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PEDS exam 3 review

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Test Review Exam 3- New for Spring 2022
Eating disorders 2

Anorexia Nervosa:
o General:

Eating disorder characterized by fear of gaining weight or
becoming overweight

Typical age of onset is late adolescence

Results from a combo of physiological and psychological
factors

Societal pressures and ideals

Genetic components
o Risk Factors:

Adolescence

Perfectionist personality

Family htx of eating disorder

Low self-esteem

Female gender

anxiety

depression

suicidal thoughts

low body mass index

dehydration

lanugo (fine, soft, unpigmented hair)

cold intolerance

hypotension

crying

compulsive exercising

Anorexia
o s/s:

Severely underweight

Cachectic appearance

Dry sallow skin

Thinning hair

Sparse body hair

Nail pitting

VS: ↓
o Decreased temp, bradycardia, hypotension

Murmur
o 1/3 will have mitral valve prolapse
o Also known as mitral valve regurgitation
o Valves do no not close properly between
each pump causing backward flow through
the valve back to left atrium

Anorexia Nervosa Tx:
o Nutritional support
o Clinical therapy (inpatient or outpatient)
o Treat fluid and electrolyte imbalances and monitor CV
involvement
o Treat with multidisciplinary team (pediatricians, behavioral health
specialists, registered dietician nutritionists)
o Involve family in counselling
o Focus on gradual weight gain of 2-3 lb per week
o May need NG feedings

Anorexia Nervosa Management:
o Outpatient or inpatient tx (requires monthd of tx)
o Nutritional consultation
o Hospitalize if:

FOOD REFUSAL

Severe wt loss

Unstable VS

Require parenteral nutrition

Arrested puberty (Real. Back when I was a teen I lost my
period for months whenever I was starving myself to lose
weight)
o Frequent monitoring labs, VS (including ortho hypotension,
irregular and decreased HR, hypothermia)
o Food journal noting intake, binging, purging episodes
o Journal: moods, behaviors, exercise
o Therapy include eval for anxiety, depression
o Support group

Bulimia Nervosa
o General:

Eating disorder characterized by periods of binge eating
followed by periods of purging

Purging = self-induced vomiting, diuretics, and/or
laxatives
o Risk factors:

Poor self-image

Societal pressures

normal weight or only slightly underweight

food rituals
o s/s:

compulsive ways in which a person interacts with
food that produces anxiety when not followed

ex: taking abnormally small bites, tearing food apart

labile mood

repeated fad dieting

diets for “fast weight loss”

thinning hair

brittle nails

hypokalemia (vomiting, diuretics, lax)

anemia

Russell sign:

calluses on backs of hands and fingers

bradycardia

esophageal irritation

poor dentition
o Treatment:

Cognitive behavioral therapy

Teaches children to change reactions so that
automatic negative thought patterns are replaced w.
alternative ones

SSRIs
Metabolic disorders- especially PKU- 2

Phenylketonuria (PKU) (info on slide)
o Rare, inherited, metabolic disorder
o Inborn error of metabolism detected thru newborn screening
o Follows and autosomal recessive inheritance pattern
o Cause:

Deficiency of a liver enzyme

Body is unable to process phenylalanine (amino acid-think
protein)
o Result:

Irreversible brain damage from high levels of
phenylalanine
o S/S:

Irritability

Vomiting of protein feedings

Musty body smell

Urine odor

Increased reflex action

Seizures

Skin rashes/eczema

Microcephaly

Developmental delays

Behavior abnormality
o Tx:

Meal plans low in phenylalanine (low protein)

Avoid protein foods: meat, milk, eggs, beans, nuts,
aspartame (in diet soda)

Level testing
Genetic disorders and genes 3

General info:
o Caused by abnorm in individual’s genetic material
o Complex:

Categorized into:

monogenic (Mendelian)
o can be autosomal dominant, autosomal
recessive, x-linked dominant, or x-linked
recessive

multifactorial
o caused by multiple gene and environmental
factors

nontraditional inheritance
o do not follow typical patterns

chromosomal disorders
o do not follow straightforward pattern of
inheritance (error in sperm or egg)
o can be structural or numeric abnormalities
o increased risk of health complications (especially cardiac)
o hearing and vision problems common

Inheritance:
o x-linked (general)

altered gene on X chromo

male inherits altered x-linked gene they develop condition;
female heterozygous or homozygous

expression similar to autosomal disorders
o x-linked recessive

affects more males than females (males only have 1 x)

no male to male transmission (hemophilia and Duchene
MD)

girls need 2 abnorm genes to have, 1 gene daughter, carrier
o autosomal recessive:

2 copies of abnormal gene in homozygous state required to
produce phenotype

Fragile X syndrome:
o Inherited gene condition and most common cause of intellectual
disability
o Boys experience more severe s/s (they only have 1 X chromo)
o X-linked dominant inheritance pattern
o s/s:

minor dysmorphic features (deformity)

developmental delay

large head

long face

prominent ears

flat feet

enlarged testes

flexible joints

sensory processing troubles

behavioral problems
o Dx: genetic testing
o Tx:


