Variability of disease activity in patients with hereditary angioedema type 1/2: longitudinal data from the Icatibant Outcome Survey.

BACKGROUND: Hereditary angioedema due to C1 inhibitor deficiency (HAE-1/2) is a chronic and debilitating disease. The unpredictable clinical course represents a significant patient burden. OBJECTIVE: To analyse longitudinal registry data from the Icatibant Outcome Survey (IOS) in order to characterize temporal changes in disease activity in patients with HAE-1/2. METHODS: Icatibant Outcome Survey (NCT01034969) is an international observational registry monitoring the clinical outcomes of patients eligible for icatibant treatment. The current analyses are based on data collected between July 2009 and July 2019. Retrospective data for attacks recorded in the 12 months prior to IOS enrolment and for each 12-month period up to 7 years were analysed. RESULTS: Included patients reported angioedema attacks without long-term prophylaxis (LTP; n = 315) and with LTP (n = 292) use at the time of attack onset. Androgens were the most frequently used LTP option (80.8%). At the population level, regardless of LTP use, most patients (52-80%) reporting <5 attacks in Year 1 continued experiencing this rate; similarly, many patients (25-76%) who reported high attack frequency continued reporting ≥10 attacks/year. However, year on year, 31-51% of patients experienced notable changes (increase/decrease of ≥5 attacks) in annual attack frequency. Of patients who reported an absolute change of ≥10 attacks from Year 1 to 2, 17-50% continued to experience a change of this magnitude in subsequent years. CONCLUSION: At the population level, attack frequency was generally consistent over 7 years. At the small group level, 28.8-34.5% of patients reported a change in attack frequency of ≥5 attacks from Year 1 to Year 2; up to half of these patients continued to experience this magnitude of variation in disease activity in later years, reflecting high intra-patient variability.

Validating accelerometry-derived proxies of energy expenditure using the doubly labelled water method in the smallest penguin species.

Understanding energy use is central to understanding an animal's physiological and behavioural ecology. However, directly measuring energy expenditure in free-ranging animals is inherently difficult. The doubly labelled water (DLW) method is widely used to investigate energy expenditure in a range of taxa. Although reliable, DLW data collection and analysis is both financially costly and time consuming. Dynamic body acceleration (e.g. VeDBA) calculated from animal-borne accelerometers has been used to determine behavioural patterns, and is increasingly being used as a proxy for energy expenditure. Still its performance as a proxy for energy expenditure in free-ranging animals is not well established and requires validation against established methods. In the present study, the relationship between VeDBA and the at-sea metabolic rate calculated from DLW was investigated in little penguins (Eudyptula minor) using three approaches. Both in a simple correlation and activity-specific approaches were shown to be good predictors of at-sea metabolic rate. The third approach using activity-specific energy expenditure values obtained from literature did not accurately calculate the energy expended by individuals. However, all three approaches were significantly strengthened by the addition of mean horizontal travel speed. These results provide validation for the use of accelerometry as a proxy for energy expenditure and show how energy expenditure may be influenced by both individual behaviour and environmental conditions.

Addressing negative psychosocial factors linked to severe injury in professional rugby players: An introduction to a group psychotherapy approach.

Background: Negative psychosocial sequelae of severe rugby injury (SRI) in professional rugby players are well documented. Unaddressed, these issues can leave players vulnerable to persistent common mental disorders (CMD) and negatively affect injury recovery processes. Objective: To introduce a psychotherapeutic group intervention aimed at addressing negative psychosocial sequelae linked to SRI in professional rugby player cohorts. Methods: Literature aimed at clarifying the potential efficacy of an integrative group therapy model, the Recovery Mastery Group (RMG), is discussed after which component parts of the intervention are presented. Case illustration: A case illustration is presented comprising examples of how the RMG framework addressed psychosocial recovery issues in a professional South African rugby team during 2019. Conclusion: The proposed Recovery Mastery Group (RMG) is presented as a cost- and time- effective psychotherapeutic intervention that integrates well-researched psychotherapeutic techniques. The RMG appears able to address multiple facets of psychosocial injury recovery, while possibly offering protection from the onset of CMD. This introduction to the RMG can be a forerunner of similar research across larger cohorts, in different team sports, to determine wider therapeutic intervention efficacy.

