GI 3 Flashcards
1
Q
Criteria for autoimmune hepatitis
A
- increased transaminases - usually 500-1000
- hypergammaglobulinemia
- positive NA Aand anti Sm antibodies (70%), antiliver-kidney antibodies in some
- liver biopsy - hepatic lobules and portal areas
2
Q
Treatment of autoimmune hepatitis
A
1st line steroids and azathioprine
2nd line: cyclosporine A, tacrolimus, mycophenolate mofetil
transplant for patients who progress to end stage
rate of remission 75% in 1-3 months, lots will relapse , can get recurrence in transplanted liver
3
Q
Wilson disease findings
A
LVR: asymptomatic hepatomegaly, subacute or chronic hepatitis, fulminant hepatic failure
Neuropsych: behavioural changes, deterioration in school or job performance, abnormal speech, tremors/dystonia
Rando: Kayser-Fleischer rings, hemolytic anemia, Fanconi syndrome
4
Q
Diagnosis of wilson disease
A
low ceruloplasmin
elevated serum and/or urine copper
increased ALP/AST but can be low with fulminant disease
5
Q
Treatment of wilson disease
A
chelation therapy - penicillamine restrict copper intake zinc to impair copper absorption liver transplant if severe without treatment it is fatal , fulminant ALF have poor prognosis
6
Q
Reye syndrome - which infections classically associated
A
influenza or varicella
often 1 week after
often associated with aspirin use
can progress to really bad neuro symptoms
labs: LFTs, NH3, prolonged PT, and/or hypoglycemia
7
Q
Treatment of nonalcoholic steatohepatitis
A
fatty infiltration of the liver in children with obesity and type II diabetes
usually asymptomatic, picked up on screening labs for obese patients
AST/ALT usually < 1, may or may not have hepatomegaly
diagnosis by liver US or biopsy
treatment by diet and exercise
8
Q
History of acute hepatic crisis precipitated by intercurrent illness, what diagnosis do you think about?
A
tyrosinemia (hereditary)
autosomal recessive
results from deficient activity of fumarylacetoacetate hydrolase, causes progressive dysfunction of LVR/KDN/Peripherap nervous system
labs: increased serum tyrosine, mehionine, elevated serum AFP
increased risk of hepatocellular carcinoma
9
Q
Treatment of tyrosinemia
A
dietary modifications, tyrosine degradation inhibitor, LVR transplanted if needed
10
Q
alpha 1 antitrypsin - how does it present in children?
A
neonatal cholestasis
childhood/adolescence- cirrhosis
diagnosis is by genetic testing
11
Q
Drugs which can cause cholestasis
A
chlorpromazine
erythromycin
estrogens
(table 12.14 in Oski) has other drugs too
12
Q
Drug which can cause biliary sludge
A
ceftriaxone
13
Q
Drugs that cause liver disease
A
acetaminophen, valproic acid, isoniazid, sulfonamids, phenytoin, methotrexate
OCP, steroids (can cause cancer)
14
Q
Causes of portal hypertension
A
extrahepatic obstruction - ie portal vein thrombosis
intrahepatic obstruction: hepatocellular disease, cirrhosis, Budd-Chiari syndrome, veno-occlusive disease, Alagill syndrome
idiopathic
definition: elevated by 10-12 mmHg of portal pressure
15
Q
Treatment of portal hypertension
A
treat underlying disease
meds to decrease portal pressure - beta blocker, vasopressin
shunt/liver transplant
treat complications - esophageal varices
16
Q
Most common cause of SBP
A
#1 E coli #2 Klebsiella, treat with IV cefotaxime while awaiting disease ID causes of ascites in children: LVR, heart filaure, protein losses, malnutrition, infectious, chylous, VP shunt dysfunction, hypo T and malignancy
17
Q
Definition of fulminant hepatic failure
A
- biochemical evidence of acute liver injury
- no evidence of chronic liver disease
- hepatic based coagulopathy
(PT >15 seconds, INR >1.5 not corrected by vitamin K in the presence of encephalopathy OR APT >20 seconds or INR >2 regardless of presence of clinical encephalopathy)
**need all 3 criteria
cause :50% idiopathic, rest are ingetsions, infections, other causes of injury
18
Q
management of patient in fulminant hepatic failure
A
neuro: avoid sedatives, limit protein, lactulose, ICP monitoring
Resp: intubation may be needed
GI: antacids, glucose control
renal: avoid hypovolemia
heme: vitamin K, FFP, plasmapheresis, platelets, liver transplantation
mortality 70% without transplant
19
Q
most common cause of cholestasis in 0-3 month term babies
A
biliary atresia
thought to be acquired disorder
HIDA scan - no excretion of tracer from liver into intestines , liver biopsy is definitive diagnosis
initial treatment: Kasai procedure, 80% ened liver transplant
20
Q
Alagille syndrome - 6 features
A
- intrahepatic bile duct paucity
- facial features - triangular shaped
- ocular (posterior embryotoxon)
- cardiac (pulmonary valve stenosis, peripherl pulmonic stenosis)
- vertebral arch - butterfly vertebrae
- renal anomalies
(don’t need all of them)
21
Q
Inheritance of Alagille syndrome
A
autosomal dominant
15-50% are spontaneous mutations
severity of liver disease is variable
treatment mainly supportive
22
Q
liver disease in born marrow transplant, mechanism?
