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125 Cards in this Set

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Iron Deficiency Anemia Patho & Etiology
patho:
➧need for Fe to produce Hgb is not met
➧↓O2 carrying capacity ⇒ hypoxia & ↓spO2

etiology:
➧↓supply: diet; inadequate at birth (premies) who have little materna; Hgb
➧impaired absorption: chronic diarrhea
➧blood loss: trauma; surgery; menstrual cycle
➧↑need: pregnancy; adolescence; premies
➧↓income: diet d/t selection/access
Iron Deficiency Anemia S/S & Dx
S/S:
➧muscle weakness
➧fatigue
➧pallor
➧CNS manifestations
➧cognitive deficiencies
➧infants may be overweight: called "milk babies" who are fat d/t whole milk intake which is a poor Fe source
➧edema: fluid loss into tissue d/t protein loss in circ
➧liner growth retardation
➧delayed sexual maturation

S/S d/t hypoxia:
➧↑HR, CO
➧murmur
➧cardiac failure during stress: esp b/c body is working at ↑level of stress already

Dx:
➧norm or RBC: microcytic (small), hypochromic (pale)
➧Hgb
➧Fe: serum Fe; total Fe-binding capacity
➧occult blood test
Iron Deficiency Anemia Tx
prevention:
➧maternal Fe stores: 4-6mon of age full term; 2-3mon premie
➧Fe rich formula, cereal
➧Fe for breastfed babies

ferrous sulfate

parent edu:
➧diet: meat, leafy greens, eggs, dried fruit, mollusks, poultry, beans, artichokes
➧Fe admin
➧↑fiber & fluid: Fe/constipation is myth
➧follow-up

Fe supplements:
➧give in divided doses b/t meals w/ citrus or fruit juice
➧store safely
➧liquid prep may stain teeth
➧tarry green/black stools = norm
Sickle Cell Disease Patho, Dx & Prognosis
patho:
➧autosomal recessive hereditary D/O of abnorm sickled Hgb that have impaired O2 carrying abilities
➧certain conditions cause sickling
➧obstruction: of vessels by sicked RBCs leads to ischemia
➧destruction: of RBCs leads to hypoxia

Dx:
➧newborn screening ⇒ Sx as toddler/school age
➧family Hx
➧CBC-anemia
➧Hgb electrophoresis
➧sickle turbidity test

prognosis:
➧risk if <5yo
➧death d/t overwhelming infection & neuro comps
➧crises tend to become less severe w/ age
Sickle Cell Crisis & Complications
crisis:
➧acute exacerbation of Sx in con't sickling process
➧vaso-occlusive crisis common
➧VERY PAINFUL

comps:
➧CVA, paralysis, death
➧retinopathy, blindness
➧avascular necrosis: shoulder, hip
➧hepatomegaly, gall stones
➧splenomegaly, splenic sequestration, autosplenectomy
➧hematuria, hyposthenuria (dilute)
➧abd pain
➧osteomyelitis pain
Sickle Cell Disease Tx
Tx:
➧goals: prevent sickling; Tx crisis; edu
➧Sx care

supportive:
➧primary needs
a) pain relief: abd pain, ↑fever, ↑HR, V/D over 3days; admin narcotics
b)adequate hydration & O2: ↑IVF
➧bed rest during crisis: to ↓O2 demands, enlarged spleen, joint pain; ↑risk THROMBI
➧lytes replacement for crisis: Tx metabolic acidosis
➧blood replacement
➧splenectomy

long term:
➧edu
➧genetic counsel
➧Folic acid
➧↓incidence of severe painful episodes & acute chest syndrome: hydroxyurea
➧prevent infection: eval fever; prophylactic ABX; immunications
➧stroke risk
Hemophilia Patho, S/S & Dx
patho:
➧bleeding d/t missing factor
➧no cure

classic hemophilia: factor VIII deficiency; X-link recessive

S/S:
➧spontaneous bleeding: upper airway, intracranial & GI tract lethal
➧hemathrosis: bleeding into joint

Dx: Hx, family, labs (Factor VIII assays)
Hemophilia Tx & When to Call MD
Tx:
➧factor replacement: recombinant factor is synthetic to ↓risk HIV/blood exposure; check HIV for kids who had multiple blood source
➧steroids
➧avoid NSAIDs & aspirin
➧regular exercise & diet
➧edu: factor admin, Sx internal hemorrhage, prevent bleed

call MD:
➧CNS: trauma to head, neck, back
➧face, throat, airway, GI bleeds
➧kidneys & GU
➧ilio psos muscle in trunk
➧hip, shoulders
➧large muscle compartments (thighs)
von Willebrand Disease Patho & S/S
patho:
➧hereditary autosomal dominant
➧deficiency of defect of von Willebrand factor protein (vWF)

S/S:
➧prolonged bleeding times: PLTs fail to adhere to walls of rupture vessels
➧bleeding can be mild, moderate, severe
➧common to bleed from mucous membranes: nose, gingival; easy bruising; excessive menstrual bleeding
von Willebrand Disease Tx
Tx:
➧demospressin (DDAVP): synthetic; vWF factor VIII secretion from storage in endothelial
➧vWF replacement (Humate-P)

nsg intrvs:
➧edu S/S bleeding & when to call ED
➧avoid unnecessary trauma
➧edu emergency care bleeding: epitaxis (nose)
➧delay circumcision for male infants
➧girls: edu management heavy flow; consider OCP
HIV/AIDS Patho & Transmission
patho: HIV attacks immune system by destroy T lymphs

transmission: intrauterine, intrapartum, postpartum

maternal risk factors:
➧↓risk: antiviral drugs; C-sec; no breastfeeding
➧↑risk: ↓maternal CD4; ↑viral load

other factors: blood transfusions; hemophilia; sexual contact; IV needles
HIV/AIDS Dx & Sx Classification
Dx:
➧ELISA: not helpful in kids <18mon d/t maternal passive immunity
➧HIV culture: takes weeks
➧polymerase chain reaction (PCR)
➧HIV neg: 2 DNA PCR tests 6 wks apart after 4mons old

HIV Pediatric Symptom Classification:
➧not Sx, mildly Sx, moderately Sx, severely Sx
➧PCP PNA significant predictor of outcome
HIV/AIDS Tx & Opportunist Infections
Tx:
➧AZT: med; poor taste; some compliance issues;
➧Bactrim: for 3-4mons old as prevention
➧immunizations: careful w/ Varicella & MMR b/c have live viruses

Opportunist Infections: PCP; CMV; Herpes simplex; Mycobacterium avium; Candidiasis
Systemic Lupus Erythematosus (SLE) Patho & Sx
patho:
➧chronic inflam disease of collagen & supporting tissue (organs)
➧autoimmune response to stress, extreme fatigue, meds
➧remissions & exacerbation
➧family Hx

Sx:
➧insidious onset
➧fever
➧arthritis
➧butterfly rash
➧skin disease: itchy rash; Raynaud phenomenon (pale extremities); cyanosis; alopecia
Systemic Lupus Erythematosus (SLE) Dx
4 or more = SLE+

