GI Flashcards

1
Q

Gastroschisis is associated with what anomaly?

A

Intestinal Atresia

1 in 10

BUT other malformations rare (versus omphalocele has many)

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2
Q

List 3 ways to tell gastroschisis from omphalocele:

A
  1. No membranous sac
  2. Periumbilical (R)
  3. No liver eviscerated
  4. Abdo developed well
  5. 1/10= intestinal atresia
    BUT other malformations rare
  6. Need more hydration due to lack of sac
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3
Q

XR bubble in stomach and left of this. None distal. XR sign? Dx? Associated syn? Heart lesion?

A
  • Double bubble
  • Congenital duodenal atresia
  • Trisomy 21
  • AV canal defect

Tx: NG decompression, fluid resus, look of other anomalies, Sx repair

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4
Q

List three things associated with chronic anal fissures in older kids.

A
  • Constipation
  • Crohn’s Dx
  • Chronic Diarrea
  • Prior Rectal Sx
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5
Q

What is the CC triad for Crohn’s Disease?

A

Diarrhea
Wt loss
Abdo Pain

Other: FTT, dermatitis herpteformis

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6
Q

Best test to tell diaphragmatic eventration versus hernia:

  • diaphragm fluoro
  • US
  • exploratory laparotomy
  • MRI
A

US

Diaphragmatic eventration= abnormal elevation= paradoxical motion of hemidisphragm
- DX usually XR but if uncertain US to confirm
Tx: Sx plication of muscle

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7
Q

13 y.o. early morning throat pain. Bad breath in am.

  • Upper GI
  • AUS
  • CXR
  • pH probe
A

pH probe

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8
Q

List three RF for GERD:

A
  • neuro impairment
  • obesity
  • lung dx
  • esophageal atresia
  • prematurity
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9
Q

Admitted for viral gastro. Intermittent scream and vomit in 8 month old. Pale, lethargic. Which is most helpful ind x:

  • AXR
  • serum lactate
  • air enema
A

Air enema
(less complication than saline)

R/O INTUSSUSCEPTION

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10
Q

T or F: intussusception is most common abdo emergency in kids < 2

A

True

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11
Q

T or F: most common site of intussusception is ileocolic

A

True

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12
Q

Abdo pain+
palpable sausage shaped abdo mass
+ bloody currant jelly stool

A

Intussusception

more common pain, vomit, mass

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13
Q

What test can you order to see intussusception? Sign on image

A

US.

Bull’s eye.

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14
Q

Swallowed nickel. In stomach on XR. Next?

  • observation
  • upper endoscopy and removal
  • cathartics
A

Observation

** once in stomach, 95% ingested without difficulty.

XR monitoring till pass for long or sharp, straight pin.

Watch out for multiple magnets - can cause pressure necrosis and perf

Note: failure to progress out within 3-4 wk= impending perf.

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15
Q

If FB in esophagus:

A

remove ASAP (battery, sharp, meat can wait 12 hr)

asymptomatic blunt and coin can be watched up to 24h to see if pass into stomach.

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16
Q

Child swallowed coin and in stomach on XR. What do you do? (1 pt)

A

Observe.

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17
Q

List three indications for FB removal in esophagus:

A
  1. Battery
  2. Magnet
  3. Sharp or pointed
  4. Meat (can wait up to 12 h)
  5. size dimension (won’t get through GI; vary per age)
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18
Q

List tests for hepatic synthetic function:

A

“Plt down -> INR up -> Alb down -> Bili up -> Glucose down”

Synthetic F’n:

  • INR, PTT up (does not improve with Vit K IM)
  • albumin low

Metabolic F’n: BG

Storage Capacity F’n:
- low cholesterol, TG, lipoprotein

Excretory F’n:
- Bilirubin

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19
Q

Stool reducing substance NOT (+) in:

  • glucose
  • sucrose
  • fructose
  • lactose
  • galactose
A

Sucrose/starch NOT reducing substances.

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20
Q

Watery diarrhea. Flatulence. Abdo distended. Abdo Pain. (+) Unabsorbed reducing sugar. Dx? Tests?

A

Carb malabsorption

  • Primary (congenital sucrose deficiency, lactase deficiency etc.)
  • Secondary (temp lactose intolerance, intractable diarrhea of infancy, toddler’s diarrhea)

Hydrogen Breath Test (correlate to degree of malabsorption)

Stool pH < 5.5
(+) reducing substance

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21
Q

List 4 test for celiac Dx?

A
  1. Anti- tissue transglutaminase IgA antibodies (anti-TTG)
  2. IgA level
  3. Anti-endomesial antibody testing (anti-EMA)
  4. IgG anti-deaminated gliadin peptide antibodies
  5. HLA DQ2 and HLA DQ8 (for unclear cases as min. one must be (+))
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22
Q

Kid with CP. Tolerate GT. Got botox in leg. P/E oral secretion and less hypertonic. Next:

  • pH probe
  • swallow study
  • observe
A

Observe.