A
GVHD, veno-occlusive disease
23
Q
liver disease in sickle cell mechanism
A
gallstones, hemosiderosis (chronic transfusions)
24
Q
Are cholodochal cysts more common in females or males?
A
4x more common in females
usually present in first 6 years of life
type 1 is the most common
25
Long term risk of cholodochal cyst
increased risk of cholangiocarcinoma
26
Conditions associated with gall bladder hydrous
1. hemoglobinopathies
2. Kawasaki
3. fasting
4. systemic infections
27
Medical conditions associated with gallstones
rare in children, usually associated with risk factor
1. hemolytic disease
2. hypercholesterolemia
3. obesity
4. CF
5. biliary tract malformations
patients with distal obstruction from gallstones can have pancreatitis at the same time
28
A patient with IBD presents with abdo pain, fever, jaundice, pruritus and hepatomegaly. ERCP shows a beaded appearance of biliary tree. What is the diagnosis
primary sclerosing cholangitis
direct hyperbili, transaminitis, increased serum Ig, + ANA, anti smooth muscle Ab
treatment: liver transplant not curable, poor prognosis due to increased risk of cholangiocarcinoma (50% 10-12 year survival)
<10% of IBD patients, rare in childhood
29
What is Charcot's triad and Reynol'd pentad?
signs for ascending cholangitis (which is treated with NPO, Abx, stone removal/dilation via ERCP vs PTC
Charcots triad: fever, jaundice, RUQ pain
Reynod's pentad: fever, jaundice, RUZ pain, hypotension, altered mental state
30
Which of the following symptoms has the highest risk of colorectal cancer?
a) familial adenomatous polyposis
b) Peutz-Jeghers
c) juvenile polyposis
a) FAP - 100% risk of colon cancer
poly - adenomals in large intestines (vs others which are large and small)
onset 2nd decade
rectal bleeding, abdo pain, bowel obstruction
**All the syndrome listed in Oski are autosomal dominant
31
Which of the following is not a feature of Peutz Jegher syndrome
a) adenoma
b) intussusception
c) mucosal pigmentation of lips
d) rectal bleeding
a) NOT adenoma, it's hamartomas of small and large intestines
the others are features
risk of colorectal cancer is 40%
32
Name 7 conditions which increase the risk of lymphoma
1. AIDS
2. ataxia telangiectasia
3. Wiscott-Aldrich syndrome
4. agammaglobulinemia
5. SCID
6. bone marrow/solid organ transplant
7. long standing celiac disease
most common GI malignancy in children
consider lymphoma in child >2 year old with intussusception
33
What type of tumour is often found incidentally during an appendectomy?
carcinoid tumour
34
What is carcinoid syndrome?
