Objective To observe the effect of inhaled nitric oxide(INO) on the nitric oxide synthase (NOS) in piglet suffered from severe meconium aspiration syndrome(MAS). Methods Severe MAS model of piglet was reproduced, the oxygenation effect and the activity of pulmonary NOS were measured, hybridization in situ of pulmonary slices was used to show the gene expression of inducible NOS(iNOS). Results INO therapy attenuated respiratory dysfunction in MAS, while discontinuation of INO induced “rebound pulmonary hypertension”. The activity of iNOS in MAS group is higher than that in control group (16.9?3.1) fmol/(mg Pro?min)vs (11.6?2.7) fmol/(mg Pro?min),( P

Objective To report the spectrum of electron transfer flavoprotein dehydrogenase (ETFDH)gene mutations in 20 Chinese RR-LsM families.Methods Twenty-four RR-LSM patients in the First Hospital of Peking University from January 2003 to May 2010 were collected and the clinical characteristics were analyzed.These patients came from 20 families in North Mainland China.Sixteen families had 1 patient each.and the other 4 families had 2 patients.ETFDH gene analysis was performed in all patients,11 family members and 100 healthy controls.Results The mean onset age was(27.9±9.9)years.The main symptoms were limb weakness(21,87.5%),dysmasesia(15,62.5%),neck weakness (14,58.3%)and myalgia(14,58.3%).Eighteen patients had high level of acyleamitine.Fifteen of 17patients had glutaric aciduria.Seventeen ETFDH mutations,including 13 missense mutations,2 splice mutations,and 2 nonsense mutations,were identified in 19 families:c.998A>G,c.1450T>C,c.1703T>C,c.1717C>T,c.821G>A,c.643G>A,c.251C>T,c.1763A>T,c.IVS7+2T>C and c.IVS6+1G>A were Hovel mutations which were not found in 100 healthy controls.Nine families had the mutation of c.770A>G(P.Y257C)and 5 families had the mutation of c.1227A>C(P.L409F).Conclusions The numerous novel mutations in ETFDH gene indicate that Chinese RR-LSM might have special mutation pattern.c.770A>G(P.Y257C)and c.1227A>C(p.L409F)may be hot spot mutations in North Mainland China.

ObjectiveTo investigate the characteristic of pathology in Chinese patients with Nonaka myopathy.MethodsThirteen patients (7 males and 6 females) diagnosed with Nonaka myopathy in our laboratory from January 2002 to March 2011 were included in this study.Their mean age was 39.5 years old and the mean duration of illness was 4.15 years.The most common symptoms were weakness of raising feet with sparing of quadriceps femoris muscles in the early stage of disease.One patient presented the initial symptoms of upper limb weakness. Muscles biopsies were obtained from all these 13 patients. Histology study including immunohistochemical (IHC) staining with antibody against amyloid 3,phosphorylated tau protein,ubiquitin,glucose-regulated protein of molecular weight 78 000(GRP78),calnexin,caspase-12and Bax were performed.Skeletal muscle samples from 3 chronic fatigue syndrome patients,2 myofibrillar myopathy patients were used for control in the IHC staining. All coding exons of uridinediphospho-N-acetylglucosamine 2-epimerase gene were directly sequenced in genomic DNA from these patients.Results The main pathological changes of tibialis anterior muscle in 12 cases were muscle dystrophy with rimmed vacuoles.The rimmed vacuoles were positive for anti-β-amyloid,tau protein and ubiquitin in IHC studies.In the atrophy fibers,IHC showed the increased expression of endoplasmic reticulum stress related proteins GRP78 and calnexin,and apoptosis proteins of caspase-12 and Bax.ConclusionsThere is accumulation of abnormal proteins in muscle fibers in Chinese patients with Nonaka myopahty.These proteins may stimulate endoplasmic reticulum stress and apoptosis,which may be a mechanism responsible for muscle damage.

Objective To investigate the effects of loop electrosurgical excision procedure (LEEP) on pregnancy and delivery outcomes of patients with cervical epithelial neoplasia (GIN). Method The delivery data of 27 pregnant women who had LEEP from May 1995 to April 2002 were retrospectively collected and analysed. Results The mean age of the 27 women was 30. 3 years ( range 23 -40 years). Ten women underwent artificial abortion. One ectopic pregnancy and two spontaneous miscarriages occurred at 6 to 8 weeks of gestation. There were 14 term pregnancies, 9 of them via vaginal delivery and 5 via caesarean section. The birth weight of newborns ranged from 2905 to 4000 g. All of newborns had a high Apgar score. No asphyxia occurred. Conclusions LEEP is a safe and effective treatment for patients with CIN. It will not increase the risk of pregnancy and delivery of the patients.

