Intestinal adaptation and rehabilitation in adults with short bowel syndrome.

PURPOSE OF REVIEW: Over the past decade, trophic gastrointestinal hormonal factors have been included in the intestinal rehabilitation programs for short bowel syndrome (SBS). Up today the only trophic factor approved for clinical practice is the glucagon-like peptide-2 (GLP-2) analogue, teduglutide. A literature review on the last 2-year data on GLP-2 analogues for the treatment of SBS in adults has been performed. RECENT FINDINGS: Several reports on real-world data on the efficacy and safety of teduglutide treatment for SBS, some case-reports on the use of teduglutide in non-SBS conditions as well as phase 2 trials on new GL-2 analogues on patients with SBS have been retrieved. SUMMARY: Real-world data confirmed the teduglutide efficacy not only in weaning off IVS in accurately selected patients but also increased the alert on the risk of development of gastrointestinal polyps related to the drug; the impact of the therapy on patients' QoL deserves further studies and the cost-utility of the treatment is still uncertain. Some case reports highlighted the potential benefit of treatment with teduglutide in non-SBS gastrointestinal diseases, such as graft-versus-host disease, primary amyloidosis and refractory microscopic colitis. Phase 2 RCTs on safety and efficacy of two new long-acting GLP-2 analogues, glepaglutide and apraglutide, were published, and phase 3 RCTs have been completed.

Combined procedures for surgical short bowel syndrome: experience from two European centres.

BACKGROUND: Autologous gastrointestinal reconstructive surgery (AGIR) has become a key component of intestinal rehabilitation programs. However, the best surgical option for short bowel syndrome (SBS) remains unknown. This paper presents our experience using combined procedures as primary treatment. METHODS: We collected data on SBS patients who underwent surgery from 2008 to 2021 in two tertiary European Centres. Combined procedures were defined as more than one technique used on the same patient. Charts were reviewed for demographics, type of procedures, complications, and outcomes. Data are presented as median and IQR. Wilcoxon signed rank was used for all paired analyses. RESULTS: Twenty-one children (12 females) underwent combined procedures. Preoperative median small bowel length was 20 cm (IQR: 15-35 cm); after lengthening, it was 35.5 cm (IQR: 30.75-50.50 cm) (P < 0.001). Combined procedures were simultaneous in 15 patients and sequential in 6. At a median of 9.2 years (IQR: 7.55-9.78 years) follow-up, complications were three bowel obstructions after strictures of anastomosis and two wound infections. Two patients achieved enteral autonomy, and others followed a weaning home parenteral nutrition regimen with a median of 4 nights off (IQR: 3-4 nights) starting with a median of 7 nights (IQR: 7-7 nights). CONCLUSIONS: Combined AGIR techniques are practical and safe in SBS treatment when tailored to meet patients' needs, combining lengthening, tailoring, and reducing transit time procedures. Therefore, combined AGIR may be considered a resource in intestinal rehabilitation units' armamentarium.

An overview of the current management of short-bowel syndrome in pediatric patients.

Short-bowel syndrome (SBS) is defined as a state of malabsorption after resection or loss of a major portion of the bowel due to congenital or acquired factors. This article presents an overview on the recent management of pediatric SBS. The pediatric SBS population is very heterogeneous. The incidence of SBS is estimated to be 24.5 per 100,000 live births. The nutritional, medical, and surgical therapies available require a comprehensive evaluation. Thus, multidisciplinary intestinal rehabilitation programs (IRPs) are necessary for the management of these complex patients. The key points of focus in IRP management are hepato-protective strategies to minimize intestinal failure-associated liver disease; the aggressive prevention of catheter-related bloodstream infections; strategic nutritional supply to optimize the absorption of enteral calories; and the management and prevention of small bowel bacterial overgrowth, nephrocalcinosis, and metabolic bone disease. As the survival rate of children with SBS currently exceeds 90%, the application of small bowel transplantation has been evolving. The introduction of innovative treatments, such as combined therapy of intestinotrophic hormones, including glucagon-like peptide-2, may lead to further improvements in patients' quality of life.

Multidisciplinary intestinal rehabilitation in acute type II intestinal failure: Results from an intestinal rehabilitation team.

