Objective Norwood StageⅠis the standard procedure to cope with hypoplastic left heart syndrome(HLHS), which continues to be the most challenging congenital heart disease .The aim of this study is to retrospectively analyse the perio-perative management of Norwood StageⅠ.Methods Between June 2010 and August 2014, totally 5 small infants with HLHS underwent the standard Norwood StageⅠprocedure.They were all boys.Age at surgeries ranged from 29 to 75 days, and weight from 2.57-3.50 kg with median of 3.13 kg.Case 1, 2 and 3 received standard medical regimen after accessing NICU, which included intravenous prostaglandin E1 and mechanical ventilation.The 3 infants underwent emergent operations because of unstable hemodynamics.Case 4 and 5 received no medical intervention before the urgent surgeries.All 5 cases underwent the standard Norwood StageⅠprocedure under deep hypothermic circulatory arrest, including 4 cases of modified Blalock-Taus-sig shunt( MBTS) and 1 case of Sano shunt.Results The case with Sano shunt died from severe hypoxemia and persistent aci-dosis 32 hours after the operation, another case died from low cardiac output syndrome after cardiopulmonary bypass.The first case underwent bidirectional Glenn procedure 12 months after Norwood Stage I, the postoperative heart function was NYHA I and the oxygen saturation was 0.90-0.95 in room air, but he died from accidental brain injury 3 months after stage Ⅱ.The second case was followed up 3 months after stage I procedure with NYHA I and oxygen saturation of 0.78-0.83 in room air, and lost the follow-up after.The fifth case was followed up 3 months after stageⅠprocedure with NYHAⅠ, confluent MBTS and oxygen saturation of 0.84, the patient is being followed up and waiting for further evaluation for stageⅡprocedures.Con-clusion The standard Norwood StageⅠprocedure is a complex procedure, which demands multidisplinary cooperation, to pal-liatively correct HLHS .Sharing expericences on perioperative managements of Norwood Stage I between heart centers in China will be helpful to decrease the mortality and morbidity in relatively short period .

Objective To study the effect of TGF?β1 gene therapy on the rat model of postpartum stress urinary incontinence and explore a novel non?operative treatment of this disease. Methods Two hundred and forty 6?month old SD female rats were used to prepare the model of postpartum stress urinary incontinence by vaginal dilation with a water sac. 148 rats from the 185 successfully prepared model rats were selected, and randomly divided into 5 groups: the TGF?β1 gene therapy, clentuterol treatment, electric stimulation therapy, injection of empty vector plasmid, and non?treated groups. In addition, 20 normal rats were selected as blank control group. Sneeze test and urodynamic test were conducted, the pelvic floor pubococcygeus muscle contractile force/muscle weight ratio was calculated, serum TGF?1 was detected by ELISA, and TGF?1 protein was detected by immunohistochemistry at 1, 21, 42 and 63 days after the treatment. Results At 21 days after treatment, all the maximum bladder capacity, leak point pressure, and urine or contractile force / muscle weight ratio of the TGF?β1 gene therapy group showed even better effects than those of the electrical stimulation group, but the differences were statistically not significant ( P>0?05 ) . Conclusions TGF?β1 gene therapy shows good therapeutic effect on the rat models of postpartum stress urinary incontinence, suggesting that TGF?β1 gene therapy may become a new type of non?surgical treatment for this disease.

Objective To observe the anticoagulant effect of low molecular weight heparin on induced hemodialysis in patients with acute renal failure.Method One hundred and eight patients with acute renal failure treated with induced hemodialysis were randomly divided into low molecular weight heparin(LMWH)group and unfractionated heparin(UFH)group.A bolus disc of UFH was given at first and then maintained by continuous infusion in UFH group,whereas a single bolus dose of LMWH with 2000AFXa IU to 4000AFXa IU in LMWH group.Results Anticoagulant effect between LMWH and UFH did not show significant discrepancy during induced hemodialysis.The bleeding from internal jugular vein catheter increased in the UFH group much more than that in the UFH group was significantly higher than that in the LMWH group.Anti-FXa blood levels were significantly higher in LMWH group than in UFH group.Conclusions LMWH has minor influence on aPTT and TT,while its anticoagulation effect approximates to that of UFH.LMWH represents a realistic alternative agent UFH in acute renal failure induced hemodialysis.

