GI

Question 1

What is eosinophilic esophagitis? Treatment?

Answer 1

Th2 inflammatory response from food antigen exposure –> difficulty swallowing
Comorbid atopy common

Cannot progress from purees to solid food –> weight loss

Treatment: Food modification, feeding therapy for maladaptive behaviors, pharmacologic PPI and topic swallowed glucocorticoids

Question 2

What is suggested by bilious emesis (green vomit)?

Answer 2

Intestinal obstruction (e.g. intussusception, malrotation)

Question 3

What is malrotation associated with?

Answer 3

Omphalocele, diaphragmatic hernia, heterotaxy syndrome

Question 4

How is gut malrotation diagnosed?

Answer 4

Upper gastrointestinal series

Question 5

Intussusception risk factors and treatment

Answer 5

Recent viral illness or rotavirus vaccination

Air or saline enema
Surgical if enema fails, signs of peritonitis, or signs of mass lesion

Question 6

When does intussusception present?

Answer 6

6-36 months

Question 7

Riboflavin (B2) deficiency

Answer 7

Cheilosis, glossitis, and seborrheic dermatitis

Question 8

Niacin (B3) deficiency

Answer 8

4Ds: Diarrhea, dermatitis, dementia, death

Dermatitis is well-demarcated in sun-exposed areas, looks like sunburn

Question 9

Signs of galactosemia

Answer 9

Jaundice and hepatomegaly
Vomiting, poor feeding/failure to thrive
Cataracts
Increased risk for E coli sepsis

Question 10

What metabolic disease predisposes to E coli sepsis?

Answer 10

Galactosemia - excessive galactose inhibits leukocyte function and superoxide release

Question 11

Galactosemia treatment

Answer 11

Soy-based formula

Question 12

What metabolic disease has impaired NADPH production?

Answer 12

Glucose-6-phosphate dehydrogenase deficiency

Question 13

Signs of biliary atresia

Answer 13

Acholic stool
Direct hyperbilirubinemia
Dark urine
Hepatomegaly

Question 14

Meckel diverticulum produces what kind of stool?

Answer 14

Bloody, painless

Question 15

What is Peutz-Jeghers syndrome?

Answer 15

GI tract hamartomatous polyposis

Question 16

How to diagnose Hirschsprung disease

Answer 16

Rectal suction biopsy (gold standard)
Anorectal manometry, contrast enema (adjuncts)

Question 17

Meconium ileus is associated with what disorder?

Answer 17

Cystic fibrosis

Question 18

How to diagnose meconium ileus

Answer 18

Water-soluble contrast enema

Question 19

What is the Meckel diverticulum rule of 2s?

Answer 19

Presentation often by age 2 (but any age)
Usually <2 inches long
Location within 2 feet of ileocecal valve

Question 20

What is most common cause of small bowel bleeding in children?

Answer 20

Meckel diverticulum - from failed closure of vitelline duct

Question 21

Why can Meckel cause bleeding?

Answer 21

Ectopic gastric mucosa –> intestinal ulceration and bleeding

Remant of the fetal vitelline duct

Question 22

How do diagnose Meckel?

Answer 22

Technetium-99m pertechnetate scan

Question 23

How to diagnose pyloric stenosis?

Answer 23

Ultrasound

Question 24

Treatment for biliary atresia

Answer 24

Kasai procedure - hepatoportoenterostomy - diverts bile to SI
Performed before age 2 months
Liver transplantation is often ultimately required

Question 25

What would liver ultrasound show in biliary atresia? What is definitive diagnosis?

Answer 25

Small/absent gallbladder Triangular cord sign (fibrous remnants above the porta hepatis) Definitive diagnosis: intraoperative cholangiography or liver biopsy

Question 26

What is hemophagocytic lymphohistiocytosis?

Answer 26

Systemic inflammation with: Cholestasis --> elevated direct bilirubin + GGT Bone marrow failure --> cytopenia and low reticulocyte count

Question 27

What is progressive familial intrahepatic cholestasis?

Answer 27

Rare genetic: elevated direct bilirubin with paradoxically normal GGT

Question 28

Why is biliary cyst dangerous?

Answer 28

30x higher risk of malignancy (cholangiocarcinoma, GB, pancreatic cancer) Must remove with surgery, then replace with roux-en-Y hepaticojejunostomy Follow up with serial labs +/- imaging

Question 29

Treatment for GERD in infants

Answer 29

Famotidine

Question 30

What fecal test is typically positive in IBD?

Answer 30

Fecal calprotectin - protein found within neutrophils

Question 31

Salicylate toxicity has what early sign?

Answer 31

Tinnitus

Question 32

Signs of iron toxicity

Answer 32

Abdominal pain, vomiting, diarrhea, bleeding (hematemesis, melena) Hypovolemic shock within hours of ingestion due to volume loss

Question 33

Diagnosis of midgut volvulus in a hemodynamically stable patient

Answer 33

Upper GI series with small bowel follow-through: gold standard

Question 34

Biliary cyst is what?

Answer 34

Intra- or extrahepatic cystic dilation of biliary tree Most common subtype (type 1) is cystic dilation of common bile duct

Question 35

How might Eastern Asians have higher indirect bilirubin at 2-4 days old?

Answer 35

Decreased UGT enzyme than others (which normally doesn't reach adult levels until 2 weeks) --> decreased conjugation of bilirubin --> decreased hepatic bilirubin clearance

Question 36

What persona/family history is commonly seen in cyclic vomiting syndrome?

Answer 36

Personal or family history of migraines Episodes often have identifiable trigger (eg infection, stress) Diagnosis of exclusion

Question 37

Cyclic vomiting syndrome - treatment

Answer 37

Abortive - triptans Supportive - antiemetics, rehydration

Question 38

PPI for infant GERD increases risk for what?

Answer 38

Pneumonia, diarrhea

Question 39

Biliary cyst is associated with what abnormality?

Answer 39

Anomalous pancreatobiliary junction - abnormality long common channel predisposes to reflux, forming biliary cyst and/or pancreatitis

Question 40

What is Reye syndrome?

Answer 40

Aspirin after viral illness --> impaired fatty acid metabolism due to mitochondrial dysfunction --> liver failure --> ammonia accumulation --> astrocyte edema Encephalopathy, cerebral edema, vomiting --> seizure, coma, death Total bilirubin is normal

Question 41

Intestinal atresia vs malrotation with volvulus

Answer 41

Intestinal atresia is within days of birth; use X-ray, double bubble Volvulus is any age, but usually <4 weeks; use upper GI series, corkscrew duodenum, right-sided ligament of Treitz

Question 42

Jejunal atresia caused by...

Answer 42

Cocaine or other vasoconstrictive substances during pregnancy