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1.
Nutrients ; 16(10)2024 May 12.
Artigo em Inglês | MEDLINE | ID: mdl-38794694

RESUMO

BACKGROUND: Although parenteral nutrition (PN) significantly improves mortality rates in pediatric short bowel syndrome (SBS), long-term PN has many possible complications and impacts quality of life. Bowel lengthening procedures (BLPs) increase the contact surface of food and the intestinal mucosa and enable the better absorption of nutrients and liquids, possibly leading to a PN decrease. METHODS: We retrospectively reviewed the data of patients with short bowel syndrome who underwent BLPs in the period from January 2016 to January 2022. Overall, eight patients, four male, five born prematurely, underwent BLPs. RESULTS: There was a significant decrease in the percentage of total caloric intake provided via PN and PN volume after the BLPs. The more evident results were seen 6 months after the procedure and at the last follow-up, which was, on average, 31 months after the procedure. Two patients were weaned off PN after their BLPs. Patients remained well nourished during the follow-up. CONCLUSIONS: The BLP led to a significant decrease in PN needs and an increase in the food intake; however, significant changes happened more than 6 months after the procedure.


Assuntos
Nutrição Parenteral , Síndrome do Intestino Curto , Humanos , Síndrome do Intestino Curto/cirurgia , Síndrome do Intestino Curto/terapia , Masculino , Feminino , Estudos Retrospectivos , Resultado do Tratamento , Lactente , Pré-Escolar , Criança , Estado Nutricional , Ingestão de Energia , Procedimentos Cirúrgicos do Sistema Digestório/métodos , Procedimentos Cirúrgicos do Sistema Digestório/efeitos adversos , Qualidade de Vida
2.
Clin Nutr ESPEN ; 61: 338-348, 2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-38777453

RESUMO

BACKGROUND & AIMS: Home Parenteral Nutrition (HPN) is the main treatment for patients with chronic intestinal failure. It is commonly prescribed for nutritional recovery, survival increase and, whenever possible, improvement of quality of life. As there are no validated instruments in Brazilian Portuguese to be used in these patients, the objective of this study was to carry out the transcultural adaptation and validation of Home Parenteral Nutrition - Quality of life (HPN-QOL©) into Brazilian Portuguese. METHODS: This observational and cross-sectional study was conducted at the multidisciplinary short-bowel syndrome clinic (AMULSIC) of the Hospital das Clínicas of the University of São Paulo Medical School (HC-FMUSP). A five-stage protocol was adopted for the transcultural adaptation: initial translation; synthesis; reverse translation; experts committee and pre-test. The adapted questionnaire was applied to a convenience (representative) sample (n = 16) and Cronbach's Alpha Coefficient, Intraclass Correlation Coefficient (ICC), and Bland-Altman Test were submitted for the analysis of the internal consistency and intraobserver and interobserver reproducibility. RESULTS: The transcultural adaptation was considered excellent (Content Validity Index = 100%). The internal consistency was satisfactory for most of the scales (16/19), and α > 0.70 was 84.21%. ICC values revealed high intraobserver and interobserver reproducibility in most of the scales. No significant difference was observed between intraobservers and interobservers in any of the questions (p > 0.05). CONCLUSIONS: The questionnaire was shown as adapted and valid for use in Brazil. Future trials with a higher sample are yet to be developed to shed light on specific scales that were inconsistent. It's expected that this would contribute for the usual quality of life assessment for individuals treated with HPN in Brazil.


Assuntos
Nutrição Parenteral no Domicílio , Qualidade de Vida , Traduções , Humanos , Brasil , Estudos Transversais , Inquéritos e Questionários , Reprodutibilidade dos Testes , Feminino , Masculino , Pessoa de Meia-Idade , Adulto , Síndrome do Intestino Curto/terapia
3.
Biochim Biophys Acta Mol Basis Dis ; 1870(5): 167221, 2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-38718845

RESUMO

BACKGROUND: Short bowel syndrome (SBS) features nutrients malabsorption and impaired intestinal barrier. Patients with SBS are prone to sepsis, intestinal flora dysbiosis and intestinal failure associated liver disease. Protecting intestinal barrier and preventing complications are potential strategies for SBS treatment. This study aims to investigate the effects of farnesoid X receptor (FXR) agonist, obeticholic acid (OCA), have on intestinal barrier and ecological environment in SBS. METHODS AND RESULTS: Through testing the small intestine and serum samples of patients with SBS, impaired intestinal barrier was verified, as evidenced by reduced expressions of intestinal tight junction proteins (TJPs), increased levels of apoptosis and epithelial cell damage. The intestinal expressions of FXR and related downstream molecules were decreased in SBS patients. Then, global FXR activator OCA was used to further dissect the potential role of the FXR in a rat model of SBS. Low expressions of FXR-related molecules were observed on the small intestine of SBS rats, along with increased proinflammatory factors and damaged barrier function. Furthermore, SBS rats possessed significantly decreased body weight and elevated death rate. Supplementation with OCA mitigated the damaged intestinal barrier and increased proinflammatory factors in SBS rats, accompanied by activated FXR-related molecules. Using 16S rDNA sequencing, the regulatory role of OCA on gut microbiota in SBS rats was witnessed. LPS stimulation to Caco-2 cells induced apoptosis and overexpression of proinflammatory factors in vitro. OCA incubation of LPS-pretreated Caco-2 cells activated FXR-related molecules, increased the expressions of TJPs, ameliorated apoptosis and inhibited overexpression of proinflammatory factors. CONCLUSIONS: OCA supplementation could effectively ameliorate the intestinal barrier disruption and inhibit overexpression of proinflammatory factors in a rat model of SBS and LPS-pretreated Caco-2 cells. As a selective activator of FXR, OCA might realize its protective function through FXR activation.