Female ; Humans ; Liability, Legal ; Middle Aged ; Occupational Diseases ; diagnosis ; Occupational Health ; legislation & jurisprudence ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; diagnosis ; etiology

Objective To evaluate the relationship between carotid artery plagues and the clinical risk factors in patients with diabetes mellitus type 2 by observing the ultrasonic characteristics of carotid atherosclerosis plagues.Methods The ultrasound results of carotid arteries in 665 patients with diabetes mellitus type 2 from January 2009 to December 2011 in Beijing Amery General Hospital were retrospectively analyzed.The location and quantity of carotid atherosclerotic plaques were observed .The relationship between the occurrence and risk factors of diabetes such as hypertension ,family history of diabetes and age were analyzed.Single factor and multi-factor analysis were applied for the related risk factors .Results The ultrasonography showed 405 cases of carotid artery plaques.Most carotid artery plaques were located in the posterior wall of carotid sinus and were hyperechoic ,hypoechoic or isoechoic.The single factor analysis showed hypertension,family history of diabetes and age are risk factors of carotid atherosclerosis (χ2 =42.322,9.682 and 140.658,all P<0.01).Multi-factor logistic regression analysis showed age was an independent risk factor of carotid atherosclerosis in patients with diabetes mellitus type 2.The risk factors scores of patients who were less than 45 years,45-65 years and over 65 years of age were (3.50 ±1.16)scores,(3.46 ±0. 92)scores and (3.21 ±0.88)scores,and the difference was statistically significant (F=5.781,P<0.001). There were significant differences between risk factors scores of different age groups ( t =2.084,2.002, 3.786,3.474,2.877 and 2.504,all P<0.05).In patients more than 65 years of age,the risk of atherosclerosis plaques is 28.732 times higher than that of patients less than 45 years of age.After controlling the age factor,the possibility of atherosclerosis plaque increased 1.201 times for each additional one point of score. Conclusions Most carotid artery plaques in patients with diabetes mellitus type 2 are located in the posterior wall of carotid sinus and are hyperechoic ,hypoechoic or isoechoic.Age is an independent risk factor for the formation of carotid plaque.Patients more than 45 years of age should undergo early intervention to avoid the occurrence of major vascular complications .

Objective To study the effect of IGF-1 gene therapy and electric stimulation therapy on the rat models of postpartum stress urinary incontinence, and explore the ideal treatment for this disease.Methods 240 SD female rats were used to establish the model of postpartum stress urinary incontinence by water sac vaginal dilation.148 model rats were randomly selected from 185 successful models and divided into 5 groups:IGF-1 gene therapy, clenbuterol treatment, electric stimulation therapy, injection of empty vector plasmid, and untreated groups.Besides, 20 non-modeled rats were used as blank control group.Urodynamic test was performed, pelvic floor pubococcygeus muscle/muscle weight ratio was calculated, and serum biochemical indices (LDH, CK) were detected, and the morphological changes of pubococcygeus muscle fibers were observed by light microscopy at 1, 21, 42 and 63 days after treatment.Results At 21 days after treat-ment, the maximum bladder capacity, leak point pressure, the contractile force/muscle weight ratio in the IGF-1 group and electric stimulation treatment group were significantly better (P>0.05), and the differences between the IGF-1 group and electric stimulation group were not significant ( P>0.05 ) .Conclusions The effect of IGF-1 gene therapy and electric stimulation on the rat models of postpartum stress urinary incontinence is better than that in the drug therapy group and oth-er groups.

