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Pediatric Board Review > Gastroenterology > Flashcards

Gastroenterology Flashcards

0
Q

+ reducing substances in urine, fulmninant neonatal hepatic failure

A

Galactosemia

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1
Q

Neonatal metabolic diseases that may present as fulminant liver failure

A

Tyrosinemia, Fructosemia, Galactosemia

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2
Q

Liver failure after introduction of fruit juices

A

Fructosemia

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3
Q

Fulminant neonatal hepatic failure + succinylacetone in urine

A

Tyrosinemia

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4
Q

Test for diagnosing hepatitis A

A

Hepatitis A IgM

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5
Q

Percentage of children infected with Hepatitis A that go on to fulminant liver failure

A

<1%

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6
Q

Extrahepatic manifestations like rash and arthralgias

A

Think Hepatitis B

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7
Q

Percentage of neonates that will not clear the hepatitis B antigen

A

90% versus <10 adults

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8
Q

This chronic hepatitis carrier state has a high association with cirrhosis and hepatocellular carcinoma

A

Hepatitis B

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9
Q

Positive HepBsAg

A

Acute infection

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10
Q

HBeAg

A

All about level of viral replication

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11
Q

HepBsAb

A

Only Ab that gives protection from the Hepatitis B virus

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12
Q

HepBcAb

A

Rises early in course of Hepatitis B, before HepBsAb

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13
Q

Hepatitis A and B treatment

A

Symptomatic management

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14
Q

Hepatitis B prevention in children born to mothers with Hepatitis B

A

Hepatitis B vaccine + HBIg shortly after birth

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15
Q

Which of the Hepatitis Viruses is a DNA virus?

A

Hepatitis B

Hepatitis A + C are RNA viruses

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16
Q

Percentage of children exposed to Hepatitis C that develop chronic disease

A

75%

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17
Q

These two hepatitides have a high rate of cirrhosis and hepatocellular carcinoma

A

Hepatitis B + C

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18
Q

Gold standard for Hepatitis C diagnosis

A

Hepatitis C DNA PCR

Hepatitis C Ab has a high rate of false positive an negative results

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19
Q

Treatment for chronic Hepatitis C

A

Ribavirin and Interferon

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20
Q

Hepatitis D

A
  • Occurs in coinfection with Hepatitis B –> worse course
  • Eastern Europe and Mediterranean
  • Diagnosis = IgM to Hepatitis D
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21
Q

Hepatitis E

A
  • Very similar to Hepatitis A
  • Short incubation period
  • Epidemics/endemics with contaminated water
  • Developing countries
  • No chronic disease
  • Hepatitis E Ab test through CDC
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22
Q

Other viruses that cause hepatitis in childhood…

A
  • EBV –> transaminitis, +/- jaundice, adenopathy + hepatoSPLENOmegaly
  • CMV, HSV, Varicella in immunocompromised pts
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23
Q

Drugs associated with hepatotoxicity

A
  • Acetaminophen
  • Isoniazid
  • Anticonvulsants - Valproic Acid
  • Anesthetics - Halothane
  • Onc - MTX, mercaptopurine, 6MP
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24
Q

This mushroom, when ingested, leads to hepatitis

A

Amanita phalloides

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25
Q
  • Viral illness
  • Aspirin use
  • Acute liver failure
  • Cerebral edema
A

Reye’s Syndrome

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26
Q

Cutoff for chronic hepatitis

A

6 months

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27
Q

Management of chronic liver failure

A
  • Symptomatic
  • Ascites –> fluid + sodium restriction
  • Encephalopathy –> limit protein to 1-1.5g/kg/day, lactulose, rifaximime
  • Liver transplantation ultimately
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28
Q

Chronic hepatitis B treatment

A
  • Interferon alone

- +/- Lamiduvine - develop resistance

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29
Q

Chronic hepatitis C treatment

A

Ribavirin and Interferon - combination therapy better

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30
Q

Liver disease in childhood, lung disease in adulthood

A

Alpha 1 antitrypsin

- ZZ phenotype

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31
Q

Findings in Wilson’s disease

A
  • Deposition of copper in CNS, liver, kidney
  • Increased: 24 urine Cu exretion, copper content in liver on biopsy
  • Decreased: serum Cu, serum ceruloplasm
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32
Q