Objectives To analyse the clinical and MRI features of cerebral autosomal dominant arteriopathy with subcortical infarct and leucoencephalopathy (CADASIL) in mainland China and compare these features with those reported in other countries.Methods All 26 CADASIL families were collected in First Hospital of Peking University from January 2003 to October 2009, and the diagnosis was confirmed by ultrastructural examination or Notch3 gene analysis.The age of onset, initial symptoms, main symptoms in 102 patients were described and the features with those reported in Germany, Japan, Arab and France were compared using x2 test.The cranial MRI changes in 35 patients were analysed and compared with the British and French patients.Results 102 patients had their initial symptoms between 22-80 years with the mean age of onset at (43.9 ± 11.0) years.There was no significant difference in the frequency of ischemic stroke or TIA (79.41% ) between our patients and the patients in other countries.Dementia rate (50.00% ) was significantly greater than that of Arab (21.05% ,x2 =5.513, P =0.020) and French patients(31.11%,x2 =4.517, P =0.034).The frequency of mood disturbances ( 14.71% ) was significantly lower than that of German patients (30.39% ,x2 =7.185, P =0.007).The frequency of migraine ( 13.73% ) was similar to that of France but lower than that of Japan (40%, x2 = 12.658, P = 0.000), Germany (38.24%, x2 =15.932, P=0.000) and Arab (42.11% ,x2 =6.869, P=0.009).Cranial MRIs were abnormal in all 35patients, but no lesion was seen in the medulla oblongata.Lacunar infarcts in the basal ganglia (82.86% )were significantly more frequent than that of French patients (60% , x2 = 5.663, P = 0.017).The frequency of leukoaraiosis in the cerebrum was similar to that of French patients.Anterior temporal involvement (68.57%) was significantly lower than of British patients ( 95%, x2 = 5.211, P = 0.022 ).Conclusion The clinical symptoms and MRI changes of CADASIL in different countries were not identical.Abnormal anterior temporal pole signal on MRI is not a sensitive marker for diagnosis of CADASIL in Chinese patients.

Objective To report the inflammatory myopathy with abundant macrophages presenting with abundant foam cells in a Chinese patient.Methods A 44 year-old man with rheumatoid arthritis manifested as progressive proximal muscle weakness in the upper limbs for 13 months and in the lower limbs for 11 months.Laboratory tests showed that serum creatine kinase level was increased,whereas neither the myositis antibody nor paraneoplasma antibodies were detectable,including anti-Jo-1 antibody.Electromyogram showed a classic myogenic pattern with irritable sarcomembrane excitement.Muscle biopsies were carried out in the left and right biceps brechii in 2 months and 13 months of the disease course respectively.The muscle sections were processed with standard histological,enzyme histochemistrical and immunohistochemical workup.Results The first muscle biopsy revealed CD8 positive T lymphocytes infiltrating into major histocompatibility complex (MHC)-Ⅰ positive fibers and distributing around endomysium,accompanied with scattered necrosis and regeneration.The second muscle biopsy showed perifascicular atrophy,fragmented perimysial connective tissue,and a large amount of CD68 positive foam cells and Touton cells accumulating within perimysial connective tissues and endomysium.Some CD20 positive B lymphocytes and plasmacytes were found around perivascular space,but CD8 positive T lymphocytes were only accumulated in the endomysium.MHC- Ⅰ was darkly expressed in the sarcolemma.Conclusions Inflammatory myopathy with abundant macrophages can present with foam cells infiltrating in perimysial and endomysium.The disease can be associated with rheumatoid arthritis and refractory to corticosteroid therapy.

ObjectiveTo report a novel HTRA1 gene mutation caused cerebral autosomal recessive arteriopathy with subcortical infarcts and leukoencephalopathy (CARASIL) with migraine,urinary retention and constipation.MethodsThe patient was a 34-year-old woman who suffered from myalgia with cramp for 16 years,lumbago for 5 years,migraine and mild alopecia for 3 years,right hemiparesis for 5 months,and urinary retention and constipation for 1 month.Vertebral MRI showed degeneration of vertebral bodies with disc herniations.Brain MRI revealed diffuse white matter lesions and lacunar infarcts.Sural nerve and skin biopsies were performed in the patient. HTRA1 gene analysis was performed in patient,her parents and 2 brothers,and Notch3 gene analysis in the patient.ResultsThe sural biopsy demonstrated discontinuous of elastica internal with thickness of intima of arterioles.The capillary basement membranes were thickness with mini granular changes.A homozygous T to A transition at position 524( c.524T ＞ A) was found in HTRA1 gene.The heterozygous c.524T ＞ A mutation appeared in the parents and 2 brothers,but not in the controls.Notch3 mutations were not found in the patient. Conclusion CARASIL caused by novel homozygous c.524T ＞ A mutation of HTRA1 gene can present with migraine,urinary retention and constipation.

