GI

Question 1

You are treating a 5 year old boy for constipation. In addition to disimpaction, his mother asks you how long he will need to be treated with PEG for?

a. 3 months
b. 6 months
c. Until he’s toilet trained
d. 3 weeks

Answer 1

6 months

Question 2

47) 2 ½ year old boy in for routine check-up. Mom mentions he has had 2 episodes of rectal prolapse, reduced in the ER, in the last 6 months. You should:
a. reassure
b. treat for constipation
c. check for CF
—————
Girl with rectal prolapse x 2 reduced easily in the ER. What do you do?
a. reassure mom
b. observe and follow in 3 months
c. sweat chloride
d. barium enema

Answer 2

Check for CF, sweat chloride

Question 3

85. Baby with delayed meconium passage, abdo distension and calcifications on AXR
    a. Hirschsprung’s
    b. CF
    c. Duodenal atresia

Answer 3

CF

Intra-abdo calcification = meconium peritonitis

Meconium ileus

• 10% of CF have MI
• 90% of MI have CF
• Dx with contrast enema

Question 4

78. What is most useful intervention in treating encopresis?
    a. pharmacologic
    b. behavioural
    c. pharmacologic and behavioural
    d. biofeedback

Answer 4

Pharmacologic + behavioural

Encopresis = voluntary/involuntary passage of feces into inappropriate places at least 1X/mo for 3 mo once developmental age of 4yo

Question 5

57. What is the most likely cause of childhood functional abdominal pain?
    a. Mild inflammation
    b. Lack of lactose digestion
    c. Slow motility with increased visceral pain response

Answer 5

Slow motility with increased visceral pain response

Question 6

2. A 3 week old baby presents with poor feeding and poor weight gain. He is jaundiced and has hepatosplenomegaly. His bilirubin is 170 with conjugated 115. Which imaging would you do next?
   a. Abdominal ultrasound with dopplers
   b. HIDA scan
   c. CT abdomen
   d. MRI abdomen
   - —————
   8) A neonate has an elevated conjugated bilirubin. What is your next step in management?
   a. Liver biopsy
   b. Abdominal ultrasound with Doppler
   c. HIDA scan
   d. Repeat liver enzymes in 2 months
   - —————-
   83) . 4 week baby presents with poor feeding and poor weight gain. He is jaundiced and has hepatosplenomegaly. His bilirubin is 150 with conjugated 100. Which imaging test would you do next?
   a. U/S with dopplers
   b. MRI
   c. CT abdomen
   d. Dis HIDA scan / Nuclear med biliary excretion scan

Answer 6

AUS

Cholestasis (conjugated bili) always pathologic

Intrahepatic (damage to liver cells or structure that secrete bile)

• Infectious: TORCH, UTI, sepsis
• Hepatitis: neonatal idiopathic , viral, bacterial
• Metabolic/genetic: CF, Alagille, alpha-1 antitrypsin deficiency, galactosemia, tyrosinemia, hypothyroidism, PFIC
• TPN-related
• Hemochromatosis
• Idiopathic
• intrahepatic bile duct or pacuity

Extrahepatic (mechanical obstruction of bile flow)

• biliary atresia
• choledochal cyst
• sclerosing cholangitis
• inspissated bile (bile/mucous plug)

AUS can detect biliary tree and other anatomical abnormalities
- triangular cord sign suggests biliary atresia

HIDA is SN but not SP for biliary atresia. Need to wait 5d for this procedure.

Question 7

59. 1 month old with normal physical but total bilirubin 280 and direct 200. Hemoglobin 98 and MCV 95. Most likely cause?
    a. breast feeding jaundice
    b. haemolytic
    c. neonatal hepatitis
    d. galactosemia
    ——————
    Baby with bili of 280 and conjugated 200? What is the most likely cause based on incidence?
    a. Breastfeeding jaundice
    b. Hemolysis ABO
    c. Neonatal hepatitis
    d. Galactosemia

Answer 7

Neonatal hepatitis

Galactosemia: also conjugated, but less common than neonatal hepatitis and in unwell infants

Question 8

75. 3 week old infant has jaundice. His birth weight was 3250g and he now weighs 3490g. He is breastfeeding well. Hemoglobin is 127 and total bilirubin is 270, conjugated bilirubin is 8. Coombs test is negative. What should be done?
    a. Admit for phototherapy
    b. Referral to gastroenterology
    c. Reassess in 1 week
    d. Recommend switching from breastfeeding to formula

Answer 8

Reassess in 1 wk

Unconjugated hyperbilirubinemia
Likely breastmilk jaundice
- Presence of glucuronidase in breast milk
- Presents after first 3-5d, peak within 2wks
- may last for 3-12wks

Feeding + gaining wt well, bili below need for phototherapy, no conjugated bili, negative Coombs, normal Hgb

DDx for unconjugated hyperbili
A. Increased RBC breakdown
1. isoimmunization: ABO, Rh, minor blood group incompatabilities
2. RBC enzyme defects: G6PD, pyruvate kinase deficiency
3. RBC structural abN: hereditary spherocytosis, elliptocytosis
4. Infection: sepsis, UTI
5. Sequestered blood: cephalohematoma, bruising, ICH
6. Polycythemia
7. IDM

B. Decreased hepatic uptake + conjugation of bili

1. Immature glucuronyl transferase activity in all newborns
2. Breastmilk jaundice (glucoronidase in breastmilk)
3. Crigler Najjar Syndrome (low/absent UGT1A1, which catalyzes conjugation of bili with glucuronic acid)
4. Gilbert syndrome (mutation in promoter region of UGT1A1 gene)
5. Hypothyroidism
6. Pyloric stenosis

C. Increased enterohepatic reabsorption

1. Breast feeding jaundice (dehydration)
2. Bowel obstruction
3. NPO

Question 9

32. A 13 year old girl with intermittent cough and early morning throat pain. She also has shortness of breath with exertion. Parents comment that she has had bad breath. What would you do?
    a. Upper GI
    b. abdominal U/S
    c. CXR
    d. pH probe
    —————
    50). A 13 year old girl with intermittent cough and early morning throat pain. Parents comment that she has had bad breath. What would you do?
    a. upper GI
    b. abdominal U/S
    c. CXR
    d. pH probe
    —————
    Bad breath, cobblestoning in pharynx, wheeze. What is the best test?
    a. pH probe
    b. upper GI series
    c. gastroscopy
    ————-
33. 7 year old with sore throat in the mornings, bad breath, chronic cough with abdominal pain for 2 weeks. Her cough is worse with activity. What test will give you the diagnosis?
    a. Throat swab
    b. Pulmonary function tests with methacholine challenge
    c. pH probe
    d. Upper GI series

Answer 9

pH probe

By NASPHAGN 2018 guidelines for child with suspected GERD (progress down steps if doesn’t improve)

1. Assess for alarm signs
2. LIfestyle + dietary education
3. Acid suppression x4-8wk
4. Refer to GI
5. Endoscopy
6. Ph-MII or pH-metry

UGI

• Can look at anatomical issues
• Can’t differentiate GER + GERD
• Can’t determine severity

Multichannel intraluminal impedance

• Can Dx GERD
• Can assess esophageal fxn
• Can determine non-acid reflux

Question 10

3. a 14 year old boy has epigastric pain, dysphagia with solids and weight loss. He is scoped and diagnosed with eosinophilic esophagitis. What management would you recommend?
   a. Referral to Allergy for skin testing to identify potential allergens
   b. PPI
   c. Oral fluticasone
   d. Oral prednisone

What is EoE?

Answer 10

Oral fluticasone

EoE (Consensus 2011)
Dx criteria
1) clinical Sx of esophageal dysfunction: feeding aversion/intolerance, vomiting/regurg, GERD refractory to medical mgmt + surgery, food/FB impaction, epigastric abdo pain, dysphagia, FTT
2) >=15 eosinophils in 1 hpf
3) Exclusion of other possible causes of esophageal eosinophilia (incl’g PPI-responsive esophageal eosinophilia)

IVx
- Dx requires upper endoscopy + Bx
- Furrowed, ring-like, multiple white plaques, strictures, crepe paper mucosa
(None are pathognomonic)
- eosinophilia
- total IgE at Dx + each endoscopic evaluation

• Ingestion of food has been assoc’d with EoE
• food-specific IgE level testing not supported
• consider skin-prick testing for foods + environmental allergens
• atopy patch testing may be promising

Tx

1. PPI as therapy (for reflux assoc’d with EoE) + r/o PPI-REE
2. short course of PO + topical .l,(swallowed fluticasone or budesonide) C/S (for emergent problems like dysphagia, dehydration, wt loss due to swallowing problems)
3. dietary restriction (specific food, 6 food elimination diet, amino acid based formula)
4. Esophageal dilatation in symptomatic ps with esophageal narrowing secondary to fixed strictures causing food impaction
5. Evaluate for aeroallergen sensitivity
   - Note: LTRA + cromolyn sodium not supported b/c no benefit or risk

Question 11

14) 8 year old boy with gastroesophageal reflux for the last 4 years, now having dysphagia on solids. Which of the following tests would reveal the diagnosis?
a. UGI
b. Upper scope + biopsy
c. Abdominal ultrasound
d. AXR

What are complications of GERD?

