Clinical and genetic analysis of a case with Thiamine metabolism dysfunction syndrome 5 / 中华医学遗传学杂志

OBJECTIVE@#To report the clinical manifestation and genetic characteristics of a child with Thiamine metabolism dysfunction syndrome 5.@*METHODS@#Clinical data and genetic results were collected and analyzed. Peripheral blood samples of the child and their parents were collected for whole exome sequencing, and the functional effect of the variants on the TPK1 enzyme activity was verified by an in vitro assay.@*RESULTS@#A four-year-old boy presented with preschool onset of ataxia were characterized. High-throughput sequencing identified a novel homozygous variant of TPK1 gene c.382G>A (p.Leu128Phe). His father and mother were both found carrying the variant. The variant protein showed a 30.9% reduction in TPK1 enzyme activity compared with the wildtype.@*CONCLUSION@#A novel pathogenic variant has been identified in a boy with thiamine metabolic dysfunction syndrome type 5.

Polioencephalomalacia (PEM) in calves associated with excess sulfur intake / Polioencefalomalacia em bezerros associada à ingestão de alimento com excesso de enxofre

Polioencephalomalacia (PEM) is the morphological characterization for softening of brain gray matter, and excess sulfur intake is one of its main causes. This study describes an outbreak of this disease in 1-to-3-month-old calves in a farm located in Santa Catarina state, Brazil. The herd consisted of 27 Jersey male calves whose diet was composed of initial feed, ground whole corn, and mineral salt. From this herd, 10 animals became ill, showing signs of apathy, anorexia and blindness, evolving to generalized weakness and death. Necropsy was performed in three of these animals, which showed flattening of the cerebral convolutions in addition to softened, yellowish areas in the cerebral cortex. Histopathological examination revealed deep laminar necrosis associated with perineuronal and perivascular edema, as well as neurons with wrinkled, eosinophilic, or vacuolated cytoplasm. The following sulfur doses were observed: 8,010mg/kg in corn, 6,385mg/kg in initial feed, 1,060mg/kg in mineral salt and 2.3mg/L in water, reaching dose values far above the accepted, totaling a daily intake of approximately 6,533.5mg sulfur/animal/day. As differential diagnosis, lead was dosed in the kidneys and liver of the three calves, with negative results. Also, the calf that sickened last was treated with 20mg/kg thiamin and 0.2mg/kg dexamethasone (IM; QID) for three days and eventually recovered. According to anatomopathological findings, excess sulfur intake and therapeutic diagnosis, sulfur poisoning was suggested as the cause of PEM in these 1-to-3-month-old calves. Occurrence of PEM is rare in calves at such a young age.(AU)

A polioencefalomalacia (PEM) é a caracterização morfológica para o amolecimento da substância cinzenta encefálica, e uma de suas principais etiologias é a ingestão excessiva de enxofre. Este trabalho descreve um surto desta enfermidade em bezerros de um a três meses de idade em uma propriedade de Santa Catarina. O lote era composto por 27 bezerros machos da raça Jersey, com alimentação composta por ração inicial, milho inteiro triturado e sal mineral. Deste lote, 10 animais adoeceram, apresentando sinais de apatia, anorexia e cegueira, com evolução para fraqueza generalizada. Nove bezerros morreram e três foram submetidos a necropsia, que demonstraram achatamento das circunvoluções cerebrais além de áreas de amolecimento e coloração amarelada no córtex cerebral. Realizou-se exame histopatológico que evidenciou necrose laminar profunda associada a edema perineuronal e perivascular, além de neurônios com citoplasma enrugado, eosinofílico ou vacuolizado. A dosagem de enxofre resultou em 8010mg/Kg no milho, 6385mg/Kg na ração, 1060mg/Kg no sal mineral e 2,3mg/L na água, atingindo valores muito acima do tolerado, totalizando a ingestão diária de cerca de 6533,5mg de enxofre/animal/dia. Como diagnóstico diferencial realizou-se dosagem de chumbo de amostras de rim e fígado dos três bezerros com resultado negativo. Ainda, o último bovino a adoecer foi tratado com 20mg/Kg de tiamina e 0,2mg/Kg de dexametasona IM, QID, durante três dias e recuperou-se. De acordo com os achados anatomopatológicos e o excesso de enxofre na dieta, sugere-se que a intoxicação por enxofre seja a causa de PEM nestes bezerros de um a três meses de idade, sendo essa enfermidade rara em bovinos tão jovens.(AU)