Botox can have systemic spread past local and cause dysphagia.

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23
Q

Progressive dysphagia with solid. List two most common cause:

A
  1. EoE
  2. Achalasia
  3. GERD with stricture
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24
Q

List two things on ddx for dysphagia for solid and two for solid + lq.

A

Solid only

  1. EoE
  2. Peptic stricture
  3. Extrinsic (vascular ring, mass)

Solid + Liquid

  1. Neuromuscular (CP, Muscular dystrophy, MS)
  2. Achalasia
  3. Systemic AI (myasthenia gravis, scleroderma)
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25
Q

List 3 AE for botox in CP:

A
  1. Transient fever or pain, bruising
  2. Weakness in injected area
  3. Spread (i.e. dysphagia, resp distress)
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26
Q

Post TEF with resp distress. 3 Dx:

A
  • Tracheomalacia
  • Re-fistulization
  • GERD w/ laryngospasm
  • tracheal stricture
  • vocal cord paralysis
  • Cardiac anomaly (VACTERL)
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27
Q

Bubbling at mouth and nose with cyanosis with feeds. Can’t pass NG. Likely Dx? Most common type?

A

TEF

Most common type: H type

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28
Q

Progressive non bilious vomit. Small palpable olive on RUQ. Likely lab issue:

  • met acid
  • resp acidosis
  • low K
  • alkalotic urine
  • high Na
A

Low K

Classic=
Hypo-Chloremia
Hypo-Kalemia
Metabolic Alkalosis

With Later Paradoxical Urinary Acidosis.

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29
Q

T or F: 75% of intussusception cases have lead point.

A

False.

90%= idiopathic.

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30
Q

What is intussusception?

A

“Telescoping” of segment of proximal bowel downstream.

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31
Q

T or F: intussusception most common abdo obstruction in kids 6 mo-3 y.o.

A

True

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32
Q

List three lead point example for intussusception:

A
  1. Meckel’s
  2. Polyp
  3. HSP
  4. Appendix
  5. Non Hodgkin’s lymphoma
  6. CF intussusception
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33
Q

What are 3 contraindications to air/ barium enema in intussusception?

A
  1. Unstable (refractory shock)
  2. Perf
  3. Peritonitis
  4. Prolonged symp
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34
Q

Infant vomit with chronic wheeze. Upper GI show dent in upper esophagus. Two dx?

A
  • Vascular Ring
  • Pulmonary Artery Sling

Other: Mediastinal mass, mediastinal lymphadenopathy, esophageal FB

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35
Q

Two indications for fundoplication:

A

Refractory Esophagitis
Chronic pul dx due to aspiration
Strictures

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36
Q

Four recommendations for 2 mo. BB with GERD that eats 6-8 oz every 6-8 hours.

A
  1. Reduce vol
  2. Increase freq
  3. Feed upright
  4. Break to burp
  5. Keep upright 20-30 min. after feed
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37
Q

Three RF for pyloric stenosis?

A
  • male
  • first born
  • prem
  • maternal smoking
  • bottle feed
  • erythro or azithro within 2 wk of life
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38
Q

T or F: Perianal dx should always make you think of Crohn’s dx?

A

True

Can be from constipation but typically then < 1 y.o.

Older pt= chronic fissure= constipation, Crohn’s, chronic diarrhea, prior rectal Sx
Tx: no constipation, sitz bath +/- topical tx

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39
Q

What are indications for G-Tube:

A
  • FTT
  • severe GERD
  • neurologic impairment

versus GJ more for delayed gastric emptying or dysmotility

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40
Q

Oral rehydration in kid w/ mod dehydration:

  • 100cc/kg over 4h
  • 50 cc/kg over 4h
  • NS bolus 20cc/kg
A

100 cc/kg over 4h

Remember % x 10
over 4 hour
moderate= up to 10%

If severe= bolus

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41
Q

Post scoliosis Sx. Bilious Emesis. Likely etiology?

A

Superior mesenteric artery syndrome.

= compress duodenum by artery against aorta

RF: thin, bed rest, abdo Sx, lumbar lordosis

Classic: thin teen with vomiting 1-2 wk after scoliosis Sx. Lengthening of vertebrate stretch of aorta and extrinsic compress duodenum.

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42
Q

Name two test to confirm GERD dx.

A

Clinical dx!

