GI and Nutrition - 2019 Updated!

Question 1

(Picture of gastroschisis) Which of the following anomalies is associated with this
condition:
a. Intestinal atresia
b. Renal anomaly
c. Cardiac anomaly

Answer 1

a. Intestinal atresia
Right of umbilicus - defect in abdominal wall (vs omphalocele which herniate abdominal contents in sac through umbilicus)
-1/10 babies have intestinal atresia (omphalocele has cardiac, renal, neural tube defects, genetic associations)

Question 2

(AXR with a large bubble of stomach gas and a second bubble of gas to the left of this, no gas distally)

a. What is this XR sign called?
b. What diagnosis is this consistent with?
c. What syndrome is often associated with this diagnosis?
d. What specific heart lesion are babies with this syndrome at risk for?

Answer 2

a. What is this XR sign called? double bubble
b. What diagnosis is this consistent with? congenital duodenal atresia
c. What syndrome is often associated with this diagnosis? Trisomy 21
d. What specific heart lesion are babies with this syndrome at risk for? AVSD

Question 3

A 16 year old female presents with decreased appetite and weight loss. There is a height-weight discrepancy. She has an anal fissure. What is the most appropriate investigation:

a. UGI and small bowel follow through
b. psychiatry consult
c. barium enema

Answer 3

a. UGI and small bowel follow through - now probably more likely to do MRE if available

• chronic anal fissures are associated with Crohn’s
• UGI can show aphthous ulcers, strictures, fistulae

Alt Q had colonoscopy as an option, which would be a better place to start.

Question 4

5 yo with cough and fever. Crackles on the RLL. CXR shows either diaphragmatic eventration vs hernia. What is the best next test for diagnosis?

a. Diaphragm fluoroscopy
b. Ultrasound
c. Exploratory laparotomy
d. MRI

Answer 4

b. Ultrasound
U/S can help differentiate CDH from diaphragmatic eventration (weakness of diaphragm, but not a hole in diaphragm - can be acquired from phrenic nerve injury or congenital); on U/S see paradoxical movement of hemidiaphragm in eventration

Question 5

13 year old with early morning throat pain. Parents comment that she has bad breath. What would you do:
A) upper GI
B) Abdo u/s
C) CXR
D) pH probe

Answer 5

D) pH probe

- best option of these to assess for GERD

Question 6

An 8 month old child is admitted for viral gastroenteritis. The child has intermittent episodes of screaming and vomiting. On examination the child is pale and lethargic.
Which of the following examinations is most useful in diagnosis of this patient:
a. AXR
b. Serum lactate
c. Air enema

Answer 6

c. Air enema

- diagnostic and curative for intussusception; air preferred to saline/contrast as lower risk of perforation

Question 7

A 6 year old boy tells his mother that he has swallowed a nickel. You do an abdominal XR and see a round radioopaque round object in the stomach. What is your
management:
a. Observation
b. Upper endoscopy and removal
c. Cathartics

Answer 7

a. Observation
- once in stomach, 95% of FBs pass spontaneously
- take the batteries and sharp objects out of trachea stat, otherwise can observe x24h for passage into stomach

Question 8

Child with Foreign body in the esophagus.

List 3 indications for removal

Answer 8

• Respiratory symptoms
• Button Battery
• Sharp object
• Multiple magnets
• blunt object not passing into stomach in 24h
• meat not passing into stomach in 12h
• failure to visualize FB on plain film in symptomatic patient

70% esophageal FBs remain trapped

Question 9

Best test of hepatic synthetic function?

a. AST
b. ALT
c. INR
d. Alk Phos
e. GGT

Answer 9

c. INR

assessment of liver synthetic function: increased PTT, INR that does not correct with vitamin K

Question 10

Confirmation of milk intolerance

a. D-xylose
b. jejunal biopsy
c. milk RAST test
d. serum IgE
e. milk challenge

Answer 10

e. milk challenge - gold standard for lactose intolerance

Presumptive if: symptoms occur within a few hours of significant lactose ingestion and resolve 5-7 days after avoidance

a. D-xylose - test for malabsortion
b. jejunal biopsy - test for lactase deficiency
c. milk RAST test - aka specific IgE test - use in suspicion of IgE mediated CMPA
d. serum IgE - not useful

Question 11

A child presents with watery diarrhea. Stool reducing substances will NOT be positive with which of the following:

a. glucose
b. sucrose
c. fructose
d. lactose
e. galactose

Answer 11

b. sucrose
- acidic stool with 2+ reducing substances suggests carbohydrate malabsorption - reducing substances include all in question plus pentose but NOT sucrose
- basic concept: you need an enzyme to convert disaccharides to monosaccharides (the small intestine can only absorb monos, not disaccharides); SO if you don’t have this enzyme you have a bunch of disacchs which end up in the colon. In the colon they get converted to methane and organic acids (reducing substances) which cause osmotic diarrhea

Question 12

What 4 screening tests would you order in a child with suspected celiac disease?

Answer 12

• anti-tissue transglutaminase IgA antibodies (can be unreliable in < 2 y.o. because relative IgA deficiency)
• IgA level (if low, TTG can be falsely negative)
• CBC, B12, iron studies, 25-OH Vitamin D
• ? TSH

Consider:

• anti-endomesial antibodies (EMA)
• if TTG antibodies are positive but <10x the ULN, should have upper GI scope

Question 13

Kid with CP presents with difficulties swallowing. She is tolerating her GT feeds but has difficulty managing her oral secretions. Of note, she got Botox injections to her legs last week. On exam, she has oral secretions and her legs feel less hypertonic. What do you do:

a. pH probe
b. do a swallow study
c. observe in hospital

Answer 13

ANSWER: c. observe in hospital (assuming due to botox)

b. do a swallow study (can esp if you think achalasia)

Botox can cause systemic effects beyond just local injection, including dysphagia

Question 14

Newborn post repair for TEF with esophageal atresia, persistent respiratory distress. 3 possible diagnoses

Answer 14

• anastomotic leak
• refistulization
• post surgical stricture
• tracheomalacia
• GERD
• vocal cord paralysis
• cardiac anomaly (VACTERL)

Question 15

A 1 month old has progressive non-bilious vomiting. On exam, there is a small palpable olive in the RUQ. What would be the most likely lab abnormality?

a. metabolic acidosis
b. respiratory acidosis
c. hypokalemia
d. alkalotic urine
e. hypernatremia

Answer 15

c. hypokalemia

Pyloric stenosis
Hypochloremic, hypokalemia metabolic alkalosis
- puke out HCl, Na and K; RAAS kicks in and preserves Na at expense of K

Question 16

What is true about intussusception?

a) Meckel’s diverticulum is the most common lead point
b) 75% of cases are idiopathic
c) most patients present with red currant jelly stools

Answer 16

ANSWER: b) 75% of cases are idiopathic - most UTD
version of Nelson’s says 90% idiopathic

c) most patients present with red currant jelly stools - blood is usually passed in the first 12 hours, though sometimes not for 1-2 days (normal stools are often still passed in the first few hours of symptoms)

Question 17

Infant has problem of vomiting with feeds and chronically wheezy.

Upper GI shows indentation of upper esophagus. What are two diagnoses you consider (2)?

Answer 17

Ddx of wheeze, vomiting and indentation of esophagus:

• vascular ring
• pulmonary artery sling
• esophageal duplication cyst
• tumor compression
• lymph node compression
• TEF

Question 18

2 indications for fundoplication in 6 mo child with GERD.

Answer 18

• intractable GERD
• refractory esophagitis
• strictures
• chronic pulmonary disease

Question 19

A mother of a 2 month old infant comes to you for the concern that the baby is difficult, fussy spitting up with feeds. The infant is developmentally appropriate, feeds avidly on a 6 ounce bottle without difficulty while in the exam room. The infant feeds every 4 hours. Has been gaining weight well. Mom is concerned but coping well.

What 5 non-pharmacologic recommendations would you give to the mom to help with the spitting up?

Answer 19

1. reduce volume of feeding
2. increase frequency of feeding
3. feed baby in more upright position
4. take breaks during feed to burp baby to allow for pacing
5. keep baby upright for 30 minutes after feed, and position prone or upright when awake
6. Reassurance that it does not have harmful effects
7. Consider thickening feeds

Question 20

Pyloric stenosis: lyte abN

a. Hyperkalemia
b. Metabolic acidosis
c. Hypochloremia
d. Hyponatremia

Answer 20

c. Hypochloremia

Hypochloremia, hypokalemia metabolic alkalosis

Question 21

Kid vomiting recurrently, missing lots of school, mom has headaches. Most likely cause?

a. abdominal migraines
b. benign paroxysal vertigo
c. separation anxiety

Answer 21

a. abdominal migraines

Question 22

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 fundoplication

Answer 22

a) g-tube

GT indications include FTT, severe GERD,
neurologic impairment.

Question 23

3 week old with pyloric stenosis, severe metabolic alkalosis (bicarbonate 34). What to do:

1. Give hydrochloric acid IV
2. OR immediately
3. Give large amounts of chloride IV
4. Give 5mmol/kg of KCl IV bolus
5. Give ascorbic acid IV

Answer 23

3. Give large amounts of chloride IV

Correct alkalosis pre-OR to prevent post-op apnea (compensate for metabolic alkalosis by not breathing)

Question 24

Oral rehydration in kid with mod dehydration

a) 100cc/kg over 4h
b) 50cc/kg over 4h
c) NS bolus 20cc/kg

Answer 24

a) 100cc/kg over 4h
- Mild 50cc/kg over 4 hours or Moderate 100cc/kg over 4 hours, 5-10cc at a time
- severe- bolus then ORT

Question 25

days post scoliosis surgery. Presents with bilious vx. Etiology? a. SMA syndrome b. Small bowel adhesions c. Pancreatitis

Answer 25

a. SMA syndrome Superior mesenteric artery syndrome - compression of duodenum by SMA against aorta

Question 26

Child with CP and GERD. 1) Name 2 tests to confirm this diagnosis. 2) Name 4 severe complications of GERD.

