O presente trabalho teve como objetivo estudar os efeitos que as fontes orgânicas e inorgânicas de cobre e enxofre possuem na interação cobre-enxofre-molibdênio, estimando a biodisponibilidade de duas fontes de cobre na dieta de ovinos. Para isso, foram utilizados 40 ovinos desmamados, com aproximadamente 3 meses e peso médio 20 kg, distribuídos em 10 tratamentos, sendo: 1) dieta basal; 2) dieta basal contendo 10 mg de molibdênio/kg de MS; 3) dieta basal + 10 mg cobre inorgânico/kg de MS + 0,2% enxofre inorgânico/kg de MS; 4) dieta basal + 10 mg cobre inorgânico/kg de MS + 0,2% enxofre orgânico/kg de MS; 5) dieta basal + 10 mg cobre orgânico/kg de MS + 0,2% enxofre inorgânico/kg de MS; 6) dieta basal + 10 mg cobre orgânico/kg de MS + 0,2% enxofre orgânico/kg de MS; 7) dieta com 10 mg molibdênio + 10 mg cobre inorgânico/kg de MS + 0,2% enxofre inorgânico/kg de MS; 8) dieta com 10 mg molibdênio + 10 mg cobre inorgânico/kg de MS + 0,2% enxofre orgânico/kg de MS; 9) dieta com 10 mg molibdênio + 10 mg cobre orgânico/kg de MS + 0,2% enxofre inorgânico/kg de MS; 10) dieta com 10 mg molibdênio + 10 mg cobre orgânico/kg de MS + 0,2% enxofre orgânico/kg de MS. O experimento teve duração total de 84 dias, sendo realizadas pesagens dos animais nos dias 1, 28, 56 e 84 dias para acompanhamento do desenvolvimento. Foram realizadas também coletas sanguíneas para estudo de teores de cobre, enxofre e molibdênio sanguíneos, teores de glicose, albumina, ureia, colesterol, triglicerídeos, hematócrito e ceruloplasmina. Foram realizadas biópsias do fígado no tempo zero para análises de cobre, enxofre e molibdênio. Ao término do período experimental os animais foram abatidos e colheu-se amostras de fígado e líquido biliar para determinação final dos teores de minerais, bem como acompanhamento do pH e peso das carcaças quente e fria. No terço final do período experimental foi realizado um balanço metabólico para cobre, enxofre e molibdênio, sendo a biodisponibilidade do cobre calculada pela técnica \"slope ratio\", utilizando-se os teores de cobre hepático. Os parâmetros foram analisados considerando-se a existência de uma estrutura de tratamento fatorial 2 x 2 x 2 (com e sem molibdênio, cobre orgânico e inorgânico e enxofre orgânico e inorgânico) e uma dieta basal e uma basal mais molibdênio, em delineamento inteiramente casualizado. As concentrações de glicose, albumina, ureia, colesterol e hematócrito sanguíneos não sofreram efeito (P>0,05) pela adição de molibdênio ou adição de cobre e enxofre, ou pela interação cobre-enxofre-molibdênio. Entretanto as concentrações de triglicerídeos sanguíneos foram alteradas (P<0,05) pela interação cobre-enxofre, mostrando redução na sua concentração quando da adição de cobre e enxofre nas fontes orgânicas. O peso vivo dos ovinos não foi influenciado (P>0,05) pelos tratamentos, porém o ganho de peso foi influenciado pela interação cobre-enxofre-molibdênio onde se observou redução no ganho de peso com a adição de molibdênio exclusivo e aumento no ganho de peso quando adicionado molibdênio com cobre e enxofre nas fontes orgânicas quando comparados com as fontes inorgânicas. A suplementação de fontes de cobre e enxofre e adição de molibdênio não influenciaram (P>0,05) os valores de pH e peso das carcaças. A presença de molibdênio na dieta diminuiu os teores de ceruloplasmina e a presença e molibdênio exclusivo reduziu as concentrações de cobre no soro dos ovinos, bem como a interação cobre-enxofre-molibdênio com as fontes inorgânicas dos minerais reduziu a ceruloplasmina e cobre no soro. Em contrapartida, a ceruloplasmina mostrou aumento nos níveis quando da administração de enxofre orgânico. Os teores de enxofre sanguíneos não foram influenciados pelos tratamentos. No líquido biliar contatou-se efeito do molibdênio exclusivo sobre os minerais, mostrando redução nas concentrações de cobre e enxofre e aumento na concentração de molibdênio. Os valores hepáticos de cobre e enxofre não foram influenciados (P>0,05) pelos tratamentos, somente o molibdênio hepático mostrou efeito na interação