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Crohn Disease

Acknowledgment The authors acknowledge the significant contributions of the prior authors of this chapter who contributed to the last edition of the book. The majority of the text has been updated, but some of the content written previously has been retained. The inflammatory bowel diseases (IBD) can be conceptualized as a spectrum, currently categorized into three main subtypes: Crohn disease (CD), ulcerative colitis (UC), and IBD…

Pediatric Polyposis Syndromes

Introduction Children and adolescents with gastrointestinal polyps may present symptomatically with rectal bleeding, abdominal pain, or intussusception. Alternatively, they may be asymptomatic and referred either because an adult family member has been affected with early-onset colorectal cancers (CRC) or there is a known family history of a polyposis syndrome. Many children fall into the latter category; they are asymptomatic and polyps are detected only as part…

Gastrointestinal Manifestations of Immunodeficiency

Acknowledgments The authors would like to dedicate this chapter to the memory of their beloved colleague, Dr. Tracie Miller, who was a coauthor on prior editions of this chapter. Introduction Advances in the understanding of the adaptive and innate immune systems have led to the characterization of a number of novel primary immune deficiencies, including interleukin (IL)-10 receptor mutation, X-linked inhibitor of apoptosis (XIAP) mutation, ICOS…

Enteric Parasites

Enteric parasites are important agents of disease throughout the world. Although the frequency and severity of parasitic diseases are most extreme in the developing world, changes in worldwide travel, immigration, commerce, day care for young children, and increasing numbers of immunocompromised patients have led to a rising incidence of parasitic diseases in the developed world. Parasitic disease may mimic other gastrointestinal (GI) disorders, such as inflammatory…

Infectious Diarrhea

According to the World Health Organization (WHO), diarrheal disease is the second leading cause of death in children younger than 5 years of age worldwide. It is also the leading cause of malnutrition in children younger than 5 years of age. Globally, diarrhea alone kills more than 2000 children daily, more than the daily death rate from other deadly diseases such as human immunodeficiency virus (HIV),…

Allergic and Eosinophilic Gastrointestinal Disease

Overview Gastrointestinal disorders involving an accumulation of eosinophils include a variety of conditions including classic Immunoglobulin E (IgE)-mediated food allergy, inflammatory bowel disease, gastroesophageal reflux (GER), and the primary eosinophilic gastrointestinal disorders (eosinophilic esophagitis, eosinophilic gastroenteritis [EoG], and eosinophilic colitis). The goal of this chapter is to provide an overview of those conditions that are characterized by an eosinophilic infiltration in the gastrointestinal tract and are…

Intestinal and Multivisceral Transplantation: Recent Advances

Continuous progress has been made over the last few decades in the management of children with intestinal failure. Significant advances include gut rehabilitation, autologous reconstruction, bowel lengthening, and visceral transplantation. Gut replacement is one of the most important milestones in the recent history of organ transplantation. Improved donor management, refinement of surgical techniques, introduction of new immunosuppressive protocols, and improved follow-up care have led to increased…

Short Bowel Syndrome

Definition and Historical Perspective Short bowel syndrome (SBS) is defined in children as a critical reduction in the length and absorptive capacity of the gastrointestinal tract, resulting in the inability of the small bowel to adequately absorb nutrients, fluids, and electrolytes that are required to support normal growth and development. , Clinical manifestations of SBS include diarrhea, weight loss, growth failure, and a higher risk of…

Celiac Disease

Definition Celiac disease (CD) is an immune-mediated systemic disorder elicited by the ingestion of wheat gliadin and related prolamins in genetically susceptible individuals, characterized by a variable combination of gluten-dependent clinical manifestations, CD-specific antibodies, human leukocytes antigen (HLA) DQ2 and/or DQ8 haplotypes, and enteropathy. Historical Background CD was first accurately described by Samuel Gee in 1888, but it was not until the early 1950s that Dicke…

Protein-Losing Enteropathy

Definition Protein-losing enteropathy (PLE) is a clinical syndrome due to a myriad of etiologies and characterized by excessive gastrointestinal (GI) protein loss. The immediate sequel of this protein loss is hypoproteinemia and edema; additional associated symptoms may include abdominal pain and cramping, diarrhea, ascites, pleural and pericardial effusions, and secondary symptoms from malnutrition. Depending on the etiology, additional constituents of lymph may be lost in the…

