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Pharmacology
Post-Graduate
04/13/2016

Additional Pharmacology Flashcards

 


 

Cards

Term
asystole
Definition
"a"= atropine(20mcg/kg)-no minimum now but old minimum was 100mcg
"e"= epinephrine(10mcg/kg)- no minimum
Term
LEAN
Definition
meds in ET tube
lidocaine=1mg/kg IV x 3 for ET(no max)= 2-3mg/kg repeat after 15min or start cont inf
Epinephrine 10mcg/kg IV(max 1mg) x 10= 100mcg/kg ET(max 2.5mg)
atropine 20mcg/kg IV(max dose 0.4mg or adult 0.5mg) x 3= 60mcg/kg ET
Naloxone 100mcg/kg IV(2mg) x 3= 300mcg/kg ET(max ?)
Term
Anion Gap
Definition
AG (mEq/L) = (Na+) - (Cl- + HCO3-)
Term
Metabolic acidosis
Definition
due to a fall in serum bicarbonate (HCO3-) concentration
Term
Respiratory acidosis
Definition
due to an elevation in arterial partial pressure of carbon dioxide (PaCO2) concentration
Term
metabolic acidosis compensation
Definition
an increased respiratory response that decreases partial pressure of carbon dioxide (PCO2, which raises the pH) begins within 30 minutes.
Term
respiratory acidosis compensation
Definition
the renal compensatory response of bicarbonate retention is much slower and may take up to three to five days.
Term
Mixed acid-base disorder
Definition
In mixed acid-base disorder, there is a simultaneous presence of more than one acid-base disorder, such as the presence of both metabolic and respiratory acidosis demonstrated by a low blood bicarbonate level (metabolic component) and elevated PCO2 due to respiratory failure.
Term
acidosis causes _______?_______
Definition
shift in the oxygen dissociation curve to the right, reducing the affinity of hemoglobin for oxygen [2]
●Decreases in myocardial contractility and cardiac output [3]
●Shift of potassium out of the intracellular space, leading to hyperkalemia [4]
●Impaired response to catecholamines [1]
●Altered mental status [5]
●Immune dysfunction [6]
●Impaired response to insulin [7
Term
metabolic acidosis
Definition
net gain of H+ or net loss of HCO3- via one or more of the following mechanisms:

