Síntesis y uso de histidinato de cobre en niños con enfermedad de Menkes en México / Synthesis and use of copper histidinate in children with Menkes disease in Mexico

Resumen La enfermedad de Menkes es una patología neurodegenerativa y letal debida a mutaciones génicas de la enzima ATP-7A trasportadora de cobre; se manifiesta por síntomas neurológicos y alteraciones del tejido conectivo de severidad variable. El uso subcutáneo oportuno de histidinato de cobre (Cu-His) es determinante en la calidad de vida. Se reportan las primeras experiencias en México en la síntesis y uso seguro de Cu-His en tres casos en los que corroboramos hipocupremia e hipoceruloplasminemia. Bajo asesoramiento del Hospital for Sick Children, Toronto, Canadá, elaboramos una solución de 500 µg/mL. En los tres casos aplicamos 250 µg de Cu-His, sin efectos indeseables relevantes durante 30 días y observamos las siguientes determinaciones séricas de cobre (Cu en µg/L) y ceruloplasmina (Cp en mg/dL): caso 1, Cu días 0 y 30, 8 y 504 µg/L; Cp días 0 y 30, 4 y 10.75 mg/dL; caso 2, Cu días 0 y 30, < 50 y 502, µg/L; Cp días 0 y 30, 2 y 15 mg/dL; caso 3, Cu días 0 y 30, 3 y 84.2 µg/L; Cp días 0 y 30, 4 y 10.7 mg/dL. En México es posible la síntesis segura de Cu-His y tratar la enfermedad de Menkes, la cual debe ser intencionalmente buscada.

Abstract Menkes disease is a neurodegenerative and lethal pathology caused by gene mutations of the copper-transporting ATP-7A enzyme; it manifests itself by neurological symptoms and connective tissue changes of varying severity. Timely subcutaneous use of copper histidinate (Cu-His) is determinant for quality of life. We report the first experiences in Mexico on Cu-His synthesis and its safe use in 3 cases where hypocupremia and hypoceruloplasminemia were corroborated. With advice of the Hospital for Sick Children of Toronto Canada, we prepared a 500 µg/mL solution. In all three cases were 250 µg of Cu-His applied without relevant undesirable effects for 30 days. Serum copper (Cu, expressed in µg/L) and ceruloplasmin (Cp, in mg/dL) were determined: case 1, Cu days 0 and 30, 8 and 504 µg/L; Cp days 0 and 30, 4 and 10.75 mg/dL; case 2, Cu days 0 and 30, <50 and 502 µg/L; Cp days 0 and 30, 2 and 15 mg/dL; case 3, Cu days 0 and 30, 3 and 84.2 µg/L; Cp days 0 and 30, 4 and 10.7 mg/dL. In Mexico, it is possible to safely synthesize Cu-His and treat MD, which must be intentionally sought.

Toxic metals in maternal blood, cord blood and meconium of newborn infants in Pakistan

Toxic metals and deficiency/excess of trace elements can have adverse effects on health. The aim of this study was to quantify toxic metals lead, cadmium and trace elements zinc, copper, aluminium [Al] and Iron [Fe] levels in pregnant women, cord blood and meconium of new-born infants from industrial zones of Karachi, Pakistan. Analytical research was performed from 2011–2012 in low socio-economic pregnant mothers and newborn infants from 20 towns near Sindh Industrial Trading Estates, Federal B industrial area and Korangi industrial areas, Karachi, where environmental pollution was anticipated. Blood samples of pregnant women [n = 416], cord blood [n = 309] and meconium [n = 309] were analyzed quantitatively for metals and trace elements. Results indicated that mothers residing in steel towns were found to have the highest levels of lead. Meconium contained high levels of toxic heavy metals and trace elements compared to cord blood and maternal blood. Maternal blood toxic metals were present in high quantities. Therefore, safety measures should be taken when industrial waste is disposed of in order to prevent population contamination

Erythrocyte zinc and serum copper in male and female adolescents according to puberty stage at different growth phases

