Carol Trahan

Vascular events associated with the appearance of the secondary center of ossification in the murine distal femoral epiphysis.

Although the formation of a secondary center of ossification is often compared with that of the primary center, there are striking differences between these processes. In the formation of the primary center, vascular invasion is always associated with the maturation of chondrocytes, whereas vascularization of the epiphysis can proceed in two different ways. In some species, the epiphysis is vascularized by cartilage canals before the appearance of the secondary center. However, in the mouse, the distal femoral epiphysis is vascularized by peripheral vascular invasion without pre-existing cartilage canals. Histological study of serial sections and studies of vascularization by injection with India ink demonstrated the relationship between hypertrophic chondrocyte formation, vascular invasion, and the formation of the secondary center of ossification in the murine distal femoral epiphysis.

Pigmented villonodular synovitis of joints

The objective of this study is to present two series of cases of pigmented villonodular disease of joints (PVNS) treated at different periods. The 215 cases from our hospital are current (1972–2009) and those 62 from the Jaffe collection from 1920 to 1968.

Histomorphometric Analysis of a Cell-Based Model of Cartilage Repair

This study examined the morphology of chondrocytes in an established model of articular cartilage repair. Articular cartilage was harvested from young sheep and seeded onto pieces of devitalized sheep cartilage. The seeded pieces were stacked in pairs and wrapped in fibrin glue, and then implanted subcutaneously in the dorsum of athymic mice. Samples were harvested after 6 weeks and examined by transmission electron microscopy (TEM) or by light microscopy. TEM revealed that the cells in direct apposition to the devitalized cartilage were elongated, with an enlarged cytoplasm, and a ruffled border. TEM of cells far from the interface with scaffold tissue revealed rounded cells with large nuclei that appeared similar to normal chondrocytes. Quantitative morphometry of histologic specimens revealed that cell area, relative amount of cytoplasm, cell aspect ratio, and relative nuclear displacement were all higher in cells near the interface with the scaffold tissue, and decreased with distance from the interface. These indices of cell morphology are all consistent with an active remodeling of the scaffold at the cell-scaffold interface.

Quantitative histologic analyses of articular cartilage and subchondral bone from osteoarthritic and normal human hips

Twelve femoral heads (two normal, four after fracture and six osteo-arthritic) were obtained at surgery or autopsy. Circumferential slices were obtained and five separate areas were analyzed in each for ash content; histological-histochemical grading of the severity of the cartilage changes; and quantitative morphometric analyses to establish the percentage of trabecular area, osteoblastic area and osteoclastic area. Analyses were performed to compare weight-bearing and non-weight-bearing areas of the femoral heads and to determine correlations between the bony and cartilaginous alterations. The data obtained showed wide variations in all parameters in the osteoarthritic specimens but consistently more marked cartilage and bony changes in the weight-bearing areas. Bone structures and metabolic parameters were distinctly increased for the osteoarthrities, differing significantly from both normal and fracture control groups. The bony change correlated directly with the severity of the cartilage lesions, as determined by the histological-histochemical grade.

Non-ossifying fibroma, fibrous cortical defect and Jaffe-Campanacci syndrome: a biologic and clinical review.

Non-ossifying fibroma of bone (NOF) is a common entity, more frequently found in male children and consisting of a solitary eccentric, lytic expanded lesion in the metaphysis of a long bone. The disorder is benign and most often asymptomatic but may result in a fracture requiring therapy. Of some importance is to distinguish NOF from another very similar but smaller lesion, fibrous cortical defect, which is almost always asymptomatic and eccentrically located. Even more striking is a very rarely encountered lesion known as Jaffe–Campanacci syndrome, which also occurs in children who present with typical non-ossifying fibromatous tumors but in multiple sites. In addition, these patients have some systemic and dermal findings resembling those seen in patients with Type 1 neurofibromatosis.

Hand and Foot Abnormalities Associated with Genetic Diseases

IntroductionThe small bones and soft tissues of the hands and feet can be affected by systemic disorders, and frequently, the findings are quite unique and virtually diagnostic for some genetic or metabolic disorders.Materials and MethodsPhotographs and imaging studies for the hands and feet are available in a digitized system, which has been approved by our hospital institutional review board. Examination of these and their description can establish a relationship with some degree of certainty to a series of highly variable and uncommon clinical disorders.ResultsDescription of the clinical, physiologic and genetic characteristics, and illustrations of hand and foot abnormalities are provided for an array of diseases, including Ellis–van Creveld syndrome, fibrodysplasia ossificans progressiva, achondroplasia, Kniest dysplasia, pseudo- and pseudo-pseudohypoparathyroidism, acromegaly, nail–patella syndrome, Marfan’s disease, cartilage–hair hypoplasia, and several forms of mucopolysaccharidosis.ConclusionsThe findings support the concept that many genetic disorders can often be diagnosed by clinical and imaging examination of the patient’s hands and feet.

A questionnaire study for 128 patients with Gaucher disease.

Gaucher disease is an uncommon autosomal recessive disorder characterized by lysosomal storage of glucosyl ceramide, a material released during cell degradation. Patients with Gaucher disease often have significant hematologic, bone structural, and visceral problems which sometimes greatly affect their health and life style. Based on some extraordinary scientific discoveries over the past 45 years, a treatment system has evolved which consists of administration of an enzyme, which destroys the lysosome‐stored material and to some extent restores the patients to good health. There are still some problems for these patients; however, and the purpose of the study is to define some of the clinical, sociologic, and psychologic problems with a specially designed questionnaire. Questionnaire data was collected for 128 patients from two institutions with complete anonymity, and the information compared against data from a National Health Inteview Survey. The results show that many of the patients still have fairly extensive problems, which could possibly be helped by some alterations in treatment protocols.