Dive behaviour and foraging effort of female Cape fur seals Arctocephalus pusillus pusillus.

While marine top predators can play a critical role in ecosystem structure and dynamics through their effects on prey populations, how the predators function in this role is often not well understood. In the Benguela region of southern Africa, the Cape fur seal (Arctocephalus pusillus pusillus) population constitutes the largest marine top predator biomass, but little is known of its foraging ecology other than its diet and some preliminary dive records. Dive information was obtained from 32 adult females instrumented with dive recorders at the Kleinsee colony (29°34.17' S, 16°59.80' E) in South Africa during 2006-2008. Most dives were in the depth range of epipelagic prey species (less than 50 m deep) and at night, reflecting the reliance of Cape fur seals on small, vertically migrating, schooling prey. However, most females also performed benthic dives, and benthic diving was prevalent in some individuals. Benthic diving was significantly associated with the frequency with which females exceeded their aerobic dive limit. The greater putative costs of benthic diving highlight the potential detrimental effects to Cape fur seals of well-documented changes in the availability of epipelagic prey species in the Benguela.

Increasing deceased organ donor numbers in Johannesburg, South Africa: 18-month results of the Wits Transplant Procurement Model.

In 2016, deceased-donor organ procurement at Wits Transplant, based at Wits Donald Gordon Medical Centre in Johannesburg, South Africa (SA), was in a state of crisis. As it is the largest-volume solid-organ transplant unit in SA, and as we aspire to provide transplant services of an international standard, the time to address our procurement practice had come. The number of deceased donors consented through our centre was very low, and we needed a radical change to improve our performance. This article describes the Wits Transplant Procurement Model - the result of our work to improve procurement at our centre. The model has two core phases, one to increase referrals and the other to improve our consent rates. Within these phases there are several initiatives. To improve referrals, the threefold approach of procurement management, acknowledgement and resource utilisation was developed. In order to 'convert' referrals into consents, we established the Wits Transplant 'Family Approach to Consent for Transplant Strategy' (FACTS). Since initiation of the Wits Transplant Procurement Model, both our referral numbers from targeted hospitals and our conversion rates have increased. Referrals from targeted hospitals increased by 54% (from 31 to 57). Our consent rate increased from 25% (n=6) to 73% (n=35) after the initiation of Wits Transplant FACTS. We hope that other transplant centres in SA and further afield in the region will find this article helpful, and to this end we have created a handbook on the Wits Transplant Procurement Model that is freely available for download (http://www.dgmc.co.za/docs/Wits-Transplant-Procurement-Handbook.pdf).

Adult liver transplant for hepatocellular carcinoma at Wits Donald Gordon Medical Centre in Johannesburg, South Africa.

BACKGROUND: For those with unresectable hepatocellular carcinoma, liver transplantation is considered the treatment of choice. Since 2006, the transplant programme at Wits Donald Gordon Medical Centre (WDGMC) has offered liver transplantation for selected patients with hepatocellular carcinoma. While the number of patients transplanted was small, we are unaware of any published data from Southern Africa describing outcomes in this group of liver transplant recipients. The aim of this study was to describe our experience as a case series. METHODS: The records of all patients with HCC who underwent deceased donor liver transplantation between April 2006 and March 2018 were reviewed retrospectively. Data were extracted from transplant clinic patient files, histopathology and pathology laboratory reports and an existing database of all liver transplant recipients at WDGMC. Patient survival was calculated from the time of transplant and survival estimates were determined by the Kaplan-Meier method. RESULTS: Thirty-one liver transplants were reviewed. The most common causes of underlying liver disease were infectious, mostly hepatitis B virus, and diseases of lifestyle including alcoholic/non-alcoholic steatohepatitis. Median age at transplant, 57 years (IQR 44-65 years), was younger than observed internationally, but consistent with reports from Africa. Male recipients predominated, in keeping with published trends. Overall, outcomes were worse than expected but for recipients who were within the University of California at San Francisco (UCSF) criteria for transplantation; survival was comparable to previously published data. CONCLUSION: Despite limitations, this is the first documented series of patients undergoing liver transplantation for HCC in South Africa and demonstrates that good results can be achieved in appropriately selected patients.

