Oral Coenzyme Q10 supplementation leads to better preservation of kidney function in steroid-resistant nephrotic syndrome due to primary Coenzyme Q10 deficiency.

Primary Coenzyme Q10 (CoQ10) deficiency is an ultra-rare disorder caused by defects in genes involved in CoQ10 biosynthesis leading to multidrug-resistant nephrotic syndrome as the hallmark kidney manifestation. Promising early results have been reported anecdotally with oral CoQ10 supplementation. However, the long-term efficacy and optimal prescription remain to be established. In a global effort, we collected and analyzed information from 116 patients who received CoQ10 supplements for primary CoQ10 deficiency due to biallelic pathogenic variants in either the COQ2, COQ6 or COQ8B genes. Median duration of follow up on treatment was two years. The effect of treatment on proteinuria was assessed, and kidney survival was analyzed in 41 patients younger than 18 years with chronic kidney disease stage 1-4 at the start of treatment compared with that of an untreated cohort matched by genotype, age, kidney function, and proteinuria. CoQ10 supplementation was associated with a substantial and significant sustained reduction of proteinuria by 88% at 12 months. Complete remission of proteinuria was more frequently observed in COQ6 disease. CoQ10 supplementation led to significantly better preservation of kidney function (5-year kidney failure-free survival 62% vs. 19%) with an improvement in general condition and neurological manifestations. Side effects of treatment were uncommon and mild. Thus, our findings indicate that all patients diagnosed with primary CoQ10 deficiency should receive early and life-long CoQ10 supplementation to decelerate the progression of kidney disease and prevent further damage to other organs.

Analysis of Eight Nutrient Elements in Whole Blood of Children and Adolescents Using Inductively Coupled Plasma-Mass Spectrometry.

Few researches have been conducted on elements in whole blood of young people. Our study was to investigate the influence of age, gender and season on the contents of magnesium (Mg), calcium (Ca), iron (Fe), copper (Cu), zinc (Zn), manganese (Mn), selenium (Se), and strontium (Sr) as well as to establish reference intervals (RIs). We conducted a retrospective study of 589 apparently healthy children and adolescents. Quantitative analysis had been carried out using inductively coupled plasma-mass spectrometry (ICP-MS). Test results were analyzed using and MannWhitney U test, Spearman and Pearson statistical analyses. RIs were defined by using 95% confidence interval. Differences between contents of Mg, Fe, Cu, and Zn in girls' and boys' whole blood were found. Positive correlations for Fe, Zn, Se, and Sr, while negative for Ca and Cu were found with age. Increasing trends were found for Fe, Zn, and Se, while for Ca and Cu, changes were even decreasing for children and teenagers. The most frequently correlating element pairs were FeZn, MgSe, and FeSe in five successive age groups. Lower contents of Mg, Ca, Fe, Zn, and Se were found in summer. Finally, the reference interval of each element was initially established according to age and gender grouping. The contents of elements in whole blood vary depending mainly on the gender and age of children and adolescents. The reference intervals of elements in whole blood grouped by age and gender provide a reference basis for clinical diagnosis and treatment of element-related diseases.

[Exploration of academic thoughts on treating myelodysplastic syndrome with combination of disease and syndrome by Prof. Ma Rou].

The diagnosis and treatment pattern using combination of disease and syndrome, fully developing the advantages of both traditional Chinese medicine (TCM) and Western medicine (WM) and being widely used clinically, has been constructed in the long history of TCM. Prof. MA Rou, as a hematology specialist of integrative medicine (IM), uses modern medical equipment to diagnose diseases and takes traditional Chinese medical methods to treat diseases. He is loyal to TCM sciences and refers to the advantages of WM. He holds the essence of MDS lies in toxic stasis according to its pathogenic features. He detoxifies and removes stasis using Qinghuang Powder. Meanwhile, according to patients' clinical manifestations, he summarized two common syndrome types, Pi-Shen yang deficiency syndrome and Gan-Shen yin deficiency syndrome. Better efficacy could be achieved by combining Chinese herbs for tonifying Pi-Shen. In recent years the application of Qinghuang Powder won some achievements in clinical study and experimental study, thus providing scientific reliance for Prof. MA Rou's academic thought on treating MDS.

