Prospective audit of treatment of paediatric febrile neutropenia in Australasia.

OBJECTIVES: Febrile neutropenia post-chemotherapy continues to impose a burden of morbidity and mortality on patients and families affected by childhood cancer, whereas these unplanned hospital admissions increase the financial cost of treating paediatric malignancies. There are currently no published national guidelines. This study comprises the first audit of current therapeutic practice in Australasia. METHODS: Information was sought prospectively from the 12 paediatric oncology tertiary referral centres in Australia and New Zealand regarding treatment of febrile neutropenia episodes commencing between 11 March and 10 May 2002. RESULTS: Data were returned on 127 episodes by nine centres. The median length of stay was 6 days and 18 different antibiotic regimens were implemented as first-line therapy. The median neutrophil count at the beginning and end of the febrile neutropenic episode was 0.0 x 10(9)/L (range 0.0 to 2.3 x 10(9)/L) and 0.7 x 10(9)/L (range 0.0 to 25.4 x 10(9)/L), respectively. Thirty per cent of episodes had positive blood cultures. Of these, 81% occurred in patients with tunnelled central venous catheters. The initial antimicrobial combination was changed in 61% of episodes. Outpatient antibiotics were used in 21% episodes after initial intravenous antimicrobial therapy. CONCLUSIONS: The current practice in Australasia is consistent with international guidelines, although changes are made more frequently to first-line therapy than in previous published studies. The central venous catheters are associated with a much higher risk of bacteraemia and consideration should be given to increased use of implanted port systems.

Evidence for susceptibility genes to familial Wilms tumour in addition to WT1, FWT1 and FWT2.

Three loci have been implicated in familial Wilms tumour: WT1 located on chromosome 11p13, FWT1 on 17q12-q21, and FWT2 on 19q13. Two out of 19 Wilms tumour families evaluated showed strong evidence against linkage at all three loci. Both of these families contained at least three cases of Wilms tumour indicating that they were highly likely to be due to genetic susceptibility and therefore that one or more additional familial Wilms tumour susceptibility genes remain to be found.

Childhood cancer in New Zealand 1990 to 1993.

An epidemiological study of childhood cancer in New Zealand identified 409 children aged 0 to 14 years with malignant neoplasms newly diagnosed between 1990 and 1993 inclusive. The original microscopic material on which the diagnoses were based was reviewed in 398 cases and the neoplasms were allocated into the 12 major groupings and 48 further subcategories of the International Classification of Childhood Cancer (ICCC). The pathology reviewers agreed with group and subcategory classification of the confirmed cancers in all but one case of acute leukemia and three cancers of the central nervous system. Changes were also made in the FAB classification of three cases of acute non-lymphocytic leukemia and in the further subcategorisation of three Hodgkin's lymphomas and ten astrocytomas. The results show a high level of diagnostic accuracy for confirmed childhood neoplasms in that time period. Nine of 15 cases of malignant melanoma notified to the study were not confirmed for various reasons, which included a change in the pathological diagnosis in four cases. Compared with Victoria (Australia), New Zealand has a high incidence rate of lymphomas in boys and an unusual female preponderance of Wilms' tumor cases.

Childhood acute lymphoblastic leukaemia: outcomes of 54 consecutive patients treated in Auckland 1985-1991.

AIMS: To report the survival and event-free survival of children with acute lymphoblastic leukaemia treated in Auckland between 1985-1991, using an international protocol, ANZCCSG ALL Study V. METHODS: The data were collected prospectively as part of the study. The analysis was carried out using standard survival methods. RESULTS: At a median follow up of 7.5 years, the overall survival is 75% and event-free survival 60%. CONCLUSIONS: For an unselected series, these outcomes are comparable to overseas series. The survival for children diagnosed in the 1990's is likely to be similar.

Good prognosis of cellular mesoblastic nephroma with hyperdiploidy and relaxation of imprinting of the maternal IGF2 gene.