Living donor liver transplantation in South Africa: the donor experience.

BACKGROUND: Living donor liver transplantation (LDLT) plays a crucial role in liver transplant programmes, particularly in regions with a scarcity of deceased donor organs and especially for paediatric recipients. LDLT is a complex and demanding procedure which places a healthy living donor in harm's way. Donor safety is therefore the overriding concern. This study aimed to report our standardised approach to the evaluation, technical aspects and outcomes of LDLT donor hepatectomy at Wits Donald Gordon Medical Centre. METHOD: The study population consisted of all patients undergoing LDLT donor hepatectomy since the inception of the programme in March 2013 until 2018. Sixty five living donor hepatectomies were performed. Primary outcome measures included donor demographics, operative time, peak bilirubin, aspartate and alanine transaminase levels postoperatively, length of hospital stay and postoperative complications using the Clavien-Dindo classification. RESULTS: The majority of the donors were female, most were parents with mothers being the donor almost 85% of the time. The median operative time was 374 minutes with a downward trend over time as experience was gained. The median length of hospital stay was 7 days. There was no mortality and the complication rate was 30% with the majority being minor (Grade 1). CONCLUSION: Living donor liver transplant from adult-to-child has been successfully initiated in South Africa. Living donor hepatectomy can be safely performed with acceptable outcomes for the donor. Wait-list mortality however remains unacceptably high. Expansion of LDLT as well as real change in deceased donor policy is required to address this issue.

Outcomes of paediatric liver transplant for biliary atresia.

BACKGROUND: Despite the widespread use of Kasai Portoenterostomy (KPE) for biliary atresia, more than two thirds of these patients require liver transplant. Liver transplantation is not widely available in South Africa, and Wits Donald Gordon Medical Centre is one of two centres performing paediatric liver transplantation in the country, and the only centre performing living related donor transplants. METHOD: A retrospective review was performed at the centre. Demographic data were collected, and tabulated. Survival analysis was performed using the Kaplan Meier method. Complication rates were categorised into biliary, vascular and enteric, and classified as early and late. RESULTS: Sixty-seven first time liver transplants were performed for biliary atresia at WDGMC from 2005 to 2017. Sixty-nine percent were female patients and thirty-one percent were male patients. Forty-eight percent of patients under the age of 5 years had a z-score of -2 or worse for mid upper arm circumference (MUAC). One year overall survival of the cohort is 84.5%, and overall graft survival is 82.9%. Overall mortality was 22%, with infection being the most common cause of death. CONCLUSION: Early referral of all patients with biliary atresia to a paediatric liver transplant centre is essential for early assessment of indications, and medical and nutritional optimisation of patients. Primary liver transplant should be considered for a select group of patients with unique clinical indications.

Reimagining liver transplantation in South Africa: A model for justice, equity and capacity building - the Wits Donald Gordon Medical Centre experience.

