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Densitometric and Ratio Spectra Methods for Simultaneous Determination of Sulfaquinoxaline Sodium and Pyrimethamine in Binary Mixture 密度法和比值光谱法同时测定二元混合物中磺胺喹啉钠和乙胺嘧啶的含量
Pub Date : 2019-06-01 DOI: 10.21608/bfpc.2019.7290.1002
S. Razeq, Asmaa O. El Demerdash, M. Fouad, Hoda F. El Sanabary
Four methods were developed for simultaneous determination of sulfaquinoxaline-Na and pyrimethamine in their combined pharmaceutical formulations. The first one was a densitometric method where chloroform-methanol (9: 1, v/v) was the developing system and the plates were scanned at 254 nm. The obtained spots appeared at Rf 0.64 and 0.35 and determined in the range of 0.5-10.0 μg/spot and 0.1-10.0 μg/spot for sulfaquinoxaline-Na and pyrimethamine, respectively. Furthermore, three spectrophotometric methods manipulating ratio spectra namely, ratio difference method, extended ratio subtraction method coupled with ratio subtraction method and mean centering method were established for the determination of the two studied drugs in the presence of propylene glycol as a solvent. Linear correlation was found over the concentration range of 2-25 μg mL-1 and 3-15 μg mL-1 for the two drugs, respectively. The proposed methods were successfully applied for analyzing the cited drugs in their veterinary pharmaceutical formulations. The obtained results were statistically analyzed and found to be in accordance with those given by a reported method. The validity of the methods was evaluated according to ICH guidelines.
建立了四种同时测定复方磺胺喹啉钠和乙胺嘧啶含量的方法。第一种是密度法,以氯仿-甲醇(9:1,v/v)为显影体系,在254 nm处扫描。得到的斑点出现在Rf为0.64和0.35的范围内,磺胺喹啉钠和乙胺嘧啶的测定范围分别为0.5 ~ 10.0 μg/斑点和0.1 ~ 10.0 μg/斑点。建立了三种操作比值光谱的分光光度法,即比值差法、扩展比值减法耦合比值减法和平均居中法,用于丙二醇存在下测定两种研究药物。两种药物分别在2 ~ 25 μg mL-1和3 ~ 15 μg mL-1浓度范围内呈线性相关。所建立的方法已成功地应用于兽药配方中被引药物的分析。对所得结果进行了统计分析,发现与报道的方法一致。根据ICH指南评估方法的有效性。
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引用次数: 3
Anti-inflammatory and Anti-apoptotic Potentials of Apigenin against Liver Injury Induced by Ischemia-Reperfusion in Rats 芹菜素对大鼠缺血再灌注肝损伤的抗炎和抗凋亡作用
Pub Date : 2019-06-01 DOI: 10.21608/BFPC.2019.13703.1040
Dalia M. El-Tanbouly, Hala F Zaki, R. Abdelsalam, A. Zaki
Apigenin is a dietary flavonoid that exists copiously in several herbs and vegetables. It exhibits anti-inflammatory, anti-mutagenesis, anti-proliferative and antioxidant properties. The present work aimed to investigate some of mechanisms underlying protective potential of apigenin in hepatic ischemia-reperfusion injury. Rats were divided into four groups; sham-operated, sham-operated pretreated with apigenin (25 mg/kg, p.o.), ischemia/reperfusion (I/R) (30 min ischemia and 1 h reperfusion) and I/R pretreated with apigenin. Compared with I/R group, pretreatment with apigenin markedly reduced transaminases levels and ameliorated tissue histopathological changes. Apigenin significantly reduced high mobility group box 1 (HMGB1) expression and suppressed liver tumor necrosis factor- α (TNF-α), nuclear factor κB (NF-κB) and myeloperoxidase (MPO) activity. Moreover, apigenin restored reduced glutathione (GSH), decreased liver lipid peroxidation, and boosted glutathione peroxidase (GPx) activity in addition to attenuation of apoptosis by increasing Bcl-2/Bax ratio. It may thus be concluded that inhibition of HMGBI by apigenin plays a role towards its antioxidant, anti-inflammatory as well as anti-apoptotic properties which are involved in conferring its hepato-protective properties
芹菜素是一种膳食类黄酮,大量存在于几种草药和蔬菜中。它具有抗炎、抗突变、抗增殖和抗氧化的特性。本研究旨在探讨芹菜素在肝缺血再灌注损伤中的保护作用机制。大鼠分为四组;假手术、假手术预处理芹菜素(25 mg/kg, p.o.)、缺血/再灌注(I/R)(缺血30 min,再灌注1 h)和芹菜素预处理I/R。与I/R组相比,芹菜素预处理显著降低转氨酶水平,改善组织病理改变。芹菜素显著降低高迁移率组盒1 (HMGB1)表达,抑制肝脏肿瘤坏死因子-α (TNF-α)、核因子κB (NF-κB)和髓过氧化物酶(MPO)活性。此外,芹菜素通过提高Bcl-2/Bax比值,恢复还原性谷胱甘肽(GSH),降低肝脏脂质过氧化,提高谷胱甘肽过氧化物酶(GPx)活性,抑制细胞凋亡。由此可见,芹菜素对HMGBI的抑制作用可能与其抗氧化、抗炎和抗凋亡特性有关,这些特性参与了其保肝特性的形成
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引用次数: 4
QbD-based design and characterization of mucoadhesive microspheres of quetiapine fumarate with improved oral bioavailability and brain biodistribution potential 基于qbd的富马酸奎硫平黏附微球的设计与表征,提高了其口服生物利用度和脑生物分布潜力
Pub Date : 2018-12-01 DOI: 10.1016/j.bfopcu.2018.09.002
Someshwar Komati , Suryakanta Swain , Muddana Eswara Bhanoji Rao , Bikash Ranjan Jena , Sambamoorthy Unnam , Vishali Dasi

