Pediatric Gastroenterology and Hepatology 2024
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A 3-month-old boy has failure to thrive and chronic diarrhea. Stools are pale, foul-smelling, and greasy, and breath sounds are coarse with rhonchi. The MOST likely diagnosis is:

  • Cystic Fibrosis (correct)
  • Schwachman-Diamond Syndrome
  • Biliary Atresia
  • Congenital Lactase Deficiency
  • Celiac Disease (correct)
  • Which of the following is NOT associated with an increased risk of Celiac Disease?

  • William’s Syndrome (correct)
  • Turner Syndrome
  • Type 1 Diabetes
  • Russell-Silver Syndrome
  • Down Syndrome
  • What are the recommended fluids for acute management of hepatitis and pancreatitis?

    1.5-2x maintenance fluids

    Which side effects are associated with Interferon-a (IFN-a) treatment for Hepatitis B?

    <p>Neutropenia</p> Signup and view all the answers

    Children with Hepatitis C have a higher rate of spontaneous clearance compared to adults.

    <p>True</p> Signup and view all the answers

    For diagnosis of Hepatitis C, confirmatory testing is required with Hepatitis C PCR and _______.

    <p>genotype</p> Signup and view all the answers

    Match the types of Cholelithiasis with their descriptions:

    <p>Cholesterol = Bile infected with enteric bacteria Brown Pigment = Bile infected with hematologic origin Black Pigment = Think coffee ground emesis</p> Signup and view all the answers

    Which of the following is NOT a unique sign of Crohn’s Disease?

    <p>Hypoalbuminemia</p> Signup and view all the answers

    Which of the following is NOT in the differential of Terminal Ileitis?

    <p>Celiac Disease</p> Signup and view all the answers

    Which of the following is NOT an organic cause of constipation?

    <p>Hyperkalemia</p> Signup and view all the answers

    Infant rumination best responds to:

    <p>Frequent holding and social interaction</p> Signup and view all the answers

    What is the main reason for managing constipation in children?

    <p>Education about constipation</p> Signup and view all the answers

    Which coagulation factor is not produced in the liver?

    <p>Factor VIII</p> Signup and view all the answers

    Probiotics are live microorganisms that have health benefits for the host.

    <p>True</p> Signup and view all the answers

    Donor milk is primarily prioritized for __________ newborns.

    <p>hospitalised</p> Signup and view all the answers

    Match the following benefits of breastfeeding with their effects:

    <p>Decreases infections in infancy = Enhanced neurocognitive testing Reduced SIDS = Decreased maternal breast and ovarian cancer Economical = Decreasing colic symptoms</p> Signup and view all the answers

    Which of the following are symptoms of irritable bowel syndrome (IBS)?

    <p>Change in frequency of stool</p> Signup and view all the answers

    What are common presentations of functional abdominal pain?

    <p>Continuous abdominal pain</p> Signup and view all the answers

    What is the most likely diagnosis for a 4-week-old baby with persistent unconjugated hyperbilirubinemia?

    <p>Gilbert’s Syndrome</p> Signup and view all the answers

    What is the main cause of prehepatic hyperbilirubinemia?

    <p>Hemolysis</p> Signup and view all the answers

    Match the following causes of hyperbilirubinemia with their types:

    <p>Hemolytic Extracellular = Sepsis/DIC, ABO/Rh Non-hemolytic Physiologic = Hypothyroid, Breast milk Non-hemolytic Gilbert’s Syndrome = Physiologic jaundice Non-hemolytic Hemoglobinopathy = Membranopathy</p> Signup and view all the answers

    What is the genetic defect associated with Gilbert’s Syndrome?

    <p>Autosomal dominant</p> Signup and view all the answers

    Individuals with chronic hepatitis B infection must have evidence of viral replication.

    <p>True</p> Signup and view all the answers

    What is the recommended surveillance frequency for measuring ALT levels in children over 2 years old?

    <p>every 6 months</p> Signup and view all the answers

    What is the lifetime risk of developing Hepatocellular Carcinoma (HCC) or cirrhosis in infectious hepatitis?

    <p>15-25%</p> Signup and view all the answers

    Which of the following is NOT a unique sign of Crohn’s Disease?

    <p>Hypoalbuminemia</p> Signup and view all the answers

    Which of the following is NOT in the differential diagnosis of Terminal Ileitis?