Answer 26

1. Tests to confirm: - pH monitoring and intraluminal impedence - Endoscopy and biopsy - 4-8 week trial of PPIs and evaluate for symptom response 2. Complications: - Erosive esophagitis - Barrett's esophagus - strictures - aspiration pneumonia - FTT - Worsened asthma - Apnea/stridor - Food refusal - laryngeal edema/granulomas

Question 27

A 14 year old black male with one year history of intermittent abdominal pain and some diarrhea. a. unlikely a primary lactase deficiency b. if reducing substances are negative then a disaccharidase deficiency is ruled out c. try avoiding milk d. do a breath hydrogen test e. do a hemoglobin electrophoresis

Answer 27

ANSWER: c. try avoiding milk a. primary lactase deficiency includes congenital and non-persistence of lactase enzyme (most common cause - 85% of black adults have this) d. could also do a breath hydrogen test, but not considered necessary for diagnosing lactose intolerance

Question 28

Why do you treat Salmonella bacteremia in a 2 month old child? a) To eliminate the carrier state b) To decrease the duration of symptoms c) To prevent meningitis d) To eliminate shedding from the GI tract

Answer 28

c) To prevent meningitis Salmonella gastro (not bacteremia) does not need to be treated and is poorly responsive to treatment since the infection is in the enterocytes and antibiotics cannot act on it (also abx prolong excretion/shedding, do not shorten duration of sx and can create chronic carrier state). ABx for immunocompromised and babies <3 months only

Question 29

A 12 yo girl presents with >5 days of fever, unwell. Hepatomegaly. HR 85 despite the temperature of 39.5, BP normal. CBC und urine tests normal. Blood culture shows gram negative bacilli. What is the cause? a) pyelonephritis b) ovarian torsion c) typhoid fever

Answer 29

c) typhoid fever - salmonella enterica typhi Salmonella bacteremia – relative bradycardia is a septic sounding patient GNB – Salmonella typhi

Question 30

A previously well 13-month-old presents with generalized puffiness and lethargy. Urine is negative for protein & blood. Albumin is 13. Initial treatment would include: a. steroids b. IV albumin c. Protein hydrolysate formula d. Lactose free formula

Answer 30

c. Protein hydrolysate formula (ddx of protein losing enteropathy is milk protein allergy) (We had answered IV albumin but I agree with this group that it wouldn't be step one - use if severe ascites or pleural effusions, severe edema)

Question 31

Shwachman-Diamond Syndrome baby. Which vitamin level would be normal? a. Vit A b. Vit e c. Vit B12 d. Vit D

Answer 31

c. Vit B12 - abnormalities: cytopenias (esp neutropenia), exocrine pancreas dysfunction (do not absorb fat soluble vitamins DEAK), bony (short stature, metaphyseal dysostosis, dental anomalies)

Question 32

A 3 mos girl BW SGA at 2200g at GA38, now wt 10th, ht 25th, HC 50 th. Which is true? 1. 2 yr for catch up growth in IUGR 2. f/u in 6 mos no nutritional intervention 3. need w/u for organic FTT 4. increase dietary protein 5. if not at 50 th %ile by 6 months then needs work up for organic FTT

Answer 32

1. 2 yr for catch up growth in IUGR

Question 33

2 year old M suspected of having Celiac disease. What is the most definitive test? a) Jejunal Bx b) Anti-gliadin Ab’s c) Anti tissue transglutaminase Ab’s d) Gluten free diet

Answer 33

a) Jejunal Bx

Question 34

A 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 34

a) Celiac disease Failure to thrive, chronic diarrhea, vomiting, abdominal distention, muscle wasting, anorexia, and irrita­bility are present in most cases. CMPA - would expect bloody stools, less likely FTT

Question 35

Child with FTT, abd distention, wasted limbs and buttocks, irritable. Diagnosed with Celiac disease. List 4 other causes of small intestinal flat villi besides celiac dz.

Answer 35

Other GI conditions - Bacterial overgrowth - Crohn disease - Eosinophilic gastroenteritis - Cow’s milk enteropathy - Small bowel ischemia Autoimmune/immunodeficiencies - Autoimmune enteropathy - Primary immunodeficiency - Graft-versus-host disease - HIV enteropathy - CVID Acquired - Tropical sprue (chronic diarrhea in the tropics, cause unknown ? frequent parasite infections) - Chemotherapy and radiation Food related - Food allergies - Malnutrition Infectious - HIV, giardiasis, TB, post viral, H Pylori

Question 36

Name 3 atypical presentations of celiac disease. List 2 conditions that are associated with celiac disease.

Answer 36

1. dermatitis herpetiformis - iron deficiency anemia, unresponsive to iron supplements - osteoporosis - short stature - arthritis/arthralgia - pathologic fractures - delayed puberty - epilepsy - peripheral neuropathies - anxiety - dental enamel hypoplasia - ecchymosis + petechiae - asymptomatic 2. T1DM - autoimmune thyroid disease - autoimmune hepatitis - Addison - Sjogren - Rheumatoid arthritis - Selective IgA deficiency T21, Williams, Turners

Question 37

Baby with delayed meconium passage, abdo distension and calcifications on AXR a) Hirschprungs b) CF c) duodenal atresia

Answer 37

b) CF - meconium peritonitis: intrauterine rupture of bowel in setting of CF or bowel atresia leads to peritoneal calcifications

Question 38

A 6 year old boy presents to your office with a history of | diarrhea and periorbital edema. He is found to have an albumin of 24 (or 12). Name 4 diagnoses on your differential.

Answer 38

- Inflammatory bowel disease - celiac disease - infectious gastroenteritis - Hypertrophic gastropathy - liver failure - giardiasis - bacterial overgrowth - SLE - nephrotic syndrome

Question 39

A mother brings in her 13-month-old child because he seems to have been getting a diaper rash and diarrhea with the introduction of new foods over the past couple months. 1. arrange for skin testing and RAST 2. carbohydrate intolerance is a common cause 3. it is due to sensitization to cow's milk protein 4. citrus fruits and tomatoes can cause this via an immune-mediated mechanism 5. if mucous is not present in the stool then it cannot be an immune problem

Answer 39

2. carbohydrate intolerance is a common cause Toddler's diarrhea - Loose watery diarrhea, flatulence, abdominal distention and pain · With onset of new foods (fruits)

Question 40

12 month old child who has passed 3 soft red stools in the past 12 hours. He is asymptomatic. The same thing happened 4 months ago but resolved. His Hb is now 70. Investigation? a. Upper endoscopy b. UGI series c. Nuclear med scan for ectopic gastric tissue

Answer 40

c. Nuclear med scan for ectopic gastric tissue - Meckels diverticulum causes 50% of lower GI bleeds in kids under 2 - presents with painless rectal bleeding

Question 41

2-week old child with a 1 week history of stool mixed with blood in an infant? Bottlefeeding well, passed stool in first 24 hours. Most likely dx? a. anal fissure b. cow milk protein allergy enterocolitis c. Meckel’s diverticulum

Answer 41

b. cow milk protein allergy enterocolitis - would say fissure most likely, but ongoing x1 week and to have fissure start at only 1 week of life seems unusual

Question 42

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 d) Bleed from peptic ulcer

Answer 42

b) Meckel’s diverticulum

Question 43

2 week old girl with rectal bleeding. HR 220, BP 60/35, abdomen tense and sensitive. What is the next step in your diagnostic approach: 1. Call general surgery 2. Abdominal ultrasound 3. IV antibiotics 4. Abdominal x-ray 5. Barium swallow with small bowel follow through

Answer 43

4. Abdominal x-ray (first step of the Diagnostic approach) AND call Gen Surgery

Question 44

``` 1 mo old baby with blood mixed in with his stool. Cause? a . Anal fissure b. Cows milk allergy c. Colitis d. Mat blood ```

Answer 44

a . Anal fissure Anal fissure is most common cause of rectal bleeding in kids under age 1; second most common is cow's milk allergy Given it is mixed in poop - CMPA might be a better answer

Question 45

What are 2 surgical complications of HSP

Answer 45

1. intussusception 2. intestinal perforation 3. Bowel ischemia 4. Pancreatitis 5. Cholecystitis 6. Renal failure requiring transplant

Question 46

3 month old boy comes in for routine newborn care. The mother describes symptoms of URTI and mentions that when he has coughed on two recent occasions, she has noticed a swelling protruding from his anus [picture of rectal prolapse]. List the most likely underlying etiology.

Answer 46

- most cases are idiopathic - onset between 1-5 years of age and resolves by 3-5 years of age - ix: rule out CF and sacral nerve root lesions

Question 47

``` An 8 month old baby presents with an abdominal mass, thrombocytopenia, systolic murmur and a murmur over the liver. Ultrasound shows a 3 cm mass in the liver. What would you do? a) DIC work-up b) Bone marrow c) Pulmonary imaging d) Liver function tests ```

Answer 47

a) DIC work-up Kasabach Merritt = hemangioma with consumptive thrombocytopenia, microangiopathic hemolytic anemia, and an acute or chronic consumption coagulopathy associated with tufted angioma and Kaposi hemangioendothelioma

Question 48

Neonate, now two weeks old, with abdominal mass palpable. Has a history of umbilical catheterization. What is the cause of hepatosplenomegaly? a. hepatic hemangioma b. portal vein thrombosis c. hereditary spherocytosis d. congenital CMV e. fungal infection

Answer 48

b. Portal vein thrombosis Rate of venous thrombosis after UVC is 30%!

Question 49

2 month old ex 32 weeker who was ventilated with Ua/Uv lines. Now has HSM but has an otherwise normal physical exam. What is the likely cause? 1. hepatic hemangioma 2. portal vein thrombosis 3. hereditary spherocytosis 4. congenital CMV 5. fungal infection

Answer 49

2. portal vein thrombosis

Question 50

Child with abdo pain, periumbilical, nonradiating. Physical exam normal. Ht and wt on 25%ile. a. chronic constipation b. IBD c. nonfunctional abdo pain d. lactose intolerance e. UTI

Answer 50

a. chronic constipation - this is most likely functional abdominal pain, but picked constipation since that's not a choice * note - ado pain in any location aside from periumbilical is suggestive of potential serious etiology

Question 51

A 5-month-old is brought to the ER because of intermittent abdominal pain for the past 5 weeks. The pain occurs several times daily, during which he screams and extends his arms and legs up simultaneously. The episodes last only seconds, and afterwards he returns to normal. His mother also feels that he has not been himself for the past month and that he doesn't smile as easily. a) colic b) constipation c) epilepsy d) intermittent intussusception e) reflux esophagitis

Answer 51

c) epilepsy concern for infantile spasms - flexion or extension of trunk or extremities sustained for 1-2 seconds; and developmental regression - classic age of onset is 4 months

Question 52

6 year old boy presents with severe abdominal pain and vomiting. On examination his abdomen is tender and he is tense and guarding. What three things do you do for management (3)?