Associations between Dietary Fiber Intake and Bone Mineral Density in Adult Korean Population: Analysis of National Health and Nutrition Examination Survey in 2011

BACKGROUND: The correlations between the amount of daily fiber intake and bone mineral densities (BMDs) in Korean adult population were investigated in our study. METHODS: Utilizing the Korean National Health and Nutrition Examination Survey in 2011, multivariable linear regression was performed to explore the association between fiber consumption and BMD of lumbar vertebrae 1 to 4 (L1–4 total), L1, L2, L3, and L4 vertebrae, femur neck, femur total, and trochanter. All models were adjusted for age, body mass index, vitamin D level, smoking, physical activity, alcohol use, contraceptive use, hormonal replacement therapy, consumption of carbohydrate, protein, fat, calcium, phosphate, iron, thiamine, riboflavin, niacin, and vitamin C. RESULTS: In males aged between 18 and 45, fiber intake significantly increased BMDs of L1 (coefficient β=0.004, P=0.040) and L2 (β=0.004, P=0.038) while daily protein consumption significantly lowered BMDs of femur neck (β=−0.001, P=0.009), femur total (β=−0.001, P=0.008), and trochanter (β=−0.001, P=0.008). In males aged 65 and older, nutrient intake shows no significant correlations with BMDs except fat consumption was inversely associated with BMD of trochanter (β=−0.001, P=0.017). In females aged between 18 and 45, fiber intake shows no significant relationship with BMDs while daily fat consumption significantly increased BMDs of L1 (β=0.001, P=0.028), L2 (β=0.001, P=0.024), L3 (β=0.001, P=0.033), and L1–4 total (β=0.001, P=0.017). CONCLUSIONS: Fiber intake was a protective factor of lumbar spine (L1 and L2) BMD in male aged between 18 and 45 but not in female participants of any age groups.

Thiamine Deficiency in a Child with Short Bowel Syndrome and Review / 대한소아소화기영양학회지

Thiamine (vitamin B₁) is a water-soluble vitamin that is not endogenously synthesized in humans. It is absorbed by the small intestine, where it is activated. Its active form acts as a coenzyme in many energy pathways. We report a rare case of thiamine deficiency in a 3.5-year old boy with short bowel syndrome secondary to extensive bowel resection due to necrotizing enterocolitis during his neonatal age. The patient was parenteral nutrition-dependent since birth and had suffered from recurrent central catheter-related bloodstream infections. He developed confusion with disorientation and unsteady gait as well as profound strabismus due to bilateral paresis of the abductor muscle. Based on these and a very low thiamine level he was diagnosed and treated for Wernicke encephalopathy due to incomplete thiamine acquisition despite adequate administration. He fully recovered after thiamine administration. After 1999 eight more cases have been reported in the PubMed mostly of iatrogenic origin.

Wernicke's Encephalopathy Onset during Diagnosis and Treatment of Peritoneal Mesothelioma:Report of One Case and Literature Review / 中国医学科学院学报

Wernicke's encephalopathy(WE),characterized by nystagmus and ophthalmoplegia,unsteadiness of stance and gait and mental-status changes,is an acute or subacute metabolic encephalopathy of the central nervous system resulting from Vitamin B1(VitB1)deficiency. A 29-year-old male patient was admitted to our hospital due to abdominal pain and fever. He remained chronically undernourished. He was complicated with WE at the late stage of diagnosis,mainly manifested as the convulsion of limbs,ataxia,and delirium. After treatment with VitB1,these neuropsychiatric symptoms were remarkably resolved. His primary disease was later pathologically confirmed as peritoneal mesothelioma.

Antibacterial effects of sodium tripolyphosphate against Porphyromonas species associated with periodontitis of companion animals

Porphyromonas species are closely associated with companion animal periodontitis which is one of the most common diseases in dogs and cats and leads to serious systemic diseases if left untreated. In this study, we evaluated the antimicrobial effects and mode of action of sodium tripolyphosphate (polyP3, Na5P3O10), a food additive with proven safety, using three pathogenic Porphyromonas species. The minimum inhibitory concentrations (MICs) of polyP3 against Porphyromonas gulae, Porphyromonas cansulci, and Porphyromonas cangingivalis were between 500 and 750 mg/L. PolyP3 significantly decreased viable planktonic cells as well as bacterial biofilm formation, even at sub-MIC concentrations. PolyP3 caused bacterial membrane disruption and this effect was most prominent in P. cangingivalis, which was demonstrated by measuring the amount of nucleotide leakage from the cells. To further investigate the mode of action of polyP3, high-throughput whole-transcriptome sequencing was performed using P. gulae. Approximately 30% of the total genes of P. gulae were differentially expressed by polyP3 (> 4-fold, adjusted p value < 0.01). PolyP3 influenced the expression of the P. gulae genes related to the biosynthesis of thiamine, ubiquinone, and peptidoglycan. Collectively, polyP3 has excellent antibacterial effects against pathogenic Porphyromonas species and can be a promising agent to control oral pathogenic bacteria in companion animals.

