CONTENT OUTLINE Pediatric Gastroenterology Subspecialty In-training, Certification, and Maintenance of Certification Examinations

Transcription

1 THE AMERICAN BOARD OF PEDIATRICS CONTENT OUTLINE Pediatric Gastroenterology Subspecialty In-training, Certification, and Maintenance of Certification Examinations i

2 INTRODUCTION This document was prepared by the American Board of Pediatrics Subboard of Pediatric Gastroenterology for the purpose of developing in-training, certification, and maintenance of certification examinations. The outline defines the body of knowledge from which the Subboard samples to prepare its examinations. The content specification statements located under each category of the outline are used by item writers to develop questions for the examinations; they broadly address the specific elements of knowledge within each section of the outline. ii

3 EXAM PERCENTAGE LIST Approximate Percent in Examination I. Gastrointestinal Signs and Symptoms; Pathophysiology II. Normal Anatomy, Physiology, and Development III. Clinical Manifestations and Management of Diseases of the Mouth IV. Clinical Manifestations and Management of Diseases of the Esophagus V. Clinical Manifestations/Management Diseases of the Stomach, Duodenum VI. Clinical Manifestations and Management of Diseases of the Intestines VII. Miscellaneous Diseases Affecting the GI Tract VIII. Clinical Manifestations and Management of Diseases of the Liver IX. Disorders of the Bile Ducts and Gallbladder X. The Pancreas XI. Diagnostic Studies XII Therapy XIII. Nutrition XIV. Psychologic Considerations XV. Ethical Considerations XVI. Core Knowledge in Scholarly Activities Page iii

4 I. Gastrointestinal Signs and Symptoms; Pathophysiology A. Growth failure and malnutrition (see also XIII.G) 1. Mechanisms Know the typical signs and symptoms present in patients with growth failure Be familiar with the gastrointestinal complications associated with malnutrition and growth failure Understand the mechanisms of growth failure in gastrointestinal diseases Understand the concept of catch-up growth Recognize the signs and symptoms of failure to thrive associated with breast feeding difficulties 2. Differential diagnosis Understand the rationale behind the energy and protein requirements for catch-up growth and know how to calculate them Know the laboratory tests useful in differentiating among various causes of growth failure Distinguish among the various causes of growth failure based on growth parameters B. Vomiting and regurgitation 1. Mechanisms Recognize the common nongastrointestinal causes of failure to thrive (eg, Williams syndrome, growth hormone deficiency, renal tubular acidosis, gonadal dysgenesis, etc) Understand the various factors (eg, metabolic, anatomic, infectious, inflammatory, neuromuscular) associated with vomiting Know the physiologic (motor) changes that occur during nausea and vomiting 2. Differential diagnosis Understand the efferent and afferent neural control of vomiting Be aware of the tests to differentiate physiologic disorders associated with vomiting Be aware of the common causes of vomiting in childhood and adolescence (eg, 1

5 brain tumor, metabolic liver disease, anatomic obstruction) Recognize the psychiatric and neurologic causes of vomiting C. Acute abdominal pain 1. Mechanisms Plan diagnostic age-appropriate evaluation of a patient who is vomiting Understand the innervation of the viscera Understand the concept of referred abdominal pain 2. Differential diagnosis Recognize the major pathologic processes producing pain in the abdomen (eg, obstruction, inflammation) Recognize the importance of and how to perform careful, repeated physical examinations in the evaluation of abdominal pain Recognize the common extra-intestinal problems that cause abdominal pain (eg, gynecologic, urologic, musculoskeletal, pulmonary) Recognize and differentiate between various causes of lower abdominal pain (eg, constipation, pelvic inflammatory disease, ectopic pregnancy) Recognize and differentiate between several causes of right upper quadrant pain Be able to select appropriate diagnostic screening tests in the child with abdominal pain D. Chronic abdominal pain 1. Mechanisms Be aware that the differential diagnosis of abdominal pain varies with age of the patient 2. Differential diagnosis Know the clinical features necessary to make a diagnosis of recurrent abdominal pain Formulate a differential diagnosis for a patient with chronic abdominal pain (eg, migraine, peptic disease, constipation, inflammatory bowel disease) Differentiate between organic and non-organic causes of recurrent abdominal pain based on signs and symptoms Understand the role of endoscopy in a patient with nonspecific abdominal pain 2

6 Be familiar with prognostic factors that predict outcome for a patient with chronic abdominal pain Recognize common extraintestinal manifestations of nonorganic chronic abdominal pain Formulate a differential diagnosis for a patient with dyspepsia Know the physiologic abnormalities identifiable in recurrent (functional) abdominal pain E. Colic and gas 1. Mechanisms 2. Differential diagnosis Know the sources of intestinal gas Formulate an appropriate differential diagnosis for fussiness in a 1-month-old infant Devise an appropriate evaluation of fussiness in a 1-month-old infant F. Diarrhea 1. Mechanisms Recognize the clinical symptoms of colic Understand the mechanisms of secretory diarrhea Understand the mechanisms of malabsorptive diarrhea Understand the mechanisms of inflammatory diarrhea 2. Differential diagnosis Understand the basic mechanisms of fluid and electrolyte transport Evaluate a stool for electrolyte content, with appropriate knowledge of the implications of the results Interpret results of stool analysis (eg, leukocytes, ova and parasites, occult blood) Differentiate invasive from secretory diarrhea by stool analysis G. Malabsorption (see also VI.G) Recognize an abnormal stool volume 3

