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Neo Board Review MAP > Gastroenterology > Flashcards

Gastroenterology Flashcards

1
Q

Pancreatic amylase

A

Present at 22 weeks gestation
Adequate amounts produced
Decreased secretion at birth

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2
Q

Glucoamylase

A

Normal action at birth

Located in intestinal brush border Removes glucose from end of starch

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3
Q

Intestinal disaccharidases

A

All except lactase reach adult levels at 28 weeks

Glucosidases - sucrase, maltase, isomaltase

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4
Q

Colonic bacteria

A

Helps ferment malabsorbed carbs to acids

Colonics salvage pathway

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5
Q

Lactase

A

Adult levels at 36 weeks gestation

Colonic salvage pathway helps limit carb malabsorption

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6
Q

Benefits to enteral feeding

A

Mucosal development/growth depends on enteral nutrients
Increases concentration of G.I. hormones
Improves gut barrier function
Increases G.I. blood cell

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7
Q

How much of the immune system does the intestinal tract make up?

A

70%

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8
Q

Nonspecific intestinal barrier defenses

A 
Mucus layer
Digestive enzymes, bile salts
IgA
Peristalsis
Tight junctions
Antimicrobial peptides
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9
Q

Epithelial cell G.I. immune function

A 
Goblet cell
Enterocytes
Enteroendocrine cells
M cells
Intraepithelial lymphocytes
Paneth cells
Gut associated lymphoid tissue (GALT)
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10
Q

Two major pancreatic proteases

A

Trypsin
Chymotrypsin

Decreased in preterm and term infants compared to older children (PT more)

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11
Q

Peptidase levels

A

Well developed early in life

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12
Q

Amino acid transport in the newborn

A

Well developed

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13
Q

Protein digestion

A

Proteins are broken down to peptides in the stomach and duodenum by pepsin and pancreatic proteases
Peptides are broken down to amino acids by peptidases

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14
Q

Fat digestion

A

Fats form micelles after bile acid emulsification
Hydrolyzed by lipase
Fatty acids are transferred across intestinal mucosa
Triglyceride re-synthesized from FA and enterocytes then forms a chylomicron which is secreted into blood

Breastmilk lipases help

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15
Q

What does a triglyceride become after it’s broken down?

A

2 free fatty acids and 1 glyceride molecule

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16
Q

Digestion of short and medium chain FA

A

Absorbed directly into the blood
Travel through portal vein
Bile acids are not required
<14 carbons

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17
Q

Salivary and pancreatic amylase

A

Hydrolyze starch, glycogen, dextrin -> glucose, maltose, limit-dextrins
Decreased in newborns

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18
Q

Carbohydrate digestion

A

Starch and glycogen are broken down by amylase

Polysaccharides and disaccharides are broken down to monosaccharides

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19
Q

Glucose and galactose Transport/absorption

A

Active transport via SGLT1 transporter into the cell
- Needs Na/K pump
- Inefficient in the newborn especially pre-term
Crosses into circulation via GLUT2 transporter

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20
Q

Fructose Transport/absorption

A

Facilitated/passive transport via GLUT5 transporter

Crosses into circulation via GLUT2 transporter

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21
Q

What is absorbed in the stomach?

A

Water
Copper
Iodide
Fluoride

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22
Q

What is absorbed in the duodenum?

A 
Calcium/Phos/Mag
Iron
Copper and selenium
Vitamins B1, B2, B3
Biotin, folate
Vitamin A, D, E, K
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23
Q

What is absorbed in the jejunum?

A 
Lipids
Monosaccharides
Amino acids/small peptides
Vitamins B1,B2,B3,B5,B6
Biotin/folate
Vitamin C
Vitamins A, D, E, K
Ca/Phos/Mag
Iron
Zinc
Chromium and manganese
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24
Q

What is absorbed in the ileum?

A 
Vitamin C
Folate
Vitamin B12
Vitamin D and K
Magnesium
Bile salts and acids

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25
Q

What is absorbed in the large intestine?

