This review explores the role of neurostimulation in the treatment of disorders of gut-brain interaction, gastroparesis, and chronic constipation. Specific neurostimulation methods including gastric electrical stimulation, percutaneous electrical nerve field stimulation, direct sacral nerve stimulation, posterior tibial nerve stimulation, and transcutaneous parasacral nerve stimulation will be highlighted and discussed. This article also evaluates the safety and efficacy of neurostimulation techniques as well as limitations of existing studies in the literature. Growing evidence suggests that neurostimulation is effective in improving a wide of gastrointestinal symptoms including nausea, abdominal pain, constipation, and fecal incontinence. Despite these encouraging results, important questions remain regarding the optimal frequency, duration, and long-term use of these therapies. Neurostimulation is emerging as a promising technology in the management of pediatric gastrointestinal disorders. Current research indicates that the major forms of neurostimulation are generally effective and safe, with noninvasive transcutaneous methods offering particularly strong safety profiles. These therapies provide the added advantage of targeted treatment while minimizing the systemic side effects commonly associated with pharmacologic interventions. As safety profiles improve, physicians are increasingly utilizing neurostimulation in the management of gastrointestinal disorders especially in patients with refractory disorders of gut-brain interaction.

Cystic fibrosis is a chronic condition that has significant effects on the nutritional status of pediatric patients. Malnutrition is frequently encountered in this population and has been shown to contribute to poor pulmonary and overall disease outcomes. This article will provide an overview of the physiologic and psychosocial challenges toward attaining optimal nutrition in pediatric cystic fibrosis patients. Newer therapies such as CFTR modulators have played significant roles in improving the nutritional status of patients with cystic fibrosis. There is also a greater focus on becoming more aware of psychosocial and cultural barriers in the care of cystic fibrosis patients. Many challenges exist in optimizing nutritional support including but not limited to the patient’s clinical manifestations and disease severity, caregiver ability, and access to care. Both gastrointestinal and non-gastrointestinal disorders lead to insufficient caloric intake, increased loss and metabolic needs, and micronutrient and macronutrient deficiency. Social factors including stressful patient and caregiver relationships and altered body image also contribute to poor nutritional status.

Dupilumab is a humanized monoclonal antibody against the IL-4 receptor alpha subunit that disrupts the type 2 inflammatory response via inhibition of IL-4 and IL-13 signaling. Dupilumab is currently approved for use in the United States for children aged 6 months and older with moderate-to-severe atopic dermatitis, 6 years and older with moderate-to-severe asthma, and 1 year and older with eosinophilic esophagitis. In this review, we summarize the pertinent findings from phase III clinical trials evaluating dupilumab’s safety and efficacy in treating these conditions. We discuss the effect of dupilumab on the disease processes as well as biochemical and histologic changes due to therapy. Dupilumab decreases disease severity and improves patient quality of life while maintaining a favorable safety profile. In addition to clinical improvement, biochemical, molecular, and histologic changes have been reported with dupilumab therapy. Dupilumab holds great promise in treating type-2 immune mediated conditions. Further research into the long-term safety and efficacy of the medication in pediatric patients is necessary.

Dysphagia is a common gastrointestinal complaint in the pediatric population and should raise concern for oropharyngeal as well as esophageal disorders. We describe a 7-year old patient who was admitted to the hospital for sudden onset dysphagia, abdominal pain, and decreased oral intake. Extensive evaluations including endoscopy eventually revealed herpes simplex esophagitis as well as eosinophilic esophagitis. Herpes simplex esophagitis is a rare condition in the immunocompetent population and is typically self-resolving. Eosinophilic esophagitis is a chronic, inflammatory condition characterized by esophageal eosinophilia and signs of esophageal dysfunction. The concurrent presentation of both conditions in the pediatric population has rarely been described.

Iron supplementation is frequently used in the treatment of iron deficiency anemia in the pediatric population. We describe a case of an 11-year old male who developed adverse side effects following treatment with oral ferrous sulfate tablets for 2 months. The diagnosis was made following findings of iron deposition on histology obtained during endoscopy. The iron supplementation was changed from tablet to liquid form, and repeat endoscopy 4 months following initial diagnosis showed resolution of the histologic findings of iron pill–induced gastritis.

Continuous progress has been made over the last few decades in the management of children with intestinal failure. Visceral transplantation is one of the most important milestones to serve this unique patient population. Refinement of surgical techniques, improvement of post-operative care, and introduction of new immunosuppressive protocols especially related to donor-specific antibodies (DSAs) have led to improved outcomes including patient and graft survival. As a result, intestinal and multivisceral transplantation are now an established treatment options for pediatric patients with gut failure. The impact of DSA on visceral allograft outcomes has received increased attention and stimulated the modification of the immunosuppression protocols. With the increasing number of patients surviving visceral transplantation, quality of life has become an important aspect of the outcome measures. In this chapter we describe recent advances in visceral transplantation, the evolving role of DSA, and the current long-term outcomes, including quality of life.

