Our results suggest that children with severe hemophilia have reduced BMD. Patients at risk are those with signs of hemophilic arthropathy. Because osteoporosis may complicate the future treatment of patients with hemophilia, screening of young patients for reduced bone density is recommended.

No abstract

Cranial irradiation therapy (CRT) and chemotherapy are associated with neurobehavioural deficits. Many studies have investigated late effects of these treatments, but few have evaluated changes in abilities over time. This study employed a longitudinal design to map abilities following these treatments. Three groups of children were studied: Group 1 (n = 35): children treated with CRT (18 Gy) + chemotherapy, aged 5 years or less at time of diagnosis; Group 2 (n = 19): children treated with chemotherapy alone, aged 5 years or less at time of diagnosis; Group 3 (n = 35): healthy children. All children were aged 7–13 years at time of initial assessment, with no pre-diagnosis history of neurologic, developmental, or psychiatric disorder. Intellectual and educational abilities were evaluated twice: T1, not less than 2 years post-treatment, and T2, 3 years later. Group 1 achieved poorest results at T1, with comparison groups performing similarly. At T2 group differences were maintained. For verbal skills differences remained stable. Group 1 exhibited deterioration on non-verbal and processing tasks, while comparison groups showed improved abilities. Group 1 exhibited increases in literacy skills, with educational intervention predicting progress. Results suggest cumulative deficits in non-verbal and information processing skills for children treated with CRT + chemotherapy, with other deficits remaining relatively stable over time. Improved literacy skills suggest that gains can occur with remediation. © 2000 Cancer Research Campaign

Cognitive and educational sequelae are inconsistently reported in children treated with cranial irradiation for acute lymphoblastic leukaemia. This study investigated differences in these skills after cranial irradiation, controlling the effects of chemotherapy and psychosocial factors. Three groups were evaluated: 100 children diagnosed with acute lymphoblastic leukaemia and treated with cranial irradiation and chemotherapy; 50 children diagnosed with acute lymphoblastic leukaemia or other cancers and treated with chemotherapy alone; and a healthy control group of 100 children. Children in the clinical groups stopped treatment at least two years before evaluation and had no history of relapse. Children were aged between 7 and 16 at the time of assessment. Evaluation included cognitive, educational, and behavioural measures.Analyses found that children receiving cranial irradiation and chemotherapy performed more poorly than non-irradiated groups on intellectual and educational tests, with verbal and attentional deficits most pronounced. Children receiving chemotherapy alone performed similarly to controls, suggesting such treatment is not associated with adverse neurobehavioural sequelae.

We investigated the effectiveness of prophylactic administration of recombinant activated factor VII (rFVIIa) for cardiopulmonary bypass surgery in children under 1 year old with congenital heart disease (CHD) in a double-blinded, placebo-controlled study. The rFVIIa dose was 40 microg/kg and all patients also received standard haemostatic replacement therapy. The primary endpoint was the time to chest closure from neutralization of heparin with protamine sulphate as this could be most objectively and accurately measured during surgery. Secondary endpoints were volumes of transfused blood, platelet concentrates and fresh-frozen plasma. All adverse events were recorded. In the intention-to-treat analysis there were 76 patients (40 in rFVIIa group and 36 in placebo group). The demographics and severity of CHD were similar in both groups. No benefit of rFVIIa prophylaxis was found in the time to chest closure, which was significantly prolonged in the rFVIIa group (rFVIIa mean +/- SE, 98.8 +/- 27.27 versus 55.3 +/- 29.15, P = 0.0263). In the 41 patients available for a follow-up visit 6 weeks after discharge, the chest closure time was also prolonged in the rFVIIa group (P = 0.0515). There were no significant differences in the secondary endpoints. Adverse events were similar in both groups.