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Flashcards in GI Deck (50):
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Schwachman diamond syndrome

Autosomal recessive. Second most common cause of exocrine pancreas dysfunction, bone marrow dysfunction, skeletal abnormalities, diagnosis by genetic testing

1

What is ideal age to have Kasai procedure if have biliary atresia?

3 months

2

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

3

Whatis abnormal direct bilirubin?

If total 2 is abnormal, if total> 5, >20% is abnormal

4

How much fiber should kids have?

Age + 5 g up to 35g

5

What is iberogast?

Herbal extract for IBS, decreases inflammation

7

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

8

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.

9

What disease classically wakes you up with epigastric pain in middle of night?

h. pylori

10

Under what age is c dif testing not reliable and why?

11

When does spitting up peak?

4 months, improves by 8

12

Which antibiotic is associated with pyloric stenosis?

erythromycin

13

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

14

What is the Ladd procedure for?

to treat malrotation

15

What is peak age of intusseception?

2 months to 5 years, but peak is 4-10months

16

Where is congenital lactase deficiency found?

Finland

17

What does the duodenum absorb?

iron, folate, calcium

18

What does the ileum absorb?

B12, bile salts (if lack get fat malabsorption)

19

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

20

What kind of malabsorption are watery stools a sign of?

CHO malabsorption

21

pigmented penile lesions, hamartomatous intestinal polyps, macrocephaly

ruvalcaba-myhre-smith syndrome

22

Cowden syndrome

PTEN mutation, many hamartomas in skin, mucous membranes, breast, thyroid

23

Omphalocele vs umbilical hernia

omphalocele is >4cm, umbilican hernia contains intestine only

24

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

25

Most common cause of chronic pancreatitis in children

hereditary pancreatitis

26

Who has highest risk of choledochal cysts?

Japanese girls

27

What cancer are patients with choledochal cysts at risk of?

chlangiocarcinoma

28

What liver abnormality do patients with ARPCKD have?

Congenital hepatic fibrosis

29

Caroli's disease

abnormality of the ductal plate - congenital dilation of the larger, segemental intrahepatic bile ducts. Recurrent cholangitis and abscesses

30

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.

31

Do you present the Kasai procedure in patients with Alagille syndrome?

NO! Liver transplant can be indicated instead

32

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

33

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

34

giant cell hepatitis is the histology for which disease?

alpha 1 antitrypsin

35

How does Wilsons present in kids vs adolescents/adults?

with hepatitis, no neuro or kayser fleisher rings (unline adults)

36

Screening for Wilsons disease?

ceruloplasmin, 24 hours copper (can be elevated early in the disease)

37

Most common reason for pediatric liver transplant?

biliary atresia

38

Which conditions can cause hydrops of the gall bladder

Kawasaki dz, strep pharyngitis, HSP, TPN, prolonged fasting

39

Treatment of hydrops of the gall bladder

treat underlying condition and will usually resolve in a few weeks

40

Which gastroenteritis causing bacteria is associated with seizures?

shigella

41

How can you diagnose sucrase-isomaltase deficiency?

No reducing substances in stool, positive hydrogen breath test. test enzyme activity on biopsy

42

#1 cause of painless bright red blood in stools age 2-5

juvenile polyp. Meckels is usually darker red blood

43

What are side effects of immodium

including lethargy, paralytic ileus, toxic megacolon, coma, and even death

44

Which patients are at risk for hepatoblastoma?

premature infants, beckwith-weidamann, FAP

45

What is the most common cause of no bowel movement by 48 hours of age?

Hirschsprungs

46

A four month old presents with severe, unremitting pruritis, diarrhea, conjugated hyperbilirubinemia, normal GGT

Progressive familial intrahepatic cholestasis type 1

47

Aagenaes syndrome

AR of norweigens, severe cholestasis and lymphedema of the lower extremities. Histologically resembles giant cell neonatal hepatitis and improves without specific therapy

48

Hamartomatous polyps, hemihypertrophy, giganticism, angiomas, pigmented nevi

Proteus syndrome

49

what bacteria can cause rectal prolapse?

Shigella

50

Cheilosis and sore tongue is associated with what vitamin deficiency?

B2 (riboflavin)