[A dyspnoeic boy with two round masses on his chest X-ray]. / Een benauwde jongen met 2 'bollen' op de thoraxfoto.

A 5-year-old boy presented with vomiting and dyspnoea. His chest X-ray showed two round consolidations in his right lung, one of them with air bronchograms, which were caused by round pneumonia. Round pneumonia presents with multiple lesions in only 2% of cases.

Daily life physical activity in long-term survivors of nephroblastoma and neuroblastoma.

The risk of metabolic late effects after childhood cancer, such as obesity, hypertension, and diabetes, can be positively influenced by a healthy lifestyle with sufficient physical activity. Nevertheless, studies on physical activity in adult survivors of childhood cancer are scarce and involve different and often nonvalidated questionnaires. We used the Short QUestionnaire to ASsess Health-enhancing physical activity (SQUASH), which was developed and validated to assess daily life physical activity in the Dutch adult population. The aim of the study was to assess daily life physical activity in Dutch adult long-term nephroblastoma and neuroblastoma survivors. Sixty-seven nephroblastoma and 36 neuroblastoma survivors (median age, 30 y; range, 18 to 51 y) and 60 sociodemographically similar healthy control subjects (median age, 32 y; range, 18 to 62 y) were asked to complete the SQUASH during their regular follow-up visit. The adjusted mean physical activity score in male neuroblastoma survivors (mean, 7155; P=0.004) was significantly lower than in male controls (mean, 10,574), whereas it was not significantly lower in male nephroblastoma survivors (mean, 9122; P=0.108). Adjusted means for physical activity scores in females were not different from their controls. In conclusions, male neuroblastoma survivors were identified as performing less daily physical activity.

Endocrine sequelae and metabolic syndrome in adult long-term survivors of childhood acute myeloid leukemia.

This study focuses on the effect of chemotherapy on endocrinopathies and the metabolic syndrome in adult survivors of childhood acute myeloid leukemia (AML). Endocrine function and metabolic syndrome were evaluated in 12 AML survivors, treated with chemotherapy, and in 9 survivors of myeloid leukemias treated with stem cell transplantation (SCT), after a median follow-up time of 20 years (range 9-31). In survivors treated with chemotherapy, no endocrinopathies or metabolic syndrome were present, although AMH and Inhibin B levels tended to be lower than in controls. In SCT survivors, pituitary deficiencies and metabolic syndrome were more frequent.

Treatment factors rather than genetic variation determine metabolic syndrome in childhood cancer survivors.

BACKGROUND: Genetic variation that regulates insulin resistance, blood pressure and adiposity in the normal population might determine differential vulnerability for metabolic syndrome after treatment for childhood cancer. OBJECTIVE: To evaluate the contribution of candidate single nucleotide polymorphisms (SNPs) relevant for metabolic syndrome in our single centre cohort of adult long-term childhood cancer survivors. METHODS: In this retrospective study 532 survivors were analysed. Median age at diagnosis was 5.7 years (range 0.0-17.8 years), median follow-up time was 17.9 years (range 5.0-48.8) and median age at follow-up was 25.6 years (range 18.0-50.8). JAZF1 gene rs864745, THADA gene rs7578597, IRS1 gene rs2943641, TFAP2B gene rs987237, MSRA gene rs7826222, ATP2B1 gene rs2681472 and rs2681492 were genotyped. The association of genotypes with total cholesterol levels, blood pressure, body mass index, waist circumference and frequency of diabetes were assessed. RESULTS: Metabolic syndrome was more frequent in cranially (23.3%, P=0.002) and abdominally (23.4%, P=0.009) irradiated survivors as compared with non-irradiated survivors (10.0%). Association of allelic variants in rs2681472 and rs2681492 with hypertension, rs987237 and rs7826222 with waist circumference and rs864745, rs7578597 and rs2943641 with diabetes were not significant. None of the SNPs was associated with the metabolic syndrome. Adjusting for age, sex, follow-up time, cranial irradiation and abdominal irradiation did not change these results. CONCLUSIONS: Treatment factors and not genetic variation determine hypertension, waist circumference, diabetes and metabolic syndrome in adult long-term survivors of childhood cancer.

Abdominal radiotherapy: a major determinant of metabolic syndrome in nephroblastoma and neuroblastoma survivors.