Options:

  • PH monitoring (document acid reflux episodes but doesn’t Dx GERD, best for acid suppression during tx)
  • Intraluminal impedance: differentiate between antegarde vs. retrograde material; good for resp symp

Non-Options:

  • Upper GI- for vomi and dysphagia; does not tell GERD
  • Manometry- good for motility, achalasia; no GERD
  • Endoscopy- tell complication like erosive esophagitis
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43
Q

Name 4 complication of severe GERD

A

** FTT

Esophagus:

  • *- esophagitis
  • *- barrett esophagus (intestinal metaplasia of distal esophagus)
  • *- stricture
  • adenocarcionma

Resp:

  • *- Asthma (Aspiration, airway hyper responsive)
  • *- recurrent pneumonia
  • laryngeal edema, granulomas
  • chronic cough

** Dental Enamel Erosion

BRUE (brief resolve unexplained event)

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44
Q

Name 5 non pharma tx for GERD:

A

Life style:

  • low vol high cal feed
  • thicken feed
  • hydrolyzed if CMPA
  • low fat meal at din
  • avoid acidic food (chocolate, mint, tomato) and carbonated or caffeinated fluid
  • upright positioning after eating
  • no tobacco, alcohol
  • wt loss if obese
  • NJ or GJ feed if aspirated
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45
Q

Teen black male. Intermittent abdo pain + diarrhea. Likely dx? Test?

A

Secondary lactase deficiency (adult type)

Do breath hydrogen test.

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46
Q

T or F: if stool reducing substances are negative than disaccharide deficiency ruled out.

A

False.

SUCROSE not stool reducing so not all = (+).

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47
Q

Why do you tx Sallmonella bacteremia in 2 month old:

  • eliminate carrier state
  • decrease duration of symp
  • prevent meningitis
  • eliminate shedding from GI tract
A

Prevent meningitis

ABX NOT recommend for uncomplicated gastroenteritis as:

  • suppress normal flora
  • prolong excretion
  • risk creating carrier state
  • does NOT shorten dx

BUT give ABX if:

  • < 3 mo. = risk of blood infection
  • immunocompromised (HIV, CA, sickle cell)
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48
Q

What are the two indication you treat salmonella gastroenteritis?

A
  1. < 3 mon. (risk of bacteraemia)

2. Immunocompromised (HIV, CA, sickle cell)

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49
Q

12 y.o. Fever. Hepatomegaly. HR 85 despite temp 39.5. CBC and urine normal. BCX show gram (-) bacilli (rode). Cause?

A

Typhoid fever.

  • Salmonella tyhpi
  • relative bradycardia
  • fever, HSM, dactylics, rose spot
  • complication: hepatic failure, intestinal perf
  • IV 2 wk (ceftriax)
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50
Q

13 mo. General puffy + lethargy. Urine (-) for protein or blood. Alb 13. Tx:

  • steroids
  • IV albumin
  • protein hydrolysate formula
  • lactose free formula
A

Protein hydrolysate formula

Low albumin:
R/O renal
R/O liver
R/O leaky gut
R/O post-fontan R side heart issue
R/O intake
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51
Q

Shwachman-Diamond Syndrome BB. Vitamin Level Normal

  • A
  • E
  • B12
  • D
A

B12

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52
Q

What is the triad for Shwachman-Diamond Syndrome?

A
  1. Exocrine Pancreatic Insuff
  2. Cytopenia (e.g. neutropenia or any line)
  3. Bone (metaphyseal dysostosis, thoracic dystrophy, short stature)

Most common CC: infancy with poor growth and steatorrhea

Definitive Dx: molecular analysis

Tx: pancreatic enzyme replacement, ADEK vit, risk of CA

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53
Q

2 y.o. with Celiac. most definitive test:

  • jejunal bx
  • anti-gliadin antibody
  • anti tissue transglutaminase antibody
  • gluten free diet
A

Jejunal bx

1st= anti-TTG IgA + IgA levels
= if (+) -> BX

Bx= gold standard

  • villus atrophy
  • crypt hyperplasia
  • increased intraepithelial lymphocytes
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54
Q

List 4 other cases of flat intestinal villi beside Celiac Dx?

A
  • *- autoimmune enteropathy
  • tropical sprue
      • giardiasis
      • HIV enteropathy
  • bacterial overgrowth
  • *- crohn’s dx
  • eosinophilic gastroenteritis
      • cow’s milk enteropathy
      • graft vs. host dx
  • TB
  • lymphoma
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55
Q

List 3 atypical ppt of Celiac dx?

A

**Iron deficiency anemia
**Dermatitis herpetiformis
Aphthous Stomatitis
Dental enamel hypoplasia
**Osteoporosis
Pubertal Delay
Arthritis
Hepatitis

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56
Q

List 2 conditions associated with Celiac Dx?