Bilateral Hearing Loss in Wernicke Encephalopathy

Wernicke encephalopathy is a syndrome caused by thiamine deficiency whose three typical symptoms are ophthalomoplegia, ataxia, and confusion. There are also rare reports of bilateral hearing loss, which can be caused by damage to the bilateral inferior colliculus or thalamic lesions, or by energy failure of the cochlea. This case suggests that thiamine should be administered based on the possibility of Wernicke encephalopathy occurring in malnourished or alcoholic patients with sudden bilateral hearing loss.

Paroxysmal crying and motor regression for more than two months in an infant / 中国当代儿科杂志

The patient was a male who was found to be abnormal at the age of 4.5 months. He presented with irritability, motor regression and opisthotonus. Brain MRI revealed bilateral abnormality in the lentiform nucleus, thalamus, deutocerebrum and cerebellar hemispheres. Novel compound heterozygous mutations of SLC19A3 gene, c.950G>A(p.G317E) and c.962C>T(p.A321V), were found in the patient. Further study showed that c.950G>A was inherited from his father and c.962C>T came from his mother. Using bioinformatics software analysis, both of the mutations were found to be harmful. His symptoms were improved remarkably after biotin, thiamine and "cocktail" therapy. One month later a brain MRI revealed that the lesions in basal ganglia and cerebellar hemispheres were improved. The patient was definitely diagnosed with biotin-thiamine responsive basal ganglia disease (BTBGD). BTBGD is a treatable autosomal recessive disease and early administration of biotin and thiamine may lead to clinical improvement.

Ácido fólico em excesso: efeitos sobre o metabolismo das vitaminas B2 e B6, o catabolismo do triptofano e a resposta imune / Folic acid excess: effects on vitamin B2 and B6 metabolism, tryptophan catabolism and immune response

Introdução: A suplementação com ácido fólico (AF) é recomendada em algumas condições para evitar a deficiência de folato, como para mulheres no período periconcepcional e durante a gestação. Atualmente, existe uma preocupação quanto ao consumo excessivo de AF pela população pelo uso de suplementos com altas doses dessa vitamina. As vitaminas B6 e B2 agem como cofatores no metabolismo de um carbono, e o uso de altas doses de AF pode influenciar o metabolismo de ambas vitaminas e, consequentemente, interferir em metabolismos importantes das quais elas participam, como a via das quinureninas, e no sistema imune. Objetivo: Avaliar os efeitos da intervenção diária com uma alta dose de AF (5 mg) por 90 dias sobre marcadores do estado das vitaminas do complexo B, e as consequências sobre os metabólitos da via das quinureninas e o sistema imune em adultos saudáveis. Material e Métodos: 64 indivíduos saudáveis foram submetidos à intervenção diária com 5 mg de AF por 90 dias. Coletas de sangue foram realizadas antes (baseline) e após 45 e 90 dias de intervenção. As concentrações séricas de folato e vitamina B12 foram avaliadas por métodos microbiológicos. As concentrações séricas das vitaminas B6 (piridoxal 5'-fosfato (PLP), piridoxal (PL) e ácido 4-piridóxico (PA)), B2 (riboflavina e flavina mononucleotídeo (FMN)), B1 (tiamina e tiamina monofosfato (TMP)) e B3 (ácido nicotínico, nicotinamida e N1-metilnicotinamida), bem como de triptofano, quinurenina e metabólitos, foram avaliadas por LC-MS/MS. A proteína C-reativa ultrassensível (PCRus) foi determinada por imunoturbidimetria, e as concentrações séricas de interleucina (IL)-6, IL-8, IL-10, interferon gama (IFN-γ) e fator de necrose tumoral alfa (TNF-α) foram avaliadas por ensaio multiplex. A expressão de RNAm de DHFR (dihidrofolato redutase), MTHFR (metilenotetrahidrofolato redutase), IL8, TNFA e IFNG em leucócitos mononucleares (PBMC) foram avaliadas por PCR em tempo real. O número de células T regulatórias (Treg) (CD3+, CD4+, CD25high, FoxP3+, CD127-) foi avaliado após incubação dos PBMC com PMA e ionomicina ou veículo por 18h, por imunofenotipagem. Resultados: Houve um grande aumento das concentrações de folato sérico após 45 e 90 dias de intervenção com AF. Não houve diferença nas concentrações de vitamina B12 antes e após a intervenção. As concentrações séricas de PLP foram semelhantes antes e após a intervenção, entretanto, um aumento de PL sérico foi observado após 45 e 90 dias, e de PA após 45 dias, quando comparado ao baseline. Riboflavina e FMN foram maiores após 45 e 90 dias em relação ao baseline. A tiamina sérica foi menor após 45 dias, e as concentrações de TMP foram maiores após 90 dias quando comparados aos períodos anteriores. Não houve diferença nas concentrações de vitamina B3 antes e após a intervenção. Dentre os metabólitos da via das quinureninas, apenas o ácido antranílico apresentou aumento após 45 e 90 dias, enquanto o ácido picolínico diminuiu após 90 dias. PCRus, IL-6, IL-8, IL-10, IFN-γ e TNF-α séricos foram semelhantes no baseline e após a intervenção. Um aumento da expressão de RNAm de DHFR e TNFA foi observado após, respectivamente, 90 dias e 45 e 90 dias de intervenção. Após 90 dias de intervenção com AF, foi observada diminuição do número de células Treg após estímulo com PMA e ionomicina. Conclusão: O uso diário de 5 mg de AF foi associado a alterações nas concentrações séricas de marcadores do estado de vitaminas do complexo B e da via das quinureninas, bem como a diminuição do número de células Treg

