GI/Bili

Question 1

What is Liley curve

Answer 1

measures amniotic fluid concentration of bilirubin by means of spectrophotometry.

Question 2

What is the most common condition requiring surgery in infancy

Answer 2

pyloric stenosis

Associated with T21

Question 3

Possible cause of breastmilk jaundice

Answer 3

• Mutations in the UGT1A1 gene
• High levels of beta-glucuronidase.

consider other non-hemolytic causes of prolonged hyperbilirubinemia, need not investigate for them unless jaundice does not resolve by 12 weeks of age

Question 4

Factors that worsen normal physiologic jaundice (5)

Answer 4

1. prematurity
2. sequestered blood
3. delayed establishment of feedings
4. maternal meds
5. DDC

Question 5

Duration of breastmilk jaundice

Answer 5

normalize over 4-12 weeks

can reach 20-30 mg/dl by 2 weeks

Question 6

Causes of pathologic jaundice

Onset before 24 hrs, inc >0.5 mg/dl/hr (>5mg/dl/day), Jaundice > 8 day for term 14 days preterm, DB >1mg/dl or >20% of TB

Answer 6

1. Increased production
   * hemolytic
   * enzyme def- G6PD, PK
   * membrane defect
2. decreased clearance: gilbert
3. impaired conjugation: crigler-najjar
4. increased enterohepatic circulation:breastmilk and breastfeeding jaundice

Question 7

Long term consequences of kernicterus

Answer 7

• extrapyramidal (choreoathetosis)
• Sensorineural hearing loss
• gaze palsies
• dental dysplasia

greatest risk with rapid rate of increase

Question 8

Phototherapy depends on (4)

Answer 8

1. spectrum of light- blue (460-490 nm)
2. irridiation- 30uW/cm2
3. surface exposed
4. distance of infant from light- 12-16 in/30-40 cm

photoherapy work by structural isomerization- irreversible

Question 9

It significantly increases surface area of the small intestine

Answer 9

Villi and microvilli

covered by columnar epithelial cells at tips- absorbtive cells, crypts- secretory

Question 10

Part of the bowel that mainly absorbs calcium and iron

Answer 10

Duodenum

• 80-100% absorb calcium via active transport
• Also initiates digestion
• production of GI hormones

Question 11

Parts of the bowel that absorbs calcium

Answer 11

1. Duodenum via active transport
2. Jejunum via concentration gradient

Question 12

What are the location of nutrient absorption in the bowel

Answer 12

Question 13

Specialized function of the distal ileum

Answer 13

Absorption of:
1. Vit B12 (needs intrinsic factor)
2. Zinc
3. Bile acid

Bile salts: not reabsorbed can cause diarrhea and impair Na and H2O

Question 14

what GI hormone cause delayed in gastric emptying

Answer 14

glucagon peptide I and petide YY

stimulated by lipids in the ileum

longer transit time, nutrient absorption in small intestine

Question 15

Function of the ileocecal valve

Answer 15

1. regulates fluids, electrolytes, nutrient
2. Prevents reflux of colonic material incl bacteria

Question 16

Greatest absorption of water and sodium in the GI tract

Answer 16

Colon

tightest intrercellular junction and slowest transt time

Question 17

morbidities/ complication of short bowel syndrome

Answer 17

1. catheter related blood stream infection- E coli and enterococci
2. Cholestasis
3. Growth failure
4. Small bowel bacterial overgrowth (SBBO)

CLABSI- d/t leaky gut mucosal barrier

Question 18

Strategies to reverse cholestasis

Answer 18

1. lipid reduction (1mg/kg 1-3/wk)
2. lipid modification (SMOF/omegaven)
3. advancement enteral feeds

Lipid supplement cannot be stopped d/t risk essential FA def

Question 19

Antibiotic therapy for SBBO

Answer 19

Metronidazole
Ciprofloxacin

Question 20

Red flags for cholestasis (3)

