Clinical manifestations of liver in congenital disorders of glycosylation / 国际儿科学杂志

Congenital disorders of glycosylation(CDG)are caused by defects in the synthesis of glycans and their binding to proteins and lipids.Up to now, over 100 types of CDG have been identified.This disease can cause functional damage to multiple organs including liver, kidney and heart.The CDG types with predominant or isolated liver involvement including MPI-CDG, TMEM199-CDG, CCDC115-CDG, and ATP6AP1-CDG.Their clinical manifestations include enlarged liver, elevated transaminase, cholestasis, liver fibrosis, liver failure, abnormal ceruloplasmin, and copper accumulation.Their pathological manifestations include steatosis, fibrosis, liver cirrhosis, cholestasis, and abnormal bile duct.This review mainly describes the types of CDG and their pathogenesis, clinical manifestations and liver involvement to provide a perspective for the diagnosis of CDG with liver manifestations.

Native Hakka speakers' identification of Cantonese tones: A comparative analysis / 中华物理医学与康复杂志

Objective To investigate the features of native Hakka speakers' ability to identify Cantonese tones.Methods Twenty native Cantonese speakers and 12 native Hakka speakers were given the Hongkong Cantonese tone identification test.Results Neither group was troubled by similar vowel and consonant sounds.The Cantonese group could identify all tones well except distinguishing tones 5 and 2 (89％) and tones 4 and 6 (89％).The Hakkas had the most difficulty distinguishing tones 3 and 6 (70.0％),but significant differences were observed between the two groups in the identification of tones 2 and 5 and 2 and 4.Conclusion A native Hakka speaker's ability to identify Cantonese tones may be closely related to the tone's pitch,tonal patterns as well as the Hakka tone system.

Clinical Evaluation of Puerarin Combining Routine Drugs in the Treatment of Unstable Angina Pectoris / 中国药房

OBJECTIVE:To provide bases for the evaluation of the therapeutic effect and pharmacoeconomics of puerarin in the treatment of unstable angina pectoris.METHODS:60patients with unstable angina pectoris were randomly divided into treatment group and control group:the control group was treated with routine drugs only while the treatment group was treated with puerarin and the routine drugs.The therapeutic effect,length of stay in hospital,total hospitalization fee and the adverse reaction were compared between two groups;and the cost-effectiveness analyses of the two groups were carried out as well.RESULTS:The respective total clinical effective rate of the treatment group and the control group were90.63%and67.86%(P