BACKGROUND: Considering that modification in body composition and growth velocity leads to alterations in zinc and copper nutritional status, the purpose of the study was to assess erythrocyte zinc and serum copper in adolescents of both genders, according to the stage of sexual maturity at different growth phases. METHODS: Two hundred and eighty-six adolescents were assessed, 157 were female, with body mass index Z-scorebetween−2 and +2. Clinical biochemical measurements were assessed using atomic absorption spectrophotometry. Student's t, Kruskal-Wallis, and Bonferroni tests were used. RESULTS Median serum copper was superior, and zinc was inferior to respective reference values in all groups. Serum copper presented significant reduction in the final stages of pubertal development in males; in turn, erythrocyte zinc values increased linearly with the evolution of puberty stages in both genders. Comparison between genders in each stage of growth showed significant difference in peak acceleration and in deceleration for erythrocyte zinc and serum copper, with both being higher in females. CONCLUSIONS: Serum copper is inversely influenced by modifications that occur with the evolution of puberty in males, whereas erythrocyte zinc is directly influenced in both genders, although always with deficient deposition. At peak acceleration and deceleration of growth, there is greater mobilization of erythrocyte zinc and serum copper in males, when compared with females, which does not occur at the onset of the acceleration of growth.

Copper-Zinc ratio and nutritional status in colorectal cancer patients during the perioperative period

PURPOSE : This study aimed to determine Cu/Zn ratio, nutritional and inflammatory status in patients during the perioperative period for colorectal cancer. METHODS: The study included patients with histological diagnosis of colorectal adenocarcinoma (Cancer Group, n=46) and healthy volunteers (Control Group, n=28). We determined habitual food intake, body composition, laboratory data of nutritional status, serum calprotectin and plasma Cu and Zn concentrations. Mann-Whitney U-test was performed between-group comparisons and Spearman correlation test for correlations between the variables. RESULTS: Individuals in the Cancer Group presented significantly lower BMI, fat mass, plasma hemoglobin, total protein and albumin as compared with the Control Group. Serum calprotectin[70.1 ng/mL (CI95% 55.8-84.5) vs.53.3 ng/mL (40.3-66.4), p=0.05], plasma Cu concentrations [120 µg/dL(CI95% 114-126) vs. 106 µg/dL(CI95% 98-114), p<0.01] and the Cu/Zn ratio [1.59 (CI95% 1.48-1.71)vs. 1.35 (CI95% 1.23-1.46), p=0.01]were higher in patients with colorectal cancer than in controls. Additionally, the Cancer Group showed negative correlations between the Cu/Zn ratio and Zn intake, hemoglobin, serum albumin, and positive correlation between the Cu/Zn ratio and serum calprotectin. CONCLUSION: These results indicate that an increased plasma Cu/Zn ratio and serum calprotectin, and decreased protein values may be a result of the systemic inflammatory response to the tumor process.

Assessment of serum copper, iron and immune complexes in potentially malignant disorders and oral cancer

Abstract Potentially malignant disorders (PMDs) of oral cavity and oral cancer remain a cause of serious concern despite intensive research and development. Diet and immunity have been identified to play a crucial role as modifying factors in these diseases. Our study intended to explore this relationship by estimating and comparing the serum levels of copper, iron and circulating immune complexes (CICs) in patients diagnosed with PMDs and oral cancer and normal healthy individuals. In this study, 40 histopathologically diagnosed cases of PMDs and oral cancer were included along with 30 healthy controls and 5 ml of venous blood was drawn using venipuncture. Serum estimation of copper, iron and CIC then followed using the colorimetric and spectrophotometric methods. The data obtained was subjected to statistical analysis using one way ANOVA and Pearson's Product-Moment Correlation Test. The mean serum copper level was measured as 138.98 ± 10.13µg/100ml in the PMD group and 141.99 ± 21.44 µg/100ml in the oral cancer as compared to 105.5 + 18.81µ/100ml in the controls. The mean serum CIC levels was highest in the oral cancer (9.65 ± 0.16OD470) followed by the PMD group (0.18 + 0.21 OD470) and least in the control group (0.048 ± 0.02OD470). Whereas, the serum levels of iron showed a significant decrease in the PMD group (110.9 ± 10.54 µg/100ml) and the oral cancer group (114.29 ± 25.83 µg/100ml) as compared with the control group (136.85 ± 14.48 µg/100ml). There was no positive correlation obtained between the three groups with respect to the chosen parameters indicating that the variables were independent of each other. It can be thus be ascertained that trace elements like copper and iron as well as humoral responses (CICs) have a close relationship with PMDs and oral cancers.

