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Introduction

Wilson's disease (WD) is a rare autosomal recessive disorder transmitted through a gene located on chromosome 13. Liver transplantation (LT) provides a therapeutic option for patients with WD presenting fulminant liver failure or drug resistance. LT in patients with WD has a twofold aim: to save the patient's life when the disorder has progressed to hepatic (or other organ) failure and to cure the underlying metabolic defect. The aim of our study was to investigate the indications, aspects and post-operative outcomes in pediatric patients (<?18 years old) with WD who underwent LT.

Methods

A meticulous search of the literature since 1971 was performed. A retrospective analysis of all the studies, presenting cases of LT in children due to WD, was conducted. Studies that did not report patients' characteristics, transplantation indications, post-operative outcomes, and complications, as well as those with small study populations (<?10 patients), were excluded.

Results

Six studies were included in the present review, which involved 290 children. The main indications for LT included chronic liver failure and fulminant liver failure. The average 1-year survival rate was 91.9%, while the average 5-year survival rate was 88.2%. Retransplantation was performed in 16 patients due to transplant rejection. In general, patients transplanted for WD displayed an excellent quality of life after LT.

Conclusion

LT is a safe and efficient procedure in selected pediatric patients with WD, demonstrating excellent long-term outcomes and quality of life.  相似文献   
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Background

Glucocorticoids play essential roles in the treatment of childhood acute lymphoblastic leukaemia (ALL); however, treatment with these agents can result in severe side-effects. This study, the first of its kind in a Saudi population, investigates associations of ABCB1 gene polymorphisms (pharmacodynamics and pharmacokinetic) with the development of toxicity and side effects (glucose abnormality, liver toxicity and infection) in a small population of Saudi children with ALL.

Methods

Three single nucleotide polymorphisms (SNPs) of the ABCB1 gene (rs 3213619 T129C, rs 2032582 G2677T and rs1045642 C3435T) were analysed in 70 Saudi children with ALL and 60 control subjects. Participants were treated according to the ALL 2000 study protocol. Toxicities were assessed and associations with genotypes were evaluated according to Common Toxicity Criteria (NCI-CTC).

Results

Significant associations were observed among carriers and the mutated genotype C3435T (ABCB1), which had an incidence of infection (p?=?0.05). Although no correlations were found between liver toxicity and glucose abnormalities for patients carrying ABCB1 SNPs, risk factors for liver toxicity were elevated by a factor of three for patients carrying the SNP G2677T, OR 3.00 (1.034–8.702). The risk factor of glucose abnormality toxicity for the patients carring T129C were increased three times OR 3.06 (0.486–19.198).

Conclusions

In terms of infection incidence, polymorphism C3435T may contribute to potential life-threatening infections during paediatric ALL therapy, through glucocorticoid usage.  相似文献   
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Routine distress screening in United States oncology clinics has been mandatory since 2015.

Objective: This study was the first to assess distress in a geographically diverse sample of cancer patients following mandated distress screening implementation by oncology social workers.

Methods: Sites were self-selected via social workers who applied to participate in the Association of Oncology Social Work’s Project to Assure Quality Cancer Care, advertised through their social media outlets and conference. Electronic screening records were collected from 55 cancer treatment centers in the United States and Canada. Cases required cancer diagnoses and Distress Thermometer (DT) scores to be included. Distress rates and rates by age, sex, cancer type, and ethnicity were examined.

Results: Of 4664 cases, 46% (2157) experienced significant distress (DT score?≥?4). Being female, age 40-59, and having diagnoses of pancreatic or lung cancer was associated with increased likelihood of distress. Half of cases experience clinically-significant distress, though this need was not evenly distributed across patient or cancer types.

Conclusion: Identifying those at risk for distress may help inform optimal resource allocation. Methods to address needs of distressed patients in cases of limited resources are discussed.  相似文献   

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At this study a multi-criteria model was developed to examine the available procedures, techniques and methods of handling infectious waste in the large healthcare unit of University Regional General Hospital of Patras, Western Greece. Particularly, this study examined the: a) current legislation and Directives issued for medical waste management at Greece and among the other EU-members, b) contribution of healthcare wastes (HCW) generation rate on social and economic parameters in selected European countries, c) available procedures, techniques, and methods upon the disposal of infectious wastes at the healthcare studied, and, d) propositions for integrated management of such hazardous wastes. Specifically, the Analytic Hierarchy Process (AHP) methodology was applied under pair wise comparison matrices in two stages: 1) the scale factors and the indicators, and 2) the criteria and their sub–criteria. The assessment of these pair wise matrices included the indicators and the sub–criteria. Subsequently, two pair wise comparison matrices, upon a) the “Fulfillment of environmental objectives” indicator and b) the “Energy consumption” sub criterion, were denoted. The AHP methodology yielded good results; however there is still space of improving the environmental performance. The normalized relative weights obtained for the criteria and sub criteria motivated specific actions that have to be handled. Particularly, the results indicated a very good value in environmental management criteria due the values obtained for the commitment towards the environmental policy standards and the waste management procedures. However, further improvements on staff awareness (such as development programs to enhance sensitivity) and more green purchasing suppliers, should be further addressed.  相似文献   
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