Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts: Gene/Protein Disease Symptom Drug Enzyme Compound   Target Concepts: 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)

To determine the adequacy of zinc and copper supplementation for infants receiving total parenteral nutrition (TPN), we performed 24-h balance studies in infants with diarrhea and infants who had recently undergone surgery. Measurements were made at base line, 1, and 2 wk. Mean serum Zn and Cu levels of the diarrhea group remained normal and were low in the postoperative group but normalized over the study period. Mean 24-h Zn and Cu balances were positive in infants with diarrhea and negative in postoperative infants. The high Zn and Cu content in the gastrointestinal fluid loss associated with surgery may have accounted in part for this finding. Normal serum levels of Zn and Cu did not guarantee positive balance. No significant changes were found in serum albumin, alkaline phosphatase, or ceruloplasmin. The current Zn and Cu recommendations may be appropriate only for hospitalized infants who have no excessive gastrointestinal fluid losses.
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PMID:Zinc and copper balance studies in infants receiving total parenteral nutrition. 249 35

Copper deficiency developed in a five-month-old prematurely born infant with an abdominal wall closure defect and a small bowel fistula under long-term parenteral nutrition. The first manifestations were typical bone changes including diffuse osteoporosis, delayed bone age, widened cupped metaphyses with beaks and a fracture, subperiosteal hematomas and ossifications in the shafts of long bones, and a diaphyseal fracture. Other findings that confirmed the diagnosis included edema of the limbs with pseudoparalysis, neurologic abnormalities, anemia, leukoneutropenia, and very low serum levels of copper and ceruloplasmin. Following initiation of copper supplementation, clinical, hematological and biological disorders resolved within a few days and roentgenologic bone abnormalities within four months. Copper deficiency develops only in the presence of specific risk factors, that are often combined in a single patient: inadequate stores due to prematurity, excessive loss due to chronic diarrhea, exudative enteropathy, a proximal stoma, or a biliodigestive fistula; and inadequate intake due to malabsorption or long-term exclusive parenteral nutrition (EPN). Appropriate copper supplementation is needed in all these high risk situations. Early, continuous copper supplementation is required for young children under EPN. Serum copper assays should be included among the periodically monitored biochemical parameters.
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PMID:[Nutritional copper deficiency. Apropos of a case]. 250 Aug 84

Red cell superoxide dismutase (SOD) activity was evaluated as a biochemical index of copper nutrition in a double-blind study of 17 infants recovering from malnutrition and receiving marginal copper intakes. Children were paired on admission by sex, birth weight, nutritional status and antecedents of diarrhea and breast feeding. Nine served as controls receiving a copper sulfate supplement (80 micrograms/kg daily for 120 d; eight received a placebo and were supplemented only if plasma copper levels dropped below 90 micrograms/dl or on d 90 for at least 30 d. After copper supplementation there was a significant rise (paired t-test; P less than 0.05) in plasma copper (96 vs. 165 micrograms/dl); ceruloplasmin (33 vs. 50 mg/dl) and SOD (1073 vs. 1371 U/g Hb). After supplementation these values were similar to those of the controls. SOD was correlated with plasma copper (r = 0.78; P less than 0.001) and not with weight-for-age or weight-for-length. Addition of copper in vitro did not modify the SOD activity. Red cell SOD is a good marker of copper nutrition in humans and correlates well with plasma copper.
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PMID:Red cell superoxide dismutase activity as an index of human copper nutrition. 393 52

Zinc and copper are quite abundant trace elements and important in many metabolic processes and enzyme systems. Preterm infants whose diet is deficient in copper and/or zinc may suffer anemia, anorexia, skin changes, diarrhea, growth retardation, impaired connective tissue formation, osteoporosis, and impaired cell mediated immunity as well as failure to thrive. The fetus stores 70% of its body reserve of copper and zinc in the last trimester, indicating that the premature infant has low reserves of cooper and zinc at delivery. They may not amass or develop body stores in the same was as does the fetus. Premature infants receiving pasteurized breast milk have a negative zinc balance for at least the first 60 days of life and a negative copper balance for the first 35 days of life. Parental nutrition without copper and zinc supplements result in deficiency conditions. Some oxidation-reduction reactions using oxygen may generate free radicals; with ventilators delivering oxygen directly into the alveoli, generated free radicals may pulmonary oxygen toxicity. This condition is called bronchopulmonary dysplasia (BPD). Preterm infants without hyaline membrane disease are more likely to support hyperopic increase of pulmonary superoxide dismutase (SOD) (a free radical scavenger) activities than those with hyaline membrane disease (e.g., plasma samples, 88.5% vs. 33%). This suggests that preterm infants with the condition do not have a plasma factor needed for pulmonary SOD response. A possible factor is ceruloplasmin. Copper and zinc are important components of both SOD and ceruloplasmin. More research is needed to examine serial levels of copper, zinc, SOD, and ceruloplasmin in newborns to determine whether postnatal levels are linked to associated morbidities. In the event of failure of vitamin E therapy, administration of bovine SOD to premature infants may prevent and reduce the severity of BPD. In developing countries, physicians should prevent BPD and other chronic problems of premature infants by administering antioxidants with copper and zinc.
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PMID:Copper, zinc and superoxide dismutase activities in premature infants: a review. 829 4

