Tieman/Brandau CIS: NNC, IBD, Neoplasia, PUD, biliary disorders, Ped GI Flashcards
most prev. IBD?
UC
ASCA
CD
also see elevated IgA and IgG
mucosal ulcers?
CD
erythema nodosum?
CD
p-ANCA elevated?
UC
tx for UC?
5-ASA, Abs, Corticosteroids
use 5-ASA for maintenance
surgery can be curative to prevent colon cancer
anal fistula/abscess
assoc. w/ CD
pain on defecation, along with BRB on the toilet paper after each BM
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)
BA
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
intrahepatic cholestasis
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
extrahepatic cholestasis
Obstructive process of the extrahepatic bile ducts
- Elevated AP, GGTP and conjugated bilirubin
most common causes of neonatal obstructive jaundice?
concerned if jaundice persists after 14 days
Biliary atresia is #1 cause!
- biliary tract anomalies, choledochal cysts and infections are other causes
Biliary Atresia:
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
Choledochal Cysts
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
what is NOT physiolgic jaundice?
if develops before 36 hours of age
if persists beyond 10 days or if direct bilirubin is > 20 % of total bilirubin
Congenital Rubella syndrome
can cause NN cholestasis - d/t rubella virus
see eye anomalies, microcephaly, heart disease, petechiae and purpura
congenital syphilis
d/t syphilis spirochete
- prematurity and intrauterine growth retardation
- hepatosplenomegaly
- nasal chondritis
- skin rash
- osteochondritis
- neuro sx
- teeth abnormalities
toxoplasmosis
NN cholestasis d/t parasite
common in developing countries, most common cause of posterior uveitis
Galactosemia
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
Glycogen storage diseas type IV
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
tyrosinemia
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
A1AT
see PIZZ genotype
cause of NN cholestasis
hypothyroidism
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
Alagille syndrome
Familial Intrahepatic Cholestatic Syndromes
AD disorder
Associated findings include:
- Cholestatic liver disease
- Pulmonary valvar stenosis or atresia
- Vasuclopathy
- Renal disease
VACTERL
birth defects that are often found in common: vertebral anomalies anal atresia cardiac defects TE fistula Esophageal atresia Renal/Radial abnormalities Limb defects
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
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
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
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