Introduction: Folic acid (FA) supplementation is recommended in some conditions to avoid folate deficiency, as women during periconceptional period and pregnancy. Currently, there is a concern about the excessive consumption of FA by population by using supplements with high doses of this vitamin. Vitamins B6 and B2 are cofactors of enzymes of one carbon metabolism and, consequently, may disturb key metabolism in which they participate, as kynurenine pathway, and the immune system. Aim: To assess the effects of a daily intervention with high dose of FA (5 mg) for 90 days on biomarkers of complex B vitamins status and its outcomes in kynurenine pathway metabolites and immune system in healthy adults. Material and Methods: 64 healthy individuals were submitted to a daily intervention with 5 mg of FA for 90 days. Blood samples were collected before (baseline) and after 45 and 90 days of intervention. Serum folate and vitamin B12 were assessed by microbiological assays. Serum vitamin B6 (pyridoxal 5'-phosphate (PLP), pyridoxal (PL) and 4-pyridoxic acid (PA)), vitamin B2 (riboflavin and flavin mononucleotide (FMN)), vitamin B1 (thiamin and thiamin monophosphate)) and vitamin B3 (nicotinic acid, nicotinamide and N1-methylnicotinamide), as well as tryptophan, kynurenine and metabolites, were assessed by LC-MS/MS. C-reactive protein (hs-CPR) was assessed by immunoturbidimetry, and serum interleukin (IL)-6, IL-8, IL-10, interferon gamma (IFN-γ) and tumor necrosis factor alpha (TNF-α) were assessed by multiplex assay. Mononuclear leukocytes mRNA expression of DHFR (dihydrofolate reductase), MTHFR (methylenetetrahydrofolate reductase), IL8, TNFA and IFNG were assessed by real time PCR. Regulatory T Cell (Treg) number (CD3+, CD4+, CD25high, FoxP3+, CD127-) was determined after mononuclear leukocytes incubation with PMA and ionomycin or vehicle for 18h, by immunophenotyping. Results: A great increase on serum folate was observed after 45 and 90 days of FA intervention. No differences in serum vitamin B12 were observed before and after intervention. Serum PLP was similar before and after intervention, however, an increase in serum PL was observed after 45 and 90 days, and in PA after 45 days, when compared to baseline. Riboflavin and FMN were increased after 45 and 90 days than in baseline. Serum thiamine was decreased after 45 days than in baseline. Serum TMP was increased after 90 days when compared with previous timepoints. No differences in vitamin B3 were observed after and before FA intervention. Among kynurenine pathway metabolites, anthranilic acid was increased after 45 and 90 days, while picolinic acid was decreased after 90 days. hs-CPR, serum IL-6, IL-8, IL-10, IFN-γ and TNF-α were similar at baseline and after intervention. An increase on mRNA expression of DHFR and TNFA was observed after, respectively, 90 days and 45 and 90 days of intervention. After 90 days of FA intervention, it was observed a decrease on Treg cell number after PMA and ionomycin stimulation. Conclusion: Daily use of 5 mg of FA was associated with changes in serum markers of B-complex vitamins status and kynurenine pathway, as well as decreased number of Treg cells

