Brain magnetic resonance imaging findings and radiologic review of maple syrup urine disease:Report of three cases

BACKGROUND Maple syrup urine disease(MSUD)is a rare autosomal-recessive disorder that affects branched-chain amino acid(BCAA)metabolism and is named after the distinctive sweet odor of affected infants’urine.This disease is characterized by the accumulation of BCAAs and corresponding branched-chain ketoacids of leucine,isoleucine,and valine in the plasma,urine,and cerebrospinal fluid.However,the mechanisms of MSUD-induced brain damage remain poorly defined.The accumulation of BCAAs in the brain inhibits the activity of pyruvate dehydrogenase andα-ketoglutarate,disrupting the citric acid cycle and consequently impacting the synthesis of amino acids,causing cerebral edema and abnormal myelination.CASE SUMMARY We report three neonates admitted to our hospital with the classic subtype of MSUD.All three patients,with a transient normal period,presented with poor feeding,vomiting,poor weight gain,and increasing lethargy after birth.Laboratory testing revealed metabolic acidosis.The serum tandem mass spectrometry amino acid profile showed elevated plasma levels of BCAAs(leucine,isoleucine,and valine).Brain magnetic resonance imaging(MRI)presented abnormal signals mainly involving the globus pallidus,thalamus,internal capsule,brainstem,and cerebellar white matter,which represent the typical myelinated areas in normal full-term neonates.CONCLUSION In our patients,MRI showed typical features,in concordance with the available literature.Early detection and timely treatment are very helpful for the prognosis of MSUD patients.Therefore,we discuss the neuroimaging features of MSUD to enhance the knowledge of pediatricians about this disease.

Studies on Effect of Syrup Concentration and Drying Modes on Quality of Kesar Mango Slices

An attempt was made to preserve mango slices by treating with 2% calcium chloride solution followed by dipping into sugar syrup of different concentrations i.e. 50°, 60° and 70 °Brix respectively. The treated slices were subjected for drying in different modes of drying （oven, microwave oven and cabinet tray drying） and analyzed for various physico-chemical and organoleptic quality characteristics. The study revealed that the osmo-air dried slices of mango produced with partial dehydration facilitated by osmotic agent （sugar syrup of 60 °Brix. A fruit： sugar syrup ratio of 1：4 （w/v） for 18 h at 40 ℃ temperature） followed by mechanical drying showed superiority in sensorial quality attributes over other concentrations of ingredients and the rest of the modes of drying. The good quality osmotically dehydrated mango slices could be preserved with maximum retentions of vitamins with better dehydration, rehydration and sensorial quality characteristics.

Physicochemical, Proximate and Sensory Properties of Pineapple (<i>Ananas</i>sp.) Syrup Developed from Its Organic Side-Stream

A major economical industrial challenge from pineapple (Ananas sp.) processing contributing to environmental pollution is the organic side-streams of pineapple. The physicochemical, proximate and sensory properties of organic sidestream pineapple syrup (OSPS) developed from Smooth cayenne, Sugar loaf and MD2 pineapple varieties were evaluated. Organic side-stream pineapple syrup developed from MD2 recorded the highest moisture content with a corresponding water activity. The colour change in OSPS was significant among the three varieties and Sugar loaf variety deviated from the standard yellow colour more than Smooth cayenne and MD2 varieties. This was buttress by the high Total Soluble Solids in 10% and 20% dilutions of Sugar loaf. The OSPS was acidic. In bread, incorporating 5% OSPS (w:w) of Sugar loaf recorded the highest percentage acceptability among the pineapple varieties. Interestingly, in the production of cakes with 15%, 20% and 30% OSPS, MD2 recorded the highest percentage overall acceptance. For bread and cake, there was varied significance (P 2 and 5% Sugar loaf was more acceptable.

