Congenital asplenia study: clinical and laboratory characterisation of adults with congenital asplenia.

Congenital asplenia is a rare disorder commonly associated with other visceral and cardiac congenital anomalies. Isolated congenital asplenia is even less common than syndromic forms. The risk of severe bacterial infections associated with asplenia is the most concerning clinical implication and carries a significant mortality risk. Prophylactic measures against the clinical syndrome known as overwhelming postsplenectomy infections (OPSI) include vaccination, prophylactic and emergency antibiotics and health education including fever management and travel advice. This case series describes fourteen adults with congenital asplenia and polysplenia syndrome, most of whom were diagnosed incidentally as adults, and outlines the nature of their diagnosis, clinical phenotype, family history and key pathology findings.

Outcomes in Medicare-ineligible people living with human immunodeficiency virus in a large healthcare network in Melbourne 2004-2019.

Australia has approximately 1.6 million Medicare-ineligible residents, of whom around 450 are living with human immunodeficiency virus (PLHIV). We examined the outcomes in a cohort of 50 Medicare-ineligible patients presenting to our hospital network over a 15-year period: 31 women (62%) and 19 men. Twenty-four were newly diagnosed. Sixteen of 24 remained in Australia more than 1 year after diagnosis. Although the mean CD4 count at initial contact was 353 cells/mm3 (range 3-2228; standard deviation (SD) = 452.88), 13 people required treatment for opportunistic infections and 21 people required hospital admissions related to HIV, incurring total estimated hospital costs of $886 310. The mean number of contact years spent with the service was 2.2 (range 0-12; SD = 2.6) and 20 people remain under care. Twenty-seven PLHIV remain in Australia, seven have transferred care within Australia, two people are known to have died and eight are lost to follow up. The median number of admissions was 0 (range 0-4; SD = 1) and median length of admission was 5 days (range 0-73; SD = 19). Many people leave Australia shortly after a diagnosis of HIV, but most Medicare-ineligible PLHIV remain. Delays in diagnosing HIV and acquiring Medicare status are associated with a significant burden of disease and cost. Keeping people well, on therapy and out of hospital is likely to be cost-saving in addition to good clinical practice.

Platelet phenotype and function in the absence of splenic sequestration (Review).

The spleen, in addition to its role in immunity, plays key roles in erythrocyte maintenance and platelet sequestration. Loss of the spleen via splenectomy occurs in approximately 6.4 to 7.1 per 100 000 people per year globally, commonly as a life-saving emergency procedure in trauma and a therapeutic procedure in hematological and hematological malignant conditions. It is associated with increased risk of life-threatening infection and thromboembolism, presumably via loss of splenic function, but the underlying mechanisms behind post-splenectomy thromboembolism are unclear. The splenectomized individual has a two-fold risk of thromboembolism as compared to non-splenectomized individuals and the risk of thromboembolism is elevated both post-operatively and in the longer term. Although those splenectomized for hematological conditions or hematological malignant conditions are at highest risk for thromboembolism, an increase in thromboembolic outcomes is also observed amongst individuals splenectomized for trauma, suggesting underlying disease state is only a partial factor. Although the physiological role of the splenic platelet pool on platelets is unclear, platelet changes after splenectomy suggest that the spleen may play a role in maintaining platelet quality and function. In hypersplenic conditions, sequestration can increase to sequester up to 72% of the total platelet mass. Following splenectomy, a thrombocytosis is commonly seen secondary to the loss of the ability to sequester platelets. Abnormal platelet quality and function have been observed as a consequence of splenectomy. These platelet defects seen after splenectomy may likely contribute to the increase in post-splenectomy thromboembolism. Here we draw upon the literature to characterize the post-splenectomy platelet and its potential role in post-splenectomy thromboembolism.

Trends in practice: attitudes and challenges in the diagnosis, treatment and management of HIV infection in Australia.

