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Hydroxocobalamin

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https://www.readbyqxmd.com/read/27905001/guidelines-for-diagnosis-and-management-of-the-cobalamin-related-remethylation-disorders-cblc-cbld-cble-cblf-cblg-cblj-and-mthfr-deficiency
#1
Martina Huemer, Daria Diodato, Bernd Schwahn, Manuel Schiff, Anabela Bandeira, Jean-Francois Benoist, Alberto Burlina, Roberto Cerone, Maria L Couce, Angeles Garcia-Cazorla, Giancarlo la Marca, Elisabetta Pasquini, Laura Vilarinho, James D Weisfeld-Adams, Viktor Kožich, Henk Blom, Matthias R Baumgartner, Carlo Dionisi-Vici
BACKGROUND: Remethylation defects are rare inherited disorders in which impaired remethylation of homocysteine to methionine leads to accumulation of homocysteine and perturbation of numerous methylation reactions. OBJECTIVE: To summarise clinical and biochemical characteristics of these severe disorders and to provide guidelines on diagnosis and management. DATA SOURCES: Review, evaluation and discussion of the medical literature (Medline, Cochrane databases) by a panel of experts on these rare diseases following the GRADE approach...
November 30, 2016: Journal of Inherited Metabolic Disease
https://www.readbyqxmd.com/read/27859624/hemolysis-index-to-detect-degree-of-hydroxocobalamin-interference-with-common-laboratory-tests
#2
Laura Fueyo, Juan Robles, Irene Aguilar, Aina M Yáñez, Magdalena Socias, Magdalena Parera
BACKGROUND: Cyanokit(®) (hydroxocobalamin OHCo) is the recommended treatment for cyanide poisoning. OHCo is a red chromophore and may cause interference with some biochemical measurements. In this study, we assessed the possible interference of Cyanokit on several cooximetric and plasma biochemistry tests and then determined the possible mathematical correction for some analytes. We studied the possibility of detecting and evaluating the degree of interference with the hemolysis index (HI) provided by our autoanalyzer because it is not possible to measure the OHCo concentration in conventional laboratories...
November 10, 2016: Journal of Clinical Laboratory Analysis
https://www.readbyqxmd.com/read/27858373/vitamin-b12-administration-by-subcutaneous-catheter-device-in-a-cobalamin-a-cbla-patient
#3
E Maines, G Morandi, G Gugelmo, F Ion-Popa, N Campostrini, A Pasini, M Vincenzi, F Teofoli, M Camilot, A Bordugo
Cobalamin A deficiency (cblA) is an inherited disorder of intracellular cobalamin metabolism, caused by impaired 5'-deoxy-adenosylcobalamin (AdoCbl) synthesis. Hydroxocobalamin (OHCbl) is the cornerstone of cblA treatment because vitamin B12 may completely restore AdoCbl deficiency. Parenteral administration, intravenous, subcutaneous or intramuscular, is generally required to achieve effect. Daily injections represent a problem for the parents and the caregivers, and this may lead to poor compliance and scarce adherence to the long-term treatment...
November 18, 2016: JIMD Reports
https://www.readbyqxmd.com/read/27851628/1994-hydroxocobalamin-for-the-treatment-of-refractory-hypotension-in-a-patient-with-septic-shock
#4
Annie Biesboer, Michael Katz, Amit Taneja
No abstract text is available yet for this article.
December 2016: Critical Care Medicine
https://www.readbyqxmd.com/read/27850516/878-treatment-of-refractory-vasoplegic-shock-with-hydroxocobalamin-and-methylene-blue
#5
Joel Feih, Joseph Rinka, Michael Zundel, Markus Kaiser
No abstract text is available yet for this article.
December 2016: Critical Care Medicine
https://www.readbyqxmd.com/read/27849860/218-evaluation-of-hydroxocobalamin-in-vasoplegia-in-cardiac-surgery
#6
Sarah Klemm, Craig Glienke
No abstract text is available yet for this article.