To delineate further the clinical spectrum of Menkes disease, an X-linked recessive disorder of copper transport, we studied 4 related males, ranging in age from 4-38 years, with a unique phenotype that combines manifestations of classical and mild Menkes disease and occipital horn syndrome (OHS). The propositus, and 18-year-old man, was evaluated following an intracerebral hemorrhage at age 15 years and was noted to have marked hypotonia, motor delay with mental retardation, bladder diverticula, failure to thrive, and diarrhea from infancy; seizures from age 3 years; and abnormal hair (pili torti) and face, cutis laxa, and multiple joint dislocations. Radiographic abnormalities included occipital exostoses, tortuous cerebral blood vessels with multiple branch occlusions, and hammer-shaped clavicles. Biochemical studies demonstrated reduced copper and ceruloplasmin levels in serum, and abnormal plasma catecholamine ratios. We reported previously the molecular defect in this family, a splice-site mutation that predicts formation of approximately 20% of the normal Menkes gene product [Kaler et al., 1994: Nat Genet 18:195-202]. Here, we detail the clinical course and physical features and radiographic findings in these 4 individuals, and compare their phenotype with classical and mild Menkes and OHS. Unusual Menkes disease variants such as this may escape recognition due to anomalies that appear inconsistent with the diagnosis, particularly prolonged survival and later onset of seizures. Males with mental retardation and connective tissue abnormalities should be evaluated for biochemical evidence of defective copper transport.
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PMID:Distinctive Menkes disease variant with occipital horns: delineation of natural history and clinical phenotype. 891 40

The objective of this study was to determine the acute gastrointestinal effects caused by the consumption of drinking water containing graded levels of added copper. Sixty healthy, adult women were randomly assigned to receive copper [Cu(II)] at four concentrations in their drinking water following a Latin-square design. Each group (n = 15) received tap water with no added copper, 1, 3, and 5 mg Cu/l of added copper sulfate for a 2-week study period, followed by 1 week of standard tap water. The subjects recorded their water consumption and gastrointestinal symptoms daily on a special form. The average daily consumption of water was 1.64 liters per subject, regardless of the amount of copper added. Final serum copper, ceruloplasmin, and liver enzymes were measured in all subjects and were not different from baseline concentrations. Twenty-one subjects (35%) recorded gastrointestinal disturbances sometime during the study, 9 had diarrhea, some with abdominal pain and vomiting, and 12 subjects presented abdominal pain, nausea, or vomiting. There was no association between copper levels in drinking water and diarrhea. However, nausea, abdominal pain, or vomiting were significantly related to copper concentrations in water. The recorded incidence rate of these symptoms was 5, 2, 17, and 15% while ingesting water with 0, 1, 3, and 5 mg Cu/l, respectively (overall [chi]2 = 11.3, p<0.01; Cu [less than/equal to]1 mg/l versus Cu [Greater than/equal to]3 mg/l, [chi]2, p<0.01). When subjects interrupted their consumption of drinking water with added copper, most symptoms disappeared. We conclude that under the conditions of the study, there was no association between aggregate copper in drinking water within the range of 0-5 mg/l and diarrhea, but a [Greater than/equal to]3 mg Cu/l level of ionized copper was associated with nausea, abdominal pain, or vomiting. Additional studies with sufficient numbers of subjects are needed to define thresholds for specific gastrointestinal symptoms with precision and to extrapolate these results to the population at large.
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PMID:Acute gastrointestinal effects of graded levels of copper in drinking water. 992 6

