Wilson's disease (WD) is a rare liver-based disorder of copper metabolism. Prognostic criteria described by our group in 1986 to predict death without transplantation have not been universally validated. The clinical features of 88 children were reviewed, retrospectively in 74 and prospectively in 14. Data from the retrospectively recruited patients that died or survived on long-term chelation were used to evaluate the validity of our old scoring system and to devise a new prognostic index, then assessed in the 14 prospectively recruited patients. Using the old scoring system, 5 children scoring > 7, the cutoff value for death without transplantation, survived, whereas 4 scoring < 7 died (sensitivity 87% and specificity 90%). A new index based on serum bilirubin, international normalized ratio, aspartate aminotransferase (AST), and white cell count (WCC) at presentation identified a cutoff score of 11 for death and proved to be 93% sensitive and 98% specific, with a positive predictive value of 88%. When the new index was evaluated prospectively in 14 patients, it predicted the need for transplantation in only the 4 who required it, although 1 child with a score of 11 survived on medical treatment. In conclusion, the new Wilson Index is more sensitive and specific in predicting mortality without transplantation than the old scoring system, but needs to be validated in a larger number of patients. (Liver Transpl 2005;11:441-448.)

As there was commonality in themes between studies strategies to lessen the burden of chronic illness during adolescence do not necessarily need to be disease specific. Nurses need to focus on treating the young person rather than their illness.

BackgroundSome countries have undertaken programs that included scaling up kangaroo mother care. The aim of this study was to systematically evaluate the implementation status of facility-based kangaroo mother care services in four African countries: Malawi, Mali, Rwanda and Uganda.MethodsA cross-sectional, mixed-method research design was used. Stakeholders provided background information at national meetings and in individual interviews. Facilities were assessed by means of a standardized tool previously applied in other settings, employing semi-structured key-informant interviews and observations in 39 health care facilities in the four countries. Each facility received a score out of a total of 30 according to six stages of implementation progress.ResultsAcross the four countries 95 per cent of health facilities assessed demonstrated some evidence of kangaroo mother care practice. Institutions that fared better had a longer history of kangaroo mother care implementation or had been developed as centres of excellence or had strong leaders championing the implementation process. Variation existed in the quality of implementation between facilities and across countries. Important factors identified in implementation are: training and orientation; supportive supervision; integrating kangaroo mother care into quality improvement; continuity of care; high-level buy in and support for kangaroo mother care implementation; and client-oriented care.ConclusionThe integration of kangaroo mother care into routine newborn care services should be part of all maternal and newborn care initiatives and packages. Engaging ministries of health and other implementing partners from the outset may promote buy in and assist with the mobilization of resources for scaling up kangaroo mother care services. Mechanisms for monitoring these services should be integrated into existing health management information systems.