A 
Water
Electrolytes
Vitamin K
Biotin
Short chain fatty acids
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26
Q

Risks for Spontaneous gastric perforation

A

Perinatal stress

Postnatal steroids

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27
Q

Etiology of spontaneous gastric perforation

A

Mechanical overdistention versus ischemia

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28
Q

Symptoms of spontaneous gastric perforation

A

Typically large and proximal
2-7 days of life
Abrupt abdominal distention, respiratory distress, shock

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29
Q

Spontaneous intestinal perforation Epidemiology

A

5% of extremely low birthweight infants
Compared to general population - more likely to have a PDA treated with indomethacin, more likely to receive vasopressor support

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30
Q

SIP vs NEC

A 
SIP:
Smaller/more immature
Earlier postnatal age
Unlikely to be fed
PDA treated with indomethacin
Vasopressor support
Received surfactant and ventilation
Maternal chorio
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31
Q

Pathophysiology of SIP

A

MC involves terminal ileum
Intestinal mucosa is robust and viable
Hypothesis
- meds and other exposures -> skewed trophism (mucosal hyperplasia, submucosal thinning, smooth muscle necrosis) -> bowel wall fragility

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32
Q

Symptoms of SIP

A 
First week of life
Abdominal distention
Black/blue discoloration
Hypotension
X-ray with pneumoperitoneum, no pneumatosis or portal venous gas
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33
Q

Prognosis of SIP

A

Increased mortality compared to controls
Decreased mortality compared to NEC
Neurodevelopmental impairment but less when compared with NC

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34
Q

Risks of pyloric stenosis

A

Mother with PS: 19% son, 7% daughter
Father with PS: 5% son, 2.4% daughter
One child with PS: 3% chance next child (4% male, 2.4% female)

Males>females 5:1

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35
Q

Symptoms of pyloric stenosis

A

1-5 weeks
Non-bilious projectile vomiting, dehydration
Hypochloremia, hypokalemic metabolic alkalosis

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36
Q

Bilious emesis

A

Due to obstruction beyond the outlet of the common bile duct (beyond sphincter of Oddi)
Bile produced in the liver, stored in gallbladder

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37
Q

Etiology of duodenal atresia

A

Failure of recanalization during 8-10th week after obliteration of lumen
Usually in second part of duodenum past sphincter of Oddi

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38
Q

What is associated with duodenal atresia?

A 
T21 81%
Malrotation 20%
Congenital heart disease 30%
Esophageal atresia 10%
Genitourinary anomalies 11%
Annular pancreas 20%
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39
Q

Symptoms of duodenal atresia

A 
In utero - polyhydramnios, distended duodenum
Bilious emesis in the first 24 hours of life
Can have meconium passage
Abdominal distension (upper abdomen)

Symptoms can also be due to an annular pancreas which usually presents later

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40
Q

What does duodenal atresia look like on x-ray?

A

Double bubble
Air fluid level
No distal intestinal air

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41
Q

Jejunal/ileal atresia epidemiology

A 
Overall incidence greater than duodenal or colonic atresias
Usually a single atresia
Distal ileum 36%
Proximal jejunum 31%
Distal jejunum on 20%
Proximal ileum 13%
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42
Q

Etiology of jejunal/ileal atresia

A

In utero mesenteric vascular occlusion

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43
Q

Symptoms of jejunal/ileal atresia

A

Polyhydramnios in 1/3 patients with jejunal atresia, more rare for distal
Often SGA
Bilious emesis
Abdominal distention worse with more distal atresias
Can fail to pass meconium

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44
Q

What does jejunal/ileal atresia look like an x-ray?

A

X-ray: triple bubble, air fluid levels
Greater the amount of air = lower the obstruction
Peritoneal calcifications = In utero perforation and meconium peritonitis

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45
Q

What is malrotation associated with?

A 
CDH
Abdominal wall defects
Intestinal atresias
Beckwith Wiedemann
Heterotaxy/situs inversus
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46
Q

Etiology of malrotation

A

Failure of normal rotation
Abnormal fixation - Ladd’s bands (fibrous bands between the cecum and right posterior retroduodenal peritoneum)
If volvulus occurs can lead to intestinal ischemia

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47
Q

What does malrotation look like on x-ray?

