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 explore the application of nurse-performed point-of-care ultrasound in abdominal distension of ICU patients with enteral nutrition.Methods Purposive sampling method was used to select 108 patients with enteral nutrition abdominal distension admitted to the ICU of a tertiary A general hospital in Hefei City from January to December 2022,and they were divided into an experimental group and a control group according to random number table method.The experimental group used bedside ultrasound to evaluate the stomach,intestine and abdominal cavity of patients with abdominal distension,and implemented targeted treatment measures according to the evaluation results.The control group was given corresponding treatment measures according to the abdominal sight,touch,tapping,listening and traditional syringe withdrawal method.Intra-abdominal pressure,abdominal circumference,feeding compliance rate and feeding interruption rate,ICU stay time and mechanical ventilation time were compared at the 1st,3rd and 5th day.Results 11 cases dropped out of the study due to transfer,automatic discharge,death,etc.,and 97 cases were included 51 cases in the experimental group and 46 cases in the control group.Repeated measurement ANOVA showed that intra-abdominal pressure and abdominal circumference time in the 2 groups had interaction with each other(F=41.724,P＜0.001;F=17.762,P＜0.001).Generalised estimating equations showed that there was only a between-group effect for the rates of feeding compliance and feeding disruption in 2 groups(Wald y2=18.033,P＜0.001;Wald x2=6.567,P＜0.001).Simple effect analysis showed that on the 3rd and 5th day after intervention.The experimental group had lower abdominal depression and the feeding interruption rate was lower than the control group(P＜0.05).On the 5th day after interven-tion,the abdominal circumference of the experimental group was lower than that of the control group,the feeding compliance rate was higher than that of control group(P＜0.05).After intervention,in the experimental group,the mechanical ventilation time was shorter than that of the control group(P＜0.05).There was no significant difference in ICU stay time between the 2 groups(P=0.095).Conclusion The application of nurse-performed point-of-care ultrasound in the management of abdominal distension in patients with enteral nutrition in ICU is feasible and scientific,which is helpful for the management of patients with enteral nutrition abdominal distension in ICU,reducing internal abdominal pressure,shortening mechanical ventilation time,and avoiding the occurrence of feeding intolerance.

Peri-implantitis, characterized by inflammation of tissues around implants and gradual loss of supporting bone tissue, has become one of the main causes for implant failure. Thoroughly removing the plaque biofilm on the implant surface is the first principle in the treatment of peri-implantitis. For this reason, various decontamination methods have been proposed, which can be divided into 2 categories: Removing biofilm and killing microorganisms according to the effect of plaque biofilm on the implant surface. However, at present, there is no decontamination method that can completely remove the plaque biofilm on the implant surface, and it lacks of clinical recommended guidelines. To understand the advantages and disadvantages, effectiveness and safety for different implant surface decontamination methods is of great significance to guide the clinical selection for peri-implantitis treatment.
Bone and Bones ; Decontamination ; Dental Implants ; Humans ; Inflammation ; Peri-Implantitis/therapy* ; Prostheses and Implants

Objective To investigate feasibility of bowel preparation for colonoscopy in endoscopic ambulatory surgery ward. Methods A total of 352 patients at endoscopic ambulatory surgery ward receiving colonoscopy at Zhongshan Hospital, Fudan University from May 25, 2018 to July 13, 2018 were surveyed by questionnaires. Data of 344 patients, including patient demographics, bowel preparation information and Boston bowel preparation scores and subjective perception of patients were analyzed. Data of 8 others were excluded because they did not complete colonoscopy for the first time due to failure of bowel preparation. Chi-square test and logistic regression analysis were conducted to analyze the quality of bowel preparation and its influencing factors. Results A total of 18. 0％( 62/344) of bowel preparations were inadequate. Morning colonoscopy ( P = 0. 005, OR = 2. 505, 95％CI:1. 312-4. 781 ) and yellow residual last stool before colonoscopy (P=0. 045,OR=0. 475,95％CI:0. 230-0. 982) were independent risk factors for inadequate bowel preparation. Patients' tolerance was 88. 1％(303/344), satisfaction was 77. 9％(268/344), and 54. 9％(189/344) were willing to be hospitalized in ambulatory surgery ward again if necessary. Conclusion Bowel preparation before colonoscopy in endoscopic ambulatory surgery ward is feasible but not optimal.

