BACKGROUND & AIMS: PURPOSE:To determine the clinical importance and relative value of reinterpreting brain CT imaging studies by subspecialty experts regarding changes in clinical management. METHODS:Computerized records were queried at two institutions during the years 2002-2003 for both primary interpretation by board-certified nonneuroradiologists and secondary interpretation by three neuroradiologists. A total of 1,081 cases were reviewed. Each case was initially interpreted as an emergent or urgent study. The reinterpreted studies were scored as concordant or discordant by the subspecialty experts. The discordant studies were then categorized as a "major discordance" if there was a change in clinical management, or as a "minor discordance" if there was no impact or change in clinical management. RESULTS:Of the 1,081 studies reviewed, 14 studies were identified as discordant (1.3%). Of those discordant studies, four were categorized as major discrepancies necessitating a change in clinical management (0.4 %). Ten were categorized as minor discrepancies (0.9%). There were no permanent adverse outcomes with respect to morbidity and mortality as a result of any discrepancy. CONCLUSION:The vast majority of interpreted head CT cases read by board-certified general radiologists do not result in discordant interpretations as verified by subspecialty experts. Discordant interpretations did not result in changes in clinical management in most cases. Double reading of head CTs by subspecialty experts appears to be an inefficient method of substantially improving imaging health quality outcomes.

背景与目标：

BACKGROUND & AIMS: BACKGROUND:Several case reports have raised serious concerns about the safety of shoulder surgery in the beach-chair position, related to global cerebral hypoperfusion. We summarize our experiences with 15,014 cases of shoulder arthroscopy over an 11-year period. Our primary aim was to evaluate the incidence of intraoperative or immediate postoperative neurologic events and secondarily to relate other perioperative complications. METHODS:We searched our online deidentified departmental quality improvement and patient safety database for adverse outcomes associated with arthroscopic shoulder surgery performed in the beach-chair position for the 11-year period between April 2001 and November 2011, as well as our hospital-system database and a statewide database. This was compared with the total number of such cases, available from our department billing database. RESULTS:The total rate of adverse events was 0.37%. Neurologic abnormalities suggestive of acute cerebral ischemia or hemorrhage did not occur in the immediate perioperative period. One new neurologic deficit was reported, secondary to ischemic stroke, which occurred 24 hours after the surgery. The most frequent complications detected were unplanned return to care (0.067%), local anesthetic systemic toxicity (0.053%), and airway compromise requiring unplanned intubation (0.033%). Complications were infrequent and did not vary in incidence over the course of the study. CONCLUSIONS:This retrospective study suggests that intraoperative or immediate postoperative stroke is rare when surgery is conducted in beach-chair position in conjunction with regional anesthesia, propofol sedation, and spontaneous respiration via natural airway.

背景与目标：

BACKGROUND & AIMS: PURPOSE:To evaluate the indications and outcomes of amniotic membrane transplantation (AMT) performed within the first 2 weeks of presentation in the management of patients with acute Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN). METHODS:A retrospective chart review from January 1998 to May 2011 identified 128 SJS/TEN patients admitted to Loyola University Medical Center Burn intensive care unit. The degree of initial ocular surface inflammation was graded as mild, moderate, or severe within the first 2 weeks of admission. Patients were managed either medically or with amniotic membrane (AM). Outcomes were graded as good [best-corrected visual acuity (BCVA)>20/40], fair (BCVA 20/40 to 20/200 or with ocular surface discomfort, requiring contact lens or reconstructive surgeries), or poor (BCVA<20/200). RESULTS:Of the 182 eyes (91 patients) with documented inpatient eye examinations, 108 eyes (59.4%) had mild or no initial ocular involvement, 37 eyes (20.3%) had moderate, and 37 eyes (20.3%) had severe inflammation. Of the 29 patients (58 eyes) with greater than 1 month of follow-up, 17 patients (33 eyes) were treated with medical management and 13 patients (25 eyes) were treated with early AM. One of the 23 eyes with moderate or severe presentation treated with early AMT (4.3%) resulted in a poor outcome within 3 months compared with 8 of 23 eyes (34.8%) that were medically managed (P=0.022). CONCLUSIONS:We present the first case-control study of the use of AM in the management of acute SJS/TEN. Early use of AMT prevents severe vision loss in SJS/TEN patients with initial moderate or severe ocular surface inflammation.