The present work aims to discuss on Quality by Design based development and characterization of the sustained release mucoadhesive microspheres of quetiapine fumarate. The microspheres were prepared by non-aqueous solvent evaporation process. Factor screening study was carried out using fractional factorial design for identifying the influential factors. Systematic optimization of microspheres was accomplished by Box-Behnken design and characterized for particle size, entrapment efficiency, in vitro drug release and ex vivo mucoadhesion strength, which indicated that microspheres were consequence to be spherical and free flowing in nature. The microspheres exhibited high drug entrapment efficiency and in vitro drug release in a sustained manner, which was considered to be dependent on the concentration of rate controlling polymers. Ex vivo wash-off test on microspheres indicated good mucoadhesive property on excised goat intestinal mucosa. Out of all the accepted formulation, F6 was preferred as the optimized formulation. In vivo pharmacokinetic and brain biodistribution study revealed significant increase in the levels of drug in blood plasma and brain homogenates from the optimized formulation vis-à-vis the pure drug suspension. Overall, current study corroborated significant improvement in the biopharmaceutical attributes of quetiapine fumarate from mucoadhesive microspheres, which can be effectively used for management of depression and schizophrenia.

本研究旨在探讨富马酸奎硫平黏附微球的质量设计与表征。采用非水溶剂蒸发法制备微球。采用分数因子设计进行因子筛选研究,确定影响因素。采用Box-Behnken设计对微球进行了系统优化,并对微球的粒径、包封效率、体外释药和体外黏附强度进行了表征,表明微球本质上是球形的、自由流动的。微球具有较高的药物包封效率和体外药物持续释放能力,这与控速聚合物的浓度有关。体外冲洗试验表明,微球对山羊切除肠黏膜具有良好的黏附性能。在所有可接受的配方中,优选F6为最佳配方。体内药代动力学和脑生物分布研究表明,与-à-vis纯药物悬浮液相比,优化后的配方显著提高了血浆和脑匀浆中的药物水平。总的来说,目前的研究证实了从黏附微球制备的富马酸奎硫平的生物制药特性有显著改善,可以有效地用于治疗抑郁症和精神分裂症。
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引用次数: 16
Formulation optimization and evaluation of fast dissolving film of aprepitant by using design of experiment 用实验设计法对阿瑞吡坦速溶膜配方进行优化及评价
Pub Date : 2018-12-01 DOI: 10.1016/j.bfopcu.2018.04.002
Rajni Bala , Shailesh Sharma , IKGPTU

