Variability of Care and Access to Transplantation for Children with Biliary Atresia Who Need a Liver Replacement

Background & Aims: Biliary atresia (BA) is the commonest single etiology indication for liver replacement in children. As timely access to liver transplantation (LT) remains challenging for small BA children (with prolonged waiting time being associated with clinical deterioration leading to both preventable pre- and post-transplant morbidity and mortality), the care pathway of BA children in need of LT was analyzed—from diagnosis to LT—with particular attention to referral patterns, timing of referral, waiting list dynamics and need for medical assistance before LT. Methods: International multicentric retrospective study. Intent-to-transplant study analyzing BA children who had indication for LT early in life (aged < 3 years at the time of assessment), over the last 5 years (2016–2020). Clinical and laboratory data of 219 BA children were collected from 8 transplant centers (6 in Europe and 2 in USA). Results: 39 patients underwent primary transplants. Children who underwent Kasai in a specialist -but not transplant- center were older at time of referral and at transplant. At assessment for LT, the vast majority of children already were experiencing complication of cirrhosis, and the majority of children needed medical assistance (nutritional support, hospitalization, transfusion of albumin or blood) while waiting for transplantation. Severe worsening of the clinical condition led to the need for requesting a priority status (i.e., Peld Score exception or similar) for timely graft allocation for 76 children, overall (35%). Conclusions: As LT currently results in BA patient survival exceeding 95% in many expert LT centers, the paradigm for BA management optimization and survival have currently shifted to the pre-LT management. The creation of networks dedicated to the timely referral to a pediatric transplant center and possibly centralization of care should be considered, in combination with implementing all different graft type surgeries in specialist centers (including split and living donor LTs) to achieve timely LT in this vulnerable population

Variability of Care and Access to Transplantation for Children with Biliary Atresia Who Need a Liver Replacement

Background & Aims: Biliary atresia (BA) is the commonest single etiology indication for liver replacement in children. As timely access to liver transplantation (LT) remains challenging for small BA children (with prolonged waiting time being associated with clinical deterioration leading to both preventable pre- and post-transplant morbidity and mortality), the care pathway of BA children in need of LT was analyzed—from diagnosis to LT—with particular attention to referral patterns, timing of referral, waiting list dynamics and need for medical assistance before LT. Methods: International multicentric retrospective study. Intent-to-transplant study analyzing BA children who had indication for LT early in life (aged < 3 years at the time of assessment), over the last 5 years (2016–2020). Clinical and laboratory data of 219 BA children were collected from 8 transplant centers (6 in Europe and 2 in USA). Results: 39 patients underwent primary transplants. Children who underwent Kasai in a specialist -but not transplant- center were older at time of referral and at transplant. At assessment for LT, the vast majority of children already were experiencing complication of cirrhosis, and the majority of children needed medical assistance (nutritional support, hospitalization, transfusion of albumin or blood) while waiting for transplantation. Severe worsening of the clinical condition led to the need for requesting a priority status (i.e., Peld Score exception or similar) for timely graft allocation for 76 children, overall (35%). Conclusions: As LT currently results in BA patient survival exceeding 95% in many expert LT centers, the paradigm for BA management optimization and survival have currently shifted to the pre-LT management. The creation of networks dedicated to the timely referral to a pediatric transplant center and possibly centralization of care should be considered, in combination with implementing all different graft type surgeries in specialist centers (including split and living donor LTs) to achieve timely LT in this vulnerable population

Variability of Care and Access to Transplantation for Children with Biliary Atresia Who Need a Liver Replacement

Background & Aims: Biliary atresia (BA) is the commonest single etiology indication for liver replacement in children. As timely access to liver transplantation (LT) remains challenging for small BA children (with prolonged waiting time being associated with clinical deterioration leading to both preventable pre- and post-transplant morbidity and mortality), the care pathway of BA children in need of LT was analyzed-from diagnosis to LT-with particular attention to referral patterns, timing of referral, waiting list dynamics and need for medical assistance before LT. Methods: International multicentric retrospective study. Intent-to-transplant study analyzing BA children who had indication for LT early in life (aged < 3 years at the time of assessment), over the last 5 years (2016-2020). Clinical and laboratory data of 219 BA children were collected from 8 transplant centers (6 in Europe and 2 in USA). Results: 39 patients underwent primary transplants. Children who underwent Kasai in a specialist -but not transplant- center were older at time of referral and at transplant. At assessment for LT, the vast majority of children already were experiencing complication of cirrhosis, and the majority of children needed medical assistance (nutritional support, hospitalization, transfusion of albumin or blood) while waiting for transplantation. Severe worsening of the clinical condition led to the need for requesting a priority status (i.e., Peld Score exception or similar) for timely graft allocation for 76 children, overall (35%). Conclusions: As LT currently results in BA patient survival exceeding 95% in many expert LT centers, the paradigm for BA management optimization and survival have currently shifted to the pre-LT management. The creation of networks dedicated to the timely referral to a pediatric transplant center and possibly centralization of care should be considered, in combination with implementing all different graft type surgeries in specialist centers (including split and living donor LTs) to achieve timely LT in this vulnerable population.Peer reviewe

Management of hepatic epithelioid haemangio-endothelioma in children: what option?

