Genetic analysis of a Chinese pedigree affected with Mucopolysaccharidosis type ⅢA / 中华医学遗传学杂志

OBJECTIVE@#To explore the clinical and genetic characteristics of a patient with hypertrophic cardiomyopathy as the initial manifestation of Mucopolysaccharidosis type Ⅲ A (MPS Ⅲ A).@*METHODS@#A female patient with MPS Ⅲ A who was admitted to the Affiliated Hospital of Jining Medical University in January 2022 and her family members (seven individuals from three generations) were selected as the study subjects. Clinical data of the proband were collected. Peripheral blood samples of the proband was collected and subjected to whole exome sequencing. Candidate variants were verified by Sanger sequencing. Heparan-N-sulfatase activity was determined for the disease associated with the variant site.@*RESULTS@#The proband was a 49-year-old woman, for whom cardiac MRI has revealed significant thickening (up to 20 mm) of left ventricular wall and delayed gadolinium enhancement at the apical myocardium. Genetic testing revealed that she has harbored compound heterozygous variants in exon 17 of the SGSH gene, namely c.545G>A (p.Arg182His) and c.703G>A (p.Asp235Asn). Based on guidelines from the American College of Medical Genetics and Genomics (ACMG), both variants were predicted to be pathogenic (PM2_Supporting +PM3+PP1Strong+PP3+PP4; PS3+PM1+PM2_Supporting +PM3+PP3+PP4). Sanger sequencing confirmed that her mother was heterozygous for the c.545G>A (p.Arg182His) variant, whilst her father, sisters and her son were heterozygous for the c.703G>A (p.Asp235Asn) variant. Determination of blood leukocyte heparan-N-sulfatase activity suggested that the patient had a low level of 1.6 nmol/(g·h), whilst that of her father, elder and younger sisters and son were all in the normal range.@*CONCLUSION@#The compound heterozygous variants of the SGSH gene probably underlay the MPS ⅢA in this patient, for which hypertrophic cardiomyopathy is an associated phenotype.

Chinese consensus of cardiopulmonary resuscitation guides prevention, treatment and rescue of cardiac arrest in pregnancy / 中华危重病急救医学

Pregnant women are a group of people in a special period, once sudden cardiac arrest (CA) occurs, it will threaten the life of both mother and child. It has become a great challenge for hospital, doctors and nurses to minimize maternal mortality during pregnancy. All the efforts should ensure the safety of both mother and child throughout the perinatal period. Because difference of the cardiopulmonary resuscitation strategies for common CA patients of the same age, the resuscitation strategies for CA patients during pregnancy need consider the patient's gestational age and fetal condition. Different resuscitation techniques, such as manual left uterine displacement (MLUD), will involve perimortem cesarean delivery (PMCD). At the same time, drugs should be reasonably used for different causes of CA during pregnancy, such as hypoxemia, hypovolemia, hyperkalemia or hypokalemia and other electrolyte disorders and hypothermia in 4Hs, as well as thrombosis, pericardial tamponade, tension pneumothorax and toxicosis in 4Ts. In view of the fact that many causes of CA in pregnancy are preventable, it is more necessary to introduce guidelines for CA in pregnancy in line with our national conditions for clinical guidance. This paper systematically reviewed the pathophysiological characteristics of CA during pregnancy, the high-risk factors of CA during pregnancy, and identified the correct resuscitation methods and prevention and treatment strategies of CA during pregnancy.

Regulatory effects of umbilical cord mesenchymal stem cells and their conditioned medium on gut microbiota of septic mice / 中华危重病急救医学

