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

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 evaluate the relationship between idiopathic Parkinson's disease (PD) and two polymorphisms (C243G and A377T) of the gamma-synuclein gene in a Chinese Han population of Shanghai area.

METHODSPolymorphic genotyping was performed with PCR-RPLP technique. Association analysis was carried out in 145 unrelated idiopathic PD patients and 184 age-matched healthy controls.

RESULTSThe authors failed to detect any distributional difference of the C243G and A377T polymorphisms of the gamma-synuclein gene between PD cases and control subjects, nor did they find any association.

CONCLUSIONThese data do not support that gamma-synuclein gene C243G and A377T polymorphisms are involved in idiopathic PD onset in the Han population of Shanghai area.

Adult ; Aged ; Aged, 80 and over ; Alleles ; Gene Frequency ; Genotype ; Humans ; Middle Aged ; Nerve Tissue Proteins ; genetics ; Parkinson Disease ; genetics ; Polymorphism, Single Nucleotide ; Synucleins ; gamma-Synuclein

OBJECTIVETo explore the methods of systemic treatment of defects of skin and soft tissue on the knees after severe trauma or burn.

METHODSTwenty patients with defects of skin and soft tissue on the knees after severe trauma or burn hospitalized in our center from January 2009 to December 2011. The injury areas on the knees ranged from 5 cm×4 cm to 30 cm×20 cm. The wounds were treated with radical debridement, vacuum sealing drainage, and douche through dripping to control infection in early stage. Then they were covered with transplantation of skin grafts plus flap or only with flap. Totally 8 local flaps (including 6 local rotation or transposition flaps and 2 saphenous artery flaps) and 12 free flaps (including 8 anterolateral thigh flaps and 4 latissimus dorsi musculocutaneous flaps) were used. The flap size ranged from 6 cm×5 cm to 32 cm×22 cm. The rehabilitation training of the knee joints was carried out in the early stage after wound healing.

RESULTSAll free skin grafts and flaps used in 15 patients survived. Thirteen of them were primarily healed, while some small parts of skin grafts of the other two patients were in poor condition because of infection, and they healed after another session of skin transplantation. Infection occurred under the free flap in one of the 5 patients transplanted with flaps only, which was healed after continuous douche through dripping and another surgical debridement following wet dressing. The knee joints were in good function during the follow-up period of 1 - 3 years.

CONCLUSIONSThe systemic therapy of radical debridement, vacuum sealing drainage technique, douche through dripping, transplantation of large autologous grafts and flaps, and the early rehabilitation training are effective and reliable in repairing defects of skin and soft tissue at the knee region after severe injuries.

Adolescent ; Adult ; Burns ; complications ; Child ; Female ; Follow-Up Studies ; Humans ; Knee Injuries ; surgery ; Male ; Middle Aged ; Skin Transplantation ; Soft Tissue Injuries ; surgery ; Surgical Flaps ; Young Adult

OBJECTIVETo explore the means for the reconstruction of extensive deep burn wounds with exposure of bone and joint in late stage.

METHODSAmong all the patients with extensive deep burn hospitalized between January 2009 and May 2011, 5 patients presented wounds with exposure of bone and joint in the late stage of treatment that could not be covered by free skin grafts or flaps. Two of the five patients had more than 2 and the other 3 patients had only one such wound(s). The wound size ranged from 8 cm×5 cm to 21 cm×8 cm. Wounds were repaired by transplantation of 7 free muscle flaps (including 4 free rectus abdominis flaps and 3 latissimus dorsi flaps) combined with split-thickness skin grafts harvested from scalp.

RESULTSAll the muscle flaps and skin grafts survived. Wounds with bone and joint exposure healed well. At one-year follow-up of some patients, good appearance of repaired areas and normal function of joints were observed with no signs of ulceration, arthritis, or osteomyelitis.

CONCLUSIONSTransplantation of free muscle flaps combined with split-thickness skin grafts harvested from the scalp provides satisfactory reconstruction for wounds with deep tissue exposure in patients with a shortage of skin donor site.

