Objective:To summarize the prenatal ultrasound features of fetal cleidocranial dysplasia (CCD) and provide references for clinical consultation.Methods:A retrospective analysis was conducted on the prenatal ultrasound features, genetic testing results, and prognosis of a CCD fetus diagnosed at Qingdao Women and Children's Hospital in June 2023. Relevant literature on CCD was retrieved from the CNKI, Yiigle, Wanfang, and PubMed databases including cases confirmed by genetic testing or postnatal clinical phenotype and imaging with relatively complete prenatal ultrasound information. The prenatal ultrasound features of CCD fetuses were summarized using descriptive statistical analysis.Results:(1) In this case, prenatal ultrasound at 25 weeks of gestation indicated widened cranial sutures with clear near-field intracranial structures, absence of the nasal bone, shortened and rigid bilateral clavicles, and mildly shortened bilateral femurs and humeri. Chromosomal karyotyping and chromosomal microarray analysis showed no abnormalities, but whole exome sequencing detected a RUNX2 gene mutation, leading to a diagnosis of CCD combined with the ultrasound phenotype. At 36 weeks of gestation, the mother experienced premature rupture of membranes and delivered a male infant vaginally. Bedside X-rays indicated bilateral wet lungs and bilateral clavicular dysplasia in the newborn. Telephone follow-up at 9 months showed no abnormalities in growth and development. (2) Literature review: Thirteen cases from 13 articles were included, along with this case, totaling 14 CCD fetuses. The main ultrasound phenotypes of CCD fetuses were clavicular dysplasia (12/14), incomplete cranial ossification (10/14), absence of the nasal bone (8/14), and shortening of the femur (12/14). Other ultrasound phenotypes included scapular dysplasia, short ribs, and increased interocular distance. Conclusion:Clavicular dysplasia and incomplete cranial ossification are the most specific signs of fetal CCD, while absence of the nasal bone and mild femoral shortening are secondary features of CCD.

【Objective】 To supervise the clinical blood use of 19 hospitals, covering a district of Shanghai, during two years, and discover the problems in the process of blood transfusion, so as to put forward suggestions for corrective methods in grades and promote continuous improvement of clinical transfusion management. 【Methods】 A total of 19 hospitals were supervised in terms of hardware facilities, management level, professional and technical level, and blood typing test on the site, according to the Administrative Blood Management Measures for Medical Institutions, Technical Specification for Clinical Transfusion and Shanghai Medical Quality Supervision Score Statistical Table.All data were analyzed. 【Results】 These hospitals can properly perform clinical blood transfusion, but there were obvious differences.Tertiary hospitals were relatively better, yet need to strengthen the management of medical documents.Secondary hospitals remained to be improved, mainly in insufficient construction of Blood Transfusion Department (blood bank), the lack of management and maintenance of key equipment and the lack of standardization in medical documents writing.However, flaws in the supervision were general in private hospitals (most of which were affiliated hospitals), so the management of clinical blood use should be further strengthened. 【Conclusion】 For secondary hospitals or above, routinized writing of medical documents and promoted construction of Blood Transfusion Department (blood bank) should be strengthened.For private hospitals, especially affiliated hospitals, the management of clinical blood use should be further improved, including the examination rules corresponding to the blood use process and strict access and exit mechanism, so as to improve the overall management level of clinical blood use and ensure the safety of clinical blood use.

