Volume 101 ·  Issue 25 ·  2021

Adult onset Still′s disease (AOSD) is a rare polygenic autoinflammatory disease mainly manifesting as high-spiking fever, rash, arthritis/arthralgia, lymphadenopathy, and leukocytosis. More importantly, life threatening macrophage activation syndrome may occur in AOSD patients. Recently, with the development of research in pathogenesis and therapy strategies of biological agents and small molecule targeted drugs, we have new recognition of AOSD. In this commentary, we attempt to place this syndrome in perspective, including data in the past year on pathogenesis, clinical and laboratory features and therapy.

Distant ischemic adaptation is to make other organs or tissues other than the organ or tissue adapt to ischemia by repeated and transient ischemic stimulation of a certain organ or tissue, thus improving its tolerance to ischemic injury. The method has the advantages of convenience, non-invasiveness, economy, practicality, few adverse reactions and easy popularization. In recent years, the application of remote ischemic adaptation in patients with ischemic cerebrovascular disease has been increasing. This consensus is based on the characteristics of prevention and treatment of cerebrovascular diseases in China, referring to the latest literature at home and abroad, and focusing on the concept and classification, mechanism of action, clinical implementation methods, applicable populations, contraindications and pre-treatment evaluation, future research and application suggestions of remote ischemia adaptation. It aims to attract the attention of neurologists and provide guiding suggestions for the standardized prevention, treatment and management of patients with ischemic cerebrovascular diseases in China.

To investigate the impact of ultrasound-guided lumbar plexus, sciatic nerve and L₁ paravertebral combined nerve blocks on the outcomes of elderly acute cerebral infarction patients with femoral neck fracture who underwent hip replacement.

The clinical data of 114 elderly acute cerebral infarction patients with femoral neck fracture and underwent artificial hip replacement surgery from May 2013 to September 2018 in Renmin Hospital of Wuhan University were retrospectively analyzed. The patients were divided into two groups based on the different anesthetic methods they received: general anesthesia (G group, n=48), lumbar plexus, sciatic nerve and L₁ paravertebral combined nerve blocks (N group, n=66). The operation time, anesthesia time, blood loss, urine volume, norepinephrine dose, length of intensive care unit (ICU) stay, hospital mortality and postoperative 6-month mortality were observed and compared between the two groups. The mini-mental state examination (MMSE) scores and the national institute of health stroke scale (NIHSS) scores were evaluated, respectively. Meanwhile, plasma D-Dimer and S100β levels were measured 1 d before surgery, 3 d and 7 d after surgery.

There were no significant differences in the baseline characteristics, operation time, anesthesia time, blood loss and length of ICU stay between the two groups (all P>0.05). Compared with group G, the dosage of norepinephrine [(86±23) μg vs (184±28) μg], hospital mortality [7.6% (5/66) vs 25.0% (12/48)] and postoperative 6-month mortality [12.1% (8/66) vs 31.3% (15/48)] were significantly decreased in group N, while the urine volume [(265±58) ml vs (160±55) ml] was significantly increased (allP<0.01). The MMSE scores (9.9±3.0 vs 14.6±2.4) in group N were significantly higher than those in the group G 3 d after surgery, while the NIHSS scores (15.3±3.2 vs 9.9±3.5), plasma D-Dimer [(10.8±2.5) mg/L vs (7.3±2.2) mg/L] and S100β levels [(326±35) ng/L vs (276±29) ng/L] were significantly lower than those in group G (allP<0.01).

Combined nerve blocks can reduce the mortality of acute cerebral infarction patients undergoing hip replacement surgery, and improve the brain function and prognosis of the patients.

To investigate the screw placement parameters, feasibility and safety of posterior atlantooccipital joint-occipital condyle-clivus screw technique in Chinese people.

Upper cervical spine CT images of 46 patients, including 24 males and 22 females, were collected with random number table from June 2019 to May 2020 in Ningbo No.6 Hospital. The patients aged 20-55 years, with a mean age of (39±9) years. Total of 92 sides of upper cervical spine models were obtained by Mimics 19.0 digital three-dimensional reconstruction, and screw placement was conducted simulately. The midpoint of transition zone between the posterior arch of atlas and the inferior articular process of lateral mass was selected as the screw entry point. The diameter and length of screws was 3.5 mm and 50 mm, respectively. Detailed morphometric measurements of the 92 atlantooccipital joint-occipital condyle-clivus screws were conducted. The distance between the screw and its surrounding important structures, screw inside and upper tilting angles, the length of screw trajectory in atlas and the length of screw trajectory on occipital side (occipital condyle-clivus) were all measured. Paired t test was performed on the parameters of left and right screw placement to confirm whether there was difference between the two sides.

