Association between cerebral blood flow changes and blood-brain barrier compromise in spontaneous intracerebral haemorrhage.

AIM: To quantitatively evaluate blood-brain barrier (BBB) permeability in the perihaematomal region of spontaneous intracerebral haemorrhage (ICH) and investigate the association between the alterations in cerebral blood flow and BBB permeability around the haematoma. MATERIALS AND METHODS: Spontaneous ICH patients underwent unenhanced computed tomography (CT) and CT perfusion (CTP) simultaneously. Haematoma volume was measured on CT. The values of cerebral haemodynamic parameters including cerebral blood flow (CBF), cerebral blood volume (CBV), mean transit time (MTT), time to peak (TTP), and permeability-surface area product (PS) were measured in the perihaematomal region and the contralateral mirror region, and then relative values were calculated for statistical analysis. Linear regression was used to evaluate associations between BBB permeability and variables. RESULTS: A total of 87 ICH patients were included in this study. The focally elevated BBB permeability was observed in the perihaematomal region in ICH patients. Linear regression showed that reduced rCBF (ß = -0.379, p=0.001) and increased rCBV (ß = 0.412, p=0.000) correlated independently with increased relative PS (rPS) value in deep ICH, while only increased rCBV (ß = 0.423, p=0.071) correlated to increased rPS value in patients with lobar ICH. CONCLUSIONS: BBB permeability is focally elevated in the region around the haematoma. Cerebral haemodynamic alterations are associated with increased BBB permeability. Cerebral hypoperfusion may aggravate BBB compromise, and a compensatory increase in CBV may lead to reperfusion injury on BBB.

[Recommendations for diagnosis and treatment of primary biliary cholangitis in China (2021)].

Primary biliary cholangitis is a chronic autoimmune cholestatic disease with a progressive course. This disease is not rare in China, but standardized diagnosis and treatment for primary biliary cholangitis are insufficient. Based on the evidence and guidelines from China and other countries, Rheumatology Branch of Chinese Medical Association developed the recommendations of diagnosis and treatment for primary biliary cholangitis in China. The aim is to help clinicians recognize clinical characters, therapeutic selection and prognosis judgement of primary biliary cholangitis, which will contribute to make diagnosis in time, to select treatment properly and to manage follow-up scientifically.

[Expert consensus for diagnosis and treatment of thrombocytopenia in China].

Thrombocytopenia is the main clinical manifestation or common complication of multiple diseases, but there is still a lack of systematic understanding of pathogenesis, underlying diseases and treatment strategies of thrombocytopenia. Based on evidence-based medicine, this consensus summarizes seven aspects related to thrombocytopenia, including definition, epidemiology, pathogenesis, clinical manifestations, laboratory examination, diagnosis and treatment. This consensus provides an important reference for the diagnosis and treatment of thrombocytopenia.

[Efficacy and safety of anti-tumor necrosis factor α monoclonal antibodies in 16 patients with severe/refractory vasculo Behcet's disease].

Objective: To explore the efficacy and safety of anti-tumor necrosis factor alpha (TNFα) monoclonal antibodies (mAbs) for severe/refractory vasculo-Behcet's disease (BD). Method: The clinical data of severe/refractory vasculo-BD patients treated with anti-TNFα mAbs were retrospectively analyzed. Response of anti TNFα mAbs was analyzed. The dosage changes of glucocorticoid, the level of erythrocyte sedimentation rate (ESR) and hypersensitive C-reactive protein (hsCRP) before and after treatment were recorded, as well as side effects. Result: Sixteen patients were enrolled. Arterial lesions were reported in 12 patients, including 9 with arterial aneurysm, 6 with arterial dilation, 2 with stenosis and 2 with occlusion. Seven patients presented venous thrombosis, including lower extremity veins (n=6), cerebral venous sinus (n=2) and inferior vena cava system (n=2). Two cases had both arterial and venous involvement. Before the application of TNFα mAbs, all 16 patients failed to response to prednisone or its equivalent dose of 40 (7.5-90) mg/d in combination with cyclophosphamide, methotrexate, thalidomide or azathioprine for median 4 (0-156) months. After a mean duration of treatment for (17.1±6.5) months, 15 patients achieved complete remission and 1 patient achieved partial remission. Three patients received surgery without any postoperative complications. After using anti TNFα mAbs, the dosage of prednisone [5(0-12.5)mg/d vs. 40(7.5-90)mg/d, P<0.01], ESR [(7.3±4.6) mm/1h vs. (33.5±26.7) mm/1h, P<0.01] and hsCRP [1.9(0.2-11.4) mg/L vs. 24.3(0.4-113.9) mg/L, P<0.01] were significantly decreased. Side effects were observed in 2 patients. One developed pulmonary infection 12 months after adalimumab with conventional treatment. Another patient had allergy to infliximab then switched to adalimumab. Conclusion: In combination with corticosteroids and immunosuppressants, anti-TNF α mAbs are effective and well-tolerated in severe/refractory vasculo-BD, with a favorable steroid -sparing effect and rare postoperative complications.

