Experience of IVIG Treatment in an Overweight 14-year-old Child With Kawasaki Disease: A Case Report.

Kawasaki disease, or mucocutaneous lymph node syndrome, is an acute systemic vasculitis involving small and medium-sized vessels. It can be complicated by varying degrees of cardiac damage, especially coronary artery disease. The disease mainly occurs in children aged < 5 years, with rarer cases in older children and adults. Intravenous immunoglobulin combined with aspirin is the widely accepted treatment regimen in the acute phase, but the dosage recommended by the American Heart Association guidelines is not suitable for heavier children. This article reports the successful management of an overweight 14-year-old child with Kawasaki disease.

6-Shogaol alleviates CCl4-induced liver fibrosis by attenuating inflammatory response in mice through the NF-κB pathway.

Liver fibrosis is a global health problem caused by a number of diseases related to liver damage. 6-Shogaol is a biologically active substance derived from the rhizome of Zingiber officinale Roscoe with anti-tumor, anti-inflammatory, and antioxidant properties. To explore the effects of 6-Shogaol on liver fibrosis, we used a mouse model of the condition in which mice were injected intraperitoneally with carbon tetrachloride (CCl4) at a dose of 2 mL/kg three times per week for a period of 4 weeks. 6-Shogaol was administered orally at two different doses (5 mg/kg, 20 mg/kg) 30 min before CCl4 injection. CCl4 induced severe liver injury and fibrosis, as indicated by significant inflammatory cell infiltration, disordered liver structure, increased activities of aspartate aminotransferase and alanine aminotransferase (liver damage markers) in serum, elevated collagen deposition, and overexpressed alpha-smooth muscle actin (α-SMA, marker of hepatic stellate cells activation) in liver tissues, whereas 6-Shogaol administration rescued those alterations dose-dependently. We found that 6-Shogaol suppressed CCl4-induced inflammatory response by inhibiting macrophage recruitment, release of pro-inflammatory factors, and activation of the NOD-like receptor family pyrin domain containing 3 (NLRP3) inflammasome in liver tissues. Additionally, we demonstrated that 6-Shogaol blocked CCl4-induced activation of the nuclear factor-kappa B (NF-κB) pathway, which is a vital transcriptional regulator of the inflammatory response. Altogether, this study demonstrates that 6-Shogaol can prevent CCl4-induced liver fibrosis by suppressing inflammatory response through the NF-κB pathway and suggests that 6-Shogaol can be used for liver fibrosis prevention.

Ligustrazine Attenuates Liver Fibrosis by Targeting miR-145 Mediated Transforming Growth Factor-ß/Smad Signaling in an Animal Model of Biliary Atresia.

To investigate therapeutic target for ligustrazine during liver fibrosis in an ethanol-induced biliary atresia rat model and transforming growth factor-ß (TGF-ß) induced hepatic stellate cell activation cell model, and the underlying mechanism, a total of 30 rats were randomly assigned into five groups (n = 6 per group): control, sham, ethanol-induced biliary atresia model, model plus pirfenidone, and model plus ligustrazine groups. The liver changes were assessed using H&E and Masson staining and transmission electron microscopy. Expression of miR-145 and mRNA and protein levels of TGF-ß/smads pathway-related proteins were detected. HSC-T6 cells were infected with LV-miR or rLV-miR-145 in the presence or absence of SMAD3 inhibitor SIS3 and treated with 2.5 ng/ml TGF-ß1 and then with ligustrazine. Collected cells were subjected to detect the expression of miR-145 and mRNA and protein expression levels of TGF-ß/smads pathway-related proteins. Ligustrazine rescued liver fibrogenesis and pathology for ethanol-caused bile duct injury, revealed by decreased α-smooth muscle actin and collagen I expression and liver tissue and cell morphology integrity. Further experiments showed that ligustrazine inhibited intrinsic and phosphorylated Smad2/3 protein expression and modification. Similar results were obtained in cells. In addition, ligustrazine altered miR-145 expression in both animal and cell models. Lentivirus mediated miR-145 overexpression and knockdown recombinant virus showed that miR-145 enhanced the TGF-ß/Smad pathway, which led to hepatic stellate cell activation, and ligustrazine blocked this activation. This work validated that ligustrazine-regulated miR-145 mediated TGF-ß/Smad signaling to inhibit the progression of liver fibrosis in a biliary atresia rat model and provided a new therapeutic strategy for liver fibrosis. SIGNIFICANCE STATEMENT: With an ethanol-induced biliary atresia rat model, ligustrazine was found to rescue liver fibrogenesis and pathology for ethanol caused bile duct injury, revealed by decreased α-smooth muscle actin and collagen I expression and liver tissue and cell morphology integrity. Furthermore, we found ligustrazine upregulated miR-145 expression and inhibited TGF-ß/SMAD signaling pathway both in vivo and in vitro. In addition, overexpression and knockdown of miR-145 confirmed that miR-145 is involved in the ligustrazine inhibition of liver fibrosis through the TGF-ß/SMAD signaling pathway.