Answer 11

Upper scope + biopsy

Look for strictures from GERD and r/o EoE

1. Esophageal
   - esophagitis
   - strictures
   - Barrett esophagus
   - adenocarcinoma
2. FTT
3. Respiratory Sx
4. Apnea + stridor
5. Dental erosions

Question 12

70. A 13 month old boy has a diaper rash and diarrhea for the last few months, since his parents have been introducing new foods. Which of the following is correct?
    a. do skin testing and RAST
    b. Carbohydrate intolerance is common at this age
    c. He is now sensitized to cow’s milk protein

Answer 12

Do skin testing and RAST

CMP is leading cause of food allergy in infants + young children <3yo
Lactose intolerance: loss of lactase activity after 3-5yo. Congenital lactase deficiency is extremely rare

Question 13

19) A 6 year old boy has recurrent vomiting episodes where he need to come to the ER and receive IV fluids. He is completely fine in between these episodes. What is the most likely diagnosis
a. cyclic vomiting
b. malrotation

Answer 13

Cyclic vomiting

ALL

1. > =2 periods of intense, unremitting nausea + paroxysmal vomiting within 6mo. Last hours to days
2. Stereotypical pattern of episodes in each pt
3. Episodes separated by wks to mo with return to baseline health between episodes
4. After appropriate medical evaluation, Sx cannot be attributed to another condition

Question 14

4). 12 year-old teen presents to your office and tells you she would like to begin a vegan diet. What is the best advice you can give her today:
a. Vitamin B12
b. Iron
c. Consult a dietitian or nutritionist
D. calcium supplements

Answer 14

Consult a dietitian or nutritionist

Question 15

5. 2 year old girl, picky eater. (long stem about how she is otherwise healthy but doesn’t like to eat certain foods). Has not gained any weight in the last few months. Has always been trending on the same percentile. What do you advise parents?
   a. Offer a variety of accepted foods and allow child to choose what to eat
   b. Offer multiple snacks throughout the day
   c. Let the child pick whatever they want

Answer 15

Offer a variety of accepted foods + allow child to choose what to eat

Physiologic decrease in appetite between 2-5yo to match slower rate of growth

AT meals, give 1 Tbsp of each food per year fo child’s age.

Question 16

7. What is the recommended vitamin D supplementation for a 6 month old baby living above the 55th latitude during winter months?
   a. 200 international units
   b. 400 international units
   c. 600 international units
   d. 800 international units

What are the CPS recommendations for vit D
A) supplementation
B) what is the goal + optimal vit D level
C) what is the mechanism
D) What are the risk of vit D deficiency?

Answer 16

800 units

A. Supplementation

• 400 units daily in breastfed infants until diet has adequate vit D
• 800 units daily if above 55th latitude during winter months
• 1L of formula/dairy milk/fortified rice + soy beverages contain 400units of Vit D3
• 2000 units daily in pregnant + lactating women, esp in winter months

B. Goal + optimal level

• goal is to provide enough Vit D for adequate Ca absorption from gut + minimize PTH secretion but avoid hypercalcemia
• optimal 25(OH)D level is 75-225nmol/L

C. Mechanism

• Vit D3 = skin of animals. Vit D2 = plant origin
• metabolized by 25-hydroxylation in liver to 25(OH)D [inactive, stable, most abundant circulating form]
• then 1-hydroxylation in kidney to 1,25 (OH)2D [active, unstable]

D. Deficiency

• Hypocalcemia + rickets
• osteoporosis
• dental malformations + caries
• SGA
• liked to asthma, autoimmune disease, disturbed muscle function, resistance to TB, pahtogenesis of specific cancer

Question 17

24. 7 wk old baby boy who cries 5h per day and has colic, reflux with feeds; parents try soy milk, baby gets diarrhea; what do you recommend?
    a. Try 2 wks of hypoallergenic milk
    b. Give PPI
    c. Try probiotics
    d. Try lactose free something

What is the definition of infant colic?
What can you try?
What are NOT recommended?

Answer 17

Try 2 wks of hypoallergenic milk

CPS statement
Infant colic
In infants birth to 4mo, ALL of:
1. Paroxysms of irritability, fussing or crying that starts or stops without cause
2. Episodes lasting >=3h/d + occur >=3d/wk for at least 1wk
3. No FTT

Consider trying hypoallergic diet in BF mother
OR hydrolyzed formulas if formula fed (Alimentum, Nutramigen)

If suspect CMPA
- eliminate cow’s milk in BF mother
OR extensively hydrolyzed formula if formula fed (Nutramigen, Alimentum)

Note: completely hydrolyzed formula = amino acid based = Puramino + Neocate

NOT RECOMMENDED

• partially hydrolyzed (Gentlease) b/c not hypoallergenic
• soy formula (only use in galactosemia or religious reasons)
• lactose-free formula
• lactase
• probiotic

Question 18

6) Baby with G-tube that looks like this: (This was the exact photo on the exam)
a. Reassure
b. Silver nitrate cautery
c. Topic ABx
d. Fungal abx cream

Answer 18

Err on side of silver nitrate cautery

Granulation usually occurs in first 2-3mo after G-tube insertion

• Mild cases: do nothing (ensure Gtube secured, area is dry, observe)
• NS or hypertonic saline dressings 3-4X/d until improved
• Topical 1% HC cream x3-4d
• Silver nitrate cautery if large granulation tissue. Put Vaseline to surrounding healthy skin to prevent injury.

Question 19

8.   	A 7 month old baby is switched from breastmilk to formula and solids. He develops diarrhea, eczema and an erythematous rash around his orifices (mouth and anus).
What is the most likely nutrient he’s deficient in?
A. Calcium
B. Zinc
C. Protein
D. Vitamin
-----------------
9) A 7 month old boy presents with failure to thrive, diarrhea, and a severe eczematous rash around his mouth and perineum. He was previously breastfed, but was switched to an appropriate combination of formula and pureed solids. Which laboratory investigation would be most important in order to make the diagnosis?
1) Immunoglobulins
2) Zinc level
3) Vitamin A level
4) Electrolytes

Answer 19

Zinc Deficiency

• decreased growth
• dermatitis of extremities + around orifices
• poor wound healing
• diarrhea
• hypogonadism, infertility
• impaired immunity

Causes
- TPN, chronic malabsorption, burns, CF, chronic renal disease, liver disease, Crohn’s disease

Acrodermatitis enteropathica

• rare AR disorder
• inability to absrob sufficient zinc from diet
• Initial SSx in first few mo of life, often after weaning from breast milk to cow’s milk
• Vesciulobullous, eczematous, dry, scaly. Symmetrically distributed. perioral, acral, perineal areas. Cheeks, knees, elbows.
• Reddish tint hair, alopecia.

Low plasma zinc
Low ALP (zinc dependent)

Tx:
PO elemental zinc 3mg/kg/d. Monitor Q3-6mo

Question 20

10.  8yo male. Duodenal ulcer. What is treatment? (Note: none of them
listed a PPI)
A. amox + clarithro
B. clinda + clarithro
C. metronidazole + clinda
D. bismuth subsalicylate + metronidazole
------------
38. Kid with peptic ulcer. His treatment would likely include
a. amoxil +clarithro
b. clinda and clarithro
c. flagyl and ????