Answer 52

Management of acute abdominal pain and vomiting - NPO - NG inserted to decompress stomach/gut - Surgical consult - IV fluids - Analgesia - Monitor ABCs - Imaging: CXR ± AUS

Question 53

Child had a URTI a week ago. He now presents with bloody diarrhea, abdominal pain and a petechial rash. What is his diagnosis?

Answer 53

HSP: palpable purpura in context of normal platelets plus one of abdo pain, arthritis, IgA deposition on biopsy, renal involvement (hematuria, proteinuria, RBC casts)

Question 54

Kid with abdominal migraine. Family history of migraines. What are two periodic syndromes associated with migraine development as an adult.

Answer 54

1. cyclic vomiting 2. abdominal migraine 3. recurrent abdominal pain 4. recurrent vomiting

Question 55

A 13 year old girl is seen with abdominal pain of 24 hour duration that she now rates as an 8/10. She has a fever. On examination she has a diffusely tender abdomen and is guarding. You call general surgery to consult. In the meantime, what is your priority in management: a. Blood C&S and antibiotics b. IV fluids and analgesia c. IV fluids and CT abd

Answer 55

b. IV fluids and analgesia

Question 56

14 yo black boy with one year history of post-prandial intermittent abdominal pains. Normal stools. No bloating. No vomiting. What test do you do? 1. hemoglobin electrophoresis 2. Hydrogen breath test 3. Gastroscopy with biopsy 4. UGI/SBFT 5. Abdo ultrasound

Answer 56

3. Gastroscopy with biopsy First line test for suspected H. pylori. This would fit with recurrent gastritis, especially if he is a new Canadian. NASPGHAN does not routinely recommend Urea breath test in children, as it has many false positives and culture and sensitivity is recommended to guide treatment. - If there was diarrhea - then do hydrogen breath test for lactose intolerance

Question 57

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 57

b) cholecystitis Fitz-Hugh-Curtis: occurs in setting of PID - inflammation of liver capsule and peritoneal surfaces - minimal hepatic cell involvement.

Question 58

Sickle cell girl who is obese, RUQ pain, increased liver enzymes, had been treated and recovers from the event. What do you recommend? a. cholecystectomy b. low-fat diet c. ursodiol

Answer 58

a. cholecystectomy

Question 59

1 m baby irritable, not feeding well, vomited once, distended abdomen. On exam VSS except mild tachycardia. There is a mass in the right scrotal, firm and non reducible. Does not transilluminate. What is the next step? a. Ultrasound b. Urgent surgical consultation c. Nuclear scan d. Testicular Doppler

Answer 59

b. Urgent surgical consultation

Question 60

A 10 yo boy has headache, abdominal pain and lethargy anytime of the day. He has missed 30 days of school in 4 months. He has: a) migraines b) school phobia c) Brain tumour

Answer 60

school phobia or migraine Not enough info here.

Question 61

A 6 year old boy with severe abdo pain 8/10, febrile, tachycardic at 160. Abdomen is rigid. As you call surgeons, what do you do? a) Culture and start antibiotics b) Bolus with normal saline and give morphine for analgesia c) Bolus with normal saline and arrange for urgent CT

Answer 61

b) Bolus with normal saline and give morphine for analgesia Fluid resuscitation immediately (this patient is in shock), then culture and antibiotics

Question 62

Lebanese girl with abdominal pain and fever for one day, recurrent episodes. During an episode you do some labs and find increased ESR, increased CRP and decreased albumin. What is a likely diagnosis? a) paroxysmal nocturnal hemoglobinuria b) familial mediterranean fever

Answer 62

b) familial mediterranean fever 1-3 days every 4-8 weeks Recurrent episodes of peritonitis, inflammation and fever PNH.- rare, manifests as complement mediated hemolytic anemia - bone marrow failure and thrombosis (get abdo venous thrombosis with recurrent abdo pain)

Question 63

What is the most specific test for pancreatitis: 1. Amylase 2. Lipase 3. Abdo U/S 4. Abdo CT scan 5. AXR

Answer 63

2. Lipase Dx of pancreatitis (⅔): - Acute onset persistent severe epigastric pain radiating to back - Elevation of lipase >3x ULN or amylase - Characteristic findings on imaging Lipase has highest sensitivity and specificity

Question 64

Kid with peptic ulcer. His treatment would likely include a. amoxil +clarithro + PPI b. clinda and clarithro c. flagyl and tobra

Answer 64

a. amoxil +clarithro + PPI other tx options: ▪ Amox + Metronidazole + PPI ▪ Clarithromycin + Metronidazole + PPI

Question 65

A 10 month old child presents to your emergency department with a history of intermittent irritability and vomiting interspersed with periods of looking relatively well. The episodes are coming closer and closer together in time. You diagnose intussusception. What are 3 contraindications to air enema? What are 2 situations in which air enema is unlikely to work?

Answer 65

``` 1. What are 3 contraindications to air enema? Refractory shock Necrosis/Perforation Peritonitis Prolonged Intussusception Pneumatosis ``` 2. What are 2 situations in which air enema is unlikely to work? - Multiple recurrences - Ilealileal (HSP) - Pathologic lead points: Meckel’s, lymphoma, polyp, intestinal duplication cysts, PTLD tissue, hemangioma, neurofibroma, hamartomas, leiomyomas

Question 66

Patients with inflammatory bowel disease lose weight due to: a) inadequate nutrient intake b) increased metabolic demands c) malabsorption d) drug-nutrient interactions

Answer 66

a) inadequate nutrient intake - patients develop food avoidance because eating causes pain and diarrhea; also pro inflammatory cytokines cause anorexia

Question 67

Teenager presents with 3 year chronic history of intermittent abdominal pain and bloody stools and also arthritis. His height and weight are less than the 5%. 1. What is his diagnosis? 2. What are two skin complications from his disease?

Answer 67

1. IBD - most likely Crohn's (more likely to present with FTT) 2. Erythema nodosum, psoriasis, pyoderma gangrenosum

Question 68

Teenager with weight loss and perianal abscess. a. ) Dx? b. ) Child had perianal abscess. Management x2 lines.

Answer 68

a. IBD (more likely Crohn’s as perianal disease more common in Crohn’s than UC) b. I+D and seton placement, - good perianal hygiene - Consider antibiotics (Amox-clav or clinda-gent or ancef/flagyl) - treatment of underlying condition (steroids) - topical tacrolimus (may be helpful in IBD)

Question 69

Teenage girl with a history of ulcerative colitis, status post total colectomy. Had a “J- pouch” and an anal pull-through ostomy that was later removed. Did have an ileostomy, but that was closed following an end-to-end anastomosis. Now she presents with a 5-day history of bloody diarrhea 8 times per day. Stool cultures are all negative. What is the diagnosis? What 1 treatment would be the best?

Answer 69

1. pouchitis 2. treatment: oral metronidazole or ciprofloxacin major complication of surgical repair = pouchitis, a chronic inflammatory reaction in the pouch - presents with diarrhea, abdo pain, occasional low grade fever more common when pouch is created in setting of UC relative to other disease processes - mgmt: flagyl, ciprofloxacin - probiotics may decrease rate

Question 70

Kid with Crohn’s. Stomach aches in morning, gone in afternoon. Crohn’s is under control. Missed 30 days of school in past two months. 3 points to management.

Answer 70

- reassurance of family that CD is under control and review of symptoms that would indicate a flare - parents should avoid reinforcing symptoms that result in secondary gain - patient should return to school and regular activities - cognitive behavioural therapy may be beneficial

Question 71

Crohns patient who has failed prednisone management. Started on azathioprine. Comes back 2 weeks later. Abdominal pain, periumbical. Vomiting. Give 2 most likely causes.

Answer 71

Medication related: - N/V are known side effects of this medication (especially if fast initiation) - hepatitis - pancreatitis Crohn's related: - stricture - intra-abdominal abscess

Question 72

Boy has had inflamed, ulcerated lips and gums. (Picture shown). Which of the following conditions is this more likely to be seen with? a. Chronic mycoplasma infection b. IBD c. Eosinophilic gastritis d. Contact dermatitis

Answer 72

b. IBD Crohns (Gum to Bum)

Question 73

5 yo with bloody stools. Hyperpigmented lesions on lip and mucosa. Diagnosed with intussusception. What is underlying cause? a. HSP b. Peutz-Jehgers c. Langerhans histiocytosis

Answer 73

b. Peutz-Jehgers - Type of Inherited Colorectal Cancer Syndrome (Autosomal dominant but rare) - Key: mucocutaneous pigmentation + extensive GI hamartomas polyposis

Question 74

Kid with crohn’s on sulfasalazine and has hairless patch with slight scale. a. alopecia areata b. trichotillomania c. tinea capitus

Answer 74

a. alopecia areata | - AI disorder, seen in IBD; treated with steroids, methotrexate or sulfasalazine

Question 75

``` 14 yo boy with R lower quadrant abdo pain, wt loss and diarrhea. RLQ mass palpable and anal fissure noted. 1. What is the most likely diagnosis? 2. List 2 diagnostic tests to confirm diagnosis. 3. List 3 acute complications of this disease. ```

Answer 75

1. Dx: Crohn’s 2. Ix: Upper endoscopy and colonoscopy with biopsy + UGI with SBFT or Magnetic Resonance Enterography 3. thrombosis/stroke, perianal fissure and fistula, bowel perforation, bowel obstruction, intra-abdominal and retroperitoneal abscess, pelvic osteomyelitis, pancreatitis

Question 76

A 16 year old boy with a known diagnosis of ulcerative colitis comes to see you in your office with his mother. He and his mother have been doing some research and are concerned about the risk of adenocarcinoma in UC. Name 2 risk factors for adenocarcinoma in the setting of a diagnosis of UC.