Objective:To investigate and compare the regulatory effects of umbilical cord mesenchymal stem cells (MSC) and their conditioned medium (MSC-CM) on gut microbiota of septic mice.Methods:Twenty-eight six-to-eight-week-old female C57BL/6J mice were randomly divided into sham operation group (Sham group), sepsis model group (CLP group), sepsis+MSC treatment group (CLP+MSC group) and sepsis+MSC-CM treatment group (CLP+MSC-CM group), with seven mice in each group. The septic mouse model was established by cecal ligation and puncture (CLP). In Sham group, CLP were not performed, and other operations were the same as CLP group. Mice in the CLP+MSC group and CLP+MSC-CM group received 0.2 mL 1×10 6 MSC or 0.2 mL concentrated MSC-CM via intraperitoneal injection 6 hours after CLP, respectively. Sham group and CLP group were given 0.2 mL sterile phosphate buffer saline (PBS) via intraperitoneal injection. Histopathological changes were evaluated by hematoxylin-eosin (HE) staining and colon length. Levels of inflammatory factors in serum were detected by enzyme-linked immunosorbent assay (ELISA). Phenotype of peritoneal macrophages was analyzed by flow cytometry, and the gut microbiota was analyzed via 16S rRNA sequencing. Results:Compared with Sham group, significant inflammatory injury in lung and colon was observed, and shorter colon was detected in CLP group (cm: 6.00±0.26 vs. 7.11±0.09), the level of inflammatory cytokine interleukin-1β (IL-1β) in serum was significantly increased (ng/L: 432.70±17.68 vs. 353.70±17.01), the proportion of F4/80 + peritoneal macrophages was increased [(68.25±3.41)% vs. (50.84±4.98)%], while the ratio of F4/80 +CD206 + anti-inflammatory peritoneal macrophages was decreased [(45.25±6.75)% vs. (66.66±3.36)%]. The α diversity sobs index of gut microbiota was downregulated significantly (118.50±23.25 vs. 255.70±6.87), the structure of species composition was altered, and the relative abundance of functional gut microbiota related to transcription, secondary metabolites biosynthesis, transport and catabolism, carbohydrate transport and metabolism, and signal transduction were decreased significantly in CLP group (all P < 0.05). Compared with CLP group, upon MSC or MSC-CM treatment, the pathological injury in lung and colon was alleviated to varying extent, the length of colon was increased (cm: 6.53±0.27, 6.87±0.18 vs. 6.00±0.26), the level of IL-1β in serum was downregulated (ng/L: 382.10±16.93, 343.20±23.61 vs. 432.70±17.68), the ratio of F4/80 + peritoneal macrophages was decreased [(47.65±3.93)%, (48.68±2.51)% vs. (68.25±3.41)%], the ratio of F4/80 +CD206 + anti-inflammatory peritoneal macrophages was increased [(52.73±5.02)%, (66.38±4.73)% vs. (45.25±6.75)%], and the α diversity sobs index of gut microbiota was increased (182.50±16.35, 214.00±31.18 vs. 118.50±23.25), and the effects of MSC-CM were more significant (all P < 0.05). At the same time, species composition of gut microbiota was rebuilt, and a tendency of increase in relative abundance of functional gut microbiota was observed upon MSC and MSC-CM treatment. Conclusion:Both MSC and MSC-CM could alleviate inflammatory injury in tissues, and showed regulatory effects on gut microbiota in septic mouse model, moreover, MSC-CM exhibited superior advantages over MSC.

Expert consensus for diagnosis and treatment of post-cardiac arrest syndrome in adults by combining traditional Chinese and Western medicine in China (2023) / 中华危重病急救医学

Reperfusion injury occurs after return of spontaneous circulation (ROSC) in patients with cardiac arrest (CA), which leads to multiple organ dysfunction, called post-cardiac arrest syndrome (PCAS). PCAS is closely related to the prognosis of CA patients, and is an independent risk factor of survival. Integrated traditional Chinese and Western medicine diagnosis and treatment is critical for improving prognosis of PCAS. In order to guide and standardize integrated traditional Chinese and Western medicine diagnosis and treatment in PCAS among clinicians, nurses and research personnel in China, the Emergency Medicine Professional Committee of the Chinese Society of Integrated Chinese and Western Medicine has established an expert group to determine 14 clinical issues related to the diagnosis and treatment of PCAS with integrated traditional Chinese and Western medicine through clinical survey. The working group formulates a search strategy for each clinical issue according to the PICO principle. Chinese and English literature were searched from CNKI, Wanfang, VIP, SinoMed, PubMed, Embase, and Cochrane Library. The grade of recommendations assessment, development and evaluation (GRADE) were used to form the level of evidence and recommendation. When the literature evidence was insufficient, the recommendations and level of recommendation were formed after expert discussion. Combined with the aspects of generalizability, suitability, and resource utilization, the expert consensus developed 28 recommendations around the 14 aspects of three stages of PCAS, including early circulation, respiratory support and reversible cause relief, mid-term neuroprotection, improvement of coagulation, prevention and treatment of infection, kidney and gastrointestinal protection and blood sugar control, post rehabilitation treatment, providing references for the integrated traditional Chinese and Western medicine of the diagnosis and treatment for PCAS.