背景与目标：

BACKGROUND & AIMS: INTRODUCTION:The goal of burn care is that 'the quality of the outcome must be worth the pain of survival'. More research is needed to understand how best to deliver care for patients with burns to achieve this aim. Loss of independence, function as well as loss of income for patients with burns and carers cause a significant burden at both individual and societal levels. Much is being done to advance knowledge in the clinical care field; however, there has been a paucity of research exploring psychosocial outcomes. This paper describes the study background and methods, as implemented in an Australian cohort study of psychosocial outcomes after major burn injuries. METHODS AND ANALYSIS:In this inception cohort study, a target sample of 230 participants, aged 18 years or over, admitted to a single statewide burns centre with a burn injury are identified by hospital staff for inclusion. Baseline survey data are collected either in person or by telephone within 28 days of the injury and participants then followed up with telephone interviews at 3, 6 and 12 months postburn. Injury and burns treatment information is collected from medical records. Social support is measured as a predictor variable using the Multidimensional Scale of Perceived Social Support. Outcome data are collected via standardised measures in the domains of Quality of Life (SF-12, EQ-5D, BSHS-B), depression (PHQ-9), post-traumatic stress disorder (PCL-C, PAS), community integration (CIQ-R) and Quality-Adjusted Life Years (EQ-5D). Additional survey questions measure life satisfaction, return to work and public services utilisation at 12 months postinjury. Data analysis methods will include analysis of variance, Pearson correlation and hierarchical multiple regression analyses. ETHICS AND DISSEMINATION:Hospital-based and University of Queensland Human Research Ethics Committees have approved the protocol. Results from the study will be disseminated at national and international conferences, in peer-reviewed journals and in a doctoral thesis. TRIAL REGISTRATION NUMBER:Australia New Zealand Clinical Trials Registry (ACTRN12616000828426). Retrospectively registered on 23 June 2016; pre-results.

背景与目标：

BACKGROUND & AIMS: OBJECTIVES:We evaluated the functional and oncological outcomes of transoral laser microsurgery (TLM) in patients with previously untreated supraglottic carcinoma compared with the outcomes in salvage cases after radiation-based treatment. METHODS:We conducted a retrospective case-control study at a single academic tertiary care institution. The functional outcomes were stratified by prior irradiation and were assessed at baseline, less than 1 week after operation, and at last follow-up. RESULTS:Five patients underwent TLM for previously untreated disease, and 5 previously irradiated patients underwent salvage TLM for local failure. No patient required tracheostomy. There was no local recurrence after TLM as primary therapy, and none of those patients required radiotherapy. One salvage patient developed local recurrence. The duration of feeding tube dependence (p = 0.049) and the rates of chronic aspiration (more than 1 month after operation; p = 0.048) were significantly higher in the salvage TLM cases than in the previously untreated cases. The median scores on the PSS-HN Understandability of Speech were 75 ("usually understandable") in the salvage group and 100 ("always understandable") in the previously untreated group. CONCLUSIONS:Both local control and function were better in the previously untreated patients than in the salvage patients. Our findings provide support for the use of TLM as a primary treatment modality for selected supraglottic carcinomas, but also suggest a potential for functional recovery in both previously untreated and salvage cases.