The challenge of providing effective and integrated liver transplant services across South Africa's two socioeconomically disparate healthcare sectors has been faced by Wits Donald Gordon Medical Centre (WDGMC) since 2004. WDGMC is a private academic hospital in Johannesburg and serves to supplement the specialist and subspecialist medical training provided by the University of the Witwatersrand. Over the past 14 years, our liver transplant programme has evolved from a sometimes fractured service into the largest-volume liver centre in sub-Saharan Africa. The growth of our programme has been the result of a number of innovative strategies tailored to the unique nature of transplant service provision. These include an employment model for doctors, a robust training and research programme, and a collaboration with the Gauteng Department of Health (GDoH) that allows us to provide liver transplantation to state sector patients and promotes equality. We have also encountered numerous challenges, and these continue, especially in our endeavour to make access to liver transplantation equitable but also an economically viable option for our hospital. In this article, we detail the liver transplant model at WDGMC, fully outlining the successes, challenges and innovations that have arisen through considering the provision of transplant services from a different perspective. We focus particularly on the collaboration with the GDoH, which is unique and may serve as a valuable source of information for others wishing to establish similar partnerships, especially as National Health Insurance comes into effect.

Management of patients with hereditary angioedema in Germany: comparison with other countries in the Icatibant Outcome Survey.

BACKGROUND: The Icatibant Outcome Survey (IOS; NCT01034969) is a Shire-sponsored, international, observational study monitoring the safety and effectiveness of icatibant, a bradykinin B2 receptor antagonist approved for the acute treatment of adults with hereditary angioedema with C1 inhibitor deficiency (HAE-C1-INH). OBJECTIVE: To report IOS data comparing demographic and icatibant treatment outcomes in patients with HAE-C1-INH from Germany to HAE-C1-INH patients from 11 other IOS countries. METHODS: A descriptive, retrospective, comparative analysis of data from 685 IOS patients with HAE-C1-INH from seven centres in Germany (n = 93) vs. centres from Austria, Brazil, Czech Republic, Denmark, France, Greece, Israel, Italy, Spain, Sweden and the United Kingdom (n = 592, July 2009-January 2017). Icatibant treatment outcomes were retrieved from patients with complete attack outcome data for time to treatment, time to resolution and attack duration (160 attacks in 42 German patients and 1442 attacks in 251 patients from other IOS countries). RESULTS: German patients reported significantly fewer severe/very severe attacks (38.7% vs. 57.5%, respectively; P < 0.001). The proportion of attacks treated with a single icatibant injection was significantly higher in German patients (97.1% vs. 91.6%, P = 0.0003). The median time to treatment (0.0 h vs. 1.5 h), time to resolution (3.0 h vs. 7.0 h) and attack duration (4.3 h vs. 10.5 h) in German patients vs. other IOS countries were all significantly shorter (all P < 0.0001). No meaningful differences were identified between patients from Germany and other countries with regard to sex, median age at enrolment, median age at symptom onset and median age at diagnosis. CONCLUSION: German IOS patients share similar demographic characteristics to patients from other IOS countries yet treat their attacks with icatibant significantly earlier and have markedly fewer severe or very severe attacks. Factors including regional access to and availability of icatibant may drive these outcomes and warrant further investigation.

Adult liver transplantation in Johannesburg, South Africa (2004 - 2016): Balancing good outcomes, constrained resources and limited donors.