Assuntos
Ácido Quenodesoxicólico , Modelos Animais de Doenças , Mucosa Intestinal , Receptores Citoplasmáticos e Nucleares , Síndrome do Intestino Curto , Animais , Ácido Quenodesoxicólico/análogos & derivados , Ácido Quenodesoxicólico/farmacologia , Síndrome do Intestino Curto/metabolismo , Síndrome do Intestino Curto/tratamento farmacológico , Síndrome do Intestino Curto/patologia , Ratos , Humanos , Masculino , Receptores Citoplasmáticos e Nucleares/metabolismo , Receptores Citoplasmáticos e Nucleares/agonistas , Mucosa Intestinal/metabolismo , Mucosa Intestinal/efeitos dos fármacos , Mucosa Intestinal/patologia , Microbioma Gastrointestinal/efeitos dos fármacos , Feminino , Ratos Sprague-Dawley , Apoptose/efeitos dos fármacos , Pessoa de Meia-Idade , Intestino Delgado/metabolismo , Intestino Delgado/efeitos dos fármacos , Intestino Delgado/patologia , Adulto , Proteínas de Junções Íntimas/metabolismo
4.
Turk J Med Sci ; 54(1): 59-68, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38812652

RESUMO

Background/aim: Intestinal neomucosa formation is a technique defined for the treatment of short bowel syndrome. This study evaluates the effect of glutamine and omega-3 fatty acids on the growth of intestinal neomucosa on the colonic serosal surface has been evaluated. Materials and methods: Thirty-two adult male Sprague-Dawley rats were randomly divided into 4 groups: sham, control, glutamine, and omega-3. Laparotomy was performed on all groups. For rats other than the sham group, a 1-cm full-thickness incision was made 4 cm proximal to the ileocecal valve, and colonic serosal surface was sutured as a serosal patch over these openings. By using the oral gavage technique, the glutamine group was ingested with 200 mg/kg/day of glutamine, and the omega-3 group was ingested with 100 mg/kg/day of omega-3 fatty acids. At the end of 14 days, the rats were euthanized, blood specimens were collected, and intestinal segments, including serosal patches, were excised. Results: Transforming growth factor-beta was significantly lower in the glutamine group compared to the control group. Similarly, fibroblast growth factor-2 was significantly lower in the glutamine group compared to the sham group. Intestinal neomucosa formation was observed in 100% of rats in the glutamine group. In the control and omega-3 groups, intestinal neomucosa formation was observed in 57.1% and 60% of rats, respectively. The inflammatory response, granulation tissue formation, and fibroblastic activity were more severe in the rats of the glutamine and omega-3 groups. Conclusion: The intestinal neomucosa formation is an experimental technique, and both glutamine and omega-3 fatty acids have the potential to positively affect inflammatory response, granulation tissue formation, and fibroblastic activity. Specifically, glutamine has a favorable effect on intestinal neomucosa formation.


Assuntos
Colo , Ácidos Graxos Ômega-3 , Glutamina , Ratos Sprague-Dawley , Animais , Glutamina/farmacologia , Ácidos Graxos Ômega-3/farmacologia , Masculino , Ratos , Colo/efeitos dos fármacos , Síndrome do Intestino Curto/tratamento farmacológico , Membrana Serosa/efeitos dos fármacos , Mucosa Intestinal/efeitos dos fármacos , Mucosa Intestinal/metabolismo
5.
Gastroenterol Clin North Am ; 53(2): 329-341, 2024 06.
Artigo em Inglês | MEDLINE | ID: mdl-38719382

RESUMO

Infants and children with intestinal failure are at risk for pediatric feeding disorders, which challenge their oral feeding development. This article explores these challenges and offers several practical strategies that can be used by multidisciplinary care teams and at-home caregivers to help support the development of oral feeding in these children and eventually lead to their attaining enteral autonomy.


Assuntos
Nutrição Enteral , Síndrome do Intestino Curto , Humanos , Síndrome do Intestino Curto/terapia , Síndrome do Intestino Curto/complicações , Lactente , Nutrição Enteral/métodos , Criança , Pré-Escolar , Insuficiência Intestinal/terapia , Insuficiência Intestinal/etiologia
6.
Clin Nutr ; 43(6): 1331-1342, 2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-38677044

RESUMO

OBJECTIVE: Fecal microbiota was investigated in adult patients with chronic intestinal failure (CIF) due to short bowel syndrome (SBS) with jejunocolonic anastomosis (SBS-2). Few or no data are available on SBS with jejunostomy (SBS-1) and CIF due to intestinal dysmotility (DYS) or mucosal disease (MD). We profiled the fecal microbiota of various pathophysiological mechanisms of CIF. METHODS: Cross-sectional study on 61 adults with CIF (SBS-1 30, SBS-2 17, DYS 8, MD 6). Fecal samples were collected and profiled by 16S rRNA amplicon sequencing. Healthy controls (HC) were selected from pre-existing cohorts, matched with patients by sex and age. RESULTS: Compared to HC, SBS-1, SBS-2 and MD patients showed lower alpha diversity; no difference was found for DYS. In beta diversity analysis, SBS-1, SBS-2 and DYS groups segregated from HC and from each other. Taxonomically, the CIF groups differed from HC even at the phylum level. In particular, CIF patients' microbiota was dominated by Lactobacillaceae and Enterobacteriaceae, while depleted in typical health-associated taxa belonging to Lachnospiraceae and Ruminococcaceae. Notably, compositional peculiarities of the CIF groups emerged. Furthermore, in the SBS groups, the microbiota profile differed according to the amount of parenteral nutrition required and the duration of CIF. CONCLUSIONS: CIF patients showed marked intestinal dysbiosis with microbial signatures specific to the pathophysiological mechanism of CIF as well as to the severity and duration of SBS.