The concept of fast dissolving dosage form has become popular as new delivery system. This system will provide maximum therapeutic efficacy, increased bioavailability and maximum stability by reducing the frequency of dosage. It will also avoid first pass metabolism of the drugs. This system provides more rapid drug absorption from the pre gastric area which may provide quick onset of action. The present research aimed to prepare fast dissolving films (FDF) of aprepitant used in the prevention and treatment of chemotherapy-induced nausea and vomiting. The FDF was prepared using solvent casting method and optimized employing central composite design considering two independent variables film forming polymer (pullulan) and PEG 400. Disintegration time, wetting time, drug release and folding endurance were taken as dependent variables. The prepared optimized formulation showed minimum disintegration time (20 s), highest dissolution rate (88.87%) and satisfactory physicochemical properties. It is evident from the above results that the developed formulation can be an innovative dosage form to improve the drug delivery, onset of action as well as improve patient compliance.

快速溶解剂型作为一种新型给药系统,已成为一种流行的给药方式。通过减少给药频率,该系统将提供最大的治疗效果,提高生物利用度和最大的稳定性。它还可以避免药物的第一次代谢。该系统提供更快的药物吸收从胃前区域,这可能提供快速起效。本研究旨在制备阿瑞吡坦快速溶解膜(FDF),用于预防和治疗化疗引起的恶心和呕吐。采用溶剂铸造法制备FDF,并考虑成膜聚合物(普鲁兰)和peg400两个自变量,采用中心复合设计对FDF进行优化。以崩解时间、润湿时间、药物释放和折叠时间为因变量。优化后的配方崩解时间最短(20 s),溶出率最高(88.87%),理化性质满意。从上述结果可以明显看出,所开发的制剂可以是一种创新剂型,以改善药物输送、起效以及改善患者依从性。
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引用次数: 42
Stability indicating methods for selective determination of Oxetacaine in the presence of its degradation products 稳定性表明选择性测定奥西他卡因降解产物的方法
Pub Date : 2018-12-01 DOI: 10.1016/j.bfopcu.2018.10.002
Eman S. Elzanfaly , Enas A. Amer , Sara A. Galal , Hala E. Zaazaa

Two methods, HPLC and TLC were presented for the determination of Oxetacaine (OXT) in the existence of its different degradation products. HPLC method was based on the separation of OXT from its degradation products using reversed phase C18 column at room temperature and isocratic elution with mobile phase mixture of acetonitrile: 5 mM sodium dihydrogen orthophosphate dihydrate, pH was adjusted to 2.4 with orthophosphoric acid (50:50 v/v). Quantitation was based on peak area at 210 nm. The second TLC-densitometric method relies on the separation and quantitation of OXT from its degradation products on TLC silica gel 60 F254 plates, using 2-propanol: triethylamine (10:0.5 v/v) as a developing system and densitometric measurement of the developed bands at 210 nm. Validation of the proposed methods was performed according to the ICH guidelines and applied to evaluate the stability of OXT under different stress conditions.

