The importance of specialized sleep investigations in children with a suprasellar tumor.

PURPOSE: Disruption of sleep has great impact on quality of life. In children with a suprasellar tumor and hypothalamic-pituitary dysfunction, the circadian rhythm may be disturbed causing sleep problems. However, also other factors may influence sleep. Awareness of these different etiologies and careful history taking with appropriate additional diagnostics will aid in restoring sleep quality. METHODS: We present the workup of 4 cases with a suprasellar tumor and disturbances of sleep initiation, sleep maintenance, and daytime sleepiness. In parallel, we developed a flowchart, to aid clinicians in the diagnostics of sleep problems in children after treatment for a (supra) sellar brain tumor. RESULTS: All four patients, known with hypopituitarism, presented with sleep complaints and increased daytime sleepiness. In all four, the cause of sleep problems showed to be different. In the first case, sleep evaluation revealed a severe obstructive sleep apnea, whereupon nocturnal ventilation was started. The second case revealed poor sleep hygiene in combination with an obsessive compulsive disorder. Sleep hygiene was addressed and psychiatric consultation was offered. Dexamphetamine treatment was started to reduce her obsessive compulsive complaints. The third case showed a delayed sleep phase syndrome, which improved by educational support. The fourth case revealed a secondary organic hypersomnia for which modafinil treatment was started. CONCLUSION: Sleep disturbances in children with hypopituitarism due to a (supra) sellar tumor can have different entities which require specific therapy. Awareness of these different entities is important to enable appropriate counseling. Referral to an expertise sleep center may be advised, if standard educational support is insufficient.

[Adolescents with acute lymphatic leukaemia achieve significantly better results when treated following Dutch paediatric oncology protocols than with adult protocols]. / Adolescenten met acute lymfatische leukemie; bij behandeling volgens Nederlandse kinderoncologische protocollen significant betere resultaten dan bij het volgen van protocollen voor volwassenen.

OBJECTIVE: To compare the results, in adolescents with acute lymphatic leukaemia (ALL), of treatment according to the protocols of the Netherlands Foundation for Paediatric Oncology (DCOG) or according to the protocols for adults of the Dutch Foundation for Adult Haemato-Oncology (HOVON). DESIGN: Retrospective. METHOD: During the period from May 1985 to November 1999, 120 15-20-year-old adolescents with ALL were treated: 47 according to a DCOG protocol and 73 according to a HOVON protocol. The records of the integrated cancer centres indicate that this represented about two-thirds of all known adolescents with ALL in the Netherlands. RESULTS: Adolescents with ALL treated according to the DCOG protocols had significantly better 5-year survival rates (79% versus 38%), a significantly lower probability of relapse (27% versus 55%) and a lower treatment-related mortality (4% versus 25%) than the adolescents treated according to the HOVON protocols. CONCLUSION: This difference in outcome was most likely related to differences in structure and content between the DCOG and HOVON protocols. The HOVON protocols consisted of relatively short, intensive chemotherapy, often followed by autologous or allogeneic bone-marrow transplantation. Several treatment elements present in the DCOG protocols, such as high-dose methotrexate, reinduction therapy and maintenance therapy were absent in the HOVON protocols. Moreover, the cumulative dosages of dexamethasone, mercaptopurine, asparaginase and vincristine were lower in the HOVON protocols.

Management of hyperuricemia with rasburicase review.

Tumor lysis syndrome (TLS) is a serious complication in patients with hematological malignancies. Massive lysis of tumor cells can lead to hyperuricemia, hyperkalemia, hyperphosphatemia and hypocalcaemia. These metabolic disturbances may result in renal failure, because of precipitation of uric acid crystals and calcium phosphate salts in the kidney. The standard prophylaxis or treatment of hyperuricemia consists of decreasing uric acid production with allopurinol and facilitating its excretion by urinary alkalinization and hyperhydration. By inhibiting the enzyme xanthine oxidase, allopurinol blocks the conversion of hypoxanthine and xanthine into uric acid. An alternative treatment is urate oxidase which oxidates uric acid into allantoin. Allantoin is 5-10 times more soluble than uric acid and is therefore excreted easily. In several clinical trials rasburicase, the recombinant form of urate oxidase, has shown to be very effective in preventing and treating hyperuricemia. Rasburicase, in contrast with the non-recombinant form of urate oxidase uricozyme, is associated with a low incidence of hypersensitivity reactions. In addition to the demonstrated clinical benefit, rasburicase also proved to be a cost-effective option in the management of hyperuricemia.