BACKGROUND Cobalamin and folate are essential micronutrients and are important in DNA and RNA synthesis, cell proliferation, growth, hematopoiesis, and cognitive function. However, data on cobalamin and folate status are lacking particularly from young children residing in low and middle income countries. OBJECTIVE To measure cobalamin and folate status and identifies their predictors among 6...

RATIONALE Methylmalonic acidemia (MMA) is a common organic acidemia, mainly due to methylmalonyl-CoA mutase (MCM) or its coenzyme cobalamin (VitB12) metabolic disorders. Cobalamin C (CblC) type is the most frequent inborn error of cobalamin metabolism; it can develop symptoms in childhood and often combine multisystem damage, which leads to methylmalonic acid, propionic acid, methyl citrate, an...

BACKGROUND Supplementation with high doses of oral cobalamin is as effective as cobalamin administered by intramuscular injection to correct plasma markers of vitamin B(12) deficiency, but the effects of lower oral doses of cobalamin on such markers are uncertain. METHODS We conducted a randomized, parallel-group, double-blind, dose-finding trial to determine the lowest oral dose of cyanocoba...

OBJECTIVE Methylmalonic acidemia is one of the inborn errors of metabolism resulting in the accumulation of acylcarnitine in blood and increased urinary methylmalonic acid excretion. This disorder can have symptoms, such as neurological and gastrointestinal manifestations, lethargy, and anorexia. MATERIALS & METHODS The patients who were diagnosed as methylmalonic acidemia in the Neurology De...

background : evaluation of exposition to volatile organic compounds (voc) and compare urinary level of urinary biological exposure index in sweepers in west of iran . methods: this study was carried out on 40 street sweepers and 40 non-exposed as control group living in hamadan city, west of iran. voc was analyzed using gas chromatography equipped with a flame ionization detector (fid). the uri...

Methylmalonic acid (MMA) concentrations are increased in cobalamin (vitamin B12) deficiency, but the relative diagnostic usefulness of determination of MMA in serum vs urine has not yet been assessed. We obtained urine collections and matched serum samples from 28 healthy volunteers and from 20 consecutive patients admitted for clinical and hematological evaluation because of low cobalamin conc...

Cobalamin neuropathy was produced in Cape fruit bats (Rousettus aegyptiacus) by a cobalamin-free diet combined with intermittent exposure to nitrous oxide, which inactivates cobalamin. Cobalamin-deficient bats had low hepatic methylmalonyl-CoA mutase holoenzyme activity, with elevated plasma and urinary methylmalonic acid levels. No significant changes could be demonstrated in the concentration...

Methylmalonic aciduria is an inborn error in the organic acids metabolism. Deficiency of methylmalonyl CoA mutase or its coenzyme, adenosylcobalamine, leads to accumulation of methymalonic acid in body fluids. The disease manifests with recurrent episodes of dehydration, metabolic acidosis, coma and death. We report a case of methylmalonic aciduria diagnosed in a female infant who presented wit...