A large congenital mesoblastic nephroma (CMN) of combined classical and and cellular histological structure was removed from a 1-month-old female infant. The tumor extended extrarenally and may have been incompletely excised. Tumor tissue showed a mosaic hyperdiploidy with 54 chromosomes in the hyperdiploid line. No other antitumor therapy was given and there has been no recurrence after 4 years. Genomic imprinting normally prevents transcription of the maternal gene for insulin-like growth factor 2 (IGF2). Relaxation of IGF2 imprinting leading to abnormal transcription of the maternal gene is found in a majority of Wilms' tumors and in other malignant neoplasms. The biallelic transcription of IGF2 demonstrated in the CMN from this case is consistent with abnormal transcription of the maternal allele. Relaxation of imprinting of the maternal IGF2 gene or abnormal expression of the gene through other mechanisms may have a role in the genesis of CMN or the cellular subtype.

Constitutional relaxation of insulin-like growth factor II gene imprinting associated with Wilms' tumour and gigantism.

We have examined the imprinting of the insulin-like growth factor II gene (IGF2) in ten normal kidney samples from children with renal embryonal neoplasms. In kidney samples from nine children with normal growth profiles, IGF2 mRNA was transcribed monoallelically, consistent with normal imprinting of the gene. But in one child who had generalized somatic overgrowth, IGF2 was transcribed from both alleles in her kidney, peripheral blood leukocytes and Wilms' tumour. These findings suggest that a defect in genomic imprinting can occur constitutionally, leading to growth abnormalities and predisposition to Wilms' tumour.

Childhood cancer among the Polynesian population.

From June 1981 through June 1989, 95 Polynesian children were seen for initial care of malignancy at the Princess Mary Hospital for Children (PMHC). The incidence of malignancy in the Polynesian populations served, the histology of the malignancies, and the outcome of therapy were reviewed and compared with 185 non-Polynesian (non-P) patients seen during the same period. Incidence figures for Polynesians and non-P were similar, but histologic patterns differed, showing an increased occurrence of leukemia, particularly nonlymphoblastic leukemia, an increased occurrence of bone tumors, and a decreased incidence of central nervous system tumors for Polynesians. Survival for Polynesian children with acute lymphoblastic leukemia was worse than for non-P. Survival in all other disease categories was similar.

Intraoperative pulse oximetry in peripheral revascularization in an infant.

Vascular patency after reimplantation has been evaluated by numerous methods. A patient is described in whom pulse oximetry was used for this purpose. Other techniques of evaluating vascular patency are mentioned, and the physics of pulse oximetry are briefly discussed.

Ultrastructural heterogeneity in cerebral amyloid of Alzheimer's disease.

Cerebral amyloid deposits from five patients with presenile or senile cerebral disease of the Alzheimer type were stained with uranyl acetate and lead citrate or with periodic acid-thiocarbohydrazide-silver proteinate, and examined with traditional high-resolution electron microscopy and with a goniometer tilting stage. In addition to a carbohydrate-rich matrix, we also consistently found local cell-derived vesicles within plaque and dyshoric amyloid. The most likely source for these vesicles appeared to be degenerate neurites. Amyloid fibrils were intimately associated with plasmalemmata, particularly those of degenerate neurites, which supported a neuronal origin for the amyloid fibril of Alzheimer's disease.

Scent of gerbil cuisine.

Preweanling and postweaning offspring of gerbil parents fed Purina Laboratory Chow or Pooch dog food show selective preference for odors coming from other animals of these same diets. Odors with differential quality include whole animal, cage material and ventral scent gland sebum. Apparently diet alters chemosignals, even those from a specialized scent gland. Species-specific pheromones may be dependent on ingested foodstuffs or other ecological chemicals.

Retention of learned temperature changes during problem solving.

In 2 studies (ns = 10, 9) college students were trained using biofeedback to lower or to raise finger temperature. Successful subjects were then asked to do distracting intellectual tasks, while simultaneously retaining the learned temperature changes. Results indicated that these changes could be maintained without detectable decreases in problem-solving efficiency in terms of either time or number of errors. These findings suggested that it might be possible for subjects to function efficiently outside the laboratory in their everyday activities while simultaneously exercising voluntary control over some autonomic processes.