Here and Now Aboriginal Assessment (HANAA): a follow-up survey of users.

OBJECTIVES: To explore users' views of, and experience with, the Here and Now Aboriginal Assessment (HANAA), an instrument developed for screening social and emotional wellbeing (SEWB) in Aboriginal adults. Over the last few years the HANAA has been widely disseminated across Australia. METHODS: All those on our database who had requested the HANAA package were contacted by email and invited to participate in the evaluation by completing an online survey. RESULTS: A total of 38 responses were received; the response rate was 36%. All 10 HANAA domains were highly rated by respondents; the overall mean score was 8.3/10. Each domain was assessed using a dichotomous scale of 'problem' or 'no problem' which respondents rated as being very useful; the mean score was 8.4/10. The mean score for the 'recommendation' section at the end of the HANAA was 7.2/10. CONCLUSIONS: Respondents reported a high level of utility and cultural applicability of the HANAA's assessment domains, semi-structured narrative style of administration and simple rating system. The HANAA is making a useful and practical contribution to the assessment of Aboriginal SEWB at a community level. Recommendations for future work on the HANAA include consideration of addition of a personality domain and development of a child and adolescent version.

Cognitive and behavioral abnormalities in children after hematopoietic stem cell transplantation for severe congenital immunodeficiencies.

Hematopoietic stem cell transplantation (HSCT) is a highly successful treatment for severe congenital immunodeficiencies. However, some studies have suggested that children may experience cognitive difficulties after HSCT. This large-scale study assessed cognitive and behavioral function for the cohort of children treated by HSCT at one center between 1979 and 2003 to determine the frequency and severity of problems and to identify risk factors. A total of 105 patients were assessed on standardized measures of cognitive and emotional and behavioral function together with a control group of unaffected siblings. The average IQ for the cohort was 85 (95% confidence interval, 81-90), significantly lower than both the population average of 100 (P < .001) and unaffected siblings. Multivariate analysis indicated that the underlying genetic defect, diagnosis of adenosine deaminase-deficient severe combined immunodeficiency, and consanguinity were associated with worse outcome but that age at transplantation and chemotherapy conditioning were not. Children treated by HSCT for severe immunodeficiency have an increased risk of long-term cognitive difficulties and associated emotional and behavioral difficulties. The specific genetic diagnosis, consanguinity, and severe clinical course are associated with poor outcome. Long-term follow-up of these patients should include screening to identify and manage these problems more effectively.

Successful reconstitution of immunity in ADA-SCID by stem cell gene therapy following cessation of PEG-ADA and use of mild preconditioning.

Gene therapy is a promising treatment option for monogenic diseases, but success has been seen in only a handful of studies thus far. We now document successful reconstitution of immune function in a child with the adenosine deaminase (ADA)-deficient form of severe combined immunodeficiency (SCID) following hematopoietic stem cell (HSC) gene therapy. An ADA-SCID child who showed a poor response to PEG-ADA enzyme replacement was enrolled into the clinical study. Following cessation of enzyme replacement therapy, autologous CD34(+) HSCs were transduced with an ADA-expressing gammaretroviral vector. Gene-modified cells were reinfused following one dose of preconditioning chemotherapy. Two years after the procedure, immunological and biochemical correction has been maintained with progressive increase in lymphocyte numbers, reinitiation of thymopoiesis, and systemic detoxification of ADA metabolites. Sustained vector marking with detection of polyclonal vector integration sites in multiple cell lineages and detection of ADA activity in red blood cells suggests transduction of early hematopoietic progenitors. No serious side effects were seen either as a result of the conditioning procedure or due to retroviral insertion. Gene therapy is an effective treatment option for the treatment of ADA-SCID.

Allozyme variation and sociogenetic structure of Polistes satan Bequaert 1940 colonies (Hymenoptera, Vespidae)

We collected 40 colonies of the wasp Polistes satan in November 1993 (Population A = 23 colonies) and October 1995 (Population B = 17 colonies) from the town of Delfinopólis in the Brazilian state of Minas Gerais. Pupae from these nests were used to assess allozyme variation and genetic relatedness among nestmates. Of the 24 gene loci sampled six showed allozyme polymorphism (Est-2, Pgm-1, 6Pgd, Hk-1, Idh and Pep-A) and all loci except Idh were at Hardy-Weinberg equilibrium. Populations A and B showed distinct intra-loci heterozygosities, but similar average heterozygosity over all loci. Significant inter-population differentiation (Fst = 0.051; p < 0.01) was detected, probably due to genetic drift driven by bottlenecking that occurred due to the first sampling. Mean intra-colony relatedness values between female broods (rA = 0,525 ± 0.063; rB = 0,456 ± 0.103) indicated that P. satan has a simple sociogenetic structure where one female takes on most of the reproductive tasks but, eventually, subordinates also reproduce in the colony.