Flashcards in GI Deck (50):
Schwachman diamond syndrome
Autosomal recessive. Second most common cause of exocrine pancreas dysfunction, bone marrow dysfunction, skeletal abnormalities, diagnosis by genetic testing
What is ideal age to have Kasai procedure if have biliary atresia?
At what age do you get worried about prolonged jaundice?
at 2weeks make sure have a fractionated bili. If no acholic stool, dark urine,etc. can watch and repeat at 3 weeks
Whatis abnormal direct bilirubin?
If total 2 is abnormal, if total> 5, >20% is abnormal
How much fiber should kids have?
Age + 5 g up to 35g
What is iberogast?
Herbal extract for IBS, decreases inflammation
Treatment for functional abdominal pain
Cyproheptadine - seratonin receptor antagonist. Takes 2 weeks to act. Trial for 4-6 weeks. Dose at nighttime. 2-6 years 2mg qhs, > 6 years. 4mg. OR amitryptiline OR levsin (hyoscyamine) OR peppermint oil OR iberogast
What is neonatal small left colon syndrome?
Neonatal small left colon syndrome presents with features of intestinal obstruction including abdominal distention, vomiting (bilious or nonbilious), and delayed passage of meconium. Contrast enema in NSLCS reveals a narrowed left colon with a transition zone at the splenic flexure similar to Hirschsprung disease. Rectal biopsy demonstrates normal bowel innervation with the presence of ganglion cells. The enema in NSLCS is curative, with bowel function returning without further surgical intervention. NSLCS may be found in up to 5% of IDMs and its cause remains unknown.
What disease classically wakes you up with epigastric pain in middle of night?
Under what age is c dif testing not reliable and why?
When does spitting up peak?
4 months, improves by 8
Which antibiotic is associated with pyloric stenosis?
What is Menetrier disease?
protein-losing gastropathy that results in hypoproteinemia. On endoscopy the fundus and body of stomach are swollen and folded. Usually presents after a viral illness. biopsy shows pits and eos in lamina propria. Almost always due to CMV infection. Resolves in week to months in kids
What is the Ladd procedure for?
to treat malrotation
What is peak age of intusseception?
2 months to 5 years, but peak is 4-10months
Where is congenital lactase deficiency found?
What does the duodenum absorb?
iron, folate, calcium
What does the ileum absorb?
B12, bile salts (if lack get fat malabsorption)
What is congenital microvillus inclusion disease?
AR disorder of congenital, profuse diarrhea. Micrsovilli within involutions of apical membrane on histology. Present early in life with FTT. Excess water and electrolyte losses. High mortality (80%). No treatment other than TPN or small bowel resection
What kind of malabsorption are watery stools a sign of?
pigmented penile lesions, hamartomatous intestinal polyps, macrocephaly
PTEN mutation, many hamartomas in skin, mucous membranes, breast, thyroid
Omphalocele vs umbilical hernia
omphalocele is >4cm, umbilican hernia contains intestine only
Second most common cause of exocrine pancreatic insufficiency in children
Schwachman-Diamond syndrome - acinar cell hypoplasia with intact ducts. Also associated with short stature, neutropenia, skeletal abnormalities
Most common cause of chronic pancreatitis in children
Who has highest risk of choledochal cysts?
What cancer are patients with choledochal cysts at risk of?
What liver abnormality do patients with ARPCKD have?
Congenital hepatic fibrosis
abnormality of the ductal plate - congenital dilation of the larger, segemental intrahepatic bile ducts. Recurrent cholangitis and abscesses
What are the features of Alagille sydrome?
peripheral pulmonary artery stenosis, occasional TOF, cholestatic liver disease with a paucity of intrahepatic ducts, butterfly vertebrae, abnormal radius/ulna, small pointed chin, prominent forehead.
Do you present the Kasai procedure in patients with Alagille syndrome?
NO! Liver transplant can be indicated instead
What do you do for a patient who has possible blood exposure to a patient with acute HBV infection?
If HbSag is still negative give HBIG and complete vaccine course
Important to know about hepatitis E
Similar to HAV, but found in far east, Africa and central america after monsoon flooding. High risk for fulminant hepatitis in 3rd trimester of pregnancy with 20% maternal fatality rate
giant cell hepatitis is the histology for which disease?
alpha 1 antitrypsin
How does Wilsons present in kids vs adolescents/adults?
with hepatitis, no neuro or kayser fleisher rings (unline adults)
Screening for Wilsons disease?
ceruloplasmin, 24 hours copper (can be elevated early in the disease)
Most common reason for pediatric liver transplant?
Which conditions can cause hydrops of the gall bladder
Kawasaki dz, strep pharyngitis, HSP, TPN, prolonged fasting
Treatment of hydrops of the gall bladder
treat underlying condition and will usually resolve in a few weeks
Which gastroenteritis causing bacteria is associated with seizures?
How can you diagnose sucrase-isomaltase deficiency?
No reducing substances in stool, positive hydrogen breath test. test enzyme activity on biopsy
#1 cause of painless bright red blood in stools age 2-5
juvenile polyp. Meckels is usually darker red blood
What are side effects of immodium
including lethargy, paralytic ileus, toxic megacolon, coma, and even death
Which patients are at risk for hepatoblastoma?
premature infants, beckwith-weidamann, FAP
What is the most common cause of no bowel movement by 48 hours of age?
A four month old presents with severe, unremitting pruritis, diarrhea, conjugated hyperbilirubinemia, normal GGT
Progressive familial intrahepatic cholestasis type 1
AR of norweigens, severe cholestasis and lymphedema of the lower extremities. Histologically resembles giant cell neonatal hepatitis and improves without specific therapy
Hamartomatous polyps, hemihypertrophy, giganticism, angiomas, pigmented nevi
what bacteria can cause rectal prolapse?