Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: EC:1.16.3.1 (ceruloplasmin)
 5,074 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Twenty-five samples obtained by amniocentesis were studied in 25 pregnant women between 35 and 40 weeks of pregnancy with hypertension. The following biochemical investigations were done in the samples: total protein, beta-lipoproteins, cholesterol, uric acid, urea, creatinine, AlAT and AspAT, total alkaline phosphatase and its thermostable isoenzyme, ceruloplasmin and alpha-amylase. The results were analysed in relation to the development of the respiratory distress syndrome in the newborn and were subjected to statistical analysis. In the amniotic fluid of hypertensive mothers in whose children the respiratory distress syndrome developed, reduction was found in the concentrations of beta-lipoproteins and cholesterol. This may have a prognostic significance in the prediction of respiratory distress in early neonatal period.
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PMID:[Respiratory distress in newborns born to hypertensive mothers and protein, lipid and renal maturity indices and enzymatic activity in the amniotic fluid]. 270 91

Serum ceruloplasmin activity in full term and premature neonates was measured on the first day of life. Preterm newborns were born more than 24 hours after antenatal corticosteroid treatment, had enzyme activity equivalent to that of the full term babies. Little or no ceruloplasmin activity was found in those prematures who were born without steroid prophylaxis. Highly significant correlation was observed between the initial ceruloplasmin activity and the morbidity and mortality of respiratory distress syndrome.
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PMID:Antenatal corticosteroid increases serum ceruloplasmin activity in premature neonates. 345 4

Oxidant injury and release of proteolytic enzymes in prematures with respiratory distress syndrome (RDS), who are treated with ventilators and oxygen, have been postulated as possible causes of bronchopulmonary dysplasia (BPD). The premature may be at particular risk due to low levels of antiproteases, such as alpha-1-proteinase inhibitor (alpha 1PI), and antioxidants, such as ceruloplasmin (CER). Both alpha 1PI and CER deficiencies have been correlated with the severity of RDS. We studied serial alpha 1PI activity as measured by trypsin inhibitory capacity (TIC) and CER in the serum 27 prematures who required ventilator therapy for RDS. Serum TIC values for day 1 were significantly lower (0.34 vs. 0.92 mg inhibited/ml of sample) in the 13 patients who developed BPD compared to the 14 who did not. No significant differences were seen on succeeding days. No significant differences in CER were seen, although both groups had levels 33-50% of adult normals (11.3 vs 9.3 mg/dl). Other significant variables included birthweight (p less than 0.005), severity of RDS (p less than 0.03), and gestational age (p less than 0.03). One way analysis of variances demonstrated day 1 TIC to be the most significant variable (p less than 0.0001), followed by weight (p less than 0.007), severity RDS (p less than 0.04), and gestational age (p less than 0.03). CER levels were not a significant variable. A formula utilizing unstandardized canonical discriminant function including day 1 TIC, birthweight, severity of RDS, and gestational age was 100% sensitive and 85% specific in the prediction of BPD for the original study group. In an additional 25 consecutive admissions with severe RDS of whom 18 survived, the formula was 100% sensitive (6/6) and 75% specific (9/12).(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Serial trypsin inhibitory capacity and ceruloplasmin levels in prematures at risk for bronchopulmonary dysplasia. 349 55

Bronchopulmonary dysplasia is a most frequent contemporary lesion of the lung in early childhood. It is characterized by clinical symptoms (neonatal respiratory distress syndrome) and by X-ray picture reflecting progressive morphological changes in the respiratory tract, i.e. in trachea, bronchi, bronchioles, and pulmonary acini, followed by interstitial pulmonary lesion. As usual, bronchopulmonary dysplasia is forerun by hyaline membranes and may be associated with or followed by interstitial emphysema. Pathogenetic participants are toxicity of highly concentrated and long administered oxygen, artificial mechanical ventilation with an intermittently positive pressure, barotrauma first of immature lung causing emphysema and pneumothorax and pneumomediastinum, lung edema, shortage of A and E vitamins and ceruloplasmin deficiency. Morphological changes in bronchopulmonary dysplasia are alike diffuse alveolar damage in bigger children or adults. Nevertheless, neonatal changes differ from later pulmonary lesion by evolving in an immature tissue and by being complicated with necrotizing "obstructive" bronchiolitis.
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PMID:[Bronchopulmonary dysplasia]. 833 23

