Cases Of Copper Deficiency Research Articles

Can Patients with Wilson's Disease Develop Copper Deficiency?

Wilson's disease (WD) is a rare genetic condition characterized by a copper overload in organs secondary to mutation in ATP7B gene. Lifelong decoppering treatments are the keystone of the treatment but must be regularly adapted to obtain a correct copper balance and could lead to copper deficiency (CD). Study the characteristics of CD in WD patients. CD cases from our cohort of 338 WD patients have been investigated. CD was defined by the association of serum copper, exchangeable copper and urinary copper excretion assays less than two standard deviations from the mean with cytopenia and/or neurological damage of spinal cord origin. A systematic review of literature about cases of CD in WD patient was performed in PubMed database according to PRISMA guidelines. Three WD patients were diagnosed with CD in our cohort. Review of the literature found 17 other patients. Most of the patients had anemia and neutropenia associated with neurological symptoms (especially progressive posterior cord syndrome). All the patients were treated with Zinc salts and the symptoms occurred more than a decade after the initiation of treatment. The adaptation of the treatment allowed a correction of the cytopenia but only a partial improvement of the neurological symptoms. WD patients can develop CD after many years of zinc therapy. Anemia and neutropenia are red flags that should evoke CD.

A case of Copper deficiency that was difficult to diagnosis and treatment in enteral nutrition.

A case of Copper deficiency that was difficult to diagnosis and treatment in enteral nutrition.

Deficiência de cobre em suínos: caracterização clínico-patológica

São relatados dois casos de deficiência de cobre em suínos (A e B), fêmeas, sem raça definida, de três meses de idade, provenientes de propriedade rural em Planaltina, GO. Os animais apresentavam incoordenação, paresia de membros pélvicos, com progressão para os membros torácicos e andar com flexão da articulação metacarpo-falangeana. Na propriedade de origem dos animais, outros dois suínos apresentavam atrofia da musculatura da coxa e posição sentada quando parados. O rebanho recebia dieta não-balanceada constituída por milho, soro de leite e sobras de lavoura. Na necropsia, não foram observadas alterações significativas, mas histologicamente havia vacuolização periaxonal multifocal no tronco encefálico e na medula espinhal, quantidade discreta de esferoides axonais e macrófagos no interior dos vacúolos. A coloração com azul rápido de luxol (ARL) para mielina demonstrou que as áreas de vacuolização caracterizavam focos de desmielinização. Amostras de fígado dos dois suínos apresentaram níveis de cobre muito abaixo do normal, de 13,1 partes por milhão (ppm) (suíno A) e 5,7ppm (suíno B). Este trabalho descreve dois casos de desmielinização medular e de tronco encefálico devido à deficiência de cobre em suínos jovens.

Copper Deficiency (Hypocupremia) and Pancytopenia Late After Gastric Bypass Surgery

Hypocupremia, or copper deficiency, is a rare and underrecognized cause of bone marrow dysplasia. Most cases of copper deficiency in adults occurred historically in patients receiving total parenteral hyperalimentation or total parental nutrition. More recently, with the obesity epidemic and the prevalence of gastric bypass, cases of malabsorption-related copper deficiency have occurred. Copper deficiency can lead to significant cytopenias and possible neurologic sequelae, which can be misdiagnosed and mismanaged. Unfortunately, a delay in diagnosis and appropriate treatment may lead to permanent neurologic damage. We describe a woman with previous gastric bypass surgery who presented with pancytopenia and bone marrow biopsy findings consistent with a myelodysplastic syndrome with excess blasts. She was found to be significantly copper deficient. With replacement copper therapy, her cytopenias quickly resolved. We discuss the distinctive clinical and hematologic features of this rare cause of significant cytopenias and provide recommendations for monitoring and treatment of such patients. Moreover, this case is an important reminder that bariatric patients should have routine follow-ups after surgery and continue dietary supplements indefinitely.

