一例“极度短肠综合征”合并肠瘘的高营养护理

“短肠综合征”是外科严重并发症之一。它可导致消化和吸收面积不足,引起腹泻、脂肪泻、营养不良和体重减轻,继之病人出现恶病质而死亡。国内曾有报道小肠切除后,仅剩25cm,存活8年以上。我院1988年3月7日曾收治一例极短小肠(21cm)综合征合并严重肠瘘的病例,经过静脉高营养等各种保守治疗后,痊愈出院。     

短肠综合征时结肠的代偿研究

目的　观察及评价短肠大鼠结肠代偿性增生及结肠对营养物质吸收的促进作用。　方法　制作切除（80～85）%的超短肠大鼠模型,用百普素(Pepti-2000)做肠内营养（EN）治疗,观察全身营养状况和结肠形态学的改变,并在术后第21天用木糖和15N-甘氨酸混合液对带血管蒂的结肠进行封闭式灌注,观察结肠对水、碳水化合物和氨基酸的吸收情况。　结果　EN组于术后第21天净氮平衡与对照组（CONT）无差异,体重仅比术前减轻(10±18)g。结肠壁明显增厚,皱襞增大增粗,结肠壁的厚度、粘膜厚度、皱襞高度和皱襞表面积与CONT组相比差异具有非常显著性意义（P＜0.01）。EN组与CONT组相比DNA指数1.21±0.11vs.1.01±0.15(P＜0.05),S期细胞百分比（52.6±5.5）%vs.（42.9±4.1）%（P＜0.05）。连续循环灌注3h之后EN组对水、木糖和氨基酸的吸收明显高于CONT组（P＜0.05）。　结论　大鼠结肠在短肠综合征时发生了明显的形态和功能上的代偿。早期适当的肠内营养不但可使超短肠大鼠获得足够营养支持,并且能够促进短肠大鼠结肠代偿。     

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。     

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。     

肠康复治疗和短肠综合征

目的探讨对短肠综合征患者进行肠康复治疗的策略。方法采用文献复习的方法对肠康复治疗在短肠综合征患者中的应用加以综述。结果肠康复治疗是指重建肠道功能从而摆脱肠外营养的过程,通常包括膳食和内科保守治疗手段,有时还包括外科治疗。最近的研究显示,药物治疗、特需营养素、生长因子等的使用促进了肠代偿和吸收功能。结论肠康复治疗有益于短肠综合征患者的恢复,并将发挥更重要的作用。     

肠外瘘661例临床分析

１９７１～１９９２年共治疗６６１例肠外瘘，其中５０９例（７７％）为手术引起，其余为外伤、炎性肠道疾病、放射性损伤与肿瘤所致。经治疗后，２０５例（３１．０％）的瘘口自愈，３８６例（５８．４％）手术治愈，手术成功率为９８．４６％，全组死亡率为８．６２％。作者认为当前改善肠外瘘治疗效果的关键是控制感染，强调在早期进行有效的引流，重视对器官功能的监测与维护。肠外与肠内营养支持在肠外瘘治疗中可互补其不足。肠部分切除吻合与肠浆肌层片修补术是主要的手术方式。     

短肠综合征的肠外营养支持治疗

报告８例短肠综合征肠外营养支持。腹泻期平均５１．４天,腹泻量平均３．８Ｌ／ｄ,ＴＰＮ供热３０Ｋｃａｌ／ｋｇ,糖脂热卡比为１∶１,非蛋白热卡∶氮为１５０（Ｋｃａｌ）∶１（ｇ）,ＴＰＮ平均持续４９．４天。腹泻量＜２．５Ｌ／ｄ则逐渐过渡为口服肠内营养制剂。据腹泻量及血生化检查,确定Ｋ＋、Ｎａ＋及ＨＣＯ－３输入量,矫正低钾、低钠及代谢性酸中毒。回肠广泛切除要补钙、镁、磷制剂。本组无１例死亡。对肠外营养支持在短肠综合征的治疗价值及具体实施进行了讨论。     

肠外瘘661例临床分析

１９７１－１９９２年共治疗６６１例肠外瘘，其中５０９例（７７％）为手术引起，其余为外伤，炎性肠道疾病，放射性损伤与肿瘤所致。经治疗后，２０５例（３１．０％）的瘘口自愈，３８６例（５８．４％）手术治愈，手术成功率为９８．４６％，全组死亡率为８．６２％，作者认为当前改善肠外瘘治疗效果的关键是控制感染，强调在早期进行有产的引流，重视对器官功能的监测与维护。肠外与肠内营养支持在肠外瘘治疗中可互补其不足。肠     

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     

益气生津法结合胃肠外营养治疗短肠综合征2例

益气生津法结合胃肠外营养治疗短肠综合征２例张静吉吉朱培庭沈平刘铭升王伟良上海中医药大学龙华医院（上海２０００３２）短肠综合征是指小肠广泛切除（小肠切除量超过７０％或残留肠管长度少于１００ｃｍ）术后出现严重的消化道吸收和全身营养障碍的临床综合征〔１，２...     

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。     

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.     

