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Royal College Peds 2021 > GASTROENTEROLOGY > Flashcards

GASTROENTEROLOGY Flashcards

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1
Q

A 17 mo old toddler presents with irritability, refusal to walk with tenderness in both of her legs. She has a low grade fever, petechiae on her skin and mucous membranes. She has a small cut that has not healed well. Radiographs of the legs show bony atrophy with epiphyseal separation. What is the most likely diagnosis?

A

Vitamin C deficiency (ie. scurvy)

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2
Q

A mom of a 2 yo girl tells you she thinks her daughter is lactose-intolerant. What is your response?

A

IDIOT. Acquired lactase deficiency is VERY rare in children < 4-6 yo

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3
Q

A patient presents to you 1 week after a viral illness with vomiting, irritability and lethargy. She then develops delirium and seizures. There is mild hepatomegaly and her bloodwork reveals elevated liver enzymes, ammonia, coagulopathy and hypoglycemia. Liver biopsy shows elevated triglyceride content and diffuse fatty infiltration of hepatocytes with minimal inflammatory changes.

What is your diagnosis?

A

Reye syndrome! Acute encephalopathy and fatty degeneration of the liver -associated with aspirin use in children with viral infections including influenza and varicella -think encephalopathy, liver failure, and FATTY LIVER on biopsy

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4
Q

A patient presents to you with severe, progressive epigastric pain with intractable vomiting. 3 days ago, he sustained a bicycle handlebar injury to the abdomen.

What is the most likely diagnosis?

  • imaging?
  • treatment?
A

Duodenal hematoma = swelling causes intestinal obstruction -diagnosis: UGI series (“coiled spring appearance”) or CT abdo -treatment: NPO, NG decompression, TPN until obstructive symptoms resolve (usually 7-10 d)

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5
Q

A patient presents with acute diarrhea after eating raw shell fish.

What organism is most likely the culprit?

-treatment?

A

Vibrio cholerae

-supportive care OR doxycycline for severe illness

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6
Q

A patient presents with acute diarrhea after playing in the dirt. He is also found to have peripheral eosinophilia and a liver abscess.

What organism is most likely the culprit?

-treatment?

A

Entamoeba histolytica

-mebendazole

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7
Q

A patient presents with acute vomiting and diarrhea after eating reheated rice.

What organism is most likely the culprit? -management?

A

Bacillus cereus -management: supportive care

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8
Q

A patient presents with focal biliary cirrhosis. What condition is this pathognomonic for and what testing should you do?

A

Pathognomonic for CF! Need to do sweat chloride

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9
Q

An adolescent who has been in a body cast following orthopedic surgery starts vomiting profusely and complains of severe epigastric pain 2 weeks post-op.

What is the most likely diagnosis?

-treatment?

A

SMA syndrome -treatment:

  1. Lateral or prone positioning to shift duodenum away from obstructing structures and resme oral intake
  2. If that doesn’t work, may need NJ tube or TPN
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10
Q

An immunocompromised patient with HIV presents to you with fever, dysphagia, odynophagia, and retrosternal pain.

What is the most likely diagnosis?

A

Infectious esophagitis -common etiologies: candida, HSV, CMV

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11
Q

Complication of chronic pancreatitis?

A

Diabetes

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12
Q

Complications of acute pancreatitis?

A
  1. Pseudocyst
  2. Multi-organ system failure
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13
Q

For reflux, when would a barium swallow be useful?

A

Useful to rule out mechanical obstruction: malrotation, achalasia

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14
Q

How can you differentiate on clinical history GERD vs. EE?

A

EE usually does NOT respond to acid blockade therapy

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15
Q

How common is lactose intolerance in asians vs. blacks?

-is testing required?

A

Asians: 40% Blacks: 85%

  • testing is not required if symptoms improve with removal of dairy
  • hydrogen breath test if you need to confirm
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16
Q

How do you diagnose H. pylori?

A
  1. Hydrogen breath test
  2. Biopsy
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17
Q

How do you differentiate upper vs. lower GI bleed anatomically?

A

Upper = above ligament of Treitz

Lower = below ligament of Treitz (suspensory ligament attaching duodenum to connective tissue)

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18
Q

How do you perform:

  • rovsing’s sign?
  • psoas sign?
  • obturator sign?
  • sensitivity of these tests?
A

Rovsing’s sign: press in the LLQ, if the patient feels pain in RRQ, this is positive

Psoas sign = seen with retrocecal appendicitis -so remember that the psoas muscle is posterior SO, get the patient to roll onto their left side and EXTEND the right hip. If this causes the patient pain, this is a positive psoas sign

Obturator sign: obturator muscle causes internal rotation of the hip SO flex the patient’s hip and knee and internally rotate. If painful, this is a positive obturator sign

****These tests have high specificity but LOW sensitivity

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19
Q

How do you diagnose eosinophilic esophagitis?

A
  1. Bloodwork: peripheral eosinophilia, elevated IgE
  2. Endoscopy = gold standard = biopsy showing >15-20 eosinophils/hpf
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20
Q

How long does breastmilk jaundice last for? -peak? -how to confirm?

