T1: Growth and Dev/Cardiac/GI/Endocrine/Metabolic Flashcards
(128 cards)
1
Q
What age range will you see the most developmental growth than any other?
A
The first year.
Infants 1-12 months.
2
Q
What Erikson theory of development is at the infant stage?
A
Trust vs Mistrust
Are needs met quickly and consistently?
3
Q
Development is head to toe and center to the outside, what are the terms explaining this?
A
Cephalocaudal: head to toe… they learn to lift their head up before arm movement.
Proximodistal: center to periphery… they learn gross motor skills before fine motor skills.
4
Q
How much weight do babies gain each week in the first 6 months?
A
5-7 ounces/wk
5
Q
By what age does the baby’s weight double? Triple?
A
Double by 6 months, triples by 1 year.
6
Q
What is the normal growth in length per month for the first 6 months? The second 6 months?
A
1-6 months = 1 inch/month
6-12 months = 1/2 per month
7
Q
What is “frontal occipital circumference”?
A
Head circumference.
There is a gain of 10 cm (4 inches) from birth to 1 year.
8
Q
What is the average wight lost the first 5 days after birth?
A
1/10 birth weight.
They should have regained it by day 14.
9
Q
When does the umbilical cord fall off?
A
10-14 days.
10
Q
For the first 2-4 weeks, what is the baby able to focus on (distance) and what is their behavior?
A
8-12 inches… faces.
Hands in tight fists, raises head slightly, first smile, fully developed hearing.
Play is stimulatory: holding, cuddling, bright colored mobiles, talking to.
11
Q
What is the anticipatory guidance for 2-4 weeks?
A
- back to sleep
- fall precautions
- car seats: rear facing in back of car for 2+ years.
12
Q
When does the posterior fontanel begin to close?
A
2 months
13
Q
When does the anterior fontanel close?
A
12-18 months.
14
Q
What is the typical behavior at 2 months?
A
Head bobs erect temporarily, grasps rattle (briefly) if placed in hand, social smile/coo; regards face, vision is 20/200, responds to loud voices.
15
Q
Nutritional and anticipatory guidance for 2 months:
A
Breastfeed or formula (low iron NOT ok), no water/juice.
- back to sleep
- fall precautions
- car seats: rear facing in back of car
- suffocation
- burns
16
Q
What is the “fat and happy” stage of the infant?
A
4 months.
They are eating well, nice weight gain, no stranger anxiety yet.
17
Q
What is the normal behavior of a 4 month old?
A
- Plays with hands
- Reaches for and regards objects
- Mouths objects
- Good head control
- Raises body on hands when prone
- Rolls prone to supine
- laughs
- initiates social contact
18
Q
Typical nutrition at 4 months:
A
- Breastfeeding/formula
- may initiate solids (4-6 months, not B4), rice cereal. From denver children’s = start with meats (iron).
19
Q
Play for 4 month olds:
A
Talking to baby.
Age appropriate toys, limit swim/bouncy time, should not be only source of entertainment.
20
Q
Anticipatory guidance for 4 months:
A
- Car seats
- Falls (rolling and no fear)
- Choking - tp roll tube as guide
- Baby proofing - mobile in next cpl of months.
21
Q
Growth rate starts to decline at this age:
A
6 months. Should have doubled weight by now.
Begin oral care w/damp washcloth: teething
- Possible anemia due to iron stores depleted.
22
Q
Normal behavior for 6 month olds:
A
No head lag
Rolls supine to prone
Sits with support
Transfers objects
Stranger anxiety begins
Separation anxiety
Imitates
Starts object permanence
23
Q
6 month nutrition:
A
Fortified cereal, slowly introduce veggies then fruits.
Introduce new food every 5-7 days.
No bottle in crib (choking, ear infections, dental caries, bonding).
May begin introduction of cup
Limit juice to 2-4 oz / day
New guidelines are to introduce eggs and peanut butter earlier to reduce allergy risk.
NO:
Cows milk
Honey
Citrus
24
Q
Anticipatory guidance for 6 months:
A
Poison control center: 800-222-1222
Put dangerous items up high, preferably locked.
Childproofing:
falls - rolling and scooting
burns (they can be “grabby”)
pool/tub
Can use PABA-free sunscreen now.
25
Anemia is often checked at this month's check up:
9 months.
Should have teeth by now.
Can begin discipline... set limits, 45 second time-out, but using redirection is best. Might be able to say "no."