    <p>Celiac Disease</p> Signup and view all the answers

    Which of the following is NOT an organic cause of constipation?

    <p>Hyperkalemia</p> Signup and view all the answers

    Infant rumination best responds to:

    <p>Frequent holding and social interaction</p> Signup and view all the answers

    What are the recommended fluid management guidelines in the acute management of infectious hepatitis?

    <p>1.5-2x maintenance</p> Signup and view all the answers

    Which statement about pancreatitis is true?

    <p>Combination EN/PN is preferred over exclusive PN.</p> Signup and view all the answers

    Cholelithiasis can be caused by black pigment formed of hematologic origin.

    <p>True</p> Signup and view all the answers

    In hepatitis C, cirrhosis takes ____ years to develop, if ever.

    <p>10-20</p> Signup and view all the answers

    Match the complications of acute liver failure to their effects:

    <p>Cerebral edema = Brain swelling Coagulopathy and hemorrhage = Risk of bleeding Bone marrow failure = Impaired blood cell production Hypoglycemia = Low blood sugar levels</p> Signup and view all the answers

    The most likely diagnosis for a 3-month-old boy with failure to thrive, chronic diarrhea, pale, foul-smelling, greasy stools, and a normal texture liver is?

    <p>Celiac Disease</p> Signup and view all the answers

    Which of the following is NOT associated with an increased risk of Celiac Disease?

    <p>William’s Syndrome</p> Signup and view all the answers

    What is the treatment for typical Celiac Disease?

    <p>lifelong gluten-free diet</p> Signup and view all the answers

    Which type of carbohydrates are highly fermentable and may cause digestive issues? FODMAP stands for Fermentable Oligosaccharides, Disaccharides, Monosaccharides, and ______.

    <p>Polyols</p> Signup and view all the answers

    What are the criteria for a diagnosis of Functional Abdominal Pain according to the New Rome IV Criteria?

    <p>Episodic or continuous abdominal pain occurring &gt;4 times per month</p> Signup and view all the answers

    What is the most likely diagnosis for a 4-week-old baby with persistent unconjugated hyperbilirubinemia?

    <p>Gilbert’s Syndrome</p> Signup and view all the answers

    What is the genetic defect associated with Gilbert’s Syndrome?

    <p>Autosomal dominant</p> Signup and view all the answers

    What is the most likely diagnosis for a 12-year-old boy with chronic transaminase elevation and diffusely echogenic liver?

    <p>Non-alcoholic Fatty Liver Disease</p> Signup and view all the answers

    What should you consider if ALP (Alkaline Phosphatase) is abnormally low?

    <p>Zinc deficiency</p> Signup and view all the answers

    If ALP is low or normal and other enzymes are high, what condition should you consider?

    <p>Wilson's Disease</p> Signup and view all the answers

    Which of the following are Vitamin K dependent factors? (Select all that apply)

    <p>Protein C</p> Signup and view all the answers

    Factor VIII is produced in the liver. True or False?

    <p>False</p> Signup and view all the answers

    What distinguishes liver disease from Disseminated Intravascular Coagulation (DIC)?

    <p>Factor VIII levels</p> Signup and view all the answers

    What is the Rome III Criteria commonly used for?

    <p>Diagnosis of constipation</p> Signup and view all the answers

    What is the primary recommendation for long-term treatment of constipation in children?

    <p>PEG3350</p> Signup and view all the answers

    According to the position statement on 'Energy and sports drinks in children and adolescents,' what is a higher risk group for energy drinks?

    <p>CVD, Renal, Liver Disease, Seizure d/o, Diabetes, Mood d/o, Hyperthyroidism</p> Signup and view all the answers

    Which of the following are proven benefits of using probiotics? (Select all that apply)

    <p>Preventing infectious diarrhea</p> Signup and view all the answers

    Human milk banking is endorsed by the Canadian Pediatric Society. True or False?

