final Flashcards
glucagon
hormone that increases the amount of glucose in your blood
insulin
hormone that decreases the amount of glucose in your blood
produced by beta cells in the pancreas
Type 1 DM
absolute insulin deficiency-> caused by autoimmune attack on beta cells, which decreases insulin secretion
peak onset 11-13 yo
higher rates in whites
etiology: genetic component/env factors
clinical manifestations of type 1 dm
reflecting an increased bl glucose, since no insulin around
- polyuria: bc sugar spilling over into urine, and water follows…increased urine
- polydipsia: body senses being hypovolemic, which triggers thirst
- polyphagia: hungry, but losing wt: bc of fluid loss and body breaking down fat stores/protein
s/s of type 1
increased thirst…polyuria; weakness/fatigue; blurred vision; nausea; tingling in hands (NS dysfunction); slow healing of wounds…incresed risk for infection; hunger…wt loss
type 1 evaluation
- increased ketone and glucose levels in urine
- random bl glucose screen >200mg/dl (+ s/s)
- fasting bl glucose >126mg/dl
- glucose conc >200mg/dl 2h post 75g OGTT (testing done @ 28w pregnancy)
- HgA1C >6.5% (glucose sticks to hgb in away that is measurable); no fasting req; measured over time (3m) v 1 d
treatment type 1
- insulin therapy (only option)
- diet/meal planning- admin insulin based on consumption
- exercise- decreases need for insulin
hypoglycemia
cause, onset, s/s
- take too much insulin
- happens quickly
- TIRED: tacky, irritable, restless, excessive hunger, diaphoresis/depression
diabetic ketoacidosis (causes, presentation)
- long term dev
- causes: infest, stress, poor insulin mgmt
- result of increased lipolysis (adipose breakdown)-> conversion to ketone bodiess->causes acidity (H+)
clinical man of diabetic ketoacidosis
- clin man (think metabolic acidosis):
1 breath fast/deep (Kussmaul) to breathe out acetone…fruity breath!
2 dehydration bc of increased urination
3 hyperkalemia: body’s buffer system will try to exchange H for K to decrease acidity, but results in excess K bc…no insulin available to regulate Na/K pump
Type 2 DM
- 90% of diabetes cases
- higher risk for non whites and elderly
- risks: aging, sedentary, obesity, genetics, pt w metabolic syndrome (pre diabetes)…obesity, htn, dyslipedimia (cholesterol)
- body becomes resistant to the action of insulin on tissues (desensitized)….which causes a requirement for more insulin AND lowered glucose utilization
Type 2 DM evaluation
- urinalysis for glucose presentation
- random bl glucose screen >200mg/dl (+ s/s)
- fasting bl glucose >126mg/dl
- bl blucose >200mg/dl 2h post 75g OGTT
- HgA1C >6.5%
type 2 DM treatment
diet/exercise…wt loss (to improve glucose tol- cells become more sensitive to insulin)
meds: oral at beginning…insulin over time
monitor for complications/chronic changes
Long term consequences of DM (hyperglycemia)
*seen less in type 1 since they’ll go into ketoacidosis before these things appear
- eyes: diabetic retinopathy; blurred vision
- kidneys: failure
- CV: hpt/failure
- cerebrovascular: risk for stroke
- neuropathy
- peripheral vasculature: decreased perfusion…vascular damage…wounds can’t heal (neuropathy)…amputation/ gangrene
- Infection
gestational diabetes
(cause, risk, treatment, complications for mom/baby)
*all prego’s screened at 28w - OGTT
- cause: placental hormones/wt gain cause insulin resistance & inability to pro amt of insulin nec during preg
- risk: fam hx; age; prev gest diabi; prev macrosomic preg
- treatment: nutrition/exercise; insulin if lifestyle changes don’t resolve
- complications for baby: >4kg; neonatal hypoglycemia when cord cut (may need IV); still birth
- comp for mom: increased risk for dev DM 10-20y post
glucocorticoids
cortisol:
E conversion; immune response, inflamm, stress
Minteralocorticoids
Aldosterone:
Na and fluid
Androgens
sex hormones
Addison’s Disease
- primary adrenocortical insufficiency
- destruction of adrenal cortex–>decreased segregation of glucocorticoids, mineralocorticoids, and androgens
- causes: removal of adrenal gland; neoplasm (abnormal tissue growth); Tb; histoplasmosis; cytomegalovirus; autoimmune disease
pathology/clinical man of Addison’s disease
- decreased cortisol = no E; no sugar…hypoglycemic
- decreased aldosterone = decreased Na/H2o–> hypotension
- decreased androgens = change body dist/hair
- hyperkalemia = acidosis…fatigue, dehydration, renal fail
- ACTH isn’t suppressed= change pigmentation (bronzing of skin)
Evaluation/Treatment of Addison’s disease
Eval:
- lab: plasma cortisol levels
- ACTH stimulation test: measure cortisol levels
- imaging: CT/MRI of adrenal glands
Treatment: replacement hormones; stress dosing
Cushing’s Disease
- hypercortisolism
- cluster of clinical abnormalities caused by excessive adrenocortical hormones or related corticosteroids
- causes:
pituitary hyper secretion of ACTH
tumor
admin of synthetic glucocorticoids/steroids
clinical man of cushing’s disease
- increased cortisol: hyperglycemia; moon face; fat deposits on back/shoulders; mood changes
- Na/H2O retention: edema; hpt
Eval/Treat Cushing’s disease
Eval:
- lab: dexamethasone suppression test: cortisol will stay high if you have the disease
- imaging: U/S, CT, MRI of pituitary or adrenal gland
treat:
- surgery/radiation for tumors
- pharmacotherapy (mgmt): anti-hpt; K; diuretics
meningitis
- inflamm/inf of the brain/sp cord
