Gastroenterology Flashcards
(90 cards)
0
Q
+ reducing substances in urine, fulmninant neonatal hepatic failure
A
Galactosemia
1
Q
Neonatal metabolic diseases that may present as fulminant liver failure
A
Tyrosinemia, Fructosemia, Galactosemia
2
Q
Liver failure after introduction of fruit juices
A
Fructosemia
3
Q
Fulminant neonatal hepatic failure + succinylacetone in urine
A
Tyrosinemia
4
Q
Test for diagnosing hepatitis A
A
Hepatitis A IgM
5
Q
Percentage of children infected with Hepatitis A that go on to fulminant liver failure
A
<1%
6
Q
Extrahepatic manifestations like rash and arthralgias
A
Think Hepatitis B
7
Q
Percentage of neonates that will not clear the hepatitis B antigen
A
90% versus <10 adults
8
Q
This chronic hepatitis carrier state has a high association with cirrhosis and hepatocellular carcinoma
A
Hepatitis B
9
Q
Positive HepBsAg
A
Acute infection
10
Q
HBeAg
A
All about level of viral replication
11
Q
HepBsAb
A
Only Ab that gives protection from the Hepatitis B virus
12
Q
HepBcAb
A
Rises early in course of Hepatitis B, before HepBsAb
13
Q
Hepatitis A and B treatment
A
Symptomatic management
14
Q
Hepatitis B prevention in children born to mothers with Hepatitis B
A
Hepatitis B vaccine + HBIg shortly after birth
15
Q
Which of the Hepatitis Viruses is a DNA virus?
A
Hepatitis B
Hepatitis A + C are RNA viruses
16
Q
Percentage of children exposed to Hepatitis C that develop chronic disease
A
75%
17
Q
These two hepatitides have a high rate of cirrhosis and hepatocellular carcinoma
A
Hepatitis B + C
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
19
Q
Treatment for chronic Hepatitis C
A
Ribavirin and Interferon
20
Q
Hepatitis D
A
- Occurs in coinfection with Hepatitis B –> worse course
- Eastern Europe and Mediterranean
- Diagnosis = IgM to Hepatitis D
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
22
Q
Other viruses that cause hepatitis in childhood…
A
- EBV –> transaminitis, +/- jaundice, adenopathy + hepatoSPLENOmegaly
- CMV, HSV, Varicella in immunocompromised pts
23
Q
Drugs associated with hepatotoxicity
A
- Acetaminophen
- Isoniazid
- Anticonvulsants - Valproic Acid
- Anesthetics - Halothane
- Onc - MTX, mercaptopurine, 6MP
24
This mushroom, when ingested, leads to hepatitis
Amanita phalloides
25
- Viral illness
- Aspirin use
- Acute liver failure
- Cerebral edema
Reye's Syndrome
26
Cutoff for chronic hepatitis
6 months
27
Management of chronic liver failure
- Symptomatic
- Ascites --> fluid + sodium restriction
- Encephalopathy --> limit protein to 1-1.5g/kg/day, lactulose, rifaximime
- Liver transplantation ultimately
28
Chronic hepatitis B treatment
- Interferon alone
| - +/- Lamiduvine - develop resistance
29
Chronic hepatitis C treatment
Ribavirin and Interferon - combination therapy better
30
Liver disease in childhood, lung disease in adulthood
Alpha 1 antitrypsin
| - ZZ phenotype
31
Findings in Wilson's disease
- Deposition of copper in CNS, liver, kidney
- Increased: 24 urine Cu exretion, copper content in liver on biopsy
- Decreased: serum Cu, serum ceruloplasm
32
Teenager with acute liver failure and hemolytic anemia
or...chronic hepatitis leading to cirrhosis
Can also present with neurological symptoms - personality changes
Wilson's disease
Kayser Fleisher rings - (liver dz with eye image -- think Wilson's or Alagille's Syndrome [posterior endotoxin])
33
Treatment for Wilson's
Penicillamine or Trientene
34
Tyrosinemia
- 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?
35
Progressive Familial Intrahepatic Cholestasis
Defects of bile acid metabolism or secretion
- mimic chronic hepatitis
- PRURITUS significant (also in Alagille's)
- Rx: symptom management, liver transplantation
36
Adolescent females
| - Elevated total protein with normal/low albumin
Autoimmune hepatitis
| Rx: immunosuppression, steroids for induction, mercaptopurine or azothioprine for maintenance
37
Most common form of obstructive jaundice occuring in the neonatal period
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
Percentage of biliary atresia pts, even with Kasai procedures, that go on to require liver transplantation
75%
39
- 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
Alagille's Syndrome - JAG1 mutation
40
School-aged female + abdominal mass + RUQ pain + intermittent jaundice
+/- conjugated hyperbilirubinemia
Choledochal cyst
41
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
Sclerosis cholangitis
| Dx: percutaneous cholangiogram or ERCP --> see "beads on a string"
42
Individuals with sclerosing cholangitis are at increased risk of developing this cancer
Cholangiocarcinoma
43
Gall stones in children
R/o hemolytic disorders, think about CF
44
Colicky RUQ pain referred to right scapula + jaundice + elevated bili + elevated AP
Cholecystitis
- E.coli, enterococcus
- Dx: US (thickening of gall bladder wall) or HIDA scan
45
Acalculous cholecystitis, associations...
- Systemic infections (enteric infections - salmonella/shigella, viral)
- Rx: cholecystectomy one inflammation improves
47
Epigastric pain, vomiting, elevated amylase + lipase
Pancreatitis
48
Basal energy needs are MOST ACCURATELY calculated by...
BSA
49
The predominant protein in BM that is easily digestible, promotes gastric emptying and is a protein with high quality biologic value.
