BRAF-V600E mutation and its clinical significance in children with Langerhans cell histiocytosis / 中国当代儿科杂志

<p><b>OBJECTIVE</b>To investigate the clinical significance of BRAF-V600E mutation in children with Langerhans cell histiocytosis (LCH).</p><p><b>METHODS</b>Real-time fluorescence quantitative PCR was used to detect BRAF-V600E mutation in paraffin-embedded tissue samples from 26 children with LCH. A retrospective analysis was performed for the association of BRAF-V600E mutation with clinical features and prognosis of children with LCH.</p><p><b>RESULTS</b>Of the 26 children, 25 received standard chemotherapy, with a 2-year overall survival (OS) rate of 100% and a 2-year event-free survival (EFS) rate of 88%. Of the 26 pathological samples, 18 (70%) came from bone tissue, and the positive rate of BRAF-V600E mutation reached 50% (13/26). The positive rate of BRAF-V600E gene mutation was not associated with age, sex, affected organ, clinical classification, early treatment response, recurrence, and 2-year OS and EFS rates of the children with LCH (P>0.05), but it was associated with clinical grouping of LCH (P<0.05).</p><p><b>CONCLUSIONS</b>Children with LCH tend to have a high OS rate and a high incidence rate of BRAF-V600E mutation. BRAF-V600E mutation is associated with clinical grouping of LCH.</p>

Roles of type II 11β-hydroxysteroid dehydrogenase and its signaling factors in pathogenesis of persistent pulmonary hypertension in neonatal rats / 中国当代儿科杂志

<p><b>OBJECTIVE</b>To study the roles of type II 11β-hydroxysteroid dehydrogenase (11β-HSD2) and it's signaling factors in the lung tissue in pathogenesis of persistent pulmonary hypertension (PPH) in neonatal rats.</p><p><b>METHODS</b>Six Sprague-Dawley rats on the 19th day of pregnancy were randomly divided into PPH and control groups (n=3 each). The PPH group was intraperitoneally injected with indomethacin (0.5 mg/kg) twice daily and exposed in 12% oxygen for three days, in order to prepare a fetal rat model of PPH. The control group was intraperitoneally injected with an equal volume of normal saline and exposed to air. Neonatal rats were born by caesarean section from both groups on the 22nd day of pregnancy. In each group, 15 neonatal rats were randomly selected and sacrificed. 11β-HSD2 expression in the lung tissue of neonatal rats were observed by Confocal laser technology, and serum cortisol levels and prostacyclin, renin, angiotensin and aldosterone in the lung tissue of both groups were measured using ELISA.</p><p><b>RESULTS</b>11β-HSD2 protein was widely expressed in the lung tissue of the control and PPH groups. The levels of 11β-HSD2 and prostacyclin in the lung tissue were lower in the PPH group than in the control group, while serum cortisol levels and renin, angiotensin and aldosterone in the lung tissue were higher in the PPH group than in the control group (P<0.05).</p><p><b>CONCLUSIONS</b>11β-HSD2 and it's signaling factors play roles in pathogenesis of PPH in neonatal rats.</p>