BACKGROUND: Researches indicate that patients with Wilson disease (WD) have abnormal skeletal metabolism, which is induced by various factors. OBJECTIVE: To probe into the changing characteristics of abnormal skeletal...BACKGROUND: Researches indicate that patients with Wilson disease (WD) have abnormal skeletal metabolism, which is induced by various factors. OBJECTIVE: To probe into the changing characteristics of abnormal skeletal metabolism in WD patients and observe the effect of decopper therapy. DESIGN: Case-contrast and self-control study. SETTING: Department of Neurology, Affiliated Hospital of Neurological Institute, Anhui College of Traditional Chinese Medicine. PARTICIPANTS: A total of 35 patients with WD including 21 males and 14 females aged from 10 to 42 years with the mean age of (20±8) years were selected from Department of Neurology, Affiliated Hospital of Neurological Institute, Anhui College of Traditional Chinese Medicine from September 2000 to February 2001. All the patients were in compliance with the diagnostic criteria: history of family heredity; cone symptoms in vitro, physical sign or liver symptoms; positive Kayser-Fleischer ring; serum copper protein < 200 mg/L or A copper oxidase < 0.2; urine copper > 1.6 μmol/24 hours; liver copper > 250 μg/g (dry weight). The control group was selected from 25 cases of health individuals including 13 males and 12 females aged from 16 to 35 years with the mean age of (22±6) years. All patients who participated in the study were informed first and consented. METHODS: Patients in treatment group were treated with venous injection of 1.0 g sodium dimercaptosulfonate, once a day for totally 6 successive days. And then, patients rested for 2 days. This procedure mentioned above was regarded as a course, and the treatment lasted for 4-8 courses. Before and after injection of sodium dimercaptosulfonate, serum calcitonin (CT), osteocalcin (BGP), parathyroid hormone (PTH) and 1,25-(OH)2VitD3 were measured with radio-immunity method; blood, urine calcium, phosphorum and urine creatinine were measured with biochemical analyzer; urine dihydropyrimidine dehydrogenase(DPD) was detected with enzyme-immunity method; bone mineral density (BMD) was checked at the one third from distal end of ulna and radius with single photon absorptiometry (SPA). MAIN OUTCOME MEASURES: Relative indexes of bone metabolism of blood and urine and results of BMD in both two groups before and after treatment. RESULTS: Among 35 patients with WD and 25 healthy subjects, 5 patients were excluded because of uncompleted decopper therapy; therefore, 30 patients with WD and 25 healthy subjects were involved in the final analysis. ① Comparisons between the two groups: Contents of serum calcium, PTH and 1,25-(OH)2VitD3 were lower in treatment group than those in control group [(2.49±0.34) mmol/L vs. (2.69±0.19) mmol/L; (218.7±50.5) ng/L vs. (262.5±88.9) ng/L; (23.53±14.21) ng/L vs. (42.78±14.44) ng/L; P < 0.05-0.01]; however, contents of serum BGP and CT were higher in treatment group than those in control group [(10.22±6.11) μg/L vs. (5.78±4.22) μg/L; (282.8±109.6) ng/L vs. (62.5±37.9) ng/L, P < 0.01]; moreover, there was no significant difference of contents of serum phosphorum, urine calcium, phosphorum and DPD/creatinine between treatment group and control group (P > 0.05). BMD of males and females was lower in treatment group than that in control group [(0.617±0.197) g/cm2 vs. (0.718±0.274) g/cm2; (0.594±0.124) g/cm2 vs. (0.677±0.157) g/cm2, P < 0.05]. ② Comparisons in treatment group before and after treatment: Contents of CT and urine calcium were lower after treatment than those before treatment [(95.3±55.4) ng/L vs. (283.3±96.7) ng/L; (2.38±1.68) mmol/L vs. (3.31±2.30) mmol/L; P < 0.01, 0.05]; however, contents of 1,25-(OH)2VitD3 and DPD/creatinine were higher after treatment than those before treatment [(33.61±19.30) ng/L vs. (24.21±14.47) ng/L; (42.95±19.92) nmol/mmol vs. (19.51±9.96) nmol/mmol, P < 0.05]; moreover, there were no significant differences among other indexes before and after treatment (P > 0.05). Furthermore, there was no significant difference of BMD before and after treatment (P > 0.05). CONCLUSION: WD patients have changes in the related indexes of abnormal skeletal metabolism. In addition, contents of CT and urine calcium are decreased remarkably after decopper therapy; however, value of BMD is not changed obviously.展开更多