As life expectancy for people living with human immunodeficiency virus (HIV) (PLWHIV) increases, management models for HIV infection are changing. To understand approaches to practice within this shifting climate and across different medical settings, in 2017 we conducted a baseline survey among the main medical practitioner groups responsible for HIV-infection care in Australia: hospital-based physicians (HBP), sexual health physicians (SHP) and 'accredited general practitioners' (referred to in 2017 study as 's100 GPs'), who are GPs authorised to prescribe HIV therapies after completing accredited national training. The follow-up survey presented here explores any changes in approaches, attitudes and challenges associated with HIV-infection management among the same practitioner groups: 17 HBP, 15 SHP and 69 accredited GP (referred to throughout as GP; includes those with sexual health diploma). Analysis of survey results showed practices remained largely similar between surveys, with a few notable exceptions. Greater consistency in attitudes, knowledge and approaches was observed between the practitioner specialty groups, with only small differences between modes of practice. A trend towards earlier initiation of HIV treatment was also identified, with a higher proportion of practitioners than baseline reporting they were comfortable beginning therapy on the day of HIV diagnosis. The impact of the introduction of two-drug therapy in Australia was also explored. Although the majority of survey respondents (and SHP in particular) expressed greater preference for three-drug compared with two-drug regimens, interest in two-drug regimens appears to be growing and may influence future prescribing practices. Addressing mental health issues for PLWHIV was again highlighted as a major priority, with practitioners overwhelmingly reporting mental health management as among their most difficult clinical challenges. Reduction in stigma/discrimination and better access to substance dependency programmes were also identified as unmet needs for this patient cohort. Consistent with our baseline survey, it appears targeted interventions and supports appropriate to this population are still required to improve overall wellbeing for PLWHIV.

Burden of infective endocarditis in an Australian cohort of people who inject drugs.

BACKGROUND: Infective endocarditis (IE) results in substantial morbidity and mortality in people who inject drugs (PWID). AIMS: To describe the burden of IE and its outcomes in PWID. METHODS: Retrospective cohort study of adults admitted to a tertiary referral centre in Melbourne, Australia, with IE due to injection drug use from 1997 to 2015. RESULTS: Ninety-seven PWID with 127 episodes of IE were identified with a median acute inpatient stay of 37 days (1-84). Admission to an intensive care unit was required in 67/127 (53%) episodes. Twenty-seven percent (34/127) of episodes occurred in patients with a previous episode of endocarditis. One third (43/127, 34%) of episodes involved left-sided cardiac valves. Antimicrobial treatment was completed in 88 (70%) episodes. Valve surgery was performed in 25/127 (20%) episodes. Predictors of surgery in univariable analysis were left-sided cardiac involvement (risk ratio (RR) 6.0), severe valvular regurgitation (RR 2.6) and cardiac failure (RR 2.2) (all P < 0.005). Twenty (16%) episodes resulted in death. Predictors of mortality on univariable analysis were left-sided cardiac involvement (RR 6.4), and not completing treatment (RR 0.12; both P < 0.001). The average estimated cost per episode was AU$74 168. CONCLUSIONS: IE causes a considerable burden of disease in PWID, with significant healthcare utilisation and cost. Surgery and death are not infrequent complications. In addition to ensuring completion of antimicrobial therapy, strategies such as opioid maintenance programmes may be useful in improving health outcomes for PWID.

Post-splenectomy sepsis: preventative strategies, challenges, and solutions.

Removal of the spleen had already been established as a routine technique to treat splenic trauma and other diseases affecting the spleen before the anatomy, physiology, and function of the spleen were known in the mid-twentieth century. It is now widely accepted that the splenectomized individual is at increased risk for infection, in particular, overwhelming post-splenectomy infection (OPSI). OPSI is a syndrome of fulminant sepsis occurring in splenectomized (asplenic) or hyposplenic individuals that is associated with high mortality and morbidity. Poorly opsonized bacteria such as encapsulated bacteria, in particular, Streptococcus pneumoniae, are often implicated in sepsis. The spleen is a reticuloendothelial organ that facilitates opsonization and phagocytosis of pathogens, in addition to cellular maintenance. Splenectomy is associated with an impairment in immunoglobulin production, antibody-mediated clearance, and phagocytosis, leading to an increased risk of infection and sepsis. Early identification of the at-risk patient, early blood cultures prior to antibiotic administration, urgent blood smears and fast pathogen-detection tests, and sepsis bundles should be utilized in these patients. Prompt management and aggressive treatment can alter the course of disease in the at-risk splenectomized patient. Overwhelming post-splenectomy infection can be prevented through vaccination, chemoprophylaxis, and patient education. This article evaluates post-splenectomy sepsis by summarizing the anatomy and function of the spleen, physiological changes after splenectomy that predispose the splenectomized patient to infection, and current management and prevention strategies.

