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Neonatal-Perinatal Boards Review > Gastrointestinal/Nutrition > Flashcards

Gastrointestinal/Nutrition Flashcards

1
Q

Prenatal lab finding in gastroschisis

A

Elevated maternal serum AFP or amniotic AFP (alpha-fetoprotein)

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2
Q

Classic presentation of pyloric stenosis (age, symptoms and signs)

A

1 month, non-bilious projectile emesis, hypochloremic/hypokalemic metabolic alkalosis and “olive” epigastric mass

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3
Q

Components of a cloaca

A

Urinary, vaginal, rectal fusion

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4
Q

Muscularization of esophagus (which parts are what type)

A

Upper: striated

Middle: striated and smooth

Lower: smooth

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5
Q

Cause of distal (jejunoileal) atresias

A

Vascular disruption leading to ischemic areas that underdevelop

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6
Q

Common prenatal and postnatal findings in TEF

A

Prenatal: polyhydramnios

Postnatal: pooling oral secretions, inability to pass NG (but presence of distal bowel gas)

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7
Q

Sequelae of 1) significant ileal resection and 2) loss of terminal ileum

A

1) Fat malabsorption due to severely decreased bile salt recycling (almost all hepatoenteric circulation is in ileum)
2) “Short gut” from faster gastric emptying/intestinal motility

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8
Q

Protein that, if deficient/absent, leads to fat malabsorption

A

Betalipoprotein

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9
Q

What is an epignathus?

A

Teratoma of upper palate or jaw, benign but can cause obstruction

Remember -gnathus/-gnathia has to do with the jaw

Treatment is excision

(sciencedirect.com)

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10
Q

The midgut loops in the ______ direction around the _____

A

Counter-clockwise

Superior mesenteric artery

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11
Q

Main cause of carbohydrate malabsorption

A

Congenital sucrase-isomaltase deficiency (CSID), an enzyme that breaks down those disaccharides in the small intestine

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12
Q

Defect that causes positive stool reducing substances that’s associated with inability for blood glucose to respond to normal feeds and/or dextrose gel

A

Sodium-glucose linked transport protein (SGLT) defect

Transport protein defect leading to carbohydrate malabsorption very rare (more commonly enzyme-related)

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13
Q

Laboratory finding (that’s non-diagnostic) in milk protein allergy

A

High cow’s milk IgE, relates to it being an immunologic hypersensitivity (does usually resolve by school age)

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14
Q

Histologic findings on rectal biospy in Hirschsprungs (2)

A

1) Aganglionosis in the myenteric and submucosal plexuses
2) Increased acetylcholinesterase (AChE) within hypertrophic cholinergic nerve fibers

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15
Q

Components of GALT (gut-associated lymphoid tissue) and their functions

A

1) Peyer patches—sites of T and B cell activity
2) Lamina propia plasma cells—make IgA (neutralize bacteria)

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16
Q

Gastroschisis is almost always on the ______ side of the umbilical cord and is associated with ______

A

Right

Rarely associated with anything, but IF it is it’s almost always another GI anomaly (like atresia)

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17
Q

What is a congenital epulis?

A

Benign granular cell tumor, comes from gingival or alveolar mucosa

(sciencedirect.com)

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18
Q

What is deficient in a patient with watery/foul stools, retinitis pigmentosa, and neuromuscular degeneration?

ncbi.nlm.nih.gov

A

Apolipoprotein beta (leading to fat malabsorption)

Rarely can be Anderson disease (lipoprotein assembly defect)

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19
Q

Primary job of colon

A

Water and electrolyte reabsorption

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20
Q

Concomitant allergy with MPA (milk protein allergy)

A

About 50% also allergic to soy

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21
Q

What causes proximal GI atresias

A

Failure of recanalization of the primitive gut tube around 10 weeks gestation

(Remember TEFs are around 4 weeks when 2 tubes separate, then 8-10 weeks is when tubes recanalize)

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22
Q

Main risk factors for small left colon/meconium plug syndrome

A

Maternal diabetes

Prematurity

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23
Q

Normal lower esophageal sphincter tone in neonate vs. 6-month-old

A

2mm Hg vs. 10-15mm Hg

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24
Q

Which abdominal wall defect is affiliated with other congenital anomalies [more frequently]?