Assuntos
Fezes , Microbioma Gastrointestinal , Síndrome do Intestino Curto , Humanos , Masculino , Feminino , Estudos Transversais , Pessoa de Meia-Idade , Fezes/microbiologia , Adulto , Síndrome do Intestino Curto/microbiologia , Síndrome do Intestino Curto/fisiopatologia , Doença Crônica , Idoso , Insuficiência Intestinal/microbiologia , RNA Ribossômico 16S/genética
7.
Pediatr Transplant ; 28(3): e14756, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-38623905

RESUMO

INTRODUCTION: Intestinal failure, defined as the loss of gastrointestinal function to the point where nutrition cannot be maintained by enteral intake alone, presents numerous challenges in children, not least the timing of consideration of intestine transplantation. OBJECTIVES: To describe the evolution of care of infants and children with intestinal failure including parenteral nutrition, intestine transplantation, and contemporary intestinal failure care. METHODS: The review is based on the authors' experience supported by an in-depth review of the published literature. RESULTS: The history of parenteral nutrition, including out-patient (home) administration, and intestine transplantation are reviewed along with the complications of intestinal failure that may become indications for consideration of intestine transplantation. Current management strategies for children with intestinal failure are discussed along with changes in need for intestine transplantation, recognizing the difficulty in generalizing recommendations due to the high level of heterogeneity of intestinal pathology and residual bowel anatomy and function. DISCUSSION: Advances in the medical and surgical care of children with intestinal failure have resulted in improved transplant-free survival and a significant fall in demand for transplantation. Despite these improvements a number of children continue to fail rehabilitative care and require intestine transplantation as life-saving therapy or when the burden on ongoing parenteral nutrition becomes too great to bear.


Assuntos
Enteropatias , Insuficiência Intestinal , Síndrome do Intestino Curto , Transplantes , Criança , Lactente , Humanos , Intestinos , Intestino Delgado , Nutrição Parenteral , Enteropatias/cirurgia , Síndrome do Intestino Curto/cirurgia
8.
Rev. colomb. cir ; 39(3): 430-440, 2024-04-24. tab
Artigo em Espanhol | LILACS | ID: biblio-1554114

RESUMO

Introducción. El intestino primitivo rota durante la vida embrionaria. Cuando ocurre de forma inadecuada aparece la malrotación intestinal que puede llevar a la obstrucción o al vólvulo del intestino medio. La incidencia disminuye con el aumento de la edad. La malrotación intestinal es una de las principales causas de complicaciones del tracto gastrointestinal en la edad pediátrica. Métodos. Estudio retrospectivo, observacional, transversal y analítico, de la experiencia durante 10 años en pacientes menores de 15 años con diagnóstico de malrotación intestinal, tratados en el Hospital Infantil de San Vicente Fundación, en Medellín, Colombia. Se buscó la asociación entre variables demográficas, clínicas e imagenológicas con los desenlaces. Resultados. Se obtuvieron 58 pacientes con malrotación intestinal, 65 % menores de 1 año. En 29,3 % de los pacientes se hizo el diagnóstico con la presentación clínica; los síntomas predominantes fueron distensión abdominal y emesis. En el 24,1 % se confirmó el diagnóstico con imágenes. Las variables con una diferencia estadísticamente significativa a favor de encontrar una malrotación complicada fueron choque séptico (OR=11,7), síndrome de respuesta inflamatoria sistémica (OR=8,4) y deshidratación (OR=5,18). Conclusiones. La malrotación intestinal tiene complicaciones como perforación, peritonitis, vólvulo e intestino corto. El vólvulo se acompaña de shock y sepsis, con mortalidad hasta del 50 %. Las imágenes diagnósticas son una ayuda, pero no se puede basar la conducta médica en estas porque ninguna imagen garantiza el diagnóstico definitivo. Los signos de alarma son poco específicos. En menores de un año con emesis, distensión y dolor abdominal se debe sospechar malrotación intestinal.


Introduction. The primitive intestine rotates during embryonic life. When it occurs inappropriately, intestinal malrotation appears, which can lead to obstruction or midgut volvulus. The incidence decreases when age increases. Intestinal malrotation is one of the main causes of complications of the gastrointestinal tract in pediatric age. Method. Retrospective, observational, cross-sectional and analytical study of the experience over 10 years in patients under 15 years of age with a diagnosis of intestinal malrotation, treated at Hospital Infantil of San Vicente Fundación, in Medellín, Colombia. The association between demographic, clinical and imaging variables with the outcomes was sought. Results. There were 58 patients with intestinal malrotation, 65% under one year of age. In 29.3% of patients, intestinal malrotation was diagnosed clinically. The predominant symptoms were abdominal distension and emesis. In 24.1% the diagnosis was confirmed with imaging. The variables with a statistically significant difference in favor of finding a complicated malrotation were septic shock (OR=11.7), systemic inflammatory response syndrome (OR-8.4), and dehydration (OR=5.18). Conclusions. Malrotation has complications such as perforation, peritonitis, volvulus, and short bowel. Volvulus is accompanied by shock and sepsis, with mortality of up to 50%. Diagnostic images are helpful, but medical conduct cannot be based on them because no image guarantees a definitive diagnosis. The warning signs are not very specific. In children under one year of age with emesis, distension and abdominal pain, intestinal malrotation should be suspected.


