Congenital long QT syndrome ( LQTS) is a cardiac ion channel dysfunction, leading to prolonged myocardial repolarization time. It is characterized by the typical ECG QT interval prolongation and torsades de pointes. It shows clinical recurrence of cardiogenic syncope and even lead to sudden death. Molecular genetic studies have revealed a total of 12 forms of congenital LQTS caused by mutations in genes of the potassium, sodium and calcium channels or membrane adapter located on chromosomes 3, 4, 7, 11, 12, 17, 20 and 21. This review summarized the studies of the pathogenesis of LQTS and gene-related treatments.

Wistar rats randomly divided into groups A,B and C were killed at 2、3 and 12 days after transplantation,respectively.The pathological changes of the donor livers were observed by light microscope and electron microscope.The findings demonstrated that:(1)in the group A there was hepatocellular regeneration including hypertrophy of nuclei and nucleoli,and hyperplasia of mitochondrions and endoplasmic reticula;(2)in the group B the morphologic changes of the allografts involved in distinet fatty degencration and mitochondrial swelling of hepatic cells and peripheral vesscl wall infiltration by monocytes and lymphocytes;(3)in the group C hepatocellular loss with the massive tissue necrosis and fibroplasia occurred in the gratfs.From the above it was concluded that hepatocellular necrosis or loss related to the rejection 12 days after the transplantation might lead to the liver failure in the rats.

The widespread use of antibiotics has been effectively controlled serious life-threatening infections.The trituration,exploitation and use of antibiotics has been rapidly developed and applied to many fields.Due to the extensive use of various antibiotics,the emergences of drug-resistant bacteria and the rates of bacterial resistance have increased unceasingly which has been a common focus in the world.Resistant pathogen can reduce the effectiveness of antibiotics,and which is a serious threat to human health.De-escalation therapy of antibiotics can quickly and effectively control serious infections,and avoid bacterial resistance,and induce fungal infections.The rational use of antibiotics in pediatrics is very imperative,which includes strengthening management of antibiotics,and improving awareness and vigilance of the adverse reactions of antibiotics,and consummating specification of medication.Then,realize the correct,safe,rational medication of antibiotics.

Bronchiolitis obliterans(BO) is chronic airflow obstruction syndrome characterized by small airway obstruction and airflow limitation.In children, BO is secondary to severe infection of respiratory tract, and its development is related with inflammation and immune response.At present, the diagnosis of BO mainly is depended on the clinical manifestation, lung function and examination results of high resolution computed tomography (HRCT) because of difficulty of lung biopsy.Pulmonary HRCT is a non-invasive examination, and worthy of generalization.Currently, BO has no accepted effective treatment,early diagnosis and treatment can block the progression of BO.Due to lack of realization of BO, doctors and parents fail to give enough attention, BO has a prolonged course and very poor prognosis.

Adult ; Humans ; Male ; Malondialdehyde ; blood ; Submarine Medicine ; Superoxide Dismutase ; blood ; Young Adult

Objective The study was to investigate the value of deduction and application of anterior compression index in evaluation of atlantoaxial dislocation and restoration. Method Twenty-eight cases of the control group and 28 cases of atlantoaxial dislocation treated with posterior restoration and screw-rod internal fixation technique before and af?ter surgery were recruited in this study and their data was retrospectively analyzed. All of the people underwent sagittal computerized tomography scan films. The anterior compression index was measured in all cases. Results The mean value of anterior compression index of the control group was 87.86±24.98. The mean value of anterior compression index of the preoperative patients was 230.44 ± 97.60 and the mean value of the postoperative patients was 106.27 ± 73.53. There was significant difference in those two parameters between the preoperative patients and the controls(t=-7.50,P<0.0001). There was no significant difference between the postoperative patients and the control group (t=-1.26, P=0.2171). Anteri?or compression index were significantly lower after surgical operation (t=10.35, compared with before, P<0.0001). Con?clusion Anterior compression index can be an excellent measurement tool for the assessment of relationship of atlas and axis in atlantoaxial dislocation patients before and after posterior restoration operation.

Objective:To determine the di-allelic polymorphisms of TNF gene and their association with Helicobacter Pylori(H.pylori) infection and gastroduodenal diseases in Chinese population with Han nationality.Methods:Two hundreds and ten patients with gastroduodenal diseases(73 chronic gastritis,78 duodenal ulcer and 59 noncardia gastric cancer) and 264 healthy controls were genotyped by the PCR-RFLP method for TNF-? 308,lymphotoxin-?(LT-?) Nco Ⅰ and AspH Ⅰ gene polymorphisms.H.pylori infection status was determined by a validated serological test.Results:H.pylori infection was detected in 90.5% of 210 patients and 62.1% of 264 healthy controls(P

Color blindness represents a group of vision disorders characterized by lack of ability to distinguish different colors.The inherited color blindness has been regarded as incurable for a long period of time.Recently,adeno-associated virus(AAV) mediated gene therapy has successfully restored cone system vision in animal models with color blindness caused by different gene mutations.These mutations are presented in human color blindness patients.It is predicted that gene therapy will become a novel treatment for these color blindness victims.In addition,a single gene transfer may achieve long-term correction of color deficiency.

Atrial Flutter ; diagnosis ; etiology ; therapy ; Electrocardiography ; Female ; Humans ; Infant, Newborn ; Infant, Premature ; Treatment Outcome

Objective This study aimed to establish an anatomic basis for the microscopic surgical anatomy of the cavemous sinus via the extended transsphenoidal approach.Methods Simulated surgery via extended transsphenoidal ap-proach was performed on seven adult cadaver heads with red--colored latex injected arteries.The cavernous segment of the ICA and its branch vessels and its relationship with cranial nerve were exposed and its anatomic parameters were measured under microscope.Results The tuberculum sellae,clivus,sellar base,ICA prominence,and optic nerve prominence are the important bone landmarks to define the surgical area.The average of the extent of bone removal of extended transsphe-noidal approach is 37.6 mm(range:28.7 mm～44.0 mm).Conclusions The bone removal from sellar base to the media edge of the foramen rotundum and over the ICA prominence can effectively expose the entire unilateral cavernous sinus.The microscopic cavernous sinus surgery via the extended transsphenoidal approach is an optimal surgical approach for the le-sions that invade the cavernous sinus from sella.