The effects of 90-day feeding of D-psicose syrup in male wistar rats

D-Psicose is a rare sugar present in small quantities in natural products. In a previous study, we showed that D-psicose suppresses increase in plasma glucose and reduces body fat accumulation in rats. Based on acute and chronic toxicity testing in rats, D-psicose is classified as an ordinary and safe substance. Recently, we developed a high D-psicose syrup (PS) made from high fructose corn syrup (HFCS) by the alkaline isomerization method. However, the safety of PS as a food additive has not been demonstrated. In this study, we investigated the effects of 90-day feeding of PS in male Wistar rats. The rats were fed diets containing 3% D-psicose (control) or 4.3% PS for 90 days. The body weight gain and intra-abdominal adipose tissue weight did not differ between the control and PS group. The weights of the tissues did not differ between the two dietary groups. In clinical chemistry and hematological analyses, no differences were found between the control and PS groups. No gross pathological findings were evident at dietary doses of 4.3% PS. Therefore, the present study found no adverse effects of PS in rats fed a diet containing 4.3% PS for 90 days.

Evaluation of the Palatability of Dry Syrups and the Bitterness Intensities of Dry Syrups Mixed with Various Beverages

The purpose of this study was to evaluate the palatability of dry syrups for pediatric use by human gustatory sensation testing and the artificial taste sensor. The bitterness intensities of dry syrups mixed with various beverages were also evaluated using the taste sensor. Twenty dry-syrup formulations of antibacterial and anti-allergic drugs containing bitter active ingredients, frequently used in pediatric medicine in Japan, were selected for testing. The main factors influencing palatability were determined by factor analysis of data from human gustatory sensation testing using the semantic differential method. It was shown that the bitterness intensity of dry syrups in water could be predicted by the artificial taste sensor. The influence of different beverages (orange juice, milk or cocoa) mixed with the dry syrups was evaluated using the artificial taste sensor. Taste and texture were found to be the principal factors influencing the palatability of dry syrups. While the bitterness intensities of some dry syrups were increased by mixing with orange juice, the bitterness intensities of most dry syrups were decreased by mixing with milk or cocoa. This suggests that one or more constituents of milk or cocoa may reduce the bitterness intensities of dry syrups.

Identification of syrup type using fourier transform-near infrared spectroscopy with multivariate classification methods

This research aimed to establish near infrared(NIR)spectroscopy models for identification ofsyrup types in which the maple syrup was discriminated from other syrup types.Thirty syruptypes were used in this research;the NIR spectra of each type were recorded with 10 replicates.The repeatability and reproducibility of NIR scamning were perfomed,and the absorbance atG940cn-1 was used for calculation,.Principal component analysis was used to group the syruptype.Identification models were developed by soft independent modeling by,class analogy(SIMCA)and partial least-squares diseriminant analysis(PLS.DA),The SiMCA models of alsyrup types exhibited accuracy percentage of 93.3-100%for identifying syrup types,whereasmaple syrup discrimination models showed percentage of accuracy between 83.2%and 100%.The PLS-DA technique gave the accuracy of syrup types classification bet ween 96.6%and 100%and presented ability on discrimination of maple syrup form other types of syrup with accuracyof 100%.The finding presented the potential of NIR spectroscopy for the syrup typeidentification.

Protective effects of honey compound syrup on busulfan-induced azoospermia in male rats

Objective:To evaluate the protective effects of honey compound syrup on sperm count and testis tissue in rats.Methods:Thirty rats were randomly assigned to five groups.The control group received 1 mL normal saline with dimethyl sulfoxide intraperitoneally;the busulfan group received busulfan 10 mg/kg body weight at the first and twenty-first days of the experiment via intraperitoneal injection;the last three groups received busulfan 10 mg/kg body weight to induce azoospermia,and then received 1.0,1.5,or 2.0 mg/kg honey compound syrup,respectively,after induction of azoospermia.After administration,the testis and epididymis of all rats were removed.Then,reproductive organ weight and sperm parameters(sperm concentration,epididymal sperm reserve and daily sperm production)were measured.After hematoxylin-eosin staining,seminiferous tubule cells and diameters were assessed.Results:Busulfan damaged the testis tissue and impaired spermatogenesis.Administration of honey compound syrup in three doses improved testis tissue and spermatogenesis.The protective effects of honey compound syrup may relate to the antioxidant properties of honey and other compounds in this syrup.Conclusions:Administration of honey compound syrup could be an ameliorative agent for the side effects of chemotherapy drugs such as busulfan on the male reproductive system.