采用高效液相色谱法和薄层色谱法测定存在不同降解产物的奥西他卡因(OXT)的含量。高效液相色谱法采用反相C18柱在室温下分离OXT及其降解产物,流动相为乙腈:5 mM正磷酸二氢钠,正磷酸(50:50 v/v)调节pH为2.4。定量依据210 nm处的峰面积。第二种TLC-密度法是在TLC硅胶60f254板上,以2-丙醇:三乙胺(10:0.5 v/v)为显色体系,在210 nm处对OXT的降解产物进行分离和定量。根据ICH指南对所提出的方法进行验证,并应用于评估OXT在不同应力条件下的稳定性。
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引用次数: 1
Cynara scolymus (artichoke) and its efficacy in management of obesity 洋蓟及其治疗肥胖症的疗效
Pub Date : 2018-12-01 DOI: 10.1016/j.bfopcu.2018.10.003
Mohaddese Mahboubi

Obesity, the most prevalent metabolic disorder is associated with elevated body fat mass and body mass index. Cynara scolymus L. is famous for its hepatoprotective properties, also it seems to have good potency as anti-obese agent. In this review article, the potency of C. scolymus as anti-obese agent has been evaluated. The evidences based information were extracted from accessible international electronic databases (PubMed, Springer, Science Direct, Wiley and Google), and books (Persian or English), by key word of Cynara scolymus and artichoke plus obesity or the mechanism of anti-obese drugs. C. scolymus inhibits the digestive enzymes such as pancreas lipase, α-amylase, α-glucosidase, increases the bile secretion, inhibits of inflammation and ROS, improves liver function, gut microbiota, enhances lipolysis and lipid metabolism, and reduces blood glucose in preclinical and clinical studies. Designing large multi-center clinical trials on C. scolymus and evaluating its effects on weight loss in comparison with famous drug such as orlistate could be the subject of future studies.

肥胖是最普遍的代谢紊乱,与身体脂肪量和身体质量指数升高有关。猪头草以其保肝作用而闻名,似乎也具有良好的抗肥胖功效。本文对猪头草作为抗肥胖药物的作用进行了评价。以Cynara scolymus和artichoke加肥胖或抗肥胖药物作用机制为关键词,从国际可及电子数据库(PubMed、Springer、Science Direct、Wiley和Google)和图书(波斯语或英语)中提取循证信息。在临床前和临床研究中,鹅牙菇抑制胰腺脂肪酶、α-淀粉酶、α-葡萄糖苷酶等消化酶,增加胆汁分泌,抑制炎症和ROS,改善肝功能,改善肠道菌群,促进脂肪分解和脂质代谢,降低血糖。设计大型多中心临床试验,评估其与奥利司酸等知名药物的减肥效果,可能是未来研究的主题。
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引用次数: 13
Evaluation of the physician’s acceptance to clinical pharmacy interventions after antibiotic stewardship implementation in the ICU in a general hospital in Egypt 埃及一家综合医院ICU实施抗生素管理后医师对临床药学干预的接受程度评估
Pub Date : 2018-12-01 DOI: 10.1016/j.bfopcu.2018.05.001
Noha Elkassas , Maggie Abbassi , Samar Farid

Background

Physicians’ perception of the role of the clinical pharmacist role plays a cornerstone in accepting interventions suggested by pharmacists to correct DRPs and in complying with guidelines of a pharmacist-led Antibiotic stewardship program (ASP). This study aimed at evaluating the acceptance of physicians’ to pharmacists interventions to antibiotic prescribing and the change in antibiotic consumption in Al-Haram general hospital Intensive care unit (ICU).

Methods

This study was performed in Al-Haram general hospital ICU from July 2014 till December 2015. Medication review of antibiotics started in Al-Haram hospital ICU in July 2014 by responsible clinical pharmacists. An on-job physician education program about antibiotics started in June 2015. Implementation of ASP started in July 2015. The antibiotic related interventions and response of physicians to interventions were recorded all along the study period. The pattern of physicians’ acceptance along with antibiotic consumption (in Daily define dose per 1000 patient-days) were reported.

Results

The number of accepted interventions had increased along the study. The overall antibiotic consumption increased after implementation of ASP, however the individual pattern of antibiotic prescribing changed.

Conclusion

The physicians in Al-Haram hospital appeared to accept the role of clinical pharmacist in correcting antibiotic-related DRPs as well as in implementing ASP.