●Increased acid generation from either an exogenous source (eg, salicylate ingestion) or endogenous production (eg, lactic acidosis)
●Loss of HCO3- either through the gastrointestinal tract (eg, diarrhea) or kidney (proximal [type 2] renal tubular acidosis [RTA])
●Reduced renal acid excretion either due to reduced glomerular filtration (eg, renal failure) or inability to maximally acidify urine (eg, distal [type 1] RTA)
Term
dobutamine vs dopamine
Definition
increases contractility in addition SVR where dopamine increases SVR and increases cardiac index
Term
dopamine vs dobutamine vs epinephrine
(catecholamines)
Definition
all increase bp at high concentrations
- all increase myocardial oxygen demand and increase risk of cardiac ischemia and arrythmias in low cardiac output which milrinone for low cardiac output
Term
milrinone
Definition
drug of choice for preheart tx and post op heart for increase inotropic and sytemic/pulmonary vasodilatory effects(no myocardial oxygen increase at all)
0.25mcg/kg/min or 0.5mcg/kg/min or 0.75mcg/kg/min or 1mcg/kg/min. Not like epinephrine for 10 fold due to half life
Term
shock in general
Definition
first is volume- give 20ml/kg up to 60ml/kg saline. (if blood loss- give colloid/blood) then low cardiac output and low svr= EPINEPHRINE
OR low cardiac output and high SVR= milrinone(after load reducer)
OR high cardiac output and low SVR =Norepinephrine
if SVR is still low after NE then add vasopressin
Term
low cardiac output and low svr for shock
Definition
low cardiac output and low svr=
Term
SIRS
Definition
systemic inflammatory response = high temp, tachycardic for >1(brady if less than 1), increased RR, decreased or increasedvleucocytes(MUST HAVE 2)
Term
SEPSIS
Definition
SIRS plus presence or suspected infection
clinical syndrome that complicates severe infection and is characterized by the systemic inflammatory response syndrome (SIRS), immune dysregulation, microcirculatory derangements, and end-organ dysfunction
Term
septic
Definition
Septic shock refers to sepsis with cardiovascular dysfunction (as described in the section on multiple organ failure below) that persists despite the administration of ≥40 mL/kg of isotonic fluid in one hour [
Term
refractory septic shock
Definition
There are two types of refractory septic shock: fluid-refractory septic shock exists when cardiovascular dysfunction persists despite at least 60 mL/kg of fluid resuscitation; and catecholamine-resistant septic shock exists when shock persists despite therapy with dopamine ≥10 mcg/kg per min and/or direct-acting catecholamines (epinephrine, norepinephrine) [3].
Term
cold shock
Definition
low SVR(ie low bp)
dopamine is drug of choice and if unresponsive epinephrine
Term
Norepinephrine
Definition
WARM shock = warm extremities and low SVR
Term
PEA
Definition
pulseless electrical activity(asystole)
Term
warm shock
Definition
Distributive shock is characterized by hyperdynamic (or high output) physiology with decreased systemic vascular resistance and elevated cardiac output as manifested by the following findings (see "Physiology and classification of shock in children", section on 'Distributive shock'):
•Flash capillary refill (<1 second)
•Bounding pulses
•Warm, dry extremities
•Wide pulse pressure (typically greater than 40 mmHg in older children and adults; lower pulse pressures may reflect widening in infants and neonates)
Term
cold shock
Definition
Cold" shock reflects increased systemic vascular resistance and decreased cardiac output as indicated by the following signs (see "Physiology and classification of shock in children", section on 'Hypovolemic shock'):
•Delayed capillary refill (>2 seconds)
•Diminished pulses
•Mottled or cool extremities
Term
serotonin syndrome
Definition
Hyperthermia commonly occurs in patients with serotonin syndrome, a potentially life-threatening condition associated with increased serotonergic activity in the central nervous system (CNS). Serotonin syndrome encompasses a spectrum of disease where the intensity of clinical findings is thought to reflect the degree of serotonergic activity. Mental status changes can include anxiety, agitated delirium, restlessness, and disorientation. Patients may startle easily. Autonomic manifestations can include diaphoresis, tachycardia, hyperthermia, hypertension, vomiting, and diarrhea. Neuromuscular hyperactivity can manifest as tremor, muscle rigidity, myoclonus, hyperreflexia, and bilateral Babinski sign. Hyperreflexia and clonus are particularly common; these findings, as well as rigidity, are more often pronounced in the lower extremities.
Term
how do IV and Intraosseous doses compare?
Definition
IV = IO doses
Term
How do ET and IV doses compare?
Definition
3x for most(lido, atropine and naloxone) and 10x for epinephrine
Term
vfib
Definition
defib then epinephrine is drug of choice
(adenosine, atropine and amiodarone are NOT used in vfib)
Term
wide QRS tachycardia
Definition
cardio/pulmonary compromise so best option cardioconversion
Term
For widened QRS with regular rhythym- whats the drug to use?
Definition
adenosine dose is 0.1mg/kg/(100mc/kg) and then double with adult dose of 6mg.
Term
widened QRS with irregular rhythym
Definition
could use amiodarone or procainamide as drug of choice
amiodarone is 5mg/kg/dose up to 300mg (may repeat)- over 20-60minutes- max is 15mg/kg/dose
Procainamide15mg/kg/dose repeat q 5min up to 1000mg
Term
procainamide dosing
Definition
15mg/kg/dose iv over 25 - 30min- may repeat up to 1000-1500mg total

