GI, Misc + CDB Flashcards
MC type of TEF
Type A: Proximal EA with distal TEF
Best to do lumbar tap at
L4-5
MC and life-threatening complication of esophageal atresia with TEF
Aspiration pneumonia (gastric contents, chemical pneumonitis)
-h/o of polyhydramnios -vomiting w/ 1st feeding -choking/coughing & cyanosis -recurrent aspiration pneumonia
EA with TEF
Inability to pass an NGT or OGT in the NB
EA with TEF
___% of EA have TEF
90
Sequence associated with EA
VACTERL V-vertebral anomalies A-anal atresia C-cardio anomalies T-TEF E-esopageal atresia R-renal anomalies L-limb anomalies
In EA, the esophagus ends blindly approximately ___ from the nares
10 to 12 cm
T/F All TEFs are congenital
F, may be acquired
T/F EA is a surgical emergency
T
Types of TEF
___ is the least common but the most likely to be seen in ED
H-type tracheoesophageal fistula (Type C)
New born infant with frothing and bubbling at the mouth with episodes of coughing and cyanosis noted to be exacerbated on feeding
EA
MC esophageal disorder in children of all ages is
GERD
Loss of normal peristalsis and failure of LES to relax in response to swallowing
Achalasia
Pathophy of achalasia
Decreased ganglion cells surrounded by inflammatory cells
Bird’s beak on barium swallow
Achalasia
Achalasia is confirmed by the most sensitive test for it which is
Manometry
Medical management for achalasia when surgery cannot be done
Nifedipine Botulinum toxin injection
Surgical management for achalasia
Heller myotomy or pneumatic dilatation
Syndrome of familial achalasia, alacrima, corticotropin insensitivity
Allgrove syndrome
What is the primary mechanism of GER
Transient LES relaxation
Main stimulus for transient LES relaxation is ___
Gastric distention
Neck contortions seen in GER is called what syndrome
Sandifer syndrome
What position worsens the infant GER
Seated position
Recommended position in infants with GER
If the patient is asleep – supine ( for risk of SIDS); if the patient is awake, prone position or upright carried position
What are the most important predictor of successful surgical outcome of GER
Preoperative accuracy of diagnosis; skill of the surgeon
It is seen in males and blood type O and B presenting as non billous vomiting after feeding at 3rd wk of life , jaundice, with olive size non movable mass above and right of the umbilicus
Hypertrophic pyloric stenosis
Shoulder sign (bulge of pyloric muscle into the antrum), double tract sign (parallel streaks of barium in the narrow channel; excess mucosa), or string sign (from elongated pyloric channel) on barium studies (upper GI series)
Hypertrophic pyloric stenosis
Management for hypertrophic pyloric stenosis
Surgery: Ramstedt pylorotomy
MC cause of pyloric stenosis
Idiopathic
T/F Hypertrophic pyloric stenosis is usually present at birth
F
Drug associated with pyloric stenosis if given to infants within the first 2 weeks of life
Erythromycin
Congenital anomalies associated with hypertrophic pyloric stenosis
Turner’s, Trisomy 18
Systemic conditions assoc with hypertrophic pyloric stenosis
Eosinophilic GE, epidermolysis bullosa
Hypertrophic pyloric stenosis may appear as late as
5 months
Most sensitive test to detect hypertrophic pyloric stenosis; initial test
Ultrasound Elongated pyloric channel (> 14 mm) Thickened pyloric wall (> 4 mm)
__ is the most common clinical association of pyloric stenosis
Unconjugated hyperbilirubenemia ( icteropyloric syndrome)
Acid-base abnormality in hypertrophic pyloric stenosis
Hypochloremic, hypokalemic metabolic alkalosis: acid loss in vomit
Mushroom sign in upper GI series
Hypertrophic pyloric stenosis; Hypertrophic pylorus against duodenum
MC cause of non bilous vomiting in children
Hypertrophic pyloric stenosis
Best test for hypertrophic pyloric stenosis
Barium swallow
Triad: S I R ( sudden onset of severe epigastric pain, inability to pass tube into stomachl, retching with emesis)
Gastric volvulus
Management for gastric volvulus
Emergency surgery
First month of life, bilious vomiting, crampy/colicky abd pain, blood/mucus in stool
Intestinal volvulus (congenital malrotation of midgut)
MC location for volvulus (twisted loop) in children
Ileum
XR: air fluid levels upper GI series: “bird beak” at rotation sight
Intestinal volvulus
Initial management for intestinal volvulus
Endoscopic decompression, fluids
Inverted U sign of volvulus represents
Distended sigmoid loop
Midline crease in the coffee bean sign of volvulus represents