    <p>False</p> Signup and view all the answers

    Study Notes

    Pediatric Gastroenterology and Hepatology 2024

    Objectives

    • Comprehensive review of RCPSC Training Objectives for Pediatric Gastroenterology
    • Review of current evidence-based guidelines for managing RCPSC recognized "problems" common to the general pediatrician
    • Preparation for MCQ/OSCE clinical scenarios

    Malabsorption

    • Failure to thrive and chronic diarrhea are common symptoms
    • Causes: fat, protein, and carbohydrate malabsorption
    • Biliary: bile emulsifies fat, pancreas: proteases, and duodenum: brush border hydrolysis
    • Examples: CF, SDS, Crohn's disease, cholestasis
    • Carbohydrate malabsorption: primary causes (enzyme deficiencies) and dietary causes (saturation of normal enzyme levels)

    Celiac Disease

    • Autoimmune enteropathy caused by systemically acting antibodies that are formed against gluten
    • Screening: bloodwork +ve, but not sufficient alone; intestinal biopsy (villous atrophy) is required for diagnosis
    • Typical symptoms: diarrhea, abdominal pain, and weight loss
    • Associated conditions: type 1 diabetes, IgA deficiency, Down syndrome, Turner syndrome, and Williams syndrome
    • Treatment: lifelong gluten-free diet

    Inflammatory Bowel Disease

    • Ulcerative colitis vs Crohn's disease: macroscopic and microscopic differences
    • Presenting signs: diarrhea, abdominal pain, weight loss, and fatigue
    • Major medication classes: INDUCE remission (tube feeds, corticosteroids, 5-ASA) and MAINTAIN remission (tube feeds, azathioprine, methotrexate, biologics)

    Constipation

    • 90% is functional, 10% is organic (hypercalcemia, hypothyroidism, lead poisoning, and Hirschsprung's disease)
    • Chronic constipation: patient eventually develops megarectum and encopresis
    • Laxatives by category: osmotic, stimulant, lubricant, and prokinetic
    • Treatment: CLEANOUT à MAINTENANCE x4-6mo à SLOW WITHDRAWAL

    Functional Gastrointestinal Disorders

    • Childhood/adolescence FGID: nausea and vomiting disorders, abdominal migraine, functional abdominal pain, and defecation disorders
    • Rumination syndrome, cyclic vomiting syndrome, and functional dyspepsia are examples of FGID### Functional Gastrointestinal Disorders in Infants and Toddlers
    • Rumination Syndrome:
      • Repeated painless regurgitation and rechewing or expulsion of food that begins soon after ingestion of a meal
      • Does not occur during sleep
      • No retching
      • No organic explanation
      • Eating disorder must be ruled out
      • Responds to relaxation therapies, avoidance of behavioral reinforcement
    • Cyclic Vomiting Syndrome:
      • At least 2 periods of intense nausea and hyperemesis or retching lasting hours to days (in the past 6 months)
      • Episodes are stereotypical
      • Return to usual state of health lasting weeks to months
      • Symptoms not attributable to other conditions
      • Family history of migraine

    Abdominal Pain Disorders

    • Irritable Bowel Syndrome:
      • Abdominal pain >4 times/month with at least 1 of the following symptoms:
        • Timing related to defecation
        • Change in frequency of stool
        • Change in form of stool
      • If patient experiences constipation (IBS-C), pain does not resolve with resolution of constipation
    • Functional Abdominal Pain:
      • Very common
      • Occurs at least 4 times per month
      • Includes:
        • Episodic or continuous abdominal pain
        • Insufficient criteria for other FGID’s

    Hepatology

    • Approach to Hepatomegaly:
      • Inflammation (Hepatitis)
      • Storage Disorders (Glycogen, Lipid, Protein, Iron)
      • Infiltrative (Benign Liver Tumors, Malignant Liver Tumors, Disseminated Tumors)
      • Biliary Obstruction
      • Posthepatic Obstruction (Cardiac, Thrombus, Intrahepatic)
    • Hyperbilirubinemia:
      • Prehepatic Causes:
        • Hemolytic (Extracellular: Sepsis/DIC, ABO/Rh, Sepsis/DIC, Intracellular: Membranopathy, Hemoglobinopathy)
        • Non-hemolytic (Physiologic, Gilbert’s Syndrome)
      • Hepatic Causes:
        • Inherited disorders (A1AT deficiency, Wilson’s Disease)
        • Acquired disorders (Viral Hepatitis, Autoimmune Hepatitis)
      • Posthepatic Causes:
        • Obstruction (Biliary Atresia, Choledochal Cyst)