December 2016: Critical Care Medicine
https://www.readbyqxmd.com/read/27749291/hydroxocobalamin-for-vasoplegic-syndrome-in-liver-transplantation-restoration-of-blood-pressure-without-vasospasm
#7
Harvey J Woehlck, Brent T Boettcher, Kathryn K Lauer, David C Cronin, Johnny C Hong, Michael A Zimmerman, Joohyun Kim, Motaz Selim
Systemic vasoplegia is common in patients undergoing liver transplantation. In this report, we present a case in which treatment with conventional vasopressors caused peripheral arterial spasm, rendering arterial blood pressure monitoring impossible. Administration of methylene blue resolved the vasospasm; however, concern for toxic dose requirements limited its use. Hydroxocobalamin administration resolved the vasospasm and increased blood pressure without the potential adverse effects seen with methylene blue...
October 5, 2016: A & A Case Reports
https://www.readbyqxmd.com/read/27568998/vitamin-b12-induced-spermatogenesis-recovery-in-cimetidine-treated-rats-effect-on-the-spermatogonia-number-and-sperm-concentration
#8
Flávia L Beltrame, Estela Sasso-Cerri
The H 2 -receptor antagonist cimetidine is an antiulcer drug also used for the treatment of cancer due to its antiangiogenic effect. However, this drug has caused structural changes in the seminiferous tubules. Vitamin B 12 has been used as a therapeutic agent for the treatment of male infertility. The supplementation of rats with vitamin B 12 during cimetidine treatment has recovered the damaged seminiferous tubules, but how this vitamin restores the seminiferous epithelium has not been clarified. In this study, we evaluated whether vitamin B 12 improves the number of spermatogonia, spermatocytes, and sperm concentration in cimetidine-treated rats...
August 26, 2016: Asian Journal of Andrology
https://www.readbyqxmd.com/read/27554631/utility-and-outcomes-of-hydroxocobalamin-use-in-smoke-inhalation-patients
#9
Lyly Nguyen, Ashkan Afshari, Steven A Kahn, Stuart McGrane, Blair Summitt
INTRODUCTION: Hydroxocobalamin has been available for use for suspected cyanide toxicity in smoke inhalation patients in the United States since 2006. Our study compares outcomes of patients who received hydroxocobalamin to historical controls who did not. METHODS: In this retrospective review, patients administered hydroxocobalamin (2008-2014) were compared to historical controls (2002-2008). Patients <18 years, patients who received an alternate antidote, and patients without suspicion of smoke inhalation injury were excluded...
August 20, 2016: Burns: Journal of the International Society for Burn Injuries
https://www.readbyqxmd.com/read/27519416/novel-mouse-models-of-methylmalonic-aciduria-recapitulate-phenotypic-traits-with-a-genetic-dosage-effect
#10
Patrick Forny, Anke Schumann, Merima Mustedanagic, Déborah Mathis, Marie-Angela Wulf, Nadine Nägele, Claus-Dieter Langhans, Assem Zhakupova, Joerg Heeren, Ludger Scheja, Ralph Fingerhut, Heidi L Peters, Thorsten Hornemann, Beat Thony, Stefan Kölker, Patricie Burda, D Sean Froese, Olivier Devuyst, Matthias R Baumgartner
Methylmalonic aciduria (MMAuria), caused by deficiency of methylmalonyl-CoA mutase (MUT), usually presents in the newborn period with failure to thrive and metabolic crisis leading to coma or even death. Survivors remain at risk of metabolic decompensations and severe long term complications, notably renal failure and neurological impairment. We generated clinically relevant mouse models of MMAuria using a constitutive Mut knock-in (KI) allele based on the p.Met700Lys patient mutation, used homozygously (KI/KI) or combined with a knockout allele (KO/KI), to study biochemical and clinical MMAuria disease aspects...
