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Jan Pfeiffenberger, Christine Marie Lohse, Daniel Gotthardt, Christian Rupp, Markus Weiler, Ulrike Teufel, Karl Heinz Weiss, Annika Gauss
OBJECTIVE: Urinary copper excretion rates and non-caeruloplasmin associated copper concentrations are increased in patients with Wilson disease. However, there is little literature describing the monitoring of these parameters over the long term. METHODS: This is a monocentric retrospective study including data collected between 2003 and 2015 from 321 patients with Wilson disease by chart review. The patients were under therapy with D-penicillamine, trientine, or zinc...
February 11, 2019: Journal of Inherited Metabolic Disease
Yumi Abiko, Yumi Nakai, Nho Cong Luong, Christopher L Bianco, Jon M Fukuto, Yoshito Kumagai
We previously reported that 9,10-phenanthraquinone (9,10-PQ), an atmospheric electron acceptor, undergoes redox cycling with dithiols as electron donors, resulting in the formation of semiquinone radicals and monothiyl radicals; however, monothiols have little reactivity. Because persulfide and polysulfide species are highly reducing, we speculate that 9,10-PQ might undergo one-electron reduction with these reactive sulfides. In the present study, we explored the redox cycling capability of a variety of quinone-related electron acceptors, including 9,10-PQ, during interactions with the hydropersulfide Na2 S2 and its related polysulfides...
February 5, 2019: Chemical Research in Toxicology
Muzamil Amin Chisti, Yousef Binamer, Abdullah Alfadley, Abdulmonem Almutawa
D-penicillamine (DPA)-induced pseudo-pseudoxanthoma elasticum (PXE) and elastosis perforans serpiginosa (EPS) has been reported in the past, but most of the treatment modalities used before have a sub.optimal response. We report a case of DPA-induced pseudo-PXE with extensive EPS who had an excellent rapid response to acitretin. To the best of our knowledge no such report has been published in the past, even though there is a single report of effectiveness of isotretinoin in elastosis perforans serpiginosa...
January 2019: Annals of Saudi Medicine
Alaeddin B Abukabda, Elizabeth C Bowdridge, Carroll R McBride, Thomas P Batchelor, William T Goldsmith, Krista L Garner, Sherri Friend, Timothy R Nurkiewicz
The fetal consequences of gestational engineered nanomaterial (ENM) exposure are unclear. The placenta is a barrier protecting the fetus and allowing transfer of substances from the maternal circulation. The purpose of this study was to determine the effects of maternal pulmonary titanium dioxide nanoparticle (nano-TiO2 ) exposure on the placenta and umbilical vascular reactivity. We hypothesized that pulmonary nano-TiO2 inhalation exposure increases placental vascular resistance and impairs umbilical vascular responsiveness...
January 31, 2019: Toxicology and Applied Pharmacology
Ting Wan, Fenglin Tang, Yanru Yin, Maoxue Zhang, Martin M F Choi, Xiupei Yang
Recently, water-soluble gold nanoclusters (AuNCs) have attracted more and more attention due to their unique properties. In this study, penicillamine-protected gold nanoclusters (Pen-AuNCs) were synthesized and initially fractionated by sequential size-selective precipitation (SSSP). The crude Pen-AuNCs and SSSP fractions were separated by capillary zone electrophoresis (CZE) with a diode array detector. The effects of key parameters, including the concentration of phosphate buffer, pH value and the ethanol content were systematically investigated...
January 25, 2019: Electrophoresis
Noppharat Phadungcharoen, Prasopchai Patrojanasophon, Praneet Opanasopit, Tanasait Ngawhirunpat, Akhayachatra Chinsriwongkul, Theerasak Rojanarata
A new, smartphone-based colourimetric method for the assay of d-penicillamine formulations relying on the Ellman's reaction was developed by performing the colourimetric reaction in a microplate. Subsequently, the plate was positioned on a white illuminating screen of an iPad placed in a dark box in order to capture a top-view image using an iPhone 5s back camera. The intensity of yellow colour was converted to Red-Green-Blue pixels using a free mobile application. Under the optimal conditions for the reaction and photography, the intensity of blue colour, which was logarithmically transformed, showed an excellent linearity over the drug concentration range of 5-40 µg/mL...
January 9, 2019: International Journal of Pharmaceutics
Mitsutaka Fukudome, Eri Watanabe, Ken-Ichi Osuki, Ryujiro Imaizumi, Toshio Aoki, Manuel Becana, Toshiki Uchiumi
The class 1 phytoglobin, LjGlb1-1, is expressed in various tissues of the model legume Lotus japonicus, where it may play multiple functions by interacting with nitric oxide (NO). One of such functions is the onset of a proper symbiosis with Mesorhizobium loti resulting in the formation of actively N2-fixing nodules. Stable overexpression lines (Ox1 and Ox2) of LjGlb1-1 were generated and phenotyped. Both Ox lines showed reduced NO levels in roots and enhanced nitrogenase activity in mature and senescent nodules relative to the wild-type (WT)...