背景与目标：

BACKGROUND & AIMS: OBJECTIVES:Monochorionic (MC) twins are at increased risk of early fetal loss secondary to vascular complications such as twin-twin transfusion syndrome (TTTS). This study compared the early perinatal loss rates between MC and dichorionic (DC) twins in an era of invasive treatment for TTTS. METHODS:This was a retrospective study of all twin pregnancies of known chorionicity from a large regional cohort of nine hospitals over a 10-year period. Ultrasound data were matched to hospital delivery records and to a mandatory national register of pregnancy losses. Prospective risk of pregnancy loss from 14 to 24 weeks' gestation was calculated and the survival trend of MC and DC twins was analyzed using Kaplan-Meier survival analysis. RESULTS:The analysis included 3117 twin pregnancies (605 MC and 2512 DC). The total risk of early pregnancy loss (miscarriage and neonatal death) before 24 weeks was significantly higher in MC twins (60.3 per 1000 fetuses) than in DC twins (6.6 per 1000 fetuses), with a relative risk of 9.18 (95% CI, 6.0-13.9). Survival analysis showed a significant difference in overall and early mortality between MC and DC twins (log-rank test, P < 0.0001), while no difference was noted after 24 weeks' gestation (log-rank test, P = 0.08). CONCLUSIONS:Early pregnancy loss is significantly more common in MC than in DC twins, but no difference in the prospective risk of mortality between MC and DC twins is evident after 24 weeks' gestation. The observed early mortality rate has almost halved in comparison with previous studies in the published literature. Early detection and prompt treatment of complications in MC twins are likely to have contributed to this improvement in outcome.

背景与目标：

BACKGROUND & AIMS: :Fetal growth, birth weight specific mortality rates and effect of sick leave or hospitalization on the fetal growth were investigated in a material of 476 twin pregnancies managed at University Central Hospital of Turku in years 1970-81. Birth weights of twin babies at any gestational age were slightly but not significantly higher than in earlier materials. When compared to growth curve of singleton fetuses, the growth rate of both twins is equal to singletons up to 30th week of pregnancy, being thereafter slower than in singleton pregnancies. Although duration of sick leave and hospitalization increased considerably during the study period, no change in the duration of pregnancy nor in the weight of twin babies occurred. Instead perinatal mortality decreased from 101/per thousand to 36.2/per thousand. Birth weight specific mortality rates did not differ from those in singleton fetuses.

背景与目标： : 在图尔库大学中央医院1970-81年管理的476例双胎妊娠材料中，研究了胎儿生长，出生体重特定死亡率以及病假或住院对胎儿生长的影响。在任何胎龄的双胞胎婴儿的出生体重均略高于但不显着高于早期材料。与单胎胎儿的生长曲线相比，两个双胞胎的生长速度等于怀孕第30周的单胎，此后比单胎妊娠慢。尽管在研究期间病假和住院时间大大增加，但怀孕时间和双胞胎婴儿的体重没有变化。相反，围产期死亡率从101/每千下降到36.2/每千。出生体重特定死亡率与单胎胎儿没有差异。

BACKGROUND & AIMS: :We performed a retrospective cohort analysis of pregnancies among women with moderate to complex congenital heart disease or pulmonary hypertension over a 12-year period, resulting in a cohort of 107 cases in 65 women. Neuraxial analgesia or anaesthesia was provided in 84%, 89% and 95% of spontaneous vaginal, operative vaginal and caesarean deliveries, respectively. The caesarean delivery rate was 43% compared to our institution average of 27% over the same period (p = 0.02), and 38% had operative vaginal deliveries compared to a 10.5% institution rate (p < 0.01). Invasive monitoring was used in 28% of all deliveries. There were one maternal and two neonatal deaths. This study provides detailed anaesthetic and peripartum management of women with congenital heart disease, a patient population in whom evidence-based practice and data are largely lacking. We observed a predominance of neuraxial anaesthetic techniques, increased caesarean and operative delivery rates, and favourable maternal and neonatal outcomes. Multicentre studies and registries to compare anaesthetic and obstetric management strategies further and delineate risk factors for adverse outcomes are required.