Developmental therapies and interventions

No cure but normal life span
Trisomy 13
o AKA Patau Syndrome
o Non-inherited chromosomal condition
o 3 copies of chromosome 13 present in each cell
o s/s:

cleft lip and cleft palate

extra digits

clenched hands

close set eyes

hernias

low set ears

scalp defects

single palmar crease

limb abnormalities

microcephaly

undescended testes
o Complications:

Apnea

Deafness

Heart failure

Seizures

Intellectual disability

Vision problems
o Prenatal testing can diagnose condition
o Management


most infants do not survive past 1st month of life

symptomatic care for infant and supportive care for family
Trisomy 18
o AKA Edwards syndrome (Edward from twilight is forever 18-(not
really he turned at 17 but whatevs lol))
o Chromosomal condition typically not inherited

Partial trisomy 18 can be inherited, but the person is
typically asymptomatic
o 3 copies of chromosome 18 in each cell
o S/S:

Intrauterine growth restriction

Low birth wt

Small head

Small jaw

Low set ears

Short eyelid fissures

Clenched and overlapping digits

Hypotonia

Short sternum

Narrow hips with limited abduction
o Management


most infants do not survive past 1st month of life

symptomatic care for infant and supportive care for family
Trisomy 21
o AKA Down syndrome
o Chromosomal condition resulting from random events that occur
during formation of reproductive cells
o 3 copies of chromosome 21 in each cell
o Degree of intellectual disability and degree of developmental delay
in severity
o S/S:

Flattened bridge of the nose

Almond shaped eyes

Cardiac defects

Hearing and vision impairments

Increased mobility of cervical spine
o Complications:

Heart defects

GI abnormalities

Soft-tissue and skeletal changes

Hypothyroidism

Atlantoaxial instability of first and second cervical
vertebrae

low muscle tone

autoimmune disorders

infectious dz

Prenatal tests:
o Dx:

Ultrasound

Amniocentesis

Chorionic villus sampling


Percutaneous umbilical blood sampling
Children:

Echocardiogram

Abdominal ultrasound

Lab testing

Sleep apnea test

Cervical x-ray

Thyroid hormone levels
o Tx:

Assess for developmental delay using appropriate
milestones based on children with trisomy 21

Refer to therapies, early intervention, and services (speech,
occupational, PT)

Turner Syndrome
o Affects only females
o Chromosomal condition caused by complete or partial absence of
second sex chromosome
o s/s:

difficulty with nonverbal communication skills and
executive function

wide or weblike neck

broad chest with widely spaced nipples

slowed growth

cardiac defects

short fingers and toes
o complications:

chronic middle ear infections

hearing loss

autoimmune disorders

heart, liver, and kidney abnormalities
o Tx:


Treat with growth hormone and estrogen therapy
Klinefelter Syndrome
o Most common sex chromosomal abnormality
o Affects only males
o Karyotype and phenotype male XXY
o 1 or more extra X chromosomes present
o Presentation:

Dx delay until adolescence or adulthood

Lack of secondary sex characteristics (physical features)

↓ facial hair

↓ pubic hair

Hypogonadism (gonads (sex glands) produce little to no
hormones)

Underdeveloped testes

Infertility

Gynecomastia (overdevelopment or enlargement of breast
tissue in males)

Taller than average

Long legs

Short torso

Learning disabilities

Motor delay

Speech/lang difficulty

ADD
o Management:

No cure

Focus on enhancing masculine characteristics and
minimizing female characteristics

Testosterone replacement

Cosmetic surg
o Nursing management:

Supportive

Education
Hemoglobin r/t pediatrics and iron

Fetal Hgb (Hgb F)
o Until 4-6 mos of life
o Shorter cell life and higher quantities
o Increase risk of anemia and O2 carrying capacity
o RBC transfer from liver to bone marrow and balance between
oxygenation and production affected

Hgb A:
o Predominant type of Hgb 6 months after birth

Heme
o The part of Hgb that has iron and carries O2
Iron
o Fetus:

Receives iron thru placenta
o Preterm infant:

Misses final wks/mo of transplacental iron transfer so risk
increases for iron deficiency anemia
o Term infant:

Physiologic anemia occurs between 2-6 mo d/t rapid
growth and maternal iron stores (in milk) depletion
between 4-6 mo
o Adolescents:

Rapid growth and starting period (females) lead to
increased risk of anemia, must increase iron intake
o Iron intake:

Critical for appropriate development of hemoglobin and
RBCs


Iron fortified cereal

Breast milk might need iron supplement
Iron deficiency Anemia:
o Causes:

Not enough iron to produce Hgb

Decreasing maternal stores and rapid growth times
(infants/adolescents)

Hgb levels decrease, o2 carrying capacity of blood
decreases, results in weakness and fatigue
o Associated with:

Delayed growth

Cognitive delays

Behavior changes
o Assessment:

Health htx:

Irritability

HA

Dizziness

Weakness

SOB

Pallor

Dietary intake of iron

Health issues
o Risk Factors:

Gestational diabetes

Prematurity

Maternal anemia

Low iron formula

Breastfed-no iron supplements

Low socioeconomic status

Antacids
o Exam:

Lethargy

Fatigue

Inspect skin for pallor

Spooning of nails

Pulse ox

HR-tachy

Murmur

Splenomegaly
o Tx:

OTC iron supplements

Severe: may need transfusion
o Nursing management:

Safety:

neurologic function r/t low O2 w/risk for fatigue

decrease eating

difficulties with ambulation (sitting, walking,
standing)