[Cardiologists' perceptions on Behcet's disease and its cardiovascular lesions: a single center analysis].

To analyze the perceptions of cardiovascular specialists about Behcet's disease and its cardiovascular lesions in Beijing Anzhen Hospital, Capital Medical University. A survey using questionnaires was conducted among cardiovascular specialists in the hospital, the differences among groups were analyzed with χ(2) analyses. Less than half of the cardiovascular specialists were familiar with the diagnostic criteria of Behcet's disease (32.6%), and its skin lesions, as acne-like rash (41.3%), erythema nodosum (42.0%), acupuncture response (47.8%). The knowledge of its cardiovascular lesions was too superficial, especially in the awareness of heart conduction block. Most of the specialists preferred to use low dose of glucocorticoids before operation in their practice. To improve the knowledge and awareness of Behcet's disease and its cardiovascular lesions is of great significance for the early diagnosis, improvement of prognosis, and reduction of postoperative complications.

[Clinical characteristics of 57 patients with polyarteritis nodosa and renal involvement].

Objective: To investigate the clinical characteristics of polyarteritis nodosa (PAN) patients with renal involvement. Methods: PAN patients admitted to the department of rheumatology, department of pediatrics, department of nephrology, general internal medicine department and department of vascular surgery at Peking Union Medical College Hospital from June 2012 to August 2018 were enrolled in this study and were divided into two groups according to renal involvement or not. The clinical characteristics were analyzed. Results: A total of 94 PAN patients were finally enrolled and 57 (60.64%) presented kidney manifestation. The mean age of onset was (37.76±17.40) years old and the interval from onset to diagnosis was 10 (0 to 240) months. Forty patients were misdiagnosed once or more times. In patients with renal involvement, 9 cases suffered from renal ischemia or infarction, 31 with microscopic haematuria, 26 with proteinuria, renal artery or its branch involved in 17 cases, renal vein thrombosis in 1 case, 4 cases with pyeloureterectasis, one case with renal fascia thickening, 33 cases with impaired renal function (serum creatinine>84 µmol/L) including creatinine>140 µmol/L in 10 patients. Renal artery branch stenosis was the most common presentation [9 cases (52.94%)] of renal vascular involvement, other abnormalities including nodular dilatation [4 cases (23.53%)], occlusion [3 cases (17.65%)]. There were significant differences (P<0.05) in the PAN patients with and without renal involvement in the following: age of onset [(33.72±16.13) years vs. (43.97±17.66) years, t(2)=2.901, P=0.005], weight loss(≥4kg since PAN onset) [25(43.86%) vs. 7(18.92%), χ(2)=6.216, P=0.013], elevation of diastolic blood pressure [22(38.60%) vs. 7(18.92%), χ(2)=4.072, P=0.044], acromegaly gangrene [18(31.58%) vs. 21(56.76%), χ(2)=5.859, P=0.015], and gastrointestinal artery involvement [20(35.09%) vs. 6(1.22%), χ(2)=3.993, P=0.046]. Laboratory parameters and the application of glucocorticoid and cyclophosphamide therapies were similar in two groups (all P>0.05). Conclusion: Young PAN patients are more likely to be associated with renal involvement, especially gastrointestinal arteries.