A

Can last up to 10 weeks -peaks at 5-15 days -confirm

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21
Q

How long should you try an H2 blocker for reflux before increasing dose, adding or switching therapies?

A

4-6 weeks of same dose

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22
Q

How many types of choledochal cysts are there?

  • which is most common type?
  • which population does it affect more: males or females?
A

5 types overall

  • Type I = most common = diltation of the common bile duct -affects females 4x more than males
  • type 5 = Caroli disease = intrahepatic bile duct cysts
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23
Q

In a patient with chronic constipation, what 3 signs are suggestive of a distal GI obstruction and should prompt further investigations?

A
  1. Narrow diameter stools (stools squeezing past an obstruction or Hirschsprung’s where not enough strength is generated to push the entire stool mass through)
  2. Abdominal distention
  3. Lack of encopresis (almost never see encopresis in Hirschsprung’s)
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24
Q

In a patient with suspected E. coli 0157:H, what test can isolate the organism?

A

Stool culture on sorbitol-MacConkey medium (won’t grow on regular stool cultures)

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25
Q

In what age group is volvulus most likely seen in?

-what is the definitive treatment?

A

Early infancy = more than 90% of cases present in first year of life BUT can present at any age

-definitive treatment: surgery (Ladd procedure)

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26
Q

What are the 3 main functional liver biochemical profiles in acute liver injury caused by hepatitis viruses?

A
  1. Cytopathic injury
    - rise in serum ALT and AST: magnitude of rise does NOT correlate with extent of hepatocellular necrosis and has little prognostic value
    - slowly improve over several weeks but lag behind serum bilirubin level (normalizes first)
    - rapidly falling ALT and AST in conjunction with increased bilirubin and INR can mean massive hepatic injury (injured or dead cells don’t produce enzymes)
  2. Cholestasis -elevated serum conjugated bilirubin from abnormal bile flow at the canalicular and cellular level due to hepatocyte damage and inflammation
    - can also have increased ALP and GGT
  3. Altered synthetic function -this should be MAIN FOCUS of monitoring -indication for prompt referral to transplant center if abnormal

a.-abnormal protein synthesis: decreased coags, decreased albumin

b.-metabolic disturbances: hypoglycemia, hyperammonemia, lactic acidosis

c.-hepatic encephalopathy: altered LOC with hyperreflexia

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27
Q

Liver failure Altered synthetic function profile?

A

Altered synthetic function-this should be MAIN FOCUS of monitoring -indication for prompt referral to transplant center if abnormal

a. -abnormal protein synthesis: decreased coags, decreased albumin
b. -metabolic disturbances: hypoglycemia, hyperammonemia, lactic acidosis
c. -hepatic encephalopathy:altered LOC with hyperreflexia

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28
Q

Liver failure Cholestasis profile?

A
  1. Cholestasis-elevated serum conjugated bilirubin from abnormal bile flow at the canalicular and cellular level due to hepatocyte damage and inflammation
    - can also have increased ALP and GGT
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29
Q

Liver failure Cytopathic injury profile?

A

  1. Cytopathic injury
    - rise in serum ALT and AST:magnitude of rise does NOT correlate with extent of hepatocellular necrosis and has little prognostic value
    - slowly improve over several weeks but lag behind serum bilirubin level (normalizes first)
    - rapidly falling ALT and AST in conjunction with increased bilirubin and INR can mean massive hepatic injury (injured or dead cells don’t produce enzymes)
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30
Q

Overview of management of acute pancreatitis?

A
  1. NPO –> can start feeds after 24-48 hrs if improved -enteral feeds are preferred to TPN if child is clinically tolerating and not septic
  2. Fluids fluids fluids –> give bolus and 1.5 maintenance
  3. Analgesia –> morphine
  4. Stop any possible aggravating factors
  5. Anti-emetics
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31
Q

What are 2 lab findings in eosinophilic esophagatisi? -appearance on endoscopy?

A
  1. Peripheral eosinophilia
  2. Elevated IgE
    - appearance on endoscopy: FURROWED esophagus
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32
Q

What are 2 metabolic causes of pancreatitis?

A
  1. Hypercalcemia
  2. Hypertriglyceridemia
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33
Q

What are 3 possible complications from untreated GERD?

A
  1. Esophageal strictures
  2. Barrett’s esophagus
  3. Adenocarcinoma
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34
Q

What are 3 types of gallstones?

A
  1. Cholesterol stones: increased secretion of cholesterol into bile (see in hyperlipidemia, obesity, pregnancy, females)
  2. Black pigment stones: increased conjugated bilirubin into bile (hemolytic disease, pancreatic insuffiency, TPN)
  3. Brown pigment stones: from bacterial and parasitic infections
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35
Q

What are 4 possible lab findings of CMPA?

A
  1. Increased IgE
  2. Increased eosinophils
  3. Increased platelets
  4. Decreased albumin
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36
Q

What are 8 intestinal manifestations of celiac disease?