26
What is the typical behavior of a 9 month old?
Can sit alone
Crawls and creeps
Bears weight on legs when supported
Pincer grasp
Object permanence
Peek a boo
Stranger and separation anxiety
1-2 vocalizations
27
Nutritional considerations at 9 months:
* 3-4 meals / day, introduce table foods
* Finger foods - chopped, soft: begin self-feed
* Encourage use of cup and weaning from bottle
28
What do we need to know about sleep at the 9th month?
- Needs decrease (growth slows=req. less sleep)
- 2-3 naps
- May begin bedtime rituals
- May awaken during the night (2ndary to object permanence: remembers caretakers when awake).
29
Anticipatory guidance for 9 month olds:
* Risk for ingestion - Poison Control Center # available
* Keep dangerous items up high/locked up.
* Childproofing: falls, burns, pool/tub
* NO: honey, citrus, peanuts, popcorn, whole grapes.
30
We may see cyanosis, pallor, chest deformities, difficult respiratory effort, clubbing, lack of pulses in legs, and visible venous pulsations in children with this condition:
Heart disease.
FTT (failure to thrive) from working to breath, not eating.
May hear murmurs, additional sounds, or thrills
At birth the foramen ovale (between R and L atria) and the ductus arteriosus (between the aorta and pulmonary artery) normally close due to increased pressure int he L atrium>R atrium, the presence of increased O2 concentration in the blood, and decreased prostaglandins.
31
Always evaluate the PATIENT
not the monitor
32
For leads:
"White is right, smoke over fire."
33
Most common risks of cardiac catheterization:
* Hemorrhage from site
* Loss of pulse in catheterized extremity
* Dysrhythmias
* Thrombus
34
Pre-op Care
- Diaper rash can cancel procedure if going through femoral
- Allergies? Iodine?
- Asses pedal pulses and make with an "x"
- O2 sats
- Discuss pain control
- Conscious sedation or even general anesthesia
- NPO for 4-8 hours (may need IV [sickle cell])
35
Post-op Care
Where can they bleed? Throw a clot?
* Watch: bleeding, lack of pulses, arrythmias
* Distal pulses may be weaker at first, color and warmth of limb
* VS, including BP (Q 15 min)
* Dressing free from urine/feces
* If bleeding, apply direct pressure, call provider, reinforce, get help.
* Keep leg STRAIGHT and still 4-8 hours (pre-op teaching should stress this)
* Pain control
* Hydrate, DAT (diet as tolerated)
* Watch blood sugars
36
This acyanotic heart defect has a hole between the atria (may be at site of foramen ovale):
Atrial Septal Defect (ASD)
Lt to Rt shunt bc pressure is greater on Lt.
Oxygenated blood from Lt atria shunts into Rt to be recycled again through pulmonary system, thus increased pulmonary flow w/no cyanosis.
Rt side enlarges bc increased flow
37
S and S of ASD:
Asymptomatic to CHF (unusual)
Murmur
Risk for emboli later if not repaired.
38
Treatment for ASD:
Surgical repair with patch, open heart or cath.
Lifelong monitoring for arrhythmias.
39
This acyanotic heart defect is from a hole between ventricles:
Ventricular Septal Defect (VSD)
Lt to Rt shunt due to greater pressure of Lt side, oxygenated blood from Lt recycled through pulmonary system (like ASD), Rt side enlargement like ASD.
This condition is often associated with other defects.
40
S and S of VSD:
* CHF common (many congenital defects have CHF as a complication)
* Murmur
41
Treatment of VSD:
- 20-60% may close spontaneously. The time they wait to see if this happens depends on their threat to life.
- Surgical repair with patch or sutures, open or cath.
42
This heart defect is due to the failure of the fetal ductus to close after birth:
Patent Ductus Arteriosus (PDA).
[In utero it's held open by low O2 and prostaglandins from the placenta. At birth, the O2 goes up (lung use), prostaglandins decline and hole closes.]
In PDA blood shunts from aorta (higher pressure) to pulmonary artery, recycling through lungs.
* Lt side hypertrophy possibly Rt-sided as well.
43
S and S of PDA:
Treatment?
- Murmur
- CHF common
Tx:
Medical management with indomethacin.
Indomethacin is a prostaglandin inhibitor. Works 75% of the time.
Surgical repair is open heart or cath.
Patent Ductus Arteriosus
44
What is the normal range of an infant's heart rate?