caused by carcinoid tumours outside the appendix which metastasize to the liver
diarrhea, vasomotor disturbances, bronchoconstriction, and/or right heart failure
dx: elevated urine serotonin metabolite - 5-hydroxyindoleacetic acid (5HIAA)
35
Please name the GI manifestations of CF
intestines - mec ileus, DIOS, GERD
pancreas - exocrine deficiency (malabsorption of fats, proteins, and lesser extent carbs; diabetes
Nutrition/Metabolism - vitamin and mineral deficiencies, edema and hypoproteinemia, salt loss
Hepatobiliary: focal biliary cirrhosis (pathognomonic for CF, can be as early as 3day, astymptomatic to hepatic failure), primary sclerosing cholangitis, portal hypertension
gallbladder - galstones - 1/3 of adult patients
36
Treatment of SMA syndrome
resolves when feeds are given in a way which bypasses the duodenum (i.e. NJ feeds) -in SMA syndrome the SMA loses it fat pad and compresses onto the duodenum
prone/lateral positioning
prokinetics
surgery in refractory cases
can confirm dx: with upper GI will demonstrate abrupt duodenal cutoff to the right of the midline
37
consequences of untreated meckel diverticulum
diverticulitis
intussusception
volvulus
38
difference between acrodermatitis enteropathica and dermatitis herpetiformis
dermatitis herpetiformis - in celiac disease
| acrodermatitis enteropathica - AR form and also form related to zinc deficiency
39
perianal fistula in a 1 year old?
relatively common in heath children of that age
usually heals on its own
if severe symptoms then might need fistulotomy and other treatment for kids > 2 years, more likely to have predisposing condition (immunocompromise, Crohn's, prior rectal surgery), may need antibiotics, surgery
40
3 possible consequences of choledochal cyst
cholangiocarcinoma
malrotation
cirrhosis
41
when can you start refeeding a patient with pancreatitis
when clinical symptoms better and amylase is decreasing
lipase elevated for 8-14 days so don't use as a marker to start feeds
clear diet then advance
42
True or false - Kayser -Fleischer rings are only caused by wilson disease
nope, other causes of cirrhosis with kayser Fleischer rings are:
1. autoimmune hepatitis
2. viarl hepatitis
3. alpha1 antitrypsin
4. drug toxicity
but most commonly associated with wilson disease
43
What are the findings of peritoneal fluid analysis in patients with exudative process causing peritonitis
```
increased total protein
increased ascites:plasma protein ratio
elevated ascites plasma LDH
low serum:ascites albumin
WBC > 500 with >50% pmns
```
44
with SBP?
```
protein is likely lower than expected
transudative process
protein 1.1
WBC <33% PMNs
no organisms on gram stain
```
45
is there evidence to keep a patient upright for 30 min after feed with GERD
not anymore, no longer recommended
GERD
when treating
1st line is ranitidine, second line is omeprazole
46
Treatment for toddler's diarrhea
decrease total fluid intake
decrease high fructose containing juices
increase dietary far - usually resolve in 2-3 days
47
classic u/s sig n in biliary atresia
triangular cord sign at the pots hepatis
3- 6 weeks old
absent stool pigment
48
stool within the rectal vault - hirschprung or functional constipation?
functional
| with hirschprung usually no stool in rectal vault
49
organisms in mastitis
```
staph aureus
E. coli
GAS
H. influenzae
klebsiella
bacteroides
Treatment: oral antibiotics and analgesic with breastfeeding
```
50
Treatment of breast abscess
IV antibiotics, I and D, temporary cessation of feeding from that breast
51
most calorically dense macronutrient
fat
main sources: animal products, vegetable oils, margarine, baked goods, fried foods
fat also helps with cholesterol for cell membranes, hormones, bile acids
helps with absorption of ADEK vitamins (fat soluble)
52
What types of fat increase LDL
saturated fatty acids
transfats - no benefits in humans
cholesterol
will increase the LDL (which increase CV risk)
starting at age 2, should do diet to optimize CV health (should have had enough fat for growth and brain development)
53
what types of fats are associated with positive health outcomes
mono and polyunsaturated fats
| fats should be <30% of total energy intake
54
deficiency of essential fatty acids, what are clinical findings
```
desquamating skin rashes
alopecia
growth deficits
but rare in the general population
arachidonic acid and DHA arein breast milk, needed for normal development
have lots of roles
```
55
Protein
needed to help with the turnover of proteins from different body compartments
impact of protein energy malnutrition (happens in developing world) :
- impairs immune function, linear growth and increase risk for morbidity and morality
- best protein for infants is breast milk
if a single amino acid is missing, need to supplement that specific amino acid ->i.e. for soy formula, supplement with methionine
- excess protein might increase gout in some patients
56
what is the glycemic index
measure of the area under the curve of blood sugar over time after ingestion of a carb (compared to reference which is white bread)
predictable effects on glucose, hgA1C, insulin, HDL cholesterol
lower glycemic index - reduces risk of insulin resistance and CV disease
57
Rold of fiber
stimulates the colon and flow and fluid and electrolyte uptake
lack of fiber increases constipation and diverticulosis
non GI benefits; help with absorption of cholesterol
maintain stool bulk
58
True or false - breast milk provides enough zinc and iron for babies until 4-6 months of age
true
enough iron and zinc
although less amount that formula, they are more bioavailable and enough to meet needs until 4- 6 months
iron from animal protein more bioavailable than veggies and there foods - since incorporated into heme in blood and muscle already
59
deficiency in ironassociations?