cobre-enxofre-molibdênio. A biodisponibilidade do cobre proteinado (145,09%) foi superior à do sulfato de cobre (100%), independente da fonte de enxofre e na ausência e molibdênio, quando determinado pela regressão múltipla \"slope ratio\". / This research aim to study the effect of organic and inorganic copper and sulfur sources in the interaction copper-sulfur-molybdenum, estimating the bioavailability of two sources of copper in the diet of sheep. For that used 40 weaned sheep at about 3 months of age and weighing 20 kg and distributed in 10 treatments, as follows: 1) basal diet; 2) diet containing molybdenum; 3) basal diet + copper inorganic + sulfur inorganic; 4) basal diet + copper inorganic + sulfur organic; 5) basal diet + copper organic + sulfur, inorganic; 6) basal diet + copper organic + sulfur organic; 7) diet with molybdenum + copper inorganic + sulfur inorganic; 8) diet with molybdenum + copper inorganic + organic sulfur; 9) diet with molybdenum + copper organic + sulfur inorganic, 10) diet with molybdenum + organic copper + organic sulfur. The experimental period lasted 84 days, animal weighing was performed on days 0, 28, 56 and 84 days to monitor its development. Blood collections to study levels of copper, sulfur and molybdenum, concentrations of glucose, albumin, urea, cholesterol, triglycerides, hematocrit and ceruloplasmin were also performed. Liver biopsies were made on zero day for copper, sulfur and molybdenum analysis. At the end of the experimental period, the animals were slaughtered and samples were collected from liver and bile fluid for final determination of mineral, and was measured pH and weight of hot and cold carcasses. At the end of the third experimental period was done a metabolic balance of copper, molybdenum and sulfur. The bioavailability of copper was calculated by \"slope ratio\" technique, using the concentration of copper in the liver. The parameters were analyzed considering the existence of a factorial structure 2 x 2 x 2 (with and without molybdenum, organic and inorganic copper and organic and inorganic sulphur) and a basal diet and a basal diet plus molybdenum. The concentrations of glucose, albumin, urea, cholesterol and blood hematocrit levels were not affected (P>0,05) by the addition of molybdenum or adding copper and sulfur, or the copper-molybdenum-sulfur interaction. However, blood triglyceride concentrations were changed (P<0.05) by copper-sulfur interaction, with a reduction in their concentration after the addition of copper and sulfur in organic sources. The live weight of the sheep was not affected (P>0,05) by the treatments, but the weight gain was influenced by the interaction copper-molybdenum-sulfur there was a reduction in weight gain with the addition of molybdenum and an increase was observed in the weight gain when added with copper and molybdenum in the organic sulfur sources compared to the inorganic sources. The supplemental sources of copper and sulfur and addition of molybdenum had no effect (P>0,05) on pH values and carcass weight. The presence of molybdenum in the diet reduced the levels of ceruloplasmin and the exclusive molybdenum presence reduced copper concentrations in the serum of the sheep. The copper-molybdenum-sulfur interaction with the inorganic sources of minerals decreased serum ceruloplasmin and copper. On the other hand, there was an increase in ceruloplasmin levels with organic sulfur administration. The blood levels of sulfur were not affected by treatments. Bile liquid was affected by molybdenum, it was a reduction in the concentrations of copper and sulfur with increase in the molybdenum concentration. The copper and sulfur liver levels were not influenced (P>0,05) by treatments, only the liver Molybdenum effect showed on copper-molybdenum-sulfur interaction. The bioavailability of organic copper (145.09%) was higher than that of copper sulphate (100%), irrespective of the source and in the absence and sulfur and molybdenum, as determined by multiple regression slope ratio.

No description available.

