The economic burden of caregiving on families of children and adolescents with cancer: a population-based assessment.

BACKGROUND: Childhood cancer represents a relevant economic burden on families. The preferred tool to investigate family expenditure is the retrospective questionnaire, which is subject to recall errors and selection bias. Therefore, in the present study the economic burden of caregiving on families of children and adolescents (0-19 years of age) with cancer was analysed using administrative data as an alternative to retrospective questionnaires. PROCEDURE: Incident cases of cancer diagnosed in children and adolescents in 2000-2005 (N = 917) were identified from the Piedmont Childhood Cancer Registry and linked to available administrative databases to identify episodes of care during the 3 years after diagnosis (N = 13,433). The opportunity cost of informal caregiving was estimated as the value of the time spent by one of the parents, and was assumed to be equal to the number of days during which the child received inpatient care, day-care or outpatient radiotherapy. Factors affecting the level of economic burden of caregiving on families were analysed in a multivariable model. RESULTS: The economic burden of caregiving increased when care was supplied at the Regional Referral Centre, or when treatment complexity was high. Families with younger children had a higher level of economic burden of caregiving. Leukaemia required a higher family commitment than any other cancer considered. CONCLUSIONS: Estimates of the economic burden of caregiving on families of children and adolescents with cancer derived from administrative data should be considered a minimum burden. The estimated effect of the covariates is informative for healthcare decision-makers in planning support programmes.

A complex karyotype including a t(2;11) in a paediatric ependymoma: case report and review of the literature.

Ependymomas are glial tumours representing approximately 5-10% of all intracranial tumours and are the third most common primary brain tumour in childhood. Only a few karyotypic studies on paediatric ependymomas have been published and no specific chromosomal aberration has been specifically related to this type of cancer. We performed cytogenetic analysis of an ependymoma in an 11-year-old boy. Our patient showed a complex karyotype, characterized by a near-tetraploidy and a sole structural unbalanced aberration: der(2)t(2;11)(q11.2;q13.1), which has not been described before. We here discuss such cytogenetic findings, comparing our data with those reported in the literature.

Inhibition of heat shock proteins (HSP) expression by quercetin and differential doxorubicin sensitization in neuroblastoma and Ewing's sarcoma cell lines.

Neuroblastoma (NB) and Ewing's sarcoma (ES) represent the most common extracranial solid tumors of childhood. Heat shock proteins (HSP) are elevated in cancer cells and their over-expression was correlated to drug-resistance. In this work we identified the HSP by a sensitive proteomic analysis of NB and ES cell lines, then, we studied the HSP response to doxorubicin. Some identified HSP were constitutively more expressed in NB than in ES cells. Doxorubicin-stimulated HSP response only in NB cells. Quercetin was found to inhibit HSP expression depleting heat shock factor 1 (HSF1) cellular stores. Quercetin caused a higher anti-proliferative effect in NB (IC(50): 6.9 +/- 5.8 mumol/L) than in ES cells (IC(50): 85.5 +/- 53.1 mumol/L). Moreover, quercetin caused a very pronounced doxorubicin sensitizing effect in NB cells (241 fold IC(50) decrease) and a moderate effect in ES cells. HSP involvement in NB cells sensitization was confirmed by the silencing of HSF1. Quercetin treatment and HSF1 silencing increased the pro-apoptotic effect of doxorubicin. In conclusion, the higher HSP levels, observed in NB cells, did not confer increased resistance to doxorubicin; on the contrary, HSP inhibition by quercetin or gene silencing caused higher sensitization to doxorubicin. These results may have a potential application in the treatment of NB.

Flow cytometric evaluation of circulating CD34+ cell counts and apoptotic rate in children with acquired aplastic anemia and myelodysplasia.

OBJECTIVE: Identification of a rapid and noninvasive test for the follow-up of aplastic anemia (AA) patients during immunosuppressive therapy (IST) to evaluate its functional effect on hematopoietic progenitors (HPC) and for early detection of progression to myelodysplasia or relapse. MATERIALS AND METHODS: Absolute count and apoptotic rate (AR) of peripheral blood (PB) CD34+ cells were evaluated by three-color flow cytometry for CD45, CD34, and annexin V in cord blood (CB), normal children, and adults, as well as in pediatric patients with AA at diagnosis and during IST, Fanconi anemia (FA), chronic immune cytopenia, and refractory anemia with excess blasts (RAEB). RESULTS: In normal subjects, the AR of PB CD34+ cells showed a progressive increase (p < 0.05), while their counts decreased (p < 0.05) from birth to adulthood. In very severe AA (vSAA) and severe AA (SAA) at diagnosis, the AR was 91.6% +/- 2.8%, higher than controls (p < 0.05), and PB CD34+ cell count was 2.6 +/- 2.4/microL. In FA patients, the PB CD34+ AR was again significantly increased (54.2% +/- 13.7%) with an absolute count of 3.7 +/- 1.2/microL. Conversely, in RAEB the AR was 11.7% +/- 3.5% and the absolute count 85.1 +/- 48.2/microL (p < 0.05). Chronic immune cytopenias did not significantly differ from controls. CONCLUSIONS: Flow cytometry evaluation of PB CD34+ AR and counts is a noninvasive and feasible first-step method for the differentiation of AA and myelodysplasia (MDS), and it might be useful for monitoring AA during IST to secure the early detection of relapse or transformation to MDS.