A kinetic assay of total lipase activity for detecting lysosomal acid lipase deficiency (LAL-D) and the molecular characterization of 18 LAL-D patients from Russia.

Unlabelled: Laboratory diagnostics of lysosomal acid lipase deficiency (LAL-D), a rare disorder associated with alterations, are based on the evaluation of LAL activity. In dry blood spots (DBS) submitted for LAL-D diagnostics (the screening cohort) over a two-year period or obtained from a cohort of retrospective LAL-D patients, we measured: (1) LAL activity using a two-reaction assay with 4-methylumbelliferone palmitate (4-MU-Palm) and Lalistat-2, a specific LAL inactivator; (2) total lipase (TL) activity by a 1-hour kinetic 4-MU-Palm cleavage reaction (no Lalistat-2). The TL activity was expressed as the area under the kinetic curve after 1 hour (TL-AUC) of the reaction and presented as the median (min-max).

HELCOM Baltic Sea Action Plan--a regional programme of measures for the marine environment based on the Ecosystem Approach.

The Helsinki Commission (HELCOM) Baltic Sea Action Plan, adopted by the coastal countries of the Baltic Sea and the European Community in November 2007, is a regional intergovernmental programme of measures for the protection and management of the marine environment explicitly based on the Ecosystem Approach. The Action Plan is structured around a set of Ecological Objectives used to define indicators and targets, including effect-based nutrient input ceilings, and to monitor implementation. The Action Plan strongly links Baltic marine environmental concerns to important socio-economic fields such as agriculture and fisheries and promotes cross-sectoral tools including marine spatial planning.