1. anemia
2. neurocognitive deficits
3. growth deficits
higher iron needs in infancy and childhood
60
deficiency of zinc
1. increased risk of stunted growth
2. impaired immune function
3. increased risk for respiratory and diarrheal diseases
associated with childhood morbidity and mortality
zinc and iron are the most common deficiencies globally
61
Some risk factors for not getting enough vitamin D
1. breast milk doesn't have vitamin D
2. darker skin
3. mother latitudes
should get 300 daily of vitamin D if ingesting less than 1000 ml/day of vitamin D fortified milk
in USA most reduced fat milks are vitamin D fortified
62
when is peak bone mineralization the most
2nd to third decade of life
63
true or false - vitamin K plays a role in bone heath
true! also plays a role in coagulation factors
| neonates have risk of vitamin K deficiency - therefore get prophylaxis
64
Some effects of potassium deficiency
decreased BP
stroke
cardiovascular disease
65
Some effects of too much Na
1. nephrolithiasis (if increased sodium/potassium ratios)
| recommend <1500 mg/day for those with renal disease)
66
Things to think about with severe or persistentt jaundice
1. breast milk jaundice - usually declines in 2nd week of life, baby should be healthy
also think of:
galactosemia
hypothyroidism
UTI
hemolysis
breast milk jaundice - happens because of inhibitors of glucoronyl transferase, or enhanced absorption of bilirubin from the gut
if persistent high bill - Nelson says may need formula for 24-48 hours and/or phototherapy without cessation of breast feeding
67
How long does nelson say you can use breast milk for in fridge, frozen and after thawing?
48 hours in fridge
frozen up to 6 months
thaw then use within 24 hours
68
Compare weight gain of breast fed and formula fed?
WHO growth charts - based on healthy breast fed infants, these SHOULD be considered the nom
infants based on healthy feeding (breast), good health care, high SES, non smokers - growth under optimal conditions
late infancy may have excess growth with bottle feeding
69
True or false - powder formula is sterile?
nope, not sterile
have had outbreaks of enterobacter (especially in perms)
use water that is boiled/sterilied and special scoop size
also the water can have fluoride - if high, should use bottled defluoridated water to avoid toxicity
70
true or false - infants need additional water ?
nope, only if super hot
71
Cow's milk protein based formulas protein content compared to breast milk?
