Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired clonal hematopoietic stem cell disease caused by abnormal expression of glycosylphosphatidylinositol (GPI) on the cell membrane due to mutations in the phosphatidylinositol glycan class A(PIGA) gene. It is commonly characterized by intravascular hemolysis, repeated thrombosis, and bone marrow failure, as well as multiple systemic involvement symptoms such as renal dysfunction, pulmonary hypertension, swallowing difficulties, chest pain, abdominal pain, and erectile dysfunction. Due to the rarity of PNH and its strong heterogeneity in clinical manifestations, multidisciplinary collaboration is often required for diagnosis and treatment. Peking Union Medical College Hospital, relying on the rare disease diagnosis and treatment platform, has invited multidisciplinary clinical experts to form a unified opinion on the diagnosis and treatment of PNH, and formulated the Expert Consensus of Multidisciplinary Diagnosis and Treatment for Paroxysmal Nocturnal Hemoglobinuria (2024), with the hope of promoting the standardization of PNH diagnosis and treatment.

Objective:To investigate the Chinese anesthesiologist′s proficiency, training experience and needs of flexible bronchoscope-guided awake flexible bronchoscopy intubation (AFBI) using a questionnaire method.Methods:The cluster sampling was used, and self-designed questionnaires that addressed 54 questions in 5 categories were distributed through WeChat and online platforms. The survey took one month, and the answers were automatically recorded by the WeChat server.Results:A total of 1 250 anesthesiologists participated in the survey in 30 provinces of China, 9 of them were not anesthesiologists, and 1 241 (99.28%) questionnaires were validated. In the valid questionnaires, 52.70% (654) of the anesthesiologists were from tertiary hospitals, and 74.78% (928) of the anesthesiologists were attending physicians or above, only 7.57% (94) of the anesthesiologists had sufficient confidence in AFBI. Twenty-five point two two percent (313) of the anesthesiologists preferred fiberoptic intubation as the first tool when dealing with the anticipated difficult airway. Forty-eight point one one percent (597) of the anesthesiologists had implemented AFBI. Among them, 80.74% (482) had experienced unsuccessful AFBI practices. Eight hundred and ninety-four anesthesiologists had received AFBI training, and the most common AFBI training strategy was theoretical lectures. In addition, the degree of satisfaction regarding the theoretical lectures quality, technical training, clinical practice relativity and non-technical skills training was 21.47% (192), 14.32% (128), 12.3% (110) and 17.90% (160), respectively. The degree of satisfaction with all the 4 training elements mentioned above was 7.27% (65).Conclusions:The awareness and practice of Chinese anesthesiologists in terms of clinical application of AFBI to treat difficult airways need to be strengthened at present, and the lack of high-quality AFBI training may be the key.

Objective:To observe any effect of repeated magnetic stimulation of the spine on lower urinary tract function and the life quality of patients with neurogenic bladder after suprasacral spinal cord injury (SCI).Methods:Fifteen suprasacral SCI patients whose lower urinary tract function was not improving were enrolled. In the first 2 weeks, all received water drinking management and intermittent catheterization, while in the following 4 weeks they were additionally provided with daily transspinal magnetic stimulation at the level of the spinous process of the first lumbar vertebra five times a week. The stimulation frequency was 1Hz. The patients kept voiding diaries. Their urodynamics were quantified using neurogenic bladder symptom scoring (NBSS) and a life quality scale.Results:The frequency of catheterization and the average voided volume, as well as the maximum detrusor pressure during the storage phase, maximum bladder capacity, maximum urethral pressure during the voiding phase and voiding efficiency at the end of the sixth week were significantly different from those at the end of the second week and before the intervention. The average NBSS and life quality scores then showed significant differences from the earlier time points.Conclusion:Repeteitive transspinal magnetic stimulation of the spine can improve lower urinary tract functioning and the life quality of persons with neurogenic bladder after a suprasacral SCI.

