胎儿动脉导管缺如的尸体解剖分析

目的:对胎儿先天性动脉导管缺如进行尸体解剖分析,以提高该疾病诊断的精准性。方法:回顾2011年1月至2019年9月北京安贞医院经超声心动图畸形筛查发现胎儿先天性心脏畸形引产而进行尸体解剖病例400例,检查胎儿心血管畸形及内脏的畸形情况,对其动脉导管缺如进行分析总结。结果:400例胎儿先天性心脏病中,动脉导管缺如24例,...     

Prenatal diagnosis of Walker-Warburg syndrome in three sibs

Walker-Warburg syndrome (WWS) is an autosomal recessive condition characterized by diffuse neurodysplasia, resulting in brain and eye abnormalities. We report on 3 prenatally diagnosed cases of this syndrome born to a consanguineous couple. An ultrasonographic examination showed hydrocephalus at the 27th week of the first pregnancy. Amniocentesis documented a normal male karyotype. The couple opted for termination of the pregnancy but declined an autopsy. Seven months later, hydrocephalus was observed at 20 weeks of the second pregnancy. Termination of pregnancy was performed at the 22nd week. Autopsy of this male fetus showed dilated ventricles, thin cortex, and type II lissencephaly with microscopic evidence of chaotic architecture. Eye examination showed retinal dysplasia. Notwithstanding the lack of demonstrable muscle change, the diagnosis of Walker-Warburg syndrome was made. Ten months later, hydrocephalus was discovered in the third fetus, a female, at 13 weeks of gestation. Termination of pregnancy was performed at 20 weeks. At autopsy, brain, eye, and muscular findings were similar to those of the previous case. In addition, cystic changes and a stenosis of the pyelo-ureteral junction were found in the right kidney. Type II lissencephaly and retinal dysplasia are characteristic of WWS. Muscular dystrophy has been pointed out as an additional abnormality in postnatal cases. By contrast, the lack of demonstrable muscle changes in the fetal period must be emphasized. Those cases illustrate practical problems in the ultrasound and pathologic diagnosis of WWS in the fetal period. Am. J. Med. Genet. 76:107–110, 1998. © 1998 Wiley-Liss, Inc.     

应用Yagel式快速心脏检查法产前筛查先心病的价值研究

目的探讨Yagel式胎儿心脏快速扫描法在胎儿心脏产前诊断中的价值。方法病例选自2002年以来在中国医科大学附属盛京医院、沈阳市妇婴医院、大连妇产医院和锦州市妇婴医院就诊的孕妇7394例,均为单胎妊娠,其中先天性心脏畸形（CHD）高危患者1276例。应用Yagel5个心脏横面检查方法进行胎儿心脏快速检查,并对引产胎儿进行尸体解剖核对产前诊断的正确性;对产前诊断未发现明显异常胎儿进行临床随访,胎儿出生后进行新生儿或婴儿心脏超声检查,判定产前诊断的正确性。结果 1.7394例孕妇中,检查发现胎儿心脏结构异常为79例（1.07%）,其中31例（39.2%）患者来自于CHD高危人群。2.79例产前诊断为CHD患者中,72例选择了终止妊娠放弃胎儿,其中56例进行尸体解剖,其中1例患者病理诊断为永存动脉干畸形,产前诊断为法洛四联症;1例右心室双流出道,产前诊断为大动脉转位;1例为部分型肺静脉异位引流,产前诊断为左心发育不良;1例为主动脉缩窄,产前诊断为左心发育不良。3.79例产前诊断为CHD患者中,7例选择继续妊娠,其中室间隔缺损（VSD）3例,法洛四联症1例,永存动脉干1例,右心室占位病变（0.8cm×0.8cm）1例,轻度肺动脉狭窄伴三尖瓣返流1例。4.6118例产前诊断为正常胎儿心脏患者,新生儿或婴儿心脏超声检查发现室间隔缺损2例（0.03%）;动脉导管未闭2例;房间隔缺损1例;部分肺静脉异位引流1例,主动脉轻度狭窄1例,肺动脉轻度狭窄1例。5.应用Yagel胎儿心脏检查方法诊断胎儿心脏异常的敏感性为90.8%,特异性为100%。结论 1.Yagel式胎儿心脏快速扫描法是产前诊断胎儿心脏畸形的安全、简单和有效方法。2.对所有人群进行胎儿心脏结构产前检查,减少严重心脏病患儿的出生,具有重大的社会和经济意义。     

