Devil Facial Tumor Disease.

Devil facial tumor disease (DFTD) is an emergent transmissible cancer exclusive to Tasmanian devils (Sarcophilus harrisii) and threatening the species with extinction in the wild. Research on DFTD began 10 years ago, when nothing was known about the tumor and little about the devils. The depth of knowledge gained since then is impressive, with research having addressed significant aspects of the disease and the devils' responses to it. These include the cause and pathogenesis of DFTD, the immune response of the devils and the immune evasion mechanisms of the tumor, the transmission patterns of DFTD, and the impacts of DFTD on the ecosystem. This review aims to collate this information and put it into the context of conservation strategies designed to mitigate the impacts of DFTD on the devil and the Tasmanian ecosystem.

Retrieval of critically ill adults using extracorporeal membrane oxygenation: an Australian experience.

PURPOSE: A retrieval program was developed in New South Wales (NSW), Australia to provide extracorporeal membrane oxygenation support (ECMO) for the safe transport of adults with severe, acute respiratory or cardiac failure. We describe the development and results of this program and the impact of the 2009 H1N1 epidemic on this service. METHODS: An observational study of all patients who were retrieved on ECMO support in NSW, from March 1, 2007 to June 1, 2010, was carried out. RESULTS: Forty adult patients were retrieved on ECMO support (median age 34 years). The indications for retrieval were respiratory in 38 patients (of whom 16 were confirmed or suspected H1N1 cases) and cardiac in 2 patients. Two other patients died after referral but before ECMO support could be established. Patients were transported by road (n = 26, 65%), medical retrieval jet (n = 10, 25%) and helicopter (n = 4, 10%). The median retrieval distance was 250 km (range 12-1,960 km). Thirty-four patients (85%) survived to hospital discharge. Survival for respiratory indications was 87% (33/38 patients) and 50% (1/2 patients) for cardiac indications. There were no deaths or major morbidity associated with these retrievals. CONCLUSIONS: Patients with very severe respiratory failure, which was considered to preclude conventional ventilation for safe transfer to tertiary centres, were managed by an ECMO referral and retrieval program in NSW and had a high rate of survival. This program also enhanced the capacity of the state to respond to a surge in demand for ECMO support due to the H1N1 epidemic, although the role of ECMO in respiratory failure is not yet well defined.

Terahertz pulsed imaging of basal cell carcinoma ex vivo and in vivo.

BACKGROUND: Terahertz radiation lies between the infrared and microwave regions of the electromagnetic spectrum and can be used to excite large amplitude vibrational modes of molecules and probe the weak interactions between them. Terahertz pulsed imaging (TPI) is a noninvasive imaging technique that utilises this radiation. OBJECTIVES: To determine whether TPI could differentiate between basal cell carcinoma (BCC) and normal tissue and to test whether it can help facilitate delineation of tumour margins prior to surgery. METHODS: A portable TPI system was used in the clinic to image 18 BCCs ex vivo and five in vivo. RESULTS: The diseased tissue showed a change in terahertz properties compared with normal tissue, manifested through a broadening of the reflected terahertz pulse. Regions of disease identified in the terahertz image correlated well with histology. CONCLUSIONS: This study has confirmed the potential of TPI to identify the extent of BCC in vivo and to delineate tumour margins. Further clinical study of TPI as a surgical tool is now required.

Comparison of costs and referral rates of 3 universal newborn hearing screening protocols.

OBJECTIVE: To investigate the costs and referral rates of 3 universal newborn hearing screening programs: transient evoked otoacoustic emissions (TEOAE), automated auditory brainstem response (AABR), and a combination, two-step protocol in which TEOAE and AABR are used. STUDY DESIGN: Clinical outcomes (referral rates) from 12,081 newborns at 5 sites were obtained by retrospective analysis. Prospective activity-based costing techniques (n = 1056) in conjunction with cost assumptions were used to analyze the costs based on an assumed annual birth rate of 1500 births. RESULTS: Referral rates differed significantly among the 3 screening protocols (AABR, 3.21%; two-step, 4.67%; TEOAE, 6.49%; P <.01), with AABR achieving the best referral rate at discharge. Although AABR had the lowest referral rate at discharge and the highest pre-discharge costs, the total pre- and post-discharge costs per infant screened (AABR, $32.81; two-step, $33.05; TEOAE, $28.69) and costs per identified child (AABR, $16,405; two-step, $16,527; TEOAE, $14,347) were similar among programs. CONCLUSION: Although AABR incurs higher costs during pre-discharge screening, it has lower referral rates than either the TEOAE or two-step program. As a result, the total costs of newborn hearing screening and diagnosis are similar among the 3 methods studied.

External chest compression in the management of acute severe asthma--a technique in search of evidence.

Compelling anecdotal evidence exists for the potentially lifesaving benefits of mechanical external chest compression (MECC), but no published trials of the technique exist. The history and technique for MECC are discussed and illustrated by a case report. Although the technique is not discussed in the Resuscitation Guideline 2000, and the need for it within the intensive care unit has reduced, the use of MECC will have its greatest impact when initiated in the prehospital setting for patients suffering from severe, sudden-onset, asphyxic asthma.

Humanized anti-CD4 monoclonal antibody therapy of autoimmune and inflammatory disease.

