Plasma level of KPTT,factor Ⅷ coagulant activity,factor Ⅷ-related antigen,fib-rinogen,antithrombin Ⅲ activity,antithrombin Ⅲ antigen,tissue-plasminogen activator activity (t-PA:C),plasminogen activator inhibitor activity(PAI:C),PAI:C/t-PA:C,plasmin activity (PLm:C),D-dimer and plasma platelet α granule membrane protein were ditermined in 26 patients treated by long-term peritoneal dialysis(PD group),17 patients with chronic renal failure under-going conservative therapy(CRF group),36 patients on maintenance hemodialysis(HD group) and 20 age & sex-matched normal controls(NC group).The results showed:1.PD group had hypercoagulable state than NC group.The prothrombotic state in PD patients was most severe in contrast with CRF patients and HD patients.2.When PD group was compared with NC group,PLm:C showed no significant differences,but they had secondary fibrinolysis following enhanced coagulation.Moreover,PLm:C in PD group was significantly depressed as compared with in CRF group.3.the activated platelets in the PD patients were significantly elevated as compared with in the normal controls.

Objective To summarize the anesthesia management and surgical characteristic of modified extended Morrow procedure in treating hypertrophic obstructive cardiomyopathy (HOCM). Methods This retrospective study was conducted in 139 patients (male 83 and female 56)aged from 1 1 to 66 years.They underwent general anesthesia by high dose intravenous fentanyl or sufentanyl combined with propofol infusion and low concentration sevoflurane inhalation.The surgeons chose ap-propriate surgical procedures,including modified extended Morrow procedure,or combined with mi-tral valve repair (replacement)and coronary artery bypass grafting,etc.All patients received intraop-erative transesophageal echocardiography (TEE ) or epicardial echocardiography monitoring. Results There was no death case in hospital.All patients were hemodynamically stable and there were no malignant adverse events such as ventricular fibrillation during perioperative period.70 (50.4%)of patients automatically recovered to cardiac rhythm after aortic unclamping,and 1 1 (7.9%)of patients needed temporary pacemaker due to atrioventricular block.Cardiopulmonary by-pass (CPB)were weaned off successfully without positive inotropic drugs in 103(74.1%)of patients, while others 36(25.9%)needed low dose dopamine,epinephrine or norepinephrine to maintain hemo-dynamics stable.The CPB time was (142 ± 5 1 )min and the time of aortic clamping was (96 ± 37 ) min.Blood protection was used in all patients and 129 (92.8%)of the patients didn’t receive any blood product.Conclusion Modified extended Morrow procedure was a safe and effective surgical pro-cedure for treatment of HOCM.Experienced teamwork was essential to achieve satisfactory clinical results.The key points of anesthesia management were administration of appropriate preoperative drugs,maintaining adequate anesthesia depth,appropriate preload and afterload,heart rate and rhythm.Intraoperative TEE monitoring was the golden standard for guiding and evaluating the effec-tiveness of the surgical procedures.Meanwhile protection of myocardium,lung,brain and blood can help to obtain satisfactory clinical outcomes.

Objective: To evaluate the efifcacy of modiifed extended Morrow procedure on hypertrophic obstructive cardiomyopathy (HOCM) in adolescent patients. Methods: We retrospectively studied 29 consecutive HOCM patients at the age≤21 years who received modiifed extended Morrow procedure in our hospital from 2011 to 2015 for their clinical conditions to assess surgical efifcacy. Echocardiography was performed to compare left atrial size, left ventricular end diastolic diameter, left ventricular ejection fraction, left ventricular outlfow tract peak pressure, ventricular septal thickness, mitral systolic anterior motion and mitral regurgitation grade before and after operation. Moreover, pre-operative and post-operative plasma NT-proBNP levels were determined. Cardiac function was evaluated by New York Heart Association functional class. Results: There were 17 (58.6%) patients received isolated modiifed extended Morrow procedure and 12 patients had concomitant operation including 8 (27.6%) with coronary artery bypass grafting. Compared with pre-operation, the post-operative thickness of ventricular septum decreased from (24.6 ± 6.8) mm to (16.9 ± 7.1) mm, left ventricular outlfow tract gradient decreased from (68.8 ± 15.7) mmHg to (10.7 ± 4.2) mmHg, bothP<0.001; mitral regurgitation degree reduced from (1.7 ± 1.3) to (0.2 ± 0.4),P<0.01; NYHA classification improved from (3.4 ± 0.8) to (1.4 ± 0.5),P<0.01; plasma level of NT-proBNP reduced from (1957.6 ± 392.5) ng/ml to (458.7 ± 161.0) ng/ml,P<0.01. There was no peri-operative death, the survival rates at 12, 24 and 36 months post-operation were 100%, 86.7% and 86.7% respectively. Conclusion: Modiifed extended Morrow procedure has been a safe and effective method for treating adolescent HOCM patients, adequate exposure is the key point to assure surgical efifcacy.