Objective:To investigate the clinical effect of using combined lumbar pelvis fixation device in the treatment of old vertical unstable fracture dislocation of posterior pelvic ring.Methods:Data of 7 patients with old vertical unstable fracture dislocation of posterior pelvic ring admitted and followed up from January 2017 to April 2020 were retrospectively analyzed, including 4 males and 3 females with an average age of 42.4 years old (range, 22-73 years old). There were 3 cases of traffic injury, 3 cases of falling injury and 1 case of tumble injury. According to Tile classification for pelvic fractures, there were 5 cases of type C1, 1 case of type C2 and 1 case of type C3. The average time from fracture to surgery was 5.4 weeks (range, 3-10 weeks). Among the 7 patients, 4 patients' posterior ring fractures were fixed by combined lumbar pelvic triangle fixation, and 3 patients' posterior ring fracture were fixed by combined lumbar pelvic fixation. 4 patients' anterior ring injury were not treated, 2 patients' anterior ring injury were treated by closed cannulated screw and internal fixation, and 1 patient's anterior ring injury was fixed by INFIX and cannulated screw. Every patient's operating time, intraoperative blood loss, length of incision and times of X-ray fluoroscopy were recorded. Pelvic X-ray and CT scan were taken postoperatively to observe the condition of reduction and screw position. Postoperative fracture reduction quality was assessed by Matta radiological criteria and Majeed criteria was used at the final follow-ups to evaluate the degree of functional recovery after pelvic fracture.Results:The average operating time of 7 patients was 143 min (range, 96-205 min); the intraoperative average blood loss was 579 ml (range, 300-1 650 ml); the average length of incisions was 12.9 cm (range, 9-15 cm) and the average time of X-ray fluoroscopy was 27 times (range, 15-52 times). Postoperative X-ray and CT scan showed that the displacements of the posterior rings were reset well and all the hollow screws were located accurately and firmly. Postoperative radiation quality was evaluated according to Matta radiological criteria, and there were 4 cases of excellent, 2 cases of good and 1 case of fair, with an excellent and good rate of 85.7% (6/7). Seven patients had good fracture union. The average followed up time for all 7 patients was 12 months (range, 6-16 months). At the last follow-up, imaging examination showed good reduction of the sacroiliac joint, and the reduction of anterior and posterior rings were not lost. The healing time was 14.2 weeks (range, 12-20 weeks). Majeed score: postoperative 4.90±6.64 points (range, 48-58 points), postoperative 3 months 71.40±7.32 points (range, 67-75 points), postoperative 6 months 84.90±8.14 points (range, 68-96 points), the difference was statistically significant ( F=0.614, P=0.004). Majeed score 6 months after operation showed that 5 cases were excellent, 1 case was good and 1 case was fair, and the excellent and good rate was 85.7% (6/7). Conclusion:Using combined lumbar pelvis fixation device in the treatment of old vertical unstable fracture dislocation of posterior pelvic ring has good reduction quality, high fixed strength and good postoperative effect.

Objective:To investigate the clinical efficacy of using INFIX combined with hollow nail in the treatment of anterior pelvic ring injury with symphyseolysis.Methods:Data of 12 patients with anterior pelvic ring injury and symphyseolysis who were treated from January 2016 to December 2018 were retrospectively analyzed. They were 8 males and 4 females with an average age of 39.5 years (range, 23-64 years). There were 4 cases of traffic injury, 3 cases of falling injury and 5 cases of crush injury; all the cases were combined with front and rear crush injury. According to Tile classification for pelvic fractures, there were 6 cases of type B1 , 3 cases of type B3, 1 case of type C1 and 2 cases of type C2. Six cases of B1 and 3 cases of B3 patients used percutaneous inter fixation with sacroiliac joint cannulated screw in posterior ring; 1 case of C1 and 2 cases of C2 used spinal-pelvic fixation. Every patients' operating time of INFIX combined with the pubic symphysis hollow nail , amount of hemorrhage, length of incisions and number of X-ray projections were recorded. Pelvic X-ray and CT scan were taken postoperatively to observe the reduction and screw position. Postoperative reduction quality was assessed using Matta radiological criteria and Majeed criteria was used at the final follow-up to evaluate the pelvic function.Results:The average operating time for 12 patients was 42 min (range, 35-56 min) ; the average amount of hemorrhage was 28 ml (range, 15-40 ml); the average length of incision at INFIX nail placement on both sides were 2.7 cm (range, 2.2-3.5 cm); the average length of incisions at the pubic symphysis hollow nail placement was 0.8 cm (range, 0.6-1.2 cm) and the average number of X-ray projections was 38 times (range, 26-55 times). Postoperative X-ray and CT scan showed that all the hollow screws are located accurately and firmly, and pubic symphysis had good reduction and no infection occurred in the surgical incision at the screw placement site. Postoperative reduction quality was evaluated according to Matta radiological criteria, and there were excellent in 10 and good in 2, giving an excellent to good rate of 100% (12/12). The average follow-up time for the 12 patients was 12 months (range, 6-15 months). At the final follow-up, all patients showed fine fracture union and no looseness of pubic symphysis occurred according to imaging test. According to the Majeed criteria, the pelvic function was excellent in 8 cases, good in 3 cases and fair in 1 case, giving a good to excellent rate of 91.7% (11/12). One case developed symptoms of femoral nerve compression after surgery and returned to normal after removal of the INFIX. Another case had damage to the lateral femoral cutaneous nerve and the symptoms disappeared after three months.Conclusion:Using INFIX combined with hollow nail in the treatment of symphyseolysis can lead to more minimally invasive with excellent postoperative efficacy and markedly reduced the morbidity of complication.

