Matsukura Hiro, Ibuki Keijiro, Nomura Keiko, Higashiyama Hiroyuki, Takasaki Asami, Miyawaki Toshio, Aikawa Atsushi, Kanegane Hirokazu
Department of Pediatrics, Saiseikai Toyama Hospital, 33-1 Kusunoki, Toyama, 931-8533, Japan.
Department of Pediatrics, Faulty of Medicine, University of Toyama, Toyama, Japan.
CEN Case Rep. 2012 Nov;1(2):86-89. doi: 10.1007/s13730-012-0019-0. Epub 2012 Jun 8.
Wilms' tumor (WT), also called nephroblastoma, is an embryonic neoplasm of the developing kidney. A previously healthy Japanese female infant had WT in a single kidney without associated congenital malformations. Preoperative chemotherapy was started for the preservation of renal tissue and function. Tumor lysis syndrome, disseminated intravascular coagulopathy, and acute renal failure were accompanying. The infant needed surgical intervention and permanent replacement therapy. At the start of emergency hemodialysis, the infant had posterior reversible leukoencephalopathy syndrome because of severe hypertension. During ongoing peritoneal dialysis, the infant suffered from anemia, dietary and fluid restriction, and restriction of time and mobility. Despite alfacalcidol and calcium supplementation, the infant had secondary hyperparathyroidism and remarkably short stature. After waiting for the completion of chemotherapy, renal transplantation from the mother was completed. Successful kidney transplantation promptly corrected preexisting metabolic abnormalities causing secondary hyperparathyroidism. Subsequently, the infant often complained of headache. Computed tomographic scanning revealed calcification in the cerebellum. Refractory secondary hyperparathyroidism was inferred as the cause. A well-functioning graft provided the infant with a greater sense of well-being and enabled her to enjoy a lifestyle free of dialysis, although the infant must continue taking transplant medications and has retained unresolved issues of short stature and ectopic intracranial calcification.
肾母细胞瘤（WT），也称为肾胚胎瘤，是发育中的肾脏的一种胚胎性肿瘤。一名先前健康的日本女婴单肾患有肾母细胞瘤，无相关先天性畸形。为保留肾组织和功能开始术前化疗。同时伴有肿瘤溶解综合征、弥散性血管内凝血和急性肾衰竭。该婴儿需要手术干预和长期替代治疗。在紧急血液透析开始时，由于严重高血压，婴儿出现了后部可逆性白质脑病综合征。在进行腹膜透析期间，婴儿患有贫血、饮食和液体限制以及时间和活动受限。尽管补充了阿法骨化醇和钙，但婴儿仍患有继发性甲状旁腺功能亢进且身材明显矮小。在等待化疗完成后，完成了来自母亲的肾移植。成功的肾移植迅速纠正了导致继发性甲状旁腺功能亢进的先前存在的代谢异常。随后，婴儿经常抱怨头痛。计算机断层扫描显示小脑有钙化。推测难治性继发性甲状旁腺功能亢进为病因。一个功能良好的移植物让婴儿感觉更健康，使她能够享受无需透析的生活方式，尽管婴儿必须继续服用移植药物，并且仍存在身材矮小和颅内异位钙化等未解决的问题。
