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MedStudy Questions 2019 - RACP Paediatrics > Gastroenterology > Flashcards

Gastroenterology Flashcards

1
Q

What does vomiting of undigested food indicate?

A

Achalasia
Delayed ­gastric emptying
Rumination

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2
Q

What does bilious vomiting suggest?

A

GI obstruction beyond the ­duodenum (e.g.,malrotation)

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3
Q

What is cyclic vomiting?

A

Cycles of vomiting
Strong FHx migraines
Median age onset ~ 5 years
Slight femalepredominance

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4
Q

The finding of “currant jelly” stool suggests what possible diagnosis?

A

Intussusception

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5
Q

True or false? Abdominal pain that is nonorganic in nature presents with growth and development problems in the child.

A

FALSE

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6
Q

What is the most common cause of abdominal pain in children?

A

Functional abdominal pain

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7
Q

What clues are useful in diagnosing functional abdominal pain?

A

Pain periumbilical
Growth is normal and appetite is not affected
Symptoms get better on weekends and during vacations
Worse in the morning before school or in the evening before bed
Social stressors - recent divorce or trouble at school

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8
Q

What clues suggest an organic cause for abdominal pain?

A 
Vomiting
Weight loss
Hematochezia
Diarrhoea
Night time awakening from pain
Fever, rash, oral ulcers, joint pain
Melena
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9
Q

What is the most common cause of diarrhea in children?

A

Infectious

- Often Rotavirus

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10
Q

What are the indications for IV therapy in a child with diarrhea?

A

Shock
High stool output
Ileus
Monosaccharide intolerance

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11
Q

Do children with resolving diarrhea require special diets?

A

NO

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12
Q

When is diarrhea considered chronic?

A

3×/day for > 14 days

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13
Q

By what age does a patient with functional diarrhea (toddler’s diarrhea) typically obtain a normal stool pattern?

A

4 years of age

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14
Q

Is it rare for a breastfed infant to pass a stool less often than once every 5 days?

A

NO

Normal is 5-10days per BO

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15
Q

What is the treatment for infant dyschezia?

A

benign
Ressurance
no Tx

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16
Q

What is the most common cause of constipation in children?

A

Functional constipation

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17
Q

How do 90% of tracheoesophageal abnormalities present?

A

Blind upper EA

Fistual between lower oesophageal segment and the lower portion of the trachea, near the carina

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18
Q

What does VACTERL stand for?

A

V - Vertebral
A - Anal atresia
C - Cardiac (PDA, ASD, VSD)
T - TracheoEsophageal fistula
E - “ “
R - Renal (e.g, urethral atresia with hydronephrosis)
L - Limb anomalies (e.g, humeral hypoplasia, radial aplasia, hexadactyly, proximally placedthumb)

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19
Q

At the bedside, how do you diagnose esophageal atresia with distal tracheoesophageal fistula?

A

Failure to insert NGT

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20
Q

What is achalasia?

A

Disorder of the esophagus characterized by incomplete relaxation of the LES and a lack of normal esophageal peristalsis

MOTOR problem

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21
Q

True or false? Achalasia in infancy can be due to a congenital disorder?

A

TRUE

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22
Q

Is GE reflux a normal process for many infants?

A

YES

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23
Q

What is the initial treatment for GERD in infants?

A

Parental reassurance

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24
Q

Which children are at risk for having infection of the esophagus?

A

HIV
DM
Cancer
Long term glucocorticoid use

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25
Q

How long after ingestion of a caustic substance should upper endoscopy be performed?

A

12-24 hours after

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26
Q

What are the most common pills that cause pill-induced esophagitis?

A

Doxycyline, tertracycline, Aspirin, NSAID, Slow K

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27
Q

What is the best way to determine if an ingested coin is in the esophagus?

A

CXR - coronal plane (face forward)

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28
Q

Spontaneous esophageal perforation is rare but has increased frequency in patients with which 2 disorders?

A

Ehlers Danlos

Marfan syndrome

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29
Q

How does pyloric stenosis typically present?

A

Between 3 weeks and 2 months of age
Progressively worsening, nonbilious vomiting
Infant is hungry and eager to feed.