Nutrition:

Formula with iron

Iron supplements at 4-5 mo for breastfed infants
(iron fortified cereal)


Limit fast food consumption
Iron rich foods:

Red meat

Tuna

Salmon

Eggs

Tofu

Enriched grains

Dried beans


Peas

Dried fruits

Green leafy veggies

Iron fortified cereal
Iron supplement admin:

Measure precisely

Liquid placed behind teeth (iron stains)

May cause constipation
o Increase fluids and adequate fiber intake)


Dark colored stool
Follow up:

Monitor iron levels and iron intake
Math problems 2-see “math to know for exams”
Allergic reaction

Allergies result from exposure to environmental or food allergens

Caused by cell damage from antigen-antibody rxns that release histamine
and causes allergic s/s

3 steps to dx:
o Personal and med htx
o Physicial examination
o Diagnostic testing

May use allergy testing to diagnosis and identify allergens

s/s:
o itchy eyes
o runny nose
o stomach cramps
o hives
o swelling
o cough
o wheezing
o SOB
o Redness
o Pain
o Feeling faint
o Throat closing

Tx:
o Antihistamines
o Bronchodilators
o Corticosteroids
o Preventative inhaled meds

Common allergens:
o Food allergens:

Cows milk

Eggs

Wheat

Chocolate

Citrus fruit
o Inhalant allergens

Mold

Pollen

House dust

Pet dander
o Drug allergens

Oral and injectable
o Contact allergens

Plants

Dyes

Chemicals
o Other:

Animal serum/venom

Insect stings
o Food and latex allergies:

Wheezing is sign of edema occurring in the airway

Sign of resp complication

Provide dietary teaching on how to read food labels for
food allergies

Children should avoid exposure to allergens

3 types of rxns to natural latex:

IgE mediated

Cell mediated contact dermatitis

Irritant dermatitis
o Children with spina bifida should never be
exposed to latex
anaphylaxis 2

Acute, immediate, and severe IgE-mediated response to allergen

Most common triggers:
o Nuts
o Shellfish
o Eggs
o Insect stings
o NSAIDs
o Radiopaque dyes
o Latex

Usually occurs within 5-10 min of contact with allergen

Tissue edema leads to vasodilation and circulatory collapse

s/s:
o pruritic urticaria
o coughing
o lip and tongue swelling
o stridor
o extreme anxiety
o asthma attack
o loss of consciousness

Tx and management:
o IV epinephrine
o Corticosteroids
o Antihistamines
o Support airway with intubation and ventilation if lip and tongue
swelling and airway compromise
o Assess circulation and administer IV fluids to provide vol
expansion
o Monitor for at least 2 hrs after anaphylactic rxn
o Use intramuscular epinephrine when in community or outpatient
settings

Send pt to ER after
General eye and ear (such as otitis media and how to look in the ear) 2

Otitis media
o Bacterial/viral infection of middle ear
o ET position increase risk secondary to URI
o Complications:

Hearing loss

Expressive speech delay

Tympanosclerosis

TM perforation

Chronic suppurative (pus) OM

Acute mastoiditis

Bacterial meningitis and abcess
o Bacterial

Antibx (amoxicillin PO) if rapid onset s/s

s/s: middle ear inflammation, ear pain, erythema of TM

may resolve wo antibx just wait and see
o Viral

Resolves on its own
o PE tubes:

Equalize pressure

Allow air in and fluid to drain

Last 6 mo

Fall on its own

Do not prevent infections

Keep ears dry w. earplugs in pool or lake
o Otitis media with effusion

Fluid in middle ear wo s/s infection

Risks:

Smoke

Absence of breastfeeding

Frequent URI

Allergy


Age (younger)

Male

adenoid hypertrophy

ET dysfunction

Congenital disorders
Complications:

Hearing loss

Deafness

AOM

Children younger than 3: pull pinna down (short) and straight back

Children older than 3: pull pinna upward (tall) and back
High and low blood sugar signs and insulin –many
Type 1 diabetes

Autoimmune condition resulting in pancreatic damage and lack of insulin

s/s:
o wt loss
o polydipsia
o polyphagia
o polyuria
o fatigue
o blurred vision
o mood changes

diagnose with lab testing (A1C, fasting glucose, plasma glucose)

management:
o insulin therapy
o glucose monitoring
o insulin education
o maintaining proper nutrition
o pt and fam education
o
Fever management

Fever usually peaks in evening

Antipyretics used for discomfort with fever
o Acetaminophen: 10-15 mg/kg/dose q 4-6 hrs limit 5 doses/24 hrs
o Ibuprofen: 5-10mg/kg/dose q 6 hrs; increase 6 months age; limit 4
doses/24 hr; never give on empty stomach

Never use aspirin (ASA) for child w. fever d/t risk for Reye’s syndrome

Monitor for s/s of dehydration

Sponging:
o Controversial; done after antipyretics; use tepid (not cold) water or
alcohol; ensure sponging does not initiate shivering

Call provider:
o Less than 3 mos and increase T 100.4 (38)
o Any child who is lethargic or listless no matter what temp is
o Fever increase 3-5 days
o More than 105
o Immunocompromised by illness w/ cancer/HIV

