Antley-Bixler syndrome (ABS) is a rare developmental malformation, usually caused by mutations in the fibroblast growth factor 2 (FGFR2) or cytochrome P450 oxidoreductase (POR) genes. This article reports an 18-year-old male patient with both ulcerative colitis and ABS to explore the possible association between the two diseases, to raise clinicians' awareness, and to provide reference value for early diagnosis and treatment in the future.

Antley-Bixler syndrome (ABS) is a rare developmental malformation, usually caused by mutations in the fibroblast growth factor 2 (FGFR2) or cytochrome P450 oxidoreductase (POR) genes. This article reports an 18-year-old male patient with both ulcerative colitis and ABS to explore the possible association between the two diseases, to raise clinicians' awareness, and to provide reference value for early diagnosis and treatment in the future.

Objective:To study the clinical features in patients with acute necrotizing pancreatitis (ANP) complicated by hemorrhage, and to analyze the treatments and their outcomes.Methods:The clinical data of 44 ANP patients with hemorrhage managed at the Department of Pancreas Center, the First Affiliated Hospital of Nanjing Medical University from September 2015 to December 2020 were retrospectively analyzed. There were 34 males and 10 females, aged (48.9±12.2) years old. Clinical data were collected on the bleeding sites, bleeding interventions, and treatment outcomes. Follow-up visits were made by outpatients visits or telephone.Results:Of the 44 patients with bleeding, 8 had gastrointestinal bleeding, 31 had intra-abdominal bleeding, and the remaining 5 had mixed bleeding sites. The median interval from onset of ANP to development of hemorrhage was 30.5(20.8, 40.3) d. For the 13 patients with gastrointestinal bleeding and mixed sites of bleeding: 4 patients were successfully treated by endoscopically for upper gastrointestinal ulcers, 5 patients were successfully treated by endovascular embolization using digital subtraction angiography (DSA) to detect the sites of bleeding, and 4 patients were successfully treated by surgery. For the 31 patients with intra-abdominal hemorrhage: 24 underwent DSA. For the 7 patients who did not undergo DSA, 3 who were hemodynamically stable were treated conservatively, 2 underwent immediate open surgery to stop bleeding within 24 h after surgical debridement of infected pancreatic necrosis, 1 did not undergo DSA because the family members decided to abandon further treatment, and 1 died while preparing for DSA. For the 29 patients who underwent DSA, vascular abnormalities were found in 69.0%(20/29), with splenic artery hemorrhage being the most common. In the 44 patients with bleeding: 29.5%(13/44) were examined by endoscopy, and 4 were successfully stopped by endoscopic treatment; 65.9%(29/44) patients were examined by DSA, and 15 patients were successfully treated by intravascular embolization; 14 patients (31.9%) were treated by open surgery and 11 patients were successfully stopped. The mortality rate was 47.7%(21/44), of which 5 patients died from hemorrhagic shock complicated by multiple organ dysfunction syndrome (MODS) and 16 patients died from sepsis complicated by MODS. The mortality rate of 55.6%(20/36) in patients with intra-abdominal and mixed sites of bleeding was significantly higher than that of the 12.5%(1/8) in patients with gastrointestinal bleeding ( P=0.048). None of the 23 surviving patients developed recurrence of intra-abdominal and/or gastrointestinal bleeding on follow-up. Conclusion:Major bleeding commonly occurred about 1 month after ANP and it was associated with a higher in-hospital mortality rate. DSA, endoscopy, and open surgery were effective means to achieve hemostasis.

Splenic lymphoma with villous lymphocytes (SLVL) or splenic marginal zone lymphoma with circulating villous lymphocytes is rare, and prolymphocytic transformation of SLVL is rarer. At present, only one case of SLVL with t(8;14)(q24;q32) translocation has been reported. In this study, we report a case of B-lymphoproliferative disorder with villous lymphocytes harboring t(8;14)(q24;q32) chromosome translocation that we inclined to SLVL with a prolymphocytic transformation. A 73-year-old female showed marked hepatosplenomegaly and high lymphocytosis (lymphocytes > 200 × 10/L). The abnormal lymphocytes had short coarse villi and round nuclei with prominent nucleoli. The immunophenotypes showed CD19, CD20, HLA-DR, CD22, CD5, Kappa, CD25, CD71, Lambda, CD7, CD10, CD23, CD34, CD33, CD13, CD14, CD117, CD64, CD103, and CD11c. The karyotype showed complex abnormality: 46XX,+ 3,-10, t(8;14)(q24; q32)[11]/46XX[9]. The cytoplasmic projection, immunological characteristics, and trisomy 3 chromosome abnormality supported the diagnosis of SLVL. However, the presence of prominent nucleoli and high lymphocytosis suggested prolymphocytic transformation, probably as a result of t(8,14) chromosome translocation. In this report, we described an unusual case of B-lymphoproliferative disorder with villous lymphocytes harboring t(8;14)(q24;q32) translocation, which could provide help in the diagnosis and differential diagnosis of B-lymphocytic proliferative diseases.
Aged ; B-Lymphocytes ; pathology ; Female ; Humans ; Immunophenotyping ; Lymphoproliferative Disorders ; genetics ; pathology ; Translocation, Genetic

