Infant disorders

Question 1

what is cleft lip?

Answer 1

unfused maxillary & nasal

Question 2

at what period does cleft lip often happen

Answer 2

approx at 5 weeks gestation (some periods of gestation where some systems are more susceptible than others)

Question 3

what does cleft lip look like?

Answer 3

indentation/fissure that is uni or bilateral (on one or both sides)

Question 4

how many babies are affected by cleft lip

Answer 4

approx 1 in 700 live births

Question 5

what causes cleft lip?

Answer 5

-genetic and congenital (but mostly congenital from TERATOGENS)

Question 6

what are is the main teratogen that causes cleft lip and some others?

Answer 6

the main teratogen is SMOKING, other ones are viral infections & folic acid deficiency

Question 7

cleft lip and cleft palate are often _____

Answer 7

combined

Question 8

what is cleft palate

Answer 8

-incomplete fusion of palatine structures

Question 9

at what time does cleft lip happen

Answer 9

in week 9-12 of gestation

Question 10

what does cleft palate look like?

Answer 10

-malformed nasal structures (these can cause problems with nasal passages & breathing)

Question 11

cleft palate has a strong link to being caused by?

Answer 11

smoking in pregnancy

Question 12

how many babies are affected by cleft palate?

Answer 12

aprox 1 in 2000 live births

Question 13

what is required to treat cleft palate

Answer 13

surgery

Question 14

what is the pyloric sphincter in the stomach

Answer 14

-pyloric region of stomach is the distal portion which as sphincter

Question 15

what is pyloric stenosis

Answer 15

muscle hypertrophy at pyloric region of stomach causing constriction at pylorus

Question 16

when does pyloric stenosis occur

Answer 16

2-8 weks (after birth)

Question 17

how many babies are affected by pyloric stenosis?

Answer 17

aprox 1 in 100 births

Question 18

are males or females more affected by pyloric stenosis

Answer 18

ratio: 4 males get it to 1 female

Question 19

what is the etiology (cause) of pyloric stenosis

Answer 19

• unclear what causes the hypertrophy (idiopathic)

- linked to hypergasternemia (increase in gastrin), PGE (prostaglandin E) & eryhromyocin exposure (antiboitic)

Question 20

path of pyloric stenosis

Answer 20

• hypertrophy at pyloric region of stomach
• causing constriction of pyloric sphincter
• inflammation
• obstruction
• projectile vomiting
• this causes projectile vomiting to cause dehydration & malnourishment)

Question 21

diagnosing pyloric stenosis

Answer 21

-Hx & Px (gently palpate mass in upper right quadrant) cannot rely on this also need to do ultrasound

Question 22

how do you treat pyloric stenosis

Answer 22

surgery

Question 23

gastroesophageal reflux is a _______ GI problem

Answer 23

common GI problem that affects approx 50% from birth to 3 months old (this is when most common)

Question 24

etiology of gastroesophageal reflux

Answer 24

neuromuscular etiology

Question 25

gastroesophageal is mostly cured by _____

Answer 25

itself because it is self limiting and appox in 1 yr

Question 26

gastroesophageal reflux can cause

Answer 26

esophagitis, whcih with severe esophagitis can have growth problems

Question 27

how to deal/treat gastroesophageal reflux

Answer 27

• symptom treatment
• modify feeds by: thickening feeds, feed baby in upright position not in supine
• if reflux becoming a problem same drugs as adults- but pediatric dosing
• Fundoplication (sx not done often, but may be done if sever GERD & drugs are not working)

Question 28

what is gastroesophageal reflux

Answer 28

• reflux via lower esophagel sphincter,
• after feeds stomach fills, sphincters close for mechanical churning, then sphincter @ bottom has to open, top sphincter also opens even though shouldnt and lets acid into esophagus (esophageal sphincter incompetent)

Question 29

simple patho of gastroesophageal reflux

Answer 29

• esophageal sphincter incompetent
• gastric contents
• esophagus
• esophagitis

Question 30

Hirschsprung disease affects how many babies?

Answer 30

approx 1 in 500 births

Question 31

what gene is effected causing Hirschsprung disease

Answer 31

RET gene, Chr 10 (has mutation on this gene)

Question 32

what does the RET gene do?

Answer 32

RET gene codes for proteins that act as mediators (helps cells to communicate with eachother) instruct cells into formation, into different types of cells & then tissues FOR HIRSCHSPRUNG DISEASE specifically affect cells differentiating into neurons

Question 33

what is the patho of Hirschsprung disease

Answer 33

• area of colon lacks parasympathetic ganglia
• no peristalsis (localized)
• accumulation of contents
• colon distention
• abdominal distention

Question 34

what do the neurons in the colon do that are affected by Hirschsprung disease

Answer 34

neurons in colon, has neurons for peristalsis with Hirschsprung disease one specific part of colon lacks neurons (parasympathetic neurons) cannot do peristalsis)

Question 35

treatment of Hirschsprung disease

Answer 35

aganglionic (part that lacks neurons) segment removed (Sx only option)

Question 36

what is intussusception?

Answer 36

-bowel invaginates into adjoining part

Question 37

how many babies are affected by intussusception

Answer 37

1 in 4 in 1000 births

Question 38

what are the serious complications that may happen from intussesception?

Answer 38

necrosis, perforation & peritonitis possible (infant cannot deal with that extent of the problem)

Question 39

patho of intussussception

Answer 39

• invagination
• obstruction
• inflm
• edema
• ischemia

Question 40

where does intussusception usually occur?

Answer 40

in the terminal ileum (right side of body in descending colon) b/c peristalsis pulls bowel that way

Question 41

Treatment of intussusception?

Answer 41

• hydrostatic reduction (not a surgery): water soluble contrast medium & air pressure DO NOT DO THIS if there is an obstruction!!! could rupture the bowel
• surgery (for complications such as ruptured colon or obstruction)