Answer 76

1. Duration of disease (increases after 8-10 years) 2. Pancolitis 3. Primary sclerosing cholangitis 4. Family history 5. Mucosal dysplasia

Question 77

2 life threatening complications of ulcerative colitis

Answer 77

1. toxic megacolon 2. bowel perforation 3. colorectal cancer

Question 78

1 year old with rectal abscess. Mgmt? a. Systemic abx b. Excision by surgery c. I and D

Answer 78

c. I and D (note: can just do nothing) | - healthy infant - can just watch and wait; sitz baths, pain management

Question 79

Which is most characteristic of encopresis: a. usually in morning b. dilated bowel c. mostly in preschool children d. stool softeners often cure the problem

Answer 79

b. dilated bowel

Question 80

3 month old boy with constipation since birth, FTT, mildly distended abdomen and no stool on rectal examination. What would be the most appropriate next test: a. rectal biopsy b. TSH c. Sweat chloride d. follow up in 6 months

Answer 80

a. rectal biopsy True constipation in the neonatal period is most likely secondary to Hirschsprung disease, intestinal pseudoobstruction, or hypothyroidism

Question 81

A child is referred for refractory encopresis. Spine exam and DTR’s are normal. The rectal ampulla is dilated with lots of stool. How should he now be treated: a) fecal emollient b) peristaltic agent c) high fibre diet d) bowel washouts e) encourage regular exercise

Answer 81

d) bowel washouts ``` fecal emollient (laxatives that incorporate more water and fat into the stool and make it pass through easier - examples include docusate) b) peristaltic agent (act on the intestinal mucosa or nerve plexus and stimulate peristalsis - examples include senna and bisacodyl) ```

Question 82

False negatives for sweat chloride a. hypoalbuminemia b. hyponatremia c. hypercalcemia

Answer 82

a. hypoalbuminemia | they are edematous

Question 83

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 83

c. sweat chloride

Question 84

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

Answer 84

c) pharmacologic and behavioural

Question 85

A 3-week-old infant has jaundice. BW 3250g, now 3490g. Breastfeeding well. Hemoglobin 127, bilirubin 270, Coombs negative. What should be done: a. stop breastfeeding and start formula feeding b. come back in 1 week for reassessment c. do further tests including liver enzymes d. refer to a gastroenterologist

Answer 85

b. come back in 1 week for reassessment Breast milk jaundice Consider: c. do further tests including liver enzymes Ham Review - Breast milk jaundice max Tbili 220 (depends on what the "further testing" included)

Question 86

A 3 week old who is brought to the office because mother thinks he is too yellow. Breastfed. Otherwise well. Total bili is 180. Direct is 8. What do you do? A) septic workup B) investigate for blood group incompatibility C) reassure mother that condition may last for 4-12 weeks D) investigate for metabolic disease

Answer 86

C) reassure mother that condition may last for 4-12 weeks

Question 87

You are called from a family doctor about a 5 day old jaundiced baby. Well looking. Total bili is 355. He is breastfed. What do you recommend: ``` A) septic work up B) investigate for a metabolic disease C) coombs test and hg D) start phototherapy E) follow up in 1 week ```

Answer 87

C) coombs test and hg - yes, you should also start photo but you need to know if RF for severe (ie. needing exchange transfusion or IVIG)

Question 88

A newborn infant was an unconjugated bilirubin of 310 at 72 hours of age. Breastfeeding is improving. Looks well, has lost 8% of birthweight. What should be done: a) stop breastfeeding and start phototherapy b) stop breastfeeding and repeat bilirubin q4h to decide on phototherapy c) continue breastfeeding, start phototherapy, and check for ABO incompatibility d) start phototherapy and work-up for metabolic disease e) recommend exchange transfusion as bilirubin level is likely to be in exchange range in 12 hours

Answer 88

c) continue breastfeeding, start phototherapy, and check for ABO incompatibility

Question 89

Unconjugated hyperbilirubinemia can be caused by all EXCEPT: a) decreased conjugation b) increased uptake by hepatocytes c) increased excretion d) increased enterohepatic circulation e) increased breakdown of hemoglobin

Answer 89

b) increased uptake by hepatocytes inefficient uptake of bill by hepatocytes (reduced blood flow, drugs) can cause hyperbili

Question 90

A 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. Hbg 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) vaso-occlusive disease of the liver

Answer 90

a) Fitz-Hugh-Curtis b) cholecystitis Depends on additional info in prompt, but one of these 2

Question 91

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

Answer 91

b. breastmilk jaundice

Question 92

2 month old M with poor growth, jaundice and conjugated hyperbilirubinemia. Best initial imaging study. a. CT abdo b. MRI c. U/S w doppler flow studies d. Nuclear med biliary excretion scan

Answer 92

c. U/S w doppler flow studies Start with US. If not clear, consider HIDA.

Question 93

What is the most common presentation of alpha 1 antitrypsin in children? a) Jaundice b) Emphysema c) Bronchiectasis d) Cirrhosis e) Pneumothorax

Answer 93

a) Jaundice Presents with GI issues in kids, lung issues don't manifest until late teens/adulthood 11% homozygous will have neonatal cholestasis w/ jaundice

Question 94

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

Answer 94

b) posterior embryotoxon (film/membrane around eye)

Question 95

A photograph is shown of Kayser-Fleischer rings in a 14-year-old boy. What should be evaluated in this patient: a) liver function b) renal function c) blood triglyceride levels d) skeletal abnormalities e) joint abnormalities

Answer 95

a) liver function Wilson's disease - degenerative changes in the brain, liver disease, and Kayser-Fleischer rings in the cornea (brown-is rings) Clinical features: unexplained neuro, hepatic or psych symptoms or FHx

Question 96

Child with fulminant liver failure. Three reasons for encephalopathy

Answer 96

- increased serum levels of ammonia - presence of false neurotransmitters (competitive inhibition from false NT that are created by a.a. metabolism) - increased GABA receptor activity - increased circulating levels of endogenous benzodiazepine-like compounds

Question 97

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

Answer 97

b) associated with splenomegaly Splenomegaly is 2nd most common finding in children with portal HTN (hemorrhage being the other) ``` Causes ▪ Extrahepatic portal vein obstruction ● Omphalitis with vein infection ● UVC- thrombosis ● Peritonitis/ Appendicitis ● IBD ● Splenic vein thrombosis ``` ``` ▪ Intrahepatic ● Cirrhosis ● Hepatitis ● Fibrosis ● Malignancy ● Granulomatous ```

Question 98

You are admitting a child to hospital with newly diagnosed liver failure. You are concerned about the possibility of portal hypertension. Name 2 features on physical examination that suggest portal hypertension.