intact cow's milk based formulas have more protein than mature breast milk
whey casein ratio varies significanty
may why protein is beta globulin in vocvine milk and alpha lactalbumin in human milk
different amino acids between human milk and formula
source of fat in formula - plant or mixture of plant and animal oils, have supplemental long chance polyunsaturated fatty acids, docosahexanoic acid and arachidonic acid (DHA and ARA)
72
effects of DHA and ARA
some studies showed positive effects on visual acuity and neurocognitive development
PUFAs no consistent effect
73
main carb content in formula and breast milk
lactose in mothers milk and in standard formulas
74
indications for soy protein based formulas
galactosemia
hereditary lactase deficiency
most previously well infants with acute gastro can continue to use BM or formula after illness (don't need special formula
no evidence than helps with colic fussiness or atopic disease
if CMPA - can also be sensitive to soy, so don't give them that
should give them hydrolized protein/synthetic amino acids
soy formulas - can contain phytoestrogens
75
Types of protein hydrolysate formulas
1. partially hydrolyzed - oligopeptides with molecular weight of <3000 d
modest evidence that infants who are at risk for atopy and not breastfed that hydrolyzed formulas might decrease/prevent atopic dermatitis (extensively might be better )
76
Indications for extensively hydrolyzed formulas (nutramigen and alimentum)
1. prevention of atopic disease (see above as well as CPS statement)
2. cow milk or soy protein intolerance
can include medium chain triglyceride, makes them useful in GI malabsoprtion due to:
3. CF
4. short gut syndrome
5. prolonged diarrhea
77
Use of amino acid formulas (i.e. neonate)
for infants with dairy protein allergy who fail to thrive on extensively hydrolyse protein formula
use of these to prevent atopy has not been studied
78
how much fruit juice is okay
don't give any before 6 months
should discourage fluids other than breast milk/formula/water
4-6 oz from 1-6 year
8-12 oz per day for 7-18 year
79
weight gain by age 1, age 2
triples by age 1
quadruples by 2 year of age
slowing in growth velocity after 1st year - increased motor activity and decrease in appetite
80
birth length - when does it double
by age 5
81
when can a child self feed with a spoon
12-18 months of age
around 15 months - chip can feed himself and drink from a cup
discourage bedtime bottles because of association with dental caries
need to offer a foot multiple times before considering rejected
82
types of vegetarian diet
vegan : no aminal products
ovobegetarianism - eats eggs not dairy
lactovegetarianism - dairy products but excludes eggs
lactoovovegetariaism - eggs and dairy
83
concerns in vegetarian diets (also have a CPS statement on this)
1. iron - same amount in vegetarian and non vegetarian diets but iron in non meat sources may have lower bioavailability and poorer absorption
vegan diets have lower iron
iron stores lower in vegetarian than non veg
more common iron deficiency in vegetarian dn vegan
(black beans, cashews, kidney beans, lentil) ->rich in iron
2. vitamin B 12: vegans don't get B12
breastfeeding by vegan mom - baby with B12 deficiency
3. risk of fatty acid deficiency (EPA and DHA)
4. calcium: impaired bone mineralizion in vegans unless enough leafy greens
5. zinc: red meat contains zinc and protein, human milk with zinc but not enough after 6 months so need to have zinc in that population, lower bioavailability of zinc in plant sources (compared to animal)
84
organic foods what to tell parents
no clear benefit or harm
lower levels of pesticides
more expensive
limited scientific evidence
85
Do kids need to receive a daily multivitamin (nelson)
unless kid has poor diet because of health, cultural or religious reasons or if heath condition that leads to micronutrient deficiency, don't need multivitamins
should not recommend routinely
86
what is nutritional programming
early nutrition affects life
undernutrition in early life - reduced adult height and academic achievement
IUGR - adult CV risk factors
rapid weight gain in infancy (after IUGR or malnutrition)-increased risk of later obesity
87
when is the risk of undernutrition the greatest
pregnancy and first 2 years
| biggest effects on health, brain development, intelligence, education, productivity
88
lady who is considering pregnancy, what nutrients should you tell her to take
1. folate - to decrease risk of birth defects
2. ensure no iodine deficiency
3. iron deficiency anemia -
89
chronic malnutrition, which measure is the best?
height for age - measure of cumulative impact of events affecting nutritional status that results in stunting
90
acute malnutrition - which measure is the best
weight for height - aka wasting
| acute malnutrition
91
weight for age -
less clinical significance but easier to measure
92
how to measure length in <2 year old
recumbent length
93
BMI for obseity
obese BMI >30
overweight if >25
chronic energy deficiency <18.5
kg/m2 is BMi
94
Micronutrient deficiencies - impact of iodine deficiency
sequelae include
goiter
hypothyroidism
developmental disabilities (including severe MR)
(can assess with clinical inspection of goitre) or by iodine concentrations in urine
95
vitamin A deficiency
low intake or retinol or precursor - beta-carotene
absorption can be inhibited by lack of fats in diet or by parasite
clinical deficiency:
- night blindness and eye changes (Bitot spots and xerophthalmia)
- associated with higher mortality in young children (prophylactic in at risk populations can reduce child mortality significantly)
96
Anemia
```
iron deficiency o from illness/parasize , malnutrition, B12 or folate deficiency
cutoffs: 110 for 6-59 mo
115 for 5-11 year old
120 for 12-14 year old
120 for non pregnant women
110 for pregnant women
130 for men
```
97
zinc supplmentation - how it can help
can reduce child mortality especially when combined with ORT for diarrhea
98
severe acute malnutrition (remember, measured best by weight for height)
inadequate dietary intake of protein/energy
almost always other nutrient deficiencies with this
most severe forms:
1. marasmus - non edematous malnutrition with severe wasting (see below for deets)
2. Kwashiorkor - aka edematous malnutrition ;
3 marasmic kwashiorkor - wasting and edema
low plasma albumin can be in either type of malnutrition
unclear why some kids get each type
some people say giving carbs to kid with non oedematous malnutrition reverses the process (mobilizes body protein stores) *
see chart table 43-4 in electronic nelson for deets
99
in America, what patients might get severe protein calorie malnutrition?
