Fatal metabolic stroke in a child with propionic acidemia 11 years post liver transplant

Propionic acidemia is a rare autosomal recessive inborn error of metabolism caused by a deficiency of propionyl CoA carboxylase which often manifests with frequent metabolic decompensations and risk of neurological injury. Outcomes with medical therapy remain suboptimal. Liver transplantation has been shown to be a therapeutic option for patients and results in a milder phenotype of the disease and partial correction of the enzyme defect. Liver transplantation has been increasingly reported over the last decade and experience in managing these patients is improving. Long-term outcomes are generally good; however, the risk of complications still exists despite transplantation. We report a child who presented with a fatal metabolic stroke 11 years post liver transplant without any biochemical evidence of decompensation. We highlight the need to closely monitor these patients lifelong despite liver transplantation and maintain multidisciplinary working between hepatology and metabolic clinicians.     

New drugs for the treatment of coronary artery syndromes: otamixaban and ticagrelor

Acute coronary syndromes are a major cause of mortality and morbidity. The objective of this evaluation is to review the clinical trials of two new drugs being developed for the treatment of acute coronary syndromes. The first drug is the anticoagulant otamixaban, and the trial compared otamixaban with unfractionated heparin and eptifibatide in acute coronary syndromes. The second drug is the antiplatelet ticagrelor, and the trial compared ticagrelor with clopidogrel in acute coronary syndromes. In the SEPIA-ACS1 TIMI 42 trial, the primary efficacy endpoint occurred in 6.2% of subjects treated with unfractionated heparin and eptifibatide, and to a significantly lesser extent with otamixaban. In the PLATO trial, the primary efficacy endpoint had occurred less in the ticagrelor group (9.8%) than in the clopidogrel group (11.7%) at 12 months. Two new drugs for acute coronary syndromes, otamixaban and ticagrelor, have recently been shown to have benefits in subjects undergoing percutaneous interventions compared to the present standard regimens for this condition.     

伏九贴敷治疗儿童反复咳喘100例临床观察

目的：观察“伏九贴敷”治疗儿童反复咳喘的临床疗效。方法：采用随机对照的方法，将200例反复咳喘患几分成观察组（贴敷组）和对照组（非贴敷组）各100例。观察组给予伏九贴敷治疗，2年为1个疗程。结果：“伏九贴敷”在平喘及消除肺部哮鸣音等方面，显效84例，有效13例，无效3例，总有效率97％，两组有显著性意义。结论：“伏九贴敷”用于治疗咳喘疗效确切，值得临床进一步推广。     

X-连锁低血磷性佝偻病的治疗现状及展望

X-连锁低血磷性佝偻病(XLH)属于遗传性代谢性骨病,发病机制复杂,在临床上较为罕见,其主要临床表现为骨骼畸形及身材矮小,致残率高,因而针对XLH进行及早诊断及治疗对患者康复具有极为重要的意义。传统治疗以补充磷酸盐和维生素D类似物为主,随着学者对XLH病理机制研究逐渐深入,分子靶向治疗等新型治疗方式横空出世,通过多学科联合诊断及治疗的方法为患者带去了福音。本文就XLH的临床特征、发病遗传机制、治疗现状及治疗的未来发展趋势进行综述。     

临床血液流变学指标检测报告单设计不合理应该修正

笔者发现虽然血液流变学广泛用于l临床已经20几年，但其指标检测报告单的指标设置仍然比较混乱。报告单设计不合理的现象普遍存在。笔者为了纠正这种现象而撰写此文。研究指出了哪些是科学合理必须的指标和设置这些指标的两方面依据。论文阐明了全血高剪变率、中剪变率、低剪变率黏度，红细胞变形性、聚集性指标的正确含义，并将若干指标的优劣性进行了分析、比较。笔者为血液流变学首次提出了红细胞变形率、红细胞聚集率这两个新概念。研究揭示报告单指标设置不合理性归纳为三个方面：指标太多，指标重复，指标无意义。产生这些不合理性的缘由是：检测仪器厂商炫耀其仪器功能齐全，利于销售；设计者和检验人员血液流变学知识浅薄，指标良莠不分。但愿此文能引起人们重视，共同促进临床血液流变学指标设置和检测规范化。     

穴位贴敷治疗变应性鼻炎的临床观察

目的观察穴位贴敷治疗变应性鼻炎的临床疗效。方法将60例患者随机分为治疗组和对照组,每组30例。治疗组采用白芥子、延胡索、细辛、甘遂按2：2：1：1混合研为细末,对照组用面粉,两组药物均用鲜姜汁调成糊状,均于大椎、风门（双）、肺俞（双）、膏肓（双）、志室（双）等穴进行贴敷,每10d1次,5次为1个疗程,共治疗1个疗程。治疗前后对患者的临床症状和体征进行评分,并于疗程结束后3个月进行随访,随访指标为临床症状和体征。结果治疗组临床症状及体征在治疗后和随访时分值均降低,治疗前后差异有统计学意义（P〈0.05）;对照组除流清涕与治疗前比较有差异外,其余症状和体征与治疗前比较差异均无统计学意义（P〉0.05）。治疗组治疗后症状总分、体征总分与对照组比较下降明显（P〈0.05）。结论穴位贴敷能有效地改善变应性鼻炎的临床症状和体征。