Pediatric/Congenital CP Disorders

Question 1

Development of lungs begin at __ of gestation f/b __ periods of lung maturation. The last period is the __ period, with the lungs being fully developed by the age of __.

Answer 1

1) 4 wks
2) 4 periods
3) Alveolar period
4) 8 y/o

Question 2

From weeks 16-26, a critical event occurs > appearance of pulmonary __. __ Production also incr. during the final 2 weeks of pregancy therefore __ babies don’t have surfactant. Therefore, be sure to ask child’s __ age during pt interview.

Answer 2

1) capillaries
2) Surfactant
3) premature
4) gestational

Question 3

Cardiac development starts in wk __ of gestation with formation of endocardial strands. By day 22-23, the heart begins to __ and by day 27 it ___ blood.

Answer 3

1) wk 3
2) beat
3) circulate

Question 4

Fetal circulation __ the lungs to go to the LV via the foramen ovale or __ __. Fetus uses __ to obtain O2/eliminate CO2. By wk __, gestation of cardiac development is complete.

Answer 4

1) bypasses
2) ductus arteriosus
3) PLACENTA (literally replaces the lungs in circulation)
4) 10

Question 5

Congenital Heart Defects (CHD) can be classified as cyanotic where SaO2 is __ , or acyanotic where SaO2 is __.

Answer 5

1) decreased
2) normal

Question 6

Acyanotic lesions (incr/decr) pulmonary blood flow & oxygenated blood is __ back into lungs and body.
(L/R) to (L/R) shunting occurs.
S&S include sweating, (incr/decr) respiratory rate, & HF. It results in (incr/decr) PaO2 to periphery, (incr/decr) SV, incr. heart workload.

Answer 6

1) increase
2) shunted
3) Left to Right
4) incr.
5) decr.
6) decr.

Question 7

Acyanotic Lesions

Name 5 acyanotic CHD lesions. Also take a moment to review the unique features about ea. (AFTER you name them :) )

Answer 7

1) Atrial septal defects – caused by patent foramen ovale
2) Patent ductus arteriosus – often assoc. w/ Down syndrome or prematurity
3) Ventricular Septal Defect – 1 or more small openings in wall separating the ventricles
4) Atrioventricular Septal Defects – atrioventricular canal defects or endocardial cushion defects
5) Coarctation of Aorta – obstruction of left ventricular outflow as a consequence of narrowing of the aorta

Question 8

Cyanotic lesions cause
(L/R) to (L/R) shunting, where most of the blood __ the lungs. This results in (incr/decr) SaO2, incr. signal for __ BC formation (polycthemia), and incr. risk for __ insult.

Answer 8

1) Right to Left
2) Bypasses
3) decr.
4) Red BC
5) CV

Question 8

Name 4 cyanotic CHD conditions. Also take a moment to review the unique features about ea. (AFTER you name them :) )

Answer 8

1) Tetralogy of Fallot – (1) pulmonary stenosis, (2) VSD, (3) overriding aorta, (4) right ventricular hypertrophy
2) Hypoplastic left heart syndrome – (1) hypoplastic left ventricle, (2) aortic and mitral valve stenosis, (3) coarctation of aorta
3) Transposition of great arteries – involves pulmonary artery leaving left ventricle and aorta exiting the right ventricle with no communication between systemic and pulmonary circulation
4) Tricuspid atresia– tricuspid valve between right atrium and ventricle is either not patent or absent
5) Truncus arteriosus– normal separation of aorta and main pulmonary artery does not occur, both right and left ventricles empty into a single large vessel
6) Total anomalous pulmonary venous return – pulmonary veins attach to right atrium

Question 9

Resp. Conditions of Infancy

Name the condition:
-Born at <37 wks of gestation
-Assoc. w/ PDA

Answer 9

Prematurity
Also periodic breathing patterns and apnea of prematurity are abnormal

Question 10

Resp. Conditions of Infancy

Name the condition:
-Seen in infants with infant respiratory distress syndrome
-can be assoc. w/ prematurity in comb w/ PDA

Answer 10

Persistent Pulmonary HTN
infant fails to adjust to breathing outside the uterus

Question 11

Resp. Conditions of Infancy

Name the condition:
-AKA Hyaline membrane disease
-Caused by deficient amount of pulmonary surfactant
-Char. by airless alveoli, inelastic lungs, respiration rate >60 bpm, nasal flaring, intercostal and subcostal retractions, grunting on expiration, peripheral edema

Answer 11

Respiratory Distress Syndrome

Question 12

Resp. Conditions of Infancy

Name the condition:
-Sudden, unexpected death during sleep of infant
-May be linked with resp.
-“Back to Sleep” campaign: infants should sleep supine until they have the ability to roll