130-140 bpm
45
What is the Moro reflex and what does it indicate?
Whole body response to noise...
Indication of healthy, intact nervous system.
46
Meaning of ecchymotic?
An ecchymosis is a subcutaneous spot of bleeding (from extravasation of blood) with diameter larger than 1-centimetre (0.39 in). It is similar to (and sometimes indistinguishable from) a hematoma, commonly called a bruise, though the terms are not interchangeable in careful usage.[1] Specifically, bruises are caused by trauma whereas ecchymoses, which are the same as the spots of purpura except larger, are not necessarily caused by trauma,[2] often being caused by pathophysiologic cell function.
47
How can you tell the difference between scalp edema (acaput succedaneum) and a cephalhematoma?
With caput succedaneum the swelling crosses a suture line .
48
What are the five areas tested for the Apgar?
Heart rate, respiratory effort, muscle tone, reflex irritability, and color.
49
What can lead to hypoglycemia in a newborn?
Being born from a diabetic mother, The pancreas of a fetus of a diabetic mother responds to the mother's hyperglycemia by secreting large amounts of insulin; this leads to hypoglycemia after birth.
50
Why are babies born to diabetic mothers larger?
The mothers extra glucose stimulates the baby to store extra tissue and fat in response. It is important that the mother keeps up with her exogenous insulin.
51
A fetal scalp pH of less than 7.2 indicates what?
Fetal hypoxia, emergency situation.
52
How many beats per minute or consider tachycardic in an infant?
>160 bpm
53
Within what timeframe does RhoGAM need to be administered?
An rH negative mother needs to receive RhoGAM within 72 hours of delivery.
54
What is Phenylketonuria?
Phenylketonuria (fen-ul-ke-toe-NU-re-uh), also called PKU, is a rare inherited disorder that causes an amino acid called phenylalanine to build up in your body. PKU is caused by a defect in the gene that helps create the enzyme needed to break down phenylalanine.
Without the enzyme necessary to process phenylalanine, a dangerous buildup can develop when a person with PKU eats foods that are high in protein. This can eventually lead to serious health problems.
55
What is the normal heart rate for a four-year-old?
| What is the normal respiratory range?
Between 80 and 125 BPM
| 20-30 breaths/min
56
How many mL's are in a teaspoon?
5 mL
57
What is the SSD for acetaminophen?
10-15 mg/kg/dose Q 4-6 hrs
58
What is the SSD for ibuprofen?
5-10 mg/kg/dose Q 6-8 hrs
59
24 hour fluid maintenance requirements... What is the amount of fluid/24 hrs? What do you multiply to the 1st 10 kg of weight? The 2nd 10 kg of weight? For each additional kg?
1. Multiply 100 mL/kg for the first 10 kg of weight. Ex: child is 24 kg, 1st 10 kg x 100 mL = 1000 mL (14 kg remains)
2. 2nd 10 kg x 50 mL / kg. Ex: 14 kg remains so 2nd 10 kg x 50 = 500 mL (4 kg remains)
3. For each additional kg x 20 mL/kg. Ex: 4 kg x 20 mL = 80 mL
So: 1000 mL + 500 mL + 80 mL = 1580 mL/24 hrs.
60
What is the weight of a 12 month old in comparison to their birth?
Should have tripled.
Birth length increased by 50%
Has 6-8 teeth = soft toothbrush, paste with no fluoride.
61
What is the typical behavior of a 12 month old?
Pulls to stand, cruises, may stand alone, walks with help, may take steps.
Pincer grasp fully developed.
Understanding of what being said develops before able to say the words: Says Mama and Dada specifically.
May say 2-3 words.
Understands 5 simple words, knows name, social games.
62
What is the nutrition requirements for a 12 month old?
3-4 meals per day.
Appetite decreases.
Feeds self, uses fingers, may use spoon.
Should be drinking mainly from a cup and weaning off of bottle and breast. No day bottles. No bottle in crib.
Okay to switch to WHOLE cow's milk (no more than 16-20 oz per day). Whole milk until 2 unless obesity or family problems with dyslipidemia.
Can have honey, citrus, peanut butter, and eggs.
Limit juice, preferably none.
63
What is the age range for toddlers?
What is Erikson's theory on their development at this stage?
1-3 years
Erikson: Autonomy vs. Shame and Doubt.
"I can do it."
Demos will be difficult for them to watch. They want to DO it, not watch it.
