Concentration, working speed and memory: cognitive problems in young childhood cancer survivors and their siblings.

BACKGROUND: Cognitive problems can have a negative effect on a person's education, but little is known about cognitive problems in young childhood cancer survivors (survivors). This study compared cognitive problems between survivors and their siblings, determined if cognitive problems decreased during recent treatment periods and identified characteristics associated with the presence of a cognitive problem in survivors. METHODS: As part of the Swiss Childhood Cancer Survivor Study, a questionnaire was sent to all survivors, aged 8-20 years, registered in the Swiss Childhood Cancer Registry, diagnosed at age <16 years, who had survived ≥ 5 years. Parent-reported (aged 8-15 years) and self-reported (aged 16-20 years) cognitive problems (concentration, working speed, memory) were compared between survivors and siblings. Multivariable logistic regression was used to identify characteristics associated with cognitive problems in survivors. RESULTS: Data from 840 survivors and 247 siblings were analyzed. More often than their siblings, survivors reported problems with concentration (12% vs. 6%; P = 0.020), slow working speed (20% vs. 8%; P = 0.001) or memory (33% vs. 15%; P < 0.001). Survivors from all treatment periods were more likely to report a cognitive problem than were siblings. Survivors of CNS tumors (OR = 2.82 compared to leukemia survivors, P < 0.001) and those who had received cranial irradiation (OR = 2.10, P = 0.010) were most severely affected. CONCLUSION: Childhood cancer survivors, even those treated recently (2001-2005), remain at risk to develop cognitive problems, suggesting a need to improve therapies. Survivors with cognitive problems should be given the opportunity to enter special education programs.

Clustering of health behaviours in adult survivors of childhood cancer and the general population.

BACKGROUND: Little is known about engagement in multiple health behaviours in childhood cancer survivors. METHODS: Using latent class analysis, we identified health behaviour patterns in 835 adult survivors of childhood cancer (age 20-35 years) and 1670 age- and sex-matched controls from the general population. Behaviour groups were determined from replies to questions on smoking, drinking, cannabis use, sporting activities, diet, sun protection and skin examination. RESULTS: The model identified four health behaviour patterns: 'risk-avoidance', with a generally healthy behaviour; 'moderate drinking', with higher levels of sporting activities, but moderate alcohol-consumption; 'risk-taking', engaging in several risk behaviours; and 'smoking', smoking but not drinking. Similar proportions of survivors and controls fell into the 'risk-avoiding' (42% vs 44%) and the 'risk-taking' cluster (14% vs 12%), but more survivors were in the 'moderate drinking' (39% vs 28%) and fewer in the 'smoking' cluster (5% vs 16%). Determinants of health behaviour clusters were gender, migration background, income and therapy. CONCLUSION: A comparable proportion of childhood cancer survivors as in the general population engage in multiple health-compromising behaviours. Because of increased vulnerability of survivors, multiple risk behaviours should be addressed in targeted health interventions.

Obesity in long-term survivors of childhood acute lymphoblastic leukemia.

BACKGROUND: Childhood acute lymphoblastic leukemia (ALL) with current cure rates reaching 80% emphasizes the necessity to determine treatment related long-term effects. The present study examines the prevalence of and the risk factors for overweight and obesity in a cohort of ALL survivors treated and living in the French speaking part of Switzerland. METHODS: In this retrospective two-center study, height and weight of 54 patients diagnosed with ALL in first complete remission and treated with chemotherapy only were recorded at specified time points during treatment and off-therapy. Body mass index (BMI) and its age- and gender-adjusted standard deviation score (BMI-SDS) were calculated for the patients and their parents separately. Overweight and obesity were defined by a threshold of BMI-SDS >1.645 and BMI-SDS >1.96, respectively. RESULTS: At last follow-up, 16 (30%) of the 54 survivors were overweight and 10 (18%) were obese. The off-treatment period was most at risk with 11 of the 16 becoming overweight and 9 of the 10 becoming obese during that period. Overweight/obesity at diagnosis and abnormal maternal BMI were significantly associated with abnormal weight at follow-up, while age at diagnosis, gender, cumulative dose of steroids and paternal BMI showed no association. CONCLUSIONS: Consistent with published evidence from other regions of the developed and developing world, there is a significant prevalence of obesity in young ALL survivors in the French speaking part of Switzerland. Factors significantly associated with this late effect were mostly related to the familial background rather than to the treatment components.

Incidence of childhood cancer in Switzerland: the Swiss Childhood Cancer Registry.

