PubMed Health⌕ Search

SEARCH · PubMed Health

Results for “PSEUDOSCLEROSIS”

Explore indexed PubMed citations for clinical trials, systematic reviews and public health research. Read source abstracts and follow each citation to its original PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 37 records · Page 2Linked to original sources
PubMed1990

[Clinical analysis of 418 patients with Wilson's disease in traditional Chinese medicine and Western medicine therapy].

From 1974 to 1988, 418 cases of Wilson's disease were treated with TCM-WM in our hospital. 147 cases were of Wilson's type; 149 cases were of pseudosclerosis type; 40 cases were of abdominal and hepatocerebral type; 21 cases were of choreoathetosis type and 21 cases of other types. After a course of treatment for 3 to 6 months, 103 patients showed marked improvement and 286 made some improvement, but no effect was found in 22 patients and 7 deaths were observed. The results were as follows: (1) The mortality in the severe and moderate groups were significantly higher than the mild (P less than 0.05) and the marked effective rate was less than the latter (P less than 0.01). (2) The marked effective rate was lower in abdominal and hepatocerebral type, and no significant difference was found in recovery rate between Wilson's type and pseudosclerosis type.

Adolescent↗
PubMed2000

[Clinical and fine motor therapy assessment in Wilson disease].

At the time of diagnosis and after therapy, we examined 33 patients suffering from Wilson's disease. We applied a standardized diagnostic score system on the basis of clinical signs. Without observing any differences between pseudoparkinsonian and pseudosclerosis subtypes, patients with neurological symptoms significantly improved by 2.33 points. Patients with initially more severe symptoms showed the same improvement as less affected patients. Fine motor disturbances were evaluated using the V-scope system. Finger tapping and drawing a spiral were compared to values of a healthy control group (n = 52). Patients with neurological symptoms showed significantly decreased frequencies in both tests. The clinical score was related to frequencies in finger tapping but not in drawing a spiral. Therefore finger tapping can be used as an objective diagnostic tool to evaluate the severity of Wilson's disease, while spiral testing appears to be a sensitive screening tool.

Adult↗
PubMed1998

Copper-chelating therapeutic effect in Wilson disease with different clinical phenotypes and polymorphisms of ATP7B gene.

AIM:To investigate the copper-chelating therapeutic effect in Wilson disease (WD) with different clinical phenotypes and polymorphisms of ATP7B gene.METHODS:One hundred and twenty-two WD patients with different clinicalphenotypes were given DMPS intravenously and Gandou copper-chelating tablet orally for one month. The therapeutic effect was judged by modified Goldstein mothod. Exon 18 of ATP7B gene extracted from the DNA of patients and 20 healthy volunteers was amplified with PCR mutation and polymorphism were screened with SSCP technique.RESULTS:Four kinds of abnormal migration bands in PCR-SSCP were observed in 37 WD patients, mutation frequencies of three different disease phenotypes, and curative effect between mutation group and non-mutation group showed no statistically significant difference (P > 0.05), but the total effectiveness rates in patients with Wilson type or pseudosclerosis type were significantly higher than those of patients with hepatic type (X(2) = 6.17, P < 0.05).CONCLUSION:Most WD patients are compound heterozygotes, the patients with different clinical phenotypes have different response to copper-chelating therapy. Specific mutation, at least in part, plays a role in influencing the disease phenotypes and therapeutic effect.

Journal Article↗
PubMed1998

[Curative effect of TCM-WM therapy on Wilson disease with different clinical phenotypes and polymorphisms of ATP7B gene].

OBJECTIVE: To investigate the curative effect of TCM-WM therapy on Wilson disease (WD) with different clinical phenotypes and polymorphisms of ATP7B gene. METHODS: One hundred and twenty-two WD patients with different clinical phenotypes were treated with unithiol 20 mg/kg per day and Gandou tablet ([symbol: see text]) for 1 month. Exon 18 of ATP7B gene extracted from patients and 20 healthy volunteers were amplified with PCR technique, mutations and polymorphisms were screened by SSCP analysis. RESULTS: Four kinds of abnormal migration bands in PCR-SSCP were seen in 37 patients, mutation frequencies of three different clinical phenotypes and curative effect between mutation and non-mutation patients did not show any significant difference, P > 0.05. But total effective rate of patients with Wilson type or pseudosclerosis type was significantly higher than that of patients with hepatic type, P < 0.05. CONCLUSIONS: The exon 18 of ATP7B gene of Chinese WD patients may be one of the regions with higher mutation frequency, most of them are compound heterozygotes and patients with different clinical phenotypes have different response to TCM-WM therapy. It indicate that specific mutations at least in part play a role in influencing clinical phenotypes and curative effect.

Adenosine Triphosphatases↗