Serum levels of copper and iron in dengue fever / Níveis séricos de cobre e ferro na dengue

SUMMARYThe role of trace elements in dengue virulence is not yet known. The present study assessed the serum levels of two micronutrients, copper and iron, in cases of dengue fever. The study involved 96 patients of whom 48 had either severe or non-severe forms of dengue (with and without warning signs), and the remaining 48 were patients with other febrile illnesses (OFI), used as controls. Serum levels of copper and iron were evaluated at admission and by the time of defervescence using commercially available kits. At admission, no difference in the level of serum copper was observed between cases and controls. In the group of dengue cases, the copper level was found to be significantly decreased in severe and non-severe cases with warning signs, compared to non-severe cases without warning signs. In contrast, by the time of defervescence the copper level was found to be increased in all dengue cases compared to OFI controls, but no difference was observed among dengue cases. Unlike OFI controls, dengue cases showed an increasing pattern of copper levels from admission until defervescence. On the other hand, no such significant differences were observed in the serum level of iron in the clinical groups, except for a decreased iron level found in severe cases, compared to non-severe dengue without warning signs. The results show that copper is associated with dengue severity and this finding emphasizes the need to investigate the involvement of trace elements in disease severity so as to improve the prognosis of dengue.

RESUMOO papel dos elementos-traço na virulência da dengue não é ainda conhecido. O presente estudo avaliou os níveis séricos de dois micronutrientes, cobre e ferro, em casos de dengue. O estudo envolveu 96 pacientes dos quais 48 apresentavam dengue grave ou não grave (com ou sem sinais de alerta), e outros 48 pacientes com outras doenças febris (OFI) representaram os controles. Níveis séricos de cobre e ferro foram avaliados na admissão e no momento da defervescência usando kits comerciais disponíveis. À admissão, nenhuma diferença nos níveis séricos de cobre foi observada entre casos e controles. No grupo com dengue, os níveis de cobre se encontravam significativamente reduzidos nos casos graves e não graves com sinais de alerta, em comparação aos casos não graves sem sinais de alerta. Contrariamente, no momento da defervescência os níveis de cobre se encontravam aumentados em todos os casos de dengue em relação aos controles com outras doenças febris (OFI), no entanto, nenhuma diferença foi observada entre os casos de dengue. Diferentemente dos pacientes com outras doenças febris, os casos de dengue mostraram um padrão de elevação dos níveis de cobre do dia da admissão até a defervescência. Por outro lado, estas diferenças não foram observadas em relação aos níveis de ferro entre os dois grupos, com exceção de níveis de ferro reduzidos encontrados nos casos graves, em comparação aos não graves com sinais de alerta. Os resultados mostram que o cobre está associado à gravidade da dengue e esta observação enfatiza a necessidade de investigação do envolvimento de elementostraço na gravidade da doença para melhorar o prognóstico da dengue.

Effects of various anesthesia maintenance on serum levels of selenium, copper, zinc, iron and antioxidant capacity / Efeitos da manutenção de várias anestesias sobre os níveis séricos de selênio, cobre, zinco e ferro e a capacidade antioxidante / Efectos del mantenimiento de varias anestesias sobre los niveles séricos de selenio, cobre, cinc y hierro y la capacidad antioxidante

BACKGROUND AND OBJECTIVES: In this study, we aimed to investigate the effects of sevoflurane, desflurane and propofol maintenances on serum levels of selenium, copper, zinc, iron, malondialdehyde, and glutathion peroxidase measurements, and antioxidant capacity. METHODS: 60 patients scheduled for unilateral lower extremity surgery which would be performed with tourniquet under general anesthesia were divided into three groups. Blood samples were collected to determine the baseline serum levels of selenium, copper, zinc, iron, malondialdehyde and glutathion peroxidase. Anesthesia was induced using 2-2.5 mg kg-1 propofol, 1 mg kg-1 lidocaine and 0.6 mg kg-1 rocuronium. In the maintenance of anesthesia, under carrier gas of 50:50% O2:N2O 4 L min-1, 1 MAC sevoflorane was administered to Group S and 1 MAC desflurane to Group D; and under carrier gas of 50:50% O2:air 4 L min-1 6 mg kg h-1 propofol and 1 µg kg h-1 fentanyl infusion were administered to Group P. At postoperative blood specimens were collected again. RESULTS: It was observed that only in Group S and P, levels of MDA decreased at postoperative 48th hour; levels of glutathion peroxidase increased in comparison to the baseline values. Selenium levels decreased in Group S and Group P, zinc levels decreased in Group P, and iron levels decreased in all three groups, and copper levels did not change in any groups in the postoperative period. CONCLUSION: According to the markers of malondialdehyde and glutathion peroxidase, it was concluded that maintenance of general anesthesia using propofol and sevoflurane activated the antioxidant system against oxidative stress and using desflurane had no effects on oxidative stress and antioxidant system. .