A

Omphalocele

VACTERL, Trisomies, and Beckwith-Wiedemann all associated and can involved kidneys and heart

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25
Q

Secondary carbohydrate enzyme deficiency seen with CSID (congenital sucrase-isomaltase deficiency)

A

Maltase-glucoamylase deficiency (MGA), because they are homologous enzymes

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26
Q

What provides stimulation of the fundus and proximal stomach

A

Vagus nerve

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27
Q

Esophageal duplication cysts are diagnosed by _____, but require ____ to confirm they’re not ______ that would communicate with CNS

A

CT

MRI

neurenteric cyst

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28
Q

Chromosome that most syndromes involving pancreatic insufficiency trace back to

A

7

Schwachman-Diamond involves mutations of 7q11 and Cystic Fibrosis of CFTR on 7

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29
Q

The other GI organs form from the ____ of the primitive gut tube

A

Endoderm (pancreas, liver)

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30
Q

Two syndromes frequently associated with TEF

A

VACTERL (vertebral anomalies, anorectal anomalies, cardiac defects, tracheoesophageal defects, renal, limb anomalies)

CHARGE (coloboma, heart defect, atresia choanae, restricted growth, genital, ear anomalies)

70% of TEFs will have at least one other anomaly

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31
Q

Most common type of tracheoesophageal fistula

A

Type C: proximal esophageal atresia with a distal fistula

(sciencedirect.com)

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32
Q

Embryonic/cellular cause of Hirschsprung’s

A

Vagal neural crest cells don’t migrate correctly to anus +/- rectum, leads to aganglionic segment

The earlier they stop [migrating], the longer the segment

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33
Q

Frequency of milk protein allergy and percentage that can later tolerate cow’s milk by ages 2 and 6.

A

5% in neonatal period (only 0.5% are breastfed)

30% can tolerate cow’s milk by age 2, 80% by age 6

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34
Q

Longterm risk if a choledochal cyst is not removed

A

Malignancy

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35
Q

Gender predominance of:

  1. Gastroschisis
  2. Choledochal cyst
  3. Imperforate anus
A
  1. NONE (omphalocele, however, has male predominance)
  2. Female
  3. Male
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36
Q

Alagille syndrome:

  1. Pathology
  2. Mutations
  3. Inheritance pattern
A
  1. Paucity of INTRAhepatic/intralobular bile ducts
  2. JAG1 and NOTCH2
  3. Autosomal dominant (less common than biliary atresia)
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37
Q

Frequent findings in Alagille syndrome

A

Renal

Cardiac (Tetralogy of Fallot and peripheral pulmonic stenosis [PPS])

Butterfly vertebrae

Triangular “shield” facies and embryotoxon (“iris strands”, faint stranding around circumference)

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38
Q

Best timeframe to perform Kasai in biliary atresia

A

First two months

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39
Q

Ultrasound findings suggestive of biliary atresia

A

Absent gallbladder (presence of one does NOT rule out BA)

“Triangular cord sign”, a fibrous remnant of extrahepatic bile duct anterior to portal vein

(radiopaedia.com)

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40
Q

Laboratory findings in alpha-1 antitrypsinogen deficiency (A1AT, AATD, A1AD)–a serine protease inhibitor made in hepatocytes

A

Cholestasis

High GGT

Low A1AT

A1AT deficiency leads to COPD/pulmonary disease in adults

41
Q

What endocrine disorders commonly present at cholestasis?

A

Hypothyroidism

Panhypopituarism

42
Q
  1. Enzyme responsible for conversion of heme to biliverdin
  2. Enzyme responsible for conversion of biliverdin to unconjugated bilirubin
A
  1. Heme oxygenase
  2. Bilirubin reductase
43
Q
  1. Location of conversion of heme to biliverdin and then unconjugated bilirubin
  2. Primary binder of bilirubin in circulation
  3. Primary carrier of bilirubin from plasma into hepatocytes
A
  1. Hepatocytes
  2. Albumin
  3. Ligandin
44
Q

List three reasons that bilirubin accumulates more in newborns

A
  1. Increased RBC turnover (average life span 60d)
  2. Decreased UDP-glucuronyl transferase (responsible for conjugation)
  3. Increased enterohepatic circulation (so its reabsorbed from intestine more)
45
Q

Areas of brain most affected by kernicterus

A
  1. Basal ganglia (especially globus pallidus)
  2. Hippocampus
  3. Cranial nerve nuclei

(These are the reasons that choreoathetosis/cerebral palsy, upward gaze palsy, and deafness are sequelae)

46
Q

Enzyme responsible for conversion to conjugated bilirubin

A

Uridine diphosphate-glucuronyl transferase (UDP-glucuronyl transferase) in hepatocytes

(Conjugated is water-soluble, then excreted in bile and secreted into intestines)

47
Q

What is the neurotoxic form of bilirubin?

