hydroxocobalamin and Hypertension

Topics: Drug Repositioning; Exanthema; Humans; Hydroxocobalamin; Hypertension; Treatment Outcome; Vasoplegia; Vitamin B Complex

Topics: Adolescent; Biopsy; Complement System Proteins; Female; Hematuria; Humans; Hydroxocobalamin; Hypertension; Intellectual Disability; Kidney; Laser-Doppler Flowmetry; Metabolism, Inborn Errors; Proteinuria; Seizures; Ultrasonography

cblC disease is a cause of hemolytic uremic syndrome (HUS), which has been primarily described in neonates and infants with severe renal and neurological lesions.. Two sisters aged 6 and 8.5 years presented with a latent hemolytic process characterized by undetectable or low plasma haptoglobin, respectively, associated with renal failure and gross proteinuria. Renal biopsies performed in both patients found typical findings of thrombotic microangiopathy suggesting the diagnosis of HUS. Both patients were free of neurologic signs.. Biochemical investigations found a cobalamin processing deficiency of the cblC type. Search for additional factors susceptible to worsen endothelial damage revealed homozygosity 677C--> T mutation in the methylenetetrahydrofolate reductase gene as well as heterozygosity for a 3254T--> C mutation in factor H in the patient with the most severe clinical presentation. Long-term subcutaneous administration of hydroxocobalamin in combination with oral betaine and folic acid resulted in clinical and biological improvement in both patients.. cblC disease may be a cause of chronic HUS with delayed onset in childhood. Superimposed mutation of factor H gene might influence clinical severity.

Topics: Anemia; Betaine; Child; Combined Modality Therapy; Complement Factor H; Drug Therapy, Combination; Endothelium, Vascular; Female; Folic Acid; Genetic Predisposition to Disease; Genotype; Haptoglobins; Hemolytic-Uremic Syndrome; Humans; Hydroxocobalamin; Hypertension; Kidney; Methylenetetrahydrofolate Reductase (NADPH2); Mutation, Missense; Nephrotic Syndrome; Plasma Exchange; Point Mutation; Proteinuria; Proto-Oncogene Proteins; Proto-Oncogene Proteins c-cbl; Renal Dialysis; Vitamin B 12

To review the mechanism of action, safety, and efficacy of hydroxocobalamin in the treatment and prevention of nitroprusside-induced cyanide toxicity.. English and foreign-language journal articles and reference texts identified from Index Medicus. Both animal and human studies were included.. High-dose or prolonged therapy with nitroprusside in patients with hepatic or renal dysfunction increases the risk for nitroprusside-induced cyanide or thiocyanate toxicity, respectively. Hydroxocobalamin has been shown to significantly reduce RBC and plasma cyanide concentrations in animals and surgical patients without producing clinically important adverse effects or toxic metabolites. Thiosulfate infusions also decrease cyanide toxicity but can cause accumulation of thiocyanate resulting in clinical toxicity. Cyanocobalamin cannot effectively remove cyanide due to poor binding.. Hydroxocobalamin is a safe and effective agent in the prevention and treatment of nitroprusside-induced cyanide toxicity. Prolonged or high-dose infusions of nitroprusside should be minimized in critically ill patients, especially if hepatic and/or renal dysfunction is present.

Topics: Animals; Critical Illness; Cyanides; Humans; Hydroxocobalamin; Hypertension; Nitroprusside; Poisoning