Research progress of the classic prescription Tianwang buxin dan in the treatment of insomnia / 中国药房

Tianwang buxin dan （TWBXD），as a classic prescription for treating insomnia ，in forms of tablet ，drop pill ，patch and others ，has been widely used in clinical practice . Its compound prescription and single ingredient can play a calming and tranquilizing role by regulating neurotransmitters ，inhibiting oxidative stress reaction and the expression of inflammatory factors . Based on the theory of traditional Chinese medicine and combined with modern pharmacological research ，this paper reviews single ingredient and active components of TWBXD ，calming and tranquilizing role of related couplet medicinals . The results showed that the single ingredients such as Rehmannia glutinosa ，the seeds of Zizyphus jujube ，the seeds of Platycladus orientalis ，Angelica sinensis，Schisandra chinensis ，Poria cocos and their effective components as well as related couplet medicinals of Z. jujube could improve the insomnia symptoms in different degrees . Except that the mechanism of the seeds of P. orientalis has not been studied ， other ingredients mainly act on nervous -endocrine-immune system . They can play the sedative and hypnotic effects by regulating neurotransmitters，inflammatory factors and oxidation .

Effect of mindfulness-based childbirth on pregnant women with anxiety and depression disorders / 四川精神卫生

ObjectiveTo explore the effect of mindfulness-based childbirth on pregnant women's anxiety and depression， and to provide references for related psychological intervention. MethodsA total of 80 pregnant women who attended the obstetrics department of the Third People's Hospital of Mianyang from October 2018 to March 2019 and met the diagnostic criteria of mild to moderate anxiety and depression in the Diagnostic and Statistical Manual of Mental Disorders， fifth edition （DSM-5） were selected， and they were divided into two groups by random number table method， each with 40 cases. Both groups received regular mental health education. On this basis， experimental group received mindfulness-based childbirth training for 8 times， once a week. Meantime， all pregnant women were assessed using Patient Health Questionnaire-9 item （PHQ-9） and Generalized Anxiety Disorder Scale （GAD-7） at the baseline and after intervention. ResultsAt the baseline and after intervention， the scores of PHQ-9 ［（6.95±1.40） vs. （2.10±1.34）， P<0.05］ and GAD-7 ［（7.23±1.29） vs. （2.08±1.51）， P<0.05］ in the experimental group had statistical significance， and the scores of PHQ-9 ［（7.00±1.43） vs. （3.65±1.35）， P<0.05］ and GAD-7 ［（7.18±1.57） vs. （3.38±1.66）， P<0.05］ in the control group had statistical significance. Moreover， the post-intervention scores of PHQ-9 and GAD-7 were significantly lower in experimental group than in control group （t=5.161， 3.666， P<0.01）. ConclusionCompared with mental health education alone， its combination with mindfulness-based childbirth achieves better effect in alleviating the anxiety and depression status of pregnant women.

Treatment of 2019 novel coronavirus pneumonia based on the theory of 'three syndromes and three methods' / 中华危重病急救医学

The latest diagnosis and treatment plan (4th edition) of 2019 novel coronavirus pneumonia has been issued. The diagnosis and treatment plan highlights the concept of integrated traditional Chinese and Western medicine, and Xuebijing injection was referred for three times. Xuebijing injection was successfully developed based on the theory of 'three syndromes and three methods'. The theory of 'three syndromes and three methods' is a theoretical system of integrated traditional Chinese and Western medicine on critical diseases proposed by Professor Wang Jinda and his team in the 1970s, and it is one of the main contents of Wang Jinda's academic thought. The theory of 'three syndromes and three methods' has a deep foundation of traditional Chinese medicine theory, and it is still being continuously enriched and improved. It is also supported by multiple evidence-based data. Therefore, 'three syndromes and three methods' has rich theoretical connotation and tenacious vitality.

