Gastrointestinal Disorders in Pediatrics
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Questions and Answers

What is the primary reason for surgery in cases of umbilical hernia?

  • Size increases after 1-2 years
  • Strangulation of the intestine
  • Persistent after age 4
  • All of the above (correct)

    • Which condition is characterized by intestines failing to return to the abdominal cavity with the gut covered in a protective sac?

  • Necrotizing enterocolitis
  • Gastroschisis
  • Omphalocele (correct)
  • Umbilical hernia

    • What is a major complication associated with gastroschisis?

  • Umbilical strangulation
  • Aging of the abdominal muscles
  • Necrotizing enterocolitis
  • Ischemia of the intestine (correct)

    • What is a pathognomonic sign of pneumatosis intestinalis on imaging?

    <p>Air in the bowel wall</p> Signup and view all the answers

    Which of the following is a common treatment approach for necrotizing enterocolitis?

    <p>Cessation of feeds</p> Signup and view all the answers

    What condition is indicated by a failure to pass stool after birth and no visible anal opening?

    <p>Imperforate anus</p> Signup and view all the answers

    What is a common finding in a 2-day-old infant with mild jaundice?

    <p>Normal stooling pattern</p> Signup and view all the answers

    Which of the following conditions is associated with the potential for genetic disorders, particularly trisomy?

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

    What is the most significant risk factor for developing necrotizing enterocolitis (NEC)?

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

    Which surgical procedure might be necessary for patients with significant necrotizing enterocolitis?

    <p>Resection of necrotic bowel</p> Signup and view all the answers

    Which condition is most commonly associated with conjugated hyperbilirubinemia in newborns?

    <p>Biliary atresia</p> Signup and view all the answers

    What is the best initial test for diagnosing biliary atresia?

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

    What is the primary treatment for indirect hyperbilirubinemia in neonates?

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

    Which enzyme is responsible for converting unconjugated bilirubin to conjugated bilirubin in hepatocytes?

    <p>Hepatic glucuronyl transferase</p> Signup and view all the answers

    What complication may arise from intensive phototherapy in neonates?

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

    In which condition is bilirubin mainly transported back to the liver after hydrolysis by intestinal beta-glucuronidase?

    <p>Enterohepatic circulation</p> Signup and view all the answers

    What is a potential sign of biliary atresia in a newborn?

    <p>Pale stools</p> Signup and view all the answers

    What effect does ceftriaxone have on bilirubin levels in neonates?

    <p>Increases free bilirubin</p> Signup and view all the answers

    What syndrome occurs due to a complete absence of glucuronyl transferase activity?

    <p>Crigler-Najjar syndrome type I</p> Signup and view all the answers

    What is an additional sign commonly seen in infants diagnosed with biliary atresia?

    <p>Dark urine</p> Signup and view all the answers

    What is the most common cause of neonatal jaundice?

    <p>Physiological rise in unconjugated bilirubin</p> Signup and view all the answers

    What is the primary purpose of the treatment for neonatal jaundice?

    <p>To reduce the risk of kernicterus</p> Signup and view all the answers

    Which condition is indicated by intestinal loops protruding through an abdominal defect in the right paraumbilical area?

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

    Which factor carries the greatest risk for a preterm baby developing necrotizing enterocolitis?

    <p>Introduction of enteral feeding</p> Signup and view all the answers

    What is likely the diagnosis for a 3-day-old infant with a total serum bilirubin of 11.8 mg/dl and direct bilirubin of 0.2 mg/dl?

    <p>Physiologic jaundice</p> Signup and view all the answers

    What is the primary cause of physiological jaundice in newborns?

    <p>Immature hepatic conjugation of bilirubin</p> Signup and view all the answers

    What should be the next step in evaluating a newborn with hemolytic disease when the mother is type O positive and baby type A positive?

    <p>Direct Coombs test</p> Signup and view all the answers

    Which of the following is NOT commonly associated with pathological neonatal jaundice?

    <p>Physiological conditions</p> Signup and view all the answers

    Which of the following is NOT a feature associated with kernicterus?

    <p>Icteric hepatitis</p> Signup and view all the answers

    Which treatment is NOT generally used for managing neonatal jaundice?

