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Yu Huang, Mitsuhisa Takatsuki, Akihiko Soyama, Masaaki Hidaka, Shinichiro Ono, Tomohiko Adachi, Takanobu Hara, Satomi Okada, Takashi Hamada, Susumu Eguchi
BACKGROUND Liver transplantation is indicated for patients with Wilson's disease (WD) who present either with acute liver failure or with end-stage liver disease and severe hepatic insufficiency as the first sign of disease. However, almost all reported cases have been treated with death donor liver transplantation. Here we report the case of a patient with WD associated with fulminant hepatic failure (WD-FHF) who underwent living donor liver transplantation (LDLT). CASE REPORT A 17-year-old female was diagnosed with WD-FHF based on high uric copper (10 603 μg/day, normal <100 μg/day), low serum ceruloplasmin (15 mg/dL, normal >20 mg/dL) and Kayser-Fleischer (K-F) corneal ring, and acute liver failure (ALF), acute renal failure (ARF) and grade 2 hepatic encephalopathy (HE)...
March 17, 2018: American Journal of Case Reports
Jung-Heun Ha, Caglar Doguer, Shireen R L Flores, Tao Wang, James F Collins
Background: Consumption of a high-iron diet causes copper deficiency in weanling rodents; however, the minimum amount of dietary iron that disrupts copper homeostasis has not been established. Objective: We tested the hypothesis that dietary iron at only several-fold above physiologic requirements would cause copper depletion. Methods: Weanling male Sprague-Dawley rats (n = 6/group) were fed AIN-93G-based diets with adequate (88 µg Fe/g = 1×), or excessive (4×, 9...
March 1, 2018: Journal of Nutrition
Iwona Sembratowicz, Katarzyna Ognik
Gold nanoparticles (AuNPs) are one of the most examined nanomaterials, but information about their immunogenic potential is still insufficient. Understanding interaction of AuNPs with immune system is essential in designing their safety and possibilities of biomedical applications. An experiment was conducted to determine immunotropic activity of gold nanocolloid (AuNPs) administered orally to chickens depending on dose and duration time. 162 birds were assigned to 9 experimental groups of 18 birds each. The control group (C) did not receive AuNPs...
May 2018: Journal of Trace Elements in Medicine and Biology
Joanna Suliburska, Katarzyna Skrypnik, Monika Szulińska, Justyna Kupsz, Paweł Bogdański
BACKGROUND: Hypotensive therapy leads to a number of trace elements metabolism disturbances. Zinc balance is frequently affected by antihypertensive treatment. AIM: To evaluate the effect of a hypotensive treatment, modified diet and zinc supplementation on mineral status and selected biochemical parameters in newly diagnosed hypertensive patients on monotherapy. METHODS: In the first stage, arterial hypertension in ninety-eight human subjects was diagnosed...
May 2018: Journal of Trace Elements in Medicine and Biology
Peter Riško, Jan Pláteník, Richard Buchal, Jana Potočková, Pavel J Kraml
BACKGROUND AND AIMS: The increased iron level and the labile iron pool (LIP) in circulating monocytes are connected to a higher frequency of cardiovascular events. METHODS: The study investigates the relationship between LIP in circulating monocytes and markers of iron metabolism and atherosclerosis (inflammation, oxidative stress, endothelial dysfunction and arterial elasticity) in long-term blood donors and non-donor volunteers. RESULTS: We found that donors had significantly higher LIP values than the control group (1...
March 3, 2018: Atherosclerosis
Omid Daneshjoo, Masoud Garshasbi
BACKGROUND: Wilson disease is an autosomal recessive disorder of copper transport and is characterized by excessive accumulation of cellular copper in the liver and other tissues because of impaired biliary copper excretion and disturbed incorporation of copper into ceruloplasmin. Hepatic failure and neuronal degeneration are the major symptoms of Wilson disease. Mutations in the ATP7B gene are the major cause of Wilson disease. CASE PRESENTATION: In this study we have screened one pedigree with several affected members, including a 24-year-old Iranian woman and a 20-year-old Iranian man, who showed psychiatric and neurological symptoms of varying severity, by amplifying the coding regions including exon-intron boundaries with polymerase chain reaction and sequencing...
March 15, 2018: Journal of Medical Case Reports
Tiandong Che, Diyan Li, Long Jin, Yuhua Fu, Yingkai Liu, Pengliang Liu, Yixin Wang, Qianzi Tang, Jideng Ma, Xun Wang, Anan Jiang, Xuewei Li, Mingzhou Li
Genome-wide transcriptomic studies in humans and mice have become extensive and mature. However, a comprehensive and systematic understanding of protein-coding genes and long non-coding RNAs (lncRNAs) expressed during pig spleen development has not been achieved. LncRNAs are known to participate in regulatory networks for an array of biological processes. Here, we constructed 18 RNA libraries from developing fetal pig spleen (55 days before birth), postnatal pig spleens (0, 30, 180 days and 2 years after birth), and the samples from the 2-year-old Wild Boar...
