Urea cycle disorder (UCD) is a group of inherited metabolic diseases with high disability or fatality rate, which need long-term drug treatment and diet management. Except those with Citrin deficiency or liver transplantation, all pediatric patients require lifelong low protein diet with safe levels of protein intake and adequate energy and lipids supply for their corresponding age; supplementing essential amino acids and protein-free milk are also needed if necessary. The drugs for long-term use include nitrogen scavengers (sodium benzoate, sodium phenylbutyrate, glycerol phenylbutyrate), urea cycle activation/substrate supplementation agents (N-carbamylglutamate, arginine, citrulline), etc. Liver transplantation is recommended for pediatric patients not responding to standard diet and drug treatment, and those with severe progressive liver disease and/or recurrent metabolic decompensations. Gene therapy, stem cell therapy, enzyme therapy and other novel technologies may offer options for treatment in UCD patients. The regular biochemical assessments like blood ammonia, liver function and plasma amino acid profile are needed, and physical growth, intellectual development, nutritional intake should be also evaluated for adjusting treatment in time.
Humans ; Child ; Citrullinemia/drug therapy* ; Urea Cycle Disorders, Inborn/therapy* ; Arginine ; Sodium Benzoate/therapeutic use* ; Liver Transplantation

Objective To establish an infrared spectroscopic method for the rapid qualitative and quantitative analysis of caffeine and sodium benzoate in Annaka samples. Methods Qualitative and quantitative modeling samples were prepared by mixing high-purity caffeine and sodium benzoate. The characteristic absorption peaks of caffeine and sodium benzoate in Annaka samples were determined by analyzing the infrared spectra of the mixed samples. The quantitative model of infrared spectra was established by partial least squares （PLS）. Results By analyzing the infrared spectra of 17 mixed samples of caffeine and sodium benzoate （the purity of caffeine ranges from 10% to 80%）, the characteristic absorption peaks for caffeine were determined to be 1 698, 1 650, 1 237, 972, 743, and 609 cm^-1. The characteristic absorption peaks for sodium benzoate were 1 596, 1 548, 1 406, 845, 708 and 679 cm^-1. When the detection of all characteristic absorption peaks was the positive identification criteria, the positive detection rate of caffeine and sodium benzoate in 48 seized Annaka samples was 100%. The linear range of PLS quantitative model for caffeine was 10%-80%, the coefficient of determination （ R²） was 99.9%, the root mean square error of cross validation （RMSECV） was 0.68%, and the root mean square error of prediction （RMSEP） was 0.91%; the linear range of PLS quantitative model for sodium benzoate was 20%-90%, the R² was 99.9%, the RMSECV was 0.91% and the RMSEP was 1.11%. The results of paired sample t test showed that the differences between the results of high performance liquid chromatography method and infrared spectroscopy method had no statistical significance. The established infrared quantitative method was used to analyze 48 seized Annaka samples, the purity of caffeine was 27.6%-63.1%, and that of sodium benzoate was 36.9%-72.3%. Conclusion The rapid qualitative and quantitative analysis of caffeine and sodium benzoate in Annaka samples by infrared spectroscopy method could improve identification efficiency and reduce determination cost.
Caffeine ; Chromatography, High Pressure Liquid ; Least-Squares Analysis ; Sodium Benzoate ; Spectroscopy, Near-Infrared

The role of food additives in chronic urticaria (CU) is still under investigation. In this study, we aimed to explore the association between food additives and CU by using the basophil activation test (BAT). The BAT using 15 common food additives was performed for 15 patients with CU who had a history of recurrent urticarial aggravation following intake of various foods without a definite food-specific IgE. Of the 15 patients studied, two (13.3%) showed positive BAT results for one of the tested food additives. One patient responded to monosodium glutamate, showing 18.7% of CD203c-positive basophils. Another patient showed a positive BAT result to sodium benzoate. Both patients had clinical correlations with the agents, which were partly determined by elimination diets. The present study suggested that at least a small proportion of patients with CU had symptoms associated with food additives. The results may suggest the potential utility of the BAT to identity the role of food additives in CU.
Basophils* ; Diet ; Food Additives* ; Humans ; Hypersensitivity ; Immunoglobulin E ; Sodium Benzoate ; Sodium Glutamate ; Urticaria*