BACKGROUND: Liver transplantation is the standard of care for the treatment of liver failure worldwide, yet millions of people living in sub-Saharan Africa remain without access to these services. South Africa (SA) has two liver transplant centres, one in Cape Town and the other in Johannesburg, where Wits Donald Gordon Medical Centre (WDGMC) started an adult liver transplant programme in 2004. OBJECTIVES:  To describe the outcomes of the adult liver transplant programme at WDGMC. METHODS:  This was a retrospective review of all adult orthotopic liver transplants performed at WDGMC from 16 August 2004 to 30 June 2016 with a minimum follow-up of 6 months. The primary outcome was recipient and graft survival and the effect of covariates on survival. Kaplan-Meier survival analysis included all adults who underwent their first transplant for end-stage liver disease (ESLD) (N=275). Proportional hazards regression analysis using hazard ratios (HRs) was conducted to determine which covariates were associated with a significantly increased risk of mortality. RESULTS:  A total of 297 deceased-donor liver transplants were performed during the study period; 19/297 (6.4%) were for acute liver failure (ALF) and the remainder were for ESLD. The median age of recipients was 51 years (interquartile range 41 - 59), and two-thirds were male. The most common cause of ESLD was primary sclerosing cholangitis. The median follow-up was 3.2 years, and recipient survival was characterised in the following intervals: 90 days = 87.6% (95% confidence interval (CI) 83.1 - 91.0), 1 year = 81.7% (95% CI 76.6 - 85.8), and 5 years = 71.0% (95% CI 64.5 - 76.5). Allograft survival was similar: 90 days = 85.8% (95% CI 81.1 - 89.4), 1 year = 81.0% (95% CI 75.8 - 85.2), and 5 years = 69.1% (95% CI 62.6 - 74.7). The most significant covariates that impacted on mortality were postoperative biliary leaks (HR 2.0 (95% CI 1.05 - 3.80)), recipient age >60 years at time of transplant (HR 2.06 (95% CI 1.06 - 3.99)), theatre time >8  hours (HR 3.13 (95% CI 1.79 - 5.48)), and hepatic artery thrombosis (HR 5.58 (95% CI 3.09 - 10.08)). The most common infectious cause of death was invasive fungal infection. CONCLUSIONS:  This study demonstrates that outcomes of the adult orthotopic liver transplant programme at WDGMC are comparable with international transplant centres. Management of biliary complications, early hepatic artery thrombosis and post-transplant infections needs to be improved. Access to liver transplantation services is still extremely limited, but can be improved by addressing the national shortage of deceased donors and establishing a national regulatory body for solid-organ transplantation in SA.

Surgery for portal hypertension in children: A 12-year review.

BACKGROUND: Portal hypertension is a common and potentially devastating condition in children. Notwithstanding advances in the nonsurgical management of portal hypertension, surgery remains an important treatment modality in select patients. We report here on our experience in the past 12 years. OBJECTIVES: To describe the profile of, indication for, and complications of shunt surgery in children with portal hypertension. METHODS: Twelve children underwent shunt surgery between 2005 and 2017. Patient records were reviewed. RESULTS: Fourteen procedures were performed on 12 patients during the study period. The median age at surgery was 6.5 (range 1 - 18) years. Six patients were male. Gastrointestinal bleeding that was not amenable to endoscopic control was the most common indication for surgery. Portal vein thrombosis was the most common cause of portal hypertension in our series (n=11). Two-thirds (8/12) of all patients had an identifiable underlying risk factor for portal vein thrombosis. One-third of all patients (4/12) underwent a meso-portal bypass procedure (Rex shunt), while 58% (7/12) were managed with a distal splenorenal shunt. All patients received postoperative thromboprophylaxis. We experienced a single mortality, 1 patient experienced shunt thrombosis that required revision shunt surgery, and 2 patients experienced anastomotic strictures, with one being managed with revision surgery and the other currently awaiting radiological venoplasty. CONCLUSIONS: Surgery is a safe and important tool in the management of children with non-cirrhotic portal hypertension and those with sufficient hepatic reserve who fail to respond to more conservative methods for the treatment of side effects of portal hypertension.

Topical herbal medicines for atopic eczema: a systematic review of randomized controlled trials.

Despite the availability of medicines with proven efficacy, many patients use complementary or alternative medicines (CAMs) to manage atopic eczema (AE). Due to the lack of objective information on topical CAMs, this systematic review evaluates the current evidence for the efficacy and safety of topical herbal preparations in AE. Using Cochrane systematic review methodology, PubMed, the Cochrane Library, the Cochrane Central Register of Controlled Trials (CENTRAL), CINAHL (via EBSCO), MEDLINE (via EBSCO), Proquest Health and Medical Complete, GREAT and CAM-QUEST were searched from inception until June 2014. Bibliographies of retrieved studies were hand searched for further relevant trials. All controlled clinical trials of topical herbal medicines for AE in humans of any age were included regardless of the control intervention or randomization. Only English-language publications were considered. Eight studies met the inclusion criteria. Seven investigated extracts of single plants and one an extract from multiple plants. Only two studies that showed a positive effect were considered to have a low risk of bias across all domains (those of liquorice gel and Hypericum perforatum). In these two, the test product was reported to be superior to placebo. Despite variations in diagnostic criteria and lack of validated tools for outcome assessments in one of these, the promising results may warrant continued research in better-designed studies. No meta-analysis was performed due to heterogeneity in all studies. There is currently insufficient evidence of efficacy for any topical herbal extract in AE. Many studies had methodological flaws and even those showing efficacy were single trials with small patient cohorts.