Hypochloremic hypokalaemic metabolic alkalosis

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30
Q

What type of acid-base disorder is seen with pyloric stenosis?

A

Hypochloremic hypokalaemic metabolic alkalosis

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31
Q

What causes stress gastropathy?

A

D/t severe physiologic stress:

  • Shock
  • Metabolic acidosis
  • Sepsis, burns
  • Head injury
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32
Q

What is the most common identifiable cause of chronic gastritis in children?

A

H pylori

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33
Q

What usually causes Ménétrier disease?

A

CMV

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34
Q

Which organism is responsible for most PUD in children?

A

H pylori

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35
Q

When and how do you diagnose H. pylori infection?

A

Urea breath test

Tissue testing

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36
Q

Are antibody tests useful in diagnosing active PUD?

A

No, only good for past exposure

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37
Q

What is the treatment for H. pylori infection?

A

2 weeks of:
PPI
Clarithromycin, plus
Metronidazole or Amoxicillin

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38
Q

What is Zollinger-Ellison syndrome?

A

Rare
Produces markedly excessive stomach acid due to a gastrin-secreting tumor (gastrinoma), typically located in the pancreas or duodenal wall

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39
Q

At what age do infants with malrotation classically present?

A

Bilious emesis in 1st month of life

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40
Q

What does an upper GI series show in an infant with malrotation?

A

Classic “bird’s beak” of the 2nd or 3rd portion of the duodenum, where the gut is twisted

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41
Q

At what age does intussusception usually occur?

A

2 months and 5 years

Peak 4 to 10 months

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42
Q

Describe the presenting symptoms of a child with intussusception?

A

Repeated episodes of severe abdominal pain interspersed with asymptomatic intervals. Vomiting (if obstruction occurs) and hematochezia (if bowel ischemia occurs) are symptoms aswell.

Current jelly stool
Sausage like mass in abdomen

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43
Q

What is the diagnostic procedure of choice in patients with suspected intussusception?

A

Air-contrast enema

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44
Q

What is the “rule of 2s” in Meckel diverticulum?

A

Present in 2% of the population (usuallyasymptomatic)
Located within 2 feet of the ileocecalvalve
Measures 2 inches inlength
Measures 2 centimeters indiameter
2:1 male-to-femaleratio
Usually symptomatic before 2 years of age (if and when symptoms are actuallypresent)

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45
Q

What test is preferred to diagnose Meckel diverticulum?

A

Technetium-99m pertechnetate scan

also called the Meckel scan

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46
Q

Which is associated with other congenital anomalies—jejunoileal or duodenal atresia?

A

Duodenal = Downs sydnrome

Jejunoileal = NOT associated with congenital

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47
Q

What do abdominal plain x-rays show in neonates with duodenal atresia?

A

Double bubble

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48
Q

In the U.S., what is the common carbohydrate metabolism deficiency that starts after 2 years of age?

A

Lactase deficiency

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49
Q

What is the most common reliable test to diagnose lactase deficiency?

A

Breath hydrogen test

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50
Q

What does the absence of apoB result in?

A

Abetalipoproteinemia

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51
Q

What is Hartnup disease?

A

Defect in transport of free neutral amino acids. It results in a deficiency of nicotinamide synthesized from tryptophan and leads to pellagra-type findings.

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52
Q

How do children with abnormal zinc absorption present?

A

Acrodermatitis enteropathica
Bullous and pustular dermatitis

Additionally, alopecia, blepharitis, conjunctivitis, diarrhea, and FTT are common.

53
Q

Which dietary protein induces celiac disease?

A

Gluten

54
Q

How does the classic GI form of celiac disease present in children < 2 years of age?

A

Malabsorptive diarrhea, poor weight gain, abdominal distention, and proximal muscle wasting.
Iron def

55
Q

Children with Type 1 DM and selective IgA deficiency should be screened for what disease?

A

Coeliac disease

56
Q

What is congenital microvillus inclusion disease?

A

AR disorder
Most common cause of congenital diarrhea
If the villi of the small intestine are examined under a microscope, they show diffuse thinning of the mucosa without crypt hypertrophy or an inflammatory cell reaction.

57
Q

What organism causes Whipple disease?

A

Gram-positive actinomycete called Tropheryma whipplei

58
Q

What is the most common surgical emergency in children?

