PEDs Exam 2
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| Infant Screening | PKU (mandatory in all states)
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| Phenylketonuria (PKU) | Autosomal recessive; deficient phenylalanine hydroxylase (used to convert phyenylalanine to tyrosine); no conversion = toxic -> CNS damage -> retardation
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| PKU - s/s | s/s may not be seen until 3mos; GI probs, V 1st, musty or mousy odor to urine, infantile eczema, hypertonia/hyperactive behavior, hypopigmentation (blond hair, blue eyes)
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| PKU - Dx | screening, before 3rd day of life may give false negative, positive result needs further testing; serum phenylalanine levels >10mg/dL
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| PKU - management | early tx; lifelong dietary restriction; keep serum phenylalanine 2-6mL/dL <12yrs; 2-15mL/dL >13yrs; d/c diet can show dec mental functions; pregnant = strict diet to avoid mental deficiency, microcephaly, retarded growth, seizures, structural defects
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| Congenital hypothyroidism - manifestations NEONATAL | skin mottling; large fontanel; large tongue; hypotonia (tongue protruding); slow reflexes; distended ABD; constipation
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| Congenital hypothyroidism - manifestations EARLY INFANCY | prolonged jaundice; constipation; feeding problems; cool skin; umbilical hernia; hoarse cry; excessive sleeping; large tongue; respiratory problems
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| Congenital hypothyroidism - untreated | s/s usually appear p 6wks; facial features (depressed nasal bridge, short forehead, puffy eyelids, large tongue); coarse, dry, lusterless hair; ABD distention, umbilical hernia; hyporeflexia; delayed neurologic development -> mental retardation
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| Congenital hypothyroidism - tx | lifelong thyroid hormone replacement; synthroid; bone age surveys to monitor optimum growth; normal growth et intelligence if tx begun shortly p birth
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| Growth Hormone deficiency (Part 1) | secreted @ night; assoc c underlying causes such as brain tumors, pituitary gland malformations; may present as hypoglycemia
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| Growth Hormone deficiency (Part 2) | growth normal 1st yr -> slowed curve below 3rd percentile; skeletal proportions normal; appear younger
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| Growth Hormone deficiency - Manifestations | ht <5th percentile; restricted growth rate; ht may be more slowed than wt if nutrition good -> obesity; immature (cherubic) faces; delayed puberty; hypoglycemia; diminished muscle mass; inc fat; micropenis; delay perm teeth; norm intelligence (precocious)
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| Growth Hormone deficiency - Dx | fam hx; child's growth hx, other health hx; physical exam; bone growth studies; endocrine studies
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| Growth Hormone deficiency - Management | synthetic growth hormone subq 6-7x/wk; stopped when growth plates fuse or when acceptable adult ht reached, give @ night; inc growth potential r/t earlier tx; given to children c short stature s GHD no major inc growth
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| Precocious puberty | Early onset of puberty (before 8yrs in girls; before 9yrs in boys); can occur in infancy or early childhood; premature appearance of secondary sex characteristics; accelerated bone growth -> early growth plate fusion -> short stature in adulthood
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| Precocious puberty - manifestations | secondary sex characteristics; acne; growth spurt in females; adult body odor; menses in girls; deepening of voice in boys; moodiness
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| Precocious puberty - management | luteinizing hormone - releasing hormone, IM once q 4wks, d/c @ chronologically appropriate time when puberty desired
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| Precocious puberty - special considerations | treat according to chronological age, not appearance; children often teased; emotional stress; inc risk of child abuse; children are fertile (oral contraceptives will initiate epiphyseal closure)
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| Congenital adrenal hypoplasia (CAH) | group of disorders characterized by deficiency of enzyme needed to make cortisol; inability of adrenal gland to make adequate glucocorticoid -> excess androgen production; autosomal recessive; cortisol needed for maintenance of BS, fluid/lytes, hormones
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| Complete (Salt-Losing) CAH | insufficient amts of aldosterone, cortisol; EMERGENCY (FTT WEAKNESS, V, DEHYDRATION IF NO TX @ BIRTH); aldosterone replacement, supplement dietary salt; frequent labs for lytes, hormone levels
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| CAH - manifestations | ambiguous genitalia of female newborn; post-natal virilization of both genders; salt-wasting crisis (inc K, hypovolemia, hypoTN)
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| Simple Virilizing CAH manifestations | no salt-wasting crisis: muscular body, adv. bone age, premature pubic hair