Light clothing

Remove blankets

Cooling blanket

Fan

Do not induce shivering/discomfort
Signs of dehydration (such as mild to severe)
Sickle cell 2

Abnorm Hgb gene (Hgb S)

Sickle-shaped RBCs (clump together)
o Not clotting, just clumping

Autosomal recessive disorder
o Asymptomatic carriers

Ethnically linked (African, Mediterranean, Hispanic, middle eastern,
Asian)

Hgb F is protectant for 1st 4-6 mo of life
o Hgb F doesn’t behave same way
o Once we switch to Hgb A we start to have problems

Occlusion and hemolysis
o RBCs are fragile and break down faster

Premature RBC death
o Low RBC counts
o Hemolytic anemia

Sickle cell crises:
o Triggered by hypoxia, acidosis, fever, dehydration, and
hypothermia
o Vaso occlusive crisis:

Clumped up sickle cells are occluding vascular circulation

Severe pain from ischemia

Dactylitis (swelling of fingers and toes)
o Splenic sequestration:

Blood gets trapped in spleen
o Silent cerebral infarct:

Neuro assessments

Stroke or silent cerebral infarct
o Acute Chest syndrome:


Pulm vessel occlusion

Mimics pneumonia but infarcts and not infiltrations
Sickle Cell Tx:
o Address triggers:

Isotonic fluid for dehydration/fluid bolus

Warm compresses for pain

Prevent infection (hand washing, antibx, vax)

Treat fevers quickly: give antipyretic
o Pain management:

Nonpharm interventions

Non-steroidal meds adjunct

Opioids when needed
o Bone Marrow transplant:

Autologous stem cell therapy w. human leukocyte antigenmatched sibling

Cord blood transplants
o Hydroxyurea:

Increases level of fetal Hgb

Reduces amount of crises and promotes splenic function

Can cause neutropenia
o Deferoxamine:

Used if hemolysis results in high levels of free iron

Binds with the excess free iron that are released by the
hemolyzed RBCS

Subq infusion:

Chelating agent to bind iron

Use with vitamin C

Monitor for SE
o Transfusion of packed RBCs
o Psychosocial support:


Sickle cell foundation

Family
Management:
o Early identification and prevention of crisis
o Family education, genetic testing, importance of follow ups
o Prophylactic penicillin: prevent infection
o Folic acid: helps make RBCs

Prevention and early detection of vaso-occlusion
o Any febrile illness: seek immediate attn
o Vax and penicillin prophylaxis
o Encourage adequate fluid intake
o Limit exposure to temp extremes
o Avoid stress and overexertion
o Contact HCP at first sign of pain crisis
o Seek tx immediately if:

Pale and listless

Abd pain

Limp or swollen joints

Cough


SOB

Chest pain

Unusual HA

Loss of feeling

Sudden weakness

Priapism (sickling in penis/ prolonged erection)
Sickle cell labs:
o Newborn screening:

Law in all states indicates possibility of sickle cell dz
requiring further eval by Hgb electrophoresis or sickle cell
turbidity test
o Hgb:

Baseline usually 7-10 mg/dL (12-17 norm)

Significantly lower w. splenic sequestration, acute chest
syndrome, or aplastic crisis
o Hgb electrophoresis:

Blood test to look at Hgb and see types
o Reticulocyte count (immature RBC)

Greatly elevated

Body is having to make lots of RBCs to replace the ones
that are bad
General cancer and neutropenic precautions

Oncological principles:
o Proto-oncogenes:

Normal genes that can mutate into cancer cells

Can regulate cell growth, division, death
o Can mutate to form oncogenes which create unchecked cellular
growth and resulting malignancy
o Tumor suppressor gene involved in cell growth and death
(apoptosis)
o Neoplasm: results from uncontrolled regulation of tumor
suppressor cells (malignant or benign)
o Process of oncogenesis is the same in children and adults
o Some genetic mutations may contribute to cancer development

Peds vs adults cancer:
o Origin

Peds: Embryonic cells, blood cells, CNS

Adults: epithelial cells
o Part of body affected

Peds: tissues, bone marrow components

Adults: solid organs
o Cause

Peds: not well understood

Adults: mainly dt environmental toxins
o Prevention

Peds: human papilloma vax; most not preventable

Adults: most preventable thru screening and limiting
exposure to toxins
o Response to tx


Peds: highly responsive

Adults: less responsive
Chemo SE/risks
o Infection:

Pts are at high risk for infection bc myelosuppression dt
chemotherapy

Monitor WBC and practice neutropenic precautions

(masks and appropriate ppe when in pts room, no
fresh fruits/veggies, no raw/rare meat, only
pasteurized dairy products, no live/active cultures in
yogurt) (this is from google and my basic
knowledge from working at the oncology unit-could
not find in PowerPoint)

Admin granulocyte colony stimulating factor
o Anemia

RBC suppression

Treat anemia with iron supplementation, epoetin alfa, blood
transfusion
o Hemorrhage

platelet suppression

Treat platelet suppression by monitoring for s/s of internal
bleeding, avoiding high impact activity, avoiding rectal
temps and other procedures
o Nausea:

Administer antiemetics before infusion and around the
clock 24-48 hrs after

Nonpharm therapies: ginger, peppermint oil. Acupuncture,
and craniosacral therapy

