新生儿胎粪性腹膜炎30例临床分析

目的 探讨新生儿胎粪性腹膜炎的临床特点和治疗经验.方法 回顾性分析1999年至2007年收治的30例胎粪性腹膜炎新生儿的临床表现、影像学特点、手术所见及治疗方法.结果 30例胎粪性腹膜炎患儿产前超声检查最常见的表现为胎儿腹水及肠管扩张.7例保守治疗,17例行肠切除肠吻合术,3例只行腹腔引流术,3例行回肠造瘘术.胎粪性腹膜炎常见型13例,纤维黏连型8例,囊肿型2例.病死率6.7%,并发症发生率36.7%.结论 新生儿胎粪性腹膜炎病死率高,超声检查有助于早期诊断.一期肠切除肠吻合术或肠造瘘术可取得较好的治疗效果.     

新生儿胎粪性腹膜炎30例临床分析

目的 探讨新生儿胎粪性腹膜炎的临床特点和治疗经验.方法 回顾性分析1999年至2007年收治的30例胎粪性腹膜炎新生儿的临床表现、影像学特点、手术所见及治疗方法.结果 30例胎粪性腹膜炎患儿产前超声检查最常见的表现为胎儿腹水及肠管扩张.7例保守治疗,17例行肠切除肠吻合术,3例只行腹腔引流术,3例行回肠造瘘术.胎粪性腹膜炎常见型13例,纤维黏连型8例,囊肿型2例.病死率6.7%,并发症发生率36.7%.结论 新生儿胎粪性腹膜炎病死率高,超声检查有助于早期诊断.一期肠切除肠吻合术或肠造瘘术可取得较好的治疗效果.     

新生儿胎粪性腹膜炎30例临床分析

目的 探讨新生儿胎粪性腹膜炎的临床特点和治疗经验.方法 回顾性分析1999年至2007年收治的30例胎粪性腹膜炎新生儿的临床表现、影像学特点、手术所见及治疗方法.结果 30例胎粪性腹膜炎患儿产前超声检查最常见的表现为胎儿腹水及肠管扩张.7例保守治疗,17例行肠切除肠吻合术,3例只行腹腔引流术,3例行回肠造瘘术.胎粪性腹膜炎常见型13例,纤维黏连型8例,囊肿型2例.病死率6.7%,并发症发生率36.7%.结论 新生儿胎粪性腹膜炎病死率高,超声检查有助于早期诊断.一期肠切除肠吻合术或肠造瘘术可取得较好的治疗效果.     

新生儿胎粪性腹膜炎30例临床分析

目的 探讨新生儿胎粪性腹膜炎的临床特点和治疗经验.方法 回顾性分析1999年至2007年收治的30例胎粪性腹膜炎新生儿的临床表现、影像学特点、手术所见及治疗方法.结果 30例胎粪性腹膜炎患儿产前超声检查最常见的表现为胎儿腹水及肠管扩张.7例保守治疗,17例行肠切除肠吻合术,3例只行腹腔引流术,3例行回肠造瘘术.胎粪性腹膜炎常见型13例,纤维黏连型8例,囊肿型2例.病死率6.7%,并发症发生率36.7%.结论 新生儿胎粪性腹膜炎病死率高,超声检查有助于早期诊断.一期肠切除肠吻合术或肠造瘘术可取得较好的治疗效果.     

新生儿胎粪性腹膜炎30例临床分析

目的 探讨新生儿胎粪性腹膜炎的临床特点和治疗经验.方法 回顾性分析1999年至2007年收治的30例胎粪性腹膜炎新生儿的临床表现、影像学特点、手术所见及治疗方法.结果 30例胎粪性腹膜炎患儿产前超声检查最常见的表现为胎儿腹水及肠管扩张.7例保守治疗,17例行肠切除肠吻合术,3例只行腹腔引流术,3例行回肠造瘘术.胎粪性腹膜炎常见型13例,纤维黏连型8例,囊肿型2例.病死率6.7%,并发症发生率36.7%.结论 新生儿胎粪性腹膜炎病死率高,超声检查有助于早期诊断.一期肠切除肠吻合术或肠造瘘术可取得较好的治疗效果.     

新生儿胎粪性腹膜炎30例临床分析

目的 探讨新生儿胎粪性腹膜炎的临床特点和治疗经验.方法 回顾性分析1999年至2007年收治的30例胎粪性腹膜炎新生儿的临床表现、影像学特点、手术所见及治疗方法.结果 30例胎粪性腹膜炎患儿产前超声检查最常见的表现为胎儿腹水及肠管扩张.7例保守治疗,17例行肠切除肠吻合术,3例只行腹腔引流术,3例行回肠造瘘术.胎粪性腹膜炎常见型13例,纤维黏连型8例,囊肿型2例.病死率6.7%,并发症发生率36.7%.结论 新生儿胎粪性腹膜炎病死率高,超声检查有助于早期诊断.一期肠切除肠吻合术或肠造瘘术可取得较好的治疗效果.     

