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Mayer-Rokitansky-Kuster-Hauser (MRKH) Syndrome – A Case ReportRT MRKH综合征1例报告
Pub Date : 2014-07-09 DOI: 10.3329/JPSB.V1I1.19468
Md. Kabirul Islam, Md. Shah Alam, Abdul Hanif, Md. Kamrul Hasan, Mahbubur Rahman
We report a case of Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome in a 25 year old married lady. Though it is a congenital abnormality, she presented as primary infertility after 2 year of her marriage and was referred to us from a Gynaecologist. She had absent vagina, rudimentary uterus with no cervix. Her ovaries were severely hypoplastic. The anus was placed anteriorly and opened into the vulva. In spite of absence of her vagina, the lady somehow maintained her married life by doing intercourse through the anteriorly placed rectum. The vagina was made from the lower end of existing rectum which opened into the vulva. The proximal end of the rectum and left colon were pulled through the pelvis and opened into the perineum. It improved her quality of life. DOI: http://dx.doi.org/10.3329/jpsb.v1i1.19468
我们报告一例梅耶-罗基坦斯基-库斯特-豪瑟(MRKH)综合征在一个25岁的已婚女士。虽然这是一种先天性异常,但她在结婚两年后表现为原发性不孕,并从妇科医生那里转介到我们这里。她没有阴道,子宫发育不全,没有子宫颈。她的卵巢严重发育不良。肛门放在前面,打开进入外阴。尽管没有阴道,这位女士还是以某种方式通过放置在前面的直肠进行性交来维持她的婚姻生活。阴道是由现有直肠的下端形成的,直肠通向外阴。直肠近端和左结肠被拉出骨盆,打开进入会阴。这提高了她的生活质量。DOI: http://dx.doi.org/10.3329/jpsb.v1i1.19468
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引用次数: 0
NEONATAL SURGERY: DEMAND AND SURVIVAL BOTH ARE INCREASING: EIGHT YEARS EXPERIENCE IN DHAKA MEDICAL COLLEGE HOSPITAL, BANGLADESH 新生儿手术:需求和存活率都在增加:孟加拉国达卡医学院医院8年经验
Pub Date : 2014-07-09 DOI: 10.3329/JPSB.V1I1.19463
A. Hanif, K. Hasina, A. Hossain, S. Huda, M. K. Hassan, M. Alam, A. Z. Hossain
Purpose: In order to achieve the Millennium Development Goal (MDG) 4 target of a two-thirds reduction in under-five mortality from 1990 to 2015, major reductions are going to be required in neonatal mortality. Congenital anomalies have become the fourth cause of neonatal deaths and most of these are curable. Dhaka Medical College Hospital is the largest public hospital of the country and serving the poor and lower middle class people where surgery and medical facilities are mostly free of cost. This study was done to see the types of neonatal surgical patients admitted in this hospital and their management out come with limited facilities and find out the ways to improve the scenario to contribute in achieving the MDG. Materials & Methods: This was a retrospective study done over a period of 8 years from July 2001 to June 2009 and carried out in the Department of Pediatric Surgery, Dhaka Medical College Hospital, Dhaka. A total of 650 neonates were admitted during this period and it was the 17.99% of total number of 3612 pediatric surgical admission upto12 years of age. Data was collected