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| Milder form of CAH manifestations | hirsutism (delayed hair growth), delayed menses, menstrual irregularities
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| CAH - management | surg reconstruction; lifelong glucocorticoid tx; mineralocorticoid replacement for children c salt-wasting CAH florinef; dbl or trpl glucocorticoids when ill, fx, or surgery; bone age studies q yr; monitor BP
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| Type 1 Diabetes | pancreas unable to produce et secrete insulin; genetic predisposition + environmental or viral trigger = autoimmune destructive process; diet (cow's milk) et viruses implicated in triggering autoimmune destructive process; no prevention/cure
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| Type 1 Diabetes - Manifestations | polyuria, polydipsia, polyphagia c wt loss et dehydration, ABD pain et V; routine UA during yearly physical exam (ketones et glucose)
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| Hyperglycemia | onset slow; N/V, confusion, lethargy, polyuria, polydipsia, polyphagia, fatigue, blurred vision, gradual wt loos , HA, hunger, emotional liability; serum glucose >160mg/dL
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| tx for Hyperglycemia | insulin; exercise; inc PO fluids
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| Hypoglycemia | rapid onset; weakness, dizziness, trembling, sweating, tachy, pallor, clammy skin (adrenergic signs); personality chg, irritability, drunken behavior, slurred speech, dec LOC, seizures (nueroglycopenic sign); serum glucose <70mg/dL
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| tx for Hypoglycemia | 15g of carbs (4oz juice); in unconscious or having seizures (glucagon SQ or IM, IV glucose)
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| Ketoacidosis Labs | serum glucose >300mg/dL; serum pH <7.25; positive serum ketones
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| Ketoacidosis | lack of insulin; excessive stress (end of illness)
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| DKA | inc BS; low pH; large urine ketones; inc serum ketones; WBC inc r/t stress; low serum CO2
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| DKA - s/s | acetone (fruity) breath; ABD et chest pain; deep, rapid resps. (kussmaul); dec LOC; N/V; dehydration
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| Progression of s/s | hyperglycemia (inc glucose, glucosuria) -> ketosis (ketones, glucose in urine; possible dehydration) -> ketoacidosis (dehydration; lyte imbalance; acidosis; kussmaul)
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| Type 2 Diabetes | peak incidence occurs c onset of puberty; females>males; inc esp. among ethnic minorities
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| Type 2 Diabetes - Manifestations | typically overwt; may have all s/s of Type 1; fatigue; frequent infections
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| Goals of Diabetes Management | appropriate growth (ht et wt); age-appropriate lifestyle; near-normal glycosylated Hgb; prevent or min acute complication (hypoglycemia, hyperglycemia)
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| Lispro | onset 15-30min; peak 60-90min; duration 2-3hr
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| Regular Insulin | onset 30min; peak 2-4hr; duration 4-6hr
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| NPH or Lente Mixed Insulin | onset 2-4hr; peak 6-8hr; duration 12-24hr
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| Ultralente Insulin | onset >2hr; peak variable; duration 24-36hr
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| Insulin Administration | need less in honeymoon phase; 2+ injections/day mimics norm insulin secretion; subq; rotate sites to dec adipose hypertrophy; location sites vary in rates of absorption
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| Insulin | store cool dry place; expiration date; draw short before mixing c long; can reuse disposable needles for 1-3days store in fridge; proper disposal of needles; rotate injection sites
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| Long-term complications of diabetes | retinopathy; diabetic nephropathy; foot problems --> good BS control, norm BP, no smoking
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| Developmental issues of Diabetes management | chronic disease; life-time management; child's perception changes over time; parent's goal is to facilitate independence in caring for self; affects entire family
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| Target ages for technical skills | 4-6yrs (urine testing); 4-8yrs (blood testing); 8-10yrs (insulin injections); 10-14yrs (nutrition decision skills); 12-18yrs (management decision skills)
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| Type 1 vs Type 2 Diabetes | 1 (immune disorder; body attacks et destroys insulin producing beta cells in pancreas); 2 (disorder either body does not produce enough insulin or the cells ignore the insulin)
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| Diabetes Dx | Prediabetes (FBS >100; random BS >140); Diabetes (FBS >126; Random BS > 200; A1C >6.5)
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| Meningocele | Saclike cyst of meninges containing CSF; translucent (flashlight able to see through sac); not assoc c neuro deficit; cover c sterile, saline gauze