Hypocupremia associated cytopenia and myelopathy: a national retrospective review

Copper is an essential trace element that is involved in a number of important enzymatic processes throughout the body. Recent single case reports and small studies have shown that deficiency of copper can cause reversible haematological changes and irreversible neurological injury. We chose to undertake a national study, looking at all cases of copper deficiency in Scotland over a 5-yr period using information from a national reference laboratory. From 16 identified patients, we determined that 86% had both haematological and neurological features of copper deficiency, while 18% had haematological features only at presentation. Twelve of the sixteen patients had high serum zinc concentrations (>18 μm/L) nine patients were using zinc-containing dental fixatives at time of diagnosis. 94% of patients had haematological features as an initial manifestation of copper deficiency, which included anaemia, thrombocytopenia and neutropenia. Patients who underwent later bone marrow testing had appearances in keeping with refractory cytopenia with multilineage dysplasia, refractory anaemia with excess of blasts, unclassified marrow dysplasia or probable myelodysplasia (MDS). 75% of patients had neurological symptoms or signs, including progressive walking difficulties and paraesthesia, or gait difficulties without sensory signs. Clinical examination was in keeping with spastic paraparesis (either with or without sensory neuropathy). Magnetic resonance imaging (MRI) showed multifocal T2 hyper intense foci in the subcortical white matter, and atrophy of the cerebrum and cerebellum was also seen on computerised tomography (CT). MRI of the spinal cord showed signal change in the dorsal columns in either the cervical or thoracic cord. 93% of cytopenias responded to copper replacement and addressing the original cause of the copper deficiency, but only 25% of patients had improvement in their neurological function, while 33% deteriorated and 42% remained unchanged. Our study demonstrates that copper deficiency is an under-recognised cause of several types of cytopenia, which are reversible but can progress to significant neurological injury if left untreated. We illustrate the importance of identifying these patients early to prevent irreversible neurological injury.

Plasma zinc and copper levels in children of families with history of cardio-vascular disease

Zinc and copper are beneficial to health, growth and development, and also for the prevention of cardiovascular- disease (CVD) with regard to improved dietary habits as a preliminary step in CVD prevention. This study was conducted among 2-18-year old children with significant family risk for premature CVD in comparison to controls.Materials and Methods: One hundred randomly selected children of parents who had premature myocardial infarctions were included in the study. The controls were 100 individuals randomly selected from the case group's neighbors and matched for age, sex and socioeconomic status. A four-day food record questionnaire was used to assess zinc and copper intakesResults: The daily average intake of zinc was significantly lower in the case than in the control group (6.89±2.97 vs. 8.30±2.45 mg, P=0.047). The mean serum zinc level was not significantly different between both groups (82.12±14.1 vs. 92.26±23.7 μg/dL, P>0.05). The daily intake and serum level of copper were not significantly different between the case and the control groups. No case of copper deficiency was found. The mean systolic blood pressure was not significantly different between the zinc-deficient and zinc-sufficient subjects.Although the mean diastolic blood pressure of the former was higher than the latter, there was no statistically significant difference. About 23.7% of all studied sample had mild to moderate degrees of failure to thrive, with significantly lower daily intake and serum zinc level than other subjects (5.41±1.06 mg, 82.09±12.74 μg/dL vs. 6.89±2.14 mg, 99.25±27.15 μg/dL, respectively, P<0.05).Conclusion: We propose that children, especially those with significant family risk of premature CVD, place increased emphasis on the consumption of foods rich in zinc.

Hematogone Hyperplasia in Copper Deficiency

Copper deficiency is likely an underrecognized cause of anemia and neutropenia and may masquerade as a myelodysplastic syndrome (MDS). We report 2 cases of copper deficiency in which the diagnosis was suggested based on the characteristic morphologic findings, such as cytoplasmic vacuolization of erythroid and myeloid precursors and iron-containing plasma cells. It is interesting that both patients had hematogone hyperplasia. This phenomenon, largely absent in MDS, may aid in distinguishing nonclonal causes of cytopenias, such as copper deficiency, from MDS. It is of crucial importance to identify treatable causes of cytopenias when MDS is suspected. We recommend copper level assessment in patients suspected of having low-grade MDS, especially patients with neuropathy and normal results of cytogenetic studies.

Copper Deficiency Misdiagnosed as Postpolio Syndrome

ABSTRACT Symptoms of progressive weakness and pain after years of stability are common in survivors of paralytic poliomyelitis. These symptoms been coined the postpolio syndrome. In the majority of patients, this condition is stable and benign. Here, a case of copper deficiency as a result of excessive zinc supplementation is presented, mimicking the post polio syndrome. This case highlights the importance of a thorough medical history and evaluation in polio survivors when they present with new progressive deficits. Postpolio syndrome remains a diagnosis of exclusion and is a benign entity in most patients. Common and atypical features of the postpolio syndrome are reviewed. In the appropriate clinical setting, a diagnosis of copper deficiency should be considered and may be confirmed with the proper serologic testing.