Answer 20

1. jaundice >2 weeks
2. hepatomegaly
3. pale stools/diarrhea

Obtain fractionated bilirubin

Question 21

Identifiable causes of cholestasis

Answer 21

• from most common
  1. biliary atresia
  2. genetic/metabolic: A1AT, alagille synd, CF
  3. Idiopathic/transient neonatal cholestasis
  4. PNAC

Question 22

Direct bilirubin level assoc with cholestasis

Answer 22

• first 5 days: >0.3-0.4 mg/dl
• 10% of TB

Question 23

Differential for GGT >150 U/L

Answer 23

1. Biliary atresia
2. choledochal cyst
3. A1AT def
4. CF

Question 24

Differential for GGT <125 U/L

Answer 24

1. PFIC (progressive familial intrahepatic cholestasis)
2. Inborn errors of bile acid synthesis
3. panhypopit

Question 25

Ideal timing for kasia procedure

Answer 25

Done before 30-45 days | chances for post-op bile flow, avoid/delay liver transplant

Question 26

Evaluation for cholestasis

Answer 26

Question 27

Most frequent indication for pediatric liver transplant

Answer 27

Biliary Atresia

Question 28

* It is an autosomal recessive d/o associated with cholestasis * Have hepatocyte retention of polymers mutant Z protein * also at risk for early emphysema

Answer 28

A1AT deficiency

Question 29

* It is an autosomal dominant d/o associated with cholestasis * Mutation with Jagged1 gene * Clinical features: * bile duct paucity * cong heart dis * dysmorphic facies- triangular face, broad forehead, deep set eyes, sm pointed chin * ocular post embryotoxon * butterfly vertebrae * renal anomalies

Answer 29

Alagille syndrome

Question 30

What is the hallmark of clinical surgical abdominal emergencies

Answer 30

Bilious emesis

Question 31

What is the most common site of gastric perforation

Answer 31

Greater curvature | Abd XR: pneumoperitoneum, NG in the pelvis ## Footnote Sx Acute abdomen, lethargy, apnea, RDS, bloody output from NG Mx: Exlap, gastrostomy

Question 32

It is due to lack of recanalization of duodenum

Answer 32

Duodenal atresia ## Footnote - normally happens 8-10 weeks GA - Dx: Abd XR: double bubble (distended stomach and prox duodenum); +/- bilious output - Mx: gastric decompression, TPN, ECHO - Repair not an emergency

Question 33

It is the most severe complicaion of intestinal malrotation

Answer 33

Midgut volvulus ## Footnote - Incidence highest first 2 months - Sx: feeding intolerance and bilious emesis, bloody stools, late abd distension - pathophysiology: obstruction and vascular compromise

Question 34

What is the normal location of: a. duodenojejunal junction b. ilocecal valve

Answer 34

a. Left upper quadrant b. right lower quadrant | In malrotaton: duodenojejunal: midabdomen, ileocecal valve: RUQ

Question 35

Gold standard Dx for malrotation/ volvulus

Answer 35

UGI (corkscrew sign) ## Footnote Ultrasound: whirlpool sign by superior mesenteric vessels

Question 36

What are the steps of the Ladd procedure

Answer 36

1. Devolvulizing the midgut 2. Adhesiolysis- separate duodenaljejunal junc and ileocecal 3. Verticalizing the duodenum 4. Performing appendectomy 5. Replacement of the small bowel on the right hemi-abdomen and the colon on the left hemi-abdomen ## Footnote done when no sigificant ischemia

Question 37

What is pathology in small bowel atresia

Answer 37

Vascular occlusion * can involve mesentery based on extent

Question 38

True or false: Passage of meconium rules out small bowel atresia

Answer 38

False * Its commonly seen symptom failure to pass meconium ## Footnote Factor associated with inc M&M prematurity, other malformation, multiple atresia (can lead to short bowel)

Question 39

What is the common site for spontaneous perforation (SIP)

Answer 39

terminal ileum | Dx: Abd XR pneumoperitoneum w/o NEC ## Footnote No reason, no vascular compromise More common in males risk factor: perinatal use and postnatal indomethacin, preeclampsia