December 28, 2018: Plant & Cell Physiology
Victoria Mainardi, Karina Rando, Marcelo Valverde, Daniela Olivari, Jorge Castelli, Gabriela Rey, Solange Gerona
INTRODUCTION AND AIM: Wilson's disease (WD) is an uncommon cause of acute liver failure (ALF). Our aim was to describe clinical features, diagnostic findings, treatments, and outcomes of patients with ALF due to WD. MATERIAL AND METHODS: Retrospective medical record reviews of all patients with ALF due to WD in eight years in Uruguay. RESULTS: WD was the cause of six (15%) of thirty-nine ALF cases. All patients were females, with a mean age of 18 years...
December 13, 2018: Annals of Hepatology
Jayantee Kalita, Usha K Misra, Vijay Kumar, Vasudev Parashar
BACKGROUND: There is paucity of studies on predictors of seizures in Wilson disease with neurological manifestation (WDNM), and none has evaluated the role of copper (Cu) induced oxidative stress, proinflammatory and excitotoxicity in the genesis of seizure. OBJECTIVES: To report frequency, refractoriness, and outcome of seizure in WDNM. We also evaluate role of Cu induced oxidative stress, excitotoxicity and cytokines in predicting seizures. METHODS: The diagnosis of WDNM was based on clinical, MRI, KF ring and 24 hour urinary Cu...
December 21, 2018: Neurotoxicology
Kessarin Ngamdee, Kanokwan Chaiendoo, Chatree Saiyasombat, Wutthikrai Busayaporn, Somlak Ittisanronnachai, Vinich Promarak, Wittaya Ngeontae
A highly selective circular dichroism sensor based on cysteamine-capped cadmium sulfide quantum dots (Cys-CdS QDs) was successfully developed for the determination of Cu2+ . Basically, the Cys-CdS QDs are not active in circular dichroism spectroscopy. However, the circular dichroism probe (DPA@Cys-CdS QDs) can be simply generated in situ by mixing achiral Cys-CdS QDs with D-penicillamine. The detection principle was based on measuring the circular dichroism signal change upon the addition of Cu2+ . The strong CD signal of the DPA@Cys-CdS QDs dramatically decreased in the presence of Cu2+ , while other cations did not result in any spectral changes...
December 14, 2018: Spectrochimica Acta. Part A, Molecular and Biomolecular Spectroscopy
Joana Vieira Barbosa, Montserrat Fraga, Joan Saldarriaga, Philippe Hiroz, Emiliano Giostra, Christine Sempoux, Peter Ferenci, Darius Moradpour
BACKGROUND AND AIM: Wilson’s disease is an inherited disorder of hepatic copper metabolism, leading to the accumulation of copper in the liver as well as the brain, cornea and other organs. Here, we describe the adult cases of hepatic Wilson’s disease diagnosed at the Division of Gastroenterology and Hepatology of the University Hospital Lausanne, Switzerland between September 2004 and August 2016. METHODS: Clinical manifestations, results of diagnostic tests, management and outcomes of adult patients with hepatic Wilson’s disease were assessed based on standardised medical records...
December 17, 2018: Swiss Medical Weekly
Hajime Asada, Mari Kojima, Takuro Nagahara, Yuko Goto-Koshino, James K Chambers, Taisuke Nakagawa, Nozomu Yokoyama, Kazuyuki Uchida, Hajime Tsujimoto, Koichi Ohno
A 9-month-old intact crossbred female cat was presented with jaundice, intermittent anorexia and lethargy, increased hepatic enzyme activities, and hyperammonemia. Abdominal ultrasound and computed tomographic examinations determined that the liver had a rounded and irregular margin, and histopathological examination identified excessive accumulation of copper hepatocytes in the liver. Concentrations of both blood and urine copper were higher than in healthy cats. The patient responded well to treatment with penicillamine...
December 18, 2018: Journal of Veterinary Internal Medicine
Rao Rao, Shan Shu, Yong Zhu Han, Yu-Jen Chiu, Yong Sheng Han
RATIONALE: Both Wilson disease (WD) and Oculocutaneous Albinism (OCA) are rare autosomal recessive disorders that are caused by mutations on chromosome 13 and chromosome 11, respectively. Here, we report on a patient with coexisting WD and OCA, initially presenting episodes of tremors. PATIENT CONCERNS: WD is a disorder of copper metabolism. The main sites of copper accumulation are the liver and the brain, resulting in hepatic symptoms. OCA is a disorder of melanin biosynthesis, characterized by a generalized reduction in pigmentation of the eyes (oculo-), skin (-cutaneous), and hair...
