Tue, Jan 27, 2015 page3

• Liver transplants tackle rare disorders: physicians

  • By Stacy Hsu / Staff reporter
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  The Taipei Veterans General Hospital yesterday touted the efficacy of liver transplants for people with metabolic liver diseases, which include an array of different, sometimes rare, conditions that often lead to lifelong medication and meticulous dietary management.

  “Metabolic liver diseases mainly involve patients whose liver is unable to sufficiently produce a certain type of enzyme to metabolize body waste because of genetic disorders, causing a number of nervous system and vascular conditions,” hospital director of surgery Lin Jen-kou (林楨國) told a news conference in Taipei.

  Lin said that while some of the diseases can be managed with medications and special diets, others cannot and can cause irreversible damage to the body, which prompted the hospital’s rare condition center a few years ago to explore abnother approach that could eradicate the disease and improve patients’ quality of life.

  Hospital Department of Pediatric Surgery physician Liu Chin-su (劉君恕) said the team then decided to opt for liver transplants and has since used the procedure to significantly improve the symptoms of 21 patients with metabolic liver diseases, with an overall success rate of 95 percent.

  “Among them were three people with ornithine transcarbamylase deficiency, a genetic disorder of the urea cycle that occurs in about one out of every 80,000 people; 10 cases with methylmalonic acidemia, an inherited illness that affects one out of every 50,000 people and in which the body is unable to properly process certain proteins; and one patient with the extremely rare disorder of homocystinuria,” Liu said.

  Lin said the latter case is a 28-year-old man surnamed Chen (陳) who is the first documented Asian case in medical history of homocystinuria detected through newborn screening.

  Homocystinuria is a rare inherited metabolic disorder caused by cystathionine beta synthase deficiency, which affects about one out of 500,000 men in Taiwan, Lin said.

  Lin added that affected people can experience abnormal accumulation of homocysteine, detachment of the crystalline lens inside the eye, a sunken chest and mental disability.

  “Chen had sought to control the disorder via medicines and stringent dietary management for the first 22 years of his life, but the approach not only greatly impeded his academic and job performances, but also failed to prevent him from developing dislocation of the crystalline lens, which meant he had to undergo two surgical procedures in 2000,” Lin said.

  After Chen and his family learned that liver transplantation could put an end to his misery, Lin said he readily agreed to the idea and received a donated liver in November 2009.

  Now, Chen has broken free of dependence on medicine and dietary control and is planning to open his own coffee shop, Lin said.
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