胎儿超声心动图诊断胎儿先心病在高危妊娠产前筛查中的意义

目的探讨胎儿超声心动图在高危妊娠产前筛查中的意义。方法应用西门子SonolineG60S型彩色多普勒超声诊断仪对570例孕龄在22-40周的高危妊娠孕妇行胎儿超声心动图检查,并将产前超声检查与胎儿尸检或分娩后超声心动图检查结果进行对照。同时检测孕龄在22-40周无明显高危妊娠因素的孕妇750例,作为平行对照组。结果检出72例心脏异常,先天性心脏畸形47例,先心病检出率为3.56%,其中高危组41例,先心病检出率为7.2%;对照组6例,检出率为0.8%。另外发现左室腔内强回声8例,心包积液3例,三尖瓣少量反流14例。结论超声心动图常规及系统诊断技术是诊断胎儿先天性心脏病较完善、可靠的方法,对检测胎儿心脏畸形有着十分重要的临床应用价值,对高危妊娠孕妇更应及早行常规胎儿心脏超声心动图检查胎儿。单纯性心包积液或单纯性少量三尖瓣反流或单纯心脏内有点状强回声,不能作为终止妊娠的指标。     

超声四切面法快速筛查胎儿心脏畸形

目的探讨超声筛查胎儿心脏畸形的简便可靠方法。方法采用胎儿上腹部横切面、四腔心切面、左、右心室流出道切面联合对9724例孕18—40周的孕妇进行胎儿心脏超声筛查,疑有异常者送上级医院行系统超声心动图检查,并追踪随访,与引产后尸检或出生后超声心动图对照。结果检出心脏畸形例18,漏诊3例;孕22—24周的胎儿,胎儿仰卧位时最容易检查。结论四切面法是筛查胎儿心脏畸形的简便可靠方法,胎儿仰卧位时容易获得标准切面发现心脏畸形,筛查时间最短,漏诊少。     

Fatal intrauterine adenoviral endomyocarditis with aortic and pulmonary valve stenosis: diagnosis by polymerase chain reaction

We report a case of fatal hydrops fetalis owing to adenoviral endomyocarditis with aortic and pulmonary valve stenosis. A 1850-g macerated male stillborn delivered 1 week after fetal ultrasonography showed hydrops, cardiomegaly, and possible aortic valve stenosis. Autopsy confirmed hydrops and showed thickened, fibrotic semilunar valves with stenosis. The myocardium was focally fibrotic with areas of calcification. Polymerase chain reaction study of myocardial and aortic valve tissue was positive for adenovirus. Intrauterine viral myocarditis has been reported only rarely, but cases owing to Coxsackie B virus, adenovirus, and parvovirus B19 have appeared in the literature. With the exception of rubella, viral causation of significant valvular lesions in humans has received scanty support in the literature. This report suggests a broader group of causative agents. HUM PATHOL 31:1433-1435.     

法洛四联症伴肺动脉瓣缺如3例报道并文献复习

目的总结法洛四联症伴肺动脉瓣缺如患儿的临床特点和诊治经验。方法收集复旦大学附属儿科医院2007年11月至2009年2月收治的法洛四联症伴肺动脉瓣缺如3例患儿的临床资料,总结其临床表现、影像学特征、治疗和预后。结果男1例,女2例,年龄39 d至4个月。临床均表现为气急、咳喘及轻度发绀;体检可闻及胸骨左缘来回杂音。超声心动图和心导管检查除法洛四联症表现外,可见肺动脉瓣环发育不良或缺如,伴肺动脉重度反流、肺动脉瘤样扩张,均未见动脉导管未闭,其中1例左肺动脉不连接伴发育不良。2例临床症状严重的患儿施行手术根治,其中1例手术效果满意,另1例左肺动脉不连接伴发育不良患儿术中死亡;1例患儿临床症状尚不明显,仍在密切随访中。结论法洛四联症伴肺动脉瓣缺如尽管少见,但由于在婴儿期甚至新生儿期即可引起严重气道受压从而危及生命,临床中遇到难以控制的呼吸窘迫新生儿和小婴儿应考虑本病的可能。尽早手术根治解除气道受压呼吸困难症状是唯一的治疗方法,无呼吸道受压症状可考虑6个月左右择期手术。手术病死率高于单纯法洛四联症,尤其是伴一侧肺动脉不连接或发育不良的患儿。     

