Nutrition

Malnutrition, health and survival?, Measuring nutritional status?, Pathophysiological consequences of severe malnutrition?, Clinical assessment of nutrition?, Medical management within inpatient therapeutic nutrition programmes?, Inpatient therapeutic nutrition programme?, Outpatient therapeutic nutrition programme?, Supplementary feeding programmes?, HIV/AIDS and malnutrition?, Severe malnutrition in infants months old?, Pregnancy?, Nutrition in emergencies?, Recipes and formulas for management of malnourished children?, Vitamin A deficiency?, Vitamin B thiamine deficiency: beriberi?, Vitamin B riboflavin deficiency?, Vitamin B niacin deficiency: pellagra?, Vitamin B pyridoxine deficiency?, Vitamin B deficiency?, Folate deficiency?, Vitamin C deficiency: scurvy?, Vitamin D deficiency: rickets/osteomalacia?, Vitamin E alpha-tocopherol deficiency?, Vitamin K deficiency?, Iodine deficiency?, Zinc xxx, Other micronutrients?, Obesity?

Pyridoxine Deficiency Exacerbates Neuronal Damage after Ischemia by Increasing Oxidative Stress and Reduces Proliferating Cells and Neuroblasts in the Gerbil Hippocampus

We investigated the effects of pyridoxine deficiency on ischemic neuronal death in the hippocampus of gerbil (n = 5 per group). Serum pyridoxal 5′-phosphate levels were significantly decreased in Pyridoxine-deficient diet (PDD)-fed gerbils, while homocysteine levels were significantly increased in sham- and ischemia-operated gerbils. PDD-fed gerbil showed a reduction in neuronal nuclei (NeuN)-immunoreactive neurons in the medial part of the hippocampal CA1 region three days after. Reactive astrocytosis and microgliosis were found in PDD-fed gerbils, and transient ischemia caused the aggregation of activated microglia in the stratum pyramidale three days after ischemia. Lipid peroxidation was prominently increased in the hippocampus and was significantly higher in PDD-fed gerbils than in Control diet (CD)-fed gerbils after ischemia. In contrast, pyridoxine deficiency decreased the proliferating cells and neuroblasts in the dentate gyrus in sham- and ischemia-operated gerbils. Nuclear factor erythroid-2-related factor 2 (Nrf2) and brain-derived neurotrophic factor (BDNF) levels also significantly decreased in PDD-fed gerbils sham 24 h after ischemia. These results suggest that pyridoxine deficiency accelerates neuronal death by increasing serum homocysteine levels and lipid peroxidation, and by decreasing Nrf2 levels in the hippocampus. Additionally, it reduces the regenerated potentials in hippocampus by decreasing BDNF levels. Collectively, pyridoxine is an essential element in modulating cell death and hippocampal neurogenesis after ischemia.

Status of water-soluble vitamins and neurological disorders in dialysis patients

The deficit of vitamins in patients receiving the long-term hemodialysis is discussed in the modern literature. Vitamin deficiency in a dialysis patient can be explained by the peculiarity of the diet recommendations, the need to take a number of medications, impaired absorption of vitamins in the digestive tract, poor appetite, uremic anorexia, depression, limited ability to buy and cook food, as well as losses of vitamins during the procedure of program hemodialysis. An analytical review of current (2011 and later) publications containing a comprehensive analysis of data on the status of water-soluble vitamins and its role in the development of neurological disorders in dialysis patients is provided. There is a high risk of deficiency of various water soluble vitamins and neurological disorders, such as vitamin B1 deficiency and thiamine deficiency encephalopathy and polyneuropathy, vitamin B6 deficiency and pyridoxine deficiency polyneuropathy, folic acid metabolism disorders, as well as vitamin B12 and the development of hyperhomocysteinemia, cognitive and depressive disorders, strokes, restless legs syndrome and dialysis polyneuropathy among the patients with end-stage chronic kidney disease and program hemodialysis. Vitamin C deficiency and the development of severe asthenic syndrome with insomnia and depression are described in dialysis patients. It seems necessary to revise the traditional nutritional approaches to the dialysis patients based on the analysis of the literature. Special attention is paid to the possible addition of such water-soluble vitamins as B1, B6, B9, B12 and C. Timely diagnosis of vitamin deficiency conditions and neurological disorders in patients on program hemodialysis, the development of methods for their correction and their introduction into clinical practice would improve the life expectancy and quality of life of dialysis patients.

