1
Q
Percent of diet comprised of carbs? proteins?
A
carbs: 50%
protein: less than 30% (2.2g/kg in baby, 0.8 in adults)
2
Q
What are micronutrients
A
vitamins and trace minerals
3
Q
Vitamin deficiency assc with night blindness and dry cornea
A
vitamin A
4
Q
Vitamin deficiency that leads to low phosphate levels
A
vitamin D
5
Q
Vitamin deficiency that leads to poor wound healing, immunodeficiency, skin lesions
A
zinc
6
Q
Vitamin deficiency that leads to dermatitis, diarrhea, dementia
A
niacin (pellagra)
7
Q
Vitamin deficiency that leads to edema, bleeding gums, poor wound healing
A
Vitamin C (scurvy)
8
Q
Describe features of Beriberi + what vitamin deficiency causes it?
A
cardiac failure
peripheral neuropathy
wernickes
B1, thiamine
9
Q
Vitamin deficiency that causes dermatitis, glossitis, microcytic anemia, peripheral neuritis
A
B6, pyridoxine
10
Q
B12 (aka ____) causes ____ anemia, elevated _____ levels, and ______sx.
A
cobalamin
macrocytic
methylmalonic acid
neuro
11
Q
Protein deficiency is called ______. Total calorie deficency causes _____.
A
protein: kawashiorkor
total: marasmus
12
Q
Kwashiorkor distinct symptoms (3)
A
hair loss
abnormal skin pigmentation
abdominal swelling
13
Q
Three general symptoms of malabsorption
A
- diarrhea
- FTT
- abdominal distention
14
Q
Test for carbohydrate malabsorption
A
- clinitest (tests for reducing sugars)
- pH below 5.6 (bacteria turn sugars to acid)
15
Q
Three causes of protein malabsorption
A
- congenital enterokinase deficiency
- protein losing enteropathy
- inflammatory disorders
16
Q
Test for protein malabsorption
A
stool levels of a1-antitrypsin
serum levels of albumin
17
Q
Causes of fat malabsorption
A
- exocrine pancreatic insufficiency (CF, pancreatitis, schwachman diamond syndrome)
- intestinal mucosal atrophy
- bile acid deficiency
- abetalipoproteinemia
18
Q
Cause of fat malabsorption assc with acanthocytes on peripheral smear
A
abetalipoproteinemia
19
Q
Schawchman Diamond Syndrome:
- inheritance pattern
- labs
- symptoms
A
- AR
- neutropenia vs pancytopenia
- FTT, fat malabosorption (PI)
20
Q
Frequency of protein intolerance in children + most common source of reactions
A
8%, cows milk
21
Q
2 possible manifestations of protein intolerance
A
enteropathy (progressive diarrhea)
enterocolitis (acute diarrhea + rectal bleeding)
22
Q
Protein intolerance prognosis
A
usually transitory and resolves within 1-2 years
acute symptoms resolve 1-2 weeks after withdrawl of stimulus
23
Q
How common is celiac disease in the US?
Most common age of onset?
A
1/250
6 months- 2 years
24
Q
Biopsy location + findings in celiac disease
A
small bowel
flat villi, deep crypts, vacuolated epi with lymphocytes
25
Antibodies assc with celiacs
IgA endomysial
serum tissue transglutaminase
antigliadin IgG
26
Celiacs long term consequences
growth failure + delayed sexual maturity
27
Causes of small bowel syndrome
- congential lesions (gastroschisis, volvulus, atresia)
- post op for nec. enterocolitis
- chrons, tumors, radiation
28
If distal small bowel is missing in SBS, ______ are not reabsorbed
B12, bile acids
29
Treatment for SBS
- TPN
| - small bowel transplant with life threatening rxn to TPN
30
Complications assc with TPN:
- TPN cholestasis
- gallstones
- renal stones
- baterial overgrowth
- secretory diarrhea
31
Pathophys of GERD (2)
TLESR
| Gastric emptying delay
32
When does emesis assc with GERD generally resolve
6-12 months of age
33
How does painful esophagitis present in a baby?