7 1. Mechanisms Understand the intraluminal causes and mechanisms of malabsorption (eg, pancreatic insufficiency) and mucosal damage Recognize the inborn errors of enterocyte enzyme deficiency with accompanying malabsorption 2. Differential diagnosis Understand the hematologic consequences of malabsorptive diseases Recognize the conditions that produce villous atrophy with accompanying malabsorption Recognize conditions that produce dysmotility with accompanying malabsorption Recognize conditions that produce bacterial contamination with accompanying malabsorption Recognize conditions that produce immune deficiency with accompanying malabsorption Recognize conditions that produce bile acid malabsorption Formulate a differential diagnosis for a patient in whom intestinal malabsorption is suspected Plan a diagnostic evaluation of a patient in whom malabsorption is suspected H. Constipation 1. Mechanisms Recognize normal small bowel mucosal histology at various stages of development and anatomic locations Know the causes of constipation, including anatomic, endocrinologic, and neuropathic Know the functional (eg, dietary, psychologic, pharmacologic) causes of constipation 2. Differential diagnosis Understand the pathophysiology of encopresis (with and without fecal withholding/retention) Differentiate functional from organic constipation based on history and physical findings 4

8 I. Gastrointestinal bleeding 1. Mechanisms Be aware of the metabolic disorders associated with constipation Recognize the various causes of gastrointestinal bleeding (eg, anatomic, inflammatory, infectious, hematologic, pharmacologic) 2. Differential diagnosis Recognize prognostic indicators for upper gastrointestinal bleeding caused by varices or ulcers Recognize the causes of rectal bleeding in different age groups Formulate a differential diagnosis of gastrointestinal bleeding, including differentiation of lower vs upper and chronic vs acute Formulate a differential diagnosis of a child with painless gastrointestinal bleeding Assess the hemodynamic status of a patient with acute gastrointestinal bleeding Understand the basis of tests that evaluate for the presence of occult blood J. Abdominal mass Know how to identify the source of gastrointestinal bleeding Formulate a differential diagnosis of an abdominal mass in an adolescent Formulate a differential diagnosis of an abdominal mass in an infant Formulate a differential diagnosis of an abdominal mass in childhood Recognize the common causes of abdominal masses in adolescence, including infectious, neoplastic, gynecologic, and traumatic causes Distinguish discrete abdominal masses from other causes of abdominal swelling K. Jaundice 1. Mechanisms Recognize the physical findings associated with abdominal masses, including those caused by infection, neoplasms, trauma, or fecal impaction Know the mechanisms of bilirubin production in early life and differences that occur during the achievement of maturity 5

9 Understand the mechanisms of hepatic bilirubin uptake, metabolism, and excretion Recognize that hemolysis is a common mechanism for the production of jaundice Formulate a differential diagnosis in a patient with jaundice associated with cardiac disease Recognize pharmacologic causes of jaundice Plan the evaluation of an infant receiving exclusive parenteral nutrition in whom jaundice develops 2. Differential diagnosis Understand the pathophysiology of breast milk jaundice Differentiate physiologic jaundice from breast milk jaundice L. Liver failure 1. Mechanisms Formulate a differential diagnosis for jaundice in an infant, child, or adolescent Understand the laboratory studies and their use in assessing hepatic failure in infants with liver disease Know the mechanisms associated with drug-induced hepatic failure Know the mechanisms responsible for infectious causes of hepatic failure Know the mechanisms responsible for disorders of coagulation in hepatic failure Distinguish between hepatic and nonhepatic coagulopathy in a patient with liver failure Know the mechanisms associated with metabolic causes of hepatic failure Know the mechanisms of hepatic failure associated with anatomic injury/insult 2. Differential diagnosis Know the mechanisms of hepatic failure associated with cardiovascular disorders Understand the stages of hepatic coma Differentiate between hepatorenal syndrome and acute tubular necrosis Identify the infectious causes of fulminant hepatic failure in infancy 6

10 Identify the infectious causes of fulminant hepatic failure in childhood and adolescence Identify the metabolic causes of hepatic failure in infancy Identify the metabolic causes of hepatic failure in childhood and adolescence Identify the pharmacologic causes of liver failure Formulate a differential diagnosis of liver failure, including metabolic, anatomic, and cardiovascular causes M. Dysphagia 1. Mechanisms 2. Differential diagnosis Understand the mechanism responsible for dysphagia Know the common causes of dysphagia N. Hepatomegaly 1. Mechanisms Diagnose dysphagia based on signs and symptoms Understand the mechanisms responsible for hepatomegaly Evaluate liver span in infants and children 2. Differential diagnosis Understand the mechanisms responsible for splenomegaly associated with hepatomegaly Plan diagnostic evaluation for a patient with hepatomegaly O. Ascites 1. Mechanisms Formulate an age-specific diagnosis for hepatomegaly in an infant, child, or adolescent 2. Differential diagnosis Understand the pathogenesis of ascites formation Know the clinical manifestations of peritonitis 7

11 Recognize the clinical situations associated with primary bacterial peritonitis Recognize clinical clues to the etiology of ascites Understand the components in the laboratory analysis of ascites fluid and the peritoneal fluid findings diagnostic of peritonitis Plan management of ascites based on mechanisms responsible for the disorder Plan diagnostic evaluation and manage a patient with spontaneous bacterial peritonitis Recognize the hemodynamic effects of withdrawal of peritoneal fluid II. Normal Anatomy, Physiology, and Development A. Anatomy (gross and microscopic) and embryology Know the normal blood supply to the liver Understand the normal development of the caudal part of the foregut (esophagus, stomach, duodenum, liver, and gallbladder), midgut (intestines, colon), and hindgut (rectum) Understand the mechanism for development of Meckel diverticulum Understand that the blood supply to the common bile duct is primarily from the hepatic artery B. Transport and secretion of ions Understand the normal development of the enteric nervous system Understand the mechanisms of intestinal transport of ions (eg, coupled/uncoupled electrolytes, calcium, phosphate) Know the location of transport and secretion of ions C. Immunology and inflammation of the gastrointestinal tract Know the characteristics and functions of different intestinal epithelial cells (eg, intraepithelial lymphocytes, mucosal lymphocytes, lymphoid cells in Peyer patches) Understand the physiology of lymphocytes and consequences of antigen uptake in the gastrointestinal tract Distinguish between the characteristics of immune function in the human immature and mature gastrointestinal tract Know how breast feeding influences immune function in the gut Understand how cytokines mediate inflammation and how their modulation may 8

