Background:Fibroscan is the noninvasive method widely used to evaluate quantitatively the liver fibrosis and monitor the long-term efficacy of anti-fibrosis therapy. Aims:To study the use of Fibroscan for evaluating the efficacy of combined therapy with FuFang BieJia RuanGan tablet and antiviral drugs in patients with hepatitis B virus( HBV)-related cirrhosis. Methods:A total of 90 patients with HBV-related cirrhosis from March 2013 to September 2014 at Shanghai Ren Ji Hospital were recruited,and divided into treatment group and control group. Patients in treatment group received FuFang BieJia RuanGan tablet,and patients in control group received conventional liver-protective drugs,all the patients took nucleoside antiviral drugs at the same time. The treatment courses in both groups were 6 months. Liver stiffness measurement( LSM)was detected by Fibroscan before and after treatment. Biochemical parameters,width of portal vein and clinical symptoms were recorded. Results:After treatment,LSM was significantly decreased in both groups( P <0.05). Liver function,width of portal vein and Child-Pugh score were improved in both groups(P <0. 05),and no significant differences were found between the two groups(P>0. 05). LSM was closely associated with Child-Pugh score both before and after treatment(r=0. 484,P<0. 01;r=0. 523,P<0. 01). Patients with Child-Pugh A had lower LSM than those with Child-Pugh B or Child-Pugh C(P<0. 01). Conclusions:FuFang BieJia RuanGan tablet combined with oral antiviral drugs can remarkably improve the liver function of cirrhotic patients and prevent progression of cirrhosis. Dynamic detection of LSM can be used for monitoring drug efficacy and disease progression in patients with cirrhosis.

Background:Q fever endocarditis,a chronic illness caused by Coxiella burnetii,can be fatal if misdiagnosed or left untreated.Despite a relatively high positive rate of Q fever serology in healthy individuals in the mainland of China,very few cases of Q fever endocarditis have been reported.This study summarized cases of Q fever endocarditis among blood culture negative endocarditis (BCNE) patients and discussed factors attributing to the low diagnostic rate.Methods:We identified confirmed cases of Q fever endocarditis among 637 consecutive patients with infective endocarditis (IE) in the Peking Union Medical College Hospital between 2006 and 2016.The clinical findings for each confirmed case were recorded.BCNE patients were also examined and each BCNE patient's Q fever risk factors were identified.The risk factors and presence of Q fever serologic testing between BCNE patients suspected and unsuspected of Q fever were compared using the Chi-squared or Chi-squared with Yates' correction for continuity.Results:Among the IE patients examined,there were 147 BCNE patients,of whom only 11 patients (7.5％) were suspected of Q fever and undergone serological testing for C.burnetii.Six out of 11 suspected cases were diagnosed as Q fever endocarditis.For the remaining136 BCNE patients,none of them was suspected of Q fever nor underwent relevant testing.Risk factors for Q fever endocarditis were comparable between suspected and unsuspected patients,with the most common risk factors being valvulopathy in both groups.However,significantly more patients had consulted the Infectious Diseases Division and undergone comprehensive diagnostic tests in the suspected group than the unsuspected group (100％ vs.63％,P =0.03).Conclusions:Q fever endocarditis is a serious yet treatable condition.Lacking awareness of the disease may prevent BCNE patients from being identified,despite having Q fever risk factors.Increasing awareness and guideline adherence are crucial in avoiding misdiagnosing and missed diagnosing of the disease.

Objective To analyze the clinical characteristics,muscle pathological features and pathogenic gene mutation in 4 cases with LMNA-related congenital muscular dystrophy (L-CMD).Methods Clinical data of the probands and the parents were collected.Skeletal muscle specimens were biopsied from the probands for pathological analysis.Genomic DNA and RNA were extracted from peripheral blood leukocytes,and PCR,reverse transcription(RT)-PCR and DNA direct sequencing were employed to analyze the LMNA gene to determine the gene mutation and confirm the pathogenicity.Results Four patients had symptoms from fetal period to several months after birth.They presented with motor retardation,muscle weakness with prominent the proximal upper limbs,distal lower limbs and neck extensor,hypotonia,contractures,with mild to moderate elevation of CK level.The muscle biopsies showed muscular dystrophic and with inflammatory changes,and the abnormal nuclear morphology was observed with transmission electron microscopy.Genetic analysis of them detected 4 dominant de novo mutations.Three of them had unreported pathogenic mutations.The same sites of the LMNA gene were wild type in their parents.Conclusions Four cases of L-CMD are genetically identified.Genetic counseling of the family can be possible.The patients should be considered LMNA gene mutation of they present themselves with muscle weakness with the proximal upper limbs,distal lower limbs and neck extensor,hypotonia,contractures,mild to moderate elevation of CK level,and if the biopsies show muscular dystrophic changes but also with inflammatory changes should be considered LMNA gene mutation.Genetic analysis is the most reliable method for diagnosing L-CMD.