背景与目标： : 我们对12年的中度至复杂先天性心脏病或肺动脉高压妇女的妊娠进行了回顾性队列分析，结果在65名妇女中进行了107例病例的队列研究。分别在84%，89% 和95% 自发阴道，手术阴道和剖腹产时提供了神经镇痛或麻醉。与同期的机构平均27% 相比，剖腹产率43% (p = 0.02)，与10.5% 机构相比，38% 进行了阴道分娩 (p <0.01)。侵入性监测用于28% 所有分娩。有1名产妇和2名新生儿死亡。这项研究提供了先天性心脏病妇女的详细麻醉和围产期管理，先天性心脏病是一个缺乏循证实践和数据的患者人群。我们观察到神经麻醉技术占主导地位，剖腹产和手术分娩率提高，孕产妇和新生儿结局良好。需要进行多中心研究和注册，以进一步比较麻醉和产科管理策略，并描述不良结局的风险因素。

BACKGROUND & AIMS: OBJECTIVE:To identify early clinical factors that are correlated with death or severe disability in paediatric patients who have sustained an injury by hanging or strangulation. METHODS:A retrospective review of all patient records from January 1, 1997, to September 30, 2007, was conducted. Patient records were identified by International Classification of Diseases and Related Health Problems, Tenth Revision, Canada diagnostic codes for asphyxia, strangulation, hypoxic-ischemic encephalopathy, hanging, hypoxemia, hypoxia or anoxia. RESULTS:A total of 109 records were identified. Of these, 41 met the inclusion criteria for the study. Of 19 (46%) children who were pulse-less and received cardiopulmonary resuscitation, 16 died and the survivors were severely disabled. Of the 22 (54%) children who were found with a pulse, 18 made a full recovery. CONCLUSIONS:Children who are pulseless at discovery for hanging injuries are at high risk of death or severe disability. Early clinical and neurophysiological indicators should be applied systematically to best guide clinicians and parents in their decision making.

背景与目标：

BACKGROUND & AIMS: :We carried out the first meta-analysis comparing the technical success and clinical outcomes of endoscopic ultrasound-guided drainage (EUD) and conventional transmural drainage (CTD) for pancreatic pseudocysts. We searched PubMed, Embase, Scopus, and the Cochrane library to identify relevant prospective trials. The technical success rate, short-term (4-6 weeks) success, and long-term (at 6 months) success in symptoms and the radiologic resolution of pseudocysts, complication rates, and death rates were compared. Two eligible randomized-controlled trials and two prospective studies including 229 patients were retrieved. The technical success rate was significantly higher for EUD than for CTD [risk ratio (RR)=12.38, 95% confidence interval (CI): 1.39-110.22]. When CTD failed because of the nonbulging nature of pseudocysts, a crossover was carried out to EUD (n=18), which was successfully performed in all these cases. All patients with portal hypertension and bleeding tendency were subjected to EUD to avoid severe complications. EUD was not superior to CTD in terms of short-term success (RR=1.03, 95% CI: 0.95-1.11) or long-term success (RR=0.98, 95% CI: 0.76-1.25). The overall complications were similar in both groups (RR=0.98, 95% CI: 0.52-1.86). The most common complications were bleeding and infection. There were two deaths from bleeding after CTD. The short-term and long-term treatment success of both methods is comparable only if proper drainage modality is selected in specific clinical situations. For bulging pseudocysts, either EUD or CTD can be selected whereas EUD is the treatment of choice for nonbulging pseudocysts, portal hypertension, or coagulopathy.

背景与目标： : 我们进行了首次荟萃分析，比较了内镜超声引导引流 (EUD) 和常规透壁引流 (CTD) 治疗胰腺假性囊肿的技术成功和临床结果。我们搜索了PubMed，Embase，Scopus和Cochrane图书馆，以确定相关的前瞻性试验。比较了症状的技术成功率，短期 (4-6周) 成功率和长期 (6个月) 成功率以及假性囊肿的放射学分辨率，并发症发生率和死亡率。检索了两项符合条件的随机对照试验和两项包括229名患者的前瞻性研究。EUD的技术成功率明显高于CTD [风险比 (RR)= 12.38，95% 置信区间 (CI): 1.39-110.22]。当由于假性囊肿的非膨出性而导致CTD失败时，对EUD进行了交叉 (n = 18)，在所有这些情况下均成功进行了交叉。所有有门静脉高压症和出血倾向的患者均接受EUD治疗，以避免严重的并发症。就短期成功率 (RR = 1.03，95% CI: 0.95-1.11) 或长期成功率 (RR = 0.98，95% CI: 0.76-1.25) 而言，EUD并不优于CTD。两组的总体并发症相似 (RR = 0.98，95% CI: 0.52-1.86)。最常见的并发症是出血和感染。CTD后有2例死于出血。只有在特定的临床情况下选择适当的引流方式，两种方法的短期和长期治疗成功才具有可比性。对于膨出性假性囊肿，可以选择EUD或CTD，而EUD是治疗非膨出性假性囊肿，门静脉高压症或凝血病的首选方法。

