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Purpose: Over the last 50 years, direct communication about cancer with adults has shifted from an approach of not telling to one of telling. Less is known about communication practices with children. The purpose of this study is to (1) describe patterns of communication at diagnosis between pediatric oncologists and children with cancer and (2) compare cultural differences in these practices in the US and Japan., Methods: This 2003 survey, developed in English and translated into Japanese was mailed to members of the American Society of Pediatric Hematology/Oncology and the two Japanese Societies of Pediatric Hematology and Oncology; there were 350 US and 362 Japanese respondents. Descriptive statistics and logistic regressions were performed., Results: US physicians had a consistent pattern of telling children (65% always told the child; less than 1% rarely or never told). Japanese physicians had greater variability in their patterns of telling (with only 9.5% always telling, 34.5% rarely or never telling). Direct communication with the child was influenced by personal attitudes, patient factors, and work culture in both countries. Many more variables emerged as influencing Japanese physicians' communication practices than for US physicians. US physicians were influenced by their own sense of responsibility for telling, while Japanese physicians were more influenced by personal attitudes, patient factors, and work culture., Conclusions: US and Japanese physicians differed when communicating directly with the child about his or her cancer. The impact of these practices on children and their parents should be explored and the parent and child's perspectives elicited. This information will help facilitate culturally sensitive patient and family centered communication., (Copyright 2006 John Wiley & Sons, Ltd.)

1. Accumulated evidence indicates that the adenylyl cyclase (AC)/cyclic adenosine monophosphate (cAMP)/protein kinase A (PKA)/cAMP-responsive element binding protein (CREB) signal transduction system may be linked to learning and memory function. 2. The effects of nefiracetam, which has been developed as a cognition enhancer, on spatial memory function and the AC/cAMP/PKA/CREB signal transduction system in rats with sustained cerebral ischaemia were examined. 3. Microsphere embolism (ME)-induced sustained cerebral ischaemia was produced by injection of 700 microspheres (48 micro m in diameter) into the right hemisphere of rats. Daily oral administration of nefiracetam (10 mg kg(-1) day(-1)) was started from 15 h after the operation. 4. The delayed treatment with nefiracetam attenuated the ME-induced prolongation of the escape latency in the water maze task that was examined on day 7 to 9 after ME, but it did not reduce the infarct size. 5. ME decreased Ca(2+)/calmodulin (CaM)-stimulated AC (AC-I) activity, cAMP content, cytosolic PKA Cbeta level, nuclear PKA Calpha and Cbeta levels, and reduced the phosphorylation and DNA-binding activity of CREB in the nucleus in the right parietal cortex and hippocampus on day 3 after ME. The ME-induced changes in these variables did not occur by the delayed treatment with nefiracetam. 6. These results suggest that nefiracetam preserved cognitive function, or prevented cognitive dysfunction, after sustained cerebral ischaemia and that the effect is, in part, attributable to the prevention of the ischaemia-induced impairment of the AC/cAMP/PKA/CREB signal transduction pathway.

Background: The p53 gene frequently is affected by point mutations, rearrangements, or deletions that contribute to the genesis or progression of a wide variety of human adult solid tumors; however, to the authors' knowledge, this gene alteration has not been analyzed in neuroblastoma., Methods: Genomic DNA samples from 20 children with neuroblastoma, including 16 patients with advanced disease, were screened for the presence of mutations in exons 5-9 of the p53 gene, where over 90% of mutations have been reported to be located in human cancer. The screening technique employed polymerase chain reaction/single-strand conformation polymorphism analysis followed by direct DNA sequencing., Results: Heterozygous mutations were detected in 2 of the 20 cases. A silent mutation (T to G transversion) at codon 172 and a missense mutation (G to T transversion) at codon 259 were found in patients with Stage II and Stage IV disease, respectively. Thus, p53 mutations were found to occur in neuroblastoma, but at a low frequency (2 of 20)., Conclusions: Our data suggest that in a minority of neuroblastomas, p53 gene mutations may play a contributing role in tumorigenesis, but other genes presumably play a major role in this tumor.