背景:医生对临床药师角色的认识是接受药师建议的干预措施以纠正DRPs和遵守药师主导的抗生素管理计划(ASP)指南的基石。本研究旨在评估Al-Haram综合医院重症监护病房(ICU)医生对药剂师干预抗生素处方的接受程度和抗生素消费的变化。方法本研究于2014年7月至2015年12月在Al-Haram综合医院ICU进行。Al-Haram医院ICU于2014年7月开始由临床负责药师对抗生素进行用药审查。2015年6月开始了一项关于抗生素的在职医师教育项目。ASP于2015年7月开始实施。在整个研究期间记录抗生素相关干预措施和医生对干预措施的反应。报告了医生接受抗生素的模式以及抗生素的消耗量(每1000病人日的每日定义剂量)。结果接受的干预措施数量随着研究的进行而增加。实施ASP后,总体抗生素消耗量增加,但个体抗生素处方模式发生变化。结论Al-Haram医院的医生对临床药师在纠正抗生素相关drp和实施ASP方面的作用表示认可。
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引用次数: 5
Evaluation of antioxidant properties of different extracts of Chaetomium cupreum SS02 铜毛囊SS02不同提取物抗氧化性能的评价
Pub Date : 2018-12-01 DOI: 10.1016/j.bfopcu.2018.08.001
Nazir Ahmad Wani , Sharmila Tirumale

To evaluate the antioxidant properties of different extracts (chloroform, ethyl acetate, n-butanol and methanol extracts) of fungus C. cupreum SS02. The antioxidant properties were evaluated by using five antioxidant methods, metal chelating assay, 2,2-azino-bis (3-ethylbenzthiazoline-6-sulphonic acid (ABTS), hydroxyl radical (HO) scavenging assay, superoxide anion (O2) radical scavenging assay, and nitric oxide (NO) scavenging assay. Methanol extract of C. cupreum showed highest metal chelating activity (63.61 ± 0.07 mg EDTAE/g DW), followed by chloroform extract (57.25 ± 0.12 mg E DTAE/g DW) and n-butanol extract (27.33 ± 0.07 mg E DTAE/g DW) whereas ethyl acetate extract showed least metal chelating activity (18.35 ± 0.07 mg E DTAE/g DW) at 50 µg/ml. In ABTS assay, n-butanol extract showed highest ABTS inhibition activity (19.91 ± 0.14 μmol RE/g DW), followed by ethyl acetate extract (13.07 ± 0.59 μmol RE/g DW) and chloroform extract (13.07 ± 0.82 μmol RE/g DW) whereas methanol extract showed the lowest activity (11.65 ± 0.16 μmol RE/g DW at 50 µg/ml). In hydroxyl radical scavenging assay, n-butanol extract showed highest scavenging activity (31.95 ± 0.21 mg RE/g DW), followed by ethyl acetate extract (28.19 ± 0.21 mg RE/g DW), methanol extract (22.93 ± 0.37 mg RE/g DW) and chloroform extract (18.04 ± 0.21 mg RE/g DW) at 50 µg/ml. In superoxide anion scavenging assay, chloroform extract showed highest scavenging activity (56.44 ± 0.03 mg RE/g DW) followed by ethyl acetate extract (49.88 ± 0.09 mg RE/g DW), n-butanol extract (19.49 ± 0.09 mg RE/g DW) and methanol extract (7.75 ± 0.06 mg RE/g DW) at 50 µg/ml. The highest inhibition of nitric oxide radical was observed in chloroform extract of C. cupreum (11.23 ± 0.11%) followed by ethyl acetate extract (7.62 ± 0.06%), n-butanol extract (4.72 ± 0.90%) and methanol extract (3.20 ± 0.06%) at 50 µg/ml. The results showed that different extracts of C. cupreum have significant antioxidant activity due to the presence of different phytochemicals. Thus, it is suggested that C. cupreum extracts should be further studied for their antioxidant properties for food and pharmaceutical applications.