新生儿胎粪性腹膜炎30例临床分析

目的 探讨新生儿胎粪性腹膜炎的临床特点和治疗经验.方法 回顾性分析1999年至2007年收治的30例胎粪性腹膜炎新生儿的临床表现、影像学特点、手术所见及治疗方法.结果 30例胎粪性腹膜炎患儿产前超声检查最常见的表现为胎儿腹水及肠管扩张.7例保守治疗,17例行肠切除肠吻合术,3例只行腹腔引流术,3例行回肠造瘘术.胎粪性腹膜炎常见型13例,纤维黏连型8例,囊肿型2例.病死率6.7%,并发症发生率36.7%.结论 新生儿胎粪性腹膜炎病死率高,超声检查有助于早期诊断.一期肠切除肠吻合术或肠造瘘术可取得较好的治疗效果.     

新生儿胎粪性腹膜炎30例临床分析

目的 探讨新生儿胎粪性腹膜炎的临床特点和治疗经验.方法 回顾性分析1999年至2007年收治的30例胎粪性腹膜炎新生儿的临床表现、影像学特点、手术所见及治疗方法.结果 30例胎粪性腹膜炎患儿产前超声检查最常见的表现为胎儿腹水及肠管扩张.7例保守治疗,17例行肠切除肠吻合术,3例只行腹腔引流术,3例行回肠造瘘术.胎粪性腹膜炎常见型13例,纤维黏连型8例,囊肿型2例.病死率6.7%,并发症发生率36.7%.结论 新生儿胎粪性腹膜炎病死率高,超声检查有助于早期诊断.一期肠切除肠吻合术或肠造瘘术可取得较好的治疗效果.     

新生儿胎粪性腹膜炎30例临床分析

目的 探讨新生儿胎粪性腹膜炎的临床特点和治疗经验.方法 回顾性分析1999年至2007年收治的30例胎粪性腹膜炎新生儿的临床表现、影像学特点、手术所见及治疗方法.结果 30例胎粪性腹膜炎患儿产前超声检查最常见的表现为胎儿腹水及肠管扩张.7例保守治疗,17例行肠切除肠吻合术,3例只行腹腔引流术,3例行回肠造瘘术.胎粪性腹膜炎常见型13例,纤维黏连型8例,囊肿型2例.病死率6.7%,并发症发生率36.7%.结论 新生儿胎粪性腹膜炎病死率高,超声检查有助于早期诊断.一期肠切除肠吻合术或肠造瘘术可取得较好的治疗效果.     

新生儿胎粪性腹膜炎30例临床分析

目的 探讨新生儿胎粪性腹膜炎的临床特点和治疗经验.方法 回顾性分析1999年至2007年收治的30例胎粪性腹膜炎新生儿的临床表现、影像学特点、手术所见及治疗方法.结果 30例胎粪性腹膜炎患儿产前超声检查最常见的表现为胎儿腹水及肠管扩张.7例保守治疗,17例行肠切除肠吻合术,3例只行腹腔引流术,3例行回肠造瘘术.胎粪性腹膜炎常见型13例,纤维黏连型8例,囊肿型2例.病死率6.7%,并发症发生率36.7%.结论 新生儿胎粪性腹膜炎病死率高,超声检查有助于早期诊断.一期肠切除肠吻合术或肠造瘘术可取得较好的治疗效果.     

Oesophageal atresia and associated anomalies.

Of 253 infants with oesophageal atresia treated over an eight year period, 122 (48%) had a total of 213 other anomalies. Most commonly affected were the cardiovascular (61 cases, 29%), anorectal (30 cases, 14%), and genitourinary (29 cases, 14%) systems. The VATER (or VACTERL) association was present in 10% of cases, but occurred more often in patients who had oesophageal atresia without an associated tracheo-oesophageal fistula (3/13, 23%). The level of the associated anorectal malformation was not associated with the type of oesophageal atresia. The presence and severity of other anomalies did not influence the basic approach to treatment of the oesophageal atresia--that is, primary repair whenever possible. Despite aggressive treatment, cardiac malformations were the most common cause of death. There were five infants with the CHARGE association, two with Potter''s syndrome, and two with ''SCHISIS'' syndrome (cleft lip and palate, omphalocoele, and hypogenitalism).     

