Short Bowel Syndrome as the Leading Cause of Intestinal Failure in Early Life: Some Insights into the Management / 대한소아소화기영양학회지

Intestinal failure (IF) is the critical reduction of the gut mass or its function below the minimum needed to absorb nutrients and fluids required for adequate growth in children. Severe IF requires parenteral nutrition (PN). Pediatric IF is most commonly due to congenital or neonatal intestinal diseases or malformations divided into 3 groups: 1) reduced intestinal length and consequently reduced absorptive surface, such as in short bowel syndrome (SBS) or extensive aganglionosis; 2) abnormal development of the intestinal mucosa such as congenital diseases of enterocyte development; 3) extensive motility dysfunction such as chronic intestinal pseudo-obstruction syndromes. The leading cause of IF in childhood is the SBS. In clinical practice the degree of IF may be indirectly measured by the level of PN required for normal or catch up growth. Other indicators such as serum citrulline have not proven to be highly reliable prognostic factors in children. The last decades have allowed the development of highly sophisticated nutrient solutions consisting of optimal combinations of macronutrients and micronutrients as well as guidelines, promoting PN as a safe and efficient feeding technique. However, IF that requires long-term PN may be associated with various complications including infections, growth failure, metabolic disorders, and bone disease. IF Associated Liver Disease may be a limiting factor. However, changes in the global management of IF pediatric patients, especially since the setup of intestinal rehabilitation centres did change the prognosis thus limiting “nutritional failure” which is considered as a major indication for intestinal transplantation (ITx) or combined liver-ITx.

Multidisciplinary Intestinal Rehabilitation for Short Bowel Syndrome in Adults: Results in a Korean Intestinal Rehabilitation Team

PURPOSE: Intense multidisciplinary team effort is required for the intestinal rehabilitation of patients afflicted with the short bowel syndrome (SBS). These include enteral and parenteral nutrition (PN) support, monitoring of complications related to treatment, and considering further medical or surgical options for intestinal adaptation. METHODS: In the Intestinal Rehabilitation Team (IRT) at the Samsung Medical Center, we have experienced 20 cases of adult SBS requiring multidisciplinary intestinal rehabilitation. This study is a retrospective review of the collected medical records. RESULTS: Of the 20 subjects treated, 12 patients were male and 8 patients were female. At the time of referral to the IRT, the mean age was 51.5 years, and the mean body weight was 50.1 kg, which was 90% of the usual body weight. The diseases or operative managements preceding massive bowel resection were malignancy in 11 cases, cardiac surgery in 2 cases, trauma in 2 cases and one case, each of tuberculosis, corrosive esophagitis, atrial fibrillation, simultaneous pancreas and kidney transplantation, and perforated appendicitis. Of these, there were 14 survivals and 6 mortalities. The fatalities were attributed to progression of disease, intestinal failure-associated liver disease, and sepsis (unrelated to intestinal failure) (2 cases each). Among the 14 surviving patients, 8 patients have been weaned off PN, whereas 6 are still dependent on PN (mean PN dependence 36%). CONCLUSION: This paper reports the results of multidisciplinary intestinal rehabilitation of adult short bowel patients treated at the Samsung Medical Center. Further studies are required to improve survival and enteral tolerance of these patients.

Treatment of short bowel syndrome in children. Value of the Intestinal Rehabilitation Program / Tratamento da síndrome do intestino encurtado na criança: valor do Programa de Reabilitação Intestinal

Summary The main cause of acute intestinal failure is short bowel syndrome, generally as a result of resection of extensive segments of small intestine. As a result, the main symptoms are watery diarrhea, malabsorption syndrome, chronic malnutrition, and death, if the patient is not properly treated. If the length of the remaining intestine is greater than 30 cm, complete adaptation is possible and the patient may not require parenteral nutrition. The currently recommended treatment includes the use of prolonged parenteral nutrition and enteral nutrition, always aimed at constant weight gain, in conjunction with surgeries aimed at elongating the dilated bowel. This set of procedures constitutes what is called an Intestinal Rehabilitation Program. This therapy was used in 16 children in periods ranging from 8 months to 7.5 years, with survival in 75% of the cases. Finally, the last resort to be used in children with complete resection of the small bowel is an intestinal transplant. However, to date there is no record of a Brazilian child that has survived this procedure, despite it being attempted in seven patients. We conclude that the results of the intestinal rehabilitation program are encouraging for the continuation of this type of treatment and stimulate the creation of the program in other pediatric care institutions.

