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311.
Karl-Josef Prommersberger J?rg van Schoonhoven Stefanie Laubach Ulrich Lanz 《European Journal of Trauma》2001,27(1):16-24
Background: In spite of the considerable information regarding the indications for, and the techniques and results of, corrective osteotomy of malunited, dorsally displaced fractures of the distal radius, there have been few reports concerning osteotomy of palmarly angulated malunions of the distal radius. The purpose of the current study was to evaluate our experience with this condition. Patients and Methods: We retrospectively reviewed 20 patients, which were operated on for a malunited, palmarly tilted fracture of the distal radius between 1993 and 1998. The investigated criteria included range of motion, grip strength, pain relief, and X-ray findings. Wilcoxon's sign-rank test was used to evaluate the extent of improvement concerning these criteria. Results: At an average of 18 months after osteotomy, radiographs revealed a statistically highly significant improvement in all radiologic parameters. The increase in ulnar and radial deviation of the wrist, and forearm supination, as well as the decrease of pain were statistically highly siginificant. The resultant improvement in grip strength in the current series was significant. Conclusion: Corrective osteotomy of palmarly displaced malunions of the distal end of the radius just as in the case of their dorsal counterparts is of great benefit and leads to improved hand and wrist function as well as diminished pain. 相似文献
312.
Development of extramedullary myeloma in the era of novel agents: no evidence of increased risk with lenalidomide–bortezomib combinations 下载免费PDF全文
Cindy Varga Wanling Xie Jacob Laubach Irene M. Ghobrial Elizabeth K. O'Donnell Matthew Weinstock Claudia Paba‐Prada Diane Warren Michelle E. Maglio Robert Schlossman Nikhil C. Munshi Noopur Raje Edie Weller Kenneth C. Anderson Constantine S. Mitsiades Paul G. Richardson 《British journal of haematology》2015,169(6):843-850
Proteasome inhibitors (PI) and immunomodulatory agents (IMIDs) have improved the overall survival (OS) of patients with multiple myeloma (MM), but concerns have been raised about increased incidence of extramedullary disease (EMD) after the combined use of PIs and IMIDs for upfront therapy. We evaluated whether the addition of lenalidomide to bortezomib‐based front‐line regimens precipitated earlier development of EMD. We reviewed the charts of 117 MM patients (median follow‐up from diagnosis 6·1 years; range 0·1–10·2 years) enrolled in eight clinical trials of first‐line treatment with bortezomib‐based regimens, with or without lenalidomide. We assessed development of EMD as extraosseous (distant from bone) or osseous (originating from bone) plasmacytomas. The primary endpoint was time from diagnosis until development of EMD, based on imaging, biopsy and/or physical examination. Any form of EMD at progression was observed in 40 (34·2%) patients, including 21 (18%) osseous, 8 (7%) extraosseous and 11 (9%) both osseous and extraosseous. Median OS was 0·9 years (range 0·1–4·8 years) after extraosseous EMD development. Sensitivity analyses with follow‐up times truncated at 5 years detected no statistically significant difference in rates of any EMD form between the two groups (P > 0·2 for each comparison). Therefore, we observed no evidence that bortezomib–lenalidomide‐based front‐line therapy precipitates earlier EMD. 相似文献
313.
Homozygous alpha6 integrin mutation in junctional epidermolysis bullosa with congenital duodenal atresia 总被引:4,自引:0,他引:4
Pulkkinen L; Kimonis VE; Xu Y; Spanou EN; McLean WH; Uitto J 《Human molecular genetics》1997,6(5):669-674
Junctional epidermolysis bullosa with congenital pyloric or duodenal
atresia is a distinct variant within this group of autosomal recessive
blistering skin diseases. In this study we demonstrate, for the first time,
a homozygous mutation in the alpha6 integrin gene (ITGA6) in a family with
three affected individuals. For this purpose, we first determined the
genomic organization of ITGA6, and placed the gene on chromosome 2q by high
resolution radiation hybrid mapping. Heteroduplex analysis of PCR products
containing the individual exons of ITGA6, followed by direct nucleotide
sequencing, revealed that the proband was homozygous for a G-to-T
transversion in the +1 position of intron 12. This mutation,
1856+1G-->T, affects an invariant base of the 5' donor splice site
predicting aberrant splicing involving exon 12. The mutation was verified
in the proband's DNA by restriction enzyme digestion which also confirmed
that the parents were heterozygous carriers of this mutation. Altered
expression of alpha6 integrin, which forms a heterodimer with the beta4
subunit at the dermal-epidermal junction, would explain fragility and
blistering as a result of minor trauma to the skin.
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