    Infectious Hepatitis

    • Chronic Hepatitis B:
      • Defined as: HBsAg+ for >6 months, ALT >2X normal, and evidence of viral replication (HBeAg+ or HBV DNA >4 log if HBeAg-)
      • Treat with either IFN-a or lamivudine
      • 20-58% spontaneous conversion/clearance
    • Surveillance Recommendations:
      • Measure ALT q6months in children >2yo
      • Measure HBeAg and HBeAb yearly in patients with normal ALT
      • Liver biopsy in children >2yo with elevated ALT
      • Examine for chronic liver disease
      • Immunize household members and patient against Hepatitis A
      • Alpha fetoprotein and ultrasound annually

    Liver Transplantation

    • Long-term Follow-up:
      • Neurodevelopmental Care
      • Medication Side Effects (Hypertension, Hyperglycemia, Seizure)
      • Secondary Graft Dysfunction
      • Infectious Disease (Common infections, Opportunistic infections)
      • Relapse of Primary Disease
      • Chronic Cellular (Graft) Rejection

    Miscellaneous Hepatology Topics

    • Non-Alcoholic Fatty Liver Disease (NAFLD):

      • Umbrella term
      • NAFLD: simple steatosis vs. NASH
      • Consider when BMI > 85%
      • Diagnosis of exclusion
      • Only effective treatments: weight loss, exercise
    • Chronic Hepatitis in Pediatrics:

      • Differential Diagnosis:
        • Autoimmune Hepatitis
        • A1AT Deficiency
        • Wilson’s Disease
        • Celiac Disease
        • Infectious Hepatitis
    • Pancreatitis:

      • Acute management:
        • Fluids (1.5-2x maintenance)
        • Enteral nutrition early (PO/GT à NJ à TPN)
        • Watch for complications (SIRS response)
      • Nutritional Considerations:
        • Early unrestricted diet should be started within 24-48 hours
        • NG > NJ as tolerated or combination EN/PN preferred over exclusive PN### Functional Gastrointestinal Disorders in Infants and Toddlers
    • Defecatory Disorders:

      • Functional Constipation: • Occurs ≥1/week for ≥2 months • Not IBS • ≥2 symptoms in a child developmentally ≥4yo: • ≤2 bowel movements/week • ≥1 episode of fecal incontinence/week • Retentive posturing • Painful or hard bowel movements • Large fecal mass in rectum • Large diameter stools that may block toilet
      • Nonretentive Fecal Incontinence: • At least 1 month of episodes • ≥1/month defecation in places inappropriate to social context • No fecal retention • Not attributable to another condition

    Complications of Acute Liver Failure

    • Encephalopathy
    • CVS effects
    • Coagulopathy and hemorrhage
    • Bone marrow failure
    • Hypoglycemia
    • Electrolyte/Acid-base imbalance
    • Renal dysfunction
    • Pancreatitis
    • Ascites

    Hepatitis B Treatments

    • Interferon-a (IFN-a): • Cytokine • Side effects: neutropenia (39%), fever, myalgia, headaches, arthralgia, anorexia with weight loss, severe mood changes
    • Nucleoside analogue (e.g., lamivudine): • Generally well tolerated • High mutation rate leading to resistance

    Infectious Hepatitis

    • Hepatitis C: • RNA virus • 0.2% of children 6-11yo and 0.4% of children 12-19yo • Spontaneous clearance in children: 20% in first 3 years of life, up to 50% by age 18 • Cirrhosis takes 10-20 years to develop, if ever • 6 genotypes • 1-3 most common in North America • 1 carries worst prognosis

    Diagnosis

    • Hepatitis C Ab (high false +ve)
    • Confirm with Hepatitis C PCR and genotype
    • In at-risk infants, continue screening until at least 18 months of age with two –ve Ab’s test in a row

    Hepatology

    • Cholelithiasis: • Types: Cholesterol, Brown Pigment, Black Pigment • Black Pigment: Hematologic origin (think coffee ground emesis)

    Chronic Pancreatitis

    • Causes: Toxic, Tropical, Metabolic, Idiopathic, Genetic, Autoimmune, Anatomic, Obstructive, Recurrent acute
    • Investigations: MRCP, IgG4, genetics

    Liver Pearls

    • If AST > ALT, think EtOH, Myopathies, Renal syndromes, Hemolysis, Intestinal inflammation, Adenovirus infection
    • If ALP is abnormally low, think Zinc deficiency
    • If ALP low or normal + other enzymes high, think Wilson’s Disease
    • Vitamin K-dependent Factors: Factor X, Factor IX, Factor VII, Factor II, Protein C, Protein S
    • Factor VIII is the only coagulation factor not made in the liver