from hospital records and analyzed retrospectively. Results: Out of these 650 neonates 533(80%) were admitted for Neonatal Intestinal Obstruction (NIO) and omphalocele were 59 (9.07%), ectopia vesicae are 14 (2.15% ), posterior urethral valves (PUV) were 19 (2.92%), gastroschisis were 7 (1.07%) , congenital diaphragmatic hernia (CDH) were 16 (2.46%),Tracheo-oesophageal fistula 1 (0.15%), and Conjoint twin 1 (0.15%). The most common cause of NIO was anorectal malformation (ARM) and 194 (66.43%) patients have high variety and 98(33.56%) patients have low variety ARM. Next was Hirschsprung disease and 137 (21.07%) neonates presented with this. Thirty-Five (5.38%) patients presented with septicemia and 40 (6.15%) presented with intestinal atresia, 20 (3.07%) neonates had meconium ileus and 9 (1.38%) patients presented with volvulus neonatorum. Five hundred and Fifty-nine patients (86.00%) were managed surgically. Out of 650 patients, 69 died, so mortality was 10.61% and before surgery 2.76% and after surgery was 7.84%. Conclusion: Pediatric surgeons by their skill and teamwork greatly improved the neonatal surgical service and contributing significantly in reducing infant mortality rate and to achieve MDG 4. But to improve further, neonatal intensive care unit (NICU) and other support systems are essential as well as support from UNICEF and World Health Organization (WHO) to include pediatric surgery and surgeons in their activities especially in developing countries. Due to socio-political and economic reasons of the developing countries the roll of pediatric surgeons are multidimensional. DOI: http://dx.doi.org/10.3329/jpsb.v1i1.19463
目的:为了实现千年发展目标4关于在1990年至2015年期间将五岁以下儿童死亡率降低三分之二的具体目标,将需要大幅降低新生儿死亡率。先天性异常已成为新生儿死亡的第四大原因,其中大多数是可以治愈的。达卡医学院医院是该国最大的公立医院,服务于穷人和中下层阶级,那里的手术和医疗设施大多是免费的。本研究旨在了解该医院收治的新生儿外科患者的类型及其在有限设施下的管理情况,并找出改善情况的方法,为实现千年发展目标做出贡献。材料与方法:这是一项回顾性研究,于2001年7月至2009年6月在达卡医学院附属医院儿科外科进行,历时8年。同期共收治新生儿650例,占12岁以下儿科外科收治患儿3612例的17.99%。从医院记录中收集数据并进行回顾性分析。结果:650例新生儿中,新生儿肠梗阻533例(80%),脐膨出59例(9.07%),膀胱异位14例(2.15%),后尿道瓣膜(PUV) 19例(2.92%),胃裂7例(1.07%),先天性膈疝(CDH) 16例(2.46%),气管-食管瘘1例(0.15%),联合双胎1例(0.15%)。肛门直肠畸形(anano直肠malformation, ARM)是NIO最常见的病因,其中高品种194例(66.43%),低品种98例(33.56%)。其次是先天性巨结肠病,137例(21.07%)新生儿出现此病。败血症35例(5.38%),肠闭锁40例(6.15%),胎粪肠梗阻20例(3.07%),新生儿扭转9例(1.38%)。手术治疗559例(86.00%)。650例患者死亡69例,死亡率10.61%,术前2.76%,术后7.84%。结论:儿科外科医生的技能和团队合作大大提高了新生儿外科服务水平,为降低婴儿死亡率和实现千年发展目标4做出了重要贡献。但为了进一步改善,新生儿重症监护病房(NICU)和其他支持系统以及联合国儿童基金会和世界卫生组织(世卫组织)的支持至关重要,以将儿科外科和外科医生纳入其活动,特别是在发展中国家。由于发展中国家的社会政治和经济原因,儿科外科医生的人数是多方面的。DOI: http://dx.doi.org/10.3329/jpsb.v1i1.19463
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引用次数: 1
Revival of Landing's Concept of Infantile Obstructive Cholangiopathy – A Plea to Hand Over the 2 Week Old Jaundiced Full Term Neonate to the Surgeon! 婴儿梗阻性胆管病概念的复兴——呼吁将2周大黄疸足月新生儿交给外科医生!