- from norm inhabitants that get into wrong place; maybe bc of bl inf, trauma, fracture
- young, old and immunocomp more susceptible
bacterial meningitis
- septic
- most common
- streptococcus pneumaniae: most common in adults, 2nd most common in peds
- neisseria meningitidis: most common in peds
- e coli/group B hemolytic streptococci: most comm newborns
bacterial vs viral meningitis
septic vs aseptic
pathophysiology of meningitis
3 phases
1 invading organism: bacteria, toxins, viruses, fungi, parasites
2 inflamm response: neutrophils come fight and bring exudate w/ them..things get clogged… CSF changes due to inability to flow easily
3 cerebral changes: inability to flow = increase in intracranial pressure…disrupt of bl supply (ischemia) …exudate continues to exacerbate inflamm response
Infectious clinical manifestations of meningitis
fever, tachycardia, chills, petechial rash
neurologic clinical manifestations of meningitis
(all related to increased ICP) decreased LOC cranial nerve involvement (assess PERRLA) seizures* irritability* delirium *everything is more sensitive bc of ICP
clinical man of meningeal irritation
(along spinal cord)
throbbing h/a
photophobia (light sensitivity)
nuchal rigidity (stiff neck…very cautious of mvmt)
Kernig’s sign
presentation of meningeal irritation
child laying…flex hip 90 deg…extend knee
if pos for meningeal irritation, this will be painful
Brudzinski’s sign
presentation of meningeal irritation
bend child’s neck up so chin to chest
if pos for meningeal irritation, pt will bend knees in response to pain
Eval of meningitis
lumbar puncture: culture/gram stain; increased pressure; CSF
culture will show:
- high… WBC, neutrophils and protein
- normal…glucose (=viral/aseptic)
- low…glucose (=bacterial/septic) *bc bacteria eating glucose!
treatment of meningitis
bacterial: IV ab’s
viral: antivirals/steroids
manage complications
provide support: rest, dark room, decreased stimuli/noise
prevention of meningitis
immunizations
Intracranial pressure
-pressure exerted by contents of the cranium (0-15 mg Hg)
-cranium has a fixed vol that holds brain matter, CSF, and blood
(not fixed in babies until fontanelles close)
common causes of increased ICP
in adults: stroke, trauma, tumor
n peds: structural malform, inf, tumor
etiology of increased ICP
1 cerebral edema: accumulation of fluid
2 space-occupying processes: tumor, hematoma, access
3 hydrocephalus: excessive accumulation of CSF
types of cerebral edema
1 vasogenic: interstitial edema (cap pressure/damage)
- extravasation of electrolytes, proteins and fluids
- results in stroke, ischemia, HTN, tumors
2 cytotoxic: intracellular edema
- ischemic tissue swells due to deficiency in cellular ATP
- results in cardiac arrest/severe hypoxia
*2 types often occurring together
clinical man of increased ICP in adults
h/a change in LOC change in eyes: pupils/vision vomiting change in VS: Cushing's Triad seizures* decreased motor function* posturing*
*late signs
Cushing’s triad
increased systolic BP
decreased pulse
altered respiratory pattern
clinical man of increased ICP in infants
bulging fontanelles increased head circumf (why we continue to measure until font close) high pitched cry poor feeding sun-setting eyes
eval of increased ICP
- imaging: tumor? bleed? what’s causing?…
- lumbar puncture:
measure ‘opening pressure’– increased ICP = fl rises higher than norm
may be a continuous drain or open/close
treatment of increased ICP
treat underlying cause
monitor/alleviate pressure
cerebral oxygenation (not SP o2)
pharm
def of seizures
alteration in membrane potential that makes certain neurons abnormally hyperactive and hypersensitive to changes
interaction of complex genetic mutations with environmental effects that cause issues
etiology of seizures
different causes across the lifespan; 1/2 are idiopathic, which makes treatment hard
neonates: birth defects, inf, injury
peds: genetic, febrile seizures, trauma
adults: degenerative brain disease/cerebrovasc disease
seizure classification
based on symptoms/ECG features
(but brain is fine in bw, which makes it hard to capture activity…why is why they provoke one to occur when they can monitor it)
Generalized seizure
entire brain surface is affected
involvement of thalamus and reticular activating system results in loss of consciousness
-absence/petite mal: staring episodes; hard to detect
-myoclonic: single/several jerking attacks
-atonic: drop attacks/fall down
-tonic-clonic: jerking of many muscles
other types: atypical absence, tonic, clonic
Partial seizure
simple v complex
limited to one brain hemisphere
- simple - no impairment of consciousness/change in LOC; can be motor, sensory, and /or autonomic
- complex - impairment of consciousness
- secondary generalized- begins partial but then involves both hemispheres
seizures triggered by…
things that activate neurons or lower ability of neurons to fire
very individual
ie: infection; drinking; trauma; drugs; increased stimuli
epileptogenic focus
focusing on where seizure starts
prodromal/aura
subjective sense a pt has that a seizure is about to occur
post ictal
period after seizure when pt has regain of consciousness; deep coma/deep sleep;
recovery can last several hours
diagnosing seizures
history (video)/physical neuro exam EEG labs: electrolyte imbalance- trigger/cause? imaging: tumor? malformation?