Clinical observation of the treatment of myelodysplastic syndrome mainly with Qinghuang Powder.

OBJECTIVE: To observe the clinical effectiveness of Qinghuang Powder (QHP) combined with Bupi Yishen Decoction (, BPYS) in treating myelodysplastic syndrome (MDS), and its relationship with France, America, and Britain (FAB) type, international prognosis scaling system (IPSS) risk, and chromosome karyotype. METHODS: There were 124 MDS patients subjected to the tests. By FAB typing, 91 patients were typed as refractory anemia (RA) type and 33 as refractory anemia with excess of blasts (RAEB) type; by IPSS scale, 21 were sorted to low risk, 77 to moderate risk I, 20 to moderate risk II, and 6 to high risk; 78 of them had normal chromosome and 46 with abnormal chromosome, including 26 of trisomy 8. All patients were treated with QHP+BPYS, and the changes of peripheral blood figure and bone marrow were observed. RESULTS: After treatment, the general effective rate was 72.58% (90/124), which in the patients of RA type was 80.22% (73/91) and in RAEB type 51.52% (17/33). The former was better than that in the later (P<0.01). For the analysis in the patients of different IPSS risk degrees, the effective rate was 95.24% (20/21) in the lowrisk group, 72.73% (56/77) in moderate risk I, 65.00% (13/20) in moderate-risk II, and 16.67% (1/6) in high-risk group. Those in the first two groups were superior to that in the latter two (P<0.01). The effective rate was 79.49% (61/78) in the patients with normal chromosome and was 60.87% (28/46) in the patients with abnormal chromosome, showing a significant difference between them. While in the patients of trisomy 8, it was 73.08% (19/26), which was parallel to that in the patients with normal chromosome. CONCLUSION: The effectiveness of QHP+BPYS comprehensive therapy for MDS is unquestionably good, and it is markedly correlated with the FAB type and IPSS risk degree of the disease, as well as the normality of chromosome in the patient.

Effect of Yiqi Bushen Granule (益气补肾颗粒) on the peripheral natural killer cell and γ δ T-cell in the patients with minimal residual leukemia.

OBJECTIVE: To analyze the changes in peripheral natural killer T-cells (NKT) and gammadelta T-cells (γ δ T-cell) in patients with minimal residual leukemia (MRL) before and after being treated with Yiqi Bushen Granule (益气补肾颗粒, YBG) in order to determine their significance in prognosis of the disease. Granule (益气补肾颗粒, YBG) in order to determine their significance in prognosis of the disease. METHODS: Before and after treatment, the changes in 36 patients (16 males and 20 females) receiving long-term (more than 3 months) YBG therapy were analyzed using multi-parameter flow cytometry, with 34 healthy persons (19 males and 15 females) acting as controls. males and 15 females) acting as controls. RESULTS: The absolute value and percentage of NKT cells and γ δ T-cells were all significantly raised after treatment, for NKT cells, 0.52%±0.39% to 0.83%±0.66% and 7.25±7.77 cells cell/µL to 12.86±11.99 cell/µL, for γ δ T-cells, 6.08%±3.03% to 7.24%±2.78% and 83.97±48.09 cell/µL 110.53±54.12 cell/µL, respectively (P<0.05 or P<0.01). CONCLUSION: YBG could regulate the immune function and elevate the amount of NKT cells and γ δ T-cells, thus to kill or suppress the residual leukemic cell in the body, which might be one of the mechanisms of YBG in prolonging the disease-free survival in MRL patients.

Kinetics of transesterification of palm oil and dimethyl carbonate for biodiesel production at the catalysis of heterogeneous base catalyst.