Answer 20

Amox + clarithro + PPI!
H pylori is a PACman making ulcers. P = PPI, A = amox, C = clarithro

H pylori prefers triple therapy
Amox + clarithro + PPI
OR amox + flagyl + PPI
OR clarithro + flagyl + PPI

PUD can be managed with PPI or H2RA

Question 21

103. 15 year old with h. pylori. You should treat with:
     a. h2 blocker
     b. amox, clarithro and proton pump inhibitor
     c. clarithro and h2 blocker
     d. amox and proton pump inhibitor
     ————
     Treatment of H. pylori is likely to consist of which combination:
     a. amoxicillin + clarithromycin
     b. bismuth salts + Abx
     c. clarithryomycin + Abx

Answer 21

Amox + clarithro + PPI
H pylori is a PACman making ulcers. P = PPI, A = amox, C = clarithro

H pylori prefers triple therapy
Amox + clarithro + PPI
OR amox + flagyl + PPI
OR clarithro + flagyl + PPI

PUD can be managed with PPI or H2RA

Question 22

103. 15 year old with h. pylori. You should treat with:
     a. h2 blocker
     b. amox, clarithro and proton pump inhibitor
     c. clarithro and h2 blocker
     d. amox and proton pump inhibitor
     ————
     Treatment of H. pylori is likely to consist of which combination:
     a. amoxicillin + clarithromycin
     b. bismuth salts + Abx
     c. clarithryomycin + Abx

Answer 22

Amox + clarithro + PPI
H pylori is a PACman making ulcers. P = PPI, A = amox, C = clarithro

H pylori prefers triple therapy
Amox + clarithro + PPI
OR amox + flagyl + PPI
OR clarithro + flagyl + PPI

PUD can be managed with PPI or H2RA

Question 23

11. GI. 3 week old blood mixed with stool, has been having since 2 weeks old,
    normal exam, growing/thriving, formula fed
    A. Anal fissure
    B. Cow’s milk allergy
    C. Meckel’s

Answer 23

CMPA

• presents at 2-8wk
• resolves by 6-18mo
• loose BM with occult or gross blood mixed

Anal fissure
- idiopathic in <1yo or passing hard BM w constipation

Meckel's
- intermittent painless rectal bleeding
- brick or currant jelly coloured
- Rule of 2s:
2% of population
<2yo
2:1 M (Meckel's):F
2 ft proximal to ileocecal valve
2 inch long
2 types of tissue (gastric + pancreatic)
Dx: Meckel scan with Tc99

Question 24

41. 1 mo old baby with blood in his stool. Cause?
    a. Anal fissure
    b. Cows milk allergy
    c. Colitis
    d. Mat blood
    ————–
    2 week old baby, well, with stool and blood mixed within. Cause?
    a. cow milk protein allergy
    b. anal fissure -
    c. gastroenteritis

Answer 24

Cow’s milk allergy

Likely FPIAP b/c no FTT

Question 25

23. 3 week old with loose stools and red blood intermixed with the stools, no FTT. What is it? a) Anal Fissure b) CMPA

Answer 25

CMPA Likely FRIAP b/c no FTT

Question 26

76. A 2 yo is in the ER with bright red rectal bleeding. He is pale-looking but otherwise well. He does not appear to be in any discomfort. On rectal exam, you find blood mixed with stool on your glove. Hemoglobin is normal. What is the most likely diagnosis? a. ulcerative colitis b. Meckel’s diverticulum c. Anal fissures

Answer 26

Meckel's diverticulum Rule of 2s 1. 2% of population 2. <2yo 3. 2:1 (M = Meckel's: F) 4. 2 ft proximal to ileocecal 5. 2 in length 6. 2 types of tissues (pancreatic or gastric) Most common congenital anomaly of GI tract Incomplete obliteration of omphalomesenteric duct Lined with ectopic mucosa Painless rectal bleeding Brick coloured or red-currant jelly stools Dx: Meckel's scan with 99Tc (taken up by ectopic mucosa) Tx: surgical diverticulectomy

Question 27

12. 15yo boy presents with severe epigastric pain that worsens after eating. He has had a few episodes of non-bilious emesis. On exam he is tachycardic and has epigastric tenderness, but is otherwise stable. Blood work reveals a lipase of 1650, wbc 12.5, and normal plt, hb, lytes, renal function and ALT. What is the next best step in management? a. NPO and IV fluids b. Surgical consult c. Ceftriaxone d. IV pantoprazole

Answer 27

NPO + IV fluids As per NASPHAGN A. Fluid resuscitation - NS/RL bolus if needed + 1.5-2X maintenance - monitor Cr, BUN, u/o for adequate fluid resusc + AKI B. Pain mgmt - NSAIDs + tylenol - IV morphine/opioids C. Nutrition - consider early (within 48-72H of presentation) PO/enteral feeds to decrease LOS + risk of GI dysfunction - If contraindication to gut use for >5-7d (ileus, abdo compartment syndrome), then parenteral nutrition D. ABx - Not recommended empirically - only if documented infected necrosis E. ERCP - indicated for choledocholithiasis causing biliary pancreatitis + pancreatic duct issues (stones, leaks) F. Surgery - cholecystectomy before D/C if mild uncomplicated acute biliary pancreatitis - if acute necrotic collections (even if infected), delay or avoid surgery >4wks (better outcomes) - if drainage or necrosectomy is necessary, use endoscopic (EUS , ERCP-assisted) or percutaneous over open surgical methods G. F/U - close F/U to monitor for complications + recurrence Not recommended - antiproteases - antioxidants - probiotics - EGD (endoscopy) - EUS

Question 28

90. What is the most specific test for pancreatitis: a. Amylase b. Lipase c. Abdo U/S d. Abdo CT scan e. AXR

Answer 28

Lipase Dx of acute pancreatitis requires at least 2 of 1. abdo pain consistent with acute pancreatitis 2. Lipase or amylase 3X ULN 3. imaging findings consistent with acute pancreatitis - 1st line AUS - CT/MRI if more complicated

Question 29

1) Mother uses marijuana for chronic pain. Breastfeeding. What do you tell her about the risks to the baby: a) continue breastfeeding. Benefits of breastfeeding outweigh risk of marijuana b) marijuana is contraindicated due to risks on the developing brain c) recent studies on the legal use of marijuana found no risk d) Counsel to stop using marijuana. Risks to baby unknown. e) Call CAS

Answer 29

Counsel to stop using marijuana. Risks to baby unknown THC absorbed in GI tract + lungs. Highly lipophilic. Rapidly distributed to brain + fat tissue. Metabolized by liver Excreted in urine + feces. Can be detected for up to 1mo after last use. Theoretically can affect brain development.

Question 30

36. A mother who just gave birth to a newborn was taking propanolol and fluoxetine during her pregnancy. She now wishes to breastfeed the child. What would be your recommendation? a. This is compatible with breastfeeding b. Change the propanolol to another drug c. Stop taking fluoxetine What are the CPS recommendations for SSRI in pregnancy + breastfeeding?

Answer 30

Compatible with BF SSRI (CPS statement) - adequate Tx of depression in pregnancy is v important - typically minimal SE in exposed infants - unlikely congenital malformations in utero - SSRI neonatal behavioural syndrome (CNS, resp, GI) in 10-30% exposed in late gestation. Present in first few hrs, resolve within 2wks * observe babies with late TM SSRI exposure for min 48H - other possible neonatal effects: early GA, low BW, resp distress, PPHN - compatible with BF BB - excreted in small quanitities, so compatible with BF

Question 31

2) 12 year-old teen presents to your office and tells you she would like to begin a vegan diet. What is the best advice you can give her today: a. Vitamin B12 b. Iron c. Consult a dietitician or nutritionist d. Calcium supplements

Answer 31

Consult a dietician or nutritionist

Question 32

23. Vegan infant, about 1 year old. Diet included two eggs per week, green leafy vegetables, soy milk. What are they deficient in? a. Vitamin B12 b. Nothing

Answer 32

Vit B12 Not getting 3 servings/d Need at least 3 servings of food rich in vit B12 in daily diet or supplement of 5-10 mcg/d Sources of vit B12: fortified soy formula, soy/nut beverages, cereals, yeasts

Question 33

51. A 13-month-old child on a vegetarian diet is at risk for which deficiency: a. vitamin C b. calcium c. folate d. zinc e. iron

Answer 33

Iron

Question 34

34. Vegan mother breastfeeding 1 mo old, what would you supplement baby ----------------- 35. A vegan mother (on no supplements) is breastfeeding her baby, and wants to know what kind of supplements should be recommended: a. Vitamin D b. Vitamin B12 c. Iron d. Vit C --------------- A mother who maintains a strictly vegan diet presents with her one month old baby who is exclusively breastfed. What supplementation do you recommend? a. vitamin D b. vitamin B12 -------------------------------------- 25. Breastfed 7 month old babe of a vegan mom. Thriving. What do you supplement baby with? (no vitamin D option) a. Vitamin B12 b. Folic acid c. calcium d. iron -------------- Breastfeeding vegan mother. Nutritional deficiency: a. folate b. iron c. B12 ------------------------------- 40. Vegan mother with infant. Mother is not on any supplements. What should be recommended for the baby? (**The question does NOT tell you if she is breastfeeding!**) a. Vit D b. Iron c. Vit B12 d. Vit C