Assuntos
Humanos , Trato Gastrointestinal , Volvo Intestinal , Enteropatias , Síndrome do Intestino Curto , Obstrução Intestinal , Perfuração Intestinal
9.
Bol Med Hosp Infant Mex ; 81(1): 23-30, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38503324

RESUMO

BACKGROUND: Short bowel syndrome (SBS) is one of the most frequent causes of intestinal failure, needing parenteral nutrition to maintain an energy-protein and water-electrolyte balance. At the Hospital Infantil de México Federico Gómez (HIMFG), the formation of two stomas is a technique used for intestinal rehabilitation, where the use of residue through the bypass technique (BT) helps to maintain gastrointestinal functionality, water-electrolyte, and nutritional stability. This study aimed to describe the technique of using intestinal residue through BT as a treatment strategy in intestinal rehabilitation and its effect on the biochemical and nutritional status of pediatric patients with SBS. METHODS: An analytical and retrospective cross-sectional study was performed in patients hospitalized at HIMFG with SBS who underwent BT during their hospital stay between 2019 and 2020 and then followed up for 8 weeks. RESULTS: A total of 10 patients were included in this study, with a mean age of 24 months; 50% were female. BT was able to reduce the inflammatory process in the liver caused by the continuous use of parenteral nutrition; enteral caloric intake increased from 25.32 kcal/kg/day to 72.94 kcal/kg/day, but it was insufficient to improve their nutritional status. CONCLUSIONS: BT is a safe and effective alternative in intestinal rehabilitation in patients with SBS to stimulate trophism and intestinal functionality, allowing a progression of enteral feeding and a decrease in the hepatic inflammatory process that occurs in these patients with prolonged parenteral nutrition.


INTRODUCCIÓN: El síndrome de intestino corto (SIC) es una de las causas más frecuentes de insuficiencia intestinal que requiere del uso de nutrición parenteral para mantener un balance energético-proteico e hidroelectrolítico. En el Hospital Infantil de México Federico Gómez (HIMFG) la formación de dos estomas es una técnica empleada para la rehabilitación intestinal, donde con el aprovechamiento de residuo mediante la técnica de puenteo (TP) se ayuda a mantener la funcionalidad gastrointestinal, equilibrio hidro-electrolítico y estabilidad nutricional. El objetivo de este estudio fue describir la técnica del aprovechamiento de residuo intestinal mediante TP como estrategia de tratamiento en la rehabilitación intestinal y su efecto en el estado bioquímico y nutricional de pacientes pediátricos con SIC. MÉTODOS: Se llevó a cabo un estudio transversal analítico y retrospectivo en pacientes hospitalizados en el HIMFG con SIC en quienes se realizó la TP durante su estancia intrahospitalaria entre 2019 y 2020. RESULTADOS: Se incluyeron 10 pacientes en este estudio, con una edad promedio de 24 meses, y el 50% de sexo femenino. La TP logró disminuir el proceso inflamatorio hepático ocasionado por el uso continuo de nutrición parenteral; la ingesta calórica por vía enteral incrementó de 25.32 kcal/kg/día a 72.94 kcal/kg/día, pero fue insuficiente para mejorar el estado nutricional. CONCLUSIONES: La TP es una alternativa segura y efectiva en la rehabilitación intestinal en pacientes con SIC para estimular el trofismo y funcionalidad intestinal, permitiendo una progresión de la alimentación enteral y disminución del proceso inflamatorio hepático que se presentan en estos pacientes con nutrición parenteral prolongada.


Assuntos
Síndrome do Intestino Curto , Humanos , Criança , Feminino , Pré-Escolar , Masculino , Síndrome do Intestino Curto/cirurgia , Síndrome do Intestino Curto/etiologia , Estudos Retrospectivos , Estudos Transversais , Derivação Jejunoileal/efeitos adversos , México
10.
Zhonghua Wei Chang Wai Ke Za Zhi ; 27(3): 231-235, 2024 Mar 25.
Artigo em Chinês | MEDLINE | ID: mdl-38532584

RESUMO

Intestinal failure is a syndrome characterized by a diminished intestinal function that is inadequate to maintain normal digestion and absorption, leading to systemic metabolic disorder and requiring long-term nutritional supplementation to sustain health and growth. Short bowel syndrome (SBS) is one of the primary causes of intestinal failure. Given the significant differences among SBS patients, nutritional treatment strategies should emphasize individualization. This review focuses on SBS, combining its anatomical and pathological characteristics, to introduce nutritional support treatment plans and experiences for patients with intestinal failure.