A
  • *Genetic
  • *T21, *Williams, *Turners

DX

    • 1DM
    • AI thyroiditis
  • Sjogren
  • Addison
  • Primary Sclerosing Cholangitis
  • Autoimmune Hep
  • Primary Biliary Cirrhosis
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57
Q

Delayed meconium. Distended above. Calcification on XR:

  • Hirschsprung
  • CF
  • Duodenal Atresia
A

CF

seen in 10-15% of pt

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58
Q

6 y.o. with hx of diarrhea. Albumin 24. Name 4 dx on DDX:

A

> Gastric inflam
- eosinophilia gastroenteropahy

> Intestinal Inlam

  • *- Celiac
  • *- Crohn’s Disease
  • *- Food protein enteropathy
  • Tropical Sprue (S. india, philippines, carribean)

> Mucosal Inflammation

  • *- post-infectious diarrhea
  • bacterial overgrowth
  • giardiasis

> Radiation Enteritis

> **Lymphangiectasia

  • Primary: Turner, Noonan
  • Secondary: constrictive pericarditis, CHF, Post-Fontan, Abdo TB, lymphoma, sarcoidosis

> Colonic Inflam

  • IBD
  • NEC
  • Congenital dx of glycosylation
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59
Q

13 mon. Diaper rash + diarrhea with new foods.

  • RAST + skin testing
  • carb intolerance common
  • cow’s milk protein
  • citrus fruit + tomato cause via immune mediated mech
  • if no mucus not immune problem
A

Carb intolerance common

  • loose watery diarrhea w/ onset of new food
  • sucrase-isomaltase deficiency- common in Aboriginal. Okay with milk + lactose but sucrose and starch (juice, fruit, starches) develop diarrhea, diaper rash
  • stool pH, reducing substance, breath test
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60
Q

What is FPIES versus Proctitis vs. Enteropathy? Is allergy testing positive for these?

A

NO HELP TO TEST.

Food protein induced enterocolitis syndrome (FPIES)

  • whole tract
  • 1 wk-3 mo.
  • hours after exposure
  • resolve by 3

Food protein induce proctitis:

  • large bowel
  • BF 2-8 wk old
  • bloody stool
  • okay by 9 mo.

Food protein induced enteropathy

  • any; cow’s milk up to 2 y.o.
  • small intestine
    i. e. Celiac
61
Q

List 3 reasons for GI bleed in infant, kid, teen:

A

Infant:

  • anal fissure
  • swallowed maternal blood
  • bacterial enteritis
  • milk protein allergy
  • intussusception
  • NEC
  • coagulation dx (hemorrhagic dx of newborn)

Child:

  • UGI: stress ulcer, gastritis, mallory weiss tear, esophagitis
  • LGI: anal fissure, colonic polyp, colitis, intussusception, Meckel’s diverticulum
  • HSP, HUS
  • sexual abuse
  • coagulopathy

Teen:

  • peptic ulcer, gastritis, mallory-weiss
  • esophageal varices
  • bacterial enteritis
  • IBD
  • colonic polyp
62
Q

T or F: meckel’s diverticulum account for 1/2 of all lower GI bleeds in kids < 2

A

True

63
Q

1 y.o. 3 soft red stool. Asymptomatic. Same happened at 8 mo. old. Hbg 70. Next:

  • Upper scope
  • UGI
  • Nuclear med scan for ectopic gastric tissue
A

Nuclear med scan

R/O Meckel’s

64
Q

What is the most common cause of rectal bleeding in kids < 1?

A

Anal fissure

65
Q

What is the Meckel Diverticulum “Rules”

A

Rule of Two’s

  • likely ppt at 2 y.o.
  • 2% of pop’n
  • 2X M>F
  • 2 feet proximal to ileoceccal valve
  • 2 inches long
  • 2 types mucosa (gastric, pancreatic)
66
Q

What is Meckel’s diverticulum

A

Embryonic out pouching of bowel

67
Q

Painless rectal bleed in 2 y.o. Dx? Test? Tx?

A

Meckel’s Diverticulum

Meckel Radionuclide (Tc99m) scan

Tx: Sx resection

68
Q

2 y.o. pass bright red blood in stool. Pale. Rectal exam has blood + stool on gloves. Hob 94. Likely Dx?

A

Meckel’s diverticulum

69
Q

2 wk old. 1 wk hx of stool mixed with blood. BF well. Passed stool within first 24h. Most likely dx?

  • anal fissure?
  • CMPA
  • Meckel’s diverticulum
A

Anal fissure

Most common cause in infant.

Could be proctocolitis, FPIES with vomiting usually, and both not most common.

70
Q

2 wk old. Tachy. Low BP. Abdomen tense. Next dx approach?

  • Gen Sx
  • AUS
  • IV Abx
  • AXR
  • Barium small with small bowel through
A

Likely NEC

= AXR

  • pneumotosis intestinalis
  • portal venous gas
  • pneumoperitoneum
71
Q

1 mo baby with blood in stool. Cause?

  • anal fissure
  • cow’s milk
  • colitis
  • mom’s blood
A

Anal fissure

Most common

Scary: NEC, Volvulus, Hirschsprung Enterocolitis, Vascular malformation or coagulopathy

72
Q

List 2 surgical complications of HSP:

A
  1. Intussusception

2. Intestinal Perf

73
Q

What is the HSP triad:

A

HSP= small vessel vasculitis. Common kindergarten. Follows URTI (usually GAS).

