Seetharaman Jayendra, Sarma Moinak Sen
Department of Pediatric Gastroenterology, Sanjay Gandhi Post-graduate Institute of Medical Sciences, Lucknow 226014, Uttar Pradesh, India.
World J Hepatol. 2021 Nov 27;13(11):1552-1567. doi: 10.4254/wjh.v13.i11.1552.
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. D-Penicillamine 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.
螯合疗法是某些儿童肝脏疾病治疗的主要方法。与铜和铁相关的疾病需要进行螯合治疗。威尔逊病（WD）是儿童肝硬化的常见病因之一，主要用D-青霉胺和曲恩汀等铜螯合剂进行治疗。D-青霉胺虽然因其在肝脏型WD中疗效高而被广泛使用，但不良反应频发，常导致停药。曲恩汀作为替代药物，在肝脏型WD中具有相当的疗效，但不良反应发生率较低。四硫代钼酸铵在肝脏型WD中的作用目前仍处于实验阶段。印度儿童肝硬化与过量摄入铜有关，在当今时代很少见。D-青霉胺在该病早期有效，可使临床状况逆转。铁螯合剂常用于溶血性贫血引起的继发性肝脏血色素沉着症。在骨髓移植期间有严格的螯合方案。由于铁螯合疗法疗效不佳且有其他治疗方式可用，其在新生儿血色素沉着症中的作用目前并不流行。遗传性血色素沉着症在儿童中很少见，在这种情况下铁螯合剂的使用有限。