Mesenteric root in a greatly distended sigmoid
T/F Ligament of treitz is abnormally located in intestinal volvulus
T
Duodenal atresia is due to failure to recanalize (lack/absence of normal duodenal apoptosis) the lumen after the ___ phase of intestinal development during the ___ weeks AOG
solid, 4th-5th
Duodenal atresia is associated with what chromosomal abnormality
Down syndrome
What is the hallmark of duodenal obstruction
Billous vomiting without abdominal distention
Double bubble sign
Duodenal atresia
What GI anomaly is commonly assoc w/ down syndrome
Duodenal atresia
Bile stained vomitus w/in 12 hrs after birth, dehydration; Annular pancreas: wraps around duodenum
Duodenal atresia
Management of duodenal atresia
Decompress w/ NG tube, correct electrolytes, treat life-threatening anomalies, surgery (duodenoduodenostomy)
Presents in infancy w/ coughing, vomiting, gagging after feeding; Resp distress and frothy bubbles in oral cavity
TEF
Distended abdomen w/ dilated loops of bowel
Malrotation
Triple bubble sign
Jejunal atresia
“Ground glass” appearance in the RLQ with trapped “soap bubbles”; egg-shell pattern
Meconium ileus
Meconium ileus is diagnostic for what other disease
Cystic fibrosis
What is meconium ileus
Obstruction of the small intestine (terminal ileum) in the newborn caused by impaction of thick, dry, tenacious meconium
Failure to pass meconium w/in first 24 hours of life; bilious vomiting, h/o polyhydramnios, family history of CF
Meconium ileus
Intestinal perforation–> pneumoperitoneum (after birth) or intrabdominal calification (before birth)
Meconium ileus (complications)
Similar presentation to meconium ileus (bilious vomit, failure to pass meconium w/in first 24 h) BUT non-cystic fibrosis babies; no complication with intestinal perforation
Meconium plug syndrome
___ % of meconium ileus are due to CF
99
The most common presentation of cystic fibrosis in the neonatal period
Meconium ileus
MC congenital GI anomaly
Meckel diverticulum
What is the cause of meckels diverticulum
Failure of omphalomesenteric duct/vitelline duct to obliterate
Omphalomesenteric duct/vitelline duct is a remnant of
Yolk sac
Significant sudden intermittent painless rectal bleeding with brick-colored or currant jelly stool
Meckel diverticulum
MCC of LGIB in children
Meckel diverticulum
Diagnostic test for Meckel
Technetium 99M scan aka Meckel scan (scintigraphy)
Management for Meckel
Diverticular resection with transverse closure of the enterotomy
The only true congenital diverticulum
Meckel diverticulum
MC tissue in Meckel
Gastric
When is a diverticulum considered “true”
If involves all layers of bowel
What is the meckel’s diverticulum rule of 2’s
-2% of population -2 feet from ileocecal valve -2 types of ectopic tissue (gastric/pancreatic) -2x more affects males than females -less than 2 years of age
MC complication of Meckel
LGIB
Omphalomesenteric (vitelline) duct should disappear by
7th week AOG
Meckel’s diverticulum may mimic ___ and also act as lead point for ___
Acute appendicitis; intussusception
MC cause for emergent surgery in childhood
Appendicitis
Perforation rates are greatest in youngest children because ___
They can’t localize symptoms
Peak age of appendicitis
10-12
Appendicitis is rare in children younger than
5
Stages of appencidicits
1) Luminal obstruction 2) Bacterial invasion 3) Necrosis of wall
Appendiceal lumen is most commonly obstructed by
Fecalith (other: lymphoid hyperplasia from viral infection, appendices carcinoid)
Appendiceal rupture usually occurs within ___ hours of onset of symptoms
48
It is the single most reliable finding in the diagnosis of acute appendicitis
Localized abdominal tenderness
Congenital lack of distal bowel innervation by auerbach plexus -> constant contracture of muscle tone
Hirschprung disease (Congenital aganglionic megacolon)
What syndrome is hirschsprung disease freq assoc w/
Down syndrome (less frequently, MEN type 2 and Waardenburg, Laurence-Moon-Bardet-Biedl)
Hirschprung disease, boys vs girls
Boys
Do not pass meconium in 1st 48 hrs or at all (normal = 1st 24 hrs); bilious vomiting
Hirschprung disease
Treatment of choice for Hirschprung
Single stage laparospcopic endorectal pullthrough is the tx of choice
How to dx hirschsprung disease, confirmatory, GOLD STANDARD
Full thickness biopsy 2 cm above the dentate line: Absence of ganglion cells on the submucosal and myenteric plexus