  1. Palpable purpura (leg or buttock)
  2. Abdo pain (+/- N/V, bloody stool)
  3. Arthritis and Arthralgia

Risk: GI bleed, bowel wall edema cause intussusception, or ischemia w/ perf, Hematuria, Proteinuria

74
Q

Most common cause of rectal prolapse?

A

Most: idiopathic

R/O CF, sacral nerve root lesion

75
Q

2 wk old BB. Abdo mass palpable. Hx of umbilical cath. Cause of hepatosplenomegaly?

A

Portal vein thrombosis.

  • associated w/ prolonged, traumatic or septic umbilical vein cath
  • associated w/ neonatal dehydration, systemic infection, or IBD in older kids
  • block portal vein and back up to spleen, portal HTN
76
Q

List 3 red flags for abdo pain:

A
  • Fever
  • Wt loss (Constitutional)
  • bile or bloody emesis
  • Jaundice
  • HSM
  • Pain outside of umbilicus
  • Wakes from sleep
  • High CRP, anemia
  • FHX IBD, Celiac
77
Q

6 y.o. w/ severe abdo pain + vomit. Tense abdo. 3 things on management:

A
  1. NPO
  2. NG to decompress gut/stomach
  3. IVF
  4. Analgesia
  5. Sx consult
78
Q

List two periodic syndrome associated with migraine develop as adult:

A
  1. Cyclical vomiting
  2. Abdominal migraine
  3. Benign Paroxysmal vertigo
79
Q

What is fitz hugh curtis?

A
Sexually active
Chlamydial and Gonococci ascend
Disseminate from fallopian tube through peritoneum to liver= Perihepatitis
RUQ Acute Pain
\+/- Vomiting
Guarding, tender
AST normal or mild elevated
Biliary dx uncommon unless sickle cell
US normal
80
Q

Irritable 1 month old. Emesis x 1. Distended abdo. mild tachy. mass in R scrotal. Firm. Non reducible. No transillumination. Next:

  • US
  • Urgent Sx consult
  • Nuclear Scan
  • Testicular doppler
A

Urgent Sx consult

Concern: Incarcerated Hernia

81
Q

6 y.o. severe abdo pain 8/10. Febrile. Tachy 160. Abdo rigid. As call Sx what do you do?

  • Cx and Abx
  • Bolus NS + Morphine
  • Bolus NS + CT
A

Bolus NS + Morphine for Analgesia

Tx:

  • ABC
  • Fluid resus
  • ABX + Sx
82
Q

What is primary versus secondary causes of peritonitis?

A

Primary:

  • spontaneous
  • seed into cavity
  • pneumococci
  • Cefotaxime

Secondary

  • Rupture appendix, hernia, Meckel’s, midgut volvulus, HUS, IBD, NEC etc.
  • polymicrobial
  • Amp, Gent, Flagyl
83
Q

Most specific test for pancreatitis:

  • amylase
  • lipase
  • AUS
  • abdo CT
  • AXR
A

Lipase

84
Q

Pancreatitis Etiologies:

A

GOTCHA IBD and CF

  • Gallstones, biliary sludge
  • Other: Meds (steroids, VPA, Imran), Idiopathic
  • Trauma
  • Calcium High
  • HSP, Hyperlipid
  • IBD
  • CF
85
Q

Peptic ulcer. Treatment?

  • amox + clarithro + PPI
  • clinda + clarithro
  • flagyl + others
A

Amox + Clarithro + PPI

86
Q

What are two situations where air enema is unlikely to work for intussusception?

A
  1. Multiple recurrences
  2. Known lead point
  3. Ileoileal intussusception
87
Q

Patients w/ IBD have wt loss due to:

  • inadequate nutrient intake
  • ++ metabolic demands
  • malabsorption
  • drug-nutrient interaction
A

Inadequate nutrient intake.

88
Q

List two skin complications of IBD:

A
  • erythema nodosum (more CD)
  • pyoderma gangrenosum (more UC)
  • psoriasis
  • perianal skin tags
  • epidermolysis bullosa
89
Q

How do you manage perianal abscess in IBD patient.

A

Infant w/ no predisposing Dx: self-limited; no Tx (even if fistula)

Older kids/predisposing Dx:

  • good perianal hygiene
  • antibiotics
  • tx underlying dx
  • topical tacrolimus may help IBD pt
  • Sx last resort
90
Q

Post total colectomy for UC patient. “J-pouch”. Now 5d bloody stool 8X per day. Stool culture negative. Dx? Tx?