OBJECTIVETo study the microsurgical method of repairing skin and soft tissue defects on head, face, and neck.

METHODSThirty-one patients with skin and soft tissue defects on the head, face, or neck were hospitalized from July 2007 to May 2010, including 10 cases of scalp defects, 4 cases of skin and soft tissue defects on face, and 17 cases of skin and soft tissue defects on neck. Among them, the cause in 20 cases was trauma, and in 11 cases they were secondary to release of cicatricial contraction. Free flaps were transplanted to repair the wounds, including 13 latissimus dorsi flaps, 3 lateral thoracic flaps, 5 scapular flaps, and 10 anterolateral thigh flaps. The area of flaps ranged from 8 cm × 5 cm to 25 cm × 18 cm.

RESULTSAll flaps survived, and all the wounds healed by first intention. The average length of hospital stay was 16.7 days. Twenty-eight patients were followed up for 2 months, and in all of them satisfactory function and appearance were restored.

CONCLUSIONSFree flap graft based on microsurgery can repair wound of skin and soft tissue defects on head, face and neck by a single operation, which eases suffering of patients, and shortens the length of hospital stay.

Adolescent ; Adult ; Aged ; Child ; Child, Preschool ; Female ; Head ; Humans ; Male ; Microsurgery ; Middle Aged ; Neck ; Reconstructive Surgical Procedures ; methods ; Skin ; injuries ; Skin Transplantation ; Soft Tissue Injuries ; surgery ; Surgical Flaps ; Young Adult

OBJECTIVETo assess the value of multiplex PCR-denaturing high-performance liquid chromatography (PCR-DHPLC) method for screening large duplications or deletions in patients with Duchenne muscular dystrophy (DMD) and spinal muscular atrophy (SMA).

METHODSDNA was extracted from peripheral venous blood samples from 35 DMD and 6 SMA patients. Large duplications or deletions were screened with multiplex PCR coupled with DHPLC method. The results were validated with testing of positive and negative controls.

RESULTSKnown duplications or deletions in all controls were reliably detected with multiple PCR coupled with DHPLC. Large duplications or deletions were found in 71.4% of 35 DMD patients, which included 5 large duplications and 20 large deletions. For SMA patients, deletions of SMN1 exon 7 were detected in 16 samples.

CONCLUSIONMultiplex PCR coupled with DHPLC method is an effective and reliable method for detecting large genomic duplications or deletions in patients with DMD or SMA.

Chromatography, High Pressure Liquid ; Dystrophin ; genetics ; Gene Deletion ; Gene Duplication ; Humans ; Multiplex Polymerase Chain Reaction ; Muscular Atrophy, Spinal ; diagnosis ; genetics ; Muscular Dystrophy, Duchenne ; diagnosis ; genetics ; Survival of Motor Neuron 1 Protein ; genetics

BACKGROUNDThe neuroprotective effect of the cyclooxygenase (COX) inhibitor has been demonstrated in acute and chronic neurodegenerative processes. But its function under cerebral ischemic conditions is unclear. This study was designed to evaluate the neuroprotective efficacy of emulsified flurbiprofen axetil (FA, COX inhibitor) and its therapeutic time window in a model of transient middle cerebral artery occlusion (MCAO) in rats.