Teenager with acute liver failure and hemolytic anemia

or…chronic hepatitis leading to cirrhosis

Can also present with neurological symptoms - personality changes

A

Wilson’s disease

Kayser Fleisher rings - (liver dz with eye image – think Wilson’s or Alagille’s Syndrome [posterior endotoxin])

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33
Q

Treatment for Wilson’s

A

Penicillamine or Trientene

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34
Q

Tyrosinemia

A
  • AR
  • can present as acute liver failure in an infant
  • untreated, by age 3, high percentage will develop hepatocellular carcinoma
  • May have neurologic symptoms and renal impairment (lose phosphorus in urine)
  • Rx: limit tyrosine in diet, NTBC stops degradation of tyrosine into toxic metabolites
  • Children with cirrhosis, transplant?
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35
Q

Progressive Familial Intrahepatic Cholestasis

A

Defects of bile acid metabolism or secretion

  • mimic chronic hepatitis
  • PRURITUS significant (also in Alagille’s)
  • Rx: symptom management, liver transplantation
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36
Q

Adolescent females

- Elevated total protein with normal/low albumin

A

Autoimmune hepatitis

Rx: immunosuppression, steroids for induction, mercaptopurine or azothioprine for maintenance

37
Q

Most common form of obstructive jaundice occuring in the neonatal period

A

Biliary Atresia

  • 1:10,000
  • Full term infants with conjugated hyperbilirubinemia in 1st month of life
  • Classic: complete atresia of external biliary system (bile ducts draining liver are not formed properly)
  • Jaundice >2 weeks of life must be worked up
  • Rx: Kasai or portoenterostomy procedure - must be done before 3 months of life because have higher success rates
38
Q

Percentage of biliary atresia pts, even with Kasai procedures, that go on to require liver transplantation

A

75%

39
Q
  • Paucity of intrahepatic bile ducts - within liver parenchyma (portal triads 50% missing bile duct)
  • Cardiac anomalies - PPS, TOF
  • Short stature
  • Typical facies - triangular, elongated facies
  • Butterfly vertebrae, especially in thoracic region
A

Alagille’s Syndrome - JAG1 mutation

40
Q

School-aged female + abdominal mass + RUQ pain + intermittent jaundice
+/- conjugated hyperbilirubinemia

A

Choledochal cyst

41
Q

Chronic inflammation around bile ducts –> focal areas of fibrosis –> narrowing and dilation of bile ducts

  • rare in children
  • associated with UC
  • may present with fever, abd pain +/- jaundice
  • liver may be enlarged
A

Sclerosis cholangitis

Dx: percutaneous cholangiogram or ERCP –> see “beads on a string”

42
Q

Individuals with sclerosing cholangitis are at increased risk of developing this cancer

A

Cholangiocarcinoma

43
Q

Gall stones in children

A

R/o hemolytic disorders, think about CF

44
Q

Colicky RUQ pain referred to right scapula + jaundice + elevated bili + elevated AP

A

Cholecystitis

  • E.coli, enterococcus
  • Dx: US (thickening of gall bladder wall) or HIDA scan
45
Q

Acalculous cholecystitis, associations…

A
  • Systemic infections (enteric infections - salmonella/shigella, viral)
  • Rx: cholecystectomy one inflammation improves
47
Q

Epigastric pain, vomiting, elevated amylase + lipase

A

Pancreatitis

48
Q

Basal energy needs are MOST ACCURATELY calculated by…

A

BSA

49
Q

The predominant protein in BM that is easily digestible, promotes gastric emptying and is a protein with high quality biologic value.