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| Myelomeningocele | sac contains meninges, CSF, portion of spinal cord or nerve roots; not translucent; first 28 days of pregnancy; any point along spine; usually lumbar or lumbosacral; severity r/t location; 80-85% also hydrocephalus
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| Myelomeningocele - s/s | depends on location
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| Myelomeningocele - tx | initially, prevent infection et surg repair; observe for postop complications of hydrocephalus (head circumference); prepare fam for long-term effects of disability
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| Myelomeningocele - nursing care | assist c dx; place infant prone; moist dressing; goal (prevent infection, preserve or enhance neuro or urologic function); postop assess for infection, hydrocephalus
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| Hydrocephalus | r/t various disease or from injury; communications (impaired absorption of CSF c/in subarachnoid space) OR non-communications (obstruction of CSF flow through ventricular system; arnold-chiari malformation of skull
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| Hydrocephalus - causes | myelomeningocele; intraventricular hemorrhage; tumors; CSF infection; head injury
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| Hydrocephalus - manifestations in infants EARLY | rapid head growth; full anterior fontanel; irritability; poor feeding; distended scalp veins; widely separated cranial sutures
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| Hydrocephalus - manifestations in infants LATE | setting sun sign; frontal bone enlargement (bossing); inc BP; dec HR; altered resp; shrill, high-pitched cry; sluggish or unequal pupil responses to light
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| Hydrocephalus - manifestations in children EARLY | strabismus; frontal HA (worse in AM, relieved by sitting up); forceful V; behavior, school cahnges; ataxia; papilledema; irritability; sluggish or unequal pupil responses; lethargy
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| Hydrocephalus - manifestations in children LATE | seizures; inc BP; dec HR; alteration in resp pattern; herniation of optic disc -> blindness; decerebrate posturing
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| Hydrocephalus - tx | ventriculoperitoneal shunt (drains excess CSF into peritoneal cavity)
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| ventriculoperitoneal shunt - nursing considerations | prevention of infection; assess for shunt malfunction; paralytic ileus or peritonitis; anticipatory guidance; will need revisions as they grow
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| Increased Intracranial Pressure (ICP) - causes | head trauma, infection, hypoxia can cause cerebral edema; overproduction of fluid or "drainage" (spina bifida) problem can affect
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| Inc ICP - s/s | dec LOC (glasgow coma scale); behavior (eventually don't recognize parents, don't respond to pain); pupils sluggish to dilate or become fixed; motor function (dec purposeful movements or posturing); vitals altered
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| Inc ICP - infant s/s | tense, bulging fontanel; separated cranial sutures (esp if approx. before); irritability; high-pitched cry; distended scalp veins; poor feeding; setting sun sign
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| Inc ICP - children s/s | HA, N/projectile V, blurred vision, seizures
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| Inc ICP - behavioral s/s | irritability, restlessness; indifference, drowsiness; inc sleep; inability to follow simple commands; lethargy; drowsiness; diminished school performance, physical activity
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| Inc ICP - late s/s | brady; dec motor response to commands; altered pupil size et reactivity; decerebrate or decorticate posturing; cheynes-stokes resp; papilledema; dec LOC -> coma
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| Levels of consciousness | conscious, confused, disoriented, lethargic, obtunded, stupor, coma
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| Closed head trauma | non-penetrating; no break b/w skull et brain
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| Open head trauma | skull, meninges open; infection a big concern
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| coup | injury directly @ point of impact
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| contrecoup | injury remote from impact; caused by rapid movement of brain away from point of impact
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| skull fx | linear (dura not involved); depressed (bone indented); basilar (base of skull, s/s battle's sign, racoon eyes, rhinorrhea, otorrhea, hemotympanum); comminuted (fragmentation)
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| contusion | bruised area; petechial hemorrhages on superficial aspects of brain
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| primary head injury | occurs at moment of impact
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| secondary head injury | brain tissue destruction secondary to hypoxia, hemorrhage, edema, etc.
