Congenital Bronchial Stenosis Presenting as Neonatal Respiratory Distress: A Case Report

BackgroundNeonatal respiratory distress has a broad differential that includes cardiac, pulmonary, anatomic, and infectious etiologies. Congenital stenotic lesions of the trachea and bronchus are rare and can occur anywhere along the tracheobronchial tree. Patients with tracheobronchial stenosis typically present in the neonatal period with respiratory distress.Case ReportWe present a case of a 10-day-old term female who presented to the emergency department (ED) with tachypnea and increased work of breathing. She was found to have congenital bronchial stenosis of her right mainstem bronchus. She was stabilized in the ED and remained in the neonatal intensive care unit until successful slide tracheoplasty was performed.Why Should an Emergency Physician Be Aware of This?Congenital bronchial stenosis is a rare etiology of respiratory distress in a neonate. Anatomic lower airway abnormalities are an important cause of neonatal tachypnea and must remain on the differential. In addition to respiratory stabilization with noninvasive or invasive support, evaluation should be directed at determining the location and anatomic characteristics of the area of stenosis.     

A multidisciplinary approach for prenatal diagnosis of FRASER SYNDROME-report of a novel variant in FRAS1

ObjectiveWith this case report, we would like to highlight the importance of a multidisciplinary approach and atypical findings of congenital high airway obstruction sequence (CHAOS), anhydramnios, and renal dysgenesis in the prenatal diagnosis of Fraser syndrome (FS).Case reportA 25-year-old primigravida at 19 weeks of routine anomaly scan revealed abnormal sonographic findings such as fetal bilateral dysplastic small kidneys and gross oligohydramnios. The further detailed evaluation revealed that both fetal lungs were hyperechogenic with prominent (dilated) trachea and bronchi suggestive of CHAOS. Based on these findings, a diagnosis of FS was suspected. The couple was counseled and the pregnancy was terminated. The postmortem evaluation and novel homozygous variant in the FRAS1 gene confirmed the diagnosis of FS.ConclusionThe diagnosis and counseling of the patient were supported by a well-coordinated, multidisciplinary approach involving an obstetrician, a fetal medicine specialist, a medical geneticist, and a fetal pathologist.     

Peritonitis esclerosante encapsulante: hallazgos en imágenes de una entidad poco frecuente

ObjectivesTo describe the most characteristic imaging findings for sclerosing encapsulating peritonitis, with an emphasis on the computed tomography findings.ConclusionThe incidence of sclerosing encapsulating peritonitis is low. The pathophysiology of this condition is unclear. Two types are recognized: idiopathic and secondary; the secondary type is generally a complication of peritoneal dialysis. Its nonspecific clinical presentation and the absence of blood markers mean that sclerosing encapsulating peritonitis is usually diagnosed late. Thus, it is important to know the imaging signs; these include thickening and calcification of the peritoneum and dilation of bowel loops with thickening and calcification of bowel walls, whether in isolation or in association with loculated ascites. Although ultrasonography allows the complexity of the collections to be evaluated, computed tomography is the most useful technique for the general assessment of the signs mentioned above.     

杜纪鸣治疗冠状动脉粥样硬化性心脏病经验

杜纪鸣老师认为,冠状动脉粥样硬化性心脏病(简称冠心病)的发生、发展,与多种因素紧密相关,其常见发病原因有寒邪内侵、饮食不节、情志失调、体虚劳倦等,与人体的代谢及生理功能失调关系紧密。心脉痹阻为本病主要病机,一方面因虚致实,另一方面因实致虚。气虚、血瘀两种病理产物贯穿本病始终。临证时,杜老师遵循益气活血、化瘀通脉止痛治疗大法,把辨脏腑、辨主症、辨兼症等辨证方法融汇其中,同时不忘借鉴现代中药药理学研究成果完善方药。杜老师提出,对有易感因素的中青年易患人群,一定要早预防,通过早期调补脾肾、调理内分泌、代谢紊乱等,能有效预防疾病的发生,延缓疾病的发展等。     

六君子汤加味联合奥利司他治疗脾虚湿阻证单纯性肥胖症临床研究

目的:观察六君子汤加味联合奥利司他治疗脾虚湿阻证单纯性肥胖症的临床疗效及对血清瘦素(leptin)和脂联素(ADPN)水平的调节作用。方法:将80例患者随机分为对照组40例和观察组40例,对照组口服奥利司他治疗,观察组在对照组的基础上加用六君子汤加味治疗,2组均连续治疗12周;观察比较2组脾虚湿阻证证候评分、肥胖指征、临床疗效及血清leptin、ADPN水平。结果:总有效率观察组为95.00%,对照组为75.00%,2组比较,差异有统计学意义(P<0.05)。治疗后,2组患者浮肿、疲乏无力、肢体困重、纳差、腹满等脾虚湿阻证证候评分较治疗前明显下降(P<0.05),且观察组上述各项评分均低于对照组(P<0.05)。治疗后,2组患者体质量、体质量指数(BMI)、体脂含量(FAT)值均较治疗前明显下降(P<0.05),且观察组上述各指标改善较对照组更显著(P<0.05)。治疗后,2组血清leptin水平均较治疗前降低(P<0.05),ADPN水平升高(P<0.05);且观察组两项指标改善较对照组更显著(P<0.05)。结论:六君子汤加味联合联合奥利司他治疗脾虚湿阻证单纯性肥胖症疗效显著,调节leptin、ADPN水平可能是其作用机制之一。     

Predicting the outcome of transcatheter mitral valve implantation using image-based computational models

BackgroundThe appropriate placement and size selection of mitral prostheses in transcatheter mitral valve implantation (TMVI) is critical, as encroachment on the left ventricular outflow tract (LVOT) may lead to flow obstruction. Recent advances in computed tomography (CT) can be employed for pre-procedural planning of mitral prosthetic valve placement. This study aims to develop patient-specific computational fluid dynamics models of the left ventricle (LV) in the presence of a mitral valve prosthesis to investigate blood flow and LVOT pressure gradient during systole.MethodsPatient-specific computational fluid dynamics simulations of TMVI with varied cardiac anatomy and insertion angles were performed (n = 30). Wide-volume full cycle cardiovascular CT images prior to TMVI were used as source anatomical data (n = 6 patients). Blood movement was governed by Navier-Stokes equations and the LV endocardial wall deformation was derived from each patient's CT images.ResultsThe computed pressure gradients in the presence of the mitral prosthesis compared well with clinically measured gradients. Analysis of the effects of prosthetic valve angulation, aorto-mitral annular angle, ejection fraction, LV size and new LVOT area (neo-LVOT) after TMVI in silico revealed that the neo-LVOT area (p < 0.001) was the most significant factor affecting LVOT pressure gradient. Angulation of the mitral valve can substantially mitigate LVOT gradient.ConclusionsComputational fluid dynamics simulation is a promising method to aid in pre-TMVI planning and understanding the factors underlying LVOT obstruction.