Comparative analysis of South African norovirus GII.4 strains identifies minor recombinant variants.

Recombination within the norovirus (NoV) GII.4 genotype is well documented as a mechanism by which novel variants evolve. Norovirus GII.4 has been the predominant NoV genotype detected in South Africa (SA) in recent years and putative NoV recombinants were previously identified in SA based on partial regions of the viral genome. The objective of this study was to determine the complete genome sequence of representative NoV GII.4 variants that have circulated in SA between 2009 and 2013 and to compare major and minor GII.4 variants based on nucleotide sequence. The complete genomes of 11/27 GII.4 strains could be amplified in three or five overlapping segments, these included major variants New_Orleans_2009 and Sydney_2012 as well as three types of minor GII.4 variants. Phylogenetic and recombination analysis identified GII.4 recombinants with breakpoints located at or near the ORF1/2 junction. Apart from recombinants already recognised as major variants (GII.P4 New_Orleans_2009/GII.4 Sydney_2012 (n=2) and GII.Pe/GII.4 Sydney_2012 (n=2)) four further recombinant strains were detected (GII.P4 New_Orleans_2009/GII.4 Hunter_2004 (n=1) and GII.P4 Yerseke_2006a/GII.4 Apeldoorn_2007 (n=3)) that were attributed to three distinct minor variants. The encoded proteins with the highest diversity were p48 (Nterm), p22, VP1 and VP2. Analysis of antigenic sites in VP1 revealed mutations at epitopes A, B, C, D and E, with epitopes A and D being most variable. The high variation at epitope D was reflected in structural differences in models of GII.4 variants in the epitope D loop region (aa 393-395). Major and minor variants could not be distinguished based on specific sequence differences. HBGA-binding studies will be necessary to assess the effect of the observed amino acid differences in the P2 domain of these GII.4 strains.

Interpreting diaphragmatic movement with bedside imaging, review article.

The diaphragm is the most important muscle of respiration. At equilibrium, the load imposed on the diaphragmatic muscles from transdiaphragmatic pressure balances the force generated by diaphragmatic muscles. However, procedural and nonprocedural thoracic and abdominal conditions may disrupt this equilibrium and impair diaphragmatic function. Diaphragmatic dysfunction is associated with respiratory insufficiency and poor outcome. Therefore, rapid diagnosis and early intervention may be useful. Ultrasound imaging provides quick and accurate bedside assessment of the diaphragm. Various imaging techniques have been suggested, using 2-dimensional and M-mode technology. Diaphragm viewing depends on the degree of robe movement, determined by the angle of incidence of the ultrasound beam and by the direction of probe movement. In this review, we will discuss the function of the diaphragm focusing on clinically important anatomical and physiological properties of the diaphragm. We will review the literature regarding various sonographic techniques for diaphragm assessment. We will also explore the evidence for the role of the tidal displacement of subdiaphragmatic organs as a surrogate for diaphragm movement.

The limitations in implementing and operating a rapid response system.

Despite the widespread introduction of rapid response systems (RRS)/medical emergency teams (MET), there is still controversy regarding how effective they are. While there are some observational studies showing improved outcomes with RRS, there are no data from randomised controlled trials to support the effectiveness. Nevertheless, the MET system has become a standard of care in many healthcare organisations. In this review, we present an overview of the limitations in implementing and operating a RRS in modern healthcare.