In the 46 cases of upper cervical spine digital three-dimensional models, 92 posterior atlantooccipital joint-occipital condyle-clivus screws were implanted. All the screws were completely fixed in the clivus, without breaking through the upper sphenoid sinus, entering into the canalis spinalis and foramen magnum, and damaging the surrounding structures such as hypoglossal canal. The screw trajectory parameters between the left and right sides were slightly different, but there was no statistical differences between the two sides (P>0.05). The vertical distance between the screw entry point and the upper edge of atlas was (12.6±1.0) mm, the vertical distance between the screw entry point and the lower edge of atlas was (6.5±0.6) mm, the distance between the screw and the medial border of atlas vertebral artery foramen was (6.7±0.6) mm, the distance between the screw entry point and the medial wall of atlas was (6.6±0.7) mm, the distance between the screw outer margin and the hypoglossal canal was (5.5±0.6) mm, screw inside tilting angle was 21.2°±2.5°, screw upper tilting angle was 52.0°±3.4°, the length of screw trajectory in atlas was (12.1±0.9) mm, the length of screw trajectory on occipital side (occipital condyle-clivus) was (37.9±0.9) mm.

The posterior atlantooccipital joint-occipital condyle-clivus screw technique can serve as a feasible and safe treatment for instability of the occipitocervical junction, which can be used as a new posterior occipitocervical fusion technique.

To compare the clinical effect of alternative-level and all-level Arch mini-plate fixation in patients undergoing unilateral open-door laminoplasty.

Clinical data of 134 patients administrated in the Peking University International Hospital for multistage cervical spondylotic myelopathy from March 2015 to March 2019 were reviewed retrospectively. There were 63 males and 71 females with an average age of (62±8) years and a mean disease course of (18±7) months (3-37 months). All the patients underwent posterior cervical unilateral open-door laminoplasty with Arch titanium plate fixation. All the patients were divided into two groups according to the different amount of titanium plates used during operation as follow: group A, Arch plates were fixed at the door sides of C₃, C₅ and C₇ (n=68) and group B, Arch plates were fixed at the door sides of C₃, C₄, C₅, C₆ and C₇ (n=66). Operation time, intraoperative blood loss, postoperative hospitalization days and the cost of consumables, the Japanese orthopaedic association (JOA) score, cervical dysfunction index (NDI), the rates of improved JOA score, cervical curvature index, C_2-7 Cobb angle, cervical range of motion, sagittal diameter of vertebral canal, opening angle of laminar, hinges bone healing and surgery related complications (axial symptoms, C₅ nerve root palsy, screw loosening, laminar re-closing, cervical kyphosis, etc.) were recorded and compared between the two groups.

There was no complications during the operation, and the mean follow-up was (20±8) months (14-48 months). There was no statistically significant difference between the two groups in terms of gender, age, course of disease and compression segments (all P>0.05). There was no statistically significant differences between the two groups in operation time, intraoperative blood loss and postoperative hospital stay too (allP>0.05). The cost of consumables in group A was (34 970±1 325) yuan, and it was (57 450±2 161) yuan in group B, the difference between the two groups was statistically significant (P<0.01). The JOA and NDI score were significantly improved 3 months and 1 year after operation in both groups (bothP<0.05). The sagittal diameter of each segment of the spinal canal at C₃, C₄, C₅, C₆ and C₇ were all significantly increased in both groups 3 months and 1 year after surgery (all P<0.05). The C_2-7 Cobb angle and cervical curvature index were all significantly reduced in both groups 3 months and 1 year after surgery (all P<0.05). The cervical range of motion in both groups was significantly reduced 3 months and 1 year after surgery compared with that before surgery (bothP<0.05). The range of motion of the cervical spine in group A was significantly higher than that in group B (P<0.05). The opening angles of C₄ and C₆ segment lamina in group A 3 months and 1 year after operation were significantly lower than those in group B (all P<0.05). At 3 months after the operation, the hinges healing rate of C₄ and C₆ in group B was significantly better than that of group A (both P<0.05). At 1 year after the operation, there was no difference in the hinges healing rate of C₄ and C₆ in the two groups (both P>0.05). There was no statistically significant difference in postoperative complications between the two groups (P>0.05).

The safety and early clinical efficacy of alternative-level and all-level Arch titanium plate fixation in posterior cervical unilateral open-door laminoplasty are comparable, alternative-level fixation can effectively reduce the cost of hospitalization. The open Angle loss and lower early hinges healing rate of the non-fixed segment in the alternative-level fixed group does not lead to laminar re-closing in early period of post operation.