This study compared plasma levels of albumin, transferrin, and ceruloplasmin in well preterm babies (n = 21) with those with respiratory distress syndrome (RDS, n = 13) and chronic lung disease (CLD, n = 13) over the first 28 postnatal days. Plasma lipid peroxidation, total radical trapping capacity (TRAP assay), and iron binding antioxidant capacity were also measured. In RDS and CLD albumin levels were decreased on days 1, 4 and 10; on day 10 albumin was lower in CLD compared to RDS (p < .05). After day 10 the levels were similar in all groups. The transferrin levels showed a similar trend. Ceruloplasmin levels did not differ, except for a higher day 28 level in CLD (p < .05). Albumin levels significantly decreased with increasing FiO2 and duration of oxygen therapy (within patient r = -0.30, p < .05 and r = -0.51, p < .005, respectively). On day 10, increasing oxygen therapy increased plasma lipid peroxidation (r = +0.49, p < .01), which was also significantly related to lower plasma protein levels (r = -0.42, p < .01). Lower plasma albumin and transferrin lowered the TRAP and iron binding antioxidant capacity, respectively (r = +0.36, p < .05, and r = +0.41, p < .005). Prediction of CLD using day 10 albumin levels had a specificity of 94%, but a sensitivity of only 50%. The interaction between oxygen toxicity and high ventilation pressures in immature babies appears to lower plasma proteins by increasing pulmonary permeability. The lower plasma albumin level was not useful in predicting the development of CLD; however, the fall in plasma transferrin and albumin will further decrease the preventive and chain-breaking antioxidant capacity of plasma of these ill babies.
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PMID:Plasma proteins in acute and chronic lung disease of the newborn. 968 Jan 78

The present study identifies proteins modified by nitration in the plasma of patients with ongoing acute respiratory distress syndrome (ARDS). The proteins modified by nitration in ARDS were revealed by microsequencing and specific antibody detection to be ceruloplasmin, transferrin, alpha(1)-protease inhibitor, alpha(1)-antichymotrypsin, and beta-chain fibrinogen. Exposure to nitrating agents did not deter the chymotrypsin-inhibiting activity of alpha(1)-antichymotrypsin. However, the ferroxidase activity of ceruloplasmin and the elastase-inhibiting activity of alpha(1)-protease inhibitor were reduced to 50.3 +/- 1.6 and 60.3 +/- 5.3% of control after exposure to the nitrating agent. In contrast, the rate of interaction of fibrinogen with thrombin was increased to 193.4 +/- 8.5% of the control value after exposure of fibrinogen to nitration. Ferroxidase activity of ceruloplasmin and elastase-inhibiting activity of the alpha(1)-protease inhibitor in the ARDS patients were significantly reduced (by 81 and 44%, respectively), whereas alpha(1)-antichymotrypsin activity was not significantly altered. Posttranslational modifications of plasma proteins mediated by nitrating agents may offer a biochemical explanation for the reported diminished ferroxidase activity, elevated levels of elastase, and fibrin deposits detected in patients with ongoing ARDS.
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PMID:Plasma proteins modified by tyrosine nitration in acute respiratory distress syndrome. 1078 26

A 16-year-old woman presented with anaemia, jaundice, vomiting and nosebleed. She had acute hepatic failure and haemolytic anaemia and developed acute respiratory distress syndrome (ARDS). Wilson's disease was diagnosed. After the ARDS resolved the patient underwent a successful orthotopic liver transplantation. Diagnostic combinations for Wilson's disease are ceruloplasmin < 0.2 g/l with Kayser-Fleischer rings, liver copper > 250 micrograms/g (dry weight) with Kayser-Fleischer rings, or homozygosity for a Wilson mutation on the 13th chromosome. In acute liver failure a copper excretion in 24 h-urine above 1 mg is diagnostic for Wilson's disease, while an elevated serum copper concentration makes this diagnosis very likely. Therapeutic options for Wilson's disease are chelation therapy and liver transplantation; in most cases of acute liver failure due to Wilson's disease orthotopic liver transplantation (preceded by albumin dialysis) is indicated. Nazer's index should be used in addition to the regular King's College criteria for liver transplantation indication.
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PMID:[An adolescent with hemolytic anemia and coagulation disorders as manifestation of Wilson's disease, treated with liver transplantation]. 1123 95

Newborns, and especially those delivered preterm, are probably more prone to oxidative stress than individuals later in life. Also during pregnancy, increased oxygen demand augments the rate of production of reactive oxygen species (ROS) and women, even with normal pregnancies, experience elevated oxidative stress and lipid peroxidation compared with nonpregnant women. Also, there appears to be an increase in ROS generation in the placenta of pre-eclamptic women. In comparison with healthy adults, newborn infants have lower levels of plasma antioxidants such as vitamin E, beta-carotene, and sulphydryl groups, lower levels of plasma metal binding proteins including ceruloplasmin and transferrin, and reduced activity of erythrocyte superoxide dismutase. This review summarizes conditions of newborns where there is elevated oxidative stress. Included in this group of conditions is asphyxia, respiratory distress syndrome and sepsis and the review also summarizes the literature related to clinical trials of antioxidant therapies and of melatonin, a highly effective antioxidant and free radical scavenger. The authors document there is general agreement that short-term melatonin therapy may be highly effective and that it has a remarkably benign safety profile, even when neonates are treated with pharmacological doses. Significant complications with long-term melatonin therapy in children and adults also have not been reported. None of the animal studies of maternal melatonin treatment or in postnatal life have shown any treatment-related side effects. The authors conclude that treatment with melatonin might result in a wide range of health benefits, improved quality of life and reduced healthcare costs and may help reduce complications in the neonatal period.
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PMID:Oxidative stress of the newborn in the pre- and postnatal period and the clinical utility of melatonin. 1905 96