PURPOSE OF REVIEW:Aerodigestive disorders encompass conditions that affect both the airway and gastrointestinal tract. These include conditions such as acquired and congenital defects of the airway and esophagus as well as neuromuscular disorders. Patients often suffer from dysphagia, aspiration, and respiratory disorders. This article will provide a review of current practices in the management of feeding disorders, oropharyngeal dysphagia, and nutritional support in the aerodigestive population. RECENT FINDINGS:Oral aversion, aspiration, and feeding-tube dependence are all commonly encountered problems in the aerodigestive population. Intensive inpatient and outpatient programs along with use of appetite stimulants and psychotropic medications may help to improve feeding-related disorders. Aspiration affects many patients and requires close monitoring of clinical symptoms along with routine assessment with video fluoroscopy. Developments in blenderized feeds and formula supplementation have also provided new options for patients with feeding intolerance. SUMMARY:Patients with aerodigestive disorders require complex medical care, and multidisciplinary teams are the most effective in addressing their medical needs. Advances in feeding, occupational, and pharmacologic therapy have allowed healthcare providers to better address the needs of these patients.

Primary hyperoxaluria type 1 is a rare genetic condition that affects predominantly the renal and urinary system. Although it can present in patients of any age, a large proportion of affected individuals are children. This condition may be challenging to diagnose, and both medical and surgical management are often required. Transplantation of the liver and kidney is the definitive treatment for this condition; however, long-term outcomes in pediatric patients are lacking. This chapter will provide a review of PH1, with a particular focus on liver and kidney transplantation.

This review provides a comprehensive overview of the diagnosis and management of common gastrointestinal conditions found in pediatric patients with aerodigestive disorders. In particular, this review will address the impact of these gastrointestinal conditions on the airway and its contribution to respiratory disease. Multidisciplinary programs help to optimize management for medically complex aerodigestive pediatric patients with gastrointestinal disorders. Aerodigestive patients are susceptible to multiple gastrointestinal conditions which may worsen pulmonary function. Timely diagnosis and treatment of these conditions have improved respiratory outcomes and decreased complications in this patient population.

Intestinal failure and transplantation may significantly impact physical, neurocognitive, and psychosocial development in pediatric patients. Currently, there is a paucity of literature on the effects of intestinal failure and transplantation on these aspects of development. This article will review the current literature and discuss the short and long-term impacts as well as interventions to improve clinical outcomes in children with intestinal failure or those undergoing transplantation. Psychological disorders, neurodevelopmental delay, and social maladaptation are frequently encountered in this patient population. While the main focus is often on medical management, equal emphasis should be placed on other aspects of development such as increasing social support and improving school performance. The transition to adulthood also presents many obstacles for patients and healthcare providers should anticipate challenges such as childbirth, employment, and raising a family. The pre-operative, perioperative, and post-operative periods all represent opportunities for medical intervention. Frequent monitoring of physical, psychosocial, and neurocognitive status helps to improve clinical outcomes and long-term quality of life. Future research should emphasize continued development of multidisciplinary programs and specialized services to help address the physical and psychosocial needs of children with intestinal failure as well as transplant recipients.

Background: The growth of academic medical centres associated with international programmes has raised questions about the benefits and harms of medical experiences abroad for medical trainees and patients. Few studies explore the potential benefits to the institution or country that send these individuals. Objective: This pilot qualitative study explores the benefits medical professionals perceive they bring back to the USA from their global health experiences abroad. Methods: Medical staff who participated in University of North Carolina (UNC)–Project Malawi and who could meet for an in-person interview from October 2016 – March 2017 were recruited. Interviews were guided by an a priori semi-structured questionnaire. Each interview was transcribed and analysed by three reviewers. Results: A total of 8 out of 13 (62%) eligible potential respondents were interviewed. Participants perceived UNC-Project Malawi to benefit themselves, partnership institutions, and patients in Malawi, North Carolina, and throughout the world. After returning to UNC, participants recalled specific instances of increased diagnostic confidence (2/8) influenced by their experiences abroad. In addition, by partnering in research, participants felt that certain concepts were advanced including the syndromic management of symptomatic patients with sexually transmitted infections (1/8), the use of antiretroviral therapy to treat and prevent HIV transmission (2/8), and malaria vaccine development (1/8). Conclusion: Participants perceived that their global health experience either influenced their thought processes concerning the medical management of patients in the USA or resulted in a better understanding of research relevant to the global standard of care for specific diseases. International academic medical centre partnerships can offer rich settings for career skills development and a unique platform for collaborating on the management of clinical diseases that affect patients globally. These benefits can be maximised by establishing bi-directional curriculum goals and defining shared research agendas.

A 13-year-old adolescent girl with a history of autism spectrum disorder presented with a 3-month history of intermittent, generalized abdominal pain and gagging. She had a history of constipation and had been evaluated 6 years previously for abdominal pain and weight loss. At that time, upper endoscopy and colonoscopy were performed to evaluate for inflammatory bowel disease. Both endoscopy and colonoscopy were visually normal; however, biopsies showed focal active inflammation in the ileum, transverse colon, ascending colon, and rectum. She was subsequently lost to follow-up.