Severe acute pancreatitis (SAP) is an ancient and fatal disease,the understanding of this disease has experienced tortuous and long-time process.Throughout the history,surgical intervention plays a dominant role in treatment of SAP.Instead of traditional “archaeological style-like” extensive resection,the strategy of minimally invasive debridement and drainage that was called “grave digging” is preferred currently.In the past two decades,with in-depth cognition about this disease,a series of interventional,minimally invasive & endoscopic techniques derived from computer-assisted imaging techniques,surgical strategy represented by “ step-up” minimally invasive methods have been developed,which has changed traditional treatment model,brought satisfied therapeutic effect,and been widely accepted.Authors reviewed the process of surgical treatment in SAP for seeking deep understanding,gaining experiences,expanding our horizons,to guide clinical practices and avoid insufficient or excessive intervention,aiming to reduce mortality & morbidity and bring the maximal benefits for patients.

Objective To study the positive end-expiratory pressure (PEEP) adjustment after recruitment maneuver during acute respiratory distress syndrome (ARDS) especially in the presence of tonic diaphragm electrical activity (Tonic EAdi) in order to obtain optimum PEEP and in turn to get successful oxygenation.Methods Rabbit model of ARDS was nade by intratracheal instillation of hydrochloric acid.After sufficient recruitment maneuvers,the rabbits were randomly (random number) divided into two groups,namely Tonic EAdi group ( n =5 ) and maximum oxygenation group ( n =5 ).In Tonic EAdi group,the regulation of PEEP was guided by Tonic EAdi.In maximum oxygenation group,PEEP was adjusted as per maximum oxygenation.The differences in magnitude of PEEP,pulmonary mechanics,gas exchange and hemodynamics were compared between two groups.The t-test was used to compare continuous variables between the two independent samples,and the difference was statistically significant when P ＜ 0.05.Results (1) PEEP:The PEEP was (10.7 + 1.4) cmH2O (1 cm H2O=0.098 kPa) in Tonic EAdi group and (10.0 ± 2.8) cm H2O in maximum oxygenation group (P ＞ 0.05). (2) Pulmonary mechanics:After PEEP adjustment,there was no significant difference in tidal volume ( Vr),peak pressure (Ppeak) and mean pressure (Pmean) between the two groups (P ＞ 0.05 ).(3) Gas exchange:After PEEP adjustment,there was no significant difference in oxygenation index (PaO2/FiO2) and partial pressure of arterial carbon dioxide ( PaCO2 ) between the two groups (P ＞ 0.05).Conclusions Tonic EAdi could be a good indicator for regulating PEEP in ARDS.

Objective To evaluate the effect of the pulmonary stretch reflex on the lung injury in acute respiratory distress syndrome (ARDS). Method ARDS models of rabbits were induced by intratracheal infusion hydrochloric acid and ventilated with neurally adjusted ventilatory assist (NAVA) with a tidal volume ( VT) of 6 mL/kg and the electrical activity of diaphragm ( Eadi)-determined PEEP level. The rabbits were randomly ( random number) divided into two groups: ( 1 ) sham operation (Sham) group ( n = 5 ),(2) bilateral vagotom (VAG) group( n = 5 ). Gas exchange and pulmonary mechanics were determined at baseline, after lung injury and ventilation 1, 2, 3 h respectively. Indices about pulmonary permeability,pathological changes and inflammatory response were also measured. Results Compared with Sham group,the PaO2/FiO2in VAG group decreased significantly at ventilation 2 h, 3 h (P ＜0.05). There was no significant difference on PaCO2 between Sham and VAG group (P ＞ 0.05 ), and VAG group had the higher VT,peak pressure ( Ppeak), mean pressure (Pm) compared with Sham group at the time point of ventilation 1 h, 2 h, 3 h (P＜0.05). Compared with Sham group, the dead space fraction (VD/VT) and the respiratory system elastance (Ers) in VAG group increased (P ＜ 0.05 ) and the static pulmonary compliance (Cst)decreased markedly (P ＜ 0.05 ) after 3 h ventilation. The wet/dry weight (W/D), lung injury score, tumor necrosis factor-α ( TNF-α), interleukin-8 ( IL-8 ), myeloperoxidase ( M PO ) and malondialdehyde ( M DA )in VAG group elevated significantly when compared with Sham group ( P ＜ 0.05 ). Conclusions The lung injury in ARDS was aggravated after bilateral vagotomy, which demonstrated that the pulmonary stretch reflex may have the lung protective effect.