by Chan Wing-Wah, Anita. / Thesis (M.Sc.)--Chinese University of Hong Kong, 1992. / Includes bibliographical references (leaves 51-55). / LIST OF TABLES / LIST OF FIGURES / LIST OF ABBREVIATIONS / ACKNOWLEDGEMENTS / ABSTRACT / Chapter CHAPTER 1. --- INTRODUCTION --- p.1 / Chapter 1.1 --- General functions of copper --- p.1 / Chapter 1.2 --- Absorption and hepatic metabolism of copper --- p.2 / Chapter 1.3 --- Distribution of copper among components of human serum --- p.2 / Chapter 1.4 --- Plasma copper concentration in disease --- p.3 / Chapter 1.4.1 --- Patients with various tumours --- p.3 / Chapter 1.4.2 --- Patients with gynaecological tumours --- p.4 / Chapter 1.5 --- Serum caeruloplasmin concentration in various malignancies --- p.5 / Chapter 1.6 --- Aim of the study --- p.6 / Chapter 1.7 --- Introduction to some laboratory methods --- p.7 / Chapter 1.7.1 --- Flame atomic absorption spectrophotometry --- p.7 / Chapter 1.7.2 --- Electrothermal (flameless) atomic absorption spectrophotometry --- p.7 / Chapter 1.7.3 --- Separation of the non-protein bound (free) copper --- p.8 / Chapter CHAPTER 2. --- MATERIALS AND METHODS --- p.11 / Chapter 2.1 --- Materials --- p.11 / Chapter 2.2 --- Methods --- p.12 / Chapter 2.2.1 --- Preparation of the ultrafiltrate --- p.12 / Chapter i. --- Ultrafiltration --- p.12 / Chapter ii. --- Determination of protein in the ultrafiltrate --- p.12 / Chapter iii. --- Verification for copper binding property of the YMT membranes --- p.12 / Chapter 2.2.2 --- Establishment of the method for the determination of copper by the graphite furnace atomic absorption --- p.13 / Chapter i. --- Parameters --- p.13 / Chapter ii. --- Method evaluation --- p.13 / Chapter a. --- Precision: within-run precision between-day precision --- p.13 / Chapter b. --- Recovery --- p.15 / Chapter c. --- Linearity --- p.15 / Chapter d. --- Detection limit --- p.15 / Chapter 2.2.3 --- Determination of ultrafiltrable copper concentration --- p.16 / Chapter 2.2.4 --- Effect of storage --- p.16 / Chapter 2.2.5 --- Comparison between healthy male and female subjects --- p.17 / Chapter 2.2.6 --- Determination of serum total copper and caeruloplasmin --- p.17 / Chapter i. --- Determination of total copper --- p.17 / Chapter ii. --- Determination of caeruloplasmin --- p.17 / Chapter 2.3 --- Blood specimens --- p.18 / Chapter 2.4 --- Patient samples --- p.18 / Chapter 2.5 --- Control subjects --- p.18 / Chapter 2.6 --- Statistics --- p.19 / Chapter CHAPTER 3. --- RESULTS --- p.20 / Chapter 3.1 --- Some experimental studies about the YMT membranes --- p.20 / Chapter 3.1.1 --- Membrane leakage --- p.20 / Chapter 3.1.2 --- Verification for copper binding property of the YMT membranes --- p.20 / Chapter 3.2 --- Method evaluation --- p.20 / Chapter 3.2.1 --- Standard addition --- p.20 / Chapter 3.2.2 --- Precision --- p.23 / Chapter 3.2.3 --- Recovery --- p.23 / Chapter 3.2.4 --- Linearity --- p.23 / Chapter 3.2.5 --- Detection limit --- p.23 / Chapter 3.2.6 --- Effect of storage of specimens --- p.23 / Chapter 3.2.7 --- Comparison between healthy male and female subjects --- p.30 / Chapter 3.3 --- "Results of ultrafiltrable (free) copper, total copper and caeruloplasmin" --- p.35 / Chapter 3.3.1 --- "Serum total copper, caeruloplasmin and ultrafiltrable (free) copper in patients with gynaecological malignancies and control subjects" --- p.35 / Chapter 3.3.2 --- "Relationship between total copper, caerulo- plasmin and ultrafiltrable (free) copper concentration" --- p.35 / Chapter 3.4 --- "Results of ultrafiltrable (free) copper, total copper and caeruloplasmin versus FIGO stage" --- p.41 / Chapter CHAPTER 4. --- DISCUSSION --- p.45 / Chapter 4.1 --- About the ultrafiltration --- p.45 / Chapter 4.2 --- Validation of the method performance --- p.46 / Chapter 4.3 --- "Discussion on ultrafiltrable (free) copper, total copper and caeruloplasmin concentration in patients with gynaecological malignancies" --- p.48 / REFERENCE --- p.51