  • what are 5 signs on physical exam of celiac disease?
  • what are 3 biopsy findings of celiac disease?
  • what are non GI manifestations of celiac disease?
A

Intestinal manifestations:

  1. FTT/wt loss
  2. Diarrhea (with occult blood loss)
  3. Irritability 4. Vomiting 5. Anorexia or excessive appetite 6. Foul-smelling, bulky stools 7. Abdominal pain 8. Rectal prolapse

Signs:

  1. Poor growth for both height and weight 2. Wasted muscles 3. Abdominal distention 4. Edema 5. Digital clubbing

Biopsy findings: 1. Villous atrophy 2. Crypt hyperplasia 3. Intraepithelial lymphocytosis

Non GI manifestations: 1. Dermatitis herptiformis 2. Dental enamel hypoplasia of permanent teeth 3. Iron deficiency anemia resistant to oral Fe

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37
Q

What are associated conditions with celiac disease? (5)

A
  1. Autoimmune thyroiditis
  2. Type 1 DM
  3. Down syndrome
  4. Turner syndrome
  5. IgA deficiency
  6. William’s syndrome
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38
Q

What are causes of small-intestinal flat villi? (8)

A
  1. Infection: Giardia 2. HIV 3. Intestinal TB 4.
  2. Diseases: Primary immunodeficiency, Crohn’s disease, Celiac’s
  3. Malnutrition
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39
Q

What are circumstances that air enema would not be effective in reducing intussusception? (2)

A
  1. Pathologic lead point
  2. Ileo-ileo intussusception (usually doesn’t respond to air enema, usually requires surgery)
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40
Q

What are clinical features of Giardia infection? (5)

A
  1. Recurrent abdmoinal pain 2. Cramping 3. Bloating 4. Weight loss 5. Intermittent diarrhea
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41
Q

What are common acute complications of Crohn’s disease?

A
  1. Abscesses.
  2. Perforation
  3. Toxic megacolon (less risk than UC)
  4. GI bleed
  5. Bowel obstruction
  6. Infection
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42
Q

What are common medication causes of pancreatitis? -2 general classes

A

anti-epileptics Valproic acid

chemotherapy

L-aspariginase Azathioprine Mercaptopurine

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43
Q

What are contraindications to air or barium enema in treating intussusception? (3)

A
  1. Perforation
  2. Hemodynamically unstable
  3. Signs of ischemia
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44
Q

What are extrahepatic causes of portal hypertension? (3)

A
  1. Portal vein thrombosis (most common)
  2. Portal vein agenesis/stenosis
  3. Splenic vein thrombosis
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45
Q

What are life threatening complications for UC? (5)

A
  1. Sepsis
  2. Primary sclerosing cholangitis
  3. Fulminant colitis
  4. Toxic megacolon/perforation
  5. Adenocarcinoma
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46
Q

What are organic causes of constipation? (6)

A
  1. Hypercalcemia
  2. Hypokalemia
  3. Hypothyroidism
  4. Celiac disease
  5. Hirschsprung’s
  6. Ulcerative colitis
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47
Q

What are risk factors for adenocarcinoma in the setting of a diagnosis of UC? (4)

A
  1. Disease > 10 years
  2. Onset before age 15 yo
  3. Pancolitis
  4. PSC
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48
Q

What are risk factors for developing peptic ulcer disease?

  • test for diagnosis?
  • treatment?
A
  1. Caffeine/alcohol/tobacco use
  2. G tubes
  3. NSAIDs
  4. Burn injuries
  5. Systemic illnesses: sepsis
  6. Steroids
    - gold standard for diagnosis: endoscopy with biopsy -

treatment:

  1. Avoid triggering substances
  2. PPIs
  3. Treatment of H pylori with clarithromycin +amoxil (abx x 2 wks) + PPI (x 1 mo) (OR amox + flagyl + PPI OR clarithro + flagyl + PPI)
  4. Surgery for severe and/or refractory cases
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49
Q

What are risk factors for TPN cholestasis? (5)

A
  1. Prematurity
  2. Low birth weight
  3. Sepsis
  4. Prolonged duration of TPN
  5. NEC
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50
Q

What are signs of meconium ileus on abdominal imaging (ie. AXR/contrast enema)?

-most common area of obstruction?

A

AXR: dilated bowel loops at level of obstructon (usually at terminal ileum)

-see bubbly, granular pattern at the level of obstruction -Contrast enema: microcolon from disuse

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51
Q

What are tests to order for diagnosing Hirschsprung’s? (3)

-which one is gold standard?

A
  1. Anorectal manometry: initial diagnostic test = will see failure of internal anal sphincter relaxation in response to dilating a balloon in the rectum
  2. Barium enema: delayed evacuation > 24 hrs
  3. Rectal biopsy = GOLD STANDARD = will see no ganglion cells, increased acetylcholinesterase staining
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52
Q

What are the 2 forms of autoimmune hepatitis?

-target population?

A

Type I autoimmune hepatitis: usually in young women aged 15-25 yrs, associated with other autoimmune conditions

-type II autoimmune hepatitis: occurs in young children

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53
Q

What are the 2 most common causes of bloody stools in infants < 6 mo?

A
  1. CMPA
  2. Anal/rectal fissure
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54
Q

What are the 2 most common complications of gastroschesis repair?

A
  1. Abdominal compartment syndrome
  2. NEC
55
Q

What are the 2 most common organisms causing spontaneous bacterial peritonitis? -empiric treatment?

