Newman HYHO

Question 1

Most helpful study to quantify the severity of reflux?

Answer 1

24-hour intra-esophageal pH and IMPEDANCE monitoring.

i. We talked in class about how pH and manometry is better than pH alone…but the absolute BEST is pH and impedance testing…the impedence testing measures the direction of bolus movement in the esophagus via measurement of changes in resistance to alternating electrical current when a bolus passes by a pair of metallic rings mounted on the catheter. IMPEDENCE MONITORING WAS NOT MENTIONED IN CLASS…BUT IT IS IMPORTANT TO KNOW.

Question 2

Presentation of Gastroesophageal reflux in Peds patient

Answer 2

a. Caused by “floppiness” of the lower esophageal sphincter.
b. Inability to “keep it down” can cause slowing of weight gain and sometimes even weight loss.
c. Get a detailed history of vomiting: timing with intake, force of emesis, amount, appearance, onset, what makes it worse/better, previous occurrence, associated symptoms (diarrhea, gas, bloating, retching, etc.)
d. A detailed history of feeding/eating is essential: schedule, type of food, amount ingested
e. Persistent reflux of acidic stomach contents up into the esophagus can cause esophagitis resulting in fussiness and sometimes (not commonly) resulting in feeding aversion.
f. GER: the happy spitter, gaining weight, refluxes while smiling

Question 3

what are signs or symptoms of gastroesophageal reflux disease in peds?

Answer 3

GER with signs/symptoms like fussiness, arching, crying, esophagitis on endoscopy, slow weight gain or weight loss.

Question 4

what is the most common location for intussusception for children?

Answer 4

Most are ileo-colic with the ileum invaginating into the colon at the ileocecal junction

Question 5

presentation of peds patients with intussception?

Answer 5

Blood supply to intestines in that area becomes compromised

80% of kids will have rectal bleeding (or “currant jelly stools”)

fairly sudden onset of intermittent severe abdominal pain and emesis

Frequently associated with a right upper quadrant “sausage-like” mass

One of the most common causes of intestinal obstruction in infancy

Often reduced with an air enema

Question 6

what are the presentations of Pyloric stenosis in Peds?

Answer 6

HYPOCHLOREMIC, HYPOKALEMIC, METABOLIC ALKALOSIS

Most common age 2-4 wks of life

Boys 4:1 over girls

Projectile vomiting

Dehydration
Poor wt gain or losing wt

Palpable olive in epigastric area

Peristaltic abdominal waves with eating

Question 7

what characteristics are suggestive of disease for Hirschsprung disease?

Answer 7

Failure to pass meconium within the first 24-48 hours of like

Palpable stool in throughout abdomen

Empty rectal vault

Hx of never having an unassisted stool

Question 8

how is a definitive diagnosis determined for Hirschsprung disease?

Answer 8

Rectal Biopsy

Question 9

what is the significance of unconjugated billirubin that is not bound to albumin?

Answer 9

It is lipid soluble and can cross the BBB and cause Billirubin Induced Neurologic Dysfunction also called kernicterus

Question 10

what is elevated conjugated bilirubin suggestive of?

Answer 10

Problems in the liver/biliary tree

Question 11

what is the function of the Direct coombs test?

Answer 11

performed directly on rbc’s from the patient. It is used in situations where hyperbilirubinemia is felt to result from hemolysis, especially ABO incompatibility in newborns. It is a test that looks for antibodies directly on the rbc’s of the baby.

Question 12

what is the function of the indirect coombs test

Answer 12

performed on the serum from a blood sample and is used to look for antibodies that could bind to certain rbc’s, leading to problems if blood mixing should occur (as in transfusions).

Question 13

what is the significance of stool passage in newborn as it relates to enterohepatic function

Answer 13

The passage of stool is an important way that the infant gets rid of excess bilirubin.

Stool remaining in the gut for a longer period of time allows for more breakdown of conjugated bilirubin and more reabsorption into the blood.

Question 14

what babies are at risk for hyperbilirubinemia secondary to blood group incompatibillity

Answer 14

The babies most at risk for hyperbili are those born to moms who are Type O or Rh negative.

since the moms have the antibodies against type A, B, and Rh

Question 15

what are the most common causes of Non-Pathologic unconjugated hyperbilirubinemia in a baby

Answer 15

physiologic jaundice (increase hemolysis of rbc’s…non pathologic)

Breast feeding/breast milk jaundice

Prematurity

Question 16

what are the most common causes of Pathologic unconjugated hyperbilirubinemia? in peds

Answer 16

Increased bili production”

1. Erythrocyte enzyme deficiencies
2. ABO incompatibility
3. Rbc structural defects

ii. Deficiency of hepatic uptake

iii. Impaired conjugation of bilirubin:
1. Gilbert syndrome
2. Crigler-Najjar syndrome type 1
3. Severe UGT1A1 deficiency

Increased enterohepatic circulation

Question 17

what is the difference between Crigler-Najjar type 1 and type 2

Answer 17

a. Type 1 (total UDPGT deficiency) results in SEVERE hyperbilirubinemia with high risk of BIND/Kernicterus
b. Type 2 (partial UDPGT deficiency) results in mild hyperbilirubinemia with low risk of BIND/Kernicterus

Question 18

what to think if you see elevated conjugated bilirubin levels in an infant and what are its symptoms

Answer 18

b. S/Sx of biliary atresia:

i. Cholestatic jaundice (conjugated hyperbili)
ii. Hepatomegaly
iii. Acholic stools

Question 19

what is the pathophysiology behind bilirubin induced neurologic dysfunction

Answer 19

aka Kernicterus

a. Lots of free (not bound to albumin) unconjugated bilirubin. Free unconjugated bilirubin in lipid soluble and can cross the BBB. At extremely high levels, the bilirubin that has crossed the BBB can be deposited in the basal ganglia/brainstem resulting in permanent neurologic dysfunction.

Question 20

what are the 3 phases of bilirubin toxicity in a neonate

Answer 20

a. Phase 1 (first 1-2 days): poor suck, high pitched cry, listless, poor tone, yellow skin, if high enough can seize
b. Phase 2 (3-5 days); hypertonia of muscles, opsithotonus (rigid arching), retrocollis, can be fever
c. Phase 3 (end of first week and beyond): hypertonia

Question 21

what are the characteristics of breast feeding jaundice

Answer 21

i. A combination of baby knowing how to nurse and mom’s milk not coming in right away
ii. Basically a physiologic jaundice that is exacerbated by mild to moderate dehydration and increased enterohepatic circulation in the baby.
iii. When everything calms down…baby learns, mom’s milk comes in…all is well.

Question 22

what are the characteristics of breast-milk jaundice

Answer 22

i. Not completely understood
ii. Likely some factor in the breast milk that inhibits the conjugation of bilirubin.
iii. Can last 3-6 weeks
iv. If the hyperbili is lasting beyond the 6-8 week period, consider other things
v. It will go away…try to nurse through it

Question 23

how does phototherapy decrease the levels of bilirubin in infants?

Answer 23

a. It isomerizes unconjugated bilirubin making it water soluble
b. Water soluble isomers can be eliminated by the body without conjugation by the liver.