Assuntos
Insuficiência Intestinal , Síndrome do Intestino Curto , Humanos , Apoio Nutricional , Intestinos
11.
J Med Case Rep ; 18(1): 122, 2024 Mar 21.
Artigo em Inglês | MEDLINE | ID: mdl-38509559

RESUMO

BACKGROUND: Short bowel syndrome (SBS) in adults is defined as having less than 180 to 200 cm of remaining small bowel. Many literature sources do not provide precise epidemiological data, and challenges in estimating the prevalence of SBS include its multifactorial etiology and varying definitions. The most common pathologies leading to SBS include Crohn disease, mesenteric ischemia, radiation enteritis, post-surgical adhesions, and post-operative complications. CASE PRESENTATION: This article presents a clinical case of a 76-year-old Lithuanian patient who underwent parenteral nutrition for four months due to SBS. Before the following diagnosis, the patient had undergone two surgeries. During the hospitalization, life-threatening conditions such as stercoral peritonitis, septic shock, and acute respiratory failure, were observed and treated. As a result of SBS, hypoproteinemia and hypoalbuminemia developed, leading to the prescription of full parenteral nutrition. After correcting the malnutrition, a third surgery was performed, resulting in the discontinuation of parenteral nutrition and the resumption of a regular diet. CONCLUSIONS: Parenteral nutrition is the sole effective method for preserving the lives of patients with a short segment of the intestine. While on parenteral nutrition, patients can be prepared for reconstructive surgery.


Assuntos
Doença de Crohn , Síndrome do Intestino Curto , Adulto , Humanos , Idoso , Síndrome do Intestino Curto/terapia , Síndrome do Intestino Curto/etiologia , Nutrição Parenteral/efeitos adversos , Intestino Delgado , Intestinos/cirurgia , Doença de Crohn/complicações
12.
Chirurgie (Heidelb) ; 95(5): 375-381, 2024 May.
Artigo em Alemão | MEDLINE | ID: mdl-38427034

RESUMO

BACKGROUND: Intestinal transplantation (ITx) is the only causal treatment for complicated chronic intestinal failure after mesenteric ischemia and impending failure of parenteral supplementation. Isolated or combined ITx with the inclusion of the intestine is associated with demanding immunological, perioperative and infection associated challenges. AIM: The characterization of chronic intestinal failure, the indications, transplant survival, transplantation techniques and success rates. MATERIAL AND METHODS: Collection, summary and critical appraisal of international guidelines, the guidelines of the German Medical Chamber, and the international literature. RESULTS: The first successful ITx were performed in 1987 and 1988 at the University of Kiel Germany and the University of Pittsburgh, USA. The number of ITx rose continuously but in phases from the end of the 1990s to over 200 per year but has currently decreased to 100-150 per year due to optimized intestinal rehabilitation. While the 1­year and 3­year transplant survival rates were 30% and 20% before 1991, they increased in phases up to 60% and 50%, respectively, after 1995 and have now achieved almost 80% and 70%, respectively. CONCLUSION: The substantial improvement in the results of ITx can be partly explained by progress in operative techniques, intensive care medicine and a better understanding of mucosal immunity; however, optimized strategies in immunosuppression as well as prevention of infectious diseases and malignancies have also made decisive contributions.


Assuntos
Enteropatias , Insuficiência Intestinal , Isquemia Mesentérica , Síndrome do Intestino Curto , Humanos , Síndrome do Intestino Curto/cirurgia , Síndrome do Intestino Curto/complicações , Isquemia Mesentérica/cirurgia , Isquemia Mesentérica/complicações , Intestinos/cirurgia , Enteropatias/complicações , Enteropatias/cirurgia , Doença Crônica
13.
Pediatr Surg Int ; 40(1): 79, 2024 Mar 16.
Artigo em Inglês | MEDLINE | ID: mdl-38492075

RESUMO

BACKGROUND: Intestinal Failure, parenteral nutrition (PN) dependence, and subsequent liver disease are the most challenging and life-threatening complications of short bowel syndrome experienced by patients with total intestinal aganglionosis. Skipped Aganglionic Lengthening Transposition (SALT) showed to be a promising procedure to overcome such problems. We herein report the results of two patients who underwent SALT at the Umberto Bosio Center for Digestive Diseases. PATIENTS AND METHODS: Between November 2019 and July 2022, 2 patients with total intestinal aganglionosis underwent SALT as autologous intestinal lengthening procedure. Perioperative data and long-term outcomes are reported. Patient #1-A 18 month-old male (PN dependant) with 30 cm of ganglionated bowel at birth experienced a 35% increase of intestinal length after SALT (from 43 to 58 cm) thanks to three 5 cm interposed aganglionic loops. Postoperative course was uneventful and he was totally weaned by PN after 28 months postoperatively. He is without PN only receiving enteric feeding 53 months after the procedure. Patient #2-A 11 year-old female (PN dependant) with 100 cm of ganglionated jejunum underwent SALT at 11 years and experienced a 19% increase of bowel length thanks to four 5 to 7 cm interposed aganglionic loops. Postoperatively she required excision of two out of the four loops due to severe strictures and inadequate perfusion with a subsequent overall 10% increase of length after SALT. Of note, she improved significantly with a progressive reduction of PN that has been stopped after 18 months. CONCLUSION: Skipped aganglionic lengthening transposition (SALT) seems to be very effective in improving nutrients absorption in patients with total intestinal aganglionosis by increasing absorptive bowel surface and decelerating intestinal flow for a longer and more effective contact of enteric material with ileal mucosa. Provided these impressive results are confirmed in the very long-term, SALT could become a valid alternative for the treatment of patients with total intestinal aganglionosis carrying at birth at least 20 to 30 cm of ganglionated jejunum.