Answer 34

Vit B12 Though Vit D also important

Question 35

50. A 6-month-old child of a vegan mother is at risk for which deficiency: a. vitamin A b. vitamin B12 c. vitamin C d. vitamin K e. folate

Answer 35

Vit B12

Question 36

32. What is the best test to look for vitamin D deficiency? a. Serum calcium b. Serum 25-OH-Vitamin D c. Serum 1-25-OH Vitamin D d. PTH

Answer 36

Serum 25 OH Vit D 25 D is an abnormal bra size, must measure this! Vit D3 = animal skin, Vit D2 = plant form - metabolized by 25-hydroxylation in liver to 25(OH)D [inactive, stable, most abundant circulating form] - then by 1-hydroxylation in kidney to 1, 25 (OH)2D [active, unstable]

Question 37

5) Who should not receive soy formula due to its phytoestrogen content? a. Baby with galactosemia b. Baby whose family is vegan c. Baby with congenital hypothyroidism What are the CPS recommendations for A) NO soy (3) B) Consider soy (2)

Answer 37

Baby with congenital hypothyroidism CPS statement A. Do not use soy formulas for 1. congenital hypothyroidism (b/c can inhibit thyroid peroxidase, lowering free T4) 2. premature infants (may not promote adequate growth) 3. Non-IgE mediated CMPA (high risk of coincident soy allergy, which is lower in true IgE mediated CMPA) B. Save soy formula for 1. infants with galactosemia or 2. who cannot consume dairy for cultural/religious reasons - Phytoestrogens have theoretical risk of mimicking estradiol (animal studies showing infertility, sex organ dvlpt, brain maturation, immune system, cancer dvlpt issues) - no proven risk

Question 38

11 What indications is it appropriate to offer Soy milk? a) Vegan family b) Fructose deficiency c) CMPA

Answer 38

Vegan family Soy milk may have high levels of fructose

Question 39

26. Phytoestrogens in soy contraindicated for: a. all males b. immune deficiency c. galactosemia d. congenital hypothyroidism -------------- In which of the following is soy formula not suggested secondary to increased phytoestrogen? a. congenital hypothyroidism b. galactosemia c. males

Answer 39

Congenital hypothyroidism

Question 40

10) Best way to decrease your intake of trans-fats: a. Avoid take out food b. Eat less butter c. Eat less margarine - ------------------ 12. Obese child mom wants to know how to reduce Trans fat? a) Cut down on takeout b) Cook with margarine c) Don’t cook with butter d) Choose food low in saturated fats What are 4 key points about trans fats?

Answer 40

Cut down on takeout Trans fat = unsaturated FA 1. No benefit for human health, not essential 2. Occurs naturally in small amounts in dairy + meat 3. Increase LDL, decrease HDL, increase risk fo CV disease 4. Largest dietary source from processed foods due to hydrogenated oils e.g. margarine

Question 41

13. A mom wants to give her 4 month old an egg. You advise her: a) Give the whole egg at 6 months b) Give the whole egg at 1 year c) Give the Yolk at 6 months d) Give the white at 6 months

Answer 41

Give the whole egg at 6mo - Introduce 1 single nutrient ingredient food at a time, observe 3-5d to observe for tolerance before next food - no good evidence for delaying "allergenic" foods - do not give fruit juices in first 6mo, limit amounts of 100% juices Foods to avoid <12mo 1. honey (botulism) 2. choking hazards (hard round foods - nuts, grapes, raw carrots, candies) 3. whole cow's milk (iron deficiency)

Question 42

14. Infant with colic a) Try hypoallergenic milk b) Try soy milk c) Give probiotic

Answer 42

Try hypoallergenic milk

Question 43

20. What is the advantage of using the new WHO growth curves? (don’t quite remember the precise options but it was some combination of breast fed vs. formula fed kids in first or latter 6 months and who do the curves better represent) a. less mislabelled failure to thrive in breastfed babies less than 6 months b. less mislabelled failure to thrive in breast fed babies more than 6 months c. less mislabelled short stature (different wording, same idea) in babies less than 6 months d. less mislabelled short stature in babies more than 6 months -------------- 21 What does the new WHO growth charts do? (Julie, do you remember this question? I don’t exactly remember the words; I may be making some of it up) a. Catch those losing weight in first 6 months b. Catch those losing weight in second 6 months c. Catch those losing ?BMI in first 6 months d. Catch those losing ?BMI in second 6 months

Answer 43

Less mislabelled FTT in breastfed babies >6mo Catch those losing weight in second 6mo I.e. If >6mo child is labelled FTT, you are more likely to be true FTT on WHO WHO (2006) charts - healthy breast-fed 0-5yo receiving adequate nutritional intake + medical care from 6 countries - growth standard! - 3, 15, 50, 85, 97%. Crossing 2 major centiles means greater loss/gain than CDC charts - "When applied to the same population, the WHO charts will result in lower rates of underweight, wasting or thinness (except during the first 6mo of life) and higher rates of stunting, overweight + obesity) - first 6mo, more infants likely to be screened as underweight using WHO growth CDC (2000) charts - pooled from both breastfed + formula fed in US - excluded VLBW (<1500g) - growth reference not growth standard - 10, 25, 50, 75, 90% Formula fed infants grow differently than breastfed infants in first yr - lighter in first 3-4mo - heavier after 4-6mo

Question 44

22. 4m breastfed and then changed to formula and rice cereal. Then had rash (eczema) and then switched to soy formula. Still has rash. What do you suggest? a. Continue breastfeeding b. Hydrolyzed formula c. Continue with current plan d. Stay on soy formula (not sure if this was the 4th option)

Answer 44

Continue breastfeeding - zinc acrodermatitis enterohepatica. Eczematous rash with typical onset in first few mo after weaning from breastfeeding to cow's milk Rash can manifest with CMPA - don't give soy b/c high risk of co-allergy in non-IgE mediated CMPA ?rice-cereal related suggesting celiac. But no options related to this

Question 45

44. Regarding breast milk versus cow milk: a. same protein source b. there is enough iron in cow’s milk for first six months c. the Ca:PO4 ratio is the same d. more calories in breast milk e. there is more carbohydrate in breast milk

Answer 45

?More carbs in breast milk Human milk has 2X as much lactose as cow's milk Breastmilk higher in whey (40C:60W) Cow's milk has more casein (80C:20W) Don't use cow's milk before 1yo b/c increased renal solute load and increased risk fo iron deficiency HUMAN milk does have enough iron in first 6mo - after 4mo, full term breastfed infant may need iron supplementation (e.g. fortified solids) - Minimum daily iron requirements > full term infant 1mg/kg/d, start suppl at 4mo > preterm + LBW infant 2-4mg/kg/d. Deplete iron stores earlier (2-3mo of age). Start suppl by 1mo Possible human milk Ca:P is higher than cow's milk Energy content of human milk = cow's milk = 20kcal/oz

Question 46

15. BMI where we would intervene for risks of obesity | a) 85%

Answer 46

85%tile Endocrine society 2017 - Diagnose overweight + obesity 1. calculate, plot + review BMI percentile at least annually during well-child +/ sick child 3. Diagnose >=2yo Use BMI on CDC %tiles a) Overweight if BMI 85-95%tile b) Obese if BMI >=95%tile c) Extremely obese if BMI >= 120% of the 95%tile or >=35kg/m2 4. Diagnose <2yo a) obese if sex-specific wt for length is >=97.7%tile on WHO charts 5. Evaluate children/adol with BMI >=85%tile for potential comorbidities 6. Do not do lab IVx for endocrine etiologies unless pt's stature +/ ht velocity are attenuated (assess genetic/family potential + pubertal stage) 7. Do not measure insulin concentrations in evaluations

Question 47

16. Autistic child, picky eater, developed gum bleeding, petechiae, tender subcutaneous nodules on legs and refusing to bear weight. What is he deficient in? a) Vitamin E b) Vitamin C c) Vitamin B d) Vitamin A

Answer 47

Vit C | SCURVY!

