Hypocupremia

BACKGROUND: Vitamin B12 deficiency is a well recognized cause of posterolateral myelopathy. In Indian subcontinent, it may coexist with nutritional copper deficiency producing partial response of patients to B12 supplementation. Hence the study was planned to look for association of hypocupremia and B12 deficiency. METHODS: Twenty-three patients with posterolateral myelopathy (Romberg sign positive) were enrolled and investigated for levels of vitamin B12, copper and zinc and followed up for six months...

MEDNIK syndrome-acronym for mental retardation, enteropathy, deafness, neuropathy, ichthyosis, keratodermia-is caused by AP1S1 gene mutations, encoding σ1A, the small subunit of the adaptor protein 1 complex, which plays a crucial role in clathrin coat assembly and mediates trafficking between trans-Golgi network, endosomes and the plasma membrane. MEDNIK syndrome was first reported in a few French-Canadian families sharing common ancestors, presenting a complex neurocutaneous phenotype, but its pathogenesis is not completely understood...

Copper is an essential trace element, playing a critical role in multiple functions in the body. Despite the necessity of adequate copper provision and data supporting the safety of copper administration during cholestasis, it remains common practice to reduce or remove copper in parenteral nutrition (PN) solutions after the development of cholestasis due to historical recommendations supporting this practice. In neonates, specifically premature infants, less is known about required copper intakes to accumulate copper stores and meet increased demands during rapid growth...

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...

OBJECTIVE: Neurological complications of celiac disease (CD) include neuropathy, myeloneuropathy, and cerebellar degeneration. The cause of neuropathy in patients with CD is not known. Prior publications describe copper deficiency in CD patients with myeloneuropathy and neuropathy and posit that hypocupremia is the cause of these neurological conditions. However, based on our clinical experience, we hypothesized that CD patients with polyneuropathy are not deficient in copper. METHODS: Patients who met diagnostic criteria for CD and peripheral neuropathy were included...

In order to evaluate the status of zinc and copper in pregnant women and in preeclampsia, we conducted a prospective case-control study. It involved 56 pregnant women compared with 30 non-pregnant women selected as controls. For pregnant women, serum levels of zinc, copper and ceruloplasmin were determined quarterly. These same measurements were performed once in controls. The comparison of various parameters in normal pregnancy and in control women revealed a statistically significant decrease in serum levels of zinc from 1st to 3rd quarter...

A 36-year-old female developed bone marrow failure diagnosed as myelodysplastic syndrome (MDS) (Sidebar), followed shortly by a peripheral neuropathy and a gait disturbance. While waiting for a bone marrow transplant, she reported to us that she had seen attorney-generated, televised advertisements concerning the role of denture adhesives relating to her malady. Labs were then obtained demonstrating she had dramatic and unsuspected hypocupremia and hyperzincemia. Administration of copper and cessation of denture adhesives resulted in recovery of her hematopoietic system and partial resolution of the neurological sequela...

BACKGROUND: Copper deficiency affects the peripheral (PNS) and central (CNS) nervous systems and can lead to neurological deficits in humans. No studies have addressed whether copper deficiency affects the enteric nervous system (ENS). We hypothesized that ENS abnormalities impair intestinal function in copper deficiency. METHODS: We induced copper deficiency in rats by nutritional deprivation. Once hypocupremia was achieved, we euthanized the animals and harvested the small and large intestine...

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...

BACKGROUND: There are limited data on the prevalence of copper and zinc deficiency in the long term after bariatric surgery. METHODS: We analysed copper and zinc serum levels in a cohort of 141 patients, 52 who underwent Roux-en-Y gastric bypass (RYGB) and 89 biliopancreatic diversion (BPD), with a follow-up of 5 years. RESULTS: Mean copper level was significantly lower in the BPD group (P < 0.0001 vs. RYGB). Forty-five (50.6%) BPD patients had, at least once, a low copper level, and half of them, 27 (30...

Bioactive copper(II) complexes with polysaccharides, like pullulan and dextran, are important in both veterinary and human medicine for the treatment of hypochromic microcitary anemia and hypocupremia. In aqueous alkaline solutions, Cu(II) ion forms complexes with the exopolysaccharide pullulan and its reduced low-molecular derivative. The metal content and the solution composition depend on pH, temperature, and time of the reaction. The complexing process begins in a weak alkali solution (pH >7) and involves OH groups of pullulan monomer (glucopyranose) units...