➧butterfly rash
➧discoid rash
➧photosensitive rash
➧oral or nasopharygeal ulcers
➧joint inflam
➧seizures/psychosis: SEVERE
➧pleuritis or pericarditis
➧abnorm urinalysis
➧abnorm CBC
➧ANA+
➧anti-DNA+
Systemic Lupus Erythematosus (SLE) Goals & Tx
goals:
➧reverse autoimmune & inflam process
➧prevent exacerbations & comps: lupus nephritis (deposition of circ immune complexities in glomerular basement membrane w/ cell infiltrates); neuropsychiatric lupus (seizure, psychosis)

meds:
➧corticosteroids: worry long term use
➧antimalarials (Hydroxychloroquine): helps remove diarrhea & joint complaints
➧NSAIDs (Naproxen, Ibuprofen)
➧immunosups (Cyclophosphamide)

family support & edu:
➧limit stress
➧body image concerns
➧diet: ↓Na & protein for renal involvement
➧skin care: avoid sun
➧eye exams: esp w/ antimalarials
➧chronic illness (remissions/exacerbations)
Juvenile Rheumatoid/Idiopathic Arthritis Patho & S/S
patho:
➧peak ages 2-5yo & 9-12yo, usually females
➧3 major courses: systemic, pauciarticular, polyarticular
➧chronic inflam of synovium w/ joint effusion & erosion
➧fibrosis of articular cartilage

S/S:
➧stiffness, swelling; esp AM
➧↓ROM
➧tender to touch
➧no erythema
➧soft tissue contractures
Juvenile Rheumatoid/Idiopathic Arthritis Goals & Tx
goals:
➧suppress inflam process
➧preserve joint function
➧prevent phys deforms (contractures)
➧relieve Sx (pain)

meds:
➧NSAIDs: analgesic & antinflam
➧Cox-2 inhibitors (Celebrex has FDA warning)
➧disease-modifying antirheumatics (DMARDs)
➧chemotherapy drugs sometimes
➧PT, exercise
➧joint replacement: not during childhood
Idiopathic Thrombocytopenic Purpura (ITP) Patho & Sx
patho:
➧acquired hemorrhagic d/o when body attacks own PLTs
➧probs autoimmune; acute, self-limiting
➧2-10yo, peak 2-5yo

Sx:
➧easy bruising w/ petechiae
➧bleeding mucous membranes
➧internal hemorrhage
➧hematomas
➧probs chronic leg ulcers
Idiopathic Thrombocytopenic Purpura (ITP) Dx & Tx
Dx: PLT<20k-30k; anti-PLT antibodies

Tx:
➧supportive care: rest; teaching & support
➧corticosteroids: PLT<50k
➧IVGG (gamma globulin): PLT<20k & minor purpura
➧splenectomy: for bleeding uncontrolled by corticosteroids & IVGG
Henoch-Schönlein Purpura (HSP) Patho & S/S
patho:
➧abnorm immunogenic response to antigen
➧allergic vasulitis, purpura & anaphylactoid purpura

S/S:
➧previously healthy
➧develops acutely abrupt or gradual
➧skin rash
➧arthritis
➧abd pain
Henoch-Schönlein Purpura (HSP) Tx & Prognosis
Tx:
➧analgesics: NSAIDs
➧corticosteroids: severe edema, arthralgia & colicky abd pain

monitor: urine, stool; I&O; dietary intake (Na); BP

prognosis:
➧most recover w/o need for hospitalization: single acute event clears w/i 1mon
➧periodic recurrences over 2-3yrs, then remission
➧death: severe GI, renal or CNS involvement
➧HSP nephritis ⇒ chronic renal insufficiency
Cancer Dx
complete Hx & phys exam

lab test

tissue biopsy

imaging: shows body functions like sugar metabolism (make sure not to give Dextrose); Iodine shows blastomas

lumbar puncture: bone marrow; L4-L5 d/t lower spinal cord in kids

bone marrow biopsy or aspirate: for leukemias or mets in bone
Pediatric Oncological Emergencies
tumor lysis syndrome (ALL or Burkitt):
➧hyperuricemia, hypocalemia, hyperphosphatemia, hyperkalemia
➧b/c rapidly growing so cells killed quickly causing hypo/hyper risks in chemo
➧weird lab draws
➧uric acid can destroy kidneys

hyperleukocytosis:
➧leuk>100k
➧managed w/ hydration, transfusion in emergency

Superior Vena Cava syndrome:
➧blood not draining so it builds up
➧Sx: fainting, dizzy, swollen eyes
➧hydration management

Spinal cord compression: pain

Disseminated intravascular coagulation:
➧↓PLT (<20k)
➧hemorrhaging & thrombi

Fever & Neutropenia
Fever & Neutropenia in Cancer
temp depends on hospital policy: >38.0C (100.4F)

neutropenia:
➧ANC<500: measurement of infection-fighting cells
➧supportive therapy & prevention

prevention: environment; hygiene

management:
➧cultures: must have w/i 30min
➧ABX
Chemotherapy
indiscriminately kills rapidly dividing cells

malignant: unable to repair themselves

norm: more successful; S/E from norm rapidly dividing cells
Radiation
use of high energy particles to destroy CA

types:
➧localized: for solid tumors, known location
➧total body: prep for HSCT
➧fractionated: piece of dose QD
➧hyperfractionated: 2-3doses/day

bradytherapy

don't scrub tattoo marks off

sometimes conscious sedation for fussy, young kids
Surgery for Cancer
Dx biopsy
Complete resections
Debulking
Debridement of necrotic tissue
Palliation of Sx
Hemapoietic Stem Cell Transplant (HSCT)
allogenic v autologous: match sibling preferred

sources:
➧marrow
➧peripheral blood stem cells
➧cord blood: more forgiving about matching; can't get more; only works w/ little kids

may provide cure but more of a stem cell rescue; can relapse

new immune system that is donated attacks host (instead of host attacks foreign donation):
➧skin: GHB; rash on palms
➧liver: weird function tests
➧GI: severe diarrhea of 3-5L
Side Effects & Nsg Intrvs of Cancer Tx
Pulm: pneumonitis; edema; fibrosis

GI: N/V/D; constipation; anorexia; mucositis; pancreatitis; lyte inbals
➧nsg: antiemetics; measure amt emesis to assess dehydration; antispasmodics & kaolin pectin preps; oral hygiene; intake as tol w/ soft, small, frequent meals; monitor weight loss

GU: hemorrhagic cystitis; renal tubular damage
➧nsg: from concomitant use of cyclophophamide; encourage liberal fluids intake & voiding; evaluate for hematuria

Repro: fertility/sterility; hormonal imbals
➧nsg: condoms to prevent ejaculating chemo; sperm/egg storage

Neuro: pain; learning difficulties; fatigue

Skin: alopecia (7-14d after, bald in 1mon, regrowth 1mon after last XRT); breakdown; pruritus; sensitivity to light; XRT recall
➧nsg: wigs; scalp hygiene; head covers; avoid sun; lubricant for dryness

Skeletal: growth issues; osteoporosis

Bone marrow: mylesuppression; anemia; neutropenia; thrombocytopenia
➧nsg: observe temp & anemia; ABX; no rectal stuff; bleeding precautions; Nadir for severe ANC

2ndary tumors

psychosocial: loss of normalcy; interruptions of milestones; relationship issues
Central Lines
Hickman/Broviac:
➧1+ lumen
➧↓risk bact migration
➧easy for self-admin
➧nonsurg pull-from-site removal
➧heparin flush QD
➧must be clamped
➧restricted heavy activity
➧protrudes outside body