Effects of Pyridoxine Deficiency on Hippocampal Function and Its Possible Association with V-Type Proton ATPase Subunit B2 and Heat Shock Cognate Protein 70

Pyridoxine, one of the vitamin B6 vitamers, plays a crucial role in amino acid metabolism and synthesis of monoamines as a cofactor. In the present study, we observed the effects of pyridoxine deficiency on novel object recognition memory. In addition, we examined the levels of 5-hydroxytryptamine (5-HT), 5-hydroxyindoleacetic acid (5-HIAA), 3,4-dihydroxyphenethylamine (DA), 3,4-dihydroxyphenylacetic acid, and homovanillic acid and the number of proliferating cells and neuroblasts in the hippocampus. We also examined the effects of pyridoxine deficiency on protein profiles applying a proteomic study. Five-week-old mice fed pyridoxine-deficient diets for 8 weeks and showed a significant decrease in the serum and brain (cerebral cortex, hippocampus, and thalamus) levels of pyridoxal 5′-phosphate, a catalytically active form of vitamin-B6, and decline in 5-HT and DA levels in the hippocampus compared to controls fed a normal chow. In addition, pyridoxine deficiency significantly decreased Ki67-positive proliferating cells and differentiated neuroblasts in the dentate gyrus compared to controls. A proteomic study demonstrated that a total of 41 spots were increased or decreased more than two-fold. Among the detected proteins, V-type proton ATPase subunit B2 (ATP6V1B2) and heat shock cognate protein 70 (HSC70) showed coverage and matching peptide scores. Validation by Western blot analysis showed that ATP6V1B2 and HSC70 levels were significantly decreased and increased, respectively, in pyridoxine-deficient mice compared to controls. These results suggest that pyridoxine is an important element of novel object recognition memory, monoamine levels, and hippocampal neurogenesis. Pyridoxine deficiency causes cognitive impairments and reduction in 5-HT and DA levels, which may be associated with a reduction of ATP6V1B2 and elevation of HSC70 levels in the hippocampus.

Novel variants in a patient with late-onset hyperprolinemia type II: diagnostic key for status epilepticus and lactic acidosis

Background Hyperprolinemia type 2 (HPII) is a rare autosomal recessive disorder of the proline metabolism, that affects the ALDH4A1 gene. So far only four different pathogenic mutations are known. The manifestation is mostly in neonatal age, in early infancy or early childhood. Case presentation The 64-years female patient had a long history of abdominal pain, and episode of an acute neuritis. Ten years later she was admitted into the neurological intensive-care-unit with acute abdominal pain, multiple generalized epileptic seizures, a vertical gaze palsy accompanied by extensive lactic acidosis in serum 26.0 mmol/l (reference: 0.55–2.2 mmol/l) and CSF 12.01 mmol/l (reference: 1.12–2.47 mmol/l). Due to repeated epileptic seizures and secondary complications a long-term sedation with a ventilation therapy over 20 days was administered. A diagnostic work-up revealed up to 400-times increased prolin-level in urine CSF and blood. Furthermore, a low vitamin-B6 serum value was found, consistent with a HPII causing secondary pyridoxine deficiency and seizures. The ALDH4A1 gene sequencing confirmed two previously unknown compound heterozygous variants (ALDH4A1 gene (NM_003748.3) Intron 1: c.62 + 1G > A - heterozygous and ALDH4A1 gene (NM_003748.3) Exon 5 c.349G > C, p.(Asp117His) - heterozygous). Under high-dose vitamin-B6 therapy no further seizures occurred. Conclusion We describe two novel ALDH4A1-variants in an adult patient with hyperprolinemia type II causing secondary pyridoxine deficiency and seizures. Severe and potentially life-threatening course of this treatable disease emphasizes the importance of diagnostic vigilance and thorough laboratory work-up including gene analysis even in cases with atypical late manifestation.