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

Azo dyes are widely used in textile, paper and packing industries, and have become one of the research hot spots in dye wastewater treatment because of their carcinogenicity, teratogenic mutagenicity, stable structure and degradation difficulty. In this study, the biodecolorization of acid orange 7 (AO7), an azo dye, by different white rot fungi was investigated, and the effect of different conditions on the decolorization rate of the dye was analyzed. At the same time, the degradation liquor was analyzed and the phytotoxicity experiment was performed to deduce the possible degradation pathway of AO7 and assess the toxicity of its degradation products. The results showed that the decolorization rate reached 93.46% in 24 h at pH 4.5, 28 ℃ by Pleurotus eryngii and Trametes versicolor when AO7 concentration was 100 mg/L. The biodegradation pathway of AO7 was initiated by the cleavage of the azo bond of AO7, generating p-aminobenzenesulfonic acid and 1-amino-2-naphthol. Subsequently, the sulfonic acid group of p-aminobenzene sulfonic acid was removed to generate hydroquinone. Moreover, the 1-amino-2-naphthol was de-ringed to generate phthalic acid and p-hydroxybenzaldehyde, and then further degraded into benzoic acid. Finally, hydroquinone and benzoic acid may be further oxidized into other small molecules, carbon dioxide and water. Phytotoxicity experiment showed that the toxicity of AO7 could be reduced by P. eryngii and T. versicolor.
Hydroquinones ; Trametes ; Azo Compounds ; Benzoic Acid

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

Objective: To explore the feasibility and practicability of establishing a rat model of premature ejaculation (PE) by injection of 8-OH-DPAT into the subarachnoid space of the lumbosacral spinal cord segments. METHODS: Twenty-four male Wistar rats were equally randomized into a PE model and a blank control group. The PE model was established by injection of 8-OH-DPAT in 10 ml normal saline at 0.8 mg per kg of the body weight per day into the subarachnoid space of the lumbosacral spinal cord segments and the control rats were injected with the same volume of normal saline only, both for 4 weeks. Another 24 female Wistar rats were injected subcutaneously with benzoic acid estradiol at 20 μg to induce estrus at 36 hours before mated with the male animals. At 2 and 4 weeks, the male rats were mated with the female ones for 30 minutes each time and meanwhile observed for their mating behavior indicators, such as mount latency, intromission latency, ejaculation latency, mount frequency, intromission frequency, and ejaculation frequency. RESULTS: Compared with the controls, the PE model rats showed a significantly lower ejaculation latency (［712.35 ± 36.77］ vs ［502.35 ± 46.72］ s, P<0.05), mount latency (［11.22 ± 3.60］ vs ［8.69 ± 2.48］ s, P<0.05), mount frequency (13.28 ± 0.24 vs 7.53 ± 1.84, P<0.05), and intromission latency (［22.33 ± 2.45］ vs ［12.08 ± 1.39］ s, P<0.05), but a remarkably higher ejaculation frequency (2.01 ± 0.48 vs 4.26 ± 0.89, P<0.05). No statistically significant difference was observed between the control and model animals in the intromission frequency (7.49 ± 2.21 vs 6.45 ± 1.89, P>0.05). CONCLUSIONS A rat model of premature ejaculation was successfully established by injection of 8-OH-DPAT into the subarachnoid space of the lumbosacral spinal cord segments, which is of great significance for further study of the mechanism of premature ejaculation.
8-Hydroxy-2-(di-n-propylamino)tetralin ; administration & dosage ; Animals ; Benzoic Acid ; administration & dosage ; Disease Models, Animal ; Ejaculation ; Estradiol ; administration & dosage ; Estrus ; Feasibility Studies ; Female ; Injections, Spinal ; Male ; Premature Ejaculation ; chemically induced ; physiopathology ; Rats ; Rats, Wistar ; Sexual Behavior, Animal ; Spinal Cord ; Subarachnoid Space