Treatment of 2019 novel coronavirus pneumonia based on the theory of "three syndromes and three methods" / 中华危重病急救医学

The latest diagnosis and treatment plan (4th edition) of 2019 novel coronavirus pneumonia has been issued. The diagnosis and treatment plan highlights the concept of integrated traditional Chinese and Western medicine, and Xuebijing injection was referred for three times. Xuebijing injection was successfully developed based on the theory of "three syndromes and three methods". The theory of "three syndromes and three methods" is a theoretical system of integrated traditional Chinese and Western medicine on critical diseases proposed by Professor Wang Jinda and his team in the 1970s, and it is one of the main contents of Wang Jinda's academic thought. The theory of "three syndromes and three methods" has a deep foundation of traditional Chinese medicine theory, and it is still being continuously enriched and improved. It is also supported by multiple evidence-based data. Therefore, "three syndromes and three methods" has rich theoretical connotation and tenacious vitality.

Expert consensus on diagnosis and treatment of septic shock with integrated traditional Chinese and Western medicine / 中华危重病急救医学

Septic shock is a pathological stage during the progress of sepsis. The mortality rate is high, and timely, standardized and comprehensive treatment is of great significance in reducing the mortality rate. At present, there are many domestic and international guidelines for the diagnosis and treatment of sepsis and septic shock. On this basis, China Association of Integrative Medicine Emergency Medicine Committee organized experts to collect evidence-based medical evidence and expert experience in the diagnosis and treatment by integrated Chinese and Western medicine, and referred to the Expert consensus on diagnosis and treatment of sepsis with integrated traditional Chinese and Western medicine published by China Association of Integrative Medicine Emergency Medicine Committee in 2013, developed Expert consensus on diagnosis and treatment of septic shock with integrated traditional Chinese and Western medicine. The consensus provided a "China approach" for the resolution of this worldwide problem. In the "China approach", the staging of septic shock is "longitude", and the "four syndromes and four methods" treatment thought is "latitude". Under the framework of interlaced latitude and longitude, we recommend a series of effective integrated Chinese and Western medicine treatment. The method embodies the wisdom of China and has certain guiding significance for the clinical treatment of septic shock.

Current status and prospect of treatment for severe community acquired pneumonia / 中华危重病急救医学

Severe community acquired pneumonia (SCAP) has raised substantial concern due to high morbidity and mortality. SCAP is usually associated with multiple organ dysfunction syndrome (MODS) and sepsis. It is suggested that organ function and coagulation/fibrinolysis improvement, organ injury reduction and organ repair facilitation are highly required besides microbiology detection and optimization of antibiotics strategy on treatment of SCAP. Neutralization antibody, steroid, immunmodulatory therapy, and Chinese medicine etc. contribute to organ function improvement from different perspective. The unique profile of multiple target and personalized therapy of Chinese medicine may become a promising new option for SCAP and MODS. The combination of Western and Chinese Medicine shed new light on SCAP treatment.

Expert consensus on application of Chinese patent medicine for acute upper respiratory tract infection / 中国中西医结合急救杂志

Expert Consensus on the Application of Chinese Patent Medicine for Acute Upper Respiratory Tract Infection was established under the joint sponsorship of Specialty Committee of Emergency of World Federation of Chinese Medicine Societies, Emergency Physician Branch of Chinese Medical Doctor Association, Emergency Medicine Professional Committee of Chinese Association of Integrative Medicine and Chinese Emergency Medical Parternerships. In the consensus, the Chinese patent medicines for treatment of acute upper respiratory tract infection (AURI) were summarized and analyzed, and after the expert writers had discussed the contents of the consensus together, they decided to formulate the experts' consensus related to the AURI, expecting to provide a reference to the clinical treatment of this disease.