treatment of seizures
- airway: risk for aspiration; have suction nearby; *don’t stick anything in their mouth in attempt to keep airway open
- injury: clear the environment
- treat underlying cause
- pharm: goal is mono therapy, but usually need 2+
- pt edu to avoid triggers
- good documentation
febrile seizure
- seizure accompanied by fever (>100.4) w/out CNS involvement (no infectious process going on)
- primarily bw 6mo-5y
- etiology: genetics and env
- simple: single/short
- complex: reoccur/prolonged
they can norm be managed at home
if you have one, likely to have more…increased risk for epilepsy
asthma
reversible obstructive airway disease characterized by 1 bronchospasms (obstruction) 2 inflammation & edema (mucus) 3 reactivity to variety of stimuli
leading cause of acute and chronic illness in children
most frequent admitting diagnosis
obstructive
air can’t get OUT; gets in fine, and then gets stuck; children with asthma have increased risk of COPD later in life
risk factors associated with astha
high risk populations: af am; hispanic living in the inner city (allergens/pollutants) premature/low birth wt fam hx allergies eczema low SES
classifications of asthma (4)
- intrinsic/non allergenic: usually adult onset; no hx of allergies; respiratory inf/psychological
- extrinsic/allergenic: triggered by things like pollen, dust, dust mites, roaches, drugs, chemicals, MSG, alcohol
- exercise induced: medicate before exercising
- status asthmaticus: ongoing, uncontrollable asthma attach; life-threatening
asthma pathophysiology: early response
INFLAMMATION
allergen binds to IgE on mast cells
degranulation of mast cells
mediators are released (histamine, leukotrienes, prostaglandins, TNF-IL-I
vasodilation, increased permeability, bronchospasm, and edema/mucus secretion
increased Ach–>constricts sm muscle and mucus
clinical manifestations of asthma
wheezing: sound of vibrations through narrow airways; don't go away w cough; sound of wheeze on insp v exp signals diff levels of severity cough (night time only) feelings of chest tightness sputum tachycardia = hypoxemia tachypnea
severe clinical man of asthma
cyanosis
retractions/nasal flaring
decreased breath sounds= ‘silent chest’- so much inflamm/airways so tight
agitation? check pulse ox…sign of low o2
can’t speak in complete sentences
pulsus paradoxus (decreased systolic pressure during insp)
bronchioloitis
- inflammation of the bronchioles (lower respiratory tract) usually 2ndary to infectious agents (ie: RSV, flu, bacteria)
- seasonal: nov to april
- premie’s esp at risk! immunizations to target
bronchioloitis @ cellular level
viral attack leads to necrosis of bronchial epithelium…mucus produced=obstruction…leads to
- inflammatory exudate
- release of chemical mediators (constriction)
- inflammation- fibrosis and narrowing
bronchiolitis: changes to breathing mechanisms
- air trapping: hyperinflation- overextended bc alveoli can’t open/close like normal); alveoli often collapsed
- decreased compliance: atelectasis (complete/partial lung collapse)
- increased work of breathing
clinical man of bronchiolitis
rhinorrhea (runny nose/snot)
tight cough: diff to feed/risk for aspiration
decreased app, lethargy
fever
tachypnea and resp distress (retractions)
abnormal ausculatory sounds: wheezing/ronchi
x-ray showing hyper expanded lunch, infiltrates, atelectasis
tx for bronchiolitis
supp o2
increased hydration (so mucus can run out easier)
inhaled hypertonic saline
bronchodilators/steroids (not so much anymore, cause not treating inflamm problem)
pulmonary embolus
undissolved detached material that occludes bl vessels of the pulmonary vasculature
90% originate from DVT’s
can also be fat, air and amniotic fluid
risk factors for pulmonary embolus
virchow’s triad
impact depends on size, area of circulatory impairment, and health status of pt
clinical man of pulmonary embolus
restlessness/apprehension/anxiety *pulse oximetry is first sign of hypoxia
DYSPNEA
late signs…..