Clinical experience of complex jejunal atresia

Purpose

Small intestinal atresia is relatively common anomaly that causes intestinal obstruction in neonates. Although surgical interventions are usually successful, critical problems could raise in certain cases. This study aimed to identify the distinct clinical characteristics of complex cases of jejunal atresia by retrospective analysis.

Methods

Overall, 91 cases of small intestinal atresia, which occured in infants between 2001 and 2010 at Pusan National University Children’s Hospital, were reviewed retrospectively. The clinical characteristics of complex jejunal atresia were analyzed.

Results

Of the 91 small intestinal atresias, 11 cases of complex jejunal atresia were found: high jejunal atresia with distal deletion, 3; high jejunal atresia with distal multiple atresias, 4; jejunal atresia with distal apple peel appearance, 1; jejunal atresia with colonic atresia, 1; jejunoileal atresia with distal volvulus, 2. Short bowel syndrome was found in four patients and bowel-lengthening procedure was performed in all. Three patients presented with an adhesive intestinal obstruction during the early postoperative period. Postoperative mortality occurred in one patient with distal volvulus.

Conclusions

From a surgical perspective, complex jejunal atresia can cause many critical problems after the correction operation. An aggressive and multidisciplinary approach is necessary for managing this condition.     

Oesophageal atresia in premature infants: an analysis of morbidity and mortality over a period of 20 years

AIM: To determine the morbidity and mortality of premature infants born with oesophageal atresia (OA) and to evaluate historical changes in morbidity and mortality over time. METHODS: Retrospective analysis of morbidity and mortality of all patients admitted for OA, with or without tracheo-oesophageal fistula, between 1982 and 2002. RESULTS: The study group consisted of 197 consecutive patients, of whom 55 (28%) were premature and 21 (11%) very premature. Type A atresia was found more often in very premature and premature infants than in those born at term (p = 0.02). Type E atresia was not found in the premature group (p = 0.004). At least one associated congenital anomaly was also present in 121 patients (61%). Postoperative complications developed more often in very premature and premature infants than in those born at term (p < 0.001). Gastro-oesophageal reflux was diagnosed in 32/76 premature infants and in 41/121 term infants (p = 0.001). Mortality among very premature and premature infants was higher than among those born at term (p = 0.003). Withdrawal of treatment was the most frequent cause of death. CONCLUSION: Premature infants with OA have a higher morbidity and mortality than term infants with OA. The complications of prematurity contribute significantly to morbidity and mortality in premature infants with OA. There is no reason to refrain from the standard treatment of OA in premature infants with no severe associated congenital anomalies.     

The Canadian Biliary Atresia Registry: Improving the care of Canadian infants with biliary atresia

Biliary atresia is the most common cause of end-stage liver disease and liver cirrhosis in children, and the leading indication for liver transplantation in the paediatric population. There is no cure for biliary atresia; however, timely diagnosis and early infant age at surgical intervention using the Kasai portoenterostomy optimize the prognosis. Late referral is a significant problem in Canada and elsewhere. There is also a lack of standardized care practices among treating centres in this country. Biliary atresia registries currently exist across Europe, Asia and the United States. They have provided important evidence-based information to initiate changes to biliary atresia care in their countries with improvements in outcome. The Canadian Biliary Atresia Registry was initiated in 2013 for the purpose of identifying best standards of care, enhancing public education, facilitating knowledge translation and advocating for novel national public health policy programs to improve the outcomes of Canadian infants with biliary atresia.     

婴儿巨细胞病毒感染与胆道闭锁的关系

目的　探讨巨细胞病毒 (CMV)感染与胆道闭锁的关系 ,了解婴儿CMV肝炎并胆道闭锁的临床特点。方法　对确诊为CMV感染并胆道闭锁 1 6例患儿的临床资料进行回顾性分析 ,并与同期诊断为单纯CMV肝炎 2 9例患儿进行比较。结果　CMV感染并胆道闭锁患儿血清CMV IgM抗体和外周血多形核白细胞中CMV pp65抗原均阳性 9例 ,IgM阳性 1例 ,仅pp65阳性3例 ,IgM和pp65均阴性 3例 (但其肝组织CMV pp65阳性 )。 1 5例肝组织标本中CMV pp65阳性 1 4例。CMV感染并胆道闭锁患儿各项指标明显重于有黄疸的单纯CMV肝炎 (P均 <0 .0 5) ,肝组织病理检查 1 5例显示胆小管增生伴肝纤维化 ,继发性胆汁性肝硬化 2例。结论　CMV感染可同时累及肝细胞和胆管上皮细胞 ,导致胆管闭锁。对以胆汁淤积为主要表现且已明确为CMV感染患儿应警惕是否并胆道闭锁 ,避免丧失手术治疗机会。     