Healthy, safe and effective international medical student electives: a systematic review and recommendations for program coordinators.

BACKGROUND: Thousands of medical students undertake international medical electives each year. These students face potentially substantial health and safety risks as well as educational and ethical challenges and therefore should undertake their electives within well-structured and coordinated programs. METHODS: We conducted a qualitative systematic review based on a pre-determined protocol. Relevant publications and guidelines relating to international medical electives were identified through a review of the literature using on-line search engines, principally PubMed. We then conducted a systematic qualitative synthesis to extract relevant publications. Finally, the literature was organized according to themes, with the aim of developing a structured set of Recommendations for Implementation for program coordinators. RESULTS: A wide range of important issues were identified which were categorized into seven themes upon which recommendations were made principally for the benefit of program coordinators: Responsibilities; General policies; Travel advisories; Occupational risk assessment; Funding & finances, Pre-departure training programs; and Post-return debriefing and screening. CONCLUSIONS: Recommendations for program coordinators on the health and safety of medical students while on international medical electives have been sourced from existing guidelines and relevant publications. There was considerable consensus from the literature and as such these synthesised recommendations could form the basis for internationally accepted standards for elective placement program coordinators.

Impact of a spleen registry on optimal post-splenectomy vaccination and care.

Objective: To evaluate quality of patient knowledge and rates of adherence to guidelines amongst splenectomised patients registered to the Spleen Australia registry. Method: Registrants recruited for assessment of residual splenic function post-splenectomy also underwent an assessment of quality of knowledge and a review of their long-term management. Eligible patients were ≥ 18 years of age, registered to the Spleen Australia clinical registry and had been splenectomised at least 1 year prior to their visit. Quality of knowledge was assessed using a validated questionnaire used in similar studies. Receipt of immunisations was validated by record review. Chemoprophylaxis use was self-reported by patients. Adherence was evaluated using Australian guidelines. Results: 77 patients were evaluated for education and adherence. 58% were female, mean age was 58 years, and median duration since splenectomy was 14 years. Most common indications for splenectomy were trauma and haematological conditions. 77% had good knowledge of key educational points to reduce chances of infection. Adherence to immunisations varied with poor adherence to vaccines introduced after 2010. Only 6 patients were adherent to all recommended immunisations. Increasing duration since registration was associated with poorer 13vPCV (p = 0.008) and 4vMenCV adherence (p = 0.001). Over 70% either currently or had previously used daily chemoprophylaxis and 66% had a supply of emergency antibiotics. Conclusions: Although registrants are receiving initial and booster vaccinations, they do not receive newly recommended vaccines. In order to maintain long-term adherence, we recommend streamlining health information systems, improving awareness strategies and improving financial access to vaccinations in the community with additional awareness of the activities of the registry.

Adherence to guidelines for prevention of postsplenectomy sepsis. Age and sex are risk factors: a five-year retrospective review.

BACKGROUND: Vaccination, education and use of long-term antibiotics are recommended in expert guidelines for the prevention of infectious complications after splenectomy. However, studies outside Australia have shown poor adherence to the guidelines. METHOD: The aim of this study was to determine overall adherence to the guidelines and to ascertain any independent risk factors for poor compliance with the guidelines. A retrospective review of hospital records between 1999 and 2004 was carried out. RESULTS: Indications for splenectomy of the 111 patients in this review included post-trauma (32), haematological (32), cancer surgery (24), iatrogenic (12) and others (11). On multivariable analysis, age was associated with a 28% less likelihood to receive education (odds ratio (OR) 0.72; 95% confidence interval (CI) 0.56-0.92; P = 0.009) and 36% less likelihood to receive long-term antibiotics (OR 0.64; 95% CI 0.52-0.80; P < or = 0.001). Women were four times more likely to receive education (OR 4.03; 95% CI 1.16-14.0; P = 0.028) and patients who had undergone splenectomy in 2004 were 22 times more likely to have received education compared with those in 1999 (OR 22.53; 95% CI 3.12-162.34; P = 0.002). CONCLUSION: Education for prevention of sepsis after splenectomy is poorly documented and may be incomplete. Older age and male sex are risk factors in non-adherence to guidelines for prevention of postsplenectomy sepsis. Strategies such as alert cards and information brochures may improve adherence to guidelines particularly in older patients.

