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what chromosomal abnormality is most at risk for AV canal defect?
trisomy 21
what is congestive heart failure? how does it happen? management (3)?
inability of heart to pump an adequate amount of blood to systemic circulation, results from a congenital heart disease
management: digoxin (improve contractility), diuretics (decrease preload), ACEi/beta blockers/calcium channel blockers (reduce afterload)
what is hypoxemia? manifestations of chronic?
arterial oxygen tension/pressure lower than normal
chronic - clubbing and polycythemia (persistent hypoxemia leads to increased viscosity of blood)
what is atrial septal defect (ASD) and ventricular septal defect? s/s for both?
ASD: abnormal opening between atria, could be asymptomatic or signs of HF depending on size of defect and how long untx, characteristic murmur
VSD: abnormal opening between ventricles, characteristic murmur, presents w HF
what is atrioventricular canal defect (what does it consist of)? s/s (2)?
consists of ASD, VSD, which creates large common AV valve; most common in children with down syndrome
s/s: mod to severe HF, mild cyanosis that increases with crying
what is patent duct arteriosus (PDA)? s/s(3)?
failure of the fetal ductus arteriosus to close in the first weeks of life
s/s: machinery-like murmur, asymptomatic or signs of HF
what is coarctation of the aorta? s/s(5)?
localized narrowing of aorta usually near insertion of ductus arteriosus
s/s: HTN upper extremities, bounding pulses in arms, weak/absent femoral pulses, cool pale lower extremities, hypotension in lower extremities
what is aortic stenosis?
narrowing or stricture of aortic valve causes resistance to blood flow in LV
what is tetralogy of fallot? s/s (2)? what are signs of tet spells (6)? how to tx tet spells (4)?
four defects (VSD, pulmonary stenosis, overriding aorta, and right ventricular hypertrophy)
s/s: characteristic murmur, some infants cyanotic at birth or mild cyanosis that progresses over 1 year
tet spells: uncontrollable cry/panic, rapid/deep breathing, cyanosis, decreased intensity of murmur, limpness, convulsions
tet spell tx: knee-chest position, 100% oxygen, iv morphine and fluids
what is tricuspid atresia? s/s (3)?
absence of tricuspid valve, complete mixing of right and left side of blood
s/s: cyanosis, tachycardia, dyspnea
what is transposition of great arteries (TGA)? must have what? tx (2)?
mixed blood flow; pulmonary artery exits left ventricle and aorta exits the RV, no communication between systemic and pulmonary circ
must have associated defect such as PDA, or septal defect for baby to sustain life
prostaglandins initiated at birth, surgical intervention (arterial switch)
what is hypolastic left heart syndrome? what allows for blood mixing? what should be kept open? when to do transplant?
left side of heart is underdeveloped, most sever and most energy used
ASD allows for blood mixing, keep ductus arteriosus open
try to push off transplant as long as possible
what is single ventricle syndrome?
defects that require staged single ventricle palliation
what is the Norwood procedure? what is it for? what should spo2 be?
establishes systemic flow by connecting RV to aorta, creates pulmonary blood flow, chest stays open for days
hypoplastic left heart syndrome
spo2: 75-85
what is the BT shunt? risks? education
blalock-taussig, connection of subclavian artery to pulmonary artery
rosk: thrombosis
education: aspirin
what is sano shunt used for? what does it require?
pulsatile blood flow and used most, less likely to clot, requires anticoags
what is aspirin? used for? requirements?
inhibits platelet function
used for anticoag in infants and children w shunts or other procedures where thrombosis is a concern
must be taken with food and PFA (platelet function assay) has to be taken to assess if dosing is therapeutic
what medication must a mechanical valve replacement go home on? how long can it take to effect INR?
coumadin
3 days
what is cardiac tamponade? dx (1)? other s/s (2)?
accumulation of fluid within pericardial space that leads to impairment of ventricular diastolic filling
dx: Beck’s triad (elevated CVP, decreased arterial pressure, muffled heart tones)
s/s: pulsus paradoxus (systolic blood pressure drops abnormally during inhalation, or breathing in), kaussmaul’s sign (a paradoxical increase in jugular venous pressure (JVP) when a person inhales)
what is infective endocarditis? what is it caused by? s/s (6)? tx (2)?
infection of endocardium and possibly the heart valves that results in vegetations of heart tissue, caused by strep and staph
s/s: malaise, low grade fever, new murmur or change in previous, splenomegaly, osler nodes, janeway spots
tx: high dose IV antibiotics (blood cultures repeated 3 days for effectiveness), or surgical debridement if does not respond to antibiotics
what is kawasaki disease? serious complication? nursing care/tx [6]?