Objective To explore the application of temporary balloon occlusion in multidisciplinary management of cesarean sec-tion for patients with pernicious placenta previa/accreta.Methods 42 patients with a diagnosis of placenta previa /accrete accepted the temporary aortic balloon occlusion in cesarean section in DSA hybrid operation room were retrospectively studied.Before cesare-an section,intravascular balloon catheter was placed in abdominal aortic.After delivery,the midpoint of filling state balloon was placed at the midpoint of the two renal arteries openings before placental dissection.The amount of blood loss and blood transfusion, operation time,the infants'radiation doses and postoperative complications were determined.Results All patients were successfully fulfilled cesarean section and conserved uteri.The amount of blood loss,blood transfusion,operation time,occlusion time and the infants’radiation doses were (586±355)mL,(422±283)mL,(75.5±1 1.9)min,(22.4±7.2)min and (4.2±2.9)mGy,respec-tively.During follow-up,complications were not found during operation and after operation.Conclusion In women with pernicious placenta previa/accreta,temporary aortic balloon occlusion can effectively control postpartum hemorrhage and reduce the risk of un-desirable hysterectomy.

Objective: To summarize the major post-operative complication of modiifed extended Morrow procedure in patients with hypertrophic obstructive cardiomyopathy (HOCM) and to explore the major factors affecting its prognosis. Methods: We retrospectively analyzed 139 consecutive HOCM patients who received the procedure by same surgeon in our hospital from 2012-06 to 2014-07. There were 87 male and 52 female patients with the age of (10-67) years, body weightof (26-105) kg and pre-operative left ventricular outlfow tract peak gradient (LVOTPG) of (84.48 ± 44.75) mmHg. Concomitant operations were performed with known cardiac disease as necessary. Pre- and post-operative echocardiography, ECG and chest X-ray were examined to assess the adequacy of resection and mitral valve structure and function. Results: There was no peri-operative death. 73/139 (53%) patients received simple modiifed expanded Morrow procedure, the other 66 (47%) patients received concomitant surgery including 21 patients with coronary artery bypass grafting, 15 mitral valve plasty, 7 mitral valve replacement, 10 tricuspid valve plasty, 2 aortic valve replacement, 3 modiifed Maze procedure, 2 unblock of right ventricular outlfow tract, 2 sub aortic membrane resection, 1 ventricular aneurysm resection. The mechanical ventilation time was (24.05±36.74) hours, post-operative ICU and in-hospital stays were (2.85±3.18) days and (10.11±4.57) days; the complications included arrhythmia in 108 cases, pleural effusion in 25 cases, secondary intubation in 1 case, tracheotomy in 1 case, hemoifltration in 1 case, intra-aortic balloon pump in 1 case, back into ICU in 3 cases; no pneumothorax, secondary thoracotomy/operation. The post-operative left atrial diameter, LVOTPG, inter-ventricular septal thickness and LVEF were all decreased; mitral valve closed well or with mild regurgitation, systolic anterior motion (SAM) basically disappeared. The major factors for delayed ICU stay included age≥55 years, female, CPB time≥120 min, AOC time≥90 min, the patients combining with arrhythmia and right ventricular dysfunction. Late follow-up presented that the patients were almost without the symptoms, NYHA classiifcation at (I-II), no late death, complication or re-operation. Conclusion: Modified expand Morrow procedure has good surgical and short/late post-operative effects, concomitant operation does not increase the complication and mortality; correction of arrhythmia and improving right ventricular function at peri-operative period are important for treating the relevant patients.