    Managing Functional Constipation in Children

    • Education about constipation important
    • Breastfeeding variability (qFeed – q10d)
    • Rome III Criteria for diagnosis (similar to Rome IV)
    • Fecal disimpaction needed to initiate treatment (PEG, enemas, mineral oil, hospitalization)
    • Investigations rarely necessary
    • PEG3350 recommended for long-term Rx/ Stool diary, fiber intake
    • Regular followup needed
    • Referral to Pediatric GI for refractory or organic pathology

    Position Statement: Energy and Sports Drinks in Children and Adolescents

    • Not recommended: contributes to obesity, mixing with alcohol
    • Health Canada: No immediate safety concerns related to caffeinated energy drinks, but more research is needed
    • Higher risk from energy drinks in CVD, Renal, Liver Disease, Seizure d/o, Diabetes, Mood d/o, Hyperthyroidism

    Position Statement: Using Probiotics in the Paediatric Population

    • Probiotics: live micro-organisms which confer health effect on host
    • Prebiotics: non-viable food components which confer health effect on host by affecting existing microorganisms
    • Proven benefits: • Preventing antibiotic-associated diarrhea (2018 data disproves) • Preventing recurrence of C.difficile • Reducing duration of acute infectious diarrhea • Preventing infectious diarrhea • Decreasing colic symptoms • Improving IBS • Preventing NEC >1000g babies
    • Small risk in immunocompromised patients

    Position Statement: The Baby-Friendly Initiative

    • Benefits of breastfeeding: • Decreases infections in infancy • Reduced SIDS • Enhanced neurocognitive testing • Decreased maternal breast and ovarian cancer • Economical
    • Requirements for “Baby-Friendly Initiative Integrated 10 Steps for Hospitals and Community Health Services”
    • Breastfeeding should be the gold standard
    • Pasteurized donor milk recommended if no BM available
    • Delay introduction of pacifiers until BF established
    • Contraindications: HIV+, cytotoxic or radioactive treatment in mother, galactosemia

    Position Statement: Human Milk Banking

    • Donor milk prioritized for hospitalized newborns
    • Statistics in this position statement are outdated (2010); cross-reference against other data
    • Description of testing, collection, and processing techniques
    • Pasteurization inactivates pathogens and partially reduces beneficial immune cells
    • Canadian Pediatric Society does not endorse unprocessed human milk

    Pediatric Gastroenterology and Hepatology

    • The course is led by Dr. Nikhil Pai, Associate Professor, Pediatric GI & Nutrition, McMaster University.

    Objectives

    • Review RCPSC Training Objectives for Pediatric Gastroenterology.
    • Review current evidence-based guidelines for managing RCPSC recognized problems.
    • Prepare for clinical scenarios that may be encountered on MCQ/OSCE.

    Outline of Topics

    • 14 topics covered, including:
      • Malabsorption
      • Celiac Disease
      • IBD
      • Constipation
      • Diarrhea
      • GERD & Dysphagia
      • Cow's Milk Protein Allergy
      • Intestinal Bleeding
      • GI Infections
      • Functional GI Disorders
      • Liver Dysfunction & Failure
      • Infectious Hepatitis
      • Liver Transplantation

    Malabsorption

    • Failure to thrive and chronic diarrhea are common OSCE/MCQ scenarios.
    • Approach to malabsorption:
      • Decreased intake
      • Malabsorption
      • Hypermetabolism
    • Malabsorption categorized by major macronutrients:
      • Fat malabsorption: Biliary, Pancreas, Ileum
      • Protein malabsorption: Pancreas, SI
      • Carbohydrate malabsorption: Primary causes (enzyme deficiencies), Dietary causes (saturated normal enzyme levels)
    • Panmalabsorption: Generalized intestinal inflammation/resection

    Celiac Disease

    • Not associated with an increased risk: William's Syndrome
    • Associated with an increased risk:
      • Type 1 Diabetes
      • IgA Deficiency
      • Down Syndrome
      • Turner Syndrome
      • First Degree Relative with Celiac
    • Diagnosis: Bloodwork +ve (not sufficient alone), Intestinal Biopsy (Duodenum/Jejunum)
    • Treatment: Lifelong gluten-free diet
    • Typical Celiac Disease: Autoimmune enteropathy caused by systemically acting antibodies against gluten
    • Extraintestinal manifestations:
      • Dermatitis Herpetiformis
      • Osteopenia/Osteoporosis
      • Short Stature
      • Delayed Puberty
      • Iron Deficiency Anemia
      • Hepatitis
      • Arthritis
      • Epilepsy with Occipital Lobe Calcifications