不同切面超声心动图检查对胎儿心血管畸形的诊断价值

目的评价不同切面超声心动图检查在胎儿心血管畸形筛查中的诊断价值。方法通过对3000例20-28周的孕妇进行不同切面超声心动图检查及部分染色体核型检查及尸检结果,统计分析不同切面超声心动图检查对各种胎儿心脏畸形的检出率及敏感性。结果诊断先天性心脏病34例,发病率为1.1%,其中明确诊断27例,漏诊6例,误诊1例,标准三切面筛查的诊断敏感性为77.8%。结论标准三个切面超声心动图检查对胎心血管畸形筛查有较高的敏感性及特异性,是胎儿心脏心血管畸形筛查的首选方法。     

Long-term follow-up of patients with extracardiac valved conduits

Seventeen patients having extracardiac valved conduits placed between the right ventricle and pulmonary artery were followed for 7 to 87 months postoperatively (mean, 42 months), at the Heart Institute, Kenritsu Amagasaki Hospital, Japan. There were no late deaths in the study group. Three conduits have been replaced, all because of conduit stenosis. In two-dimensional echocardiographic examinations, commissural fusion and calcification of the valve were noted in 6 out of 16 xenograft valved conduits. Mechanical valve immobility was found in one patient. Neointimal peel of the dacron graft was noted in 6 out of 17 cases, and marked left ventricular deformity in the short axis view was found in 6. Late cardiac catheterization was done in 6 patients who were suspected of having valve failure and right ventricular hypertension by two-dimensional echocardiography. All 6 of these patients showed a high pressure gradient between the pulmonary artery and right ventricle and also had elevated right ventricular pressure. In conclusion, two-dimensional echocardiography is a simple, non-invasive and very accurate method for detecting conduit stenosis and valve failure. An echocardiographic series should be performed for a long-time postoperatively because obstructions of valved conduits may be progressive, and an operation may be advisable in order to prevent the development of advanced right ventricular hypertrophy and deterioration.     

Morphometric study of the human fetal heart. I. Arterial segment

This study measured the dimensions of the great arteries of normal human fetal hearts at an early fetal stage (between 13-20 weeks post-fertilization) in 103 fetuses obtained by necropsy. Different segments of the aorta and the pulmonary artery were dissected and their external diameters measured under stereoscopic magnification. All segments showed linear growth during this period of fetal development. Ranges in median values of external diameters associated with the pulmonary artery were: 2.1-4.2 mm for the valve ring; 2.2-4.2 mm for the main pulmonary trunk; 1.2-2.5 mm for the right pulmonary artery; 0.9-2.18 mm for the left pulmonary artery; and 4.0-8.0 mm for the length of the main pulmonary trunk. Similarly, in the aorta, median diameters were: 2.1-4.2 mm for the ascending aorta; 1.92-3.8 mm for the aortic arch; 1.45-3.0 mm for the aortic isthmus; and 1.75 -3.35 mm for the descending aorta. The diameter of the ductus arteriosus ranged between 1.2-2.45 mm. The growth rates of the pulmonary artery and the aorta were similar. The diameter of the ascending aorta was found to be greater than that of the descending aorta and the right pulmonary artery was wider than the left pulmonary artery. In addition, the magnitude of growth in the various aortic segments was different and the ratios obtained between the aortic isthmus and the ascending and descending aortae, ranged between 0.66-0.93. This study provides important morphometric reference information concerning the dimensions and growth of the great arteries of the fetal heart and has clinical application in pediatric cardiac surgery and echocardiography.     

