Single institution outcomes of treatment of severe aplastic anaemia

Background: In severe aplastic anaemia, the treatment of choice for young patients with a human leucocyte antigen-matched sibling is now established as allogeneic bone marrow transplantation (BMT). In older patients and in those without a matched sibling donor, immunosuppressive therapy is the usual first option. 'Alternative' marrow donors are emerging as an option for those without a matched sibling donor. Aims: To review 10 years of local experience in treating severe aplastic anaemia with BMT and immunosuppressive therapy with emphasis on long-term outcomes. Methods: A retrospective analysis was performed of all patients with severe aplastic anaemia presenting to the Royal Brisbane and Royal Children's Hos- pitals between 1989 and 1999. Data were abstracted regarding patient demographics, pretreatment characteristics and outcome measures, including response rates, overall survival and long-term complications. Results: Twenty-seven consecutive patients were identified, 12 treated with immunosuppression alone and 15 with BMT. In these two groups, transfusion independence was attained in 25% and 100%, respectively, with overall survival being 36% and 100%, respectively. Those treated with immunosuppression were significantly older (median 41.5 versus 22 years, P = 0.008). Long-term survivors of either treatment had extremely low morbidity. Three patients carried pregnancies to term post-transplant. Three patients received alternative donor BMT with correspondingly excellent survival. Conclusions: Patients treated with allogeneic BMT for severe aplastic anaemia enjoyed extremely good long-term survival and minimal morbidity. Patients treated with immunosuppressive therapy had a poorer outcome reflecting their older age and different usage of therapies over the past decade. Optimal treatment strategies for severe aplastic anaemia remain to be determined.

Liver transplantation: current status and future prospects

The enormous progress that has been made in liver transplantation over the past two decades has culminated in survival approaching 90% at 12 months. The success of the procedure combined with the widening spectrum of disease processes deemed amenable to liver transplantation has meant that there are too few donors for those awaiting transplantation. This has extrapolated to many patients having such advanced disease by the time a suitable donor liver is available, that they are almost non-transplantable. The immediate options facing the transplant community are to decrease the number of patients listed or to increase the number of living donor transplants. Alternatives to liver transplantation such as hepatocyte transplantation, gene therapy, xenotransplantation and the bioartificial liver are being sought but, at best, are some way from clinical application. It is anticipated that a number of liver diseases that are indications for liver transplantation at this time will have progression arrested or will be cured by medical therapy in the future.

High prevalence of asthma in five remote indigenous communities in Australia

Data on the prevalence of asthma in children residing in remote indigenous communities in Australia are sparse, despite the many reports of high prevalence in nonindigenous children of this country. Two previous Australian studies have had poor participation rates, limiting interpretation of their results. A study of children in the Torres Strait and Northern Peninsula Area of Australia was conducted to document the prevalence of asthma symptoms. Five indigenous communities were randomly selected and trained interviewers, who were local indigenous health workers, recruited participants using a house-by-house approach. Information was collected by a structured face-to-face interview based on standardized questionnaire constructed from the protocol International Study of Asthma and Allergy in Childhood; 1,650 children were included in the study with a 98% response rate. Overall, the prevalence of self-reported ever wheezing was 21%,; 12% reported wheezing in the previous year; and 16%, reported ever having asthma, There was significant variation in the prevalence of asthma symptoms between communities. It is concluded that there are significant intercommunity variations in the prevalence of asthma symptoms in remote communities and that the prevalence in these communities is as high as in nonindigenous groups.

Pediculus humanus capitis infestations in the community: A pilot study into transmission, treatment and factors affecting control

Head lice (Pediculus humanus capitis) infestations affect schoolchildren worldwide, creating social, economic and health consequences for families. Problems with self-detection, chronic infestations and classroom transmission are compounded by increasing resistance of the lice to pediculicides. Public health strategies are based on limited research and little is known about transmission dynamics. Mismanagement and transmission in the general community are blamed for control failure. The purpose of this study was to explore community head-lice experience in Brisbane, Australia, and to identify critical factors underlying control failure. A home-based pilot survey used physical examination to verify transmission and treatment patterns which were self-reported by a group of trace-contact families in addition to other unconnected participants. The survey was enlarged to further compare therapy outcomes and suspected risk factors. The findings reinforce those of previous studies - that children attending school and early childhood centres, and subsequently their families, are most at risk of contracting pediculosis capitis, and some may carry lice for years. First-line (pediculicidal) treatment and even additional physical methods of hand-picking and fine-toothed combing usually fail to eradicate lice quickly and completely (overall cure-rate 39 per cent, n = 84 cases). Failures were linked to hair characteristics. Public education alone may not control pediculosis. Accurate diagnosis requires considerable experience; a strong case exists for returning to institutional surveillance.