1. fat diets/parents who think kid has milk allergy/rice milk diets
2. chronically ill PICU/NICU
3. burns
4. HIV
5. CF
6. FTT
7. chronic diarrhea
8. malignancy
9 bone marro
10 Inborn error metabolism
100
what is marasmus?
non edematous malnutrition with severe wasting ; inadequate intake
failure to gain weight and irritability - then weight loss and listens, get emaciated
skin wrinkled, loose sc fat
face might look normal compared to the rest
usually constipation - but can have starvation diarrhea
abdo can be distended or flat
muscle atrophy and hypoteonia
temp low and slow pulse eventually
101
kwashiorkor
`inadequate PROTEIN intake - edematous malnutrition
1. initially vague - leathargy, apathy, irritability
2. advanced: lack of growth, stamina, muscle tissue,
3. increased susceptibility to infections, vomiting, diarrhea, anorexia, flabby sc tissues and edema
4. liver enlargement - early or late in course of disease
dermatitis - darkening of the skin in irritated areas
pellagra in areas not exposed to sunlight
can get depigementation in these areas after desquamation
5. hair is sparse and thin, in dark-haired children, can become streaky red or gray
6. eventually stupor, coma, death:(
102
What is noma
chronic necrotizin ulceration of gingiva and the cheek - in malnourished kids who get an infection (i.e. measles, TB etc)
can cause disfiguration, wound care/penicillin/metronidazole for treatment
103
edematous malnutrition
eventually get defect in albumin synthesis - hypo albumin and dedema
fatty liver from secondary lipogenesis perhaps
free radical damage also potentially related
104
Treatment of severe malnutrition
1. 1st phase - week 1 - stabilize - treat dehydration and antibiotic therapy for infection, prefer oral rehydration therapy; high calory formula (80-100 kcal/kg/day), in developed countries 24-27 calorie/oz formulas ; or ready to use therapeutic foods
2. 2nd phase - week 2-6 - can start iron here (don't usually start earlier), can continue Abx for infection, feed ad lib
3. final follow up phase - feed to cover catch up growth as well as emotional /sensory stimulation
(3 phases)
105
What is refeeding syndrome
1. hypophophatemia - cellular uptake of phosphate during the 1st week of starting referring; symptoms of phosphate <0.5 mmol/L can produce weakness, rhabdomyolysis, neutrophil dysfunction, cardioresp failure, arrythmias, seizures, altered LOC, sudden death
monitor phosphate during referring
2. hypokalemia
3. hypomagnesemia- can get arrhtyhmias, tremors/tetany/seizures, refractory hypokalemia/hypocalcemia, death
4. vitamin/thiamine deficiency
5. sodium retention
6. hyperglycemia
106
risk of obesity related to
SES
race
maternal education - higher maternal education confers protection against chi hood obesity
gender
higher in AA teen girls, mexican american 6-12 year old boys
parental obesity correlates with higher risk for obesity
prenatal factors: weight gain during pregnancy, high birth weight, gestational diabetes increase risk for later obesity
IUGR with early infant catch up growth - associated with central adiposity and CV risk
107
BMI percentile for obesity in kids > 2 year old
>95th percentil - obesity
between 85-95 percentile - overweight
in adults BMI >30 obesity, 25-30 is overweight
108
why does decreased sleep lead to increased chance of obesity?
kjh
