term benefit. Clinical vigilance, prompt multimodal diagnosis and multidisciplinary management are paramount for the treatment of immune-mediated myocarditis.
Infection with Mycobacterium avium, M. intracellulare and M. scrofulaceum (MAIS) organisms in normal children may result in cervical lymphadenopathy. There is a poor response to anti-tuberculous therapy and surgical excision of infected nodes is necessary. The diagnosis therefore requires consideration in children with cervical lymphadenopathy. A simple in vitro assay is described which may be useful for diagnosis prior to excision. Whole blood is cultured with M. avium purified protein derivative. After 24 h the plasma is removed and the concentration of gamma interferon (IFN-gamma) quantified by a radioimmunometric assay. In a prospective study of 38 children with neck lesions, 16 showed MAIS organisms isolated from cultures of excised tissue. The level of IFN-gamma produced by peripheral blood lymphocytes from children in the MAIS group was significantly greater when compared with children having operations for neck lesions that were not due to MAIS organisms (P < 0.001).
At the time of Indonesian withdrawal in 1999, there were only twenty general doctors for a population of seven hundred thousand (2). By comparison, in Australia at the same time there were 1,787 doctors per seven hundred thousand people (3). In Timor-Leste today, the density of skilled health professionals -1.3 per 1000 population -remains well below the World Health Organisation's recommended lower threshold of 2.3 per 1000 population, and far below Australia's current density of 16.2 health-workers per 1000 population (4, 5). The majority of specialist medical care over the last two decades in East Timor has been provided by outreach medical services from Australian physicians and surgeons (6).The East Timor Hearts Fund (ETHF) commenced work in Timor-Leste from 2002, and was formally created as a charitable foundation in 2010. Specialist cardiologists volunteer in Timor-Leste and perform clinical and echocardiographic assessment . Patients identified as suitable candidates are referred for cardiac intervention (surgical or percutaneous) in Australia, with costs paid for by charitable donations (7). Through ETHF's efforts, Timor-Leste has been identified to have one of the highest rates of rheumatic heart disease in the world; approximately 2% prevalence identified on aThis article is protected by copyright. All rights reserved. screening study of school children in 2016 (8). Two key goals of the ETHF are to provide high-quality specialist cardiac care to Timor-Leste's citizens and to develop in-country resources and training in cardiology, with the ultimate goal that Timor-Leste does not require the services of Australian physicians for the provision of cardiac care. We undertook a review of one decade of clinical activity in order to assess demographics and patterns of cardiac disease presenting in Timor-Leste, quantify our clinical coverage, and assess evolving trends.This study is intended to 'take the pulse' of Timor-Leste's cardiac needs by highlighting areas of evolution to facilitate healthcare planning for the future decade. We anticipate that our experience will be of interest to physicians working in a range of developing countries with limited healthcare resources. Methods:Australian cardiologists travel to East Timor with the ETHF three times per year during the non-rainy season (7). The medical team comprises up to four adult cardiologists and ideally at least one paediatric cardiologist and echocardiographer . Additional medical volunteers may include a general practitioner or cardiac nurses, who assess vital signs and perform six-minute walk tests. Two Timorese interpreters are present at all clinics: one at the reception desk and one moving between clinical rooms. Clinics are primarily based at the national hospital in Dili (the Hospital Nacional Guido Valadares (HNGV) with one outreach day to a different rural area each trip.
thromboembolism (VTE) in SCD, with a higher mortality observed in patients with VTE compared to those without this complication [5].There are few studies specifically addressing treatment of PHT in SCD. Current strategies include optimizing SCD therapy and identifying potentially modifiable etiologies such as obstructive sleep apnea, pulmonary thrombosis, and left ventricular dysfunction. Hydroxyurea, approved for treatment of severe anemia and frequent vaso-occlusive episodes, has the potential benefit of being an NO donor. Endothelin receptor antagonists and prostaglandin therapy are approved for treatment of pulmonary arterial hypertension and have been recommended for select SCD patients with marked elevation of pulmonary vascular resistance and normal pulmonary artery occlusion pressure on RHC, and related symptoms [1]. Phosphodiesterase-5 inhibitor therapy is presently not recommended as first-line treatment for RHC-confirmed PHT due to the association of sildenafil with increased hospitalizations for pain crises. Riociguat, a soluble guanylate cyclase stimulator, is a vasodilator that bypasses the NO pathway. It has shown promise in CTEPH and PAH, but has not yet been studied in SCD. Although a study of the low molecular weight heparin, tinzaparin showed a reduction in the duration of pain crisis and hospitalization [6], most studies of anticoagulants in SCD have been small and poorly controlled. Treatment with the factor Xa inhibitor, rivaroxaban, as well as PAR-2 or IL-6 deficiency have been reported to attenuate PHT in animal models [7]. A study of rivaroxaban in SCD is ongoing to assess its pharmacodynamics effects (www.clinical trials.gov. identifier NCT02072668). Finally, pulmonary endarterectomy is standard for non-SCD patients with CTEPH, but the experience in SCD is limited, possibly due to concerns for a high risk of perioperative complications. Pulmonary endarterectomy requires cardiopulmonary bypass, hypothermia, and periods of circulatory arrest, which will likely increase the risk of sickling. These two cases, along with the previously published reports of four patients who underwent pulmonary endarterectomy [8][9][10], demonstrate that patients can undergo this procedure safely, with symptomatic and hemodynamic benefit. In summary, SCD patients with CTEPH, should be considered for pulmonary endarterectomy. AcknowledgmentAuthors thank Ertan Pamuklar, MD for help with the figure.
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