Pub Date : 2014-07-09 DOI: 10.3329/JPSB.V1I1.19465
Shilpa Sharma, D. Gupta
The concept of infantile obstructive cholangiopathy started in 1974 by Landing and remained till early 1980s. However, the concept has not been used much since then as nothing new has developed over the last 40 years sufficient enough to change the course of the disease or identify its etiology. This review article is an attempt to consolidate evidence on review of literature pointing towards a common etiopathological process leading to the spectrum of anomalies constituting neonatal cholestasis. The importance of early referral of any case with neonatal jaundice in a full term neonate, preferably at 2 weeks age is highlighted. This will help to identify and treat more cases within the correctable range and prevent the disease from progressing to a life threatening situation especially in developing countries where the resources for liver transplantation are meagre. DOI: http://dx.doi.org/10.3329/jpsb.v1i1.19465
婴儿梗阻性胆管病的概念始于1974年,一直持续到20世纪80年代初。然而,从那时起,这个概念就没有被广泛使用,因为在过去的40年里,没有任何新的发展足以改变疾病的进程或确定其病因。这篇综述文章是试图巩固文献综述的证据指向一个共同的病因病理过程导致频谱异常构成新生儿胆汁淤积症。早期转诊的重要性与新生儿黄疸的任何情况下,足月新生儿,最好是在2周龄被强调。这将有助于识别和治疗更多的病例在矫正范围内,防止疾病发展到危及生命的情况下尤其在发展中国家的资源为肝移植是微薄的。DOI: http://dx.doi.org/10.3329/jpsb.v1i1.19465
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引用次数: 0
Ambiguous Genitalia with Perineal Rhabdomyosarcoma: A Case Report 阴唇模糊伴会阴横纹肌肉瘤1例
Pub Date : 2014-07-09 DOI: 10.3329/JPSB.V1I1.19473
T. Banu, T. Chowdhury, M. Hoque
Individuals having ambiguous genitalia have been described since antiquity. But their association with the occurrence of any cancer is not well established. Here we report a case of perineal rhabdomyosarcoma associated with ambiguous genitalia. DOI: http://dx.doi.org/10.3329/jpsb.v1i1.19473
自古以来,就有描述具有模糊生殖器的个体。但它们与任何癌症的发生之间的联系还没有得到很好的证实。我们在此报告一例会阴横纹肌肉瘤合并阴唇不清。DOI: http://dx.doi.org/10.3329/jpsb.v1i1.19473
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引用次数: 0
Two Case Reports of Peutz Jegher's Syndrome Peutz Jegher综合征2例报告
Pub Date : 2014-07-09 DOI: 10.3329/JPSB.V1I1.19471
A. Hanif, K. Hasina, Md. Kamrul Hassan, S. Talukder, Md. Kabirul Islam
We are reporting two cases in a family, Elder sister was 10 year old & younger one was 8 years old. Both of them were presented to us with the features of gastric outlet obstruction. Further examination revealed that there is pigmentation on their muco–cutanious junction of lips. Their father also had pigmentation on the muco– cutanious junction of lips. DOI: http://dx.doi.org/10.3329/jpsb.v1i1.19471
我们报告了一个家庭中的两个病例,姐姐10岁,妹妹8岁。两例患者均以胃出口梗阻为特征。进一步检查发现他们的嘴唇粘膜-皮肤交界处有色素沉着。他们的父亲在嘴唇的粘膜交界处也有色素沉着。DOI: http://dx.doi.org/10.3329/jpsb.v1i1.19471
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引用次数: 1
Endoscopic Removal of Vesical Calculus Through Suprapubic Route: A Case Report 耻骨上径路膀胱结石内镜切除1例报告
Pub Date : 2014-07-09 DOI: 10.3329/JPSB.V1I1.19469
Md. Akbar Hossain Bhuyian, Md. Ashraf Ul Huq, M. Kabir, S. H. Chowdhury
For decades, vesical stone has been removed by traditional cystolithotomy. It gives a big scar and adds considerable morbidity to the patient. Here we tried a new indigenous technique to remove the bladder stone with a laparoscopic grasper . The aim was to minimize the morbidity and an almost invisible scar to the patient. DOI: http://dx.doi.org/10.3329/jpsb.v1i1.19469
几十年来,膀胱结石一直是通过传统的膀胱取石术去除的。它会留下很大的疤痕,给病人增加相当大的发病率。在这里,我们尝试了一种新的本土技术,用腹腔镜抓手去除膀胱结石。其目的是尽量减少发病率和病人几乎看不见的疤痕。DOI: http://dx.doi.org/10.3329/jpsb.v1i1.19469