To investigate the efficacy and tolerability of Polatuzumab vedotin+rituximab±bendamustine (Pola-(B)R) in relapse/refractory diffuse large B cell lymphoma (R/R DLBCL) patients.

The clinical data of 21 patients enrolled in Chinese Pola compassionate use program (CUP) in 4 centers from November 2019 to August 2020 were collected. There were 15 males and 6 females, and the median age was 56 years (ranged 25-76 years). Of the patients, 10 cases received Pola-BR regimen and the other 11 received Pola-R. Their clinical features, regimens, efficacy, and adverse events (AEs) were retrospectively analyzed.

Twenty-one patients with at least one efficacy evaluation were included. At data analysis cut-off point (12 Aug. 2020), the best overall response (BOR) rate was 81.0% (17/21) and the complete response (CR) rate was 19.0% (4/21). Kaplan-Meier survival estimation was performed, at a median follow-up of 54 days, three patients (14.3%) had disease progressed, and 18 patients (85.7%) were censored; the median progression-free survival (mPFS) was estimated to be 148 days. The incidence of adverse effects (AEs) of any grade was higher in Pola-BR group than Pola-R group (80.0% vs 63.6%). However, the incidence of grade 3-4 AEs were close in the two groups (30.0% vs 29.3%). The most common hematological toxicities were thrombocytopenia (28.6%, 6/21), neutropenia (28.6%, 6/21) and anemia (14.3%, 3/21), respectively. One patient with pneumonia and 1 patient with hemophagocytic syndrome recovered after symptomatic treatment. No peripheral neuropathy of grade≥2 was observed.

The preliminary data suggested that, for heavily treated Chinese R/R DLBCL, the Pola-(B)R regimen still achieves promising efficacy and tolerable safety.

The clinical data of 22 patients with giant renal hamartoma in Zhejiang Provincial People′s Hospital who underwent robot-assisted laparoscopic nephron-sparing surgery from October 2014 to January 2020 were retrospectively analyzed. All the patients successfully completed the operation. The operation time and renal artery occlusion time was (179±34) min and (19.8±2.5) min, respectively. The intraoperative blood loss was (117±62) ml, and the postoperative hospital stay was (9.0±1.5) d. All cases were confirmed as renal angiomyolipoma by postoperative pathology. No urine leakage, postoperative bleeding and other complications occurred. Postoperative telephone follow-up was performed for 6 to 52 months, and no tumor recurrence on the surgical side was reported.

This paper reports a case of Behcet's disease with multiple thrombosis of lower extremity artery, celiac artery and aorta as the first manifestation. During the diagnosis and treatment, the new thrombosis of superior mesenteric artery and Behcet's disease involving gastrointestinal tract caused small intestinal perforation. The condition improved after active surgical management and combined high-dose glucocorticoid and cyclophosphamide treatment. Finally, small intestinal pathology revealed ulcer and perforation of small intestinal mucosa, and proliferation of granulation tissue around ulcer, which confirmed the diagnosis of Behcet's disease.

The incidence of polymyositis is low, the onset is concealed, and it is easy to be misdiagnosed and missed. This paper reports a patient with fatigue and abnormal transaminase onset, suspected to be diagnosed as autoimmune liver disease. Because of the mismatch between the characteristics of his abnormal liver function and autoimmune liver disease, it was further found that the patient's muscle strength was decreased and pulmonary interstitial lesions were complicated. At the same time, combined with the positive antibody of anti-signal peptide recognition granule, it was diagnosed as polymyositis. The patient's myositis was effectively treated, but there was persistent eosinophil elevation during the recovery period. Detailed investigation did not find the cause. Until about 3 years after the course of the disease, whole body positron emission computed tomography was performed again to find left axillary breast cancer metastasis, and no in situ solid tumor was found, which is extremely rare in clinical practice. Finally, the patient underwent left mastectomy and ipsilateral axillary lymph node dissection in time under the condition of maintenance treatment of myositis, and his condition is relatively stable at present.

This paper reports a severe neurological case of spontaneous cerebral hemorrhage caused by thrombocytopenia, venous sinus thrombosis and disseminated intravascular coagulation secondary to medical system disease. Accurate and timely judgment of the critical degree and changing trend of different organ diseases, careful weighing of the advantages and disadvantages and conflicts of treatment, combining multidisciplinary consultation, early prevention and active treatment of related complications are the keys to obtaining a good prognosis for patients.

Chronic altitude sickness (CMS) refers to a clinical syndrome in which people living in high altitude areas are not adapted to the hypoxic environment. With more and more people participating in the construction of plateau areas, the clinical diagnosis and treatment of CMS and its molecular pathogenic mechanism have attracted more and more attention. Detection of protein expression profiles by proteomics can help explore changes in tissue-specific proteins related to health and disease, and understand the functional adaptation mechanisms of cells, tissues, organs and entire organisms in extreme environments. By summarizing the changes of proteomics under high altitude hypoxia, this paper provides a reference for the diagnosis and pathogenesis of CMS.

