肝移植后1b型糖原贮积病患者中性粒细胞功能改善。
Improved neutrophil function in a glycogen storage disease type 1b patient after liver transplantation.
作者信息
Adachi Masanori, Shinkai Masato, Ohhama Youkatsu, Tachibana Katsuhiko, Kuratsuji Tadatoshi, Saji Hiroh, Maruya Etsuko
机构信息
Department of Endocrinology and Metabolism, Kanagawa Children's Medical Centre, Mutsukawa 2-138-4, Minami-ku, 232-8555 Yokohama, Japan.
出版信息
Eur J Pediatr. 2004 Apr;163(4-5):202-6. doi: 10.1007/s00431-004-1405-1. Epub 2004 Feb 11.
UNLABELLED
Patients with glycogen storage disease type 1b (GSD1b) not only show hepatomegaly, hypoglycaemia and lactic acidosis, but also neutropenia and neutrophil dysfunction. Here, we report improvement of neutropenia and neutrophil function in a 22-year-old male GSD1b patient who had undergone living-related partial liver transplantation (LT) at 18 years of age. After LT, the patient's infectious episodes decreased, gastrointestinal symptoms ameliorated, neutrophil counts increased, and neutrophil function tests normalised.
CONCLUSION
Although it is not known whether this improvement was causally related to liver transplantation, this may be the first recorded case of restoration of neutrophil dysfunction in a glycogen storage disease type 1b patient.
未标注
1b型糖原贮积病（GSD1b）患者不仅表现为肝肿大、低血糖和乳酸性酸中毒，还伴有中性粒细胞减少和中性粒细胞功能障碍。在此，我们报告了一名22岁男性GSD1b患者中性粒细胞减少和中性粒细胞功能的改善情况，该患者在18岁时接受了活体亲属部分肝移植（LT）。肝移植后，患者的感染发作减少，胃肠道症状改善，中性粒细胞计数增加，中性粒细胞功能测试恢复正常。
结论
虽然尚不清楚这种改善是否与肝移植存在因果关系，但这可能是首例记录的1b型糖原贮积病患者中性粒细胞功能障碍恢复的病例。
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