Objective:To explore the effect of humanistic care in patients with hematopoietic stem cell transplantation.Methods:Totally 51 patients with hematopoietic stem cell transplantation were recruited from the First Affiliated Hospital of Xi'an Jiaotong University,between September 2014 and July 2016,and were randomly divided into two groups.Patients in the intervention group (n =25) received humanistic care,while those in the control group (n =26) received routine nursing.The incidence of adverse psychological status and nursing satisfaction were compared between the two groups.Results:The incidence rate of adverse psychological state in the intervention group (16％) was significantly lower than that in the control group (53％),while the nursing satisfaction in the interventiongroup (92％) was significantly higher than that in the control group (69％),P ＜0.05.Conclusion:Application of humanistic care in the patients with hematopoietic stem cell transplantation can significantly improve nursing satisfaction and reduce the incidence of adverse psychological state,which will improve the confidence of the patients and the quality of nursing satisfaction.Consequently,it will promote the relationship between the nurses and patients.

non-myeloablative BuCy+fludarabine conditioning regimen,and another one was treated with TBI+VP-1 6 +CTX+CCNU conditioning regimen.Only one case received short-term MTX,cyclosporin A and ATG regimen for prevention of graft-versus-host disease (GVHD).The GVHD prevention regimens of the other patients were based on short-term MTX,cyclosporin A,ATG and mycophenolate mofetil regimen.The hematopoietic reconstitution, complications and prognosis were observed.Results One patient died of intracranial hemorrhage,and hematopoi-etic reconstitution was achieved in the other 20 patients.The median time for hematopoietic reconstitution shortened by one day in large-dose group compared with that in low-dose group.Adverse reactions included high fever, shivering,gastrointestinal tract adverse reaction,liver injury,oral mucositis and other rare side effects.GVHD occurred more frequently in patients with HLA mismatched transplantation.Nine patients with aGVHD and 9 patients with cGVHD recovered after effective treatment.Within 100 days after transplantation,18 patients had bacterial or fungal infection,mainly upper respiratory tract infection;7 patients had cytomegalovirus infection;2 had EB viremia,and one had urinary BK virus infection.Only one patient died of VOD.Hemorrhagic cystitis occurred in 5 patients and improved after treatment.The median survival time was 24 months (ranging from 136 days to 9 years).One-year and 3-year overall survival rates were 85.2% and 63.9%,the disease free survival rates were 81% and 23.8%,recurrence free survival rates were 71.4% and 14.3%,respectively.Conclusion URD-HSCT was an effective method to treat leukemia.Conditioning regimen of BuCy and modified BuCy2 were safe and effective,the adverse reactions were reversible and well tolerated.Hematopoietic reconstitution time shortened in large-dose MNC and CD34 + cell number groups compared with that in low-dose group.The occurrence rate of GVHD with HLA mismatched transplantation was more than that of HLA matched transplantation.Low-dose heparin,prostaglandin E1 and Danshen injection can effectively prevent VOD.

Objective To assess the safety and efficacy of adopting different vascular access during autologous peripheral blood stem cells collection.Methods 87 patients received autologous peripheral blood stem cells collection were divided into two groups:One was peripheral vein harvesting group (43 cases),which used the 16G disposable fistula needle for autolo-gous peripheral blood stem cell collection and the other central venous harvesting group (44 cases),which used double cavity of femoral vein catheter for autologous peripheral blood stem cell collection.The observation indicators included venous ac-cesses,collection efficiency,patient tolerance,the number of mononuclear cell and CD34 positive cells.Results The numbers of mononuclear cells and CD34 positive cells in two groups were all above the standard and there was no significant differ-ence (P >0.05).However,the success of venous accesses,the efficiency smooth of collection and-patient tolerance were bet-ter in double cavity of femoral vein catheter group (P <0.05).Conclusion Harvesting the autologous peripheral blood stem cell through central venous by using double cavity of femoral vein catheter had the advantages as follows:high success rate of puncture,acquisition smoothly and reducing the suffering extent of the patients,and also it did not affect the acquisition effi-ciency and effectiveness.