We have investigated the biological and therapeutic properties of a humanized anti-CD4 MoAb, hIgG1-CD4, in patients with refractory psoriasis and rheumatoid arthritis (RA). hIgG1-CD4 is a modulating, non-depleting MoAb, which induced a first-dose reaction in most patients treated. It provided brief symptomatic relief in both conditions, and psoriasis appeared easier to control with conventional agents after MoAb therapy. At the doses used, hIgG1-CD4 did not synergize therapeutically with the panlymphocyte MoAb CAMPATH-1H (C1H) in patients with RA treated sequentially with both agents. There were no serious adverse effects definitely attributable to therapy. Our results are compared with those of other CD4 MoAb studies, and factors influencing the outcome of therapy are discussed.

Treatment of extramammary Paget's disease by radiotherapy.

Extramammary Paget's disease (EMPD) is a rare cutaneous malignancy, which usually occurs in the elderly. Wide local excision is the recommended treatment, although this may not always be feasible. We report our experience of EMPD treated by radiotherapy in five patients. The radiotherapy was well tolerated in each case, and there were no signs of recurrence during follow-up (6 months-8 years). This study shows that radiotherapy is a useful alternative therapy for EMPD, and should be considered particularly in elderly patients who may not tolerate surgery.

Mid-dermal elastolysis.

We report a case of mid-dermal elastolysis in which dermal inflammation was a mild but definite feature. The aetiology of this condition remains unknown, although inflammatory destruction of elastic fibres appears to be the final result. In this study, areas of inflammation exhibited features of a specific immune response, with induction of a number of immune accessory molecules.

Auricular embryonal rhabdomyosarcoma.

Rhabdomyosarcoma is an uncommon tumour that may present at a wide variety of different sites. We report a 4-year-old girl who developed an embryonal rhabdomyosarcoma arising in the left pinna which was clinically indistinguishable from a lymphangioma. The case illustrates that this neoplasm can be easily misdiagnosed because of its variable morphology. Early recognition is important as successful treatment is now possible with a combination of chemotherapy, surgery and/or radiotherapy.

Scleroderma and the carcinoid syndrome.

The follow up of a case of the carcinoid syndrome complicated by scleroderma is reported, in which progress of the disease may have been halted by treatment with a combination of cyproheptadine, parachlorophenylalanine and prednisolone. Impairment of tryptophan and 5-hydroxytryptamine (serotonin) metabolism appears central to the development of skin fibrosis in the carcinoid syndrome and may be indicative of important mechanisms in the pathogenesis of idiopathic scleroderma.

Multiple primary cutaneous plasmacytomas.

BACKGROUND: Cutaneous plasmacytoma is an uncommon tumor and is mostly seen in the context of end-stage multiple myeloma. Only 20 cases of primary cutaneous plasmacytoma have been documented. A significant proportion of these patients went on to develop systemic disease with a poor prognosis. In a number of patients, however, the abnormal clone of plasma cells may arise in the skin and never progress to multiple myeloma involving the bone marrow. OBSERVATIONS: We describe a patient who developed multiple primary cutaneous plasmacytomas after a possible insect bite reaction. The monoclonality of the tumor cells is demonstrated using immunohistochemical techniques. He has been treated vigorously with chemotherapy and local radiotherapy and remains well 3 years after diagnosis. Bone marrow has been harvested for use as an autologous bone marrow transplant in the event of systemic relapse. CONCLUSIONS: Unlike previous reports of this rare entity, this case documents the monoclonality of tissue plasma cells with immunohistochemical techniques. As cutaneous plasmacytomas have been reported with an early significant mortality, unlike extramedullary plasmacytomas elsewhere, we have advocated combination chemotherapy and cryopreservation of uninvolved bone marrow for future autologous bone marrow transplantation should systemic myelomatosis develop in the patient.

Recognition of two distinct major antigens by bullous pemphigoid sera.

Sera from 17 patients with bullous pemphigoid identified a range of polypeptides of relative molecular mass (Mr) 240,000, 230,000, 190,000, 180,000, 120,000 and 100,000 from extracts of SCaBER cells, cultured human keratinocytes or human epidermis, using an immunoblotting technique. The pattern of polypeptides was characteristic for the patient serum and individual sera identified similar polypeptides from all three substrates. All 17 sera recognized major polypeptides of either Mr 230,000 (11 sera) or Mr 180,000 (seven sera) under the denaturing conditions used for immunoblotting studies. Sera from 12 patients were also examined using an immunoprecipitation technique. Polypeptide(s) of Mr 230,000 were immunoprecipitated from extracts of SCaBER cells by 11 of these sera, despite immunoblotting patterns of Mr 180,000 (or less) for three of the 11 sera. None of the minor polypeptides recognized in immunoblotting studies were immunoprecipitated by these sera. Localization of antigens was determined by binding of sera to intact or permeabilized SCaBER cells in an ELISA. Sera which recognized the Mr 230,000 polypeptide under denaturing conditions also identified an intracellular epitope in SCaBER cells, while sera which identified the denatured Mr 180,000 polypeptide bound to a cell surface epitope. Two distinct major antigens are recognized by bullous pemphigoid sera. These both appear as molecules of Mr 230,000 under non-denaturing conditions, but only one of the molecules is dissociated to produce a Mr 180,000 polypeptide under denaturing conditions. Epitopes on these two major antigens are localized on either side of the cell membrane.

Keratinocyte culture.

The in vitro growth of keratinocytes has proved to be an important tool in the study of the normal biology and disease processes involving the skin, e.g., the influence of extrinsic regulators of growth and differentiation, effects of pharmacological agents, dermo-epidermal interactions, tissue antigenicity, and models of carcinogenesis.