A
  1. E. coli
  2. Klebsiella -empiric treatment: IV ceftriaxone
56
Q

What are the 3 most common causes of chronic/recurrent abdominal pain in children?

A

In order of prevalence:

  1. Functional abdominal pain
  2. Lactose intolerance
  3. Constipation
57
Q

What are the clinical features of Alagille syndrome? (6)

A
  1. Ocular abnormalities: posterior embryotoxin
  2. Vertebral anomalies: butterfly vertebrae
  3. Intrahepatic bile duct paucity
  4. Triangular face
  5. Cardiac defects: peripheral pulmonic stenosis, pulmonary valve stenosis, etc.
  6. Renal abnormalities
58
Q

What are the clinical features of esophageal perforation?

  • CXR findings? (4)
  • diagnosis?
  • treatment?
A
  1. Acute onset of severe epigastric/retrosternal pain and dysphagia
  2. Looks unwell with fever, tachycardia, abdominal pain -diagnose by obtaining C spine and CXR

-CXR findings:

  1. Retrosternal free air
  2. Subcutaneous emphysema
  3. Pneumomediastinum
  4. Free air under the diaphragm
    - Diagnosis: if xrays are normal but you have a high suspicion, get an esophagram with water-soluble contrast
    - treatment:

NPO, gastric drainage, broad spectrum IV abx for small esophageal tears in stable patients. If unstable or large esophageal teams, need emergent surgical repair

59
Q

What are the clinical manifestations of Wilson’s disease? -diagnosis?

-treatment?

A
  1. Hepatic: -asymptomatic hepatomegaly -subacute or chronic hepatitis -fulminant hepatic failure
  2. Neuropsychiatric: -behavioural changes -dystonia -tremors -Abnormal speech -deteriotation in school or job performance 3. Kayser-Fleischer rings
  3. Hemolytic anemia
    - diagnosis:

low serum ceruloplasmin, high serum or urine copper -

definitive diagnosis:

liver biopsy for copper content

-treatment: chelation therapy with penicillamine + zinc + vitamin B6 (penicillamine decreases zinc and vitamin B6 levels so need to replace AND zinc decreases GI absorption of copper)

60
Q

What are the common infectious causes of pancreatitis (5)?

A
  1. Viral:

EBV CMV

Hepatitis A & B

Mumps

  1. Mycoplasma
61
Q

What are the differences between Crohn’s disease and ulcerative colitis on gross pathology?

-on histology?

A

Gross pathology:

  • CD: cobble stoning, strictures, fistulas, fissures
  • UC: diffuse continuous inflammation

Histology:

  • CD: transmural involvement
  • UC: mucosal and submucosal inflammation, crypt abscesses
62
Q

What are the indications for a pH probe study in assessing for GERD?

A
  1. Assessing efficacy of acid suppression during treatment
  2. Evaluating apneic episodes in conjunction with a CXR
  3. Evaluating atypical GERD presentations such as chronic cough, stridor and asthma
63
Q

What are the infectious causes of bloody (ie. inflammatory) diarrhea? (7)

A

Inflammatory diarrhea: organisms and cytotoxins invade mucosa, killing mucosal cells = bleeding

-think EECYSTS

E coli (0157:H7)

E. histolytica

Campylobacter/C. diff

Yersinia

Shigella

Salmonella

Typhi

Strongyloides

64
Q

What are the limitations to barium contrast study in GERD investigation?

A

Good for studying the anatomy of the upper GI tract (esophageal strictures, hiatal hernia, gastric outlet or intestinal obstruction) but has poor sensitivity and specificity in the diagnosis of GERD since it has limited duration and the inability to differentiate between physiologic GER from GERD

65
Q

What are the management principles for fulminant hepatic failure? (6)

A
  1. Neurologic: avoid sedatives, limit protein intake (to decrease ammonia), neurovitals and monitor for increased ICP due to cerebral edema
  2. Respiratory: intubation to prevent aspiration due to impaired LOC
  3. GI: antacids, glucose control
  4. Renal: avoid hypovolemia (risks hepatorenal syndrome)
  5. Hematology: give vitamin K, FFP, platelets, etc
  6. Liver transplant (mortality is 70% without transplant)
66
Q

What are the possible extraintestinal manifestations seen in Crohn’s disease? (6)

A
  1. Spondyloarthropathy
  2. Pyoderma gangrenosum (painless, necrotic tissue)
  3. Erythema nodosum (PAINFUL)
  4. Aphthous ulcers
  5. Uveitis
  6. Primary sclerosing cholangitis
67
Q

What are the similarities (4) and differences (1) between Familial Adenomatous Polyposis and Gardner syndrome?

A

Similarities:

  1. Both are AD 2. Both have average onset in 2nd decade 3. Both present with rectal bleeding, abdo pain, bowel obstruction 4. Both have 100% risk of colon cancer

Difference: 1. FAP has adenomas in large intestine only while Gardner syndrome has adenomas in both small and large intestine

68
Q

What are the usual causes of and location of ulcers in:

  • primary peptic ulcer disease (1)
  • secondary peptic ulcer disease (2)
  • how to differentiate between gastric and duodenal ulcers clinically?
A

Primary PUD:

usually caused by H. pylori, duodenal location

Secondary PUD: gastric in location, usually caused by:

  1. hypergastrinemia
  2. Zolllinger-Ellison syndrome: autonomous gastrin secretion by gastrinoma, associated with MEN-I (recurrent, multiple, atypically located ulcers and treated with PPI and octreotide to inhibit tumor growth)

***gastric ulcers = pain worsens with meals, especially with spicy foods

***duodenal ulcers = pain improves with meals

69
Q

What condition must always be ruled out in a neonate presenting with bilious emesis?