chest pain
tachy/tachypnea
hemoptysis (not nec every case)
…progress to heart failure, shock, respiratory arrest
tx for pulmonary embolus
PREVENTION
resp support
thrombolytic therapy
heparin
dx for pulmonary embolus
hx/physical
chest xray/CT
ABG (respiratory alkalotic)
elevated d-dimer (sensitive but not specific)
saturable PK
half life is dep on the dose
a lower dose will hav a shorter half life
ie: phenytoin *very small therapeutic range, so easy to reach toxicity
auto induction (as related to PK)
as therapy continues, the drug’s half life will decrease
therefore in order to practice effective therapy, you must start with a low dose and slowly titrate (over time/weekly) to avoid kickstarting pt’s metabolism and not reaching a therapeutic range (which administering a big bolus would do)
chromosomal anomalies
mutations
abnormal number
abnormal structure
can be autosome or sex* chromosome
*more common/less debilitating than autosomal
mendelian single-gene disorders
autosomal dominant autosomal recessive x linked (sex-linked); gametes
euploidy
46 chromosomes
aneuploidy
anything other than 46 chromosomes
monosomy
deficiency of a chromosome
*loss of chromosome more serious than too much
polysomy
too many chromosomes
ie: down’s sydrome
nondisjunction
not forming correctly
usually the cause of genome mutations during meiosis
ie: down’s–>disjunction of chromosome 21
abnormal structure (chromosome mutations)
has a normal number, but abnormal structure: deletion duplication inversion translocation point mutation frameshift mutation
point mutation
substitution of a single base pair
2 poss outcomes:
1 base pair change causes a change in amino acid coded (bad)
2 base pair change creates same amino acid as originally intended to be coded (not bad)
frameshift mutation
addition or deletion of 1 or 2 nucleotides–> changes the reading frame, and all codons after are now incorrect
autosomal recessive disorders
due to mutation of a recessive gene on one of the autosomes
-males/fem equally affected
-unaffected (carrier) may transmit the disease to offspring
-may experience…delay of onset, incomplete penetrance (gene expression), and variable expressivity
ie: cystic fibrosis, sickle cell
AA= nothing
Aa= carrier
aa= affected
autosomal dominant disorders
due to mutation of a dominant gene on one of the autosomes
males/fem equally affected
*affected ind usually has affected parent; no generation skipped; only takes 1 dominant allele to affect offspring
unaffected ind (ie carriers) do not transmit the disease
ie: huntingtons disease
AA (homozygous) affected
Aa (heterozygous) affected
aa nothing
x linked recessive inheritance
- seen more often in males (bc they only have one X)
- can skip generations via carrier female
- never passed from father to son (bc father’s only pass a Y to the son)
- if father affected, it will be passed to all his daughters; -whether or not the daughter is affected or simply a carrier then depends on mom
multifactorial disorders
polygenic
polygenic traits: traits that result from several genes acting together
multifactorial: can’t predict the outcome; genes make the individual susceptible (ie: diabetes, hptn)
-env factors may trigger susceptibility or infl the expression of a trait
difficult to predict occurrence but “runs in families”
penetrance
re multifactoral disorders
% in which gene is expressed…
% of individuals w/ a given genotype who exhibit the phenotype associated with that genotype
expressivity
re multifactoral disorders
level of expression…
extent of variation in phenotype associated with a particular genotype
penetrance v expressivity
both terms quantify the modification of gene expression by varying environmental and genetic background
variable penetrance
explains things that skip generations
variable expressivity
explained by the env and other genes expressing the same trait
variable penetrance and expressivity
(??)
congenital
present at birth
may or may not be hereditary
de novo - from new
could be result of deviation in utero development
heriditary
inherited genetically
down syndrome
“trisomy 21”
extra copy of chromosome 21
most common of the chromosomal disorders (1 in 700)
risk increases with maternal age
pathophysiology of down syndrome
- caused by nondisjunction (95% of cases): pair of 21st chromosome fails to separate in meiosis
- caused by translocation (4%): full or partial copy of chromosome 21 attaches to another chromosome
- caused by mosaicism (1%): some cells have 46 chromosomes (normal), and some cells have 47 chromosomes (abnormal- an extra 21st chromosome)
characteristics of down syndrome
cognitive disability
low set ears, wide nasal bridge, protruding tongue (hypotonic), upward slant to eyes, epicanthal folds, single palmar crease, small stature
nuchal changes (neck) can be spotted with US
hypotonia in baby - joints relaxed/flaccid
those with disease have increased risk for congenital heart disease, leukemia, and respiratory issues
Duchenne Muscular Dystrophy (DMD)
x link recessive affecting only males apparent by age of 3 life exp ~20y goal: maintain fct and independence (surgery, PT, prevent obesity which would make it even more diff to move, address infection quickly, med w/ steroids)