Question 48

17. 8 month male, quadriplegic, has GER treated with ranitidine. FTT. NG placed and now starting to grow. Next to consider? a. G tube b. GJ tube ------------------------------------ 24. 8 month male, quadriplegic, has GER treated with ranitidine. FTT. NG placed, starting to grow, doing well with NG. Next to consider? a. g-tube b. J-tube c. pH probe d. G-tube + nissen ---------------- Infant with FTT, started on NG feeds the last 2 months and now thriving. Has difficulty swallowing. Also has severe GERD that is responding well to ranitidine. What do you suggest? ----------- a. Gastrostomy b. Jejunostomy c. Fundoplication and gastrostomy ------------------- A 5 year old child with spastic CP who is known for reflux has recently started NG feeds and he is doing much better – growing, healthier, etc. What is the next step to consider? a. Jejunostomy tube b. Gastrostomy tube c. Fundoplication

Answer 48

G tube CPS statement Consider - Context: characteristics unique to the family's decision making (e.g. family structure, parental understanding) - Values: values, attitudes, beliefs + beliefs systems affecting decision - Processes of care: decision-making in concert with child's health team.

Question 49

30. 3 week old baby brought by mother for persistent jaundice. Mom is O+ and baby is A+. Baby is breastfeeding well and thriving. Unremarkable gestational and postnatal history. Likely cause for jaundice: a. ABO incompatibility b. Breastmilk jaundice c. Sepsis --------------- 3 week old child breastfeeding and growing well, jaundiced now. Mom is O and baby is A+. What is the cause? a. ABO incompatibility b. breastmilk jaundice c. hypothyroidism ------------------------ 28. 3 week old breastfeeding and growing well. Persistent jaundice. Baby A+, Mom O+. What is the cause? a. ABO incompatibility b. Breastmilk jaundice

Answer 49

Breast milk jaundice

Question 50

31. Infant with skin breakdown around every orifice, glossitis, stomatitis, corneal changes and looks unwell. Most likely diagnosis: a. Zinc deficiency b. Vitamin A deficiency c. Vitamin C deficiency d. Vitamin B12 deficiency --------------- 3 month old baby with severe diaper dermatitis eczema, mucositis/stomatitis and some eye findings. What deficiency: a. Vitamin A b. Zinc --------------- Breastfed baby, presents with rash around his orifices. Also has glossitis, stomatitis, corneal dystrophy. What is the cause? a. Vitamin C deficiency b. Vitamin A deficiency c. Vitamin B12 deficiency d. Zinc deficiency ------------------ 27. Breastfed baby with rash around his orifices. Also glossitis. What is the cause? a. vitamin C deficiency b. vitamin A deficiency c. zinc deficiency d. vitamin B12

Answer 50

Zinc deficiency

Question 51

48. An infant is brought for assessment of eczema, failure to thrive, diarrhea, and a rash around his mouth and anus. What is the likely deficiency: a. vitamin A b. zinc c. copper d. niacin (B3) e. riboflavin (B2)

Answer 51

Zinc Vit A: night blindness, xerophthalmia, keratomalacia, Bitot spots Niacin B3: pellagra. Diarrhea, dementia, dermatitis photosensitive Riboflavin B2: cheilosis, corneal revascularization magenta tongue, sore throat Copper: peripheral neuropathy, myelopathy, anemia, neutropenia. Can mimic B12 def. Cu absorbed in stomach + prox SI.

Question 52

38. Adopted 3 y.o. from Caribbean country is pale, edematous, with hepatomegaly, abdominal distention, sparse yellow tinged hair. What is the cause? a. Marasmus b. Kwashiorkor c. Zinc deficiency --------------------------------------- 39. Adopted 3 y.o. from Caribbean country is pale, edematous, with hepatomegaly, abdominal distention, sparse yellow tinged hair. What is the cause? a. Marasmus b. Kwashiorkor c. Zinc deficiency -------------------------- 33. Adopted kid from Caribbean with edema, abdominal distention, yellow tinged rusty hair. What is the most likely? a. Kwashiorkor b. Marasmus c. B1 deficiency (beriberi) --------------- 52. Adopted child from third world country with distended abdo, edematous, lethargic and rusty hair. What does the child have: a. B6 deficiency b. Marasmus c. Kwashiorkor d. nephrotic syndrome e. zinc deficiency --------------- Couple adopts a 2 year old female from a Caribbean country. They come to you with concerns about her condition. On exam she is lethargic, edematous, has hepatosplenomegaly, a protuberant abdomen and sparse rusty coloured hair. Most likely diagnosis: a. Marasmus b. Kwashiorkor B1 deficiency Vitamin A deficiency