The synthesis of nanostructured materials, especially metallic nanoparticles, has accrued utmost interest over the past decade owing to their unique properties that make them applicable in different fields of science and technology. The limitation to the use of these nanoparticles is the paucity of an effective method of synthesis that will produce homogeneous size and shape nanoparticles as well as particles with limited or no toxicity to the human health and the environment. The biological method of nanoparticle synthesis is a relatively simple, cheap and environmentally friendly method than the conventional chemical method of synthesis and thus gains an upper hand. The biomineralization of nanoparticles in protein cages is one of such biological approaches used in the generation of nanoparticles. This method of synthesis apart from being a safer method in the production of nanoparticles is also able to control particle morphology. In this study, a comparative biological synthesis, characterization and biomedical effects of metallic nanoparticles of platinum, gold and silver were investigated. Metallic nanoparticles were biologically synthesized using cage-like (apoferritin), barrel-like (GroEL) and non-caged (ribonuclease) proteins. Nanoparticles generated were characterized using common techniques such as UV-visible spectroscopy, scanning and transmission electron microscopy, inductively coupled optical emission spectroscopy, Fourier transform infra-red spectroscopy and energy dispersion analysis of X-rays (EDAX). Nanoparticles synthesised biologically using apoferritin, GroEL and RNase with exhibited similar chemical and physical properties as thoses nanoparticles generated chemically. In addition, the metallic nanoparticles fabricated within the cage-like and barrel-like cavities of apoferritin and GroEL respectively, resulted in nanoparticles with relatively uniform morphology as opposed to those obtained with the non-caged ribonuclease. The enzymatic (ferroxidase) activity of apoferritin was found to be greatly enhanced with platinum (9-fold), gold (7-fold) and silver (54-fold) nanoparticles. The ATPase activity of GroEL was inhibited by silver nanoparticles (64%), was moderately activated by gold nanoparticles (47%) and considerably enhanced by platinum nanoparticles (85%). The hydrolytic activity of RNase was however, lowered by these metallic nanoparticles (90% in Ag nanoparticles) and to a higher degree with platinum (95%) and gold nanoparticles (~100%). The effect of synthesized nanoparticles on the respective enzyme activities of these proteins was also investigated and the potential neurotoxic property of these particles was also determined by an in vitro interaction with acetylcholinesterase. Protein encapsulated nanoparticles with apoferrtin and GroEL showed a decreased inhibition of acetylcholinesterase (<50%) compared with nanoparticles attached to ribonuclease (>50%). Thus, it can be concluded that the cavities of apoferitin and GroEL acted as nanobiofactories for the synthesis and confinement of the size and shape of nanoparticles. Furthermore, the interior of these proteins provided a shielding effect for these nanoparticles and thus reduced/prevented their possible neurotoxic effect and confirmed safety in their method of production and application. The findings from this study would prove beneficial in the application of these nanoparticles as a potential drug/drug delivery vehicle for the prevention, treatment/management of diseases associated with these enzymes/proteins.

Measurements of relaxin and acute-phase proteins have not been validated for use in canine serum as a method of pregnancy diagnosis. This means that handling and anesthesia is still necessary to check the pregnancy status of most non-domestic canines. Therefore, the intention of this study was to determine whether relaxin and/or acute-phase proteins could be detected in the urine and/or feces of the domestic dog in order to evaluate the potential for a noninvasive pregnancy test in canines. Blood, urine and feces were collected from 18 domestic dogs and assayed for the presence of relaxin, fibrinogen, alpha-1 acid glycoprotein, and ceruloplasmin. Urinary relaxin appeared to be significant for detecting pregnancy of 30 Days or more in the domestic dog. Additionally, further research might shed light on the presence of relaxin in the feces and fibrinogen and AGP in the urine of the domestic dog and their significance for pregnancy diagnosis.