BACKGROUND: Type II (acute) intestinal failure (IF) is usually caused by complications of abdominal surgery resulting in enteric fistulas or proximal stomas and requires parenteral nutrition (PN) for several months. This study aimed to evaluate clinical management and outcome of type II IF patients in a single center. METHODS: Medical records of patients referred to the Intestinal Rehabilitation Team (IRT) at Samsung Medical Center (Seoul, Korea) were retrospectively analyzed. RESULTS: From 2014 to 2019, 34 patients with IF were referred. 28 patients were type II IF and were included in the analysis. There were 17 males and 11 females. Mean age of patients was 56.7 years. Pathophysiology of IF were high-output stoma in 16 cases, extensive bowel resection (with bowel in continuity) in 7 cases, and enterocutaneous fistula in 5 cases. The catastrophic events necessitating abdominal surgery in the patients were adhesive ileus in 9 cases, superior mesenteric artery thrombosis in 8 cases, internal herniation of bowel in 5 cases, traumatic bowel injury in 3 cases, and ischemic enteritis in 3 cases. Following medical and surgical rehabilitation, 10 patients (35.7%) were weaned off PN and overall mortality was 28.5%. Deaths were related to progression of underlying malignancies in 4 cases, liver failure in 3 cases, and sepsis in 1 case. Thirteen patients underwent surgery to restore bowel continuity. Six postoperative complications occurred in 4 patients (30.7%) and there were no postoperative mortalities. CONCLUSION: Standardized care including restorative surgery resulted in successful outcomes in type II IF patients in this cohort.

Síndrome do intestino curto: desafios e estratégias para um cuidado continuado / Short bowel syndrome: challenges and strategies for ongoing care

A síndrome pediátrica do intestino curto (SIC) é uma condição que ocorre em neonatos e crianças cujo cuidado demanda acesso a centros especializados. A SIC constitui-se um desafio para os profissionais de saúde, pois para que a criança alcance a autonomia enteral, deve ter seu cuidado planejado e realizado por uma equipe experiente e especializada. Esta pesquisa teve como objetivo descrever os principais desafios e estratégias para organização dos serviços especializados visando o cuidado integrado e continuado à criança com SIC. Foram utilizadas as seguintes técnicas metodológicas: a) revisão integrativa de literatura; (b) análise da casuística das crianças com SIC atendidas e internadas no Instituto Fernandes Figueira (IFF) nos últimos 6 anos; e (c) grupo nominal. Na revisão integrativa foram selecionadas 8 revisões sistemáticas que continham as práticas e intervenções clínicas que se desejava examinar e 11 estudos foram recuperados manualmente para complementar a análise dos pontos críticos do cuidado à criança com SIC. Na análise da casuística do IFF foram encontrados 23 pacientes que preencheram os critérios de elegibilidade, sendo a maioria destes nascidos no IFF (74%), cuja patologia de base predominante foi a gastrosquise (86,9%), com idade gestacional (IG) entre 34 e 36 semanas (47,8%). O tempo médio de internação hospitalar foi de 179 dias, com 2 reinternações em média, devendo-se considerar que a maior parte dos pacientes (n= 14, 60,8%) não reinternaram. As crianças com SIC/Falência Intestinal (FI) ocuparam na maior parte do tempo (70%) leitos da enfermaria de cirurgia pediátrica, sob os cuidados da equipe cirúrgica. A casuística apresenta 13 crianças (56,5%) que permaneceram internadas com nutrição parenteral (NP) por um período superior a 90 dias e que seriam elegíveis para a nutrição parenteral domiciliar (NPD). A média de utilização de cateteres por paciente ficou em torno de 6,5 e o tempo médio de permanência dos cateteres foi de 116,82 dias. Na análise da infecção de corrente sanguínea relacionada a cateter (ICSRC), o número de episódios de infecção foi prejudicado pela ausência de dados anteriores ao ano de 2016 no Epimed. Contudo, foi observado uma maior percentagem de pacientes (21,7%) que apresentaram o primeiro episódio de infecção antes de completarem um mês de vida. Somente 1 paciente foi submetido à Serial transverse enteroplasty (STEP), tendo como desfecho o óbito. Já o transplante intestinal não foi realizado em pacientes. Dentre os 15 pacientes que tiveram alta hospitalar, apenas 4 foram atendidos pela gastroenterologia pediátrica (27%), enquanto um maior número pela cirurgia pediátrica (87%) a nível ambulatorial. O grupo nominal (GN) foi composto por 5 especialistas do IFF e 2 especialistas externos que atuam em programas de reabilitação intestinal (PRI). Todos os especialistas reforçaram por meio dos dados obtidos nas estratégias anteriores, que as crianças de SIC/FI se beneficiam de um PRI estruturado no hospital de referência (serviço de internação e cuidado ambulatorial especializado), o qual também deve estar organizado para o cuidado de longo prazo que estas crianças demandam. Como contribuição para o SUS e no intuito de fortalecer a criação de uma linha de cuidado à criança com SIC/FI em outras unidades de referência, foram referidos os principais aspectos que devem ser pensados nas instâncias de saúde do país.