Most hospitalized patients with novel coronavirus pneumonia are in a state of hypercoagulability, and their abnormal coagulation indexes and coagulation dysfunction are often closely related to poor prognosis. Disseminated intravascular coagulation exists in most death cases, and the incidence of arteriovenous thrombosis is significantly higher than that of general hospitalized patients. The monitoring of coagulation indicators will help early intervention and reduce the mortality rate. This article describes the application of coagulation test in coagulopathy of novel coronavirus pneumonia.

Respiratory infection is the most common type of infectious disease, and the sudden outbreak of novel coronavirus pneumonia in 2020 once again reminds us of the importance of preventing and treating respiratory infectious diseases. Early detection of pathogens causing respiratory infection is of great value for the treatment and prognosis of the disease. As a new pathogen detection method, nanopore sequencing has the advantages of rapid detection and real-time analysis. In this paper, the application status of nanopore sequencing technology in the etiological diagnosis of respiratory infectious diseases is reviewed and its application prospect is further analyzed, in order to provide a perspective for the application of nanopore sequencing and other novel molecular diagnostic technologies in the diagnosis and treatment of infectious diseases.

Mild cognitive impairment is considered a transitional state between normal aging and dementia and is a critical stage in the preventive treatment of dementia. The default network of the brain refers to the region of the brain where spontaneous neuronal activity is relatively active in the resting state, and is considered to be the most important network for maintaining normal cognitive function. REST-STATE FMRI technology can accurately locate spontaneous neuronal activity in the brain. Because of its simplicity, non-invasiveness, high resolution and repeatable scanning, it has been widely used in the research of cognitive impairment diseases. This paper reviews the research progress of brain default network based on resting-state functional magnetic resonance imaging in cognitive impairment diseases.

Sepsis is a life-threatening organ dysfunction caused by a dysregulated host response to infection. However, as a syndrome caused by underlying infection, the global burden of disease research data is still relatively limited. This study is the first to use data from the Global Burden of Disease Study (GBD2017) to assess the incidence and mortality of sepsis globally, in different regions and countries. The study included 282 potential causes of death from sepsis in 109 million death records in GBD2017, using a mixed-effect linear regression model to model different factors such as age, sex, location, and year (1990 – 2017). The results showed that a total of about 48.9 million sepsis events were reported globally in 2017, of which 11 million related deaths occurred, accounting for 19.7% of all deaths globally. Age-standardized sepsis rates decreased by 37.0% and mortality by 52.8% in 2017 compared to 1990. Despite the decline in age-standardized morbidity and mortality, sepsis remains a major threat to global health, and the burden of disease is particularly acute in sub-Saharan Africa.

Mortality in patients with cardiogenic shock due to sepsis remains high, and this study evaluated the supportive role of venous-arterial extracorporeal membrane oxygenation (VA-ECMO) in patients with septic cardiogenic shock who are not effective with conventional therapy. The study included 82 adult septic cardiogenic shock patients treated with VA-ECMO and 130 control patients who did not receive ECMO in 5 ECMO centers worldwide. All patients had severe cardiac dysfunction [cardiac index (CI) ≤3 L/ (min·m²)] or left ventricular ejection fraction (LVEF) ≤35%] and hemodynamic impairment [positive inotropic drug score ≥75 μ g/ (kg min), lactic acid ≥4 mmol/L]. The primary endpoint of the study was 90-day survival. The results showed that at the baseline level of the disease, the treatment group was more severe than the control group: cardiac dysfunction [mean CI 1.5 L/ (min·m²) ratio 2.2 L/ (min·m²), LVEF: 17% vs. 27%]; Hemodynamic impairment [positive inotropic drug score 279 μ g/ (kg·min) vs 145 μ g/ (kg·min), lactate concentration 8.9 mmol/L vs 6.5 mmol/L]; Organ failure (Sequential Organ Failure Assessment Score 17 vs 13). However, the 90-day survival rate of patients treated with VA-ECMO was significantly higher than that of controls (60% vs. 25%). After propensity score weighting, ECMO treatment still improved survival (51% vs. 14%) and significantly improved patient lactate and catecholamine clearance. Of the 49 patients who survived VA-ECMO, 32 had participated in the Health Survey Short Form (SF-36) assessment at follow-up 1 year later and had a good health assessment. The results of this study suggest that VA-ECMO significantly improves survival in patients with severe septic cardiogenic shock compared to controls who did not receive ECMO.