Hereditary systemic immunoglobulin light-chain amyloidosis

Several members of a family died from renal failure as a result of systemic amyloidosis. Extensive studies to detect previously documented gene mutations associated with amyloidosis failed to identify a causative factor. In search of the genetic basis for this syndrome, amyloid fibrils were isolated from renal tissue of a member of the kin who died while on renal dialysis. Amino acid sequencing of isolated amyloid protein identified sequences compatible with the constant region of the immunoglobulin κ light-chain. Isolation and characterization of κ light-chain protein from serum of an affected member of the kindred revealed mutation in the constant region of κ light-chain, with cysteine replacing serine at amino acid residue 131. This mutation (Ser131Cys) was confirmed by DNA analysis, which identified a single-base change of cytosine to guanine at the second position of codon 131 of the κ light-chain gene (TCT131TGT). DNA analysis of members of the extended family revealed transmission of the Ser131Cys mutation and association with systemic amyloidosis. This amyloid light-chain (AL) amyloidosis, which is a hereditary type of amyloidosis and not the result of a monoclonal plasma cell dyscrasia, may be misdiagnosed and lead to inappropriate chemotherapy

Technical and vocational education and training (TVET) for young people in low- and middle-income countries: a systematic review and meta-analysis

The world is facing a worsening youth employment crisis. In response, technical and vocational education and training (TVET) is back on the development agenda after years of neglect. This systematic review examined the evidence from studies evaluating the impacts of TVET interventions for young people in low- and middle-income countries (LMICs). The 26 included studies evaluated 20 different interventions, predominantly from Latin America. Meta-analyses of the effectiveness of TVET on five outcome measure categories were conducted. The overall mean effects on overall paid employment, formal employment, and monthly earnings were small, positive, and significant; however, significant heterogeneity was observed. Moderator analysis was performed in an attempt to explain between-study differences in effects. The overall paucity of research in this area, together with specific gaps and methodological limitations, affirm the need for strengthening the evidence base. Implications for policy, practice and research are discussed