Distinguishing the "Wei Qi Ying Xue" syndrome differentiation from the "three certificates and three methods" to the diagnosis of sepsis / 中华危重病急救医学

The?"three?certificates?and?three?methods"?is?a?combination?of?Chinese?and?Western?medicine?for?the?treatment?of?sepsis?proposed?by?Professor?Wang?Jinda,?a?pioneer?in?Chinese?emergency?medicine.?The?"Wei?Qi?Ying?Xue"?syndrome?differentiation?is?a?dialectical?theory?proposed?by?Ye?Tianshi,?a?famous?doctor?in?the?Qing?Dynasty,?for?the?treatment?of?warm?diseases.?The?"three?certificates?and?three?methods"?theory?of?"toxin-heat?syndrome"?(inflammation?storm)?to?the?"blood?stasis?syndrome"?(coagulation?storm)?conversion?mainly?focus?on?the?"Wei?Qi?Ying?Xue"?syndrome?differentiation?of?the?"Wei,?Qi?syndrome"?to?"Ying,?Xue?syndrome",?and?poison.?The?conversion?of?"toxin-heat?syndrome"?to?"blood?stasis?syndrome"?is?a?watershed?in?the?progression?of?common?infections?to?sepsis.?The?timely?application?of?Xuebijing?injection?can?cut?off?this?transformation,?which?is?one?of?the?reasons?why?Xuebijing?injection?can?treat?sepsis.

Investigation report on the effect of large hospital's group teaching in a hospital located in the remote area / 中国中西医结合急救杂志

Objective To evaluate the effectiveness of group teaching of Third Hospital of Peking University (Beijing Medical Third Hospital) for a hospital located in remote area. Methods From 2016 to 2017, 19 medical staff from Peking University Third Hospital gave their support to Huanxian People's Hospital and provided respectively the theoretical support, guidance of clinical skills, medical consultation, free paid clinic, outpatient service, and teaching rounds, etc. A questionnaire survey was carried out to realize the subjective evaluation of the 155 clinical front-line staff who had participated in the class regarding the effect of group teaching and the most welcome teaching mode and contents; the objective data on the monthly outpatient visits, number of patients admitted into the hospital, the number of surgical patients, etc were calculated and compared between the pre-support (2014 to 2015) and post-support (2016 to 2017) period to evaluate the effect of counterpart support. Results One hundred and fourteen of the 155 medical staff in their respective departments were trained by the experts, and the percentage of medical staff believed that the counterpart support helped clinical work very much accounting for [58.8% (67/114)] being significantly higher than those who considered of no help [9.6% (11/114)], a little help [16.7% (19/114)] and some help [14.9% (17/114), χ2= 94.082, P = 0.000]; after support, the number of monthly outpatient visits compared to that of pre-support period (case times:43 816.5±3 406.9 vs. 37 319.5±4 302.1) and the number of monthly patients hospitalized compared to that of pre-support period (case times: 2 978.3±235.1 vs. 2 250.9±218.1) were significantly higher than those before the support (all P < 0.05), no statistical significant difference in monthly surgery volume (case times: 449.8±107.3 vs. 459.0±85.4, P > 0.05) between the above 2 periods was seen. The survey showed that the mostly demanding teaching method was theoretical teaching [65.8% (102/155)], followed by teaching rounds [50.3% (78/155)] and outpatient teaching the least [18.1% (28/155)]. That the ratios of various teaching contents with their sequence from high to low the medical personnel required were as follows: to learn new technologies [76.8% (119/155)], medical frontier knowledge [68.4% (106/155)], and standard diagnosis and treatment manipulations [67.7% (105/155)], cultivation of clinical thinking [66.5% (103/155)] and passing on surgical skills [56.1% (87/155)]. There were statistical significant differences between each teaching method and teaching content (all P < 0.05). Conclusions Teaching to cultivate qualified health personnel is the key to the development of hospitals located in remote areas, according to their needs, combining local conditions and the establishment of long-term cooperation mechanisms, we can comprehensively improve the professional quality of medical personnel and the overall strength of hospitals located in remote areas.