September 23, 2016: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/27416488/hibiscus-sabdariffa-increases-hydroxocobalamin-oral-bioavailability-and-clinical-efficacy-in-vitamin-b12-deficiency-with-neurological-symptoms
#11
Zouhayr Souirti, Mouna Loukili, Imar D Soudy, Kaies Rtibi, Aslihan Özel, Nicolas Limas-Nzouzi, Seloua El Ouezzani, Bruno Eto
The aim of the study was to evaluate the bioavailability and clinical benefits of oral new formulation (HB12 ) of hydroxocobalamin (Hdrx) with Hibiscus sabdariffa (HS). First, in an observational study, a cohort of 30 vitamin B12 -deficient patients (vit B12 < 200 pg/mL) with neurological symptoms received oral fixed dose of Hdrx containing 15 mg Hdrx daily for 10 days followed by 15 mg monthly. Clinical benefits were evaluated on haematological and biochemical parameters, and neurological improvement at days 10 and 90 compared to day 0...
December 2016: Fundamental & Clinical Pharmacology
https://www.readbyqxmd.com/read/27355231/sublingual-vitamin-b12-compared-to-intramuscular-injection-in-patients-with-type-2-diabetes-treated-with-metformin-a-randomised-trial
#12
Amber Parry-Strong, Fali Langdana, Sylvan Haeusler, Mark Weatherall, Jeremy Krebs
UNLABELLED: MAIM: To compare a single 1mg intramuscular hydroxocobalamin injection with a 3-month course of 1mg/day sublingual methylcobalamin supplements on serum vitamin B12 concentrations in participants withtype 2 diabetes treated with metformin. METHOD: Participants on metformin treatment with vitamin B12 concentrations below 220pmol/L were recruited through hospital diabetes clinics and primary care practices. They were randomised to receive either the injection or sublingual treatment...
2016: New Zealand Medical Journal
https://www.readbyqxmd.com/read/27338592/wine-colored-plasma-and-urine-from-hydroxocobalamin-treatment
#13
Sophia L Wong, Morris Pudek, Dailin Li
No abstract text is available yet for this article.
June 23, 2016: Journal of General Internal Medicine
https://www.readbyqxmd.com/read/27065874/the-anti-fibrotic-actions-of-relaxin-are-mediated-through-a-no-sgc-cgmp-dependent-pathway-in-renal-myofibroblasts-in-vitro-and-enhanced-by-the-no-donor-diethylamine-nonoate
#14
Chao Wang, Barbara K Kemp-Harper, Martina Kocan, Sheng Yu Ang, Tim D Hewitson, Chrishan S Samuel
INTRODUCTION: The anti-fibrotic hormone, relaxin, has been inferred to disrupt transforming growth factor (TGF)-β1/Smad2 phosphorylation (pSmad2) signal transduction and promote collagen-degrading gelatinase activity via a nitric oxide (NO)-dependent pathway. Here, we determined the extent to which NO, soluble guanylate cyclase (sGC) and cyclic guanosine monophosphate (cGMP) were directly involved in the anti-fibrotic actions of relaxin using a selective NO scavenger and sGC inhibitor, and comparing and combining relaxin's effects with that of an NO donor...
2016: Frontiers in Pharmacology
https://www.readbyqxmd.com/read/26989453/alternatively-spliced-methionine-synthase-in-sh-sy5y-neuroblastoma-cells-cobalamin-and-gsh-dependence-and-inhibitory-effects-of-neurotoxic-metals-and-thimerosal
#15
Mostafa Waly, Verna-Ann Power-Charnitsky, Nathaniel Hodgson, Alok Sharma, Tapan Audhya, Yiting Zhang, Richard Deth
The folate and cobalamin (Cbl-) dependent enzyme methionine synthase (MS) is highly sensitive to oxidation and its activity affects all methylation reactions. Recent studies have revealed alternative splicing of MS mRNA in human brain and patient-derived fibroblasts. Here we show that MS mRNA in SH-SY5Y human neuroblastoma cells is alternatively spliced, resulting in three primary protein species, thus providing a useful model to examine cofactor dependence of these variant enzymes. MS activity was dependent upon methylcobalamin (MeCbl) or the combination of hydroxocobalamin (OHCbl) and S-adenosylmethionine (SAM)...