Purpose:To discuss the function of Gd-BOPTA on the deferment of manifestation of MRI,which bring influence to the immune mensuration of antigen PCNA,PTEN.Materials and Methods:Scanning with MRI on the 35 patients with pathologically verified HCC the image were analyzed concretely.Pathologic diagnose was made with Edmondson pathologic classification standard,and it was expressed with index of PCNA and PTEN of immune quantitative analysis.Results: HCC was negatively related to the index of PCNA,and most patients with high index were lightly or not deferred enhanced(13/16),Patients with low index were evidently enhanced ( 10/19).The degree of HCC deferred enhancing was positively related to the expression of PTEN.Most HCCs( 15/17) with negative PTEN were lightly or not deferred enhanced,while positive patients of PTEN were mostly enhanced obviously(11/18).Conclusion: Deferment of Gd-BOPTA can be used to basically estimate the tincture of tumor biology,therefore,the enhanced degree of which,the PCNA,PTEN,can offer great help in choosing the therapuetic method and estimating the outcome of the the therapy.

OBJECTIVE: To observe the characteristics of vertical cast-off bloodstain pattern by different hitting-tools. METHODS: The regular hitting tools, a kitchen knife, a dirk, a plane set-hammer and an iron pipe, were selected. At a distance of 30 cm away from the wall, the hitting tool with 5 mL fresh chicken blood made the cast-off bloodstain from top to bottom. Then the holistic distribution characteristics (length, width and density) of cast-off bloodstain and morphology characteristics (length, width and contact angle) of first single cast-off bloodstain were analyzed. RESULTS: The distribution length of cast-off bloodstain formed by dirk was minimum (P < 0.05). The distribution width of cast-off bloodstain formed by kitchen knife was minimum (P < 0.05). Except the pair of kitchen knife and plane set-hammer, the distribution density between each two tools had statistical differences (P < 0.05). The length of first single cast-off bloodstain formed by plane set-hammer was longest compared (P < 0.05). The width of first single cast-off bloodstain had statistical differences between kitchen knife and plane set-hammer, and between dirk and plane set-hammer (P < 0.05). CONCLUSION The type of hitting tool could be inferred by the specific characteristics of cast-off bloodstain pattern formed by every specific type of hitting tool in crime scene.
Blood Stains ; Computer Simulation ; Crime ; Forensic Ballistics/methods* ; Forensic Medicine/methods* ; Humans

Objective To explore the clinical feature, gene mutations and treatment of isolated hypogonadotropic hypogonadism (IHH). Methods The clinical data from a case of IHH and his families were retrospectively analyzed. The related literatures were reviewed. Results The propositus was a 7-year-old boy with a small penis and testes, whose younger brother was 5-year-old with a small penis and cryptorchidism. In both boys testosterone, luteinizing hormone (LH), follicle stimulating hormone (FSH) levels were low. The GnRH provocation test was not reactive. Their parents denied the consanguineous marriage. Illumina sequencing revealed that both of the brothers had homozygous mutation of GnRHR gene in C.806C>T, and their parents were heterozygous mutations in C.806C>T gene. After confirmation of the diagnosis, human choronic gonadotropin (hCG) treatment was given. The levels of testosterone and dihydrotestosterone were significantly increased after 6 weeks. Conclusion The combination of clinical phenotype, biochemical analysis, and gene detection is helpful for early diagnosis of IHH.

[Summary] Microcephalic or Majewski's osteodysplastic primordial dwarfism type Ⅱ ( MOPD Ⅱ) is an extremely rare genetic disease mainly caused by pericentrin ( PCNT) gene mutations. This paper reported one 13-year-old boy, who was admitted because of the slow growth for more than 13 years and deepened skin color over six months. He was diagnosed as MOPD Ⅱ associated with a combination of growth hormone deficiency, type 2 diabetes, hypertension, acanthosis nigricans, multiple café-au-lait spots. On magnetic resonance imaging of brain, no vascular malformations such as aneurysms were shown. There were novel compound heterozygous mutations of PCNT gene in the patient, with the nonsense mutations of c. 502C > T ( p. Gln168 * heterozygous variation) and c. 3103C > T (p. Arg1035* heterozygous variation). His father carried a nonsense mutation c. 3103C > T ( p. Arg1035 *heterozygous variation ) and his mother had a nonsense mutation c. 502C > T ( p. Gln168 * heterozygous variation). After treatment with metformin for three months, his blood glucose returned to normal, and acanthosis nigricans was improved. It seems critical to evaluate the abnormal condition of blood vessels regularly for MOPD Ⅱpatients with PCNT gene mutations.