[The evaluation of training program "resident team leader in the Department of General Internal Medicine" at Peking Union Medical College Hospital].

Objective: To evaluate senior resident training program "resident team leader in the Department of General Internal Medicine" at Peking Union Medical College Hospital. Methods: We surveyed the residents or the fellows who had been selected as resident team leaders and received the training from October 2014 to September 2018 on their comments and suggestions. Results: Twenty-two rotated senior residents who were selected as team leaders in the Department of General Internal Medicine completed the survey. Almost all (21/22, 95.5%) of the respondents reported that they learnt more in general as team leaders by Visual Analog Scale (VAS). The mean VAS scores of clinical skills were 7.23±1.27, 7.86±1.32 in teaching abilities, 8.14±0.89 in leadership evaluation. Scales as chief resident assistants were 8.44±1.26. Sixteen respondents (72.7%) considered that pre-job training by attending doctors was necessary. Another 8 (36.4%) respondents addressed their demands on training of teaching skills. Conclusions: The senior resident training program "resident team leader in the Department of General Internal Medicine" improves the competency of rotated senior residents. It is a valuable pilot study on senior resident training and worthy of further application in other departments and hospitals.

Clinical outcomes and predictive relapse factors of IgG4-related disease following treatment: a long-term cohort study.

OBJECTIVES: The aim of this study was to investigate the predictive factors for relapse of IgG4-related disease (IgG4-RD) and observe the long-term clinical outcomes in patients with IgG4-RD. METHODS: We included in the present analysis 122 patients who were newly diagnosed with IgG4-RD, treated with glucocorticoid (GC) monotherapy or GC and immunosuppressant combination therapy, and followed for at least 3 years. Clinical relapse, response and side effects were recorded. RESULTS: The cumulative relapse rates of patients in this study were 10.66%, 22.95% and 27.87% at 12, 24 and 36 months, respectively. Complete drug withdrawal was an independent risk factor for disease relapse. Higher serum IgG4 concentrations, involvement of more organs, higher IgG4 RI scores and elevation of eosinophils at baseline were closely associated with disease relapse. Re-elevation of serum IgG4 concentrations and low GC maintenance dosage during the follow-up period were significantly associated with clinical relapse. The GC dosage should be more than 6.25 mg day-1 as monotherapy during the maintenance stage; moreover, combining with immunosuppressants can reduce the GC dosage. Adding GC or immunosuppressants for patients with re-elevation of serum IgG4 levels could prevent later disease relapse. No serious complications were noted during long-term follow-up. CONCLUSIONS: The combination of GC with immunosuppressants was more effective than GC monotherapy during the steroid tapering and maintenance stages. Higher serum IgG4 levels, involvement of more organs, higher IgG4 RI scores, history of allergy, eosinophil elevation at baseline, re-elevation of serum IgG4 levels and lower GC maintenance dosage at follow-up might be predictive of relapse.

[The 466th case:myasthenia,fever,blurred vision and pancytopenia].

A 45-year-old woman was admitted to the Department of Rheumatology and Immunology, Peking Union Medical College Hospital, due to weakness of the upper limbs, fever, and blurred vision. She was clinically diagnosed as systemic lupus erythematosus overlapped primary biliary cirrhosis, with renal, retinal, hematological and musculoskeletal involvement, combined with severe pulmonary infection and respiratory failure. Treated with glucocorticoids, ursodeoxycholic acid, antibiotics and respiratory support, the patient got better. A couple of days later, her fever recurred and platelets count dropped to 30×10(9)/L, hemoglobin to 78 g/L, fibrinogen to<1.5 g/L, ferritin to 1 640 ng/ml, natural killer (NK) cell count to 8/µl, the activity of NK cells 2% (reference value 9.5%-23.5%), considering the occurrence of hemophagocytic lymphohistiocytosis (HLH). Cytomegalovirus pp65 antigenemia test: 13 positive cells/2×10(5) WBC. Considered the possibility of HLH caused by cytomegalovirus infection and treated by 250 mg ganciclovir intravenous drip twice a day for a full course. The temperature of the patient was gradually reduced to 36.5 ℃, the count of platelets were increased to 229 ×10(9)/L, the hemoglobin was increased to 94 g/L, and the fibrinogen was increased to 3.26 g/L. When there were unexplained critical signs of the primary disease during systemic lupus erythematosus treatment, severe complications such as infection, HLH, thrombotic thrombocytopenic purpura should be taken into account.