Cluster care to minimize interruptions
o Mucositis

White patches, ulcer, redness

Suck on ice chips/ice pops for pain or meds

Avoid spicy, acidic, salty, hot foods

Eat moist and soft foods

Rinse mouth 5-6x daily

Use soft toothbrush
o Pain:


Admin pain meds
Oncological emergencies:
o Tumor lysis syndrome

Metabolic abnormalities from release of intracellular
contents after malignant cells are destroyed

hyperkalemia, hyperphosphatemia, hypocalcemia,
hyperuricemia

hydrate with IV fluids b4 chemo

treat with allopurinol: for uric acid buildup
o Sepsis

Progression of infection in children w. neutropenia

Neutrophil count <500 at higher risk

Tx w. fluids, antibx, vent support
o Superior vena cava syndrome

Compression of vena cava

s/s:

dyspnea, cough, HA, pleural effusion, edema in
neck and UE

treat tumor to reduce pressure

may need to intubate
o Increased ICP

From primary tumor, metastases, surg procedure

May result in brainstem herniation wo intervention
ALL (leukemia)

Acute Lymphoblastic Leukemia

Most common cancer in children in U.S

Immature nonfunctioning WBC (lymphoblasts) dominate bone marrow
production

RBC and platelet suppression from WBC production

s/s:
o fatigue
o pallor
o fever
o anorexia
o petechiae

3 phases of chemo for Tx:
o Induction phase (4 wks):

induce remission
o Consolidation phase (variable):

Strengthen remission and CNS prophylaxis w. intrathecal
chemo
o Maintenance phase (2-3 yrs)


Eliminate residual cancer cells w. intermittent chemo
Ensure pain management for procedures
Hodgkin’s lymphoma

Characterized by presence of Reed-sternberg (RS) cells
o B lymphocytes that have lost immune function

Typically starts in lymph nodes and spreads thru lymph system (rarely
occurs below the diaphragm)

s/s:
o swollen, painless, rubbery lymph nodes in cervical and
supraclavicular region
o fever
o night sweats
o cough and dyspnea (mediastinal mass)

staged based on spread

tx: combo of chemo and low dose radiation
Ticks

Use fine-tipped tweezers

Protect fingers w. tissue, paper towel, latex gloves

Grasp tick as close to skin as possible and gently pull upward w. slow
steady, even pressure

Do not twist or jerk tick

After removal, clean site q. soap and water

Wash hands

Save tick for identification if illness occurs
lice

Type of parasite

Pediculosis capitis
o Aka head lice

Pediculosis pubis
o Pubic lice

s/s
o itching
o nits
o look like dandruff but do not comb away
General psych

Disorders result from genetics, physiological changes, and environmental
exposures

Mental health:
o State of well-being in which every individual realizes his or her
own potential, can cope with the normal stresses of life, can work
productively and fruitfully, and is able to contribute to his/her
community

Cognition:
o The process of thought and knowing that is acquired thru
experiences and maturation

Pediatric vs Adult Mental Health
o Definitions consistent for children and adults
o Anatomical changes to fetal brain yield cognitive and behavioral
deficits
o Children differ from adults in the scope, presentation, and
progression of mental and cognitive disorders
o Access to good nutrition, positive relationships, and safe housing
affects development of mental illness and cognitive disability

Gen Nursing Interventions:
o Provide supportive care

Play therapy

Form of psychotherapy that encourages children to
express feelings and emotions thru play


Used for 3-12 yrs of age
Therapeutic play

Technique employed by child life specialists for
hospitalized children

Art therapy

Incorporates creativity in healing and expressing
childhood emotions (all ages)
o Maintain safe environment

Ensure safety from SH

Environmental assessments


No sharp objects or tubing

Standard safety measures

Adult supervision
Assess the safety or home environment if outpatient and
hospital environment if inpatient
Special needs

Learning Disabilities:
o Difficulties in receiving and processing info and generating
appropriate responses
o Most common disorder: dyslexia (disorder of receptive lang that
creates difficulty using letters to decode written lang)
o Common s/s may not appear until child enters school
o S/S:

Slower acquisition of lang and math skills

Difficulty recognizing letters and numbers

Problems with reading comprehension
o Identify thru standardized developmental eval

Assess for sensory difficulties
o Treat with an individualized plan (IEP) to foster appropriate
growth and development
Stem cell transplant

Intense chemotherapy and radiation to create myelosuppression followed
by infusion of stem cells

Autologous transplantations use the child’s own stem cells

Allogenic transplantations use donor stem cells

Before transplant:
o Myelosuppression to decrease potential rejection
o Protective iso in positive pressure room
o Prophylactic antibx, antifungals, and immunoglobulins

After transplant complications:
o Graft-versus-host dz (acute or chronic)
o Growth retardation, hypothyroidism, and secondary cancers
Death perception by age

Infants/toddlers (birth to 3 yrs)
o Have little to no concept of death
o Egocentric thinking prevents their understanding death (toddlers)
o Mirror parental emotions (sadness, anger, depression, anxiety)
o React in response to changes brought about by being in hospital
(change of routine, painful procedures, immobilization, less
independence, separation from family)
o Can regress to an earlier stage of behavior

