Tieman/Brandau CIS: NNC, IBD, Neoplasia, PUD, biliary disorders, Ped GI Flashcards
1
Q
most prev. IBD?
A
UC
2
Q
ASCA
A
CD
also see elevated IgA and IgG
3
Q
mucosal ulcers?
A
CD
4
Q
erythema nodosum?
A
CD
5
Q
p-ANCA elevated?
A
UC
6
Q
tx for UC?
A
5-ASA, Abs, Corticosteroids
use 5-ASA for maintenance
surgery can be curative to prevent colon cancer
7
Q
anal fistula/abscess
A
assoc. w/ CD
8
Q
pain on defecation, along with BRB on the toilet paper after each BM
A
anal fissure - sore that develops just beyond dentate line -
usually assoc. w/ constipated bowel mvmt thats painful –> increased symp NS –> increased pressure in internal sphincter –> makes fissure worse (most req. lateral internal sphincterotomy)
9
Q
BA
A
biliary atresia = obstruction extrahepatic bile duct during first few months after birth, progressive biliary cirrhosis and eventual death if left untreated
it is an idiopathic fibrosing cholangiopathy of UKE
- leading cause of neonatal cholestasis, most common reason for pediatric liver translpant
developed around 3-6 weeks of age
80% surgery success rate if done at less than 30-45 days of age
10
Q
intrahepatic cholestasis
A
Usually a failure of formation of bile at the hepatocyte level
- Elevated transaminases and unconjugated bilirubin
Can also be an obstructive process confined to the intrahepatic bile ducts
11
Q
extrahepatic cholestasis
A
Obstructive process of the extrahepatic bile ducts
- Elevated AP, GGTP and conjugated bilirubin
12
Q
most common causes of neonatal obstructive jaundice?
A
concerned if jaundice persists after 14 days
Biliary atresia is #1 cause!
- biliary tract anomalies, choledochal cysts and infections are other causes
13
Q
Biliary Atresia:
A
Post-natal destruction of EH bile ducts with resultant injury and fibrosis of IH bile ducts
Unknown etiology
~30% of neo-natal cholestatic jaundice
Females > males
Associated with extra-hepatic anomalies: cardiac, chromosomal anomalies
Most common cause of hepatic death and reason for liver transplantation in children
Treated by Porto-enterostomy (Kasai procedure): connect porta hepatis to SI
Best results if done at < 60 days of age (best if done 20-45 days)
If Kasai procedure fails, liver transplant
14
Q
Choledochal Cysts
A
Less common cause of pediatric obstructive jaundice (2-4%)
5 types—type V (intrahepatic) called Caroli’s disease
60-70% are found at age <10
- Present with obstructive jaundice, acholic stools, pruritis, abd. pain, and/or fever
- Diagnosed by typical lab pattern of obstructive jaundice, US, MRCP, and ERCP
- Should usually be surgically resected because of risk of malignancy and cholangitis
15
Q
what is NOT physiolgic jaundice?
A
if develops before 36 hours of age
if persists beyond 10 days or if direct bilirubin is > 20 % of total bilirubin
16
Q
Congenital Rubella syndrome
A
can cause NN cholestasis - d/t rubella virus
see eye anomalies, microcephaly, heart disease, petechiae and purpura
17
Q
congenital syphilis
A
d/t syphilis spirochete
- prematurity and intrauterine growth retardation
- hepatosplenomegaly
- nasal chondritis
- skin rash
- osteochondritis
- neuro sx
- teeth abnormalities
18
Q
toxoplasmosis
A
NN cholestasis d/t parasite
common in developing countries, most common cause of posterior uveitis
19
Q
Galactosemia
A
metabolic cause of NN cholestasis
see brain damage, cataracts, jaundice, enlarged liver, kidney damage d/t child given milk, unable to metabolize milk - sugars build up and damage organs
20
Q
Glycogen storage diseas type IV
A
met cause of NN cholestasis
d/t deficiency of glycogen branching enzyme (GBE) –> results in formation of compact glycogen molecule w/ fewer branching points
- pt. has early progressive liver disease and neuromuscular manifestations
21
Q
tyrosinemia
A
can’t convert phenylalaline to fumarate –> cause of NN cholestasis
Common Manifestations - Diarrhea and bloody stools
- Vomiting
- Poor weight gain
- Extreme sleepiness
- Irritability
- “cabbage-like” odor to the skin or urine
Liver problems
- Enlarged liver
- Jaundice
- Tendency to bleed and bruise easily
- Swelling of the legs and abdomen
22
Q
A1AT
A
see PIZZ genotype
cause of NN cholestasis
23
Q
hypothyroidism
A
cause of endocrine NN cholestasis
see:
jaundice, poor feeding, enlarged tongue, hypotonia, large fontanelles w/ delayed closure, course facial features, mental retardation, short stature
screen for elevated TSH w/ decreased T4 in blood
24
Q
Alagille syndrome
A
Familial Intrahepatic Cholestatic Syndromes
AD disorder
Associated findings include:
- Cholestatic liver disease
- Pulmonary valvar stenosis or atresia
- Vasuclopathy
- Renal disease
25
VACTERL
```
birth defects that are often found in common: vertebral anomalies
anal atresia
cardiac defects
TE fistula
Esophageal atresia
Renal/Radial abnormalities
Limb defects
```
26
what are 85% of cases of esophageal atresia?