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*

OBJECTIVES: Automobile exhaust gases contain benzene and toluene, which are excreted in human urine as trans,trans-muconic acid and hippuric acid, respectively. Sorbic acid and benzoic acid, used as food preservatives, are also metabolized into trans,trans-muconic acid and hippuric acid in the human body. The purpose of this study is to estimate the level of benzene and toluene exposure according to the commuting mode and duration, residential environment, and preservative-added foods intake in university students who are not occupationally exposed to benzene or toluene. METHODS: Spot urine samples were collected from 211 university students who had no occupational exposure to volatile organic compounds. Information about their smoking history, residence type, traffic environments, commuting mode and duration, and their intake of bottled or canned food or beverages was gathered through a self-administered questionnaire. Urinary concentrations of trans,trans-muconic acid and hippuric acid were measured and statistically correlated to the individual's life style factors. RESULTS: There was no significant difference in the urinary concentrations of trans,trans-muconic acid or hippuric acid according to the smoking history. Mean urinary concentration of hippuric acid in females was higher than that found in males. Subjects living in districts with a population density of 1,000~4,999 people/km2 showed the highest urinary hippuric acid level; individuals living in cities of 5,000~9,999 people/km2 population density were next. The mean urinary trans,trans-muconic acid concentration was higher in students who were living where traffic jams are common compared to those who did not, and in subjects whose residence were within 149 m from a 4-lane road compared to those whose residence was not. However, neither mode nor duration of the commute showed any effect on the urinary trans,trans-muconic acid or hippuric acid concentrations of the students. Urinary hippuric acid levels increased when consuming canned fruit or canned coffee, and urinary trans,trans-muconic acid levels increased when consuming fruit juices or pickled radishes. CONCLUSIONS: The inhalation of vehicle exhaust and the ingestion of canned or pickled food may increase urinary hippuric acid and trans,trans-muconic acid levels in individuals who are not occupationally exposed to benzene or toluene.
Benzene ; Benzoic Acid ; Beverages ; Coffee ; Eating ; Female ; Food Preservatives ; Food, Preserved ; Fruit ; Gases ; Hippurates ; Human Body ; Humans ; Inhalation ; Life Style ; Male ; Occupational Exposure ; Occupations ; Population Density ; Questionnaires ; Smoke ; Smoking ; Sorbic Acid ; Toluene ; Transportation ; Vehicle Emissions ; Volatile Organic Compounds

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

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

OBJECTIVETo study on the effect of different processing methods on the contents of seven major constituents in wild and cultivated Paeonia lactiflora, gallic acid, catechin, albiflorin, paeoniflorin, pentagalloylglucose, benzoic acid and paeonol, in order to provide reference basis for different efficacy and formation mechanism of Paeonia Radix Rubra and Paeonia Radix Alba.

METHODWild and cultivated P. lactiflora were dealt with by four processing methods, direct drying, drying after boiling, drying after decorticating and boiling, and drying after boiling and decorticating. HPLC was use to simultaneously determine the contents of seven chemical constituents.

RESULTWild P. lactiflora showed notable higher content of paeoniflorin and catechin than cultivated P. lactiflora, whereas cultivated P. lactiflora showed higher content of albiflorin than wild P. lactiflora. Both of them were less affected by process methods in above three constituents. Drying after boiling, drying after decorticating and boiling, and drying after boiling and decorticating methods reduced the content of benzoic acid and paeonol to trace in both wild and cultivated P. lactiflora. Clustering analysis results showed that all processing methods assembled wild and cultivated P. lactiflora in 2 groups.

CONCLUSIONThe content differences of Paeonia Radix Rubra and Paeonia Radix Alba are mainly caused by their own differences and less affected by processing methods.

Acetophenones ; chemistry ; Benzoic Acid ; chemistry ; Chromatography, High Pressure Liquid ; Cluster Analysis ; Medicine, Chinese Traditional ; methods ; Paeonia ; chemistry

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