Design, Development and Phytochemical Evaluation of a Poly Herbal Formulation Linkus Syrup

The present work has been carried out on polyherbal formulation named as Linkus Syrup. The herbal formulation consists of Glycyrrhiza glabra, Hyssopus officinalis, Piper longum and Alpinia galangal. The Linkus syrup physico-chemical evaluation such as pH, density, identification of polysaccharide, tanning agents, ascorbic acid and shelf life was complied. The TLC and quantitative determination of alkaloid were quantified. Determination of biomarker has been validated for the analysis of vasicine. The study result revealed that Linkus syrup formulation was well standardized at different levels such as physic-chemical consistency and assay of bio marker compound.

Diagnosis of an intermediate case of maple syrup urine disease:A case report

BACKGROUND Maple syrup urine disease(MSUD)is an autosomal recessive genetic disorder caused by defects in the catabolism of the branched-chain amino acids(BCAAs).However,the clinical and metabolic screening is limited in identifying all MSUD patients,especially those patients with mild phenotypes or are asymptomatic.This study aims to share the diagnostic experience of an intermediate MSUD case who was missed by metabolic profiling but identified by genetic analysis.CASE SUMMARY This study reports the diagnostic process of a boy with intermediate MSUD.The proband presented with psychomotor retardation and cerebral lesions on magnetic resonance imaging scans at 8 mo of age.Preliminary clinical and metabolic profiling did not support a specific disease.However,whole exome sequencing and subsequent Sanger sequencing at 1 year and 7 mo of age identified bi-allelic pathogenic variants of the BCKDHB gene,confirming the proband as having MSUD with non-classic mild phenotypes.His clinical and laboratory data were retrospectively analyzed.According to his disease course,he was classified into an intermediate form of MSUD.His management was then changed to BCAAs restriction and metabolic monitoring conforming to MSUD.In addition,genetic counseling and prenatal diagnosis were provided to his parents.CONCLUSION Our work provides diagnostic experience of an intermediate MSUD case,suggesting that a genetic analysis is important for ambiguous cases,and alerts clinicians to avoid missing patients with non-classic mild phenotypes of MSUD.

Study on quality standard of Danmu exract syrup

Objective:To establish the quality standard of Danmu exract syrup.Methods:TLC method was used to qualitatively identify Nauclea officinalis in Danmu exract syrup.The content of strictosamide and chlorogenic acid in Danmu exract syrup was determined by high efficiency axillary chromatography.Results:The spots were clear and there was no negative interference.The linear relationship of strictosamide was good in the range of 100~300ug/ml(R^(2)=0.9997).The average recovery was 99.1%and RSD was 1.16%(n=9).Chlorogenic acid had a good linear relationship in the range of 6.7~20.2ug/ml(R^(2)=0.9991).The average recovery was 99.9%and RSD was 1.10%(n=9).Conclusion:The standard is safe and reasonable,the method is simple,accurate and reproducible,and it can be used for the quality control of Danmu exract syrup.

