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Goals Of Work: To describe the course of hepatitis C in a cohort of 105 survivors after childhood cancer.Patients and Methods: Data on chemo/radiotherapy, clinical status, serial alanine aminotransferase (ALT) evaluation, and virological parameters after the end of treatment were collected for each patient. Liver biopsies, when performed, were centrally evaluated by a pathologist.Main Results: All patients were alive at the end of follow-up and did not show hepatic insufficiency. ALT evaluation along the entire follow-up showed a moderate (87%) or a remarkable (13%) cytolytic pattern. Young age at diagnosis, hematopoietic stem cell transplantation, and duration of infection significantly correlate with a worse hepatic activity. Type of tumor and chemo and/or radiotherapy regimens did not influence the pattern of hepatic cytolysis. Liver biopsy, centrally reviewed in 30% of the cohort, showed one case of cirrhosis and mild fibrosis in 71% of the group. Higher degrees of fibrosis did not seem to be related to any exposition to chemo/radiotherapy but correlated significantly with the more remarkable cytolytic course.Conclusions: The outcome of hepatitis C in our patients is comparable to the one described in European cohorts of adult cancer survivors and perinatally infected subjects. Nevertheless, progression to high degrees of hepatic damage has to be monitored by a careful follow-up. [ABSTRACT FROM AUTHOR]

The objective of this study was to assess the efficacy of an injection of 100 μg/kg of pegfilgrastim in haematopoietic recovery and mobilization in children following 32 courses of chemotherapy. End points were duration of neutropaenia, myeloid recovery and PBMC collection. Neutropaenia lasted a mean of 4.7 days (±2.13 days). Myeloid recovery occurred at a median of 10 days (inter quartile range (IQR) 8–11). Febrile neutropaenia complicated 13 courses (40.6%). Mobilization was observed in 20 out of 26 assessable courses (76.9%). The rise in CD34+ cells occurred at a median of 6 days (IQR 4–7) after PEG and remained >20 per μl for 6 days (IQR 4–8), with a median value of 80 per μl (IQR 48–170.5). The median CD34+ cell peak was 165 per μl (IQR 82.5–331), 9 days (range 6–14) after PEG. PBMC were collected on average at day +5 (+4 to +9) after PEG. In 93.3% of collections, at least 3 × 106 per kg CD34+ cells were collected through a single apheresis. Myeloid recovery occurred in all cases within 15 days, without concomitant thrombocytopaenia. The incidence of primary febrile episodes is in line with data in the literature and with our own historical experience. A long-lasting period of circulating CD34+ cells allowed for more accurate scheduling of apheresis.Bone Marrow Transplantation (2008) 42, 507–513; doi:10.1038/bmt.2008.206; published online 21 July 2008 [ABSTRACT FROM AUTHOR]

Invasive mycoses represent a rare but severe complication following hemopoietic SCT (HSCT) in children. Their incidence is related to the type of donor, being higher after allogeneic transplant, especially from alternative donors. Moreover, the incidence of invasive mycoses varies in the different post transplant phases. Neutropenia, lymphopenia, GvHD, high-dose steroids or other immunosuppressive drugs represent well-known risk factors. The clinical features of invasive mycoses after HSCT in children are similar to those observed in adults, and the diagnostic tools, including Aspergillus galactomannan antigen detection, are feasible also in pediatrics. Mortality due to invasive mycoses after HSCT in children is high.Bone Marrow Transplantation (2008) 41, S107–S111; doi:10.1038/bmt.2008.67 [ABSTRACT FROM AUTHOR]

We performed a retrospective single center study to define the epidemiology of bacteremias or invasive mycoses in pediatric allogeneic hematopoietic SCT (HSCT) from matched related donors (MRD) or alternative donors (AD). During 119 213 days of follow-up, 156 infections were observed: 130 bacteremias (27 in MRD-HSCT and 103 in AD-HSCT recipients) and 26 invasive mycoses (8 in MRD-HSCT and 18 in AD-HSCT recipients). Overall, the risk of bacteremia was fivefold that of invasive mycosis (P<0.001). AD-HSCT recipients had a higher percentage of infections (89 vs 27%; P<0.001), a higher rate/100 days of immunosuppression (infection rate (IR): 0.21 vs 0.06; P<0.001) and a higher proportion of repeated infections (44 vs 9%; P=0.001). In AD-HSCT, the relative risk of bacteremia was 2.87 in the pre-engraftment period, 5.84 in the early post-engraftment period and 6.46 in the late post-engraftment period (P<0.001) compared to MRD-HSCT. Only after 1 year did the epidemiology become similar. The epidemiology of invasive mycoses did not differ significantly between the two types of transplant.Bone Marrow Transplantation (2008) 41, 339–347; doi:10.1038/sj.bmt.1705921; published online 19 November 2007 [ABSTRACT FROM AUTHOR]