METHODSForty-eight male SD rats were randomly assigned into six groups (n = 8 in each group); three FA groups, vehicle, sham and ischemia/reperfusion (I/R) groups. Three doses of FA (5, 10 or 20 mg/kg, intravenous infusion) were administered just after cerebral ischemia/reperfusion (I/R). The degree of neurological outcome was measured by the neurologic deficit score (NDS) at 24, 48 and 72 hours after I/R. Mean brain infarct volume percentage (MBIVP) was determined with 2, 3, 5-triphenyltetrazolium chloride (TTC) staining at 72 hours after I/R. In three other groups (n = 8 in each group), the selected dosage of 10 mg/kg was administrated intravenously at 6, 12 and 24 hours after I/R.

RESULTSThe three different doses of FA improved NDS at 24, 48 and 72 hours after I/R and significantly reduced MBIVP. However, the degree of MBIVP in the FA 20 mg/kg group differed from that in FA 10 mg/kg group. Of interest is the finding that the neuroprotective effect conferred by 10 mg/kg of FA was also observed when treatment was delayed until 12 - 24 hours after ischemia reperfusion.

CONCLUSIONCOX inhibitor FA is a promising therapeutic strategy for cerebral ischemia and its therapeutic time window could last for 12 - 24 hours after cerebral ischemia reperfusion, which would help in lessening the initial ischemic brain damage.

Animals ; Cyclooxygenase Inhibitors ; administration & dosage ; pharmacology ; Disease Models, Animal ; Flurbiprofen ; administration & dosage ; analogs & derivatives ; pharmacology ; Infusions, Intravenous ; Ischemic Attack, Transient ; chemically induced ; drug therapy ; pathology ; Male ; Random Allocation ; Rats ; Rats, Sprague-Dawley ; Time Factors

OBJECTIVETo investigate sperm DNA integrity in male infertility patients with hepatitis B virus (HBV) infection.

METHODSThis study included 90 infertile men with HBV infection (group A), 82 infertile men without HBV infection (group B) and 70 normal fertile men (group C). We detected sperm DNA integrity among the subjects, including DNA fragmentation index (DFI) and high DNA stainability (HDS), by sperm chromatin structure assay (SCSA), and compared them among the three groups.

RESULTSDFI was higher in group A ([28.17 +/- 13.06]%) than in B ([26.64 +/- 9.79]%) and C ([15.67 +/- 4.73]%), significantly higher in A and B than in C (P < 0.05) but with no significant difference between A and B (P > 0.05). HDS was higher in group A ([10.83 +/- 5.601]%) than in B ([9.04 +/- 3.48]%) and C ([8.04-2.25]%), with significant difference between A and C (P < 0.05).

CONCLUSIONSperm DNA integrity of infertile males is significantly different from that of normal fertile men, and infertility with HBV infection further impairs sperm DNA, which is manifested by abnormal sperm nuclear maturity.

Adult ; Case-Control Studies ; Chromatin ; DNA ; genetics ; DNA Damage ; Hepatitis B ; pathology ; Hepatitis B virus ; Humans ; Infertility, Male ; genetics ; virology ; Male ; Sperm Count ; Spermatozoa ; pathology ; Young Adult

To explore new measures for functional reconstruction of multiple severe deformities as a result of extensive deep burn (total burn surface area > or = 90% TBSA, including deep burn > or = 70%TBSA) in late stage. Twelve severe burn patients with above-mentioned deformities were hospitalized in our ward during 1960--2005, the scars resulted from burns were distributed from head to foot with 173 deformities, including 27 scar ulcers. All patients lacked of self-care ability, among them some could not stand. Due to inadequate skin source, deformities were corrected by skin from matured scars expanded with subcutaneous balloon at late postburn stage. Following our former clinical experience, anatomic investigation and experimental research, we chose the following methods to correct deformities and restore functions: application of split-thickness scar skin after expansion (88 wounds); use of scar skin flap/scar-Achilles tendon flaps (59 wounds); combination of thin split-thickness skin grafts from scar and allogeneic acellular dermal matrix (composite skin, 40 wounds). All grafts survived, the appearance and function were improved obviously without complications. Follow-up 1-40 years, all patients could take care themselves with satisfactory function and appearance, and among them 8 patients returned to work (one had worked for 40 years), 2 patients married and had children. The above-mentioned measures are safe, reliable and effective for functional reconstruction of deformities.
Adult ; Burns ; complications ; surgery ; Cicatrix ; etiology ; surgery ; Contracture ; etiology ; surgery ; Female ; Humans ; Male ; Middle Aged ; Reconstructive Surgical Procedures ; methods ; Recovery of Function ; Skin Transplantation ; Skin, Artificial ; Surgical Flaps ; Wound Healing

OBJECTIVETo investigate the therapeutic effects of upper airway stenosis after Le Fort III osteotomy and midfacial distraction osteogenesis (DO).