12 affect disease processes Understand the secretory IgA immune system D. The gut as an endocrine organ Recognize the role of growth factors in mediating inflammation and cytotoxic responses Understand the stimuli responsible for the secretion of gastrointestinal hormones Understand the mechanism of action responsible for the physiologic effects of various gastrointestinal hormones Know the functions of the gut-derived polypeptides Know the location of hormone-secreting cells in the gastrointestinal tract E. Gastrointestinal motility Understand the process of intracellular hormone processing in the gastrointestinal tract Understand the components of motility, including esophageal, gastric, small intestinal, colonic, anorectal Understand the neuronal and hormonal peptides that modulate gastrointestinal motility Understand the ontogeny of gastrointestinal motility Understand the nonpeptide chemical neurotransmitters that modulate gastrointestinal motility (including acetylcholine, norepinephrine, dopamine, serotonin, etc) Understand how nitric oxide modulates gastrointestinal motility and in which disease states its function may be altered Understand the differences between motility in the fed and fasted states F. Flora of the gut and protective function Understand the role of the extrinsic nervous system and the enteric nervous system in modulating gastrointestinal motility Understand the effects of enteric flora on intestinal morphology and function Know the mechanisms that prevent bacterial overgrowth in the small intestine (eg, motility, acid secretion, mucus, immunologic factors) Identify the organisms that are the predominant constituent of the colonic flora 9

13 G. Feeding, tasting, sucking H. Gastric function Know the normal developmental pattern of sucking in preterm and term infants Know the anatomic features of the stomach (gross, microscopic, neuromuscular) Understand that peptic acidity (ph < 4.0) is present as young as 24 weeks' gestation Understand the normal gastric secretory processes I. Digestive and absorptive function Know how to evaluate gastric secretory processes Understand the developmental sequence of digestive function Understand the role of the terminal ileum in regulating the enterohepatic circulation of bile acids Understand the digestion and absorption of fat Understand the digestion and absorption of carbohydrates Understand the digestion and absorption of protein Understand the absorption of vitamins and minerals Understand the phenomenon of changes in nutrient absorption (mucosal hypertrophy and changes in specific transport mechanisms) with luminal nutrient concentration Understand the phenomenon of changes in nutrient absorption with changes in body stores of selected nutrients J. Hepatobiliary system, hepatic structure and function Understand the concept of physiologic immaturity of hepatic function (eg, excretory function, bilirubin metabolism, bile acid metabolism) Know the pathways of hepatic drug metabolism Understand that taurine is a conditionally essential amino acid in early life Know the normal histology of liver tissue from a young infant (extramedullary hematopoiesis and double hepatocytes) Know the various biochemical parameters useful to assess hepatic integrity Understand the segmental anatomy of the liver 10

14 Understand hepatic carbohydrate metabolism Understand hepatic synthesis and metabolism of fatty acids and the role of the liver in lipid transport Know serum proteins synthesized by the liver Understand mechanisms of hepatic regeneration K. Exocrine pancreas: structure and function Understand the development of stimuli causing pancreatic secretion Be aware of age-related deficiency in exocrine pancreatic function Know the mechanisms by which pancreatic enzymes are activated Know the co-factors necessary for pancreatic enzyme function III. Clinical Manifestations and Management of Diseases of the Mouth A. Diseases of the oral cavity Know and recognize the oral lesions commonly associated with nutritional deficiency B. Disorders of deglutition Know and recognize the oral lesions commonly associated with gastrointestinal disease Know how to manage disorders of deglutition in preterm infants Understand and recognize the differential diagnosis of dysfunctional swallowing in the pediatric patient (eg, neuromuscular disorders, food refusal, systemic disorders, infection, psychologic disorders) Understand the clinical assessment of a child with impaired deglutition (eg, motility studies, ph studies, observation of feeding) Evaluate a patient with feeding difficulty Understand the methods, risks, and complications of nutritional support in a patient with dysphagia IV. Clinical Manifestations and Management of Diseases of the Esophagus A. Congenital anomalies Recognize the clinical manifestations of various esophageal anomalies 11

15 Understand embryology of the various esophageal anomalies Plan the management of a patient with tracheoesophageal fistula, with consideration of pathogenesis and long-term sequelae Plan the management of a patient with esophageal web B. Gastroesophageal reflux and esophagitis Recognize congenital thoracic vascular anomalies that cause esophageal dysfunction Be aware of the natural history of gastroesophageal reflux in infancy and childhood Recognize the clinical, endoscopic, and histologic features of esophagitis Know the pathogenesis of gastroesophageal reflux Know the spectrum of symptoms and presentations of gastroesophageal reflux disease at various ages Plan the management of a patient with gastroesophageal reflux, with an understanding of the principles guiding dietary, medical and surgical therapy Be familiar with the tests diagnostic of esophageal reflux and their limitations Recognize the postoperative complications and long-term prognosis associated with antireflux surgery Understand the definition, pathogenesis, diagnosis, and management of Barrett esophagus Understand the medical and surgical conditions predisposing to gastroesophageal reflux disease Recognize the clinical, endoscopic, and histologic features of eosinophilic esophagitis C. Motor disorders Plan the management of a patient with eosinophilic esophagitis, with an understanding of the principles guiding dietary and pharmacologic therapy Be aware of the pathophysiology of achalasia Know the association of other systemic or endocrine diseases with motor disorders of the esophagus (eg, scleroderma) Recognize the esophageal motility findings in specific esophageal disorders Understand the pathophysiology and management of cricopharyngeal achalasia 12