Preschool children (3-6 yrs)
o Egocentric thinking
o Magical thinking allows for the belief that thoughts can cause an
event such as death (as a result, child can feel guilt and shame)
o Interpret separation from parents as punishment for bad behavior
o View dying as temporary bc of lack of a concept of time and bc
dead person can still have attributes of the living (sleeping, eating,
breathing)

School-age children (6-12 yrs)
o Start to respond to logical or factual explanations
o Begin to have an adult concept of death (inevitable, irreversible,
universal) which generally applies to older school age children (912)
o Fear often displayed thru uncooperative behavior
o Can be curious about funeral services and what happens to body
after death

Adolescents (12-20 years)
o Can have adult-like conept of death
o Can have difficulty accepting death bc they are discovering who
they are, establishing an identity, and dealing with issues of
puberty
o Rely more on peers than the influence of parents which can result
in the reality of a serious illness causing adolescnets to feel
isolated
o Can be unable to relate to peers and communicate with parents
o Can become increasingly stressed by changed in physical
appearance due to medications or illness than the prospect of death
o Can experience guilt and shame
Autism (ASD) 3

Genetic basis combined with environmental factors (no link to vax)

Implement standardized developmental screenings into well-child
appointments (MCHAT, Ages and Stages, Denver developmental test, etc)

s/s:
o stereotypy (repetitive movements)
o obsessive behavior
o difficulty with sensory integration
o echolalia (repetition of words w/out meaning)
o avoidance of eye contact

Impairments:
o 3 specific areas:

Communication

Socialization interaction

Repetitive & characteristic behavior

Perseverative/stereotypy:
o Rocking, flapping, lip smacking
o + some have intellectual disability, others do
not

ASD tx:
o Early identification and referral to early intervention programs are
the cornerstones of effective tx
o Plan educational activities that limit overstimulation of senses
o Collab with school personnel to align child’s needs for maximal
learning and limited distress
o Consult with speech therapist to improve communication skills
o Utilize multidisciplinary approach (developmental pediatricians,
behavioral health specialists, occupational therapists)
Idiopathic thrombocytopenia purpura (ITP)

Clotting disorder-IMMUNE RESPONSE

Immune response approximately 1-4 wks after viral infection causing
production of antiplatelet antibodies causing platelet destruction

More common in young children, most self-limiting, recover in a few
months

Presentation:
o Bruising
o Petechiae (pinpoint)
o Purpura (larger areas)

Complications:
o Severe hemorrhage
o Intracranial hemorrhage
o Organ bleeding

Platelet count & Tx:
o <10,000 (norm 150,000-400,000): give corticosteroids (prednisone
or prednisolone: po 2-3 wks or count >30,000)
o IVIG
o Platelet transfusions not indicated unless life threatening

Assessment:
o Previously healthy child with recent increase bruising, gum
bleeding, epistaxis (nose bleeds)
o Recent viral infection
o MMR immunization
o Meds with thrombocytopenia SE

Note: petechiae/purpura and progression include lips and oral mucosa

Management:
o Observation
o Re-eval
o Avoid ASA and NSAIDs
o Give acetaminophen for pain PRN
o Avoid trauma (EX: CONTACT SPORTS)
o Know s/s serious bleeding and when to call HCP
Henoch Schoenlein

Clotting disorder

Develops in association with viral or bacterial infection (typically
respiratory)

Benign, good prognosis
o Unless ongoing nephrotic syndrome d/t renal injury and possible
HTN, pulm, cardiac, neurologic complications

If renal injury:
o Renal function tests
o Eval BP
o Tx as needed

Presentation:
o Vasculitis (leakage) with IgA(antibody) dominant immune
deposits affecting small vessels
o Skin (purpura rash)
o kidney, gut, and joint pain

Tx:
o As needed with corticosteroids if severe joint/GI manifestations

Management:
o Treat s/s
o Pain management
o Hydration if norm renal function
o I&O
o Teach s/s renal injury to parents and when to notify HCP
Beta thalassemia aka Cooley’s Anemia

Abnorm Hgb beta chains lead to rapid RBC destruction

Autosomal recessive condition

Ethnically linked (Mediterranean, middle eastern, Asian)

Hemolytic anemia with bone marrow hyperplasia d/t erythropoiesis (thin
cortical bone w. new growths)
o Bone marrow makes lots and lots of RBCs resulting in expansion
of bone marrow

Characteristics of dz:
o Severe anemia
o Cellular damage
o Free iron buildup (hemosiderosis)
o Bronze skin
o Frontal bossing

Manifestations:
o Bone malformations (maxillary, frontal lobe)

Caused by the bone marrow expanding trying to make
more RBCs
o Osteoporosis and osteopenia
o Splenomegaly and hepatomegaly
o Cardiomyopathy
o Short statues
o Yellow or bronze skin pigmentation

Tx:
o Transfusion therapy
o Chelation therapy
o Bone marrow transplantation
o Monitor effects on growth and development

Tx (2nd slide)
o Frequent blood transfusions to replace deficient RBCs

Infection control with central venous access devices

Follow blood bank protocols and keep in blood refrigerator

Monitor for transfusion rxns
o Chelation therapy to reduce free iron

Oral or parenteral agents

Take with vit C

Monitor for SE, kidney damage, liver damage

Use a supervised care approach to improve compliance
o Splenectomy
o Growth hormone replacement

Nursing Management
o Blood transfusion, education, monitor for transfusion rxns
o Chelation:

Admin with transfusion

Iron—ferrous; chelation products have fer in their name
o IV Desferal (deferoxamine)

Chelating agent

Binds to iron and allows its removal via stool & urine

Given subq at home by family via battery pack infusion
pump
o Exjade

Oral deferoxamine

Well tolerated

Minimal SE

Monitor renal and liver function

Monitor for GI bleeding

Dissolve in juice or water
o Family teaching:

Adherence to transfusion and chelation schedule
Chelation therapy for iron and lead- drugs used

Iron:
o IV Desferal (deferoxamine) or subq
o Exjade (oral deferoxamine)

Lead:
o EDTA-ethylene diamine tetra-acetic acid
Autosomal recessive inheritance

2 copies of abnormal gene in homozygous state required to produce
phenotype

Both parents of affected person must be carriers of abnorm gene (25%)

In other words, requires 2 copies of the mutated gene (one from each
parent) to cause the disorder
Humoral vs. cellular immunity

Humoral immunity:
o Specific immune function
o Involves B cells, which recognize specific antigens and secrete
antibodies
o Training

Cellular immunity:
o Specific immune function
o Involves T cells which attack antigens marked by B cells
o Automatic
Organ donation

Open casket funeral usually possible for organ, eye, and tissue donors

Thry entire donation process, body is treated with care, respect, and
dignity and will not be disfigured

There is no cost to donors or their families

Federal law prohibits buying and selling organs in US
o Violators punished with prison sentences and fines
o Dang, plan b is out the window 

Majority of deceased organ donors are pts who have been declared brain
dead

National computer system matched donated organs to recipients

Most major religions in US support organ donation and consider donation
as the final act of love and generosity towards others

Anyone, regardless of age or med htx, can be a donor
Childhood infectious diseases (basic definitions and cause)

Types of infections:
o Bacterial: staph, strep, sepsis

MRSA

Staph infection resistant to certain antibx

Acquired thru contact, resp droplets, blood, sharing
personal items, contaminated surfaces

Scarlet fever
o Sandpaper rash and strawberry tongue

Diphtheria

Pertussis (Bordetella)

Tetanus (lockjaw)

Botulism

Osteomyelitis

Septic arthritis
o Viral: in general (viral exanthems: a rash caused by virus)

Mumps: inflammation of parotid glands; fever and
parotiditis

Spread by resp secretions

Varicella

Measles(rubeola)

Highly contagious

Spreads thru resp secretions

Mild fever, conjunctivitis, coryza, cough, kolpik
spots (clustered white lesions in oral mucosa),
maculopapular rash

Rubella

German measles

Spread by aerosolized particles

Irregular macular rash, fever, malaise, HA, sore
throat, red eyes, lymphadenopathy
o Zoonotic:

Cat scratch dz: painful swollen nodes

rabies
o Vector-borne:

Lyme dz: from deer tick bite, ring like bull0eye rash around
bite, remove tick, treat w. antibx

West nile: mosquito bite, febrile illness, may lead to
meningitis

Rocky mountain spotted fever: dog and wood tick bite, tx
with tetracyclines

Malaria: mosquito bute; high fever and chills may have
cyclic pattern

Zika: mosquito bite; can cause microcephaly
o Parasitic and helminthic:

pediculosis (lice)

scabies

helminth

worms

pinworms (fecal-oral route-rectal night itchingscrotch tape) (everyone had this in cuba)

roundworms, hookworms (pores, hair follicles, skin,
eating)
Chickenpox (varicella)

Itchy red macules then papules-vesicles-scabs

As initial lesions progress thru stages, new lesions form on trunk and
extremities even as some scab dev 2 wks after exp

Contagious 1-2 days before eruption

Not contagious once all lesions scab and dry

Extremely contagious herpes virus

Prevented thru vax

Can remain latent and reactivate later as shingles

Transmitted thru contact with weeping lesions or nasopharyngeal
secretions

Children contagious 1-2 days before rash appears and until all lesions are
crusted over

s/s management:
o acyclovir for severe cases
Treat hypoglycemia

15 g of carbs

Posterior pit gland disorder

Not enough ADH

Most common form

No cure

Life-long tx

Characteristics:
DI
o Excessive thirst
o Excessive urination not affected by decreasing fluid intake
(polyuria/polydipsia)

Cause:
o Head trauma
o Post cranial surg
o Genetic mutation
o Granulomatous dz (immune deficiency)
o Infections (meningitis/encephalitis)
o Vascular anomalies
o Congenital malformations
o Leukemia
o Meds inhibiting vasopressin(adh) release (phenytoin)
o Idiopathic/hereditary

Antidiuretic hormone (ADH) is called vasopressin and comes from
hypothalamus and posterior pit gland
o In DI body does not produce enough adh
o Pt is not able to hold on to urine or concentrate it
o Pt will urinate until dehydrated
o Drink excessively to compensate for water loss

s/s:
o weight loss
o ftt
o dehydration (dry MM, decreased tears, poor skin turgor)
o increased UO
o tachycardia
o tachypnea

Tx:
o Daily replacement of ADH with DDAVP (desmopressin) which is
vasopressin/ADH

Nursing management:
o Individualized care
o I&O
o Monitor for hypernatremia (thirst, neuromuscular excitability,
confusion, seizures)
o hydration
o promote activity
o education
o daily wt