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| Intracranial hemorrhage | epidural (b/w dura et skull; arterial damage; develops rapidly); subdural (b/w dura et cerebrum; acute or chronic depending on type of vessels involved)
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| concussion | transient, reversible, neuronal dysfunction; instantaneous loss of awareness et responsiveness; posttraumatic amnesia reflects the extent of injury (retrograde - period of time before injury; anterograde - period of memory loss p injury)
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| Post-concussion syndrome - infants | pallor, sweating, irritability, sleepiness, V
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| post-concussion syndrome - children | behavioral disturbances, sleep disturbances, phobias, emotional lability, irritability, seizures, altered school performance
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| post concussion syndrome | diffues s/s of brain dysfunction (not working); HA; dizziness; impaired conc.; variable duration
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| Meningitis | most common infectious process affecting CNS; usually bacterial; may be primary infection or may occur secondary to trauma, surgery, migration, from other infection sites
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| Meningitis - prevention | HIB vaccine
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| Bacterial meningitis | acute inflammation of meninges et CSF; HIV vaccine; greatest mortality in children birth-4yrs; extension of other illness; nasopharynx common site of entry
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| Bacterial meningitis- manifestations in infants, young children | fever, V, marked irritability, restlessness, seizures, high pitched cry, bulging ant fontanel, nuchal rigidity, Brudzinski (bend neck, knees come up), Kerning signs (s/cannot extend leg at knee when this is flexed)
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| Bacterial meningitis- manifestations in children, adolescents | abrupt onset of fever, chills, HA, V -> alter in sensorium; photophobia, confusion, drowsiness, stupor, coma; nuchal rigidity -> opisthotonos; Brudzinki et Kernig signs; cyanotic, cool skin c poor periph perfusion
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| Bacterial meningitis - complications | sepsis (disseminated intravascular coagulation, shock, bilat adrenal hemorrhage, purpura, mortality rate 90%); inc ICP, hydrocephalus, SIADH, destructive changes in cerebral cortex, cranial nerve damage (hearing, vision losses)
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| Bacterial meningitis - dx | lumbar puncture, full septic workup usually done in any child c signs (urine, blood, CSF cultures, CBC)
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| Bacterial meningitis - tx | antibiotics, steroids, supportive care, hydrations, tx of complication, auditory evals in 6mos, prevention (vaccinations)
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| Seizures | more common 1st 2yrs; prenatal insults/birth injuries most common cause in very young infants; seizure threshold can be altered
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| Seizures - etiology | altered neuronal activity -> seizures; primary (occur in absence of underlying brain structural abnormality; secondary (symptomatic; provoked by temp or perm structural or metabolic abnormality, underlying cause can be found); idiopathic (no known cause)
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| Seizures - causes | cerebral lesions, metabolic disorders, anoxia, trauma, stroke, infections, degenerative disorders, toxic disturbances
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| epileptogenic focus | area of the brain where brief paroxysmal behavior caused excessive abnormal discharge of neurons
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| status epilepticus | prolonged seizure or series of seizures c loss of consciousness at least 30 min
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| epilepsy | recurrent seizure activity not assoc c other illness or procedure like insulin OD
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| refractory seizures | last more than 60min
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| generalized seizures | abnormal excessive impulses THROUGHOUT THE BRAIN; no apparent focal point of onset; altered LOC; can be "convulsive" or not; may begin at a focal point but we don't know where it started
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| partial seizures | begin in ONE PLACE IN BRAIN; may or may not alter LOC, depending on which part of brain is affected
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| generalized tonic-clonic seizures | grand mal (abrupt arrest of activity et LOC); tonic phase (sustained stiffening of muscles); clonic phase (axial symmetric, jerking asymmetric et rhythmic); postictal phase (period of confusion, lethargy, sleep)
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| generalized atonic seizures | abrupt loss of postural tone, confusion, lethargy, sleep
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| generalized myoclonic-infantile spasms | brief, random contractions of a muscle group; occur on both sides of body, singly or in clusters
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| generalized absence seizures | petit mal - very brief alterations in consciousness; may have eyelid twitching, head bobbing; day dreamer, cannot easily bring back