Research progress of acute cardiorenal syndrome / 中国中西医结合急救杂志

The heart and kidney damage is a clinical disease commonly seen, the 2 organs can interact with each other as cause and effect, leading to a series of clinical symptoms which is the cardiorenal syndrome (CRS). In 2008, according to the connection between the heart and kidney, the nephrologists Ronco, etc, completed the definition and classification of CRS, including type Ⅰ and type Ⅲ of CRS being acute cardiorenal syndrome (ACRS). ACRS refers to the fact that when the damage of heart or kidney dysfunction influences each other leading to a clinical syndrome caused by a sharp deterioration of cardiorenal function. At present, no definite diagnostic criteria for ACRS have yet been made. The pathogenesis of ACRS may be related to the renin-angiotensin-aldosterone system (RAAS), nitric oxide-reactive oxygen species (NO-ROS) system, inflammatory reaction, the excessive activation of sympathetic nervous system and so on. Clinically, about 50% of ACRS patients are accompanied by acute decompensated cardiorenal dysfunction or failure, that seriously impact on the patients' clinical prognosis and survival rate, so it is necessary to find an effective therapeutic regimen. At present, the treatments of ACRS have mainly the diuretic, angiotensin converting enzyme inhibitor (ACEI), angiotensin receptor inhibitor (ARB), β-receptor blocker, positive inotropic drugs, recombinant human erythropoietin, recombinant human brain natriuretic peptide, continuous blood purification (CBP) etc, and traditional Chinese medicine (TCM) also has a certain effect for improving the clinical symptoms of ACRS patients. Now the pathogenesis, diagnosis, and combined treatment of TCM and western medicine for treatment of ACRS are summarized.

Some suggestions on standardized training of resident doctors in a primary hospital / 中国中西医结合急救杂志

Objective To explore the issues related to the standardized training of resident doctors at the primary hospital and provide basis for the standardization of doctors' training. Methods Questionnaires were used to investigate the resident titles, ages, and education background in the Huanxian People's Hospital. The self-assessment of the competency of resident doctors and professional contents necessary for their improvement were analyzed to identify the medical topics should be recommended to improve their clinical knowledge and techniques. Results Forty-six of 52 registered residents in the Huanxian People's Hospital participated in the survey with a participation rate of 88.5%. In this hospital, mainly the title of physicians was resident, accounting for 43.3% (52/120), the ages were chiefly in the range of 31 - 35 years, and their education levels were college degree 42.3% (22/52) and bachelor degree 48.1% (25/52). 76.1% (35/46) of the residents found they had difficulties in working and not competent enough on the present post, and 71.7% (33/46) of residents considered that they were in urgent need to strengthen their trainings of clinical thinking and clinical skills. Conclusions Strengthening the residents' self academic qualifications, improving the hospital teaching system, enhancing the teaching management, and focusing on clinical thinking and clinical skills training are the main issues in the current standardized training in primary hospitals. By accelerating the standardized training of residents, they may have the chance to transform to be the middle-level physicians and improve the unreasonable structure of doctor title distribution that will play the key role in the development of primary hospitals.

Experimental basis and clinical practice of "bacterial poison and cure" sepsis / 中华危重病急救医学

Since the 20th century, research team of Professor Wang Jinda in the Tianjin First Central Hospital has been using blood circulation drugs to rescue critically ill patients for 70 years. With the deepening of the understanding of sepsis, put forward the "bacteria and inflammation and governance" theory, and the use of blood circulation drugs Xuebijing injection with anti-endotoxin and inflammatory mediators effect in the treatment of sepsis, some clinical efficacy has been found. However, in recent years, the progress of treatment of sepsis at home and abroad is slow, we review a series of animal experiments since the late of 1980s, and found that small thrombosis in the microcirculation and coagulation disorders play a more important role in the development of local infection to sepsis transition. Treatment combined with Xuebijing injection is proved effective with more and more clinical practice, so the important status of blood stasis and blood circulation method in sepsis must be paid attention to again.

Non-congenital heart disease related pulmonary arterial hypertension in 45 children / 中华实用儿科临床杂志

