短肠综合征患者家庭肠外营养体验的质性研究

目的 了解短肠综合征患者行家庭肠外营养的真实体验,为临床制订有效的护理干预方案提供依据。方法 目的抽样法选取12例短肠综合征行家庭肠外营养患者,对其进行半结构式访谈,并用Colaizzi 7步法分析资料。结果 提炼出3个主题：家庭肠外营养的积极体验（改善营养状况、获得家庭归属感、自我管理意识及能力增强）;家庭肠外营养的消极体验（睡眠障碍、活动受到限制、伴随和/或潜在的并发症、负性心理情绪）;家庭肠外营养患者的需求（对专业医护人员培训及指导的需求、对医疗资源及政策支持的需求）。结论 医护人员应重视患者的负性体验,通过有效干预方法解决患者在家庭肠外营养支持过程中出现的问题,以保障家庭肠外营养支持顺利安全实施。     

实施长期肠外营养和家庭肠外营养的体会

为探讨长期肠外营养和家庭肠外营养的实施。我们采用经中心静脉导管输全合一营养液行长期肠外营养30例，其中10例经住院治疗后转为家庭肠外营养。每例行肠外营养6～120个月，平均12.4个月。10例家庭肠外营养者每例行家庭肠外营养6～114个月，平均95．2个月。共置上腔静脉导管71根，每根留置时间1～18个月，平均4．6个月。平均每留管26．3个月发生1次导管性感染。我们认为长期肠外营养和家庭肠外营养是肠衰竭病人维护生命的有价值治疗，可在我国安全、有效地应用。     

富含谷氨酰胺二肽的新型氨基酸对全肠外营养短肠综合征大？…

目的 观察富含谷氨酰胺二肽的新型氨基酸对Ｗｉｓｔａｒ大鼠短肠模型血清蛋白和肝功能的影响及探讨其减轻脂肪乳剂引起肝损害的作用机制。方法 ３０只Ｗｉｓｔａｒ雄性在鼠随机分为富含谷氨酰胺二肽新型氨基酸组（２０ＡＡ组）、标准组（１７ＡＡ组）、无氮组,观察不同组动物之间血清总蛋白、白蛋白、前白蛋白（ｐｒｅ－ａｌｂｕｍｉｎ,ＰＡ）、纤维结合蛋白（ｆｉｂｒｏｎｅｃｔｉｎ,ＦＮ）以及丙氨酸转氮酶和胆红素的变化。结     

富含谷氨酰胺二肽的新型氨基酸对全肠外营养短肠综合征大鼠血清蛋白及肝功能的影响

目的观察富含谷氨酰胺二肽的新型氨基酸对Wistar大鼠短肠模型血清蛋白和肝功能的影响及探讨其减轻脂肪乳剂引起肝损害的作用机制.方法30只Wistar雄性大鼠随机分为富含谷氨酰胺二肽新型氨基酸组(20AA组)、标准组(17AA组)、无氮组,观察不同组动物之间血清总蛋白、白蛋白、前白蛋白(pre-albumin,PA)、纤维结合蛋白(fibronectin,FN)以及丙氨酸转氨酶和胆红素的变化.结果20AA组血清总蛋白、纤维结合蛋白与17AA组相比无明显差别,但高于无氮组(P＜0.05),白蛋白、前白蛋白明显高于17AA组和无氮组(P＜0.05);总胆红素和直接胆红素3组之间均有差别,20AA组最低(P＜0.05).结论富含谷氨酰胺二肽新型氨基酸能提高短肠综合征大鼠血清白蛋白、前白蛋白的含量,抑制全肠外营养(TPN)引起的肝内淤胆作用,减轻脂肪乳剂对肝功能损害,增强其生存能力.     