64
What are some behavioral considerations with toddlers?
They are easily frustrated bc they know what they want but may not be able to effectively communicate it.
Their attention span is about 1 min for every year old they are.
Can tolerate separation from parent a little, in beginning stage, but do NOT want to be touched or held by a stranger.
65
How should we communicate with a toddler patient?
Speak to the parent, then the child.
Give the child space.
Initially, do not touch.
Stoop to their level, talk softly.
May use a puppet to demonstrate procedure. Demos may be difficult for them to watch. Keep it short.
66
Developmental considerations with toddlers:
- Recognizes themselves as a separate person.
- Begins to learn about impulse control and how to get along in society.
- Physical growth decreases while cognitive and motor growth is huge.
- They gain about 4-6 lbs a year
- Birth weight quadruples by 2.5 years.
- Grow 3 inches a year... we can now start using a standing scale to measure height (after 2).
- Visual acuity is 20/20 - 20/40
- Digestive tract more mature and can handle larger meals = less frequent.
67
Physical milestones for toddlers:
* Mature digestion
* Control sphincters due to myelination of spinal cord (12-24 months).
* Bladder capacity increased by 14-18 months.
* Myelanation and bladder capacity allows for potty training around 2.
* Concept of time is related to events "after lunch."
68
Concerns at the toddler age?
- Toilet training
- Sibling rivalry (new sibling?)
- Temper tantrums (frustration of expression)
- "No" is their favorite
- Regression occurs often when change in their world or routine. (baby, divorce, hospitalization, etc.)
69
Tips on assessing toddlers:
* Try toe to head instead of head to toe, may be less threatening.
* Give 1-2 choices that you are willing to accept their choice on.
* Transport in crib
70
What sorts of things should we be concerned with toddlers?
SAFETY
Unsafe items out of reach, out of sight, prefer locking up. Especially poisons and guns.
Constant supervision. Childproof home (pot handles, plugs covered, small items away).
71
What is the age range of a preschooler?
What is Erikson's theory of development on this age group?
3 - 5 years.
Initiative vs. Guilt. They want to do things on their own. If they can't or not allowed they will begin feeling guilt.
72
What is the age range of school age children?
What is Erikson's theory of development on this age group?
6 - 12 years.
* Concrete thinkers.
* Often rigid about rules.
Industry vs. Inferiority. The need to feel competent. They will often start collections bc they are easy to measure.
By 8 they know right from wrong.
73
What is the age range of the adolescent stage?
What is Erikson's theory of development?
12 - 20 years.
Identity vs. Role Confusion.
Who am I? What do I stand for?
74
This reflex disappears around 2-4 months:
Moro.
When they throw their arms and legs out. The "startled" reflex.
75
3 divisions of adolescence:
1. Early 12-14
2. Middle 14-17
3. Late 17-20 years.
There are 5 stages for each sex for sexual maturity.
There is a predictable length but it still varies between individuals.
76
This reflex occurs when a hand/finger is put in the baby's mouth and they react with sucking:
Suck reflex.
Disappears between 2-5 months.
77
Walking/stepping reflex ends:
1-2 months
78
Rooting reflex ends:
3-4 months
79
Tonic neck reflex ends:
4-5 months
80
Palmar grasp reflex ends:
4-6 months
81
Plantar grasp ends:
9-12 months
82
Babinski ends:
12 months.
83
When should we be concerned about reflexes not fading?
Within 2 months of time expected.
Further exploration is needed.
84
What's a food jag?
When the child only wants to eat one food.
3-5 years
85
What heart defect is common in Down Syndrome?
Atrioventricular Canal Defect (AVC)
It's like a combined ASD and VSD creating one large hole between the left and right side of the heart with a large AV valve.
S and S are CHF and murmur
86
What is the treatment of Atrioventricular Canal Defect?
Surgical repair with patch and possibly a valve replacement.
87
This OBSTRUCTIVE acyanotic defect causes CHF, right ventricular hypertrophy, and can be cyanotic if severe:
Pulmonic Stenosis (PS)
From the narrowing (stenosis) at pulmonary artery entrance/valve.
Rt ventricular hypertrophy due to increased resistance against it.
S and S: asymptomatic to severe CHF
- Can be cyanotic with severe PS
- Murmur
88
How do we treat Pulmonic Stenosis?
Surgery. Often balloon angioplasty, sometimes Brock procedure (a valvotomy).
* Great prognosis.