Resumo A principal causa da falência intestinal aguda é a síndrome do intestino encurtado, decorrente, em geral, de ressecção de extensos segmentos de intestino delgado. Em consequência, os principais sintomas são diarreia aquosa, síndrome de má absorção, desnutrição crônica e óbito, caso o paciente não seja adequadamente tratado. Se o comprimento do intestino remanescente for superior a 30 cm, poderá haver adaptação completa e o paciente poderá ficar livre da nutrição parenteral. O tratamento atualmente preconizado inclui a utilização de nutrição parenteral prolongada e de nutrição enteral, objetivando sempre o ganho ponderal constante, em paralelo a cirurgias que visem ao alongamento do intestino dilatado. Esse conjunto de procedimentos constitui o que se denomina Programa de Reabilitação Intestinal. Essa terapia foi utilizada em 16 crianças, em períodos que variaram de 8 meses a 7 anos e meio, com sobrevida em 75% dos casos. O último recurso utilizado em crianças com ressecção completa do intestino delgado é o transplante intestinal. Até o momento, não há registro de criança brasileira que tenha sobrevivido a esse procedimento, a despeito de sete pacientes terem sido submetidos a ele. Os resultados do Programa de Reabilitação Intestinal nos anima a continuar com esse tipo de tratamento e estimular a criação do programa em outras instituições de atendimento pediátrico.

Nutrition Therapy for Adult Intestinal Failure Patients

Intestinal failure (IF) is a condition, in which the intestinal function or length remaining is below the minimum amount required for the absorption of sufficient nutrients and fluid to maintain normal life. The nutritional supply of IF depends on the anatomical site, length, and function of the remaining bowel. The goals of nutritional therapy for patients with IF are to achieve bowel adaptation to absorb nutrients sufficiently to live a healthy life with the current intestinal condition, and to promote the enteral autonomy to control nutrient digestion, absorption, excretion, and bowel movement. To stabilize and recover the patient's nutrition condition after a huge bowel resection, the intestinal rehabilitation team (IRT) for individual nutritional therapy should be established. IRT carefully monitors the changes in body weight, medication use, patient's symptoms, nutrient deficiency, hydration status, function of the remaining bowel, degree of bowel adaptation, adverse effects due to nutritional therapy, and enteral balance. To achieve intestinal adaptation and enteral autonomy through complicated and difficult nutritional intensive therapy in IF patients, it is essential to manage the patients through multidisciplinary collaboration involving physicians, pharmacists, dietitians, and nurses.

Quality Improvement Activities for Establishment of Intestinal Rehabilitation in Intestinal Failure Patients

PURPOSE: Intestinal failure (IF) is a complex clinical condition requiring a multi-disciplinary team approach. Our objective was to set up the treatment protocols and education documents for IF patients for development of intestinal rehabilitation programs in our hospital. METHODS: We compared the number of inpatients, length of hospital stay, mode of nutrition and calorie supply at discharge, and the frequency of blood transfusions before and after quality improvement of multidisciplinary activities, in order to evaluate the indirect effects of new protocols and training materials and for development of the intestinal rehabilitation system. RESULTS: We integrated eleven protocols for treatment and monitoring and seven educational materials for patients and caregivers. We compared indirect effects before and after the quality improvement activities. The number of IF patients hospitalized was reduced from 12 to 9. The mean days of hospital stay was decreased from 322 days to 73 days, the average number of monthly blood transfusions was also reduced from 1.8 to 0.3. In addition, the percentage of patients administered enteral nutrition and calories supplied was increased at discharge. CONCLUSION: By integrating IF protocols and education materials for IF patients, we found possible indirect effects of intestinal rehabilitation using a multidisciplinary team approach.

Intestinal rehabilitation in the management of acute gastrointestinal injury / 中华消化外科杂志

Acute gastrointestinal injury (AGI) is a malfunctioning of the gastrointestinal tract in intensive care patients due to their acute illness.AGI could be divided into 4 types,including AGI grade Ⅰ:increased risk of developing gastrointestinal dysfunction or failure; AGI grade Ⅱ:gastrointestinal dysfunction; AGI grade Ⅲ:gastrointestinal failure; AGI grade Ⅳ:gastrointestinal failure complicated with distal organ dysfunction.The symptoms of AGI include vomiting and regurgitation,gastric retention,diarrhea,gastrointestinal bleeding,paralytic intestinal obstruction,bowel dilatation and abnormal bowel sounds.Intestinal rehabilitation therapy was used to promote the patients with short bowel syndrome to restore enteral or oral feeding,and it could be used to improve the gastrointestinal function in patients with AGI.The procedure of intestinal rehabilitation therapy include total parenteral nutrition,parenteral and enteral nutrition,total enteral nutrition and oral feeding.The specific therapy could be implemented according to the severity of AGI and early enteral nutrition should be tried frequently.Intestinal tissue specific nutrients should also be provided either through enteral or parenteral approaches.