16 Understand the diagnosis and management of achalasia Be familiar with medical and surgical treatment of esophageal motility disorders D. Infections Be able to diagnose infections of the esophagus in otherwise healthy children Be able to diagnose infections of the esophagus in immunocompromised children (eg, herpes, Candida) Be able to treat infections of the esophagus E. Trauma Be aware of the medications which can cause esophageal mucosal injury Know how to identify and treat caustic injuries of the upper gastrointestinal tract Recognize the clinical manifestations of esophageal foreign body Know how to treat various esophageal foreign bodies Know the clinical manifestations of esophageal tear or perforation (eg, Mallory-Weiss tear, Boerhaave syndrome) Know how to manage an esophageal tear or perforation, including Mallory-Weiss tear Understand the mechanism(s) by which caustic injury to the esophagus occurs Recognize the clinical manifestations of esophageal diverticula, and understand the causes of same Plan the evaluation and treatment of esophageal lesions in a patient with epidermolysis bullosa F. Hiatal hernia Differentiate a hiatal hernia from a para-esophageal hernia on barium swallow examination and recognize how their management differs V. Clinical Manifestations/Management, Diseases of Stomach, Duodenum A. Congenital anomalies Recognize the clinical presentation of congenital anomalies of the stomach Know how to diagnose congenital anomalies of the stomach Know the indications for surgery for congenital anomalies of the stomach and 13

17 duodenum Recognize the clinical features of gastric volvulus, and manage appropriately Recognize the clinical features of gastric and duodenal duplication, and manage appropriately Recognize the clinical features of duodenal stenosis/atresia, and manage appropriately Recognize the clinical presentation of congenital anomalies of the duodenum Know how to diagnose congenital anomalies of the duodenum Plan the management for congenital anomalies of the stomach B. Pathogenesis of gastroduodenal inflammation Plan the management for congenital anomalies of the duodenum Be able to recognize generalized gastroduodenal inflammation endoscopically Understand the mechanisms that protect the gastroduodenal mucosa from chemical irritation Be able to diagnose gastritis endoscopically Be able to diagnose duodenitis endoscopically and histologically Be able to diagnose gastritis histologically C. Helicobacter pylori-related peptic disease Know how to diagnose Helicobacter pylori infection Plan the management of a patient with Helicobacter pylori infection Know the mechanisms by which Helicobacter pylori causes disease Be able to diagnose the spectrum of Helicobacter pylori gastroenteritis histologically Know the diseases associated with Helicobacter pylori (gastritis, ulcer, gastric carcinoma, MALT) Understand the risk factors for Helicobacter pylori infection Identify the symptoms associated with Helicobacter pylori disease D. Gastroduodenitis unrelated to Helicobacter pylori Formulate a differential diagnosis in a patient in whom gastroduodenitis is 14

18 suspected Plan the diagnostic evaluation of a patient in whom cytomegalovirus infection of the stomach is suspected Plan the diagnostic evaluation of a patient in whom hypertrophic gastropathy is suspected Plan the diagnostic evaluation of a patient in whom gastritis is suspected, and manage appropriately Recognize systemic disorders than can cause gastroduodenitis (eg, Crohn disease, chronic granulomatous disease) Understand that stress ulcers occur in patients with acute CNS injury Recognize the causes of erosive gastropathy E. Peptic ulcer Recognize the causes of nonerosive gastropathy Understand the pathophysiology of childhood acid-peptic disease (eg, stress, burns, familial incidence) Understand the pathologic conditions that affect the neuroendocrine regulation of the stomach Identify the x-ray characteristics of peptic ulcer disease in children Understand the management of acid-peptic disease in childhood Understand the factors that effect the prognosis for peptic ulcer disease in a child F. Trauma and foreign substances Recognize complications of peptic ulcer disease Know substances that cause corrosive gastritis Recognize the signs and symptoms of corrosive gastritis Know how to diagnose and manage corrosive gastritis in children Recognize the signs and symptoms of duodenal hematoma Know how to diagnose and manage duodenal hematoma in children Know the management of a foreign body in the stomach of children (eg, coin, battery, sharp object, bezoar) Know the causes of gastrointestinal perforation 15

19 Recognize the clinical manifestations of gastrointestinal perforation G. Neoplasia Formulate the differential diagnosis of neoplasms of the stomach in children H. Motor disorders including pyloric stenosis Know how to diagnose gastric neoplasms in children, including the use of endoscopy Know how to evaluate normal and abnormal gastric motor activity Recognize pyloric stenosis based on physical and radiologic findings Know how to manage the fluid and electrolyte requirements in pyloric stenosis Know the surgical management of pyloric stenosis Know the differential diagnosis of gastroduodenal dysmotility Know how to manage gastroduodenal dysmotility Evaluate a patient in whom gastroduodenal dysmotility is suspected VI. Clinical Manifestations and Management of Diseases of the Intestines A. Congenital anomalies Know the characteristics, presentation, diagnosis, and management of duplication cysts Recognize the early and late clinical manifestations of malrotation Recognize associated congenital anomalies of the gastrointestinal tract in children with Down syndrome Know how to differentiate gastroschisis from omphalocele Recognize the clinical manifestations of gastroschisis, and manage appropriately Recognize the clinical manifestations of omphalocele, and manage appropriately Recognize long-term complications of gastroschisis B. Infections 1. Bacterial infections Recognize long-term complications of omphalocele Know the signs and symptoms of intestinal tuberculosis 16