Background:Gastrointestinal dysmotility is frequently seen in patients with chronic obstructive pulmonary disease ( COPD). Simo Decoction is used for promoting gastrointestinal motility in clinical practice. Aims:To study the effect of Simo Decoction on gastrointestinal symptoms and motility in patients with COPD. Methods:Thirty-six patients with stable COPD in Shiyan Renmin Hospital from Sep. 2012 to Jun. 2013 were enrolled and received a 28-day course treatment of Simo Decoction oral solution,19 healthy volunteers were served as controls. All subjects were assessed with gastrointestinal symptom rating scale(GSRS). Electrogastrography was performed and levels of vasoactive intestinal peptide(VIP)and intestinal fatty acid-binding protein( I-FABP)in peripheral blood were measured. Patients with COPD were re-examined for the above-mentioned parameters after treated with Simo Decoction. Results:The baseline GSRS score and serum I-FABP level in COPD patients were significantly increased as compared with controls(P<0. 05),while the baseline serum VIP level,as well as the fasting and postprandial normal gastric slow wave percentage in COPD patients were significantly decreased(P <0. 05). After 28-day course of Simo Decoction treatment,all these parameters in COPD patients were improved as compared with those before treatment(P<0. 05),serum levels of VIP,I-FABP and normal gastric slow wave percentage were similar to those of controls. Conclusions:Simo Decoction can ameliorate the gastrointestinal symptoms and dysmotility in patients with COPD. The regulatory effect of Simo Decoction on gastrointestinal motility might be bidirectional.

Objective To investigate the gastrointestinal symptoms in patients with chronic obstructive pulmonary disease (COPD) and explore its possible reasons.Methods Thirty-six patients with stable COPD were divided into mild group [21 cases,forced expiratory volume in first second (FEV1) measured values/predictive values ≥50％] and severe group (15 cases,FEV1 measured values/predictive values ＜ 50％) according to the pulmonary function.Replacement of 19 healthy volunteers as control group.The patients and volunteers completed the Gastrointestinal Symptom Rating Scales (GSRS) scores and received electrogastrography,the plasma vasoactive intestinal peptide (VIP) and intestinal fatty acid binding protein (Ⅰ-FABP) were detected.Results The GSRS scores in severe group and mild group was higher than that in control group[(17.73 ± 4.64),(14.29 ± 5.44) scores vs.(8.00 ± 2.29) scores],and severe group was higher than that in mild group,there was significant difference (P ＜ 0.05).The percentage of normal slow waves of fasting and postprandial,the percentage of bradygastria in severe group and mild group were lower than those in control group,there were significant differences(P ＜ 0.05).The level of VIP in severe group was lower than that in mild group and control group [(36.07 ± 9.22) μ g/L vs.(44.16 ± 7.88),(46.53 ± 4.54) μ g/L],the level of I-FABP was higher than that in mild group and control group [(35.80 ± 11.69) μ g/L vs.(27.40 ±9.53),(23.58 ±8.18) μg/L],there was significant difference (P ＜0.05),but there was no significant difference between mild group and control group (P ＞ 0.05).Conclusion The patients with COPD are accompanied with serious gastrointestinal symptoms and gastric dysrhythmia,severe COPD can be associated with VIP decrease and Ⅰ-FABP rise in peripheral blood.

Objective To investigate the expression of Foxp3+ Treg in liver cancer patients receiving liver transplant after immunosuppressive protocols FK506 or Rapamycin and evaluate the rejection prevention.Methods Liver transplant patients were randomly divided into FK506 group (10 cases),and Rapamycin group (10 cases).Real time quantitatve PCR was used to examine Foxp3 mRNA expression of patients' PBMC starting the 2nd month for consecutively 10 months.Foxp3 mRNA and incidences of acute rejection were compared between the two groups.Results Foxp3 mRNA expression was significantly lower in the FK506 group (0.1032 ±0.0943) as compared to that in the Rapamycin group (1.2136 ±0.6738)(t =5.1610,P ＜ 0.01) ;The incidences of acute rejection was significantly lower in the Rapamycin group as compared to that in the FK506 group after operation in the same period (x2 =2.2222,P ＜ 0.05).Conclusions FK506 may suppress the induction of immune tolerance after liver transplantation,while Rapamycin may play an important role in inducing and maintaining graft immune tolerance.Rapamycin is better than FK506 in preventing rejection reaction in liver cancer patients receiving liver transplant.