The transesterification of palm oil with dimethyl carbonate (DMC) for preparing biodiesel has been studied in solvent-free system at the catalysis of potassium hydroxide (KOH) as heterogeneous catalyst. Fatty acid methyl esters (FAMEs) were analyzed by GC with internal standard method. The effects of reaction conditions (molar ratio of DMC and palm oil, catalyst amount and time) on FAMEs yield were investigated. The highest FAMEs yield could reach 96.2% at refluxing temperature for 8h with molar ratio of DMC and oil 9:1 and 8.5% KOH (based on oil weight). Kinetics of the KOH-catalyzed transesterification of palm oil and DMC was researched over a temperature range of 65-75 degrees C. A pseudo first-order model was proposed. The activation energy (E(a)) was 79.1 kJ mo1(-1) and the pre-exponential factor (k(o)) was 1.26 x 10(9) min(-1) from Arrhenius equation. Further, a plausible reaction mechanism for the catalytic process with DMC as acyl acceptor was proposed.

Intervention of Tongxinluo capsule against vascular lesion of atherosclerosis and its effect on lectin-like oxidized low density lipoprotein receptor-1 expression in rabbits.

OBJECTIVE: To investigate the prevention by Tongxinluo capsule (TXL) of vascular lesions and its effect on the levels of protein and gene expression of lectin-like oxidized low-density lipoprotein receptor-1 (LOX-1) of vascular wall in rabbits with atherosclerosis (AS), and to explore its possible mechanism against AS. METHODS: AS models were established by feeding New Zealand white rabbits with high-cholesterol diet, and 24 immature rabbits were randomly divided into the control group, model group and treated group (treated with TXL capsule). The indexes of total cholesterol (TC) and low density lipoprotein (LDL) levels were measured at the 16th week. The intima thickness and the plaque area of abdominal aorta were quantitatively analyzed by pathological morphological analysis, the expression of macrophage and smooth muscle cell (SMC) in intima were detected by immunohistochemical method and histologic segments were stained by Hematoxilin-Eosin (HE) to identify the degree of atherosclerotic lesion in the model group and the prevention by TXL. The LOX-1 gene and protein expression in abdominal aorta was detected by semi-quantitative RT-PCR and immunohistochemistry, respectively. RESULTS: In the model group, the levels of TC and LDL were significantly elevated, aortic intima thickened extensively, the intima area enhanced, and macrophages expression increased; the levels of LOX-1 gene and protein expression was up-regulated in endothelium and neo-intima of the abdominal aorta. The treatment with TXL reduced blood lipids, attenuated arterial intimal proliferation, markedly inhibited the expression of macrophage and excessively expressed the level of LOX-1. CONCLUSION: TXL has an inhibitory effect on blood lipids, and it can prevent the occurrence of vascular lesion and cure its development, and its protection against AS was possibly associated with a crucial endothelial protective action through lowering the expression of LOX-1 in vascular walls.

[Effect of tongjingning granule on primary dysmenorrhea].

OBJECTIVE: To study the mechanisms of tongjingning granule (TJN) on primary dysmenorrhea (PD). METHODS: One hundred and twenty PD patients were treated with TJN, and 40 patients treated with aspirin as controls. The levels of serum estrogen (E2) and progestin (P) content in the luteal metaphase and anaphase (MI/AI) as well as endothelin (ET) and calcitonin gene related peptide (CGRP) in the metaphase and menstrual stage before and after treatment in partial patients were determined. RESULTS: The therapeutic effect , and the curative markedly-effective rate and improvement rate of main accompanied symptoms of severe and moderate PD patients in the treated group were superior than those in the control group (P < 0.01). The level of E2 and ET were significantly decreased, and the content of P and CGRP were significantly increased in the treated group after treatment respectively (P < 0.01). CONCLUSION: JTN not only can regulate ovarian hormone, ET and CGRP, but shows advantages in adjusting spirits and emotions, improving homeostasis and consolidating the therapeutic effect.