XR: distended bowel loops + no air in rectum Contrast enema: barium retention for >24 hrs, narrow distal colon, proximal dilation Manometry: high pressure anal sphincter
Hirschprung disease
Most common cause of lower intestinal obstruction in neonates
Hirschprung disease
Currarino triad: Anorectal malformation, sacral bone anomaly, presacral masses
Hirschprung disease
Abd distention, *explosive discharge* of stool following a rectal exam
Hirschprung
How to dx hirschsprung disease, initial
Barium enema
DRE, rectal vault is empty
Hirschprung
It is the most common cause of intestinal obstruction in children between 3 mos to 6 years and most common abdominal surgery in children less than 2 years
Intussusception
2 types of intussusception
Ileocolic and ileoileocolic
What does the doughnut sign specify in GI tract imaging
Intussusception
MCC of Intussusception
Idiopathic (Other: Polyp, hard stool, lymphoma, viral inflammation)
Currant jelly stool + bilious vomiting
Intussusception
Right upper quadrant sausage-shaped abdominal mass
Intussusception
Initial test for Intussusception
UTZ: Target sign (echogenic mucosa + hypoechogenic submucosa)
Diagnostic & therapeutic modality for Intussusception
Barium enema
Coiled spring sign
Intussusception
T/F Intussusception is a pediatric emergency
T, do air-contrast barium enema
Syndrome associated with Intussusception with polyp as leading intussuseptum
Peutz-Jeghers syndrome
Mode of inheritance of Peutz-Jeghers syndrome
Autosomal dominant
Management for Intussusception
Hydrostatic reduction in stable patients; if not stable do not do hydrostatic reduction
Intussusception, upper part of bowel that invaginated into distal part, dragging its mesentery along
Intussuseptum (distal part, intussusipiens)
Light colored (echoic) stools at 3 to 6 weeks, dark urine, hepatomegaly, jaundice due to increased direct hyperbilirubinemia (direct hyperbilirubinemia)
Biliary atresia
___ is an indication for evaluating neonatal jaundice even if the infant seems normal otherwise
Direct hyperbilirubinemia (>2)
What are the two inborn disorders of bilirubin metabolism that lead to a CONJUGATED bilirubinemia?
Dubin-Johnson, Rotor syndrome (biliary atresia and alpha1 antitrypsin def. can present this way also)
Lack of gallbladder and triangular cord sign on ultrasound
Biliary atresia
Extrahepatic cholestasis
Biliary atresia, choledochal cyst
Management for biliary atresia
Kasai procedure before 8 weeks of life
MCC of conjugated hyperbilirubinemia (direct) in neonates
Idiopathic neonatal hepatitis (diagnosis of exclusion) and biliary atresia
Distal segmental bile duct obliteration with patent extrahepatic ducts up to porta hepatis
Extrahepatic Biliary Atresia
Prolonged elevated levels of conjugated bilirubin after 14th day of life
Neonatal cholestasis
Omphalitis in newborn is associated with
Leukocyte adhesion deficiency (no pus with minimal wound inflammation) , inadequate care of umbilical cord (with bacterial colonisation from maternal genital tract and envt; often results in sepsis)
Delayed separation of umbilical cord is associated with
Leukocyte adhesion deficiency
Leukocyte defect in leukocyte adhesion deficiency
Leukocyte chemotaxis
It is the most common life threatening emergency of the GI in newborns characterized by mucosal necrosis of the intestines
NEC
Gas accumulation in the submucosa of the bowel wall produced by bacteria seen in NEC is called
Pneumatosis intestinalis
Intestinal ischemia Enteral nutrition Bacterial translocation
NEC triad
The finding of __ confirms the clinical suspicion of NEC and is diagnostic
Pneumatosis intestinalis
Most significant risk factor for NEC
Prematurity
Diseases associated with prematurity
NEC, ROP
MC site of NEC
Distal ileum, proximal colon
Mgt for NEC
NPO, bowel decompression, antibiotics (G-, G+, anaerobic)
mechanism of diarrhea with normal osmolality
secretory
mechanism of diarrhea that stops with fasting
osmotic diarrhea
diarrhea that persists even with fasting
secretory diarrhea
it is seen if the mechanism of diarrhea is mucosal invasion
dysentery evident — blood wbc mucus in stool
diarrhea common in less than 4 years old with, associated with hemolytic uremic syndorme
enterohemorrhagic e.coli (O157H7)
___ causes diarrhea from inadequately pasteurized milk
listeria monocytogenes
salmonella typhi specimen of choice after 1st week of infection
urine, stool