89
What Obstructive Acyanotic defect is more serious because it is progressive and can result in sudden death due to MI?
Aortic Stenosis (AS)
Narrowing of the aortic VALVE
* Lt ventricular hypertrophy due to increased resistance against it
* Pulmonary vascular congestion (due to lack of CO)
** Strenuous activity may be curtailed FOR LIFE
S and S:
- Faint pulses
- Tachycardia
- Poor feeding
- Exercise intolerance
- Chest pain
- Murmur
90
What is the treatment for Aortic Stenosis?
Balloon angioplasty.
or
Valvotomy (may leave abnormal valve)
- poor risk for infants and often needs repeating.
91
This defect is usually found on the distal end of the aorta, causing increased pressure proximally (in head and upper extremities) and decreased pressure distally:
Coarctation of the Aorta (COA)
Coarctation also means stenosis... stenosis of aorta.
RISKS:
* Stroke
* HTN
* Ruptured aneurysm
* Intracranial Pressure
S and S:
- Cool, pale lower extremities with weak pulses
- High BP in upper extremities.
92
How do we treat Coarctation of the Aorta?
- Balloon angioplasty
- Closed heart surgery to resect the coarct. This is done within the first 2 years to prevent HTN.
- Monitor BP in all extremities.
93
This cyanotic heart defect consists of 4 defects:
Tetralogy of Fallot (TOF)
1. Ventricular Septal Defect
2. Pulmonary Stenosis
3. Overriding aorta (aortic valve is enlarged, overriding the VSD, making unoxygenated blood enter systemic circulatory system)
4. Right ventricular hypertrophy (due to PS)
Blood can shunt either way, depending on the size of VSD and resistance to flow in the vessels.
94
S and S of Tetralogy of Fallot:
* Murmur
* Cyanosis, mild to severe, often progressive, with acute episodes called "blue spells" or "tet spells."
During these spells, the patient will squat down, knees to chest, to keep blood in the core part of their body. If baby, then we pull their legs up to be in that position.
95
How is Tetralogy of Fallot treated?
Palliative shunts (Blalock-Taussig) shunt from an aortic branch to the pulmonary artery allowing more blood to enter lungs for gas exchange.
* Avoid BP in the arm affected
* Pulse of the arm affected won't be detectable.
Surgical repair: open heart to close VSD and resect stenosis.
* Monitor for ventricular arrhythmias after corrective repair.
96
This congenital heart defect shows S and S of cyanosis, tachycardia, and clubbing. It is due to a valve failing to develop:
Tricuspid Atresia.
No flow from RT atria to RT ventrical.
Blood flows via ASD or patent foramen ovale to left side of heart, and via VSD to RT ventricle and then to lungs.
* Complete mixing of all blood in LT side of heart.
RISKS:
Stroke
Endocarditis
97
How do we treat Tricuspid Atresia?
Keep PDA open with prostaglandins.
Palliative shunt or surgery to correct.
98
What do we call the congenital heart defect that is composed of 2 separate systems where the pulmonary artery leaves the LT ventricle and the aorta leaves the RT ventricle?
Transposition of the Great Vessels/Arteries (TGV/TGA).
* This is not compatible with life unless there are septal defects or PDA present. Sometimes they'll go in and make the defects (holes) larger to keep the baby alive until ready for surgery.
S and S:
Cyanosis
CHF
99
How do we treat Transposition of the Great Vessels/Arteries?
Given prostaglandins to keep holes/PDA open.
Cardia cath with balloon insertion to make ASD (Rashkind procedure).
Surgery: arterial switch procedure is choice procedure.
100
This defect is due to the underdevelopment of the LT side of the heart with PDA and patent foramen ovale:
Hypoplastic Left Heart Syndrome
The blood flows LA via patent foramen ovale to RA, then RV and out to the pulmonary artery.
S and S:
Cyanosis
CHF within a week.
101
What treatment is available for Hypoplastic Left Heart Syndrome?
Prostaglandins to keep ducts open.
Surgery to repair.
Heart transplant is treatment of choice.
102
When you think strep, think what?
Rheumatic fever and AGN (Acute poststreptococcal GlomeruloNephritis).
These are inflammatory diseases occuring after group A beta-hemolytic strep infection... body develops antibodies which react with tissue antigens = autoimmune response.
Not contagious.
Involves joints, skin, brain, heart, and can cause valve damage (rheumatic heart disease).