Adult ; Burns ; surgery ; Free Tissue Flaps ; Humans ; Male ; Middle Aged ; Muscle, Skeletal ; injuries ; Reconstructive Surgical Procedures ; methods ; Rectus Abdominis ; transplantation ; Wound Healing ; Young Adult

Alteration in the balance between cell apoptosis and proliferation is one of the pathophysiological mechanisms of the myelodysplastic syndromes (MDS). The question of whether the excessive apoptosis and/or proliferation predominantly involve the subset of progenitor cells (CD34(+) cells) or mature cells (CD34(-) cells) remains a controversial issue. This study was purpose to analyze the apoptosis and proliferation status of CD34(+) and CD34(-) cells in bone marrow (BM) of patients with MDS, to investigate the pathogenesis of MDS and to determine the relation of apoptosis and proliferation status of CD34(+) and CD34(-) cells with prognosis of MDS. The proportion of CD34(+) cells, the apoptosis and proliferation ratio (A/P) of CD34(+) and CD34(-) cells in BM of 40 patients with MDS, including 20 cases of high-risk MDS and 20 cases of low-risk MDS, and 10 normal persons as control were detected by flow cytometry; the influence of CD34(+) and CD34(-) cell apoptosis and proliferation levels on prognosis of MDS was evaluated by univariate and multivariate analysis of survival. The results showed that the proportion of CD34(+) cells in BM of high-risk MDS patients was significantly higher than that in BM of low-risk MDS patients and in normal BM [(1.92 ± 0.10)%, (1.09 ± 0.04)%, (1.03 ± 0.05)% respectively]. The apoptotic rates (AR) of both CD34(+) and CD34(-) cells were significantly higher in low-risk MDS [(54.75 ± 2.18)%, (80.36 ± 1.68)%] than in high-risk MDS [(24.87 ± 2.69)%, (23.12 ± 1.23)%] and in normal BM [(18.51 ± 2.74)%, (20.98 ± 2.21)%]. When compared between CD34(+) cells and CD34(-) cells in low-risk MDS, a greater AR of CD34(-) cells was found. However, the higher proliferative rate of CD34(+) cells was observed in high-risk MDS. In low-risk MDS, a higher A/P ratio was found in CD34(-) cells than in CD34(+) cells; whereas this ratio was equalized or inverted in high-risk MDS. In addition, the survival and prognosis correlated significantly with AR of CD34(+) cells. It is concluded that the early MDS is predominantly associated with excessive apoptosis of the mature CD34(-) cells. The proliferation rate of cells increases with the disease progression in MDS subsets, especially, in the subset of CD34(+) cells. Surprisingly, the apoptosis of CD34(+) cells may be a useful prognostic factor, and the inhibition of apoptotic mechanisms may induce the transformation of MDS to leukemia.
Adolescent ; Adult ; Aged ; Antigens, CD34 ; Apoptosis ; Bone Marrow Cells ; immunology ; pathology ; Case-Control Studies ; Cell Proliferation ; Child ; Female ; Humans ; Male ; Middle Aged ; Myelodysplastic Syndromes ; mortality ; pathology ; Prognosis ; Survival Rate ; Young Adult

OBJECTIVETo observe the therapeutic effects of Le Fort III osteotomy and midface distraction osteogenesis (DO) on the upper-airway narrow.

METHODSSince 2000, 11 cases (10 cases of Crouzon syndrome and 1 case of Apert syndrome) with severe midface deficiency were treated with Le Fort III osteotomy and midface DO. The section area of different parts of upper-airway were tested by computer assistants image measurement preoperatively and postoperatively. Some patients received sleep function monitoring.

RESULTSThe face appearance and the function of upper-airway improved significantly after Le Fort III osteotomy and Midface DO. The section area at the level of posterior nasal spine and uvula increased obviously after treatment (P < 0.05), however the section area at the level of epiglottis and separation between airway and esophagus were not obviously enlarged (P > 0.05).

CONCLUSIONSMidface DO after Le Fort III osteotomy can effectively improve the upper-airway narrow, especially the upper part from uvula.

Adolescent ; Airway Obstruction ; etiology ; surgery ; Child ; Child, Preschool ; Craniofacial Dysostosis ; complications ; surgery ; Epiglottis ; pathology ; Female ; Humans ; Male ; Nasopharynx ; pathology ; Osteotomy, Le Fort ; methods ; Palate, Soft ; pathology ; Postoperative Period ; Sleep Apnea, Obstructive ; etiology ; surgery ; Treatment Outcome