考察铜铜菌(C. cupreum SS02)不同提取物(氯仿、乙酸乙酯、正丁醇和甲醇提取物)的抗氧化性能。采用金属螯合法、2,2-氮基-双(3-乙基苯并噻唑-6-磺酸(ABTS))法、羟基自由基(HO)清除法、超氧阴离子(O2)自由基清除法和一氧化氮(NO)清除法等5种抗氧化方法评价其抗氧化性能。甲醇提取物c cupreum显示,金属螯合活性最高(63.61 ±0.07  mg EDTAE / g DW),紧随其后的是氯仿提取物(57.25 ±0.12  E mg DTAE / g DW)和正丁醇提取物(27.33 ±0.07  E mg DTAE DW / g),而乙酸乙酯提取显示至少金属螯合活动(18.35 ±0.07  E mg DTAE / g DW)在50岁 µg / ml。abt化验,正丁醇提取物显示abt抑制活性最高(19.91 ±0.14  μ摩尔RE / g DW),其次是乙酸乙酯提取物(13.07 ±0.59  μ摩尔RE / g DW)和氯仿提取物(13.07 ±0.82  μ摩尔RE / g DW)而甲醇提取显示最低的活动(11.65 ±0.16  μ摩尔RE / g DW 50岁 µg / ml)。氢氧自由基清除实验,正丁醇提取物显示清除活性最高(31.95 ±0.21  mg RE / g DW),其次是乙酸乙酯提取物(28.19 ±0.21  mg RE / g DW),甲醇提取物(22.93 ±0.37  mg RE / g DW)和氯仿提取物(18.04 ±0.21  mg RE / g DW)在50岁 µg / ml。超氧化物阴离子的清除试验,氯仿提取物显示清除活性最高(56.44 ±0.03  mg RE / g DW)其次是乙酸乙酯提取物(49.88 ±0.09  mg RE / g DW),正丁醇提取物(19.49 ±0.09  mg RE / g DW)和甲醇提取物(7.75 ±0.06  mg RE / g DW)在50岁 µg / ml。的最高抑制一氧化氮自由基在氯仿提取物c cupreum(11.23 ± 0.11%)其次是乙酸乙酯提取物(7.62 ± 0.06%),正丁醇提取物(4.72 ± 0.90%)和甲醇提取物(3.20 ± 0.06%)在50岁 µg / ml。结果表明,铜柏不同提取物具有明显的抗氧化活性,这是由于其含有不同的植物化学物质。因此,铜铜提取物在食品和医药领域的抗氧化性能有待进一步研究。
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引用次数: 7
Formulation and evaluation of gastro retentive tablets of clarithromycin prepared by using novel polymer blend 新型聚合物共混法制备克拉霉素保胃片的配方及评价
Pub Date : 2018-12-01 DOI: 10.1016/j.bfopcu.2018.07.001
Sharwaree Hardikar , Ashok Bhosale

Clarithromycin is a semi synthetic macrolide antibiotic that is proved to be extremely effective mono therapy in treating H. pylori infection. One of the various reasons reported for partial eradication of H. pylori infection is that the short dwelling time of antimicrobial agents in the stomach. Hence the principal object of the present work was to design and develop floating and swellable gastro retentive tablet of clarithromycin to obtain its prolonged release in the stomach. HPMC K 100LV was blended in the present study with the Gel isolated from the seeds of Ocimum basilicum to overcome the drawback of time dependent erosion of former. This novel polymer blend was investigated and exploited as a matrix material for the development of gastro retentive tablets of clarithromycin as drug delivery system. Hydrogen bonding between the Glucomannan – a component of the Gel and HPMC K100LV was envisaged and further confirmed by ATR-FTIR. Tablets of clarithromycin prepared by using the polymer blend of Gel and HPMC K100LV exhibited fast swelling with almost instantaneous buoyancy followed by prolonged release of drug from the swollen, floating dosage form. The retention of the optimized tablet formulation of clarithromycin in the stomach was confirmed by γ scintigraphy.