Whey
50
Protein content in human milk
9g/L --> 7.2g/L nutritionally available
| - IgA, lactoferrin and lysozyme (30%)
51
This type of triglyceride does not require intraluminal digestion or bile salts for absorption
MCT (8-10 carbons)
52
Essential fatty acids
1. Linoleic
2. Linolenic
Both needed to make arachnodonic acid
53
1. Scaly dermatitis
2. Hair loss
3. Diarrhea
4. Poor wound healing
5. Growth failure
Essential Fatty Acid Deficiency (EFAD)
In infants, can present within 2 weeks of not receiving EFAs
54
Patients at high risk for EFAD
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
What is the protein content in breastmilk
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
1. CHF
2. Tachycardia
3. Peripheral edema
Wt beriberi = fluid overload
THIAMIN (B1) DEFICIENCY
57
1. Neuritis
2. Peripheral paresthesia
3. Irritability
4. Anorexia
Dry beriberi = more neurologic
THIAMIN (B2) DEFICIENCY
Seen in chronic EtOH related encephalopathy
58
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
Riboflavin (B2) deficiency
[described in premies on prolonged phototx]
59
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
Pyridoxine (B6) Deficiency
60
1. Macrocytic anemia/hypersegmented neutrophils
| 2. CNS changes (ataxia, peripheral neuropathy)
B12 CYANOCOBALAMIN DEFICIENCY
High risk: BF infants of vegan mothers, Patients s/p distal sm bowel resection (B12 absorbed in ileum)
61
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)
ASCORBIC ACID (C) DEFICIENCY/SCURVY
62
3D's - dermatitis (sun-exposed skin), diarrhea, dementia
NIACIN (B3) DEFICIENCY/PELLAGRA
63
1. Macrocytic anemia/leukopenia
2. Poor growth
3. Impaired cellular immunity
4. Glossitis
5. Diarrhea
6. Neural Tube Defects
FOLATE (B9) DEFICIENCY
64
Goat's milk is deficient in this
Folate (B9)
| Also Methotrexate impairs folate absorption
65
1. Dermatitis
2. Anorexia
3. Glossitis
4. Muscle pain
5. Insomnia
6. Alopecia
BIOTIN DEFICIENCY
[seen in infants given TPN free of biotin]
66
Only 3 water-soluble vitamins that cause toxicity when given in excess:
1. Niacin
2. Vitamin B6/pyridoxine
3. Vitamin C
67
1. Skin flushing
2. Tingling
3. Itching
4. Dizziness
5. Nausea
6. Liver test abnormalities
Niacin toxicity
68
1. Sensory neuropathy
2. Progressive ataxia
3. Altered sense of touch & pain
Vitamin B6 (pyridoxine) toxicity
69
1. Nausea
2. Diarrhea
3. Cramps
4. Kidney Stones (oxalate and cysteine nephrocalcinosis)
Vitamin C toxicity
70
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
VITAMIN A (RETINOL) DEFICIENCY
71
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
VITAMIN D DEFICIENCY/RICKETS
72
1. Hemolytic anemia in premature infants
2. Neurological changes - ataxia, hyporeflexia, decreased vibratory and position sensation, proximal muscle weakness, ophthalmoplegia/retinal dysfunction, neurocognitive changes
VITAMIN E DEFICIENCY
73
1. Easy bruisability/soft-tissue hemorrhage
| 2. Defective bone & collagen production
VITAMIN K DEFICIENCY
II, VII, IX, X, protein C&S
74
- HA
- Anorexia
- N/V
- increased ICP (pseudotumor cerebri)
- HSM
- Bone pain
- On retinoic acid
Vitamin A toxicity (can be teratogenic)
75
- Hypercalcemia
- Anorexia
- Vomiting, abd pain, constipation
- Convulsions
Vitamin D
76
1. Myositis
2. Cardiomyopathy
3. Macrocytic anemia
4. Loss of skin and hair pigmentation
SELENIUM DEFICIENCY
77
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)
ZINC DEFICIENCY
78
SKIN - bullous and pustular lesions of oral, anal, genital areas & extremities
Alopecia
Diarrhea
EYES - blepharitis, conjunctivitis, photophobia, corneal opacities
ACRODERMATITIS ENTEROPATHICA
- AR
- Defect in zinc absorption
79
1. Microcytic anemia which does not respond to Fe supplementation
2. Neutropneia
3. Depigmentation of hair and skin
4. Bone abnormalities
COPPER DEFICIENCY
80
2-3m/o M with:
- loss of milestones/neurodegenerative disease/truncal hypotonia/seizures
- FTT
- Lightly pigmented kinky hair
- Connective tissue and skeletal abnormalities
Menkes Disease
- X-linked
- Impaired transport of dietary Cu from the intestine
81
This type of malnutrition results from a severe deficiency of protein and less than adequate caloric intake
Kwashiorkor
82
This type of malnutrition results from inadequate caloric intake only
Marasmus
83
What nutrients are deficient in Crohn's disease?
- Iron
- Folic acid
- Vitamin B12
- Zinc
- Selenium
- Vitamin ADEK
- Calcium
- Magnesium
84
Premature neonates should consume formula with increased ________-chain FA
Medium
85
Breath hydrogen test
- 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
Small intestinal biopsy
Detects celiac disease
87
Colonoscopy
Detection of IBD
88
Juices with high sorbitol
Prune
Apricot nectar
Apple
Pear nectar
89
Presents at birth with diarrhea, dehydration and metabolic acidosis in breast and lactose containing formula fed infants
Congenital glucose-galactose malabsorption
- AR
- Fructose = only carbohydrate tolerated
90
- 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
Congenital sucrase isomaltase deficiency
| - AR
