Genetic, Clinical, and Pathologic Backgrounds of Children With X-Linked Alport Syndrome in China: A Monocenter Study.

Background. Characteristics of X-linked Alport syndrome (XLAS) in a cohort of Chinese children. Methods. This work is a retrospective study covering the clinical information, pathological data, and gene sequencing results of 32 cases with XLAS from 2011 to 2022. Results. Among these 32 patients, the youngest age of onset was 3 months. Renal biopsy was performed on 29 children. The lamellated glomerular basement membrane was observed in 19 children using electron microscopy (65.5%). Of the 26 samples tested, 73.1% were found to be negative for collagen-a5 under immunohistochemical staining, showing clinical significance. Next-generation sequencing (NGS) detected 27 pathogenic gene mutations. A total of 15.4% of patients carried de novo mutations. Conclusions. The boys with XLAS showed more typical pathological performance than the girls. Patients with severe mutation were more likely to have proteinuria and hearing impairment. Renal pathology combined with NSG is an important means of diagnosis of AS.

Analysis of effect of oil and S2- impurities on corrosion behavior of 16Mn steel for storage tanks by electrochemical method.

Corrosion is a common problem of storage tanks, and different storage media and impurities have different effects on the corrosion behavior of steel used for tanks. In this paper, electrochemical impedance spectroscopy (EIS) and polarization curves were used to study the corrosion behavior of 16Mn steel in 3.5% sodium chloride solution after immersion in heavy oil, diesel oil and gasoline, considering the influence of the main impurities, S2-, on the corrosion process. The results showed that the self-corrosion current density of 16Mn steel in the solution after the immersion in gasoline was higher than that in heavy oil and diesel oil, due to the higher dissolved oxygen concentration promoting the cathodic reaction. The comparison of blank sample in 3.5% sodium chloride solution and the immersed samples showed that the corrosion rate of the 16Mn steel decreased in the presence of an oil film on the surface of the steel. With the increase of S2- concentration in the oil, the corrosion rate of 16Mn steel tended to be stable after it gradually increased. This was mainly related to the composition of FeS and FeS1-x in the sulfide film formed on the surface of the steel as well as the change of the lattice structure.

Comparative effect of electroacupuncture and moxibustion on the expression of substance P and vasoactive intestinal peptide in patients with irritable bowel syndrome.

OBJECTIVE: To compare the impacts of electroacu puncture (EA) and moxibustion (Mox) on the prima ry gastrointestinal symptoms and the expressions of colonic mucosa-associated neuropeptide substance P (SP) and vasoactive intestinal peptide (VIP) in patients with either diarrhea-predominant or constipation-predominant irritable bowel syndrome (IBS-D and IBS-C, respectively). METHODS: Eighty-five IBS patients were randomly allocated to the EA and Mox groups. Zusanli (ST 36) and Shangjuxu (ST 37) were selected as acupoints for electroacupuncture or warm moxibustion treatment once a day for 14 consecutive days. Before and after the treatment sessions, a Visual Analog Pain Scale and the Bristol Stool Form Scale were used to evaluate gastrointestinal symptoms. There were four dropout cases, leaving 81 participants (41 with IBS-D and 40 with IBS-C) who volunteered to undergo colonoscopy before and after the treatment sessions. During colonoscopy, sigmoid mucosa were collected to detect SP and VIP expression using immunohistochemistry assay. RESULTS: Both EA and Mox treatments were effective at relieving abdominal pain in IBS-D and IBS-C patients. However, Mox was more effective at reducing diarrhea in IBS-D patients, whereas EA was more effective at improving constipation in IBS-C patients. EA and Mox treatments both down-regulated the abnormally increased SP and VIP expression in the colonic mucosa, with no significant difference shown between the two treatments. CONCLUSION: Both EA and Mox treatments are effective at ameliorating gastrointestinal symptoms by reducing SP and VIP expression in the colonic mucosa of IBS patients.

miR-495 mediates metabolic shift in glioma cells via targeting Glut1.

Cancer cell metabolism is often characterized by a shift from an oxidative to a glycolytic bioenergetics pathway, a phenomenon known as the Warburg effect. Whether the deregulation of microRNAs contributes to the Warburg effect remains largely unknown. Here, we show that miR-495 expression is decreased and thus induces a metabolic shift in glioma cells. miR-495 performs this function by increasing the expression of Glut1, leading to the increase of glucose uptake and lactate production. The altered metabolism induced by miR-495 results in the rapid growth of cancer cells. These results identify miR-495 as a molecular switch involved in the orchestration of the Warburg effect in glioma cells via targeting the expression of Glut1.

Effects of Selenium-Enriched Protein from Ganoderma lucidum on the Levels of IL-1 ß and TNF- α , Oxidative Stress, and NF- κ B Activation in Ovalbumin-Induced Asthmatic Mice.

The purpose of this study was to compare the effect and toxicity of organic selenium (Pro-Se) with inorganic selenium (IOSe) in preventing asthma in ovalbumin-induced asthmatic mice. After the mice were treated orally with Pro-Se and IOSe, respectively, the plasma Se levels, Se accumulation in liver and kidney, tumor necrosis factor alpha (TNF- α ), interleukin 1 beta (IL-1 ß ), oxidative stress, and NF- κ B activation in lung were examined. The results showed that the serumal Se levels in the mice fed the Pro-Se were significant (P < 0.01) elevations. It results in restoration of the level of endogenous antioxidant enzyme, lower levels of TNF- α and IL-1 ß , and activated NF- κ B in the asthmatic mice. Our experiments have demonstrated profound differences between the activities of organic selenium and inorganic selenium in experimental conditions. These data provide an important proof of the concept that organic selenium might be a new potential therapy for the management of childhood asthma in humans.