Objective: To investigate the origin of infection and risk factors of a case with SARS-CoV-2 infection associated with overseas countries in the Ningbo-Zhoushan Port, Zhejiang Province, so as to provide the evidence for improving the COVID-19 control measures at ports. Methods: Ningbo Center for Disease Control and Prevention ( CDC ) and Beilun CDC conducted case finding and epidemiological surveys immediately after being informed. The general information, history of vaccination and the travel during the latest 14 days were collected from the positive case, and all close contacts were tracked. Saliva samples were collected for SARS-CoV-2 nucleic acid testing and whole-genome sequencing, and the sequencing results were aligned with the GISAID's EpiCoV database. The origin of infection and transmission route of the positive case was investigated. Results: A case was identified positive for SARS-CoV-2 nucleic acid during company M's routine screening in the Ningbo-Zhoushan Port on August 10, 2021, and was confirmed positive for SARS-CoV-2 nucleic acid by Beilun CDC and Ningbo CDC on August 11. Whole-genome sequencing showed SARS-CoV-2 B.1.617.2 ( Delta ) variant, which shared the highest homology with the virus sequence uploaded by Russia on June, 2021 ( Russia/MOW-RII-MH27356S/2021 ). The case was a bundling worker for overseas container ships, and reported communicated with foreign boatmen and contacted materials without protected interventions on the SINOKOR AKITA Container Ship between August 4 and 5, 2021. This ship anchored at Vladivostok, Russia from July 27 to 29, anchored at Ningbo Harbor on August 4, and departed on August 5. Then, 11 boatmen from this ship were tested positive for SARS-CoV-2 nucleic acid on August 8. One asymptomatic case was reported in this epidemic; 254 close contacts and 617 secondary close contacts were identified, and all were tested negative for SARS-CoV-2 nucleic acid. No new cases with SARS-CoV-2 infections were detected until August 25, 2021, and the emergency response was therefore terminated. Conclusions The infection was a sporadic COVID-19 epidemic associated with overseas countries, which was caused by Delta variant infection through contacts with foreign boatmen or materials by a bundling worker in Ningbo-Zhoushan Port; fortunately, no epidemic spread occurred. Intensified closed-loop management and increased frequency of SARS-CoV-2 nucleic acid test among high-risk populations, and improving the precision and rapid emergency treatment of COVID-19 epidemics are required for the containment of COVID-19 at ports.

Sepsis is a serious life-threatening organ dysfunction disease caused by the body′s response to infection, which is the main cause of death in patients admitted to ICU.The occurrence, development and prognosis of sepsis are closely related to metabolism and regulation of inflammatory response.Adipose tissue not only participates in energy storage and metabolism, but also, as an important endocrine organ, secretes a variety of adipokines with pro-inflammatory or anti-inflammatory activities, and thus participates in the occurrence and development of sepsis.There are many kinds of adipokines, and different adipokines play different roles in sepsis and sepsis-related organ damage.Some adipokines such as adiponectin, adipokine complement Clq/tumor necrosis factor-associated protein 3, vaspin, irisin and Apelin are closely related with the pathogenesis and prognosis of organ injury in sepsis.

【Objective】 To study the annual financial expenditure in blood stations with different scales, and to establish the regression equation between blood collection units and total expenditure. 【Methods】 The annual total expenditure, the per capita cost of serving population, as well as the collection units of whole blood and apheresis platelet of 24 blood stations were collected. The financial expenditure required for collecting 10 000U blood was calculated.The statistical analysis was carried out with SPSS statistical software. 【Results】 From 2017 to 2020, the total annual financial expenditure of 24 blood stations showed an upward trend. The total expenditure among blood stations was different. The per capita cost of servicing population in the areas where the 24 blood stations were located had been increasing year by year. The 24 blood stations were divided into two grades according to the blood collection volume as 50 000 U, and the relationship equation between the blood collection volume and the annual total expenditure had been established. After testing, each equation was effective(P<0.05); There was no difference in the financial expenditure required for collecting 10 000U blood among blood stations with different scales. 【Conclusion】 From 2017 to 2020, the blood stations with an annual collection volume more than 50 000 U demonstrated a higher financial expenditure and the per capita cost of serving population than those <50 000 U. The blood collection volume of blood stations is significantly correlated with the annual total expenditure and the per capita cost of serving population.

