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Short bowel syndrome children

Federica Pederiva, Basem Khalil, Antonino Morabito, Sarah J Wood
BACKGROUND:  Short bowel syndrome (SBS) has an impact on children and their families not only physically, but also emotionally, mentally, and socially. This study aimed to evaluate quality of life and family impact in patients with SBS, using the Pediatric Quality of Life Inventory (PedsQL) measurement model. MATERIALS AND METHODS:  PedsQL questionnaires were administered to patients with SBS followed at the Pediatric Surgery of Royal Manchester Children's Hospital...
January 30, 2018: European Journal of Pediatric Surgery
Mitchell A Rees, Nikhil B Amesur, Ruy J Cruz, Amir A Borhani, Kareem M Abu-Elmagd, Guilherme Costa, Anil K Dasyam
Intestinal transplantation has evolved from its experimental origins in the mid-20th century to its status today as an established treatment option for patients with end-stage intestinal failure who cannot be sustained with total parenteral nutrition. The most common source of intestinal failure in both adults and children is short-bowel syndrome, but a host of other disease processes can lead to this common end-point. The development of intestinal transplantation has presented multiple hurdles for the transplant community, including technical challenges, immunologic pitfalls, and infectious complications...
March 2018: Radiographics: a Review Publication of the Radiological Society of North America, Inc
Amber Smith, Mary Beth Feuling, Catherine Larson-Nath, Catherine Karls, Megan Van Hoorn, Cassandra L S Walia, Carly Leon, Elaine Danner, Pam Opichka, Lori Duesing, Alfonso Martinez, Praveen S Goday
BACKGROUND: The primary hypothesis of this article is that a team approach in creating a protocolized laboratory monitoring schedule for home parenteral nutrition (PN) patients improves patient safety by decreasing the occurrence of nutrition deficiencies and is cost-effective. METHODS: In this prospective cohort study of home PN patients, each patient followed an established protocol of laboratory monitoring and weekly review by an interdisciplinary team of dietitians, nurses, and physicians...
January 2018: JPEN. Journal of Parenteral and Enteral Nutrition
Lorenzo Norsa, Solene Artru, Cecile Lambe, Cecile Talbotec, Benedicte Pigneur, Frank Ruemmele, Virginie Colomb, Carmen Capito, Christophe Chardot, Florence Lacaille, Olivier Goulet
BACKGROUND & AIMS: Intestinal rehabilitation is the preferred treatment for children with short bowel syndrome (SBS) whatever the residual bowel length, and depends on the accurate management of long-term parenteral nutrition (PN). If nutritional failure develops, intestinal transplantation (ITx) should be discussed and may be life-saving. This study aimed to evaluate survival, PN dependency and nutritional status in children with neonatal very SBS on PN or after ITx, in order to define indications and timing of both treatments...
February 15, 2018: Clinical Nutrition: Official Journal of the European Society of Parenteral and Enteral Nutrition
Antje Allendorf, Ruth Dewitz, Joy Weber, Shahrzad Bakthiar, Rolf Schloesser, Udo Rolle
PURPOSE: Necrotizing enterocolitis (NEC) in very low birth weight infants is a risk factor for developmental delay. To our knowledge, there are no studies published investigating the neurodevelopmental outcome of patients with NEC comparing surgically treated and conservatively treated patients versus match paired controls. The aim of this retrospective case control study was to measure the neurodevelopmental outcome of patients with NEC who were treated surgically or conservatively METHODS: All patients were identified, who have been diagnosed with NEC (ICD-10 code, P77) born between 2006 and 2013...
January 31, 2018: Journal of Pediatric Surgery
Katarzyna Olszewska, Janusz Ksiazyk, Dariusz Kozlowski, Magdalena Pajdowska, Malgorzata Janusz, Maciej Jaworski
AIM: Children with ultra-short bowel syndrome (USBS) have not been extensively studied to date because the condition is rare. The aim of the study was to assess the nutritional status of children with USBS receiving home parenteral nutrition, by using citrulline serum concentration and cholestasis. METHODS: We studied 17 patients with USBS, with a median age of 6.6 years and median duration of parenteral nutrition of 6.6 years. The study was carried out at The Children's Memorial Health Institute, Warsaw, from January 2014 to January 2015...
February 6, 2018: Acta Paediatrica
Elie Abi Nader, Cécile Lambe, Cécile Talbotec, Liping Dong, Bénédicte Pigneur, Olivier Goulet
BACKGROUND: Infants with intestinal failure (IF) are at increased risk of malnutrition and require adapted nutrition support. Optimal weight gain during nutrition rehabilitation should occur at the velocity of statural age (adjusted to the 50th percentile of height) and not chronological age. The aim of this study was to assess the relationship between weight gain during catch-up growth, nonprotein energy intake (NPEI) provided by total parenteral nutrition (TPN), and resting energy expenditure (REE) in children with severe malnutrition due to IF...