PREVENTION IS KEY
Teach: May develop chorea (quick movements) that will eventually resolve but is difficult for kids to deal with.
103
What is the treatment for Rheumatic Fever?
- Kill strep (penicillin/erythromycin)
- Prevent cardiac damage
- Tx symptoms (bedrest while febrile, salicylates - anti-platelet reduces inflammation and lowers threshold.
- Prevent recurrence through prolonged Pen-G... may be on for years.
104
What are the treatments for artherosclerosis in childhood?
Called hyperlipidemia or hypercholesterolemia
Dietary: restriction of fats and cholesterol intake AFTER 2 years of age (not before bc will impede development)
Drugs: cholestyramine, colestipol bind bile acids
Take MVI with iron daily because drugs interfere with fat-soluble vitamins.
105
When you see a strawberry tongue, big red inflammed lips, and conjunctivitis (due to acute systemic vasculitis), what should you be thinking?
Kawasaki Disease
Etiology unknown.
Usually in toddlers.
If untreated:
Can damage heart (esp. infants), bc it inflames the blood vessels and leads to aneurysms.
106
S and S of Kawasaki Disease (besides strawberry tongue, etc.):
* Abrupt onset of high fever that is unresponsive
* Irritability
* Rash on groin, stomach, chest
* Arthritic symptoms
* Hands and feet may peel (desquamate)
* When the fever passes they are at very high risk for MI. S and S for MI in children:
- abdominal pain
- vomiting
- inconsolable crying
- pallor
- shock
- restlessness
- BP going down?
* 6-8 weeks later, lab values return to normal and eventually irritability resolves over a few months.
107
Treatment of Kawasaki Disease:
-- High dose IV gamma globulin
-- Salicylates ( may be for several weeks ) High dose to prevent coronary aneurysms.
-- Quiet and rest, support family
-- Monitor I's and O's, weights, respiratory status carefully.
--* No live vaccines for a while.
salicylates are group of anti-inflammatory, mildly ANALGESIC and antipyretic drugs that includes aspirin, sodium salicylate and BENORYLATE (benorilate).
108
This is increasing in children. Most are asymptomatic. There are two types: essential (no known cause) and secondary (identifiable cause):
HTN
Children should get routine screenings for BP and try to reduce anxiety.
S and S:
- Frequent headaches
- Vision changes
- Dizziness
- Infants: irritability, head-rubbing/banging, wake screaming.
109
Treatment of hypertension:
Dietary, lifestyle, exercise, drugs are last resort.
Correct the cause
YW should avoid BCP (birth control pills)
110
What is a normal digoxin level?
Normal levels of K+?
0. 5 - 2 ng/mL
| 3. 5 - 5
111
This condition is when the heart fails to pump adequate oxygenated blood:
Congestive Heart Failure (CHF)
S and S are different for LT and RT sided failures:
LT is lung (cough, crackles)
RT is systemic: peripheral edema
We do not want them crying... respond to their needs right away.
112
What is the treatment for CHF?
* Digoxin (apical pulse for 60 sec, follow parameters)
* Any dose greater that 1 mL needs to be questioned.
- - toxicity: vomiting, bradycardia
If K+ falls, dig toxicity more likely.
* Rest and increased calories
* O2 as needed
* Sitting up position
113
What do we call the slipping of one part of the intestine into another part just distal to it?
Intussusception
Common site is ileocecal valve. Causes obstruction and bowel necrosis (pulls in mesentery and blood supply).
MEDICAL EMERGENCY
S and S, healthy child suddenly presents with:
* Severe abdominal pain, straining, flexed legs
* Bowel sounds hyperactive proximal to obstruction, hypoactive distal to it.
* Emesis, starting bilious, progressing to fecal
* Currant jelly stools from blood and mucous leaking into the intestine
* Abdominal rigidity
* A more chronic picture: vomiting, D, pain that is intermittent, accompanied by weight loss.
114
How do we Dx Intussusception?
How do we treat it?
History and X-ray
Some resolve spontaneously.
* Ba+ enema, air enema, or CO2 enema relieves in 75% of cases.
* Surgery: bowel resection if can't quickly fix it
Child is at risk for another.
115
What do we call the condition where the bile is blocked and unable to drain from the liver into the intestine due to a progressive inflammatory process?
Biliary Atresia.
Often fatal by 2 years old.
Etiology is unknown.