A

Whey

50
Q

Protein content in human milk

A

9g/L –> 7.2g/L nutritionally available

- IgA, lactoferrin and lysozyme (30%)

51
Q

This type of triglyceride does not require intraluminal digestion or bile salts for absorption

A

MCT (8-10 carbons)

52
Q

Essential fatty acids

A
  1. Linoleic
  2. Linolenic

Both needed to make arachnodonic acid

53
Q
  1. Scaly dermatitis
  2. Hair loss
  3. Diarrhea
  4. Poor wound healing
  5. Growth failure
A

Essential Fatty Acid Deficiency (EFAD)

In infants, can present within 2 weeks of not receiving EFAs

54
Q

Patients at high risk for EFAD

A
  1. Premies who receive inadequate linoleic acid (no IL)
  2. Children with fat malabsorption from hepatobiliary or pancreatic disease
  3. Children receiving long term TPN without IL
55
Q

What is the protein content in breastmilk

A

60 whey:40 casein - mature BM
90 whey:10 casein - early BM
[cow’s milk formula 40:60]
[cow’s milk 20:80]

BM IS THE WHEY TO GO!

56
Q
  1. CHF
  2. Tachycardia
  3. Peripheral edema
A

Wt beriberi = fluid overload

THIAMIN (B1) DEFICIENCY

57
Q
  1. Neuritis
  2. Peripheral paresthesia
  3. Irritability
  4. Anorexia
A

Dry beriberi = more neurologic

THIAMIN (B2) DEFICIENCY

Seen in chronic EtOH related encephalopathy

58
Q
  1. Seborrheic dermatitis
  2. Angular stomatitis
  3. Photophobia
  4. Loss of visual acuity
  5. Burning and itching of eyes
  6. Corneal vascularization
  7. Glossitis
  8. Poor growth
A

Riboflavin (B2) deficiency

[described in premies on prolonged phototx]

59
Q
  1. Newborns –> convulsions (1951-infant formula prep destroyed B6)
  2. Older children –> peripheral neuritis (INH administration), seb deem, cheilosis, SIDEROBLASTIC ANEMIA 2/2 failure of iron utilization for Hgb synthesis
A

Pyridoxine (B6) Deficiency

60
Q
  1. Macrocytic anemia/hypersegmented neutrophils

2. CNS changes (ataxia, peripheral neuropathy)

A

B12 CYANOCOBALAMIN DEFICIENCY

High risk: BF infants of vegan mothers, Patients s/p distal sm bowel resection (B12 absorbed in ileum)

61
Q
  1. Bleeding problems (petechiae)
  2. Poor wound healing (collagen synthesis)
  3. Easy fractures of bone
  4. Friable bleeding gums with loose teeth
  5. Microcytic anemia (aids in Fe absorption)
A

ASCORBIC ACID (C) DEFICIENCY/SCURVY

62
Q

3D’s - dermatitis (sun-exposed skin), diarrhea, dementia

A

NIACIN (B3) DEFICIENCY/PELLAGRA

63
Q
  1. Macrocytic anemia/leukopenia
  2. Poor growth
  3. Impaired cellular immunity
  4. Glossitis
  5. Diarrhea
  6. Neural Tube Defects
A

FOLATE (B9) DEFICIENCY

64
Q

Goat’s milk is deficient in this

A

Folate (B9)

Also Methotrexate impairs folate absorption

65
Q
  1. Dermatitis
  2. Anorexia
  3. Glossitis
  4. Muscle pain
  5. Insomnia
  6. Alopecia
A

BIOTIN DEFICIENCY

[seen in infants given TPN free of biotin]

66
Q

Only 3 water-soluble vitamins that cause toxicity when given in excess:

A
  1. Niacin
  2. Vitamin B6/pyridoxine
  3. Vitamin C
67
Q
  1. Skin flushing
  2. Tingling
  3. Itching
  4. Dizziness
  5. Nausea
  6. Liver test abnormalities
A

Niacin toxicity

68
Q
  1. Sensory neuropathy
  2. Progressive ataxia
  3. Altered sense of touch & pain
A

Vitamin B6 (pyridoxine) toxicity

69
Q
  1. Nausea
  2. Diarrhea
  3. Cramps
  4. Kidney Stones (oxalate and cysteine nephrocalcinosis)
A

Vitamin C toxicity

70
Q
  1. Eye symptoms - NIGHT BLINDNESS, retinal degeneration, xerophthalmia (dryness), photophobia, conjunctivitis, keratomalacia, BITOT’s SPOTS
  2. Follicular hyperkeratosis
  3. Poor growth
  4. Impaired resistance to infection
A

