A new familial immunodeficiency disorder characterized by severe neutropenia, a defective marrow release mechanism, and hypogammaglobulinemia.
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Biomedical subjects
Publications and source records attributed to M Wetzler.
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This is a retrospective nonrandomized study of 66 open tibial fracture patients that compares comparable population treated at one trauma center. The fractures were treated with either Ender rods or external fixation. Of these, 29 fractures were eliminated from the study due to incomparable variables. Thus, the study group consisted of a total of 37 tibial fractures: 20 treated by Ender rods and 17 treated by external fixation. Open tibial fractures treated with Ender rods had a significantly lower number of complications than those treated with external fixation. The group treated with Ender rods also had a significantly lower number of surgical procedures per patient in Grades I and II open tibial fractures. There were no statistical differences regarding weeks to union or hospital days. Ender rods are superior to external fixation removed early in the treatment of Gustilo's Grades I, II, and IIIA open fractures with either stable or unstable fracture configuration (Orthopaedic Trauma Association Grades IA-IC, IIA-IID, and IIIA-IIIC). Perhaps future study will allow sufficient numbers to determine whether Ender rods can be used in Grades IIIB and IIIC open tibial fractures, but there are no data at this time to support this conclusion.
Acute lymphocytic leukemias (ALL) of infants and children were found to preferentially survive in coculture with a cloned cell line of endothelial adipose cells (14F1.1) from mouse bone marrow. One of these ALLs expressed a phenotype compatible with an early stage of differentiation (HLA-DR+, CD19+, and CD34+) and exhibited extensive growth in the presence of the mouse stromal cells during a period greater than 25 weeks following seeding. These ALL cells were strictly dependent upon the mouse stromal clone 14F1.1 and failed to proliferate in the absence of the endothelial adipocytes or with a variety of "feeder cells." Throughout the culture period the cells died if removed from the stroma. No similarly proliferative cell population with strict dependence upon stromal cells was found among a variety of other leukemias including hairy cell, acute myeloid, and chronic lymphocytic leukemia. The 14F1.1 clone has been previously found to promote the renewal of mouse and human stem cells. It is therefore possible that leukemias with a stem cell-like phenotype depend upon stromal cell factors similar to those affecting the growth of normal stem cells. These factors appear to operate across genetic barriers.
Of four patients who were treated between May 1985 and November 1987 and who had both a lateral malleolar fracture and a tear of the lateral ligaments of the ipsilateral ankle, three could be followed. The fractured malleolus was treated with rigid internal fixation, but the injury to the lateral ligaments was not diagnosed until the ankle was stressed to test for stability after the internal fixation. We believe that if disruption of the lateral ligaments is noted at operation, repair is indicated. This involves only a slight prolongation of time under anesthesia and markedly increases the chances for a stable ankle. It is important to check intraoperatively for a possible tear of the lateral ligaments in a patient who has a fracture of the ankle, especially when the injury was caused by high-energy trauma.
The HER2/neu protooncogene was found to be amplified in 6 of 109 primary adenocarcinoma tumors. No HER2/neu amplification was found in 29 other primary nonadenocarcinomatous tumors. In two colon tumors, in addition to the amplification, DNA rearrangement of HER2/neu gene was also observed. The rearrangement was explored in detail in one tumor and it was shown to be confined to the 3' region of the gene. Moreover, this tumor expressed an aberrant HER2/neu polypeptide with a molecular weight of 190,000, which is larger by approximately 5,000 than the molecular weight of the normal HER2/neu protein. The aberrant HER2/neu protein was immunoprecipitated with site-specific antibodies against a synthetic peptide from the COOH-terminal end of the normal HER2/neu protein; it also displayed intrinsic protein tyrosine kinase activity leading to self-phosphorylation.
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A 60-year-old woman presented with relapse of polycythemia vera associated with hypereosinophilic syndrome (HES) with abnormal immunologic measures, including increased serum IgE and IgG levels, high levels of circulating immune complexes, rheumatoid factor, and antinuclear antibodies. Treatment with hydroxyurea was followed by a dramatic response of both the polycythemia vera and the HES, with return to normal of the abnormal immunologic measures. This case report documents that evidence of immunologic and myeloproliferative causes of HES may coexist in the same patient.
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