Port-a-cath:
➧totally implantable metal/plastic device w/ self-sealing injection port w/ side/top access
➧placement in large blood vessel, completely under skin
➧↓risk infection
➧heparin Qmon & after each infusion
➧no activity limits
➧cath may dislodge from port
➧surg removal

PICC:
➧single or double lumen
➧inserted into antecubital fossa and passed thru basilic or cephalic vein into SVC
➧fast surg appli, some resistance to removal
➧not for rapid fluid replacement b/c small lumen
➧5-10mL syringe for flushes
Leukemia Patho
Acute Lymphoblastic Leukemia: 2-3yr Tx, very curable w/ oral therapy maintenance
➧may develop to AML d/t Tx

Acute Myelogenous Leukemia:
➧9mon Tx but more aggressive form/less survival
➧transplant first if match sibling, chemo first if not

malignancy of blood-forming cells:
➧uncontrolled proliferation of blasts
➧↓production of norm cells
➧accumulation of blasts in body organs & tissue
Leukemia S/S, Dx &Tx
S/S (vague): fatigue, pallor, anorexia; bruising, bleeding; fever, infection; abd pain; HA, V, visual disturbances; tachycardia

Dx:
➧CBC w/ DIFF, lytes
➧bone marrow aspiration/biopsy
➧LP
➧chest Xray: may reveal infiltration of lungs

Tx: chemo; HSCT
Central Nervous System Tumors
S/S: morning HA; mental status changes, restlessness, anxiety, confusion; ↑ICP; N/V; nerve palsies; ataxia, aphasia; hemiparesis; visual probs

Dx: scans, blood work, biopsy

Tx:
➧surgery
➧XRT if >3yo
➧chemo: may give to <3yo d/t brain damage assoc w/ XRT
➧immune/biotherapy
➧HSCT
➧NGT to keep gut going/poor appetite
Neuroblastoma
patho: extracranial tumor; from neural crest cells; nontender firm abd mass that crosses midline

Dx: scans, labs, biopsy, MIBG

S/S:
➧abd mass
➧fevers
➧irritability
➧excessive sweating
➧anemia/fatigue
➧anorexia/weight loss
➧Hutchinson syndrome: mets to skin
➧Blueberry muffin spots: mets in bone marrow ⇒ limp in walk
➧Raccoon eyes: swelling/bruising (looks like abuse)

Tx:
➧chemo if stage III/IV
➧surgical resection
➧HSCT
➧XRT
➧biological/antibody therapy
➧MIBG: for relapse; Tx w/ higher XRT dose
Osteosarcoma
patho:
➧malignant tumor of bone derived from mesenchyme (connective tissue): distal femur, proximal tibia, proximal hummerus
➧assoc w/ teen growth spurt

S/S:
➧pain/tenderness w/ ↑activity
➧weight bearing may show limp/↓ROM
➧local edema/redness/pulsation/bruit
➧imaging looks like new bone growth
➧may present w/ multiple fractures from minimal impact where tumor grows

Tx:
➧chemo
➧surgery (sometimes multiple): amputation; limb salvage (removed bone and up in prothesis, can be expanded); rotationplasty (amputate above log, use ankle as functioning knee joint)
Wilms Tumor
kidney tumor

S/S:
➧nontender, firm flank mass not crossing midline
➧aSx or pain, V, gross hematuria, anemia or HTN

surgery:
➧unilateral: complete nephrectomy
➧bilateral: complete nephrectomy of more involved kidney, partial of other

chemo: all stages

XRT: stages III-IV

don't palpate abd in order not to rupture capsule & spread tumor

high survival
Important Pediatric Nursing Considerations
professional boundaries

developmental considerations

care of family w/ chronically ill child: school; siblings; parents need to utilize their role; parent meeting own needs; coping/strress

long term effects: phys; psych/social; financial
Forms of Child Maltreatment
physical abuse

Münchausen syndrome by proxy, Factitious disorder by proxy, Pediatric condition falsification: when caretaker uses harm of child to get medical attn; very rare by highly emphasized in media

Neglect: phys; emotional; medical

Sexual Abuse

Emotional/psychological abuse
Etiology of Child Maltreatment
parental factors:
➧Hx severe punishment as child (intergenerational)
➧poor impulse control, free expression of violence, Hx cruelty to animals, domestic/intimate partner violence
➧low self-esteem
➧poor social-emotional support
➧substance abuse

child factors:
➧temperament mismatch
➧illness, disability, developmental delay
➧unwanted or prob pregnancy: when fetus expected to die so no maternal-fetal bondng, but baby survives instead
➧failure to bond w/ kid or other partner

environmental: chronic stress; poverty, poor housing, unemployment; divorce; frequent relocation
Making the Diagnosis of Child Maltreatment
difficult to Dx

S/S may not be obvious

kids often inly witnesses: will have harder time disclosing/recalling, esp if they are the ones harmed & dissociate

delayed disclosure

social resistance to report: fear of harm of others

other causes for S/S must be considered but may delay Dx

disabled kids
Child Physical Abuse
deliberate infliction of phys injury on a child; def differs by state

includes: bruises, burns, fractures, Shaken Baby Syndrome

S/S:
➧injuries: repetitive pattern of injury; injuries not consistent w/ Hx
➧child behavior: uncomfortableness of speaking to health care provider (more so than devel age)
➧unusual behavior by parents
Differential Diagnosis of Child Maltreatment
Mongolian spot: over butt

Hemangioma: growth of vasculature under tissue; will not change in color but may shrink

Café au Lait spots: looks like big freckle

Prominent veins

Cultural/folk practices

Skin disease

ink, paint, dye

Bleeding d/o

Infection/vasculities
Bruises & Others in Child Maltreatment
numerous bruises in various stages

bruising over soft tissue: buttocks & thighs not typical accident areas

loop marks: whipping w/ cord

injuries not consistent w/ Hx
Burns in Child Maltreatment
child development inconsistent w/ Hx given

burn to soles of feet, palms of hands, back or buttocks

patterned burns or caused by "holding down"

absence of splash marks

scalds
Fractures in Child Maltreatment
suspicious: multiple frx, in various stages of healing

Dx: xrays; bone scans

non-abuse causes of frx:
➧osteogenisis imperfecta (abnorm collagen)
➧accidental injury
➧birth trauma
➧osteoporosis 2ndary to neuromus d/o; osteomylitis
➧calcified cephalhematoma
➧metabolic bone disease
➧copper deficiency
Shaken Baby Syndrome/Abusive Head Trauma
patho: head injury from shaking
➧brain rebounds against skull ⇒ tissue sheared ⇒ blood vessels ruptured

S/S:
➧CNS injury from: changes in behavior, irritability, lethargy, LOC, vomiting, seizures
➧retinal hemorrhages
➧bruises
➧soft tissues swelling
➧skull &/or other bone frx
➧abd injuries: from squeezing

Dx:
➧radiology (frx, soft tissue)
➧ortho exam (retina)
➧rule out: SIDS, seizure, infectious cause for neuro changes
Neglect in Child Maltreatment
physical: deprivation of necessities such as food, clothing, shelter, supervision, medical care or edu

emotional: failure to meet child's need for affection, attention & emotional nurturance

Dx (difficult): maybe growth delays; feeding d/o; enuresis; sleep d/o; social/emotional probs; medical probs