Grape Syrup

Grape syrup is a new type of healthdrink produced by the Zhuguang Drink Factoryin Wuhai City,Inner Mongolia. Grapes contain protein,amino acids,tartrate,vitamins and multi-minerals.Thisgrape syrup is prepared from the big Wuhaigrapes,quality mineral water and the extractof seaweed.It contains 30% grape juice.With rich nutrients and sweet and refreshingtaste,it has the effect of nourishing

不同品种梨膏营养成分的综合分析与评价研究

为检测地方不同品种梨所制梨膏的主要营养成分,并进行综合评价,对原产我国的25个代表性梨地方品种所制梨膏的总酚、总黄酮、有机酸、可溶性糖和矿物质元素等主要营养成分含量进行测定,研究不同品种梨膏主要营养成分含量的变异系数和相关性,采用主成分分析法建立梨膏主要营养成分的评价模型,对梨膏的主要营养成分进行综合评价。结果表明,不同品种梨所制梨膏主要营养成分间存在较大差异。梨膏总酚平均含量(6.72 mg/g)大于总黄酮(2.15 mg/g),但总黄酮的变异系数(66.93%)大于总酚(34.18%)。有机酸平均含量由高到低依次为苹果酸>奎宁酸>草酸>柠檬酸>抗坏血酸>莽草酸,可溶性糖平均含量由高到低依次为果糖>山梨糖醇>葡萄糖>蔗糖;莽草酸变异系数最大(233.25%),果糖变异系数最小(11.26%)。矿质元素平均含量由高到低依次为K>Na>P>Mg>Ca>B>Fe>Zn>Mn>Cu;矿质元素Fe的变异系数最大(158.05%),Na的变异系数最小(15.37%)。相关性分析发现梨膏22个主要营养成分之间具有较强相关性,其中K与Na相关性最强,相关系数r=0.983。建立了主成分分析的综合数学模型F=0.3609F1+0.1372F2+0.0997F3+0.0823F4+0.0630F5+0.0493F6。据此计算综合得分并排序确定梨膏主要营养成分较高的品种为‘火把梨’‘巍山红雪梨’‘云和雪梨’。本研究为地方品种梨膏主要营养成分的综合评价提供了参考。

强力枇杷露治疗慢性支气管炎模型小鼠作用机制研究

目的 探讨强力枇杷露治疗慢性支气管炎模型小鼠的作用机制。方法 将72只NIH小鼠随机分为空白对照组(A组,12只,等体积纯净水)和模型组(60只),采用烟熏联合脂多糖吸入法复制慢性支气管炎模型小鼠建模45 d后,将模型组小鼠随机分为模型对照组(B组,等体积纯净水),盐酸氨溴索组(C组,10 mL/kg),强力枇杷露低、中、高剂量组(D1组、D2组、D3组,7.5,15,30 mL/kg),各12只,雌雄各半。各组小鼠按30 mL/kg灌胃给药,每日1次,连续14 d。末次给药24 h后,采集小鼠血清,测定白细胞介素6(IL-6)、白细胞介素8(IL-8)水平;取右肺组织,测定肿瘤坏死因子-α(TNF-α)、丙二醛(MDA)及超氧化物歧化酶(SOD)含量;对左肺支气管组织进行病理组织学检查。结果 与B组比较,D1组小鼠肺组织MDA含量显著降低(P <0.01);D2组小鼠血清IL-8的水平、肺组织MDA含量均显著降低(P <0.05);D3组小鼠血清IL-8水平、肺组织TNF-α和MDA含量均显著降低(P <0.05),SOD含量显著升高(P <0.05)。与C组比较,D1组小鼠血清IL-8水平显著升高(P <0.05),肺组织SOD含量显著降低(P <0.05)。病理组织学染色结果显示,与B组比较,D1组、D2组、D3组小鼠慢性支气管炎病变程度减轻。结论 强力枇杷露治疗慢性支气管炎模型小鼠的作用机制可能与降低IL-8,TNF-α,MDA含量和升高SOD含量相关。