January 2018: JPEN. Journal of Parenteral and Enteral Nutrition
Carmina Wanden-Berghe Lozano, Nuria Virgili Casas, Esther Ramos Boluda, Cristina Cuerda Compés, José Manuel Moreno Villares, José Luis Pereira Cunill, Carmen Gómez Candela, Rosa Burgos Peláez, Mª Ángeles Penacho Lázaro, Antonio Pérez de la Cruz, Julia Álvarez Hernández, Montserrat Gonzalo Marín, Pilar Matía Martín, Ceferino Martínez Faedo, Eva Ángeles Sánchez Martos, Alejandro Sanz Paris, Cristina Campos Martín, Tomás Martín Folgueras, M Ángela Martín Palmero, María de Los Ángeles Martín Fontalba, Luis Miguel Luengo Pérez, Ana Zugasti Murillo, María José Martínez Ramírez, Fátima Carabaña Pérez, Cecilia Martínez Costa, Patricia Díaz Guardiola, Cristina Tejera Pérez, Rosa Mª Parés Marimón, José Antonio Irles Rocamora, Carmen Garde Orbaiz, Miguel Ángel Ponce González, María Victoria García Zafra, Rebeca Sánchez Sánchez, Juan Ramón Urgeles Planella, Antxón Apezetxea Celaya, Olga Sánchez-Vilar Burdiel, Clara Joaquín Ortiz, José Pablo Suárez Llanos, Begoña Pintor de la Maza, Pere Leyes García, Mª Carmen Gil Martínez, Silvia Mauri Roca, María José Carrera Santaliestra
OBJECTIVE: To communicate HPN data obtained from the HPN registry of the NADYA-SENPE group ( for the year 2016. MATERIAL AND METHODS: Descriptive analysis of the data collected from adult and pediatric patients with HPN in the NADYA-SENPE group registry from January 1st, 2016 to December 31st, 2016. RESULTS: There were 286 patients from 42 Spanish hospitals (54.2% women), 34 children and 252 adults, with 294 episodes, which represent a prevalence rate of 6...
November 24, 2017: Nutrición Hospitalaria: Organo Oficial de la Sociedad Española de Nutrición Parenteral y Enteral
Raghav Chandra, Anil Kesavan
Pediatric short bowel syndrome (SBS) is a serious condition which occurs in children with congenital or acquired reduction in length of the small intestine. SBS results in excessive fluid loss, nutrient malabsorption, electrolyte abnormalities, increased susceptibility to infections, parenteral nutrition associated complications and affects weight gain and growth. In children, SBS is debilitating and uniformly fatal without treatment. The primary goal of treatment is to restore enteral autonomy and reduce long-term dependence on parenteral support by increasing the absorptive potential of the remnant intestine...
December 26, 2017: Clinical Journal of Gastroenterology
Christina Belza, Kevin Fitzgerald, Nicole de Silva, Yaron Avitzur, Karen Steinberg, Glenda Courtney-Martin, Paul W Wales
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...
December 4, 2017: Annals of Surgery
Shweta S Namjoshi, Sarah Muradian, Hannah Bechtold, Laurie Reyen, Robert S Venick, Elizabeth A Marcus, Jorge H Vargas, Laura J Wozniak
BACKGROUND: Home parenteral nutrition (PN) is a lifesaving therapy for children with intestinal failure (IF). Our aims were to describe the prevalence of micronutrient deficiencies (vitamin D, zinc, copper, iron, selenium) in a diverse population of children with IF receiving PN and to identify and characterize risk factors associated with micronutrient deficiencies, including hematologic abnormalities. METHODS: Data were collected on 60 eligible patients through retrospective chart review between May 2012 and February 2015...
February 1, 2017: JPEN. Journal of Parenteral and Enteral Nutrition
Federica Porcaro, Maria Giovanna Paglietti, Antonella Diamanti, Francesca Petreschi, Alessandra Schiavino, Valentina Negro, Valentina Pecora, Alessandro Fiocchi, Renato Cutrera
BACKGROUND: Medications with methyl-prednisolone sodium succinate containing lactose, which potentially contains traces of cow's milk proteins (CMP), could cause allergic reactions or compromise treatment of acute allergic reactions in sensitized patients. CASE PRESENTATION: We describe the unusual case of a one-year-old child affected by short bowel syndrome and history of severe cow's milk allergy (CMA) and anaphylactic reaction due to intravenous administration of methyl-prednisolone sodium succinate (Solu-Medrol 40 mg, Pfizer)...