Experimental infection of Australian brushtail possums, Trichosurus vulpecula (Phalangeridae : Marsupialia), with Ross River and Barmah Forest viruses by use of a natural mosquito vector system

Brushtail possums, Trichosurus vulpecula Kerr, were experimentally infected with Ross River (RR) or Barmah Forest (BF) virus by Aedes vigilax (Skuse) mosquitoes. Eight of 10 animals exposed to RR virus developed neutralizing antibody, and 3 possums developed high viremia for < 48 hr after infection, sufficient to infect recipient mosquitoes. Two of 10 animals exposed to BF virus developed neutralizing antibody. Both infected possums maintained detectable neutralizing antibody to BF for at least 45 days after infection (log neutralization index > 2.0 at 45 days). Eight possums did not develop neutralizing antibody to BF despite exposure to infected mosquitoes. These results suggest that T. vulpecula may potentially act as a reservoir species for RR in urban areas. However, T. vulpecula infected with BF do not develop viremia sufficient to infect mosquitoes and are unlikely to be important hosts for BF.

A decade of Taiwanese migration in Australia: Comparisons with Mainland Chinese and Hong Kong settlers

The arrival of Taiwanese migrants to Australia represents the second major wave of Chinese immigration to this nation. Many who entered Australia did so as business migrants. They were typically well educated, affluent professionals, managers, &/or entrepreneurs who were looking for new business opportunities as well as a lifestyle characterized by open space, clean air, a good education for their children, & personal & political safety. Yet, the settlement experiences of many Taiwanese migrants, despite their affluence & (business) skills, have been typified by stress & hardship, particularly in making adjustments in social, business, & economic relationships. A review of statistical data compiled from census & government reports in Australia has revealed that after a decade Down Under, the Taiwanese settler group was still characterized by high unemployment, even when compared to other Chinese migrant groups from Hong Kong & Mainland China. It is suggested that the Taiwanese migrants' persistent high nonparticipation in Australia's labor force is indicative & poignant of their highly distinctive, albeit not exclusive in the broader Chinese migrant terms, experience of migration settlement. There seems to be an increasing number of Taiwanese settlers returning to resettle in Taiwan in recent years, because of perceived better employment & business opportunities or for family & personal reasons. Recent interviews with Taiwanese settlers have also suggested that the most recent arrivals, being more aware of the obstacles in achieving work or business satisfaction during settlement, seem less likely to commit themselves to lifelong settlement in Australia. 16 Tables, 1 Figure, 37 References. Adapted from the source document.

Conservative management of mesenchymal hamartomas of the chest wall

Mesenchymal hamartomas of the chest wall are rare benign lesions usually discovered in infancy. The authors present their experience with 3 cases. All of these cases were managed initially conservatively, although 1 child required a thoracotomy and partial tumour resection at 5 months of age because of respiratory compromise. The other 2 children have now reached 5 and 6 years of age with the tumors becoming less prominent. The authors believe many cases can be managed conservatively because malignant change has not been reported, and the lesions often become relatively smaller as the child grows. J Pediatr Surg 36:1346-1349, Copyright (C) 2001 by W.B. Saunders Company.