Four Cases of Wernicke's Encephalopathy Following Hematopoietic Stem Cell Transplantation / 中国实验血液学杂志

<p><b>OBJECTIVE</b>To make through introduction of Wernicke's encephalopathy (WE) following hematopoietic stem cell transplantation (HSCT) in terms of clinical characteristics, diagnostic process and treatment.</p><p><b>METHODS</b>The clinical charactaristics, diagnostic and therapeutic process and prognostic follow-up in 4 patients diagnosed of WE following HSCT between January 2016 to January 2017 at Department of Hematology, Chinese Aerospace Center Hospital were retrospectively analyzed.</p><p><b>RESULTS</b>Four patients included 2 ALL and 2 AML, and 3 males and 1 female, their age ranged from 8 to 20 years old. 4 patients accouted for about 3% of all petients who received HSCT at that time. Typical triad syndrome consisting of ocular motility disorders, ataxia, global confusion was seen in only 1 patient. However, confusion and heterophthongia as onset of this complication were seen in all patients. Cerebral computed tomograph scan was universally unremarkable and useless. Cerebral MRI scan disclosed that typical involvement including thalamus, fourth ventricle, third ventricle, middle cerebral aqueduct was seen in 3, while untypical site including mamillary body was in the remaining 1 patient. All received vitamin B supplement therapy by intramuscular injection at a dose of 100 mg each day. Initial response was observed at 2, unknown, 3, 4 days after treatment and all obtained complete remission within 2 weeks without any event of relapse after median follow-up period of 8 (7-12) months.</p><p><b>CONCLUSION</b>Any recipient of HSCT with clinical signs or symptoms of central nervous system should receive vitamin B supplementary therapy immediately to decrease risk of mortality of WE even if the diagnosis of WE is uncertain.</p>

Metronidazole-Induced Encephalopathy with Thiamine Deficiency

Metronidazole-induced encephalopathy (MIE) can be caused by excessive dose or prolonged metronidazole administration. The signal abnormalities in the cerebellar dentate nuclei, midbrain, dorsal pons and corpus callosum on magnetic resonance imaging are considered as the characteristic feature of MIE. Although the mechanism of MIE remains to be elucidated, various hypothesis have been proposed including the role of metronidazole as a thiamine antagonist. Here we report a 58-year-old woman with MIE who coincidentally presented with thiamine deficiency.

Cereal intake status and nutritional status of adults: results from the Korean National Health and Nutrition Examination Survey, 2013 ~ 2016 / 한국영양학회지

PURPOSE: This study was performed to evaluate the cereal intake status and explore its relationship with the nutritional status of the Korean adults from 2013 ~ 2016, Korean National Health and Nutrition Examination Survey (KNHANES). METHODS: We analyzed dData from the combined 2013 ~ 2016 Korean National Health and Nutrition Examination Survey were analyzed. The analysis included 12,199 adults aged from 19 to 64 years (male, n = 5,127, female, n = 7,072). In this study, according to cereal intake frequency (From food frequency questionnaire), we classified tThe subjects were classified according into the “non-cereal intake (NCI)” group (male, n = 4,290, female, n = 5,578), and “cereal intake (CI)” group (male, n = 837, female, n = 1,494) according to cereal intake frequency (From food frequency questionnaire). RESULTS: Typically, 79.6% of adults did not consume cereals (male 81.6%, female 77.3%);, 13.4% consumed cereals 1–3 times a month (male 12.2%, female 14.8%) and 7.1% consumed cereals more than once a week (male 6.2%, female 7.9%). In the case of both males and females, the nutrient density (nutrient intake per 1,000 kcal) and Index of Nutritional Quality (INQ) of protein, calcium, phosphorous, potassium, vitamin A, vitamin B1, vitamin B2, niacin, and vitamin C in the Non-Cereal group were significantly lower than those of the CI group (respectively, p < 0.01). CONCLUSION: Our These results reveal highlight the low frequency of cereal intake in Korean adults and the better nutritional status in adults who consume cereals. It is hypothesized that tThe data of the present study would is expected to serve as basic data to improve the nutritional status of Korean adults.