Objective To investigate the causes of acute renal failure(ARF)after orthtopic liver transplantation(OLT)in patients of acute liver failure(ALF)and the effects of systemic therapy based on continuous renal replacement(CRRT).Methods Clinical data of 412 patients who underwent liver transplantations between January 2001 and June 2006 were analyzed retrospectively (all the cases were followed up to June 2007).According to UNOS grading scale,54 patients were of acute liver failure(UNOS 1 and 2A).Posttransplant ARF developing in 17 cases underwent a systemic therapy based on CRRT as well as anti-rejection,anti-infection and nutrition support.The perioperative courses,complications,causes of death and follow up results were analyzed.Results There were no severe complications during CRRT.Perioperative mortality was 5.4%and 58.8%in patients without ARF and those with ARF respectively.the rate of complications was 35.1%vs 100%.1 year survival rate Was 89.2% vs 41.2%.3 year survival rate was 81.1% vs 41.2%.Condusions The effect of surgery mainly depends on the function of liver and other vital organs.The ALF recipients suffered from a high perioperative mortality,especially those with posttransplant ARL.The systemic therapy based on CRRT benefits patients with postoperative ARF.

OBJECTIVE: Three genes including the OTOF, the DFNB59 and the DIAPH3 have been implicated previously in human non-syndromic auditory neuropathy. In this study, we aim to investigate whether DIAPH3 gene or the known deafness loci of 25 cloned autosomal dominant deafness (DFNA) genes contribute to the nonsyndromic hearing loss of a Chinese pedigree with dominantly inherited auditory neuropathy (AN). METHOD: Nine members of the kernal pedigree in this family were selected. Genomic DNA was isolated from the peripheral leukocytes of the subjects using the Puregene DNA Isolation Kits. Firstly, the 5'UTR of DIAPH3 gene was PCR amplified in all subjects. Then, the DNA fragments spanning the entire coding regions of DIAPH3, GJB2 and GJB3 genes, and 50 exons in other 23 cloned DFNA genes were amplified using specific primers. Each fragment was purified and analyzed by direct sequencing. The resultant sequence data were compared with the standard sequence to identify deafness-associated mutations. RESULT: PCR amplifications were successfully conducted. We failed to detect the presence either of c. --172G > A mutation in the 5'UTR that have been reported, or any other deafness-associated mutations in the whole DIAPH3 gene, by sequence analysis. We also did not find any known deafness-causing mutations among the 25 cloned DFNA genes. CONCLUSION The DIAPH3 gene, and the known deafness loci of 25 cloned DFNA genes seem not contribute to the pathogenesis of this Chinese AN family in this study, which suggesting new gene(s) involvement.
Adaptor Proteins, Signal Transducing ; genetics ; Asian Continental Ancestry Group ; China ; Connexins ; DNA Mutational Analysis ; Deafness ; Exons ; Hearing Loss ; Hearing Loss, Central ; genetics ; Hearing Loss, Sensorineural ; genetics ; Humans ; Mutation ; Pedigree ; Polymerase Chain Reaction

OBJECTIVE: To investigate if the DFNB59 gene contributes to the hearing loss of a Chinese pedigree with dominantly inherited auditory neuropathy (AN). METHOD: Nine members in four generations of the family were selected for this study. Genomic DNA was isolated from the peripheral leukocytes of the patients using the pure gene DNA isolation kits. Firstly, the subjects DNA fragment was PCR amplified using specific primers corresponding to exon 2 and 4 of the DFNB59 gene. Each fragment was purified and subsequently analyzed by direct sequencing in an applied biosystems 3730 automated DNA sequencer. The whole coding sequence of DFNB59 gene of one family patient were then PCR amplified and submitted for sequence analysis as described above. The resultant sequence data were compared with the standard sequence to identify deafness-associated mutations. RESULT: PCR amplifications were successfully conducted in all the subjects. We failed to detect the presence either of mutations T54I and R183W in the exon 2 and exon 4 that have been reported, or any other deafness-associated mutations in the whole DFNB59 gene, by sequence analysis. CONCLUSION The DFNB59 gene seems not contribute to the pathogenesis of this Chinese AN family, which suggesting new gene(s) involvement.
Asian Continental Ancestry Group ; genetics ; Base Sequence ; DNA Primers ; Female ; Humans ; Male ; Mutation ; Nerve Tissue Proteins ; genetics ; Pedigree ; Sequence Analysis, DNA ; Vestibulocochlear Nerve Diseases ; genetics