acute systemic vasculitis that lasts 6-8 wks with unknown cause
serious complication: coronary artery aneurysm
care: IVIG, aspirin, I&Os, symptomatic relief, address pt irritability
what is supraventricular tachycardia (SVT)? tx (3)?
most common dysrhythmia in children
tx: valsalva maneuvers, adenosine rapid IVP, synchronized cardioversion (do not shock without sedation)
what is the difference between a child’s hand bones compared to adult? what to teach if fracture occurs to growth plate?
growth plate present and thick periosteum (allows for faster healing and holds bones better)
follow up to see if growth plate was injured if fracture occurs
clinical manifestations of fracture (7)
edema, pain w point tenderness, deformity, ecchymosis, decreased use, warmth, crepitus
what is neurovascular assessment? what are the 6 Ps and what is it assessing?what causes this syndrome? tx (3)?
done routinely after child gets cast.
6 Ps of Compartment syndrome (Pain, Paresthesia, Pulselessness, Paralysis, Pallor, Poikilothermia)
edema causes compartment syndrome
tx: loosen or remove tight clothing or restrictions, notify doctor immediately, do not elevate
what are physiologic effects of immobilization (5)?
dependent edema, atrophy, decreased ventilation, osteoporosis, renal calculi
what is developmental dysplasis of hip? s/s (5)? tx (3)? care (3)?
abnormality in development of proximal femur, acetabulum or both
s/s: ortolani and barlow test (infants), asymmetry of gluteal folds, galeazzi sign (affected leg is lower), older child w limp
tx: pavlik harness (<6mo), bracing/spica cast (>6 mo), hip surgery
nursing care: maintain harness 24/7, check skin integrity, educate on skin care
what is tales equinovarus club foot? what are the 4 types of deformities? tx (3)?
club foot, involves soft tissue, 4 types (inward twist at ankle/foot, contracture of achilles tendon, or atrophied gastrocnemius)
tx: ASAP, serial casting for 4-12 wks, achilles tenotomy, bracing after casting and tenotomy until 4-6 yrs
what is osteogenesis imperfecta? characteristics (6)? what are the 4 classifications? med tx?
genetic d/o that affects body’s production of collagen that leads to weak bones
characteristics: demineralization, multiple fractures, exuberant callus formation, blue sclera, wide sutures, pre-senile deafness
classifications: Type I (mild bone fragility, blue sclera, poor dentition), Type II (lethal), Type III (severe bone fragility, progressive growth failure), Type IV (mild-mod bone fragility, normal sclera, short statue, possible abnormal dentition)
med tx: biphosphonates
what is legg-calve-perthes disease? unilateral or bilateral? what do they usually present with? tx (4)? nursing considerations (3)? common ages affected?
avascular necrosis of femoral head, unilateral
present with knee pain or intermittent painless limp due to sciatic nerve
tx: rest and non-weightbearing, may need brace, surgical tx
nursing: identification, education of disease and compliance, high incidence of hip replacement
ages: 3-12, boys 4-8
what is slipped capital femoral epiphysis? when does it occur? tx (4)?
spontaneous displacement of proximal femoral epiphysis in a posterior and inferior direction
occurs shortly before or during accelerated growth periods or puberty
tx: rest, no weight-bearing, surgery, keep femur in acetabulum
what is scoliosis? dx (4)? tx (3)? post op (4)?
complex spinal deformity in three planes (lateral, spinal rotation, thoracic hypokyphosis)
dx: standing xray (gold standard), forward bending, asymmetry of shoulders, scapula, ill fitting clothes
tx: bracing (stops progression), exercise, surgery
post op: pain control, early motion, NV checks, drainage
what is osteomyelitis? s/s (3)? dx (2)? difference between exogenous and hematogenous? tx (2)?
inflammation and infection of bony tissue
s/s: abrupt, marked leukocytosis, pain, fever
dx: bone scans, MRI
types: exogenous (infectious agent invades bone), hematogenous (preexisting infection spreads)
tx: prompt IV antibiotics, pain control
what are juvenile idiopathic arthritis signs (5)?
clinical manifestations: stiffness, swelling, loss of mobility, warm to touch, growth retardation
what is cerebral palsy? what can it cause (5)? s/s (5)? tx (2)?
non-progressive impairment of motor function, especially muscle control, posture and coordination
can cause: seizures, speech impairments, abnormal vision, hearing, and sensation and perception
s/s: poor head control, clenched fists, stiff limbs, unable to sit w/out support, no smiling
tx: baclofen and diazepam
what is duchenne muscular dystrophy? s/s (7)? most common cause of death?
most severe and common; gradual degeneration of muscle fibers, progressive weakness, and wasting of skeletal muscle
s/s: contractures, waddling gait, Gower’s sign (crawl up legs to stand up from sitting), lordosis, enlarged thighs and upper arms, muscular atrophy, mental deficiency
death by respiratory or cardiac failure
what are the 3 phases of a chemotherapy action plan?