A

Appendicitis

59
Q

If the pain of appendicitis suddenly resolves, what has likely happened?

A

Rupture

60
Q

What is typhlitis?

A

Inflammation of the cecum

Mainly seen during periods of neutropenia in patients who are being treated for leukemia

61
Q

What are the 2 forms of IBD?

A

UC

Crohns

62
Q

What are the differences between UC and Crohn disease regarding bowel involvement, tissue involvement, granulomas, and perianal disease?

A

UC = PR bleeding, Colon only, Superficial ulcers, No granuloma, No perianal disease

Crohns = abdo pain, top to bottom, perianal disease common, full thickness skip lesions, Yes granulomas

63
Q

How do you diagnose UC?

A

Endocolon

64
Q

Which drugs are used for both the remission and maintenance of UC?

A

5 ASA

& Infliximab

65
Q

Which is transmural—Crohn’s or UC?

A

Crohns

66
Q

In which part of the GI tract does Crohn disease most commonly occur?

A

Terminal ileum

67
Q

Which is more likely to cause weight loss and growth problems, Crohn’s or UC?

A

Crohns!

68
Q

Is surgical therapy curative for Crohn disease?

A

No

69
Q

What is the difference in cancer risk between a solitary juvenile polyp and juvenile polyposis?

A

Solitary = no cancer risk

Polyposis = High long term risk of malignancy

70
Q

What are the presenting symptoms of Peutz-Jeghers syndrome?

A

GI hamartomatous polyps

& Mucocutaneous hyperpigmentation of the lips and gums.

71
Q

Is there an increased risk of cancer in patients with Peutz-Jeghers syndrome?

A

YES
Most common involving the breast, cervix, ovary, testicle, and pancreas;
2–13% develop colon cancer. Adenomatous changes are found in about 5% of Peutz-Jegherspolyps

72
Q

What does Gardner syndrome consist of?

A

Adenomatous polyposis of the colon, small bowel, duodenum, and stomach. Other findings include soft tissue tumors, extra teeth, osteomas, and congenital hypertrophy of the retinal pigment epithelium (CHRPE)

73
Q

In children, what is the most common malignant tumor of the small intestine?

A

Lymphoma

74
Q

What is an omphalocele?

A

Omphalocele is a ventral, midline defect in the abdominal wall at the umbilical region that can contain both hollow and solid visceral abdominal organs.
Omphaloceles are larger than 4 cm and are covered by peritoneal membrane internally and amniotic membrane externally

75
Q

If an omphalocele is present, should you suspect another congenital anomaly or is it likely an isolated event?

A

50–75% of neonates with an ­omphalocele have an associated congenital anomaly, including thoracoabdominal syndrome (known as the pentalogy of Cantrell), lower midline syndrome, and Beckwith-Wiedemann syndrome.
About 25% have major chromosomal abnormalities, including thetrisomies.

76
Q

What is a gastroschisis?

A

Gastroschisis presents as a 2–5 cm lateral abdominal wall defect, just to the right of the umbilicus, with exposed (no membrane!) loops of small and large intestines that are short and thick due to an inflammatory reaction of the serosa
No solid organs involved
Umbi cord normal

77
Q

True or false? Congenital anorectal disorders are usually isolated findings.

A

FALSE

Often part of VACTERYL

78
Q

Which chromosomal abnormality has an increased risk of imperforate anus?

A

Trisomy 21

79
Q

What are the 3 most common causes of rectal prolapse?

A 
1st = Constipation
2nd = Infectious diarrhoea
3rd = CF
80
Q

What is the most common cause of rectal bleeding in children?

A

Anal fissures

81
Q

What is the etiology of Hirschsprung disease?

A

Occurs in utero when neural crest cells fail to migrate from the craniocaudal region to the distal intestine.

82
Q

A term infant is 48 hours old and has not passed meconium. Is it necessary at this point to evaluate for Hirschsprung’s?

A

YES

83
Q

How does enterocolitis present in infants with Hirschsprung’s?

A

Occurs during the 2nd to 4th weeks of life and is characterized by fever with explosive, foul-smelling stools. Bloody diarrhea is common, as is abdominal distention
X-ray shows dilated loops of bowel with air-fluid levels

84
Q

What is the gold standard procedure used to diagnose Hirschsprung’s?

