OBJECTIVE: To describe a case of Ehlers-Danlos syndrome type IV and its pedigree in China. METHODS: Clinical materials of a case of Ehlers-Danlos syndrome type IV and a pedigree of 6 members within 4 generations were ...OBJECTIVE: To describe a case of Ehlers-Danlos syndrome type IV and its pedigree in China. METHODS: Clinical materials of a case of Ehlers-Danlos syndrome type IV and a pedigree of 6 members within 4 generations were analyzed. Dilated internal jugular vein in the proband was removed by operation. The diagnosis, surgical treatment, and postoperative complications were retrospectively reviewed. RESULTS: Vessels of the proband in the pedigree were crisp and easily lacinated during the procedure of removing his internal jugular vein. Repeating postoperative hematomas were found though complete stanching was achieved during the operation. The patient was successfully recovered by promptly debridgement and needle sucking. The other 5 members of the pedigree all had the triads of Ehlers-Danlos syndrome. CONCLUSIONS: Though it was of extremely low morbidity rate, the high mortality rate and complication of Ehlers-Danlos syndrome deserve great attention during surgical management, especially in patients with Ehlers-Danlos syndrome type IV. Surgeons should be aware of the ponderance of its complications and combined diseases to avoid fatal intraoperative vascular lascination and incontrollable hemorrhage.展开更多
Uncertainty exists widely in hydrological analysis, and this makes the process of uncertainty assessment very im- portant for making robust decisions. In this study, uncertainty sources in regional rainfall frequency ...Uncertainty exists widely in hydrological analysis, and this makes the process of uncertainty assessment very im- portant for making robust decisions. In this study, uncertainty sources in regional rainfall frequency analysis are identified for the first time. The numeral unite spread assessment pedigree （NUSAP） method is introduced and is first employed to quantify qual- itative uncertainty in regional rainfall frequency analysis. A pedigree matrix is particularly designed for regional rainfall frequency analysis, by which the qualitative uncertainty can be quantified. Finally, the qualitative and quantitative uncertainties are com- bined in an uncertainty diagnostic diagram, which makes the uncertainty evaluation results more intuitive. From the integrated diagnostic diagram, it can be determined that the uncertainty caused by the precipitation data is the smallest, and the uncertainty from different grouping methods is the largest. For the downstream sub-region, a generalized extreme value （GEV） distribution is better than a generalized logistic （GLO） distribution; for the south sub-region, a Pearson type III （PE3） distribution is the better choice; and for the north sub-region, GEV is more appropriate.展开更多
文摘OBJECTIVE: To describe a case of Ehlers-Danlos syndrome type IV and its pedigree in China. METHODS: Clinical materials of a case of Ehlers-Danlos syndrome type IV and a pedigree of 6 members within 4 generations were analyzed. Dilated internal jugular vein in the proband was removed by operation. The diagnosis, surgical treatment, and postoperative complications were retrospectively reviewed. RESULTS: Vessels of the proband in the pedigree were crisp and easily lacinated during the procedure of removing his internal jugular vein. Repeating postoperative hematomas were found though complete stanching was achieved during the operation. The patient was successfully recovered by promptly debridgement and needle sucking. The other 5 members of the pedigree all had the triads of Ehlers-Danlos syndrome. CONCLUSIONS: Though it was of extremely low morbidity rate, the high mortality rate and complication of Ehlers-Danlos syndrome deserve great attention during surgical management, especially in patients with Ehlers-Danlos syndrome type IV. Surgeons should be aware of the ponderance of its complications and combined diseases to avoid fatal intraoperative vascular lascination and incontrollable hemorrhage.
基金the National Natural Science Foundation of China，the Zhejiang Provincial Natural Science Foundation of China
文摘Uncertainty exists widely in hydrological analysis, and this makes the process of uncertainty assessment very im- portant for making robust decisions. In this study, uncertainty sources in regional rainfall frequency analysis are identified for the first time. The numeral unite spread assessment pedigree （NUSAP） method is introduced and is first employed to quantify qual- itative uncertainty in regional rainfall frequency analysis. A pedigree matrix is particularly designed for regional rainfall frequency analysis, by which the qualitative uncertainty can be quantified. Finally, the qualitative and quantitative uncertainties are com- bined in an uncertainty diagnostic diagram, which makes the uncertainty evaluation results more intuitive. From the integrated diagnostic diagram, it can be determined that the uncertainty caused by the precipitation data is the smallest, and the uncertainty from different grouping methods is the largest. For the downstream sub-region, a generalized extreme value （GEV） distribution is better than a generalized logistic （GLO） distribution; for the south sub-region, a Pearson type III （PE3） distribution is the better choice; and for the north sub-region, GEV is more appropriate.