A

Unbound circulating unconjugated

(Vast majority bound to albumin, which cannot cross blood-brain barrier–this is the reason albumin:bilirubin ratio is important)

48
Q

Extraneural manifestations of severe bilirubin toxicity

A

Renal, intestinal mucosa, and pancreatic necrosis

49
Q

Non-intrinsic risk factors that increase bilirubin toxicity (AKA other pathologies/abnormalities)

A

Hypoxia

Acidosis

Hyperosmolarity (hypernatremia, dehydration)

Asphyxia

50
Q

Complications of double-volume exchange transfusion

A

Portal vein and renal vein thrombosis

Necrotizing enterocolitis

Thrombocytopenia

Hypocalcemia (due to binding from citrate anticoagulant in pRBCs)

51
Q

Which presents earlier, breastmilk or breastfeeding jaundice?

A

Breastfeeding (the pathologic one); breastmilk jaundice is more 1-2 week timeframe

(Most commonly cited cause for breastmilk jaundice is increased beta-glucuronidase which deconjugates intestinal bilirubin, increasing reuptake)

52
Q

What are some evidence-based iatrogenic risk factors for ELBW infants to develop necrotizing enterocolitis (NEC)?

A

Prolonged antibiotic exposure, in the study defined as 5 or more days

Formula

(Rapid feed advancement is believed to be a risk, but not defined or fully proven)

53
Q

What are the normal sodium and potassium requirements for preterm neonates?

A

Sodium: 3-7 mEq/kg/day

Potassium: 2-5 mEq/kg/day

54
Q

Which of the three nutrient groups has predominantly normal enzyme levels at birth?

A

Carbohydrates: Glucosidases are normal level by term

For preemies lactase is deficient but contained in breastmilk

55
Q

What organ contributes to the second step in protein digestion

A

The pancreas: trypsin and chymotrypsin, which are severely deficient in preterm (and even term only have 25% of adult activity)

Breastmilk has these (and others) to make up for low levels

56
Q

What are the first two steps in fat digestion and what organs help

A

Bile salts (liver) form micelles

Pancreatic lipase hydrolyzes them into absorptive pieces

Both decreased in preemies but lingual/gastric and beastmilk lipase help

57
Q

Which types of fatty acids do NOT require bile salts

A

Short- and medium-chain

This is why patients with cholestasis/liver disease do best with MCT supplementation

58
Q

Why does it matter that glucose and galactose are absorbed via active transport (from intestine)

A

This is inefficient the earlier in gestation, one of many reasons for hypoglycemia

59
Q

What transporter do all monosaccharides go through to enter circulation

A

GLUT2 transporter

60
Q

Of the two–digestion and absorption–which one tends to be less efficient in newborns

A

Digestion

Enzymes tend to be at low levels but transporters normal (exception is the active transport of glucose/galactose)

61
Q

Beckwith-Wiedemann is associated with what two GI anomalies

A

Omphalocele

Malrotation

62
Q

___% of patients with meconium ileus have _______

A

90% of patients with meconeum ileus have Cystic Fibrosis

(It’s usually obstructive at the terminal ileum)

63
Q

What should you think of for an otherwise well baby who develops acute enterocolitis (foul-smelling bloody stools, NEC-like picture)

A

Hirschsprung’s disease

Rare but if occurs has around same mortality rate as preemie NEC (30%)

No _P_arasympathetic innervation to _P_oop (most are only rectosigmoid)

64
Q

Week of gestation of 1) TEF and 2) Hirschsprung’s

A

TEF: Four (embryonic stage for lungs, two tubes don’t separate correctly)

Hirschsprung’s: Eight

65
Q

If you see pyloric stenosis on an XR, what names is the sign given?

A

String sign or apple core (from the contrast leaking through I highly narrowed segment)

66
Q

Omphalocele and gastroschisis associations

A

Omphalocele: Beckwith-Wiedemann, Trisomies, OEIS

Gastroschisis: almost n**one

67
Q

Name the most common intra- and extra-hepatic causes of cholestasis

A

Intra: idiopathic neonatal cholestasis

Extra: biliary atresia

68
Q

Consider what diagnosis in a jaundiced infant with acholic stools and a palpable mass near the RUQ

A

Choledochal cyst

(EXTRA hepatic cause, most easily diagnosed with ultrasound)

69
Q

Mutations associated with Allagille Syndrome

A

JAG1 or NOTCH2

70
Q

What is the estimated energy requirement to grow for an LBW neonate?

A

100-130 kcal/kg/day (90-120 is estimated “need” but higher-end for growth)

71
Q

How much of the energy (calories) is fat in human milk or formula

A

50%

Most common fatty acids are SOaP=Stearic, Oleic and Palmitic

72
Q

Which two fatty acids are essential in all neonates? Which two are hard for preemies to synthesize?