salmonella is seen in blood cs on what day
1st 1 week
incubation period of vibrio cholerae toxin causing diarrhea
24-72 hrs
cholera etiologic agent common in immunocompromised or with chronic liver diase
vibrio vulnificus
ciguatera fish poisoning is common in what type of fish
barracuda
the most common complication of shigellosis
dehydration
SIADH is associated with ____ diarrhea
shigella dysenteriae
this syndrome is caused by shiga toxin mediated endothelial injury
HUS
syndrome of severe toxic convulsion, extreme hyperpyrexia, headache, brain edema, dehydration caused by shigella
Ekiri syndrome or lethal toxic encephalopathy
presumptive data supporting the diagnosis of bacillary dysentery include the____
finding of fecal leukocytes and blood leukocytosis with left shift ( bands> neutrophils)
treatment regimen of shigellosis
ceftriaxone 50mkd as single dose for empiric therapy ( continued for 5days if patient responds despite negative culture results); zinc 20mg/day for 14 days
travelers diarrhea is caused by
ETEC
major cause of infant acute and persistent diarrhea
enteropathogenic e. coli
this bacteria cause copious watery diarrhea, having fishy odor without the abdominal cramps
v. cholera
bacteria associated with IgA nephropathy
campylobacter
In IMCI, the treatment of choice for bloody diarrhea is
Ciprofloxacin
What is the dose of ciprofloxacin if given to treat bloody diarrhea
15mg/kg bid for 3 days
Zinc supplement for diarrhea should be given for how many days
10-14
cause of toddlers diarrhea
drinks excessive carbohydrate containing drinks
occurs when the transfer of food bolus from the mouth to the esophagus is impaired
oropharyngeal dysphagia
occurs when there is difficulty in transferring down to the esophagus
esophageal dysphagia
vomiting begin with involuntary ___
retching
acute diarrhea is best defined as onset of excessive loose stools of ___ ml/kg/day and ___ g/day which lasts for ___
>10mlkgday; > 200g/day; less than 14 days
a young infant normally has ___ of stool output
5ml/kg/day
secretory diarrhea is often caused by __
secretagogue
diarrhea caused by ingestion of poorly absorbable solute
osmotic diarrhea
anion gap of osmotic diarrhea
> 100 mOsm
7 yr old who have soft stool every 3 days but without difficulty. is this constipation?
No. HARD stool passed every 3 days WITH difficulty is treated as constipation
in visceral pain, the pain and tenderness is felt over the site of the disease. true or false
FALSE
when will central incisors errupt
6.5 months for mandibular (lower); 7.5 months (upper)
cleft lip is more common in what gender
males
surgical correction of cleft lip is done at what age?
3 months
what technique is commonly used in the repair of cleft lip
mod of millard rotation technique
what happens when patients with velopharyngeal dysfunction phonates the vowel U
the soft palate does not contact the posterior pharyngeal wall
what organsim is associated with dental carries
streptococcus mutans
children should only consume juices at mealtime and not to exceed ___ per day to prevent dental carries
6 oz
necrotizing peridontal disease or trench mouth is caused by
spirochetes and fusobacterium
well circumscribed ulcerative lesion with a white necrotic base and surrounded by red halo that last 10-14 days and heals without a scar
apthous ulcer (canker sore)
disease of the salivary gland is rare except for ___
mumps
suppurative parotitis is caused by
staph aureus
swallowing is seen in utero at what age of gestation
16-20
7 year old male presenting with feeding problems, vomiting, associated with atopic disease and on laboratory there is peripheral eosinophilia and IgE elevation. on microcopy, esophagus has eosinophilia. it is refractory to anti reflux medication and has no esophageal erosion on endoscopy
eosinophilic esophagitis
what is the treatment of eosinphilic esophagitis
6 food elimination ( seafood, egg, wheat, nuts, soy,milk)
position of the coin if inside the esophagus in AP view
we see the flat surface ( round shaped)
position of the coin if inside the trachea in AP view
the edge is seen ( line shaped)
position of the coin if inside the esophagus in lateral xray view
we see the edge ( line shaped)
the intrinsic factor required for GI absorption is ___
vit b12
apple peel appearance is seen in ___
jejunoileal atresia
what is the most common type of malrotation
malrotation that involves failure of the cecum to move into the right lower quadrant
