Fivush B, Green O C, Porter C C, Balfe J W, O'Regan S, Gahl W A
Am J Dis Child. 1987 Oct;141(10):1087-9. doi: 10.1001/archpedi.1987.04460100065027.
Nephropathic cystinosis causes renal death by approximately age 10 years. With increased life span due to kidney transplantation, ten to 25 years of cystine accumulation has resulted in pancreatic complications in individuals with cystinosis. We noted severe hyperglycemia in five posttransplant patients, three of whom remained insulin-dependent diabetics several years after transplant. The clinical findings were not consistent with steroid-dependent or insulin-resistant diabetes. Pancreatic cystine deposition was detected histologically and biochemically on post-mortem examination of two other patients. We conclude that hyperglycemia may be anticipated in the immediate posttransplant period in cystinotic patients and that some patients will require insulin therapy years later. The use of cystine-depleting agents should be considered in posttransplant cystinosis as an attempt to prevent potential damage to the pancreas and other organs from cystine deposition.
肾性胱氨酸病通常会在10岁左右导致肾衰竭死亡。随着肾移植技术的发展延长了患者寿命，患胱氨酸病的个体因10至25年的胱氨酸积累而出现胰腺并发症。我们注意到5例肾移植术后患者出现严重高血糖，其中3例在移植后数年仍依赖胰岛素治疗。临床症状与类固醇依赖型或胰岛素抵抗型糖尿病不符。对另外2例患者进行尸检时，通过组织学和生化方法检测到胰腺中有胱氨酸沉积。我们得出结论，胱氨酸病患者在肾移植术后短期内可能会出现高血糖，部分患者在数年后需要胰岛素治疗。对于肾移植术后的胱氨酸病患者，应考虑使用降低胱氨酸的药物，以防止胱氨酸沉积对胰腺和其他器官造成潜在损害。
