Ehrich J H, Brodehl J, Byrd D I, Hossfeld S, Hoyer P F, Leipert K P, Offner G, Wolff G
Kinderklinik, Abteilung Pädiatrische Nieren- und Stoffwechselkrankheiten, Medizinische Hochschule, Hannover, Federal Republic of Germany.
Pediatr Nephrol. 1991 Nov;5(6):708-14. doi: 10.1007/BF00857880.
In 1989, 22 children (11 boys, 11 girls aged 8-23 years) with nephropathic cystinosis, who had received a total of 28 renal allografts over the previous 14 years, were reviewed. Nineteen were alive, of whom 17 had functioning grafts 5 months to 13 years after transplantation. The mean serum creatinine level in these 17 was 135 mumol/l. Patient and graft survival did not differ from non-cystinotic children. Persistent hypothyroidism was found in 3 patients, transient diabetes mellitus in 1, severely disturbed vision in 1 and brain atrophy in 11. Arterial hypertension was present in 16 patients. Growth retardation was universal, although in 4 patients on cyclosporin A post-transplant catch-up growth occurred. Five patients over 15 years completed puberty. Readjustment in terms of school performance was good but was less good for psychosocial development. None of the patients had ever been treated with cystine-depleting agents; the data will therefore provide a historical control group with which to compare the results from a group treated with these agents.
1989年，对22名患有肾病型胱氨酸病的儿童（11名男孩，11名女孩，年龄8 - 23岁）进行了回顾性研究，这些儿童在过去14年中共接受了28次肾移植。19名儿童存活，其中17名在移植后5个月至13年拥有功能正常的移植肾。这17名儿童的平均血清肌酐水平为135μmol/l。患者和移植肾的存活率与非胱氨酸病儿童无异。3名患者出现持续性甲状腺功能减退，1名出现短暂性糖尿病，1名视力严重受损，11名出现脑萎缩。16名患者患有动脉高血压。生长发育迟缓普遍存在，不过4名移植后使用环孢素A的患者出现了追赶生长。5名15岁以上的患者完成了青春期发育。学业成绩方面的调整良好，但心理社会发展方面的调整则不太理想。这些患者均未接受过耗竭胱氨酸药物的治疗；因此，这些数据将提供一个历史对照组，以便与接受这些药物治疗的一组患者的结果进行比较。