a blind proximal pouch and a fistula between the distal end of the esophagus and the distal one third of the trachea - type C
27
what is seen with esophageal atresia?
- Shortly after birth, the infant with esophageal atresia is noted to have excessive salivation and repeated episodes of coughing, choking, and cyanosis.
* * assoc. w/ polyhydramnios
- abdominal distention (coughing/crying causes air to go through fistula into stomach/bowel)
- Attempts at feeding result in choking, gagging, and regurgitation.
- Infants with tracheoesophageal fistula in addition to esophageal atresia will have reflux of gastric secretions into the tracheobronchial tree, with resulting pneumonia.
- Pulmonary infiltrates are usually noted first in the right upper lobe.
ddx? insert a catheter and see how far it goes down
tx: place a sump-suction catheter in upper esophageal pouch w/ bed elevated and use thoracotomy to make repair
complications: death may occur due to complications such as assoc. anomalies (VACTERL) or pneumonia/sepsis
28
Intestinal Obstruction in the Newborn
- presence of polyhydramnios
- vomiting after birth that is bile stained
- may see abdominal distension
- check to see if anus is present, or if there is Hirshprung disease
29
Hypertrophic Pyloric Stenosis
- see projectile vomiting at 2-4 weeks of birth
- vomit contains NO bile
- stools are infrequent and dehydration occurs (sunken in fontanelles, dry mucus membrane, poor skin turgor)
- pyloric "olive" palpated
- gastric perstaltic waves seen moving from left costal margin to the area of pylorus
** more common in males
Causes:
- d/t hypertrophy of circular/longitudinal muscularis of the pylorus and distal antrum of stomach
- maternal erythromycin exposure?
Imaging:
- abdominal ultrasound is the most specific and sensitive, will ID hypertrophic mm. (** sonography!)
- second choice is contrast UGI series (positive findings include: "pyloric beak sign" "string sign" )
complications:
- hypochloremic hypokalemic alkalosis, dehydration, starvation, gastritis
tx: pyloromyotomy after dehydration is corrected
30
causes of nonbilious vomiting?
```
pyloric stenosis
overfeeding
intracranial lesions
antral web
GER
duodenal stenosis
malrotation of bowel
adrenal insufficiency
```
31
congenital duodenal obstruction?
d/t atresia, stenosis, mucosal web, anular pancreas, malrotation, peritoneal bands
Duodenal atresia is seen in Downs and w/ congenital heart disease
32
atresia/stenosis of jejunum, ileum and colon
may result from volvulus, hernia, intussusception --> necrotic bowel
most cases occur in distal ileum/proximal jejunum
clinical findings:
- vomiting w/ bile
- abdominal distention
- failure to pass meconium
- may see enlarged colon using contrast enema
ddx?
- use plain abdominal radiograph
33
Disorders of Intestinal rotation?