  • diagnostic test?
  • Where is the obstruction in a patient with bilious emesis?
A

Malrotation with volvulus

  • UGI series with small bowel follow through and STAT gen surg consult
  • obstruction: distal to the ampulla of vater (halfway along the duodenum, formed by the pancreatic duct and common bile duct)
70
Q

What feeds are appropriate for short-gut syndrome?

-how to start feeds?

A

Protein-hydrolyzed formula with MCT in order to ensure absorption (ie. alimentum with medium chain triglycerides)

-start with TPN, then small enteral feeds, then slowly increase volume in order to increase pancreatic-biliary flow

71
Q

What findings on physical examination can help differentiate between constipation vs. Hirschsprung’s? (3) -on history? (2)

A

Hirschsprung’s:

  1. Abdominal distension
  2. Empty rectum on DRE with possible explosive passage of stool
  3. Possible poor weight gain
    - on history:
  4. Onset of constipation at birth (for functional constipation, usually after 2 yr of age)
  5. Encopresis VERY rare
72
Q

What imaging should always be ordered for all cases of direct hyperbilirubinemia and should always be your initial imaging study of choice?

A

Abdominal U/S

73
Q

What imaging study can help differentiate between biliary atresia and neonatal hepatitis in a neonate presenting with conjugated hyperbilirubinemia?

A

HIDA scan (radionuclide scan)

-in biliary atresia:hepatic uptake of radionuclide is normal but there is no excretion into the intestines

-in neonatal hepatitis: hepatic uptake of radionuclide is normal but excretion into the intestines is normal

74
Q

What is a common cause of perianal dermatitis in toddlers and school-age children?

  • clinical features?
  • diagnosis?

–work-up to rule out other causes?

-treatment?

A

GAS!

  • clinical features: perianal erythema, pain, pruritis
  • diagnosis: rapid strep test or culture
  • workup: CBC, CRP, ESR, gonorrhea/chlamydia swabs, tape test for pinworms
  • treatment: 10 d course penicillin
75
Q

What is achalasia?

  • gold standard for diagnosis?
  • classic sign on UGI?
A

Loss of LES relaxation due to high resting LES pressure and absent or non-peristaltic esophageal contractions

  • gold standard for diagnosis: esophageal manometry
  • UGI = “bird’s beak” = tapering of esophagus
76
Q

What is ascending cholangitis? -treatment?

A

Bacterial infection of obstructed common bile duct usually due to stricture of stones -see Charcot’s triad or Reynold’s pentad -treatment: after diagnosis via ultrasound/ERCP/percutaneous transhepatic cholangiography, do NPO, antibiotics, stone removal or dilation via ERCP or PTC

77
Q

What is Charcot’s triad?

  • What is Reynold’s pentad?
  • seen in what condition?
A

Charcot’s triad: 1. Fever 2. RUQ pain 3. Jaundice

Reynold’s pentad: 1. Fever 2. RUQ pain 3. Jaundice 4. Hypotension 5. Altered mental status

****See both in ascending cholangitis

78
Q

What is malrotation and why does it increase risk for volvulus?

A

Developmental abnormality in which there is incomplete rotation of the midgut and thus, the cecum is in the RUQ instead of the RLQ and the mesentary is fixed on a narrow base -this places the small bowel at increased risk for twisting on the pedicle of mesentary

79
Q

What is Meckel diverticulum?

  • clinical features?
  • diagnostic test?
  • treatment?
A

Meckel diverticulum: remnant of embryonic yolk sac that results from failure of the omphalomesenteric/vitteline duct to involute.

  • clinical features:
    1. PAINLESS rectal bleeding (gastric mucosa secrets acid leading to ulceration and bleeding of intestinal mucosa)
    2. Can also present with intussusception or volvulus (acts as lead point)
  • diagnostic test: Meckel scan (technietium-99 radionuclide scan)
  • treatment: surgical excision
80
Q

What is NASH?

  • 2 risk factors?
  • treatment?
A

Non-alcoholic steatohepatitis -fatty infiltration of the liver

  • 2 risk factors:
    1. obesity
    2. type 2 DM
  • treatment: weight loss through diet and exercise
81
Q

What is perianal disease (tags, fissures, abscesses) more commonly seen in: CD or UC?

A

CD!

82
Q

What is Peutz-Jehgers?

  • clinical features
  • potential complications? (3)
A

Autsomal dominant disease causing hamartomas

  1. Hyperpigmented lesions on lips and oral mucosa (other mucosal areas can be involved too)
  2. Hamartomas in small and large bowel (also elsewhere in GI)

Complications:

  1. hamartomas can serve as lead point for intussusception
  2. GI malignancy: yearly colonoscopy needed
  3. Breast/testicular malignancy
83
Q

What is primary sclerosing cholangitis?