Lysinuric protein intolerance (LPI) is a rare inherited metabolic disease, caused by defective transport of dibasic amino acids. Failure to thrive, hepatosplenomegaly, hematological abnormalities, and hyperammonemic crisis are major clinical features. However, there has been no reported Korean patient with LPI as of yet. We recently encountered a 3.7-yr-old Korean girl with LPI and the diagnosis was confirmed by amino acid analyses and the SLC7A7 gene analysis. Her initial chief complaint was short stature below the 3rd percentile and increased somnolence for several months. Hepatosplenomegaly was noted, as were anemia, leukopenia, elevated levels of ferritin and lactate dehydrogenase, and hyperammonemia. Lysine, arginine, and ornithine levels were low in plasma and high in urine. The patient was a homozygote with a splicing site mutation of IVS4+1G > A in the SLC7A7. With the implementation of a low protein diet, sodium benzoate, citrulline and L-carnitine supplementation, anemia, hyperferritinemia, and hyperammonemia were improved, and normal growth velocity was observed.
Amino Acid Metabolism, Inborn Errors/complications/diet therapy/*genetics ; Antifungal Agents/therapeutic use ; Antigens, CD98 Light Chains/genetics ; Asian Continental Ancestry Group/*genetics ; Carnitine/therapeutic use ; Child, Preschool ; Citrulline/therapeutic use ; Diet, Protein-Restricted ; Disorders of Excessive Somnolence/complications/*diagnosis/drug therapy ; Female ; Growth Disorders/complications/*diagnosis ; Homozygote ; Humans ; Hypercalcemia/complications/*diagnosis ; Metabolic Diseases/complications/*diagnosis ; Mutation ; Nephrocalcinosis/complications/*diagnosis ; Republic of Korea ; Sequence Analysis, DNA ; Sodium Benzoate/therapeutic use ; Vitamin B Complex/therapeutic use

Ornithine carbamoyltransferase (OTC) deficiency is a urea cycle disorder that causes the accumulation of ammonia, which can lead to encephalopathy. Adults presenting with hyperammonemia who are subsequently diagnosed with urea cycle disorders are rare. Herein, we report a case of a late-onset OTC deficient patient who was successfully treated with arginine, benzoate and hemodialysis. A 59-yr-old man was admitted to our hospital with progressive lethargy and confusion. Although hyperammonemia was suspected as the cause of the patient's mental changes, there was no evidence of chronic liver disease. A plasma amino acid and urine organic acid analysis revealed OTC deficiency. Despite the administration of a lactulose enema, the patient's serum ammonia level increased and he remained confused, leading us to initiate acute hemodialysis. After treatment with arginine, sodium benzoate and hemodialysis, the patient's serum ammonia level stabilized and his mental status returned to normal.
Age of Onset ; Ammonia/blood ; Arginine/therapeutic use ; Citrulline/blood ; Humans ; Hyperammonemia/*etiology ; Male ; Middle Aged ; Ornithine/blood ; Ornithine Carbamoyltransferase Deficiency Disease/complications/*diagnosis/drug therapy ; Pedigree ; Renal Dialysis ; Sodium Benzoate/therapeutic use

