Peds: GI

Question 1

Colicky abdominal pain, bilious vomitting, current jelly stools. Suasage-like mass palpalbe. Dx? First step in management? Test? Rx? Complication?

Answer 1

Intussception. First step in management is IVF. Best initial test US (Doughnut sign.) Confirm with Air Contrast enema (Air Enema)- both therapeutic and diagnostic. Peritonitis- Surgery.

Question 2

Polyhydramnios, Non-billous vomtting AND NO Respiraotry distress. Double-Bubble on AXR. Dx? First step in management? Test? Rx?

Answer 2

Duodenal Atresia (Associated with anular pancrease and Down Syndrome.) First intial step is IVF. NPO, NG to decompress bowel. Best initial test is AXR. Surgery definitive.

Question 3

Painless rectal bleeding. Bright red blood per rectum. Dx? What are the rules of 2s. Test? Rx?

Answer 3

Meckels Diverticulum (persistent vitelline duct in the small intestine.) 2% prevalence, <2 years old, 2 inches long, 2 ft from ileocecal valve, 2 types of tissue, males 2x more affected. Tech-99 scan. Surgery.

Question 4

Severe fat malabsorption from birth, Acanthocyes on Peripheral Blood Smear, Very Low to absent plasma cholesterol, triglycerides. Dx?

Answer 4

Abetlipoproteinemia.

Question 5

All cause watery diarrhea, fever and emesis. 1. Daycare associated, occurs in winter months, need vaccine to prevent. 2. Endemic occurs all year round. 3. Epiddemic?

Answer 5

1. Rotavirus. 2. Adenovirus. 3. Rotavirus. First step in management is IVF. Rx with Supportive Care.

Question 6

Pt presents with Respiratory Distress and Tachypneia, frothing, coughing and choking and vomiting alone no diarrhea AFTER FiRST FEEDING. Dx? First step in management? Test? Rx? What is this associated with?

Answer 6

Esophgeal Atresia and Tracheoesphgeal. IV fluids first step in management, NPO. NG tube cant be passed seen on CXR. Surgery.

Question 7

4 month old presents with apnea 20-30 min after feeds. Has been spitting up since birth. 5th percentile for weight. Dx? Test? Rx?

Answer 7

GERD. Esophgeal PH monitoring. 1. Supportive first ( decrease feedings, spread them apart). 2. H2 or PPI Nissen Fundolipocaiton.

Question 8

Non-bilious PROJECTILE vomiting. Still hungry and has desire to eat more. >3weeks of age. Small mass found in the epigastric area. Dx? First step in management? Best initial test? Most accurate test? What is the metabolic derailment. Rx?

Answer 8

Pyloric stenosis. IVF is the first step in management. Best initial test Abdominal US (shows thickened pylorus). Barium/Contrast is Diagnostic. Hypokalemic Hypochloremic Metabolic Alkalosis (Hypo everythin. Pylorotomy.

Question 9

Newborn presents with vomiting, abdominal distention, no passage of meconium within 48 hrs. Palpaiton of doughy cordlike masses/Inssipated meconium. Dx? Test? Rx? Complication?

Answer 9

Meconium Ileus. AXR to rule out perforation. Next step is Water Soluble Contrast Enema to check for the small contracted colon, if newborn is STABLE. Test for CF (Sweat Chloride Testing is usually equivocal in neonates.) IVF is the first step in management. Definitive treatment is Hypertonic Water Soluble contrast enemia to wash out. Peritonitis. - need to do emergency laparotomy.

Question 10

Associated with infants with polycythemia, dehydration, small left colon. Associated with maternal opiate use. Dx? Test? Rx? First step in management?

Answer 10

Meconium plug. AXR r/o peroforation. IV fluids first step in management. NPO, NG.

Question 11

Bilious emesis, recurrent “colicky” abdominal pain with vomiting + Blood in Stools. Acute small bowel obstruction in a patient s/p surgery suggest this. Dx? Test? Rx? Complication?

Answer 11

Malrotation/Volvulus. 1. AXR double bubble + distal gas and r/o perforation/pneumoperitineum 2. “Birds Beak” on Barium/Contrast. Rx: Endoscopic or Surgical Decompression. Obstruction, ischemia and necrosis can lead to life threatening sepsis.

Question 12

6 yo boy recently had gastroenteritis, now presents with hard bowel movements. He is normal for height and weight. Abdomen is soft, hard stool is palpable on rectal examination. Dx? Test? Rx?

Answer 12

Functional constipation (delay or difficultly passing stool for 2 weeks. Voluntary witholding due to pain. Physicall exam diagnosis. Rx: Bowel training program. Stool softner, laxative, enema.

Question 13

Most common reason for bowel obstruction in neonates. Dx? Best initial Test? Most accurate Test? Rx?