Objective To evaluate the predictive performance of neuro-mechanical coupling (NMC) and neuro-ventilatory coupling (NVC) in the weaning outcome in patients with chronic obstructive pulmonary disease (COPD). Methods Sixteen patients were enrolled when the criteria for their first spontaneous breathing trial (SBT) was met. A 30-minute SBT was attempted, with the measurement of electrical activity of the diaphragm (Edi) , NMC, NVC, NVC ×NMC, index of rapid shallow breathing (f/Vt) , airway occlusion pressure (P0.1) and f/Vt ×P0.1 at 0, 5 and 30 min. The receiver operating characteristic (ROC) curve was calculated to evaluate the predictive performance of each index. Results Successful weaning(S group) was observed in 6 patients while weaning failure(F group) in 10 patients. (1)The predictive capacity of Edi: at 30 min of SBT, Edi showed higher values in the F group (P ＜ 0. 05), the area under the ROC curves(AUC) was 0. 817(P ＜0. 05). (2) The predictive capacity of NVC and NMC:at 5, 30 min of SBT, NVC and NMC showed higher values in the S group (P ＜0. 05); at 30 min of SBT NVC presented the largest AUC than any other time of SBT (0. 822, P ＜ 0. 05), while the AUC of NMC was 0. 800 (P ＞ 0. 05). (3) The predictive capacity of NVC × NMC: at 30 min of SBT, the AUC of NVC × NMC was larger than NVC (0. 864, P ＜ 0. 05) , showing greater sensitivity (100. 0%) and specificity (83. 3%) .(4) The predictive capacity of f/Vt and P0.1: f/Vt and f/Vt × P0.1 presented poor predictive performance in the failed patients. Conclusions Edi, NVC and NVC × NMC were good predictor for the weaning outcome in patients with COPD.

Objective:To introduce our experience on extra-operating room anesthesia of children with grave congenital heart diseases during cardiac catheterization an d ventriculography. 　　Methods:Three hundred and sixty-nine children with grave congenital heart d iseases undergoing cardiac catheterization or ventriculography were selected for this study,among whom 86 are presented with left to right shunt and pulmonary a rtery hypertention,11 with pulmonic stenosis and primary pulmonary artery hypert ention,and 272 with right to left shunt.They were routinely fasted before the pr ocedure.O2 Saturation(SpO2) and electrocardiogram were monitored and blood p ressure were recorded.With oxygen inhalated by mask and venous route established ,the children were injected with scolapamine (0.02 mg/kg) and ketamine (1-2 mg /kg) for anesthesia induction.When patients lost conciousness,ketamine (6-8 mg/ kg) and droperidol (0.15-0.30 mg/kg) were given intromascularly for maintaine nce.During the procedure,ketamine (1-2 mg/kg) were given to deepen anethesia. 　　Results:The procedures were fufiled steadily.Eighteen patients presented wit h different kinds of complications such as abdomen distention,vomitting,arrhymia and refractory anoxia to defferent degrees.One patient died,and the mortality w as 0.27%. 　　Conclusions:Anesthesia of children with grave heart diseases during cardiac catheterization and ventriculography is particular.It is important for the anest hesiologists to be familiar with the pathophysiology of heart diseases,and suppl y sufficiant oxygen,keep airway open and unobstructed,avoid stomach regurgitatio n and inhalation and maintain sufficient sedation during the procedure.Oxygen sh ould be given continuously and SpO2 monitored posoperatively to avoid any comp lication.