Non-alcoholic fatty liver disease (NAFLD) is the most common paediatric liver disease with a prevalence of almost 10%; therefore, the majority of affected patients are under the care of general practitioners and non-specialists. The condition is caused by central obesity with insulin resistance with additional factors influencing inflammatory activity (steatohepatitis). Ongoing inflammation leads to fibrosis and end-stage liver disease, though this will usually occur after children have transitioned into adult care. However, their main morbidity and mortality is from type 2 diabetes and complications of atherosclerosis. The minority of children undergo biopsy but currently there is no other method to accurately assess the stage of disease. Management is focused at weight loss through a combination of diet and exercise. Here, we present a current review of paediatric NAFLD aimed at non-specialists, with practice points for implementation.

Permanent neonatal diabetes mellitus (PNDM) is caused by reduced b-cell number or impaired b-cell function. Understanding of the genetic basis of this disorder highlights fundamental b-cell mechanisms. We performed trio genome sequencing for 44 patients with PNDM and their unaffected parents to identify causative de novo variants. Replication studies were performed in 188 patients diagnosed with diabetes before 2 years of age without a genetic diagnosis. EIF2B1 (encoding the eIF2B complex a subunit) was the only gene with novel de novo variants (all missense) in at least three patients. Replication studies identified two further patients with de novo EIF2B1 variants. In addition to having diabetes, four of five patients had hepatitis-like episodes in childhood. The EIF2B1 de novo mutations were found to map to the same protein surface. We propose that these variants render the eIF2B complex insensitive to eIF2 phosphorylation, which occurs under stress conditions and triggers expression of stress response genes. Failure of eIF2B to sense eIF2 phosphorylation likely leads to unregulated unfolded protein response and cell death. Our results establish de novo EIF2B1 mutations as a novel cause of permanent diabetes and liver dysfunction. These findings confirm the importance of cell stress regulation for b-cells and highlight EIF2B1's fundamental role within this pathway.

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In the ancient Greek city states people have participated in decision making known as direct democracy. The expansion of population over time made this mode of direct decision making impossible and so representative democracy was born. It evolved to have the separation of powers; legislative, executive and judicial.The separation of powers of the State enables the judiciary keeps the executive and administrative functions in check. The courts however, have restricted the right to intervene to those persons whose interests were directly affected rather than allow busy bodies wanting fame and fan fare waste the time of court. Thus while decisions of the executive and measures taken by the administration affect the rights of individuals on matters such as land acquisition, licencing, issuing of permits, taxation, and the nationalization of industries, only those affected and not the public could petition court.The present Constitution is a step in facilitating direct democracy. The gradual willingness of the judiciary to allow the public to intervene in matters that they would traditionally have been shut out of, have enabled both the courts and the public to affect the decision making process and its implementation. The Eppawala Phosphate mining case, the Southern Expressway case, the Waters Edge case, cancellation of privatization of Sri Lanka Insurance Corporation, the cancellation of sale of Lanka Marine Service to John Keels Holdings and de-merging of North and East Provincial Councils are among notable instances of public interest litigation. This paper will analyze the expansion of public interest litigation, the principles of good governance propounded by court including the public trust doctrine, and the impact it has had on administrative and policy decision making.

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