No description available.

No description available.

Orientadora: Mirela Tinucci Costa / Banca: Suely Nunes Esteves Beloni / Banca: José Jurandir Fagliari / Resumo: A erliquiose canina é uma doença de alta incidência na região nordeste do Estado de São Paulo, sendo responsável pela morte de muitos cães. O diagnóstico precoce favorece a pronta instituição do tratamento e melhora o prognóstico do animal. Estudos têm apontado que a determinação da concentração de proteínas de fase aguda (PFA) pode contribuir para detecção precoce de doenças e auxiliar na predição do prognóstico. O presente estudo objetivou avaliar o perfil de proteínas de fase aguda (PFA) em cães experimentalmente infectados com Ehrlichia canis, amostra Jaboticabal, no início da infecção e após o tratamento. Para tanto, foram utilizados 10 cães sem contato prévio com hemoparasitas. Deste, cinco foram infectados e cinco serviram de controle da infecção. Hemogramas, nested PCR, detecção de anticorpos anti-E. canis e eletroforetograma de proteínas séricas foram realizados em períodos pré-determinados. Os resultados mostraram que a proteína-C reativa, ceruplasmina e a α1-glicoproteína ácida tiveram suas concentrações aumentadas antes do aparecimento dos sinais clínicos e das alterações de hemograma nos cães infectados. Os sinais clínicos da doença tornaram-se evidentes por volta do 17º dia de infecção. Trombocitopenia foi registrada a partir do 3º dia de infecção. Mórulas intracitoplasmáticas foram detectadas a partir do 15º dia. Títulos sorológicos anti-E. canis, variando de 1:2560 a 1:5120, e nPCR positiva foram evidenciados no 18º dia. Nas avaliações após o tratamento os cães infectados estavam assintomáticos, com nPCR negativo e acentuada redução dos títulos de anticorpos específicos em quatro dos cinco cães. Todas as PFA estudadas reduziram suas concentrações a títulos próximos aos iniciais. Este estudo mostrou que as mensurações das PFA contribuem para o diagnóstico precoce e predição de cura... (Resumo completo, clicar acesso eletrônico abaixo) / Abstract: Canine ehrlichiosis is an endemic disease, with high incidence in the Northwest area of Sao Paulo State - Brazil and it is responsible for the death of numerous dogs. The rapid and accurate diagnosis helps the prompt establishment of treatment and improves the prognosis of the animal. Evidence has shown that the measurement of acute phase proteins (APP) can contribute for the early detection of the disease and help to predict its prognosis. This study evaluated the profile of APP in dogs experimentally infected with Ehrlichia canis, Jaboticabal - SP - Brazil sample, at the onset of the infection and after its treatment. Ten dogs, with no previous hemoparasitosis, were randomly assigned in either the infected group (5 animals), which received the bacteria, or the control group (5 animals). In pre-determined intervals, their blood was colected and hemogram, nested PCR, anti-E. canis antibodies detection and tritation and eletrophoresis of serum proteins were performed. We showed that, in infected dogs, the concentration of C-reactive protein, ceruplasmin and α-1 acid glycoprotein were increased previous to the clinical signs forthcoming and hemogram alterations. The clinical signs were evident around the 17th day of infection. Thrombocytopenia was found on the 3th day of infection. Intra-cytosolic morules were detected on the 15th day of infection. Sorologic tritation of anti-E. canis, ranging from 1:2560 to 1:5120, and positive nPCR were found on the 18th day of infection. In the post-treatment evaluation, the infected dogs were asymptomatic with negative nPCR and decreased tritation of specific antibodies in four out of five dogs. All APP analyzed were similar to basal levels. This study showed that the measurement of APP can, indeed, contribute for the early diagnosis and prediction of cure, after treatment, of the experimental acute phase of canine Ehrlichiosis. / Mestre