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引用次数: 1
Postoperative Results Following Surgery for PUJO in Children 儿童PUJO手术后的结果
Pub Date : 2014-07-09 DOI: 10.3329/JPSB.V1I1.19466
P. Goel, D. Gupta
Pelvi-ureteric junction obstruction (PUJO) is among the most common causes of congeni tal urinary obstruction. With the introduction of antenatal sonography and its universal acceptance a larger number of cases of asymptomatic hydronephrosis are picked up early in the natural history of the disease. However, this hydronephrosis tends to improve or resolve spontaneously in a large number of cases and has forced the treating personnel to alter their understanding of the disease process and redefine the indications for surgical management. In today’s scenario, we are equipped with a lot many options directed towards the management of PUJ obstruction. The spectrum of these options ranges from watchful waiting at the conservative end to balloon dilatation, endopyelotomy and pyeloplasty which could be open, laparoscopic or robotic. Then we have certain exclusively salvage options like ureterocalicostomy or even nephroureterectomy at the extreme end of the spectrum. However, there are a lot many controversies that surround the management of PUJO. The main indication for performing pyeloplasty include; a symptomatic child with pain, sepsis, lump and deteriorating renal functions. A frusemide induced renal scan showing not only the dilatation but also the retention of the isotope even after 4 hours and an obstructive pattern of the drainage curve (in absence
盆腔输尿管交界处梗阻(PUJO)是先天性尿路梗阻最常见的原因之一。随着产前超声检查的引入及其普遍接受,大量的无症状肾积水病例在疾病的自然史早期被发现。然而,在许多病例中,这种肾积水往往会自行改善或消退,这迫使治疗人员改变他们对疾病过程的理解,并重新定义手术治疗的指征。在今天的情况下,我们有很多针对PUJ阻塞管理的选择。这些选择的范围从保守端观察等待到球囊扩张,肾盂内切开术和肾盂成形术,可以是开放的,腹腔镜的或机器人的。然后我们有一些专门的抢救选择,比如输尿管造口术甚至是最极端的肾输尿管切除术。然而,围绕PUJO的管理存在很多争议。进行肾盂成形术的主要指征包括;有疼痛、败血症、肿块和肾功能恶化症状的儿童。氟塞胺诱导的肾脏扫描显示,即使在4小时后,不仅扩张,而且同位素保留,并且引流曲线呈阻塞性模式(没有)
{"title":"Postoperative Results Following Surgery for PUJO in Children","authors":"P. Goel, D. Gupta","doi":"10.3329/JPSB.V1I1.19466","DOIUrl":"https://doi.org/10.3329/JPSB.V1I1.19466","url":null,"abstract":"Pelvi-ureteric junction obstruction (PUJO) is among the most common causes of congeni tal urinary obstruction. With the introduction of antenatal sonography and its universal acceptance a larger number of cases of asymptomatic hydronephrosis are picked up early in the natural history of the disease. However, this hydronephrosis tends to improve or resolve spontaneously in a large number of cases and has forced the treating personnel to alter their understanding of the disease process and redefine the indications for surgical management. In today’s scenario, we are equipped with a lot many options directed towards the management of PUJ obstruction. The spectrum of these options ranges from watchful waiting at the conservative end to balloon dilatation, endopyelotomy and pyeloplasty which could be open, laparoscopic or robotic. Then we have certain exclusively salvage options like ureterocalicostomy or even nephroureterectomy at the extreme end of the spectrum. However, there are a lot many controversies that surround the management of PUJO. The main indication for performing pyeloplasty include; a symptomatic child with pain, sepsis, lump and deteriorating renal functions. A frusemide induced renal scan showing not only the dilatation but also the retention of the isotope even after 4 hours and an obstructive pattern of the drainage curve (in absence","PeriodicalId":137868,"journal":{"name":"Journal of Paediatric Surgeons of Bangladesh","volume":"15 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2014-07-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"127172798","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Laparoscopic Pyeloplasty - Our Early Experience 腹腔镜肾盂成形术-我们的早期经验