Clinical aspects on neonatal cholestasis based on observations at a Swedish tertiary referral centre

The aim of the study was to investigate the clinical aspects of neonatal cholestasis. The medical records of 85 cholestatic infants were retrospectively reviewed. A majority of the patients were referred from other parts of the country. The most common diagnoses were extrahepatic biliary atresia (n = 30 patients), alpha₁-antitrypsin deficiency (n = 11) and progressive familial intrahepatic cholestasis (n = 11). On presentation, the biliary atresia group had higher mean serum values of bilirubin, G-GT and cholesterol than the patients with intrahepatic cholestasis, with no significant differences noticed for any other biochemical parameter. A lack of excretion on hepatobiliary scintigraphy was noticed in all investigated patients with biliary atresia, but also in 9 of 34 patients with intrahepatic neonatal cholestasis. There was no statistical correlation between the age at portoenterostomy and the outcome in patients with biliary atresia. However, both the detection of a partial flow on perioperative cholangiogram and the establishment of a non-icteric phase within 6 mo after the portoenterostomy correlated to a good outcome. Eight of 11 patients with progressive familial intrahepatic cholestasis were treated with a biliary diversion procedure, five of eight experienced a sustained cholestatic remission. Conclusions: Progressive familial intrahepatic cholestasis may be a more common cause of neonatal cholestasis in Sweden than reported elsewhere and that the experience with biliary diversion is positive. While early referral in patients with extrahepatic biliary atresia remains important, a portoenterostomy should be attempted also in patients referred after 3 mo of age.     

Intestinal absorption of calcium and magnesium in hepatobiliary disease in infancy

The intestinal absorption of calcium and magnesium was measured by metabolic balance studies in 6 normal infants, 13 infants with biliary atresia, 5 infants with successfully repaired biliary atresia, 7 infants with neonatal hepatitis, and 2 infants with choledochal cyst. The absorption of both elements was impaired in these disorders. The malabsorption of these elements was most marked in biliary atresia. In successfully repaired biliary atresia the absorption was increased to the normal levels. In neonatal hepatitis the degree of the malabsorption was variable in individual cases. In choledochal cyst the reduction of the absorption was less marked than in biliary atresia and neonatal hepatitis. In biliary atresia parenteral vitamin D increased moderately the absorption of both elements, though oral vitamin D had little effect. In infants with biliary atresia receiving a milk containing medium-chain triglycerides the absorption was moderately raised. There was a clear relation between the absorption of calcium and that of magnesium: the per cent. absorption of magnesium was almost the same as that of calcium in most cases. The serum calcium level determined during the studies was within the normal ranges in hepatobiliary diseases. The serum magnesium level was, however, found to be generally reduced in these conditions. It was greatly reduced in the patients with biliary atresia.     

Portal hypertension.

Increased portal pressure is the product of both increased resistance to splanchnic flow through the liver and increased blood flow in the portal circuit. Although portal hypertension in children is less common than in adults, the important clinical end results are the same, ie, esophageal variceal hemorrhage, ascites, and hypersplenism. The etiology of portal hypertension in children is very different from adults in whom cirrhosis (most commonly secondary to alcohol) is the predominant cause. In children, extrahepatic obstruction due to portal vein thrombosis is the most common cause. However, as children survive longer with biliary atresia, cystic fibrosis, and other liver diseases, the incidence of intrahepatic obstruction causing portal hypertension is increasing. The treatment has also undergone a dramatic evolution over the last decade with the near extinction of portosystemic shunt procedures and their replacement with endoscopic treatment of esophageal varices and liver transplantation.     

血小板活化因子在新生儿坏死性小肠结肠炎发病中的作用

坏死性小肠结肠炎是新生儿,尤其是早产儿或极低出生体重儿最常见的胃肠道急症,至今仍是早产儿发病和死亡最常见的原因.大量流行病学、动物实验模型及临床研究发现NEC是与早产、配方奶喂养、肠缺血缺氧、细菌异常增殖等多种因素相互作用的结果,但其确切的发病机制尚不完全清楚.目前有研究者通过模拟上述因素建立NEC动物模型,发现模型鼠中NEC组回肠中血小板活化因子水平较对照组明显升高,予以PAF拮抗剂预处理后NEC的发病率显著降低,表明PAF在NEC发病中有关键作用.PAF通过与其受体特异性结合后,可激活多条信号传导通路,导致多种炎症介质合成与释放增加;肠道黏膜屏障受损,大量毒素吸收,进一步增加内源性PAF合成;通过依赖线粒体途径凋亡肠上皮细胞;激活核因子NF-κB通路放大级联炎症反应;诱导活性氧产生,引起细胞凋亡导致肠管坏死.