Dietary Intake of Thiamine in Migraine Patients and Healthy Subjects: a Case-Control Study

The migraine headache is a disease related to the neurovascular system, which affects 10%–20% of people, worldwide. Recent evidences suggested a relation between thiamine status and migraine headaches. The current study was undertaken to assess dietary intake of the thiamine in migraine patients and to evaluate its association with the frequency of migraine attacks. In a case-control design, the current study was performed on 50 migraine patients and 50 healthy people, 20–60 years old in Isfahan, Iran, in 2017. Information about dietary intake was collected by Food frequency questionnaire and analyzed using the Nutritionist version 4 (N4) software (Tinuviel Software). Information about the history of disease was collected by demographic questionnaire. Analysis of covariance and independent t-test were used for data analysis and p value less than 0.05 was considered significant. Mean age, weight, height, and body mass index of participants were 35.1 ± 9.8 years, 65.3 ± 10.4 kg, 162.5 ± 8.4 cm, and 24.7 ± 4.0 kg/m², respectively. Dietary intake of thiamine among the migraine patients was lower than that in the healthy participants (p < 0.001). Migraine patients with the high frequency attacks had significantly lower intake of thiamine compared with moderate frequency attacks group (p = 0.010), however, it was not significant after adjusting for energy intake (p = 0.410, p = 0.240). Dietary intake of thiamine in migraine patients was not significantly different in comparing with healthy subjects. In addition, no significant correlation between thiamine intake and the frequency of migraine attacks was observed.

Nutrient intake and dietary quality of Korean adults according to chronic obstructive pulmonary disease (COPD): Based on the 2012~2014 Korea National Health and Nutrition Examination Survey / 한국영양학회지

PURPOSE: Food intake of COPD patients decreases because of appetite loss, depression, and breathing problems while eating, which increase the likelihood of patients becoming malnourished. This study investigated the nutrient intake and dietary quality of people with and without COPD in Korea. METHODS: There were 2,160 adults aged 50 and over who participated in the 5~6th Korea National Health and Nutrition Examination Survey. The subjects were divided into the COPD group and non-COPD group according to the rate of forced expiratory volume at 1 second (FEV1) against forced vital capacity (FVC). Consumption of food and nutrients were calculated based on the nutrition examination survey. Statistical analyses were conducted using SPSS 19.0. RESULTS: Among individuals in their 50s, intake of vitamin B1 in the COPD group was significantly less than the NCOPD group, while intake of sodium in the COPD group was significantly greater than the NCOPD group. Among individuals in their 60s, the energy intake of the COPD group was significantly less than that of the NCOPD group. The COPD group showed significantly less intake of vitamin B1 and vitamin C, but greater intake of sodium than the NCOPD group. In the group aged 70 and over, The COPD group had significantly less intake of vitamin B1 than the NCOPD group and showed lower nutrient adequacy ratio values in protein, vitamin B1, vitamin B2, and iron than the NCOPD group. CONCLUSION: The COPD group consumed less energy and certain nutrients, and their dietary quality was poor compared to the NCOPD group. Thus, more detailed research is required to understand the concrete relationship between COPD and malnutrition.

Health and nutritional status of Korean adults according to age and household food security: Using the data from 2010~2012 Korea National Health and Nutrition Examination Survey / 한국영양학회지

PURPOSE: This study was performed to identify the health and nutritional status of Korean adults according to food security by age group. METHODS: The subjects were 20~79 year old adults (n = 16,595) who participated in the fifth Korea National Health and Nutrition Examination Survey (2010~2012). Subjects were divided into three groups based on food security such as secure, mildly insecure, and moderately/severely insecure groups. Dietary intake was estimated by 24-hour dietary recall. As for mental health status, the data on mental stress, sleep hours, depression symptoms, and suicide ideation were used. RESULTS: Korean adults who were exposed to high food insecurity typically had low income levels, lived alone, and were recipients of basic welfare. In the 20~39y group, people with higher food insecurity had lower concentrations of hemoglobin and higher iron-binding capacity. In the 40~59y group, people with higher food insecurity had lower HDL-cholesterol. In the 60~79y group, people with higher food insecurity had higher total cholesterol levels, more stress, more experiences of depression symptoms, and were more suicidal. Mean intakes of energy, protein, calcium, phosphorus, potassium, vitamin A, carotene, vitamin B1, vitamin B2, niacin, and vitamin C were lower in the food insecure groups. Amounts of vegetables, fruits, and seasoning intakes tended to be lower in people with higher food insecurity. The effects of food security on nutrition intake were greater in the 40~59y and 60~79y groups than the 20–39y group. CONCLUSION: Food insecurity was related to certain health indicators such as anemia and cholesterol levels and affected mental health. The effects of food insecurity on health and nutritional status were different by age group.