A

If complete obstruction- dilated bowel loops, air fluid levels, decreased air distal to the obstruction

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48
Q

Etiology of meconium ileus

A

Obstruction of the terminal ileum due to hyperviscous secretions from mucous glands of small intestine
Meconium with decreased water -> adheres to intestinal lining

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49
Q

How many patients with CF have meconium ileus?

A

10-15%

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50
Q

How many patients with meconium ileus have CF?

A

90%

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51
Q

Symptoms of meconium ileus

A 
24 to 48 hours
Usually no passage of meconium
Abdominal distention at birth
Bilious emesis
Palpable bowel loops
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52
Q

What does an x-ray of meconium ileus look like?

A

Descendent intestinal loops without air fluid levels

Can appear granular or bubbly

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53
Q

What does malrotation look like on barium enema?

A

Cecum seen in RUQ instead of RLQ

Corkscrew appearance of proximal jejunum

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54
Q

Management of meconium ileus

A

Uncomplicated treat with enema (hypertonic barium, gastrografin, or Hypaque)
Draws fluid into the intestines and allows for disimpaction
Successful in 60%

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55
Q

Etiology of meconium peritonitis

A

In utero intestinal perforation with meconium spillage into peritoneal cavity
Usually secondary to meconium ileus, intestinal atresia, volvulus, gastroschisis

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56
Q

Meconium pseudocysts

A

Type of meconium peritonitis
Wall of fibrous tissue surrounds meconium
Meconium present for many weeks

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57
Q

Adhesive meconium peritonitis

A

Widespread contamination of peritoneal cavity
Days-weeks before birth
X-ray with calcifications
Vascular adhesion seen in surgery
Often associated with intestinal obstruction

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58
Q

Meconium ascites

A

Type of meconium peritonitis
Perforation only a few days before birth
No calcifications on x-ray

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59
Q

Infected meconium peritonitis

A

Intestinal perforation that does not seal

Seating of micro organisms into peritoneal cavity

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60
Q

Etiology of microcolon

A

Functional immaturity of ganglion cells
Affects descending and rectosigmoid colon
Transient functional obstruction

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61
Q

What is microcolon associated with?

A

Maternal diabetes*
Maternal hypothyroidism
Maternal magnesium exposure

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62
Q

Symptoms of microcolon

A

Abdominal distention

Failure to pass meconium

63
Q

What does microcolon look like on x-ray?

A

Multiple dilated loops of bowel

64
Q

What does microcolon look like on a barium enema?

A

Small colon segment with dilated bowel proximal, rectum is normal

65
Q

Epidemiology of Hirschsprung’s

A

If one child has Hirschsprung’s = 3-5% risk to next child

80% male

66
Q

What is Hirschsprung’s associated with?

A 
T 21
Heterochromia
Cartilage-hair hypoplasia
Goldberg-Shprintzen syndrome
Hirschsprung’s disease with limb anomalies
MEN 2
Waardenburg syndrome
Congenital deafness
13q deletion
Pheochromocytoma
Neurofibromatosis
Neuroblastoma
Congenital hypoventilation syndrome
Riley-Day syndrome
67
Q

Etiology of Hirschsprung’s

A

Failure of cranial to caudal migration of neural crest cells
8-10 weeks gestation
Aganglionic = no parasympathetic innervation to distal colon

68
Q

Symptoms of Hirschsprung’s

A

No meconium in first 24 hours
Abdominal distention, failure to thrive
Constipation or foul smelling liquid stool seepage
Can have urinary obstruction due to large colon
Can be complicated by acute bacterial enterocolitis (25-30% mortality rate)

69
Q

What does Hirschsprung’s look like on x-ray?

A

Large colon with absence of air in rectum

70
Q

What does Hirschsprung’s look like on barium enema?