Congenital nephrogenic diabetes insipidus arginine vasopressin receptor 2 gene mutation at new site: A case report.

BACKGROUND: Congenital nephrogenic diabetes insipidus (CNDI) is a rare hereditary disorder. It is associated with mutations in the arginine vasopressin receptor 2 (AVPR2) gene and aquaporin 2 (AQP2) gene, and approximately 270 different mutation sites have been reported for AVPR2. Therefore, new mutations and new manifestations are crucial to complement the clinical deficiencies in the diagnosis of this disease. We report a case of a novel AVPR2 gene mutation locus and a new clinical mani-festation. CASE SUMMARY: We describe the case of a 48-d-old boy who presented with recurrent fever and diarrhea 5 d after birth. Laboratory tests showed electrolyte disturbances and low urine specific gravity, and imaging tests showed no abnormalities. Genetic testing revealed a novel X-linked recessive missense mutation, c.283 (exon 2) C>T (p.P95S). This mutation results in the substitution of a proline residue with a serine residue in the AVPR2 protein sequence. The diagnosis of CNDI was confirmed based on the AVPR2 gene mutation. The treatment strategy for this patient was divided into two stages, including physical cooling supplemented with appropriate amounts of water in the early stage and oral hydrochlorothia-zide (1-2 mg/kg) after a clear diagnosis. After follow-up of one and a half years, the patient gradually improved. CONCLUSION: AVPR2 gene mutations in new loci and new clinical symptoms help clinicians understand this disease and shorten the diagnosis cycle.

The safety and effectiveness of salvianolate in preventing perioperative venous thromboembolism in China: A PRISMA-compliant meta-analysis based on RCTs.

BACKGROUND: Salvianolate, a common drug for stabilizing heart disease and Angina Pectoris, is considered to be off-label for preventing venous thromboembolism (VTE) or anticoagulation at present. However, many clinical studies have showed that salvianolate can effectively inhibit the deep-vein thrombosis (DVT) incidence, and prevent VTE of perioperative patients in the real world in China. OBJECTIVE: This analysis aimed to evaluate the effectiveness and safety of salvianolate in preventing VTE in perioperative patients. METHODS: Databases of PubMed, Cochrane Library, Embase, CNKI, Wanfang and VIP were searched until July 2019. Literature retrieval, data extraction and quality assessment were independently completed by two researchers and checked with each other. Review Manager 5.2 software was applied for meta-analysis. RESULTS: A total of 429 studies were retrieved, including 11 randomized controlled trials (RCTs) with a total of 1149 subjects. Compared with low molecular weight heparin (LMWH) group alone, salvianolate combined LMWH group had lower DVT incidence in preventing perioperative thrombosis (2.75% and 14.23%, OR: 0.21, 95% CI:[0.08,0.53]; P = .0009). The incidence of adverse reactions of experimental group was similar to that of control group (1.79% and 2.31%, OR: 0.65, 95% CI:[0.18,2.35]. P = .51). Compared with the control group, D-dimer level (D-D), platelet count (PLT), fibrinogen (FIB), whole blood high shear viscosity (WBHSV), and whole blood low shear viscosity (WBLSV) were all significantly decreased (P < .01), and prothrombin time (PT) was significantly increased (P < .05). CONCLUSION: Salvianolate combined LMWH has better effectiveness and the same safety in preventing venous thromboembolism in perioperative patients. However, due to the small number of included literatures, large sample studies are still needed to further verify this conclusion.