双胎妊娠-胎宫内死亡20例临床分析

目的探讨双胎妊娠-胎宫内死亡的原因、临床处理及预后。方法回顾性分析1994年3月至2008年2月北京大学第三医院双胎妊娠-胎宫内死亡的病例共20例。结果双胎-胎宫内死亡的发生率为3．2％,其中双卵双胎17例,单卵双胎3例。28w前发现-胎宫内死亡者6例,平均期待治疗95．8天,平均分娩孕周为34．4w（32^＋4-39w）;28w后发现-胎宫内死亡者14例,平均期待治疗10．1天,平均分娩孕周为34．6w（29^＋4-39^＋2w）;仅2例新生儿发生轻度窒息。无一例孕妇发生凝血功能障碍。追踪新生儿6月至14年,1例新生儿因合并隐性脊柱裂及早产原因早期死亡,1例患儿3岁时发生运动障碍性脑瘫（分娩孕周为29^＋6w）,4例失访,其余均健康存活。结论双胎妊娠-胎宫内死亡后可采取期待治疗,严密监测存活胎儿宫内状况,尽量延长孕周,可提高存活胎儿的生存质量。     

结合心超声研究的胎儿心脏测量

对40例16-42周引产的胎儿心脏进行了17项与胎儿心超声研究直接有关的项目的测量。36例为正常胎儿心脏。动脉导管长度随胎龄增加而加长,内径下端大于上端。卵园孔全部开放,一半为孔,一半为裂隙;瓣膜覆盖孔的面积为20～100%。一例卵园孔瓣膜向右房侧脱垂。四个瓣环周长随胎龄而增大。三尖瓣环周长大于二尖瓣环周长,肺动脉瓣环周长大于主动脉瓣环周长。左室壁比右室壁厚,比值约为1.2.3例畸形胎儿的心脏形态正常。1例正常胎儿心脏的动脉导管长而弯曲。     

完全性大动脉错位动脉转位术后新主肺动脉发育的随访

目的报道应用动脉转位术（ASO）治疗完全性大动脉错位（TGA）术后超声心动图的随访结果,评价TGA术后主、肺动脉的发育情况。方法以2001年3月至2007年3月于复旦大学附属儿科医院心血管中心实施ASO治疗TGA术后存活患儿作为研究对象,于2008年8～9月进行随访,行超声心动图检查,测定新主动脉根部内径、新主动脉瓣环内径、新肺动脉内径和新肺动脉瓣环内径,获取的数据与正常参考值做比较。结果研究期间ASO治疗TGA患儿共72例,术中死亡6／72例（8．3％）,存活率为91．7％。失访10／66例,有效数据56例,其中室间隔完整的完全性TGA（TGA／IVS）32例,完全性TGA合并室间隔缺损（TGA／VSD）24例。随访距手术18—168（86．46±23．51）个月。新主动脉根部内径为（16．97±2．71）mm（t=6．936,P〈0．001）,12／56例（21．4％）〉正常参考值90％CI的上限,44／56例（78．6％）在正常参考值90％CI内,平均Z值为1．10±0．70。新主动脉瓣环内径为（16．27±2．38）mm（t=4．52,P〈0．001）,56例均在正常参考值90％CI内,平均Z值为0．66±0．65。新肺动脉内径为（14．29±1．92）mm（t=-3．2,P=0．005）,40／56例（71．4％）在正常参考值90％CI内,16／56例（28．6％）〈正常参考值90％CI的下限,平均Z值为-0．95±1．33。新肺动脉瓣环内径为（14．00±1．92）mm（t=0．132,P=0．897）,42／56例（75．0％）在正常参考值90％CI内,14／56例（25．O％）〈正常参考值90％CI的下限,平均Z值为0．05±1．85。16／56例（28．6％）存有主动脉瓣轻度反流,均为TGA／VSD病例,平均随访（46．0±22．2）个月;40／56例（71．4％）未见主动脉瓣反流,平均随访（46．8±25．3）个月,两组随访时间差异无统计学意义（P=0．899）。随访中未见主动脉狭窄病例。肺动脉瓣上狭窄7／56例（12．5％）,2／56例（3．6％）为轻度狭窄,5／56例（8．9％）为极轻度狭窄。结论目前在复旦大学附属儿科医院心血管中心开展ASO治疗TGA术后新主动脉根部和瓣环内径均有所扩张,新肺动脉根部内径有所缩减,但其瓣环发育良好。主动脉瓣反流常见于TGA／VSD病例,但其反流程度较轻。肺动脉瓣上狭窄是TGA术后常见的并发症,但大多数病例狭窄程度极轻。     

Echocardiographic features of adult tetralogy of Fallot with natural palliative correction by patent ductus arteriosus