20 Recognize the clinical manifestations of common infections of the gastrointestinal tract (eg, Salmonella, Shigella, Yersinia, Campylobacter, Escherichia coli) Understand the pathophysiology of Escherichia coli infections and hemolytic-uremic syndrome Plan the therapy for common infections of the gastrointestinal tract (eg, Salmonella, Shigella, Yersinia, Campylobacter, Escherichia coli) Understand the mechanisms of action of bacterial pathogens 2. Viral infections Order appropriate diagnostic tests for common bacterial pathogens of the intestine and colon Know the signs and symptoms of viral gastroenteritis Know the differential diagnosis of viral gastroenteritis Recognize the clinical manifestations of infections with viral pathogens (eg, rotavirus, enteric adenovirus, calicivirus, Norwalk virus) Know how to diagnose viral gastroenteritis Understand the pathophysiology of viral gastroenteritis 3. Fungal infections Know how to manage viral gastroenteritis (eg, intravenous fluids, refeeding, oral rehydration) Recognize that fungal infection of the intestine is rarely a cause of acute diarrhea in immunocompetent hosts Recognize common fungal pathogens invading the small intestinal mucosa (eg, Candida, Aspergillus, mucormycosis, coccidioidomycosis) Recognize the risk factors for intestinal fungal infections 4. Parasitic infections Know the appropriate therapy for fungal infections of the intestine Know how to treat giardiasis Know the clinical symptoms of Giardia lamblia infestation Know the methods of diagnosis of Giardia lamblia infestation 17

21 Know the factors predisposing to Giardia lamblia infestation Understand the clinical presentation and complications of common intestinal parasites (eg, Ascaris, tapeworm, hookworm, whipworm) Recognize that the stool may contain harmless commensal organisms, and manage appropriately 5. GI manifestations of sexually transmitted diseases C. Antibiotic-associated diarrhea Recognize the gastrointestinal manifestations of sexually transmitted diseases and manage appropriately Know the risk factors for and pathogenesis of pseudomembranous enterocolitis Know the clinical manifestations of pseudomembranous enterocolitis Know the therapy for pseudomembranous enterocolitis Know how to diagnose pseudomembranous enterocolitis (eg, cultures, studies for toxins, endoscopy) Plan management for a patient with recurrent C. difficile enteritis (including how to prevent spread of the disease) D. Inflammatory bowel disease Compare the clinical and laboratory characteristics of antibiotic- associated diarrhea with those of pseudomembranous enterocolitis 1. Pathogenesis of intestinal inflammation Know the mechanisms for inflammatory mediators evoked by immune reactions within the gut (eg, cytokines) Know the familial contribution to inflammatory bowel disease 2. Crohn disease Recognize that infection may be a contributing cause of inflammatory bowel disease Be able to distinguish Crohn colitis from chronic ulcerative colitis Know the signs and symptoms of Crohn disease, including extra-intestinal manifestations Interpret radiologic findings in the diagnosis of Crohn disease Recognize the complications of medical and surgical treatment of Crohn disease 18

22 Know the long-term outcome of patients with Crohn disease Understand the epidemiology of Crohn disease Know how to interpret endoscopic findings in Crohn disease Know how to interpret histologic findings in Crohn disease Know how to manage Crohn disease (eg, nutritionally, medically, surgically) Understand the mechanism of action of the drugs used to treat Crohn disease (eg, corticosteroids, 5-ASA, 6-MP, metronidazole, biologic agents) Recognize that perianal disease may be a symptom of Crohn disease 3. Ulcerative colitis Understand the pathogenesis of Crohn disease Interpret the radiologic findings in a patient with ulcerative colitis Recognize the extra-intestinal manifestations of ulcerative colitis Know the signs and symptoms of ulcerative colitis Know how to interpret biopsies of the colon for the diagnosis of chronic ulcerative colitis Know how to interpret endoscopy of the colon for the diagnosis of chronic ulcerative colitis Understand the pathophysiology of chronic ulcerative colitis Understand the mechanism of action of the drugs used to treat ulcerative colitis Recognize the role of medical and surgical treatment of ulcerative colitis Know the long-term outcome of patients with ulcerative colitis Understand the epidemiology of ulcerative colitis Plan an appropriate therapeutic regimen for a patient with toxic megacolon Recognize signs and symptoms of toxic megacolon E. Other inflammatory disorders Recognize the association of hepatobiliary disease with ulcerative colitis Know the clinical manifestations of inflammatory lesions of the bowel (eg, protein-losing enteropathy, Behcet, HSP, hemolytic- uremic syndrome, drugs) 19

23 F. Necrotizing enterocolitis Formulate a differential diagnosis of inflammatory lesions of the bowel (eg, Behcet, HSP, hemolytic-uremic syndrome, drugs) Recognize the conditions that predispose to necrotizing enterocolitis (eg, prematurity, bowel ischemia) Identify the radiographic features of necrotizing enterocolitis Know the short-term and long-term complications of necrotizing enterocolitis G. Malabsorption (see also I.G) Plan the management of a patient in whom necrotizing enterocolitis is suspected 1. Congenital enzyme and transport deficiencies Plan the therapy of a patient with suspected bile acid malabsorption Plan the evaluation of a patient with congenital disorder of carbohydrate absorption Know the clinical manifestations of congenital enzyme and transport defects Understand the therapy for congenital transport defects Recognize clinical and laboratory manifestations of abetalipoproteinemia 2. Secondary enzyme deficiencies and maldigestion Be familiar with the types of secondary enzyme deficiencies Interpret the results of laboratory tests for secondary enzyme deficiencies Know the physiologic consequences of secondary enzyme deficiencies 3. Bacterial overgrowth Understand the mechanisms of drug-induced malabsorption Be familiar with several methods to make the diagnosis of bacterial overgrowth in the small intestine Know the mechanisms by which bacterial overgrowth produces malabsorption 4. Celiac disease Be familiar with ways to treat bacterial overgrowth Know the morphologic and histologic features of untreated celiac disease 20