2018: PloS One
Tomás Urrego, Blanca Ortiz-Reyes, Adriana L Vanegas-García, Carlos H Muñoz, Luis A González, Gloria Vásquez, José A Gómez-Puerta
BACKGROUND AND OBJECTIVE: Diagnosis of lupus nephritis (LN) is usually based on renal biopsy, which is an invasive technique that involves multiple risks. Therefore, different biomarkers have emerged as alternatives for the diagnosis of LN. Nonetheless, studies regarding urinary biomarkers in Latin American patients are limited. The objective of this study was to assess the diagnostic value of urinary transferrin and ceruloplasmin to differentiate patients who have renal involvement from those who do not...
March 9, 2018: Reumatología Clinica
Ruhsen Öcal, Serkan Öcal, Mahir Kırnap, Gökhan Moray, Mehmet Haberal
OBJECTIVES: Wilson disease is an autosomal, recessive, inherited disorder of copper metabolism that results in the accumulation of copper in many organs and tissues. This disease is mainly characterized by dysfunction due to copper accumulation in the liver, kidney, brain, cornea, bone, heart, and blood cells. The clinical spectrum is broad in Wilson disease. Asymptomatic Wilson disease may be present, but findings related to the involvement of an individual organ or multiple organ failure can be seen...
March 2018: Experimental and Clinical Transplantation
L De Riccardis, A Buccolieri, M Muci, E Pitotti, F De Robertis, G Trianni, D Manno, M Maffia
Although many studies have been carried out in order to understand the implication of copper (Cu) in the pathogenesis of multiple sclerosis (MS), the exact role that this metal plays in the disease is not still clear. Because of the lack of information in this subject, the present study compared the serum and cerebrospinal (CSF) levels of copper in MS patients in respect to a control group, matched for age and sex, finding a significant increase of metal concentrations, in both biological fluids of MS subjects...
March 7, 2018: Biochimica et Biophysica Acta
Nehal El-Koofy, Hanan M Fouad, Mona E Fahmy, Heba Helmy, Olfat Shaker, Hanaa M El-Karaksy, Nabil Mohsen
BACKGROUND AND STUDY AIMS: Hepatobiliary cholestatic disorders produce excess copper (Cu) retention in the liver, which is toxic and may cause hepatitis, fulminant hepatic failure, cirrhosis and death. In this study, we measured hepatic Cu and tested its correlation with serum Cu (S. Cu) and serum ceruloplasmin (S. ceruloplasmin) in cholestatic infants. PATIENTS AND METHODS: 41 cholestatic infants were enrolled as cases and 11 healthy infants as control subjects...
March 6, 2018: Arab Journal of Gastroenterology: the Official Publication of the Pan-Arab Association of Gastroenterology
Orhan Gozeneli, Faik Tatli, Ali Erdal Gunes, Muhammed Emin Guldur, Abdullah Taskin, Osman Bardakci, Mehmet Yilmaz
PURPOSE: To investigate thymoquinone, curcumin and a combination of these two drugs were effective or not at the growth of liver. METHODS: Forty female Wistar-Albino rats distributed into five groups of eight rats each, control, thymoquinone, curcumin, and thymoquinone/curcumin groups. Pathological specimens were studied using the Ki-67 Proliferation Index(PI); and arginase(Arg), tissue plasminogen activator(tPA), ceruloplasmin(Cer) and nitric oxide(NO) were studied in biochemical analysis...
February 2018: Acta Cirúrgica Brasileira
Clement Bresteau, Sophie Prevot, Gabriel Perlemuter, Cosmin Voican
Drug-induced liver injury is the fourth most common cause of liver disease in industrialised countries. Methylprednisolone is often considered to be a treatment with a low hepatotoxicity. We report a case of methylprednisolone-induced liver injury in a 35-year-old woman. She was admitted to our department for acute liver injury 2 months after a treatment with high dose of methylprednisolone (1 g/day) for a multiple sclerosis relapse. No other cause of liver injury could be found (screening for hepatotropic viruses, autoimmune antibodies, ceruloplasmin, abdominal ultrasonography and liver biopsy)...
March 5, 2018: BMJ Case Reports
Sara Pelucchi, Raffaella Mariani, Giulia Ravasi, Irene Pelloni, Massimo Marano, Lucio Tremolizzo, Massimo Alessio, Alberto Piperno
INTRODUCTION: Aceruloplasminemia is an ultra-rare hereditary disorder characterized by iron-restricted microcytic anemia and tissue iron overload associated with diabetes, retinal and progressive neurological degeneration. We describe genotypes and phenotypes at diagnosis, and disease evolution of seven Italian patients. METHODS: Anagraphical, biochemical, genetic, clinical and instrumental data were collected at diagnosis and during a long-term follow-up. Mutations, ferroxidase activity and Western Blot analysis of ceruloplasmin were performed according to standard protocols...