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| partial seizures | abnormal electrical activity in small area of brain; usually temporal, frontal, or parietal lobes; s/s assoc c area of brain affected
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| simple partial seizures | most common syndrome - benign focal epilepsy; varies b/w people; s/s r/t area of brain affected; motor, autonomic or sensory s/s; no change in LOC; odd taste or smell, ABD discomfort, unexplained fear/dread, motor movements may occur
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| complex partial seizures | may begin c/s an aura or c/s simple paritla seizure; impaired consciousness, transient staring, altered mental status, feeling of detachment or unreality; teeth-grinding, lip smacking, followed by variable postictal state
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| febrile seizures | usually young children; 2/3 not have subsequent seizures c 3% develop epilepsy; level et rapidity of temp rise are keys to seizure (usually occurs during rise); simple febrile seizures are familial
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| neonatal seizures | r/t incomplete myelinization; sustained eye opening; horizontal deviation of eyes; sucking, smacking; drooling; tongue thrusting; apnea
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| Seizures - assessment (part 1) | loss of conscious or awareness; color (pale, cyanotic, flushed); unusual movements (tonic-clonic, muscle spasms, head drops); eyes (pupil dilated, rolling upward or deviations to one side); incontinent GI/GU; postictal state; how long return; length
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| Seizures - assessment (part 2) | order of events; duration of seizure activity; significant precipitating events (lights, noise, stress); behaviors; position of eyes, head, body, extremities; color changes; resp changes; incontinence; any postictal state
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| Seizures - ketogenic diet | limit carbs et protein; 4:1 fat:carb
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| Seizures - Medications | block sodium; enhance GABA; inhibit glutamate; type of drug depends on type of seizure
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| Seizures - medications SEs | CNS effects (drowsiness, Keppra - moodiness); GI (N/V, anorexia); skin (Lamictal, Neurontin - rash); blood (anemia, leukopenia, etc.); resp depression; liver damage; hypocalcemia; lymphadenopathy; gingival hyperplasia c dilantin
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| Phenytoin (Dilantin) | prototype; initial drug of choice; CNS et GI SEs; gingival hyperplasia common; oral care
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| Carbamazepine (Tegretol) | r/t tricyclic antidepressants; pharm similar to dilantin
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| ethosuximide (zarontin) | ZZZ for sleep; drug of choice for absence seizures
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| Benzos - Lorazepam (Ativan) | drug of choice for status epilepticus; all have potential for dependence et withdrawal s/s; abrupt withdrawal may precipitate seizure activity (all antiseizure meds)
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| Seizure - management | airway, O2 suction; DO NOT PLACE ANYTHING IN MOUTH; turn on side; seizure meds/ketogenic diet, seizure surgery; prevent injury; referral; teach CPR to parents; side rails up/padded; waterproof mattress; protect during possible hazardous activities; med ID
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| Cerebral palsy | disorders characterized by early onset, impaired movement et posture; NONPROGRESSIVE; may be accompanies by perceptual problems; language deficits; intellectual involvement
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| CP - muscle movements | ataxia; spasm; spasticity; tremors; twitching; dystonia
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| CP - causes PRENATAL | maternal diabetes; Rh or ABO incompatibility; rubella in 1st trimester; intrauterine ischemia; toxoplasmosis; cytomegalovirus; congenital brain abnormality
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| CP - causes L&D | preeclampsia; complication of L&D
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| CP - causes PERINATAL | sepsis/CNS infection; asphyxia; prematurity; low birth wt; prolonged labor/aggressive labor
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| CP - causes CHILDHOOD | meningitis; traumatic brain injury; toxins; et other non-obvious causes
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| CP - manifestations | poor head control p 3mos; stiff or rigid arms/legs; push away/arch back; floppy/limp body posture; can't sit s support p 8mos; uses only 1 side of body/only arms; clenched fists p 3mos
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| CP - behavioral manifestations | extreme irritability or crying; failure to smile by 3mos; feeding difficulties; persistent tongue thrusting p 6mos or persistent gagging or choking
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| CP - clinical manifestations | delayed gross motor; abnormal motor performance - asymmetry or spasticity; alterations in muscle tone (exaggerated arch of back, scissoring of legs); abnormal postures; reflex abnormalities
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| CP - classifications | spastic (most common); athetoid (worm like); ataxic