Efficacy and safety of azithromycin combined with glucocorticoid on refractory Mycoplasma pneumoniae pneumonia in children: A PRISMA-compliant systematic review and meta-analysis.

INTRODUCTION: The aim of this study was to evaluate the efficacy and safety of azithromycin (AZI) combined with glucocorticoid (GC) in the treatment of children with refractory Mycoplasma pneumoniae. METHODS: Computer search for PubMed, EMbase, Cochrane Library, China Biomedical Literature Database (CBMdisc), China Knowledge Network (CNKI), Wanfang, VIP (VIP), and a randomized controlled trial (RCT) of AZI combined with GC in the treatment of children with refractory Mycoplasma pneumoniae pneumonia test (RCT), the search time limit is built until March 20, 2019. Two researchers independently performed literature screening, data extraction, and literature risk bias, and meta-analysis was performed using RevMan 5.3 software. RESULTS: A total of 12 RCTs were included, including 1130 patients. Meta-analysis showed that AZI combined with GC therapy significantly improved the total effective rate of the disease compared with the conventional treatment group (odds ratio [OR] = 6.37; 95% confidence interval [CI] 4.03, 10.07; P < .00001; I = 0%), effectively shortened the antipyretic time (SMD = -2.29; 95% CI -2.70, -1.88; P < .0001); promoted lung inflammation absorption (SMD = -1.89; 95% CI -2.38, -1.40; P < .0001), reduced cough time (SMD = -2.39; 95% CI -2.80, -1.99; P < .0001); shortened hospital stay (SMD = -2.19; 95% CI -3.21, -1.17; P < .0001); improved imaging findings (OR = 5.38; 95% CI 1.09, 26.51, P = .04); reduced inflammation index (SMD = -3.15; 95% CI -4.93, -1.36; P = .004); improved immune function (SMD = 1.29; 95% CI -0.02, 2.60; P < .0001); had no significant adverse reactions (OR = 1.18; 95% CI 0.71, 1.98; P = .53). CONCLUSIONS: According to the current limited research evidence, the addition of GCs to the conventional treatment of refractory Mycoplasma pneumoniae in children can improve the clinical efficacy to a certain extent, and the safety is better. However, due to the quality and quantity of the included literature, the conclusions of this study need to be confirmed by more high-quality studies.

Effect of combined ursodeoxycholic acid and glucocorticoid on the outcome of Kasai procedure: A systematic review and meta-analysis.

INTRODUCTION: Multiple studies have investigated the effect of ursodeoxycholic acid (UDCA) or glucocorticoid (GC) on the outcome of the hepatoportoenterostomy (Kasai procedure) in patients with biliary atresia (BA). However, the combined effect of these drugs (UDCA + GC) is little understood. METHODS: This meta-analysis specifically evaluated the effect of UDCA + GC after the Kasai procedure in patients with BA. A comprehensive literature search was conducted for all relevant articles in the electronic databases Medline, PubMed, Cochrane, Excerpta Medica Database (EMBASE), China National Knowledge Infrastructure (CNKI), Chinese Biomedical Literature Database on Disc (CBM-disc), and Vendor Information Pages (VIP). RESULTS: Eight studies with BA patients were finally included in our meta-analysis. The 8 identified studies consisted of 3 case-control, 3 cohort, and 2 randomized controlled trials (RCTs) with overall 530 subjects (144, 152, and 234 subjects, respectively). Among them, 312 patients were treated with UDCA + GC, while 218 received placebo or other intervention. The meta-analysis indicated that groups that received UDCA + GC had significantly lower rates of postoperative jaundice relative to the controls (pooled, odds ratio [OR] = 2.41; 95% confidence interval [CI] 1.44-4.04; Z = 3.34; P = .0008), while rates of cholangitis were similar (pooled, OR = 0.87; 95% CI 0.43-1.74; Z = 0.40; P = .69). CONCLUSIONS: Combined UDCA and GC intervention was superior to that of the control in accelerating the clearance of serum bilirubin in patients with BA after the Kasai procedure. However, this conclusion requires further confirmation using RCTs of high methodological quality.