Answer 13

Hirschusprung Diseae. AXR to check for peforation/pneumoperitineum. Then Water Soluble-Contrast Enema (check for contracted sigmoid and dilated large colon.) Rectal biopsy (to check for absence of ganglion cells) confirms diagnosis. Manometry is done when rectal biopsy is equivocal.

Question 14

Imperfect closure of the umbilical ring. Occurs in females, LBW and African Americans. Dx? Rx?

Answer 14

Umbilical hernia. Will disappear spontaneously within 1 year. If still present by age 5 then surgery is needed.

Question 15

Benign condition causing mildly low UGT (Uridine Glucoronyl Transferase) activity in the liver resulting elevated UCB and increat T. Bili. Jaundice occurs during stress. Dx?

Answer 15

Gilbert Syndrome.

Question 16

Complete absence of the enzyme UGT (Uridine Glucoronyl Transferase) resulting in Eleveted levels of UCB, kernicterus and pale stools. Dx?

Answer 16

Crigler-Najjar I Syndrome (UCB is fat soluble and can deposit in the basal ganglia. Diagnosed by extremely high level of bili without any hemolysis in early age. Exchange transfusion. Phototheraphy.

Question 17

Child has URI then develops projectile vomiting, fever and enceplopathy 5 to 7 days later. AST and ALT enzymes are elevated. Dx? Test? Rx?

Answer 17

Reye’s syndrome (Mitochondrial damage of the hepatocytes) 2/2 Aspirin Use. Clinical. Supportive.

Question 18

Pt vomits 8-10 times a day with no apparent cause. No HA, abdominal pain or diarrhea. Dx?

Answer 18

Cyclical Vomitting.

Question 19

3 day old preterm infant presents with increased gastric residual volume and abdominal distension. Stool is heme positive. Lactate 2.9. AXR shows air in bowel all but no free air in peritoneum. Dx? Next step in management? Test? Rx?

Answer 19

Necrotizing Entercolitis. START ABX BEFORE fluids. AXR “ shows “pneumatosis intestinalis.” Rx: Abx, IVF, NPO, NG can help decompress the bowel. If medical management fails then surgery.

Question 20

Open end of the anus is missing and the rectum ends in a blind pouch. Dx? Associated with?

Answer 20

Imperforate Anus. VACTERL (veterbral anomalies, Anus Imperforate, Cardiac, TE fistula, Renal anomalies, limb anomalies.) Associated with Down Syndrome as well.

Question 21

6 Month old with occasional stridor and episodes of respiratory distress. Stridor does not change with respiration. The family has also noted mild difficulty in swallowing. Dx? Best initial test? Most accurate test? Rx?

Answer 21

Vascular Ring. Barium Swallow. Bronchoscopy to confirm the Segmental Tracheal Compression and to r/o diffuse Tracheomalacia. Surgery.

Question 22

Child presents with GI harmatomatous polyps Hyperpigmented lesions on Lips Oral mucosa and Genital skin. Dx?

Answer 22

Peutz-Jeghers Syndrome.

Question 23

Can be a 1. single benign poly 2. Multiple polyps that occur in the colon before the age of 5. Dxs?

Answer 23

Juvenile Polyp and Juvenile Polyposis Syndrome - increase risk of colon cancer and breast cancer.

Question 24

100s to 1000s Adenomatous polyps throughout colon and rectum associated with APC mutation on chromosome 5. Dx? Management?

Answer 24

Familial Adenamtous Polyposis Syndrome. Sigmoidoscopy starts at age 12 Management: All polyps are removed prophylatically otherwise pts develop colon cancer by age 40.

Question 25

Inherited mutation in the DNA repair enzymes associated with increase risk of colon, ovarian and endometrial carcinoma. Dx? When should pt have there first colonscopy?

Answer 25

HNPCC (Microsatletite Instability- defect in DNA repair enzymes.) Lynch Syndrome. Colon cancer arises de novo not from an adematous polyp. Age25.

Question 26

Benign condition where there is a defect of hepatic storage of CB resulting in leakage into the plasma. Chronic and mild hyperbilirubinemia of both the UCB and CB forms then develop, without any suggestions of hemolysis. The liver is not pigmented. Dx?

Answer 26

Rotor Syndrome. - Deficiency in the bilirubn canalicular transport protein.

Question 27

Benign condition where there is a defect of hepatic storage. There is CB with a D bili fraction of at least 50% (T bili 4.5 and D. Bili 3.1.) Normal liver function profile. Urinary Coproporphyrin I (Diagnostic) is present in high levels. The liver appears pigmented. Dx?

Answer 27

Dubin-Johnson Syndrome. - Deficiency in the bilirubin canalicular transport protein.