A thirty-year-old man with the diagnosis of the tetralogy of Fallot and patent ductus arteriosus was admitted to our hospital because of a syncope. He reported no previous symptoms. We diagnosed adult tetralogy of Fallot, which included all four characteristic anomalies: ventricular septal defect, overriding aorta, pulmonary artery stenosis, and right ventricular hypertrophy. The associated persistent ductus arteriosus and the presence of compensatory arteriovenous communications produced a continuous flow load on the left ventricle, which resulted in moderate left ventricular hypertrophy, but without symptoms of pulmonary congestion or cardiac decompensation. Anatomic diagnosis and hemodynamic assessment were established by transthoracic and transesophageal echocardiography, with incidental finding of a quadricuspid aortic valve. To the best of our knowledge, our case of the adult form of Fallot's tetralogy associated with both patent ductus arteriosus and quadricuspid aortic valve is the first one ever described. It is well known that patients with tetralogy of Fallot who do not undergo operation in childhood have short survival, which depends predominantly on the degree of pulmonary artery stenosis and early development of collateral circulation to the lungs. Long-term persistence of natural aortopulmonary anastomosis with systemic collateral circulation to the lungs and remodeling of the heart, with better hemodynamic balance as well as the presence of mild pulmonary artery stenosis probably enhanced the survival of our patient.     

胎儿超声心动图对胎儿室上性心动过速的诊断和治疗评价

目的应用胎儿超声心动图宫内诊断胎儿快速心律失常并对宫内干预治疗室上性心动过速(SVT)进行跟踪评价。方法对已检出的1900名心律失常胎儿中的121例快速心律失常者,分为窦性心动过速、SVT、SVT合并心衰3组,对后两组胎儿进行宫内地高辛转律治疗。结果胎儿心动过速121例(心率≥180bpm),其中SVT29例(含房颤、房扑7例);余为窦性心动过速92例。SVT组中合并复杂先天性心脏病者7例,胎儿水肿7例。合并先心病均于检查后中止妊娠,其余18例行常压氧治疗或经胎盘给药,首选药物为地高辛。新生儿随访期最长16个月,头颅CT、超声心动图等各项生理指标均正常。结论胎儿超声心动图是目前唯一可迅速检出胎儿心律失常并明确性质的诊断手段,且重复性好。地高辛经胎盘治疗可有效转律并同时控制胎儿心衰减轻水肿且无致畸作用。     

超声心动图对胎儿三尖瓣下移畸形的诊断价值

目的探讨超声心动图对胎儿三尖瓣下移畸形的诊断价值。方法回顾性分析5例经胎儿超声心动图检查诊断为三尖瓣下移畸形的声像图与随访结果,总结其二维及彩色多普勒血流声像图特点。结果 5例胎儿经尸解均证实为三尖瓣下移畸形。胎儿三尖瓣下移畸形的主要声像图特点表现为四腔心不对称,右心房增大,三尖瓣叶附着点下移,三尖瓣重度反流,反流起源点降低,反流面积大、反流速度低。结论超声心动图诊断胎儿三尖瓣下移畸形方法简单、可靠,对胎儿三尖瓣下移畸形的诊断具有重要的临床价值。     

Primary cardiac sarcoma in pregnancy: a case report

Primary cardiac sarcoma is a rare disease in adults. It is also associated with poor prognoses, due to diagnostic delay, therapeutic difficulty, and high metastatic potential. The coincidence of pregnancy and a primary cardiac intimal sarcoma is extremely rare. We report a pregnant woman at 27(+5) weeks gestation who was admitted to the hospital with acute-onset dyspnea. A mass was found on the left atrium by transthoracic echocardiography. Subsequently, the intracardiac mass was removed, and mitral valve replacement and modified DeVega tricuspid annuloplasty were performed. The patient was diagnosed with a undifferentiated sarcoma, and gave birth to a 1,230 g living baby boy by Caesarean section from preterm contraction at 29(+5) weeks gestation. The patient then received systemic chemotherapy. However, 10 months after the initial clinical onset, the patient suddenly died. Surgery is the standard treatment for cardiac tumors, and their removal should always be attempted, even in pregnant women. Although the overall survival rates of the patients are rather poor, palliative cardiac surgery allows the prolonging of pregnancy, until an acceptable fetal viability level is reached.     