BACKGROUND & AIMS: OBJECTIVE:Of pediatric patients with Crohn disease, 20% to 30% undergo surgery within 10 years. Although disease relapses and reoperations are common, long-term functional outcomes and quality of life (QoL) are unclear. METHODS:In 2010, we reviewed the hospital records of all pediatric patients with CD who had undergone intestinal resections during childhood in 2 major tertiary care hospitals between 1985 and 2008 and mailed out questionnaires that asked about health outcomes and QoL. We compared the QoL of the patients and a group of matched controls randomly chosen from the Population Register Centre. RESULTS:In total, 36 children had undergone bowel resection a median of 10 years earlier and had at least 2 years of follow-up. Disease activation (verified at endoscopy) requiring medical or surgical treatment occurred in 94% (median 1.8 years after primary resection). At least 1 surgical complication occurred in 77%, and 54% underwent re-resection. The patients reported a median stool frequency of 3 stools during the day and zero at night, with 33% being totally continent. Overall, 96% were completely or moderately satisfied with the outcome of the surgery. The QoL was comparable between the patients and controls, but school or work absences diminished the QoL of the patients. CONCLUSIONS:Surgery for pediatric-onset CD is risky even under expert care. Disease relapses and bowel re-resections are common during the first decade after primary surgery. In the long term, however, bowel function is acceptable and the QoL is comparable between patients and their peers.

