简介：Congenitalheartdisease(CHD)-associatedpulmonaryarterialhypertension(PAH)includesaheterogeneouspatientpopulationthatcanbecharacterizedbytheunderlyingcardiacmalformation.CHD-associatedPAHhasanestimatedprevalenceof5–10%inadultpatients,withanincreasingnumberofpatientssurvivingtoadulthoodbecauseofadvancesinthesurgicalmanagementandthedevelopmentofpulmonaryarterialhypertension(PAH)-targetedpharmacotherapy.Althoughlimiteddataexist,targetedPAHpharmacotherapyhasproventobebeneficialinpatientswithCHD-associatedPAH,withobservedimprovementinfunctionalclass,increaseinexercisecapacity,andimprovementinqualityoflifeandcardiopulmonaryhemodynamics.Additionally,therehasbeenincreasinginterestinthe“treat-to-close”strategy.PAH-targetedpharmacotherapymaybeusedtooptimizecardiopulmonaryhemodynamicssoastoimprovepatients’operabilityinrepairingthecardiacdefect.Althoughtherehavebeensignificantadvancesinthemanagementofthisdiseasestateinthepast2decades,mortalityremainshigh,andongoingclinicaltrialsareneededtobetterunderstandthetreat-to-closestrategy.

简介：AbstractPurpose:The aim of this study is to evaluate the application value of virtual surgical planning in the management of mandibular condylar fractures and to provide a reliable reference.Methods:This was a prospective randomized controlled study and recruited 50 patients requiring surgical treatment for their mandibular condylar fractures. The inclusion criteria were patients (1) diagnosed with a condylar fracture by two clinically experienced doctors and required surgical treatment; (2) have given consent for the surgical treatment; and (3) had no contraindications to the surgery. Patients were excluded from this study if: (1) they were diagnosed with a non-dislocated or only slightly dislocated condylar fracture; (2) the comminuted condylar fracture was too severe to be treated with internal reduction and fixation; or (3) patients could not complete follow-up for 3 months. There were 33 male and 17 female patients with 33 unilateral condylar fractures and 17 bilateral condylar fractures included. The 50 patients were randomly (random number) divided into control group (25 patients with 35 sides of condylar fractures) and experimental group (25 patients with 32 sides of condylar fractures). Virtual surgical planning was used in the experimental group, but only clinical experience was used in the control group. The patients were followed up for 1, 3, 6 and 12 months after operation. Variables including the rate of perfect reduction by radiological analysis, the average distance of deviation between preoperative and postoperative CT measurements using Geomagic software and postoperative clinical examinations (e.g., mouth opening, occlusion) were investigated for outcome measurement. SPSS 19 was adopted for data analysis.Results:The average operation time was 180.60 min in the experimental group and 223.2 min in the control group. One week postoperatively, CT images showed that the anatomic reduction rate was 90.63% (29/32) in the experimental group and 68.57% (24/35) in the control group, revealing significant difference (X2= 4.919, p = 0.027). Geomagic comparative analysis revealed that the average distance of deviation was also much smaller in the experimental group than that in the control group (0.639 mm vs. 0.995 mm; t = 3.824, p < 0.001).Conclusion:These findings suggest that virtual surgical planning can assist surgeons in surgical procedures, reduce operative time, and improve the anatomic reduction rate & accuracy, and thus of value in the diagnosis and treatment of condylar fractures.

简介：AbstractCholedochal cysts is a rare congenital cystic dilatation of common biliary duct. The most common prenatal diagnosed form type- I (85%-90%), consists of fusiform dilation of the common bile duct. There is communication between the bile duct and the cyst. In antenatal period diagnosis may be made by the presence of a cyst in the upper right side of the fetal abdomen on ultrasound. To differentiate it with other cystic lesions prenatal ultrasonography (USG), three-dimensional USG and magnetic resonance imaging may be helpful. By early diagnosis and prompt removal of cyst in postnatal period long term complications like development of biliary cirrhosis, portal hypertension, calculi formation or adenocarcinoma can be avoided in later life. The operative mortality is about 10%. We are reporting a case of antenatal diagnosis type I congenital choledochal cysts at 21 weeks, its follow up and successful postnatal management.