Sex chromosome trisomies in Europe: prevalence, prenatal detection and outcome of pregnancy

This study aims to assess prevalence and pregnancy outcome for sex chromosome trisomies (SCTs) diagnosed prenatally or in the first year of life. Data held by the European Surveillance of Congenital Anomalies (EUROCAT) database on SCT cases delivered 2000–2005 from 19 population-based registries in 11 European countries covering 2.5 million births were analysed. Cases included were livebirths diagnosed to 1 year of age, fetal deaths from 20 weeks gestation and terminations of pregnancy for fetal anomaly (TOPFA). In all, 465 cases of SCT were diagnosed between 2000 and 2005, a prevalence of 1.88 per 10,000 births (95% CI 1.71–2.06). Prevalence of XXX, XXY and XYY were 0.54 (95% CI 0.46–0.64), 1.04 (95% CI 0.92–1.17) and 0.30 (95% CI 0.24–0.38), respectively. In all, 415 (89%) were prenatally diagnosed and 151 (36%) of these resulted in TOPFA. There was wide country variation in prevalence (0.19–5.36 per 1000), proportion prenatally diagnosed (50–100%) and proportion of prenatally diagnosed resulting in TOPFA (13–67%). Prevalence of prenatally diagnosed cases was higher in countries with high prenatal detection rates of Down syndrome. The EUROCAT prevalence rate for SCTs diagnosed prenatally or up to 1 year of age represents 12% of the prevalence expected from cytogenetic studies of newborn babies, as the majority of cases are never diagnosed or are diagnosed later in life. There is a wide variation between European countries in prevalence, prenatal detection and TOPFA proportions, related to differences in screening policies as well as organizational and cultural factors.     

先天性血管环12例诊断与治疗分析

目的探讨先天性血管环的临床特征、诊断与治疗。方法回顾性分析2008年7月至2011年12月在首都医科大学附属北京安贞医院诊断为先天性血管环患儿的临床特征、影像学检查、诊断和治疗。结果12例先天性血管环患儿进入分析,男3例,女9例。就诊年龄1个月至21岁,〈1岁7例。反复咳喘11例,喂养困难2例,青紫3例,心脏杂音10例。12例超声心动图示主动脉双弓4例、右位主动脉弓伴左位动脉导管未闭1例、肺动脉吊带7例,其中10例伴其他心脏畸形。10例64排CT气道重建示主气管狭窄5例、左主支气管狭窄1例,右侧气管狭窄3例,食管狭窄2例;2例纤维支气管镜示主气管狭窄1例,右主支气管狭窄1例。7例行手术治疗,根据血管环的病理类型选择术式,包括2例切断双主动脉弓中的次弓、1例切断右位主动脉弓伴随的左位动脉导管、4例左肺动脉移植术。1例术后死亡,6例术后3—6个月呼吸道症状逐渐好转,生长发育改善。结论儿童中反复出现原因不明的咳喘等呼吸道症状者,应考虑到先天性血管环的可能,超声心动图联合多排CT可明确诊断,及早手术解除气管狭窄是改善预后的关键。     

先天性左冠状动脉主干闭锁3例超声心动图特征及文献复习

目的 通过研究先天性左冠状动脉主干闭锁（LMCAA）的超声心动图表现,提高对LMCAA诊断的准确性.方法 回顾性分析经冠状动脉造影证实的3例LMCAA患儿的超声心动图检查结果,并复习相关文献,总结LMCAA超声心动图特征.结果 LMCAA特异性超声心动图特征：①主动脉左冠状动脉窦内无左冠状动脉主干开口,左冠状动脉主干近心端闭锁呈盲端,远心端内径细窄,发育不良;②右冠状动脉内径增宽;③多切面未显示左冠状动脉与肺动脉确切连接的证据.LMCAA非特异性超声心动图特征：①左心室明显扩大,左室收缩功能可正常或减低;②二尖瓣腱索、乳头肌回声显著增强,可伴有二尖瓣脱垂.彩色多普勒超声特征：①收缩期二尖瓣口可见中至大量反流信号;②左、右冠状动脉之间形成细小侧支循环; ③左冠状动脉前降支和回旋支血流为逆向灌注（向心性）;④发育不良的左冠状动脉虽然在肺动脉周围分布,但彩色多普勒超声不能显示其与肺动脉连接的确切逆灌血流信号.结论 LMCAA有特异性的超声心动图特征,提高对LMCAA的全面认识是诊断本病的关键.