背景与目标：

BACKGROUND & AIMS: OBJECTIVES:To estimate the agreement between prenatal ultrasonography observations at 16 - 21 gestational weeks and fetal autopsy findings in pregnancies terminated because of fetal anomalies. STUDY DESIGN:This 4½ year retrospective study includes consecutive fetuses that were terminated because of fetal malformation and/or chromosomal anomaly diagnosed in the second trimester. Only fetuses that had undergone fetal anatomy scanning by an obstetrician trained in fetal ultrasound before the termination and with available fetal autopsy reports were included. The cases were identified through the malformation registry database of our ultrasound unit. The sensitivity and specificity of ultrasound were calculated per organ system. When estimating the agreement between ultrasound results and autopsy findings, the cases were allocated to one of four categories according to the degree of concordance between ultrasound and autopsy findings: full agreement, near match, partial agreement and unconfirmed ultrasound findings. RESULTS:71 of 95 pregnancy terminations due to fetal malformations met the inclusion criteria and constitute our study population. The sensitivity of ultrasonography with regard to malformations in the brain and spine was 100 % (27/27) and with regard to malformations in the internal organ system (including malformations in the urogenital and gastrointestinal systems and in the abdominal wall and diaphragm) was 91 % (30/33). The corresponding figures for malformations in the cardiovascular and skeletal organ systems were 63 % (17/27) and 71 % (25/35), respectively. The specificity was lowest for malformations in the central nervous system and internal organ system (33/38, 87 % and 39/44, 89 %, respectively). There was full agreement between the ultrasound and autopsy findings in 44 % (31/71) of all cases and a near match in 46 % (33/71) of cases. In almost 10 % (7/71) of the pregnancies, the ultrasound findings were only partially confirmed or not confirmed by autopsy. In one case the discrepancy between the ultrasound and autopsy findings suggests that the pregnant woman might have decided to terminate the pregnancy on the basis of incorrect interpretation of ultrasound findings. CONCLUSION:Even though the agreement between ultrasound and autopsy findings was acceptable from a clinical point of view, agreement with regard to the detailed description of malformations was far from perfect. The detection rates were suboptimal for the cardiovascular and skeletal organ systems. :ZIEL:: Bewertung, inwieweit bei Schwangerschaftsabbrüchen aufgrund fetaler Anomalien die Befunde der pränatalen Ultraschalluntersuchung in der 16.–21. SSW mit dem Autopsiebefund des Feten übereinstimmen. METHODEN:Diese 4½ jährige retrospektive Studie schließt fortlaufend Feten ein, bei denen im 2. Trimenon ein Schwangerschaftsabbruch (TOP) aufgrund fetaler Fehlbildungen und/oder aufgrund von diagnostizierten Chromosomenanomalien erfolgt ist. Es wurden nur Feten einbezogen, bei denen vor dem Abbruch eine anatomische Untersuchung durch einen in der Fetalsonografie geübten Geburtshelfer durchgeführt wurde und bei denen fetale Autopsiebefunde vorlagen. Die Fälle wurden durch die Datenbank unserer Sonografieabteilung zur „Registrierung von Fehlbildungen“ ermittelt. Die Sensitivität und Spezifität der Sonografie wurde für jedes Organsystem berechnet. Zur Abschätzung der Übereinstimmung von Ultraschall- und Autopsiebefunden wurden die Fälle einer der vier Kategorien zugewiesen, je nach Grad der Übereinstimmung der Befunde: Solche mit völliger, sich fast entsprechender und nur teilweiser Übereinstimmung sowie nicht bestätigte Ultraschallbefunde. ERGEBNISSE:Die Einschlusskriterien erfüllten 71 von 95 Schwangerschaftsabbrüchen aufgrund fetaler Anomalien und bildeten somit unsere Studienpopulation. Die Sensitivität der Sonografie in Bezug auf Malformationen des Gehirns und Wirbelsäule betrug 100 % (27/27) und in Bezug auf Anomalien der inneren Organe (einschließlich der Fehlbildungen des Urogenital- und Magen-Darm-Systems, der Bauchdecke und des Diaphragmas) 91 % (31/33). Die entsprechende Rate für Anomalien des Herz-Kreislauf-Systems betrug 63 % (17/27) und für die des Skelettsystems 71 % (25/35). Die niedrigste Spezifität wurde für Malformationen des zentralen Nervensystems (33/38, 87 %) und der inneren Organe (39/44, 89 %) ermittelt. Eine völlige Übereinstimmung von Ultraschall- und Autopsiebefunden wurde in 44 % (31/71) und eine sich fast entsprechende Übereinstimmung in 46 % (33/71) aller Fälle gefunden. Bei beinahe 10 % (7/71) der Schwangerschaften konnten die Ultraschallbefunde nur teilweise beziehungsweise gar nicht durch die Autopsie bestätigt werden. In einem Fall mit diskrepanten Ultraschall- und Autopsiebefunden kann angenommen werden, dass die Schwangere die Entscheidung für einen Abbruch vermutlich aufgrund der fehlerhaften Interpretation des Ultraschallbefundes fällte. SCHLUSSFOLGERUNG:Obwohl die Übereinstimmung von Ultraschall- und Autopsiebefunden vom klinischen Standpunkt betrachtet akzeptierbar schien, war sie bezüglich der genauen Beschreibung der Fehlbildungen bei weitem nicht perfekt. Die Trefferquoten beim Herz-Kreislauf- und Skelettsystem waren suboptimal.

背景与目标：

BACKGROUND & AIMS: BACKGROUND:Severity of illness scores are helpful in predicting mortality; however, no standardized scoring system has been validated in patients with Staphylococcus aureus bacteremia (SAB). The modified Rapid Emergency Medicine Score (REMS), the CURB-65 (confusion, urea, respiratory rate, blood pressure and age 65) and the Charlson weighted index of comorbidity (CWIC) were compared in predicting outcomes at the onset of SAB. METHODS:All adult inpatients with SAB from July 15, 2008, to December 31, 2009, were prospectively assessed. The 3 scoring systems were applied: REMS, CURB-65 and CWIC. The end points were attributable and overall mortality. RESULTS:A total of 241 patients with SAB were reviewed during the study period. The all-cause mortality rate was 22.8% and attributable mortality 14.1%. Patients who died had higher mean CURB-65 score and REMS than those who lived, whereas the difference in the CWIC score was not significant. Two logistic regression models based on CURB-65 score or REMS, after controlling for CWIC, revealed that both scores were independent predictors of mortality, with an odds ratio of 3.38 (P < 0.0001) and 1.45 (P < 0.0001) for CURB-65 and REMS, respectively. Receiver operating characteristic analysis revealed that a cutoff point of 3.0 (CURB-65) and 6.0 (REMS) provided the highest sensitivity and specificity. The area under the curves for all-cause mortality were 0.832 and 0.806, and for attributable mortality 0.845 and 0.819, for CURB-65 and REMS, respectively. CONCLUSIONS:REMS and CURB-65 scores outperformed CWIC as predictors of mortality in SAB and may be effective in predicting the severity of illness at the onset of bacteremia.