- 2x more common in males
- intestinal obstruction, peptic ulceration, malabsorption
- usually seen in infants
vomiting of bile occurs, thin/underweight, steatorrhea
- may see bloody stools and signs of necrosis
Types:
1. nonrotation: midgut is suspended from the superior mesenteric vessels; the small bowel is located predominantly on the right side of the abdomen and the large bowel in the left abdomen
- usually found w/ omphalocele, gastroschisis, diaph. hernia
- no adhesive bands
2. incomplete rotation "malrotation":
- adhesive bands usually present
- predisposed to volvulus
3. reversed rotation
- bowel rotates clockwise
- cecum may be found on left side, often se obstruction of right colon
4. fixation of mesentary
34
Meconium Ileus
seen in 10-20% of infants with CF (though almost all w/ this HAVE CF)
- thick mucous secretions of small bowel caused by CF obstruct the meconium in terminal ileum
- Sx: distended abdomen, vilious emesis early on, loops of thick distended bowel
Imaging: plain radiographs show loops of bowel with increased diameter
- "soap bubble sign" RLQ
Complications: segmental volvulus, and complications of CF
tx: NON-barium enema w/ mucolytic agent (make sure pt. is well hydrated)
tx: tx for CF, pancreatic enzyme replacement, Abs, watch out for lungs
35
Hirschprung disease:
- due to failure of myenteric nerve cell migration to distal bowel --> aganglionic bowel causes obstruction
* males more commonly affected
* see down syndrome in 10-15% of patients
most common: rectum + sigmoid colon (75%)
- short segment: just recum - 10%
- proximal colon: 10% - more common in females
sx:
- occur shortly after birth
- pass little or no meconium
- abdominal distention, bilious emesis, diarrhea, irritability, problems w/ growth
imaging:
- radiographs show dilated loops of bowel
- contrast enema should be performed: demonstrates narrow lower segment rectum w/ enlarged upper segment and cecum
ddx: made by rectal biopsy of mucosal and submucosal areas of posterior rectal wall
36
neonatal small left colon syndrome (meconium plug syndrome)
- low intestinal obstruction associated with a left colon of narrow caliber and a dilated transverse and right colon. The infants are in most cases otherwise normal, though approximately 30–50% are born to diabetic mothers and are large for gestational age.
Contrast enema shows a very small left colon, usually to the level of the splenic flexure. Proximal to this point, the colon and commonly the small bowel are greatly distended. In about 30% of cases, a meconium plug is present at the junction of the narrow and dilated portion of the bowel, and the enema (using water-soluble contrast) will dislodge it.
- little or no meconium is passed
37
Intussuscpetion
Telescoping of a segment of bowel (intussusceptum) into the adjacent segment (intussuscipiens)
***most common cause of intestinal obstruction in children between 6 months and 2 years of age (more common in males)
* most common form is intussusception of the terminal ileum into the right colon
- 95% of pts is idiopathic
- hypertrophied Peyer patches are noted on the leading edge of bowel and Adenovirus correlation
- Mechanical factors:Meckel diverticulum, polyps, hemangioma, enteric duplication, intramural hematoma (Henoch-Schönlein purpura), and intestinal lymphoma are present with increasing frequency in patients over 2 years old.
findings:
- healthy child that suddenly cries and has abdominal pain episodes lasting for a minute
- reflex vomiting
- blood in stool: "currant jelly stool"
- pallor, sweating, dehydration
tx: contrast enema is ddx as well as therapeutic (if pt. is well hydrated! )
38
imperforate anus
result from faulty division of cloaca into the urogenital sinus and rectum by the urorectal septum
Low IA: When an orifice is evident at the perineum or distal introitus
- rarely have sacral anomalies
- presents as a dimpile
- tx: anoplasty
High IA: absence of orifice at the perineal level - often results in fistula of the rectum and bladder in male or with vagina in females
- often have deficient innervation and high ammounts of sacral anomalies w/ poor prognosis
*** Assoc. anomalies occur in 70% with high anomaly: VACTERL - most commonly see esophageal atresia, GI tract problems or vertebrae problems
ddx: use fistulagram
NOTE: watch out for necrotizing entercolitis!!
