Turk J Pediatr； Decreased antioxidant capacity of serum natural thiol and total thiol levels in children with hemophilia A: a prospective case-control study

Experimental studies have addressed the role of oxidative stress in the pathogenesis of hemophilia one
.
This study aimed to identify dynamic thiol-disulfide exchange, a recently discovered cell defense system against oxidative stress that is disturbed
in children with hemophilia A.
The study reported in this report aimed to determine whether serum levels of native mercaptans, total mercaptans, and disulfides in children with hemophilia A and the ratios between these variables changed compared to normal healthy controls
.

This prospective case-control study included boys with hemophilia A (n = 62) and randomly selected healthy age- and sex-matched controls (n = 62).

Serum natural mercaptan, total mercaptan, and disulfide levels were analyzed
by a new spectrophotometry, respectively.
Disulfide/total mercaptan, disulfide/natural mercaptan ratio, and calculate natural/total mercaptans
.
Use an independent-sample t-test or a Mann-Whitney U test, depending on whether the data are normally distributed
.

Table 1: Clinical features of hemophilia A in children (n=62)

Table 2: Comparison
of oxidative markers in children with hemophilia A versus healthy controls.

Studies suggest that serum natural thiol and total thiol levels are significantly lower compared to
the control group.
The serum levels of natural thiols and total thiols in children with hemophilia A were significantly lower than those in the control group (p=0.
024).

The serum disulfide level of children with hemophilia A was close to that of the control group, while the ratio of disulfide bonds to natural mercaptans (p=0.
024) and the ratio of disulfide to total thiols (P=0.
024) were significantly increased
.

Overall, studies suggest that reduced serum levels of native thiols and total thiols and decreased antioxidant capacity in children with hemophilia A may be considered some evidence
of a disturbance in the mercaptan/disulfide balance.
In addition to gene therapy and new therapeutic products, antioxidant therapy may be the subject
of future research in the development of inhibitors and the prevention of complications in children with hemophilia A.

Original source:

Taşar S, Güven AG, Olgaç A, Neşelioğlu S, Erel Ö, Alioğlu B.
Decreased antioxidant capacity with serum native thiol and total thiol levels in children with hemophilia A: a prospective case-control study.
Turk J Pediatr.
2022; 64(5):876-881.
doi: 10.
24953/turkjped.
2022.
73.
PMID: 36305437.