  • labwork?
  • imaging?
  • treatment:
A

Inflammation and fibrosis of hepatobiliary system (occurs in < 10% of IBD patients, mainly UC)

  • labwork: conjugated hyperbilirubinemia, elevated liver enzymes, positive ANA, positive antismooth muscle Ab -
    imaging: ERCP (beaded appearance of biliary tree)
  • treatment: None..liver transplant is not curable and there is poor prognosis due to increased risk of cholangiocarcinoma
84
Q

What is Sandifer syndrome?

A

Neck contortions (arching, turning of head) secondary to GERD

85
Q

What is SMA syndrome?

  • main risk factors (2)?
  • clinical features?
A

SMA syndrome = compression of the duodenum by the SMA against the aorta

-2 main risk factors:

  1. rapid weight loss from malnutrition or catabolic states = loss of mesenteric fat pad which collapses the duodenum in between the SMA and the aorta
  2. full body cast (extra-abdominal compression)
    - clinical features:
  3. Severe epigastric pain
  4. Vomiting
86
Q

What is the “hamburger sign” in appendicitis?

A

Refers to the fact that patients with appendicitis will usually not even want to eat their favourite food due to nausea/anorexia. Thus, if they’re hungry, it’s probably NOT appendicitis

87
Q

What is the antibiotic treatment for severe shigella acute diarrhea?

What is the risk of Antibiotic Rx

A

Azithromycin

-if it is NOT severe, do NOT treat as this may worsen chance of HUS (endotoxin release)

88
Q

What is the antibiotic treatment for severe yersinia acute diarrhea?

A

Ceftriaxone

89
Q

What is the black box warning for metoclopramide?

A

Chronic use (>3 mo) linked with tardive dyskinesia

90
Q

What is the charactsteristic pattern of tooth decay seen with prolonged use of a baby bottle?

-when should weaning from bottle occur?

A

Extensive maxillary decay (especially frontal) and posterior maxillary and manibular delay but NORMAL mandibular frontal teeth

-weaning from bottle: when they turn 1 yo

91
Q

What is the clinical criteria for diagnosis of fulminant hepatic failure?

-what percentage of cases are idiopathic?

A

Need 3/3:

  1. Biochemical evidence of acute liver injury
  2. No evidence of chronic liver disease
  3. Hepatic-based coagulopathy defined as:
    a. In presence of hepatic encephalopathy: PT > 15 sec or INR > 1.5 not corrected by vitamin K
    b. In absence of hepatic encephalopathy: PT > 20 sec or INR > 2 -50% of cases are idiopathic
92
Q

What is the criteria for acute pancreatitis?

A

Need 2/3

  1. Acute RUQ abdominal pain
  2. Elevated amylase or lipase at least 3x normal
  3. Imaging findings consistent with pancreatitis
93
Q

What is the definitive diagnostic test for peptic ulcer disease? -supportive test?

A

Endoscopy for biopsies and H pylori cultures

-supportive test: Hydrogen breath test

94
Q

What is the diagnostic criteria for irritable bowel syndrome?

A
  1. More than 1 week of symptoms for 2 or more months
  2. Abdominal discomfort or pain associated with at least 2 of the following:
    a. Improved with defecation
    b. onset associated with change in frequency of stool
    c. onset associated with change in form of stool
  3. No organic cause
95
Q

What is the differential diagnosis for conjugated hyperbilirubinemia?

  • intrahepatic (9)
  • extrahepatic (4)
A

Results from direct obstruction of bile flow from the liver into the biliary system, then into the intestine (can be from obstruction inside the liver or outside the liver) = aka cholestasis

Intrahepatic: 1. Alagille syndrome (congenital abnormalities of the intrahepatic biliary tract) 2. TPN 3. Wilson disease 4. Viral hepatitis 5. Autoimmune hepatitis 6. Metabolic disorders (ie. Dubin-Johnson syndrome) 7. Drug-induced cholestasis 8. Alpha-1 antitrypsin deficiency 9. Neonatal hepatitis

Extrahepatic: 1. Biliary atresia 2. Choledochal cyst 3. Abdominal mass 4. Obstructive gallstone

96
Q

What is the differential diagnosis for hematemesis +/- melena? (7)

A
  1. Swallowed blood (epistaxis, dental work, etc.)
  2. esophagitis
  3. gastritis
  4. peptic ulcer disease
  5. mallory-weiss tear
  6. esophageal varices
  7. vascular malformations
97
Q

What is the differential diagnosis for hematochezia? (9)1

A
  1. CMPA 2. NEC 3. Meckel diverticulum 4. Vasculitis (HSP) 5. Polyp 6. Intestinal or colonic ulcer 7. Colitis (infectious vs. IBD) 8. Vascular malformation 9. Anal fissure
98
Q

What is the differential diagnosis for unconjugated hyperbilirubinemia? (8)

A

Results from increased RBC turnover or impaired conjugation

  1. Breastmilk jaundice 2. Breastfeeding jaundice (neonatal physiologic jaundice) 3. Gilbert disease 4. Hemolytic anemia 5. Crigler-Najjar syndrome 6. Hypothyroidism 7. Cystic fibrosis 8. Viral hepatitis
99
Q

What is the differential diagnosis of hepatitis?