A

Assesses for transition zone - Area between end of normal proximal zone and beginning of abnormal distal zone

71
Q

Definitive diagnosis of Hirschsprung’s

A

Biopsy to detect the absence or presence of ganglion cells with acetylcholinesterase staining

72
Q

Treatment of Hirschsprung’s

A

Diverting colostomy
Definitive treatment at 1-1.5 years
Final repair is a pull through

73
Q

Meconium plug

A

Colonic obstruction 2/2 a viscous, congealed mass of meconium

Associated with CF, maternal diabetes, and prematurity

74
Q

Symptoms of meconium plug

A

Abdominal distension
No meconium in first two days
Can have bilious vomiting

75
Q

What does a meconium plug look like on x-ray?

A

Multiple dilated loops of bowel

No rectal gas

76
Q

What does a meconium plug look like on a barium enema?

A

Empty distal colon
Dilated proximal bowel
Filling defects caused by plugs

77
Q

Treatment of meconium plugs

A

Abdominal decompression
Contrast enemas can be therapeutic
Consider Hirschsprung’s if no improvement after repeated enemas

78
Q

Epidemiology of imperforate anus

A

Includes wide spectrum of anorectal abnormalities

Usually no familial pattern

79
Q

Etiology of imperforate anus

A

Anorectum derives from cloaca

Failure of normal separation and division of cloaca into anterior urogenital sinus and posterior intestinal canal

80
Q

Symptoms of imperforate anus

A

High and intermediate lesions often without an anal opening
If rectal atresia anal opening will appear normal
Rocker bottom perineum suggest sacral agenesis
If the lesion is high 10% have a tethered cord

81
Q

Etiology of gastroschisis

A

Controversial, several mechanisms proposed

  1. Involution of right umbilical vein creates a weak spot
  2. Teratogenic exposures might be vasoconstrictive agents
  3. Genetic influences
82
Q

Prenatal findings with gastroschisis

A

Elevated AFP, oligohydramnios, fetal growth restriction, MSAF
If intestinal atresia is present may develop polyhydramnios
Dilated loops of bowel correlate with bowel edema, longer repair time, higher rate of post operative complications

83
Q

Defects associated with gastroschisis

A

All infants have malrotation
16% have other G.I. anomalies
Chromosomal or non-G.I. anomalies are very rare


84
Q

Management of gastroschisis

A

Cover bowel loops with a plastic wrap

Silo placement or immediate surgical repair

85
Q

Prognosis of gastroschisis

A

Survival 90%
Most have good long-term health and growth
Prematurity, low birthweight, stage silo repair, intestinal atresia -> all a/w longer time to full feedings and prolonged hospital stays

86
Q

Epidemiology of omphalocele

A

3:1 male to female

Higher incidence of prematurity and FGR

87
Q

Defects associated with omphalocele

A 
As many as 80%
Chromosomal 50% (T13,T18,T21)
Cardiac 28%
Genitourinary 20%
Craniofacial 20%
88
Q

Syndromes associated with omphalocele

A

Pentalogy of Cantrell
Beckwith-Wiedemann syndrome
OEIS complex (omphalocele, bladder exstrophy, imperforate anus, spinal deformity)

89
Q

Etiology of omphalocele

A

Intestinal loops fail to return to abdominal cavity at 11 weeks
OR somatic folds failed to complete formation of abdominal wall at 18 weeks

90
Q

Prenatal findings of omphalocele

A

Elevated AFP
Can be seen in second trimester
Amniocentesis recommended to rule out chromosomal abnormalities

91
Q

Symptoms of omphalocele

A

Amniotic/peritoneum membrane protects intestinal loops
Covering sac may be ruptured
Abdominal wall musculature is normal
All have malrotation

92
Q

Prognosis of omphalocele

A

Mortality is high is 30-40%
Large defect, ruptured sac, low birthweight, additional congenital anomalies, early respiratory failure -> all effect survival

Survival rate 90% with isolated omphalocele

93
Q

Complications of omphalocele

A 
Decreased G.I. motility
Bowel obstruction
Perforated viscous
GE reflux
Sepsis
94
Q