Pediatric short bowel syndrome (SBS) is a condition that occurs in neonates and children whose care requires access to specialized centers. SIC is a challenge for health professionals, because for the child to achieve enteral autonomy, his care must be planned and carried out by an experienced and specialized team. This research aimed to describe the main challenges and strategies for the organization of specialized services aimed at integrated and continuous care for children with SBS. The following methodological techniques were used: a) integrative literature review; (b) analysis of the sample of children with SBS treated and hospitalized at Instituto Fernandes Figueira (IFF) in the last 6 years; and (c) nominal group. In the integrative review, 8 systematic reviews were selected that contained the clinical practices and interventions that were desired to be examined and 11 studies were manually retrieved to complement the analysis of the critical points of care for children with SBS. In the analysis of the IFF series, 23 patients were found who met the eligibility criteria, most of whom were born in the IFF (74%), whose predominant underlying pathology was gastroschisis (86.9%), with gestational age (GA) between 34 and 36 weeks (47.8%). The mean length of hospital stay was 179 days, with an average of 2 readmissions, considering that most patients (n=14, 60.8%) were not readmitted. Children with SBS/Intestinal Failure (IF) occupied most of the time (70%) beds in the pediatric surgery ward, under the care of the surgical team. The series has 13 children (56.5%) who were hospitalized on parenteral nutrition (PN) for a period longer than 90 days and who would be eligible for home parenteral nutrition (HPN). The average use of catheters per patient was around 6.5 and the average length of stay of the catheters was 116.82 days. In the analysis of catheter-related bloodstream infection (CRBSI), the number of episodes of infection was hampered by the absence of data prior to 2016 in Epimed. However, a higher percentage of patients (21.7%) were observed who presented the first episode of infection before completing one month of life. Only 1 patient underwent serial transverse enteroplasty (STEP), resulting in death. Intestinal transplantation was not performed in patients. Among the 15 patients who were discharged from the hospital, only 4 were treated by pediatric gastroenterology (27%), while a greater number by pediatric surgery (87%) were treated on an outpatient basis. The nominal group (NG) was composed of 5 specialists from the IFF and 2 external specialists working in intestinal rehabilitation programs (IRP). All the specialists reinforced, through the data obtained in the previous strategies, that the children of SBS/FI benefit from a structured IRP in the referral hospital (inpatient service and specialized outpatient care), which must also be organized for the care of long term that these children demand. As a contribution to the SUS and to strengthen the creation of a line of care for children with SBS/IF in other reference units, the main aspects that should be considered in the country's health bodies were mentioned.

Long-Term Outcomes and Disease Burden of Neonatal Onset Short Bowel Syndrome.

PURPOSE: The study aims to describe long-term outcomes and disease burden of neonatal onset short bowel syndrome (SBS). METHODS: Utilizing the WHO criteria for adolescence, patients 10-19 years of age with neonatal onset SBS requiring parenteral nutrition (PN) for >90 days and followed by our multidisciplinary intestinal rehabilitation center between 2009 and 2018 were included for analysis. RESULTS: Seventy adolescents with SBS were studied. Median (IQR) age at last follow up in our center was 15 (11, 17) years. There was 0% mortality in the cohort, and 94% remained transplant free. Fifty-three patients (76%) achieved enteral autonomy. Three patients were weaned from PN without transplantation after six years of follow-up and another four after ten years of care at our multidisciplinary center. Disease burden remained higher in adolescents receiving PN, including inpatient hospitalizations (p < 0.01), procedures (p = 0.01), clinic visits (p < 0.01), and number of prescribed medications (p < 0.01). CONCLUSION: Survival for adolescents with neonatal onset SBS is excellent. Of the cohort studied, there was no mortality, and more than 75% achieved enteral autonomy. Disease burden remains high for adolescents who remain dependent on PN. However, achievement of enteral autonomy is feasible with long-term multidisciplinary rehabilitation. TYPE OF STUDY: Prognosis study. LEVEL OF EVIDENCE: Level II.