December 2018: Medicine (Baltimore)
Madelene Lindkvist, Ulrika Fernberg, Liza U Ljungberg, Knut Fälker, Maria Fernström, Anita Hurtig-Wennlöf, Magnus Grenegård
INTRODUCTION: Platelet aggregation and secretion can be induced by a large number of endogenous activators, such as collagen, adenosine diphosphate (ADP) and epinephrine. Conversely, the blood vessel endothelium constitutively release platelet inhibitors including nitric oxide (NO) and prostacyclin. NO and prostacyclin are also well-known vasodilators and contribute to alterations in local blood flow and systemic blood pressure. MATERIALS AND METHODS: In this study we investigated individual variations in platelet reactivity and arterial functions including blood pressure and flow-mediated vasodilation (FMD) in 43 young, healthy individuals participating in the Lifestyle, Biomarkers and Atherosclerosis (LBA) study...
December 6, 2018: Thrombosis Research
Meng-Che Lu, Shyh-Dar Shyur, Lee-Wen Lee, Timothy Hsu
Generalised morphea (GM) is a subtype of localised scleroderma that usually manifests with bilateral involvement. Unilateral generalised morphea (UGM) is a rare variant of GM. This is a case report of a Taiwanese girl with UGM over the left side of her body. She presented with hyperpigmentation, tightness, and skin atrophy over the left extremities and trunk. Mild range of motion (ROM) limitation over the left knee was also noted. At the clinic, the patient was given oral prednisolone, oral methotrexate (MTX), and oral D-penicillamine...
December 9, 2018: Asian Pacific Journal of Allergy and Immunology
Lu Huang, Qi Lin, Yanxia Li, Guocai Zheng, Yiting Chen
Two new chiral membranes were prepared by modification of gold nanochannel membranes with D-penicillamine and N-acetyl-L-cysteine and were characterized by scanning electron microscopy and X-ray photoelectron spectroscopy. The effects of key factors such as the gold deposition time, the pH, and the concentration of sodium dihydrogen phosphate on the separation factor are discussed. Chiral resolution of amino acid enantiomers by the chiral membranes was investigated. The experimental results show that the D-penicillamine-modified membrane has good enantioselectivity toward tyrosine and phenylalanine enantiomers, whereas the N-acetyl-L-cysteine-modified membrane has good enantioselectivity toward tyrosine and tryptophan enantiomers...
November 19, 2018: Analytical and Bioanalytical Chemistry
Richard Seto, Lauren M Mathias, Andrew Kelly, Richard S Panush
We cared for a woman with sero-positive rheumatoid arthritis (RA), in clinical remission on oral methotrexate (MTX) and hydroxychloroquine, who wished to donate a kidney to a brother with end-stage renal disease (ESRD). We could find scant literature about this unusual clinical circumstance, and therefore review pertinent aspects of renal disease in RA, perioperative medical management, maintenance of disease remission, outcomes for RA patients who have donated kidneys, and relevant ethical issues. Renal complications in RA are not uncommon, with as many as 50% of patients at risk of reduced eGFR...
January 2019: Clinical Rheumatology
Zahra Shojaeifard, Nahid Heidari, Bahram Hemmateenejad
Assays of ferric ion (Fe3+ ) with high sensitivity and selectivity have been required to evaluate its amount in environmental and biological systems. Herein, a novel fluorometric penicillamine-capped bimetallic gold-copper nanoclusters (PA-AuCu bi-MNCs) sensor was constructed for facile, environmentally friendly and quantitative detection of Fe3+ through inner filter effect (IFE) mechanism. One-step green synthetic approach was applied for the synthesis of AuCu bi-MNCs by using d-penicillamine (D-PA) as template and stabilizer...
February 15, 2019: Spectrochimica Acta. Part A, Molecular and Biomolecular Spectroscopy
Jakob Damsgaard, Fin Stolze Larsen, Henriette Ytting
Wilson's disease (WD) is a genetic disorder resulting in accumulation of copper in the liver and other organs. When presenting as acute liver failure (WD-ALF), urgent orthotopic liver transplantation (OLT) is considered the only life-saving option. The revised WD prognostic index is highly accurate in predicting death due to WD-ALF (1,2). For a score ≥11 acute listing for OLT is recommended in European and American guidelines. Here we report a patient with WD-ALF and poor prognostic score that was rescued by high-volume plasma exchange (HVP)...
October 23, 2018: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
Hongwei Ge, Kui Zhang, Huan Yu, Ji Yue, Long Yu, Xinfeng Chen, Tianxin Hou, Khalid A Alamry, Hadi M Marwani, Suhua Wang
This paper reported a dual-mode probe for D-penicillamine on the basis of pH-mediated gold nanoparticles aggregation and fluorescence resonance energy transfer (FRET) from carbon dots. D-penicillamine is a zwitterionic compound and has different forms depending on specific pH ranges. The thiol group of D-penicillamine has high affinity towards the surface of gold nanoparticles and can replace other surface ligands. When pH values were close to its isoelectrical point (pH(I)), the D-penicillamine capped gold nanoparticles aggregated through hydrogen bonding or electrostatic interactions, resulting in the releasing of carbon dots from gold nanoparticles...
November 2018: Journal of Fluorescence
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