VITAMIN A (RETINOL) DEFICIENCY

71
Q
  1. Widening of epiphyses with thickening of wrists and ankles
  2. Chostochondral beading
  3. Bowing
  4. Craniotabes (persistently open anterior fontanelle/softening of the skull
A

VITAMIN D DEFICIENCY/RICKETS

72
Q
  1. Hemolytic anemia in premature infants
  2. Neurological changes - ataxia, hyporeflexia, decreased vibratory and position sensation, proximal muscle weakness, ophthalmoplegia/retinal dysfunction, neurocognitive changes
A

VITAMIN E DEFICIENCY

73
Q
  1. Easy bruisability/soft-tissue hemorrhage

2. Defective bone & collagen production

A

VITAMIN K DEFICIENCY

II, VII, IX, X, protein C&S

74
Q
  • HA
  • Anorexia
  • N/V
  • increased ICP (pseudotumor cerebri)
  • HSM
  • Bone pain
  • On retinoic acid
A

Vitamin A toxicity (can be teratogenic)

75
Q
  • Hypercalcemia
  • Anorexia
  • Vomiting, abd pain, constipation
  • Convulsions
A

Vitamin D

76
Q
  1. Myositis
  2. Cardiomyopathy
  3. Macrocytic anemia
  4. Loss of skin and hair pigmentation
A

SELENIUM DEFICIENCY

77
Q
  1. Rash
  2. Poor wound healing
  3. Impaired cellular immunity
  4. Anorexia
  5. Diarrhea
  6. Growth failure
  7. Co-factor for many enzymes

(low alk phos in malnourished pt)

A

ZINC DEFICIENCY

78
Q

SKIN - bullous and pustular lesions of oral, anal, genital areas & extremities
Alopecia
Diarrhea
EYES - blepharitis, conjunctivitis, photophobia, corneal opacities

A

ACRODERMATITIS ENTEROPATHICA

  • AR
  • Defect in zinc absorption
79
Q
  1. Microcytic anemia which does not respond to Fe supplementation
  2. Neutropneia
  3. Depigmentation of hair and skin
  4. Bone abnormalities
A

COPPER DEFICIENCY

80
Q

2-3m/o M with:

  • loss of milestones/neurodegenerative disease/truncal hypotonia/seizures
  • FTT
  • Lightly pigmented kinky hair
  • Connective tissue and skeletal abnormalities
A

Menkes Disease

  • X-linked
  • Impaired transport of dietary Cu from the intestine
81
Q

This type of malnutrition results from a severe deficiency of protein and less than adequate caloric intake

A

Kwashiorkor

82
Q

This type of malnutrition results from inadequate caloric intake only

A

Marasmus

83
Q

What nutrients are deficient in Crohn’s disease?

A
  • Iron
  • Folic acid
  • Vitamin B12
  • Zinc
  • Selenium
  • Vitamin ADEK
  • Calcium
  • Magnesium
84
Q

Premature neonates should consume formula with increased ________-chain FA

A

Medium

85
Q

Breath hydrogen test

A
  • One of the best ways to detect carbohydrate malabsorption
  • Breath sampled after patient ingests carbohydrate substrate
  • Rise > 10 to 20ppm in expired hydrogen = carbohydrate malabsorption
86
Q

Small intestinal biopsy

A

Detects celiac disease

87
Q

Colonoscopy

A

Detection of IBD

88
Q

Juices with high sorbitol

A

Prune
Apricot nectar
Apple
Pear nectar

89
Q

Presents at birth with diarrhea, dehydration and metabolic acidosis in breast and lactose containing formula fed infants

A

Congenital glucose-galactose malabsorption

  • AR
  • Fructose = only carbohydrate tolerated
90
Q
  • 10% eskimos have it
  • diarrhea, abd pain and or bloating with ingestion of sucrose
  • starch and cornstarch (isomaltose) also cause symptoms
  • symptoms variable in onset, depends on time of introduction of juices, fruits, cereals
A

Congenital sucrase isomaltase deficiency

- AR

Pediatric Board Review (17 decks)

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