Objective To improve the knowledge of paediatric pulmonary arterial hypertension(PAH) and to elevate the level of early diagnosis and treatment.Methods The clinical data of 45 PAH patients admitted in Shanghai Children's Hospital from Jan.2006 to Dec.2012 were reviewed,including clinical manifestation,laboratory examination,diagnosis,treatment and prognosis.Results Of the 45 PAH patients,21 cases (46.7％) were male and 24 cases (53.3 ％) were female,with an average age of 2.5 years old.Among them idiopathic PAH was in 24 cases (53.3 ％) and secondary PAH was in 21 cases [including interstitial lung disease in 4 cases,upper airway obstruction in 3 cases,systemic lupus erythematosus in 3 cases,hepatic disease in 3 cases,including hepatic hemangioma 1 case,liver cirrhosis portal hypertension 1 case and autoimmune hepatic sarcoidosis 1 case,tachycardia induced cardiomyopathy in 2 cases,extensive pulmonary small artery stenosis in 2 cases,human immunodeficiency virus (HIV) infection in 1 case,hypothyroidism in 1 case,and familial PAH in 2 cases].Main clinical manifestations were anhelation after exercise (71.1％),fatigue (68.9％),cough (48.9％),chest tightness (26.7％),chest pain (33.3％),syncope (8.9％),et al.The most common physical signs were splitting of the second sound in pulmonary valve area (93.3％),followed by tricuspid murmur (77.8％),cyanosis (53.3％),hepatomegaly (42.2％),hydropericardium (28.9％),and oedema (11.1％),et al.Twenty-seven cases did cardiac catheterization,compared with idiopathic PAH and secondary PAH,pulmonary artery systolic pressure,mean pulmonary artery pressure,pulmonary capillary wedge pressure,pulmonary arteriolar resistance index had significant difference(P ＜ 0.05).Thirty-one cases' condition improved after treatment,11 cases without any improvement and 3 cases died during follow-up visit.Conclusions PAH is a rare disease with no specific symptom and can be easily misdiagnosed in children.Ultrasonic cardiogram and cardiac catheterization are helpful in diagnosis.Actively looking for the cause can improve the prognosis.

Effect of Rho kinase inhibitor Y27632 on the cytoskeleton of airway smooth muscle in young asthmatic rats with airway remodeling / 中国医师杂志

Objective To investigate the alteration of the cytoskeleton of airway smooth muscle cells in young asthmatic rats with airway remodeling and the effect of RhoA/ROCK signal pathway.Methods Airway smooth muscle cells (ASMCs) were primary cultured and purified from Sprague-Dawley(SD) rats that were induced by ovalbumin (OVA) inhalation for 8w,then incubated by Pho kinase inhibitor Y27632.Real time quantitative polymerase chain reaction (RT-PCR),Western blot,and immunohistochemistry were used to measure the alteration of F-actin,and α-tubulin in the cytoskeleton of airway smooth muscle.Results (1) The asthma group showed a high average gray value of F-actin in ASMC than control groups,especially 8 weeks;and were significantly down in the group after adding Y27632(P ＜0.01).(2) The intension and intensity of fluorescence signal of α-tubulin in asthma groups in 8 weeks were higher than control greup(P ＜0.01),and were significantly decreased in Y27632 group.(3) A higher expression of α-tubulin protein was shown in the asthma group in 8 weeks relative to control group(P ＜0.01),and was significantly down-regulated in Y27632 group(P ＜0.05).Conclusions Alteration of the cytoskeleton of airway smooth muscle exists in young asthmatic rats and the RhoA/ROCK signal pathway possibly plays a significant role.

Effect of pre-arrest and post-arrest mild hypothermia on myocardial function of ventricular fibrillation after restoration of spontaneous circulation in rabbits / 中华危重病急救医学