Question 40

Goal mgt of SIP

Answer 40

for <1kg: Percutaneous drain at bedside to: 1. release pressure- eliminate hypoperfusion 2. outlet spilled intestinal content For >1kg: Laparotomy (Primary closure vs temp ostomy) ## Footnote Long term consequence: enterocutaneous fistula, stricture

Question 41

True or false: NEC exclusively occurs in preterm infants

Answer 41

False * 5% of preterm<30 wks * 10% of term (from hypoperfusion- CHD, sepsis) ## Footnote findings: intestinal ischemia leading to damage to intestinal mucosa, bacterial invasion, sepsis - poor mucin production overpermeability of intercellular junction - risk factors: H2 blockers, nonhuman milk, hyperosomolar meds/formula

Question 42

NEC staging and mgt

Answer 42

Question 43

What are the parameters to determine need for exploration (SIP/NEC)

Answer 43

1. worsening acidosis 2. inc need cario-resp support 3. worsening coagulopathy 4. inc erythema/ bluish discoloration ## Footnote Rationale: ischemic (not necrotic) intestinal tissue keeps the patient in a vicious circle

Question 44

Signs of esophageal atresia

Answer 44

early postnatal period: * difficult feeding * excessive oral secretions * cyanosis/ apnea during feeds ## Footnote Prefer Dx test: flexible esophagoscopy/ bronchoscopy UGIS after surgical repair- leak and stricture

Question 45

Ultrasound finding for hypertrophic pyloric stenosis

Answer 45

Thickness: 3mm Length: 15 mm

Question 46

What are the functions of the GI tract

Answer 46

Question 47

What is the GI cellular origin and function | function at maturity

Answer 47

## Footnote Endoderm: digestion and absorption **M**esoderm: **m**uscle **E**ctoderm: **e**nteric nervous system-Auerbach plexus is between muscle layers, **M**eissner plexus adjacent **m**ucosal layer.

Question 48

What are the sections of the GI tract embryologically

Answer 48

## Footnote common sources of blood supply, innervation, and lymphatic drainage with those derived from the same precursor Foregut: celiac artery Midgut: superior mesentery artery Hindgut: inferior mesentery artery

Question 49

What are the ultrasound findings of biliary atresia

Answer 49

* sclerosed bile ducts * absence of a gallbladder * "triangular cord" sign

Question 50

Findings in chylous ascites

Answer 50

* milky fluid * elevated triglyceride * cell count with lymphocyte predominance * low glucose.

Question 51

Predictors of outcome of CDH

Answer 51

1.Degree of pulmonary hypoplasia * Lung to head ratio * Observed vs expected total lung volume 2. Pulmonary hypertension 3. involvement of the liver 4. Prematurity

Question 52

Delivery room mgt of CDH

Answer 52

1. place a NGT soon after delivery for decompression of the stomach and intestines 2. prompt intubation for respiratory distress ## Footnote Goals in vent setting: PIP<25, preductal sats >70%

Question 53

What are the fetal predictors of outcome of CDH

Answer 53

1. Associated anomalies 2. Extent of lung hypoplasia 3. Position of the liver- liver up worst prognosis, highly predictive of ECMO ## Footnote LHR and survival * >1.35- 100% * 1.35-0.6 - 61% * <0.6- no survival

Question 54

long term complications of CDH

Answer 54

1. sev pulm hypoplasia 2. pulm htn 3. chronic lung disease 4. neuro cog delay 5. hearing loss- can be late 6. chest wall deformity 7. scoliosis 8. poor growth 9. hernia reoccurence

Question 55

What is the most common primary hepatic malignancy in childhood

Answer 55

Hepatoblastoma ## Footnote * usually isolated, but may be associated with Beckwith-Wiedemann syndrome, isolated hemihyperplasia, familial adenomatous polyposis coli, or trisomies. * Treatment with initial surgical resection versus neoadjuvant chemotherapy is determined by risk stratification based on extent, location, and histology.

Question 56

What is the serum marker for hepatoblastoma

Answer 56

Serum α1-fetoprotein (AFP) can be a useful marker for response to treatment ## Footnote useful marker for response to treatment