Sandifer syndrome (torticollis, arched back)
34
Symptoms of esophagitis in older kids:
- chest pain
- morning nausea
- hoarseness
- halitosis
35
Respiratory sequelae assc with GERD
-upper + lower airway disease worsened with GERD due to induced bronchopulmonary constriction
36
Describe progression of esophagitis --> cancer
chronic inflammation leads to : squamous cell --> columnar cell (barretts) --> adenocarcinoma of the esophagus
37
Gold standard for diagnosis of GERD
pH probe measurement
| constant measurement of esophageal pH over 18 hours
38
Conservative management of GERD:
thicken feeds, sit up after feeds
| H2 blockers, PPis
39
Pyloric stenosis:
- MC symptom
- MC sex
- nonbilious projectile vomiting in the second or third week of life
- first born males
40
PE findings in pyloric stenosis + acid base status
"olive" to the upper right of the umbilicus
visible abdominal peristaltic waves
hypochloremic hypokalemic alkalosis
41
Gold standard for diagnosing pyloric stenosis
U/S = string sign
42
Treatment of pyloric stenosis
partial pyloromyotomy
43
Midgut volvulus:
gut rotates around _____
more common in what population?
SMA (assc with Laads bands); males
44
Normal rotation of gut in utero
-counterclockwise around SMA at 10 weeks
45
Diagnosis of volvulus
UI contrast imaging shows ligament of treitz right/ jejunum to the right of midline
46
Classic symptom malrotation:
sudden (bilious) vomiting in otherwise healthy baby
47
Treatment of malrotation:
surgery +/- TPN
48
Cause duodenal atresia + assc condition
failure of small bowel to recanalize at 8-10 weeks
| downs syndrome
49
Clinical features duodenal atresia
- polyhydramnios in utero
- scaphoid abdomen
- emesis/ FTT
- double bubble sign on imaging
50
Treatment duodenal atresia
surgical
51
Jejunoileal atresia:
cause
symptoms
dx
mesenteric vascular accident
bilious vomiting in first few days of life
radiographs show air fluid levels at lower SI
52
Treatment of jejunoileal atresia
surgical resection and anastomosis
53
Peak incidence of intussusception; MC location
5-9 month old males; ileocolic
54
How commonly is lead point identified in IS
5% of cases (ie Meckels diverticulum)
55
Clinic features of intussusception
- currant jelly stool
- sudden onset colicky pain
- sausage shaped mass
56
Dx and tx of intussusception
-constrast enema to dx, acutally relieves 80-90% cases, surgical treatment of remaining cases
57
Acute abdomen:
- signs of intestinal obstruction
- signs of peritonitis
obstruction: high pitched bowel sounds
peritonitis: diminished or absent bowel sounds, rigidity
58
In an acute abdomen, CONSTANT pain suggests _____.
-strangulation, torsion
59
Labs for acute abdomen:
- CBC
- U/A
- AST/ALT
- amylase/ lipase
- pregnancy, STDs
60
Peak incidence of appendictis occurs at what age?
10-12 years
61
How soon after onset of symptoms does perforation occur in appendicitis?
+ Location McBurneys
36-48 hours
2/3 between umbilicus and ASIS
62
#1 and #2 causes of pancreatitis in kiddos:
- trauma
| - idiopathic
63
Two PE "signs" assc with pancreatitis
Gray Turner- blueish flank regions
| Cullens- bluish periumbilical region
64
What lab is most specific for pancreatitis?
lipase (elevated longer than amylase as well)
65
Describe pseudocyst assc with pancreatitis
collection of fluid containing pancreatic enzymes, self resolves in most cases
66
MC management of pancreatitis
supportive
67
Two types of cholecystitis
- calculous
| - acalculous (salmonella, shigella, e coli)
68
Three conditions predisposing kiddos to chole
SCA
prolonged TPN
CF
69
Describe "sign" assc with chole:
Murphys- pain with inspiration, causes inspiratory effort to stop with palpation of RUQ
70
Define Chronic abdominal pain:
3+ consecutive months pain
71
Function CAP more common in what sex?
females
72
Describe functional CAP symptoms
5+ year old with periumbilical pain that does not interfere with sleep
73
CAP caused by IBS presents how?