patho of DMD
muscle cells deficient in protein dytrophin–> weak cell membranes–> leak creatinine kinase and take in calcium–> proteases and inflamm processes activated–> leads to muscle fiber necrosis and muscle degeneration
clinical man of DMD
present w/ muscle weakness/difficulty walking
large calves- normal fiber replaced w/ fat and connective tissue
gradual loss of ability to ambulate by 8-13yo (Gower’s sign)
scoliosis (when disease has progressed)
osteoperosis…fractures
muscle weakness progresses = respiratory weakness = premature death
very complex disease mgmt…many disciplines need to be involved in care
diagnosing DMD
H&P
initially shows an elevated creatinine kinase
muscle biopsy
genetic testing
sickle cell anemia
chromosome 11 comes from a point mutation (sub of single base carrier = diff amino acid coded for) autosomal recessive (aa = affected; Aa = carrier) homozygous inheritance (aa) = disease...affected heterozygous inheritance (Aa) = trait...carrier
patho of sickle cell
- genetic mutation causes defective hgb molecule (Hb S)
- when o2 is low, Hb S undergoes polymerization and causes it to change into sickled shape
1st: sickling- occlusion of small arteries–tissue damage–pain
2nd: anemia- shorter life span due to hemolysis; hyperbilirubinemia (jaundice) as RBC’s breaking down
potential triggers for cells to sickle
dehydration, infection, fever, acidosis, hypoxia, exposure to cold
clinical man of sickle cell
(typ don't present until 4-6mo, once fetal hgb is gone and infant's body is trying to make it's own) chronic anemia pallor jaundice (bilirubin levels) fatigue delayed growth/puberty infections (most common cause of death) sickle cell crises: vaso-occlusive, acute sequestration, aplastic anemia
vaso-occlusive
sickled cells and vasospasm block the blood flow–> thrombosis and infarction
extremely painful, lasting for days/weeks
pot locations: joint/bone pain; acute chest syndrome; dactylitis (hand and food syndrome); priapism (penis); cerebrovascular (brain)
acute sequestration
usually in children 6m-4y
blood pools causing enlargement–>hypovolemic shock–> Ex surgery (splenectomy)
pot locations: spleen, liver, lungs
asplastic
increased destruction or decreased production–> profound anemia
associated with viral infections and fever
Dx of sickle cell
newborn screen
h&p (related to crisis-like symptoms…pain/anemia)
labs: anemia; sickled and target cells (pale w/ bulls eye)
Tx of sickle cell
stem cell transplant edu to prevent sickling crises vaccinations penicillin prophylaxis (to prevent pot inf) transfusions splenectomy pain mgmt, hydration, and oxygenation
sickle cell complations
S strokes, swelling of hands/feet, spleen
I infections, infarctions (bl clogged in vessels)
C crisis, chest syndrome, cardiac probs
K kidney disease
L liver and lung probs
E eyes/erection
cystic fibrosis
chromosome 7 (1300 diff mutations)
most common single gene disorder
autosomal recessive (aa = affected; Aa = carrier)
most often in caucasian americans
defect in membrane trans for chloride ions in epithelial cells
production of abnormally thick secretions in glandular tissues (which is what’s causing most of the probs)
autosome
name for ordinary paired chromosome, not a sex chromosome
carrier
person that transmits a genetic condition but suffers no symptoms of that condition
heterozygous vs homozygous
dissimilar pair of genes (Aa)
same pair of genes (AA or aa)
genotype
genetic makeup of an organism
phenotype
appearance of an organism resulting from the interaction of the genotype and the environment
chromosome
structure of DNA molecules that consists of 2 strands that wind around each other (double helix)
gene
basic physical unit of inheritance that is passed from parent to offspring
meiosis
cell div used in gamete formation
mutation
change in DNA sequence that results from copy mistakes or exposure to radiation, chemicals, or infection
Mendel
Austrian monk who worked out basic laws of inheritance
inherited
trait that is genetically determined
pedigree
genetic representation of a family tree
Punnett Square
diagram used to predict probability outcome of a genetic cross in parents
allele
diff forms of the same gene
epigenetics
how genes are turned on or off by their environment
inflamm bowel disease (IBD)
chronic illnesses that are more commonly onset in childhood and young adults
ie: crohns and ulcerative colitis
ulcerative colitis
- inflamm disease of the mucosa of the colon and rectum
- continuous exacerbations and remissions
- genetic, environmental and immunologic factors (fam hx, Jewish decent, white race)
pathology of ulcerative colitis
- inflamm at base of crypts of Lieberkuhn (due to activated neutrophils/macrophages)
- inflammation leads to access formation
- accesses coalesce–> ulcer formation
- they can repair, but it’s fragile and highly vascular tissue, so it will occur repetitively
- superficial ulceration only (vs transmural)
clinical man of ulcerative colitis
- diarrhea - bc of inability of colon to absorb water… which produces more watery stools
- rectal bleeding (2ndary to ulcers)
- abdominal pain
dx and tx of ulcerative colitis
- dx with biopsy (view ulcerations)