JUSTIFICATIVA E OBJETIVOS: Investigar os efeitos da manutenção de sevoflurano, desflurano e propofol sobre nos níveis séricos de selênio, cobre, zinco, ferro e malondialdeído, as mensurações de glutationa peroxidase e a capacidade antioxidante. MÉTODOS: Foram alocados em três grupos 60 pacientes agendados para cirurgia unilateral de membros inferiores, feita com torniquete sob anestesia geral. Amostras de sangue foram coletadas para determinar os níveis séricos basais de selênio, cobre, zinco, ferro, malondialdeído e glutationa peroxidase. A anestesia foi induzida com 2-2,5 mg kg-1 de propofol, 1 mg kg-1 de lidocaína e 0,6 mg kg-1 de rocurônio. Na manutenção da anestesia, sob gás de transporte de 50% O2 e 50% N2O (4 L min-1), sevoflurano a 1 CAM foi administrado ao Grupo S e desflurano a 1 CAM ao Grupo D e, sob gás de transporte em mistura de 50% O2 e 50% ar (4 L min-1), 6 mg kg h-1 de propofol e 1 mg kg h-1 de fentanil foram administrados ao Grupo P. No pós-operatório, amostras de sangue foram novamente coletadas. RESULTADOS: Apenas nos grupos S e P os níveis de MDA diminuíram em 48 horas de pós-operatório; os níveis de glutationa peroxidase aumentaram em comparação com os valores basais. Os níveis de selênio diminuíram no Grupo S e no Grupo P, os níveis de zinco diminuíram no Grupo P, os níveis de ferro diminuíram em todos os grupos e não houve alteração nos níveis de cobre em nenhum grupo no período pós-operatório. CONCLUSÃO: De acordo com os marcadores de malondialdeído e glutationa peroxidase, concluímos que a manutenção da anestesia geral com propofol e sevoflurano ativou o sistema antioxidante contra o estresse oxidativo e o uso de desflurano não teve efeitos sobre o estresse oxidativo e o sistema antioxidante. .

JUSTIFICACIÓN Y OBJETIVOS: Investigar los efectos del mantenimiento de sevoflurano, desflurano y propofol sobre los niveles séricos de selenio, cobre, cinc, hierro y malondialdehído, las medidas de glutatión peroxidasa y la capacidad antioxidante. MÉTODOS: Fueron ubicados en 3 grupos 60 pacientes programados para cirugía unilateral de miembros inferiores, realizada con torniquete bajo anestesia general. Fueron recogidas muestras de sangre para determinar los niveles séricos basales de selenio, cobre, cinc, hierro, malondialdehído y glutatión peroxidasa. La anestesia fue inducida con 2-2,5 mg/kg-1 de propofol, 1 mg/kg-1 de lidocaína y 0,6 mg/kg-1 de rocuronio. En el mantenimiento de la anestesia, bajo gas portador de 50% de O2 y 50% de N2O (4 L/min-1), sevoflurano a 1 CAM fue administrado al grupo S; y desflurano a 1 CAM al grupo D y bajo gas portador en mezcla de 50% O2 y 50% aire (4 L/min-1), 6 mg/kg/h-1 de propofol y 1 µg/kg/h-1 de fentanilo fueron administrados al grupo P. En el postoperatorio se recogieron de nuevo muestras de sangre. RESULTADOS: Solamente en los grupos S y P los niveles de malondialdehído disminuyeron en las 48 h del postoperatorio; los niveles de glutatión peroxidasa aumentaron en comparación con los valores basales. Los niveles de selenio disminuyeron en el grupo S y en el grupo P, los niveles de cinc disminuyeron en el grupo P, los de hierro disminuyeron en todos los grupos y no hubo alteración en los niveles de cobre en ningún grupo en el período postoperatorio. CONCLUSIÓN: De acuerdo con los marcadores de malondialdehído y glutatión peroxidasa, llegamos a la conclusión de que el mantenimiento de la anestesia general con propofol y sevoflurano activó el sistema antioxidante contra el estrés oxidativo y el uso de desflurano no tuvo efectos sobre el estrés oxidativo y el sistema antioxidante. .

