Pediatric systemic lupus erythematosus: management issues in primary practice

PURPOSE: To provide nurse practitioners (NPs) with an update on pediatric systemic lupus erythematosus (SLE) with an emphasis on management considerations for primary care practitioners. DATA SOURCES: An extensive literature review was conducted using both Medline and CINAHL databases. Research articles reflecting the most compelling findings were included in this review. CONCLUSIONS: NPs who care for children with SLE may be able to prevent or delay the morbidities associated with this disease and its treatments, if they keep abreast of the new information evolving in this realm of rheumatologic diseases. IMPLICATIONS FOR PRACTICE: As more is learned about pediatric SLE, better treatments have evolved such that the survival rates have increased. The primary care of pediatric SLE patients that is focused on preventing or delaying SLE morbidities may help to restore, maintain, or improve the quality of life for these patients.     

Coordinated β-globin expression and α2-globin reduction in a multiplex lentiviral gene therapy vector for β-thalassemia

A primary challenge in lentiviral gene therapy of β-hemoglobinopathies is to maintain low vector copy numbers to avoid genotoxicity while being reliably therapeutic for all genotypes. We designed a high-titer lentiviral vector, LVβ-shα2, that allows coordinated expression of the therapeutic β^A-T87Q-globin gene and of an intron-embedded miR-30-based short hairpin RNA (shRNA) selectively targeting the α2-globin mRNA. Our approach was guided by the knowledge that moderate reduction of α-globin chain synthesis ameliorates disease severity in β-thalassemia. We demonstrate that LVβ-shα2 reduces α2-globin mRNA expression in erythroid cells while keeping α1-globin mRNA levels unchanged and β^A-T87Q-globin gene expression identical to the parent vector. Compared with the first β^A-T87Q-globin lentiviral vector that has received conditional marketing authorization, BB305, LVβ-shα2 shows 1.7-fold greater potency to improve α/β ratios. It may thus result in greater therapeutic efficacy and reliability for the most severe types of β-thalassemia and provide an improved benefit/risk ratio regardless of the β-thalassemia genotype.     

New fatty acid esters originate during storage by the interaction of components in prasaplai, a Thai traditional medicine

Prasaplai is a Thai traditional preparation composed of twelve ingredients; during storage three newly emerging compounds were detected by HPLC. Their structures were established as the new (E)-4-(3,4-dimethoxyphenyl)but-3-en-1-yl linoleate, (E)-4-(3,4-dimethoxyphenyl)but-3-en-1-yl oleate and the known (E)-4-(3,4-dimethoxyphenyl)but-3-en-1-yl palmitate on the basis of spectral data and chemical evidence. The artificial esters were already observable after one day of storage of the freshly prepared dry mixture, and steadily increased during a storage period of 30 days and more. A systematic search indicated that the three esters arose from the interaction of compounds contained in the rhizomes of Zingiber cassumunar and the seeds of Nigella sativa.