Objective: To evaluate the clinical application effects of an iliac-crest-preserving iliac wing bone graft harvesting technique and the bone regenerative ability of the donor site after harvesting. Methods: From January 2016 to June 2017, a series of 39 patients including 28 men and 11 women aged between 16 and 59 y (35±13 y in average) were prospectively collected to treat bone defects using the iliac-crest-preserving iliac wing bone graft harvesting technique. The patient cohort included 38 ipsilateral and 1 bilateral bone harvests taken from 15 femoral fracture non-unions, 8 tibial fracture non-unions, 11 femoral head necroses (hip preserving surgery), 1 tuberculosis deriving hip arthritis with bone defect (total hip arthroplasty), 2 hip prosthesis loosenings (revision), and 2 proximal femoral benign tumors. All patients were treated through comprehensive surgeries containing autologous iliac bone grafting. The surgical time, blood loss, bone graft volume, 24 h post-operative visual analogue scale (VSA) at the iliac surgical site, complications, and bone regeneration of the donor site were documented and evaluated. Results: In the 39 patients (40 sides), the average surgical time was 25±4 min, average blood loss was 79±23 ml, average bone graft volume was 27±6 cm³. The average 24 h post-operative VAS at the iliac surgical site was 1.8±0.7 points. The VSA at the 6 week later and thereafter were 0 in all patients. The iliac incisions in 38 patients (39 sides) were healed uneventfully. However, seroma at the iliac surgical site emerged in 1 patient at the 6th post-operative day in the manner of serous exudation and was treated successfully with non-operative measures. None infections and lateral femoral cutaneous nerve injuries took place. Iatrogenic non-displaced iliac crest fractures happened in the very first 2 patients when taking the crest as a pivot to pry up the graft, which united without special cure at the 3 month post-operative. This complication was completely avoided when shifting the pivot to the anterior pillar containing the anterior iliac spines. None pelvic fractures and heterotopic ossifications took place. Post-operative radiographical examinations revealed that the bone defects at the donor site shrank through bone regeneration, that mamillary or canine-tooth-shaped bone formation occurred in some of the cases, and that none complete bone regeneration took place to eliminate the bone defect at the donor. There were 12 patients in whom pre- and post-operative computed tomographic scans necessitated by the disease were prescribed, which facilitated the measuring of the bone defect at the donor site. The measurement of 13 sides revealed that the bone defects were decreased more or less: the average immediate post-operative bone defect was 25.7±6.5 cm², the average 12 month post-operative bone defect was 12.7±5.3 cm². Conclusion The iliac wing bone graft harvesting technique suggested here is safe and less invasive, in the premise of preserving the iliac crest and retaining the figure of the surgical site, it can harvest a large amount of iliac wing bone graft and reserve the bone regenerative ability of the donor site

Objective To introduce an efficient way to orientate S1 iliosacral screw guide-pin verified by CT.Methods The pelvic axial CT data in DICOM format of 180 adults between July 2017 and June 2018 were retrieved from the database of Imaging Center,Luoyang Orthopaedic Hospital of Henan Province.The data were input into the software Mimics 20.0 to display the axial,coronal and sagittal sectional views of the pelvis.On the axial CT sectional view displaying the largest osseous pathway in the S1 segment,a virtual iliosacral screw and its virtual guide-pin were accurately placed into the sacral body in an oblique fashion.In the design of ideal insertion,the virtual screw and guide-pin were truly in the pelvic transverse plane when they were located exactly in the pelvic axial CT sectional view,and they intersected the outer iliac table at the start-point which restricted the guide-pin's location,and angulated with the pelvic coronal plane (represented by a line connecting the most dorsal points of bilateral ilia) at an angle (α) which limited the guide-pin's orientation.After three-dimensional pelvic models of the standard lateral sacral view and the pelvic outlet and inlet views in each patient were calculated,they were displayed in a transparent manner using the software,followed by the virtual insertion of the screw and guide-pin.After the start-point was established on the standard lateral sacral view,the guide-pin was orientated into the pelvic transverse plane and at the guide-pin's α angle relative to the pelvic coronal plane,and subsequently inserted into the ilium shallowly for stabilization.The pelvic outlet and inlet views were taken to judge the guide-pin's orientation.If fine orientation was verified,the guide-pin was advanced to its final position,followed by virtual insertion of an iliosacral screw over the guide-pin.After the virtual insertion was completed,the axial,coronal and sagittal CT sectional views of the pelvis were scrutinized to evaluate the accuracy of insertion.Results After all the guide-pins were orientated in the 180 adults (360 sides) on the true sacral lateral view according to the above way,their orientations on the pelvic outlet and inlet views were 100％ fine,leaving further adjustment unnecessary.The intraosseous insertions of the virtual screws and guide-pins were 100％ accurate and safe on the CT sectional images.Conclusion The way introduced here can theoretically guarantee accurate orientations of the guide-pin on the pelvic outlet and inlet views with no more complex guide-pin adjustments,assuring insertion accuracy and enhancing surgical efficiency.