A

Rectal biopsy - absence of any ganglion cells

85
Q

A biopsy shows histologic absence of what structures in a patient with Hirschsprung disease?

A

Absence of any ganglion cells

86
Q

What is Shwachman-Diamond syndrome?

A

AR disorder
2nd most common cause of exocrine pancreatic insufficiency
-> Acinar cell hypoplasia, with intact function of the pancreatic ducts

Short stature
Intermittent or persistent neutropenia -> infections
Skeletal abnormalities
1/3 develop myeloproliferative malignancies

87
Q

What are the commonly identified causes of acute pancreatitis in children?

A

Biliary obstruction from gallstones
Idiopathic (many of these are probablyviral)
Blunt abdominaltrauma
Multisystemdisease

88
Q

What is Cullen sign? Grey Turner sign?

A

Signs of acute hemorrhagic pancreatitis

Cullen sign (bluish patchy discoloration around/near the umbilicus)
Grey Turner sign (bluish discoloration of the flanks from retroperitoneal hemorrhage—“Turn to your side to see your flank!”)
89
Q

Which radiologic test is usually most helpful in diagnosing acute pancreatitis?

A

USS

90
Q

What is the most common cause of chronic pancreatitis?

A

Idiopathic - most common

Hereditary causes

91
Q

Which radiologic modality is useful for diagnosing choledochal cysts?

A

Use ultrasound to show both intrahepatic and extrahepatic biliary tree dilatation.
Radionuclide scans can show cysts with accumulation of tracer.
ERCP or MRCP can be useful for determining theanatomy.

92
Q

What type of cancer commonly occurs in a choledochal cyst if it is not removed?

A

Cholangiocarcinomata

93
Q

What is Caroli disease?

A

Abnormality of the ductal plate and is due to a congenital dilatation of the larger, segmental intrahepatic bile ducts
AR
Presents with recurrent cholangitis and abscess

94
Q

What is Alagille syndrome?

A

AD with variable penetrance
Caused by mutations in a single gene, JAG1 (which encodes protein ligands for NOTCH1), on chromosome 20p.

Peripheral pulmonary artery stenosis
Occasionally TOF
Neonatal cholestasis - Conjugated

Classically, present with chronic cholestatic liver disease with a paucity of small intrahepatic ducts,
“butterfly” vertebrae,
abnormal radius/ulna,
posterior embryotoxon of the eye (a developmental abnormality marked by a prominent white ring of Schwalbe and iris strands that partially obscure the chamber angle), and
characteristic facies (prominent forehead; moderate hypertelorism; a small, pointed chin; and a saddle or straightnose)

95
Q

What are the cardiovascular findings seen with Alagille syndrome?

A

TOF

96
Q

How is hepatitis A transmitted from person to person?

A

Faecal oral route

97
Q

Which laboratory test is used to diagnose acute hepatitis A?

A

anti HAV IgM

98
Q

What can be given to household contacts to prevent spread of hepatitis A once a case has been identified?

A

Hep A Vaccine or Immunoglobulin within 2 weeks of exposure

99
Q

How is hepatitis B transmitted?

A

Sexual contact - most common in adolescents and adults
Contaminated body fluids
Contaminated needles
Transplacentally

100
Q

Which laboratory test indicates immunity to hepatitis B?

A

Anti HBS IgG positive

101
Q

Which laboratory test correlates with increased infectivity of the patient with hepatitis B?

A

Detecting both HBsAg and HBeAg indicates active virions and high infectivity

102
Q

What is the window period for hepatitis B infection?

A

Several weeks

103
Q

Hepatitis B is associated with which autoimmune reaction?

A

?

104
Q

Once infected with acute hepatitis B, who is more likely to develop chronic hepatitis B—an infant or an adolescent?

A

Children! 90% if affected at birth

5% if affects as teen

105
Q

In adults, chronic hepatitis B is associated with which 2 serious conditions?

A

Cirrhosis

HCC

106
Q

Prior to 1992, what was a common mode of transmission of hepatitis C?

A

Blood transfusion

107
Q

Which is more likely to cause chronic hepatitis—hepatitis B or hepatitis C?