A 6-year-old boy presented with pancytopenia. Bone marrow morphology showed dyspoiesis and cytoplasmic vacuolation in myeloid precursor cells. Cytoplasmic vacuoles are described in erythroid cells in myelodysplastic syndrome (MDS) but are extremely rare in myeloid precursor cells. We ruled out viral and autoimmune etiology, hypocupremia, Pearson syndrome, and chromosomal abnormalities. Finally, a diagnosis of MDS of refractory cytopenia of childhood subtype was made. The patient then underwent an allogenic stem cell transplant that resulted in normalization of the complete blood counts and bone marrow morphology...

Gastrojejunal bypass surgery may be complicated by nutritional deficiencies, including trace elements. Copper, which is absorbed in the duodenum and proximal jejunum, is poorly absorbed when a significant portion of the proximal small intestine is bypassed. However, despite this theoretical risk of hypocupremia in the gastrojejunal bypass patient, reports of symptomatic copper deficiency are very rare, and even then the deficiency is often potentiated by an accelerant, such as concomitant zinc ingestion. We report a case of hypocupremia resulting in hematologic abnormalities and myeloneuropathy after gastrojejunal bypass surgery for morbid obesity...

Although seizures are rarely encountered in Wilson's disease (WD), seizures related to hypocupremia have not been reported before. We report a patient presenting with partial status epilepticus who was on strict low-copper diet and chelating therapy for WD. Despite other rare causes of seizures in WD including penicillamine-induced pyridoxine deficiency, cerebral copper deposition and metabolic encephalopathy, the most probable cause of resistant status epilepticus in this patient was found as hypocupremia from overzealous treatment...

We report two patients, both with a history of gastric surgery, who presented with progressive optic neuropathy and myelopathy. The patients' symptoms were initially attributed to vitamin B12 deficiency and/or neuromyelitis optica; however, after the neurologic deficits continued to progress with the use of conventional treatments, further evaluation was initiated, and a severe copper deficiency was revealed. Copper deficiency is a rare cause of progressive optic neuropathy and myelopathy and should be considered in the differential diagnosis...

It is increasingly evident that a multitude of etiologies can give rise to signal abnormality in the dorsal and lateral columns of the spinal cord, apart from pernicious anemia. We report a case of dorsal and lateral columns signal abnormality related to hypocupremia resulting in progressive sensory ataxia and weakness in the lower and upper limbs, compounded by a recent diagnosis of Sjögren's syndrome.

Neurodegeneration of the central and peripheral nervous system associated with hypocupremia and hyperzincinemia has been widely recognized but the origin of high zinc remained unknown. Denture cream has been recently suggested as one possible source of zinc, but the frequency with which denture fixative alone accounts for this syndrome is unknown. We analyzed the origin of excessive zinc in eleven patients with a progressive myelopolyneuropathy and unexplained hypocupremia with hyperzincinemia. These patients had a detailed clinical assessment, determination of zinc and copper levels, and analyzed use of denture cream with the estimates of daily zinc exposure...

Pseudomonas aeruginosa was isolated from infected burn patients and characterized by standard biochemical tests. The in vitro copper uptake was compared between this isolated pathogenic strain and two non-pathogenic control strains of gram positive bacteria Bacillus thuringiensis strain Israelis as well as gram negative bacteria Enterobacter aerogenes. Maximum copper uptake of 470 ppm/g biomass was obtained by P. aeruginosa strain, while the control strains B. thuringiensis and Enterobacter aerogenes had copper uptake of 350 and 383 ppm/g biomass, respectively...

BACKGROUND: : Acquired copper deficiency in adults leads to hematological and neurological manifestations that mimic vitamin B12 deficiency. A significant number of patients with copper deficiency syndrome have a history of gastric surgery, often remote. We sought to determine whether copper deficiency is present in a population of individuals with longstanding partial gastric resection. METHODS: : Serum copper, ceruloplasmin, and zinc levels were determined in 20 patients with a history of partial gastric resection and 50 controls, randomly selected from the Oklahoma City Veterans Affairs Medical Center electronic database...

BACKGROUND: Chronic, excess zinc intake can result in copper deficiency and profound neurologic disease. However, when hyperzincemia is identified, the source often remains elusive. We identified four patients, one previously reported, with various neurologic abnormalities in the setting of hypocupremia and hyperzincemia. Each of these patients wore dentures and used very large amounts of denture cream chronically. OBJECTIVE: To determine zinc concentration in the denture creams used by the patients as a possible source of excess zinc ingestion...