克拉霉素是一种半合成的大环内酯类抗生素,被证明是治疗幽门螺杆菌感染非常有效的单一疗法。据报道,部分根除幽门螺杆菌感染的各种原因之一是抗菌药物在胃中的停留时间短。因此,本研究的主要目的是设计和研制克拉霉素浮胀胃留置片,使克拉霉素在胃中缓释。本研究将HPMC k100lv与从罗勒种子中分离的凝胶混合,以克服前者随时间侵蚀的缺点。研究了这种新型聚合物共混物,并将其作为制备克拉霉素胃留置片的基质材料。设想了凝胶组分葡甘露聚糖与HPMC K100LV之间的氢键,并通过ATR-FTIR进一步证实。凝胶与HPMC K100LV聚合物共混制备的克拉霉素片剂表现出快速肿胀,几乎瞬间浮力,随后药物从肿胀的浮动剂型中释放时间延长。通过γ闪烁图证实了优化后的克拉霉素片剂在胃中的保留率。
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引用次数: 9
Differential diagnosis of mucopolysaccharidosis and oligosaccharidosis of a sample of Egyptian children 埃及儿童粘多糖病和寡糖病的鉴别诊断
Pub Date : 2018-12-01 DOI: 10.1016/j.bfopcu.2018.10.005
Ekram Fateen , Manal Fouad Ismail , Noha Ahmed El-Boghdady , Mona Aglan , Mona Ibrahim , Amira Radwan

Mucopolysaccharidosis (MPS) and oligosaccharidosis are lysosomal storage disorders (LSDs) that share many clinical features. The present study aimed to establish a protocol for the biochemical diagnosis of these disorders and their subtypes in affected Egyptian children as well as in pregnant females, in order to prepare children or fetus for enzyme replacement therapy. Urine, plasma and leukocyte samples were collected from 280 children with symptoms suggestive of LSDs. Fourteen amniotic fluid samples were collected from pregnant females having positive family history or having one affected sibling. Assessment of urinary glycosaminoglycans (GAGs) followed by two dimensional electrophoresis (2-DEP), thin layer chromatographic (TLC) for separation of oligosaccharides and plasma or leukocyte enzyme activity were performed. Six of pregnancies were diagnosed to have affected fetuses. 84 children had abnormal 2-DEP and classified as 26 MPS I, 10 MPS II, 24 MPS III and 24 MPS VI. Two were diagnosed as α-mannosidosis and 2 as GM1 gangliosidosis. In conclusion; MPS should be excluded before suspecting oligosaccharidosis. 2-DEP and TLC alone cannot rule out the diagnosis of either MPS or oligosaccharidosis and confirmation must be done by specific lysosomal enzymatic assay. Analysis of GAGs by 2-DEP in amniotic fluid can be promising method for prenatal diagnosis of MPS.

粘多糖病(MPS)和寡糖病是溶酶体储存障碍(lsd),具有许多共同的临床特征。本研究旨在为受影响的埃及儿童和孕妇建立一种对这些疾病及其亚型的生化诊断方案,以便为儿童或胎儿进行酶替代治疗做好准备。收集了280名有lsd症状的儿童的尿液、血浆和白细胞样本。从有阳性家族史或有一个患病兄弟姐妹的孕妇中收集了14份羊水样本。通过二维电泳(2-DEP)、薄层色谱(TLC)分离低聚糖和血浆或白细胞酶活性评估尿糖胺聚糖(GAGs)。其中6名孕妇被诊断为胎儿受影响。2- dep异常84例,分为MPS I型26例、MPS II型10例、MPS III型24例、MPS VI型24例,其中2例诊断为α-甘露糖醇病,2例诊断为GM1神经节脂醇病。结论;在怀疑寡糖病之前应排除MPS。单独的2-DEP和TLC不能排除MPS或寡糖病的诊断,必须通过特定的溶酶体酶测定来确认。羊水中2-DEP测定GAGs是产前诊断MPS的一种有前景的方法。
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引用次数: 2
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Bulletin of Faculty of Pharmacy, Cairo University
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