基于JAK2/STAT3信号通路探究利咽糖浆对慢性咽炎大鼠咽喉组织损伤及咽黏膜修复的影响

目的:基于Janus激酶2(JAK2)/信号转导和转录激活因子3(STAT3)信号通路探究利咽糖浆对慢性咽炎大鼠咽喉组织损伤及咽黏膜修复的影响。方法:取SD大鼠随机分为对照组、模型组、利咽糖浆组、RO8191(JAK2/STAT3激活剂)组、利咽糖浆+RO8191组,模型组与药物干预组大鼠采用氨水刺激咽部构建慢性咽炎模型,对照组大鼠咽部注射等剂量生理盐水,经利咽糖浆与RO8191干预后,检测大鼠一般情况及咽部表观状态,并进行咽部表观状态评分;HE染色检测大鼠咽部病理形态变化;流式细胞术检测大鼠外周血T淋巴细胞亚群CD4^(+)T与CD8^(+)T表达、CD4^(+)T/CD8^(+)T;试剂盒检测大鼠血清肿瘤坏死因子-α(TNF-α)、IL-6、IL-10、丙二醛(MDA)、活性氧(ROS)、总抗氧化能力(T-AOC)水平;以免疫印记法检测大鼠咽部组织JAK2/STAT3通路相关蛋白表达。结果:与对照组比较,模型组大鼠咽部组织出现明显病理形态损伤,外周血CD4^(+)T与CD4^(+)T/CD8^(+)T、IL-10、血清T-AOC水平降低(P<0.05),咽部表观状态评分、CD8^(+)T、血清TNF-α、IL-6、MDA与ROS水平、咽部组织p-JAK2/JAK2与p-STAT3/STAT3水平升高(P<0.05);与模型组、利咽糖浆+RO8191组相比,利咽糖浆组大鼠咽部组织病理形态损伤减轻,外周血CD4^(+)T表达与CD4^(+)T/CD8^(+)T、IL-10、血清T-AOC水平均升高(P<0.05),咽部表观状态评分、CD8^(+)T、血清TNF-α、IL-6、MDA与ROS水平、咽部组织p-JAK2/JAK2与p-STAT3/STAT3水平均降低(P<0.05);RO8191组大鼠各指标变化趋势与利咽糖浆组相反。结论:利咽糖浆可通过抑制JAK2/STAT3信号激活减轻炎症及氧化应激,增强抗炎及抗氧化能力,缓解慢性咽炎大鼠咽喉组织损伤,修复咽黏膜。

苋黄洗剂联合复方敏维糖浆治疗儿童湿疹的疗效及对炎性因子的影响

目的探讨苋黄洗剂联合复方敏维糖浆对小儿湿疹的治疗效果并分析其对炎性因子的影响。方法选择2023年1月至2023年9月在我院接受治疗的108例湿疹患儿,按照随机数字表法将病例组分为治疗A组(54例)和治疗B组(54例),A组采用复方敏维糖浆治疗,B组采用苋黄洗剂联合复方敏维糖浆治疗,对两组患儿的临床疗效、EASI评分及治疗前后细胞因子白介素-2(IL-2)、白介素-4(IL-4)、白介素-10(IL-10)、干扰素-γ(IFN-γ)水平进行比较。结果治疗一疗程后两组EASI评分均较治疗前下降,其中B组EASI评分低于A组(P<0.05),B组治疗总有效率高于A组,复发率低于A组(P<0.05)。治疗后,2组IL-4、IL-10水平均较治疗前降低,IL-2与IFN-γ水平均较治疗前升高,且B组降低或升高的程度大于A组。结论苋黄洗剂联合复方敏维糖浆对小儿湿疹的治疗效果较单一复方敏维糖浆治疗显著,联合治疗可降低复发率,提高有效率,减轻炎症反应,改善免疫平衡,安全可靠,值得临床推广应用。