Study of Serum Sialic Acid and Copper as Inflammatory Markers in Type 2 Diabetes Mellitus.

The study aims to understand the relationship between increased serum levels of acute- phase reactants like Sialic Acid (SA) and transitional metals like Copper (Cu) which are indicated to be associated with the etiology and pathogenesis of type 2 diabetes mellitus (DM) and its various chronic complications. Serum SA and Cu levels, measured colorimetrically using Ehrilch’s reagent and Atomic Absorption Spectrophotometer (AAS) respectively among 30 cases each of type 2 DM with and without long-term complications, were significantly higher in all the cases compared to controls. The values are even higher in cases with complications, compared to diabetics without complications. Serum lipid profile was significantly altered in both the experimental groups. Serum SA and Cu levels are positively correlated to duration and degree of impaired glycemic status and altered lipid profile in type 2 DM. Measurement of these new serum markers can explain the inflammatory process, implicated towards the development of diabetic complications like cardiovascular diseases and microangiopathies thereby helping in early medical intervention.

Serum copper and ceruloplasmin levels in systemic sclerosis and various types of morphea in the Kashmir valley

To find the serum copper and ceruloplasmin levels in patients of systemic sclerosis and morphoea in the Kashmir Valley. This was a cross sectional study conducted in the department of dermatology of SKIMS Medical College over a period of one year involving 12 patients of systemic sclerosis and morphea and equal number of healthy controls. All the individuals were taken up for serum copper and ceruloplasmin levels. In patients with systemic sclerosis, the mean levels of copper and ceruloplasmin were increased. No alterations were found in patients with morphea and healthy controls. The increase in serum copper is probably secondary to increase in ceruloplasmin level, which occurs as a nonspecific response to inflammation

Assessment of antioxidants status and superoxide dismutase activity in HIV-infected children

Objective: This study aims to assess the nutritional status of selenium, copper and zinc; and also the erythrocyte superoxide dismutase activity of HIV-infected children compared to a control group. Methods: A cross-sectional study was carried out with prepubertal HIV-infected children (n = 51) and their healthy siblings (n = 32). All biochemical measurements including plasma selenium, serum copper levels, serum and erythrocyte zinc levels and erythrocyte super-oxide dismutase activity were evaluated according to dietary, clinical and biochemical parameters. Results: Compared to the control group, the HIV-infected children had lower z-score values for height-for-age (p = 0.0006), higher prevalence of stunting (11.8%) (p = 0.047), lower selenium levels (p = 0.0006) and higher copper levels (p = 0.019). No difference was found concerning superoxide dismutase activity (p > 0.05). The HIV-infected group presented a higher proportion (45.1%) of children with zinc intakes below the estimated average requirement (p = 0.014); however, no association with zinc biochemical parameters was found. Conclusion: HIV-infected children have an inadequate selenium and copper nutritional status, which could influence the progression to AIDS. An adequate micronutrient status could improve the clinical conditions in these patients and minimize free radical production and cellular oxidative stress. .

Association of Parkinson's disease with altered serum levels of lead and transition metals among South Indian subjects.

Several epidemiologic studies have suggested an association between the Parkinson’s disease (PD) and exposure to heavy metals, such as lead, iron, copper, manganese, etc. A growing body of evidence suggests that heavy metals stimulate free radical formation in the brain and can lead to neurodegeneration. In the present study, we investigated whether such association exists in PD cases from rural and urban areas in our study population. The plasma levels of copper, iron, manganese and lead in PD cases (n = 150) and controls (n = 170) were determined by inductively coupled plasma mass spectrometry (ICP-MS) and correlated with the oxidative stress markers like malondialdehyde (MDA), protein carbonyl and total glutathione. Results indicated significant increase in the levels of copper (17.73 ± 4.48 vs. 13.0 ± 3.22 ng/ml) and iron (554.4 ± 123.8 vs. 421.7 ± 126.1 ng/ml) in PD cases compared to controls, whereas no significant differences in the levels of manganese and lead were observed. Further, the data based on urban or rural residence showed that plasma copper, iron, manganese levels were comparatively higher in rural subjects, whereas plasma lead levels were significantly higher in urban subjects. Increased plasma iron showed positive correlation with marker of lipid peroxidation (MDA), suggesting that increased iron levels induced oxidative stress in PD. These results substantiated the earlier observations about the role of environmental exposure and metal-induced oxidative stress in the etiology of PD.