肠外营养相关性肝损伤机制及防治

肠外营养相关性肝损伤(PNALD)是持续肠外营养(PN)相关的严重代谢性并发症,也是常见并发症。其病理生理改变主要表现为胆汁淤积和肝脂肪浸润,临床上表现为肝酶异常、胆汁淤积和黄疸,严重者可导致肝功能发生不可逆的损害,甚至可引起肝衰竭及死亡,目前尚无早期识别方法。PNALD的相关机制尚不清楚,目前认为包括胆汁淤积、肠外营养制剂、肠道相关性感染。对于除外其他引起肝损的常见病因且有PN输注史的病人,可考虑诊断PNALD。PNALD的治疗原则是尽早恢复肠内营养,同时加用保肝药物。     

生长激素对短肠大鼠残留小肠形态及生长代谢的影响

目的　探讨单纯肠外营养 (PN)与添加生长激素 (GH)的PN对短肠大鼠残留小肠代偿的作用及作用机制。方法　将 2 0只短肠SD大鼠随机分成PN组及PN rhGH组。行细胞增殖核抗原 (PCNA)测定、原位末端标记 (TUNEL)染色及bcl 2、BaxmRNA测定。结果　PN组残留小肠粘膜明显萎缩 ,PN rhGH组肠萎缩显著改善。PCNA表达在PN组降低 [(8.37± 2 .2 3)个 /视野 ],PN rhGH组增高 [(19.2 8± 3.2 5 )个 /视野 ],差异有非常显著性 (P <0 .0 1)。凋亡指数在PN组增高 [(2 2 .32± 3.84)个 / 10 0细胞 ],PN rhGH组降低 [(8.0 6± 2 .2 3)个 / 10 0细胞 ],差异有非常显著性(P <0 .0 1)。bcl 2mRNA表达在PN组降低 (0 .2 0± 0 .0 3) ,在PN rhGH组增高 (0 .44± 0 .0 6 ) ,BaxmRNA表达则相反。结论　单纯PN使短肠大鼠残留小肠粘膜明显萎缩 ,rhGH通过促进肠粘膜上皮细胞增生与抑制肠粘膜上皮细胞凋亡 ,显著促进残留小肠的代偿适应。     

家庭肠外营养支持治疗

家庭肠外营养的历史及现状自1968年起,肠外营养(parenteral nutrition,PN)在全世界广泛实施[1-5]。其中,家庭肠外营养(home parenteral nutrition,HPN)将肠外营养的实施地点由医院转入家中,具有明显的社会效益与安全性。据统计,1992年美国已经有40 000例HPN病人,增长比例约为25%/年[6];欧洲肠外肠内营养学会(European Society for Parenteral and Enteral Nutrition,ESPEN)调查发现,1997年欧洲每100万人中仅有3~4例HPN,到2009年时,HPN实施率已达2~40/100万人[3]。     

Relationship between serum citrulline levels and progression to parenteral nutrition independence in children with short bowel syndrome

Objective

Although bowel length is an important prognostic variable used in the management of children with short bowel syndrome (SBS), reliable measurements can be difficult to obtain. Plasma citrulline (CIT) levels have been proposed as surrogate markers for bowel length and function. We sought to evaluate the relationship between CIT and parenteral nutrition (PN) independence in children with SBS.

Study Design

A retrospective chart review performed for all patients seen in a multidisciplinary pediatric intestinal rehabilitation clinic with a recorded CIT between January 2005 and December 2007 (n = 27).

Results

Median age at time of CIT determination was 2.4 years. Diagnoses included necrotizing enterocolitis (26%), intestinal atresias (19%), and gastroschisis (22%). Citrulline levels correlated well with bowel length (R = 0.73; P < .0001) and was a strong predictor of PN independence (P Wilcoxon = 0.002; area under the receiver operating characteristic curve = 0.88; 95% confidence interval, 0.75-1.00). The optimal CIT cutoff point distinguishing patients who reached PN independence was 15 μmol/L (sensitivity = 89%; specificity = 78%).

Conclusion

Plasma CIT levels are strong predictors of PN independence in children with SBS and correlate well with a patient's recorded bowel length. A cutoff CIT level of 15 μmol/L may serve as a prognostic measure in counseling patients regarding the likelihood of future PN independence.     

The role of enteral nutrition in the reversal of parenteral nutrition-associated liver dysfunction in infants

Background

Liver dysfunction in children dependent on parenteral nutrition (PN) is well established, and the extent of hyperbilirubinemia has been shown to correlate with morbidity and mortality. The aim of this study was to assess whether increasing provisions of enteral nutrition can improve PN-associated hyperbilirubinemia over time.

