Anti-inflammatory and Immunosuppressive Effects of Panax notoginseng

Here, we designed to examine the anti-inflammatory effects on RAW264.7 cells and the immunosuppressive effects by evaluating interleukin-2 (IL-2) production in Jurkat T cells using a MeOH extract of Panax notoginseng roots. The results showed that the MeOH extract inhibited the synthesis of nitric oxide (NO) in a dose-dependent manner (IC₅₀ value of 7.08 µg/mL) and displayed effects on T cell activation at a concentration of 400 µg/mL. In efforts to identify the potent compounds, bioactivity-guided fractionation of the MeOH extract and chemical investigation of its active CH₂Cl₂-, EtOAc-, and butanol-soluble fractions led to the successful isolation and identification of eleven compounds, including two polyacetylenes (1, 2), a steroid saponin (3), seven dammarane-type ginsenosides (4 – 10), and an oleanane-type ginsenoside (11). Among them, compound 11 was isolated from this plant for the first time. Compound 2 exhibited potent inhibitory effects on NO synthesis and an immunosuppressive effect with IC₅₀ values of 2.28 and 65.57 µM, respectively.

The Effect of Levamisole in Steroid-Dependent Nephrotic Syndrome in Children

PURPOSE: Long-term use of steroid, cyclophosphamide and cyclosporin, which are frequently used in the therapy of SDNS, might cause severe side effects. Recently, the immune-modulator levamisole has been tried as a substitute therapy and it has been reported as a method with less side effects and more effectiveness. We started this research in order to observe the effects of levamisole and compare it to other therapy results. PATIENTS AND METHODS: We chose 16 steroid dependent nephrotic syndrome children, those who had shown frequent relapse during the immunocompromised therapy period. Mean age was 9.1+/-.4 years in children and the male to female ratio was 15:1. All of subjects were diagonized with MCNS and had received cyclophosphamide or cyclosporin before receiving levamisole. Levamisole at a dose of 2.5mg/kg was used every other day for 1 year and the relapse rate was observed. RESULTS: On average of 14 days after treatment, complete remission was visible in all of the children, and the relapse percentage was 50%, which represents 8 children, while remaining 8 children representing 50% of the cases showed no relapse during treatment. During the levamisole therapy period, the average relapse rate was reduced significantly from 2.18+/-.9/year to 0.77+/-.9/year(p=0.027). Also the average relapse rate after the therapy was reduced to 1.34+/-.1/year, which was a significant level compared to the level before treatment(p=0.003). There was no significant difference in terms of duration of remission maintenance. Duration of remission maintenance showed an average of 12.2+/-.1 months before the use of levamisole, but it was also 10.1+/-.9 month after therapy. No other side effects such as leukopenia, skin disease and other clinically significant symptoms appeared at all during therapy. CONCLUSION: The long-term medication of levamisole for the therapy of SDNS children is thought to be able to maintain stable remission by reducing the relapse frequency without causing severe side effects. Further study with a broader range of subjects is required to eluccidate the long-term effects of this treatment.

The Effects of Intravenous Methylprednisolone Pulse Therapy by Mendoza Protocol in Primary and Secondary Nephrotic Syndrome

PURPOSE: Since Mendoza(1990)'s report that long term methylprednisolone pulse therapy by Mendoza protocol (MP therapy) is a good treatment option in focal segmental glomerulo -sclerosis(FSGS), there have been reports of the effects of this therapy in steroid-resistant nephrotic syndrome. However, no studies have been performed on the effects of MP therapy in steroid-dependent nephrotic syndrome and secondary nephrotic syndrome. In this study, we investigated the effects of long term MP therapy in primary and secondary nephrotic syndrome in which previous treatment options were not effective. METHODS: We chose 10 children who were diagnosed with steroid-dependent minimal change nephrotic syndrome(SD-MCNS), who had shown frequent relapse during the immunocompromised or cytotoxic therapy period, and 6 children with FSGS and 5 children with secondary nephrotic syndrome children, who had shown no response during the previous therapy period. We treated these patients according to Mendoza protocol involving infusions of high doses of methyl- prednisolone, often in combination with oral cyclophosphamide for 82 weeks. RESULTS: In all the 10 children with SD-MCNS, complete remission was visible on average of 18+/- days after MP therapy was started. However, all these children relapsed during or after MP therapy. In these children, the mean relapse rate prior to MP therapy was 2.1+/-.0 relpases/year, which was reduced to 1.4+/-.9 relapses/year during MP therapy(P>0.05) and rose to 2.7+/-.0 relapse/year after MP therapy. Of the 6 children with FSGS, 4 children(67%) showed complete remission, of whom 3 children(50%) remained in the remission status during the follow up period, 1.2+/-.7 years, after the end of MP therapy. 2 children(33%) showed no response. All of the 5 children with secondary nephrotic syndrome showed remission and remained in the remissiom status during the follow up period, 1.7+/-.6 years. The only side effect of MP therapy was transient hypertension in 10 children of all subjects during the intravenous infusion of methylprednisolone. CONCLUSION: We conclude that although long term MP therapy is not effective in the treatment of SD-MCNS, it is an effective therapy against intractable FSGS and secondary nephrotic syndrome.

A Case of Triple A Syndrome / 대한소아소화기영양학회지

Achalasia is very uncommon in children, and cases accompanied with alacrima and adrenal insufficiency is even more uncommon. When these three disorders are seen altogether, it is called triple A syndrome. It is inherited in an autosomal recessive manner and has potentially life-threatening sequelae. So, pediatricians should always consider the possibility of triple A syndrome when seeing children with achalasia. Neurological abnormalities such as autonomic neuropathy, peripheral neuropathy, sensory impairment and mental retardation occasionally accompany. We report a 2-year-old girl who presented with repeated vomiting, short stature and alacrima. Diagnosis of achalasia was made after perfoming esophagogram and endoscopy and was confirmed with esophageal manometry. After pneumatic dilatation, she became asymptomatic.