Serum zinc, copper, selenium, and lead levels in children with chronic renal failure

In the last two centuries, chronic kidney disease [CKD] and renal failure [RF] have been considered as the main medical problems which were fatal in many cases. Trace elements such as selenium, copper, and zinc are the components of biological enzymes which have a crucial role in decreasing reactive oxygen. The levels of these trace elements reduce in CKD patients. Close and careful nutritional support of children with CKD, particularly in the early stages of the disease, is necessary

Co-administration of trientine and flaxseed oil on oxidative stress, serum lipids and heart structure in diabetic rats.

The administration of flaxseed oil or flaxseed oil plus trientine in diabetic rats reduced triglyceride, very low density lipoprotein, and total cholesterol. Furthermore, the combined treatment significantly increased superoxide dismutase activity and attenuated serum Cu2+. The results suggest that the administration of flaxseed oil plus trientine is useful in controlling serum lipid abnormalities, oxidative stress, restoring heart structure, and reducing serum Cu2+ in diabetic rats.

Trace mineral status related to levels of glycated hemoglobin of type 2 diabetic subjects in Jeddah. Saudi Arabia

Diabetes mellitus [DM] is of major and increasing global public health importance. People with diabetes are at increased risk of premature disability and death associated with vascular, renal, retinal, and neuropathic complications. Direct association of trace elements in both type 1 and type 2 diabetes has been observed in many research studies. An alteration in the metabolism of these minerals has been demon- strated in diabetes. The aim of the present study was to investigate zinc [Zn], copper [Cu], chromium [Cr], manganese [Mn] and magnesium [Mg] levels in the serum of patients with type 2 diabetes and age-matched healthy subjects and also to assess the association between these elements and glycated hemoglobin [HbA1c]. The study population consisted of 55 type 2 diabetic patients and 55 age-matched non-diabetic healthy subjects within the age range of 45-65 years. In this study, we found significantly higher Cu levels [P < 0.001], lower Zn levels [P < 0.05], and also lower Cr, Mn, Mg levels [P < 0.05] in patients with DM in comparison with healthy subjects. Statistical analysis showed a positive correlation between serum levels of Cu and Zn in the group of healthy subjects [r = 0.97, P < 0.0001]. In contrast, we found a negative correlation between these metals [r = -0.59, P < 0.0001] in patients with DM. We also found significant higher levels of HbA1c [P< 0.001] in the DM group than in the healthy group. Positive correlations between levels of HbA1c and Cu [r= 0.71, P < 0.001] as well as Cr [r = 0.54, P < 0.0001] and negative correlations between levels of HbA1c and Zn [r = -0.65, P < 0.001] and also Mg [r = -.52, P < 0.001] in the DM group were obtained. Patients with DM had altered metabolism of Zn, Cu, Cr, Mn, and Mg, and this may be related to increased values of glycated hemoglobin. We concluded that imbalance in the levels of studied metals may play an important role in the pathogenesis of DM

Analysis of clinical and biochemical spectrum of Wilson Disease patients.

Background and Aims: Wilson disease (WD) is autosomal recessive disorder of copper metabolism. Wilson disease patients usually suffer from hepatic or neuropsychiatric complications. The symptoms appear between ages five to 35 but it can vary from two years to 72 years. Materials and Methods : Study was carried out from June 2008 to November 2010. This study included nine families with eleven cases of WD to determine clinical presentation, diagnostic findings (including laboratory results) and liver histology. It included 11 patients who presented with hepatic manifestations and/or Neuropsychiatric manifestations and/or family history suggesting features of WD. Patients with hepatitis B and C and those with history of taking antipsychotic drugs were excluded from the study. Patient's data was included in a well designed performa. Liver function test, serum ceruloplasmin, serum copper, 24 hour urinary copper, blood complete picture were analyzed. Quantitative data such as age, hemoglobin etc were expressed as mean with ± SD and quantitative variables such as sex, movement disorders, hepatic involvement etc were expressed as frequency and percentage. Results: There were five male and six female patients with evidence of various manifestations here (i) hepatic in which they had only liver dysfunction (ii) hepatic and neurological (iii) neurological. The mean age of presentation was 8.7±3.92 years (range 4-19 years) and 45% were male patients. Decreased serum ceruloplasmin, enhanced 24-h urinary copper excretion and signs of chronic liver damage were confirmed in all patients and Kayser-Fleischer rings (KF rings) in 72% of patients. In severe WD patients, serum prothrombin activity was less than 50%, serum ceruloplasmin were low and serum copper levels were high than those in non-severe WD patients. High degree of suspicion leads to early treatment with good outcome. Conclusions: The WD is rare but important cause of chronic liver disease. Clinical and biochemical analysis in cases of patients with unexplained liver disease with high degree of suspicion can lead to early treatment with good outcome.