Decopperization in molten steel with FeS-Na2 S slag was investigated in the laboratory. It was found that with an increase in FeS content of the FeS-Na2 S slag, the decopperization ratio increased. The decopperization...Decopperization in molten steel with FeS-Na2 S slag was investigated in the laboratory. It was found that with an increase in FeS content of the FeS-Na2 S slag, the decopperization ratio increased. The decopperization ratio could reach the maximum of 71.6% in hot metal. The decopperization ratio was closely related to the carbon content, and the higher the carbon content, the higher was the decopperization ratio. Sulphur addition was found in the process of decopperization, and the increase in sulphur content of molten steel was about 0. 2%-0. 42%. However, by adding 30%-50% of BaO to the FeS-Na2 S slag, the addition of sulfur content in molten steel could be reduced by about 50%. The finer slag system composition was FeS ： NazS ： BaO=40 ： 20 ： 40.展开更多
Copper is difficult to separate from nickel electrolyte due to low concentration of copper (0.53 g/L) with high concentration of nickel (75 g/L). Manganese sulfide (MnS) was used to deeply remove copper from the elect...Copper is difficult to separate from nickel electrolyte due to low concentration of copper (0.53 g/L) with high concentration of nickel (75 g/L). Manganese sulfide (MnS) was used to deeply remove copper from the electrolyte. Experimental results show that the concentration of copper (ρ(Cu)) decreases from 530 to 3 mg/L and the mass ratio of copper to nickel (RCu/Ni) in the residue reaches above 15 when the MnS dosage is 1.4 times the theoretical valueDt,MnS (Dt,MnS=0.74 g) and the pH value of electrolyte is 4?5 with reaction time more than 60 min at temperatures above 60 °C. The concentration of newly generated Mn2+(ρ(Mn)) in the solution is also reduced to 3 mg/L by the oxidation reaction. The values ofρ(Cu),ρ(Mn)andRCu/Ni meet the requirements of copper removal from the electrolyte. It is shown that MnS can be considered a highly effective decoppering reagent.展开更多
Background: Medical therapy is rarely effective inpatients with fulminant Wilson's disease (FWD). Livertransplantation is limited by the lack of donor liver inmost patients with FWD at the time of diagnosis. NewWi...Background: Medical therapy is rarely effective inpatients with fulminant Wilson's disease (FWD). Livertransplantation is limited by the lack of donor liver inmost patients with FWD at the time of diagnosis. NewWilson's index, model for end-stage liver disease (MELD)and Child-Pugh score are useful tools for decisionmakingof liver transplantation;however, none of them isan independent decisive tool. It is worthwhile to explorea more effective and practical therapeutic strategy andreevaluate the prediction systems for patients with FWD.Methods: Nine patients with FWD associated withhemolytic crisis and fulminant hepatic failure (FHF) wereinvestigated. The clinical presentation, prognostic scoreand medical therapies of the patients were analyzed.Results: In 7 of the 9 patients with FWD who receivedthe comprehensive therapy of corticosteroid, copperchelatingagent (dimercaptopropansulfonate sodium)and therapeutic plasma exchange (TPE), 6 patientsrecovered from FHF. The remaining one had beenimproved through the comprehensive therapy but died ofsepticemia 51 days later. Two patients with spontaneousbacterial peritonitis (SBP) died from liver failure inthree or five hospital days without plasma exchangeor chelating therapy. All of the 9 patients with FWDpresented with acute hepatic failure, severe jaundice andmild to severe hemolytic anemia. No marked differencein the incidence of severe hemolytic anemia was detectedbetween the survival and deceased groups. However,the incidence and the degree of hepatic encephalopathy(HE) in the non-survival group were higher than thosein the survival group. Unlike the deceased group, thesurvival group had no complications induced by bacterialinfection. Compared to new Wilson's index, Child-Pughscore and MELD score, the variation of prothrombinactivity (PTA) between the survival and deceased groupswas more evident.Conclusions: For patients with FWD, the episodeof severe hepatic encephalopathy or/and spontaneousbacterial peritonitis indicates worse prognosis, andPTA is a recommendable predictor. An emergent livertransplantation should be considered for patients whosePTA is below 20%, or for those with severe HE or/and SBP. The comprehensive therapy of corticosteroid,copper-chelating agent and TPE is effective for patientswithout SBP and whose PTA is higher than 20%.展开更多