A

1) Linoleic and linolenic acid
2) docohexanoic acid (DHA) and arachidonic acid (ARA)

73
Q

What percentage of the energy in human milk are carbohydrates and protein

A

40% carbohydrates

10% protein

(fat is 50%)

74
Q

The predominant forms of fatty acids, carbohydrate, and protein in breastmilk

A

Fatty acids: Stearic, Oleic, and Palmitic (SOaP)

Carbohydrate: Lactose

Protein: Beta-casein (although for general groups, whey>casein)

75
Q

What is the main fetal energy source and how is it delivered

A

Maternal glucose by facilitated diffusion (passive, requires no ATP)

The other two sources are maternal lactate and amino acids

76
Q

Four amino acids essential in premature infants (per Brodsky)

A

CATT:

Cysteine

Arginine

Tyrosine

Taurine

77
Q

How do whey:casein change as milk production continues

A

In colostrum whey:casein is 80:20, then ends up being around 50:50 with beta-casein being the main protein form

78
Q

How many calories come from 1g of fat, carbohydrate and protein

A

20% IL: 9kcal/g

CHO: 3.4kcal/g

AA: 4kcal/g

These are listed in order of how much they contribute to ENTERAL nutrition compositin; in TPN CHO > FAs >> AA

79
Q

What amino acid is most important in fatty acid metabolism

A

Carnitine

This is why you add it in TPN cholestasis and why you supplement it in fatty acid oxidation defects

80
Q

Both vitamin ____ deficiencies and EFAD cause _____ as a main presenting symptom

A

Vitamins B (B2, B3, B6)

EFAD: essential fatty acid disorder from lack of linoleic and linolenic acid

Dermatitis

81
Q

Two main drugs/classes that cause bilirubin dissociation

A

Ceftriaxone

Sulfa

(Matters because they compete for albumin binding, and only unconjugated unbound albumin crosses the blood-brain barrier)

82
Q

Mechanism of phototherapy

A

Photoisomerization of unconjugated bilirubin to lumirubin, which is water-soluble and leaves in bile/urine

83
Q

Name three important proteins and enzymes in bilirubin metabolism

A

Albumin: binds unconjugated bilirubin to safely transport to liver

Ligandin: transport molecule from plasma into liver

UDP-glucuronosyltransferase (UDPGT): enzyme that conjugates bilirubin

(UDGPT only 1% of adult function at birth)

84
Q

Gilbert Syndrome facts

A

1) UGT1A1 mutation: decreased expression of UDPGT
2) less glucoronc acid conjugation means higher unconjugated bilirubin; usuallu just at times of stress

85
Q

Genetic syndrome that causes dysfunction of UDPGT

A

Crigler-Najjar

(Unconjugated hyperbilirubinemia beyond first 2 weeks of life, severe form requires liver transplant)

86
Q

As gestational age increases, what happens to 1) total body water, 2) extracellular water and 3) intracellular water

A

As gestational age increases, TBW and extracellular water decrease

Intracellular water increases (cells are hypertrophic at this phase, not increasing in number)

87
Q

Sequelae of Copper deficiency

A

Anemia

Leukopenia

Myeloneuropathy

88
Q

Sequelae of Selenium deficiency

A

Cardiomyopathy

89
Q

What is the respiratory consequence of giving excess carbohydrates

A

Excess CO2 production (ie increased Respiratory Quotient)

Keep ratio CHO:IL 60:40 in terms of calorie contribution (and maximum GIR 13)

90
Q

Why is it that carbohydrate excess increases the RQ (respiratory quotient)

A

Converting CHO to fat is energy inefficient, results in more CO2 byproduct

91
Q

What enzymes does the stomach produce to start protein digestion

A

Pepsin and gastrin

92
Q

How glucose is absorbed in the GI tract

A

Facilitated diffusion

Also true for glucose to cross placenta

93
Q

Name the deficiency: Scaling dermatitis, alopecia, thrombocytopenia, increased susceptibility to infections

A

Essential fatty acid disorder (EFAD) Presents very similarly to Biotin deficiency (especially kind of dermatitis) but thrombocytopenia helps tell them apart Occurs if getting <0.5g/kg/day of IL

94
Q

Name the deficiency: Photophobia, abnormal enamel formation, abnormal epiphyses

A

Vitamin A Also important for pulmonary epithelial growth (remember it’s proven to decrease BPD!)

95
Q

Name the deficiency: Megaloblastic macrocytic anemia, maternal vegetarian diet

A

Vitamin B12 (cobalamin) deficiency Supplement with methylmalonic acidemia

96
Q

Name the deficiency: Megaloblastic macrocytic anemia, failure to thrive, goat’s milk diet

A

Folate deficiency Remember to supplement in homocystinuria

97
Q

Name the deficiency: Osteopenia/Rickets, failure to thrive

A

Vitamin D Remember, vitamin D is NOT required for maternal transfer of Ca to fetus (increased intestinal absorption in her is completely independent of vit D!)

98
Q

In TPN-associated cholestasis, what two trace elements are decreased

A

Manganese and Copper

Neonatal-Perinatal Boards Review (11 decks)

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