    <p>Direct bilirubin infusions</p> Signup and view all the answers

    Which condition requires a work-up for pathologic hyperbilirubinemia in newborns?

    <p>Bilirubin rising &gt;5 mg/dL/day</p> Signup and view all the answers

    What lab finding would indicate a potential Rh incompatibility in a newborn?

    <p>High reticulocyte count</p> Signup and view all the answers

    What is one of the common causes of increased hemolysis leading to jaundice in newborns?

    <p>Rh incompatibility</p> Signup and view all the answers

    Which of the following is a key sign of kernicterus in infants?

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

    Which laboratory finding is typically associated with immune-mediated hemolysis in newborns?

    <p>Increased direct bilirubin</p> Signup and view all the answers

    In which scenario would twins be at risk for hyperbilirubinemia due to delayed cord clamping?

    <p>If there is twin-twin transfusion syndrome</p> Signup and view all the answers

    Which of the following indicates a significant risk for developing kernicterus?

    <p>Bilirubin level rising &gt;5 mg/dL/day</p> Signup and view all the answers

    What type of bilirubin is primarily increased in cases of kernicterus?

    <p>Unconjugated bilirubin</p> Signup and view all the answers

    What is the suggested treatment for significant hyperbilirubinemia in newborns due to increased red blood cell volume?

    <p>Partial exchange transfusion</p> Signup and view all the answers

    Study Notes

    Gastrointestinal and Hepatobiliary Disorders

    • Presented by Professor Abdellatif Abdelmoez, Professor of Pediatrics, Asyut University.

    Abdominal Wall Defects

    • Umbilical hernia
    • Omphalocele
    • Gastroschisis

    Umbilical Hernia

    • Failure of the umbilical ring closure; weakness of abdominal muscles.
    • Most are small and resolve within 1-2 years without treatment.
    • Surgery is required if the hernia continues to grow after 1-2 years, shows symptoms (such as strangulation or incarceration), or persists after age 4.

    Omphalocele

    • Failure of intestines to return to the abdominal cavity; gut protrudes through the umbilicus.
    • Covered by a sac, providing protection.
    • Often associated with other major malformations and potential genetic disorders (e.g., trisomy).
    • Large omphaloceles require staged surgical reduction (using a silo) to prevent respiratory failure and ischemia.

    Gastroschisis

    • Defect in the abdominal wall, lateral to the umbilicus.
    • Any part of the gastrointestinal (GI) tract may protrude.
    • Not covered by a sac.
    • Associated with significant intestinal abnormalities (atresia, stenosis, ischaemia, short gut).
    • Surgical intervention varies based on the condition of the intestines: large lesions with no ischemia typically needing staged reduction, similar to omphalocele.

    Necrotizing Enterocolitis (NEC)

    • Transmural intestinal necrosis.
    • Prematurity is the biggest risk factor; rare in full-term infants.
    • Commonly arises from the introduction of feeds, often characterized by bloody stools, apnea, lethargy, and abdominal distention (once perforation has occurred).
    • Pneumatosis intestinalis (air in the bowel wall) is a hallmark sign, visible on plain abdominal X-rays.
    • Treatment includes cessation of feeds, using gut decompression, systemic antibiotics, and supportive care.
    • Surgical resection of necrotic bowel is sometimes necessary.

    Imperforate Anus

    • Failure to pass stool after birth; absence of an anal opening.
    • Surgical correction is the treatment.
    • Can be part of a VACTERL association.

    VACTERL Association

    • A constellation of congenital anomalies involving Vertebral defects, Anal atresia, Cardiac defects, Tracheo-Esophageal fistula, Renal anomalies, and Limb abnormalities.
    • A diagnosis is usually made based on presence of three or more features in the clinical presentation.
    • Associated with a normal level of intelligence in patients and the mode of inheritance appears to be sporadic.
    • Ventricular septal defects (VSDs) are the most common cardiac defects associated.

    Neonatal Jaundice

    • Yellowish discoloration of the eyes, skin, and mucous membranes in the first month of life due to elevated bilirubin levels in the blood.
    • Pathophysiology includes increased production of bilirubin from fetal red blood cell breakdown, along with immature hepatic conjugation and elimination in the first week of life.
    • Increased unconjugated bilirubin can cross the blood-brain barrier, leading to kernicterus (bilirubin encephalopathy).
    • Jaundice in the first 24 hours or rapidly increasing bilirubin levels (>5 mg/dL/day) or bilirubin >13 mg/dL in term infants, or direct bilirubin >2 mg/dL at any time warrants further investigation.