A doença de Wilson (DW) é distúrbio da excreção biliar de cobre, de herança autossômica recessiva, devido a mutações no gene ATP7B. O cobre que não se liga à apoceruloplasmina circula no organismo ligado a aminoácidos, deposita-se principalmente no fígado e no cérebro e é excretado pelos rins. A cuprúria maior que 100ug/24h pode auxiliar no diagnóstico, embora cerca 20% dos pacientes com DW apresentem níveis anormais de cuprúria basal. A administração da d-penicilamina (DPA) pode promover, em crianças, valores maiores que 1.600ug/24h. A fim de se conhecer os níveis de cuprúria de possíveis indivíduos heterozigotos adultos, foram avaliados 25 pais e 25 mães de pacientes (média 61 anos em homens; 57 anos em mulheres) com diagnóstico de DW. Foram obtidos os níveis séricos de enzimas hepáticas, cobre e ceruloplasmina, e quantificada a cuprúria de 24h. A seguir, os indivíduos receberam DPA 1,0g por via oral dividido em duas tomadas, durante nova coleta de urina para dosagem de cuprúria de 24h. Esta análise foi realizada pelo método de espectrometria de absorção atômica eletrotérmica. Os níveis de enzimas hepáticas foram semelhantes nos dois grupos, exceto o nível médio de fosfatase alcalina que foi maior nas mulheres (H= 68,72 UI/L; M=81,68 UI/L). Os níveis de ceruloplasmina (H=21,72mg/dL; M=27,78mg/dL) e de cobre sérico (H=71,38 ?g/dl; M=88,0 ug/dl) foram maiores em mulheres do que em homens (p<0,001). Os níveis de cuprúria basal foram 22,43ug/24h (média) e 21,40ug/24h (mediana); e, após a DPA, de 523,54 ug/24h (média) e 511,5 ug/24h (mediana). A cuprúria média basal masculina foi de 26,19. ug/24h, enquanto a feminina foi de 18,28. ug/24h (p=0,005). Com o presente estudo, ficou definida possível faixa de variação da cuprúria antes e depois da administração de dpenicilamina em pais de portadores com doença de Wilson; que os pais apresentaram cupremia e níveis de ceruloplasmina menores e cuprúria basal maior do que as mães; que as faixas de variação de normalidade para os parâmetros ceruloplasmina, cobre sérico e urinário deveriam ser diferenciadas de acordo com o sexo. / Wilson disease is a biliary copper excretion disturbance, of recessive autossomic heritage, due to ATP7B gene mutations. The copper not bound to apoceruloplasmin circulates in the organism bound to amino acids and accumulates mainly in the liver and brain being excreted by the kidneys. Urinary copper higher than 100ug/24h can be useful in the diagnosis, but only about 20% of Wilson disease patients have abnormal basal levels. In this case, d-penicillamine (DPA) administration can lead, in children, to levels higher than 1.600ug/24h. Twenty five fathers and twenty five mothers of wilson disease patients (mean 61 years for male, and 57 years for female) were assessed in order to obtain urinary copper levels of probable heterozygote adults. Fasting liver enzymes, copper and ceruloplasmin serum levels were obtained along with 24h urinary copper excretion. After, patients got DPA 1.0g by oral route, twice a day, while collecting urine for 24h urinary copper excretion dosage. These analyses were performed by elethrotermic atomic absortion spectrometry method. Liver enzyme levels were similar in men and women but those of alkaline phosphatase were higher in women (M= 68.72 UI/L; F=81.68 UI/L). Serum ceruloplasmin (F=21.72mg/dl; F=27.78mg/dl) and copper (M=71.38 ug/dl; F=88.0 ug/dl) levels were higher in women than in men (p<0.001). Urinary copper levels before DPA were, 43ug/24h (mean) and 21.40 ug/24h (median); and, after DPA, 523.54 ug/24h (mean) and 511.5 ug/24h (median). Basal urinary copper levels in men were 26.9 ug/24h, and in women were 18.67 ug/24h (p=0.005). With the results of this study, we defined a possible range for urinary copper before and after oral intake of DPA in parents of Wilson disease patients. Furthermore, fathers had lower levels of serum copper and of ceruloplasmin, and greater levels of baseline cupriuresis than mothers; and finally the normal range for serum copper and ceruloplasmin, and urinary copper should be differentiated according to the gender.

