Two children were weaned from long-term tube feeding after liver transplant because of Alagille syndrome. The children were successfully weaned, one in seven days and the other in 13 days, using our standard and highly specialized intensive treatment protocol. Normal feeding behavior and stabilization of body weight were established. Children fed by long-term enteral tubes can be weaned from enteral feeding even after a long period of treatment. The return to age-appropriate self-feeding should be introduced as early as possible. Our weaning program time is brief and effective and can be recommended generally to improve quality of life and withhold unintended side-effects of enteral nutrition.
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Division of Gastroenterology, Hepatology and Nutrition, The Hospital for Sick Children and the University of Toronto, Toronto, Ontario, Canada.
Background: Alagille syndrome (ALGS) is a multisystem cholestatic disorder. Maralixibat is approved for the treatment of cholestatic pruritus in ALGS with limited data in adults.
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Background: Alagille syndrome (ALGS) is a rare disease. The variable clinical manifestations make the diagnosis of ALGS difficult. This study aimed to provide a basis for the early diagnosis of ALGS patients whose clinical identification is difficult and to enrich the spectrum of genetic variants implicated in Chinese children with ALGS.
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Guy's & St Thomas' NHS Foundation Trust, London, UK.
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