Methods

A retrospective review was conducted on infants in our institution's Short Bowel Syndrome Clinic from 1999 to 2004. Inclusion criteria included PN duration more than 1 month, serum direct bilirubin more than 3 mg/dL while on PN, and tolerance of full enteral nutrition with eventual discontinuation of PN. Paired t tests were used for statistical analyses.

Results

Twelve infants were identified with a PN duration of 5 ± 1 months. Five patients underwent liver biopsy while on PN, and histological evidence of cholestasis was found on all specimens. Peak total and direct bilirubin levels were 10.5 ± 1.9 and 7.0 ± 1.6 mg/dL, respectively, and occurred at time of PN discontinuation. Only 2 patients had improvement in serum bilirubin levels before initiation of full enteral nutrition. After initiation of full enteral nutrition and discontinuation of PN, all patients achieved permanent normalization of bilirubin levels by 4 months (P < .05) after a 1-month plateau phase. Alkaline phosphatase levels approached reference range within this time but were not significant.

Conclusion

These data demonstrate for the first time that although PN-dependent infants can achieve normalization of marked hyperbilirubinemia with enteral nutrition, the improvement in liver function usually begins only after full enteral nutrition is tolerated and PN is withdrawn. These findings support the aggressive weaning of PN to enteral nutrition in infants with short bowel syndrome.     

Massive small bowel resection in neonates—Is weaning from parenteral nutrition the final goal?

Eight surviving cases from an original 15 pediatric patients who underwent massive small bowel resection during their neonatal period were reviewed for a period of 2–19 years to assess long-term prognosis. the primary diseases were congenital intestinal atresia in 6 cases and midgut volvulus in 2. The length of the residual small intestine ranged from 27–75 cm and the ileocaecal valve had been resected in 3 cases. All cases were able to be weaned from parenteral nutrition and at present, 6 of the children can tolerate normal meals but 2 are still receiving enteral nutrition, in the form of a low residue diet at home. Near normal somatic growth was achieved in the most recent 5 cases who received intensive nutritional treatment in the immediate postoperative period, whereas 3 who did not receive nutritional management exhibited growth retardation. The D-Xylose absorption test revealed gradual improvement and finally normal absorption in all except one case with the shortest remaining intestine (27 cm). However, the fat absorption test revealed abnormal absorption in cases with a residual intestine of less than 45 cm. Late metabolic complications such as renal calculi, cholelithiasis and pathologic fractures were encoutered in 3 cases.     

短肠综合征病人的营养支持及肠道代偿治疗

目的探讨短肠综合征病人的营养支持以及肠道代偿、康复治疗体会。方法回顾性分析1986~2005年复旦大学附属中山医院64例短肠综合征病人治疗过程及随访情况,其中26例联合应用生长激素[GH,每天(0.10±0.06)mg/kg]和谷氨酰胺[GLN,每天(0.30±0.17)g/kg]进行肠道促代偿治疗。结果64例病人中死亡6例,存活58例,存活时间3个月至19年,平均(6.6±9.4)年。9例长期接受家庭肠外营养(HPN),13例接受部分PN或肠内营养(EN)支持,完全摆脱PN者36例,平均摆脱PN的时间为(8.6±14.2)个月。26例接受GH GLN治疗的SBS病人,其中9例(34.6%)治疗后近期内完全摆脱PN;8例(30.8%)经治疗后明显减少了PN用量[从每周需要PN支持(6.5±1.0)d下降至(4.2±1.0)d,每周PN需要量从(13.6±5.2)L降至(8.2±3.3)L];9例(34.6%)在治疗后仍依赖PN支持。64例共发生286次各种并发症。结论经过适宜的营养支持和肠道促代偿治疗,大多数短肠综合征病人的残留肠道可充分代偿,完全摆脱PN或减少PN用量,长期健康存活。     

Reversal of intestinal failure-associated liver disease in infants and children on parenteral nutrition: experience with 93 patients at a referral center for intestinal rehabilitation

Purpose

Intestinal failure (IF)-associated liver disease (IFALD) complicates the treatment of children with IF receiving parenteral nutrition (PN). We hypothesized that prevention or resolution of IFALD was possible in most children and that this would result in improved outcomes.