induction: 4-6 weeks
consolidation: eradicate residual leukemic cells and prevent resistant leukemic clones
maintenance: preserves remission
what are 4 other modes of therapy besides chemotherapy?
radiation: breaks down cellular DNA, shrink tumor or curative, palliative for symptom relief
biotherapies: immunotherapies response (immune checkpoint inhibitors, monoclonal antibodies, vaccines)
transplant: allogenic bone marrow transplant, stem cell transplant umbilical cord blood (stem cells have high frequency in circulation of newborns)
peripheral stem cell transplant: autologous, colony-stimulating factor given to stimulate production of stem cells then collected by apheresis
what are 7 oncological emergencies? details on tumor lysis only
tumor lysis syndrome: metabolic abnormalities result from rapid release of intracellular contents during lysis of malignant cells; electrolyte imbalance, oliguria, tetany, acute renal failure and death
risk factors: large tumor burden, sensitivity to chemo, high proliferative rate, high WBC at diagnosis
hematologic
structural
hyperleukocytosis
SVC syndrome
spinal cord compression
disseminated intravascular coagulation
when should a child receiving chemotherapy receive vaccines?
given 2 weeks before or during therapy should be considered inactive, should be revaccinated or receive live virus 3 months after chemo has stopped
what is leukemia? more frequent in what sex and age group? who is at greater risk of developing ALL? what are consequences of leukemia (5)? diagnostic (5)? therapeutic management (2)?
broad group of malignant disease of bone marrow, unrestricted proliferation of immature WBC in blood forming tissues; more frequent in males older than 1, peak onset at 2-5 y.o.; children with down syndrome are 20x higher risk for ALL
consequences: anemia, infection, bleeding tendencies, pancytopenia; spleen, liver and lymph glands show marked infiltration
dx: history, physical manifestations, peripheral blood smear, LP, bone marrow aspiration or biopsy
tx: IV and intrathecal chemotherapeutic agents
hodgkins (age group, origination, staging, dx) vs non-hodgkins lymphoma (age group
hodgkins: prevalent in 15-19 y.o, originates in lymphoid system and metastasizes to spleen, liver, bone marrow, lungs and etc; swollen lymph nodes NT and firm
clinical staging: stage A (asymptomatic), stage B (fever, night sweats, wt loss of 10% or more)
dx: s/s or lymph node biopsy
non-hodgkins: prevalent in <14y.o, disease usually diffuse, cell type undifferentiated or poorly differentiated; dissemination occurs early, often and rapid; mediastinal involvement and invasion of meninges
brain tumors clinical manifestations (3)? what are 2 classifications of brain tumors and where do they occur? dx (7)? what to watch for post op?
s/s related to anatomic location, size and age; increased head circumference, headache, symptoms associated w/ increased ICP
60% are infratentorial (posterior third of brain, primarily cerebellum or brainstem)
40% are supretentorial (anterior two thirds, mainly cerebrum)
dx: clinical signs, neurologic assessment, MRI, CT, EEG, LP, histologic
watch for clear, colorless drainage
what is neuroblastoma? where does it primarily develop? when does metastasis happen? dx (4)? what age has better prognosis?
malignant extracranial solid tumor, develops mostly in adrenal gland or retroperitoneal sympathetic chain, metastasis may have occurred before dx (silent tumor)
dx: s/s depend on location and stage, radiologic studies, bone marrow eval, IV pyelogram to eval renal involvement
younger children have better prognosis
what is osteosarcoma? what sex does it occur more in? primary site of growth? dx and explain why(5)?
bone tumor, occurs more in males during accelerated growth, primary site is metaphysis of long bones and femurs
dx: CT scan, bone scans, bone biopsy (definitive dx), MRI (eval neuromuscular and soft tissue), labs (elevated alkaline phosphatase)
what is wilms tumor/nephroblastoma? more common in what race and sex? peak age? what is most common sign? important priority?
malignant renal and intra-abdominal tumor, more common in african americans and males, peak age 2-3 y.o.
most common sign is abdomen is firm, NT, confined to one side
priority ensuring abdomen is protected from palpation
what is retinoblastoma? Manifestations (4)? what is used for staging? tx (3 stages)?
congenital malignant tumor from the retina, mainly nonhereditary and unilateral
Manifestations: cat’s eye reflex (most common, whitish glow or pupil), strabismus; red, painful eye w/glaucoma, blindness (late sign)
staging: Reese-Ellsworth classification
tx: early stage unilateral (irradiation, cryotherapy), bilateral (attempt to preserve useful vision in least affected), advanced (enucleation, chemo)
common manifestations in immunologic deficiency d/o (7)? transmission?