A

HCV 70-80% vs. HBV 1%

108
Q

Hepatitis C is associated with which vasculitides?

A

Small vessel vasculitis with glomerulonephritis and neuropathy
Mixed cryoglobulinemia
Porphyria cutanea tarda

109
Q

Which coinfection does hepatitis D require to cause infection?

A

HBV infection

110
Q

How is hepatitis E transmitted?

A

Faecal oral

Just like Hep A

111
Q

In whom is hepatitis E most virulent?

A

3rd trimester pregnancy

112
Q

Can EBV cause significant liver disease?

A

occasionally, more if immunocompromised

113
Q

What is Gilbert syndrome?

A

Caused by a less severe mutation in UGT1A1 or its promoter.
This results in a mild, indirect hyperbilirubinemia, usually < 5 mg/dL.
No Tx

114
Q

How does Crigler-Najjar syndrome Type I differ from Crigler-Najjar syndrome Type II?

A

Crigler-Najjar syndrome Type I (CN I; severe deficiency inconjugation)

  • > kernicterus
  • unconjugated

Crigler-Najjar syndrome Type II (CN II; mild-to-moderate deficiency inconjugation)
- unconjugated

115
Q

What is Dubin-Johnson syndrome?

A

Causes conjugated hyperbilirubinemia
- (unlike Gilbert and Crigler-Najjar, which are unconjugated hyperbilirubinemias)

D/t deficiency in the cMOAT/MRP2 gene, which encodes the canalicular transporter of conjugated bilirubin

116
Q

How does α1-antitrypsin deficiency present?

A

Liver disease can present as neonatal jaundice, juvenile cirrhosis, chronic hepatitis, or HCC
Cholestatic jaundice in 10–15% of infants who are homozygous
50% have abnormal LFTs

117
Q

What are the eye findings in Wilson disease?

A

Kayser-Fleisher rings

118
Q

How do you diagnose Wilson disease?

A

Liver biopsy

119
Q

How does PFIC1 present?

A

3 and 6 months
Conjugated hyperbilirubinemia and severe, unremitting pruritus

But GGT is normal!!

120
Q

What drug is associated with Reye syndrome?

A

Aspirin

  • when have intercurrent viral illness with Chickenpox or Influenza
  • > Acute liver failure and hyper ammonia encephalopathy
121
Q

What drug is administered to patients with acetaminophen toxicity in order to replenish glutathione stores?

A

N-acetylcysteine replenishes glutathione stores and allows the liver to metabolize acetaminophen without generating toxic metabolites

122
Q

How do you diagnose autoimmune hepatitis?

A

Serum antibody markers
Elevated aminotransferases
Elevated total protein (due to hypergammaglobulinemia)

123
Q

What is associated with primary sclerosing cholangitis?

A

IBD - especially UC

ANCA +ve

124
Q

What is the best radiologic test to confirm primary sclerosing cholangitis?

A

Endoscopic retrograde cholangiography

- which shows alternating normal strictures along with dilated portions of the biliary tree, known as “beading”

125
Q

What tests should be done quickly to diagnose extrahepatic biliary atresia?

A

Abdominal ultrasound—this can show an absentgallbladder
Liver biopsy—this will often show ductular ­proliferation, bile plugs, and portalfibrosis

If these tests are suggestive of biliary ­atresia, do an intraoperative cholangiogram. If this is positive, the surgeon can perform a Kasai portoenterostomyimmediately.

126
Q

What procedure should be performed as soon as possible for extrahepatic biliary atresia?

A

Kasai procedure

127
Q

Which children are more likely to develop gallstones?

A 
Obesity
Hemolytic disease (particularly sickle cell)
Chronic TPN
Short bowel syndrome
DM
Oral contraceptives
And pregnancy in adolescent females
128
Q

Under what conditions are hydrops of the gallbladder found?

A 
Kawasaki syndrome
Streptococcal pharyngitis
Prolonged fasting
TPN
Henoch-Schönlein purpura (HSP)
129
Q

What is the most common malignant liver tumor of childhood? Which laboratory test is elevated?

A

Hepatoblastomata

Alpha fetoprotein HIGH

MedStudy Questions 2019 - RACP Paediatrics (4 decks)

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