[Guidelines for diagnosis and treatment of dengue in China].

Dengue is the most prevalent and rapidly spreading mosquito-borne viral disease. As a dengue non-endemic country, China has experienced several dengue outbreaks in recent years. However, dengue patients in China displayed distinct clinical characteristics compared to patients in endemic countries. To standardize the diagnosis and treatment of dengue fever, the experts of the Society of Infectious Diseases, Society of Tropical Medicine and Parasitology of Chinese Medical Association, and the Society of Emergency Medicine, China Association of Chinese Medicine have reached this guideline based on guidelines for diagnosis, treatment, prevention and control of dengue (World Health Organization, 2009); guidelines for diagnosis and treatment of dengue (National Health and Family Planning Commission of the People's Republic of China, 2014, Edition 2), health industry standard of the People's Republic of China "diagnosis for dengue fever (WS216-2018)" and systemic reports on dengue. The guideline includes 8 aspects: introduction, terminology, epidemiology and prevention, etiology and pathogenesis, clinical features, diagnosis, treatment and problems to be solved.

The postoperative effect of sevoflurane inhalational anesthesia on cognitive function and inflammatory response of pediatric patients.

OBJECTIVE: To investigate the effect of sevoflurane on cognitive function and inflammatory response of children after general anesthesia at different times. PATIENTS AND METHODS: Ninety-three pediatric patients who underwent general anesthesia surgery were enrolled and divided into groups based on time under general anesthesia: group A (<1 h, n=27), group B (1-3 h, n=36), and group C (≥ 3 h, n=30). Changes in cognitive function and serum inflammatory index were compared. RESULTS: The occurrence of postoperative cognitive dysfunction (POCD) in group A and B was lower than in group C and the difference was statistically significant (p<0.05). The levels of caspase-3, TNF-α, and IL-6 in the POCD group at the different time points were significantly higher than in the non-POCD group and the differences were statistically significant (p<0.05). Caspase-3, TNF-α, and IL-6 levels in the POCD group at the different time points significantly changed and were highest during the recovery period, while there were no significant changes in the non-POCD group at the different time points. CONCLUSIONS: The prolonged sevoflurane inhalational anesthesia time (≥ 3 h) enhanced the occurrence of POCD and was related to the expression levels of serum caspase-3, TNF-α, and IL-6.

[An analysis of residents' self-evaluation and faculty-evaluation in internal medicine standardized residency training program using Milestones evaluation system].

Objective: To assess the value of internal medicine residency training program at Peking Union Medical College Hospital (PUMCH), and the feasibility of applying revised Milestones evaluation system. Methods: Postgraduate-year-one to four (PGY-1 to PGY-4) residents in PUMCH finished the revised Milestones evaluation scales in September 2017. Residents' self-evaluation and faculty-evaluation scores were calculated. Statistical analysis was conducted on the data. Results: A total of 207 residents were enrolled in this cross-sectional study. Both self and faculty scores showed an increasing trend in senior residents. PGY-1 residents were assessed during their first month of residency with scores of 4 points or higher, suggesting that residents have a high starting level. More strikingly, the mean score in PGY-4 was 7 points or higher, proving the career development of residency training program. There was no statistically significant difference between total self- and faculty-evaluation scores. Evaluation scores of learning ability and communication ability were lower in faculty group (t=-2.627, -4.279, all P<0.05). The scores in graduate students were lower than those in standardized training residents. Conclusions: The goal of national standardized residency training is to improve the quality of healthcare and residents' career development. The evaluation results would guide curriculum design and emphasize the importance and necessity of multi-level teaching. Self-evaluation contributes to the understanding of training objectives and personal cognition.