Answer 52

``` Kwashiokor - Protein-deficient MEALS M - malnutrition E - edema (decreased plasma oncotic pressure) A - anemia L - liver - enlarged fatty liver S - skin lesions (hyperkeratosis, hyperpigmentation, enamel/flaky paint sign of diffuse fine reddish brown scaling, itchy) - reddish or pale hair - Subcutaneous fat preserved - Protuberant belly - Lethargic - Poor appetite ``` Tx: protein ``` Marasmus - protein + calorie deficiency - MUSCLE wasting - decreased subcutaneous fat - prominent ribs - alert + irritable - ++ appetite Tx: protein, fats + carbs ```

Question 53

41. 2mo old child who is currently being fed goat’s milk. What do you supplement with? a. Folic acid b. Iron c. Multivitamin d. Folate e. Ca ------------- Mom feeding goat’s milk to a 2 month old baby. What do you need to supplement with? a. Ca b. Fe c. Folate ------------------------------------- 4) What should you supplement in a baby who is being fed only goat’s milk? a. B12 b. Folate c. Zinc

Answer 53

Folate

Question 54

47. Some vitamins are stored in large amounts and there may be no biochemical or clinical evidence of deficiency for many months. Which of the following vitamins behaves as such: a. thiamine b. vitamin C c. vitamin B12 d. riboflavin e. folate

Answer 54

Vitamin B12 Stores can provide adequate levels fo 5-10y Exceptions - Newborn to vit B12 deficiency mother. Has no stores - Exposure to nitrous oxide

Question 55

37. Infant being fed iron-fortified formula. What do you anticipate: a. No problems b. Constipation c. Black stool d. Colic

Answer 55

No problems Peds In Review - iron-fortified vs low-iron formulas - no differences in fussiness, cramping, colic, GER, or flatulence

Question 56

49. A 3-week-old infant is going to be fed formula, and you suggest an iron-fortified formula. What problems should the mother expect: a. colic b. constipation c. anal fissure d. black stools e. no problems ------------------------------------- A 3 week old bottle fed baby presents to you for their first well baby check. You recommend an iron-fortified formula and mother asks about side effects. The most likely outcome: a. no problems b. constipation c. GI upset

Answer 56

No problems Peds In Review - iron-fortified vs low-iron formulas - no differences in fussiness, cramping, colic, GER, or flatulence

Question 57

54. Which of the following is true regarding iron supplementation in a preterm infant who is breast-fed: a. do not use iron as it interferes with absorption of iron in breastmilk b. start iron supplementation at one month of age c. start only if on a non-iron fortified supplemental formula When should preterm + term infants have iron supplementation and at what age? What are the SSx of iron deficiency?

Answer 57

Start iron supplementation at 1mo of age CPSP - prem + LBW infants born with lower iron stores + grow faster during infancy, so iron stores depleted by 2-3mo (term babies deplete by 4mo) - term exclusively breast fed infants get 1mg/kg/d at 4mo - prem exclusively breast fed should get iron supplement 2mg/kg/d by 1mo until weaned to iron-fortified formula or starting complementary foods SSx - irritable, apathetic, poor appetite - pallor of conjunctivae, tongue, palms, nailbeds - if severe, then signs of CHF

Question 58

46. The best form of nutrition for an infant with short gut syndrome: a. breast milk - ? b. long-term total parenteral nutrition c. elemental formula with MCT d. soy formula with MCT e. whey hydrolysate formula - ------------------------------- 53. Child with short gut. Which is the best form of feeding? a. elemental formula with MCT b. soy formula with MCT c. TPN d. lactose free formula e. gluten free diet

Answer 58

Elemental formula with MCT Short gut syndrome - congenital malformations: congenital short gut, gastroschisis, multiple atresias - resection of bowel: NEC, volvulus +/- malrotation, Crohn's disease Management A. Replenish fluids + lytes losses - oral glucose electrolyte solutions improve intestinal Na absorption - may need TPN B. Bowel rehabilitation - gradual reintroduction of feeds - continuous small volume trophic enteral feeds - protein hydrolysate + MCT-enriched formula (stimulate gut hormones + promote mucosal growth) - advance feeds as tolerated

Question 59

43. Competitive sports and nutrition in pediatrics - which is true? a. give high carbo drinks b. give protein supplements c. provide a well-balanced diet d. give Calcium and vit D supplements e. change child's diet according to the sport in which they're involved. - ----------------------- 45. Competitive sports and nutrition in pediatrics-which is true? a. high carbo drinks b. protein supplements c. regular balanced diet d. change the child’s diet according to the sport in which they are involved ``` What does the CPS recommend for A) Macronutrient % of calories B) Calories/d C) Vit D, Ca, Iron D) Fluids E) Food ```

Answer 59

Provide a well balanced diet CPS Statement: sport nutrition for young athletes - well balanced diet is essential to maintain proper growth + optimize performance In 4-18yo: - carbs 50% [45-65%] of calories. 1g = 4kcal - protein 20% [10-30%] of calories. 1g = 4kcal - fats 30% [25-35%] of calories. 1g = 9kcal Calories (kcal/d) - 4-6yo: 1800 - 7-10yo: 2000 - male: 11-14yo 2500, 15-18yo 3000 - female: 11-18yo 2200 - Vit D: 600units - Ca: 1000mg/d (4-8yo), 1300 mg/d (9-18yo) - Iron: 8mg/d (9-13yo), 14-18yo: males 11mg/d, females 15mg/d - screen female athletes, vegetarians, distance runners periodically for iron status Fluids - 2-3H before: 400-600mL - during: 150-300mL Q15min - after: 4mL/kg - if >1h or hot: sport drink with 6% carb + 20-30mEq/L salt Food - meals 3H before event (limit fibre + high fat) - snacks 1-2h before - during: sports drinks/granola bars - recovery food (prot + carb) 30min and again 1-2H post event to allow muscle rebuilding

Question 60

13. 8yo boy with type 1 diabetes presents with nonspecific abdominal pain. He has had normal stools with no hematochezia. There has been a 1kg weight loss over the past 3 months. On exam he has a pruritic bullous lesions on his extensor surfaces of his arms and on his trunk. What is the most likely diagnosis? a. Celiac disease b. IBD c. eosinophilic gastroenteropathy d. parasitic infection

Answer 60

Celiac disease Dermatitis herpetiformis - vesicobullous eruption - severe itch followed by small blisters (look like HSV vesicles) - elbows, knees, buttocks, neck, scalp

Question 61

26. 7y boy with low ferritin and low hgb. Other cell lines normal, exam normal. Has been on iron treatment for 3mo with good compliance. His ferritin and hgb are still low. What is the next step? a. Anti-TTG b. Bone marrow c. Upper GI d. Upper endoscopy

Answer 61

Anti TTG

Question 62

80) 2yo suspected of having Celiac. What is the definitive test for celiac disease a. Anti transglutaminase antibody b. Jejunal biopsy c. Gluten free diet trial d. Antigliadin antibody

Answer 62

Jejunal biopsy | would need multiple samples

Question 63

55. Picture of emaciated child with ftt and muscle wasting (it is celiac). Best test for dx? a. immunoglobulins b. small bowel bx

Answer 63

Small bowel Bx

Question 64

77. 6 mo baby with FTT is very irritable. Was breastfed initially and solids were introduced at 4 months of age. What is the likely diagnosis? a. Celiac disease b. CF c. Cow’s milk allergy

Answer 64

Celiac disease CF: no comment on changes in stool to suggest steatorrhea, no resp Sx CMPA: unusual age. FPIES does have FTT, should have more GI Sx.

Question 65

22. 15 yo boy with nocturnal diarrhea and diarrhea 3 months. No pain, tried lactose free didn’t help a) UGI b) Colonoscopy c) Barium d) Lower Scope

Answer 65

Colonoscopy Need to r/o IBD

Question 66

52. Teenager with weight loss, poor appetite, anal fissure. What to do? a. colonoscopy b. upper GI series

Answer 66

Colonoscopy Need to r/o IBD

Question 67

33. Cause of malnutrition in IBD a. low intake of nutrients b. malabsorption

Answer 67

Malabsorption

Question 68

71. Teen girl with weight loss, vomiting, fistula in ano. (No diarrhea, but makes it really sound like Crohn’s). What test to make diagnosis? a. Ultrasound or CT (I forget) b. Colonoscopy c. Upper GI series -------------- Teenaged girl with weight loss, abdominal pain and an anal fissure. Which investigation will MOST LIKELY reveal the diagnosis? a. abdominal ultrasound b. colonoscopy c. upper GI with follow-through

Answer 68

Colonoscopy Only 25% present with triad of diarrhea, wt loss, abdo pain

Question 69

25. Crohn’s kid on azathioprine; present w r flank pain; stools normal. What is your dx? a. Renal colic b. Cholecystitis c. Crohn’s flare

Answer 69

Renal colic Doesn't meet PCDAI for Crohn's flare b/c normal stools and just flank pain

Question 70

15) A 7yo boy has been complaining intermittently over the last 2 days of RUQ pain and has had non-bilious emesis. He has had several dark red mucousy stools. Which imaging test would reveal the diagnosis? a. UGI b. Technetium 99 scan c. Abdo U/S d. Colonoscopy ``` A. What is the diagnosis? B. What types + causes? C. What SSx? D. How to Dx? E. How to manage? ```

Answer 70