OBJECTIVE: To investigate if the OTOF gene contributes to the non-syndromic hearing loss of a Chinese pedigree with dominantly inherited auditory neuropathy (AN). METHOD: The subjects included were 9 live individuals in an autosomal dominant AN pedigree, 3 sporadic AN patients and 3 normal-hearing controls. Genomic DNA was isolated from the peripheral leukocytes of the subjects using the Pure gene DNA Isolation Kits. Firstly, the whole coding sequence of OTOF gene of one family patient were PCR amplified using specific primers. Each fragment was purified and subsequently analyzed by direct sequencing in an Applied Biosystems 3 730 automated DNA sequencer. The resultant sequence data were compared with the standard sequence to identify deafness-associated mutations. Other DNA samples were then screened for these mutations by PCR amplification and sequence analysis. RESULT: PCR amplifications were successfully conducted in all the subjects. Comparison of the resultant OTOF sequence in one family patient with the standard sequence identified 10 nucleotide variants which do not lead to amino acid change. These mutations were also detectable in other family individuals, 3 sporadic AN patients and 3 normal-hearing controls. CONCLUSION The OTOF does not seem to contribute to the pathogenesis of this Chinese AN family, which suggest new gene(s) involvement.
Asian Continental Ancestry Group ; genetics ; Base Sequence ; Case-Control Studies ; Chromosome Disorders ; ethnology ; genetics ; DNA Mutational Analysis ; Female ; Genetic Testing ; Hearing Loss ; genetics ; Humans ; Male ; Membrane Proteins ; genetics ; Mutation ; Pedigree ; Sequence Analysis ; Vestibulocochlear Nerve Diseases ; ethnology ; genetics

OBJECTIVETo investigate the relationship of mitochondrial DNA mutations with inherited deafness in a maternally inherited pedigree with non-syndromic deafness.

METHODSThe diagnosis was validated by hearing tests. Blood samples were collected from 18 maternal members of the family and 53 controls including 6 paternal members, 7 spouses and 40 unrelated individuals. DNA was extracted from the leukocytes in blood samples. The gene fragments of mitochondrial DNA 12S rRNA, tRNA(Ser(UCN)) and GJB2 gene were amplified by polymerase chain reaction(PCR). PCR products were analyzed by sequencing. Computerized 12S rRNA secondary structure modeling was carried out to characterize the mutation found in the family.

RESULTSA novel mitochondrial DNA 12S rRNA 709 G to A transition was detected from all maternal members including 8 patients with hearing loss and other 10 symptom-free maternal members. Non-maternal members and other controls did not carry this mutation. In addition, the tRNA(Ser(UCN))A7445G, 12S rRNA A1555G and GJB2 gene mutations were not observed in the study. Computerized modeling showed that this mutation changed the eighth and ninth loop-stem structure of the 12S rRNA secondary structure.

CONCLUSIONIn this family, 8 deaf patients carried the mitochondrial DNA 12S rRNA 709 G to A mutation, which is highly conservative in healthy adults. It was confirmed that the mitochondrial DNA 12S rRNA gene G709A was associated with non-syndromic inherited hearing loss. The other 10 maternal members carried the mutation, but they did not suffer from deafness, which might suggest that the G709A mutation may cause hearing impairment in combination with a synergistic effect of some other nuclear modifier genes.

Adolescent ; Adult ; Aged ; Aged, 80 and over ; Base Sequence ; Child ; Connexins ; DNA Mutational Analysis ; DNA, Mitochondrial ; chemistry ; genetics ; Deafness ; congenital ; genetics ; Female ; Genomic Imprinting ; Humans ; Male ; Middle Aged ; Molecular Sequence Data ; Nucleic Acid Conformation ; Pedigree ; Point Mutation ; RNA, Ribosomal ; chemistry ; genetics ; Young Adult