Chelation therapy in liver diseases of childhood: Current status and response  

作　　者：Jayendra Seetharaman Moinak Sen Sarma 

机构地区：[1]Department of Pediatric Gastroenterology,Sanjay Gandhi Post-graduate Institute of Medical Sciences,Lucknow 226014,Uttar Pradesh,India

出　　处：《World Journal of Hepatology》2021年第11期1552-1567,共16页世界肝病学杂志（英文版）（电子版）

摘　　要：Chelation is the mainstay of therapy in certain pediatric liver diseases.Copper and iron related disorders require chelation.Wilson’s disease(WD),one of the common causes of cirrhosis in children is treated primarily with copper chelating agents like D-penicillamine and trientine.D-Penicillamine though widely used due its high efficacy in hepatic WD is fraught with frequent adverse effects resulting discontinuation.Trientine,an alternative drug has comparable efficacy in hepatic WD but has lower frequency of adverse effects.The role of ammonium tetra-thiomolybdate is presently experimental in hepatic WD.Indian childhood cirrhosis is related to excessive copper ingestion,rarely seen in present era.DPenicillamine is effective in the early part of this disease with reversal of clinical status.Iron chelators are commonly used in secondary hemochromatosis of liver in hemolytic anemias.There are strict chelation protocols during bone marrow transplant.The role of iron chelation in neonatal hemochromatosis is presently not in vogue due to its poor efficacy and availability of other modalities of therapy.Hereditary hemochromatosis is rare in children and the use of iron chelators in this condition is limited.

关 键 词：Wilson’s disease D-PENICILLAMINE Trientine Indian childhood cirrhosis DEFEROXAMINE DEFERASIROX HEMOCHROMATOSIS 

分 类 号：R725.7[医药卫生—儿科]