```
S and S:
May seem healthy at birth,
Jaundice that doesn't clear
Pale stools
Dark urine
Abdominal distention from hepatomegaly and ascites
Clotting problems
Malnutrition
FTT
Intense puritis (itching)
```
116
How do we Dx and Tx biliary atresia?
Dx:
- Early dx is essential to survival
- Ultrasound
- Nuclear scan of liver
- Biopsy
- Cholangiogram
Tx:
- Surgery preferable within 2 months
- Vitamins A,D,E,K and caloric support
- Kasai procedure (uses part of intestine to create new duct).
- Liver transplant (often necessary even after Kasai) in 90% of children.
117
If child is severely dehydrated, what are we able to increase fluid maintenance to?
We are able to administer 1.5 times the normal fluid maintenance level.
Ex. If their maintenance is 1,000 then we are able to push 1,500.
Hypotension is a sign of severe dehydration.
Other S and S:
Poor turgor, Furrowed tongue, Dry membranes, Lethargy, Slow capillary refill?, Sunken eyes or fontanel, Absent tears, Tachycardia, Decreased LOC.
shock is very late sign.
118
Hypothyroidism S and S and Tx:
Sleepy baby!
Lethargic, constipated, hypotonic, feeding difficulties, enlarged tongue, dry skin.
Tx:
Detect early with newborn screenings to prevent MENTAL RETARDATION
Lifelong therapy with drug replacement (synthroid)
119
Hypoparathyroidism S and S and Tx:
```
S and S:
Tetany
HA
Seizures
Laryngospasms
Apnea
```
Tx:
Correct the Ca+ defficiency, then long-term therapy with massive doses of Vit D
Watch for: weakness, lassitude, HA, N/V/D from toxicity)
120
S and S of DM:
* Acanthosis Nigricans in Type II
* S and S mostly show when 90% of islet cells destroyed.
* Recurrent infections
* High Hgb A1C. Normal is 1.8 - 4
We want to stay below 7 - 8 (it's a little higher because we want growth... as they age, the # will go down.
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S and S of hypoglycemia at night:
restless
awake
nightmares... check BS levels
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Tx for PKU
Dietary restrictions
NO meats, eggs, milk, beans
OK cereals, breads, fruits, vegetables, milk substitute
usually lifelong dietary restrictions
special icky formula
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Urine smells like maple syrup... defect in amino acid metabolism that affects the way the body breaks down proteins:
Maple Syrup Urine Disease
Common in mennonites in PA
S and S:
can be picked up by newborn screening test but its not routine in most states.
- poor feeding
- irritability
- lethargy
- high-pitched cry
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Tx of maple syrup urine disease
dietary restrictions w/only carefully controlled amounts of leucine, isoleucine and valine allowed (special formula)
As they grow, no meats, nuts, eggs, dairy.
Peritoneal dialysis to remove high levels of amino acids
Infections precipitate exacerbations
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The Tx for this condition include no galactose in diet for life; soy-based, meat-based formula is used:
Galactosemia
Inability of body to utilize galactose, resulting in increased serum and urine galactose... leading to liver cirrhosis, cataracts, mental retardation and kidney failure.
Untreated can cause DEATH in 3 DAYS.
```
S and S:
Poor feeding
Lethargy
Hypotonia
Vomiting
Diarrhea
FTT
Jaundice
Seizures
```
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This metabolic disorder is due to the body's inability to process amino acids lysine, hydroxylysine, adn tryptophan... the build up results in damage to basal ganglia (movement centers) in brain:
Glutaric Aciduria
Usually appear normal until several months old when infection of some kind causes presentation of disorder with rapidly appearing S and S:
```
hypotonia
loss of motor control
seizures
vomiting
acidosis
```
* Can be mistaken for CP or child abuse in ER (hemorrhages in brain).
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What is Tx for Glutaric Aciduria:
Diet - Carnitine - may help, but damage is usually done and irreversible unless picked up on newborn screening (optional).
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This metabolic disorder is from the deficiency of enzyme hexosaminidase required for fat metabolism, develops deposits of lipids on nerve cells:
Tay-Sachs
Leads to blindness, mental retardation, and death within 2-4 years.
More commonly seen in east European Jews (1:27 is carrier).
S and S:
Usually normal 5-6 months then start noticing delays
Progressive destruction of nervous system with regression (can't sit up/roll over/reach out/hold objects) Usually die by 5 years.
Tx: NO CURE, NO Tx to stop symptoms or progression.
