Assessment of dynamic thiol-disulfide homeostasis in patients with lipoid proteinosis (Urbach-Wiethe syndrome)
Rev. Assoc. Med. Bras. (1992, Impr.)
;
68(9): 1259-1263, Sept. 2022. tab, graf
Article
in English
|
LILACS-Express
| LILACS
| ID: biblio-1406651
ABSTRACT
SUMMARY OBJECTIVE: Lipoid proteinosis is a rare autosomal recessive genetic dermatological disease that occurs due to the accumulation of hyaline material in the skin and mucous membranes. This study aimed to investigate whether dynamic thiol-disulfide homeostasis is a new marker of oxidative stress in patients suffering from lipoid proteinosis. METHODS: The study group involved 17 patients with lipoid proteinosis and 17 healthy controls with same gender and age. Native thiol, total thiol, disulfide levels, and thiol-disulfide indexes were measured with the fully automated spectrophotometric method described by Erel and Neselioglu, and the results of the two groups were statistically analyzed. RESULTS: Serum total thiol and native thiol levels were significantly lower in lipoid proteinosis group compared to the control group (p=0.020 and p=0.014, respectively). The disulfide levels were found to be higher in lipoid proteinosis group, but there was no significant difference between two groups. CONCLUSIONS: Impaired dynamic thiol-disulfide homeostasis was observed in lipoid proteinosis patients, suggesting that thiol-disulfide homeostasis may have a role in the pathogenesis of this disease.
Full text:
Available
Index:
LILACS (Americas)
Language:
English
Journal:
Rev. Assoc. Med. Bras. (1992, Impr.)
Journal subject:
EducaÆo em Sa£de
/
GestÆo do Conhecimento para a Pesquisa em Sa£de
/
Medicine
Year:
2022
Type:
Article
Affiliation country:
Turkey
Institution/Affiliation country:
Harran University/TR
/
Lokman Hekim University/TR
/
Sanlıurfa Training and Research Hospital/TR
Similar
MEDLINE
...
LILACS
LIS