A
  1. Infectious: -viral: Hep A/B/C/D/E, adenovirus, coxsackie, enterovirus, EBV, HSV, HIV, VZV, paramyxovirus (RSV, mumps, measles) -non-viral: abscess, amebiasis, bacterial sepsis, histoplasmosis, leptospirosis, TB
  2. Autoimmune: autoimmune hepatitis, sclerosing cholangitis, SLE, JIA
  3. Metabolic: alpha-1 antitrypsin deficiency, tyrosinemia, Wilson’s
  4. Toxic: drug-induced (anti-seizure, chemo, acetaminophen)
  5. Anatomic: choledochal cyst, biliary atresia
  6. Hemodynamic: shock, congestive heart failure
  7. Non-alcoholic fatty liver disease: idiopathic, Reye syndrome
100
Q

What is the initial imaging study of choice for a patient presenting with hepatosplenomegaly?

A

Abdominal U/S

101
Q

What is the initial testing for celiac disease?

-definitive diagnosis?

A

Initial testing: elevated tissue transglutaminase (can be falsely negative in IgA deficiency so ALWAYS order an IgA with tissue transglutaminase)

-definitive diagnosis: small intestinal biopsy showing villous atrophy

102
Q

What is the management principles of portal hypertension? (4)

A
  1. Treament of underlying disease
  2. Medications to decrease portal pressure (beta blockers, vasopressin)
  3. Surgical portosystemic shunt +/- liver transplant
  4. Treat complications (ie. esophageal varices)
103
Q

What is the most common cause of acute liver failure in children?

A

Viral hepatitis

104
Q

What is the most common cause of diarrhea resulting in hospitalization in children?

A

Rotavirus

105
Q

What is the most common cause of indirect hyperbilirubinemia in a patient > 1 month of age?

A

Gilbert disease = benign disorder of mildly impaired bilirubin conjugation found in 5-10% of population

106
Q

What is the most common cause of protein losing enteropathy?

  • clinical features?
  • treatment?
A

CMPA

  • clinical features: edema, hypoalbuminemia, iron deficiency anemia, vomiting, bloody stools, irritability
  • Treatment:

quick response to removal of cow’s milk protein (remember that 50% react also to soy protein)

  • need hydrolyzed protein formula (nutramigen, progestamil, alimentum) OR if this fails, then use amino-acid based formula (neocate)
  • most grow out of CMPA and tolerate cow’s milk by 2 yr
107
Q

What is the most common condition requiring liver transplant in the pediatric population?

  • clinical presentation?
  • diagnosis?
  • treatment?
A

Biliary atresia: atresia of any portion of the extrahepatic biliary system = 50% of liver transplants are because of biliary atresia!

  • clinical presentation: conjugated hyperbilirubinemia in an otherwise healthy, growing infant in the first few months of life
  • diagnosis: elevated liver enzymes, conjugated hyperbilirubinemia, HIDA scan showing lack of excretion of tracer from liver into the intestinal tract -definitive diagnosis: liver biopsy showing bile duct proliferation and widened portal area
  • treatment: Kasai procedure, eventual liver transplant
108
Q

What is the most common lead point in an older child with intussusception?

A

Meckel diverticulum

109
Q

What is the most common of lower intestinal obstruction in neonates?

-male:female ratio?

A

Hirshsprung/s disease

-M:F = 4:1 for short segment disease

110
Q

What is the most common presenting complaint of a patient with hepatitis?

A

Jaundice

111
Q

What is the most common syndrome associated with TEF?

A

VACTERL

  • Vertebral anomalies
  • Anal atresia
  • Cardiac defects
  • TracheoEsophageal fistula
  • Renal abnormalities
  • Limb abnormaliies
112
Q

What is the most serious complication of ulverative colitis?

A

Toxic megacolon

113
Q

What is the order of teeth eruption in children?

A
  1. Mandibular central incisors: 5-7 mo
  2. Maxillary central incisors: 6-8 mo
  3. Lateral incisors (mandibular first, then maxillary): 7-11 mo
  4. First molars: 10-16 mo
  5. Cuspids: 16-20 mo
  6. 2nd molars: 20-30 mo
114
Q

What is the pathophysiology of hereditary tyrosinemia?

  • clinical features?
  • what do you see on labwork?
  • treatment?
A

AR condition = deficient fumarylacetoacetate hydrolase = affects liver, kidney, peripheral nervous system

  • clinical features (begin in infancy):
    1. FTT 2. Jaundice 3. HSM with increased risk of hepatocellular carcinoma
  • labwork: 1. Elevated tyrosine 2. Elevated AFP 3. Elevated urine succinylacetone
  • treatment: dietary restriction of tyrosine, phenylalanine, methionine, medical management with tyrosine degradation inhibitor, supportive care for liver failure
115
Q

What is the pathophysiology underlying Hirschsprung disease?

A

Absence of ganglion cells in the submucosal and myenteric plexus = inadequate relaxation of the bowel wall with bowel wall hypertonicity

116
Q

What is the perforation rate for appendicitis in infants compared to the general population?