ObjectiveTo study the effect of pre-arrest and post-arrest mild hypothermia after restoration of spontaneous circulation (ROSC) on myocardial function, ultrastructure, apoptosis of myocardial cells in rabbits with ventricular fibrillation.Methods Sixty-two male New Zealand rabbits were randomly allocated into five groups: namely normothermic control group (NTC group,n = 10), hypothermia control group (HTC group,n = 10), normothermic resuscitation group (NTR group,n = 14), hypothermia pre-arrest group (HPRA group,n = 14), and hypothermia post-arrest group (HPOA group,n = 14). The normal temperature was controlled at (39.0±0.5)℃, and the hypothermia (33.5±0.5)℃. Ventricular fibrillation cardiac arrest (CA) was reproduced in rabbits by transcutaneous epicardium electrical stimulation. The parameters of hemodynamics were monitored dynamically for 4 hours in all the groups, including heart rate (HR), left ventricular end diastolic and systolic pressure (LVEDP/LVESP), maximal rate of increase/decrease in left ventricular pressure (±dp/dt max), and mean arterial pressure (MAP). The body temperature of rabbits in hypothermia groups was maintained by surface cooling for 4 hours followed by rewarming. The survived rabbits were sacrificed at 48 hours after resuscitation, and myocardial apical tissue was harvested for observation of ultrastructure with electronic microscope, and to observe apoptosis by terminal deoxynucleotidyl transferase-mediated dUTP nick end labeling (TUNEL) staining.Results① Resuscitation investigation: there was no significant difference in rate of ROSC, time of CPR and energy of defibrillation among HPRA, HPOA, and NTR groups [rate of ROSC: 85.71%, 71.43%, 71.43%; time of CPR (seconds): 45.3±30.2, 61.2±41.3, 82.3±63.8;energy of defibrillation (J): 14.3±8.9, 22.0±15.5, 25.0±15.8, allP> 0.05].② Hemodynamics: compared with normal temperature groups, animals in hypothermia groups exhibited lower levels of HR (allP< 0.05). Compared with NTR group, HPRA group exhibited higher levels of LVESP (mmHg, 1 mmHg = 0.133 kPa) at 0.5, 1, 2 and 3 hours post ROSC (0.5 hour: 103.8±14.3 vs. 91.6±13.3, 1 hour: 107.2±14.1 vs. 82.7±8.5, 2 hours: 109.0±16.9 vs. 88.8±12.9, 3 hours: 109.1±14.6 vs. 89.3±14.3, allP< 0.05). Compared with NTR group and HPOA group, HPRA group exhibited lower levels of LVEDP (mmHg) at 0.5 hour post ROSC (3.70±0.85 vs. 7.61±2.73, 7.02±3.12, both P< 0.05). Compared with NTR group, HPRA group exhibited lower levels of LVEDP at 1 hour post ROSC (4.34±1.44 vs. 6.99±1.96,P< 0.05). In HPRA group, the level of+dp/dt max (mmHg/s) was higher than that of NTR group and HPOA group at 1 hour and 2 hours post ROSC (1 hour: 2 759.5±321.6 vs. 2 123.0±304.5, 2 283.7±234.2, 2 hours:2 730.6±425.1 vs. 2 221.5±392.9, 2 252.6±476.0, allP< 0.05). There were no significant differences in -dp/dt max and MAP levels among three CPR groups.③ The survival rate at 48 hours post ROSC of NTR, HPRA and HPOA groups was 60%, 75%, and 100%, respectively. Compared with NTR group, higher survival rate was found in HPOA group at 48 hour post ROSC (P< 0.05).④ Compared with NTR group, less damage to myocardial ultrastructure was found in HPRA and HPOA groups. Apoptosis index (AI) was lower in HPRA and HPOA groups than that in NTR group [(28.05±9.82) %, (26.39±8.98) % vs. (42.02±13.36) %, bothP< 0.05].Conclusions Our study shows that mild hypothermia has no effect on ROSC rate. Pre-arrest hypothermia can ameliorate myocardial systolic function of rabbit in early stage after ROSC, and it has no negative influence on diastolic function. Post-arrest mild hypothermia produces no negative influence on myocardial function of rabbit, but it improves 48 hours survival rate in ROSC rabbits. Both pre-arrest and post-arrest mild hypothermia therapy can attenuate myocardial injury in CA model of rabbits by ameliorating mitochondrial injuries and suppressing apoptosis of myocardial cells.

Significance of serum anti-gp210 antibody detection in diagnosis of primary biliary cirrhosis / 临床肝胆病杂志

Primary biliary cirrhosis (PBC)is a chronic disease characterized by progressive destruction of intrahepatic small bile ducts, which may progress to liver cirrhosis.Anti-mitochondrial antibodies,especially anti-M2 antibody,have a high diagnostic value for PBC, but they are unrelated to the severity and prognosis of the disease and are negative in some patients.There have been reports from around the world that anti-nuclear antibodies,especially anti-gp210 antibody,are closely associated with PBC.It showed that anti-gp210 antibody has high specificity and sensitivity for the diagnosis of PBC,especially for the patients with negative anti-M2 antibody tests;in addition,it has a high predictive value for the prognosis and development model of the disease.Anti -gp210 antibody has a high diagnostic value for PBC,with great clinical significance,so its detection holds promise for clinical application.