39
Gastroesophageal Reflux
- absence of LES high pressure zone
sx:
- regurgitation when feeding, baby is has poor weight gain
- vomiting/coughing
- may cause aspiration
- difficulty sleeping
imaging:
- LEL 24 hour pH monitoring
- upper GI series, less sensitive
tx: none needed, adjust feedings, maybe an antacid
40
Vitelline duct remains?
results in fistula near distal end of umbilicus thorugh which urine will leak
most often assoc. w/ Meckels diverticulum
41
Necrotizing enterocolitis
* most serious GI disorder of premature infants
- necrosis, ulceration and sloughing of intestinal mucosa
- usually affects terminal ileum and right colon first
findings:
- bilious vomiting
abdominal distension
blood stools
lethargy
apnea, bradycardia, hypoglycemia, temperature instability
42
Gastrointestinal bleeding in children
Upper GI bleeding: presence of melena in the stools (think ulcers, erosions, esophageal varices)
Lower GI bleding (diverticulitis, cancer, angiodysplasia)
43
umbilical hernia
fascial defect at umbilicus
surgical repair only indicated if there is intestinal involvment, but this is rare
44
omphalocele
- midline abdominal wall defect noted in 1:5000 live births. (defect in umbilicus)
- abdominal viscera (commonly liver and bowel) are contained within a sac composed of peritoneum and amnion
* * freq. assoc. w/ other cardiac anomalies (trisomy 13, 18, 21)
- see high amniotic fluid AFP levels
- assoc. w/ advanced maternal age
45
Gastroschisis
- assoc. w/ anterior abdominal wall defect: to the right of umbilicus
- not covered in amnion
- more common
- young maternal age
- NOT assoc. w/ other anomalies
46
pancreatic ADCA mutation?
KRAS
genetic: PRSS1/SPINK1 (hereditary pancreatitis)
STK11 = Peutz-Jeghers
47
ab pain, w/l, asthenia, jaundice, new onset diabetes...
ADCA in HEAD of pancreas - results in jaundice!
48
marker used to follow pancreatic treatment?
CA19-9
** surgical resection is only potential curative therapy
>1000 = unresectable disease
49
ZE syndrome
gastrinoma located in pancreas or duodenum
Diagnosis is dependent on elevated serum gastrin & elevated gastric acid levels
gastrin stimulates pareietal cells to secrete acid --> chornic hypergastrinemia--> abdominal pain due to ulcers
think if see peptic ulcer disease not related to H. pylori w/ diarrhea present
ddx tests:
- basal acid output
- overnight acid output
- elevated serum gastrin
- secretin test (causes gastrin level to go up at least 200)
tx: resection
50
neuroendocrine pancreatic tumors = "islet cell tumors"
nonfunctional: often ddx d/t nonspecific ABD sx attributable to mass or mets
Functional: produce dramatic sx d/t xs hormone release
- insulinoma
- gastrinoma
- glucagonoma
- VIPoma
- somatostatinoma
51
insulinoma
Fasting hypoglycemia (< 40 mg/dL) associated w/ an elevated insulin level (in the absence of exogenous administration of insulin) is pathognomonic
tx: surgical excision
52
glucagonoma
triad:
Necrolytic migratory erythema
Hyperglycemia
Venous thrombosis
diagnosis made?
Serum glucagon > 1000 pg/mL confirms diagnosis
tx? surgical resection
53
somatostatinoma
SS: increases gastric acid secretion and inhibits SI function
Often present w/ diarrhea, steatorrhea, diabetes, and/or gallstones
Diagnosis:
Serum somatostatin > 100 pg/mL
54
road map for pancreatic cysts?
pseudocyst: hx of pancreatitis, alcohol abuse, stones
Cystic neoplasm: no hx of pancreatitis/trauma and cyst has internal septa/solid component
1. Mucinous cystic neoplasm:
- unilocular cyst filled w/ mucin
- only in women (40-50)
- premalignant!
2. Serous cystic neoplasm:
- microcystic lesion
** contains serous fluid, has central scar that may calcify
- can look like IPMN, but has no comm. w/ pancreatic duct
(NOT premalignant)
- GRANDMA (60-70)
3. intraductal papillary mucinous neoplasm: (IPMN)
- arise in head of pancreas in males
- can look like SCN but no central scar
- distends pancreatic duct
- often harbor KRAS mutation and lead to ADCA
MRI is usually of more diagnostic than CT.
MRI can show the cystic nature of a pancreatic lesion and it's internal structure.