24 Know the pathogenesis of celiac disease, including immunologic features and genetics Recognize the clinical manifestations of celiac disease Know the laboratory abnormalities commonly found in celiac disease (including diagnostic tests) Understand the treatment of celiac disease Know the assessment of therapeutic success in a patient with celiac disease Know the long-term complications of celiac disease 5. Tropical sprue Recognize the risk factors and conditions associated with celiac disease Recognize the hematologic and pathogenetic characteristics of tropical sprue 6. Idiopathic prolonged diarrhea Differentiate tropical sprue from celiac disease a. Protracted diarrhea of infancy and villous atrophy Know the signs and symptoms of idiopathic protracted diarrhea of infancy (with or without villous atrophy) Know the differential diagnosis and plan the management of protracted diarrhea of infancy Interpret the histologic features of a small bowel biopsy of an infant with idiopathic villous atrophy Understand the pathophysiology of the malabsorption present in infants with idiopathic villous atrophy Recognize the clinical manifestations and histopathology of microvillus inclusion disease Recognize the clinical manifestations and histopathology of autoimmune enteropathy Identify laboratory findings of intractable diarrhea of infancy b. Chronic nonspecific diarrhea of childhood (toddler's diarrhea) Know the signs and symptoms and plan the management of chronic nonspecific diarrhea of childhood (toddler's diarrhea) 21

25 Know the differential diagnosis of prolonged diarrhea in childhood without failure to thrive 7. Short gut syndrome Understand the management of chronic nonspecific diarrhea of childhood (toddler diarrhea) Recognize the complications of short gut syndrome in infancy Understand the various methods to assess bowel function in infants with short gut syndrome Plan the short-term and long-term therapy of an infant with short gut syndrome Recognize the mechanism of immediate and late gut adaptation in short gut syndrome Know the relative importance of resection length vs loss of ileocecal valve in a patient with short gut syndrome Understand the role of intestinal transplantation in a patient with short gut syndrome Understand the importance of linear growth in adaptation to short bowel Understand the special nutritional needs of a patient with short gut syndrome Know the regulators of immediate and long-term adaptation to gut resection H. Motility disorders Formulate a differential diagnosis of altered intestinal motility Recognize motility abnormalities associated with intestinal pseudo-obstruction Be familiar with the histopathology of various forms of intestinal pseudo-obstruction Recognize the clinical features of chronic intestinal pseudo-obstruction syndrome Understand the role of surgery in the management of a patient with intestinal pseudo-obstruction Plan the treatment of a patient with intestinal pseudo-obstruction I. Neoplasms (excluding tumors of liver and pancreas) Be familiar with the types and locations of malignancy of the intestine, and know their relative importance throughout infancy and childhood Differentiate between risk factors (including ulcerative colitis, polyps, genetic 22

26 J. Gastrointestinal polyps markers) and protective factors in carcinoma of the colon Recognize the clinical presentation of various types of gastrointestinal polyps that occur in children Know the accepted methods of treating gastrointestinal polyps Understand inheritance and/or genetic defects in the intestinal polyposis syndromes Understand the malignant potential and function of oncogenes relative to gastrointestinal polyps K. Appendicitis Identify the histologic characteristics of childhood gastrointestinal polyps Recognize the typical historical and physical findings in acute appendicitis Recognize the association of appendicolith with appendicitis Select appropriate diagnostic tests for appendicitis L. Protein-losing enteropathies Recognize the complications of acute appendicitis (eg, perforation, abscess) Plan the management of a patient with protein-losing enteropathy Plan therapy for an infant with congenital intestinal lymphangiectasia Recognize the clinical symptoms and biopsy findings of protein- losing enteropathy Recognize the laboratory findings associated with protein-losing enteropathy Formulate a differential diagnosis of protein-losing enteropathy based on age of the patient M. Acute obstruction Know the clinical situations in which protein-losing enteropathy occurs Know the causes of acute intestinal obstruction in children of various ages Recognize acute intestinal obstruction by physical examination and on plain abdominal x-ray studies Devise a management plan for a child with acute intestinal obstruction (eg, intussusception, volvulus) 23

27 Recognize the association between prenatal and neonatal intestinal obstruction, meconium peritonitis, and cystic fibrosis Identify congenital intestinal abnormalities associated with maternal polyhydramnios Plan the treatment of a patient with cystic fibrosis and distal intestinal obstruction syndrome N. Perianal lesions Recognize the perianal signs of sexual abuse Recognize the symptoms of anal fissure in a healthy infant Know how to treat anal fissures Recognize anterior ectopic anus and its symptoms Differentiate between a high and low imperforate anus and the treatment of each Know that imperforate anus is part of the VACTERL anomaly Plan the management of a patient with acute perianal streptococcal infection Be able to recognize and treat perianal candidiasis Know the significance of hemorrhoids in a child Identify the causes of and be able to diagnose rectal prolapse and manage appropriately Recognize perianal rashes as a clue to systemic disease O. Hirschsprung disease Plan the management of a patient with rectal abscess Understand the pathophysiologic mechanisms of Hirschsprung disease Be familiar with the genetics of Hirschsprung disease Recognize the clinical manifestations of Hirschsprung disease Be able to identify the radiologic manifestations of Hirschsprung disease Interpret the manometric findings of Hirschsprung disease Know the histopathology of Hirschsprung disease Be able to treat a patient with Hirschsprung disease utilizing medical and 24