Failure to thrive: must rule out organic cause
➧lack of financial/support resources, knowledge
Child Sexual Abuse
contact or interaction b/t child & adult when child is used for sexual stimulation or exposed to sexually explicit material

often norm phys exam w/ delayed disclosure about someone known to family/child

S/S:
➧genital: bruises, bleeding, lacerations, irritation; torn, stained or bloody underclothes; pain or urination; STIs; recurrent UTIs
➧non-genital: age-inaprop behavior; changes in behavior; sudden onset of phobias; substance abuse; SI; pregnancy

Dx: Hx; disclosure; phys exam

non-abuse causes:
➧genital injury
➧precocious puberty
➧Lichen sclerosis
➧eczema
➧vulval hematoma
➧vaginal foreign body
➧Pin worms
Nurse's Role in Child Maltreatment
obtain: Hx from caretaker, descri of injury from child

doc injuries objectively: location, size, shape, color, any distinguishing characteristics, degree of pain

discuss concerns w/ MD or social worker

report as indicated by hospital/state:
➧health ppl are all mandatory reporters
➧call local child protection agency/Dept of Social Services

to help kids talk:
➧ASK
➧provide private place to talk
➧do NOT promise not to tell b/c you're mandated to tell
➧do NOT act surprised or shocked
➧use their words
➧tell them the abuse is NOT their fault and they were right to disclose
Endocrine System Glands & Regulations
glands: pituitary; thyroid; parathyroids; adrenals; pancreas; ovaries/testes

controls:
➧*energy production: disrupt peds function the most b/c they are in process of growing
➧*growth: affected by adrenals
➧*F&E balance
➧response to stress
➧reproductive: must be preserved for future
Hypopituitarism/Growth Hormone Deficiency
patho: pituitary not releasing GH for various reasons (like tumor)

S/S:
➧short
➧may appear obese d/t ht:wt ratio
➧appear well-nourished
➧permanent teeth come late
➧norm IQ
➧delayed sexual maturation

Dx: family/child Hx; phys exam (need good linear measurements); xray (look at bones & plate closure); endocrine studies

Tx:
➧remove tumor if that is prob
➧early Tx is necessary for good linear growth

Nsg:
➧self-esteem: males worry about being short
➧edu:
a) injections: not painful; indep; same time QD to simulate norm release
b) ↑growth in 1st yr; slower pace after
c) Human Growth Foundation

EMERGENCY: Slipped Femoral Capitol Epiphysis
Cushing's Syndrome Patho, S/S & Dx
patho: hypercortisolism of adrenal tumors or excess ACTH

S/S:
➧growth failure & muscle wasting: protein catabolism for fuel; see fat gain but protein loss
➧delayed skeletal maturation
➧excessive hair
➧obesity: rapid wt gain w/ arrest in linear growth
➧HTN
➧CHO intol
➧moon face
➧abd straie

Dx:
➧exogenous supply: Dexamethazone suppress test in which will see NO ↓production ACTH
➧24 urine for cortisol: adrenals & cortisol affect lytes
➧electrolytes
➧CT or MRI to rule out tumor
Cushing's Syndrome Tx
d/c steroid therapy by tapering: b/c body needs time to start producing its own cortisol or will ⇒ comps

surgery to remove adrenal glands or tumors: may need replacement therapy

hormones therapy

edu: give steroids in AM & alternate days to ↓Cushing development as this mimics the body
Juvenile Hypothyroidism
causes: deficiency in TH secretion; congenital; acquired (XRT, thyroiditis, thyroidectomy)

S/S:
➧may appear after 1-2yrs
➧decelerated growth
➧↑weight
➧dry skin
➧sparse hair
➧constipation
➧↓mental if not Tx

Dx (thyroid function tests): ↑TSH; ↓T3 & T4

Tx:
➧early to help cognitive development
➧TH replacement
➧monitor G&D: different doses as they grow
➧edu on S/S of both hypo/hyperthyroidism (to need more or less Tx)
Hyperthyroidism/Grave's Disease Patho & S/S
patho:
➧accelerated metabolism of all body tissues d/t ↑TH
➧peak during teens, 5x often in females

S/S:
➧onset over 6-12mon
➧r/t EXCESSIVE MOTION: emotional liability; weight; accelerated linear growth; irritability; hyperactivity; short attn span; insomnia; skin warm, flushed
➧V/D
➧CV: murmurs, widened pulse pressure, cardiomegalyn
➧heat intol
➧fine hair growth
➧goiter: enlargement in throat
➧exothalmus: eyes bug out
Hyperthyroidism/Grave's Disease Dx & Tx
Dx: ↓TSH; ↑T3 & T3

Tx:
➧antithyroid meds (PTU & Tapazole): give in AM; monitor leukopenia esp w/ fever/sore throat
➧subtotal thyroidectomy: need TH replacement

Nsg:
➧assist in making Dx
➧Tx Sx until meds take effect: quiet, calm enviro; address sleep issues; ↑food if w/o meds but ↓food when w/ meds b/c PTU helps slow metabolism ⇒ wt if not
➧body image: Sx will end w/ Tx
➧eye care: Lacrilube or patch at night to protect from dryness & abrasions
➧teen issues/compliance
Diabetes Mellitus I Patho & Characteristics
patho:
➧↓gluc transport from blood to cells ⇒ ↑bloodstream concentration
➧protein wasted: broken down & converted to gluc by liver ⇒ further ↑blood gluc
➧fats break down into fatty acids for energy ⇒ glycerol (in fat cells) converted to ketone

characteristics:
➧<20yo abrupt onset, males slightly more
➧underweight
➧little pancreatic content or insulin
➧diet not effective; always need insulin
➧prone to ketoacidosis
Diabetes Mellitus I S/S, Potential Complications & Dx
classic S/S: polydipsia; polyuria; polyphagia; fatigue

other S/S: ↓weight; abd pain; enuresis; irritability; ketoacidosis

complications:
➧hypoglycemia
➧↑weight: depends on if pt feels that wt is not/helpful
➧ketosis
➧DKA: gluc>330; pH<7.30; Bicarb<15; Kussmaul resp
➧retinopathy; neuropathy
➧nephropathy
➧heart disease
➧PVD
➧impotence