Objective To explore the time of application of hemoperfusion (HP) for the treatment of acute serious organophosphorus pesticide (ASOPP). Methods One hundred and four patients with ASOPP were randomly divided into two groups, 46 patients accepted traditional treatment(control group), 58 patients were treated with traditional treatment and HP (HP group). The patients in HP group were again divided into three groups according the different time of treatment (time of beginning HP after poisoning), the 4-8 hours group (HP-1 group, 27 patients), the 9-16 hours group (HP-2 group, 19 patients), the 17-32 hours group (HP-3 group, 12 pafients).Tbe coma period, the dosage of atropine, the time of regaining the vitality of cholinesterase, the time of hospitalization and the rate of fatality and curing among groups were observed. Results The coma period, the dosage of atropine, the time of regaining the vitality of cholinesterase, the time of hospitalization and the rate of fatality of the HP group were less than those of the control group (P<0.05). Compared with HP-1 group, the eoma period, the dosage of atropine, the time of regaining the vitality of eholinesterase and the time of hospitalization of the HP-2 group and the HP-3 group were higher (P<0.05), but the difference of the rate of fatality and curing between the HP-1 group and the other HP groups was not statistically significant (P>0.05). The difference of all of the above indicators between HP-2 group and HP-3 group was not statistically significant (P>0.05). Conclusion Application of hemoperfusion among 4-32 hours after poisoning for the treatment of ASOPP can improve the efficacy of therapy, and the efficacy of application of hemoperfusion among 4-8 hours is the best.

Objective:To illustrate the effectiveness of Teachermate platform combined with problem-based learning (PBL) in gynecology and obstetrics teaching.Methods:Students from different classes were randomly divided into experimental group and control group, and the general data of the two groups were compared. The two groups respectively adopted Teachermate platform combined with PBL and traditional lecture teaching methods. The class attendance rate and the scores of relevant chapters in the final examination were analyzed through statistical methods for assessing the enthusiasm of students and teaching quality in class in terms of the same content and the same class hours. A questionnaire survey was conducted to know students' evaluation of this teaching mode, evaluating its teaching effect and satisfaction comprehensively.Results:There was no difference in general information between the two groups. The class attendance rate of the experimental group (274/279) was significantly higher than that of the control group (61/90) ( P=0.00), while the scores of the relevant chapters in the final exam were also significantly higher ( P=0.00). Questionnaire survey results among 93 students in the experimental group were taken back 93 valid questionnaires (the effective rate, 100%), showing that most of the students were very satisfied with this teaching mode (90/93) and hoped to apply it to more subjects teaching (89/93). Conclusion:The application of Teachermate platform combined with PBL in gynecology and obstetrics class teaching contributes to improve students' participating enthusiasm and can effectively improve the quality and efficiency of teaching. Most students highly recognize the teaching method, which is worth applying and popularizing.

Objective:To illustrate the effectiveness of Micro-Teachermate platform combined with problem-based learning (PBL) in gynecology and obstetrics teaching.Methods:Students from different classes were randomly divided into experimental group and control group, and the general data of the two groups were compared. The two groups respectively adopted Micro-Teachermate platform combined with PBL and traditional lecture teaching methods. The class attendance rate and the scores of relevant chapters in the final examination were analyzed through statistical methods for assessing the enthusiasm of students and teaching quality in class in terms of the same content and the same class hours. A questionnaire survey was conducted to know students' evaluation of this teaching mode, evaluating its teaching effect and satisfaction comprehensively. SPSS 17.0 was used for t test and chi-square test. Results:There was no difference in general information between the two groups. The class attendance rate of the experimental group (274/279) was significantly higher than that of the control group (61/90), while the scores of the relevant chapters in the final exam were also significantly higher ( P<0.01). Questionnaire survey results among 93 students in the experimental group were taken back 93 valid questionnaires (the effective rate, 100%), showing that most of the students were very satisfied with this teaching mode (90/93) and hoped to apply it to more subjects teaching (89/93). Conclusion:The application of Micro-Teachermate platform combined with PBL in gynecology and obstetrics class teaching contributes to improve students' participating enthusiasm and can effectively improve the quality and efficiency of teaching. Most students highly recognize the teaching method, which is worth applying and popularizing.