A new experimental model of intrinsic denervation in ileum from wistar rats through intramural microinjections of benzalkonium chloride / Um novo modelo experimental de desnervação intrínseca em íleo de ratos wistar através de micro injeções intramurais de cloreto de benzalcônio

Extensive literature is available about the intrinsic denervation of segments of the digestive tube through the application of CB in the serosa of the viscera. However, this technique has some disadvantages like causing peritonitis, flanges and high mortality, limiting its use in humans. The aim of the present study was to evaluate the feasibility of benzalkonium chloride (CB) to induce intrinsic chemical denervation, through applications of CB in the intramural ileum of wistar rats, as well as deepen the knowledge about the evolution of neuronal injury caused in the process. We used 40 rats, divided into two groups (control-GC and benzalkonium-GB) of 20 animals each, divided into four sub-groups according to the time of postoperative assessment of 24, 48 hours, 30 and 90 days. The animals were submitted to intramural microinjections of sterile saline solution 0.9% (GC) or benzalkonium chloride (GB) in ileal portion, and subsequent histopathological analysis and immunohistochemistry for evaluation of neuronal injury. A significant decrease (p<0.05) was found of the neuronal myenteric count over time in groups, GB3, GB4 and GB2. The specific positive immunolabeling for H2AX and Caspase-3 confirmed the results obtained in the histopathological evaluation, denoting the ignition of irreversible cell injury in 24 hours, evolving into neuronal apoptosis in 48 hours after application of the CB 0.3%. Under the conditions in which this work was conducted, it can be concluded that the application of CB 0.3% by means of microinjections intramural in the ileal wall is able to induce intrinsic chemical denervation of the diverticulum of wistar rats and that the main mechanism of neuronal death is induction of apoptosis.(AU)

Existe vasta literatura sobre a desnervação intrínseca de segmentos do tubo digestório através da aplicação de CB na serosa da víscera. Entretanto, essa técnica tem a desvantagem de causar peritonite, formação de bridas e alta mortalidade, não sendo factível para eventuais utilizações em humanos. O objetivo do presente estudo foi avaliar a viabilidade do Cloreto de benzalcônio (CB) induzir desnervação química intrínseca, por meio de aplicações intramurais em íleo de ratos wistar, além de aprofundar o conhecimento sobre a evolução da lesão neuronal causada neste processo. Foram utilizados 40 ratos, distribuídos em dois grupos (controle- GC e benzalcônio- GB) de 20 animais cada, subdivididos em quatro subgrupos de acordo com o tempo de avaliação pós-operatória de 24, 48 horas, 30 e 90 dias. Os animais foram submetidos à microinjeções intramurais de solução salina estéril 0,9% (GC) ou de cloreto de benzalcônio (GB) em porção ileal, e posterior análise histopatológica e imuno-histoquímica, para avaliação da lesão neuronal. Houve diminuição significativa (p<0,05) na contagem neuronal mientérica ao longo do tempo nos grupos GB2, GB3 e GB4. A imunomarcação específica positiva para H2AX e Caspase-3 confirmou os resultados obtidos na avaliação histopatológica, denotando início da lesão celular irreversível em 24 horas, evoluindo para apoptose neuronal em 48 horas após a aplicação do CB 0,3%. Nas condições em que este trabalho foi conduzido, é possível concluir que a aplicação de CB 0,3% por meio de microinjeções intramurais na parede ileal é capaz de induzir desnervação química intrínseca da porção ileal de ratos wistar e que o principal mecanismo de morte neuronal é a indução de apoptose.(AU)

Short bowel syndrome in children and adults: from rehabilitation to transplantation.