Answer 98

``` - Pallor ▪ Caput Medusa ▪ Splenomegaly - Ascites - Growth impairment - Spider nevi - Clubbing - Dyspnea - Jaundice - Palmar erythema ```

Question 99

A 13 y.o. with mild jaundice and fatigue. Lab results: AST=222; ALT=150 (increased); Bilirubin 35/20; alpha-1-antitrypsin=2.5 g/l (normal); hepatitis B surface antigen negative; hepatitis C IgM negative. The most likely diagnosis: a. autoimmune hepatitis b. hepatitis C c. Wilson’s disease d. sclerosing cholangitis e. alpha-1-antitrypsin disease

Answer 99

Not enough info c. Wilson Disease Previous Group a. autoimmune hepatitis - insidious onset - fatigue, malaise, anorexia - aminotransferases often range from 100-300 (but can be over 1000 in symptomatic patients) - bili can be normal or elevated - ALP and GGT normal to slightly increased - low albumin - prolonged PTT, INR - normochromic, normocytic anemia, leukopenia and TCP

Question 100

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. ALP

Answer 100

b. INR - INR most useful test for liver synthetic function; focus of monitoring in hepatitis is for liver injury (altered synthetic function is most important indicator of liver injury) - rapidly falling AST/ALT but rising GGT and bill can be poor prognosis - fall in AST/ALT usually falls behind fall in bilirubin

Question 101

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

Answer 101

d. Autoimmune ( associated with anti-smooth muscle antibody ) - work-up should include: o R/O viral serologies, alpha1 antitrypsin and wilsons, celiac, IBD, PSC (MR choliangiography)

Question 102

A 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

Answer 102

B) amox, clarithro and proton pump inhibitor - treatment: eradication is necessary even in asymptomatic patients - amox, clarithro and PPI - amox, flagyl and PPI - clarithro, flagyl and PPI - antibiotics are taken for 14 days, PPI for 1 month

Question 103

Competitive sports and nutrition in pediatrics - which is true? 1. give high carbo drinks 2. give protein supplements 3. provide a well balanced diet 4. give Calcium and vit D supplements 5. change child's diet according to the sport in which they're involved.

Answer 103

3. provide a well balanced diet A well balanced diet containing appropriate amounts of macronutrients (protein, carbs, and fat) and micronutrients (vitamins and minerals) is essential to provide enough energy for growth and activity. Fluids are also essential for hydration to support growth and athletic performance

Question 104

28 wk prem now 28 days old and on oxygen for CLD. On 150 cc/kg/d EBM by gavage, also on Fe, aldactazide, vit E and fluticasone. Calculate the caloric intake, what should his caloric intake be? What 2 ways do you recommend optimizing nutrition.

Answer 104

- 150ml/kg/day x 1 ounce/30 mls = 5 ounces/kg/day x 20kcal/1 ounce = 100kcal/kg/day - energy requirements for growth in BPD patients are 130-150kcal/kg/day - can use fortified human milk or fortified preterm formula to increase caloric density as high as 30kcal/ounce - diuretics in patients with BPD cause loss of Na, K, Cl and Ca in the urine - recommend supplementation with KCl and NaCl to maintain normal levels - ensure normocalcemia to prevent osteopenia of prematurity

Question 105

You work north of 55 th parallel. What do you counsel mothers regarding vit D in an 8 month old bottle fed baby? a. 400 units all year long b. 400 units in winter c. 800 units all year long d. No supplementation necessary

Answer 105

b. 400 units in winter (assume already getting 400IU for Vit D fortified formula, so need to top up to 800 in winter

Question 106

A mother comes in with her newborn baby and tells you that she lives out in the country where they have well water. What should she do about vitamins and fluoride? What do you tell her about each (be specific) (2)

Answer 106

Vitamins- supplement with Vit D 400IU if breastfeeding (no need if formula) - If the fluoride concentration in the well water is <0.3ppm then starting at 6 months of age teeth should be brushed twice daily; if this does not happen then supplemental fluoride should be given (best preparations have topical effect - mouthwash, lozenges or drops (dilute drops in water before squirting on teeth))

Question 107

Phytoestrogens in soy contraindicated for: a) all males b) immune deficiency c) galactosemia d) congenital hypothyroidism

Answer 107

d) congenital hypothyroidism -soy can interfere with uptake of thyroid hormone and prolongs increase in TSH Note: galactosemia is the only condition in which soy formula is indicated

Question 108

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

Answer 108

a. breast milk ``` Breast Milk (optimal macronutrient composition and trophic factors to foster adaptation, immunoglobulins to enhance barrier function, hypoallergenic, low osmotic load) ▪ Extensively hydrolyzed with MCT-enriched formula is also good if no EBM ```

Question 109

An otherwise well 6-month-old presents with a one month history of passing small hard stools once per week. She is exclusively breast-fed. Appropriate management would include: a. careful use of laxatives b. enema c. change to lactose free formula d. introduce solids e. change to iron free formula

Answer 109

a. careful use of laxatives

Question 110

A 1 week old breastfed baby comes into your office for newborn care. There is bright red blood mixed in with the stool. The baby did not have any bleeding or bruising with the Vitamin K injection at birth or with the newborn screen. The anus appears normal and there is no evidence of a fissure. List the most likely cause of the blood in the stool.

Answer 110

- milk protein allergy/intolerance | note: swallowed maternal blood common, but does not usually present with BRBPR

Question 111

A 3-week-old infant is going to be formula-fed, and you suggest an iron-fortified formula. The mother asks you what problems she should expect. How do you counsel her: a. colic b. constipation c. black stool d. no problems

Answer 111

d. no problems * key is that there is no evidence that iron fortified formula causes constipation, colic, GI cramping, fussiness or reflux

Question 112

Absolute contraindications of breastfeeding for the infant. a. Hep B b. Bilateral mastitis c. Active TB

Answer 112

c. Active TB | Active TB contraindication until completed 2 weeks of treatment

Question 113

Describe 3 maternal infections that represent absolute contraindications to breast-feeding.

Answer 113

- HIV - Human T-cell lymphotrophic virus type I and II (HTLV) - HSV lesions that cannot be covered - active untreated TB until completed 2 weeks of treatment - can give EBM, just don't breastfeed - untreated maternal brucellosis

Question 114

A new mother is hoping to breastfeed. She has been on paroxetine for her depression and was recently started on propranolol for her hypertension. Which of the following is true: a. Neither of these medications is a contra-indication to breast-feeding b. The propranolol should be changed to another anti-hypertensive before breast-feeding c. The paroxetine should be discontinued before breast-feeding

Answer 114

a. Neither of these medications is a contra-indication to breast-feeding -may be excreted in breast milk but no evidence that the presence of these medications will cause harm to baby Some contraindications to BF: ● High dose metronidazole (d/c for 12-24h to allow excretion of dose) ▪ Mothers on cytotoxic chemo ▪ Radioactive isotopes or radiation therapy ▪ Infants w/ classic galactosemia (need soy formula)

Question 115

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

Answer 115

b. start iron supplementation at one month of age | - supplement from 1 month until 12 months

Question 116

Contraindication to breastfeeding: a. vegan mother b. erythromycin c. prednisone d. alcohol e. active tuberculosis

Answer 116

e. active tuberculosis - no BF until 2 weeks of treatment completed; can still give EBM - re vegan diet: not a problem but mom may need supplementation (B12, Vit D, Fe) - if mom's levels are fine then it's all good

Question 117

At a routine well-baby visit, a mother tells you that she has been mixing honey into her baby's formula to make it more sweet. You advise her that her child: a. does not need the excess calories b. is at risk for Clostridium botulinum c. is at risk for E. coli d. is at increased risk for allergies e. is at increased risk for dental caries

Answer 117

b. is at risk for Clostridium botulinum - no honey until 1 year of age - immature digestive system cannot get rid of spores from c botulinum - once 1 year old, able to get rid of them so risk decreased

Question 118

A vegan mother comes to you and is hoping to breastfeed her baby. Which of the following vitamins do recommend supplementation with: a. iron b. vitamin B12 c. vitamin C d. folate

Answer 118

b. vitamin B12 if she is breastfeeding | and vitamin D

Question 119

What nutrient is vegan diet deficient in?

Answer 119

Vitamin B12

Question 120

What nutritional deficiency is a 12 year old vegan child at risk for?

Answer 120

B12 deficiency

Question 121

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 121

e. iron

Question 122

Breastfed 7 month old babe of a vegan mom. Thriving. What do you supplement baby with? a. Vitamin B12 b. Folic acid c. calcium d. iron

Answer 122

a. Vitamin B12 CPS statement does recommend supplementing baby with B12 - 0.4mcg/kg/day

Question 123

Mom feeding goat’s milk to a 2 month old baby. What do you need to supplement with? a. Ca b. Fe c. Folate d. multivitamin

Answer 123

c. Folate Goat milk has been shown to cause significant electrolyte disturbances and anemia because it has low folic acid concentrations; goats milk is high in sodium and protein

Question 124

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 124

a. elemental formula with MCT | * remember if it's an option, breast milk is the most ideal form of feeding

Question 125

Neonate on TPN. List four things that will increase his incidence of cholestasis.

Answer 125

- lower birthweight (especially <1000g) - Prematurity - Sepsis - NEC - longer duration of therapy (especially >2 weeks) - short bowel syndrome - Respiratory distress - Lipid therapy (intralipid worse than smof) - Prolonged fasting

Question 126

TPN – list four metabolic complications

Answer 126

- hypertriglyceridemia - cholestasis - hyperglycemia - hypokalemia

Question 127

A 1 mo baby with FTT and diarrhea. WBC 5 x10 9 (80% neutrophils, 10% lymphocytes, 6% basophils, 4% eosinophils). a. ) What does this make you think of? (i.e. interpret the labs) b. ) List 2 causes

Answer 127

a.) What does this make you think of? Primary Immunodeficiency b.) List 2 causes SCID WAS Sepsis

Question 128

``` 27 mos female, bone age is 17 mos, short stature. Ht and wt graphs provided show N bw and length and a gradual fall to below the 3 rd percentile for wt A) constitutional B) familial short stature C) hCG deficiency D) malnutrition ```

Answer 128

A) constitutional - so long as continues to follow the curve and weight loss is not > height loss

Question 129

14 mos male, FTT, vomiting, met acidosis, pH 7.31, bicarb 14, K 3.5, Na140, Cl 118, urine pH 6.3 a. distal RTA b. Bartters c. organic acidopathy d. nutrit. deprivation

Answer 129

Interpretation = Non anion gap metabolic acidosis with alkalotic urine. a. distal RTA - kidneys pushing out less H+ so urine is alkalotic (pH>5.5) - Bartter get hypoK metabolic alkalosis and dysmorphic - organic acidopathy: high AG metabolic acidosis

Question 130

Changing to the WHO growth will result in what when looking at the growth of breast fed babies?

Answer 130

WHO charts are based on breast fed babies so they will look normal; formula fed babies will look FTT in the first 6 months because formula fed babies grow slower than BF babies in the first 6 months, and then catch up after (CDC curves were based on North American babies who are more likely to be formula fed)

Question 131

3 yo only gained 2 kg last year a. reassure b. consult dietician for intake c. malabsorption work-up

Answer 131

a. reassure Increases of approximately 2 kg (4-5 lb) in weight and 7-8 cm (2-3 in) in height per year (from age 2 years until puberty)

Question 132

A 13 year old girl comes to you with a BMI of 31. Which of the following is she at risk for? a. type 2 diabetes mellitus b. delayed onset of menarche c. AVN of the femoral head d. Pancreatitis

Answer 132

a. type 2 diabetes mellitus Obesity a/w Early Menarch, SCFE (not AVN) Most pancreatitis is idiopathic, however obesity can put at increased risk for gall stones

Question 133

You are counseling the mother of a child who is significantly overweight about her child’s diet and exercise habits. a. What BMI is considered overweight for a child? b. What BMI is considered obese for a child?

Answer 133

a. overweight: BMI 85-95th percentile | b. obese: BMI >95th percentile

Question 134

Features of childhood obesity include all EXCEPT: a. 50% chance of being obese if one parent is obese, 80% if both parents are obese b. premorbid psychological problems predate the onset of obesity c. increased incidence in lower socioeconomic class d. associated with decreased exercise

Answer 134

b. premorbid psychological problems predate the onset of obesity - lower SES associated with obesity in developed countries, but failure to thrive in developing countries; high SES in developing countries is associated with obesity (if you're rich in a poor country you're fat; if you're poor in a rich country you're fat)

Question 135

Adopted kid from caribbean with edema, abdominal distention, yellow tinged rusty hair. What is the most likely? a. Kwashiorkor b. Marasmus c. B1 deficiency d. B6 deficiency e. zinc deficiency f. nephrotic syndrome

Answer 135

a. Kwashiorkor Edematous acute malnutrition (protein deficiency) * marasmus get severe wasting with scaphoid abdomen * B1 deficiency (thiamine) - leads to beriberi (peripheral neuropathy) * B6 = pyridoxine - goat milk deficient in B6 - seizures, FTT

Question 136

Mom brings 10mo son from another country. Worried re: malnutrition. Baby has thick costochondral junctions. a) Diagnosis? b) List 2 physical findings that would support the diagnosis

Answer 136

a) Diagnosis: Vitamin D deficiency rickets b) List 2 physical findings: - craniotabes - short stature - enlargement of wrists or ankles - frontal bossing - protruding abdomen - FTT - sternal groove

Question 137

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

Answer 137

d. Zinc deficiency - zinc in breast milk sufficient until 7 months of age, then infants need to get it from solid food diet - this could specifically be acrodermatitis enteropathica - AR disorder with zinc deficiency - get findings in question

Question 138

All are features of iron deficiency anemia EXCEPT: a. Pica b. Koilonychia c. cheilitis d. mild scleral icterus e. psychomotor retardation

