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What is a key characteristic of Hirshprung's Disease?
What is a key characteristic of Hirshprung's Disease?
Which of the following conditions is commonly associated with Hirshprung's Disease?
Which of the following conditions is commonly associated with Hirshprung's Disease?
What typically indicates a diagnosis of Hirshprung's Disease in newborns?
What typically indicates a diagnosis of Hirshprung's Disease in newborns?
What complication is a leading cause of death in children with Hirshprung's Disease?
What complication is a leading cause of death in children with Hirshprung's Disease?
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Which statement about the demographic prevalence of Hirshprung's Disease is incorrect?
Which statement about the demographic prevalence of Hirshprung's Disease is incorrect?
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What is a common sign of enterocolitis in a child?
What is a common sign of enterocolitis in a child?
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Which diagnostic procedure is considered definitive for Hirschsprung disease?
Which diagnostic procedure is considered definitive for Hirschsprung disease?
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What distinguishing feature is observed during a rectal exam of a child with Hirschsprung disease?
What distinguishing feature is observed during a rectal exam of a child with Hirschsprung disease?
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What complication can arise from Hirschsprung disease that may lead to significant morbidity?
What complication can arise from Hirschsprung disease that may lead to significant morbidity?
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What is a typical sign of bowel obstruction in infants?
What is a typical sign of bowel obstruction in infants?
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What is the recommended initial treatment approach for managing enterocolitis in children?
What is the recommended initial treatment approach for managing enterocolitis in children?
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What characteristic of stool may indicate the presence of Hirschsprung disease?
What characteristic of stool may indicate the presence of Hirschsprung disease?
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Which of the following should be avoided if a patient has enterocolitis during a barium enema?
Which of the following should be avoided if a patient has enterocolitis during a barium enema?
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What is the recommended initial treatment for a child with enterocolitis?
What is the recommended initial treatment for a child with enterocolitis?
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Which condition is indicated by the esophagus failing to connect to the stomach?
Which condition is indicated by the esophagus failing to connect to the stomach?
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What are the expected age ranges for the peak occurrence of gastroesophageal reflux in infants?
What are the expected age ranges for the peak occurrence of gastroesophageal reflux in infants?
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What symptom is most commonly associated with gastroesophageal reflux in infants?
What symptom is most commonly associated with gastroesophageal reflux in infants?
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What potential complication can arise from Nissen fundoplication surgery?
What potential complication can arise from Nissen fundoplication surgery?
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What triggers are commonly associated with chronic inflammatory diseases of the GI tract?
What triggers are commonly associated with chronic inflammatory diseases of the GI tract?
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In terms of demographics, which group is more likely to develop inflammatory bowel diseases?
In terms of demographics, which group is more likely to develop inflammatory bowel diseases?
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What medication class is preferred for the healing of severe gastroesophageal reflux?
What medication class is preferred for the healing of severe gastroesophageal reflux?
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What might indicate a child is experiencing Sandifer's syndrome related to reflux?
What might indicate a child is experiencing Sandifer's syndrome related to reflux?
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Which symptom is more indicative of clinical failure to thrive due to severe gastroesophageal reflux?
Which symptom is more indicative of clinical failure to thrive due to severe gastroesophageal reflux?
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What is the primary goal of treatment for patients with Crohn's disease?
What is the primary goal of treatment for patients with Crohn's disease?
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Which of the following medications is used as part of the first-line therapy to control inflammation in Crohn's disease?
Which of the following medications is used as part of the first-line therapy to control inflammation in Crohn's disease?
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Which lab tests are commonly used in the diagnosis and management of inflammatory bowel disease?
Which lab tests are commonly used in the diagnosis and management of inflammatory bowel disease?
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What is a significant long-term complication associated with Crohn's disease?
What is a significant long-term complication associated with Crohn's disease?
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Which nutritional strategy is indicated for patients with Crohn's disease to support their health?
Which nutritional strategy is indicated for patients with Crohn's disease to support their health?
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What is the role of immunomodulators in managing Crohn's disease?
What is the role of immunomodulators in managing Crohn's disease?
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What role does nutritional supplementation play in the management of children with Crohn's disease?
What role does nutritional supplementation play in the management of children with Crohn's disease?
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Which of the following is a major characteristic of Crohn's disease in pediatric patients?
Which of the following is a major characteristic of Crohn's disease in pediatric patients?
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What is the primary reason for the soft and frequent stools observed in infants?
What is the primary reason for the soft and frequent stools observed in infants?
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At what age do infants typically start producing sufficient digestive enzymes like amylase and lactase?
At what age do infants typically start producing sufficient digestive enzymes like amylase and lactase?