Iron, zinc, copper and magnesium nutritional status in Mexican children aged 1 to 11 years / Estado nutricio de hierro, zinc, cobre y magnesio en niños mexicanos de 1 a 11 años de edad

OBJECTIVE: To describe the micronutrient nutritional status of a national sample of 1-11 year old Mexican children surveyed in 2006 in National Health and Nutrition Survey (ENSANUT 2006) and their association with dietary and sociodemographic factors. MATERIALS AND METHODS: Serum samples were used (n=5 060) to measure the concentrations of ferritin, transferrin receptor, zinc, copper and magnesium. RESULTS: Prevalence of deficiencies in 1-4 and 5-11y old children were for iron (using low ferritin) 26.0 and 13.0%; zinc, 28.1 and 25.8%, respectively; and copper, ≈30% in both age groups. Magnesium low serum concentrations (MLSC), were found in 12.0% and 28.4% of the children, respectively. Being beneficiary of Liconsa (OR=0.32; C.I.95%, 0.17-0.61) or belonging to higher socioeconomic status (OR=0.63; C.I.95%, 0.41-0.97) were protective against iron deficiency. Increasing age (OR=0.59; C.I.95%, 1.19-1.32) and living in the Central Region (OR=0.59; C.I.95%, 0.36-0.97) were protective against MLSC. CONCLUSIONS: Deficiencies of iron and zinc are serious public health problems in Mexican children.

OBJETIVO: Describir el estado nutricio de micronutrimentos en niños de 1-11 años de edad de la Encuesta Nacional de Salud y Nutrición 2006 y su asociación con factores dietéticos y sociodemográficos. MATERIAL Y MÉTODOS: Se usaron muestras séricas (n=5060) para medir las concentraciones de ferritina, receptor de transferrina, zinc, cobre y magnesio. RESULTADOS: La prevalencias de deficiencias en niños de 1-4 y de 5 a 11 años fueron para ferritina, 26.0 y 13%; zinc, 28.1 y 25.8% respectivamente y cobre ≈30% en ambos grupos. Las concentraciones bajas de magnesio (CBM) fueron 12.0 y 28.4%, respectivamente. Ser beneficiario de Liconsa (RM=0.32; IC 95%: 0.17-0.61) y pertenecer al nivel socioeconómico alto (RM=0.63; IC, 95%: 0.41-0.97) fueron protectores para deficiencia de hierro. La edad (RM=1.26; IC, 95%: 1.19-1.32) y vivir en la región Centro (RM=0.59; IC, 95%: 0.36-0.97) fueron protectores para CBM. CONCLUSIONES: Las deficiencias de hierro y zinc son serios problemas de salud pública en niños mexicanos.

Atomic absorption spectrometry in Wilson's disease and its comparison with other laboratory tests and paraclinical findings

Wilson's disease [WD] is an autosomal recessive disease with genetic abnormality on chromosome 13 causing defect in copper metabolism and increased copper concentration in liver, central nervous system and other organs, which causes different clinical manifestations. The aim of this study was to determine the sensitivity of different clinical and paraclinical tests for diagnosis of Wilson's disease. Paraffin blocks of liver biopsy from 41 children suspicious of WD were collected. Hepatic copper concentrations were examined with atomic absorption spectrophotometry [Australian GBC, model: PAL 3000]. Fifteen specimens had hepatic copper concentration [dry weight] more than 250 micro g/g. Clinical and laboratory data and histologic slides of liver biopsies of these 15 children were reviewed retrospectively. Liver tissue was examined for staging and grading of hepatic involvement and also stained with rubeonic acid method for copper. Patients were 5-15 years old [mean age=9.3 years, standard deviation=2.6] with slight male predominance [9/15=60%]. Five [33%] patients were 10 years old. Three [20%] of them were referred for icterus, 8 [54%] because of positive family history, 2 [13%] due to abdominal pain and 2 [13%] because of hepatosplenomegaly and ascites. Serum AST and ALT levels were elevated at the time of presentation in all patients. In liver biopsy, histological grade and stage was 0-8 and 0-6 respectively, 2 [13%] had cirrhosis, 1 [7%] had normal biopsy and 12 [80%] showed chronic hepatitis. Hepatic copper concentrations were between 250 and 1595 micro g/g dry weight. The sensitivity of various tests were 85% for serum copper, 83% for serum ceruloplasmin, 53% for urinary copper excretion, 44% for presence of KF ring and 40% for rubeonic acid staining on liver biopsies. None of the tests stated in the article were highly sensitive for diagnosis of WD, so we suggest that diagnosis should be based on combination of family history, physical examination and different tests