Pub Date : 2014-07-08 DOI: 10.3329/JPSB.V1I1.19448
Gazi Md. Zakir Hossain, Mofizur Rahman, Rashedul Hasan
Objectives: To present our initial experience with laparoscopic pyeloplasty and to evaluate the safety and short-term outcome of this technique. Methods: Five patients underwent laparoscopic dismembered pyeloplasty for the management of ureteropelvic junction obstruction (UPJO) at Chittagong between July’2007 to Mach’2009. Patient age at surgery was 8–22 years. There were two boys and three girls. All had unilateral UPJO with a normal contralateral kidney. We used 5 mm instruments for grasping, blunt dissection, incising and suturing to facilitate safe and precise surgery. The outcome was measured by the operative time, perioperative complications and resolution of obstruction and symptoms. Results: Mean operative time was 195 minutes (range 175–220 min). No major perioperative complications occurred in any cases. Overall, successful resolution of UPJO was observed in all the five cases evident by renogram. Conclusions: Laparoscopic pyeloplasty represents a safe and effective option in the surgical treatment of UPJO. DOI: http://dx.doi.org/10.3329/jpsb.v1i1.19448
目的:介绍腹腔镜肾盂成形术的初步经验,并评价该技术的安全性和短期疗效。方法:2007年7月至2009年3月,在吉大港接受腹腔镜下肢解肾盂成形术治疗输尿管肾盂连接处梗阻(UPJO)的患者5例。患者手术年龄8-22岁。有两个男孩和三个女孩。所有患者均为单侧UPJO,对侧肾脏正常。我们采用5mm器械进行抓握、钝性剥离、切开缝合,确保手术安全、精准。观察手术时间、围手术期并发症、梗阻及症状缓解情况。结果:平均手术时间195分钟(175 ~ 220分钟)。所有病例均无重大围手术期并发症发生。总体而言,5例患者均通过肾重图观察到UPJO的成功消退。结论:腹腔镜肾盂成形术是UPJO手术治疗中一种安全有效的选择。DOI: http://dx.doi.org/10.3329/jpsb.v1i1.19448
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引用次数: 0
Laparoscopically Assisted Anorectoplasty Using Perineal Ultrasonographic Guide 会阴超声引导下腹腔镜辅助肛肠成形术
Pub Date : 2014-07-08 DOI: 10.3329/JPSB.V1I1.19447
A. Kubota
Background/Purpose : As minimal invasive surgery,laparoscopy assisted anorectal pull-through has been reported with new devices. However, it is not easy to create an accurate pull-through canal (PTC), because of the narrow space between the urethra and puborectal sling. The authors describe a new method employing perineal ultrasonography. Methods: The rectourethral prostatic fistula was dissected laparoscopically. Externally, electrostimulation identified the center of the muscle contraction, over which a 1.2 cm of skin incision was made, and the lower part of PTC was created by hemostat forceps guided by electrostimulation. An ultrasonographic probe applied to the perineum demonstrated the urethra, and the forceps was advanced behind the urethra into the pelvic cavity using the ultrasonographic guide. Anorectal pullthrough was performed following dilatation of the PTC with dilators. Results: The authors applied this procedure in 9 cases of male high  anomalies. Surgical damages to the urethra, the levator and vertical muscles were not encountered. Postoperative MRI demonstrated a good location of the rectum, and fluoroscopic study showed a good anterior angulation and intact contraction and relaxation of those muscles. Conclusion: The combination of laparoscopic dissection, pinpointing the center of anal sphincter by electrostimulation and identification of the urethra by ultrasonographic images from the perineum facilitated creation of appropriate PTC in the muscle complex. DOI: http://dx.doi.org/10.3329/jpsb.v1i1.19447