Objective To evaluate the safety and accuracy of modified percutaneous retrograde intramedullary screwing into the superior pubic ramus or anterior acetabular column for pelvic and acetabular injury.Methods A retrospective analysis was conducted of the 23 patients with pelvic and acetabular injury who had been treated with modified percutaneous retrograde intramedullary screwing into the superior pubis ramus or anterior acetabular column from June 2015 to June 2017 in Luoyang Orthopaedic Hospital of Henan Province.They were 12 men and 11 women,aged from 22 to 88 years (mean,53 years).The injury included 17 pelvic fractures,4 acetabular fractures,and 2 acetabular plus pelvic fractures.The safety and accuracy of screwing were assessed by postoperative physical and imaging examinations.The number of fluoroscopy and operative time were documented for each retrograde intramedullary screwing into the pubic symphysis or acetabular anterior column.The quality of fracture reduction was evaluated by the Mata criteria postoperatively.The Majeed and the Harris scores were used to evaluate the functional recovery of the pelvis and acetabulum at the last follow-ups.Results A total of 31 intramedullary screws were placed in the 23 patients.For insertion of one screw,the operative time ranged from 15 to 50 min (average,35 min) and the number of fluoroseopy from 32 to 55 times (average,45 times).Postoperative physical examinations revealed no iatrogenic neurovascular lesion and uneventful healing of incisions in all the patients.Postoperative imaging examinations showed that 29 screws were located completely in the bone and 2 ones protruded the cortical bone of the pubic anterior border;all the screws did not protrude into the acetabulum or broke through the pubic symphysis or the pubic cortex.By the Matta criteria,the postoperative fracture reduction was rated as excellent in 17 cases,good in 5 and fair in one,giving an excellent to good rate of 95.7％.Of this series,19 were followed up for 6 to 24 months (mean,13 months).All the fractures healed after 3 months.The Majeed scores at the last follow-up for the 13 patients with pelvic fracture were excellent in 12 and good in one.The Harris scores at the last follow-up for the 6 patients with acetabular fracture (including the 2 with pelvic fracture) were excellent in 5 and fair in one.Conclusion The modified percutaneous retrograde intramedullary screwing into the superior pubic ramus or anterior acetabular column is safe,convenient and precise,effectiyely reducing radiation and operative time.

Objective: To survey the conduction and evaluate the effectiveness of extracorporeal membrane oxygenation (ECMO) therapy in pediatric intensive care unit (PICU) in China mainland. Methods: In a questionnaire-based survey, we retrospectively reviewed the application of ECMO in children's hospital and general hospital in China mainland to summarize and analyze the categories of diseases and prognosis of children treated with ECMO therapy. Results: By December 31, 2017, a total of 23 hospitals using ECMO, including 22 tertiary referral hospitals and 1 secondary hospital, among which 16 were children′s hospitals and 7 were general hospitals. Thirty-seven ECMO equipment was available. A total of 518 patients treated with ECMO, within whom 323 (62.4%) successfully weaned from ECMO and 262 (50.6%) survived to discharge. Among 375 pediatric patients, 233 (62.1%) were successfully weaned from ECMO and 186 (49.6%) survived to discharge. Among 143 newborn patients, 90 (62.9%) successfully weaned from ECMO, 76 (53.1%) survived to discharge. ECMO was applied in veno-arterial (VA) mode to 501 (96.7%) patients, veno-venous (VV) mode to 14 (2.7%) patients, and VV-VA conversion mode to 3 (0.6%) patients. Sixty-nine patients required extracorporeal cardiopulmonary resuscitation (ECPR), including 20 newborn patients (29.0%) and 38 pediatric patients (71.0%), who were all with cardiovascular disease. Neonatal respiratory distress syndrome (26/61), persistent pulmonary hypertension of the newborn (PPHN) (12/61), and meconium aspiration syndrome (MAS) (11/61) are the most common pulmonary diseases in newborn patients; among whom, infants with PPHN had highest survival rate (10/12), followed by MAS (9/11). Among newborn patients with cardiovascular diseases, those who admitted were after surgery for congenital cardiac disease were the most common (54/82), while those with septic shock had the highest survival rate (2/3). In pediatric pulmonary diseases, acute respiratory distress syndrome was the most common (42/93), while plastic bronchitis was with the highest survival rate (4/4), followed by viral pneumonia (13/16). Among pediatric cardiovascular diseases, congenital cardiac defect was the most common (124/282), while fulminant myocarditis had the highest survival rate (54/77). Conclusion The application of ECMO as a rescue therapy for children with severe cardiopulmonary failure has dramatically developed in China mainland.