简介：AbstractPlacenta percreta with bladder bleeding can occur during gestation or postpartum, posing a great threat to both mother and fetus. But it is rare and lacks standard management strategies. We reported four cases suffering from bladder bleeding caused by placenta percreta even with hemorrhagic shock admitted between January 1st, 2011 and December 31th, 2020 in The First Affiliated Hospital of Zhengzhou University. Clinical information, including age, gravidity and parity, ultrasound and magnetic resonance imaging manifestations, onset gestational age, bladder bleeding volume, clinical manifestations under bleeding, diagnosis, hemostatic methods, hospital stay, treatment cost, and prognosis, are presented. Two cases had bladder bleeding during the second trimester, respectively on the 22+3 and 23+5 weeks. Pregnancy was terminated timely. The other two cases had bladder bleeding on the 2nd day post near-term cesarean section when activity. All the four cases achieved successful hemostasis following angiography and concomitant embolization for iliac vessels, and one of them received electrocoagulation hemostasis under cystoscopy, but failed. They all had favorable clinical outcomes and had no long-term complications. The neonatal outcome in the two cases that bladder hemorrhage occurred after near-term c-section was good. The newborns did not survive in two cases in which bladder hemorrhage occurred at the second trimester of pregnancy. Timely termination of pregnancy is recommended when such a condition develops during gestation. Diagnosis of bladder bleeding is relatively easy, for it is characterized by fast speed and large volume, with concomitant distension of the lower abdomen, blood discharge from the urethral orifice, or the indwelling catheter. Interventional embolization is an effective means to treat bladder bleeding caused by placenta percreta, while electrocoagulation hemostasis under cystoscopy must be applied with great caution. For the pregnant women with a high risk of placenta percreta, timely and accurate diagnosis should be achieved during the gestational age, and bladder bleeding should be concerned when placenta penetrates through the anterior wall of uterus.

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简介：AbstractObjective:Endoscopic approaches for sinus and skull base surgery are increasing in popularity. The objective of this narrative review is to characterize risk factors for internal carotid artery injury in endoscopic endonasal surgery (EES), highlight preventative measures, and illustrate key management principles.Data Sources:Comprehensive literature review.Methods:Relevant literature was reviewed using PubMed/MEDLINE.Results:Carotid artery injury in EES is rare, with most studies reporting an incidence below 0.1%. Anatomic aberrancies, wide dissection margins, as well as specific provider and hospital factors, may increase the risk of injury. Multidisciplinary teams, comprehensive preoperative imaging, patient risk assessment, and formal training in vascular emergencies may reduce the risk. Management protocols should emphasize proper visualization of the injury site, fluid replacement, rapid packing, angiography, and endovascular techniques to achieve hemostasis.Conclusions:While EES is a relatively safe procedure, carotid artery injury is a devastating complication that warrants full consideration in surgical planning. Important preventative measures include identifying patients with notable risk factors and obtaining preoperative imaging. Multidisciplinary teams and management protocols are ultimately necessary to reduce morbidity and mortality.

简介：AbstractImmunoglobulin G4-related disease (IgG4-RD) is a newly recognized chronic fibro-inflammatory autoimmune disease, and its recognition has been constantly increasing worldwide over the last few years. A correct and timely recognition, as well as appropriate intervention, is crucial for the treatment of IgG4-RD. For certain subtypes of IgG4-RD, organ-specific criteria are formulated to make the diagnosis more accurate. New biomarkers have emerged in the recent years to aid the disease diagnosis, its prognosis prediction, as well as therapy response monitoring. Although recurrence is very common in IgG4-RD, glucocorticoid is still the first-line treatment for the majority of patients. The factors that affect the likelihood of disease relapse are multifaceted. The selection strategy of various steroid-sparing agents is still being explored. Besides, when patients have special sites involvement leading to severe clinical conditions, surgical operation or interventional therapy should also be considered. An update on classification, diagnosis, and management of IgG4-RD is provided in the current study to fully elucidate the recommended clinical practice of this mysterious disease.