A

Dx: Pouchitis

CC: bloody stool, abdo pain, +/- low grade fever

Tx: PO metronidazole or ciprofloxacin

Note: probiotics decrease rate of pouchitis

91
Q

Crohn’s pt on azathioprine (Imuran). 2 wk later abdo pain, vomiting. Two causes:

A

AE for azathioprine:

  • *- N/V, abdo pain
      • pancreatitis
  • hepatitis

Crohn’s complication: stricture, fistula, abscess

92
Q

5 y.o. bloody stool. Hyper pigmented lesion on lip + mucosa. Intussusception Dx. Underlying Cause:

  • HSP
  • Peutz-Jehgers
  • Langerhans Histiocytosis
A

Peutz-Jegher Syn

    • inherited colorectal CA syndrome
    • mucocutaneous pigmentation + extensive GI hamargtoma polyposis
93
Q

Name 2 RF for adenocarcinoma in setting of dx UC IBD.

A
  • Pancolitis
  • Primary Sclerosing Cholangitis

Note:

  • risk increase after 8-10 yr of dx
  • proctitis alone has no increased risk
  • once > 10 yr dx, screen colonoscopy q 1-2 yr (if you get PSC then annual regardless of UC onset)
94
Q

List 2 life threatening complications of UC:

A
  • Htypercoaguloable state resulting in stroke or PE
  • Toxic megacolon
  • Perforation
  • Primary sclerosing cholangitis
  • Adenocarcinoma
95
Q

Which is true of encopresis:

  • am usually
  • usually preschool
  • colon dilated
  • soft softeners cure the problem
A

Colon Dilated

96
Q

6 y.o. 2-3 loose BM a day. Leaks into underwear. No red flags. P/E normal except fecaloma on DRE.

  • TSH
  • Stool Cx
  • Rectal Bx
  • Psych consult
  • No investigations
A

No investigations

Tx: PEG3350, balanced diet with fibre (0.5 g/kg/d), regular activity

97
Q

Red flags for encopresis:

A
  • delayed passage of meconium
  • FTT
  • bloody stools
  • severe abdo distension
  • perianal fistula
  • absent anal wink
  • sacral dimple
98
Q

T or F: true constipation as neonate likely due to: Hirschsprung disease, intestinal pseudo obstruction, hypothyroidism.

A

True

99
Q

3 month with constipation since birth, FTT, no stool in rectal exam. Next:

  • rectal bx
  • TSH
  • sweat chloride
  • F/u in 6 mo.
A

Rectal Bx

R/O hirshsprung

100
Q

T or F: low albumin results in false (-) for sweat chloride.

A

True.

101
Q

Girl with rectal prolapse x 2 reduced easily in ER. What 1 test to do?

A

Sweat chloride.

102
Q

3 mo. BB with constipation and FTT. what test to do:

  • TSH
  • Anal manometry
  • Sweat Chloride
A

Anal Manometry

Even better= Barium enema or BX

103
Q

Most useful intervention in encopresis?

  • pharm
  • behav
  • pharm + behav
  • biofeedback
A

Pharm + Behavioural

Steps:

  1. Relief impaction
  2. Soften stool
  3. Behav (regular postprandial toilet sitting, no punishment, avoid power struggle)
  4. Balanced diet + activity
  5. Psychiatric/Behav Therapy
104
Q

What is threshold for term baby with no RF for photo at 5 d?

A

360

If don’t know should do Coombs and Hgb as this would change your risk line and photo.

105
Q

4 wk old with conjugated hyperbili. Next imaging test:

  • US w/ doppler
  • MRI
  • CT abdo
  • HIDA Scan
A

US w/ Doppler

** Key R/O sepsis, Biliary atresia, galactosemia, A1AT

W/U
= AST, ALT, GGT
- INR, Alb, Glucose, Ammonia
- Urine (R/O UTI), urine reducing substance (galactosemia)
- Newborn screen or sweat chloride
- AUS
- HIDA Scan
106
Q

what % of conjugated bill is considered too high?

A

20

107
Q

List 7 things on your ddx for conjugated hyperbili

A

Syndrome
> **Alagille
> PFIC (progressive familial intrahepatic cholestasis)

Obstructive
> ** Biliary atresia
> ** Choledochal cyst
> Inspissated Bile Syndrome
> Perf of bile duct

Infection
> **TORCH
> **UTI
> Sepsis

Endo
> **Hypothyroidism
> Hypopituitarism

Metabolic
> **CF
> **Galactosemia
> Alpha-1-antitrypsin
> Tyrosinemia
> IEM

Drugs/Toxin
> **TPN, Meds

Systemic: CHD, Shock

Non-cholestatic: **Dubin-Johnson, Rotor

108
Q

The earliest and most common ppt of alpha-1 antitrypsin in children?

A

Persistent Jaundice as newborn

109
Q

Butterfly vertebrae. Heart murmur. Cholestatic jaundice at 2 wk of life. Eyes shows:

  • chorioretinitis
  • posterior embryotoxon
  • cataract
  • glaucoma
A

Posterior embryotoxon (film or memb around eye)

110
Q

List 5 features of Alagille Syndrome:

A

Facies: broad forehead, deep wide set eyes, long straight nose

Eyes: posterior embryotoxon, micro cornea, shallow anterior chamber

Cholestasis- prolonged conjugated bili

Peripheral pul stenosis

Butterfly vertebrae

Interstitial nephro

Pancreatic insuff

Short

111
Q

What does the liver bx in Alagille show?