Aim: To evaluate clinical and genetic factors, besides pancreatic insufficiency, associated with increased risk of cystic fibrosis-related diabetes. Methods: Case-control (1:1) study on 138 cystic fibrosis patients. Data were collected on gender, age at diagnosis, reason for cystic fibrosis diagnosis, family history of type 1 or 2 diabetes mellitus, pre-existing severe liver disease, and class of cystic fibrosis transmembrane regulation mutation. Moreover, information was obtained on lung involvement and degree of exocrine pancreatic insufficiency evaluated 1 year before the diagnosis of cystic fibrosis-related diabetes in patients and age-matched controls. Results: Compared to controls, patients with cystic fibrosis-related diabetes had a higher probability of having already been diagnosed with liver disease (16.7% versus 1.7%, OR = 11.6, 95% CI 1.43–93.0). Moreover, in the year before diabetes onset, cases had slightly worse pulmonary function compared to controls (FEV1= 58.4 ± 27% predicted versus 67.4 ± 21% predicted; p = 0.05). No significant effects related to the other factors considered were found. Conclusion: Severe liver disease was found to significantly increase the risk of developing cystic fibrosis-related diabetes. Patients with liver disease should be scheduled for earlier diabetes screening in order to identify and possibly treat glucose intolerance. [ABSTRACT FROM AUTHOR]

Survivors of retinoblastoma (Rb) are at high risk of dying from second malignant tumour. The occurrence of second malignant neoplasm (SMN) and related mortality in a cohort of 1111 cases from the Italian Retinoblastoma Registry was analysed, considering the possible role of both genetic and iatrogenic causes. Rb patients had a greater than 10-fold excess in overall mortality compared with the general population (standardized mortality ratio (SMR) 10.73, 95% CI 9.00–12.80). Their excess risk attributable to cancers other than Rb was 14.93 95% CI 10.38–21.49). Survivors of hereditary Rb had an SMR for all causes of 16.25 (95% CI 13.20–20.00), whereas their SMR for all cancers was 25.72 (95% CI 17.38–38.07). Survivors of unilateral sporadic Rb had an SMR of 4.12 from all cancers (95% CI 1.55–10.98) and a much higher excess for overall mortality (SMR 13.34, 95% CI 10.74–16.56). As expected, survivors of hereditary Rb had higher mortality from cancers of the bone (SMR 391.90, 95% CI 203.90–753.20) and soft tissue (SMR 453.00, 95% CI 203.50–1008.40), small intestine (SMR 1375.50, 95% CI 344.00–5499.70), nasal cavity (SMR 13.71, 95% CI 1.93–97.35) and cancers of the brain and central nervous system (SMR 41.14, 95% CI 13.2–127.55)Oncogene (2006) 25, 5350–5357. doi:10.1038/sj.onc.1209786 [ABSTRACT FROM AUTHOR]

Aims: To evaluate cardiopulmonary exercise tolerance in a large cohort of apparently healthy paediatric cancer survivors in order to determine their participation in sporting activities. Methods: A total of 84 young (<21 years) asymptomatic childhood cancer survivors, who had been exposed to anthracyclines (mean dose 212 mg/m²) and/or chest irradiation (median dose 2000 cGy), with normal left ventricular systolic function at rest (fractional shortening >29%), and 79 healthy controls were studied. Exercise testing was performed on a treadmill ergometer. Gas exchange analysis and derived variables were measured on a breath-by-breath basis. Pulmonary functional evaluation was performed before exercise. Echocardiographic evaluation at rest was performed within one month before the exercise test. Results: There were no differences in exercise responses between patients and controls. In boys <13 years, mean VO2 max was slightly but significantly lower than in controls. This finding was thought to be a result of decreased physical fitness as all the other exercise parameters were similar to those in the controls. Conclusions: Results show that apparently healthy survivors of paediatric cancer can take part in dynamic sporting activities if they exhibit a normal response to cardiopulmonary exercise testing, white those that exhibit a reduced VO2 max should be re-evaluated after an aerobic training programme, and should undergo tailored dynamic physical activity if the VO2 max does not normalise. [ABSTRACT FROM AUTHOR]

Summary: Aplastic anemia (AA) is a rare disease with a major autoimmune pathogenetic component. CTLA4 is a T-lymphocyte surface molecule involved in the maintenance of immune tolerance. Some polymorphisms associated with a reduced expression of CTLA4, and thus presumably with increased tendency to autoimmunity, have been associated with various autoimmune diseases. In this study, we evaluated the distribution of the low expression polymorphisms −318C>T and 49A>G of CTLA4 in a population of 67 patients with acquired AA and in 100 normal controls. There was no difference in the distribution of the tested polymorphism between patients and controls and, within the patient group, between those who responded to immunosuppression vs those who did not respond. This study indicates that the polymorphisms −318C>T and 49A>G of CTLA4 do not affect the risk of developing AA and do not influence the response to immunosuppression. [ABSTRACT FROM AUTHOR]

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