Four Cases of Wernicke's Encephalopathy with Impaired Horizontal Vestibular Ocular Reflexes / 대한평형의학회지

Wernicke's encephalopathy (WE) is a neurological disorder induced by a dietary vitamin B1 (thiamine) deficiency which is characterized by encephalopathy, gait ataxia, and variant ocular motor dysfunction. In addition to these classical signs of WE, a loss of the horizontal vestibulo-ocular reflex (VOR) is being reported as the major underdiagnosed symptoms in WE. In this retrospective single center study, we report four cases of WE initially presented with impaired horizontal VOR in addition to the classical clinical presentations, and imaging and neurotological laboratory findings were described.

Repetitive Transcranial Magnetic Stimulation for Wernicke-Korsakoff Syndrome: A Case Report

A 57-year-old man who was diagnosed with Wernicke-Korsakoff syndrome showed severe impairment of cognitive function and a craving for alcohol, even after sufficient supplementation with thiamine. After completing 10 sessions of 10 Hz repetitive transcranial magnetic stimulation (rTMS) at 100% of the resting motor threshold over the left dorsolateral prefrontal cortex, dramatic improvement in cognitive function and a reduction in craving for alcohol were noted. This is the first case report of the efficacy of a high-frequency rTMS in the treatment of Wernicke-Korsakoff syndrome.

Clinical role of dietary thiamine on regulation of renal response to metabolic acidosis in adult rats / Papel clínico de tiamina na dieta sobre a regulação da resposta renal de acidose metabólica em ratos adultos

Kidney plays a central role in maintaining the composition of body fluids by regulating water, NaCl, acid base, and solute reabsorption and excretion, respectively. The study was done to investigate the physiological role of thiamine in regulation of renal response to metabolic acidosis induced by NH4Cl in adult male rats. For this experiment, fifty rats were used. They were divided into five groups. Control rats received basal diet; rats fed on basal diet mixed with NH4Cl (4g NH4Cl/100g diet) to induce severe metabolic acidosis, rats fed on basal supplemented diet with thiamine (600 mg/kg diet), and rats fed on basal supplemented diet with thiamine before and after induction of metabolic acidosis by NH4Cl for 14 days. The results showed that the plasma levels of chloride, urea, and creatinine were significantly elevated in metabolic acidosis induced by NH4Cl. Thiamine supplementation at high dose before or after induction improved the chloride values. Feeding diets supplemented with thiamine modulated the plasma sodium and bicarbonate values. Supplementation with vitamin B1 as preventive agent significantly restored these changes to near control value and when used as curative agent improved plasma creatinine and urea levels. Urinary pH and potassium levels were decreased significantly in metabolic acidotic rats when compared to all experimental groups. Urinary ammonia and aldosterone levels were decreased by thiamine supplementation as protective agent. Supplementation with vitamin B1 as preventive and curative agents, restored the affected parameters and regulate the response of kidney to metabolic acidosis induced by ammonium chloride.

O rim desempenha um papel central na manutenção da composição de fluidos corporais através do controlo de água, NaCl, ácido-base, e reabsorção do soluto e excreção, respectivamente. O estudo foi realizado para investigar o papel fisiológico de tiamina na regulação da resposta renal à acidose metabólica induzida por NH4Cl em ratos machos adultos. Para esta experiência, foram utilizados cinquenta ratos. Eles foram divididos em cinco grupos. Os ratos de controlo receberam dieta basal; os ratos alimentados com dieta basal misturado com NH 4 Cl (4 g NH 4 Cl / 100 g de dieta) para induzir uma grande taxa de acidose metabólica , os ratos alimentados com dieta suplementada basal com tiamina (600 mg / kg de ração), e ratos alimentados com dieta suplementada basal com tiamina antes e após a indução de acidose metabólica por NH4Cl durante 14 dias. Os resultados mostraram que os níveis plasmáticos de cloreto, ureia e creatinina foram significativamente elevados em acidose metabólica induzida por NH4Cl. A suplementação de tiamina em doses elevadas antes ou após a indução aumentou os valores de cloreto. O fornecimento de dietas suplementadas com valores de tiamina modulado o sódio plasmático e bicarbonato. A suplementação com vitamina B1 como agente preventivo restaurou significativamente estas alterações para aproximar o valor de controle e, quando utilizado como agente curativo melhorou os níveis de creatinina e uréia plasmática. Os níveis de pH e de potássio na urina foram reduzidos significativamente em ratos acidóticos metabólicos, quando comparado com todos os grupos experimentais. os níveis de amônia e de aldosterona urinário foram reduzidos pela suplementação de tiamina como agente de proteção. A suplementação com vitamina B1 como agentes preventivos e curativos, restaurou os parâmetros afetados e regulou a resposta do rim a acidose metabólica induzida por cloreto de amónio.