A

Paucity of bile duct

“Disappearing bile ducts”

112
Q

Brown ring around eye. Kayser Fleischer Rings. Dx? Complication?

A

Wilson Disease

  • cooper accumulate
  • Complication: hepatic dx, neuro dx (tremor, parkinsonism)
  • N/V, AST/ALT, portal HTN, hemolytic anemia
  • high ceruloplasmin
  • 24h quantitive urine copper test of choice
  • restrict dietary copper
  • oral copper chelation tx
  • liver transplant
113
Q

3 reasons for liver failure encephalopathy?

A
  • elevated serum ammonia
  • glutamine accumulation in astrocyte causing cerebral edema
  • increased GABA receptor activity causing hepatic encephalopathy
114
Q

What is true re: extra-hepatic portal HTN:

  • associated w/ hepatomegaly
  • associated w/ splenomegaly
  • LFT abnormal
  • most common cause splenic vein thrombosis
A

Associated w/ splenomegaly

115
Q

List 1 cause of extra hepatic versus intrahepatic causes of portal HTN.

A

Extra-hepatic:

  • portal vein thrombosis
  • splenic vein thrombosis
  • AV fistula

Intra-hepatic:

  • hepatocellular dx
  • hepatitis
  • cirrhosis
  • hepatic fibrosis
116
Q

T or F: you MUST R/O portal HTN in child w/ asymptomatic splenomegaly without hepatocellular dx?

A

TRUE

Bleeding may not occur till > 6 y.o.

Dx: US with Doppler (portal vein, esophagus)

Long term complication:

  • bleed
  • hypersplenism
  • biliary obstruction
  • growth slow
  • DD
  • neuropsych
  • hepatopulmonary syn
117
Q

Name 2 features on P/E that suggest Portal HTN

A

Hepatic etiology signs:

  • jaundice
  • palmar erythema
  • vascular telangiectasia
  • ascites
  • hepatomegaly

Extra-hepatic etiology signs:

  • caput medusa
  • splenomegaly
  • anorectal varices
118
Q

13 y.o. mild jaundice + fatigue. HIGH AST, ALT. Bili mild high. Negative a-1-a, hep B, hep C. Dx:

  • Autoimm Hep
  • Hep C
  • Wilson’s
  • Sclerosing Cholangitis
  • A-1-A
A

Autoimmune Hepatitis
* link with anti-smooth muscle antibody*

R/O viral, a-1-a, wilsson, IBD, celiac, IBD, PSC.
Tx: steroids

Re: Question

  • ALP, GGT up if biliary tree issue.
  • Wilson’s rare.
119
Q

Viral hepatitis. What to monitor serially?

  • INR
  • GGT
  • Albumin
  • AUS
  • Alk-phos
A

INR

Hepatocyte= AST, ALT but elevation does not correlate with necrosis.

F’n: low albumin, PTT, INR, low BG, low lipid

Cholestasis= Bili, GGT, alk-phos

120
Q

H pylori dx. Treatment:

  • H2 blocker
  • amox, claritho, PPI
  • claritho + H2
  • amox + PPI
A

Amox + Clarithro + PPI

CC: asymp. or pain, vomit, Fe deficiency, growth issue
Dx: Bx
must tx all pt (including asymp.)
Treatment: amox, clarithro, PPI
- Abx x 2 wk
- PPI x 1 mo.
121
Q

Which is NOT associated with increased risk of Celiac Dx?

  • T21
  • Turner
  • Russell-Silver
  • William’s
  • Type 1 DM
A

Russell-Silver

122
Q

What has gluten:

A

Whey
Rye
Barley

This turns into gliadin which binds with our endogenous made TTG that activate antigen presenting cel.

123
Q

What is the gold standard to Dx Celiac

A

Bx

Bx= gold standard

  • villus atrophy
  • crypt hyperplasia
  • increased intraepithelial lymphocytes
124
Q

Which IBD has transmural inflammation

A

Crohn’s

Versus UC= superficial submucosa

125
Q

NOT on Terminal Ileitis DDX:

  • Crohn’s
  • Lymphoma
  • TB
  • Yersinia
  • Celiac
A

Celiac Dx= NOT

DDX:

  • crohn’s
  • lymphoma
  • yersinia
  • TB
  • chronic granulomatous dx
  • servere eosinophilia gastroenteropathy
  • lymphonodular hyperplasia
126
Q

List organic causes of constipation:

A
  • HIgh Ca
  • LO K
  • LO Thyroid
  • Celiac Dx
  • Lead poisoning
  • Mercury poisoning
  • UC
  • Medications
  • CNS (CP, Neurotube dx, Hirschsprung)
127
Q

Most common cause of diarrhea in 3 y.o.