Ten leukemia cases with mixed phenotype were investigated in terms of clinical characteristics and cellular origin. Three patients were infants and six patients were older children. Six of them had a high leukocyte count and a mediastinal mass was found in three cases. All but one showed hepatosplenomegaly and/or lymphoadenopathy. In spite of intensive chemotherapy, most of them responded poorly. Cytochemical analysis of their leukemic cells revealed a low percentage of positivity for myeloperoxidase reactivity (less than 25%) in two cases and electron microscopic platelet peroxidase reactivity was found in one of three analyzed cases. Phenotypically, these cells all expressed CD7, and other T-lineage-associated, B-lineage-associated, and/or myeloid-associated antigens were also detected to some extent. In addition, three cases expressed CD41 and one case expressed CD56. The T-cell receptor (TCR) genes and immunoglobulin gene were in the germline configuration in seven cases. In three rearranged cases, two showed only the TCR-delta gene rearrangement, and one had both TCR-gamma and delta gene rearrangements. Cell culture studies with 12-0-tetradecanoyl-phorbol-13-acetate (TPA) revealed differentiation to the T-lineage in two cases and to a myeloid lineage in one case. Megakaryocytic differentiation was detected in two cases in culture without TPA. These results suggest that the cells from these cases arose from stem cells capable of both lymphoid and nonlymphoid differentiation. Although the cells were heterogeneous with regard to their potency of differentiation, they have similar clinical characteristics. Because of poor prognosis, it is important to identify this type of leukemia, and allogenic or autologous bone marrow transplantation should be considered.

We analyzed multidrug-resistance gene (mdr1 gene) expression in a patient with undifferentiated sarcoma of the liver using the cloned cDNA for the mdr1 gene. Tissue samples were available at the time of initial diagnosis and of two intracranial relapses after chemotherapy with a regimen including doxorubicin and teniposide. The level of mdr1 gene expression was increased sevenfold in the intracranial tumor at the time of first relapse and 11-fold at the second relapse. This case may be an example of acquired multidrug resistance associated with overexpression of the mdr1 gene.

A 2-year-old boy with B-lineage non-Hodgkin's lymphoma is described. He presented with growing skin tumors on his head, and biopsy specimens showed a malignant lymphoma of diffuse lymphoblastic type. Sixty-four percent of bone marrow cells were replaced with lymphoblasts, and they expressed B-lineage markers (CD19 and HLA/DR). Southern blot analysis demonstrated immunoglobulin heavy chain gene rearrangements (two rearranged and one germline) with the germline configuration of the T-cell receptor beta chain gene. Ten months later he relapsed with blasts of M5 morphologic type and a myeloid phenotype with the germline configuration of the immunoglobulin genes. During the next 2 months, myeloid blasts with immunoglobulin gene rearrangement which was identically rearranged with one of the two rearranged bands detected at diagnosis appeared. The most likely explanation for these findings is that initially the patient seemed to have at least two different clones of blasts, and clonal selections occurred during the treatments.

Nine children with mediastinal non-Hodgkin's lymphoma (NHL) were treated according to our new regimen which is characterized by intensified therapy with high-dose cytosine arabinoside (HDCA). After induction therapy with a combination of five drugs, such as vincristine, doxorubicin, cyclophosphamide, 1-asparaginase, and prednisolone, intermediate dosages of methotrexate (MTX) (1 g/m2) and HDCA (1.5 g/m2 x 12 doses) were administered. All but one patient (88.9%) achieved complete remission and then received this intensified therapy. With a median follow-up period of 25.5 months, five patients are still in complete remission, but three patients have relapsed. From the phenotypic point of view, these relapsed patients showed only very immature T-cell differentiation antigens such as CD2 and CD7 (or CD5). These results suggest that HDCA as intensified therapy for children with mediastinal NHL seems to be effective. However, for patients with an immature phenotype of T-lineage cells, more sophisticated regimens should be prepared.