DDAV
o Keep refrigerated
o Clear nostril before giving
o Proper dosage
o Repeat dose if child sneezes
o Measure specific gravity
o Monitor s/s of overdose (siadh)

Confusion

HA

drowsiness

Rapid wt gain secondary to fluid retention
SIADH

Excessive secretion of ADH resulting from central nervous system
changes

Water is reabsorbed and urine output is decreased leading to decreased
serum sodium from hemodilution

s/s:
o water intoxication
o hyponatremia
o jugular vein distension
o increased BP
o sudden wt gain
o fluid in lungs
o concentrated urine

Tx:
o Fluid monitoring
o Restriction and demeclocycline (reduce reabsorption of water)

Teach pts and families about water restriction

Encourage child to wear medical alert bracelet
Normal range for specific gravity

1.005-1.030
Lupus

Chronic autoimmune dz consisting of remissions and exacerbations

s/s:
o butterfly rash on bridge of nose and cheeks (malar rash)
o fever
o joint inflammation/arthritis
o fatigue
o weight loss
o splenic enlargement
o vasculitis
o leukopenia
o anemia
o nephrotic syndrome
o Raynaud disorder
o Alopecia
o Photosensitivity
o Stomatitis
o Pleurisy
o seizures

Management:
o Encourage rest during lfare ups
o Help pts and caregivers identify signs of impending flare up
o Protect against cold weather and ultraviolet light

Medications based on severity:
o Mild to moderate:

Corticosteroids

Antimalarial agents (chloroquinine)

NSAIDs
o Severe:

High-dose corticosteroids and/or immunosuppressive drugs
ADHD (Attention Deficit Hyperactivity Disorder)

Neurobehavioral disorder characterized by inattentiveness with or w/out
hyperactivity

Pathophysiology is unclear, but appears to have genetic component

s/s:
o short attention span
o impulsivity
o difficulties with movement

Rule out sensory and organic causes of s/s before diagnosing

Diagnose with formal eval and psychological testing (include educators,
caregivers, and child in eval process)

ADHD Tx:
o Caregiver education, development of individualized education
plans, pharmacotherapy
o Meds:




Stimulants:

Ritalin

Adderall

Focalin
Controlled substances:

These meds are controlled substances

strict prescribing practices and restrictions
SE:

Weight loss

Appetite suppression

Tachycardia

Hypertension
Labs to monitor:

Serum liver function levels

Serum kidney function levels
Sensory integration dysfunction

Neurologic disorder where child cannot organize sensory input used in
daily living

Develops hyposensitivity or hypersensitivity to sensory input:
o How brain processes multiple sensory modality inputs including:

Proprioception (position awareness)

Vision


Auditory system (sound)

Tactile (touch)

Olfactory (smell)

Vestibular system (balance)

Interoception (internal sense- pain, hunger, emotions, etc)

Taste
Result:
o No or limited or overreaction to diff textures, sounds, sensations,
etc

Risks:
o Preterm infants
o Low birth weight
o Autism spectrum disorder

Therapy:
o OT and other therapies to ↑ ability to function, respond
appropriately, & control responses
Neurofibromatosis

Neurocutaneous genetic disorders of nervous system

Characterized by abnormalities of skin & CNS

Primarily affect growth & development of neural cells

Progressive & usually worsens over time

Unpredictable

Type 1:
o Most common type
o Tumors grow on nerves and produce abnormalities including skin
changes & bone deformities

Characteristics:

Café-au-lait spots (light brown macules, often present at
birth)

↑ size, #, and pigmentation

Usually spare face

Pigmented nevi (moles)

Axillary freckling

Slow growing cutaneous & subq or dermal neurofibromas
(lumps)

Complications:
o Headache
o Hydrocephalus
o Scoliosis
o Cardiac defects
o HTN
o Seizures
o Vision/hearing loss
o Neurocognitive deficits
o Learning disabilities
o ADD
o Fine and gross motor delays
o ASD
o Behavioral and psychosocial issues
o Speech abnormalities
o ↑ risk of neoplasms (abnorm growth of tissue)

Management:
o No cure
o Control symptoms
o Manage complications

Nursing management:
o Supportive care
o Early detection
o Support and education
Anxiety disorders

Worry, fear, and anxiety extend past normal adaptive coping mechanisms
and cause stress and significant impairment

s/s:
o Abdominal pain
o Palpitations
o Nausea
o Vomiting
o Dyspnea
o Aggression
o Defiance
o Dizziness

Categorized by causes and manifestations:
o Generalized Anxiety Disorder
o Separation Anxiety Disorder
o Panic Disorder
o Obsessive-compulsive disorder (OCD)

Anxiety disorders tx:
o Exposure-based cognitive behavioral therapy to treat separation
anxiety disorder and school refusal

Find out what’s causing school refusal and solve the
problem
o SSRIs often used for generalized anxiety disorders
o SSRIs may increase risk of suicide in pediatric population
Obsessive compulsive disorder

Repeated behaviors (checking curling iron unplugged, washing hands,
cleaning) done to ↓ anxiety from obsessions
Growth and Development 3 questions from module 1
Neuro 2 questions from module 1, especially seizures, increased ICP, and
meningitis page in Canvas
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