Assuntos
Doença de Hirschsprung , Insuficiência Intestinal , Síndrome do Intestino Curto , Criança , Feminino , Humanos , Lactente , Masculino , Intestino Delgado , Intestinos/cirurgia , Síndrome do Intestino Curto/cirurgia , Resultado do Tratamento
14.
J Clin Pharmacol ; 64(6): 744-754, 2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-38465515

RESUMO

Renal impairment is a common complication in patients with short bowel syndrome with intestinal failure (SBS-IF). Glucagon-like peptide-2 analogs, such as apraglutide, have been developed as a treatment option for SBS-IF. This study assessed the potential for apraglutide overexposure in individuals with severely impaired renal function versus healthy volunteers with normal renal function. In this phase 1, open-label, multicenter, nonrandomized, parallel-group study, a single dose of apraglutide 5 mg was administered subcutaneously to individuals with severely impaired renal function (<30 mL/min/1.73 m2) and healthy volunteers with normal renal function (≥90 mL/min/1.73 m2). Primary pharmacokinetic endpoints were maximum observed concentration (Cmax) and exposure to apraglutide (area under the curve [AUC] from time 0 to infinity [AUCinf], and AUC from time 0 to the last quantifiable concentration [AUClast]). Each group comprised 8 individuals. Results show that patients with severe renal impairment do not have increased apraglutide exposure. Apraglutide achieved a lower Cmax and AUCinf in individuals with severe renal impairment versus those with normal renal function (Cmax = 36.9 vs 59.5 ng/L; AUCinf = 3100 vs 4470 h · ng/mL, respectively). The respective geometric mean ratios were 0.620 and 0.693 for Cmax and AUCinf, and the upper bound of their 90% confidence intervals were <2, indicating patients with severe renal impairment were not overexposed to apraglutide versus those with normal renal function. Adverse events were mild or moderate in severity. Apraglutide does not require dose reduction for any degree of renal impairment and could be used in a broader patient population of renally impaired patients without dose adjustment.


Assuntos
Área Sob a Curva , Insuficiência Renal , Humanos , Masculino , Feminino , Pessoa de Meia-Idade , Adulto , Insuficiência Renal/metabolismo , Idoso , Peptídeo 2 Semelhante ao Glucagon/farmacocinética , Peptídeo 2 Semelhante ao Glucagon/administração & dosagem , Peptídeo 2 Semelhante ao Glucagon/efeitos adversos , Síndrome do Intestino Curto/tratamento farmacológico , Síndrome do Intestino Curto/fisiopatologia
15.
J Pediatr Gastroenterol Nutr ; 78(5): 1149-1154, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-38511559

RESUMO

Patients with short bowel syndrome (SBS) have multiple risk factors for eosinophilic gastrointestinal diseases (EGIDs) including increased risk for intestinal dysbiosis and food allergy compared to their counterparts with normal anatomy. However, there is limited data on the prevalence of EGIDs in children with SBS. We aimed to define the prevalence of EGIDs in an SBS cohort and its association with different risk factors via a retrospective chart review of patients with SBS at Children's National Hospital. The prevalence of eosinophilic esophagitis in our SBS cohort was 10%, eosinophilic gastritis was 4.9%, and eosinophilic enteritis was 4.9%. SBS patients with history of allergy or atopy were more likely to have esophageal and intestinal eosinophilia on biopsy than patients without allergy. The prevalence of EGIDs in our SBS cohort is significantly higher than in the general population and may be associated with allergic polarization.


Assuntos
Enterite , Eosinofilia , Esofagite Eosinofílica , Gastrite , Síndrome do Intestino Curto , Humanos , Masculino , Feminino , Estudos Retrospectivos , Prevalência , Eosinofilia/epidemiologia , Eosinofilia/complicações , Criança , Síndrome do Intestino Curto/complicações , Síndrome do Intestino Curto/epidemiologia , Gastrite/epidemiologia , Gastrite/complicações , Enterite/epidemiologia , Enterite/complicações , Pré-Escolar , Esofagite Eosinofílica/epidemiologia , Esofagite Eosinofílica/complicações , Adolescente , Fatores de Risco , Lactente
16.
Am J Clin Nutr ; 119(5): 1187-1199, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-38431119

RESUMO

BACKGROUND: Short bowel syndrome with intestinal failure (SBS-IF) is a rare but devastating medical condition. An absolute loss of bowel length forces the patients into parenteral support dependency and a variety of medical sequelae, resulting in increased morbidity and mortality. Interdisciplinary treatment may include therapy with the effective but expensive intestinotrophic peptide teduglutide. OBJECTIVES: A time-discrete Markov model was developed to simulate the treatment effect [lifetime costs, quality-adjusted life years (QALYs), and life years (LYs)] of teduglutide plus best supportive care compared with best supportive care alone in patients with SBS-IF. METHODS: The health status of the model was structured around the number of days on PS. Clinical data from 3 data sets were used: 1) an Austrian observational study (base case), 2) pooled observational cohort studies, and 3) a prospective study of teduglutide effectiveness in parenteral nutrition-dependent short bowel syndrome subjects. Direct and indirect costs were derived from published sources. QALYs, LYs, and costs were discounted (3% per annum). RESULTS: Under the base case assumption, teduglutide is associated with costs of 2,296,311 € per patient and 10.78 QALYs (13.74 LYs) over a lifetime horizon. No teduglutide is associated with 1,236,816 € and 2.24 QALYs (8.57 LYs). The incremental cost-utility ratio (ICUR) amounts to 123,945 €. In case of the pooled clinical data set, the ICUR increases to 184,961 €. If clinical data based on the study of teduglutide effectiveness in parenteral nutrition-dependent short bowel syndrome subjects were used, the ICUR increased to 235,612 €. CONCLUSIONS: Teduglutide in treating patients with SBS-IF meets the traditional cost-effectiveness criteria from a European societal perspective. Nevertheless, the varying concentrations of teduglutide efficacy leave a degree of uncertainty in the calculations.


