Jason S. Soden

Failure of resolution of portal fibrosis during omega-3 fatty acid lipid emulsion therapy in two patients with irreversible intestinal failure.

Parenteral omega-3 fatty acid lipid emulsions have been evaluated for their potential role in reversing intestinal failure-associated liver disease. We report our experience using Omegaven in 2 patients with irreversible intestinal failure and intestinal failure-associated liver disease. Despite biochemical and histologic improvement in cholestasis, both patients had persisting, significant portal fibrosis on liver biopsy.

Subcutaneous vitamin E ameliorates liver injury in an in vivo model of steatocholestasis

Several genetic metabolic liver diseases share the pathological features of combined steatosis and cholestasis, or steatocholestasis. The aims of this study were to develop and characterize an in vivo model for steatocholestasis and to evaluate the effects of an antioxidant treatment on liver injury, oxidative stress, and mitochondrial perturbations in this model. Obese and lean Zucker rats received intravenous (IV) injections of glycochenodeoxycholic acid (GCDC) and were killed 4 hours later. Liver enzymes were measured; the liver histology was assessed, and hepatic mitochondria were analyzed for mitochondrial lipid peroxidation. In separate experiments, rats received daily injections of subcutaneous (SQ) vitamin E before GCDC infusion. Bile acid–induced injury (serum AST and ALT and liver histology) was more severe in the obese rats than in the lean rats, characterized predominantly by extensive cell necrosis with minimal evidence of apoptosis. SQ vitamin E provided significant protection against IV GCDC‐induced hepatic injury, in vitro GCDC‐induced permeability transition, and cytochrome C and apoptosis‐inducing factor release from isolated mitochondria. Conclusion: Steatosis sensitizes the liver to bile acid–induced necrotic hepatocyte injury, which is responsive to vitamin E therapy. (HEPATOLOGY 2007.)

Clinical assessment of the child with intestinal failure

The management of the child with intestinal failure is complex, and it is developing into a multispecialty field of its own led by expert teams of both transplant and nontransplant surgeons, gastroenterologists, and dieticians. Patients are at risk for medical, surgical, and nutritional complications that should be anticipated so that they can be prevented or managed appropriately. Catheter associated infections and intestinal failure associated liver diseases are important complications that impact the likelihood of bowel adaptation and long-term survival. The clinical assessment of a pediatric intestinal failure patient should include evaluation of the child within the context of recognized prognostic factors.

Multidisciplinary care of children with repaired esophageal atresia and tracheoesophageal fistula.

Children with congenital esophageal atresia with tracheoesophageal fistula (TEF) require complex medical and surgical care, but few guidelines exist to guide the long term care of this population. The purpose of this study is to describe the findings and initial management of a comprehensive aerodigestive team in order to understand the ongoing needs of children with repaired TEF.

First successful antegrade single-balloon enteroscopy in a 3-year-old with occult GI bleeding.

BACKGROUND Balloon enteroscopy is an emerging technique to allow access to the small intestine for both diagnostic and therapeutic purposes. To date, there have been few published data documenting the safety and efficacy of balloon enteroscopy in small children. OBJECTIVE To describe our experience with single-balloon enteroscopy (SBE) in a 37-month-old toddler with occult GI bleeding. DESIGN A single case report. SETTING A free-standing, academic childrens hospital in Denver, Colorado. PATIENT The patient was a 37-month-old, 13.5-kg toddler with persistent heme-positive stools, severe microcytic anemia, and hypoalbuminemia. Previous workup was significant for eosinophilic inflammation in the antrum and a video capsule study showing erythematous lesions in the small bowel. INTERVENTION An antegrade SBE was performed with the child under general endotracheal anesthesia, with biopsy specimens obtained from identified lesions in the jejunum and ileum. MAIN OUTCOME MEASUREMENTS Complications and successful treatment of symptoms were the primary endpoints. RESULTS The procedure was performed successfully in 85 minutes, passing an estimated 200 cm beyond the pylorus, without complications. Identification of the lesions as consistent with eosinophilic enteropathy led to successful treatment with an elimination diet and corticosteroids. LIMITATIONS The primary limitation of this study is that it is a single case report. Therefore, it is difficult to make a generalized statement regarding the safety and efficacy of balloon enteroscopy in toddlers of this size. CONCLUSIONS Antegrade SBE can be a well-tolerated and effective procedure to evaluate occult GI bleeding in children as young as 3 years of age. Further study is needed to better establish safety parameters for balloon enteroscopy in small pediatric patients.

