Mevalonic aciduria: Does stem cell transplant fully cure disease?

MA is a rare, autosomal recessive disorder characterized by episodes of inflammation and periodic fevers. In its most severe form, it can result in facial dysmorphism, growth inhibition, ataxia, liver dysfunction, intellectual disability, and at times can be fatal. A number of case reports exist stating that SCT is curative in these patients. We present the case of a patient diagnosed with MA at birth, who underwent SCT at the age of 14 months with intent to cure. She achieved complete engraftment and urine mevalonate became undetectable. However, 18 months following transplant, she developed frequent episodes of fevers, rashes, arthritis, and a rising urinary mevalonate. She was subsequently diagnosed with relapse. She now requires treatment with steroids and canakinumab to manage her disease. This case is the first report of disease relapse following transplant for MA. It runs contrary to prior reports that SCT is fully curative of MA and suggests that transplant may instead provide a means of decreasing disease severity without entirely eradicating the condition.     

小儿推拿“六腑”穴位考

[目的]通过考证相关文献，更全面认识小儿推拿特定穴“六腑”，正确指导临床应用。[方法]通过整理研究小儿推拿经典古籍和现代文献，对六腑的定位、操作、功效、配伍及现代临床应用、机制研究进行详细解析，从多个方面深入了解该穴。[结果]归纳古籍发现六腑的定位和操作存在争议，普遍认为穴性为寒凉，有退热、通腑之效，常与三关配伍合用于寒热、虚实夹杂之证。现代医家认为该穴有通腑、泻热、解毒的作用，临床可用于小儿多系统病变的治疗，尤以肺、脾胃系疾病多见，效果显著，但目前对于作用机制的研究较少，退热机制可能与神经反馈调节、免疫调节等相关，通腑、解毒机制尚未有前人研究。[结论]小儿推拿六腑穴退热、通腑功效卓著，古今广泛用于临床多种小儿疾病的治疗，备受医家推崇。全面了解该穴，正确指导辨证施术，才能发挥其最佳临床疗效。     

清金降火汤加减联合阿奇霉素对痰湿闭阻型小儿支原体肺炎患者的临床疗效

目的探讨清金降火汤加减联合阿奇霉素对痰湿闭阻型小儿支原体肺炎患者的临床疗效。方法 210例患者随机分为清金降火汤加减组、阿奇霉素组、联合组,每组70例,疗程14 d。检测临床疗效、肺功能指标(PEF、VPTEF、TPTEF、PFV)、血清抗炎因子(IL-2、IL-4、IL-10、IL-13)、血清促炎因子(TNF-α、IFN-γ、IL-6、IL-8)、血清免疫功能指标(CD^3+、CD^4+、CD^8+、RBC-ICR)、不良反应发生率变化。结果联合组总有效率、VPTEF、TPTEF、PFV、IL-2、IL-13、CD3+、CD4+高于清金降火汤加减组、阿奇霉素组(P<0.05),PEF、IL-4、IL-10、TNF-α、IFN-γ、IL-6、IL-8、CD^8+、RBC-ICR降低(P<0.05),不良反应发生率低于阿奇霉素组(P<0.05)。结论清金降火汤加减联合阿奇霉素可有效改善痰湿闭阻型小儿支原体肺炎患者肺功能、血清炎性因子、免疫功能,安全性较高。     

Neighborhood effect and obesity in adult survivors of pediatric cancer: A report from the St. Jude lifetime cohort study

Survivors of childhood cancer are at risk for obesity, a condition potentially modifiable if dietary intake and physical activity are optimized. These health behaviors are likely influenced by neighborhood of residence, a determinant of access to healthy, affordable food and safe and easy exercise opportunities. We examined associations between neighborhood level factors and obesity among survivors in the St. Jude Lifetime cohort and community comparison group members. Persons with residential addresses available for geocoding were eligible for analysis (n = 2,265, mean age 32.5 [SD 9.1] years, 46% female, 85% white). Survivors completed questionnaires regarding individual behaviors; percent body fat was assessed via dual X-ray absorptiometry (obesity: ≥25% males; ≥35% females); neighborhood effect was characterized using census tract of residence (e.g., neighborhood socioeconomic status [SES], rurality). Structural equation modeling was used to determine associations between neighborhood effect, physical activity, diet, smoking, treatment exposures and obesity. Obese survivors (n = 1,420, 62.7%) were more likely to live in neighborhoods with lower SES (RR: 1.23, 95% CI: 1.10–1.38) and rural areas (RR: 1.22, 95% CI: 1.07–1.39) compared to survivors with normal percent body fat. Resource-poor neighborhoods (standardized effect: 0.06, p < 0.001) and cranial radiation (0.16, p < 0.001) had direct effects on percent body fat. Associations between neighborhood of residence and percent body fat were increased (0.01, p = 0.04) among individuals with a poor diet. Neighborhoods where survivors reside as an adult is associated with obesity. Interventions targeting survivors should incorporate strategies that address environmental influences on obesity.     

刘晓鹰教授“扶中”外治法治疗儿科疾病临床经验浅析

小儿"脾常不足",脾胃之体成而未全,脾胃之气全而未壮,脾胃不足在儿科疾病发生发展、病机转归中占有相当重要的地位。刘晓鹰教授在继承全国名老中医倪珠英学术思想基础上,提出"扶中"外治法,立法取穴方面强调时时顾护小儿中焦脾胃,采用小儿推拿、扶阳罐(含温推、温灸和温刮痧)、针刺(含腹针、揿针)等外治手段治疗儿科脾、肺、肾等系列疾病,取得满意疗效。