Assuntos
Análise Custo-Benefício , Fármacos Gastrointestinais , Cadeias de Markov , Peptídeos , Anos de Vida Ajustados por Qualidade de Vida , Síndrome do Intestino Curto , Síndrome do Intestino Curto/tratamento farmacológico , Síndrome do Intestino Curto/economia , Síndrome do Intestino Curto/terapia , Humanos , Peptídeos/uso terapêutico , Peptídeos/economia , Fármacos Gastrointestinais/uso terapêutico , Fármacos Gastrointestinais/economia , Adulto , Europa (Continente) , Feminino , Masculino , Nutrição Parenteral/economia , Pessoa de Meia-Idade , Estudos Prospectivos
17.
Clin Nutr ; 43(5): 1043-1050, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-38554476

RESUMO

BACKGROUND & AIMS: Acid-base disturbances are common in short bowel (SB) patients due to increased intestinal bicarbonate loss. However, the resulting systemic acid load has not been quantified. Base excess is used to monitor metabolic acid-base disturbances but inadequately reflects the acid load. Our aim was to investigate the systemic acid/base load in SB-patients to obtain quantitative estimates to guide the composition of parenteral support. METHODS: We calculated total acid load in SB patients by summing 24-h urinary net acid excretion (NAE) and the provision of base equivalents in parenteral support. We then compared differences among anatomical SB-types: jejunostomy (SB-J), jejunocolostomy (SB-JC), and jejunoileostomy (SB-JIC). 47 urine samples from 34 SB patients were analyzed for bicarbonate (HCO3-), ammonium (NH4+), and titratable acid (TA) concentrations. NAE was calculated as (TA + NH4+) - HCO3-. Mixed-effects repeated-measures models were used to statistically examine differences between SB-types and associations with parenteral nutrition and NAE. A healthy cohort served as control. RESULTS: In comparison to SB-J, SB-JC patients had a 4.1 mmoL/l lower base excess (95% CI: -6.3 to -1.8) and an 84.5 mmol/day higher total acid load (CI: 41.3 to 127.7). There were no significant differences between SB-JIC and SB-J regarding base excess, NAE, or total acid load. Higher amounts of infused acetate, sodium, and chloride, but not the acetate/chloride ratio, were associated with lower NAE and higher base excess. CONCLUSIONS: Due to increased colonic bicarbonate loss, patients with SB-JC have a ∼4.4-fold higher acid load than healthy controls. The ion transport mechanisms mediating this bicarbonate loss from the remaining colon need further experimental investigation. NAE could be a useful tool to adjust base infusion in SB.


Assuntos
Bicarbonatos , Colo , Nutrição Parenteral , Humanos , Masculino , Feminino , Pessoa de Meia-Idade , Nutrição Parenteral/métodos , Colo/cirurgia , Colo/metabolismo , Adulto , Síndrome do Intestino Curto/cirurgia , Síndrome do Intestino Curto/terapia , Anastomose Cirúrgica , Desequilíbrio Ácido-Base , Idoso , Equilíbrio Ácido-Base
18.
Nutr Clin Pract ; 39(3): 634-640, 2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-38491966

RESUMO

BACKGROUND: Chronic hepatic complications are common in patients with short bowel syndrome-associated intestinal failure (SBS-IF). Teduglutide, a glucagon-like peptide-2 analogue, demonstrated efficacy in reducing parenteral nutrition and/or intravenous fluid dependence among patients with SBS-IF in phase 3 clinical studies. METHODS: This was a post hoc analysis of pooled data from two separate randomized, double-blind, placebo-controlled, multinational phase 3 clinical studies. Adult patients with SBS-IF with parenteral nutrition and/or intravenous fluid dependence without liver disease at baseline were randomized to treatment with the glucagon-like peptide-2 analogue teduglutide (0.05 or 0.10 mg/kg/day) or placebo subcutaneously once daily for 24 weeks. Mixed-effects models assessed the baseline predictors of change in liver chemistries. RESULTS: Between baseline and week 24, teduglutide treatment (n = 109) was associated with least squares mean reductions in aspartate aminotransferase (-7.51 IU/L; P = 0.014), alanine aminotransferase (-12.15 IU/L; P = 0.002), and bilirubin (-5.03 µmol/L [-0.057 mg/dl]; P < 0.001) compared with that of the placebo (n = 59). These values were independent of reductions in parenteral nutrition and/or intravenous fluid dependence. CONCLUSION: Teduglutide treatment was associated with reductions in liver chemistries by week 24, which is beneficial for patients with SBS-IF beyond improvements in parenteral nutrition and/or intravenous fluid dependence. Future studies should examine how long-term teduglutide might mitigate the risk of liver disease in patients with SBS-IF.