Dx:
➧fasting blood gluc>126
➧random blood sugar>200 w/ classic Sx
➧gluc>200 2hrs after oral gluc tol test
Diabetes Mellitus I Tx
insulin admin:
➧SQ injections/pens: BID or multiple
➧insulin infusion pump: delivers fixed amts insulin con't to imitate islet cells; must be programed; needle sits in SQ tissue of abd or thigh & changed Q48-72hr

other Tx:
➧monitor blood gluc QID+: for good control & kids need more
➧balanced diet: huge issue w/ toddlers/teens; ETOH consumption

edu:
➧gluc testing
➧how to admin insulin
➧S/S hypogly/hypergly
Handling Chemotherapeutic Agents
use great care & strict aseptic technique in handling chemos to prevent phys contact w/ them

prepare drugs in ventilated room or biological safety cabinet (to ↓inhalation)

wear disposible gloves & protective clothing; discard in special container after each use

use sterile gauze pad when priming IV tubes, connecting & disconnecting tubes, inserting syringes into vials, breaking glass ampules or performing any other procedure in which antineoplastic drugs may be inadvertently discharged

dispose of all contaminated needles, syringes, IV tubes & other contaminated equipment in a leakproof & puncture-resistent container; do not recap or break needles
Monitoring Chemotherapeutic Agents
look for hypersensitivity

shown w/: urticaria, angioedema, flushing, rashes, difficulty breathing, hypotension, N/V

prevent by taking careful Hx of allergies & family Hx

if rxn suspected:
a) d/c drug
b) flush IV & maintain w/ saline
c) observe VS & further rxns
Musculoskeletal Assessment
health Hx:
➧delayed walking or motor development
➧pain, stiffness or both
➧↓ROM

observ: posture, symmetry of extremities & ROM, spots on skin, gait

palpation:
➧test muscle strength/reflexes
➧spine alignment: for scoliosis
➧pain: tender areas
➧affect on neurovascular: PAIN, PALLOR, PULSELESSNESS, PARESTHESIA, PARALYSIS

labs: CBC; Alkaline phosphate & Creatinine kinase (injury to bone/muscle); C-reactive protein; Rheumatoid factor; biopsies; imaging (CT, MRI, U/S, nuclear med studies/bone scans)
General Tx for Musculoskeletal Problems
Immobilization

Heat/Cold application

Casts

Traction

surgery: external or internal fixation

mobilization devices: orthotics; prothetics
Issues with Immobilization
resp:
➧↓chest & lung expansion
➧↓resp effort
➧effects of gravity

CV:
➧VD & impaired venous return
➧redistribution of body fluids

musculoskeletal:
➧↓bone stress & muscle tension: be aware of positioning
➧bone demineralization: bones fragile & losing Ca from not being active

metabolic:
➧↓metabolic rate & O2 consumption
➧↓protein from ↓muscle mass

skin: con't pressure

elimination:
➧general muscle weakness & atrophy
➧inactivity slows peristalsis: issues w/ constipation ⇒ automatically put on stool softeners
➧urinary stasis: sometimes b/c kids have difficulty w/ bedpan; may get UTI; give fluids

psychosocial
Heat/Cold Application
heat:
➧chronic & subacute
➧warms muscles, VD, relieve inflam & pain

cold:
➧prevent swelling, edema & O2 needs
➧VC; acute injuries

may prefer either for whatever

best if intervals of application & removal for skin protection
Nursing Considerations of Casts
drying:
➧don't have anything pressing against cast while drying or will indent
➧notify MD

skin assessment/care: check moisture, cap refill, 5Ps

safety:
➧be careful of GU area (like w/ spica)
➧kids will shove stuff (give Benadryl)

cast removal: kids have anxiety b/c of noise so reassure that tool won't harm their bodies

5Ps of Ischemia
Nursing Considerations of Traction
know purpose

assess child & apparatus; document

maintain traction:
➧check device & bed position
➧DO NOT release traction unless emergency w/ MD nearby or MD order

maintain alignment:
➧emphasis on shoulder, hip, leg
➧check joints

care for skin traction: assess straps, tape & boot; replace if ordered or absolutely necessary

care for skeletal traction: check & clean pins/sites

discomfort w/ traction initiation: analgesics & muscle relaxants: d/t muscle spasms from pulling

complications:
➧neurovascular checks (5Ps): call MD for emergency if there are changes
➧encourage deep breathing
➧assess skin integrity
Fractures Patho, Types & S/S
patho:
➧resistance of bone against stress yields to stress force
➧bones in kids more easily injured than soft tissue

types:
➧spiral: slanting & circular twisting around bone shaft
➧bend: bone bends to breaking point
➧buckle: compression causes a raised area
➧greenstick: incomplete, broken bone as a result of bending
➧simple: break of bone
➧open: breaks into skin
➧compound: fragments, more problematic

S/S: swelling; pain, tenderness; limited ROM; open wound or ecchymosis; limp or refusal to use limb
Fractures Dx, Tx & Complications
Dx:
➧Hx, Exam
➧radiographic: Xray, MRI, CT; may film unaffected limb to compare
➧blood: ↓Hgb & HCT esp d/t blood loss in femur break; ↑AST & LDH

Tx:
➧emergency management: assess 5Ps; immobilize; call 911
➧assess: circ, neuro impairment; skin integrity (wounds)
➧admin analgesics
➧prevent complications
➧edu

complications:
➧circulatory impairment: sometimes by device
➧nerve compression: during injury, realignment or by device; classified by nerve; Tx w/ correcting alignment or surgery
➧compartment syndrome: pressure on muscles/nerves comprises circ there; cause include tight dsgs, skin traction, bleeding, trauma, burns, surgery; pain exceeds injury & only relieved w/ opiods; Tx is relief of pressure
➧epiphyseal damage: damage to epiphyseal can result in unequal lengths; Tx suregry
➧nonunion/malunion: must cut fresh surface to fractural bone & see if bone will grow again
➧infection: osteomyelitis
➧kidney stones: usually when urinary stasis from imbol or hypercalce
➧pulm embolism: fat or blood
Developmental Dysplasia of the Hip Patho, S/S & Dx
includes:
➧Acetabular dysplasia (preluxation): mildest; fermoral head remains in acetabulum
➧Subluxation: most common; femoral head remains in contact w/ acetabulum but head of femur partially displaced
➧Dislocation: most severe; femoral head loses contact w/ acetabulum & displaced

S/S:
➧unequal skin folds: gluteal & thigh
➧limited abduction
➧Allis sign: unequal knee height
➧Ortolani click: heard on exit or entry of femur from acetabulum
➧Barlow test: femoral head slips out w/ pressure from the front & slips back when pressure released

Dx:
➧Ortolani & Barlow for 2-3mon
➧U/S
➧Xray if >4mon: wait for ossification of femoral head
Developmental Dysplasia of the Hip Tx & Nsg Considerations
Tx
➧improved success if Tx initiated before 2mon
➧kids < 6mon: Pavlik harness (abduction device)
➧6-18mon: traction; OR for manual reduction & hip spica cast after for 2-4mon
➧>18mon: reduction w/ cast

considerations:
➧promote norm G+D
➧maintain phys mobility
➧skin/cast care
➧proper use of reducing device
➧sleep
Clubfoot
patho: unknown; may occur w/ other abnorms (DDH, spina bifida)

S/S: complex deformity of foot
➧forefoot adduction
➧inversion of heel
➧plantar flexion

Dx: phys exam; Xray type/severity

goals: strengthen foot; maintain; observe for recurrence

Tx mild or positonal:
➧serial casting, exercises or wait: want to loosen inner side & tighten outer side
➧Denis Browne splints

Tx teratological & congenital: surgery

nsg considerations: cast care; surgical care; neurovascular checks
Legg Calvé-Perthes Patho & S/S
patho:
➧unknown
➧aseptic necrosis of femoral head; self-limiting d/o; often unilateral
➧skeletal age < chronological age
➧disturbance of circ to femoral capital epiphysis ⇒ ischemic necrosis

S/S:
➧insidious onset: mon-yrs
➧intermittent limp
➧hip soreness, ache or stiffness: pain in hip, thigh, knee
Legg Calvé-Perthes Dx & Tx
Dx: radiographic exam

