PS2-58: A Survey of HMORN VDW Tumor Data Sources

Background There are currently fifteen HMORN sites that have a VDW tumor table. The VDW tumor specifications standardize this data. However, there is variation in each site's cancer registry, and hence the types of data that can be accessed. Methods During 2011 two metadata surveys have been sent out to the VDW implementation group. The participation rate has been over a dozen sites. The surveys ascertain each site's registry source type, (internal local registry, external central registry, such as SEER, NPCR, State, etc), the type of software used at the registry, whether all patients are captured by the registry, whether NAACCR is used to format and populate VDW tumor, the table update frequency, and other factors. Results All responding sites populate their data by means of a cancer registry All but two sites maintain their own facility registry. Just about half the respondents claimed to be a SEER site, Six sites use the NAACCR manual as a dictionary to aid the population of VDW tumor, four sites use the FORDS manual, Planned VDW Tumor file update schedules vary a great deal, from weekly to annually, with four sites each reporting annual and monthly update schedules, There is a diverse collection of software vendors used to collect cancer data at the registries, Geographic central registry coverage area may miss some patients treated for cancer at some sites. Conclusion It is instructive to understand the diversity in tumor data sources. Different central registry requirements dictate which fields are available to the site and may vary in content. While the data is standardized across sites by a common specification, there may still be nuances between sites; understanding the source data will help researchers and programmers navigate these nuances, and help shape requests for new fields or derivations.     

Life-threatening extrarenal lupus in children despite improvement in serologic findings

Systemic lupus erythematosus (SLE) is an autoimmune disorder with the potential for multiorgan involvement. Serologic tests are helpful in establishing the diagnosis of SLE and predicting disease flares. However, there are few data on the relationship between the onset of new organ involvement and lupus serologies, especially in children. This report details our experience in managing two children with lupus nephritis. Both developed life-threatening extrarenal complications (cerebritis and carditis) soon after receiving high-dose immunosuppressive therapy and despite normalizing serologies. This lack of concordance between serologies and the development of carditis and cerebritis needs to be recognized so that health care professionals treating children with SLE can promptly intensify immunosuppressive medications and avoid life-threatening delays from seeking alternative explanations for symptomatology.     

Children presenting with end-stage renal disease of unexplained etiology: implications for disease recurrence after transplantation

With longer graft and patient survival, recurrent disease is becoming recognized as an increasingly important contributor to long-term graft loss in renal transplant recipients. However, patients may present for the first time in end-stage renal disease (ESRD) leading to uncertainty as to their underlying diagnosis and the risk of recurrence. The purpose of this study was to describe the features of children who presented for the first time in ESRD and to determine the predictive value of investigations in differentiating diseases with and without a recurrence risk. From 7/99 to 11/04, 13 children presented to our center in ESRD. Their median age was 13.3 yr; 77% were male. The majority were hypertensive (77%) and oligoanuric (69%). All had proteinuria (median urine protein to creatinine ratio [Up/c] 7.0), and 92% had microhematuria. Only seven had small kidneys on ultrasound. All children underwent a serologic work-up and six (46%) were biopsied. Of the 13 children, seven had a glomerular disease; in five the diagnosis was made on biopsy, in one on serologic testing and one by family history. Of the remaining six children, three had non-glomerular diseases: obstructive uropathy in one and primary hyperoxaluria type 1 in two, and 3 had an unknown disease. When patients with glomerular diseases were compared with those with non-glomerular diseases, the two predictors for glomerular disease were a lower serum albumin (p = 0.004) and a higher serum bicarbonate level (p = 0.01). Comparing patients with and without a risk of recurrence, there were no differences between the two groups in any of their demographic, clinical, or biochemical parameters by analysis of variance (including serum albumin or proteinuria). In summary, the vast majority of children presenting in ESRD have hematuria and proteinuria, even with non-glomerular diseases. The significant overlap in clinical features between patients with and without a risk of recurrence emphasizes the need for all children presenting in ESRD to be evaluated extensively so that disease recurrence after transplantation can be anticipated or even prevented.     

Acute humoral rejection in pediatric renal transplant recipients receiving steroid minimization immunosuppression

SM protocols have increasingly gained acceptance owing to their favorable side effect profile with comparable cellular rejection rates. After encountering SM patients with AHR, we performed a case-control study to identify predictors associated with AHR in this cohort. Patients with (n = 4) and without (n = 19) biopsy proven AHR on a SM regimen were compared using the Student's t-tests. The median age at transplant was 13.8 yr. Compared to controls, the AHR cohort was older (15.9 vs. 12.1 yr, p = 0.01). Children with AHR had a lower mean tacrolimus trough level and were more likely to have a sub-therapeutic trough at six months (3.5 vs. 5.5 ng/mL, p = 0.05); mean MMF doses were lower at all times points except three months in the AHR group (not statistically significant). This occurred in spite of higher MPA trough levels at all study points in the AHR group (significant at 3 [p = 0.019] and 6 [p = 0.03] months). Children receiving a SM regimen have a lower safety net and may benefit from more intensive monitoring of tacrolimus exposure. MMF dose modifications based on MPA trough determinations should be resisted in the setting of SM.     

Extended experience with a steroid minimization immunosuppression protocol in pediatric renal transplant recipients

Lau KK, Berg GM, Schjoneman YG, Perez RV, Butani L. Extended experience with a steroid minimization immunosuppression protocol in pediatric renal transplant recipients.
Pediatr Transplantation 2010: 14:488–495. © 2009 John Wiley & Sons A/S. Abstract: Purpose: To determine the safety and efficacy of a novel steroid minimization protocol after renal transplantation at a single Northern California center. Introduction: We have previously reported our experience on the short‐term outcomes in eight children using our steroid minimization protocol. Herein, we present our ongoing experience in using this regimen in 20 children. Methods: Children receiving immunosuppression with a steroid minimization protocol at our center from 1/04 – 12/08 (Group 2) were retrospectively compared with 20 controls (Group 1). Results: At one‐month follow‐up, Group 2 was observed to have lower eGFR, hemoglobin, white cell count, and cholesterol. The incidence of adverse events during the first yr was comparable. Three patients in Group 1 displayed histological evidence of acute rejection, one patient in Group 2 developed humoral rejection; another patient in Group 2 had sub‐clinical rejection. Surgical complications were observed in 20% of patients in both groups. While 10% of patients in Group 1 developed diabetes mellitus, none was observed in Group 2. Thirty and 40% of patients in Groups 1 and 2, respectively, suffered from infectious complications during the first yr. Conclusions: Our novel steroid minimization immunosuppression is safe in children and associated with no increased risk of rejection and infection.