28 surgical options Be familiar with the complications of Hirschsprung disease Understand the prognosis for patients with Hirschsprung disease Understand the mechanism of peristaltic failure in Hirschsprung disease P. Intussusception Plan the management of a patient with intussusception Recognize the causes and clinical manifestations of intussusception Identify the radiologic manifestations of intussusception Know the complications of intussusception Understand the prognosis for a patient with intussusception Identify the various gastrointestinal lesions that can lead to intussusception VII. Miscellaneous Diseases Affecting the Gastrointestinal Tract A. Gastrointestinal allergy Know the gastrointestinal manifestations of food allergy (eg, eosinophilic gastroenteritis, malabsorption, vomiting) Be aware of the familial component of gastrointestinal allergy Differentiate neonatal allergic colitis from other causes of gastrointestinal bleeding in infancy Know the dietary treatment of food allergy Be aware of the role of breast feeding in the management of food allergy Know which pharmacologic agents to use in gastrointestinal allergy Recognize the influence of age on acquisition and resolution of food allergy B. Endocrine disorders Recognize the contribution of intestinal mast cells to gastrointestinal diseases 1. The gut in systemic endocrinopathies Recognize the gastrointestinal manifestations of systemic endocrine disorders (eg, hypothyroidism, hyperthyroidism, Cushing syndrome, adrenal insufficiency, hypoparathyroidism, hyperparathyroidism) 25

29 Know the diagnostic criteria for endocrine disorders which have gastrointestinal manifestations (eg, hypo- or hyperthyroidism, Cushing syndrome, adrenal insufficiency, hypo- or hyperparathyroidism) 2. Secretory tumors affecting the gut Plan appropriate diagnostic evaluation for a patient with gastrointestinal complications of diabetes mellitus (eg, bacterial overgrowth, gastric paresis, malabsorption, celiac disease) Formulate a differential diagnosis for a patient in whom multiple endocrine neoplasia syndrome is suspected Know the inheritance pattern and risk factors of multiple endocrine neoplasia syndrome Recognize the features of Zollinger-Ellison syndrome, and manage appropriately Know the diagnostic criteria for Zollinger-Ellison syndrome Formulate a differential diagnosis for tumors causing secretory diarrhea and manage appropriately Recognize the major features of multiple endocrine neoplasia syndrome C. Drug-induced bowel injury Know the types of secretory tumors affecting the gut (eg, VIPoma, gastrinoma) and their secretory proteins D. Radiation enteritis Know the effects of various drugs upon the bowel (eg, antiemetics, antibiotics, corticosteroids, NSAIDs, antispasmodics, antidiarrheals, and medications to treat constipation) Understand the pathophysiology of radiation injury to the intestine E. Trauma and foreign bodies Know the appropriate management and long-term prognosis for radiation injury to the intestine Know the abdominal lesions associated with blunt abdominal trauma Know the most common locations in the gastrointestinal tract where foreign bodies may impact and obstruct Know the most common sites in the esophagus where foreign bodies tend to become lodged and why Plan the management for ingestions of specific types of foreign bodies (eg, 26

30 coins, batteries, sharp elongated objects, meat) Recognize the clinical manifestations of intestinal foreign bodies, including complications F. The intestine in immune deficiency Recognize the clinical manifestations of bezoar, and manage appropriately Know which immune deficiency disorders leave a patient susceptible to opportunistic intestinal pathogens Recognize the gastrointestinal manifestations of selective IgA deficiency Formulate a differential diagnosis of immunodeficiencies that may cause gastrointestinal symptoms (eg, T-cell deficiency, graft- versus-host disease, chronic granulomatous disease, AIDS, drug-induced) Recognize the gastrointestinal manifestations of AIDS Understand the difference between viral infections in immunocompetent hosts and immunocompromised hosts Recognize the clinical manifestations of graft-versus-host disease G. Munchausen syndrome (factitious disorder) by proxy Recognize the variety of clinical presentations of Munchausen syndrome (factitious disorder) by proxy (eg, apnea, gastrointestinal bleeding, vomiting, diarrhea, failure to thrive) Plan the evaluation of a patient with diarrhea in whom laxative administration by the parent is suspected Understand the criteria for the diagnosis of Munchausen syndrome (factitious disorder) by proxy Recognize the characteristics of caretakers of patients with Munchausen syndrome (factitious disorder) by proxy Plan the evaluation of gastrointestinal bleeding in a patient with suspected Munchausen syndrome (factitious disorder) by proxy Differentiate idiopathic protracted diarrhea of infancy from Munchausen syndrome (factitious disorder) by proxy H. GI manifestations of specific systemic disorders Plan the evaluation of emesis in a patient with suspected Munchausen syndrome (factitious disorder) by proxy Recognize the gastrointestinal manifestations of specific chronic systemic 27