Protracted Diarrhea

Introduction The intestine is a complex organ system holding the unique ability to digest and absorb food while also coordinating complicated motility and hosting a distinctive immune system. When abnormalities occur in any of these intestinal functions, diarrhea can ensue. Diarrheal illness is a common childhood event which can lead to significant morbidity and even mortality. Infectious diarrhea remains one of the leading causes of childhood…

Maldigestion and Malabsorption

Maldigestion and Malabsorption Many of the common digestive symptoms in pediatrics, including diarrhea and weight loss, are the result of disruption of nutrient digestion or absorption. As such, maldigestion and malabsorption are not specific diagnoses but rather mechanistic categories that result from one or more pathophysiologic events that impair nutrient processing, resulting in compromised nutriture or growth, or other abnormality. Digestive or absorptive compromise can present…

Anatomy and Physiology of the Small and Large Intestines

The small and large intestines are contiguous and occupy most of the abdominal cavity. Working in concert, and with remarkable efficiency, they are responsible for several complex functions including digestion and the absorption of nutrients, among them vitamins and trace elements. Other functions include fluid and electrolyte transport, excretion, and physical and immunologic defense mechanisms. The intestines are morphologically adapted to serve these functions, with distinct…

Gastric Motility Disorders

Introduction The stomach is a complex organ with unique mechanical, sensory, endocrine, and immunological properties. Alterations in gastric motility and sensation have been implicated in several gastrointestinal disorders, including gastroparesis, functional dyspepsia, rumination syndrome, and dumping syndrome. 1 Understanding the evaluation and management of these disorders requires a thorough knowledge of the physiology of gastric motility and sensation. Physiology of Gastric Motility and Sensation Normal gastric…

Helicobacter pylori in Childhood

Four decades ago, pediatric gastritis and peptic ulcer disease (PUD) lacked a putative microbial causative agent. Pediatric flexible upper endoscopy was becoming more available. In 1983, Warren and Marshall proposed that colonization of the human stomach with an organism, now known as Helicobacter pylori , was associated with human disease, specifically PUD. The earliest reports of this bacterium in children noted its coincidence with gastritis and…

Gastritis, Gastropathy, and Ulcer Disease

Gastritis, gastropathy, and peptic ulcer diseases (PUDs) in children are a spectrum of acid-related disorders that can affect the esophagus, stomach, and duodenum. This chapter focuses on gastroduodenal acid–related peptic diseases, excluding Helicobacter pylori gastritis, which is addressed in a separate chapter. Gastritis is characterized by the presence of inflammatory cells, whereas gastropathies demonstrate gastric mucosal damage and abnormalities in the absence of inflammatory cells. PUD…

Congenital Anomalies and Surgical Disorders of the Stomach

Acknowledgment We thank Louisa W. Chiu, MD, for the authorship of the chapter from the fourth edition. Embryology A general understanding of the embryologic development of the stomach is essential to the management of congenital and acquired surgical disorders of the stomach. The primordial stomach is a foregut organ that begins as a pharyngeal structure and then dilates and elongates to form the esophagus and stomach.…

Developmental Anatomy and Physiology of the Stomach

The gastrointestinal tract begins as a primitive tubular system and is one of the first organs to polarize the embryo by forming an entry and exit with an anterior and posterior axis, also known as the craniocaudal axis, extending from the mouth to the cloaca ( Fig. 24.1 ). The nonneural elements of the gut are derived from endodermal and mesodermal cells. Bilateral folding of these…

Other Diseases of the Esophagus

Esophageal Symptoms Pathologic processes in the pediatric esophagus can result in a variety of symptoms. These range from the classic esophageal symptoms of heartburn, odynophagia, dysphagia, chest pain, and regurgitation to nonspecific symptoms such as feeding difficulties, recurrent abdominal pain, or increased fussiness in infants. Dysphagia is defined as difficulty swallowing, whereas odynophagia is defined as painful swallowing. Esophagitis encapsulates more than just chemical damage to…