文摘BACKGROUND: Researches indicate that patients with Wilson disease (WD) have abnormal skeletal metabolism, which is induced by various factors. OBJECTIVE: To probe into the changing characteristics of abnormal skeletal metabolism in WD patients and observe the effect of decopper therapy. DESIGN: Case-contrast and self-control study. SETTING: Department of Neurology, Affiliated Hospital of Neurological Institute, Anhui College of Traditional Chinese Medicine. PARTICIPANTS: A total of 35 patients with WD including 21 males and 14 females aged from 10 to 42 years with the mean age of (20±8) years were selected from Department of Neurology, Affiliated Hospital of Neurological Institute, Anhui College of Traditional Chinese Medicine from September 2000 to February 2001. All the patients were in compliance with the diagnostic criteria: history of family heredity; cone symptoms in vitro, physical sign or liver symptoms; positive Kayser-Fleischer ring; serum copper protein < 200 mg/L or A copper oxidase < 0.2; urine copper > 1.6 μmol/24 hours; liver copper > 250 μg/g (dry weight). The control group was selected from 25 cases of health individuals including 13 males and 12 females aged from 16 to 35 years with the mean age of (22±6) years. All patients who participated in the study were informed first and consented. METHODS: Patients in treatment group were treated with venous injection of 1.0 g sodium dimercaptosulfonate, once a day for totally 6 successive days. And then, patients rested for 2 days. This procedure mentioned above was regarded as a course, and the treatment lasted for 4-8 courses. Before and after injection of sodium dimercaptosulfonate, serum calcitonin (CT), osteocalcin (BGP), parathyroid hormone (PTH) and 1,25-(OH)2VitD3 were measured with radio-immunity method; blood, urine calcium, phosphorum and urine creatinine were measured with biochemical analyzer; urine dihydropyrimidine dehydrogenase(DPD) was detected with enzyme-immunity method; bone mineral density (BMD) was checked at the one third from distal end of ulna and radius with single photon absorptiometry (SPA). MAIN OUTCOME MEASURES: Relative indexes of bone metabolism of blood and urine and results of BMD in both two groups before and after treatment. RESULTS: Among 35 patients with WD and 25 healthy subjects, 5 patients were excluded because of uncompleted decopper therapy; therefore, 30 patients with WD and 25 healthy subjects were involved in the final analysis. ① Comparisons between the two groups: Contents of serum calcium, PTH and 1,25-(OH)2VitD3 were lower in treatment group than those in control group [(2.49±0.34) mmol/L vs. (2.69±0.19) mmol/L; (218.7±50.5) ng/L vs. (262.5±88.9) ng/L; (23.53±14.21) ng/L vs. (42.78±14.44) ng/L; P < 0.05-0.01]; however, contents of serum BGP and CT were higher in treatment group than those in control group [(10.22±6.11) μg/L vs. (5.78±4.22) μg/L; (282.8±109.6) ng/L vs. (62.5±37.9) ng/L, P < 0.01]; moreover, there was no significant difference of contents of serum phosphorum, urine calcium, phosphorum and DPD/creatinine between treatment group and control group (P > 0.05). BMD of males and females was lower in treatment group than that in control group [(0.617±0.197) g/cm2 vs. (0.718±0.274) g/cm2; (0.594±0.124) g/cm2 vs. (0.677±0.157) g/cm2, P < 0.05]. ② Comparisons in treatment group before and after treatment: Contents of CT and urine calcium were lower after treatment than those before treatment [(95.3±55.4) ng/L vs. (283.3±96.7) ng/L; (2.38±1.68) mmol/L vs. (3.31±2.30) mmol/L; P < 0.01, 0.05]; however, contents of 1,25-(OH)2VitD3 and DPD/creatinine were higher after treatment than those before treatment [(33.61±19.30) ng/L vs. (24.21±14.47) ng/L; (42.95±19.92) nmol/mmol vs. (19.51±9.96) nmol/mmol, P < 0.05]; moreover, there were no significant differences among other indexes before and after treatment (P > 0.05). Furthermore, there was no significant difference of BMD before and after treatment (P > 0.05). CONCLUSION: WD patients have changes in the related indexes of abnormal skeletal metabolism. In addition, contents of CT and urine calcium are decreased remarkably after decopper therapy; however, value of BMD is not changed obviously.