-infraumbilical + stool alterations
74
Screening tests for CAP
- CBC, AST/ALT, BMP
- CRP, ESR
- H pylori
- Lactose breath testing
75
Prognosis CAP
25% persists into adulthood
76
MCC encopresis
```
severe constipation
(liquid stool leaks around a hard mass of retained stool through distended AR canal)
```
77
Cause of more frequent stools in infant
breast feeding
78
Average # stools in baby, 1 year, kiddo
baby: 4x/day, 2x/day 1 year, 1x/day kiddos
adults= variable (3x/day-->week)
79
MC form of constipation in kiddos
FFR, caused by sentinel event
| if organic, MC is hirschsprungs
80
Symptoms of prolonged FFR:
- encopresis
- fecal halitosis
- anorectal distention
81
Define delayed meconium passage
more than 48 hours after birth
82
Some important systemic causes of constipation
| 7
- DM
- hypothyroid
- lead poisoning
- botulism
- CF
- dehydration
- celiacs
83
IBD age of onset
bimodal (15-20, 50+)
84
Males more commonly suffer from which IBD?
CD has 2:1 male: female predominance
85
Location of inflammation: CD/UC
CD: transmural
UC: mucosa
86
Which has skip lesions? UC/CD?
UC: contiguous
CD: skip lesions
87
Complications of UC:
toxic megacolon
risk colon cancer
pyoderma gangrenosum
sclerosing cholangitis
88
Location of intestine affected by UC:
rectum --> colon, can get proctitis vs pancolitis
89
Location of intestine affected by CD:
any part, most commonly terminal ileum
90
Which IBD is assc with abscesses/ fistulas etc?
CD
91
Antibodies assc with UC
ANCA, 80% of cases
92
Antibodies assc with CD
antisaccharomyces cerevisiae, 70% cases
93
Rectal bleeding is more common in which IBD?
UC
94
Medical treatment for mild IBD
sulfasalazine
95
Treatment of perianal involvement in IBD
metronidazole
96
How is intractable colitis cured in UC?
total proctocolectomy
97
Which is surgery not commonly used in CD?
high recurrence rate post bowel resection
98
Melena is suggestive of blood proximal to _____.
Ligament of Treitz
99
GI bleed is confirmed by _____.
Cause of false negative (1).
Cause of false positive (3)
guiac testing
false negative: excess vitamin C
false positive: beets, iron, red meats, etc.
100
Causes of upper GI bleeding: (4)
- swallowed blood
- ulcers
- mechanical (mallory weiss)
- varices
101
How is ongoing bleding of the upper GI tract assessed?
NG tube + inspect nose, oropharynx for sources
102
Labs for diagnosing GI bleed/ stability
CBC, Hb, platelets, coag, AST/ALT, BUN(elevated)
103
Fluid bolus dose for stabilization in acute GI bleed
20 ml/kg normal saline
104
Drugs for treating GI bleed (4)
- octreotide to constrict varices
- abx for H. pylori
- H2i/PPi for gastritis/esophagitis/ ulcers
105
Newborn + rectal bleed always think _____.
| Kid + rectal bleed think _____.
Nec enterocolitis
| juvenile polyps most common source in kids
106
Causes of lower GI bleeding (6)
- necrotizing/ infectious enterocolitis
- Juvenile polyps
- allergic colitis
- meckels diverticulum
- HUS, HSP
- IBD
107
CBC findings in HUS
- microangiopathic hemolytic anemia
| - thrombocytopenia
108
```
HSP is _____ mediated.