背景与目标：

BACKGROUND & AIMS: OBJECTIVE:The aim was to investigate birth characteristics, obstetric and neonatal outcomes of the first childbirth in women with Marfan syndrome by use of Swedish national registers since pregnancy-related outcomes in women with Marfan syndrome have only been sparsely investigated. STUDY DESIGN:In this national population-based cohort study and matched case-control study of Swedish women born 1973-1993, women with Marfan syndrome (n=273) were compared to women without the condition (n=1 017 265). The study population was followed until 2013. A total of 364 553 mother-firstborn-offspring pairs were analyzed. Sixty-one women with Marfan syndrome became mothers. Women with Marfan syndrome were also compared to 543 healthy controls. RESULTS:Women with Marfan syndrome were more often born preterm (p<0.001), small-for-gestational age (p<0.001), and delivered by cesarean section (p=0.001). Women with Marfan syndrome had no increased risk of giving birth by cesarean section (p=0.079). No increased neonatal risks in their children were found. Women with Marfan syndrome were less likely to give birth than those without (p<0.001). There were no maternal deaths. CONCLUSIONS:Women with Marfan syndrome were more likely to be born preterm, SGA and by cesarean section. These increased risks of preterm birth and SGA babies were not found in connection with their own first childbirth. Pregnancies with known fetal Marfan syndrome have to be carefully monitored. The results are important for obstetricians giving preconception counseling and treating women with Marfan syndrome. Further studies are needed to evaluate risks during pregnancy and long-term effects of pregnancy on the cardiovascular status of women with Marfan syndrome.

背景与目标：

BACKGROUND & AIMS: :A 31-year-old woman complained of dyspnea and orthopnea at 38 weeks of gestation. A grade 3/6 pansystolic murmur was heard, and echocardiography revealed severe mitral regurgitation with a hyperechoic obstacle on the posterior mitral valve leaflet, consistent with a diagnosis of acute heart failure due to a ruptured chordae tendineae or an infectious endocarditis. An emergency cesarean section was performed under general anesthesia. A male infant was born weighing 2928 g with Apgar scores of 7 and 8 at 1 and 5 min, respectively. The patient was managed in the intensive care unit and underwent open-heart surgery for mitral valve repair on postpartum day 3. The two chordal tendineae appeared torn and frail, and a mitral annuloplasty was performed. No finding of infectious endocarditis was observed. Because it is a dramatic and life-threatening clinical situation, proper diagnosis and treatment in the intensive care unit assure a good outcome for both mother and fetus.

背景与目标： : 一名31岁的妇女在妊娠38周时抱怨呼吸困难和正呼吸。听到了3/6级的全收缩期杂音，超声心动图显示严重的二尖瓣反流，二尖瓣后叶上有高回声障碍，这与由于腱索破裂或感染性心内膜炎引起的急性心力衰竭的诊断一致。在全身麻醉下进行紧急剖宫产。出生时体重为2928  g的男婴，分别在1和5  min时Apgar评分为7和8。该患者在重症监护病房接受治疗，并于产后第3天接受心脏直视手术进行二尖瓣修复。两个腱索出现撕裂和脆弱，并进行了二尖瓣环成形术。未发现感染性心内膜炎。由于这是一个戏剧性的，危及生命的临床情况，因此在重症监护病房中进行适当的诊断和治疗可确保母亲和胎儿的良好结局。