Cryptococcemia in primary HIV infection.

Opportunistic infections have been reported infrequently in primary HIV infection. We report a case of cryptococcemia in primary HIV infection. To our knowledge there has not been such a case reported. Our case highlights the need for clinicians to be wary of other opportunistic infections, including cryptococcosis, in primary HIV infection.

Disseminated mycobacterium avium-intracellulare complex (MAC) infection in the era of effective antiretroviral therapy: is prophylaxis still indicated?

Before highly active antiretroviral therapies (HAART) were available for the treatment of persons with HIV infection, disseminated Mycobacterium avium-intracellulare complex (MAC) infection was one of the most common opportunistic infections that affected people living with AIDS. Routine use of chemoprophylaxis with a macrolide has been advocated in guidelines for the treatment of HIV-infected individuals if they have a circulating CD4+ cell count of < or =50 cells/microL. In addition, lifelong prophylaxis for disease recurrence has been recommended for those with a history of disseminated MAC infection. The introduction of HAART has resulted in a remarkable decline in the incidence of opportunistic infections and death among persons living with AIDS. Considerable reconstitution of functional immune responses against opportunistic infections can be achieved with HAART. In the case of infection with MAC, there has been a substantial reduction in the incidence of disseminated infections in the HAART era, even in countries where the use of MAC prophylaxis was never widely accepted. Moreover, the clinical picture of MAC infections in patients treated with potent antiretroviral therapies has shifted from a disseminated disease with bacteraemia to a localised infection, presenting most often with lymphadenopathy and osteomyelitis. Data from several recently conducted randomised, double-blind, placebo-controlled trials led to the current practice of discontinuing primary and secondary prophylaxis against disseminated MAC infections at stable CD4+ cell counts >100 cells/microL. These recommendations are still conservative as primary or secondary disseminated MAC infections are only rarely seen in patients who respond to HAART, despite treatment initiation at very low CD4+ cell counts. Potential adverse effects of macrolide therapy and drug interactions with antiretrovirals also metabolised via the cytochrome P450 enzyme system must be critically weighed against the marginal benefit that MAC prophylaxis may provide in addition to treatment with HAART. These authors feel that, unless patients who initiate HAART at low CD4+ cell counts do not respond to HIV-treatment, routine MAC prophylaxis should not be recommended. Nevertheless, the patient population for whom MAC prophylaxis may still be indicated in the era of HAART needs to be identified in prospectively designed clinical trials.

Clostridium septicum bacteremia without gas gangrene as a late consequence of a contaminated wound.

BACKGROUND: Clostridium septicum is an unusual human pathogen associated with colorectal malignancy and gas gangrene. METHODS: A case compilation and literature review are presented. RESULTS: We report the case of an individual with a comminuted tibial fracture complicated by a superficial surgical site infection with C. septicum nine weeks after the original injury and internal fixation, which was complicated by a secondary bacteremia. CONCLUSIONS: This is a unique case in the literature, but it is suggested that the use of cephalosporins as prophylaxis for contaminated wounds may be inferior to penicillins to prevent clostridial infections.

Fragility fractures in HIV-infected patients: need for better understanding of diagnosis and management.

HIV infection, AIDS, and antiretroviral therapy (ART) have been associated with bone fragility fractures, although the prevalence and incidence are not well studied by researchers. In HIV and ART, osteopenia and osteoporosis are multifactorial, and health promotion or medical health maintenance should anticipate and prevent morbidity of bone fragility fractures.