Sx include (4)
```
- IgA
| - rash at buttocks, arthralgias, renal involvement, GI bleeding
109
Tissue + most common location of Meckels diverticulum
- ectopic gastric tissue
| - MC in terminal ileum
110
Presentation of Meckels diverticulum
acute painless rectal bleed
111
Three liver enzymes + which is most sensitive/ specific
- AST (senstitive)
- ALT (specific)
- LDH
112
Three biliary enzymes
- alk phos
- gamma glutamyl transpeptidase (GGTP)
- 5 nucleotidase (5NT)
113
Enzyme responsible for bilirubin conjugation
UDP glucuronyl transferase
114
How to measure liver fxn
- albumin
- prothrombin time
- lactate
- ammonia
115
Define infant jaundice
-more than 3mg/dl bili after the neonatal period
116
Cholestatic jaundice is retention of _____.
| It is defined as ______.
bile in the liver
| more than 2mg/dL conjugated bili, or greater than 15% total bili being conjugated.
117
Percentage of neonates with unconjugated jaundice?
| Pattern of spread
50%
| cranial to caudal
118
Test of choice for suspected cholestasis
HIDA scan
119
Three pathologic causes of decreased UDP glucuronyl transferase
- Gilbert, 50% enzyme reduction, mild, jaundice with stress
- Crigler Najar I, AR, 100% reduction
- Crigler Najar II, AD, 90% reduction
120
Causes of newborn cholestasis (6)
| + which is most common?
- infection
- mechanical obstruction
- a1at deficiency
- TPN assc disease
- metabolic derangements
- idiopathic hepatitis**
121
```
Neonatal hepatitis:
cause
predominant sex
incidence
diagnosis
prognosis
```
```
idiopathic
male
1/5k-10k
exclusion, biopsy
self resolves in 70%
```
122
How commonly is jaundice seen in neonatal hepatitis during week 1 of life?
50% of cases, 33% asx until FTT/ more liver symptoms later
123
Treatment for neonatal hepatitis
ursodeoxycholic acid
| transplant when refractory
124
Biliary atresia:
incidence
most common age of presentation
dx
1/10,000
2/3 present between week 4-6 of life
intraoperative cholangiogram
125
Biliary atresia:
- treatment
- prognosis if left untreated
Kasai portoenterostomy by 50-70 days of life, transplant if failed or repeated cholangitis occurs
complete obilteration and cirrhosis by 4 months if not treated.
126
Alagille Syndrome
- inheritance pattern
- chromosome
- gene
AD, 20, jagged 1 gene
127
List features of Alagille syndrome
- cholestatic liver disease
- debilitating pruritis
- unusual face
- cardiac disease
- renal/eye/MSK abnormalities
- FTT
128
Two major strains of hep viruses affected kids
A, B
129
What virus types are hep ABCDE
```
A-picorna
B- DNA
C- flavi, RNA
D- delta, RNA
E- hepe, RNA
```
130
What hep strain causes disease in 50% of adolescents in undeveloped countries? Also causes hepatic failure in 20% preggos.
Hep E
131
Route of infection for:
A,E
B,C
A,E: fecal oral
| B,C: vertical, body fluids, parenteral
132
Antigens positive in early Hep B infxn
HbsAg, HbeAg
133
Antibody assc with vaccination AND/ OR live infection, against Hep B
HbsAb
134
Antibody assc with LIVE infection
HbcAb
135
Antibody assc with lade infection of Hep B
HbeAb
136
Early and late antibodies assc with Hep A infection
IgM, persist for 6 months
| IgG, life long, late
137
Hep type most responsible for transfusion assc hepatitis
Hep C
138
Hep D requires _____ to replicate
HbsAg
139
Type 1 autoimmune hepatitis antibodies
ANA, ASMA
140
Type 2 autoimmune hepatitis antibodies
antiliverkidney
| anti liver cytosol type 1
141
Most common population suffering from autoimmune hepatitis
females, pre-pubertal
142
PResentation of autoimmune hep
50% acute hep, 50% chronic liver disease
143
Nonhepatic signs autoimmune hep
rash, nephritis, vasculitis, arthritis
144
Three labs used to dx autoimmune hep
autoantibodies
elevated liver enzymes
hypergammaglobulinemia
Pediatrics
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