INTRODUCTION: Short bowel syndrome (SBS) is a dramatic clinical condition in both children and adults; the residual bowel length is not sufficient to avoid intestinal failure, with subsequent malnutrition and growth retardation, and intravenous support is required to provide the nutrients normally coming from the intestine. Apart from the primary disease, the medical status can be worsened by complications of intestinal failure: if there are irreversible, the prognosis is poor unless a successful intestinal rehabilitation is achieved. Areas covered: The rescue of the remnant small bowel requires a multidisciplinary expertise to achieve digestive autonomy. The use of intestinal trophic factors has shown encouraging results in improving the intestinal adaptation process. Whenever the residual bowel length is inadequate, in a well-selected population weaning parenteral nutrition (PN) off could be attempted by surgery through lengthening procedures. A further subset of patients, with total and irreversible intestinal failure and severe complications on PN, may have an indication to intestinal transplantation. This procedure is still affected by poor long-term results. Expert commentary: Novel approaches developed through a multidisciplinary team work, such as manipulation of microbiota or tissue bioengineering, should be added to current therapies to treat successfully SBS.

Predicting Intestinal Adaptation in Pediatric Intestinal Failure: A Retrospective Cohort Study.

OBJECTIVE: The primary goal in intestinal failure (IF) is adaptation and enteral autonomy (EA). Our goals were to determine the proportion of patients treated for IF by an established intestinal rehabilitation program who achieved EA and to assess the predictors of EA. BACKGROUND: There have been considerable advancements in the management of IF over the last 15 years, children with short bowel syndrome with a reduction in mortality. Several studies have discussed variables that may influence the ability to attain EA; however, majority were written when mortality rates were considerably higher compared with the current contemporary experience. METHODS: A retrospective analysis of infants <12 months with short bowel syndrome referred between 2006 and 2013 (n = 120). Data was collected on IF-related factors and nutritional intake. The cohort was stratified based on achievement of EA. Statistical testing completed using t test, Chi Square, and Cox Proportional Hazards regression (P < 0.05). RESULTS: EA was achieved in 84 (70.0%) patients. Patients who remained parenteral nutrition dependent were more likely to have volvulus (1.2 vs 22.2%, P < 0.001), shorter percent residual small bowel (29.4 vs 68.6%; P < 0.0001) and colon length (64.6 vs 86.0%; P = 0.001), and no ileocecal valve (61.1 vs 29.8%; P = 0.05). Mortality was also decreased in those who achieved EA (4 vs 22%; P = 0.004). Percent residual small bowel (HR = 1.03; 95% CI 1.02-1.03) and colon (HR = 1.01; 95% CI 1.00-1.02) length were positively associated with EA, while number of septic episodes was negatively associated (HR = 0.95; 95% CI 0.91-0.99). CONCLUSION: Seventy percent of infants with IF achieved EA. Residual small and large bowel length were the most important predictors of EA and septic events had a negative impact.

Improvements in intestine transplantation.

Transplantation of the intestine in children has presented significant challenges even as it has become a standard to treat nutritional failure due to short gut syndrome. These challenges have been addressed in part by significant improvements in short and long-term care. Noteworthy enhancements include reduced need for intestine transplantation, drug-sparing immunosuppressive regimens, immune monitoring, and improved surveillance and management of PTLD and non-adherence.

Intestinal failure: the evolving demographic and patient outcomes on home parenteral nutrition.

AIM: We performed this study to examine and understand the evolving demographics and changing outcomes of intestinal failure (IF) and its implications for healthcare delivery. METHOD: We conducted a retrospective analysis of outcome data of children on home parenteral nutrition (HPN), over a 15-year period. RESULTS: A total of 31 patients received HPN: 15 for short bowel syndrome (SBS), eight neuromuscular disease (NMD) and eight for other causes. The HPN prevalence increased from 1.54 per million children in 2000 to 21.5 in 2016. The outcomes over last 5 years were better than those of previous 10 years. The rate of catheter-related bloodstream infection (CRBSI) had fallen from 4 to 1.3 and IF liver disease (IFALD) from 20% to 7.7%. The aetiology changed over years from SBS being the main cause to NMD contributing 43% to the total in 2016. This was especially relevant as NMD was associated with greater numbers of IFALD (38% vs 6.7%), CRBSI (1.51 vs 0.64/1000 PN days) and mortality. CONCLUSION: The outcome of long-term parenteral nutrition (PN) has improved. The increasing number of patients with NMD, coupled with their higher burden of care, results in an increasing health care burden, and the planning of intestinal rehabilitation services needs to reflect this.