Pharmacological and pharmacokinetic studies with vitamin D-loaded nanoemulsions in asthma model.

Vitamin D (VD) was studied for its anti-inflammatory activities with prepared VD-loaded nanoemulsions (VDNM) in ovalbumin-induced asthmatic mice in this paper. In this study, we prepared VDNM for the delivery of VD from the established composition of solid self-emulsifying drug delivery systems (sSEDDS) by spray-drying technique and evaluated its bioavailability (BA) and anti-inflammatory activities in experimental allergic asthma. After the mice were treated orally with VD or VDNM, the plasma 25(OH) D levels, polymorphonuclear cells, tumor necrosis factor alpha (TNF-α), interleukin 1 beta (IL-1ß), total antioxidant activity, and C3 and C4 complement protein levels were studied, respectively. Treatment with VDNM reduced MPO activity, oxidative stress, C3 protein level, O2(-) level as well as the production of IL-1ß and TNF-α. Pharmacokinetic studies showed that a significant increase in the maximum concentration (Cmax) and AUC0→24 h were observed in VDNM group when compared with VD group (P < 0.01). The result revealed that VDNM led to an improvement in oral BA of VD in a murine ovalbumin-induced asthma model. These data provided an important proof that VDNM might be a new potential therapy for the management of asthma in humans.

Relationship between serum aminotransferase levels and metabolic disorders in northern China.

BACKGROUND/AIMS: Increasing evidence suggests an association between elevated serum aminotransferase levels and metabolic disorders (metabolic syndrome, hyperlipidemia and diabetes mellitus). However, the significance of relatively low levels of aminotransferases in relation to metabolic disorders has not been fully investigated in the general population. We investigated the association between serum aminotransferase levels and metabolic disorders using data from a survey in Jilin Province, China. MATERIALS AND METHODS: In 2007, a prospective survey was conducted throughout Jilin, China, covering both urban and rural areas. A total of 3835 people, 18-79 years old, were undergoing real-time ultrasonography, blood tests, and interviews with a structured questionnaire. RESULTS: Serum aminotransferase levels within the normal range were associated with metabolic syndrome independent of age, occupation, cultural and educational level, income, body mass index, waist circumference, smoking, and alcohol intake. Compared with the lowest level (< 20 IU/L), the adjusted odds ratios for alanine aminotransferase levels of 20-29, 30-39, 40-49, and >50 IU/L were 1.92, 2.50, 2.97, and 3.52 in men, and 1.38, 1.54, 3.06, and 2.62 in women, respectively. Near-normal serum aminotransferase levels associated with hyperlipidemia, non-alcoholic fatty liver disease, and diabetes mellitus were also found in the study. CONCLUSIONS: Normal to near-normal serum aminotransferase levels are associated with metabolic disorders. Serum alanine aminotransferase levels of 21-25 IU/L for men and 17-22 IU/L for women are suggested as cut-off levels that detect metabolic disorders affecting the liver.

Mechanism governing reversal of multidrug resistance in human breast carcinoma cells by chelerythrine.

OBJECTIVE: To explore the mechanism governing the reversal of multidrug resistance in human breast carcinoma cells by chelerythrine. METHODS: Reverse transcription-polymerase chain reaction (RT-PCR) and Western blot were used to determine the expressions of protein kinase Cα (PKCα) and multidrug resistance-related genes ABCG2, ABCC1, MDR1, and P-glycoprotein (P-gp) in MCF-7Taxol cells after treatment with chelerythrine and phorbol-12-myristate-13-acetate (PMA). Also, the antitumor effect of PMA or chelerythrine and effects of PKCα activator or inhibitor in combination with paclitaxel or adriamycin on multidrug resistance in MCF-7Taxol cells were evaluated by MTT. RESULTS: RT-PCR or Western blot showed that the expressions of MDR1 and P-gp were significantly higher in MCF-7Taxol cells exposed to PMA stimuli (both P0.05). CONCLUSION: PKCα inhibitor chelerythrine can reverse multidrug resistance in breast carcinoma cells by inhibiting the expressions of MDR1 and P-gp expression in vitro.

The effect of amiodarone on cardiac rhythm after cardiopulmonary bypass in patients with atrial fibrillation.

BACKGROUND AND AIM OF THE STUDY: The intraoperative use of amiodarone in patients with atrial fibrillation (AF) undergoing valvular heart surgery was unclear. Hence, a randomized clinical trial was designed to evaluate the effect of amiodarone on cardiac recovery and rhythm following mitral valve surgery. METHODS: A total of 120 elective mitral valve replacement patients with AF received either amiodarone (5 mg/kg) or the same volume of saline during cardiopulmonary bypass (CPB), in a randomized manner. Cardiac recovery was assessed by the heart auto re-beat ratio, the time from reperfusion to heart beat, the post-reperfusion rhythm, pacemaker use, and vasoactive drug use. RESULTS: A higher heart auto re-beat ratio, higher sinus rhythm ratio, lower electric defibrillation ratio and less dopamine usage were found in the amiodarone treatment group. However, the proportion of temporary pacemaker use was lower in the control group. CONCLUSION: Amiodarone had a good effect on cardiac recovery and the rhythm of AF patients after CPB.