Effect of selective serotonin re-uptake inhibitor [sertraline] on some reproductive aspects of mature male rats and plasma trace elements concentration

Administration of selective serotonin reuptake inhibitors [SSRI] interferes with male reproduction and trace element concentrations. The mechanism of action of antiserotonin [sertraline] is presumed to be linked to its ability to inhibit the neuronal reuptake of serotonin. The aim of the present study is to evaluate the effects of sertraline on reproductive system of mature male rats and on some plasma trace element concentrations. Twenty mature male rats were divided into 2 equal groups [10 rats/group], one group was administrated with sertraline [10mg/kg.b.w.]via stomach tube for 70 days while the other group administrated 1ml saline and considered as control groups. Results showed that seminal vesicles were significantly [P<0.05] decreased in weight. Also, sertraline resulted in significant [P<0.05] decrease of sperm motility [30%] compared to [70%] in control group. Histological sections prepared from sertraline treated rats showed degenerative and necrotic changes with few spermatocytic giant cells. Seminal vesicles were activated in sertraline treated rats and increased eosinophilic secretions. Plasma concentration of testosterone was significantly [P<0.05] decreased in sertraline treated rats as compared to its level in control rats. Moreover plasma concentration of iron in sertraline treated rats showed significant [P<0.05] decrease compared to control group, while plasma concentration of copper in sertraline treated rats showed significant [P<0.05] increase, but was concluded that serotonin inhibitor adversely affect the reproductive aspects of male rats and induced alteration in some plasma trace element concentration

relationship between serum lipid profile and selected trace elements for adult men in Mosul city

To evaluate the correlations of the serum concentrations of copper, zinc, and manganese with lipid profile parameters of adult men in Mosul City, Iraq. The study included 51 apparently healthy adult men as a control group aged 34-62 years [group 1], and 31 hyperlipidemic patients aged 37-60 years [group 2]. Trace elements copper, zinc and manganese were determined using atomic absorption spectrometry. Concentrations of total cholesterol, triglyceride and high density lipoprotein cholesterol were determined using enzymatic method. Indirect serum concentration of low-density lipoprotein cholesterol were calculated via the Friedewald formula. Data were evaluated as mean and standard deviation by analysis of variance [ANOVA] and t-test. The results indicated that there is a significant lower level of serum zinc in hyperlipidemic patients compared with the control group, while copper and manganese showed no significant differences between the two groups. A significant negative correlation was found between serum zinc and total cholesterol, low-density lipoprotein cholesterol, triglyceride and low/highdensity lipoprotein cholesterol ratio; while a significant positive correlation was found between serum zinc and high density lipoprotein cholesterol. In addition, a significant positive correlation between copper and triglyceride existed in the patient group, while the control group showed no such correlation. Hyperlipidemia may possibly be related to a decrease in the level of serum zinc in hyperlipidemic adult men. The data also supports the concept that zinc supplementation might be useful in improving metabolic complications in subjects with hyperlipidemia

Histopathological and biochemical findings of congenital copper deficiency: are these similar to those of caprine arthritis-encephalitis?

This study was done after identifying animals with a twisted carpal joint in goat herd. These included a kid goat walking on its articulus carpii and a newborn goat with a stiff leg. Necropsies of the diseased goats revealed swollen carpal joints that were twisted backwards. Arthritis was observed during microscopic examination of the carpal joints. Very low levels of eosinophil, leucocyte, and lymphocyte cell infiltration were found in the central nervous system and meninges. Serum copper levels were significantly decreased in most of the animals. All of these results led us to diagnose the animals with swayback disease.