Maternal antecedents for cerebral palsy in extremely preterm babies: A case-controlled study

The study aimed to identify significant antenatal risk factors for cerebral palsy (CP) among extremely preterm infants with a matched case-control design. Infants born between 1989 and 1996 at 24 to 27 weeks' gestation who survived to hospital discharge were evaluated: 30 with a proven diagnosis of CP at 2 years corrected for prematurity and 120 control children matched for gestational age without CP. Information on maternal obstetric risk factors and medication was obtained. Matched analyses were performed and odds ratios (OR) and 95% confidence intervals (CI) were calculated. An antenatal diagnosis of intrauterine growth restriction was associated with an increased risk of CP (OR 6.6; 95% CI 1.8 to 25.2), while maternal administration of corticosteroids was associated with a reduced risk of CP (OR 0.4; 95% CI 0.1 to 0.98). A high rate of placental histopathology was achieved but no relation between clinical or histological chorioamnionitis or funisitis and CP was demonstrated. Maternal preeclampsia was not associated with a statistically significant reduction in the risk of CP. It is concluded that a reduced risk of CP in extremely preterm infants is associated with the antenatal use of corticosteroids.

A case study of partial agenesis of the corpus callosum: Audiological implications

This case study represents four years of audiological observations, testing and aural habilitation of a female child with a partial agenesis of the corpus callosum (ACC). The ACC was diagnosed by MRI scans to eliminate neurological causes for developmental delay at six months of age. This child was also born with a cleft palate and was diagnosed with Robinow Syndrome at 3 years and 3 months of age. The audiological results showed an improvement in hearing thresholds over the four-year period. The child’s opthamologist also reported an improvement in visual skills over time. The most interesting aspect of the child’s hearing was the discrepancy between the monaural and the binaural results. That is, when assessed binaurally she often presented with a mild to moderate mixed loss and when assessed monaurally she showed a moderate to severe mixed loss for the right ear and a severe mixed loss for the left ear. This discrepancy between binaural and monaural results was evident for both aided and unaided tests. Parental reports of the child’s hearing were consistent with the binaural clinical results. This case indicates the need for audiologists to: (a) carefully monitor the hearing of children with ACC, (b) obtain monaural and binaural hearing and aided thresholds results, and (c) compare these children’s functional abilities to the objective test results obtained. This case does question whether hearing aids are appropriate for children with ACC. If hearing aids are deemed to be appropriate, then hearing aids with compression characteristics should be considered.

Two new classes of conopeptides inhibit the alpha 1-adrenoceptor and noradrenaline transporter

Cone snails use venom containing a cocktail of peptides ('conopeptides') to capture their prey. Many of these peptides also target mammalian receptors, often with exquisite selectivity. Here we report the discovery of two new classes of conopeptides. One class targets alpha (1)-adrenoceptors (rho -TIA from the fish-hunting Conus tulipa), and the second class targets the neuronal noradrenaline transporter (chi -MrIA and chi -MrIB from the mollusk-hunting C. marmoreus). rho -TIA and chi -MrIA selectively modulate these important membrane-bound proteins. Both peptides act as reversible non-competitive inhibitors and provide alternative avenues for the identification of inhibitor drugs.

Childhood neglect and cognitive development in extremely low birth weight infants: A prospective study

Objective. To examine the relationship between child maltreatment and cognitive development in extremely low birth weight infants, adjusting for perinatal and parental risk factors. Methods. A total of 352 infants with birth weight of

A highly conserved c-fms gene intronic element controls macrophage-specific and regulated expression

The c fins gene encodes the receptor for macrophage colony-stimulating factor-1. This gene is expressed selectively in the macrophage cell lineage. Previous studies have implicated sequences in intron 2 that control transcript elongation in tissue-specific and regulated expression of c -fms. Four macrophage-specific deoxyribonuclease I (DNase I)-hypersensitive sites (DHSS) were identified within mouse intron 2. Sequences of these DHSS were found to be highly conserved compared with those in the human gene. A 250-bp region we refer to as the fins intronic regulatory element (FIRE), which is even more highly conserved than the c-fins proximal promoter, contains many consensus binding sites for macrophage-expressed transcription factors including Spl, PU.1, and C/EBP. FIRE was found to act as a macrophage-specific enhancer and as a promoter with an antisense orientation preference in transient transfections. In stable transfections of the macrophage line RAW264, as well as in clones selected for high and low-level c -fms mRNA expression, the presence of intron 2 increased the frequency and level of expression of reporter genes compared with those attained using the promoter alone. Removal of FIRE abolished reporter gene expression, revealing a suppressive activity in the remaining intronic sequences. Hence, FIRE is shown to be a key regulatory element in the fins gene.