![]() ![]() ![]() Conclusions Heterozygous living donor liver transplantation is a safe and effective treatment of maple syrup urine disease, which reduces the possibility of sudden acute metabolic events, significantly improves the quality of life of the recipient and provides a novel idea for surgical treatment of maple syrup urine disease. As of the submission date, the recipient grew well in a stable condition and achieved high quality of life. The amino acid level was decreased to normal level immediately after operation, and BCAA was continually declined after normal diet for postoperative 1 month. Results The liver function of the recipient was recovered to normal range at postoperative 1 month, and basically stabilized at 3 years after surgery. A 12-day-old male infant was examined at the Cleveland Clinic on October 24, 1963, because of continuous convulsions for 6 days. Glucocorticoid administration was terminated at approximately 6 months after operation. The dose of tacrolimus was adjusted according to biochemical parameters and cytochrome P450(CYP)3A5 genotype of the recipient. Postoperatively, routine immunosuppression, anti-virus, anti-infection therapies, maintenance of fluid, electrolyte, and acid-base balance and other necessary nutritional support were given. ![]() Living donor liver transplantation from his biological father was performed. The child was diagnosed with maple syrup urine disease type b based on gene detection combined with BCAA assay. Serum branched chain amino acid (BCAA) assay detected that the level of leucine was 684 μmol/L and 559 μmol/L for the valine. No other growth abnormality or mental defects were observed. Maple syrup urine disease (MSUD) is an inborn error of branched chain amino acids (BCAAs) metabolism. The child presented with paroxysmal dysarthria and motor dysfunction of the lower limbs under fasting status for half a year, accompanied with obvious maple syrup urine odor and slow language development. (especially in case of higher intake levels), patients were studied with a 5. Methods A 3-year-old boy was admitted to the hospital on Jdue to maple syrup urine disease for half a year. Maple syrup urine disease (MSUD) is an autosomal recessive disorder caused. Objective To evaluate the clinical efficacy of heterozygous living donor liver transplantation for pediatric maple syrup urine disease. ![]()
0 Comments
Leave a Reply. |
AuthorWrite something about yourself. No need to be fancy, just an overview. ArchivesCategories |