谷氨酰胺和生长激素对短肠综合征患者肠道代偿作用

目的探讨谷氨酰胺和生长激素对短肠综合征(SBS)患者的肠道代偿作用。方法26例短肠综合征患者残余小肠长度为0～100(中位数42.5)cm,手术后接受肠外营养(PN)支持3-52个月,联合应用生长激素(GH)(0.10±0.06)mg·kg-1·d-1和谷氨酰胺(GLN)(0.30±0.17)g·kg-1·d-1进行肠道促代偿治疗。结果26例接受GH加GLN治疗的SBS患者,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(6.0±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维持。结论经过合适的营养支持和肠道促代偿治疗,大多数短肠综合征患者残留肠道能充分代偿,完全摆脱PN或减少PN用量,长期健康生存。     

Relationship between biopsy-proven parenteralnutrition-associated liver fibrosis and biochemical cholestasis in children with short bowel syndrome

Purpose

The aim of the study was to determine the frequency of biochemical cholestasis (direct bilirubin [DB] ≥2 mg/dL) in children with short bowel syndrome and biopsy-proven parenteral nutrition (PN)-associated liver disease and to define predictive factors for the occurrence and degree of hepatic fibrosis.

Methods

After institutional review board approval, a retrospective review was conducted of patients followed by 2 multidisciplinary intestinal rehabilitation programs between January 1, 2000, and September 30, 2008. Inclusion criteria were exposure to PN (>30 days) and having undergone a liver biopsy. Liver biopsy specimens were graded from 0 to 3 based upon degree of fibrosis in the pathology report. The most recent DB within 10 days before biopsy was recorded.

Results

A total of 66 children underwent 83 liver biopsy procedures. The most common diagnoses included necrotizing enterocolitis (NEC) (36.4%), gastroschisis (22.7%), and intestinal atresia (15.1%). Median age at biopsy was 6.1 months with a median duration of PN of 4.7 months. Of the patients, 70.3% had a history of exposure to parenteral ω-3 lipid emulsion. Of the liver biopsy specimens, 89% (74/83) demonstrated some degree of fibrosis (fibrosis scale 1-3), including 9.6% (8/83) with evidence of cirrhosis. 83% of biopsies without fibrosis and 55% of biopsies with fibrosis were obtained in patients without evidence of biochemical cholestasis (P = .20). Three (37%) of the 8 patients with cirrhosis on liver biopsy had no evidence of biochemical cholestasis. Univariate analysis identified only gestational age (GA) at birth as significantly associated with the degree of liver fibrosis (P = .03). A multivariate logistic regression model accounting for multiple biopsy procedures in patients revealed that GA was a predictor of fibrosis only in patients with a diagnosis other than NEC (P < .01).

Conclusions

In children with short bowel syndrome, biochemical cholestasis does not reflect the presence or degree of histologically confirmed PN-associated liver fibrosis. Careful follow-up, combined with further refinement of diagnostic and hepatoprotective strategies, may be warranted in this patient population.     

Neonatal short bowel syndrome: a cohort study

Background

To date, our knowledge of morbidity and mortality in neonatal short bowel syndrome (SBS) is based on individual case series. Shortcomings of the published literature include long patient recruitment time, selection bias, variable SBS definitions, failure to account for gestational age, and incomplete follow-up. By applying more rigorous methodology, our aim was to determine outcomes of SBS neonates compared with a control group of neonates without SBS.

Methods

A cohort study of all neonates with abdominal pathology requiring laparotomy between January 1, 1997, and December 31, 1998, with observation through July 1, 2001. Short bowel syndrome was defined as patients requiring parenteral nutrition for more than 42 days or residual small bowel length of less than 25% predicted by gestational age. Student's t test, Mann-Whitney U test, and χ² were used where appropriate. Kaplan-Meier curves were used to determine cumulative survival. Covariates important in the development of SBS were examined using forward step-wise logistic regression.

Results

There were 175 patients (with SBS = 40, without SBS = 135) with a mean gestational age of 30.7 ± 4.6 weeks vs 35.9 ± 4.8 weeks, respectively (P < .0005). The patients with SBS suffered significantly more morbidity than the group without SBS in all categories of investigation (surgical complications, septic events, central venous line complications, duration to adaptation and parenteral nutrition independence, cholestasis and liver failure, and duration of hospitalization). The case fatality rate was 37.5% in patients with SBS vs 13.3% in patients without SBS (P = .001). Most of the deaths were caused by liver failure or sepsis and occurred within 1 year from the date of surgery. Presence of an ileostomy (exp(B) = 12.29; P < .0005) and a residual small bowel length less than 50% of the original length (exp(B) = 26.84; P < .0005) were the only 2 variables in a logistic regression analysis found to be independently associated with the development of SBS.

Conclusion

This cohort study clearly illustrates the tremendous morbidity experienced by infants with SBS relative to other surgical neonates. Accurate estimates of the morbidity associated with SBS enables clinicians to appropriately counsel parents, allocate resources and initiate therapeutic trials.     

Cryptosporidium parvum diarrhea in an infant with short bowel syndrome

Cryptosporidium parvum is an underdiagnosed cause of diarrhea in children. The case of a 1-year-old girl with short bowel syndrome presenting with severe dehydrating diarrhea with a protozoon named C parvum is reported. Although the resection of the small bowel in this patient seemed to cause this severe infection with C parvum, more cases are needed to include the resection of the small bowel as a risk factor for Cryptosporidium infection and/or for a more severe form of diarrhea. Awareness of this infection among clinicians will help to diagnose this infection since special acid fast staining is made on special request.