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48 Cards in this Set
- Front
- Back
What are 2 causes of congenital intestinal obstruction (atresias and stenoses)?
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1. failure in development
2. intrauterine vascular accidents |
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What is atresia?
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Complete obstruction
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What is stenosis?
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Narrowing of intestine due to a diaphragm or web that partially obstructs
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How does atresia present clinically?
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vomiting, abdominal distention
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What does esophageal atresia commonly present with?
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Tracheo-esophageal fistula
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What does esophageal atresia and fistula present with?
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Aspiration, regurgitation, respiratory distress
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Is duodenal stenosis more commonly proximal or distal?
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Proximal
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What is the major cause of duodenal stenosis?
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Web- resulting from failure of recanalization
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What is Annular Pancreas?
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Another cause of duodenal stenosis due to surrounding of the duodenum by pancreas
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What causes jejuno-ileal atresia?
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Intrauterine vascular accidents
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How are jejuno-ileal atresias classified?
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1. Whether the blind ends are connected
2. Whether there is a corresponding defect in the mesentery |
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How are anorectal atresias classified?
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High (above levator sling) or Low
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How do anorectal atresias present?
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Abnormal perineum, imperforate anus, and often fistula to bladder, urethra, vagina
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What is an omphalocele?
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Defect of the abdominal wall where the umbilical cord inserts - sac containing intestinal loops, liver
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What is gastroschisis?
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Defect in abdominal wall to the right of the umbilical cord - evisceration of bowel, stomach, gonads but NO SAC
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What is short bowel syndrome?
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Massive loss of bowel
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Most children with short bowel syndrome require what?
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Total parenteral nutrition
Most eventually need transplantation |
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What are duplications in the GI tract?
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cystic or tubular outgrowths anywhere along GI tract
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Do duplications communicate with the intestine?
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NO! but may share wall
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Where do neuroenteric remnants originate?
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Dorsal midline GI
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Where are neuroenteric remnants most common?
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Cervical and lumbar regions
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What are the clinical signs of neuroenteric remnants?
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GI obstruction, respiratory distress, and CNS manifestations such as paralysis
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What is a diverticulum?
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remnant of the vitelline duct (most common- Meckel's)
Communication between yolk sac and endoderm in normal devo |
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What is the most common GI congenital anomaly?
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Meckel's diverticulum
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Where is a Meckel's diverticulum located?
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antimesenteric border of terminal ileum
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What causes most symptoms of Meckel's?
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Gastric mucosa - pain, rectal bleeding, perforation
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How many degrees and to what direction does the gut rotate in development?
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270 degrees CCW
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What does malrotation cause?
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abnormal fixation
Sometimes, volvulus and internal strangulation |
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What is pseudobstruction?
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Signs and Sx of obstruction without obstruction
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What causes pseudobstruction?
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Enteric neuropathy (Hirschsprung) (problem in nerves)
Visceral myopathy (problem in muscles) Secondary causes |
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What gives rise to the enteric NS?
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Vagal Neural crest cells
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What does failure of migration of neural crest cells result in?
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Aganglionosis (Hirschsprung)
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Does aganglionosis result in loss of Meissner's plexus or Auerbach's?
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Both.
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What are 2 genetic susceptibilities to Hirschsprung?
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1. Mutations in RET
2. Down's syndrome |
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How is Hirschsprung diagnosed?
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Absence of ganglion cells on biopsy
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What is the most common GI emergency in newborns?
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Necrotizing enterocolitis
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What is a common finding in newborns with NEC?
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Low birthweight
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What are the signs of NEC?
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abdominal distention, bloody stools, apnea, gas in bowel on radiology
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What is pneumatosis intestinalis?
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Gas in the bowel wall (found in NEC)
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What are long term results of NEC?
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Strictures, atresia, Short Bowel Syndrome
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What is intussusception?
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invagination of one intestinal segment into another
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What is the most common cause of intestinal obstruction?
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Intussusception
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Compression of the mesentery in intussusception results in what?
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Ischemia
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What is the primary defect in cystic fibrosis?
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abnormal chloride channel protein coded by CFTR gene
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What are the 4 areas of the GI tract that CF affects?
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1. Intestinal
2. Pancreatic 3. Hepatic 4. Nutritional |
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What are CF's affects on the intestines?
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Dense secretions
Meconium ileus (obstruction by viscid meconium) |
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What are CF's affects on the pancreas?
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Duct obstruction
Acinar destruction and loss Loss of exocrine function |
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What are CF's affect on the liver?
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Elevated liver enzymes (ASx)
Steatosis Cirrhosis Microgallbladder Gallstones |