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How does the stomach position in infants differ from that of adults?
How does the stomach position in infants differ from that of adults?
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Why is regurgitation common in infants?
Why is regurgitation common in infants?
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What role does the immature liver function play in infant health?
What role does the immature liver function play in infant health?
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What anatomical characteristic of infants contributes to their increased intestinal permeability?
What anatomical characteristic of infants contributes to their increased intestinal permeability?
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Which of the following is a common assessment characteristic of infants regarding abdominal appearance?
Which of the following is a common assessment characteristic of infants regarding abdominal appearance?
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Which observation is NOT indicative of potential abdominal issues in children?
Which observation is NOT indicative of potential abdominal issues in children?
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What is the recommended method for assessing an infant's abdomen?
What is the recommended method for assessing an infant's abdomen?
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What is characteristic of normal bowel sounds in infants and children?
What is characteristic of normal bowel sounds in infants and children?
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What condition is associated with absent bowel sounds?
What condition is associated with absent bowel sounds?
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Which statement regarding the umbilical cord is incorrect?
Which statement regarding the umbilical cord is incorrect?
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What is the appropriate method for assessing the spleen in infants?
What is the appropriate method for assessing the spleen in infants?
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Which abdominal palpation technique is recommended when assessing a child?
Which abdominal palpation technique is recommended when assessing a child?
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What clinical sign should be noted when inspecting the skin of infants?
What clinical sign should be noted when inspecting the skin of infants?
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When percussing an infant's abdomen, what sound is expected due to their propensity to swallow air?
When percussing an infant's abdomen, what sound is expected due to their propensity to swallow air?
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Which symptom is least likely to indicate enterocolitis in a child with Hirschsprung disease?
Which symptom is least likely to indicate enterocolitis in a child with Hirschsprung disease?
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What is a major concern that necessitates a rectal biopsy for diagnosis in Hirschsprung disease?
What is a major concern that necessitates a rectal biopsy for diagnosis in Hirschsprung disease?
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In the context of managing Hirschsprung disease, what is the primary purpose of irrigation of the colon before surgery?
In the context of managing Hirschsprung disease, what is the primary purpose of irrigation of the colon before surgery?
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Which diagnostic method assesses nerve function in the anal sphincter for Hirschsprung disease?
Which diagnostic method assesses nerve function in the anal sphincter for Hirschsprung disease?
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What is a potential long-term complication following surgery for Hirschsprung disease?
What is a potential long-term complication following surgery for Hirschsprung disease?
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What is the primary cause of the mechanical obstruction in Hirshprung's Disease?
What is the primary cause of the mechanical obstruction in Hirshprung's Disease?
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Which demographic group is at a higher risk of having Hirshprung's Disease?
Which demographic group is at a higher risk of having Hirshprung's Disease?
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What is a typical outcome if a newborn fails to pass meconium within 24 to 48 hours after birth?
What is a typical outcome if a newborn fails to pass meconium within 24 to 48 hours after birth?
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What is the consequence of the internal anal sphincter failing to relax in Hirshprung's Disease?
What is the consequence of the internal anal sphincter failing to relax in Hirshprung's Disease?
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What significant complication arises from intestinal distention and ischemia of the bowel wall in children with Hirshprung's Disease?
What significant complication arises from intestinal distention and ischemia of the bowel wall in children with Hirshprung's Disease?
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What should be avoided during the first 2 to 4 weeks after surgery for Hirschsprung disease?
What should be avoided during the first 2 to 4 weeks after surgery for Hirschsprung disease?
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Which treatment is recommended for infants with severe gastroesophageal reflux who fail to respond to medical therapy?
Which treatment is recommended for infants with severe gastroesophageal reflux who fail to respond to medical therapy?
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What systemic issue can be a consequence of untreated gastroesophageal reflux in infants?
What systemic issue can be a consequence of untreated gastroesophageal reflux in infants?
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Which group is at the highest risk for developing inflammatory bowel diseases?
Which group is at the highest risk for developing inflammatory bowel diseases?
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What is a notable cause of aspiration risk in infants with gastroesophageal reflux?
What is a notable cause of aspiration risk in infants with gastroesophageal reflux?
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Which symptom is associated with Sandifer's syndrome in children with reflux?
Which symptom is associated with Sandifer's syndrome in children with reflux?
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What is the mechanism by which proton pump inhibitors help in managing gastroesophageal reflux?
What is the mechanism by which proton pump inhibitors help in managing gastroesophageal reflux?
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What are the common triggers for chronic inflammatory diseases of the GI tract?
What are the common triggers for chronic inflammatory diseases of the GI tract?
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What is the primary purpose of using corticosteroids in the management of inflammatory bowel disease?