    Inflammatory Bowel Disease (IBD)

    • Not a unique sign of Crohn's Disease: Hypoalbuminemia
    • Inflammatory Bowel Disease:
      • Ulcerative Colitis
      • Crohn's Disease
    • Ulcerative Colitis vs Crohn's:
      • Macroscopic: Rectum, Distribution, Terminal Ileum, Serosa, Bowel Wall, Mucosa, Stricture, Fistula, Erythema Nodosum, Uveitis, PSC
      • Microscopic: Inflammation, Lymphoid Hyperplasia, Crypt Abscess, Mucus Depletion, Granuloma, Submucosal Fibrosis
    • Major IBD Medication Classes:
      • Induce Remission: Tube feeds, Corticosteroids, 5-ASA, Biologics
      • Maintain Remission: 5-ASA, Tube feeds, Azathioprine, Methotrexate, Biologics

    Constipation

    • Not an organic cause of constipation: Hyperkalemia
    • Chronic Constipation:
      • Functional (>90%): Patient eventually develops megarectum, *Encopresis (“diarrhea”) due to overflow
      • Organic (10%): Not necessary to investigate on first visit, screen if patient fails first-line treatment
    • Laxatives by Category:
      • Osmotic: Lactulose, PEG3350, Magnesium Citrate
      • Stimulant: Picosalax, Glycerine suppository, Bisacodyl, Senokot
      • Lubricant: Mineral Oil
      • Prokinetic: Prucalopride, Linaclotide

    Functional Gastrointestinal Disorders (FGIDs)

    • Childhood/Adolescence FGIDs:

      • Nausea and vomiting disorders
      • Abdominal Migraine
      • Functional abdominal pain
      • Defecation disorders
      • Functional constipation
      • Nonretentive fecal incontinence### Functional Gastrointestinal Disorders in Infants and Toddlers
    • Rumination syndrome: repeated painless regurgitation and rechewing or expulsion of food that begins soon after ingestion of a meal, does not occur during sleep, no retching, no organic explanation, and responds to relaxation therapies and avoidance of behavioral reinforcement.

    • Rumination syndrome is treated with frequent holding and social interaction.

    Nausea & Vomiting Disorders

    • Rumination syndrome: begins soon after ingestion of a meal, does not occur during sleep, no retching, no organic explanation, and responds to relaxation therapies and avoidance of behavioral reinforcement.
    • Cyclic vomiting syndrome: ≥2 episodes of intense nausea and hyperemesis or retching lasting hours to days, episodes are stereotypical, return to usual state of health lasting weeks to months, symptoms not attributable to other conditions, and family history of migraine.

    Abdominal Pain Disorders

    • Irritable bowel syndrome: abdominal pain >4 times/month with ≥1 of the following symptoms: timing related to defecation, change in frequency of stool, and change in form of stool; if patient experiences constipation (IBS-C), pain does not resolve with resolution of constipation.
    • Functional abdominal pain: very common, occurs at least 4 times per month, includes episodic or continuous abdominal pain, and insufficient criteria for other FGID's.

    Hepatology

    • Hepatomegaly approach: 1) hepatitis (inflammation), 2) storage disorders (glycogen, lipid, protein, iron), 3) infiltrative (benign liver tumors, malignant liver tumors, disseminated tumors), 4) biliary obstruction, and 5) posthepatic obstruction (cardiac, thrombus, intrahepatic).
    • Gilbert's syndrome: autosomal dominant, genetic defect in glucuronyl transferase UDP1A1 promoter region, fasting bilirubin >40, and 10yo Wilson's (90%).
    • Causes of acute liver failure by age group: autoimmune, viral hepatitis, shock/ischemia, and others.

    Infectious Hepatitis

    • Chronic hepatitis B: HBsAg+ for >6 months, ALT >2x normal, and evidence of viral replication; treat with IFN-α or lamivudine, 20-58% spontaneous conversion/clearance.
    • Surveillance recommendations: measure ALT q6months in children >2yo, measure HBeAg and HBeAb yearly in patients with normal ALT, liver biopsy in children >2yo with elevated ALT, examine for chronic liver disease, immunize household and patient against Hepatitis A, alpha fetoprotein and ultrasound annually.