背景/目的:作为一种微创手术,腹腔镜辅助肛肠通拔术已被报道使用了新的器械。然而,由于尿道和耻骨直肠吊带之间的空间狭窄,要建立一个准确的拉过管(PTC)并不容易。作者介绍了会阴超声检查的新方法。方法:腹腔镜下切开直肠尿道前列腺瘘。外部电刺激识别肌肉收缩中心,在其上切开1.2 cm皮肤切口,电刺激引导止血钳形成PTC下半部分。超声探头置于会阴处显示尿道,超声引导下将镊子置于尿道后进入盆腔。用扩张器扩张PTC后进行肛肠牵引。结果:应用该方法治疗男性高畸形9例。尿道、提肛肌和垂直肌未见手术损伤。术后MRI显示直肠位置良好,透视检查显示直肠前角良好,肌肉收缩舒张完整。结论:腹腔镜解剖、电刺激定位肛门括约肌中心和会阴超声图像识别尿道相结合,有助于在肌肉复合体中形成合适的PTC。DOI: http://dx.doi.org/10.3329/jpsb.v1i1.19447
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引用次数: 1
Extradural Haematoma in Children: Surgical Experiences and Prospective Analysis of 138 Cases 儿童硬膜外血肿:138例手术经验及前瞻性分析
Pub Date : 2014-07-08 DOI: 10.3329/JPSB.V1I1.19450
Sharif Md. Noman Khaled Chowdhury, A. Hanif, K. T. Islam, Ehsan Mahmud, S. S. Hossain
Objective: The authors present their experiences in the management of xtradural haematoma in children which involved an aggressive diagnostic approach, prompt surgical evacuation of the haematoma results in an excellent outcome. Subjects and methods: 138 consecutive patients with cranial extradural haematoma who underwent surgery in department of Neurosurgery from 1st January 2006 to 31st July 2009 were included in this prospective study. Each of the patients were evaluated in term of age, sex, mode of injury, localization of haematoma, clinical presentation, CT findings, operative measures and outcome. Results: Out of 138 cases 72.47 % were boys and 13.78 % were girls. The boys and girls ratio was 2.64: 1. Age ranges from 1.8 to 15 years with a mean age of 9.49 years. Most of the victims are in first half of second decade of life and closely followed by the 5-10 years age group. The most common mode of injury was fall 40.58 %, (n = 56) followed by Road traffic Accident (RTA) 31.89 %, (n = 44) .The Most common clinical presentation was altered sensorium 59.43 %, (n = 82), followed by Headache / Vomiting 56.53 %, (n = 78). Conclusion: Extradural haematoma in children is a recognized and one of the most rewarding neurosurgical emergencies. It must be recognized and evacuate early to prevent potential mortality and morbidity. Many factors affect the outcome of extradural haematoma surgery. In addition to influence of presence cranial fractures, associated brain lesions and pre-operative neurological condition of patient, duration of time interval between onset of coma and surgical intervention, morbidity and mortality have also been shown to be affected by age – with better prognosis in patients under 10 years of age. DOI: http://dx.doi.org/10.3329/jpsb.v1i1.19450
目的:作者介绍了他们在处理儿童硬膜外血肿方面的经验,其中包括积极的诊断方法,及时手术清除血肿的效果很好。对象和方法:本前瞻性研究纳入了2006年1月1日至2009年7月31日神经外科收治的连续138例颅硬膜外血肿患者。对每位患者的年龄、性别、损伤方式、血肿部位、临床表现、CT表现、手术措施和预后进行评估。结果:138例中男孩占72.47%,女孩占13.78%。男女性别比为2.64:1。年龄介乎1.8至15岁,平均9.49岁。大多数受害者处于生命第二个十年的前半期,紧随其后的是5-10岁年龄组。最常见的伤害方式为跌倒(40.58%),其次为道路交通事故(RTA) (31.89%) (n = 44),最常见的临床表现为感觉改变(59.43%)(n = 82),其次为头痛/呕吐(56.53%)(n = 78)。结论:儿童硬膜外血肿是公认的、最有价值的神经外科急症之一。必须及早发现并撤离,以防止潜在的死亡率和发病率。影响硬膜外血肿手术结果的因素很多。除了存在的颅骨骨折、相关的脑部病变和患者的术前神经状况、昏迷开始和手术干预之间的时间间隔、发病率和死亡率也受到年龄的影响——10岁以下患者的预后更好。DOI: http://dx.doi.org/10.3329/jpsb.v1i1.19450
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引用次数: 3
期刊
Journal of Paediatric Surgeons of Bangladesh
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