what is the imaging test of choice for malrotation
Upper GI series
treatment of duplications
surgical resection
there is intentional or subconscious withholding of stool
functional constipation
it is the failure of intestinal peristalsis caused by coordinated gut motility without obstruction
ileus
abdominal xray shows air fluid levels through out the abdomen
abdominal ileus
primary ulcer is associated with ___ infection
helicobacter pylori infection
secondary peptic ulcer is a result of
stress, sepsis, shock
what is the method of choice in diagnosing peptic ulcer disease
esophagogastroduedenoscopy
peptic ulcer caused by gastrimona
zollinger ellison syndrome
what disease is associated with zollinger ellison syndrome
MEN1, tuberous sclerosis; neurofibromatosis
perianal disease is common in what IBD
crohns diasease
___may be the only manifestation of Crohns disease
growth failure
__ is the mainstay therapy for acute exacerbations of crohns
corticosteroids
__ is the main clinical expression of malabsorption
diarrhea
if celiac disease is suspected what test should be determined
serum IgA and transglutamate TG2
measurement of carbohydrate using this reagent identifies carbohydrate reducing substances
clinitest reagent
breath hydrogen test is used to identify the specific ___ malabsorbed
carbohydrate
screening tool for protein loosing enteropathy
stool a1 antitrypsin
what is the most common cause of exocrine pancreatic insufficiency in children and what test must be done
cystic fibrosis; sweat chloride test
what is the gold standard for exocrine pancreatic function
direct analysis of duodenal aspirate
what is the hallmark of celiac disease
increase density of CD8 cytotoxic intraepithelial lymphocytes
what is the most common extraintestinal manifestation of celiac diases
iron def anemia unresponsive to iron therapy
the most commonly recognized cause of congenital diarrhea
congenital microvillus atrophy ( microvillous inclusion disease)
what are the nutrients absorbed in duodenum and proximal jejunun
FIMP-C; folic acid, iron, magnesium, phosphorus, calcium
nutrient absorbed in distal ileum
vit b12; bile acids
test that is useful to differentiate between lack of intrinsic factor or malabsorption of cobalamin
schilling test
severe deficiency of body zinc after birth in bottle fed infants or after weaning from breasteeding
acrodermatitis enteropathica
it is the 2nd most common cause of child death worldwide
diarrhea
persistent diarrhea is defined as episodes beginning acutely but lasts for __
more than or equal to 14 days
management for minimal diarrhea
*if less than 10kg –> 60-120 ml ors for each vomiting, diarrhea; * if more than 10 kg –> 120-240 ml ORS
management of mild to moderate dehydration
* ors 50-100 ml/kg over 3-4 hr; * same replacement of loss as no dehydration
management of severe dehydration
* LR 20ml/kg until perfusion and mental status improve; *100 ml/kg D5.5 NSS twice the maintenance fluid OR ors over 4 hours; * for replacement of loss NGT same as no dehydration OR D5.35 NSS with 20 meq kcl IV
lactose load exceeding ___ may be associated with higher purging in children with diarrhea
5 d/kg/day
oral zinc after acute diarrhea is given how?
10mg/day for infants and 20mg/day for more than 6 months for 10-14 days
chronic diarrhea is defined as diarrhea episode that last for ___
more than or equal to 14 days
it is the most common cause of severe and protracted diarrhea in AIDS
enteric cryptosporodiosis
what is the gold standard for imaging in patients with appendicitis
ct scan
most common intestinal tumor of childhood
hamartomas
the most common primary GI carcinoma
colorectal Ca
the most common GI malignancy ( wether benign or malignant)
lymphoma
what is the hallmark of hepatorenal syndorme
renal vasoconstiriction
persistence of unconjugated bilirubin (>20) after the 1st week of life without hemolysis, jaundice with normal stool color, is seen in what disease
crigler-najar I ( gluc transferase def)
wilsons disease there is a decreased biliary ___ excretion
copper
what is the firs serologic marker to appear in hepa b infection
hbsAg
what is the marker of infectivity of hepa B
HbeAg
wha vaccine is given in infants with hsbAg + mother
HBIG and hepa vaccine within 12 hours of birth
preterm infants with HsBAg negative mother should receive vaccine when?
unti 1 month or before discharge
HBIG can be given until __ after sexual exposure
14 days
