Left ventricular noncompaction(LVNC)describes a rare cardiomyopathy of unknown etiology characterized by the presence of prominent trabeculations and deep intertrabecular recesses.It is one of the three major cardiomyopathies among children.LVNC can have different inheritance patterns, including X-linked recessive, autosomal dominant, and mitochondrial inheritance.Mutations in genes encoding sarcomere or cytoskeletal proteins have been identified as causes of LVNC.As a comprehensive cardiac event with serious complications such as life threatening arrhythmias, heart failure and thromboembolism, LVNC is mainly diagnosed through echocardiography, cardiac magnetic resonance and other examinations.Early improvement of genetic evaluation, screening of family members for the mutation, close follow-up of high-risk patients, and early management of complications will help to improve patients′ quality of life and prognosis.

Drugs, Chinese Herbal ; therapeutic use ; Humans ; Indoles ; therapeutic use ; Liver Neoplasms ; drug therapy ; Male ; Middle Aged ; Pyrroles ; therapeutic use

Bronchiolitis ; epidemiology ; virology ; Case-Control Studies ; Child, Preschool ; China ; epidemiology ; DNA, Viral ; Female ; Human bocavirus ; isolation & purification ; Humans ; Infant ; Male ; Metapneumovirus ; isolation & purification

Objective To investigate the effects and the mechanism of telmisartan and pyridoxamine on oxidative stress in spontaneously hypertensive rats(SHR).Methods SHRs(male,20 weeks of age) were randomly divided into four groups (n＝ 12 for each):hypertension control (HC) group (2 ml of distilled water),telmisartan group[T,6 mg/(kg · d)],pyridoxamine group[P,200 mg/(kg · d)]and combined group(TP,6 mg/kg telmisartan and 200 mg/kg pyridoxamine per day).Treatments were continued for 16 weeks.The normal control group included 13 WKY rats and received gastric lavage with distilled water.SBP of tail artery was measured during the intervention ervey 2 weeks.The levels of AGEs,SOD and MDA were measured by ELISA,xanthine oxidase and thiobarbituric acid methods after the intervention.Expressiones of NF-κBp65,ERK1 and ERK2 in renal tissue were detected by immunohistochemistry.Expression of RAGE in the renal cortex was investigated by Western blot.Results SOD activity was decreased in the HC group.The levels of AGEs,MDA,RAGE and the activations of NF-κBp65 and ERK1/2 were increased in the HC group (t＝4.53,5.52,2.93,al1 P＜0.05).After the 16 weeks' intervention,SOD activity was elevated in T,P and TP groups compared to that in HC group (P＜0.05).The positive expressiones of NF-κBp65,ERK1 and ERK2 were significantly reduced in T,P and TP groups compared to those in HC group (F＝20.13、148.82、18.70,all P＜0.05).All the positive expressiones of NF-κBp65,ERK 1and ERK2 were lowest in the TP group versus T and P groups (t ＝ 3.58、2.84,P ＜ 0.05).Conclusions Telmisartan and pyridoxamine can alleviate the oxidative stress in spontaneously hypertensive rats,which may result from the blocking effect of Ang Ⅱ,the reduction of AGEs-RAGE and inhibiting the signal pathways of ROS,NF-κBp65 and ROS-ERK1/2.

Objective:To explore influence of monotherapy or combined use of telmisartan and pyridoxamine on aor-tic remodeling in spontaneously hypertensive rats (SHR)and its possible mechanism.Methods:A total of 48 male SHE were randomly and equally divided into hypertension control group,telmisartan group,group,and telmisartan+ group (combined treatment group). Kyoto Wistar rats of the same age and gender were regarded as normal blood pressure control group (normal control group). Thoracic aortic section were examined by related staining af-ter 16 weeks intervention to calculate the ratio of aortic wall thickness to radius of lumen (Tw/Rl),the ratio of wall area to lumen area (W/L),and the area ratio of media elastic fiber/collagen fiber. Concentrations of related en-zymes and receptor etc. of abdominal aortic were measured.Results:Compared with hypertension control group, there was significant rise in ratio of media elastic fiber/collagen fiber area and significant reduction in media collagen fiber/media area ratio in telmisartan group,pyridoxamine monotherapy group and combined treatment group,and there were significant decrease in Tw/Rl [(0.17±0.02)vs. (0.12±0.01)]and W/L [(0.29±0.03)vs. (0.22± 0.02)]ratios in combined treatment group,P <0.05 or <0.01;immunohistochemistry indicated that there were significant reductions in thoracic aortic receptor of advanced glycation end products (RAGE) [(0.24±0.03)vs.(0.17±0.03)]and extracellular signal-regulated kinase 1/2 (p-ERK1/2 )expression [(0.63 ± 0.06)vs. (0.37± 0.04)]in combined treatment group,P <0.05,<0.01. Fluorescence quantitative PCR indicated that medication can significantly reduce nicotinamide adenine dinucleotide phosphate (NADPH)oxidase subunit p47phox mRNA ex-pression (P <0.01 all),especially in combined treatment group (P =0.001).Conclusion:Combined use of telmis-artan and pyridoxamine is superior to the single use of either drug on improving thoracic aortic remodeling in SHR, the mechanism may be related to it reduces local expression of RAGE and p-ERK1/2 ,and inhibits oxidase subunit p47 of NADPH.