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| Spastic CP | unilateral/bilateral; hypertonicity c poor control of posture, balance, coordinated motion; impaired fine et gross motor skills
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| Athetoid CP | constant, slow, worm-like writhing movements; extremities, trunk, neck, face, tongue; might have inc caloric needs
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| ataxic CP | wide-based gait; rapid, repetitive movements performed poorly
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| CP - gait abnormalities | ataxia (unable to coordinate movements, staggered, postural imbalance); spastic paraplegic (narrow-based, scissor gait, walk on toes c flexed knees); cerebellar (staggered, unsteady, veers in 1 direction)
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| CP - therapeutic management | PT (ortho devices, adoptive equipment); OT; speech/lang therapy; special ed; surg intervention (contractures, g-tubes); med therapy; case management
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| Adolescence Developmental Question | Look at notes from development based on ATI reading ~week 1
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| Spina Bifida (Part 1) | congenital neural tube defect - incomplete closure of vertebrae et neural tube during fetal development; may be very mild c no s/s or severe c sensory et motor defects
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| Spina Bifida (Part 2) | maternal folic acid deficiency is linked to disorder; manifestations r/t degree et level deficit; spina bifida occulta/cystica
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| Spina Bifida Occulta | NOT VISIBLE EXTERNALLY; vertebrae do not fuse completely; may have no sensory/motor deficits; usually discovered accidentally c routine xray; tuft of hair or dimple common
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| Spina Bifida Cystica | VISIBLE DEFECT; external saclike protrusion; meningocele (no nerves in sac); myelomeningocele (nerves in sac)
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| Delegation of PEDs pt care with any dx listed here |
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| Down Syndrome | most common genetic cause of mod-severe mental retardation; late maternal age primary factor; cause specific unknown; facial/body features common to all children make IDable; also have common set of potential concomitant conditions
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| Down Syndrome - Characteristics | Intelligence (mild-mod mental retardation); social (2-3yrs delay; sociable c easy temperaments); congenital anomalies (esp. septal defects); sensory (vision, hearing); growth (short stature); sexual development (often delayed)
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| Down Syndrome - common features FACIAL | brachycephaly c flat profile; upward-slanted palpebral fissures; inner epicanthal folds; wide, flat nasal bride; protruding tongue; low-set, small ears; short, broad neck
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| Down Syndrome - common features BODY | short ht; short, broad hands c transverse palmar crease (simian); clinodactyly; broad, stubby feet et wide gap b/w 1st et 2nd toes
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| Down Syndrome - common features CARDIAC | tetralogy of fallot; ASD; VSD; PDA
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| Down Syndrome - common features GI | TE fistula; pyloric stenosis; Hirschprung's disease; imperforate anus; duodenal atresia
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| Down Syndrome - common features MISC. | obesity; alopecia; thyroid dysfunction; male infertility; craniofacial defects, esp. involving jaw, teeth; joint laxity et dislocations
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| Down Syndrome - 3 types | nondisjunction (extra copy 21st chromosome); mosaic (improper division 21st chromosome); translocation (additional 21st chromosome attached to another chromosome; ONLY FORM THAT CAN BE INHERITED)
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| Down Syndrome - Dx | review clinical features for suspected dx; definite diagnosis (prenatal - CVS, amniocentesis; postnatal - blood drawn for chromosomes)
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| What to remember for Down Syndrome | physiologic (heart, pulm HTN, vision, hear, speech, apnea, feeding, small airways); person 1st speech c congrats et positivity info truthful et honest but not overwhelming --> resources
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| Hypoxic-Ischemic Encephalopathy (HIE) | LOOK AT PPT ON MOODLE
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| EXTRA CREDIT!!! | MUSCULAR DYSTROPHY
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| REVIEW ATI!!! | CHAPTERS 12-14, 27-29, 33-34
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If you know all the data on any row, you can temporarily remove it by tapping the trash can to the right of the row.
To hide a column, click on the column name.
To hide the entire table, click on the "Hide All" button.
You may also shuffle the rows of the table by clicking on the "Shuffle" button.
Or sort by any of the columns using the down arrow next to any column heading.
If you know all the data on any row, you can temporarily remove it by tapping the trash can to the right of the row.
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kdrummond08
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