Implantação de um método para determinação da atividade da transcetolase eritrocitária para avaliação indireta da tiamina / Implementation of a method for determining the erythrocyte transketolase activity for indirect evaluation of thiamin

O teste de ativação da transcetolase eritrocitária (TK-E) pelo pirofosfato de tiamina (TPP) exógeno é um método indireto para mensurar a tiamina (vitamina B1). A diminuição da atividade da transcetolase eritrocitária e o aumento da estimulação in vitro com o TPP maior do que 17% indicam deficiência de tiamina. Este é um método plausível, pois são nos eritrócitos que estão concentradas a maior parte desta vitamina. Em virtude de surtos de beribéri que tem ocorrido no Brasil desde 2006, o Instituto Adolfo Lutz (IAL), como Laboratório Central de Saúde Pública, propôs a implantação desse método para auxiliar na investigação de novos surtos ou de casos isolados. Foram avaliados o teste de precisão, a linearidade, a estabilidade do hemolisado e da amostra, e estimados os limites de detecção e de quantificação. A atividade da TK-E sem ativação pelo TPP foi de 0,732 UI/gHb e com ativação foi de 0,827 UI/gHb. Todos os resultados dos parâmetros avaliados neste estudo apresentaram-se dentro dos critérios de aceitabilidade garantindo-se a confiabilidade do método. Fica, assim, disponível mais um ensaio bioquímico para a Rede Pública de Saúde, mas ainda necessário definir os valores de referência para estabelecer os limites clínicos da deficiência de tiamina.

Erythrocyte transketolase activation test (TK-E) by exogenous thiamine pyrophosphate (TPP)is an indirect method to measure thiamine (vitamin B1). The decrease in the erythrocyte transketolase activity and the increase of in vitro stimulation with TPP greater than 17 % indicate thiamine deficiency. It is a reasonable method as the major portion of this vitamin are concentrated in erithrocytes. Due to the beriberi outbreaks that have occurred in Brazil since 2006, the Adolfo Lutz Institute (IAL), as a Central Public Health Laboratory, proposed the implementation of this method to give support to the investigation on the new outbreaks or isolated cases. The evaluated parameters were precision, linearity, hemolysate and sample stability, and the limits of detection and quantification were estimated. The TK-E activity without activation by TPP was 0.732 UI/gHb, and with activation was 0.827 UI/gHb. All of the results obtained from the evaluated parameters showed to be within the eligibility criteria, ensuring the reliability of the proposed methods.Thus, this method showed to be adequate as biochemical assay for the Public Health Network. However, there is a need to define the reference values to establish the clinical limits of thiamine deficiency.

Efficacy and Safety of "URSA Complex" in Subjects with Physical Fatigue: A Multicenter, Randomized, Double-blind, Placebo-controlled Trial / 中华医学杂志(英文版)

<p><b>BACKGROUND</b>Fatigue is a common symptom both in diseases status and in healthy subjects. Various supplements and nutraceuticals for relieving of fatigue have been used. However, there are a few studies to evaluate the efficacy and the safety of the drug for fatigue alleviation, we conducted using URSA Complex to evaluate the efficacy on physical fatigue via score changes in the checklist individual strength (CIS).</p><p><b>METHODS</b>The study was designed as a multicenter, randomized, double-blind, placebo-controlled trial, with subjects randomized to one of the two arms, receiving either placebo or URSA Complex administered as identical capsules. The primary efficacy endpoints of this clinical trials are the ratio of improving CIS scores < 76 points in patients at the end (4 weeks). Secondary efficacy variables are as follows one is an improvement of fatigue and the other is an improvement of the liver enzyme.</p><p><b>RESULTS</b>The fatigue recovery rate in who had improved CIS scores of < 76 points were 70.0%, 50.9% in the therapy group and placebo group, respectively (P = 0.019). The fatigue recovery rate in CIS score was higher in URSA Complex therapy group than placebo group. The difference between therapy group and placebo group was statistically significant at 4 weeks later, but not 2 weeks.</p><p><b>CONCLUSIONS</b>Our results provided that the URSA Complex was effective in alleviating physical fatigue. The adverse event frequency in the therapy groups was similar to that in the placebo group.</p>