Etiology of cholestasis

A

Increased direct fraction of bilirubin caused by abnormal bilirubin excretion
Conjugated should be <15% of total bilirubin

95
Q

Symptoms of cholestasis

A

Conjugated hyperbili
Jaundice
Hepatomegaly
Acholic stools (2wks)

96
Q

Causes of intrahepatic cholestasis

A

Neonatal idiopathic hepatitis
Alagille syndrome
Non-syndromic paucity of bile ducts

97
Q

Causes of extrahepatic cholestasis

A 
Biliary atresia
Sclerosing cholangitis
Bile duct stenosis
Choledochal cyst
Bile plug syndrome
98
Q

What to etiologies cause 60-70% of cases of cholestasis?

A

Biliary atresia and idiopathic neonatal hepatitis

99
Q

Biliary atresia

A

Obstruction of biliary tree 2/2 progressive sclerosis of extrahepatic bile ducts (possibly after viral infection)
On histology will see bile duct proliferation

100
Q

Idiopathic neonatal hepatitis

A

Inflammatory condition of neonatal liver

Histology: multinucleated giant cella

101
Q

PN associated liver disease

A

Inversely related to birthweight
Related to duration of PN use (>2 wks)
Initiate enteral feedings as soon as possible (stimulus for bile flow, gallbladder contraction, intestinal motility)

102
Q

What are choledochal cysts?

A

Congenital bile duct anomalies

Cystic dilations of biliary tree

103
Q

Symptoms of choledochal cysts

A

Jaundice
Acholic stools
Palpable mass in RUQ
Hepatomegaly

104
Q

Diagnosis of choledochal cysts

A

Ultrasound

105
Q

Management of choledochal cysts

A

Complete excision

Construction of biliary-enteric anastomosis

106
Q

Causes of term NEC

A

Cocaine
Congenital heart disease
Things that decrease perfusion to the gut

107
Q

Most common location of NEC?

A

Terminal ileum and proximal colon

108
Q

Timing of NEC

A

PMA 31 to 32 weeks, preterm infants can get it later compared to full-term

109
Q

Pathophysiology of NEC

A 
Prematurity
Formula
Abnormal bacterial colonization
Reduced commensal organisms
Final pathway to injury seems to be activation of inflammatory cascade
110
Q

Esophageal sphincter tone

A

Preterm 4mmHg

Term 18mmHg

111
Q

Gastroesophageal reflux

A

Physiological event

Gastric contents move into the esophagus

112
Q

Gastroesophageal reflux disease

A

Pathologic state, complications arise from GER
Pain
Mucosal damage
Potential for aspiration

113
Q

Risk factors for GERD

A

Prematurity
Caffeine
Sepsis
Neurologic impairment

114
Q

Which comes first - apnea or reflux?

A

Apnea preceeds reflux more often

Desaturation with leads to relaxation of sphincter

115
Q

Histamine antagonists

A

Reduce gastric acidity
Does not stop reflux
Increased levels of NEC

116
Q

PPIs

A

Reduce acidity and reflux episodes
No improvement in clinical symptoms
No evidence for efficacy or safety in neonates

117
Q

Prokinetics

A

Erythromycin - increased risk of pyloric stenosis, sepsis, cardiac dysrhythmias

Metoclopramide - Tardive dyskinesia, extrapyramidal symptoms, bradykinesia, tremor, rigidity

118
Q

When is the esophageal lumen reestablished?

A

10 weeks

119
Q

When is swallowing seen in utero?

A

16 weeks

120
Q

When does nonnutritive sucking appear?

A

20 weeks

121
Q

When does nutritive sucking appear?

A

32-34 weeks

122
Q

When does stomach start secreting things?

A 
16 weeks
Hydrochloric acid
Intrinsic factor
Pepcid
Gastrin
Mucus
123
Q

What does caudal portion of foregut give rise to?

A

Upper duodenum

124
Q

What does midgut give rise to?

A 
Lower duodenum
Jejunum
Ileum
Appendix
Ascending colon
Proximal 2/3 of transverse colon
125
Q

What does hindgut give rise to?