Answer 138

d. mild scleral icterus - would be more in hemolytic b. Koilonychia = spoon nails c. cheilitis (angular cheilitis= inflammation at corners of mouth) e. psychomotor retardation (yes - delayed psychomotor development, irreversible behavioural and IQ deficiency)

Question 139

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 (B1) b. vitamin C c. vitamin B12 d. riboflavin (B2) e. folate

Answer 139

c. vitamin B12 - A, D and B12 store in large amounts in liver, B12 last years Vit C- one month of no VitC and you develop scurvy Folate/Thiamine - weeks before deficient

Question 140

The reason maxeran (metaclopramide) is not used commonly is: a. side effects b. cost c. interactions with other drugs

Answer 140

a. side effects increased risk in younger patients of dystonic reactions/extrapyramidal symptoms

Question 141

Fat absorption in childhood: a. fats are exclusively digested by pancreatic lipase b. medium-chain triglycerides require solubilization c. triglycerides require tryptic predigestion d. triglycerides require hydrolysis to free fatty acids e. lipolysis is enhanced by gastric acid

Answer 141

d. triglycerides require hydrolysis to free fatty acids Long chain Triglycerides are broken down into glycerol and FFA by lipase. o almost all dietary fat absorbed IN PROXIMAL JEJUNUM Trypsin is important for protein digestion, not fat. MCTs absorbed passively from GI tract (Why they're good for kids with fat malabsorption syndromes)

Question 142

Meckles Rules of 2s

Answer 142

- Most present before 2 yrs - 2% of the population - Male: female 2 (-4):1 - Within 2 feet of ileocecal valve - 2 inches in length - 2 types of heterotopic mucosa (gastric, pancreatic)

Question 143

Stuff that's absorbed in Duodenum and Proximal Jejunum

Answer 143

``` Ca++ Mg++ Phos Iron Folic Acid ```

Question 144

Stuff that's absorbed in Distal ileum

Answer 144

B12 Bile acids Because they start with B... so they're absorbed closer to your butthole.

Question 145

A mother is concerned about her 6-year-old son who has been having 2 or 3 loose bowel movements per day for the past year. He usually has to change his underwear at school. He does not have abdominal pain, vomiting, or rectal bleeding. Growth is normal. When you examine the child he is shy but cooperative. Physical exam is normal except for a very dirty anus and a fecaloma on digital rectal examination. What investigations are necessary: a) TSH b) stool culture c) rectal biopsy d) psychology consult e) no investigations

Answer 145

e) no investigations

Question 146

6 yo girl with Greek background has severe abdominal pain with fever of 40. Her respiratory rate is 44, HR 160 and BP 100/50. She has RUQ pain on exam but no guarding with muscles. WBC is 40. Her ultrasound of liver is normal. Liver and renal function are normal. What is her diagnosis? First episode FMF Pneumonia Acute cholecystitis Bornholm's (Pleurodynia)

Answer 146

Pneumonia FMF - would expect sterile peritonitis (serositis) Pleurodynia - Coxsakie, Echovirus. Paroxysmal thoracic pain due to myositis involving chest and abdo wall muscles. ± Pleural friction rub - WBC seems too high for this.

Question 147

3 mo baby with constipation and failure to thrive. What test to do? TSH Anal manometry Sweat chloride

Answer 147

TSH - Most infants with congenital hypothyroidism are asymptomatic at birth - The anterior and posterior fontanels are open widely; observation of this sign at birth can serve as an initial clue to the early recognition of congenital hypothyroidism - There may be constipation that does not usually respond to treatment. The abdomen is large, and an umbilical hernia is usually present

Question 148

Etiology of functional abdominal pain. a. Enhanced visceral sensitivity and increased mobility of gut b. Migraine headache and vasospasm of SMA c. Lactose intolerance

Answer 148

a. Enhanced visceral sensitivity and increased mobility of gut

Question 149

``` Child with constipation. How long do you treat with PEG3350? A) A few days B) 1 year C) 6 mths D) till he has one soft stool daily ```