AUS Intussusception - ileocolic (90%) > ceco-colic > ileo-ileal - 90% idiopathic - ~5% have lead points, more common in >2yo: Meckel's, polyp, neurofibroma, hemangioma, lymphoma SSx - classic triad (<30%): 1) paroxysmal pain, 2) palpable sausage-shaped abdo mass, 3) bloody or currant jelly stool Diagnosis - AUS: target sign, tubular mass - Plain AXR: target sign, crescent sign - Air, saline, or water-soluble contrast enemas: coiled spring appearance Management 1. Reduction - Spontaneous in 10% - hydrostatic (saline) or pneumatic (air) - contraindications: prolonged intussusception, signs of shock, peritoneal irritation, intestinal perforation, pneumatosis intestinalis 2. Surgical - Indications for reduction: refractory shock, suspected bowel necrosis, perforation, peritonitis, suspected lead point - Indications for resection: reduction is not possible or bowel not viable 3. Steroids - decrease frequency of recurrent intussusception 5% recur, most within 72H of reduction

Question 71

29. Kid w hepatitis, which would be an indicator of function? a. INR

Answer 71

INR ``` Liver function W Low plt High INR Low albumin HIgh bili Low glucose ```

Question 72

17) In a patient with liver failure, which of the following is most concerning regarding need for transplantation? a. Acute onset lethargy b. Acute liver volume loss c. Increase in liver enzymes

Answer 72

Acute liver volume loss Rapid decrease in liver size without clinical improvement is an ominous sign

Question 73

20. A 4 year old patient presents with lethargy, bruising, hepatosplenomegaly and elevated LFT’s. He has signs of acute liver failure (this is given in the question). Which would make you most worried? a) Sudden decrease in liver size b) Development of asterixis A. What is acute liver failure? B. What are 7 causes? C. What are the SSx? D. How to manage?

Answer 73

Sudden decrease in liver size Rapid decrease in liver size without clinical improvement is an ominous sign Acute liver failure (fulminant hepatic failure) - biochemical evidence of liver injury <8wks - Hepatic-based coagulopathy (prolonged INR) +/- clinical hepatic encephalopathy Causes 1. Vascular: hypoxia + ischemia d/t hepatic vascular occlusion, severe heart failure, circulatory shock 2. Idiopathic (50%) 3. Drugs: acetaminophen, Amanita mushroom, isoniazid, VPA, tetracycline 4. Viral hepatitis 5. Autoimmune hepatitis (5%) 6. HLH (increased ferritin, increased TG, low fibrinogen) 7. Metabolic: Wilson, galactosemia, tyrosinemia, hereditary fructose intolerance, FAOD, mitochondrial disorders SSx - progressive jaundice, fetor hepaticus, fever, anorexia, vomiting, abdo pain - hepatic encephalopathy: d/t increased ammonia. Lethargic, confused, asterixis. Onset occurs 8-24wks after onset of jaundice. Cerebral edema is severe complication ``` Management A. Treat the cause (Amanita mushrooms -> penicillin) B. Supportive - Avoid renal dysfxn - Manage coagulopathy - vit K. May need FFP, cryo, plts. - Monitor for infections - Prevent encephalopathy C. Liver transplant ```

Question 74

95. A child has resolving viral hepatitis. AST and ALT have normalized. Bilirubin is still elevated. Which of the following is best to monitor serially: a. GGT b. INR c. Albumin d. Abdo ultrasound e. Alk phos

Answer 74

INR (marker of synthetic function) Albumin is a marker of chronicity

Question 75

68. Child with hepatitis, initially elevated ALT and AST but now resolving. What is the best thing to follow a. INR b. GGT c. ALT, AST d. albumin ------------- A child has resolving viral hepatitis. AST and ALT have normalized. Bilirubin is still elevated. Which of the following is best to monitor serially: a. GGT b. INR c. Albumin d. Abdo ultrasound

Answer 75

INR

Question 76

74) . 11 year old girl with vague abdominal pain, vomiting, and jaundice. Her labs show ALT 1000, total bilirubin 100. What test will likely confirm the diagnosis? a. Hep A IgM b. CMV urine c. Heb B serology d. Monospot

Answer 76

Hep A IgM Hep A is most prevalent hepatitis viruses Fecal-oral transmission Contangious for 2wks before + 7d after onset of jaundice Exclude from daycare/school Responsible for acute hepatitis only When Sx: acute febrile illness with abrupt onset of anorexia, nausea, vomiting, malaise, jaundice Duration 7-14d Dx: anti-HAV IgM (detectable when Sx clinically apparent, pos for 4-6mo) Tx - supportive - monitor for signs of acute liver failure + prolonged cholestatic syndrome - prevent with HAV vaccine (2 inactivated) No RFs for Hep B - blood + sex transmission - IVDU, blood products, acupuncture, tattoos, sexual contacts, institutional care, perinatal

Question 77

28. Teenage girl presents with hepatitis, bili 42 conjugated, 55 total. Anti Smooth muscle antibodies positive to a titre of 1:512. What is the most likely diagnosis? a. Alpha 1 antitrypsin deficiency b. Wilson’s disease c. Autoimmune hepatitis

Answer 77

Autoimmune hepatitis Chronic hepatic inflammatory process Can be triggered by infections (Hep B), drugs, envo toxins in genetically susceptible host - ASx (w just biochemical liver dysfxn) to hepatic failure - extrahepatic: arthritis, vasculitis, nephritis, thyroiditis, Coombs-pos anemia, rash ``` Elevated AST, ALT, bili (hepatocellular) Normal ALP/GGT Low albumin + plts High INR **Hypergammaglobulinemia **Serum autoAb: ANA, anti-SM, antiactin antibody, anti LKM ``` Prednisone +/- azathiorpine or 6MP

Question 78

39. Child with increased unconjugated bili, increase conjugated bili, increased ALT, AST. Normal alpha1antitrysin, 1:512 anti-sm. Most likely dx? a. Gilberts b. Alpha 1 antitrypsin c. PSC d. Autoimmune

Answer 78

Autoimmune - Gilbert is unconjugated bili, not conjugated - Normal alpha 1 antitypsin - PSC would see more increased GGT+ALP

Question 79

40. Adolescent female presents with jaundice. Bloodwork findings: bili 55, direct 40, AST 250, ALT 200. Copper, ceruloplasmin, Hep A, B, C normal. Alpha 1 antitrypsin done (2.5 U). what is the cause? a. alpha-1 antitrypsin b. autoimmune hepatitis c. Gilbert’s

Answer 79

Autoimmune hepatitis normal alpha 1 antitrypsin is 150-350mg/dL Gilbert is unconjugated hyperbili Normal copper + ceruloplasmin does not suggest Wilsons Normal Hep A,B,C does not suggest viral hepatitis

Question 80

98. What is true regarding extrahepatic portal hypertension: a. associated with hepatomegaly b. associated with splenomegaly c. Liver transaminases will be abnormal d. most common cause is splenic vein thrombosis ``` A. What are the 2 causes of portal hypertension B. What are the 2 most common SSx? C. What are 3 complications? D. How to diagnose? E. How to treat? ```

Answer 80

Associated with splenomegaly Portal hypertension portal pressure >10-12mmHg (normal is 7) Extrahepatic - most common cause is portal vein thrombosis - Other: splenic vein stenosis/thrombosis, omphalitis Intrahepatic (poor prognosis) - viral hepatitis, cirrhosis, A1AT, Wilson, CF, PSC, biliary atresia SSx - esophageal bleeding (#1 most common) - splenomegaly (#2 most common) - intrahepatic has large hard liver, abnormal liver transaminases Complications - hepatopulmonary syndrome (intrapulmonary microvascular dilation -> oxygenation defect. Dx: microbubbles seen after saline bolus into peripheral vein) - bleeding - peritonitis Dx - Doppler U/S - Endoscopy for GIB Tx - Treat bleeding: fluids, vit K or FFP, NG, IV PPI, octreotide, vasopressin, endoscopic band ligation of esophageal varcies - Surgical procedures to divert portal blood flow - Liver transplant

Question 81

21. Girl with facial edema, pallor. Albumin 26. Urine negative for protein. Most likely test to reveal diagnosis: a) serum trypsinogen b) stool alpha 1 antitrypsin c) echocardiogram d) 24 hr creatinine clearance

Answer 81

Stool alpha 1 antitrypsin - synthesized by liver, not ingested in diet - Screening test for protein losing enteropathy Serum trypsinogen for CF. Not usually edema ECHO for CHF. Should have other cardiac + resp findings. 24H creatinine clearance. Not particularly helpful, particularly as urine neg for protein, less likely nephrotic syndrome

Question 82

67. 14 month old child with puffy eyes, albumin of 14, urine negative for protein. Looks well. On an unrestricted diet. How would you treat? (no mention of any GI symptoms in the question) a. Prednisone b. High protein, high calorie diet c. Protein hydrolysate formula d. IV albumin ---------------- 15 month old boy is very edematous but has no proteinuria. Height & weight are at the 75th percentile. Your management? a. high protein, high calorie diet b. protein hydrolysate formula

Answer 82

Protein hydrolysate formula Protein-losing enteropathy If give high protein, will just lose Since he looks well, don't need IV albumin

Question 83

27. 5 years old kid w abdo pain, on u/s see intussusception; has hyperpigmented macules on lips a. Peutz jegher b. Crohn’s

Answer 83

Peutz Jegher Need 2/3: 1. Pucker by puberty (mucocutaneous hyperpigmentation - dark black/blue, often fade by puberty), 2. Polyps in SI -> intussusception 3. Positive FHx (AD) Increased risk of cancer: CRC, breast, gyne/testicular

Question 84

61. A child with intussusception has melanotic lesions on his lips. What is the most likely diagnosis? a. Peutz Jegers b. HUS c. HSP - --------------- 62) 5 yo with bloody stools. Hyperpigmented lesions on lip and mucosa. Diagnosed with intusseption. What is underlying cause? a. HSP b. Peutz-Jehgers c. Langerans’ histiocytosis

Answer 84

Peutz-Jehgers Need 2/3: 1. Pucker by puberty (mucocutaneous hyperpigmentation - dark black/blue, often fade by puberty), 2. Polyps in SI -> intussusception 3. Positive FHx (AD) Increased risk of cancer: CRC, breast, gyne/testicular