基金Item Sponsored by National Natural Science Foundation and Steel Research Combination Foundation of China (50574002)
文摘Decopperization in molten steel with FeS-Na2 S slag was investigated in the laboratory. It was found that with an increase in FeS content of the FeS-Na2 S slag, the decopperization ratio increased. The decopperization ratio could reach the maximum of 71.6% in hot metal. The decopperization ratio was closely related to the carbon content, and the higher the carbon content, the higher was the decopperization ratio. Sulphur addition was found in the process of decopperization, and the increase in sulphur content of molten steel was about 0. 2%-0. 42%. However, by adding 30%-50% of BaO to the FeS-Na2 S slag, the addition of sulfur content in molten steel could be reduced by about 50%. The finer slag system composition was FeS ： NazS ： BaO=40 ： 20 ： 40.
基金Project(51104183)supported by the National Natural Science Foundation of ChinaProject supported by the China Scholarship Council
文摘Copper is difficult to separate from nickel electrolyte due to low concentration of copper (0.53 g/L) with high concentration of nickel (75 g/L). Manganese sulfide (MnS) was used to deeply remove copper from the electrolyte. Experimental results show that the concentration of copper (ρ(Cu)) decreases from 530 to 3 mg/L and the mass ratio of copper to nickel (RCu/Ni) in the residue reaches above 15 when the MnS dosage is 1.4 times the theoretical valueDt,MnS (Dt,MnS=0.74 g) and the pH value of electrolyte is 4?5 with reaction time more than 60 min at temperatures above 60 °C. The concentration of newly generated Mn2+(ρ(Mn)) in the solution is also reduced to 3 mg/L by the oxidation reaction. The values ofρ(Cu),ρ(Mn)andRCu/Ni meet the requirements of copper removal from the electrolyte. It is shown that MnS can be considered a highly effective decoppering reagent.
基金supported by Hunan Province Scientifi c Fund of Department of Health(B2006-052)the Project of New Clinic Techniques of Central South University.
文摘Background: Medical therapy is rarely effective inpatients with fulminant Wilson's disease (FWD). Livertransplantation is limited by the lack of donor liver inmost patients with FWD at the time of diagnosis. NewWilson's index, model for end-stage liver disease (MELD)and Child-Pugh score are useful tools for decisionmakingof liver transplantation;however, none of them isan independent decisive tool. It is worthwhile to explorea more effective and practical therapeutic strategy andreevaluate the prediction systems for patients with FWD.Methods: Nine patients with FWD associated withhemolytic crisis and fulminant hepatic failure (FHF) wereinvestigated. The clinical presentation, prognostic scoreand medical therapies of the patients were analyzed.Results: In 7 of the 9 patients with FWD who receivedthe comprehensive therapy of corticosteroid, copperchelatingagent (dimercaptopropansulfonate sodium)and therapeutic plasma exchange (TPE), 6 patientsrecovered from FHF. The remaining one had beenimproved through the comprehensive therapy but died ofsepticemia 51 days later. Two patients with spontaneousbacterial peritonitis (SBP) died from liver failure inthree or five hospital days without plasma exchangeor chelating therapy. All of the 9 patients with FWDpresented with acute hepatic failure, severe jaundice andmild to severe hemolytic anemia. No marked differencein the incidence of severe hemolytic anemia was detectedbetween the survival and deceased groups. However,the incidence and the degree of hepatic encephalopathy(HE) in the non-survival group were higher than thosein the survival group. Unlike the deceased group, thesurvival group had no complications induced by bacterialinfection. Compared to new Wilson's index, Child-Pughscore and MELD score, the variation of prothrombinactivity (PTA) between the survival and deceased groupswas more evident.Conclusions: For patients with FWD, the episodeof severe hepatic encephalopathy or/and spontaneousbacterial peritonitis indicates worse prognosis, andPTA is a recommendable predictor. An emergent livertransplantation should be considered for patients whosePTA is below 20%, or for those with severe HE or/and SBP. The comprehensive therapy of corticosteroid,copper-chelating agent and TPE is effective for patientswithout SBP and whose PTA is higher than 20%.