Objective To analyze the clinical value of low molecular heparin calcium combined with early rehabilitation intervention in the prevention of deep vein thrombosis after internal fixation of intertrochanteric fractures.Methods 100 patients with internal fixation for treatment of femoral intertrochanteric fracture from January 2014 and October 2015 were selected and randomly divided into two groups:the control group of 50 cases, treated with low molecular weight heparin low molecular weight heparin by abdominal subcutaneous injection of drug therapy and routine nursing intervention service, observation group of 50 cases, in the control group based on the use of early rehabilitation intervention.The incidence of deep venous thrombosis after operation in two groups was recorded, and the changes of platelet count and coagulation markers were observed before and after intervention in the two groups.Results The observation group of patients with postoperative deep venous thrombosis incidence rate of 4%, significantly lower than the control group 16%, the difference was statistically significant (P<0.05), the two groups after the intervention each time the platelet count was higher than that of before intervention, four weeks after the intervention, the observation group of platelet count (239.51 ±34.62) ×109/L, significantly higher than the control group (235.23 ±28.87) ×109/L, the difference was statistically significant (P<0.05), the two groups before the intervention of each marker concentration, the difference was not statistically significant, patients in the observation group after the intervention, the blood coagulation markers concentrations were significantly lower than before treatment, and were significantly lower than the control group after the intervention.The comparison between the two groups, the difference was statistically significant (P<0.05).There was no chest pain, chest tightness, dyspnea and other symptoms of pulmonary embolism in the two groups , and no adverse reactions were found.Conclusion The application of low molecular weight heparin calcium combined with early rehabilitation intervention can effectively reduce the incidence of deep venous thrombosis after intertrochanteric fracture fixation.

BACKGROUND:Artificial knee joint replacement is the main surgical method for the treatment of severe knee joint deformation, but due to the limited existing knee prosthesis type. The stature of Fujian population in southeast China was shorter than that of the north. The size of artificial knee joint usual y did not fit for patients in clinic. The substitution effect was poor.
OBJECTIVE:To measure the geometric parameters of proximal surface and cross-section of adult female tibia in southeastern area of Fujian Province of China, and provide basic data for design and replacement of artificial knee joint in females.
METHODS:We chose 85 cases of dry female skeleton in southeastern area of Fujian Province of China, measured tibial plateau width, medial tibial plateau width and anteroposterior diameter, lateral tibial plateau width and anteroposterior diameter. We col ected clinical normal proximal tibial CT data of 36 patients (38-65 years old), with scan range of 30 cm (intercondylar eminence 30 cm) and layer thickness of 0.625 mm. Mimics 16.0 three-dimensional reconstruction software was used to measure above indexes and cut the reconstructed models with thickness of 3 mm, total y 10 layers. We divided its widest distance into four equal parts, and measured tibial anteroposterior diameter on three points. Statistical software was used to analyze the linear relationship between the data.
RESULTS AND CONCLUSION:The experiment has successful y obtained geometric parameters of tibial specimens of adult females in southeastern area of Fujian province of China. Tibia platform inside and outside diameters were greater than the width inside and outside;inside and outside diameter and inside and outside width were similar. Ten segment planes of tibia, inside and outside diameters were always less than middle anteroposterior diameter. Three groups of data increased firstly, and then reduced. There were positive correlations between tibia length and width of the medial tibia platform, the lateral tibia platform width and anteroposterior diameter, the medial tibia platform width and anteroposterior diameter, and the lateral tibia platform width and anteroposterior diameter (P<0.05). These results suggested that relevant parameters of tibial plateau adult women in southeastern area of Fujian province of China showed some regular changes. Our results may provide quantitative reference data for the design and operation of artificial tibial plateau.

Objective To evaluate the efficiency of second trimester screenings for Down syndrome using alpha-fetoprotein and β-human chorionic gonadotropin duplex.MethodsPregnant women of south Zhejiang were screened for Down syndrome fetuses by maternal alpha-fetoprotein and β-human chorionic gonadotropin duplex during second trimester.The high-risk women underwent prenatal diagnosis by amniocentesis,cell culture and chromosome analysis.The newborns followed up by the maternal and child tertiary health care network and suspected to have Down syndrome were diagnosed by peripheral blood chromosome analysis.Statistical analysis was performed using two-sample t test and x2 test.Risk probability of Down Syndrome was calculated by random screening software. Results From Oct.2007 to May 2009,1130 of 32 188 singleton pregnant women in second trimester received prenatal screening were discovered with high risk(≥1 ∶ 270).Prenatal diagnosis was performed in 90.79％ cases (1026/1130) of high risk women and seven fetuses were diagnosed as Down syndrome by amniotic fluid chromosome analysis,and the pregnancies were terminated.Among the other 104 cases without prenatal diagnosis one Down syndrome baby was delivered.Six of 31 058 pregnancy women with low risk delivered Down syndrome babies with the incidence of Down syndrome of 0.19‰ (6/31 058).Detection rate of second trimester screenings for Down syndrome using alpha-fetoprotein and β-human chorionic gonadotropin duplex was 57.14％(8/14).False positive rate was 3.48％ (1122/32 188).Positive predictive value was 7.08‰(8/1130).During the same period,there were 23 813 pregnant women who didn't receive screening and 15 fetuses with Down syndrome were diagnosed after birth.There was no statistical difference in the prevalence rate of Down syndrome between those pregnant women who received prenatal screening or not [0.43‰ (14/32 188) vs 0.63‰ (15/23 813),x2 =1.004,P＞0.05].The prevalence of Down syndrome was 0.52‰ (29/56 001) in this area. ConclusionsThe prenatal screening and diagnosis could reduce the birth rate of Down syndrome patients.However,detection rate,false positive rate and positive predictive value of which were lower than reports in other studies.It's possible that the reference data might be not suitable for Chinese.