Hepatic epithelioid haemangio-endothelioma (HEHE) is an endothelium-derived tumour of low-to-medium grade malignancy. It is predominantly seen in adults and is unresponsive to chemotherapy. Liver transplantation is an accepted indication when the tumour is unresectable. Hepatic epithelioid haemangio-endothelioma is very rare in children and results after transplantation are not reported. The aim of this study is to review the experience of three European centres in the management of HEHE in children. A retrospective review of all paediatric patients with HEHE managed in three European centres is presented. Five children were identified. Four had unresectable tumours. The first had successful resection followed by chemotherapy and is alive, without disease 3 years after diagnosis. One child died of sepsis and one of tumour recurrence in the graft and lungs 2 and 5 months, respectively, after transplant. Two children who had progressive disease with ifosfamide-based chemotherapy have had a reduction in clinical symptoms and stabilisation of disease up to 18 and 24 months after the use of platinum-based chemotherapy. HEHE seems more aggressive in children than reported in adults and the curative role of transplantation must be questioned. Ifosfamide-based chemotherapy was not effective. Further studies are necessary to confirm if HEHE progression in children may be influenced by platinum-based chemotherapy

Old and new cluster designs in emergency field surveys: in search of a one-fits-all solution

<p>Abstract</p> <p>Introduction</p> <p>Cluster surveys are frequently used to measure key nutrition and health indicators in humanitarian emergencies. The survey design of 30 clusters of 7 children (30 × 7) was initially proposed by the World Health Organization for measuring vaccination coverage, and later a design of 30 clusters of 30 children (30 × 30) was introduced to measure acute malnutrition in emergency settings. Recently, designs of 33 clusters of 6 children (33 × 6) and 67 clusters of 3 children (67 × 3) have been proposed as alternatives that enable measurement of several key indicators with sufficient precision, while offering substantial savings in time. This paper explores expected effects of using 67 × 3, 33 × 6, or 30 × 7 designs instead of a "standard" 30 × 30 design on precision and accuracy of estimates, and on time required to complete the survey.</p> <p>Analysis</p> <p>The 67 × 3, 33 × 6, and 30 × 7 designs are expected to be more statistically efficient for measuring outcomes having high design effects (e.g., vaccination coverage, vitamin A distribution coverage, or access to safe water sources), and less efficient for measuring outcomes with more within-cluster variability, such as global acute malnutrition or anemia. Because of small sample sizes, these designs may not provide sufficient levels of precision to measure crude mortality rates. Given the small number (3 to 7) of survey subjects per cluster, it may be hard to select representative samples of subjects within clusters.</p> <p>The smaller sample size in these designs will likely result in substantial time savings. The magnitude of the savings will depend on several factors, including the average travel time between clusters. The 67 × 3 design will provide the least time savings. The 33 × 6 and 30 × 7 designs perform similarly to each other, both in terms of statistical efficiency and in terms of time required to complete the survey.</p> <p>Conclusion</p> <p>Cluster designs discussed in this paper may offer substantial time and cost savings compared to the traditional 30 × 30 design, and may provide acceptable levels of precision when measuring outcomes that have high intracluster homogeneity. Further investigation is required to determine whether these designs can consistently provide accurate point estimates for key outcomes of interest. Organizations conducting cluster surveys in emergency settings need to build their technical capacity in survey design to be able to calculate context-specific sample sizes individually for each planned survey.</p

Emerging Disease Burdens and the Poor in Cities of the Developing World

Patterns of future urban growth, combined with advances in the treatment of traditional scourges of communicable diseases, will cause a shift in the burden of disease toward category 2 (noncommunicable) and 3 (injury) conditions over the next 30 years. Communicable diseases, particularly HIV/AIDs, will continue to be the most important killers among the poor. However, new risks will emerge for several reasons. First, the marked sprawl of cities in the developing world will make access to care more difficult. Second, increasing motor vehicles and the likelihood of inadequate infrastructure will make air pollution and accidents in road traffic more common than in the past. Third, impoverished urban populations have already shown a propensity toward undernourishment, and its obverse, obesity, is already emerging as a major risk. Also, the large projected increase in slums suggests that violence and homicide will become a more important burden of health, and very large hazards will be created by fire-prone, insubstantial dwellings that will house nearly two billion people by 2030. In addition, decentralized governance will exacerbate the tensions and discontinuities that have plagued the management of health issues on the urban fringe over the past decade. Accordingly, public health agencies will need to adjust to the regional and country-specific factors to address the changing profile of risk. This analysis suggests that four factors – levels of poverty, speed of city growth, sprawl in cities, and degree of decentralization – will have importance in shaping health strategies. These factors vary in pace and intensity by region, suggesting that health care strategies for Category II and III conditions will need to be differentiated by region of the world. Also, interventions will have to rely increasingly on actors outside the ranks of public health specialists

