Reduced Free-Radical-Trapping Capacity and Altered Plasma Antioxidant Status in Cystict Fibrosis

Langley, Simon C.; Brown, R K; Kelly, Frank J.

doi:10.1203/00006450-199303000-00008

Cited by 46 publications

(23 citation statements)

References 18 publications

(24 reference statements)

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“…Because it is well established that neutrophils, when activated, are a major source of free radicals (ll), it follows that those CF patients with marginal or depleted antioxidant defenses are likely to exhibit evidence of increased oxidative stress, especially during episodes of acute pulmonary infection. Results from our previous study (6) support such a hypothesis; we found that C F plasma had a reduced capacity to withstand increased oxidative damage in vitro, indicating that patients may be more sensitive to free radical-mediated attack in vivo.…”

supporting

confidence: 67%

“…This is not surprising, because all the patients were receiving vitamin supplements and the pancreatic enzyme Creon (Reid-Rowell, Marietta, GA). The program of dietary management and nutrient supplementation instituted at Southampton General Hospital has stabilized the plasma concentrations of vitamin E in C F patients as reported previously (6).…”

Section: Resultsmentioning

confidence: 89%

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Evidence for Increased oxidative Damage in Patients with Cystic Fibrosis

1994

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Patients with cystic fibrosis (CF) may be more susceptible to oxidative cell injury than normal healthy children due to both the impaired absorption of antioxidant nutrients and the increased oxidative stress caused by chronic pulmonary infections. The purpose of this study was to examine whether markers of oxidative damage to lipids (malondialdehyde-like substances and lipid hydroperoxides) and proteins (protein carbonyls) were present in the plasma of CF patients. Mean values (? SD) of thiobarbituric acid-reactive substances were significantly higher in patients (6.93 & 1.47 prnol/L, n = 25) than in controls (5.84 2 0.59 ~mol/L,; n = 10). FFA hydroperoxides were not detected in control subjects (the detection limit of the assay was 0.02 pmol/L), but in 11 of the 33 CF patients studied they were found in a range of 0.03-0.34 pmoVL. Plasma protein carbonyl concentrations did not differ significantly between the two groups @ = 0.076), although a much wider distribution was observed in the CF patients (range 0.17-5.64 nmollmg protein) than in the control group (range 0.24-1.55 nmollmg protein). No correlation was found between thiobarbituric acid-reactive substances and FFA

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confidence: 67%

Section: Resultsmentioning

confidence: 89%

Evidence for Increased oxidative Damage in Patients with Cystic Fibrosis

1994

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“…Accordingly, it is also possible that ascorbic acid and sulfhydryl groups acted as pro-oxidants rather than antioxidants in WD patients during hemolytic crisis. In recent studies, the TRAP assay has provided useful information about plasma antioxidant activity in newborns (23) and patients with cystic fibrosis (39). Plasma antioxidant capacity calculated as TRAP was significantly decreased in WD patients during a hemolytic episode.…”

Section: Discussionmentioning

confidence: 99%

Erythrocyte Metabolism and Antioxidant Status of Patients with Wilson Disease with Hemolytic Anemia

et al. 2006

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ABSTRACT:Wilson disease (WD) is an autosomal recessive disorder due to the defect in ATP7B gene characterized by excessive accumulation of copper in the liver with progressive hepatic damage and subsequent redistribution to various extrahepatic tissues including the brain, kidneys, and cornea. Strikingly, the total serum copper concentration is always low in WD, even though the nonceruloplasmin copper level is still expected to be high. To assess the role of free radical reactions catalyzed by non-ceruloplasmin copper, we investigated erythrocyte metabolism and oxidative stress as a mechanism for hemolysis in eight WD patients during episodes of acute hemolysis and compared them with eight follow-up cases of WD on D-penicillamine therapy and eight healthy, age-matched children. Elevated levels of non-ceruloplasmin copper were found in all the WD patients during an episode of hemolytic anemia. There was marked inhibition in erythrocyte enzymes, namely, hexokinase, total adenosine triphosphatase (ATPase), and glucose-6-phosphate dehydrogenase (G-6-PD) from WD patients compared with patients on penicillamine and healthy children, indicating altered erythrocyte metabolism during a hemolytic crisis. Antioxidant status was also found to be compromised as is evident from decreased glutathione (GSH) levels, decreased antioxidant enzymes (namely, superoxide dismutase, catalase, glutathione peroxidase, and glutathione reductase), increased lipid peroxidation, and deranged plasma antioxidants. Uric acid showed maximum decrease followed by ascorbic acid. These findings suggest that the free radical production by elevated non-ceruloplasmin copper through transition metal catalyzed reactions leads to oxidative injury resulting in altered erythrocyte metabolism and severely compromised antioxidant status of WD patients during hemolytic anemia. (Pediatr Res 59: 593-597, 2006)

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“…The total antioxidant capacity of plasma is dependent on the synergism between various antioxidants (11) in addition to the capaci ty of each single antioxidant actin g alon e. Alth ough a test has been developed to measure the TRAP of plasma (12), these potenti ally important interactions have only been explored in a few clini cal studies (13)(14)(15). In this stud y, we also measured the TRAP value to evaluate the total plasma antioxidant capacity in Wilsonian patients.…”

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confidence: 99%