Favourable outcomes for the first 10 years of kidney and pancreas transplantation at Wits Donald Gordon Medical Centre, Johannesburg, South Africa.

BACKGROUND: It is important for centres participating in transplantation in South Africa (SA) to audit their outcomes. Wits Donald Gordon Medical Centre (WDGMC), Johannesburg, SA, opened a transplant unit in 2004. The first 10 years of kidney and pancreas transplantation were reviewed to determine outcomes in respect of recipient and graft survival. METHODS: A retrospective review was conducted of all kidney-alone and simultaneous kidney-pancreas (SKP) transplants performed at WDGMC from 1 January 2004 to 31 December 2013, with follow-up to 31 December 2014 to ensure at least 1 year of survival data. Information was accessed using the transplant registers and clinical records in the transplant clinic at WDGMC. The Kaplan-Meier method was used to estimate 1-, 5- and 10-year recipient and graft survival rates for primary (first graft) kidney-alone and SKP transplants. RESULTS: The overall 10-year recipient and graft survival rates were 80.4% and 66.8%, respectively, for kidney-alone transplantation. In the kidney-alone group, children tended towards better recipient and graft survival compared with adults, but this was not statistically significant. In adults, recipient survival was significantly better for living than deceased donor type. Recipient and graft survival were significantly lower in black Africans than in the white (largest proportion in the sample) reference group. For SKP transplants, the 10-year recipient survival rate was 84.7%, while kidney and pancreas graft survival rates were 73.1% and 43.2%, respectively. CONCLUSION: Outcomes of the first 10 years of kidney and pancreas transplantation at WDGMC compare favourably with local and international survival data.

Cognitive function, quality of life and mental health in survivors of our-of-hospital cardiac arrest: a review.

There is growing interest in the long-term outcomes of patients surviving out-of-hospital cardiac arrest (OHCA). This paper aims to summarise the available literature on the long-term cognitive, health-related quality of life (QoL) and mental health outcomes of survivors of OHCA. Between 30% and 50% of survivors of OHCA experience cognitive deficits for up to several years post-discharge. Deficits of attention, declarative memory, executive function, visuospatial abilities and verbal fluency are commonly reported. Survivors of OHCA appear to report high rates of mental illness, with up to 61% experiencing anxiety, 45% experiencing depression and 27% experiencing post-traumatic stress. Fatigue appears to be a commonly reported long-term outcome for survivors of OHCA. Investigations of long-term QoL for these patients have produced mixed findings. Carers of survivors of OHCA report high rates of depression, anxiety and post-traumatic stress, with insufficient social and financial support. The heterogeneous range of instruments used to assess cognitive function and QoL prevent any clear conclusions being drawn from the available literature. The potential biases inherent in this patient population and the interaction between QoL, cognitive performance and mental health warrant further investigation, as does the role of post-discharge support services in improving long-term patient outcomes.

Heterozygote to homozygote related living donor liver transplantation in maple syrup urine disease: a case report.

Liver transplantation is an accepted treatment modality in the management of MSUD. To our knowledge, ours is only the second successful case to date of a patient with MSUD receiving an allograft from an RLD who is a heterozygous carrier for the disease. In view of the worldwide shortage of available organs for transplantation, heterozygote to homozygote transplantation in the setting of MSUD may provide a viable alternative for those awaiting transplantation. We report on the case of a two-yr-old infant with MSUD, who received a left lateral segment (segments II and III) liver transplant from his mother, a heterozygote carrier of one of the three abnormal genes implicated in MSUD. Post-operative BCAA levels normalized in our patient and remained so on an unrestricted protein diet and during times of physiological stress. To date, this is only the second case of a successful RLD liver transplant in a child with MSUD. Preliminary results indicate that RLD liver transplants are at least equivalent to deceased donor liver transplants in the treatment of MSUD, although longer term follow-up is required. Heterozygote to homozygote RLD transplant in patients with MSUD presents a new pool of potential liver donors.