Assuntos
Fármacos Gastrointestinais , Fígado , Peptídeos , Síndrome do Intestino Curto , Humanos , Síndrome do Intestino Curto/complicações , Síndrome do Intestino Curto/tratamento farmacológico , Masculino , Feminino , Pessoa de Meia-Idade , Peptídeos/uso terapêutico , Método Duplo-Cego , Adulto , Fígado/efeitos dos fármacos , Fígado/metabolismo , Fármacos Gastrointestinais/uso terapêutico , Fármacos Gastrointestinais/farmacologia , Aspartato Aminotransferases/sangue , Nutrição Parenteral/métodos , Alanina Transaminase/sangue , Idoso , Bilirrubina/sangue , Insuficiência Intestinal/tratamento farmacológico , Resultado do Tratamento , Hepatopatias
19.
Clin Nutr ESPEN ; 60: 281-284, 2024 04.
Artigo em Inglês | MEDLINE | ID: mdl-38479922

RESUMO

BACKGROUND AND AIMS: Short bowel syndrome is a malabsorption disorder typically caused by the physical loss of a portion of the intestine, whereafter the body is unable to adequately absorb nutrients, fluids, and electrolytes. Many patients with short bowel syndrome are reliant on home parenteral nutrition through a tunneled or peripherally inserted central catheter to ensure sufficient hydration and nutrition. Central venous catheters are a nidus for bacteria, and patients are at risk for infections associated with high levels of morbidity and mortality. Lactobacillus is a ubiquitous microorganism that most frequently colonizes mucosal surfaces such as the gastrointestinal tract. Lactobacillus bacteremia is rare, with limited occurrence in current medical literature. METHODS: Our patient is a 60-year-old female with a past medical history significant for multiple abdominal surgeries resulting in short bowel syndrome, with subsequent dependence on home parenteral nutrition via peripherally inserted central catheter. She had type III chronic intestinal failure, category D2, and stage 1 moderate malnutrition. She was originally admitted to the hospital for a presumed pulmonary embolism and was found to have a deep vein thrombosis in the setting of her peripherally inserted central catheter. On admission her abdominal exam was unremarkable, she denied abdominal pain, and her only gastrointestinal complaint was chronic stable diarrhea. During the hospitalization she developed severe left lower quadrant abdominal pain and noted decreased frequency of her bowel movements. A computed tomography scan of her abdomen revealed chronic stable intestinal distension and was concerning for obstruction. Clinically she remained without symptoms of acute obstruction or ileus. During the admission she became febrile, with blood cultures from her peripherally inserted central catheter and peripheral IV growing out gram negative rods determined to be lactobacillus bacteremia. The infectious disease team recommended removal of her peripherally inserted central catheter given their concern for a line infection. RESULTS: The patient was treated with broad-spectrum antibiotics, did well clinically, and was ultimately discharged following reinsertion of her peripherally inserted central catheter after negative repeat blood cultures. Though she initially did well in the outpatient setting, she ultimately passed away ten months later after re-presenting to the hospital in septic shock, secondary to bowel ischemia and suspected fungemia of her peripherally inserted central catheter. CONCLUSIONS: In this case report, we describe an unusual case of a patient with short bowel syndrome on chronic parenteral nutrition who developed catheter-associated lactobacillus bacteremia - the first reported case in an adult patient on parenteral nutrition.


Assuntos
Bacteriemia , Cateteres Venosos Centrais , Enteropatias , Nutrição Parenteral no Domicílio , Síndrome do Intestino Curto , Humanos , Adulto , Feminino , Pessoa de Meia-Idade , Lactobacillus , Síndrome do Intestino Curto/complicações , Síndrome do Intestino Curto/terapia , Cateteres Venosos Centrais/efeitos adversos , Cateteres Venosos Centrais/microbiologia , Bacteriemia/epidemiologia , Nutrição Parenteral no Domicílio/efeitos adversos , Dor Abdominal/etiologia
20.
Clin Nutr ESPEN ; 60: 298-302, 2024 04.
Artigo em Inglês | MEDLINE | ID: mdl-38479925

RESUMO

BACKGROUND & AIMS: Disruptions in the home parenteral nutrition (HPN) process may lead to failure to achieve the intended treatment purposes. This study aimed to evaluate the mid-term clinical outcome in a group of patients with short bowel syndrome (SBS) after a sudden change in the type of home parenteral nutrition programs from customized to commercially premixed admixtures. METHODS: The study conducted in 2020 identified 51 patients with SBS: 27 (53%) women and 24 (47%) men. The SBS were classified as SBS with end jejunostomy 23 (45%) patients, with jejuno-colon anastomosis 23 (45%) patients, with jejuno-ileo anastomosis and total colon in continuity 5 (10%) patients. The following therapeutic program-related data were analysed: changes in nutritional status, body mass index (BMI), Controlling Nutritional Status (CONUT) score, and biochemical assessment. For statistical analysis, the Wilcoxon rank-sum and signed-rank paired tests with continuity corrections were used to compare the results. A p-value of <0.001 was considered statistically significant. RESULTS: There was no statistically significant difference between the analysed groups in total energy, amino acid concentrations, and intravenous volume supplementation. BMI and CONUT assessments of nutritional status and selected biochemical parameters were stable during the study period. CONCLUSIONS: The study demonstrated that a sudden change in the HPN therapy program from parenteral admixtures, tailored to meet individual patients' needs, to commercially premixed admixtures had no significant impact on the mid-term clinical condition of patients with SBS.


Assuntos
Nutrição Parenteral no Domicílio , Síndrome do Intestino Curto , Masculino , Humanos , Feminino , Síndrome do Intestino Curto/terapia , Estado Nutricional , Índice de Massa Corporal
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