Diagnosis and management of fulminant Wilson's disease: a single center's experience

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%.

Acupuncture for dystonia in brain-type Wilson’s disease with internal retention of damp heat pattern:A randomized clinical trail

Objective:To observe the clinical efficacy of acupuncture on dystonia in brain-type Wilson’s disease(WD)with internal retention of damp heat pattern.Methods:A total of 60 patients with WD dystonia with internal retention of damp heat pattern were randomized into acupuncture and medication groups using a random number table,with 30 participants in each group.All patients had a low-copper diet and consumed dimercaptopropanesulfonate sodium(DMPS) for copper excretion.In the acupuncture group,on the base of the same treatment as that given to the medication group,acupuncture was applied at Baihui(百会GV20),Shenting(神庭GV24),Chéngjiāng(承浆CV4),Jiānyú(肩髃L15),Nàoshū(臑俞SI10),Wàiguān(外关TE5),Nèiguān(内关PC6),Shǒusānlǐ(手三里LI10),Hégǔ(合谷LI4),Yángxī(阳溪LI5),Huántiào(环跳GB30),Bìguān(髀关ST31),Yánglíngquán(阳陵泉GB34),Fēnglóng(丰隆ST40),Zúsānlǐ(足三里ST36),Sānyīnjiāo(三阴交SP6),Xuánzhōng(悬钟GB39),and Xíngjiān(行间LR2).Before and 24 days after treatment,the modified Ashworth scale(MAS) and BurkeFahn-Marsden dystonia rating scale(BFMDRS) were used to evaluate dystonia symptoms.The allocation scheme was concealed for the outcome assessors.Results:The data of 30 cases were analyzed in each group.Before treatment,the MAS score difference between the acupuncture and medication groups was not statistically significant(P> 0.05).Compared with the score before treatment,the MAS score was lower significantly in both the acupuncture group(2.63±0.76 vs 4.50 ± 0.78) and medication group(3.30 ± 0.65 vs 4.40 ± 0.77) after treatment(both P <0.05).After treatment,the MAS score in the acupuncture group was significantly lower than that in the medication group(P <0.01).Before treatment,the BFMDRS score was not significantly different between the two groups(P> 0.05).Compared with the score before treatment,the BFMDRS score was significantly lower in both the acupuncture(64.97 ± 14.26 vs 85.23± 16.99) and medication groups(11.33 ± 2.60 vs 75.40 ± 16.25) after treatment(both P <0.05).The BFMDRS score of the acupuncture group was lower than that of the medication group after treatment(P <0.05).During treatment,1 case had allergic reaction of DMPS in the acupuncture group,manifested as reddish skin and skin rashes,and the allergic symptoms disappeared after anti-allergic treatment.There was no any adverse reaction in the medication group.Conclusion:Combined treatment with acupuncture and Western medication is significantly effective against dystonia in brain-type WD with internal retention of damp heat pattern.