Many different additives include preservatives, stabilizers, conditioners, thickeners, colorings, flavorings, sweeteners, and antioxidants. Despite the multitude of additives known, only a small number has been associated with hypersensitivity reactions. A number of investigators have suggested that a significant population of patients with allergic diseases has symptoms related to the ingestion of food additives. However, the incidence and mechanism of reactions to additives in patients with chronic urticaria, angioedema, and atopic dermatitis remain unknown. A few studies of monosodium glutamate is reported to be associated with atopic dermatitis, but their relationship remains unknown. The best known dye is tartrazine. The group of azo dyes includes ponceau and sunset yellow. Amaranth (FD&C red no. 5) was banned from use in the US in 1975 because of claims related to carcinogenicity. Most of them are reported to be associated with aggravation of atopic dermatitis. Parabens are aliphatic esters of parahydroxybenzoic acid. Sodium benzoate is a closely related substance usually reported to cross-react with these compounds. These agents, which are widely used as preservatives in both food and drugs, are well recognized as causes of severe contact dermatitis. Additives would have to act as haptens to create a response mediated by IgE. The majority of these reactions are not of the immediate hypersensitivity type. Many cases of additive-provoked urticaria or dermatitis occur as late as 24 hours after challenge, arguing against an IgE-mediated mechanism. In conclusion, the exact relationship between food additives and the allergic diseases still remains to be solved.
Angioedema ; Antioxidants ; Azo Compounds ; Coloring Agents ; Dermatitis ; Dermatitis, Atopic ; Dermatitis, Contact ; Eating ; Esters ; Food Additives ; Food Hypersensitivity ; Haptens ; Humans ; Hypersensitivity ; Hypersensitivity, Immediate ; Immunoglobulin E ; Incidence ; Parabens ; Research Personnel ; Sodium Benzoate ; Sodium Glutamate ; Sweetening Agents ; Tartrazine ; Urticaria

Atomic force microscope (AFM) was used to study biotoxicity of food preservative sodium benzoate (SB) at the single cellular level. Lymphocyte morphology and membrane ultrastructure treated with SB at different concentrations and time were analyzed visually. As compared to the normal lymphocyte, the cell morphology and membrane was significantly changed and its ultrastructure was also complicated. After treated with SB, the Rp-v, Rq, Ra and Z values were changed. The statistical analysis of lymphocytes after treated with SB was studied, and discussed its mechanism.
Animals ; Cell Membrane ; ultrastructure ; Lymphocytes ; drug effects ; pathology ; Mice ; Mice, Inbred BALB C ; Microscopy, Atomic Force ; Sodium Benzoate ; toxicity

Hepatic encephalopathy, one of the major complications of cirrhosis, is a neuropsychiatric syndrome caused by accumulation of toxins in the central nervous system following dysfunction in liver detoxification. Various manifestations makes it difficulty to diagnose and categorize hepatic encephalopathy. For the consistency in diagnosis and staging of hepatic encephalopathy, standardized nomenclature regarding forms of hepatic encephalopathy was proposed recently. Due to the poor understanding of pathophysiology, effective prevention or treatment options are limited. Based on the theory that intestinal-derived ammonia plays major role in the development of hepatic encephalopathy, therapeutic approaches are directed at reducing intestinal bacterial production of ammonia and facilitating its elimination. Non-absorbable disaccharides, antibiotics such as rifaximin and L-ornithine-L-aspartate are main therapies for hepatic encephalopathy. Alternative therapies such as benzodiazepine receptor antagonists, branched-chain amino acids, sodium benzoate and acarbose have limited data supporting their use. Precipitating factors must be looked into carefully and corrected immediately because most patients are usually present with precipitating factors. Hepatic encephalopathy without precipitating factors shows poor prognosis and could be candidate of liver transplantation.
Acarbose ; Amino Acids, Branched-Chain ; Ammonia ; Anti-Bacterial Agents ; Central Nervous System ; Complementary Therapies ; Dipeptides ; Disaccharides ; Fibrosis ; Hepatic Encephalopathy ; Humans ; Liver ; Precipitating Factors ; Prognosis ; Receptors, GABA-A ; Rifamycins ; Sodium Benzoate