- tx corticosteroids and immunosuppressants (bc inflamm disease); Ab if systemic toxicity (infection)
- manage with nutrition: no milk; low fiber/fat diet; high in protein; similar to hypoallergenic diet
- risk for colon cancer increases; colectomy if high grade dysplasia found (abnormal growth)
Crohns disease
- inflamm of the GI tract from lips to anus
- transmural: extends thru all layers of intestinal wall
- *cardinal features: granulomas (deep linear ulcers)
- genetic, env and immunologic factors: genetic predisposition, Jewish decent, white race, cig smoking, living urban (higher rate) vs rural; virus/bacteria
pathology of crohns
- blockage of GI lymphoid and lymphatic structures
- engorgement/inflamm –> granulomas (deep linear ulcers)
- thickened by fibrous score, observe deep fistulas (secretions from intestines in places they shouldn’t be (ie stool discharge out of vagina…increases risk of UTI/inf)
- cobblestone look to tissue on biopsy - bumpy surface
clinical man of crohns
- nutritional deficiency: incapable of adequately absorbing nutrients, AEB wt loss, malnutrition, ht/wt changes over time
- biopsy shows “skipping lesions”
- perianal fissures (tears in skin - pt will complain of pain when going to bathroom), fistulas and abcesses
dx and tx of crohns
hx and physical: wt, nutrition, s/s
biopsy: granulomas, skipping lesions
- tx: corticosteroids, immunosuppressants
Ab if systemic toxicity
manage with nutrition: diet like ulcerative colitis
- risk for colon cancer increases; colectomy if high grade dysplasia found
Gastroesophageal reflux disease (GERD)
def and 3 causes
backflow (reflux!) of gastric contents into the esophagus through the lower esophageal sphincter (LES)
3 causes:
1 issues with LES: closure strength and efficacy, ie: high fat diet, caffeine, alcohol, smoking, obesity, meds
2 increase in intrabdominal pressure, ie prego, tumor, overfed babies, constipation, crying, bearing down to poop, positioning
3 delayed gastric emptying, ie infants
clinical man of GERD
heartburn regurgitation chest pain dysphagia (painful swallowing, eating) may have no s/s- physiologic reflex (esp in infants)- see when difficulty eating/not enough wt gain
2 complications of GERD
- long term exposure of esophagus to highly acidic gastric content–> leads to strictures/narrowing
- aspiration –> respiratory issues like asthma, cough, inflamm of larynx (laryngitis)
tx of GERD
dietary/behavior changes: low fat diet; decrease smoking, all, caffeine
pharm: antacids and histamine blockers; proton pump inhibitors
GERD in peds
- reflux is normal in newborns and part of dev
- CP, head injury, neural signaling issue (can’t close LES) all increase risk of GERD
- highest rates in premie, and decreases during first 6-12 mos (until LES can mature)
tx: small, frequent feedings (1-2hr); burping; positioning, meds - s/s: feeding refusal (pain), recurrent vom, poor wt gain, irritability, sleep disturbance (reflux is likely when lying flat), resp s/s
- s/s older children: same as above, plus asthma, recurrent pneumonia, and chronic cough/hoarse voice (upper airway effected)
osteomyelitis
- severe infection of bone and local tissue
- infection can reach the blood by:
bloodstream (infection coming from somewhere else)
adjacent soft tissue injury
direct introduction of the organism into the bone - most common pathogens (directs tx): S aureus & S pneumaniae
osteomyelitis
high risk groups and clinical man
high risk: surgery (contamination risk); open fractures; penetrating wound, IV drug users; kids <16yo (risky play), older adults (change in bones/increased risk for falls)
clinical man: pain, fever, muscle spasms, swelling, refusal to use affected limb (bc of pain)
pathophysiology of osteomyelitis
1 bacteria enters
2 inflammation
3 pus formation…spreads inward (unaccessible to tx)
4 access forms
5 interrupted bloods supply –> necrosis
6 cavities or sinus forms when bone heals around necrotic tissue (again, inaccessible)
osteomyelitis: dx and tx
dx: xray; labs (increased WBC, CRP, ESR) indicate inf/inflamm; bone scan/MRI; blood cultures (systemic) or bone aspiration (if just in the bone)
tx: 4-6w IV Ab’s or IV then switch to PO (oral Ab’s aren’t strong enough to reach inaccessible areas, so must have IV component); debridement if needed/not clearing up; removal of prosthesis or other materials (if that’s what’s causing it), ie new hip not jiving in body
fractures
break in continuity of a bone, epiphyseal plate, or cartilaginous joint surface
generated by trauma
risk groups: young males 15-24 and older adults
differences in peds bones
1 less brittle, higher collagen to bone ratio (+)
2 stronger periosteum helps bones heal faster (+)
3 presence of epiphyseal plate (where bone growth occurs); if break occurs here, poss of altering/stunting growth (-)
transverse fracture
break occurs at right angles to the long axis of the bone
serial fracture
twisted or circular break that affects the length; s shaped; *suspicion for child abuse
longitudinal fracture
fracture along the length of the bone
oblique fracture
45 edge angle diagonal or slanting that occurs bw horizontal and perpendicular plates of the bone
comminuted fracture
splintered into pieces