[Effect of Jiawei Yinchen Sini Decoction on the Production of Smad7 and Collagenâ ,â ¢ in Carbon Tetrachloride Induced Hepatic Fibrosis Model of Mice].

Objective: To explore the preventive effects of Jiawei Yinchen Sini Decoction on the gene expression of Smad7 and Collagen,Ⅲ in mice with hepatic fibrosis. Methods: 48 male ICR mice were randomly divided into normal group, model group, colchicine group, JWYSN high dose group, medium dose group and low dose group, with 8 mice in each group. The hepatic fibrosis model was established by intraperitoneal injection of 30% CCl4( 1. 5 mg/kg, dissolved in olive oil). At the same time, the mice were treated with intragastric adminstration of drugs. After 14 days, the liver index was calculated; the levels of serum hyaluronic acid( HA) and laminin protein( LN) were detected by Enyme-linked immunosorbent assay; the pathological fibrosis change was observed by performing HE staining and was made a grading of hepatic fibrosis. mRNA expression of Smad7 and Collagen I, Ⅲ were detected by RT-q PCR analysis. Results: (1) Compared with model group, the liver index and levels of HA and LN in JWYSN medium dose group and low dose group were obviously decreased( P < 0. 05).( 2) The pathological results showed that, compared with model group, the hepatic fibrosis were significantly relief in different dose of JWYSN group( P < 0. 05).( 3) The expressions of Collagen Ⅰ mRNA in each treatment group were significantly lower than model group( P < 0. 05); In addition to JWYSN high dose group, the expressions of Smad7 mRNA in each treatment group increased significantly( P < 0. 05); In addition to JWYSN low dose group, the expression of CollagenⅢmRNA in each treatment group decreased significantly( P < 0. 05). Conclusion: Jiawei Yinchen Sini decoction can up-regulate the expressions of Smad7 mRNA and inhibit expressions of mRNA and CollagenⅢmRNA in mice with hepatic fibrosis,thus attenuate the development of hepatic fibrosis.

[Risk factors for hypertension and impact of postoperative hypertension on medium survival after heart transplantation].

OBJECTIVE: To access the prevalence and risk factors for hypertension after heart transplantation (HT), and the impact of post-transplant hypertension on medium-term survival among HT patients. METHODS: Data from 265 consecutive patients underwent HT between June 2004 and May 2012 in Fuwai hospital and survived for at least 6 months were retrospectively analyzed. Hypertension was defined as systolic pressure ≥ 140 mm Hg (1 mm Hg = 0.133 kPa) and/or diastolic pressure ≥ 90 mm Hg or current treatment with antihypertensive drugs. Patients were divided into post-HT hypertension group and non-hypertension group. Logistic regression analysis was used to determine preoperative and postoperative risk factors for hypertension after HT. Kaplan-Meier method and log rank test were used for survival analysis. RESULTS: Hypertension was present in 17.4% (46/265) patients before HT and in 57.4% (152/265) patients post HT. The median follow-up time was 37 months (20 - 57 months). Logistic regression analysis showed that male gender (OR: 2.27, 95%CI: 1.16 - 4.42, P < 0.05), history of pre-HT hypertension (OR: 2.22, 95%CI: 1.05 - 4.71, P < 0.05), and cyclosporine A based immunosuppressive therapy (OR: 2.54, 95%CI: 1.51 - 4.29, P < 0.01) were independent risk factors for the development of post-HT hypertension. At the end of 1, 3, 5 years, the survival rate of heart transplant patients by Kaplan-Meier method estimation were 100%, 97.2%, 86.7% in post-HT hypertension group; 98.1%, 93.8%, 93.8% in non-hypertension group. Log rank test displayed that there was no significant difference between the two survival curves (P > 0.05). CONCLUSIONS: Hypertension is a frequent comorbidity after HT. Male gender, pre-HT hypertension together with cyclosporine A based immunosuppressive therapy are independent predictors for the development of post-HT hypertension. By adjusting the controllable risk factors and active control of blood pressure, the medium-term survival is similar between patients with or without postoperative hypertension in this cohort.

