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Clinical and pathologic studies on adults with uremic neuropathy are numerous, but less is known about this disorder in children and adolescents. We report the clinical, electrophysiologic, and pathologic findings in an adolescent female with uremic neuropathy. Electrophysiologic findings were consistent with a primarily axonal sensorimotor polyneuropathy. Sural nerve biopsy revealed areas of focal depletion in myelin sheaths and loss of axons. Axonal degeneration with secondary myelin changes appears to be the characteristic pathology in this case, one of the youngest to our knowledge for which nerve biopsy data are available. Our patient experienced dramatic recovery after renal transplantation, similar to the reports of older patients.

We report a case of a pediatric en bloc liver-double kidney transplant in a patient with IVC thrombosis below the renal veins. The patient is an 11-month-old girl diagnosed with congenital nephrotic syndrome at two months of age. Multifocal liver masses were identified on routine ultrasound at eight months of age. Alpha fetoprotein level was 55 319. Biopsy confirmed hepatoblastoma. CT scan confirmed multiple lesions in both lobes, which would require liver transplantation for resection. She was also found to have thrombosis of her infrarenal IVC secondary to multiple central lines. She was listed for combined liver-kidney transplant and began chemotherapy. After four cycles of chemotherapy, she underwent bilateral nephrectomies followed by a combined en bloc liver-double kidney transplant from a size matched donor. In order to provide adequate venous outflow from the kidneys in the absence of a recipient infrarenal IVC, the donor liver and kidneys were procured en bloc with a common arterial inflow via the infrarenal aorta and common outflow via the suprahepatic IVC. Kidney transplantation in the absence of adequate recipient venous drainage may require unusual vascular reconstruction techniques. This case demonstrates a novel approach in patients who may require combined liver-kidney transplantation.