Infant disorders Flashcards
what is cleft lip?
unfused maxillary & nasal
at what period does cleft lip often happen
approx at 5 weeks gestation (some periods of gestation where some systems are more susceptible than others)
what does cleft lip look like?
indentation/fissure that is uni or bilateral (on one or both sides)
how many babies are affected by cleft lip
approx 1 in 700 live births
what causes cleft lip?
-genetic and congenital (but mostly congenital from TERATOGENS)
what are is the main teratogen that causes cleft lip and some others?
the main teratogen is SMOKING, other ones are viral infections & folic acid deficiency
cleft lip and cleft palate are often _____
combined
what is cleft palate
-incomplete fusion of palatine structures
at what time does cleft lip happen
in week 9-12 of gestation
what does cleft palate look like?
-malformed nasal structures (these can cause problems with nasal passages & breathing)
cleft palate has a strong link to being caused by?
smoking in pregnancy
how many babies are affected by cleft palate?
aprox 1 in 2000 live births
what is required to treat cleft palate
surgery
what is the pyloric sphincter in the stomach
-pyloric region of stomach is the distal portion which as sphincter
what is pyloric stenosis
muscle hypertrophy at pyloric region of stomach causing constriction at pylorus
when does pyloric stenosis occur
2-8 weks (after birth)
how many babies are affected by pyloric stenosis?
aprox 1 in 100 births
are males or females more affected by pyloric stenosis
ratio: 4 males get it to 1 female
what is the etiology (cause) of pyloric stenosis
- unclear what causes the hypertrophy (idiopathic)
- linked to hypergasternemia (increase in gastrin), PGE (prostaglandin E) & eryhromyocin exposure (antiboitic)
path of pyloric stenosis
- hypertrophy at pyloric region of stomach
- causing constriction of pyloric sphincter
- inflammation
- obstruction
- projectile vomiting
- this causes projectile vomiting to cause dehydration & malnourishment)
diagnosing pyloric stenosis
-Hx & Px (gently palpate mass in upper right quadrant) cannot rely on this also need to do ultrasound
how do you treat pyloric stenosis
surgery
gastroesophageal reflux is a _______ GI problem
common GI problem that affects approx 50% from birth to 3 months old (this is when most common)
etiology of gastroesophageal reflux
neuromuscular etiology
gastroesophageal is mostly cured by _____
itself because it is self limiting and appox in 1 yr
gastroesophageal reflux can cause
esophagitis, whcih with severe esophagitis can have growth problems
how to deal/treat gastroesophageal reflux
- 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)
what is gastroesophageal reflux
- 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)
simple patho of gastroesophageal reflux
- esophageal sphincter incompetent
- gastric contents
- esophagus
- esophagitis
Hirschsprung disease affects how many babies?
approx 1 in 500 births
what gene is effected causing Hirschsprung disease
RET gene, Chr 10 (has mutation on this gene)
what does the RET gene do?
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
what is the patho of Hirschsprung disease
- area of colon lacks parasympathetic ganglia
- no peristalsis (localized)
- accumulation of contents
- colon distention
- abdominal distention
what do the neurons in the colon do that are affected by Hirschsprung disease
neurons in colon, has neurons for peristalsis with Hirschsprung disease one specific part of colon lacks neurons (parasympathetic neurons) cannot do peristalsis)
treatment of Hirschsprung disease
aganglionic (part that lacks neurons) segment removed (Sx only option)
what is intussusception?
-bowel invaginates into adjoining part
how many babies are affected by intussusception
1 in 4 in 1000 births
what are the serious complications that may happen from intussesception?
necrosis, perforation & peritonitis possible (infant cannot deal with that extent of the problem)
patho of intussussception
- invagination
- obstruction
- inflm
- edema
- ischemia
where does intussusception usually occur?
in the terminal ileum (right side of body in descending colon) b/c peristalsis pulls bowel that way
Treatment of intussusception?
- 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)