manifestations: lymphadenopathy, hepatosplenomegaly, oral candidiasis, chronic diarrhea, infections, FTT, malabsorption
horizontal and vertical transmission
what is sepsis? septic shock involves what? tx?
systemic infectious organism that involves pts immune, inflammatory and coagulation responses
septic shock: involves CV organ dysfunction
tx: broad spectrum antibiotics, fluids
what is sickle cell anemia? what causes cells to sickle (acronym)? 2 types of crisis, their signs and causes? dx (4)? tx(3)? what med is contraindicated?
autosomal recessive hereditary hemoglobinopathy, partial or complete replacement of normal Hgb with abnormal Hgb S
hypoxia occurs and causes hemoglobin to sickle ( Severe trauma/bleeding, Infection, Climbing high altitudes, Keeping stress, Limited liquid intake, Extreme temp changes)
types of crisis: splenic sequestration (life threatening, blood pools in spleen, profound anemia, hypovolemia, shock);
aplastic (diminished production and increased destruction of RBC, triggered by viral infection or depletion of folic acid, profound anemia and pallor)
dx: cord blood, newborn screening, genetic testing, sickle turbidity test
tx: penicillin, hydration, hydroxyurea
meperidine (demerol) is contraindicated
pre-blood (6) and active (3) transfusion guidelines?
pre-blood: leukocyte filter, confirm w another nurse, verify identity of recipient and donor’s blood, monitor VS, use blood within 30 mins, infuse over 4 h max
active transfusion: transfuse slowly for first 15-20 mins, VS, stop immediately if reaction occurs
what is hemophilia? manifestations (6)? 2 types of hemophilia? dx (3)? tx (4)?
x-linked recessive, males dominant
manifestations: bleeding tendencies, symptoms may not occur until 6mo, hemoarthrosis (bleeding into joint spaces of knee, ankle, elbow, leading to impaired mobility), ecchymosis, epistaxis, bleeding after procedures
types: A (deficiency of factor VII, classic hemophilia), B (caused by deficiency of factor IX)
dx: amniocentesis, genetic testing of family members, labs (low levels of VIII or IX, prolonged PTT)
tx: factor replacement therapy, DDAVP/desmopressin, prevent bleeding, RICE (rest, ice, compression, elevation)
what are the 3 types of parenting styles?
authoritative: balanced control, shared authority, emphasize on reason
permissive: low control, high warmth, children regulate their own activities
authoritarian: high control, unquestioned mandates
what are the 8 types of family structures?
traditional: man and woman married with ONLY bIological children
nuclear: 2 parents and children
blended: at least one 1 step parent or step/half sibling
extended: grandparents, cousins, aunts/uncles etc in same household
single-parent
LGBTQIA
binuclear: parents not together but co-parent in diff houses
polygamous: one or more spouses are married to other people at same time
what is most important consideration for infants to pre-school?
separation anxiety (6-30 months)
special needs core concepts (normalization and mainstreaming)? what causes for a better outcome?
normalization: any effort to maintain typical family (adapt, incorporating child into community and school)
mainstreaming: integrating children with disabilities into traditional classroom settings
EARLY INTERVENTION
how does different age groups react to hospitalization?
toddler: negativism
preschool: blame themselves, egocentric
school age: choices, personification
adolescent: withdrawn, need independence
visual impairments (myopia, hyperopia, astigmatism, strabismus, cataracts and glaucoma definitions)
myopia: near-sightedness
hyperopia: far-sightedness
astigmatism: unequal curvature of eye in cornea or lens
strabismus: eyes are misaligned or not aligned when looking at object
cataracts: prevents light from refracting
glaucoma: increased intraocular pressure
what is expected and collected assessment data for visual impairments? how to care for visual impairment?
response to light, tracking, rubbing eyes, vertigo, headaches, closing one eyes
care: prevent perinatal infections, vision screening, promote independence adaptations and safety
auditory impairments (conductive and sensorineural definitions, ototoxic drugs) what is red flag for auditory impairment?
conductive: middle ear, caused by recurrent ear infection, decreased loudness
sensorineural: inner ear or auditory nerve, structural defects, distorted sound
ototoxic drugs: aminoglycosides, vancomycin, loop diuretics, some chemo
no babbling by 6 months
2 types of communication impairments
receptive: difficulty understanding and comprehending language
expressive: speaking through verbal and nonverbal
autism spectrum d/o (what is it?) and what should be done?
deficit of social interaction, communication and behavior
EARLY INTERVENTION (highly structured routines, and modifying discipline)
fragile X syndrome (manifestations, dominant or recessive)
cognitive and intellectual impairment, X-linked dominant
manifestations: long head, big ears
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