows one to state that multislice spiral computed tomography (MSCT) can define the type of pelvic ring instability and the nature of fragment displacement, visualize all types of fractures of pelvic bones and cotyloid cavity walls, and reveal intrapelvic hematomas, as well as changes in adjacent soft tissues, sacroiliac joints, intrapelvic vessels and organs. MSCT angiography is a technique that can be successfully used at different stages of diagnosis and as the first imaging method or as a pre-examination one if the diagnosis is established and as a control study. The scope and quality of obtained information are generally sufficient to make a decision on treatment policy.     

The effect of rigid fixation on growth of the neurocranium

The effects on skull growth of plating the coronal suture and frontal bone were studied in New Zealand White rabbits. Three-dimensional coordinate landmarks were digitized and analyzed to determine the differences in form between operated and unoperated animals using Euclidian distance matrix analysis. This method compares sets of interlandmark distances in three dimensions and was used to demonstrate changes induced by plating. We interpret these changes in morphology to be the result of differences in growth between the operated and unoperated groups. Periosteal elevation alone (n = 6) resulted in a minimal local growth increase. Coronal suture plating (n = 8) resulted in local growth restriction with contralateral and adjacent size increases. Frontal bone plating (n = 6) without crossing a suture line also resulted in local growth restriction and adjacent bone size increases. The timing of intervention in relation to the completion of bone growth may explain the magnitude of clinically apparent effects. Changes in bones adjacent to those directly manipulated may be an attempt to maintain a normal skull volume.     

BMP and Hedgehog signaling during the development of scleral ossicles

Bone development is a complex process, involving multiple tissues and hierarchical inductive interactions. The study of skeletal development has largely focused on endochondral bones while intramembranous bones, such as the scleral ossicles within the avian eye, have received less attention. Our previous research directly demonstrated the involvement of sonic hedgehog and suggested the involvement of bmp2 and 4 during the development of scleral ossicles. The bones of the sclerotic ring are induced by overlying conjunctival papillae at HH 35 and 36. Here, we examine the spatial and temporal expression patterns of ptc1, ihh, bmp2, bmp4 and bmp7. We show that the cells of conjunctival papillae express ptc1, ihh and bmp2 at these stages; coincident with shh expression previously described. Interestingly, both ihh and ptc1 are also expressed in the mesenchyme underlying the papillae unlike shh and bmp2. Bmp4 and bmp7 are not expressed in these regions at any stages examined. Furthermore, using Noggin soaked beads implanted adjacent to papillae, we provide direct evidence that the BMP family of genes are important factors in the development of scleral ossicles. Localized inhibition of BMPs in this way causes a reduced expression of ihh in the surrounding tissue demonstrating that the BMP and Hedgehog pathways interact. Our data also demonstrates that the sclerotic ring has an intrinsic ability to compensate for missing elements. The scleral ossicle system provides a unique opportunity to investigate the epithelial-mesenchymal induction of intramembranous bones of the vertebrate skull.