Intestinal Rehabilitation Programs in the Management of Pediatric Intestinal Failure and Short Bowel Syndrome.

Intestinal failure is a rare, debilitating condition that presents both acute and chronic medical management challenges. The condition is incompatible with life in the absence of the safe application of specialized and individualized medical therapy that includes surgery, medical equipment, nutritional products, and standard nursing care. Intestinal rehabilitation programs are best suited to provide such complex care with the goal of achieving enteral autonomy and oral feeding with or without intestinal transplantation. These programs almost all include pediatric surgeons, pediatric gastroenterologists, specialized nurses, and dietitians; many also include a variety of other medical and allied medical specialists. Intestinal rehabilitation programs provide integrated interdisciplinary care, more discussion of patient management by involved specialists, continuity of care through various treatment interventions, close follow-up of outpatients, improved patient and family education, earlier treatment of complications, and learning from the accumulated patient databases. Quality assurance and research collaboration among centers are also goals of many of these programs. The combined and coordinated talents and skills of multiple types of health care practitioners have the potential to ameliorate the impact of intestinal failure and improve health outcomes and quality of life.

Ultrashort Bowel Syndrome Outcome in Children Treated in a Multidisciplinary Intestinal Rehabilitation Unit.

Aim Short bowel syndrome (SBS) is the leading cause of intestinal failure (IF) in the pediatric population. Our aim was to review long-term outcome of ultrashort bowel syndrome (USBS) in an Intestinal Rehabilitation Unit (IRU). Patients and Methods Retrospective study of patients with USBS (defined as < 10 cm of remnant small bowel) treated between 2000 and 2015. Demographic data, clinical, and treatment variables including parenteral nutrition (PN), surgical techniques, and intestinal transplantation (IT) were analyzed. Results Out of 250 children, 30 referred to the IRU met inclusion criteria. Upon first assessment, patients had a median age of 3 (1-217) months and had undergone 3 (1-6) previous laparotomies that left 5 (0-9) cm of remnant small bowel. The main cause of USBS was neonatal midgut volvulus (50%). Follow-up was 28 (4-175) months. Advanced IF-associated liver disease (IFALD) was documented in 63%. None of the patients achieved digestive autonomy and was consequently considered for IT. One patient was excluded, five died before IT, and three are still on the waiting list. Six patients received an isolated IT, 6 a combined liver IT, and 18 a multivisceral graft. Digestive autonomy was achieved in 71% after 31 (14-715) days after IT and currently 62% are alive and off total PN. A significant drop in IFALD progression prior to IT was observed with the introduction of new lipid emulsions in 2010 (SMOF or Soy oil MCT (mid-chain triglycerides) Olive oil Fish oil). Conclusion A multidisciplinary IRU including an IT program offers a comprehensive approach for patients with IF and is crucial to improve survival rate of USBS. New PN lipid emulsions had an impact on IFALD progression and may eventually reduce overall mortality.

Short bowel syndrome: treatment options / Síndrome Intestino Curto: abordagens terapêuticas

Introduction: Short bowel syndrome (SBS) refers to the malabsorptive state that occurs following extensive intestinal resection and is associated with several complications. Methods: The research for this review was conducted in the Pubmed database. Relevant scientific articles dated between 1991 and 2015 and written in Portuguese, Spanish or English were selected. Results: Several therapies, including nutritional support, pharmacological options and surgical procedures have been used in these patients. Conclusions: Over the last decades new surgical and pharmacological approaches emerged, increasing survival and quality of life (QoL) in patients with SBS. All SBS patients ought to have an individualized and multidisciplinary care that promotes intestinal rehabilitation.