Answer 149

6 months

Question 150

``` 15 year old wants to become vegan. What is the best advice to meet her nutritional needs? B) Start folic acid supplementation C) Start iron supplementation D) Start vitamin B12 supplementation E) Start Calcium supplement F) See a nutritionist/dietician ```

Answer 150

F) See a nutritionist/dietician

Question 151

A mom brings in her toddler because she is concerned he is not eating well. He refuses many foods but is thriving. What do you tell her? A) Offer a variety of foods at each meal including something he likes and let him decide what he wants to eat B) Give him plenty of snacks between meals

Answer 151

A) Offer a variety of foods at each meal including something he likes and let him decide what he wants to eat

Question 152

3 mo. with 1 mo. history of progressive regurgitation, most recently projectile vomiting, last two episodes were bilious. Kid looks unwell, eyes sunken. What’s the diagnostic test? A. Abdominal ultrasound B. AXR C. Upper GI series D. Contrast enema

Answer 152

First Test = AXR Best test = Upper GI

Question 153

C diff confirmed in child with mild diarrhea after being on antibiotics. What is the next step in management after stopping the offending antibiotics? A) Supportive B) Oral metronidazole C) IV vancomycin D) Cefalexin

Answer 153

A) Supportive → mild illness: watery diarrhea without systemic toxicity; typically < 4 abn BM/day Initial Moderate - >4 stools/day, no systemic toxicity = Metronidazole 30 mg/kg/day ÷ QID for 10-14 days Initial Severe - systemically unwell: Vancomycin 40 mg/kg/day ÷ QID PO for 10-14 days Severe + Complicated (colitis, hypotension, shock) - add IV metronidazole

Question 154

2-year-old child with nausea and vomiting for a few days, being given only free water. Serum sodium 108, cl 85, elevated urea and creatinine, low urine output. How best to manage? A) Correct with NS to 138-140 over 24 hours B) Treat with 3% NS with an aim to normalize sodium over 4-6 hours C) Correct with NS to 118-120 over 24 hours D) Fluid restrict

Answer 154

C) Correct with NS to 118-120 over 24 hours Correct Na by 8-12 in 24h

Question 155

7-year-old kid post-appendectomy. How do you best manage pain? B) IV ketorolac C) rectal Tylenol D) IV morphine

Answer 155

D) IV morphine

Question 156

You're asked to investigate a child for Celiac. His TTG is low. What do you do next? B) no further investigation required C) Biopsy D) IgA

Answer 156

IgA | should have done this with your TTG

Question 157

A boy presents with epigastric pain after meals. His lipase is 1000. What is your management? B) Surgical consult C) IV antibiotics D) CT scan E) NPO and IV fluids

Answer 157

E) NPO and IV Fluids Analgesia Fluid/electrolytes NG useful in patients who are vomiting NPO while vomiting Refeeding as soon as vomiting has resolved→ early refeeding by nasogastric tube or on demand decreases the complication rate and length of stay severe pancreatitis → antibiotics are used to treat infected necrosis but prophylactic antibiotics are not recommended

Question 158

A child presents with 4 episodes of vomiting, 2 requiring IV fluids over the last 15 months. Between episodes she is other well, growing and thriving, with a normal examination. What do you do? A) neuro imaging B) supportive care C) refer to GI

Answer 158

B) Supportive care Sounds like cyclic vomiting syndrome

Question 159

A mother delivers prematurely. If her breast milk were to be compared to that of a woman who delivers at term, it would be found to be: A) Higher in lactose C) Higher in lipids D) The same E) Higher in protein

Answer 159

E) Higher in protein - Prem milk higher in protein, sodium and chloride. Lower lactose and lipids.

Question 160

A 12 year old boy presents with progressive dysphagia. He is known for severe GERD. However, he now must drink large quantities of liquids to swallow solids. There are no particular trigger foods or solids that are worse than others. Which of the following will be diagnostic? A) Allergy skin testing B) Upper GI studies C) CT chest D) Upper endoscopy with esophageal biopsies

Answer 160

D) Upper endoscopy with esophageal Bx Sounds like EoE - differentiated from GERD by lack of erosive esophagitis and ++ eosinophilia

Question 161

Which of the following patients requires 800 IU daily of vitamin D supplementation during the winter? A) A breastfed Aboriginal baby B) A breastfed baby whose mother is vegetarian C) A baby who is exclusively formula fed D) A premature baby

Answer 161

A) A breastfed Aboriginal baby Prems: 200-400 IU/kg/day 800 for those above 55th parallel including northern native communities during the winter months

Question 162

In a patient with hepatic disease, what is the most specific sign concerning for acute, impending hepatic failure? 1. Rapid decrease in the liver span 2. Increase in the liver span 3. Decreased LOC 4. Anorexia

Answer 162

3. Decreased LOC ``` Sx of fulminant hepatic failure: Progressive jaundice fetor hepaticus fever, anorexia Vomiting abdominal pain A rapid decrease in liver size without clinical improvement is an ominous sign hemorrhagic diathesis and ascites can develop ```

Question 163

A 9 year old girl presents to your clinic with swelling of her face, lower limbs, and abdominal fullness. A urine dipstick is done in your clinic and is negative for proteinuria. The lab adjacent to your clinic confirms that there is no proteinuria on formal urinalysis. Bloodwork reveals plasma albumin levels of 26. What investigation will you request next? 1. Fecal chymotrypsin 2. Fecal alpha-1-antitrypsin 3. 24 hour urine collection for protein 4. Serum prealbumin

Answer 163

Fecal alpha-1-antitrypsin Protein losing enteropathy: - eg. celiac disease, intestinal lymphangiectasia and causes hypoalbuminemia and edema also leading to microcytic anemia, decreased fat soluble vitamins (A, D, E, K) - investigations guided by history and physical -- stool for O+P, stool occult blood and leukocytes (r/o inflammatory disorder), stool pH and reducing substances (for carbohydrate malabsorption), fecal fat test for fat malabsorption, and alpha 1 antitrypsin for protein malabsorption, fecal elastase for exocrine pancreatic insufficiency

Question 164

A 12-year-old girl is hospitalized with a 24 hour history of intractable non-bilious emesis. She has had two similar episodes in the past which resolved without sequelae after 36 hours of rehydration. Her physical exam is reassuring. What is the most likely diagnosis? Abdominal tumour Brain tumour Cyclic vomiting Meningitis

Answer 164

Cyclic vomiting

Question 165

An obese child is followed in your clinic. His mother asks you how she could decrease his intake of trans fats. You recommend: Cooking with margarine Reducing his fast-food intake Reducing his soft drink intake Purchase low fat dairy products

Answer 165

Reducing his fast-food intake

Question 166

A 10-month-old is referred to your clinic for failure to thrive. On further history, you note that he has large, foul smelling stools several times each day. What is the most likely underlying cause? Cystic Fibrosis Celiac disease Cow’s milk protein allergy Infectious diarrhea

Answer 166

Cystic Fibrosis CF intestinal manifestations: - meconium ileus, meconium plug, meconium peritonitis - rectal prolapse (rare now because of early diagnosis) exocrine pancreatic insufficiency → protein and fat malabsorption (85%) - frequent, bulky, greasy stools and failure to gain weight fecal elastase - fat soluble vitamin deficiencies

Question 167

A 4 month old child with past medical history of neonatal SVT presents to the emergency department with a gastroenteritis. He has had a 24 hour history of vomiting and diarrhea. Which of the following is an absolute contraindication for the prescription of ondansetron in this child? Age Expected increase in diarrhea Past history of SVT

Answer 167

Past history of SVT Do not use ondansetron in patients with congenital long QT or who are at risk of malignant hyperthermia

Question 168

Use of ondansetron for gastro in what age group Mod dehy 3mos – 12 years Mod dehy 6 mos – 12 years Severe dehy 3mos – 12 years Severe dehy 6 mos – 12 years

Answer 168

Mod dehy 6 mos – 12 years oral ondansetron therapy, as a single dose for paediatric gastroenteritis, is effective in reducing the frequency of vomiting and IV fluid administration in infants and children six months to 12 years of age who present to the ED with mild to moderate dehydration or who have failed a trial of oral rehydration therapy - most common SE is diarrhea

Question 169

15 year old with diarrhea three times a day. Nocturnal BMs in past week. No weight loss. Normal exam. Normal labs including inflammatory markers and TTG. What do you do? No further investigation required UGI with SB follow through Lower scope Duodenal biopsy

Answer 169

Lower Scope Nocturnal BMs are a red flag for IBD

Question 170

Autistic child more irritable, petechiae painful to touch, difficulty walking, gum bleeding what is the nutritional deficiency Vitamin K Vitamin C Vitamin E Vitamin D

Answer 170

Vitamin C | Scurvy

Question 171

Fe deficient on supplements but no improvement what to do next, said he was very compliant, what is next investigation TG for Coeliac Upper GI Scope Bone Marrow

Answer 171

TG for Celiac Iron absorption occurs in the duodenum, which could be affected by celiac or crohns Celiac: anemia (iron malabsorption) unresponsive to therapy - most common extra-intestinal manifestation

Question 172

CP child with NG feed and GERD that is responsive to ranitidine, what is the next step Gastrostomy tube Jejunal Tube change the reflux med G tube and fundoplication

Answer 172

Gastrostomy tube

Question 173

7 week baby crying for 4-5 hours/day. Tried soy formula and got diarrhea what is the next step: 1. 2 weeks trial of hydrolysed formula 2. Reassure 3. give probiotics 4. go back to breastfeeding

Answer 173

2 weeks trial of hydrolysed formula Definition: Rome III criteria 1. infants < 4 months 2. paroxysms of irritability, fussiness or crying that start and stop without obvious cause 3. episodes lasting >3 h/day and occurring >3 days per week for > 1 week 4. no failure to thrive NO role for soy, insufficient data on probiotics. Consider maternal consumption of hypoallergenic diet or trial of extensively hydrated formula

Question 174

Indication for soy formula

Answer 174

Galactosemia

Question 175

14 year old, post fontan 10 years ago, with low albumin and diarrhea.... what is the diagnosis protein losing enteropathy Coeliac Heart Failure IBD

Answer 175

protein losing enteropathy PLE Causes - include intestinal loss of lymphatic fluid due to lymphatic obstruction (ie. Fontan)

Question 176

6 y old, jaundice, clinical hepatitis. Test most likely to give diagnosis: Hep A IgM EBV PCR

Answer 176

Hep A IgM HepA most common cause of clinically apparent acute hepatitis

Question 177

A photography is shown eye findings (Kayser-Fleisher Ring) in a 14 year old boy. What should be evaluated: ``` Liver function Renal function Blood triglyceride levels Skeletal abnormalities Joint abnormalities ```

Answer 177

Liver function Wilson Disease Should check for serum copper, urine excretion of copper and ceruloplasmin levels - Liver function to look for inflammation - slit lamp

Question 178

A 5 month old is brought to the ER because of intermittent abdominal pain for the past 5 weeks. The pain occurs several times daily, during which he scream and extends his arms and legs up simultaneously. The episodes last only seconds, and afterwards he returns to normal. His mother also feels that he has not been himself for the past month and that he doesn’t smile easily. ``` Colic Constipation Epilepsy Intermittent intussusception Reflux esophagitis ```

Answer 178

Epilepsy Bizarre question - ?developmental regression with lack of smile

Question 179

6 day old term newborn male presents to the ED with blood in his diaper. Thought to be rectal in origin. Baby born term and at home with no apparent complications. On examination in the ED he is tachycardic and pale. What is the most likely diagnosis? 1. CMPA 2. Anal fissure 3. Hemorrhagic disease of the newborn 4. Meckles

Answer 179

3. Hemorrhagic disease of the newborn Results from severe transient deficiencies in vitamin K dependent factors Characterized by bleeding: GI, nasal, subgaleal, intracranial, post-circumcision Prodromal or warning signs may occur before serious intracranial bleeding

Question 180

Trichobezoar - what is definitive management? Endoscopy Laparoscopy Observe Laparotomy

Answer 180

1. Endoscopy If that fails then surgery

Question 181

7yo kid with history of GERD, and new onset abdominal pain. How do you diagnose 1. Esophagoduodenoscopy and biopsy 2. Scope 3. Urea breath test 4. Stool for H pylori

Answer 181

1. Esophagoduodenoscopy and biopsy EGD and Bx are gold standard for Dx of H Pylori

Question 182

Previously well 6 month old presents with a 1 week history of acholic stools with total bilirubin 350 and direct bilirubin of 200. Urine is cola coloured. RUQ mass. What is the most likely diagnosis: 1. Hepatoblastoma 2. Alagille 3. biliary atresia 4. choledochal cyst

Answer 182

4. Choledocal cyst - 75% appear during childhood as cholestatic jaundice, severe liver dysfunction including ascites and coagulopathy can happen rapidly In neonates, abdominal mass is rarely palpable - Older child has classic triad: abdominal pain, jaundice and mass (even so… only 33% have palpable mass)

Question 183

Adolescent girl with intermittent epigastric pain for months. Normal weight and diet. Father has history of peptic ulcer disease. Tried dad’s antacids and experienced some relief. What would be the best management? Amox, clarithro, and PPI Scope + biopsy Urea Breath Test? Stool antigen test

Answer 183

Scope and Biopsy - Should ensure you have the correct Dx first before triple therapy.

Question 184

2 year old boy swallowed a button battery. Mom calls you asking what to do? 1) Follow up with xray in 48h 2) Reassurance that lots of kids swallow small objects 3) Urgent Xray to determine where battery is 4) Urgent endoscopy

Answer 184

3) Urgent Xray to determine where battery is Do imaging for all children <12, all patients ingestested button battery >12mm, all patients where diameter of battery is unkown. Urgent Endoscopic Removal : esophageal impaction, gastric if signs or symptoms of esophageal or gastric injury at presentation. Consult GI if asymptomatic, generally they'll fish out any >20 mm or if in stomach for 48h

Question 185

You are seeing a 2 month old in your clinic being seen due to concerns with poor weight gain. Brith weight approx 3kg, currently weighs 4 kg. She is drinking 60mL of regular formula every 3 hours. What is her daily caloric intake? 60 kcal/kg/day 80 kcal/kg/day 100 kcal/kg/day 120 kcal/kg/day

Answer 185

80 kcal/kg/day 60 ml = 2 ox = 16 oz per day 16 x 20 = 320 kcal day 80 kcal/kg/day

Question 186

Day old female baby with jaundice, Bili 420. Both parents well. Mom O-, baby B+. Coomb’s negative. Of Northern European ancestry. What is most likely cause of jaundice? Rh incompatibility ABO incompatibility Hereditary spherocytosis G6PD deficiency

Answer 186

Hereditary spherocytosis If Coombs is negative - rules out Rh, ABO and other blood group problems. HS - common in northern europeans. Manifests as anemia and hyperbilirubinemia

Question 187

You see a teenager in your clinic with progressive dysphagia to solids and epigastric pain. He has been diagnosed with eosinophilic esophagitis, and a referral has been sent for allergy testing. In the meantime, what should you do? Elimination diet Oral fluticasone by MDI Proton-pump inhibitor Oral Prednisone

Answer 187

Oral Fluticasone by MDI Topical steroids is first line (swallow your inhaler), systemic steroids have better histologic improvement (but similar symptomatic improvement) but have more SE so are reserved for failure of topic steroids or need for rapid improvement of symptoms. - Some kids will respond to PPI - Elimination diets are difficult and $$

Question 188

Description of a 2 month old baby with colic. Tolerating breastfeeding well, normal exam. What is the best management? 1. Encourage mom to continue breast feeding 3. Add cows milk based formula in diet 3. Add soy based formula in diet 4. Simethicone

Answer 188

1. Encourage mom to continue breast feeding - Start with parental support- feeding techniques, soothing techniques. - If that fails consider limited time trial of maternal hypoallergenic diet

Question 189

Adolescent with gastroesophageal reflux disease. Give 5 non pharmacologic treatments

Answer 189

- avoid acidic or reflux inducing foods such as tomatoes, chocolate and mint - avoid caffeine and carbonated drinks - avoid alcohol - lose weight - don’t smoke - don’t eat right before bed - smaller volume meals - elevate head of bd

Question 190

4 signs a kid is ready for toilet training.

Answer 190

- Able to walk to potty - Stable while seated on potty - Able to remain dry for a few hours - Receptive language skills (can follow simple commands) - Expressive language skills (communicated need to use potty) - Desire to please - Desire for independence

Question 191

8yr male with constipation since age 2. 1 yr of fecal soiling. Exam normal except large hard stool in rectum. 1. List 4 management strategies. 2. List 3 meds with long-term safety in children

Answer 191

1. - Behavioural interventions (timed stooling BID) - Education for parent + child - PEG 3350 clean-out (1-1.5g/kg/day) x 3 days - Enema x 6 days - Go-Lytely until effluent clear - Use of foot stool - Balanced diet (0.5g/kg/day of fiber) - prune/pear/apple juice ``` 2. PEG 3350 Lactulose Docusate sodium Glycerin Supp Mineral oil (avoid in babies) ```

Question 192

18mo with fulminant hepatitis without neurologic symptoms. | List 3 supportive care measures.

Answer 192

- PPIs to reduce risk of GI bleed (they are all at high risk) - Monitor for, avoid and treat hypovolemia - Monitor for Coagulopathy: give Vit K, FFP, cryo, platelets. Consider plasmapheresis - Maintain glucose in normal range (may need IV dextrose) - Nutritional support (may need TPN) - Intubation: prevent aspiration, reduce cerebral edema by hyperventilation - Avoid sedatives as much as possible: can aggravate or precipitate encephalopathy - Avoid lyte imbalance: can precipitate encephalopathy - Monitor closely for infection: most common pathogen are Gram positives - If encephalopathy present: restrict/eliminate protein; give lactulose at doses sufficient to cause diarrhea - lowers ammonia levels by decreasing microbial ammonia production

Question 193

A mother comes in with her newborn baby and tells you that she lives out in the country where they have well water. What should she do about vitamins and fluoride?

Answer 193

- Consider fluoride supplementation if water level <0.3ppm | - Vitamin D

Question 194

Mom brings 10mo son from another country. Worried re: malnutrition. Baby has thick costochondral juctions 1) Dx: 2) Other Physical findings

Answer 194

1) Rickets (Vit D) 2) - Widened epiphyses of long bones - Craniotabes - Short stature - Frontal bossing - Sternal groove - Windswept deformities - fractures

Question 195

28 wk prem now 28 days old and on oxygen for CLD. On 150 cc/kg/d EBM by gavage, also on Fe, aldactazide, vit E and fluticasone. 1. Calculate the caloric intake, what should his caloric intake be? 2. What 2 ways do you recommend optimizing nutrition.

Answer 195

1. 150 ml = 5 oz/kg/day = 100 kcal/kg/day Prem with CLD has higher needs (120-140 kcal/kg/day) Healthy term 100-110 Prem and well 90 - 120 2. Concentrate the feeds Increase feed volume Ensure Vit D

Question 196

Child presents with an ammonim level in the 400-range. What 3 things would you do in your management?

Answer 196

NPO IVF with dextrose (D10NS) at 1.5 maintenance w/ K+ Ammonia scavenger (Ammonul) BW: Gas, lytes, lactate, glucose, liver enzymes, urine organic acids, serum amino acids

Question 197

AXR of bird’s beak. What’s the condition?

Answer 197