Follow-Up Survey on Functionality of Nutrition Documentation and Ordering Nutrition Therapy in Currently Available Electronic Health Record Systems

BACKGROUND This is a follow-up survey to reassess the safety and efficacy of nutrition content in the available electronic health record (EHR) systems. MATERIALS AND METHODS Members of the American Society for Parenteral and Enteral Nutrition (A.S.P.E.N.), American Society for Nutrition, and the Academy for Nutrition and Dietetics were asked to participate in an online survey. The survey included questions from a 2012 EHR survey on the safety and efficacy in 5 nutrition content areas as well as questions from previous 2003 and 2011 A.S.P.E.N. parenteral nutrition (PN) surveys. RESULTS Percent of respondents using an EHR and using the EHR for less than 1 year increased between 2012 and 2014 (86%-94%, P < .05; 11%-16%, P < .05, respectively). However, there was no improvement in the safety and efficacy of the 5 nutrition content areas, with a significant decrease in 2 of these areas, ordering oral nutrition supplements and ordering PN. The top-rated EHR vendors had a higher average favorable response rate in regards to safety and efficacy in the nutrition content areas but even the top-rated EHR vendor had only a 60% average in favorable responses. Reported use of electronic PN ordering and a direct interface between the EHR and the automated compounding device (ACD) significantly increased from 2003 to 2011 to 2014 (29% to 33% to 63% and 16% to 19% to 28%, respectively, P < .05). CONCLUSIONS This is a call to action to nutrition support clinicians, societies, and organizations to proactively be involved in initiatives to educate clinicians and collaborate with EHR vendors to enhance the EHR systems to improve the safety and efficacy of providing nutrition therapy in hospitalized patients.

Hepatic veno-occlusive disease and human herpes virus 7 infection in primary agammaglobulinemia.

Hepatobiliary disease has been described in Brutons x-linked agammaglobulinemia; however, veno-occlusive disease has not been reported in this setting. We report a case of end-stage liver disease in a patient with x-linked agammaglobulinemia who was found to have VOD and evidence of human herpes virus 7 infection in the explanted liver after transplantation.

Resistance of Young Rat Hepatic Mitochondria to Bile Acid-Induced Permeability Transition: Potential Role of Alpha Tocopherol

Retention of bile acids within the liver is a primary factor in the pathogenesis of cholestatic liver disorders, which are more common in human infants. The objective of this study was to evaluate developmental changes in mitochondrial factors involved in bile acid-induced hepatocyte injury. Hepatic mitochondria from adult rats (aged 9 wk) underwent a mitochondrial permeability transition (MPT) and release of cytochrome c upon exposure to glycochenodeoxycholic acid. In contrast, mitochondria from young rats (age 6–36 d) were resistant to MPT induction and cytochrome c release. Neither mitochondrial levels of MPT-associated proteins (voltage-dependent anion channel, cyclophilin D, or adenine nucleotide translocase), Bcl-2 family proteins, nor antioxidant enzymes explained this resistance. Mitochondria from young rats contained 2- to 3-fold higher α-tocopherol (α-TH). In vivo α-TH enrichment of adult hepatic mitochondria increased their MPT resistance. Tetra-linoleoyl cardiolipin (TL-CL), the primary molecular species of CL, was reduced in mitochondria of the young rat; however, enrichment with CL and TL-CL only modestly increased their MPT susceptibility. In conclusion, we observed an unexpected resistance in young rats to bile acid induction of mitochondrial cell death pathways, which may be related to developmental differences in membrane composition.

Evaluating the yield of gastrointestinal testing in pediatric patients in aerodigestive clinic

To improve understanding of the interrelatedness of airway and esophageal diagnoses by evaluating the yield of procedural and radiographic testing of the gastrointestinal tract in children with airway conditions by their referring diagnoses in a pediatric aerodigestive clinic.

Bacterial Overgrowth and Intestinal Microbiome

Alterations in the gut microbiome, including bacterial overgrowth and dysbiosis, play a central role in the pathophysiology of short bowel syndrome and intestinal failure. By definition, patients with intestinal failure require TPN due to significant anatomic, mucosal, or motility disturbances. These factors in addition to pharmacologic and systemic comorbidities remodel the intestinal flora, creating a cascade of downstream pathophysiologic and clinical sequelae. The unique relationship between microbiota, epithelial barrier function, and mucosal and systemic inflammatory pathways likely drives a multitude of symptoms in intestinal failure, from intraluminal malabsorptive symptoms to severe extraintestinal complications, including liver disease. At present, diagnostic evaluations including hydrogen breath testing and duodenal aspiration/culture are considered, but may be limited in accuracy and yield. Therefore, antibiotic therapy is commonly used by intestinal rehabilitation centers. Advancements in microbiomics are likely to yield improved understanding of pathogenesis and applicable clinical evaluations that may direct future therapies in this field.