Introdução: A Síndrome do Intestino Curto (SIC) resulta da perda da capacidade de absorção do intestino após resseção intestinal extensa e está associada a diversas complicações. Métodos: Esta revisão foi realizada com base em artigos científicos originais pesquisados na base de dados MEDLINE via Pubmed, na língua portuguesa, inglesa e espanhola, com o limite temporal de 1991 a 2015. Resultados: O tratamento instituído pode ser a nível nutricional, farmacológico ou cirúrgico. Conclusões: Ao longo das últimas décadas surgiram novas abordagens terapêuticas cirúrgicas e não-cirúrgicas que melhoraram a sobrevivência e a qualidade de vida (QoL) destes pacientes. Deve-se estabelecer uma abordagem multidisciplinar e individualizada para garantir a melhor reabilitação.

Insuficiencia intestinal secundaria a síndrome de intestino corto: resultados de un programa multidisciplinario de rehabilitación intestinal / Intestinal failure due to short bowel syndrome: impact of a multidisciplinary intestinal rehabilitation program

Background: In patients suffering intestinal failure due to short bowel, the goal of an Intestinal Rehabilitation Program is to optimize and tailor all aspects of clinical management, and eventually, wean patients off lifelong parenteral nutrition. Aim: To report the results of our program in patients suffering intestinal failure. Patients and Methods: A registry of all patients referred to the Intestinal Failure unit between January 2009 and December 2015 was constructed. Initial work up included prior intestinal surgery, blood tests, endoscopic and imaging studies. Also demographic data, medical and surgical management as well as clinical follow-up, were registered. Results: Data from 14 consecutive patients aged 26 to 84 years (13 women) was reviewed. Mean length of remnant small bowel was 100 cm and they were on parenteral nutrition for a median of eight months. Seven of 14 patients had short bowel secondary to mesenteric vascular events (embolism/thrombosis). Medical management and autologous reconstruction of the bowel included jejuno-colic anastomosis in six, enterorraphies in three, entero-rectal anastomosis in two, lengthening procedures in two, ileo-colic anastomosis in one and reversal Roux-Y gastric bypass in one. Thirteen of 14 patients were weaned off parenteral nutrition. Conclusions: Our Multidisciplinary Intestinal Rehabilitation Program, allowed weaning most of the studied patients off parenteral nutrition.

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.

Pharmacological strategies to enhance adaptation in intestinal failure.

PURPOSE OF REVIEW: Intestinal failure because of more or less extensive resection of parts of the small and large intestine (short bowel syndrome) results from the reduction of absorptive surface of the remaining intestine and frequently results in dependence on parenteral nutrition. Parenteral nutrition, although lifesaving, is associated with short and long-term complications as well as with reduced quality of life and overall survival. RECENT FINDINGS: Pharmacological enhancement of the physiological intestinal adaptive response by subcutaneous application of the glucagon-like peptide 2 analogue teduglutide results in an improved, hyperadaptive response. This is reflected by decreased parenteral calorie and fluid requirements, decreased parenteral nutrition infusion days per week including complete weaning off parenteral nutrition with complete oral autonomy, improved quality of life, and metabolic and nutritional stability. SUMMARY: The advent of teduglutide as an authority-approved specific medication for intestinal failure in parenteral nutrition-dependent short bowel syndrome offers an effective and beneficial treatment for these patients. As a result, patients are more stable whether for medical or further surgical management including intestinal transplantation. Long-term efficacy and safety still have to be proven.

Surgical Rehabilitation Techniques in Children with Poor Prognosis Short Bowel Syndrome.