Objective To evaluate the causes,prevention and treatment ot laparoscopic comphca- tions in urologic surgery.Methods From May 2000 to May 2004,135 urologic laparoscopies were per- formed,including 17 transperitoneal laparoscopies and 118 retroperitoneal laparoscopies.Of the135 cases,26 had adrenal adenoma extirpation;31 had roof-removal and decompression of renal cyst;25 had radical ne- phrectomy;6 had pyeloplasty;2 had partial resection of renal capsula and encapsulation of the great omen- tum;2 had partial nephrectomy;4 had pelviureteral resection;13 had ligation of renal pedicle lymphatic ves- sel;2 had ureterolysis;7 had ureterolithotomy;12 had ligation of spermophlebectasis;5 had exploration,re- duction and fixation of undescended testis.The intra-and post-operative complications were retrospectively reviewed and clinically analyzed.Results Overall,14 cases(10.4%)had complications.Intraoperative complications occurred in 10 cases,including 6 cases of peritoneal injuries,which were clamped during oper- ation;4 of vascular injuries,which resulted in conversion to open surgery.Postoperative complications oc- curred in 4 cases,including 2 of subcutaneous emphysema,which was spontaneously absorbed at 5 and 7 d after operation;1 of incision seepage,which was drained for 40 d and then disappeared;and 1 of urine reten- tion,for which urethral catheterization was kept for 3 d and then the patient had voluntary voiding.No death occurred in this series.Conclusions Understanding the characteristics of laparoscopic complications in u- rologic surgery and systematic training of the surgeons can reduce the occurrence of complications.

Objective To explore the treatment of children with EB virus infection accompanied by facial paralysis. Method The clinical data of a child with EB virus infection accompanied by facial paralysis was analyzed retrospectively, and the related literature were reviewed. Results A 2-year-old boy was admitted to hospital due to fever and mouth askew for 4 days. After admission, he was confirmed to have EB virus infection and viremia by serology and polymerase chain reaction, and then treated with acyclovir. The symptoms of facial paralysis and EB viremia disappeared completely 14 days after antiviral treatment. There was no recurrence in the short-term follow-up. Interestingly, the literature analysis shows that there is still limited evidence for the antiviral treatment by acyclovir in children with acute infection of EB virus associated with facial paralysis. Conclusion Antiviral treatment may be beneficial to EB viremia with facial paralysis.

Objective: To observe the effect of pyridoxamine and telmisartan on the indexes of myocardial remodeling in spontaneous hypertensive rats (SHR). Methods: A total of 48 male SHR at 22 weeks of age were randomly divided into 4 groups:①Hypertension (H) control group, the rats received distilled water 2 ml/d,②telmisartan (T) group, the rats received telmisartan 6 mg/(kg.d),③pyridoxamine (P) group, the rats received pyridoxamine 200 mg/(kg.d),④Combination (TP) group, the rats received both telmisartan and pyridoxamine. All animals were treated for 16 weeks and the systolic blood pressure (SBP) was measured before and after treatment. After treatment, the serum levels of nitric oxide (NO) and super oxide dismutase (SOD) were examined by chemiluminescent method, advanced glycation end-products (AGEs) was detected by ELISA, left ventricular weight index and collagen volume fraction (CVF) in myocardial tissue were calculated, the mRNA expression of myocardial receptor of advanced glycation end products (RAGE) was evaluated by real time RT-PCR. Results: Compared with H group, SBP levels were decreased in T and TP groups,P<0.01, while SBP in P group stayed similar,P>0.05. Compared with H group, the serum levels of NO and SOD were increased in T, P and TP groups,P<0.01; while compared with T group and P group, the levels of NO and SOD were further increased in TP group,P<0.05. Compared with H group, left ventricular weight index and CVF were decreased in T, P and TP groups,P<0.01; while compared with T group and P group, left ventricular weight index and CVF were further decreased in TP group,P<0.05. Pyridoxamine and telmisartan had the coordinative effect on left ventricular weight index and CVF, P<0.05. The serum levels of AGEs were lower in T group (5.99 ± 0.51) mg/L, P group (5.57 ± 0.91) mg/L and TP group (5.24 ± 0.63) mg/L than that in H group, (6.71 ± 0.50) mg/L,P<0.01; while compared with T group and P group, AGEs was further decreased in TP group,P<0.05. The mRNA expressions of RAGE were reduced in T group (0.035 ± 0.010), P group (0.036 ± 0.005) and TP group (0.024 ± 0.007) than that in H group (0.053 ± 0.010),P <0.01; while compared with T group and P group, the mRNA of RAGE was further reduced in TP group, P<0.05. Conclusion: Pyridoxamine could improve the oxidative stress and the indexes of myocardial remodeling independently from decreasing blood pressure in SHR. Combined intervention of P and T may break the coordination systems between AGEs-RAGE and renin-angiotensin, therefore further improve the indexes of myocardial remodeling in SHR.

Child ; Child, Preschool ; Contrast Media ; adverse effects ; Coronary Angiography ; Female ; Humans ; Infant ; Iohexol ; adverse effects ; Kidney ; drug effects ; physiology ; Male ; beta 2-Microglobulin ; urine

The effect of recombinant human phospholipase D2(rhPLD2)in vivo was investigated on the secretion of serum glycosyl phosphatidylinositol-specific phospholipase D(GPI-PLD)in guinea pigs of chronic asthma．Ater treating the guinea pigs attacked by chronic asthma with rhPLD2,the GPI-PLD activity detection was canrried out by phase separation of human placental alkaline phosphatase in Triton X-114．Compared with the healthy guinea pigs(NS group),the serum GPI-PLD in the guinea pigs of chronic asthma are much higher than that of control groups,P≤0．01．Our results showed that rhPLD2 could significantly reduce the secretion of GPI-PLD when the guinea pigs were attacked by chronic asthma．