31 I. Irritable bowel syndrome disorders (eg, connective tissue, cardiac, renal, hematologic pulmonary) Recognize genetic and environmental risk factors for irritable bowel syndrome Be familiar with the symptoms-based criteria for irritable bowel syndrome Plan appropriate evaluation of a child with symptoms suggestive of irritable bowel syndrome J. Food poisoning Plan the treatment of a child with irritable bowel syndrome Recognize the symptoms of food poisoning VIII. Clinical Manifestations and Management of Diseases of the Liver A. Cholestatic liver disease 1. Neonatal cholestasis (see also VIII.M and VIII.0) Plan the evaluation of a 1-month-old infant with conjugated hyperbilirubinemia Understand that sepsis may be a cause of neonatal cholestasis Plan the evaluation of a child suspected of having a choledochal cyst as a cause of prolonged conjugated hyperbilirubinemia Plan the evaluation of a patient suspected of having galactosemia Know the indications for surgery in a patient with neonatal cholestasis Recognize the clinical and histologic features of extrahepatic biliary atresia Recognize the clinical and histologic features of neonatal hepatitis Distinguish an infant with cholestasis secondary to panhypopituitarism from other patients with various forms of neonatal cholestasis Know the factors contributing to the pathogenesis of cholestasis associated with total parenteral nutrition Recognize the clinical features of cholestasis of various origins (eg, TPN-induced, short bowel syndrome/bacterial overgrowth) 2. Cholestasis beyond the neonatal period (see also IX.A and IX.B) Plan the evaluation of an 11-month-old infant with conjugated hyperbilirubinemia 28

32 Plan the evaluation of an 11-year-old child with conjugated hyperbilirubinemia Know the nutritional consequences of chronic cholestasis (eg, vitamin deficiency, fat malabsorption, impaired carbohydrate metabolism) Recognize that cholangitis occurring in a patient who has undergone hepatoportoenterostomy may contribute to progressive scarring and hepatic decompensation Plan the therapy of a patient with pruritus associated with intrahepatic cholestasis Understand the mechanisms of malabsorption in cholestatic liver disease Plan the management of prolonged/chronic cholestasis B. Congenital infections of the liver (excluding hepatitis B and C) Understand the serologic diagnosis of congenital infections of the liver C. Viral hepatitis Recognize the histologic and clinical findings associated with congenital viral infections of the liver Know the risk factors for and modes of transmission of hepatitis A, B, C, D, E Plan the management of a child with hepatitis A virus infection in order to prevent the spread of infection Plan the preventive management of an HBsAg-positive pregnant woman and the management of her infant Understand the serologic diagnosis of hepatitis B virus infection Understand the methods for diagnosis of hepatitis C Plan the management for a patient with hepatitis C Recognize the clinical manifestations of hepatitis C Know the mechanism, clinical manifestations, diagnostic evaluation, and clinical course of hepatitis delta infection Recognize the clinical manifestations of and be able to diagnose hepatitis E Know the long-term considerations related to the hepatitis carrier state Know the limitations of liver transplantation for treatment of chronic hepatitis B infection Know the management of a needle-stick injury in the setting of viral hepatitis 29

33 D. Molecular biology of hepatitis viruses Know the role of molecular biology in the understanding of the pathophysiology of hepatitis viruses Understand the pathophysiology of hepatitis B infection Understand the structure of hepatitis C virus as it relates to immune response Know that hepatitis B and C are precursors of hepatocellular carcinoma Understand the value of various serologic markers of hepatitis C (eg, PCR, ELISA, RIBA) E. Bacterial, parasitic, and other infections of the liver Recognize the clinical manifestations of liver abscess Understand the mechanisms and pathogenesis for the pyogenic liver abscesses Recognize the clinical manifestations of Fitz-Hugh-Curtis syndrome Recognize systemic diseases that predispose a patient to pyogenic liver abscesses Plan the treatment of pyogenic liver abscess Recognize parasitic causes of hepatic disease Plan the treatment of parasitic liver infestation Recognize and differentiate between the characteristic radiologic findings associated with bacterial, parasitic, and other infections of the liver F. Drug-induced liver injury Recognize the characteristic histologic findings associated with bacterial, parasitic, and other infections of the liver Know the laboratory findings for drug-related hepatotoxicity (eg, acetaminophen, antituberculous drugs, anticonvulsants, methotrexate) Know how to treat drug-related hepatotoxicity (eg, acetaminophen, antituberculous drugs, anticonvulsants, methotrexate) Differentiate among the characteristic clinical features associated with drug-related liver injury Know that chronic acetaminophen administration can be associated with severe hepatotoxicity in children 30

34 Understand the mechanisms of liver injury by hepatotoxic drugs (eg, acetaminophen) Recognize the histologic manifestations of various toxic liver injuries G. Liver tumors Judge the value of alpha-fetoprotein determinations in screening for hepatoblastoma and hepatocellular carcinoma Describe the disorders associated with hepatoblastoma Recognize the characteristic findings on computed tomography in a patient with vascular lesions of the liver Recognize the clinical features of hepatic malignancy in childhood (eg, hepatoblastoma, hepatocellular carcinoma) Recognize therapeutic options for treatment of hepatic malignancies in childhood (eg, hepatoblastoma, hepatocellular carcinoma) H. Noncholestatic hyperbilirubinemia Interpret the laboratory findings associated with hepatic malignancies in childhood (eg, hepatoblastoma, hepatocellular carcinoma) Evaluate the pattern of unconjugated hyperbilirubinemia in a patient with Gilbert syndrome Describe the characteristic hepatic histology of a patient with the Dubin-Johnson syndrome Understand the clinical manifestations and pathogenesis of the Dubin-Johnson syndrome Plan treatment and know the prognosis for a child with Crigler- Najjar type I and II syndrome Understand the implication of fasting-induced unconjugated hyperbilirubinemia in patients with Gilbert syndrome Differentiate the pathogenesis and clinical manifestations of unconjugated hyperbilirubinemia due to Crigler-Najjar (type I and type II) and Dubin-Johnson, Gilbert, and Rotor syndromes Plan the evaluation of an adolescent patient with unconjugated hyperbilirubinemia Understand the molecular basis of the inherited hyperbilirubinemias (eg, Crigler-Najjar syndrome types I & II, Gilbert syndrome, Dubin-Johnson syndrome) 31