Methods

We reviewed prospectively gathered data on all children referred to the intestinal rehabilitation and transplantation center at our institution. Total bilirubin level (TB) was used as the marker for IFALD. Patients were grouped based on TB at referral and at subsequent inpatient stays and outpatient visits. Standard treatment consisted of cycling of PN, limiting lipid infusion, enteral stimulation, use of ursodeoxycholic acid, and surgical intervention when necessary. Outcomes such as mortality, dependence on PN, and need for transplantation were assessed. Statistical analyses were performed using Fisher's exact, Mann-Whitney U, and Wilcoxon signed rank tests.

Results

Ninety-three patients with intestinal failure and on PN were treated at our center from 2003 to 2009. Median age at referral was 5 months (0.5-264 months). Prematurity was a complicating factor in 63 patients and necrotizing enterocolitis was the most common diagnosis. Eighty-two children had short bowel syndrome, whereas the remaining 11 had extensive motility disorders. 97% of children required significant alteration of their PN administration. At referral, 76 of 93 children had TB 2.0 mg/dL or higher, and 17 had TB below 2.0 mg/dL. TB normalized in 57 of 76 children with elevated TB at referral, and TB remained elevated in 19. Normalization of TB was associated with a mortality of 5.2%, and transplantation was needed in 5.2%. Conversely, when TB remained elevated, mortality was 58% (P = .0002 vs TB normalized), and transplantation occurred in 58% owing to failure of surgical and medical rehabilitation.

Conclusions

Most children referred for treatment of IF have IFALD. A dedicated IF rehabilitation program can reverse IFALD in many children, and this is associated with improved outcome.     

短肠综合征患者的肠道代偿及康复治疗

目的 总结短肠综合征(SBS)的治疗经验。方法 分析38例SBS患者的治疗过程,随访其目前饮食情况,肠外营养(PN)或肠内营养(EN)的时间,了解并发症情况,对部分患者作有关检测,并联合应用生长激素(GH)和谷氨酰胺(GLN)治疗,采用稳定核素示踪检测残余肠道对单糖、脂肪酸及氨基酸的吸收情况。结果 本组患者死亡5例;存活33例,存活时间为6个月～17年,平均(5.9±4.3)年。目前有3例长期接受家庭PN,6例需部分或间歇性接受PN或EN补充,完全摆脱PN的有24例,其平均摆脱PN的时间为(9.5±6.6)个月。GH加GLN治疗只能在短时间内促进残余肠道对营养物质的吸收能力。结论 经过合适的肠道康复治疗,大多数SBS患者残留肠道能充分代偿,可完全摆脱PN或减少PN用量,长期健康地生存。     

短肠综合征的肠内营养支持

目的探讨短肠综合征患者肠内营养支持的临床意义、疗效及注意事项。方法回顾性总结1999至2005年收治的40例短肠综合征患者的临床资料。所有患者均存活至今,并随访2年以上。统计分析其肠内营养用量、费用、脱离肠外营养时间及目前营养状况。结果40例患者平均残存小肠（50．8±29．4）cm,脱离肠外营养平均时间为（29．1±9．2）个月。肠内营养用量为（3284．0±1408．8）kJ／d,其费用显著低于肠外营养（P〈0．01）。目前本组患者平均体质指数为（17．8±3．2）kg,／m^2,血红蛋白（113．3±14．8）g／L,血清白蛋白（35．0±4．1）g／L。平均大便次数为（3．4±1．7）次／d,平均大便量为（720．2±350．3）ml／d。结论肠内营养对于维持短肠综合征患者营养状况、减少并发症具有重要意义,但在具体实施时需掌握方法。     

低氮低热量肠外营养对胃肠道恶性肿瘤病人术后恢复的影响

目的 探讨胃肠道恶性肿瘤切除术后行低氮低热量肠外营养的临床效果.方法 将胃肠道恶性肿瘤切除手术病人120例随机分为对照组和研究组,分别接受传统肠外营养和低氮低热量肠外营养治疗,分析两组的血糖水平、氮平衡、血浆蛋白水平、免疫指标以及并发症的发生率.结果 经过术后6d的肠外营养支持治疗,研究组血糖明显低于对照组,术后并发症的发生率也明显降低.而在血浆蛋白水平和免疫指标方面两组没有显著性差异.结论 胃肠道恶性肿瘤切除术后行低氮低热量肠外营养有利于病人的恢复.