Multifocal Epstein-Barr Virus-Negative Posttransplantation Lymphoproliferative Disorder Treated With Reduction of Immunosuppression.

Posttransplantation lymphoproliferative disorder (PTLD) is associated with significant mortality in kidney transplant recipients. PTLD cases associated with poor prognostic factors that are refractory to reduction of immunosuppression generally require chemotherapy and immunotherapy. We present a patient with PTLD who achieved complete remission after reduction of immunosuppression alone despite having a poor prognosis. A boy with a mutation in the WT1 gene developed bilateral Wilms tumor at 15 months and received a kidney transplant at the age of 4 years. At 13 years of age, the patient's condition was managed with methylprednisolone, tacrolimus, and mycophenolate mofetil. He developed Epstein-Barr virus-negative monomorphic PTLD with numerous nodular lesions in the liver, vertebral bodies, and gastric wall. To reduce immunosuppression, we discontinued mycophenolate mofetil treatment, decreased tacrolimus dosage to 1mg/d, and increased methylprednisolone dosage to 2mg/d. The PTLD lesions drastically diminished in size within several days and disappeared 144 days after reduction of immunosuppression, although the patient had several factors indicating a poor prognosis. As of 13 months after reduction of immunosuppression for PTLD, the transplanted kidney was still functional. We conclude that even when patients with PTLD have a poor prognosis, reduction of immunosuppression alone may result in complete remission when the early response is excellent.

Estimation of 24-hour urine protein quantity by the morning-urine protein/creatinine ratio.

BACKGROUND: Many reports have described a correlation between the morning-urine protein /creatinine ratio (morning urine P/Cr) and the quantity of 24-h urine protein (Up), as well as regression formulas for Up with morning-urine P/Cr. However, there is no universal regression formula that can be used at all facilities. It is still controversial whether a qualitative calculation is required at outpatient clinics. To develop a practical and universal method, we used receiver operating characteristic (ROC) analysis to estimate Up from morning-urine P/Cr. METHODS: The subjects were 34 children (309 specimens) with kidney disease who had been admitted to Miyazaki Prefectural Hospital. We examined the correlations of P/Cr with Up and Up/body surface area (Up/BSA) using morning and daytime urine. We determined the cutoff values to estimate Up/BSA from morning-urine P/Cr with an ROC analysis. Next, we applied the values to specimens obtained from other facilities to show the universality of this approach. RESULTS: Up/BSA for samples in one hospital was significantly correlated with morning-urine P/C. When the morning-urine P/Cr ratio is >or=1.0 or >or=2.0, the Up/BSA ratio will exceed 0.5 or 1.0 (g/m(2) per day), respectively, and the efficiency was sufficiently high (efficiency for Up/BSA of >or=0.5: 88.0%, efficiency for Up/BSA of >or=1.0: 90.9%). When we analyzed samples from two other facilities with these cutoff values, both the sensitivity and specificity were greater than 80% for both facilities. CONCLUSIONS: The use of cutoff values of 1.0 and 2.0 for morning-urine P/Cr determined by ROC analysis could be a universal method for quantitatively estimating Up/BSA >or=0.5 and 1.0, respectively.

Fetal ultrasonography to prevent irreversible neurological sequelae of neonatal neuroblastoma.

An intra-abdominal mass was observed by fetal ultrasonography at 32 weeks of gestation. The baby was diagnosed as having neuroblastoma at the time of delivery at 39 weeks and its lower extremities were completely paralyzed. The chemotherapy after birth was quite effective to reduce the mass volume but neurological sequelae failed to improve. By carefully monitoring the movement of extremities, it may have been possible to prevent irreversible by inducing delivery before that state was reached.