November 17, 2017: Italian Journal of Pediatrics
Lynn M Malec, James Cooper, Jeffrey Rudolph, Marian G Michaels, Margaret V Ragni
BACKGROUND: Central venous access devices (CVADs) are essential for total parenteral nutrition administration in patients with short bowel syndrome (SBS). However, they are fraught with complications including infection and venous thromboembolism (VTE), which increases associated morbidity and mortality in this population. There is evidence linking the development of CVAD-associated thrombosis and line-related infection. Thus, it has been postulated that prevention of catheter-related clot formation could minimize the risk of infection originating from the catheter...
November 10, 2017: Journal of Pediatric Gastroenterology and Nutrition
Lissette Jimenez, Danielle A Stamm, Brittany Depaula, Christopher P Duggan
We describe 3 patients with short bowel syndrome who had persistently elevated serum methylmalonic acid (MMA) levels while being treated for vitamin B12 deficiency. Following treatment for presumed small bowel bacterial overgrowth, MMA levels normalized. Among patients with short bowel syndrome, MMA levels may have limited specificity for vitamin B12 deficiency.
January 2018: Journal of Pediatrics
Elisa Ylinen, Laura Merras-Salmio, Riikka Gunnar, Timo Jahnukainen, Mikko P Pakarinen
OBJECTIVE: Although impaired renal function has been a frequent finding among adults with intestinal failure (IF), the data on children is scarce. The aim of this study was to assess renal function in pediatric-onset IF. METHODS: Medical records of 70 patients (38 boys) with pediatric-onset IF due to either short bowel syndrome (n = 59) or primary motility disorder (n = 11) and a history of parenteral nutrition (PN) dependency for ≥1 mo were evaluated. Renal function at the most recent follow-up was studied using plasma creatinine, cystatin C, and urea concentrations and estimated glomerular filtration rate (eGFR)...
January 2018: Nutrition
Javier Bueno, Rosa Burgos, Susana Redecillas, Manuel López, Joaquin Balsells
BACKGROUND: We have recently demonstrated the feasibility of lengthening the duodenum in children with short bowel syndrome and a dilated duodenum. This procedure gains additional intestinal length in a challenging area of autologous gut reconstruction. Herein, we report the successful application of this technique in an adult with ultra-short bowel syndrome. METHODS: A 25-year-old man with a history of mid-gut volvulus was referred to our center for intestinal transplant evaluation...
November 6, 2017: Revista Española de Enfermedades Digestivas
Leel Nellihela, Mohamed Mutalib, David Thompson, Kammermeier Jochen, Manasvi Upadhyaya
BACKGROUND: Pneumatosis intestinalis (PI) is an uncommon and poorly understood condition. Although it can be an incidental finding in asymptomatic individuals, it can also be secondary to life-threatening bowel ischaemia and sepsis. In premature infants, it is a pathognomonic sign of necrotising enterocolitis. There is no consensus regarding management and long-term outcome of children with PI. AIM: Review of our experience of PI in children beyond the early infantile period...
October 7, 2017: Archives of Disease in Childhood
Cristine S Velazco, Brenna S Fullerton, Joseph A Brazzo, Charles R Hong, Tom Jaksic, Carol E Barnewolt
PURPOSE: Small bowel length is the most reliable predictor of enteral independence in pediatric short bowel syndrome. Retrospectively measured bowel lengths on upper GI with small bowel follow-through (UGI/SBFT) were compared to operative measurements. METHODS: A pediatric radiologist and surgical trainees blinded to operative measurements retrospectively analyzed UGI/SBFT studies using the digital radiography curved measurement tool. Children with SBS and severe intestinal failure (parenteral nutrition >90days) at a multidisciplinary intestinal failure program 2002-2015 were included...
December 2017: Journal of Pediatric Surgery
Bruna Bergmann Santos, Isabela Heineck, Giovanna Webster Negretto
OBJECTIVE: To describe how children respond to oral anticoagulation with warfarin, verifying the influence of age, clinical condition, route of administration of warfarin and use of total parenteral nutrition (TPN), as well as to describe risk factors for the occurrence of thrombotic events (TE) in childhood. METHODS: A retrospective descriptive study including all patients ≤18 years old for whom warfarin was prescribed in a university hospital. Patients were divided according to clinical condition, age, route of medication administration and use of TPN...
October 2017: Revista Paulista de Pediatria: Orgão Oficial da Sociedade de Pediatria de São Paulo
Cory M McLaughlin, Nandini Channabasappa, Jesse Pace, Hoa Nguyen, Hannah G Piper
OBJECTIVES: Infants with short bowel syndrome (SBS) require diligent nutritional support for adequate growth. Enteral independence is a primary goal, but must be balanced with ensuring sufficient nutrition. We aimed to describe growth trajectory in infants with SBS as function of nutritional intake during first 2 years of life. METHODS: Infants with SBS were reviewed (2008-2016). z Scores for weight, height, and head circumference (HC) were recorded at birth, 3, 6, 12, 18, and 24 months...
March 2018: Journal of Pediatric Gastroenterology and Nutrition
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