Adolescents with Chronic Kidney Disease: A Model for Transition to Adult Care.

Successful health care transition from pediatric to adult care emphasizes the need for a collaborative effort to employ systematic processes. The development of a structured health care transition program for adolescents with chronic kidney disease (CKD) was the goal of this quality improvement program. The non-experimental design included development of an individualized health care transition treatment plan and TRxANSITION Scale™ application of the transition plan with patient evaluation; success designated full transition to adult care. Of the 19 patients enrolled, 74% had CKD, and 26% were renal transplant recipients. TRxANSITION Scale variables with the highest Pearson Correlation coefficients for total scores and strong positive relationships were self-management, insurance, and school. Four participants successfully transitioned. Purposeful, interprofessional health care transition preparation provides youth with CKD ongoing access to subspecialists, promotes self-care, and allows continued support of long-term health care planning. This evidence-based project adds to the body of knowledge for a topic that has proven to be challenging and often difficult for patients, families, and providers.

Physical confinement during cancer cell migration triggers therapeutic resistance and cancer stem cell-like behavior.

Metastasized cancer cells have an increased resistance to therapies leading to a drastic decrease in patient survival rates. However, our understanding of the cause for this enhanced resistance is lacking. In this study, we report that physically tight confinement during cancer cell migration triggers therapeutic resistance and induces cancer stem cell-like behavior including up-regulation in efflux proteins and in cancer stem cell related markers. Moreover, the re-localization of Yes-associated protein (YAP) to the cell nucleus indicated an elevated level of cytoskeletal tension. The increased cytoskeletal tension suggested that mechanical interactions between cancer cells and tight surroundings during metastasis is one of the factors that contributes to therapeutic resistance and acquisition of cancer stem cell (CSC) like features. With this system and supporting data, we are able to study cells with therapeutic resistance and CSC-like properties for the future purpose of developing new strategies for the treatment of metastatic cancer.

Post-hematopoietic stem cell transplant immunization practices in the Pediatric Blood and Marrow Transplant Consortium.

BACKGROUND: A survey of National Marrow Donor Program transplant centers in 1995 demonstrated a wide range of immunization practices in post-hematopoietic stem cell transplant (HSCT) recipients, which led to the 2000 Centers for Disease Control and Prevention (CDC) recommendations for vaccination after HSCT. We surveyed the principal investigators of the Pediatric Blood and Marrow Transplant Consortium (PBMTC) to identify immunization practice patterns after HSCT and assess compliance with the 2000 CDC guidelines. PROCEDURE: Approval was obtained from the Medical University of South Carolina Institutional Review Board. A 33 question survey using surveymonkey.com was distributed by email to principal investigators in the PBMTC. RESULTS: Forty-one (40%) of the 102 pediatric HSCT centers participating in the PBMTC responded. Thirty of the responding centers completed the entire survey. For individual vaccines, compliance with the CDC guidelines ranged from 22% to 93%. Less than 20% of the centers reported schedules consistent with the 2000 CDC recommendations for both allogeneic and autologous HSCT recipients. CONCLUSION: Despite the 2000 CDC guidelines, wide variation in post-HSCT immunization practices still exists. Updated guidelines have been needed, particularly to address the use of the pneumococcal conjugate vaccine. In conjunction with multiple other groups, the CDC recently released new immunization guidelines in October 2009. Additional data are still needed to adequately address the utility of incorporating immunologic parameters with the timing of vaccination after HSCT.

Clear cell atypical fibroxanthoma:a clinicopathologic study.

INTRODUCTION: The atypical fibroxanthoma (AFX) is considered by most authorities to represent a superficial or minimally invasive variant of malignant fibrous histiocytoma that most often presents as a solitary nodule on the sun-exposed skin of the elderly. Among the rarest variants is the clear cell AFX, a lesion which raises consideration to a differential diagnosis encompassing a variety of neoplastic and non-neoplastic clear cell proliferations. METHODS: We describe three cases of a distinctive cutaneous neoplasm arising in the sun-exposed skin of elderly patients. In all cases, formalin-fixed, paraffin-embedded tissue was available for analysis. The histology in concert with the immunophenotype was held to be diagnostic of the clear cell variant of AFX. RESULTS: All tumors comprised sheets of large cells with foamy cytoplasms and hyperchromatic, polyploid nuclei manifesting frequent and atypical mitoses. The critical cells in our cases expressed CD68 but none of CD3, CD20, CD34, S-100 protein, muscle-specific actin, factor XIIIa, Melan-A, carcinoembryonic antigen, or cytokeratin. CONCLUSION: Although typical examples of AFX provoke diagnostic consideration of spindle cell cancers of the skin (most often spindle cell melanoma, spindle cell squamous cell carcinoma, and leiomyosarcoma), the clear cell variant raises other differential diagnostic considerations instead. These include balloon cell melanoma, sebaceous carcinoma, pleomorphic liposarcoma, chordoma, parachordoma, tricholemmal carcinoma and clear cell squamous cell carcinoma. A diagnosis of AFX is one of exclusion; one must employ immunohistochemical markers to rule out the aforementioned differential diagnostic considerations. By reporting the fifth, sixth and seventh cases of clear cell AFX, we hope to alert dermatopathologists to this distinctive and unusual neoplasm, recognition of which is essential to avoid under- or over-diagnosis and inappropriate therapy.