A doença de Wilson (DW) é distúrbio da excreção biliar de cobre, de herança autossômica recessiva, devido a mutações no gene ATP7B. O cobre que não se liga à apoceruloplasmina circula no organismo ligado a aminoácidos, deposita-se principalmente no fígado e no cérebro e é excretado pelos rins. A cuprúria maior que 100ug/24h pode auxiliar no diagnóstico, embora cerca 20% dos pacientes com DW apresentem níveis anormais de cuprúria basal. A administração da d-penicilamina (DPA) pode promover, em crianças, valores maiores que 1.600ug/24h. A fim de se conhecer os níveis de cuprúria de possíveis indivíduos heterozigotos adultos, foram avaliados 25 pais e 25 mães de pacientes (média 61 anos em homens; 57 anos em mulheres) com diagnóstico de DW. Foram obtidos os níveis séricos de enzimas hepáticas, cobre e ceruloplasmina, e quantificada a cuprúria de 24h. A seguir, os indivíduos receberam DPA 1,0g por via oral dividido em duas tomadas, durante nova coleta de urina para dosagem de cuprúria de 24h. Esta análise foi realizada pelo método de espectrometria de absorção atômica eletrotérmica. Os níveis de enzimas hepáticas foram semelhantes nos dois grupos, exceto o nível médio de fosfatase alcalina que foi maior nas mulheres (H= 68,72 UI/L; M=81,68 UI/L). Os níveis de ceruloplasmina (H=21,72mg/dL; M=27,78mg/dL) e de cobre sérico (H=71,38 ?g/dl; M=88,0 ug/dl) foram maiores em mulheres do que em homens (p<0,001). Os níveis de cuprúria basal foram 22,43ug/24h (média) e 21,40ug/24h (mediana); e, após a DPA, de 523,54 ug/24h (média) e 511,5 ug/24h (mediana). A cuprúria média basal masculina foi de 26,19. ug/24h, enquanto a feminina foi de 18,28. ug/24h (p=0,005). Com o presente estudo, ficou definida possível faixa de variação da cuprúria antes e depois da administração de dpenicilamina em pais de portadores com doença de Wilson; que os pais apresentaram cupremia e níveis de ceruloplasmina menores e cuprúria basal maior do que as mães; que as faixas de variação de normalidade para os parâmetros ceruloplasmina, cobre sérico e urinário deveriam ser diferenciadas de acordo com o sexo. / Wilson disease is a biliary copper excretion disturbance, of recessive autossomic heritage, due to ATP7B gene mutations. The copper not bound to apoceruloplasmin circulates in the organism bound to amino acids and accumulates mainly in the liver and brain being excreted by the kidneys. Urinary copper higher than 100ug/24h can be useful in the diagnosis, but only about 20% of Wilson disease patients have abnormal basal levels. In this case, d-penicillamine (DPA) administration can lead, in children, to levels higher than 1.600ug/24h. Twenty five fathers and twenty five mothers of wilson disease patients (mean 61 years for male, and 57 years for female) were assessed in order to obtain urinary copper levels of probable heterozygote adults. Fasting liver enzymes, copper and ceruloplasmin serum levels were obtained along with 24h urinary copper excretion. After, patients got DPA 1.0g by oral route, twice a day, while collecting urine for 24h urinary copper excretion dosage. These analyses were performed by elethrotermic atomic absortion spectrometry method. Liver enzyme levels were similar in men and women but those of alkaline phosphatase were higher in women (M= 68.72 UI/L; F=81.68 UI/L). Serum ceruloplasmin (F=21.72mg/dl; F=27.78mg/dl) and copper (M=71.38 ug/dl; F=88.0 ug/dl) levels were higher in women than in men (p<0.001). Urinary copper levels before DPA were, 43ug/24h (mean) and 21.40 ug/24h (median); and, after DPA, 523.54 ug/24h (mean) and 511.5 ug/24h (median). Basal urinary copper levels in men were 26.9 ug/24h, and in women were 18.67 ug/24h (p=0.005). With the results of this study, we defined a possible range for urinary copper before and after oral intake of DPA in parents of Wilson disease patients. Furthermore, fathers had lower levels of serum copper and of ceruloplasmin, and greater levels of baseline cupriuresis than mothers; and finally the normal range for serum copper and ceruloplasmin, and urinary copper should be differentiated according to the gender.