impacted fracture
telescopes or drives one fragment into the other (into itself); may be referred to as compression or buckle fracture
greenstick fracture
break through the periosteum on one side while only bowing or buckling on the other side
(see pics in vocab doc)
stress fracture
fracture on the cortical surface- can become complete
avulsion fracture
small fragment of bone fragments
complete v incomplete fracture
break through entire bone vs partial break
open wound vs closed wound fracture
break in the skin near the fracture (also called compound fracture) vs no open wound
healing of cortical bone
(triggered by inflammation - 1 and 2 are triggers for things to come to the site and start the healing process)
1 bleeding
2 hematoma forms
3 osteoblasts and calcium come a runnin
4 callus forms…new bone built/old bone destroyed
5 callus reabsorbs
compartment syndrome
a potential comp of fractures;
accumulation of pressure;
surgical Ex;
check for 5 P’s: pain, paralysis, parenthesis (decreased sensation), pallor (interrupted bl supply), pulses
complications of fractures
nonunion/malunion osteonecrosis osteomyelitis fat emboli syndrome DVT and/or pulmonary embolism neuromuscular injury
function of the ductus arteriosus
pathway of blood from the pulmonary artery to the aorta (fetal circulation, before birth)
fct of the foramen ovale
pathway of bl from the R atrium to L atrium and out to the body
(fetal circulation, before birth)
key things affecting CHD
pressure gradient, oxygenation, workload
pressure gradient: fl (blood) is lazy! will move from high to low pressure; @ birth the pressure is greater on the systemic side (vs pulmonic side)- this means pressure is greater on the L side of the heart
oxygenation: oxygenated bl is on the L side, headed out to the body; unoxygenated blood on the R side
workload: an increased workload = hypertrophy of the heart; an upstream workload comes before the defect, and is what’s causing the defect; a downstream workload comes after the defect
Patent Ductus Arteriosus (PDA)
defect and patho
- defect: ductus arteriosus doesn’t close after birth (meant to close 24-48h post birth due to oxygenation changes in fetus and hormones)
- pressure: bl goes into pulmonary artery vs aorta (less pressure)
- oxygenation: acyanotic; no oxygenation issue; bl is circulating back into the lungs/picking up more O2
- workload: can cause pulmonary hptn –> R sided heart failure- more work required from R ventricle to push blood through the pulmonary artery; this is a workload issue
PDA
clinical man and eval/tx
clinical man: heart failure; murmur; widened pulse pressure; bounding pulses; cardiac enlargement/hypertrophy
eval/tx: echocardiogram to observe bl flow/structures; *admin Indomethacin to close the ductus arteriosus; if med not successful, cardiac cath or surg
Atrial Septal Defect (ASD)
defect and patho
an opening in the septum bw the atria, usually where the foramen ovale was (is)
- pressure: bl goes from L side of the heart to the R atrium
- oxy: acyanotic/no issue; bl is just moving to the other side
- workload: R sided hypertrophy/heart failure- working harder to pump extra blood
ASD
clinical man and eval/tx
cm:
size of defect guides interventions (if small enough may just be left alone; big requires surgery)
fatigue/dyspnea on exertion (crying/eating)
recurrent respiratory inf
murmur
HF in young adulthood
eval/tx: Echo; spontaneous closure if poss; manage HF; cardiac cath/surg
Ventricular Septal Defect (VSD)
defect and patho
an opening in the septum bw the R and L ventricles *most common heart defect
pressure: blood flows L to R
oxy: acyanotic, no issue
workload: R sided HF/hypertrophy
VSD
clinical man and eval/tx
cm: size of defect guides; murmur; HF
eval/tx: may close spontaneously; manage HF; cardiac cath/surg
pharm: Digoxin (output); Diuretics (fl status); ACE inhibitors
Coarctation of the heart
a narrowing of the aorta, commonly around the ductus arteriosus
pressure: higher behind the defect; lower after defect
oxy: acyanotic, no issue; decreased amt of bl getting out, but bl that is still circulating is oxygenated
workload: increased on L side–> L sided ventricular hypertrophy–> pulmonary manifestations (edema) due to the extra fluid vol
Coarctation of Aorta
clinical man and eval/tx
cm: murmur
poor lower extremity peripheral perfusion
pulse and BP diffs in upper and lower extremities (must check all pulses when assessing! MD order: “BP on all 4 extremities”
left sided HF
eval/tx: echo; improve CO with diuretics and digoxin; *admin Prostaglandin E to keep ductus arteriosus open (so bl can choose to go path of least resistance, and therefore decrease the workload of the heart); cardiac cath/surg
Tetralogy of Fallot
(tetra=4…4 defects)
1 VSD
2 overriding aorta
3 pulmonary stenosis
4 R ventricular hypertrophy
pressure: like a washing machine, everything is all over the place
oxy: cyanosis; unoxygenated bl in aorta being transported to the body
workload: R sided HF; primarily bc of stenosis…working hard to get past
Tetralogy of fallout
clinical man and eval/tx
cm: severity related to pulmonic stenosis
becomes worse after DA closes (24-48h…after baby has gone home from hospital!)