A 
1/3 of transverse colon
Descending colon
Sigmoid colon
Rectum
Superior anal canal
126
Q

When does midgut loop herniate through umbilical ring?

A

Six weeks

127
Q

Esophageal duplication cysts

A

Can lead to esophageal obstruction

Can present with respiratory or digestive issues

128
Q

Neonatal intussusception

A

Very rare, often confused with NEC
Present with abdominal distention, feeding intolerance, vomiting, bloody stools
Diagnosis with Ultrasound

129
Q

Congenital sucrase-isomaltase deficiency

A

Chromosome 3
Appears at 3-6 months - introduced to foods containing sucrose
Severe watery diarrhea, acidosis, abdominal extension, cramping, FTT
Stool pH <7
Reducing substances negative

130
Q

Congenital lactase deficiency

A

Very rare
Autosomal recessive, most in Finland
Seen when starting to feed
Severe watery diarrhea, vomiting, FTT, lactosuria, aminoaciduria, lethargy
Endoscopy with biopsy for disaccharidase activity

131
Q

Maltase-glucoamylase deficiency

A

Very rare

Presents with diarrhea and distention after starch introduced to diet

132
Q

Glucose-galactose malabsorption

A

Very rare, autosomal recessive Chromosome 22
Defect in SGLT protein
Severe watery diarrhea when infant is fed breastmilk or glucose containing formula
Stools positive for reducing substances

133
Q

Hypobetalipoproteinemia and abetalipoproteinemia

A

Autosomal dominant
Low or absent apolipoprotein B and LDL cholesterol
Fat malabsorption, acanthocytosis, retinitis pigmentosa, neuromuscular degeneration

134
Q

Congenital chloride diarrhea genetics

A

Autosomal recessive

Defect in brush border Cl/HCO3 exchange

135
Q

Electrolyte abnormalities in congenital chloride diarrhea

A

Hyponatremia
hypokalemia
hypochloremia
metabolic alkalosis

136
Q

Congenital sodium diarrhea genetics

A

Autosomal recessive

Defect in Na/H exchanger in jejunal brush border

137
Q

Electrolyte abnormalities in congenital sodium diarrhea

A

Hyponatremia
Hypokalemia
Metabolic acidosis

138
Q

Neonatal hemachromatosis diagnosis

A

Biopsy parotid gland

139
Q

Gastric pH in neonates is higher or lower than adults?

A

Higher
More alkalotic


140
Q

Symptoms of congenital chloride diarrhea

A

Severe watery diarrhea high in chloride immediately after birth
Persist when NPO

141
Q

Symptoms of congenital sodium diarrhea

A

High sodium content in severe watery diarrhea immediately after birth

142
Q

Neonatal hemachromatosis

A

Alloimmune process
Can lead to IUFD
Liver failure at birth

143
Q

Treatment of neonatal hemachromatosis

A

IVIG
Exchange transfusion
Liver transplant

144
Q

Recurrence risk of neonatal hemachromatosis

A

Up to 90%

145
Q

Symptoms of neonatal hemachromatosis/GALD

A 
Refractory hypoglycemia
Severe coagulopathy
Hypoalbuminemia
Elevated ferritin >1000
LFTs near normal
Extrahepatic iron deposition
Elevated AFP >80,000
Elevated transferrin saturation
146
Q

What is another, newer term for neonatal hemachromatosis?

A

Gestational alloimmune liver disease (GALD)

This is to distinguish it from juvenile hemachromatosis which is a genetic disease

147
Q

Secretory IgA plays a role in ___

A

Immunity

Uptake of proteins in GI system

148
Q

How many carbons do short chain fatty acids have?

A

<6

149
Q

How many carbons do medium chain fatty acids have?

A

6-12

150
Q

What are Fatty acids with double bonds known as?

A

Unsaturated

151
Q

What are fatty acids without double bonds known as?

A

Saturated

152
Q

How many carbons do long chain fatty acids have?

A

13-21

153
Q

How many carbons do very long chain fatty acids have?

A

> 22

Neo Board Review MAP (18 decks)

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