The number of cystic fibrosis (CF) patients undergoing lung transplant has risen over the past decade, because of a clear-cut survival benefit. However, patients with Burkholderia cepacia complex are often excluded from transplantation because of increased mortality. To determine the influence of B. cepacia complex genomovar type on transplant outcome, we undertook a retrospective study in 121 CF patients transplanted at UNC. Twenty-one and three patients, respectively, were infected pre- or postoperatively with B. cepacia complex. All posttransplant acquisitions were successfully treated. However, excess mortality occurred over the first 6 postoperative months in those infected preoperatively with B. cepacia complex compared with those not infected (33% versus 12%, p = 0.01). The 1-, 3-, and 5-yr survival were significantly lower in the B. cepacia complex cohort. Of the patients infected preoperatively, genomovar III patients were at the highest risk of B. cepacia complex-related mortality (5 of 12 versus 0 of 8, one isolate not typed; p = 0.035). Each of the B. cepacia complex-related deaths was caused by a unique genotype as determined by pulsed-field gel electrophoresis. All isolates were negative for the cable pilin gene. These results warrant a multicenter analysis of B. cepacia complex-infected patients with genomovar-typing to confirm that genomovar III patients are at highest risk for post-transplant complications.

Urachal anomalies present and progress differently in pediatric and adult populations. Adults are at high risk for urachal cancer but children usually incur lower morbidity. Urachal lesions noted early in childhood should be excised to prevent problems in adulthood.

Even after correcting for increases in total patient volume at Pediatric Health Information System hospitals, there has been a significant increase in the number of children diagnosed with and treated for urolithiasis at these hospitals in the last decade.

BACKGROUND:The objective of this study was to evaluate local control for patients with intermediate-risk rhabdomyosarcoma (RMS) treated on Children's Oncology Group (COG) protocol ARST0531. METHODS: This study analyzed 424 patients with intermediate-risk RMS. Patients were randomized to chemotherapy with either vincristine, dactinomycin, and cyclophosphamide (VAC) or VAC alternating with vincristine and irinotecan. With the goal of improving local control, radiation therapy (RT) was delivered early at week 4 and was concurrent with irinotecan in the experimental arm. Individualized local control plans for children 24 months old or younger were allowed. Local failure on ARST0531 was compared with local failure on the preceding COG intermediate-risk study, D9803. RESULTS: For patients with group I/II alveolar RMS (n = 55), the 5-year cumulative incidence of local failure was 13.4%; for group III alveolar RMS (n = 141), it was 20.2%; and for group III embryonal RMS (n = 228), it was 27.9% (P = .03). Among patients with group III disease, local failure did not differ by histology, site, nodal status, RT modality, or treatment arm. Local failure was worse for a tumor size >5 cm (32.3% vs 16.7%; P = .001). Among patients with group III embryonal RMS, local failure was higher on ARST0531 than D9803 (27.9% vs 19.4%; P = .03). After the exclusion of patients 24 months old or younger or patients who did not receive radiation, local failure remained significantly increased on ARST0531 (P = .02). After adjustments for clinical prognostic factors, event-free survival and overall survival were worse on ARST0531 (P = .004 and P = .05, respectively). CONCLUSIONS: Despite interventions designed to enhance local control, local control was inferior on ARST0531 in comparison with D9803. The reason for this is unclear, but it could be the reduced cyclophosphamide dose on ARST0531. Cancer 2019;125:3242-3248.

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OBJECTIVE Current clinical guidelines recommend that orchidopexy be performed by the age of 1 in patients with congenital undescended testis. We sought to examine trends in surgical timing and to determine what factors are associated with age at surgery. METHODS The Pediatric Health Information System (PHIS) is a national database of >40 freestanding children’s hospitals. We searched the PHIS to identify boys with cryptorchidism who underwent orchidopexy between 1999 and 2008. Patient age at orchidopexy was evaluated, and we used multivariate models to determine factors associated with timing of surgery. RESULTS We identified 28 204 children who underwent orchidopexy at PHIS hospitals. Of these, 14 916 (53%) were white, and 17 070 (61%) had public insurance. Only 5031 patients (18%) underwent orchidopexy by the age of 1 year; only 12 165 (43%) underwent orchidopexy by the age of 2 years. These figures remained stable over time (P = .32). After adjusting for patient clustering, race (P < .001) and insurance status (P < .001) remained associated with patient age at orchidopexy; however, the treating hospital (P < .001) was the most important factor in predicting the timing of the procedure. CONCLUSIONS Only 43% had surgery by 2 years of age, which suggests that either significant numbers of boys with congenital cryptorchidism do not undergo surgery in a timely fashion or late-onset testicular ectopy is more common than generally recognized. Factors associated with the timing of orchidopexy include patient race, insurance status, and the hospital in which surgery is performed.

The overall per-ureter Dx/HA success rate was 77% after 3 months, although success rates varied widely among studies. Increased VUR grade negatively affected success rates, whereas COI, patient age, and injected Dx/HA volume were not significantly associated with treatment outcome after adjustment for VUR grade. There is a significant need for improved reporting of VUR treatments, including comparative studies of Dx/HA and other VUR treatments.

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