Tx:
➧eliminate hip irritability
➧restore & maintain adequate hip ROM
➧prevent capital femoral epiphyseal collapse, extrusion or subluxation
➧ensure well-rounded femoral head at time of healing
➧nonsurg: abduction casts, leg casts, leather harness sling; abduction-ambulation braces/casts after bed rest & traction
➧surg: reconstruction & containment procedures

better prognosis for kids <5yo b/c have more cartilage; risk for degenerative arthritis in kids >9yo
Slipped Femoral Capitol Epiphysis
patho:
➧displacement of proximal femoral epiphysis (posterior-inferior direction); slides up or down
➧around time of teen growth spurts
➧also in obese kids & hypopituirary/↓GH

S/S:
➧pain in hip referred to groin, knee or thigh
➧consistent limp
➧restriction of internal rotation on adduction & loss of abduction w/ external rotation

Dx: phys exam; Xrays confirming displacement; EMERGENCY requiring early Dx

surg nsg:
➧no wheel chair: don't want downward pressure of perpendicular position & shoving of femoral head; crutches instead
➧pre-suregry bed rest/traction
➧hardware to correct deformity

post-suregry nsg: non-weight bearing until painless ROM; serial xrays of new bone growth
Scoliosis Patho, S/S & Dx
patho:
➧involves: lateral curvature of spine & spinal rotation causing rib asymm
➧may be from: leg length diff; hip/knee contractures; pain; neuromusc d/o; congenital

S/S:
➧often aSx
➧uneven hips
➧1 scapula more prominent
➧may have: pain; sacral dimpling; hairy patches; abnorm reflexes; incontinence; left thoracic curve (but check if d/t spine issues 1st)
➧can interfere w/ resp & CV function

Dx:
➧phys exam: repeat screens or parental obsrv
➧xray
Scoliosis Tx & Nsg Considerations
Tx:
➧depends on degree of curvature, location, type & age
a) <20degrees: obsrv q3-6mon, exercise
b) 20-40degrees: bracing
c) >40degrees: spinal fusion/surgery

considerations:
➧brace: 16-23hrs/day; off for shower, bath & swim
➧phys: safety/enviro hazards; need for exercise; shirt under brace to protect skin
➧psych: self-concept issues

post-op:
➧ICU admin
➧ROM & log roll q2hr
➧NPO/NGT
➧pain control
➧d/c instructions: may not be allowed to lift certain wt over certain amt time

prevent comps:
➧close monitoring of neurological deficits
➧monitor for breathing difficulties
➧skin integrity: d/t surgical incision
➧elimination: foley or bedpan
Infantile Spinal Muscular Atrophy (SMA)/Werdnig-Hoffmann Disease
patho:
➧progressive weakness & wasting of skeletal muscles: degeneration of anterior horn cells of spinal cord & motor nuclei of brain stem
➧autosomal recessive terminal disease
➧onset 6mon: regression of motor devel
➧death 2yo (resp failure)

Dx: genetic test; muscle EMG & biopsy

Tx: palliative & prevent complications
➧resp probs: G tubes to prevent aspiration PNA; ventilator & trach
➧cardiac issues
Duchenne Muscular Dystrophy (DMD)
patho:
➧most common & severe musc dystrophy
➧X-linked recessive
➧onset 3-5yo

S/S:
➧progressive muscle weakness, wasting, contractures
➧calf muscle hypertrophy common
➧Gower's sign: loss of independent ambulation by 9-12yo; to stand, kneels ⇒ pushes torso upright ⇒ walks hands up legs
➧slowly progressive, generalized weakness during teens until death from resp or cardiac failure

Dx: S/S; genetic testing; EMG

Tx:
➧no effective Tx
➧palliative care
➧corticosteroids: MIGHT improve musc strength, performance & pulm function
Pain Management Options
pharmacologic:
➧oral
➧IV
➧PCA pumps: may be on med baseline & Tx more when kids want ⇒ BAD if parents give b/c give too much
➧EMLA: topical numbing lotion
➧epidural

nonpharm:
➧parent involvement
➧cognitive behavioral interventions
➧biophysical interventions

bring BOTH options
Croup Syndromes
infection & inflamm in larynx region

"barky" "brassy" cough sounds worse than actually is

described according to primary anatomic area affected

pts can last sickness for awhile and suddenly CRASH (usually after fatigue)
Acute Spasmodic Laryngitis
etiology:
➧3mon-3yo
➧viral w/ allergen component repetitive w/ flares
➧sudden onset at PM when worse

S/S: URI; Croupy cough; stridor (inhale, high pitch); hoarseness; dyspnea; restlessness; awakes w/ Sx/none in AM; tends to recur

Tx: humidity (from hot shower, freezer's dry/cold air, outside)
Acute Laryngotracheobronchitis (Acute LTB) Etiology & S/S
etiology:
➧viral
➧>5yo
➧follows URI
➧low grade fever if at all

S/S:
➧mucous lining swells, airway narrows ⇒ struggles to breathe
➧cough
➧hoarseness
➧stridor
➧retractions
➧resp dist
Acute Laryngotracheobronchitis (Acute LTB) Tx/Nsg Care
maintain airway/adequate gas exchange: bag, mask & O2 at bedside

close observation & assessment: esp w/ meds

cool mist, cool temp therapy

NPO until RR improved & - stridor; encourage drinking

comfort, avoid agitation

reassure parent about sound/illness

meds:
➧Racemic epi aerosol: thru airslides method; worry for ↑HR & tremors
➧corticosteroids: inflam
➧heliox (He & O): for SEVERE
Acute Epiglottitis Etiology & S/S
etiology:
➧very serious obstructive, inflam process
➧2-5yo
➧bacteria, but now more viral & older kids d/t immunizations

S/S:
➧abrupt onset
➧fever, lethargy, dyspnea
➧agitated*, restless
➧drool*, can't swallow
➧no cough**
➧red throat, inflamm
➧tripod position (trying to get air in)*
Acute Epiglottitis Dx & Tx/Nsg Care
Dx:
➧see practitioner for Xray Dx before checking throat when suspected to prevent occluding airway
➧Xray: swelling & airway at epiglottis

Tx:
➧OR for artificial airway
➧antibiotics
➧corticosteroids
➧comfort
➧teaching: vaccinate at 2mon
Cystic Fibrosis Patho & Dx
patho:
➧Autosomal recessive trait
➧Exocrine gland dysfunction w/ multisystem involvement
➧↓ability of the epithelial cells in the airways & pancreas to transport Cl

Dx:
➧Sweat Chloride test
➧Newborn Screening
➧Genetic testing
➧Abnorm nasal potential difference
measurement
Cystic Fibrosis Manifestations
Resp:
➧Thick bronchial mucus ⇒ bacterial colonization: have trouble moving mucous
➧↓O2-CO2 exchange: chronic hypoxia
➧Chronic cough: airways constantly inflam or very productive when moving
➧Dyspnea
➧Hyperaeration (barrel chest)
➧Clubbing
➧Sinusitis, nasal polyps

GI:
➧Steatorrhea (fatty), azotorrhea (nitrogenous waste)
➧Intestinal obstruction: meconium ileus, distal intestinal obstruction syndrome: N/V, pain (complete or partial)
➧Failure to thrive: burn a lot of calories
➧Rectal prolapse
➧CF diabetes
➧Liver involvement: bile duct issues, portal HTN

