Quiz 5 NEC/PDA/Pain/IEM

Question 1

What is NEC

Answer 1

Ischemic and inflammatory necrosis of the bowel

Question 2

NEC morbidity

Answer 2

short bowel syndrome, TPN related liver disease, poor growth, poor neurodevelopment outcome

Question 3

What % of NEC are term?

Answer 3

10%
gastroschisis 5%
CHD 3-7%

Question 4

Who is at greatest risk for NEC

Answer 4

<28 weeks

Question 5

Age of onset of NEC

Answer 5

28-33 weeks

Question 6

Spontaneous intestinal perforation

Answer 6

Occurs earlier during first 2 weeks, isolated areas of hemmorhagic necrosis with perforation in terminal ileum

Question 7

What is the main reason for NEC

Answer 7

immaturity of intestinal tract

Question 8

what is the function of the toll-like receptors?

Answer 8

pattern recognition; send out signals to immune system

Question 9

Preterm gut characteristics

Answer 9

dec GI motility; patchy mucous coating with loose junctions; decreased gastric acidity and altered circulatory regulation

Question 10

What causes bacterial proliferation?

Answer 10

enteral feeding

Question 11

What happens when bacteria adhere?

Answer 11

toll-like receptors are stimulated

Question 12

Stimulation of toll-like receptors results in:

Answer 12

local inflammation and release of inflammatory mediators (PAF and TNF)

Question 13

Inflammatory mediators result in:

Answer 13

inflammation, vasoconstriction, permeability

Question 14

pathognomonic sign for NEC

Answer 14

pneumatosis intestinalis

Question 15

Most frequent location for NEC

Answer 15

terminal ileum and ascending colon

Question 16

Where is the most likely site for perforation?

Answer 16

Ileocecal valve

Question 17

Risk factors for NEC

Answer 17

black and hispanic race, outworn, no standard feeding protocol, NPO, formula, hypertonic meds, jejunal feedings, abnormal bacterial colonization, blood transfusions

Question 18

Stage II NEC

Answer 18

proven NEC
abd distention, absent bowel sounds, mild metabolic acidosis, thrombocytopenia, cellulitis, pneumatosis intestinalis, intestinal dilation, ileum, ascites

Question 19

Stage III NEC

Answer 19

impending bowel perforation, hypotension, A/B’s, respiratory and metabolic acidosis, pneumoperitoneum

Question 20

Triad of late non-specific symptoms of NEC

Answer 20

thrombocytopenia, increased lactate (metabolic acidosis), hyponatremia

Question 21

Left lateral decubitus X-ray for NEC

Answer 21

best to detect pneumoperitoneum; place left side down so air rises over liver

Question 22

Portal venous gas

Answer 22

hydrogen gas has gotten into the portal blood system

Question 23

Sentinal loop

Answer 23

area that loop has gotten necrotic and nothing can pass, need serial X-rays to diagnose

Question 24

NEC sequelae

Answer 24

recurrent NEC, strictures, malabsorption, short bowel syndrome

Question 25

Short bowel syndrome outcome dependent on:

Answer 25

presence of ileocecal valve, residual length <10% for GA

Question 26

When does the infants bowel double in length?

Answer 26

23-25 weeks

Question 27

Short bowel syndrome and nutrition

Answer 27

early! Breastmilk or elecare, continuous

Question 28

Duodenum absorption:

Answer 28

iron, Ca, Mg, ADEK

Question 29

Jejunum absorption:

Answer 29

water soluble vitamins B and C, carbs, fats, and proteins

Question 30

Ileum absorption:

Answer 30

vit B and C, carbs, fats, proteins, B12 and bile acids

Question 31

Colon absorption:

Answer 31

short chain fatty acids from fermentation, water

Question 32

Dumping syndrome

Answer 32

80%; maintain normal Na and K for growth, monitor for acidosis, refeed osmotic output through mucous fistula

Question 33

Post op gastric acid hypersecretion

Answer 33

50%; can disrupt surgical site, peptic ulceration, inactivate pancreatic enzymes. May need a few days of pepcid in TPN

Question 34

Short bowel syndrome mortality primarily due to :

Answer 34

Parenteral nutrition sequelae. Length of time = central line sepsis, steatosis (fatty liver), cholestasis (direct bili>2)

Question 35

Cholestasis may be prevented with:

Answer 35

20-30% enteral nutrition, actigall, omegaven, cycling TPN

Question 36

Surgical procedures to improve short bowel outcome:

Answer 36

stricture resection, bowel tapering, bowel lengthening

Question 37

Procedures for lengthening bowel

Answer 37

bianchi and STEP procedure

Question 38

Preventative measures for NEC

Answer 38

breastmilk, standardized feeding protocols, holding feeds during blood transfusions, pre/probiotics

Question 39

Which immunoglobulin correlates with a decrease in bacterial translocation?