    Liver Transplantation Long-term Follow-up

    • Prognosis: spontaneous seroconversion and clearance may be as high as 70-80%, risk of hepatocellular carcinoma (HCC) is 15-35X higher, lifetime risk of HCC or cirrhosis is 15-25%.
    • Surveillance recommendations: measure ALT q6months in children >2yo, measure HBeAg and HBeAb yearly in patients with normal ALT, liver biopsy in children >2yo with elevated ALT, examine for chronic liver disease, immunize household and patient against Hepatitis A, alpha fetoprotein and ultrasound annually.

    Miscellaneous Hepatology Topics

    • Non-alcoholic fatty liver disease (NAFLD): umbrella term, NAFLD: simple steatosis vs. NASH, consider when BMI >85%, diagnosis of exclusion, only effective treatments: weight loss, exercise.
    • Chronic hepatitis in pediatrics: differential diagnosis, consider... Doppler U/S, adolescent history, autoimmune hepatitis, A1AT deficiency, Wilson's disease, celiac disease, infectious hepatitis.

    Pancreatitis

    • Acute management: fluids (1.5-2x maintenance), enteral nutrition early (PO/GT à NJ à TPN), watch for complications (SIRS response).

    Delayed Gastric Emptying

    • Descriptive term with several causes, idiopathic, postinfectious, inflammatory/autoimmune, neurologic, medication/toxins, and postsurgical.### Rome IV Criteria for Functional Gastrointestinal Disorders in Infants and Toddlers
    • Defecatory Disorders:
      • Functional Constipation: occurs ≥1/week for ≥2 months, not IBS, ≥2 symptoms in a child developmentally ≥4yo
        • £2 bowel movements/week
        • ≥1 episode of fecal incontinence/week
        • Retentive posturing
        • Painful or hard bowel movements
        • Large fecal mass in rectum
        • Large diameter stools that may block toilet
      • Nonretentive Fecal Incontinence: ≥1/month defecation in places inappropriate to social context, no fecal retention, not attributable to another condition

    Complications of Acute Liver Failure

    • Encephalopathy
    • Cerebral edema
    • Pulmonary effects
    • Coagulopathy and hemorrhage
    • Bone marrow failure
    • Hypoglycemia
    • Electrolyte/Acid-base imbalance
    • Renal Dysfunction
    • Pancreatitis
    • Ascites

    Hepatitis B Treatments

    • Interferon-a (IFN-a): cytokine, side effects: neutropenia (39%), fever, myalgia, headaches, arthralgia, anorexia, severe mood changes with weight loss
    • Nucleoside analogue (e.g., lamivudine): generally well-tolerated, but high mutation rate leading to resistance

    Hepatitis C

    • RNA virus
    • 0.2% of children 6-11yo and 0.4% of children 12-19yo
    • Spontaneous clearance in children: 20% in first 3 years of life, up to 50% by age 18
    • Cirrhosis takes 10-20 years to develop, if ever
    • 6 genotypes, 1-3 most common in North America, genotype 1 carries worst prognosis

    Diagnosis of Hepatitis C

    • Hepatitis C Ab (high false +ve)
    • Confirm with Hepatitis C PCR and genotype
    • In at-risk infants, continue screening until at least 18 months of age with two –ve Ab’s tests in a row

    Cholelithiasis

    • Cholesterol stones
    • Brown Pigment stones
    • Black Pigment stones (20%): Hematologic origin, think coffee ground emesis

    Liver Pearls

    • Liver Pearl #1: If AST > ALT, think EtOH, myopathies, renal syndromes, hemolysis (e.g., capillary blood sample), intestinal inflammation, adenovirus infection
    • Liver Pearl #2: If ALP is abnormally low, think zinc deficiency; if ALP low or normal + other enzymes high, think Wilson's Disease
    • Liver Pearl #3: Vitamin K dependent Factors are: Factor X, Factor IX, Factor VII, Factor II, Protein C, Protein S
    • Liver Pearl #4: Factor VIII is the only coagulation factor not made in the liver, distinguishing liver disease from DIC

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    A quiz focused on pediatric gastroenterology and hepatology, covering topics related to the diagnosis and treatment of digestive system disorders in children. Led by Dr. Nikhil Pai, a renowned expert in the field.

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