Objective To explore the effect of enteral nutrition applied to bowel preparation of diabetes patients before electronic colonoscopy. Methods A total of 172 diabetes patients were randomly allocated to the experimental group and the control group with 86 patients in each group. The experimental groups executed no dietary restriction within 3 days before the examination, and received Ensure 1h before colonoscopy. However,the control group was given low residue diet within 3 days before the examination, and received no Ensure before colonoscopy. The enteral cleanness and incidence of hypoglycemic reaction, abdominal pain and horror was compared between the two groups. Results 72 cases(83.72%) of the experimental group and 70 cases (81.40%) of the control group had better enteral cleanness, and there was no statistical difference in intestinal cleanness between the two groups. In the experimental group the incidence of hypoglycemic reaction, abdominal pain, horror was significantly lower than those in the control group. Conclusions Bowel preparation with unrestricted diet and ingested ensure before colonoscopy can not only ensure intestinal cleanness but also enhance endurance of colonoscopy.

Objective Repair of aortic arch aneurysm is technically demanding and usually requiring complex circulatory management. Operative morbidity and mortality may be prohibitive with traditional surgical intervention. We described our experience with 5 hybrid endovascular procedure for aorta repair with different kinds of bypass followed by concomitant placement of stent graft in the aorta. Methods We retrospectively reviewed the clinical data of 5 consecutive patients presenting with aortic aneurysm or dissection from 2007 to 2008 treated by the hybrid aorta repair. Complete surgical rerouting of the supra-aortic vessels was followed by endovascular repair of aortic arch aneurysm with a Zenith TX2 stent graft. Hybrid left carotid-subclavian bypass with Zenith stent graft deployment covering the ostium of the LSA was performed in a Debakey type Ⅲ aortic dissection case. Procedures were successfully completed with exclusion of the aortic aneurysm. All stent grafts were deployed retrograde from the femoral artery in these patients. Results Technical success with complete aneurysmal exclusion was achieved in all patients (100%). At a follow-up period of 2-10 months, there was no incidence of endoleak. Documented perioperative neurelogic events did not occurred in all patients. Postoperatively one patient suffered from ARDS and cardiac failure and recovered. One patient died of myocardial infarction. Conclusions Hybrid arch repair provides an alternative to patients otherwise considered prohibitively high risk for traditional open arch and thoracoabdominal aorta repair.

Objective To evaluate arterial reconstruction of traumatic iliac-femoral artery pseudoaneurysm in parenteral drug abusers.Methods Data of 21 consecutive patients who presented with iliac-femoral artery pseudoaneurysm with the diameter ranging from 3.0 cm～7.5 cm secondary to parenteral drug abuse from 2004 to 2007 were analyzed.Fifteen patients were male and the median age was 3 1.3 years.the aneurysm involved the common femoral artery and distal external iliac artery.Autologous greater saphenous vein was used as a graft for arterial reconstruction after iliac-femoral artery pseudoaneurysm excision through a single curved inguinal incision.Results The surgical procedure was successful in all 21 eases without intraoperative mortality and severe complications.All the 21 patients were free of postoperative claudication symptoms except 1 case with preoperative popliteal artery stenosis.Wound infection and tissue fistula developed in one ease.Ingunal incisional hematoma and cutaneous abnormal sensation developed in one each eases.Conclusions The use of autologous greater saphenous venous grafts for aaefial reconstruction after false aneurysm excision in drug abusers is safe and effective.The harvest of the greater saphenous vein is precondition of this precedure.These preliminary results indicate that the implementation of this technique offers advantages compared with the artery ligation alone without revascularization,that is frequently associated with later intermittent claudication.