February 24, 2018: Parkinsonism & related Disorders
Marc C Hines, Herbert L Bonkovsky, Sean R Rudnick, Justin T Mhoon
No abstract text is available yet for this article.
February 27, 2018: Annals of Internal Medicine
F A Guardiola, A Bahi, A M Jiménez-Monreal, M Martínez-Tomé, M A Murcia, M A Esteban
Improving fish defense through the preventive administration of immunostimulants has an important role in controlling the outbreak of the disease in aquaculture. As a continuity of our previous studies, this paper describes the effects of dietary fenugreek (Trigonella foenum graecum) seeds on the skin mucosal antioxidant status and immune response of gilthead seabream (Sparus aurata L.). Fish were fed with four experimental diets: one a basal diet (control) and three diets with powdered fenugreek seeds incorporated in the fish feed at 1%, 5%, and 10%...
February 17, 2018: Fish & Shellfish Immunology
Wouter C Meijers, Manuel Maglione, Stephan J L Bakker, Rupert Oberhuber, Lyanne M Kieneker, Steven de Jong, Bernhard J Haubner, Wouter B Nagengast, Alexander R Lyon, Bert van der Vegt, Dirk J van Veldhuisen, B Daan Westenbrink, Peter van der Meer, Herman H W Silljé, Rudolf A de Boer
Background -Heart failure (HF) survival has improved and nowadays many patients with HF die from non-cardiac causes, including cancer. Our aim was to investigate whether a causal relationship exists between HF and the development of cancer. Methods -HF was induced by inflicting large anterior myocardial infarction (MI) in APCmin mice, which are prone to develop precancerous intestinal tumors, and tumor growth was measured. In addition, to rule out hemodynamic impairment, a heterotopic heart transplantation model was employed, where an infarcted or sham-operated heart was transplanted into a recipient mouse, while the native heart was left in situ...
February 19, 2018: Circulation
Maria Carmela Bonaccorsi di Patti, Antimo Cutone, Fabio Polticelli, Luigi Rosa, Maria Stefania Lepanto, Piera Valenti, Giovanni Musci
In the last 20 years, several new genes and proteins involved in iron metabolism in eukaryotes, particularly related to pathological states both in animal models and in humans have been identified, and we are now starting to unveil at the molecular level the mechanisms of iron absorption, the regulation of iron transport and the homeostatic balancing processes. In this review, we will briefly outline the general scheme of iron metabolism in humans and then focus our attention on the cellular iron export system formed by the permease ferroportin and the ferroxidase ceruloplasmin...
February 16, 2018: Biometals: An International Journal on the Role of Metal Ions in Biology, Biochemistry, and Medicine
Dhaval Patel, Chuan Xu, Sureshbabu Nagarajan, Zhengchang Liu, Wayne O Hemphill, Runhua Shi, Vladimir N Uversky, Guy A Caldwell, Kim A Caldwell, Stephan N Witt
We probed the role of alpha-synuclein (α-syn) in modulating sorting nexin 3 (Snx3)-retromer-mediated recycling of iron transporters in Saccharomyces cerevisiae and Caenorhabditis elegans. In yeast, the membrane-bound heterodimer Fet3/Ftr1 is the high affinity iron importer. Fet3 is a membrane-bound multicopper ferroxidase, whose ferroxidase domain is orthologous to human ceruloplasmin (Cp), that oxidizes external Fe+2 to Fe+3; the Fe+3 ions then channel through the Ftr1 permease into the cell. When the concentration of external iron is low (< 1 µM), Fet3/Ftr1 is maintained on the plasma membrane by retrograde endocytic-recycling; whereas, when the concentration of external iron is high (> 10 µM), Fet3/Ftr1 is endocytosed and shunted to the vacuole for degradation...
February 14, 2018: Human Molecular Genetics
Ayako Yamamura, Yoshitaka Kikukawa, Kenji Tokunaga, Eiko Miyagawa, Shinya Endo, Hirosada Miyake, Hiroyuki Hata, Hiroaki Mitsuya, Kunihiro Yoshida, Masao Matsuoka
A 72-year-old Japanese woman suffered from mild pancytopenia 3 years before her initial hospitalization. On admission, the levels of trace elements, particularly copper, and ceruloplasmin were significantly decreased in her blood serum. Abdominal lymphadenopathy and bone marrow dysplasia were detected. Hemosiderin deposition was observed in her lymph nodes and bone marrow, and magnetic resonance imaging suggested its deposition in various organs. A novel missense pathogenic variant (c.T1670G) was detected in the ceruloplasmin gene, resulting in an amino acid change (p...
February 9, 2018: Internal Medicine
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