-reason for the difference?

A

Perf rate in infants: 50% Perf rate in general population: 10% -

due to delayed diagnosis/atypical presentation

117
Q

What is the purpose of always treating salmonella bacteremia in infants < 2 yo?

A

Salmonella bacteremia can quickly become invasive and cause sepsis, meningitis, especially in infants

118
Q

What is the rate of success of replantation of permanent teeth at: -30 minutes ->2 hrs

  • what to do if a permanent tooth is knocked out?
  • management of loss of primary tooth?
A
  • @ 30 minutes: 90% chance
  • @ > 2 hrs:

Loss of primary tooth - do not replace it since it may damage the underlying permanent tooth if shoved back in

119
Q

What is the rule of 2s for meckel diverticulum? (6)

A

2:1 M:F ratio 2.

2% of population 3.

2 feet above ileocecal valve 4.

2 year of age most common presentation 5.

2 inches in size 6.

2 types of tissue: gastric and pancreatic

120
Q

What is the the differential diagnosis for terminal ileitis? (5)

A
  1. Crohn’s disease
  2. Yersinia infection
  3. TB
  4. Lymphoma
  5. CGD
121
Q

What is the treatment for eosinophilic esophagitis?

A
  1. Dietary restriction of major food allergens (usually milk, soy, wheat, egg, peanuts and treenuts, seafood)
  2. Topical and systemic corticosteroids
122
Q

What is the treatment for IBD, in order of increasing disease severity? (7)

A
  1. Steroids
  2. Mesalamine
  3. Sulfasalazine
  4. azathioprine
  5. Methotrexate
  6. Infliximab
  7. Surgical resection
123
Q

What is the treatment for ruptured esophageal varices? -pharmacological vs. surgical

A

Pharmacological: IV octreotide

surgical: endoscopic sclerotherapy, band ligation or thrombin injection

124
Q

What is the treatment of a perforated appendicitis?

A

IV antibiotics = amp/gent/flagyl until symptomatically improved +/- percutaneous drainage of abscess if present

125
Q

What is thet treatment approach to GERD?

A
  1. Conservative therapy and lifestyle modification -infants: thickening of feeds or use of commercially prethickened formulas (ie. add 1 tbsp of rice cereal per oz of formula), short trial of hypoallergenic diet can be used to exclude milk allergy, positioning measures -older children: avoid reflux inducing foods such as tomatoes, chocolate, mint, juice, carbonated and caffeinated drinks, alcohol; weight reduction, stop smoke exposure
  2. Pharmacotherapy

-mild-moderate reflux esophagitis: histamine-2 receptor antagonists

  • severe reflux esophagitis: PPIs (more potent than H2 blockers but more expensive)
  • 2nd line: prokinetic agents (metoclopramide, erythromycin) = increase LES pressure or improve gastric emptying
126
Q

What is your ddx if AST >> ALT? (5)

-what is the most likely cause of an ALP is abnormally low?

A
  1. ETOH 2. Myopathy 3. Renal syndromes 4. Hemolyasis 5. Adenovirus infection

****Abnormally low ALP: think zinc deficiency

127
Q

What labwork confirms a diagnosis of autoimmune hepatitis? (3)

  • definitive diagnosis?
  • treatment?
A
  1. Hypergammaglobulinemia
  2. Positive ANA and anti-smooth-muscle antibodies (70% positive on presentation); some patients have anti-liver-kidney antibodies
  3. Elevated transaminases
    - definitive diagnosis: liver biopsy
    - treatment: steroids +/- azathioprine, may need liver transplant if ESLD
128
Q

What medication should you start when you’re making a patient NPO for a long period of time?

A

Ranitidine or prevacid

129
Q

What test should you order if you want to diagnose a carbohydrate malabsorption condition?

A

Stool reducing substances

130
Q

What three extraintestinal manifestations are more commonly seen in UC than CD?

A
  1. Pyoderma gangrenosum
  2. Primary sclerosing cholangitis
  3. Thrombosis
131
Q

Which do you see the following more commonly in, UC or CD? -toxic megacolon

  • risk for cancer
  • pANCA positive
A

All 3 are more common in UC

132
Q

Which medication classes are the most common causes of pill esophagitis? (3)

  • treatment?
  • prevention?
A

Pill esophagitis: irritation/erosion of the esophageal wall due to a pill temporarily lodging in the esophagus

  1. Antibiotics - specifically tetracycline, doxycycline, cilndamycin
  2. Anti-inflammatories - aspirin
  3. Bisphosphanates
    - treatment: stop the offending drug if possible; if not, switch to liquid formulation
    - prevention: always take pills standing upright, with at least 8 oz of water
133
Q

Which test has high sensitivity and positive predictive value for bacterial gastroenteritis?

A

Stool fecal leukocytes

134
Q

You have just completed an air enema with successful reduction of an intussusception. What do you do now? a. admit for observation b. d/c home -what is the reduction rate with air enema? -what is the recurrence rate?

A

Admit for observation x 24-48 hrs = recurrence can occur -reduction rate with air enema: 80-95% -recurrence rate after reduction: 10%

Royal College Peds 2021 (55 decks)

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