Skin:
➧Salty taste d/t ↑loss of Na & Cl: electrolyte replacement necessary if will sweat a lot
➧↑risk for hyponatremic and
hypochloremic alkalosis
➧Edema d/t hypoalbuminemia: fluid leaks out of vessels ⇒ edema ⇒ skin breakdown

Reproductive:
➧Females: Delayed puberty; ↓fertility; ↑incidence of premature labor, LBW
➧Males: sterile
Cystic Fibrosis Therapeutic Management
Goals: Prevent/minimize pulm complications (bact, viral infections, pneumothorax); Ensure adequate nutrition for growth (calorie-dense); Encourage physical activity; Promote reasonable quality of life

Respiratory:
➧Pulm Hygiene: CPT** (loosens mucous), Vest, Flutter valve, exercise
➧Antibiotics
➧Nebulized/inhaled meds: Pulmozyme, TOBI, bronchodilators, anti-inflam
➧Cepacia precautions: a bacteria sometimes in CF pts' lungs
➧Lung transplant

GI:
➧pancreatic enzymes w/ meals, snacks, enteral feedings
➧↑protein, ↑calorie diet, salt supplementation
➧fat soluble vitamins A, D,
E, K w/ water-miscible forms
➧Monitor for & Tx intestinal obstruction
Pancreatic Enzyme Administration for Cystic Fibrosis Patients
➧1-5caps per meal, fewer for snacks

➧caps can be taken whole, split, or sprinkled over small amt food at start of meal

➧amt enzymes depends on growth & amt of stools, amt of fat in food

➧should be taken w/i 30min of eating
Types of Dehydration (Sodium)
1. isotonic (isonatremic): water & electro deficits in balance; most common; easiest to manage

2. hypotonic: electrolyte deficit > water deficit

3. hypertonic: water deficit > electrolyte deficit
➧hardest to manage
➧shock presents as neuro changes b/c fluid from inside cells to out maintain HR
Compensatory Mechanisms for Dehydration
interstitial fluid movement:
➧responds to hemoconcentration & hypovolemia
➧fluid moves to intravascular space
➧vasoconstriction maintains pumping pressure

kidneys:
➧reduce blood flow (↓urine)
➧↑serum osmolality (ADH & renin)
Acute Diarrhea
Tx:
➧correct F&E imbalances
➧PO fluids (better than solids): low sugar; milk ok
➧IVF for severe or if emesis
➧no meds unless really severe so kid can poop illness out
Chronic Diarrhea
etiology: Hx viral infection; dietary restrictions; abx use (Cdiff)

patho:
➧impaired intestinal motility
➧excessive fluid intake
➧dietary fat restriction
➧CHO malabsorption (sorbitol, fructose)

Dx: exclusionary

Tx:
➧avoidance of certain foods/liquids
➧↑fiber, fat
➧limit fluid intake: b/c will give more diarrhea
Vomiting
Dx: good Hx/descriptions (parents report more)

S/S:
➧classic presentation to vom & feel hungry right after (except w/ virus)
➧fatigued when VERY sick
➧HR does not improve right away ⇒ bolus

Tx:
➧PO clear fluids
➧antiemetic meds given (high risk, b/c of surgery)
Severity of Burns
1st degree: superficial; scalds, sunburn

2nd: partial thickness; can still blanch w/ pressure

3rd: full thickness; burn not painful b/c nerves died but associated 1st/2nd around it hurt

4th: down to/including bone
Area of Burn Wound (Pathology)
edema:
➧injury to vessels causes caps permeability
➧VD causes ↑hydrostatic pressure in caps

fluid loss:
➧no skin to hold moisture

circulatory:
➧↓blood flow d/t fluid shifts ⇒ ↓CO & edema
➧thrombi
Systemic Consequences of Burns
cardiovas (burn shock):↓CO d/t circulating myocardial depressant factor ⇒ ↓blood vol

renal:
➧renal VC occurs d/t loss intravas fluid ⇒ compensates w/ ↓UO ⇒ ↓renal plasma flow ⇒ ↓GFR
➧can result in renal failure

GI: acid production stops; ↑metabolism d/t N loss

g&d: delays in >40%TBSA burn for as long as 3yrs post
Major Burns Management
airways (consider smoke damage):
➧establish airway
➧give 100% O2
➧blood gas
➧bronchodilators

fluid:
➧15%-20%+TBSA need Tx
➧crystalloid to keep electrolytes; colloids for protein & keeping fluids
➧cap refill, mental status & UO (Parkland) indicate adequate management

nutrition:hypomet then hypermet; more protein & calories; supplemental feeds if >25%

meds: abx, sedation & analgesia
Major Burn Wound Care
wound: primary excision to reduce infection; debridement in OR allows new skin to grow

topical antimicrobial agents: silver nitrate; silver sulfadiazine; bacitracin

biological skin coverings: allograft, xenograft, synthetic; permanent skin coverings (donor site more painful)
Esophageal Atresia & Tracheoesophageal Fistula Patho, S/S & Dx
patho:
➧defective separation or incomplete fusion of the tracheal fold
➧may have blind pouches or fistulas

S/S:
➧frothy, drooling, can't pass feeding tube, abd distension, apnea, tachypnea after feeds
➧3 C's: coughing, choking, cyanosis

Dx:
➧careful assessment before discharge
➧Hx polyhydramnios
➧bronchoscopy w/ endoscopy
Esophageal Atresia & Tracheoesophageal Fistula Pre- and Post-Op Treatment
pre-op:
➧patent airway
➧supportive therapy: NPO (to protect lungs), NGT; ↑HOB; IVF; ABX (to protect lungs)
➧incubation to protect from saliva

post-op:
➧reposition q2hr: often sedated & paralyzed
➧suction only w/ measured catheter
➧↑HOB
➧promote adequate nutrition b/c usually pt has feeding aversion d/t of long NPO status
➧pain control
➧family support
GER & GERD
S/S: emesis (feel better after); poor weight gain*; blood in emesis*; heartburn; gagging/choking; recurrent PNA* (reactive airway disease); esophagitis

Dx: Hx, pH probe, UGI/endoscopy

Tx:
➧small thickened feeds
➧↑HOB
➧burping, sucking, no feeds before bed

meds: antacids & histamine-receptor agonists/proton pump inhibitors (change pH); prokinetics (not proven)

fundoplication if cannot maintain airway
Hypertropic Pyloric Stenosis
patho: enlarged muscular tumor nearly obliterates pyloric channel

S/S:
➧regurgitating ⇒ projectile vomiting
➧appears hungry/irritable
➧late signs: dehydration, lethargy, lyte imbalance

Dx:palpable olive-like mass; U/S; labs

Tx (post-op):
➧IVF, NG
➧clear liquids ad lib
➧teach incision care
➧expect emesis
➧manage pain
➧monitor weight/feeds
Causes of CHF
↑vol workload d/t altered PRELOAD (volume)

↑workload d/t altered AFTERLOAD (resistance), ↑PVR or ↑SVR

impaired function or contractility
CHF Signs and Symptoms
CV: tachycardia; murmur; cool extremities; slow cap refill; diaphoresis; hepatomegaly; periorbital edema; pallor

Resp: pulm edema; tachypea; resp distr; resp infection

GI/Nutrition: feeding intolerance; failure to thrive

Behavior: irritability; lethargy; exercise intolerance
Ventricular Septal Defect (VSD)
patho: abnorm opening b/t RV & LV