BACKGROUND: This report describes the incidence of childhood cancer in Switzerland, based on the data from the Swiss Childhood Cancer Registry (SCCR), a national hospital-based cancer registry with very high coverage, founded in 1976 by the Swiss Paediatric Oncology Group (SPOG). PROCEDURE: Malignancies were coded according to the International Classification of Childhood Cancer (ICCC-3). Incidence rates per 100,000 person-years were calculated for all malignancies and groups of malignancies in Swiss residents less than 15 years of age for the decade 1995-2004. RESULTS: The SCCR annually registered on average 174 new cases of cancer in Swiss residents aged <15 years, with a median age at diagnosis of 5.6 years. The crude incidence of childhood cancer in children aged <15 years was 13.5, higher for boys (15.0 per 100,000) than for girls (12.1 per 100,000), and was nearly twice as high in the first 5 years of life (19.3 per 100,000) than in the age group 5 to 14 years (10.8 per 100,000). CONCLUSION: Incidence of childhood cancers in the SCCR was similar to neighbouring countries and to data published by regional cancer registries in Switzerland for the same period, suggesting good completeness of registration. This makes the SCCR a valuable resource for national and international research on childhood cancer.

Severe nose bleeding after intake of acetylsalicylic acid: von Willebrand disease type 2A. Case 9.

This case report of a school boy with a history of severe and repeated episodes of epistaxis presents a short overview of the clinical and laboratory findings which lead to confirm the suspected diagnosis of von Willebrand disease (vWD). Suspicion of defective primary haemostasis should arise when unusual (because of their number or duration) mucosal bleeds appear in an otherwise normal and healthy patient. Because of its definitive inhibitory effect on platelet aggregation, acetylsalicylic acid (more than other non-steroidal anti-inflammatory drugs exerting unselective inhibition of cyclooxygenase) is a strong factor in triggering or sustaining the bleeding disorders in these patients. Among the congenital disorder of primary haemostasis, vWD is by far the most frequent one. The difficulties of laboratory diagnosis of vWD are stressed; the promises and pitfalls of new in vitro methods for measuring primary haemostasis (PFA-100 analyzer) are discussed. An accurate diagnosis of the specific type of vWD is of critical importance for correct patient management as well as for genetic counseling.

Cisplatin therapy in childhood: renal follow up 3 years or more after treatment. Swiss Pediatric Oncology Group.

BACKGROUND: In childhood, cisplatin is an essential component of solid tumour therapy such as in neuroblastomas, germ cell tumours, bone tumours, liver tumours and brain tumours. The potential nephotoxicity of cisplatin is widely recognized, but little information is available on permanent sequelae. METHODS: Of the 500 children included in the Swiss Pediatric Oncology Group Late Effect Study, a group of 46 patients (27 males and 19 females) aged 5.7-28 years (median 14 years) surviving the above-mentioned solid tumours entered the present study. The patients were disease-free and off antineoplastic medication for at least 3 years. No recent gastrointestinal or urinary disturbances had occurred, and diets as well as appetites were normal. RESULTS: Blood pressure and plasma or urinary calcium and phosphate were similar in 17 patients treated with cisplatin (dose 142-717, median 400 mg/m2), in 19 patients without cisplatin and in 20 control subjects. A tendency (P<0.02) towards increased plasma creatinine (79 (69-89) micromol/l; median and interquartile range) and low plasma magnesium (0.80 (0.78-0.85) mmol/l) was noted in patients treated with cisplatin as compared with those without cisplatin (68 (58-80) micromol/l; 0.84 (0.79-0.90) mmol/l) and controls (71 (64 80) micromol/l; 0.83 (0.80-0.90) mmol/l). No correlation was noted between the dosage of cisplatin and circulating magnesium or creatinine. CONCLUSIONS: The study demonstrates that the permanent renal disturbances ((i) decreased renal function and (ii) hypomagnesaemia) noted after treatment with cisplatin during infancy or childhood are mild. Furthermore, the study does not demonstrate renal sequelae in patients with the same malignancies who had been treated without cisplatin.

Acute fatal myeloencephalopathy after combined intrathecal chemotherapy in a child with acute lymphoblastic leukemia.

We report a case of a fatal toxic encephalomyelopathy in a 12-year-old girl due to prophylactic intrathecal injection of methotrexate and cytosine arabinoside, with a characteristic progressive symptomatology leading to death after 28 days. The location and type of neuropathological changes support the hypothesis of a direct toxic effect of methotrexate and/or cytosine arabinoside on structures directly exposed to the cerebrospinal fluid.