Answer 39

IgA (closes enterocyte gaps)

Question 40

What are inborn errors of metabolism?

Answer 40

disorders caused by deficiency of one or more enzymes involved in a biochemical pathway

Question 41

What are the consequences of an enzyme deficiency?

Answer 41

won't be able to break down that particular thing

Question 42

Acute inborn errors of metabolism:

Answer 42

metabolic disease of the newborn

Question 43

Progressive

Answer 43

organomegaly, developmental delay (develops slowly over several years)

Question 44

Chronic

Answer 44

Failure to thrive, mental retardation

Question 45

Late

Answer 45

first indication in adults

Question 46

What is an important red flag in diagnosing metabolic disease?

Answer 46

Developmental delay

Question 47

Labs for Fatty acid oxidation defect:

Answer 47

HYPOGLYCEMIA WITH ABSENT KETONES

Question 48

gluconeogenesis (fatty acid oxidation pathway)

Answer 48

takes fats and converts them to glucose

Question 49

why does fatty acid oxidation not get noticed till 3-5 months?

Answer 49

doesn't get to catabolic phase until feeds have lengthened when baby is sleeping through the night. presents with hypoglycemia

Question 50

Labs for Urea cycle defects:

Answer 50

``` High ammonia (300-2000 range). No acidosis or hypoglycemia normal ammonia (40-70) ```

Question 51

Test for urea cycle defects

Answer 51

plasma amino acids

Question 52

Labs for Organic acidemias

Answer 52

ACIDOSIS, HIGH ANION GAP, KETONES, HYPOGLYCEMIA, INCREASED AMMONIA

Question 53

Galactosemia

Answer 53

1/60,000 | must have lactose feeding prior to screening

Question 54

Galactosemia clinical signs

Answer 54

vomiting, diarrhea, jaundice, hepatomegaly, gram negative sepsis, liver dysfunction

Question 55

Galactosemia treatment

Answer 55

galactose/lactose restriction

Question 56

Phenylketonuria

Answer 56

1/15,000 | must have protein intake prior to screening

Question 57

Phenylketonuria clinical signs

Answer 57

mental retardation, autism, seizures, hypopigmentation, eczema

Question 58

Phenylketonuria treatment

Answer 58

low phenylalanine diet, tx for life

Question 59

Pinprick studies

Answer 59

1941, greater response as GA increases

Question 60

1985 first event

Answer 60

Jeffrey Lawson PDA ligation without anesthesia

Question 61

1985 second event

Answer 61

Dr. Anand in United Kingdom proved that infants do feel pain. Sufentanyl vs control

Question 62

Cutaneous sensory receptors

Answer 62

7 weeks - face 11 weeks - palms and soles 15 weeks - trunk and proximal arms and legs 20 weeks - cutaneous and mucous surfaces Synapses begin at 6 weeks, neurotransmission begins before 13-14 weeks, completes by 30 weeks

Question 63

C-polymodal fibers

Answer 63

unmyelinated; transfer pain even more than delta A fibers which are thinly myelinated

Question 64

Pain pathways to brain and hypothalamus are completely myelinated by when

Answer 64

30 weeks

Question 65

consequences of unmanaged pain

Answer 65

increased HR and BP, decreased O2 sat, dec GI motility, dec UO

Question 66

Repeated production of cortisol from the adrenal cortex does what to the immune system?

Answer 66

suppresses the immune system, production of ACTH and cortisol persist and increased risk of infection

Question 67

Secretion of human growth hormone and thyroid hormone

Answer 67

increases rate of protein synthesis in cells, mobilizes fatty acids from adipose tissue

Question 68

Hypoglycemia leads to secretion of what:

Answer 68

human growth hormone and thyroid hormone