2016: Oxidative Medicine and Cellular Longevity
https://www.readbyqxmd.com/read/26988471/stability-of-added-and-in-situ-produced-vitamin-b12-in-breadmaking
#16
Minnamari Edelmann, Bhawani Chamlagain, Marco Santin, Susanna Kariluoto, Vieno Piironen
Vitamin B12 exists naturally in foods of animal origin and is synthesised only by certain bacteria. New food sources are needed to ensure vitamin B12 intake in risk groups. This study aimed to investigate the stability of added cyanocobalamin (CNCbl, chemically modified form) and hydroxocobalamin (OHCbl, natural form) and in situ-synthesised vitamin B12 in breadmaking. Samples were analysed both with a microbiological (MBA) and a liquid chromatographic (UHPLC) method to test applicability of these two methods...
August 1, 2016: Food Chemistry
https://www.readbyqxmd.com/read/26907495/n-carbamylglutamate-is-an-effective-treatment-for-acute-neonatal-hyperammonaemia-in-a-patient-with-methylmalonic-aciduria
#17
Sufin Yap, Huey Yin Leong, Fadzlina Abdul Aziz, Haszlin Hassim, Pavai Sthaneshwar, Ser Huy Teh, Ili Syazwana Abdullah, Lock Hock Ngu, Zulqarnain Mohamed
N-carbamylglutamate (NCG) has been used in combination with ammonia scavengers (sodium benzoate, sodium phenylbutyrate) and dialysis to treat hyperammonaemia in methylmalonic aciduria (MMA). The sole use of NCG for acute neonatal hyperammonaemia secondary to MMA is demonstrated in a neonate presenting at day 9 with encephalopathy, severe metabolic acidosis, hyperammonaemia (1,089 μmol/l), ketonuria and urinary methylmalonic acids. Emergency treatment included discontinuing protein feeds, providing high calories, carnitine and hydroxocobalamin...
2016: Neonatology
https://www.readbyqxmd.com/read/26891675/risk-of-oxalate-nephropathy-with-the-use-of-cyanide-antidote-hydroxocobalamin-in-critically-ill-burn-patients
#18
LETTER
Matthieu Legrand, Thibault Michel, Michel Daudon, Mourad Benyamina, Axelle Ferry, Sabri Soussi, Véronique Maurel, Maïte Chaussard, Marc Chaouat, Maurice Mimoun, Jérôme Verine, Vincent Mallet, Alexandre Mebazaa
No abstract text is available yet for this article.
June 2016: Intensive Care Medicine
https://www.readbyqxmd.com/read/26865262/photolytic-properties-of-cobalamins-a-theoretical-perspective
#19
Pawel M Kozlowski, Brady D Garabato, Piotr Lodowski, Maria Jaworska
This Perspective Article highlights recent theoretical developments, and summarizes the current understanding of the photolytic properties of cobalamins from a computational point of view. The primary focus is on two alkyl cobalamins, methylcobalamin (MeCbl) and adenosylcobalamin (AdoCbl), as well as two non-alkyl cobalamins, cyanocobalamin (CNCbl) and hydroxocobalamin (HOCbl). Photolysis of alkyl cobalamins involves low-lying singlet excited states where photodissociation of the Co-C bond leads to formation of singlet-born alkyl/cob(ii)alamin radical pairs (RPs)...
March 21, 2016: Dalton Transactions: An International Journal of Inorganic Chemistry
https://www.readbyqxmd.com/read/26735010/recovery-of-elemental-tellurium-nanoparticles-by-the-reduction-of-tellurium-oxyanions-in-a-methanogenic-microbial-consortium
#20
Adriana Ramos-Ruiz, Jim A Field, Jean V Wilkening, Reyes Sierra-Alvarez
This research focuses on the microbial recovery of elemental tellurium (Te(0)) from aqueous streams containing soluble tellurium oxyanions, tellurate (Te(VI)), and tellurite (Te(IV)). An anaerobic mixed microbial culture occurring in methanogenic granular sludge was able to biocatalyze the reduction of both Te oxyanions to produce Te(0) nanoparticles (NPs) in sulfur-free medium. Te(IV) reduction was seven times faster than that of Te(VI), such that Te(IV) did not accumulate to a great extent during Te(VI) reduction...
February 2, 2016: Environmental Science & Technology
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