Copper can induce acute and chronic intoxications in humans. Copper in tap water has caused a series of severe systemic diseases in Germany in recent years (copper induced liver cirrhosis). Besides cirrhosis, another type of disease with predominantly gastrointestinal symptoms has occurred which likewise appeared to be induced by copper in tap water. - In a retrospective investigation we looked for additional indications and proof that chronic copper poisoning has been the cause of the observed gastrointestinal diseases. All patients suffering from this type of disease had copper plumbing in their houses. - The patients (children and adults) suffered from nausea, vomiting, colic, and diarrhoea. In the group of infants, one refused formula milk (prepared with tap water) and the others suffered from persistent restlessness, unexplainable screaming (especially at night) and/or long lasting diaper rash. - We accept the diagnosis of chronic copper intoxication as the cause of the gastrointestinal symptoms when at least one of the following criteria were fulfilled: 1. first manifestation, remission and relapse of the disease depend on intake and a non-intake of water containing copper, respectively. 2. hypercupric state of the patients (i.e. pathological high concentrations of the non-ceruloplasmin-bound copper in serum and/or elevated copper levels in urine) 3. signs of systemic copper intoxication in the same patient 4. signs of systemic copper intoxication or hypercupric states in members of the patient s family or in his neighbourhood (non-relatives) - We found that the disease can even be caused by copper concentrations below the allowed concentration given by the German Guidelines for Drinking Water (Trinkwasserverordnung). - The data prove that copper in drinking water can cause gastrointestinal diseases and not only the better known systemic diseases (i.e. copper induced liver cirrhosis). Copper poisoning must be considered as a possible cause of chronic gastrointestinal diseases in those countries in which copper plumbing is common.
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PMID:Chronic poisoning by copper in tap water: I. Copper intoxications with predominantly gastointestinal symptoms. 1057 26

The aim of this study was to determine whether total copper or soluble copper concentration is associated with gastrointestinal signs and symptoms. Forty-five healthy adult women (18-55 years of age), living in Santiago, Chile, ingested tap water with 5 mg/L of copper containing different ratios of soluble copper (copper sulfate) and insoluble copper (copper oxide) over a 9-week period. Three randomized sequences of the different copper ratios (0:5, 1:4, 2:3, 3:2, and 5:0 mg/L) were followed. Subjects recorded their water consumption and gastrointestinal symptoms daily on a special form. Mean water consumption was similar among groups. Serum copper levels, ceruloplasmin, and activities of liver enzymes were within normal limits. No differences were detected between the means of biochemical parameters at the beginning and at the end of the study. Twenty subjects presented gastrointestinal disturbances at least once during the study, 9 suffered diarrhea (with or without abdominal pain and vomiting), and the other 11 subjects reported abdominal pain, nausea, or vomiting. No differences were found in incidence of abdominal pain, nausea, vomiting, and diarrhea regardless of the ratio of copper sulfate to copper oxide. In conclusion, both copper sulfate (a soluble compound) and copper oxide (an insoluble compound) have comparable effects on the induction of gastrointestinal manifestations, implying that similar levels of ionic copper were present in the stomach.
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PMID:Gastrointestinal effects associated with soluble and insoluble copper in drinking water. 1167 25

IDA is still the major medico-social problem in pediatric hematology, especially in early childhood. In this correction ferroresistant forms of IDA are interesting. The aim of our investigation was: studying the Efficacy of Tot'hema in the treatment of Iron Deficiency Anemia in Early childhood with concomitant copper deficiency. We observed 42 patients with IDA (age 0,4 - 3 years) in open control investigation. The carried-out investigations revealed that IDA in early childhood is often proceeded by the concomitant copper deficiency and ceruloplasmin, mainly in premature infants and in children with prolonged diarrhea in anamnesis. In such cases it is important to investigate the copper metabolism together with the peripheral blood index and iron metabolism. Tot'hema improves hematologic and biochemical index, completely supplies iron and copper deficiency, prevents of iron resistant form of IDA. Tot'hema has no side effects.
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PMID:[Efficacy of to'thema in the treatment of iron deficiency anemia in early childhood with concomitant copper deficiency]. 1590 30

The duodenal switch (DS) procedure is a type of restrictive-malabsorptive bariatric surgery that is typically reserved for severe morbidly obese people (body mass index >50 kg/m(2)) with obesity-related comorbidities, when diet, lifestyle changes, and pharmacologic therapy fail to achieve adequate weight loss. Patients who undergo the DS procedure are at risk for malabsorption, malnutrition, and nutrient deficiencies. Copper deficiency is a commonly reported long-term complication of Roux-en-Y gastric bypass (RYGB) surgery. However, data are limited on copper deficiency-associated complications and their treatment in DS patients. This article presents a case of a patient who developed hypocupremia with associated pancytopenia, myeloneuropathy, and leukoencephalopathy following DS and reviews the literature related to the pathophysiology of copper deficiency and copper replacement in bariatric surgery patients. When severe diarrhea was present, intravenous elemental copper 4 mg (as cupric chloride)/d in addition to daily oral copper gluconate was necessary to correct the hypocupremia and improve the hematologic indices and neurologic symptoms of copper deficiency. When diarrhea subsided, oral elemental copper 4 mg (as copper gluconate) 3 times daily maintained normal serum copper concentrations and avoided the relapse of severe neurologic dysfunction. Regular monitoring of serum copper and ceruloplasmin concentrations is recommended following DS surgery to detect any copper deficiency before irreversible neurologic damage occurs. Long-term copper supplementation is likely necessary to maintain normal copper status in DS patients.
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PMID:Neurologic dysfunction and pancytopenia secondary to acquired copper deficiency following duodenal switch: case report and review of the literature. 2194 41


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