[Comparison of Dengue viral nonstructural protein 1 antigen testing kits].

Objective: To investigate the sensitivity and specificity of commercial nonstructural protein 1 (NS1) testing kits for Dengue fever diagnose, and provide the evidence for diagnostic criteria revision. Methods: 300 PCR or virus isolation positive blood samples for dengue virus were collected from sentinel hospitals for dengue surveillance in Guangzhou, Dongguang and Zhongshang from May 2015 to Nov. 2016. At the same time, 308 PCR negative samples for Dengue virus were collected as control group. The information of the sample was collected using questionnaires. These samples were tested using imported and domestic ELISA and the colloidal gold-labeled kits that were widely used for detecting dengue NS1. Sensitivity, specificity and coincidence were calculated and analyzed, and Z hongshan's result was regarded as the reslut of the third part. Results: The positive group includes 133 males and 167 females, average ages are 47.2±13.3, 179, 110 and 11 of them is Dengue Ⅰ, Ⅱ and Ⅲ respectively. The negative group includes 154 males and 154 females, average ages are (40.1±11.6) years old. The sensitivity of domestic ELISA Kits (94.5%) is less than imported (99.5%), and the result has statistical significance (χ(2)=8.59, P=0.030), the specificity is 99.7% and 97.7% respectively; The sensitivity of imported and domestic the colloidal gold-labeled Kits is 97.5% and 96.5% respectively, both of specificities are 100%. The sensitivity and specificity of Dengue Ⅰ for NS1 test are more than 97.0%. The sensitivity of domestic ELISA and gold-labeled Kits is 90.0% and 95.0%, and the specificity is 96.8% and 100% respectively for Dengue Ⅱ test. The sensitivity of imported ELISA and gold-labeled Kits is 100% and 98.0%, and the specificity is 99.4% and 100% respectively for Dengue Ⅱ test. The result of the third party show the sensitivity and specificity of domestic ELISA and gold-labeled Kits are 90.0% and 98.0%, the differences has statistical significance (χ(2)=5.67, P=0.020). Conclusion: NS1 testing can be used as early dengue fever diagnose for higher sensitivity and specificity.

Influence of laryngeal mask airway (LMA) insertion anesthesia on cognitive function after microsurgery in pediatric neurosurgery.

OBJECTIVE: To compare the influence of laryngeal mask airway (LMA) insertion anesthesia and endotracheal intubation on cognitive function during anesthesia for neurosurgery microscopy. PATIENTS AND METHODS: A total of 76 pediatric patients who underwent neurosurgery microscopy were selected. They were randomly divided in the LMA insertion group with 35 cases and the endotracheal intubation group with 41 cases. Before the operation, the two groups were injected with 0.02 mg/kg atropine and 2 mg/kg phenobarbital. A combination solution of 2 mg/kg ketamine and 0.1 mg/kg midazolam was then given to induce anesthesia. The inhalation of 4-6% sevoflurane was used to maintain anesthesia. The hemodynamics, complications, cognitive functions, and expression levels of serum NSE and S-100ß protein after anesthesia and extubation were compared. RESULTS: After comparing the average heart rate, average arterial pressure and average oxygen saturation of the LMA insertion group at different times, the difference was not statistically significant (p>0.05). At T2 and T4, compared with the endotracheal intubation group, the average heart rate and arterial pressure of the LMA insertion group were significantly reduced and the average oxygen saturation was significantly increased. The difference was statistically significant (p<0.05). The prevalence of complications from postoperative cognitive dysfunction (POCD) of the LMA insertion group was significantly lower than that of the endotracheal incubation group. The difference was statistically significant (p<0.05). CONCLUSIONS: Compared with the endotracheal intubation group, in the LMA insertion group, the hemodynamics is more stable, the prevalence of postoperative complications and the POCD are lower during pediatric neurosurgery microscopy. The occurrence of POCD is related to the reduction of protein expression levels of NSE and S-100ß during serum anesthesia and the recovery period.

[Feasibility and value of index of microcirculatory resistance in patients with acute myocardial infarction after primary percutaneous coronary intervention].