Combination therapy with angiotensin-converting enzyme inhibitors and indapamide impairs glucose tolerance in Chinese hypertensive patients.

BACKGROUND: Elevated blood glucose (BG) induced by antihypertensive agents increases the risk of cardiovascular events. This study was designed to investigate whether fosinopril+indapamide combination therapy has any effect on glucose tolerance (GT), and if it did, whether conversion to fosinopril alone could reverse the impaired GT. METHODS: Included in the present study were 124 hypertensive patients, of whom 62 patients were treated with fosinopril plus indapamide (F/I group) and the remaining 62 patients were treated with fosinopril alone (F group). Of them, 89 patients completed a mean of 14-month follow-up. In the F/I group, 29 patients were converted to the use of fosinopril for 4-12 months after they completed the follow-up. RESULTS: In the F group, fasting BG decreased significantly from 5.1+/-0.5 to 4.8+/-0.7 mmol/l (p<0.01), and 2-h postprandial BG decreased significantly from 7.2+/-1.6 to 6.4+/-1.4 mmol/l (p<0.01), while in the F/I group, fasting BG increased significantly from 5.1 +/-0.6 to 5.3+/-0.9 mmol/l (p<0.05), and 2-h postprandial BG increased significantly from 7.2+/-1.7 to 7.7+/-1.8 mmol/l (p<0.05). In 29 patients of the F/I group who completed the follow-up and were converted to fosinopril, fasting BG decreased significantly from 5.5+/-1.0 to 5.3+/-1.0 mmol/l (p<0.05), and 2-h postprandial BG decreased significantly from 7.5+/-2.0 to 7.0+/-2.7 mmol/l (p<0.05). CONCLUSION: Fosinopril+indapamide combination therapy impaired GT in Chinese hypertensive patients, and fosinopril alone was able to reverse fosinopril+indapamide-induced GT impairment in part of these patients.

Effect of metformin on weight gain during antihypertensive treatment with a beta-blocker in Chinese patients.

BACKGROUND: Overweight and obesity are associated with cardiovascular disease (CVD). This study was designed to investigate whether combined use of nitrendipine and atenolol has any effect on body weight (BW) and whether metformin can prevent antihypertensive medication-induced weight gain and has any effect on blood glucose (BG). METHODS: Included in the present study were 94 hypertensive patients with a body mass index (BMI) > or =25 kg/m(2), of whom 45 patients were treated with nitrendipine plus atenolol (N/A group), and the remaining 49 patients were treated with nitrendipine, atenolol, and metformin (N/A/M group). The mean follow-up duration was 14 months. BW and glucose tolerance were measured. RESULTS: In N/A group, BW and fasting BG significantly increased from 73.5 +/- 9.6 kg to 74.2 +/- 9.7 kg (P < 0.05) and from 94.2 +/- 10.5 mg/dl to 97.9 +/- 11.3 mg/dl (P < 0.01), respectively, whereas postprandial BG did not change significantly. In N/A/M group, BW slightly decreased from 72.7 +/- 10.1 kg to 72.3 +/- 10.2 kg (P = 0.30), and fasting BG did not change significantly (93.5 +/- 10.4 mg/dl vs. 92.7 +/- 10.2 mg/dl, P = 0.59), whereas 2-h postprandial BG significantly decreased from 133.7 +/- 30.5 mg/dl to 124.0 +/- 29.6 mg/dl (P < 0.05). Furthermore, a significant difference was observed in difference value of BW before and after treatment between the two groups (0.7 (95% confidence interval, 0.1-1.3) kg in N/A group vs. -0.4 (95% confidence interval, -1.3 to 0.4) kg in N/A/M group, P < 0.05). CONCLUSIONS: Combination therapy of nitrendipine and atenolol may significantly increase BW and fasting BG in overweight or obese patients with hypertension. Metformin may prevent BW gain and improve BG levels in hypertensive patients who received combination therapy of nitrendipine and atenolol.