GI

Question 1

which organs does GALD not impact

Answer 1

spleen, lymph nodes and bone marrow are relatively spared from deposits

Question 2

When is the ideal time for the kasai procedure

Answer 2

30-45 days. follow bilirubin levels for need for hepatic transplant

Question 3

between duodenal atresia and ileal atresia, what is the etiology?

Answer 3

Duodenal atresia: failure to recanalize
obliteration of lumen should be 6-7 wks, recanalization 8-10w
Jejunal -ileal atresia: ischemia (Ilial, Ischemia) - this is more common than duondenal atresia

Question 4

what does maternal intrahepatic cholestasis put the fetus at risk for?

Answer 4

many pulm things: RDS, MAS, bile acid pneumonia

Question 5

what is an obese mother’s placenta at risk for?

Answer 5

hypermature (increased villi), large, decreased perfusion

Question 6

most common cause of flank mass?

Answer 6

hydronephrosis

Question 7

where is copper absorbed

Answer 7

stomach

Question 8

what is absorbed in the jejunum

Answer 8

fat, protein, carbs

Fe, Mg, Ca

Question 9

What is absorbed in the ileum

Answer 9

B12, bile salts, Zn

fat and fat soluble vitamins

Question 10

what is absorbed in the colon

Answer 10

water and electrolytes

Question 11

what is neonatal hemochromatosis

Answer 11

aka GALD - maternal alloab against baby hepatocytes. deposition of Fe in liver and other tissues.
Dx: anemia, low plts, low fib. High ferritin, high lfts
Bx: salivary gland or liver bx
presents with liver failure and cardiac impairment, or iufd, iugr, pt delivery

Question 12

what is hemophagocytic lymphohistocytosis (HLH)

Answer 12

abnormality of hyperactive T cells and macrophages, where macrophages are phagocytosing hematopoietic precursors (imagine wbc and rbc)

dx: anemia, low plts and neutropenia. low fib, high ferritin, high lfts
dx: hemophagocytosis in BM, spleen and ln tissue

Question 13

what are the lengths of the different FA

Answer 13

short : <6 C
medium 6-12 C
long: 13-21 C

Question 14

what does CCK do

Answer 14

released by small intestine

stimulates pancreatic enzyme secretion and contracts gall bladder

Question 15

what does gastrin do

Answer 15

gastric acid secretion

Question 16

what does secretin do

Answer 16

released by duodenum

stimulates liver bile production, pancreas to release bicarb and inhibits gastric acid

Question 17

what does motilin do

Answer 17

released by small intestine, increases motility and gastric emptying

Question 18

what does glucose dependent insulinotropic peptide do

Answer 18

released by jejunum, post-prandial release of insulin

Question 19

what does neurotensin do

Answer 19

released by ileum, inhibits gastric secretion and increases motility

Question 20

what trace elements should you remove in renal dysfunction

Answer 20

chromium and selenium (think music, kidneys are headphones)

Question 21

which trace elements should you remove in cholestasis

Answer 21

manganese and copper

Question 22

what is manganese involved in

Answer 22

cho metabolism and bone structure

Question 23

what is selenium involved in

Answer 23

glutamine peroxidase. deficiency cause cardiac problems

Question 24

what is chromium involved in

Answer 24

glucose levels through insulin metabolism

Question 25

what is the treatment of choledochal cyst and risks after treatment

Answer 25

prompt surgical removal of cyst, anastamose hepatic ducts to pancreatic duct and remove the cyst. Still at risk for malignancy even after, so need careful lifelong surveillance. Increased risk of malignancy if had cyst drainage via cystenterostomy. If surgery needs to be delayed, can place a drain and start abx.

Question 26

what is a choledochal cyst and how does it present

Answer 26

anomaly of bile duct, can involve intra or extrahepatic. 75% involved CBD. More common in females and asian populations. Present with increased d. bili. Juncture of pancreatic duct and CBD may be abnormal, allowing for pancreatic reflux which can cause inflammation and malignancy.