Objective: To evaluate the feasibility of detecting index of microcirculatory resistance (IMR) and the relationship between IMR and left ventricular (LV) systolic function after acute myocardial infarction (AMI) undergoing primary percutaneous coronary intervention (PCI). Methods: The patients with first AMI received primary PCI in Peking University Third Hospital were enrolled from January 2014 to March 2016. IMR were measured immediately after PCI by using pressure/temperature wire. The relationship between IMR and left ventricular ejection fraction (LVEF) assessed by echocardiography at first day and 6 months after admission was evaluated. Results: Twenty-eight patients with anterior wall AMI were enrolled, with an average age (56±13) years. The success rate of IMR detection was 100%. The mean IMR was (33±18 )mmHg·s. There was no complication related to intravenous adenosine triphosphate (ATP) (140 µg· kg(-1)· min(-1)). The IMR was negatively correlated with TIMI blood flow grade after primary PCI (r=-0.386, P=0.043), and positively correlated with female gender, CK peak value and TnT peak value (r=0.430, P=0.022; r=0.431, P=0.025; r=0.434, P=0.024). After 6 months of follow-up, no adverse cardiovascular events (including cardiac death, nonfatal myocardial infarction, malignant arrhythmia, unplanned revascularization, hospitalization for unstable angina pectoris and severe heart failure requiring hospitalization) occurred. LVEF increased significantly compared with the first day after PCI (0.54±0.08 vs 0.47±0.06, P=0.001), and IMR was negatively correlated with LVEF after 6 months (r=-0.477, P=0.014). Multivariable linear regression analysis showed that CK peak and IMR were predictors of LVEF after six months ( ß=-0.595, t=-3.814, P=0.01; ß=-0.352, t=-2.26, P=0.036). Conclusions: Immediate detection of IMR in patients with anterior wall AMI after PCI is safe and feasible. The immediate IMR after PCI reflects the extent of myocardial necrosis and myocardial perfusion, and is a predictor of LVEF at 6 months after PCI.

Retinal vasculopathy in patients with systemic lupus erythematosus.

A retrospective case control study was conducted in the Peking Union Medical College Hospital. Medical records were reviewed for demographic data, clinical features, laboratory results, systemic lupus erythematosus (SLE) disease activity evaluations, and ophthalmic examinations to investigate the clinical characteristics and significance of retinal vasculopathy (RV) in Chinese patients with systemic lupus erythematosus. The prevalence of RV was approximately 0.66% (35/5298) in SLE patients. A total of 60 eyes were involved. The ocular presentations included decrease of visual acuity (48/60, 80%), visual field loss (7/60, 11.7%), and diplopia (3/60, 5%). Ophthalmic fundoscopic examination revealed cotton-wool spots (30/60, 50%), retinal vascular attenuation (31/60, 51.6%), and hemorrhages (41/60, 68.3%). Retinal angiogram showed that 72.7% (16/22) eyes had vaso-occlusion. The ophthalmic episodes could occur at any stage of SLE duration, with a median of 12 months (0-168 months) following SLE onset. Twenty-one (35%) eyes did not recover, or even worsened, during hospital stay. RV was found to be significantly associated with neuropsychiatric lesions (51.4% vs. 21.3%, p = .005) and hematological disturbance (62.9% vs. 34.3%, p = .005). SLE patients with RV had significantly higher SLE disease activity index scores than controls (19.9 ± 0.9 vs. 10.2 ± 0.7, p < .001). An inverse association of anti-SSA antibody with RV was detected (34.3% vs. 67.1%, p = .001). Nervous system disturbance (odds ratio (OR) = 4.340, 95% confidence interval (CI) 1.438, 13.094, p = .009) and leukocytopenia (OR = 6.385, 95% CI 1.916, 21.278, p = .003) were independent risk factors, while anti-SSA antibody positivity (OR = 0.249, 95% CI 0.087, 0.710, p = .009) was a protective factor for RV in SLE patients. In certain cases, RV is a threatening condition for SLE patients presenting with clinical ocular manifestations. Ophthalmo-fundoscopic detection is recommended as soon as SLE is diagnosed.