简介：AbstractBackground:Civilian gunshot wounds to the head refer to brain injury caused by projectiles such as gun projectiles and various fragments generated by explosives in a power launch or explosion. Gunshot wounds to the head are the deadliest of all gun injuries. According to literature statistics, the survival rate of patients with gunshot wounds to the head is only 9%. Due to the strict management of various types of firearms, they rarely occur, so the injury mechanism, injury and trauma analysis, clinical management, and surgical standards are almost entirely based on military experience, and there are few related reports, especially of the head, in which an individual suffered a fatal blow more than once in a short time. We report a case with a return to almost complete recovery despite the patient suffering two gunshot injuries to the head in a short period of time.Case presentations:We present a case of a 53-year-old man who suffered two gunshot injuries to the head under unknown circumstances. On initial presentation, the patient had a Glasgow Coma Scale score of 6, was unable to communicate, and had loss of consciousness. The first bullet penetrated the right frontal area and finally reached the right occipital lobe. When the patient reflexively shielded his head with his hand, the second bullet passed through the patient’s right palm bone, entered the right frontotemporal area, and came to rest deep in the lateral sulcus. The patient had a cerebral hernia when he was admitted to the hospital and immediately entered the operating room for rescue after a computed tomography scan. After two foreign body removals and skull repair, the patient recovered completely.Conclusions:Gunshot wounds to the head have a high mortality rate and usually require aggressive management. Evaluation of most gunshot injuries requires extremely fast imaging examination upon arrival at the hospital, followed by proactive treatment against infection, seizure, and increased intracranial pressure. Surgical intervention is usually necessary, and its key points include the timing, method, and scope of the operation.

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简介：AbstractPurpose:Gunshot wounds are the second leading cause of spinal cord injuries. Surgical intervention for gunshot injury to the spine carries a high rate of complications. There is a scarcity of data on civilian gunshot injuries to the spine in Pakistan. Approximately 60 cases over the last 10 years have been recoded, with unusual presentation and neurological recovery. Thus it is imperative to fill this gap in data, by reviewing cases of civilian gunshot injuries to spine presenting at a tertiary care hospital (Aga Khan University Hospital, Karachi).Methods:This is a retrospective cohort study. Patients of all ages who presented to the emergency department of Aga Khan University Hospital, with gunshot injuries to spine between January 2005 and December 2016 were included in the study. Data were collected on neurological status (American Spinal Injury Association score was used for the initial and follow-up neurological assessment), extent of cord transection, motor and sensory deficits. The patients were further grouped into those with cord transection, and those with fractures of the bony spine but an intact spinal cord. These patients were then followed and the outcomes were recorded.Results:A total of 40 patients were identified. The mean ± SD of patients age was (30.9 ± 9.5) years. Of the 40 patients with gunshot wounds, 31 had the medical imaging performed at the facility, and hence they were included in this categorization. The remaining 9 patients were excluded from this additional grouping. Thirteen patients were managed surgically and 27 patients underwent the conservative management. The mean ± SD of follow-up was (8.7 ± 7.2) months. In our study, the thoracic spine was the most commonly injured region in gunshot injuries. Of the 31 patients with medical imaging performed at our institute, 17 (54.8%) had cord transection, of whom 8 (47%) ultimately developed paraplegia.Conclusion:The prognosis of gunshot injuries to the spine can be varied depending on whether the spinal cord is intact or transected. This will help healthcare providers to plan the further management of the patient and counsel them accordingly.

简介：Advancedgastriccancer(GC)hasbeenrecognizedaslethaldiseasewhenperitonealmetastases(PM)occurred.ThereisnostandardtreatmentforadvancedGCwithPM.Until1980s,thetherapeuticarenaforthesepatientshadremainedstagnant,withnotherapeuticapproachhavingshownasurvivalgaininGCwithPM.However,cytoreductivesurgery(CRS)withperitonectomyproceduresandintraperitonealchemotherapy(IPC)promisingnewcombinedtherapeuticapproachtoachievediseasecontrolforGCwithPM.TherecentpublicationschangedtheGCwithPMtreatmentlandscapebyprovidinganevidencethatCRSandIPCledtoprolongationinoverallsurvival(OS).ThisreviewwillprovideanoverviewoftheevolvingroleofCRSandIPCinthemanagementofadvancedGCwithPMinthecurrentera.