55
cyst w/ elevated CEA leves?
indicates that its mucinous cystic neoplasm - its premalignant
seen in older women
56
what kind of drug gives people high gastrin levels
omeprazole
57
associated urologic malformations of imperforate anus?
VUR is most common - must check kidneys
58
IRT
CF screening tool
59
CF mutation?
delta F508 resp. for 70%
60
Beckwith-Wiedemann syndrome
microcephaly, macroglossia, umbilical hernia
* large infants w/ seizures, cyanosis, hypoglycemia, high risk for malignancy
61
barium esophagography
reveals both anatomic and physiologic information like lesions, malignancies and also how the person swallows (physiologic)
62
esophageal manometry
measures pressure changes generated by esophageal wall
- useful to exclude/ ddx achalasia and motility disorders
63
GERD sx / tx?
- Epigastric/lower chest with radiation to neck, throat or back
- 2 days/week or more
- Post-prandial, especially after large meals, alcohol, rich or spicy foods, citrus, chocolate
- increases in sleep/suping positiion
"water brash" = onset of salivary secretions in mouth
- dysphagia, odynophagia = "alarm sx", need an aggressive workup
tx: PPI's, can also use as DDx
- esophageal pH monitoring (abnormal if pH - shows anatomic information such as hiatal hernia, webs, and also shows physiologic motility
- EGD scope: visualizes the mucosa to detect Barrets: used for alarm sx
Note: assoc. w/ adult onset asthma: Any adult with new-onset asthma, without allergic component, and with poor response to bronchodilators or steroids should be investigated for GERD
64
tx of GERD?
1. lifestyle modifications: elevate head of bed, alcohol avoidance, don't eat late/spicy foods, w/l, stop smoking, avoid tight-fitting clothes
2. Meds:
- antacids- Gaviscon
- H2RA: delayed onset, cimetidine and ranitidine (raise warfarin levle)
* *** PPI: Esomeprazole is most effective
3. Laproscopic Nissen Fundoplication surgery
65
PUD sx/tx?
- epigastric/upper abdominal pain
- duodenal: pain relieved by food/antacid - appetite/weight preserved (dont worry about cancer)
- gastric: pain immediately after eating, see w/l and anorexia (worry about cancer)
ddx testing:
- EGD: high sensitivity, allows for H. pylori biopsy
- Barium swallow/UGI series, less specific: only ddx 50%
66
most common site for gastric ulcer?
angular incisura, followed by lesser curvature and antrum
67
H. pylori, ddx? tx?
- gram negative, flagellated spiral bacterium
- produces urease which splits urea into CO2 and ammonia
- 20% develop ulcers (duodenal and gastric)
ddx: (need to take off PPI first)
- biopsy
- urea breath test (C13 or C14)
- serology shows IgG for H. pylori
- Stool antigen
tx:
- Triple therapy (PPI + Metranidazole + Amox/Clarith)
- Quadruple therapy (PPI + bismuth + tetracycline + metranidazole)
68
where should GE junction be located?
40 cm from the incisors
69
choledocholithiasis
stone in common bile duct - results in cholangitis, pancreatitis, biliary obstruction and jaundice
sx: ab. pain, jaundice, pruritis, acholic stools
Ascending (supporative) cholangitis:
- Charcot's triad: RUQ pain, jaundice fever, chills
- Reynold's phenomenon = Charcots + hypotension + mental changes (BAD!!)
ddx: bili>4, elevated amylase, elevated alk phos and GGP, WBC elevated
US: only 50% accurate
ERCP = ddx AND therapeutic!
tx: ERCP w/ sphincertotomy - remove the stone
70
ddx of calculous cholelithiasis ?
US >90% sensitivty
71
HIDA scan/cholescintigraphy
ddx: acute calculous cholecystitis
combined w/ CCK ejection fraction
72
tx for chronic acalculous/calculous cholecystitis?
laparoscopic cholecystectomy
73
gallbladder carcinoma
usually ADCA - presents w/ acute cholecystitis
- often seen in people w/ long-standing gall stones or porcelain gallbladder
- more common in elderly females
- surgery is only cure
74
cholangiocarcinoma
"Katskin tumor" at junction of left and right hepatic ducts (60-80%)
clinical pres:
- obstructive jaundice w/ w/l, anorexia and CA 19-9 is elevated
only 50% resectable