复方氢溴酸右美沙芬糖浆治疗小气道功能障碍致咳嗽的临床效果

目的:分析复方氢溴酸右美沙芬糖浆治疗小气道功能障碍致咳嗽的临床效果。方法:选取2022年8月—2024年1月于北京市垂杨柳医院呼吸与危重症医学科就诊的80例小气道功能障碍致咳嗽患者作为研究对象,随机分为对照组和观察组,各40例。对照组给予川贝枇杷糖浆治疗,观察组给予复方氢溴酸右美沙芬糖浆治疗。比较两组咳嗽程度、小气道功能指标和肺功能指标。结果:治疗前,两组日间咳嗽、夜间咳嗽评分及总分比较,差异无统计学意义(P>0.05);治疗后,两组日间咳嗽、夜间咳嗽评分及总分均低于治疗前,且观察组低于对照组(P<0.05)。治疗前,两组用力呼出50%肺活量的呼气流量(FEF50%)、用力呼出75%肺活量的呼气流量(FEF75%)、最大呼气中期流量(MMEF)比较,差异无统计学意义(P>0.05);治疗后,两组FEF50%、FEF75%、MMEF均高于治疗前,且观察组高于对照组(P<0.05)。治疗前,两组第1秒用力呼气容积(FEV_(1))、用力肺活量(FVC)、呼气流量峰值(PEF)比较,差异无统计学意义(P>0.05);治疗后,两组FFEV_(1)、FVC、PEF均高于治疗前,且观察组高于对照组(P<0.05)。结论:复方氢溴酸右美沙芬糖浆治疗小气道功能障碍致咳嗽的临床效果显著,能够缓解咳嗽症状,改善小气道功能和肺功能。

炎宁糖浆联合干扰素治疗对急性上呼吸道感染患儿炎症和症状恢复的影响

目的:观察炎宁糖浆联合干扰素治疗对急性上呼吸道感染患儿炎症和症状恢复的影响。方法:选取华中科技大学协和江北医院2021年12月—2023年12月收治的120例急性上呼吸道感染患儿作为研究对象,按照随机数表法分为研究组和对照组,每组各60例。对照组采用干扰素治疗,研究组在对照组基础上联用炎宁糖浆,比较两种方案的临床疗效。结果:研究组治疗总有效率和CD3^(+)、CD4^(+)水平高于对照组,中医证候积分、CD8^(+)、白细胞计数(WBC)、中性粒细胞、C反应蛋白(CRP)水平低于对照组,止咳时间、鼻塞缓解时间和退热时间短于对照组,差异有统计学意义(P<0.05)。结论:炎宁糖浆联合干扰素治疗急性上呼吸道感染效果较好,能够显著缓解患儿临床症状,减轻呼吸道炎症刺激,提高其免疫功能,治疗实用性更高。

微滤联合大孔树脂纯化蓝莓果脯糖浆中花色苷的工艺优化

本文使用微滤技术联合大孔树脂技术对蓝莓果脯糖浆分离纯化,以回收其中的蓝莓花色苷。首先通过静态试验对大孔树脂进行筛选,并在动态试验中以花色苷回收率和总糖去除率为指标,在单因素和Box-Behnken响应面试验的基础上,优化了上样流速、乙醇浓度和洗脱流速等工艺参数。结果表明:微滤透过液中花色苷含量为6.84 mg/100 mL,总糖含量为31.76 mg/mL。AB-8、D101、HPD600三种树脂中,优选出AB-8用于蓝莓果脯花色苷的纯化,大孔树脂纯化花色苷最佳工艺条件为:上样流速2.0 mL/min、乙醇浓度83%、洗脱流速2.0 mL/min,该条件下花色苷的回收率为86.68%,总糖去除率为84.13%。花色苷解吸液经旋转蒸发浓缩后,花色苷质量浓度为5.56 mg/100 mL,总糖浓度为5.24 mg/mL,花色苷的色价为31.43,约为原料中花色苷色价的5倍。微滤联合大孔树脂纯化蓝莓果脯糖浆花色苷效果明显,本研究为蓝莓花色苷的分离纯化提供了一定的技术参考。

儿童咳嗽变异性哮喘气道重塑发病机制及抗支糖浆作用机制研究进展

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