简介：AbstractPatients with aspirin exacerbated respiratory disease (AERD) experience a severe and recalcitrant form of chronic rhinosinusitis with nasal polyposis (CRSwNP) and asthma, which are exacerbated by aspirin/NSAID ingestion. As compared with aspirin-tolerant CRSwNP, patients with AERD experience more severe olfactory dysfunction, which is one of the key contributors to the observed decrease in quality of life (QOL) in this disease. The objective of this paper is to review the published olfactory outcomes observed with various treatment modalities.

简介：客观Tracheobronchial损害被定义为从延长直到支气管的部门的环状的软骨的水平包含气管或支气管的损害。我们在场有大多数tracheobronchial损害的一个盒子系列发现了在渗透损伤以后被支撑。回顾的评论在统帅中亚细亚大学被执行的方法，喀拉蚩，巴基斯坦。从2004年1月到2009年12月，有胸的损伤的168个病人被对待，谁15被认出有主要tracheobronchial和肺的损害。结果平均年龄是与大多数是男性的病人(14:1)一起的31年。在他们之中，11个病人让了作为损害的主要原因渗透损伤，有的3个病人从道路交通事故弄钝损伤，仅仅1个病人联合了损伤(钝、渗透的损伤)。八个病人基于放射学的调查结果被诊断。所有病人通过手术被对待。叶切除术是在7个病人执行的最普通的干预。死亡率是7%(1个病人)。当5与残疾幸存时，大多数病人没有sequelae(10个病人)幸存。我们发现渗透损伤是在我们的系列的损害的领先的原因。损害的严厉取决于引起损伤的武器。在我们的系列的病人有多重损害并且要求了外科的管理。结论Tracheobronchial损害是稀罕的但是潜在地威胁的生活。他们要求快诊断和管理。自从没有目前可得到的专业化诊断形式，诊断趋于困难。

简介：AbstractThe gold standard of cancer diagnosis has long been based on histological characteristics. With the rapid advancement of genetic medicine, such standard algorithm of diagnostic approach is facing a challenge. The genetic findings have been changed from being a "supporting character" into the role of a "main character" . More and more disease diagnosis and classification has to be defined by genetic basis. In this article, we focus on the challenges in the field of pediatric oncology. We cited 2 scenarios where genetic information plays a pivotal role in identifying the underlying pathology. The first scenario is that same genetic mutation can lead to variable clinical phenotypes, this includes EWSR1-PATZ1 fusion related neoplasms; BCOR neoplasms; and GATA-2 deficiency related immunodeficiency and myelodysplastic syndrome. Another scenario is relatively more common that is the same clinical and histopathological phenotype with different underlying genotypes. The genotypes actually impact on the treatment response and outcome. We used medulloblastoma as an example. In fact, we can also find similar scenario in many pediatric cancers such as Ewing sarcoma, ependymoma, etc. The essence of this article is to remind clinicians of the rapid development in genetic medicine and it has been reshaping the landscape of the modern disease classification and therapeutic approach. In the near future, it may even lead to a paradigm shift in our disease diagnostic algorithm.

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简介：Despitethedevelopmentofnoninvasivetoolsasechocardiography,right-sidedheartcatheterizationremainsanintegralpartofthecardiovascularevaluation.Inthelastdecade,betterunderstandingofthehemodynamicprocessbeforeheartfailuredecompensationledtoimprovementofoutpatientstrategiestopreventit.Advancesinimplantablewirelesstechnologynowallowfrequentanddirectmeasurementofintracardiacfillingpressures,whichcanbemonitoredbyhealthcareproviderstohelptailortherapytoreducefillingpressuresandhospitalreadmissionrates.

简介：INTRODUCTIONWiththedevelopmentofeconomyandimprovementoflifequality,theincidencesofhypertension,hyper-cholesterolemia,diabetes,obesityandsmokinghavebeenincreasedinChina,whichhasledtoasignificantincreaseinthemorbidityandmortalityofcoronaryarterydisease(CAD)~1.SinceitwasintroducedintoChinain1984,coronaryintervention(PCI)hasdevelopedrapidlyandhasbecomethemajortreatmentofCADbecauseofitsuniquecharacteristicsofminimalinvasiveand

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