BACKGROUND: Reports on metabolic syndrome in nephroblastoma and neuroblastoma survivors are scarce. Aim was to evaluate the occurrence of and the contribution of treatment regimens to the metabolic syndrome. PATIENTS AND METHODS: In this prospective study 164 subjects participated (67 adult long-term nephroblastoma survivors (28 females), 36 adult long-term neuroblastoma survivors (21 females) and 61 control subjects (28 females)). Controls were recruited cross-sectionally. Waist and hip circumference as well as blood pressure were measured. Body composition and abdominal fat were assessed by dual energy X-ray absorptiometry (DXA-scan). Laboratory measurements included fasting triglyceride, high density lipoprotein-cholesterol (HDL-C), glucose, insulin, low-density lipoprotein-cholesterol (LDL-C) and free fatty acids (FFA) levels. RESULTS: Median age at follow-up was 30 (range 19-51) years in survivors and 32 (range 18-62) years in controls. Median follow-up time in survivors was 26 (6-49) years. Nephroblastoma (OR = 5.2, P<0.0001) and neuroblastoma (OR 6.5, P<0.001) survivors had more components of the metabolic syndrome than controls. Survivors treated with abdominal irradiation had higher blood pressure, triglycerides, LDL-C, FFA and lower waist circumference. The latter can not be regarded as a reliable factor in these survivors as radiation affects the waist circumference. When total fat percentage was used as a surrogate marker of adiposity the metabolic syndrome was three times more frequent in abdominally irradiated survivors (27.5%) than in non-irradiated survivors (9.1%, P = 0.018). CONCLUSIONS: Nephroblastoma and neuroblastoma survivors are at increased risk for developing components of metabolic syndrome, especially after abdominal irradiation. We emphasize that survivors treated with abdominal irradiation need alternative adiposity measurements for assessment of metabolic syndrome.

Adrenal function in adult long-term survivors of nephroblastoma and neuroblastoma.

BACKGROUND: Adrenal insufficiency, or relative insufficiency, might partly explain increased mortality rates in nephroblastoma and neuroblastoma survivors after unilateral adrenalectomy. OBJECTIVE: To assess adrenal function and its metabolic effects in survivors after adrenalectomy. METHODS: In this cross-sectional study, 67 adult long-term survivors of nephroblastoma, 36 survivors of neuroblastoma and 49 control subjects participated. Adrenal function was assessed by a 1µg short Synacthen-test. Levels of cortisol, adrenocorticotrophic hormone (ACTH), low (LDL-C) and high-density lipoprotein-cholesterol (HDL-C), triglycerides, apolipoprotein-B, glucose and insulin were assessed in blood samples taken at baseline. In addition, cortisol levels were assessed after 30 (t=30) and 60 min. Homoeostatic Model Assessment (HOMA) was calculated. RESULTS: Adrenal insufficiency was not present in survivors. Interestingly, baseline serum cortisol levels were higher in survivors after unilateral adrenalectomy (mean 503 nmol/l) (N=46) than in survivors with both adrenals intact (mean 393 nmol/l, P=0.002) (N=52), and than in controls (mean 399 nmol/l, P=0.013) (N=49). After correcting for age, sex and use of oral oestrogens, unilateral adrenalectomy was independently associated with elevated baseline cortisol and ACTH levels. Baseline cortisol levels were positively associated with triglycerides (P<0.001), LDL-C (P=0.004), apolipoprotein-B (P<0.001) and HOMA (P=0.008). CONCLUSIONS: No adrenal insufficiency was observed in survivors of nephroblastoma and neuroblastoma. Survivors treated with unilateral adrenalectomy had relatively high basal cortisol and ACTH levels, indicating a higher central setpoint of the hypothalamic-pituitary-adrenal axis. This higher setpoint was associated with lipid concentrations and insulin resistance and can therefore influence the cardiovascular risk profile in long-term survivors of nephroblastoma and neuroblastoma.

The metabolic syndrome in adult survivors of childhood cancer, a review.

The number of adult survivors of childhood cancer in the general population has increased. As reports on the prevalence of the metabolic syndrome in adult survivors of childhood cancer are scarce, we reviewed the available literature on the components of the metabolic syndrome in adult survivors of childhood cancer. Although there is a lack of studies estimating the prevalence of metabolic syndrome directly, especially prevalence of insulin resistance, obesity, and dyslipidemia is increased in certain groups. Therefore, adult survivors of childhood cancer are at increased risk of developing cerebrovascular and cardiovascular diseases. Accordingly, it is important to identify the predisposing factors of the metabolic syndrome in cohorts of survivors, to introduce medical interventions, and to subsequently decrease the risk of cerebrovascular and cardiovascular events.