Intestinal failure (IF) requires a multidisciplinary management based on nutritional support, surgical and medical rehabilitation, and transplantation. The aim of this study is to review our experience with surgical rehabilitation techniques (SRTs: enteroplasty, Bianchi, Serial Transverse Enteroplasty Procedure [STEP]) in patients with short bowel syndrome (SBS) and poor prognosis due to complex abdominal pathology. We performed a single-center retrospective study of patients with IF evaluated for intestinal transplantation in the Intestinal Rehabilitation Unit who underwent an SRT. Nonparametric tests were used for statistical analysis.A total of 205 patients (107 males/98 females) with mean age of 25 ± 7 months were assessed for IF. A total of 433 laparotomies were performed on 130 patients including intestinal resection, enteroplasties, adhesiolysis, and transit reconstruction. SRT were performed in 22 patients: 12 enteroplasties, 8 STEPs, and 4 Bianchi procedures. All patients were parenteral nutrition (PN) dependent with different stages of liver disease: mild (13), moderate (5), and severe (4). The adaptation rate for patients who underwent enteroplasty, STEP, and Bianchi were 70, 63, and 25%, respectively, although the techniques are not comparable. Overall, intestinal adaptation was achieved in nine (41%) patients, and four (18%) patients showed significant reduction of PN needs. One child did not respond to SRT and did not meet transplantation criteria. The remaining eight (36%) patients were included on the waiting list for transplant: four were transplanted, two are still on the waiting list, and two died. Better outcomes were observed in milder cases of liver disease (mild 77%, moderate 40%, severe 25%) (p < 0.05). Conversely, a trend toward a poorer outcome was observed in cases with ultrashort bowel (p > 0.05). One patient required reoperation after a Bianchi procedure due to intestinal ischemia and six needed further re-STEP or adhesiolysis procedure several months later. The median follow-up was 62 (3-135) months. Overall mortality was 19%, and was due to end-stage liver disease and/or central venous catheter-related sepsis. SRT led to intestinal adaptation in a significant number of patients with poor prognosis SBS referred for intestinal transplantation. However, SRT requires a multidisciplinary evaluation and should be attempted only in suitable cases. Careful assessment and optimal surgical timing is crucial to obtain a favorable outcome.

[Intestinal failure due to short bowel syndrome: impact of a multidisciplinary intestinal rehabilitation program]. / Insuficiencia intestinal secundaria a síndrome de intestino corto: resultados de un programa multidisciplinario de rehabilitación intestinal.

BACKGROUND: In patients suffering intestinal failure due to short bowel, the goal of an Intestinal Rehabilitation Program is to optimize and tailor all aspects of clinical management, and eventually, wean patients off lifelong parenteral nutrition. AIM: To report the results of our program in patients suffering intestinal failure. PATIENTS AND METHODS: A registry of all patients referred to the Intestinal Failure unit between January 2009 and December 2015 was constructed. Initial work up included prior intestinal surgery, blood tests, endoscopic and imaging studies. Also demographic data, medical and surgical management as well as clinical follow-up, were registered. RESULTS: Data from 14 consecutive patients aged 26 to 84 years (13 women) was reviewed. Mean length of remnant small bowel was 100 cm and they were on parenteral nutrition for a median of eight months. Seven of 14 patients had short bowel secondary to mesenteric vascular events (embolism/thrombosis). Medical management and autologous reconstruction of the bowel included jejuno-colic anastomosis in six, enterorraphies in three, entero-rectal anastomosis in two, lengthening procedures in two, ileo-colic anastomosis in one and reversal Roux-Y gastric bypass in one. Thirteen of 14 patients were weaned off parenteral nutrition. CONCLUSIONS: Our Multidisciplinary Intestinal Rehabilitation Program, allowed weaning most of the studied patients off parenteral nutrition.

Early Intestinal Rehabilitation Therapy Ameliorates Intestinal Adaptation in Children with Short Bowel Syndrome: The Long-Term Outcome.

In the management of short bowel syndrome (SBS), the benefits of treatment with growth hormone (GH), glutamine, and enteral nutrition (EN) on intestinal adaptation among children patients is still controversial. The aim of present study is to determine whether GH, glutamine, and EN have positive effect on intestinal adaptation in children with SBS. Sixteen children with SBS (small bowel remnant length, 56.75 ± 8.09 cm; mean ± SE) were treated with GH (0.05 mg/kg/d), glutamine (0.45 mg/kg/d), plus EN-enriched fiber diet for four weeks. After four weeks of treatment, patients were discharged home; GH was discontinued, but the EN with glutamine was continued. Repeated treatment was performed if there were lose weight, dysplasia, or severe diarrhea. All patients completed the treatment. Body weight, intestinal absorptive capacity, and plasma levels of proteins were significantly improved after complete treatment, without any major adverse effects. On follow-up, no death was reported. Treatment with GH, glutamine, and EN in early stage significantly improved intestinal adaptation in pediatric patients with SBS. Furthermore, the positive effect of the treatment does not seem to be sustained once GH discontinued until the residual intestinal adaptation reaches its maximum.