Answer 12

Sudden Infant Death Syndrome (SIDS)

Question 13

Resp. Conditions of Infancy

Name the condition:
-Factors may cause fetal rectal sphincter muscles to relax, resulting in meconium in the amniotic fluid
-infant then may aspirate amniotic fluid and meconium

Answer 13

Meconium Aspiration syndrome

Question 14

Resp. Conditions of Infancy

Name the condition:
-Chronic respiratory disorder char. by lung scarring, thickened pulmonary arterial walls, & lung V/Q mismatch
- >1 mo. of resp. s&s, abnormal imaging, & dependence on supp. O2
-Mechanical ventilation is etiologic factor

Answer 14

Bronchopulmonary Dysplasia (BPD)

Question 15

Resp. Conditions of Infancy

Name the condition:
-Genetic, autosomal recessive disease affecting exocrine gland function

Answer 15

Cystic Fibrosis

Question 16

Resp. Conditions of Infancy

Name the condition:
-Rare disease in which mucociliary clearance is impaired because of defective motility of cilia

Answer 16

Primary Ciliary Dyskinesia

Question 17

PEDIATRIC DIAGNOSES WITH SECONDARY CARDIOPULMONARY ISSUES

Name the condition:
-Assoc. w/ endocardial cushion defect, VSD, ASD, Tetralogy of Fallot
-Cardiac defects and sleep apnea are more common
-Exercise throughout life span is critical

Answer 17

Down Syndrome

Question 18

PEDIATRIC DIAGNOSES WITH SECONDARY CARDIOPULMONARY ISSUES

Name the condition:
-Primary immunodeficiency disease, caused by deletion of chromosome 22

Answer 18

DiGeorge Syndrome

Question 19

PEDIATRIC DIAGNOSES WITH SECONDARY CARDIOPULMONARY ISSUES

Name the condition:
-Vertebral defects, imperforate anus, tracheoesophageal fistula, radial and renal dysplasia

Answer 19

VATER Association

Question 20

PEDIATRIC DIAGNOSES WITH SECONDARY CARDIOPULMONARY ISSUES

Name the condition:
-Connective tissue disease that is commonly assoc. w/ aortic aneurysm and aortic/mitral insufficiency

Answer 20

Marfan Syndrome

Question 21

PEDIATRIC DIAGNOSES WITH SECONDARY CARDIOPULMONARY ISSUES

Name the condition:
-Involves deletion of long arm of chromosome 7
-Assoc. w/ supravalvular aortic stenosis and supravalvular pulmonary stenosis

Answer 21

Williams Syndrome

Question 22

PEDIATRIC DIAGNOSES WITH SECONDARY CARDIOPULMONARY ISSUES

Name the condition:
-chronic alcohol exposure in utero

Answer 22

Fetal Alcohol syndrome

Question 23

Pediatric conditions with decr. activity levels are d/t muscle weakness, tone issues, motor planning, motor __, & __.

Answer 23

1) motor learning
2) medical fragility

Question 24

Pediatric conditions with decr. activity levels

Name the condition:
-Disorder caused by nonprogressive but permanent lesion of brain, occurring in utero, during birth, or immediately after birth

Answer 24

Cerebral Palsy

Question 25

Pediatric conditions with decr. activity levels

Name the condition:
-Various types of myelodysplasia, ranging from lack of posterior vertebral arch fusion (occulta) to open spinal defects (myelomeningocele)

Answer 25

Spina Bifida

can include Arnold-Chiari type II malformation – ventilatory problems

Question 26

Pediatric conditions with decr. activity levels

Name the condition:
-Characterized by progressive, symmetrical wasting of musculature

Answer 26

Duchenne Muscular Dystrophy

Question 27

Pediatric conditions with decr. activity levels

Name the condition:
-LMN condition; severe weakness, muscle atrophy, contractures lead to progressive respiratory failure

Answer 27

Spinal Muscular Atrophy

Question 28

What are the HR, RR, & BP (systolic) norms for a premature and newborn?

Answer 28

Premature:
HR: 100-180
RR: <40
BP: 42 +/- 10 (only diff)
Newborn:
HR: 100-180
RR: <40
BP: 60 +/- 10 (only diff)

Question 29

What is a score that can quick assesses an infant’s health?
Hint: it can be taken 1 min and 5 mins after birth.
1 min = necessity for resuscitation
5 mins = predicting mortality and neurologic deficits

Answer 29

APGAR Score

Question 30

1) What does APGAR mean?
2) How is the score interpreted
3) Take a moment to review the APGAR score chart.

Answer 30

1) Appearance, Pulse, Grimace (reflex irritability), Activity (tone), Respiration
2) Lower scores = higher risk of neurological impairment
3) refer to pic