Total ankle replacement by the Ankle Evolution System: medium-term outcome.

We present the outcomes in 38 consecutive patients who had total ankle replacement using the Ankle Evolution System with a minimum follow-up of four years. Pain and function were assessed using the American Orthopaedic Foot and Ankle Society (AOFAS) score and regular standardised anteroposterior and lateral weight-bearing radiographs were obtained. Patient satisfaction and complications were recorded and the survival of the implants was demonstrated by the Kaplan-Meier method. The mean follow-up was for 57.8 months (48 to 80). The cumulative survival rate at six years was 94.7% (95% confidence interval 80.3 to 98.7). The mean total AOFAS score was 88.1 (53 to 100). The mean score for pain was 35.8 (20 to 40). Ten patients presented with edge-loading of whom nine had corrective surgery. Two ankles were revised, one to an arthrodesis and the other to replace the tibial component. Nine patients showed radiological evidence of osteolysis. They had minimal non-progressive symptoms and further surgery was not undertaken. Nevertheless, the concerns about osteolysis led to the implant being withdrawn by the manufacturer. The medium-term results of the ankle evolution system ankle replacement are satisfactory with high patient satisfaction, but the rate of osteolysis is of some concern. The long-term benefit of this procedure has yet to be determined.

A rare presentation of sarcoidosis, back pain and spondylolisthesis.

A 48-year old man presented with back pain that was resistant to treatment. An MR scan showed spondylolisthesis at L4-5 and narrowing of the exit foraminae. He had a posterior fusion which did not relieve his symptoms. He continued to have back pain and developed subcutaneous nodules in both forearms. Biopsy from the skin revealed cutaneous sarcoidosis, and one from the lumbar spine showed sarcoidosis granuloma between the bone trabeculae. A CT scan of the abdomen and chest revealed axillary lymphadenopathy, mediastinal enlarged nodes, apical nodular nodes and splenomegaly. The patient was started on large doses of methotrexate and steroids. His angiotensin-converting enzyme and calcium levels returned to normal and the back pain resolved.

Characterization of Wnt-1 and Wnt-2 induced growth alterations and signaling pathways in NIH3T3 fibroblasts.

Members of the Wnt family induce mouse mammary tumors and partially transform mammary epithelial cells in culture. However, their mechanism of transformation remains to be elucidated. In NIH3T3 mouse embryo fibroblasts, a standard transformation model, Wnt-1 and Wnt-2 were shown to induce altered properties including increased saturation density and growth in soft agar. Such cells also exhibited increased cell-cell adhesiveness. However, unlike oncogenes such as PDGFB or ras, Wnt-1 and -2 failed to induce detectable transformed foci following transfection, and stable NIH3T3 transfectants lacked tumor forming capacity. Wnt-1 and -2 transfectants exhibited increased uncomplexed, cytosolic beta-catenin, which was not observed with PDGFB, ras or erbB2 transfectants. In transient transfection, Wnt-1 and -2 induced a rapid increase in cytosolic beta-catenin but no detectable increase in the phosphorylated activated forms of MAP kinase. In contrast, ras was a potent activator of MAP kinase but had no effect on free beta-catenin levels. These findings establish that both Wnt signaling and pattern of growth alterations differ from those of oncogenes which activate proliferative signaling pathways in NIH3T3 cells.

Trisomy 5 as sole anomaly in acute lymphoblastic leukemia.

We present a case of B-cell acute lymphoblastic leukemia (ALL) in whose leukemic cells trisomy 5 (+5) was the only cytogenetic anomaly observed. This is the first report of +5 as the sole cytogenetic abnormality in ALL; two cases (one questionable) of acute nonlymphocytic leukemia with such a change have been reported. The findings are presented in relation to other cases with +5 as part of a more complicated cytogenetic picture in hematologic disorders.

Fragile sites at 4q23 and 7q11.23 unique to bone marrow cells.

Fragile sites in chromosome bands 4q23 and 7q11.23 were discovered in bone marrow cells. Expression of these fragile sites was induced by treatment of the cells sequentially with 10(-7) M methotrexate and then 10(-5) M thymidine. No expression was observed in bone marrow cells without treatment. The fragile sites at 4q23 and 7q11.23 were seen individually, together, and in the homozygous state in a total of 20 bone marrow samples. The bone marrow karyotypes were normal in all cases. Expression of these common fragile sites at 4q23 and 7q11.23 could not be induced in blood lymphocytes from two subjects using methotrexate and thymidine, or by any other means including the addition of bromodeoxyuridine and fluoro-deoxyuridine or growth in folate-deficient medium. Because the fragile sites at 4q23 and 7q11.23 have never been observed in lymphocytes treated with methotrexate and thymidine for high-resolution chromosome analysis, it appears that these fragile sites are not expressed under these conditions in T lymphocytes. We propose that the differential expression of these fragile sites in bone marrow cells reflects genes active in bone marrow cells but not in blood lymphocytes.

Enhanced expression of chromosome fragile site 10q25 in chronic myelogenous leukemia.

Spontaneous expression of a BrdU-sensitive fragile site at 10q25 was observed in normal lymphocytes and malignant blood and bone marrow cells in chronic myelogenous leukemia (CML). The cells were marked by a Philadelphia chromosome rearrangement due to insertion of 22q11----q13 at 11q13. The fragile site at 10q25 was expressed in larger proportions of malignant than normal cells. Although this fragile site is not at a cancer chromosome breakpoint, malignancy enhanced its expression, consistent with a cascade effect.

Euryblepharon: a case report with photographs documenting the condition from infancy to adulthood.

A case of euryblepharon with lateral ectropion is reported, with photographs from infancy to adult life. The value of obtaining photographs, if available, in cases such as this is stressed, to avoid unnecessary clinical studies being done. The photographs indicate the stability of the condition. They also indicate that, at least in some cases, the ectropion will become less apparent with body growth and may need no therapy.