    Physiologic vs. Pathologic Jaundice

    • Physiologic jaundice: Usually appears on the second or third day of life in full-term newborns, peaks on the second or third day and resolves by the 7th day (in term infants). Bilirubin levels are usually low (<13 mg/dL). Rise is normally <5 mg/dL/day
    • Pathologic jaundice: Can appear within the first 24 hours of life, persist beyond the 7th day (or 3 weeks in premature babies). It’s potentially due to an underlying condition (can be conjugated or unconjugated). Rises are typically greater than 5 mg/dL/day, or above 13 mg/dL in term infants.

    Biliary Atresia

    • Progressive obliterative cholangiopathy leading to obstruction of the extrahepatic bile ducts.
    • Characterized by conjugated hyperbilirubinemia (direct bilirubin is elevated), poor feeding, vomiting, lethargy, hepatosplenomegaly, acholic (clay-colored) stools, and dark urine.
    • Generally presents in the first 2 weeks of life.
    • Diagnosis involves an ultrasound and liver biopsy.
    • Treatment typically involves the Kasai procedure initially, followed by liver transplantation as an option for long-term management.

    Bilirubin Metabolism

    • Bilirubin is a byproduct of red blood cell breakdown.
    • Unconjugated (indirect-acting) bilirubin is fat-soluble and binds to albumin for transport.
    • Hepatic conjugation converts unconjugated bilirubin to conjugated (direct-acting) bilirubin, making it water-soluble, and enabling excretion.
    • Conjugated bilirubin is eliminated in bile and urine.

    Breast-feeding Jaundice vs. Breast Milk Jaundice

    • Breastfeeding jaundice: Results from poor feeding and inadequate calorie intake, often in first-time mothers, characterized by early appearance.
    • Breast milk jaundice: Due to glucuronidase in breast milk, typically develops a few days after adequate breastfeeding has begun, resolves spontaneously over a few weeks or months.

    Hemolytic Disease of the Newborn

    • An immune-mediated hemolytic disorder typically triggered by Rh or ABO incompatibility between mother and fetus.
    • Incompatibility causes the mother to create antibodies which attack the red blood cells of the fetus.
    • Symptoms include jaundice and increased unconjugated bilirubin, likely to develop in the first 24 hours of birth.
    • Diagnosis involves testing for the presence of antibodies (anti-Rh, anti-A, anti-B, etc.).

    Additional Information

    • Diagnostic approach to neonatal jaundice involves assessing various factors to determine whether the jaundice is physiological or abnormal.
    • Workup for pathologic jaundice should consider factors such as: age of manifestation, rate of bilirubin increase, or presence of direct bilirubin.
    • Specific treatments of various types of neonatal jaundice are mentioned.

    Quiz Questions

    • A new-born displaying uncovered intestinal loops in the right paraumbilical area likely has what condition? (Gastroschisis)
    • A preterm baby with IV nutritional support initially, followed by enteral feeding, presenting with lethargy, apnea, bloody stools and abdominal distension has what risk factor prominent? (Prematurity and IV feeding)
    • A 3-day-old breastfed infant with yellowish skin, type O blood (baby & mother) has a total serum bilirubin of 11.8 mg/dL, and direct serum bilirubin of 0.2 mg/dL. What diagnosis is most likely? (Physiological jaundice)
    • A primiparous woman with a type O positive blood gives birth to a child with A positive blood. The serum bilirubin level is 10 mg/dL, and Reticulocytes 8%, at 18 hours of age. What next step would be taken? (Direct Coombs’ test, measuring G6PD level and blood smear)

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    Gastrointestinal and Liver Disease Final PDF

    Description

    This quiz covers key topics related to gastrointestinal and hepatobiliary disorders in pediatrics, including abdominal wall defects such as umbilical hernia, omphalocele, and gastroschisis. It discusses their definitions, symptoms, and treatment options, providing a comprehensive overview for medical students and professionals.

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