cyanosis/fatigue; hypercyanotic episodes “Tet spells”- pressure changes when baby eats/cries/poops and there’s even more unoxygenated bl traveling out to body- blue baby
murmur and thrill
boot shaped heart on X-ray
eval/tx: echo, Prostaglandin E to keep PDA; mgmt of tet spells; series of surgery
Transposition of the Great Arteries
transposition = mixed..in wrong place; defect involves switched aorta and pulmonary artery - unconnected circles
happening separately and simultaneously:
RV–>aorta–>unox bl–>body
LV–>pulm artery–>oxyg bl–>lungs
Transposition of the Great Arteries
cm (a very quick progression): cyanosis at birth, hypoxemia with O2, progressive desaturation and acidosis, HF
eval/tx: Prostaglandin E to keep PDA and get oxyg bl out to body; cardiac cath/surg
heart failure
inability of the heart to maintain sufficient cardiac output to meet metabolic demands of tissues and organs
CO = stroke volume x heart rate
treat HF to increase CO
right sided heart failure
ineffective R ventricular function
bl backs up R atrium and into peripheral circulation
most often caused by L sided HF
-cm: (all affecting peripheral circulation)
jugular vein distension
hepato-splenomegaly
weight gain (edema)
L sided HF
ineffective L ventricular fct
CO decreases– bl backs up into L atrium, then lungs
-cm:
(pulmonary s/s) dypsnpea, cough/crackles, hemoptysis
tacky; s3/s4
cool, pale skin
Biventricular HF
R & L sides not working; symptoms of both R and L HF
Systolic HF
ventricles don’t pump out enough blood into systemic circulation
reduced myocardial contractility = low EF
(ejection fraction: % bl ejected out during systole)
norm EF <40-50%
(only measuring systole)
diastolic HF
ventricles don’t fill up properly/not enough bl to pump out;
will see a change in stroke vol
congestive HF
dx: history/physical; chest xray; check electrical activity with EKG/ECG; echo for structure/function
tx: manipulate preload, afterload and contractility
- improve CO: digitalis and digoxin
- minimize congestive s/s: diuretics
- minimize cardiac workload: pacemaker, ACE inhibitors, ARV’s
CHF in peds
-most common comp of CHD and acquired cardiac disease
-most often result of decreased L ventricular systolic fct:
L atrial and pulmonary venous hptn
pulmonary venous congestion
tx: underlying cardiac defect guides; manage fl balance - increased caloric content of feeds/diuretics; increased CO with meds
serotonin (5-HT)
fct/effect
feeling of well being *mood sleep appetite sexual desire/fct memory
when you’re lacking you’ll see c/m of depression
dopamine
cognitive fct; “sluggish” when lacking
motivation
interest/attn
pleasure/euphoria
Norepinephrine
stress hormone
attention
fight/flight
when you’re lacking you’ll see anxiety disorder; fi/fl mechanism is dysregulated
depression
a clinically and etiologically heterogeneous disorder- everyone is clinically different/diff etiology
leading cause of global disability; higher in girls and older children; *directly related to suicide
r/f: genetic; relational/interpersonal stressors; chronic illness; poor coping mech; SDOH like school failure/lower edu, abuse/neglect and ses;
monoamine deficiency theory
monoamines = norepinephrine, serotonin and dopamine
- not a usual amt avail in the synaptic space; decreased baseline/amt to begin with
- overactive reuptake (SSRI)
- enzymes are eating up the nerotransm. before they make it to the postsynaptic neuron
- not enough transport molecules to accept the neurotransmitter
major depressive disorder
- 5+ s/s have been present during the same 2-week period and represent a change from previous functioning
- s/s cause clinically sig distress/impairment in social, occupational or other imp areas of functioning
- episode is not attributable to the physiologic effects of a substance or other med condition
- not better explained by schizo/other psychotic disorder
- there has never been a manic episode
anxiety, fears and worries
anxiety = NECESSARY physiologic response; keeps us safe in dangerous situation fears = NORMAL emotional response to threat; part of dev worries = cognitive manifestation of fear and anxiety
labeled an anxiety disorder when the fear, worries or anxiety are outside of normal dev response- are extreme and cause sig distress/impair fct (ie social/school situation)
–>persistent, excessive and unrealistic worry
anxiety disorder in peds
most comm psychiatric illness
can start in preschool but doesn’t impair until school age
girls 2x more likely
r/f: genetic predisposition; temperament, modeling, physiologic factors, exposure to psychosocial/env stressors
high rate of comorbidity with depression
Fight or Flight with anxiety disorder
fi/fl reg through the limbic system: sensory input (thalamus) contect/memory (hippocampus) emotional reg (amygdala) prefrontal cortex helps modulate
in anxiety disorder there is a dysregulation - it’s activated in situations that are not actually a threat; involvement of neurotransmitters: norepinephrine, GABA, serotonin, dopamine
release of stress hormones and adrenaline without reg
generalized anxiety disorder
excessive anxiety/worry occurring more days than not for at least 6mos
physical s/s (only one required in peds) such as restlessness, diff concentrating, irritability, muscle tension, sleep disturbance
causes clinically sig distress/impairment
not attributable to physiological effects of substance/med
not better explained by another medical disorder
*if not treated approp, major depressive disorder/ substance abuse l ikely down the road
long term physiologic effects of stress cause GI, CV and other probs later in life