Measles outbreaks in displaced populations: a review of transmission, morbidity and mortality associated factors

<p>Abstract</p> <p>Background</p> <p>Measles is a highly contagious infectious disease with a significant public health impact especially among displaced populations due to their characteristic mass population displacement, high population density in camps and low measles vaccination coverage among children. While the fatality rate in stable populations is generally around 2%, evidence shows that it is usually high among populations displaced by disasters. In recent years, refugees and internally displaced persons have been increasing. Our study aims to define the epidemiological characteristics and risk factors associated with measles outbreaks in displaced populations.</p> <p>Methods</p> <p>We reviewed literature in the PubMed database, and selected articles for our analysis that quantitatively described measles outbreaks.</p> <p>Results</p> <p>A total of nine articles describing 11 measles outbreak studies were selected. The outbreaks occurred between 1979 and 2005 in Asia and Africa, mostly during post-conflict situations. Seven of eight outbreaks were associated with poor vaccination status (vaccination coverage; 17-57%), while one was predominantly due to one-dose vaccine coverage. The age of cases ranged from 1 month to 39 years. Children aged 6 months to 5 years were the most common target group for vaccination; however, 1622 cases (51.0% of the total cases) were older than 5 years of age. Higher case-fatality rates (>5%) were reported for five outbreaks. Consistent factors associated with measles transmission, morbidity and mortality were vaccination status, living conditions, movements of refugees, nutritional status and effectiveness of control measures including vaccination campaigns, surveillance and security situations in affected zones. No fatalities were reported in two outbreaks during which a combination of active and passive surveillance was employed.</p> <p>Conclusion</p> <p>Measles patterns have varied over time among populations displaced by natural and man-made disasters. Appropriate risk assessment and surveillance strategies are essential approaches for reducing morbidity and mortality due to measles. Learning from past experiences of measles outbreaks in displaced populations is important for designing future strategies for measles control in such situations.</p

Мова українських обрядових пісень

Intestinal failure (IF) is the reduction in functioning gut mass below the minimal level necessary for adequate digestion and absorption of nutrients and fluids for weight maintenance in adults or for growth in children. There is a paucity of epidemiologic data on pediatric IF. The purpose of this study was to determine the prevalence, incidence, regional distribution and underlying diagnosis of pediatric chronic IF (CIF) requiring home parenteral nutrition (HPN) in Italy. METHODS: Local investigators were selected in 19 Italian centers either of reference for pediatric HPN or having pediatric gastroenterologists or surgeons on staff and already collaborating with the Italian Society for Pediatric Gastroenterology, Hepatology and Nutrition with regard to IF. Data requested in this survey for children at home on Parenteral Nutrition (PN) on 1 December 2016 included patient initials, year of birth, gender, family's place of residence and underlying diagnosis determining IF. RESULTS: We recorded 145 CIF patients on HPN aged ≤19 years. The overall prevalence was 14.12/million inhabitants (95% CI: 9.20-18.93); the overall incidence was 1.41/million inhabitant years (95% CI: 0.53-2.20). CONCLUSION: Our survey provides new epidemiological data on pediatric CIF in Italy; these data may be quantitatively useful in developing IF care strategy plans in all developed countrie

Consequences of Cold-Ischemia Time on Primary Nonfunction and Patient and Graft Survival in Liver Transplantation: A Meta-Analysis

Introduction: The ability to preserve organs prior to transplant is essential to the organ allocation process. Objective: The purpose of this study is to describe the functional relationship between cold-ischemia time (CIT) and primary nonfunction (PNF), patient and graft survival in liver transplant. Methods: To identify relevant articles Medline, EMBASE and the Cochrane database, including the non-English literature identified in these databases, was searched from 1966 to April 2008. Two independent reviewers screened and extracted the data. CIT was analyzed both as a continuous variable and stratified by clinically relevant intervals. Nondichotomous variables were weighted by sample size. Percent variables were weighted by the inverse of the binomial variance. Results: Twenty-six studies met criteria. Functionally, PNF%=-6.678281+0.9134701*CIT Mean+0.1250879*(CIT Mean-9.89535) 2 - 0.0067663*(CIT Mean-9.89535) 3, r2=.625, p<.0001. Mean patient survival: 93 % (1 month), 88 % (3 months), 83 % (6 months) and 83 % (12 months). Mean graft survival: 85.9 % (1 month), 80.5 % (3 months), 78.1 % (6 months) and 76.8 % (12 months). Maximum patient and graft survival occurred with CITs between 7.5-12.5 hrs at each survival interval. PNF was also significantly correlated with ICU time, % first time grafts and % immunologic mismatches. Conclusion: The results of this work imply that CIT may be the most important pre-transplant information needed in the decision to accept an organ. © 2008 Stahl et al