This paper presents the developmental process and preliminary results of testing a game-based intervention aimed at promoting emotional health among adolescents between the ages of 13-17 years. There are more than 20 million adolescents between those ages living in the United States. Stress plays a significant factor in the physical, emotional, and psychological well-being of individuals and may particularly affect the quality of life of children. Previous 2D serious games have been developed to give children an avenue to escape from stressors, however many of these games lacked realism and were not as attractive. We designed and developed a virtual reality (VR) game aimed at improving the social and emotional well-being of adolescents. Due to its immersive nature, our preliminary data showed that the VR game was effective in enhancing the emotional well-being of adolescents. Further studies will be needed to be undertaken to confirm its effectiveness in other groups of people.

Eosinophilic esophagitis is a chronic, immune-mediated esophageal condition that may lead to impairment of quality of life in pediatric and young adult patients. We performed a prospective, cross-sectional study on 40 patients between the ages of 2-21 years with an established diagnosis of eosinophilic esophagitis. The study evaluated physical, emotional, social, and school functioning in patients undergoing treatment with proton pump inhibitors, dietary elimination, or swallowed corticosteroids. There were no statistically significant differences in total or domain-specific quality of life scores between proton pump inhibitors, dietary elimination, and swallowed corticosteroid therapy. Overall, total and domain-specific quality of life were well-preserved in patients with eosinophilic esophagitis, with the highest scores reported in social functioning. There were also no statistically significant associations between clinical, endoscopic, and histologic features and quality-of-life measures.

APS-1 is an extremely rare, autosomal recessive condition that often presents with candidiasis, adrenal insufficiency, and hypoparathyroidism. This condition is associated with autoimmune hepatitis in less than 20% of cases, and there have only been a few reports of children with the condition who developed ALF. We present a unique case of an infant with APS-1 who developed ALF and subsequently required liver transplantation.

Acute colonic dilation in pediatric patients with ulcerative colitis (UC) raises a concern for toxic megacolon, but other rare conditions such as sigmoid volvulus may present in a similar manner. We report a rare case of a teenager with UC without prior surgery who developed an obstructing sigmoid volvulus managed with endoscopic detorsion and decompression. Colonic inflammation in patients with UC may result in a volvulus in the absence of other predisposing factors and should be considered in the differential diagnosis of patients with UC who present with obstructive symptoms with an atypical presentation.

Introduction: Autism spectrum disorders (ASD) are associated with a myriad of challenges that include social disabilities, language deficits, and behavioral disorders. Pica and foreign body ingestion have been frequently described in pediatric patients with ASD, developmental delay and psychiatric disorders. The awareness of organic etiologies behind unexplained worsening of non-verbal behavioral alterations allows early intervention which can prevent significant morbidity from complications. Case Description/Methods: A 13-year-old female with ASD, pica and constipation presented to clinic for evaluation of a 3-month intermittent, generalized abdominal pain and gagging following a failed empiric gluten-free diet at home. Six years previously, she underwent an upper endoscopy and colonoscopy to evaluate for inflammatory bowel disease (IBD) in the setting of abdominal pain, constipation and weight loss. At that time, biopsies showed focal active inflammation in the ileum, transverse colon, ascending colon, and rectum. Cyproheptadine had been prescribed with improvement in weight, but she was subsequently lost to follow-up. She was nonverbal and developmentally delayed, with an otherwise non-focal physical examination. Labs were unremarkable except for mild thrombocytosis (500 k/uL). Despite optimizing her bowel regimen and a famotidine trial, her persistent symptoms again raised concern for IBD, and she underwent another upper endoscopy and colonoscopy. This time, she was found to have a foreign body spanning the ileocecal (IC) valve that appeared to be a tampon, which was removed with biopsy forceps. The rest of her endoscopy and colonoscopy were visually and histologically normal. The patient’s mother subsequently recalled that the patient had ingested the contents of a brightly colored package approximately three months earlier after mistaking it for candy. Following foreign body removal, her abdominal pain and gagging resolved. Discussion: Foreign body ingestion affects more than 100,000 patients annually in the United States. Potential sites of foreign body impaction include the pyloric sphincter and IC valve, though majority pass through without complications. In this case, both the size as well as texture of the feminine hygiene product contributed to its inability to pass further into the large intestine. This case highlights the importance of considering foreign body ingestion in the differential diagnosis for patients with developmental delay who present with GI symptoms.

Albumin plays a critical role in a wide range of disease processes; however, the role of albumin in pediatric patients has not been well described. This article aims to review albumin physiology and kinetics in children, albumin’s impact on pediatric diseases, and the utility of albumin as a predictor of clinical outcome. Hypoalbuminemia is seen in a wide range of conditions, including protein-losing enteropathy, hepatic synthetic failure, malnutrition, inflammatory states, and renal disease. While the impact of hypoalbuminemia has been more extensively studied in adult patients, there is a relative paucity of literature in the pediatric population. Hypoalbuminemia is a marker of poor outcome in critically ill children and those undergoing a wide range of medical interventions. Albumin infusions may be an effective therapy for fluid resuscitation and for patients with severe hypoalbuminemia.