S/S: failure to thrive; murmur; pulm over-circulation (pulm endothelial damage); HF common

repair:
➧timing dependent on degree of shunt; can close on its own
➧device closure
➧stitch closure or patch closure

post-op complications: arrhythmias; ventricular function
Tetrology of Fallot Pathology
1) ventricular septal defect

2) pulmonary stenosis

3) overriding aorta

4) right ventricular hypertrophy: results from PS when RV has to work to get past this
Tetrology of Fallot S/S & Management
S/S:
➧murmur
➧"Tet Spell": acute episodes of hypoxia/cyanosis when blood supply < O2 requirements; worsened w/ crying, after feeding, etc

management:
➧knee-to-chest position
➧O2: vasodilator; to ↓PVR
➧morphine: pain; to ↓PVR
➧fluid bolus: to ↑SVR
➧phenylephrine: alpha agonist to constrict vessels; to ↑SVR
➧intubation
Tetrology of Fallot Repair
timing dependent on frequency of Tet spells & level cyanosis; typically 6mon

Corrective Procedure v Palliative Care:
➧RV-PA conduit
➧Transannular patch: makes PA bigger
➧Blalock Taussig Shunt

post-op:
➧residual VSD
➧residual obstruction
➧ventricular dysfunction & low CO: cut in ventricle to repair
➧arrhythmia
➧RV-PA conduit: anti-PLT Tx; future conduit replacements (b/c is like foreign body)
Enuresis
types:
➧primary: not successful at complete dryness
➧secondary: not successful even w/ dryness; nocturnal/diurnal
➧sometimes DM as cause

management:
➧organic: Tx cause (DM)
➧nonorganic: behavioral Tx (PM alarm); meds (Tofranil for ACh affects, DDAVP for ↓urine production)
Acute Glomerulonephritis Etiology, Patho & S/S
etiology: usually after strep infection

patho:
➧inflamm: edema & occlusion of glomeruli & capills; ↓GFR; Na & H2O retention; vascular congestion; HTN; FACIAL EDEMA
➧malfunction: PROTEINURIA; HEMATURIA

S/S:
➧good health
➧nephrotic rxn: puffy face, anorexia, dark ↓urine
➧pale, lethargic, irritable
➧generally unwell
➧mild to moderate HTN
➧azotemia
Acute Glomerulonephritis Clinical Course & Dx
clinical course:
➧acute edematous phase for 2-3wks, usually 4-10days
➧small ↑UO & ↓weight, followed by diuresis
➧↑BUN for several wks
➧hematuria persists for wks to months

Dx:
➧urinalysis: hematuria (+3-+4), proteinuria, ↑spec gravity
➧biopsy
➧CXR: cardiac enlargement
➧blood: ASO titer-+ (for strep), ↑BUN, ↑CREAT
Acute Glomerulonephritis Tx/Nsg & Prognosis/Complications
Tx/Nsg:
➧supportive for: edema, HTN, hematuria, oliguria
➧fluid restrictions, I&Os: to prevent HTN
➧Na restriction, low K if oliguric: to help w/ edema
➧diuretics: HTN, edema
➧ABX if persistent strep
➧weight QD
➧urine drip: some blood

prognosis: good following Dx & supportive care

comps:
➧HTN encephalopathy
➧cardiac decompensation
➧acute renal failure
Nephrotic Syndrome Etiology & Patho
etiology: 2-7yo; idiopathic, secondary, congeital

patho:
➧↑glomerular permeability
➧proteinuria & ↓plasma oncotic pressure: hypoalbum & hyperlip
➧IVF into interstitial spaces edema
➧renin-angiotensin system & ADH
➧retain Na & fluid
Nephrotic Syndrome S/S & Dx
S/S:
➧periorbital edema w/ weight gain
➧urine changes
➧norm BP
➧GI probs: anorexia, poor food absorp
➧ascites, pleural effusion (d/t edema), labia/scrotal swelling
➧irritable, fatigued, lethargic

Dx:
➧based on S/S
➧urine: proteinuria
➧blood work: ↓protein
➧biopsy: no nephrotic changes
Nephrotic Syndrome Tx/Nsg & Complications
Tx/Nsg:
➧goals: ↓excreted protein; prevent skin breakdown; promote adequate nutrition; prevent infection
➧rest & diet: conserve energy; well balanced but Na/fluid restricted
➧corticosteroids (Prednisone): 1) protein-free urine for 10-15d ⇒ con't steroids for 4wks ⇒ biopsy ⇒ immunos
➧immunosuppressant Tx: if steroid unresponsive

comps:
➧2/3 relapse but usually complete remission by 30s
➧infection d/t long term steroids
➧sometimes kidney damage
ICP Monitoring and Drains
monitoring (does NOT help pressure):
➧non-invasive transducer
➧invasive device: epidural transducer; subarachnoid bolt or screw; intra-ventricular catheter transducer

drains: ventricular drain
➧helps w/ pressure by working w/ gravity so head > bag
Early Increased ICP Symptoms
distended scalp veins; HA; nausea; vomiting; fatigue; personality changes; seizures; bulging fontanel; high-pitched cry; Macewen's sign (hollow sound when skull percussed); Sun setting eyes (no botton scelera); diplopia (double vision)
Late Increased ICP Symptoms
LOC; change in pupils; motor/sensory responses; papilledema (vessels in back of eye as distended and full d/t chronic ↑ICP)
Goal of Care with Increased ICP
keep level down

↓stimuli

quiet environment

control hyperthermia: ↑temp will ↑ICP

neutral head position: not flat d/t skin breakdown (use donut or gel protectant)

protect airway but don't suction often: will cough with ↑ICP
Seizure Activity Assessment & Dx
assessment:
order of events (before, during, after); duration of seizure; onset; behavior; movements; face; eyes; respiratory effect; other (incontinence); postial observations

Dx:
➧type of seizure based on Sx
➧determine etiology/Hx
➧EEG: maps brain activity after seizure w/i certain time
Seizure Tx/Nsg
meds:
➧acute: valium, activan, dilantin, phenobarbital (to stop seizure)
➧long term: tegretol, klonopin, depakene (loading doses needed)

ketogenic diet: ↑fat, ↓CHO, adequate protein; shifts primary Gluc from CHO to fat

vagus nerve stimulator: for pts not controlled w/ meds, partial onset, +12yo; cranial nerve 10, prevents seizure

surgical: for unresponsive, troubles w/ statues; no issue to remove a specific troubling area

safety: protect form injury; nothing in mouth; helmets; water safety; sleeping safety
Spina Bifida
patho: neural tube defect that affects small area of spinal cord or entire length; incomplete closure/abnorm development

types:
➧occulta: internal; bones fail to close
➧cystica: external sac; meningocele (fluid); myelomeningocele (fluid + tissue)

S/S: depends on degree of affect; many systems; sensory/motor dysfunction

Dx: examine sac; in utero w/ AFP or U/S

surgery: 1-3d post-birth

Tx/Nsg:
➧skin care: prevent decub & infection
➧prevent neuro injury/infection
➧prevent ortho injury: contractures; determine functional expectations; promote best locomotor function
➧promote bowel elimination: regime-diet; regular toileting (won't feel); prevent constipation/impaction
➧manage neurogenic bladder: clean cath; antispasmotics?