What is the primary purpose of using corticosteroids in the management of inflammatory bowel disease?
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Which laboratory test is NOT typically included in the assessment for inflammatory bowel disease?
Which laboratory test is NOT typically included in the assessment for inflammatory bowel disease?
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What are the key goals of managing a pediatric patient with Crohn's disease?
What are the key goals of managing a pediatric patient with Crohn's disease?
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Which medication is specifically indicated for children 6 years or older who have not responded to other treatments for Crohn's disease?
Which medication is specifically indicated for children 6 years or older who have not responded to other treatments for Crohn's disease?
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In the context of active inflammatory bowel disease, what is a potential complication that can significantly affect patient outcomes?
In the context of active inflammatory bowel disease, what is a potential complication that can significantly affect patient outcomes?
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What is considered an essential component of the nutritional management for pediatric patients with Crohn's disease?
What is considered an essential component of the nutritional management for pediatric patients with Crohn's disease?
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What is the significance of performing mucosal biopsies during diagnosis of inflammatory bowel disease?
What is the significance of performing mucosal biopsies during diagnosis of inflammatory bowel disease?
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Study Notes
Hirschsprung's Disease (Congenital Aganglionic Megacolon)
- Mechanical obstruction due to inadequate motility of part of the intestine
- Congenital, a condition diagnosed in the womb.
- Lack of nerve fibers in the aganglionic portion eliminates sensation of bowel movement.
- Accumulation proximal to the aganglionic area leads to megacolon (enlarged colon).
Incidence
- Affects 1/4 of all neonatal obstructions, but diagnosis may not occur until later.
- More frequent in children with Down Syndrome and leukemia.
- Four times more frequent in boys.
- One in 5,000 births
- Also associated with T1D, congenital HD.
- Meconium within 24-48 hours of birth is a significant indicator.
Pathophysiology
- Lack of ganglionic cells in one or more segments of the colon
- The cause is unknown.
- This results in a lack of propulsive movements (peristalsis), causing intestinal content buildup and distension proximal to the defect (megacolon).
- The internal anal sphincter fails to relax.
- Intestine inflammation (enterocolitis) is caused by distension and ischemia (lack of blood flow), and may be fatal in children with Hirschsprung's.
Signs and Symptoms (Newborn Period)
- Failure to pass meconium within 24–48 hours after birth.
Signs and Symptoms (Infancy)
- Reluctance to take fluids
- Bile-stained vomit
- Firm, distended abdomen
- Tracking abdominal girth
- Failure to thrive (not growing or gaining weight)
- Constipation
- Abdominal distention
- Episodes of diarrhea and vomiting
- Fever
- Severe exhaustion
Signs and Symptoms (Childhood)
- Constipation
- Ribbon-like, foul-smelling stool
- Stool leaks
- Abdominal distention
- Visible peristalsis
- Fecal masses (easily palpable)
- Poorly nourished and anemic child
Diagnosis
- Physical examination (rectal exam – tight internal sphincter, absence of stool)
- Barium enema (contrast enemas should be avoided if enterocolitis is present)
- Anorectal manometry (catheter passed into rectum, balloon inflated to assess nerve function in anal sphincter)
- Rectal biopsy (definitive diagnosis)
Treatment
- Pre-surgical colon irrigation
- Surgery to unlock colon and preserve bowel control
- Removal of aganglionic colon between rectum and sigmoid colon approximately 2 cm above the pectinate zone
- Healthy intestine connected to rectum.
- 30% of patients may still experience colitis and need further irrigations for months or years.
- Temporary ostomy may be needed before connecting the bowel.
Enterocolitis
- Inflammation of intestine and colon, most common cause of death.
- Signs of perforated bowel, including vital signs — shock, absent bowel sounds, distension, tenderness, vomiting, irritability, dyspnea, cyanosis, and fever. (No rectal temps)
Other Important Notes
- Hirschsprung's disease patients are at risk for infection in the large intestine (called enterocolitis)
- Symptoms of enterocolitis include fever, swollen belly (distention), constipation, vomiting, explosive diarrhea, and foul-smelling stool or gas.
- Colonic irrigation is used to clear the bowels when enterocolitis is suspected, involves using salt water (saline) to irrigate the colon through a tube. -Post-surgical care includes colostomy care—preventing skin breakdown and infection, and addressing any urinary incontinence, and pain control as needed.
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Description
This quiz covers Hirschsprung's disease, a congenital condition characterized by inadequate intestinal motility due to the absence of nerve fibers. The content discusses its incidence, pathophysiology, and key indicators for diagnosis. Test your knowledge on this crucial topic in pediatric gastroenterology.