METHODSEleven cases (age, 5-16 yrs) with severe midface dysostosis complicated with exophthalmos, anterior crossbite and upper airway stenosis were treated by using Le Fort III osteotomy and midfacial DO from August 2000 to February 2007. The 3D reconstruction of the upper-airway from CT data was used to evaluate the upper airway volume before and after the operation. And meanwhile polysomnography was carried out to demonstrate the upper airway functional changes.

RESULTSThere was a 64.3% mean increase [mean, (9.13 +/- 6.94) ml, P < 0.05] in upper airway volume in the 11 cases after the operations. It showed that there was significant improvements in the indexes of polysomnography after the operations, such as apnea and hypopnea index, average SaO2, minimum oxygen saturation and snore index.

CONCLUSIONSThe Le Fort III osteotomy and midfacial distraction osteogenesis can efficiently relieve the symptoms of upper-airway stenosis in severe midfacial dysostosis.

Acrocephalosyndactylia ; complications ; Adolescent ; Airway Obstruction ; etiology ; surgery ; Child ; Child, Preschool ; Craniofacial Dysostosis ; complications ; Female ; Follow-Up Studies ; Humans ; Male ; Osteogenesis, Distraction ; methods ; Osteotomy, Le Fort ; Treatment Outcome

OBJECTIVETo study an intervention model of "schools without infected students with schistosoma japonica", to control and prevent students from schistosoma infection.

METHODSTwelve primary schools of four heavy endemic counties (districts) with schistosomiasis in the Poyang Lake areas were selected as the study fields, of which, ten schools were the experimental groups, and the other two schools were the control groups by cluster random sampling. All enrolment students were the target population. The baseline survey was carried out in 2005, and an intervention model, "information dissemination + behavior participation + behavior encouragement", was applied in the experiment groups in 2006 - 2008, then the effect of intervention was assessed.

RESULTSBefore intervention (2005), the anti-schistosomiasis knowledge awareness rate of experimental and control groups were 14.75% (324/2196) and 16.58% (91/549), and the different was not significant (χ(2) = 1.14, P > 0.05); the rate of accurate attitude of anti-schistosomiasis were 14.71% (323/2196) and 11.84% (65/549) in experimental and control groups, and the difference was not significant (χ(2) = 2.98, P > 0.05); the rate of contacting infected water were 15.44% (18 988/122 976) and 15.03% (4622/30 744) in experimental and control group and the difference was not significant (χ(2) = 3.13, P > 0.05); and the infection rate of schistosomiasis of experiment control groups were 9.65% (212/2196) and 10.56% (58/549), the difference was not significant (χ(2) = 0.41, P > 0.05). After one year intervention (2006), the anti-schistosomiasis knowledge awareness rate of experimental and control groups were 97.79% (2032/2078) and 18.11% (98/541), and the different was significant (χ(2) = 1794.31, P < 0.01); the rate of accurate attitude of anti-schistosomiasis were 99.09% (2059/2078) and 13.49% (73/541) in experimental and control group, and the difference was significant (χ(2) = 2077.45, P < 0.01). After 1 - 3 years intervention (2006 - 2008), there were no any contactors with infected water and infectors with schistosome in students of the experiment group in successive 3 years. While in the control group of the same period, the rate contacting infected water were 16.12% (4884/30 296), 11.11% (3079/27 720) and 12.25% (3451/28 168); the infection rate of schistosomiasis were 8.87% (48/541), 7.47% (37/495) and 7.95% (40/503), respectively.

CONCLUSIONThe intervention model of health promotion, "information dissemination + behavior participation + behavior encouragement", can effectively control and prevent students from infecting schistosoma japonica in heavy endemic areas with schistosomiasis.

Animals ; Health Promotion ; Humans ; Schistosomiasis ; prevention & control ; Schistosomiasis japonica ; School Health Services ; Schools ; Students