Treatment of Wilson's disease with penicillamine and zinc salts : a follow-up study

OBJECTIVE: Wilson's disease (WD) is an autosomal recessive disorder characterized by excessive accumulation of copper in the liver and later in the brain and other organs. Penicillamine acts as a reductive chelator. Zinc salts induce the synthesis of metallothionein in cells. Thus these two dru...

Description complète

Détails bibliographiques
Publié dans:Zhonghua er ke za zhi = Chinese journal of pediatrics. - 1960. - 41(2003), 2 vom: 15. Feb., Seite 119-22
Auteur principal: Li, Ming (Auteur)
Autres auteurs: Zhang, Yue-hua, Qin, Jiong
Format: Article
Langue:Chinese
Publié: 2003
Accès à la collection:Zhonghua er ke za zhi = Chinese journal of pediatrics
Sujets:Clinical Trial English Abstract Journal Article Antidotes Penicillamine GNN1DV99GX Zinc J41CSQ7QDS
LEADER 01000caa a22002652c 4500
001 NLM144642425
003 DE-627
005 20250205071539.0
007 tu
008 231223s2003 xx ||||| 00| ||chi c
028 5 2 |a pubmed25n0482.xml 
035 |a (DE-627)NLM144642425 
035 |a (NLM)14759316 
040 |a DE-627  |b ger  |c DE-627  |e rakwb 
041 |a chi 
100 1 |a Li, Ming  |e verfasserin  |4 aut 
245 1 0 |a Treatment of Wilson's disease with penicillamine and zinc salts  |b a follow-up study 
264 1 |c 2003 
336 |a Text  |b txt  |2 rdacontent 
337 |a ohne Hilfsmittel zu benutzen  |b n  |2 rdamedia 
338 |a Band  |b nc  |2 rdacarrier 
500 |a Date Completed 22.04.2004 
500 |a Date Revised 07.06.2016 
500 |a published: Print 
500 |a Citation Status MEDLINE 
520 |a OBJECTIVE: Wilson's disease (WD) is an autosomal recessive disorder characterized by excessive accumulation of copper in the liver and later in the brain and other organs. Penicillamine acts as a reductive chelator. Zinc salts induce the synthesis of metallothionein in cells. Thus these two drugs are theoretically synergistic for the treatment of the disease. However, the two drugs may also have some unfavorable interactions. In this study, the effect of the therapy with combined penicillamine and zinc salts was evaluated based on the follow-up observations of 21 patients with Wilson's disease 
520 |a METHODS: Using the combined therapy of penicillamine [10-30 mg/(kg.d)] and zinc (22.5 mg, 3 times per day), follow-up study by hospitalization or communication with telephone or mail 
520 |a RESULTS: Before treatment, all the 21 patients were suffered from chronic liver disorder. Among them, 13 patients (62%) showed to be reactive to the treatment for their liver disorder, 5 patients (24%) died, and 3 patients (14%) dropped off our follow-up study. Among the 5 patients who died, 3 died within 40 days after treatment, one had taken penicillamine only 8 mg/(kg.d), and one died after discontinuation of the treatment by the parents. Of the 12 patients having neurological involvement, neurological symptoms disappeared or markedly improved in 11 patients after treatment. One patient dropped off the follow-up study. The patient with renal tubular acidosis responded well to the treatment. Urine routine analysis was followed up in 6 of the 7 patients with hematuria. Hematuria disappeared in one, became less severe in 1, and remained unchanged in 4 patients. Hypersensitivity to penicillamine was found in one patient. WBC and platelet were found decreased further in 3 patients after the medications 
520 |a CONCLUSIONS: The combined therapy with penicillamine and zinc salts was effective in treatment of patients with Wilson's disease 
650 4 |a Clinical Trial 
650 4 |a English Abstract 
650 4 |a Journal Article 
650 7 |a Antidotes  |2 NLM 
650 7 |a Penicillamine  |2 NLM 
650 7 |a GNN1DV99GX  |2 NLM 
650 7 |a Zinc  |2 NLM 
650 7 |a J41CSQ7QDS  |2 NLM 
700 1 |a Zhang, Yue-hua  |e verfasserin  |4 aut 
700 1 |a Qin, Jiong  |e verfasserin  |4 aut 
773 0 8 |i Enthalten in  |t Zhonghua er ke za zhi = Chinese journal of pediatrics  |d 1960  |g 41(2003), 2 vom: 15. Feb., Seite 119-22  |w (DE-627)NLM136249191  |x 0578-1310  |7 nnas 
773 1 8 |g volume:41  |g year:2003  |g number:2  |g day:15  |g month:02  |g pages:119-22 
912 |a GBV_USEFLAG_A 
912 |a SYSFLAG_A 
912 |a GBV_NLM 
912 |a GBV_ILN_11 
912 |a GBV_ILN_20 
912 |a GBV_ILN_22 
912 |a GBV_ILN_24 
912 |a GBV_ILN_31 
912 |a GBV_ILN_39 
912 |a GBV_ILN_40 
912 |a GBV_ILN_50 
912 |a GBV_ILN_61 
912 |a GBV_ILN_65 
912 |a GBV_ILN_69 
912 |a GBV_ILN_70 
912 |a GBV_ILN_72 
912 |a GBV_ILN_120 
912 |a GBV_ILN_130 
912 |a GBV_ILN_227 
912 |a GBV_ILN_244 
912 |a GBV_ILN_285 
912 |a GBV_ILN_294 
912 |a GBV_ILN_350 
912 |a GBV_ILN_665 
912 |a GBV_ILN_813 
951 |a AR 
952 |d 41  |j 2003  |e 2  |b 15  |c 02  |h 119-22