A study of oxidative stress, cytokines and glutamate in Wilson disease and their asymptomatic siblings

Kalita, Jayantee; Kumar, Vijay; Ranjan, Abhay; Khan, Hamidullah; Konwar, Rituraj

doi:10.1016/j.jneuroim.2014.06.013

Cited by 38 publications

(22 citation statements)

References 37 publications

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“…Plasma lipid peroxidation (LPO) was measured by assessing MDA level, according to the method described by Janero (1990). The GSH level in normal controls is 2.73 AE 0.04 mg/dl and MDA 3.03 AE 0.52 nmol/ml which were derived from 64 healthy individuals and have been reported in our earlier study (Kalita et al, 2014b).…”

Section: Background and Aimmentioning

confidence: 99%

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MRI and oxidative stress markers in neurological worsening of Wilson disease following penicillamine

et al. 2015

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Section: Background and Aimmentioning

confidence: 99%

“…Their clinical, biochemical and repeat MRI findings were noted. Serum free copper and plasma GSH and MDA levels were measured at baseline, at the time of deterioration and when they stabilized (Kalita et al, 2014b). Plasma GSH was measured by spectrophotometer at 412 nm according to the method described by Tietze (1969).…”

Section: Background and Aimmentioning

confidence: 99%

MRI and oxidative stress markers in neurological worsening of Wilson disease following penicillamine

et al. 2015

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“…In our earlier study, we have reported increased MDA, cytokines (interleukin 6, 8, 10 and tumor necrosis factor a), glutamate and reduced GSH and TAC in the WD patients. These changes were more marked in the neurologic WD patients who were untreated (Kalita et al 2014b). The follow-up of these parameters and their correlation with clinical response, however, were not done in the above-mentioned study.…”

Section: Discussionmentioning

confidence: 94%

“…TAC measures the hydrophilic antioxidants and is highly influenced by serum uric acid level (Bruha et al 2012). In a study on WD with neurologic manifestation, reduced levels of GSH and TAC and elevated levels of glutamate, cytokines and MDA have been reported (Kalita et al 2014b).…”

Section: Introductionmentioning

confidence: 99%

Role of Oxidative Stress in the Worsening of Neurologic Wilson Disease Following Chelating Therapy

2015

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Patients with neurologic Wilson disease (NWD) may worsen on treatment, but there is no study evaluating the role of oxidative stress. We report the role of plasma glutathione (GSH), total antioxidant capacity (TAC) and malondialdehyde (MDA) in the worsening of NWD following treatment. Fifty-one treatment-naïve NWD patients were subjected to detailed clinical evaluation. The severity of NWD was noted, and dystonia was measured by Burke-Fahn-Marsden (BFM) score. Their hematological, serum chemistry, ultrasound abdomen and cranial MRI changes were noted. Plasma GSH, TAC and MDA, serum free copper (Cu) and 24-h urinary Cu were measured at admission and at 3 and 6 months after treatment. The patients were considered worsened if there was one or more grade deterioration in severity scale, >10 % deterioration in BFM score or appearance of new neurologic signs. The median age of the patients was 11 (5-37) years, and 12 were females. Following treatment, 25 patients improved, 12 worsened, and 14 had stationary course. The worsened group at 3 months had lower GSH (1.99 ± 0.17 vs. 2.30 ± 0.30 mg/dl; P = 0.004) and TAC (1.59 ± 0.12 vs. 1.82 ± 0.17 mmol Trolox equivalent/L; P = 0.001) and higher MDA (5.24 ± 0.22 vs. 4.34 ± 0.46 nmol/ml; P < 0.001) levels compared to the improved group. These changes were associated with increased serum free Cu (41.81 ± 3.31 vs. 35.62 ± 6.40 µg/dl; P = 0.02) and 24-h urinary Cu (206.42 ± 41.61 vs. 121.99 ± 23.72 µg/24 h; P < 0.001) in the worsened compared to the improved group. All the patients having worsening were on penicillamine. Worsening following chelating treatment in NWD may be due to oxidative stress which is induced by increased serum free Cu. These results may have future therapeutic implication and needs further study.

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“…During the administration of PA or trientine, enhanced oxidative stress and increased free copper levels were found and were associated with neurological disorders and deterioration [4,7,10,11]. When treated with TM, there were no increases in serum free copper, and neurological worsening was rare [4].…”

Section: Introductionmentioning

confidence: 99%

Effects of tetrathiomolybdate and penicillamine on brain hydroxyl radical and free copper levels: A microdialysis study in vivo

Zhang

Liu

Hou

et al. 2015

Biochemical and Biophysical Research Communications

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A study of oxidative stress, cytokines and glutamate in Wilson disease and their asymptomatic siblings

Cited by 38 publications

References 37 publications

MRI and oxidative stress markers in neurological worsening of Wilson disease following penicillamine

MRI and oxidative stress markers in neurological worsening of Wilson disease following penicillamine

Role of Oxidative Stress in the Worsening of Neurologic Wilson Disease Following Chelating Therapy

Effects of tetrathiomolybdate and penicillamine on brain hydroxyl radical and free copper levels: A microdialysis study in vivo

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