``` Achalasia (esophagus) Sigmoid volvulus (Abdomen) ``` Achalasia: Regurgitation and dysphagia for solids and liquids Mean age 8 May have FTT and chronic cough CXR - air fluid level in dilated esophagus Barium fluorscopy: tapering of lower end esophagus

Question 198

Child with tall, mitral valve prolapse, hyperextensible joints, long arms, who has rectal prolapse. 1. Diagnosis 2. Differential

Answer 198

``` 1. Ehlers-Danlos (though being tall is a MARFAN thing... not ED) 2. Marfan CF Chronic constipation Diarrhea Pertussis (cough it out) Idiopathic Ulcerative Colitis Malnutrition ```

Question 199

Teen with dysphagia, odynodysphagia with solids, name 3 differentials

Answer 199

``` Esophageal foreign body GERD Eosinophilic Esopagitis Esophageal stricture Esophageal tumor Pill esophagitis Achalasia ```

Question 200

This is a baby with jaundice and significant conjugated hyperbilirubinemia. Two tables with one column where you had to write the differentials and then the corresponding definitive investigation?

Answer 200

Biliary Atresia - Liver Biopsy Choledochal cyst - U/S or MR cholangiopancreatography Hypothyroidism : TSH/FT4 Alagille: Genetic testing (JAG1 mutation) Infection: PCR for CMV, Toxo, Rubella, HSV, Parvo Sepsis: Blood Culture, Urine Culture Alpha-1-antitripsin deficiency (do a level) Galactosemia - galactose 1 phosphate uridyl transferase activity in RBC Neonatal Hepatitis- Liver Bx

Question 201

5 Skin findings of IBD

Answer 201

``` Erythema Nodosum Pyoderma gangrenosum Psoriasis Perianal skin tags Vitiligo Apthous stomatitis Chelitis Alopecia areata ```

Question 202

There was an 8mm kidney stone in an Crohn’s patient. 1. What 2 indications for surgical removal 2. What is the most likely type of stone 3. Give 3 Recommendations to patient

Answer 202

1. Obstructive stone, Infection 2. Calcium oxalate 3. Increase fluid intake Reduce sodium Avoid Ca++ supplement, eat dietary calcium Avoid high oxalate foods (ie. beets, stawbs, sweet potatoes) -

Question 203

Child from a Vegan family. List 5 nutritional deficiencies this child might be at risk for aside from assuming their protein and calorie content is ok. Why would you recommend a high protein amount for this child vs a non-restricted diet.

Answer 203

``` Iron deficiency Vitamin D deficiency Vitamin B12 deficiency Calcium deficiency Zinc Omega 3 Fatty acids ``` -Lower digestibility of plant protein

Question 204

2 year old in your office, ex-25 week prem with short gut secondary to NEC. Aside from malnutrition and nutrient deficiency: 1. What are 3 long term complications seen with short gut?

Answer 204

1. - central catheter infection - thrombosis - cholestasis and cirrhosis - gallstones - Repeat surgeries - Renal stones due to hyperoxaluria (ca++ binds to excess fat and not to oxalate, so more oxalate is absorbed and excreted into urine) - Sepsis 2. Of the fat soluble vitamins, which one will not be deficient and why? Vitamin K - intestinal bacteria synthesize vitamin K

Question 205

A teenager has been complaining of recurrent epigastric pain. Examination is otherwise normal. Name 3 criteria for functional dyspepsia.

Answer 205

Any combination of: Postprandial fullness Early satiation Epigastric pain or burning not associated with defecation Severe enough to interfere with usual activities At least 3 days per week over last 3 months (double stuffed, double pain)

Question 206

4 year old girl has been stable on NG tube feeds. She gets 4 bolus feeds a day. Lately she has been getting sweaty, crampy abdo pain and diarrhea one hour after feeds. a) What is the reason for her dumping syndrome? (1 line) b) Name 2 pathophysiologic mechanisms for dumping syndrome.

Answer 206

a) NG tube may have gone post-pylorus. Large volume in short period of time, or high osmolarity formula. End up hypoglycaemic b) High concentration of carbs in the small bowel, rapid absorption of glucose, with hyperinsulin response - Altered gastric mucosa and tone from surgery - increased secretion of GI hormones

Question 207

4-week old infant with bloody stools. He is formula fed. He is otherwise well and gaining weight. a. What is the most likely diagnosis? b. What is your NEXT step in management? c. What do you tell the mother regarding the child's prognosis?

Answer 207

a. CMPA (Food protein induced proctocolitis) b. Extensively hydrolyzed formula c. 90% will outgrow by 1 year, progression to food allergy or IBD is rare

Question 208

Anemia with Ferritin of 5. Determined to be nutritional. a) What are 2 nutritional pieces of advice to give? b) What is the appropriate treatment? c) How long should the treatment be provided? d) When should the patient be brought back for follow-up?

Answer 208

a) Increase iron rich foods (iron fortified formulas, red meats, green leafy vegetables) Limit cow's milk to no more than 20oz/day b) Treatment dose of elemental iron 3-6 mg/kg/day for 3-4 mos d) Recheck CBCd (+retics) at 1 month

Question 209

A 13 year old girl presents to you with jaundice. She is otherwise well, and has episodes like this in the past. Bloodwork is done, which shows a mildly elevated unconjugated bilirubin at 60 and a conjugated bilirubin of 12. a) What is her most likely diagnosis? b) What are two things that can incite jaundice in her condition c) How to treat her?

Answer 209

a) Gilbert b) Menses, Infection, Medications, fasting, prolonged exercise c) Reassurance

Question 210

Boy (6 years old) whose dad says that he ‘stinks’ all the time and he has feces in his underwear. a) What are the two most important components of your physical exam (2)?

Answer 210

a) Spine exam to look for spinal dysraphism Neurological exam of lower limbs DRE

Question 211

What are x-ray findings consistent with a coin that would need an urgent removal? What if it was in the esophagus - what anticipatory guidance would you provide the family and your next step (2)

Answer 211

a) Double halo (ie. not a coin), step off on lateral view, mediastinal free air, peritoneal free air Lodged in trachea b) If no respiratory distress and can swallow, endoscopic removal in 24 hours with XR prior to endoscopy (¼ will pass in 8-16 hours)

Question 212

2 yo M with bloody diapers since yesterday. He is asymptomatic otherwise, in no pain and has normal physical exam. Mother shows you a diaper with large amounts of blood. a) What is the diagnosis? b) What are two other conditions it could be? c) What investigation do you do?

Answer 212

a) Meckel's diverticulum b) Anal fissure, food protein enterocolitis, infectious colitis, juvenile polyp c) mocker's scan with 99m technetium pertechnetate

Question 213

Name 2 screening investigations for Celiac Disease (2). Name 5 extra intestinal manifestations of Celiac Disease (5).

Answer 213

Screening anti-TTG anti-endomysial antibodies IgA level ``` Non-GI: Short stature Delayed puberty Hypotonia, developmental delay, learning disorders, HA, ataxia, epilepsy LIver disease Iron deficiency Dermatitis herpetiformis Dental enamel defects Metabolic bone disease Arthritis ```

Question 214

Causes of Infectious bloody diarrhea

Answer 214

Campylobacter Shigella Shiga toxin producing E. coli