Zinc and Copper Status in Children with High Family Risk of Premature Cardiovascular Disease

Zinc and copper are beneficial to health, growth and development, and also for the prevention of cardiovascular disease (CVD) with regards to improved dietary habits as a preliminary step in CVD prevention. This study was conducted among 2-18-year-old children with high family risk of premature CVD in comparison to controls. One hundred randomly selected children whose parents had premature myocardial infarction were included in the study. The controls were 100 individuals randomly selected from the case group's neighbors and matched for age, sex, and socioeconomic status. A four-day food record questionnaire was used to assess zinc and copper intakes, and their serum levels were determined using Flame-Atomic Absorption Spectrophotometry. The data were analyzed by SPSS/Windows V6 software, using the Student's t and Mantel-Hanzel tests. Significance of differences was considered at P<0.05. The daily zinc intake was significantly lower in the case than control group (6.89+/-2.97 vs. 8.30+/-2.45 mg, P=0.047). The mean serum zinc level was not significantly different between both groups (82.12+/-14.1 vs. 92.26+/-23.7 microg/dL, P>0.05). Zinc deficiency was more prevalent among the case in boys than their controls (58% vs. 18%, P=0.04). This difference was not significantly in girls (44% vs. 40%). The daily intake and serum level of copper were not significantly different between the case and control groups. No case of copper deficiency was found. The mean systolic blood pressure was not significantly different between the zinc-deficient and zinc-sufficient subjects. Although the mean diastolic blood pressure of the former was higher than the latter, there was no statistically significant difference. About 23.7% of all studied sample had mild-to-moderate degrees of failure to thrive, with significantly lower daily intake and serum zinc level than other subjects (5.14+/-1.06 mg, 82.09+/-12.74 microg/dL vs. 6.89+/-2.14 mg, 99.25+/-27.15 microg/dL, respectively, P<0.05). It is recommended that emphasis be placed on the consumption of food rich in zinc by children, especially those with high family risk of premature CVD.

A Case of Copper Deficiency during Enteral Nutrition

Copper deficiency during enteral nutrition is very rare. In this case, we treated a patient who developed leukocytopenia and anemia caused by copper deficiency during long-term enteral nutrition.A 67-year-old woman was admitted for treatment of brain damage after cardiopulmonary resuscitation. After one month, her condition improved and she started rehabilitation. As she could not eat spontaneously, she received forced enteral feeding (Fiberen YH(R)). However, She developed leukocytopenia after 15 weeks enteral feeding, and then had anemia for 23 weeks (white blood cell count; WBC 2.5×103μl-1, red blood cell count; RBC 2.68×106·μl-1, hemoglobin; Hb 8.4gdl-1, hematocrit; Ht 25.1% for 24 weeks). Bone marrow was tapped and found to be normal. The plasma copper level was markedly below normal (9μg·dl-1). After 4 weeks of intravenous (0.3mg·day-1) and enteral (6.4mg·day-1) copper supplementation, the plasma copper level increased to 97μg·dl-1 and hematologic values improved dramatically (WBC; 9.0×103μl-1, RBC; 3.95×106μl-1, Hb; 12.0g·dl-1, Ht; 36.0%). In conclusion, the copper deficiency should be considered during not only total parenteral nutrition but also enteral nutrition when hematologic disorder develops.

Copper as an essential nutrient

Animal and human studies have shown that copper is involved in the function of several enzymes. Studies have also shown that copper is required for infant growth, host defense mechanisms, bone strength, red and white cell maturation, iron transport, cholesterol and glucose metabolism, myocardial contractility, and brain development. Copper deficiency can result in the expression of an inherited defect such as Menkes syndrome or in an acquired condition. Acquired deficiency is mainly a pathology of infants; however, it has been diagnosed also in children and adults. Most cases of copper deficiency have been described in malnourished children. The most constant clinical manifestations of acquired copper deficiency are anemia, neutropenia, and bone abnormalities. Other, less frequent manifestations are hypopigmentation of the hair, hypotonia, impaired growth, increased incidence of infections, alterations of phagocytic capacity of the neutrophils, abnormalities of cholesterol and glucose metabolism, and cardiovascular alterations. Measurements of serum copper and ceruloplasmin concentrations are currently used to evaluate copper status. These indexes are diminished in severe to moderate copper deficiency; however, they are less sensitive to marginal copper deficiency. Erythrocyte superoxide dismutase and platelet cytochrome c activities may be more promising indexes for evaluating marginal copper deficiency.

A Case of Copper Deficiency in Long Term Total Parenteral Nutrition

A Case of Copper Deficiency in Long Term Total Parenteral Nutrition