  • lactose into
  • IBD
  • IBS
  • Toddler’s diarrhea
  • parasitic infection
A

Toddler’s diarrhea

= fructose intolerance

128
Q

List symptoms of reflux in older child:

A
  • Halitosis
  • Reswallowing
  • Retrosternal chest pain
  • Nocturnal cough
129
Q

Fun Fact: What are extensively hydrolyzed formula versus a.a. based?

A

Extensively hydrolyzed = 9/10

  • Nutramigen
  • Alimentum
  • Pregestimil

A.A based= 99/100

  • Neonate
  • Puramino
130
Q

What are bugs that cause bloody stool? Which treated?

A

E coli (no tx)

Shigella (no tx)

Salmonella

  • Tx if: <3 month OR immunocompromised
  • Amox or TMP-Sx

Campylobacter (consider)

    • Abx if bloody stool, fever, severe course, immunosuppressed
  • Azithro/erythro x 5d (shorten duration, and prevent relapse)
  • Cipro can be used (high resistance)

Yersinia

Stenotrophomonas

131
Q

Which is Gilbert’s Syndrome?

A

AD

Unconjugated

132
Q

Most common indication for liver transplant in kids:

A

Biliary Atresia

133
Q

Top three causes of fulminant hepatic failure in kids?

A

Infection: hepatitis
Toxin: acetaminophen
IEM: Neonatal hemochromatosis, Wilson’s, Tyrosinemia type 1

134
Q

List liver failure complications:

A
  • encephalopathy
  • cereal edema
  • coag.
  • bone marrow failure
  • low BG
  • lyte imbalance
  • renal dysfunction
  • ascites
  • pancreatitis
135
Q

EXTRA: Liver BW tips:

  • AST> ALT
  • ALP LOWW
  • ALP HIII
  • ALP Norma but all other high
A

AST> ALT:
Myopathy, renal, hemolysis, adeno

Alk-P
> Low=Zinc
> HI but other BW Normal= familial hyperphos
> Normal but all rest high= Wilson’s

136
Q

If low Factor 8 only. Is it liver failure?

A

No- made in blood vessels.

  • low only= DIC
  • 8 normal but 5+7 low= liver
137
Q

Can we treat hepatitis B?

A

Yes.

If > 2, BW abnormal.

= IFN-alpha

OR

= Lamivudine

138
Q

What is the risk of hepatitis B?

A

Hepatocellular CA risk up (30X)

Cirrhosis (25%)

139
Q

How is Hep C dx?

A

Hep C Antibody

Confirm w/ PCR

140
Q

12 y.o. chronic LFT elevation. Hyperechogenic liver. Likely dx?

  • AI hepatitis
  • NASH
  • Wilson’s
  • Hep B
  • Recurrent viral myositis
A

Non-alcoholic fatty liver dx

** esp if high BMI
= Do US
= R/O AI hep, AIAT, Wilson’s, Hep C

141
Q

Initial W/U for IBD

A
CBC + diff
ESR
CRP
Albumin
\+/- US with doppler (terminal ileum, flow, abscess, liver, pancreatitis)
Upper endoscopy and colonoscopy
MRE

**No small bowel follow through.

142
Q

Treatment of IBD: UC vs. Crohn’s

A

** Rescue Steroids anytime!

CD

  • rare: 5-ASA (mesalamine)
  • Immune modulators: MTX (M), Azathioprine (Imuran)
  • Biologics: Infliximab (Remicade)
  • Sx complications

UC

  • 5-ASA (PO, PR)(mesalamine)
  • Immuno modulators: Azathioprine (Imuran)
  • Biologic: Infliximab (Remicade)
  • Sx
143
Q

What is gold standard to dx Biliary atresia?

A

HIDA Scan + Bx

Scan can be negative. BX key to ensure intrahepatic fine (not Alagille)

Need to do Kasai within 1-2 mo.

144
Q

Post Kasai Nutrition:

A

ADEK

Progestrimil (MCT oil) or BF with MCT

145
Q

Way to remember Alagille?

A

Intrahepatic biliary atresia!

Triangle face
Smooth broad forehead
posterior embryotoxon
long nose
pancreatic insuff
butterfly vertebrae
146
Q

Triad of Eosinophilia Esophagitis?

A

GERD
Hx of atopy
FHx of EoE or atopy

147
Q

How do you manage EoE?

A

ALL PPI

Severe= Food impact=
= PPI
+ Inhaled steroid (no aerochamber and turn upside down thens swallow)
+/- food protein elimination diet

Mild-Mod= PPI to see if responsive or not
If no change= Bx and then start same treatment as severe.

148
Q

NG now dumping. What’s up?

A
  • feed given too fast
  • formula too hyperosmolar
  • tube displaced (put Jejunum)