Beef luncheon meat is one of the most popular meals in several countries in the world including Egypt. Thirty one fungal species and 3 species varieties were recovered from 30 samples of beef luncheon meat collected from different supermarkets in Qena. Alternaria, Aspergillus, Emericella, Mucor, Mycosphaerella, Penicillium and Rhizopus were the most common genera on the two types of media. From the above genera, the most prevalent species were Alternaria alternate, Aspergillus flavus, A. fumigatus, A. niger, A. terreus, Emericella nidulans, Mucor racemosus, Mycosphaerella tassiana, Penicillium chrysogenum and Rhizopus stolonifer. Screening of fungi for their abilities to produce lipase enzyme showed that, ten isolates represented 32.26% of total isolates appeared high lipase production, while sixteen isolates (51.61%) were moderate and 5 isolates (16.13%) were low producers. Aspergillus niger, Fusarium oxysporum and Nectria haematococca produced the highest amount of lipase enzyme, so these fungi were used in further studies. The incorporation of five food preservatives (Disodium phosphate, sodium benzoate, citric acid, potassium sorbate and sodium citrate) individually in the culture medium of lipase production exhibited an inhibitive effect on the mycelial growth and enzyme production by Aspergillus niger, Fusarium oxysporum and Nectria haematococca.
Alternaria ; Aspergillus ; Aspergillus flavus ; Aspergillus niger ; Citric Acid ; Egypt ; Emericella ; Food Preservatives ; Fungi ; Fusarium ; Lipase ; Mass Screening ; Meals ; Meat ; Mucor ; Nectria ; Niger ; Penicillium ; Penicillium chrysogenum ; Rhizopus ; Sodium ; Sodium Benzoate ; Sorbic Acid

Many different additives are added to the food that we consume, and the number of additives is estimated to range from 2,000 to 20,000. These substances include preservatives, stabilizers, conditioners, thickeners, colorings, flavorings, sweeteners, and antioxidants. Despite the multitude of additives known, only a surprisingly small proportion of them has been associated with hypersensitivity reactions. A number of investigators have suggested that a significant proportion of patients with chronic urticaria, angioedema, atopic dermatitis, and asthma have symptoms related to the ingestion of food additives. However, the incidence and mechanisms of reactions to additives in patients with chronic urticaria, angioedema, and atopic dermatitis remain unknown. Monosodium glutamate(MSG) produces the flavor-enhancing properties of seaweed, the traditional component of Japanese and Chinese cooking. A few studies on MSG have reported an association between MSG and atopic dermatitis, but the exact nature of the relationship remains unknown. Dyes approved under the Food Dye and Coloring Act are coal tar derivatives, the best known of which is tartrazine(FD&C yellow no. 5). The group of azo dyes includes ponceau(FD&C red no. 4) and sunset yellow (FD&C yellow no. 6). Amaranth(FD&C red no. 5) was banned from use in US in 1975 due to the claims related to carcinogenicity. Most of them were reported to be associated with an aggravation of atopic dermatitis. Parabens are aliphatic esters of parahydroxybenzoic acid. Sodium benzoate is a closely related substance usually reported to cross-react with these compounds. These agents, which are widely used as preservatives in both foods and drugs, are well recognized as the cause of severe contact dermatitis. Additives can serve as haptens to create a response mediated by IgE. Only a few reports have suggested IgE-mediated reactions, notably to sulfites and parabens. Instead, the overwhelming majority of these reactions are not of the immediate hypersensitivity type. Many cases of additive-provoked urticaria or dermatitis occur as late as 24 hours after the challenge,arguing against an IgE-mediated mechanism.
Angioedema ; Antioxidants ; Asian Continental Ancestry Group ; Asthma ; Child ; Coal Tar ; Coloring Agents ; Cooking ; Dermatitis ; Dermatitis, Atopic* ; Dermatitis, Contact ; Eating ; Esters ; Food Additives* ; Food Hypersensitivity ; Haptens ; Humans ; Hypersensitivity ; Hypersensitivity, Immediate ; Immunoglobulin E ; Incidence ; Parabens ; Research Personnel ; Seaweed ; Sodium Benzoate ; Sodium Glutamate ; Sulfites ; Sweetening Agents ; Urticaria