Question 85

51. Alpha-1 antitrypsin. Most likely presentation in children? a. jaundice b. emphysema c. bronchiectasis d. pneumonia

Answer 85

Jaundice

Question 86

45. 2 days of fever and cough, mother concerned with yellow eyes. No abdo pain. Indirect bili 58, direct 9, ALT 40, AST 42, Normal wbc. What do you do? a. Do nothing b. HIDA ? c. Direct Coombs --------------- 46) Early adolescent presenting with jaundice, indirect hyperbili (bili 56, direct 12?), fever. what to do? a. Hep A, B, C serology b. blood smear c. do nothing --------------- Jaundiced child with elevated unconjugated bili, Normal Hb, Nr retics. What investigations: a. Hep A,B,C serology b. Blood smear

Answer 86

Direct coombs, blood smear to look for hemolytic anemia

Question 87

30. A 3 week old patient is being worked up for cholestasis and is found to have a pointy chin, a broad forehead and butterfly vertebrae. Which of the following is most consistent with the diagnosis? a. chorioretinitis b. posterior embryotoxon c. cataract d. glaucoma

Answer 87

Posterior embryotoxon ``` Alagille Pointed chin Posterior embryotoxon Paucity of bile ducts PPPS Butterfly vertebrae ```

Question 88

37. Eye finding in Alagille’s syndrome a. posterior embryotoxin ---------------- Eye finding in child with cholestasis, triangular facies, frontal bossing a. glaucoma b. cataract c. posterior embryotoxin ------------- Description of kid with alagille (butterfly vtb, heart murmur, cholestatic jaundice at 2 weeks of life), eyes show a. chorioretinitis b. posterior embryotoxon c. cataract d. Glaucoma -------------- Kid investigated for cholestasis. Frontal bossing. Triangular face. Most likely eye findings? a. Cataract b. Chorioretinitis c. Embryotoxin d. Aniridia

Answer 88

Posterior embryotoxin

Question 89

31. 3 month old boy with constipation since birth, FTT, and a mildly distended abdomen (RECTAL EXAM NOT DESCRIBED). What would be the most appropriate next test: a. rectal biopsy b. TSH c. Sweat chloride d. follow up in 6 months

Answer 89

Rectal biopsy R/O hirschsprung's. Most common cause of lower intestinal obstruction in neonates. Hypothyroidism possible, but no other signs on exam (e.g. prolonged jaundice)

Question 90

3 month old baby boy who has been breastfeeding well, but now 3rd percentile in growth parameters. Constipated since birth. a. TSH b. sweat test c. anal manometry - ----------------- 60. A 3 month old baby has failure to thrive and constipation. What do you order?? a. sweat chloride b. rectal manometry c. TSH, T4

Answer 90

Rectal manometry - Bx would be better answer Evaluate internal anal sphincter while ballon is distended in rectum Normal pts: rectal distension initiates relaxation of internal anal sphincter Hirschprung's: failure of anal sphincter relaxation

Question 91

54) 3 m.o. infant with weight loss, abdominal distension and infrequent stools since birth. Next investigation: a. rectal biopsy b. U/S abdomen c. Sweat test

Answer 91

Rectal biopsy Hirshsprung - most common cause of lower intestinal obstruction in neonates - neural crest cells fail to complete cranial to caudal migration - no parasympathetic innervation to distal colon -> abN peristalsis, functional constipation - 2 main assoc'ns: T21, CCHS SSx - fail to pass meconium, bilious emesis, distended abdo - FTT possible - tight empty rectal vault - blast sign (explosive stool output with DRE) Dx 1. barium contrast enema: transition zone 2. Anal manometry - Normal: rectal distention with ballon means internal anal sphincter relaxes - Hirshsprungs: failure to relax 3. Rectal Bx (definitive): absent ganglion cells, hypertrophic nerve fibres Tx: surgery Complication: enterocolitis (fever, bloody stool, abdo distension)

Question 92

72) 3 mo baby with constipation and failure to thrive. What test to do? a. TSH b. Anal manometry c. Sweat chloride

Answer 92

Anal manometry

Question 93

36. Kid with bloody diarrhea 1 month ago which has now resolved. Now has weight loss and abdominal pain. Which was the most likely organism? a. Yersinia b. Campylobacter c. E Coli d. Shigella ---------------- Teenager who has had low grade fever, malaise, and weight loss in the last 3 weeks. it started with non-bloody diarrhea x 1 week, now resolved. What is cause? a. e coli b. campylobacter c. c difficile d. yersinia

Answer 93

Yersinia Bloody diarrhea Your stool smells extremely crappy Yersinia: GN coccobacilli - food, water, animal contact (pigs) - most common <5yo - fever, vomiting, diarrhea. - exudative pharyngitis, mesenteric lymphadenitis, arthritis, erythema nodosum. - appendicitis like - Diarrhea can last 2-3wks. - usu self limiting. Septra for systemic infection or <3mo Shigella: GN bacilli - high fever, lower abdo pain, diarrhea (water to bloody), PAINFUL defecation. - Last 4-7d - ABx! Salmonella - contam food, water - Fever, abdo cramps, V/D - Last 4-7d - ABx for S typhi = paratyphi. Otehrwise only if extraintestinal spread E.coli: - diarrhea, vomiting (fever rare) - Last 5-10d - Rarely ABx Campylobacter: GN S shaped bacilli - contaminated chicken (poultry). - FEVER, diarrhea, abdo pain. - Last 2-10d - Gullain-Barre! - Azithro if severe

Question 94

58. Child who lives on a farm presents with abdominal pain and diarrhea. His small bowel follow through reveals narrowing of his distal ileum. Which of the following organisms would be important to rule out prior to getting a colonoscopy. a. Entamoeba histolytica b. Campylobacter c. Yersinia d. Shigella

Answer 94

Yersinia

Question 95

63) . A 6y M has always lived on a farm. For the last 3 months he has had intermittent fever, vomiting, diarrhea and weight loss. A test shows narrowing of distal ileum. Which is the causative agent? a. Entameoba histolytica b. Yersinia c. Salmonella d. Giardia

Answer 95

Yersinia Entamoeba histolytica: presents with 1) amebic colitis, or 2) amebic liver abscess. Abdo pain, diarrhea + tenesmus. Salmonella: illness duration seems too long Giardia; fever doesn't really fit

Question 96

43. Most important reason to remove a foreign body in the esophagus within 24 hours is to: a. Decrease risk of GERD b. Decrease risk of aspiration c. Decrease risk of perforation d. Risk of Esophagitis --------------- Most important indications to remove a foreign body in the esophagus after 24 hours? a. Esophageal perforation b. Esophagitis --------------- Reason why we remove esophageal foreign bodies that have been sitting for >24 hours a. esophagitis b. risk of aspiration c. risk of esophageal perforation

Answer 96

Decrease risk of esophageal perforation Choke/gag/cough then excessive salivation, dysphagia, food refusal, emesis or pain in neck - children: coins, adolescents: meat/food ABCS - is it in trachea? Can they handle secretions? - CXR + AXR to assess position - Eso: flat surface of coin seen in AP view, edge on lat view - Trachea: edge of coin seen in AP view, flat surface on lat view - If eso/stomach -> endoscopically remove - If past pylorus -> no intervention unless trapped in ileocecal valve or perforation -> gen surg FB Esophagus - Most FB lodge at level of UES, aortic arch, or LES - All esophageal FB need to be removed within 24H - Battery = emergency -> remove within 6H - If cannot handle secretions or sharp object -> remove immediately FB Stomach - can observe small FB, repeat Xray in 4wks -> if not passed, then endoscopic removal - Battery or 2 magnets must be removed within 24H - If >2cm thickness, >5cm diameter, or >6cm in length, need endoscopic removal

Question 97

69. A child swallowed a coin. There is a coin in the stomach on the AXR. What would you do? a. Observe b. Endoscopy ------------ A child presents after having been witnessed to swallow a nickel. X-ray confirms the coin is in the stomach. What do you do? a. Notify surgery for urgent laparotomy b. Admit, repeat x-ray in 3 days c. Notify GI for endoscopic removal d. Observation only

Answer 97

Observe Once in stomach ~95% of ingested objects pass w/o difficulty Usually takes 4-6d. Ask parents to observe stools If >2cm thickness, >5cm diameter, or >6cm in length, need endoscopic removal

Question 98

87) Child swallowed a coin and it is in the stomach on X-ray. What do you do? a. Repeat X-ray in 2 days b. Consult surgery c. Observe d. Give him a pro-motility agent

Answer 98

Observe Once in stomach ~95% of ingested objects pass w/o difficulty Usually takes 4-6d. Ask parents to observe stools If >2cm thickness, >5cm diameter, or >6cm in length, need endoscopic removal

Question 99

89. Sexually active adolescent female with sickle cell disease and a history of previous transfusions presents with an acute history of fever, jaundice and vomiting. She also develops right shoulder pain. Hb 79 (prev 89), AST and ALT slightly elevated. Unconjugated bilirubin is increased. What is the most likely diagnosis? a. fitz hugh curtis b. cholecystitis c. hepatitis C d. HIV e. vasoocclusive disease of the liver

Answer 99

Vasoocclusive disease FItz hugh curtis - perihepatitis in setting of PID - RUQ pain + pleuritis. Sometimes referred to right shoulder - normal aminotransferases - Laparoscopy: violin string adhesions Cholecystitis - RUQ pain, can radiate to right shoulder or back - Fever, N/V, anorexia - Fatty food >1H before pain - Elevated ALP + direct bili