Question 27

what other anomalies is duodenal atresia associated with

Answer 27

congenital heart disease (30%), Tri 21 and other GI anomalies

Question 28

how does duodenal atresia present

Answer 28

in utero, 75% with polyhydramnios, 20% noted on prenatal US, 80% are atretic after ampula so present with bilious vomiting

Question 29

where and how does gi duplication present | what is treatment

Answer 29

MC in small intestine (40%) - mc in ileum > colon (15%) can be cystic or tubular cystic: can be a lead point for volvulus tubular: can retain intestinal contents and cause obstruction with gastric mucosa: can ulcerate treatment: resection and f/u for pancreatitis, bleeding, malignancy

Question 30

how does biliary atresia present

Answer 30

more common in females and asians presents with elevated d. bili, jaundice beyond 2nd week, acholic stools after 1st week, dark urine (from d bili) and gi sx and hsm after 4th week

Question 31

when should the kasai procedure be performed and what is the success rate

Answer 31

ideally before 2 months, 30-50% success rate but may still need liver transplant

Question 32

what is allagile syndrome

Answer 32

AD intrahepatic hypoplasia of biliary ducts - mutation in JAG and NOTCH gene - Triangular facies, Posterior embryotoxon (iris strands), butterfly vertebrae, renal disease, Cardiac defects (peripheral pulmonic stenosis, tetralogy of Fallot), Direct hyperbilirubinemia, May have acholic stool, elevated ALT, bile acids and GGT

Question 33

In NEC, what goes up, what goes down? - microbio diversity - protebacteria - firmicutis

Answer 33

decreased: microbio diversity and firmicutis increased: proteobacteria

Question 34

what is nec totalis, what are treatment options, and what are sequelae?

Answer 34

Nec totalis: widespread disease with <25% viable bowel either resect all necrotic bowel and have diversion, OR divert and then come back later 6-8w for bowel almost all have SBS and long term TPN dependence

Question 35

What are risk factors for inguinal hernias? When is the best time to treat? What is normal descent process of testes? What is incarceration vs strangulation?

Answer 35

Risk factors include PT (40% in 24w vs 4% in 32w), bw (40% in Elbw vs 4% in term), male (3-5x), ab pressure, constipation, cld and dex. Controversial about when to treat but >55w Pma associated with less recurrence and need for vent support without increasing risk of incarceration. At 8-15w descend from diaphragm to abdomen when and contents reenter. At 25-35w descend to scrotum when it gets developed. Incarceration is difficult to reduce, (80% should be reducible) and strangulation is loss of blood flow (20% risk)

Question 36

Gastroschisis: more common in which pregnancies? Presentation? Over how much time do you reduce contents? What is ideal abd pressure?

Answer 36

More common in younger moms (Omphalocele Older). Over all gastroschisis is mc and wall defect. Almost always to the right of the umbillicus. Babies frequently sga. Reduce over 3-5d. And pressure should be 15-20mmhg. Takes 4-6 weeks for bowel improvement. Closed gastroschisis is where defect narrows in utero —> strangulation —> atresia

Question 37

What is a “giant omphalocele”? What is an important genetic association?

Answer 37

Greater than 5cm and with liver. Commonly have Pulm hypoplasia. 12% with omphalocele have BW.

Question 38

what is the most common late complication of NEC and where is it located?

Answer 38

intestinal strictures, usually in left colon (not TI! even though this is most common site of NEC)

Question 39

what is a good vs bad LHR. when is it most accurate

Answer 39

<25% lung to head ratio is assc with <30% survival, vs >50% LHR assc with >85% survival. most accurate at 22-26w ega.

Question 40

what length of bowel is normal in pt and term, what is pathalogic for SBS?

Answer 40

PT: 100cm, Term 160cm | <25% is associated with SBS, <75cm at risk for SBS

Question 41

if you can't pass an OG beyond what depth do you worry about TEF?

Answer 41

10cm

Question 42

what are complications of TEF?

Answer 42

1) esophageal peristalsis 2) esophageal dysmotility 3) GERD 4) leak (most resolve spontaneously but can result in stricture) 5) stricture (more common if gap >2.5 cm) 6) recurrence (usually at 2-18 m and in spot of leak or original fistula pouch) 7) asthma

Question 43

what conditions are associated with hirschsprungs?

Answer 43

wardenburg, slo, cchs