for widened qrs tachycardia
Term
amiodarone
Definition
5mg/kg/dose iv over 20-60min. may repeat up to 15mg/kg-
ventricular tachycardia
Term
Normal ICP
Definition
normal is less than 20mmhg(greater than 20 for more than 5 minutes you should treat)
Term
increased ICP
Definition
bolus of 3% NS 10ml/kg (mannitol is second choice to 3% but 0.25gm-1gm/kg)- mannitol only lasts for an hour but doesn't stay. continuous of 3% 0.2ml/kg/hour(0.1-1ml/kg/hr)
Term
supraventricular tachycardias
Definition
WPW(wolf parkinson white)
AVNTR(atrioventricular tachycardia(rentrant or entrant)
Term
atrial fibirillation
Definition
common in s/p fontan procedure- hypoplastic left heart
Term
ectopic atrial tachcardia names(2)
Definition
FAT= focal atrial tachycardia
JET-junctional ectopic tachycardia(can happen with post op hearts)- JET has higher mortality others are very low
Term
what do you do for supraventricular tachycardia in peds?
Definition
vagal maneuvers (knee to chest) by baby's ice to face.
adenosine is first line(0.1-2mg/kg/dose or 6mg max dose)
Term
what is first line after adenosine dose for SVT if doesn't respond or continuous?
Definition
procainamide(10-15mg/kg/dose bolus and then drip 20-80mcg/kg/min or esmolol 100-500mcg/kg/min
Term
if need continued chronic suppresion of SVT for infants?
Definition
propranolol is 0.5-1mg/kg/day with dose escalation to 16mg/kg/day
OR digoxin(not as common 4-6mcg/kg/day div 1-2 doses)
OR atenolol for older patients
Term
JET treatment
Definition
amiodarone 5mg/kg/dose up to 15mg/kg/dose with ice to face and then possilby pacer because this can be fatal
Term
atrial fibrillation
Definition
cardioconversion and beta blockers or for refractory dig or amiodaraone
Term
torsades des Pointes- how does it look and what to treat?
Definition
Torsades des Pointes will have a ”party-streamer” appearance to the ECG. and use magnesium to treat
IV, IO: 25 to 50 mg/kg/dose; if pulseless, administer as a bolus; if pulse, administer over 10 to 20 minutes (Hegenbarth 2008; PALS [Kleinman 2010])
Term
how does vetricular fibrillation look?
Definition
Ventricular fibrillation will have no QRS complexes and a ‘flat-line’ appearance.= asystole- epinephrine amiodarone or procainamide(vasopressin now out or last resort)
Term
LCOS
Definition
Low Cardiac Output Syndrome after congenital heart surgery
Cardiac Output (CO) = Heart Rate x Stroke Volume
Three Components to Stroke Volume: Preload, Afterload, Contractility
Each of these is manipulated by pharmacotherapy to treat and prevent LCOS
Preload – optimized by fluid status (diuretics, fluid restriction)
Afterload – decrease to increase CO (vasodilators)
Contractility – increase to increase CO (inotropic medications)
Term
atrial septal defect
Definition
opening between atriums causing left to right shunt
-preop waiting to grow- diuretics, dig(increase contractility, ace inhib or milrinone(reduce afterload)
Term
atrial septal defect repair- what do they have?
Definition
LOCOS, hypertension is common so vasodilators(ACE inhib/milrinone- immed postop - nipride) and aspirin d/t hardware
Term
VSD
Definition
ventricular septal defect(between vetricles0- pulm hypertension and pulm recirculation and heart failure- same as ASD)- preop same(diuretics, dig and ACE/milrinone) and post op same but can have arrythmias
Term
Transposition of Great Arteries
Definition
prerepair- need prostaglandins to keep open(PDA-patent ductus arteriosis)- heart is working ok but not as much diuretics are needed compared ASD/VSD
Post op LCOS, nipride or milrinone (afterload reduce and increase CO- not often ACE)
Term
tretralolgy of Fallot
Definition
Pulmonary artery stenosis or atresia; ventricular septal defect; aorta overriding both ventricles; ventricular hypertrophy
Term
aspirin ingestion
Definition
can use gastric lavage if no ms changes and charcoal- use bicarb in fluid for acidosis
Term
reason for decrease of HIV infections diagnosis in infants
Definition
universal prenatal HIV counseling and testing, antiretroviral prophylaxis, scheduled cesarean delivery, and avoidance of breastfeeding, the rate of perinatal transmission of HIV has dramatically diminished to <2% in the U.S. and Europe.
Term
babies that don't have perinatal precautions in US have increase risk of infection by 21-25%
Definition
Mom with higher viral load

2. Duration of exposure (ruptured membranes, vaginal delivery)

3. Factors that facilitate transfer of virus from mom to child (maternal breast pathologic lesions, infant oral candidiasis)
Term
Selection of an ARV regimen for a pregnant woman, at least one nucleoside reverse transcriptase inhibitor (NRTI) with high placental transfer should be included.
Definition
Abacavir, Emtricitabine, Lamivudine, Tenofovir, Zidovudine
Term
For HIV moms Intrapartum
Definition
IV zidovudine should be administered to HIV-infected women with HIV RNA >1000 copies/mL near delivery. This is not required for women receiving ARV regimens who have HIV RNA < 1000 copies/mL

2. Cesarean delivery is recommended for women with an HIV RNA >1000 copies/mL
Term
Postpartum for HIV moms
Definition
1. Infant antiretroviral prophylaxis

Six week’s of zidovudine chemoprophylaxis is recommended to reduce perinatal transmission and should be started as close to the time of birth as possible.

b. Infants born to HIV-infected women who have not received cART should receive prophylaxis with zidovudine given for 6 weeks combined with 3 doses of neviripine in the first week of life (i.e. at birth, 48 hours, and 96 hours after second dose)
Term
Henoch-Schönlein purpura (HSP)
Definition
acute, systemic, immune complex-mediated vasculitis
Term
HSP
Definition
cutaneous purpura, arthritis, abdominal pain, and renal disease
last 4 weeks
Term
hyperbinielmia
Definition
In the liver, uridinediphosphogluconurateglucuronosyltranserase (UGT1A1)

helps transform bilirubin into conjugated (direct) bilirubin
Term
Conjugated(direct)bilirubin
Definition
is excreted in bile into the duodenum
Gut bacterial enzymes break conjugated bilirubin down and it gets excreted
Term
newborn jaundice
Definition
Bile production is higher in neonates due to increased hemolysis

a. More blood cells overall and fetal blood cells have shorter half-life

b. Fetal blood cells have shorter half-life

2. Neonates have decreased clearance

a. Deficiency in UGT activity that is responsible for conjugating bilirubin
Term
Bilirubin peaks at day of life 3-5.
Definition
Mean peak unconjugated bilirubin

i. White and African American infants: 5.5 mg/dL

ii. Asian infants: 10 mg/dL

b. By 96 hours of life, most have bilirubin < 17 mg/dL

4. Bilirubin > 17 mg/dL is not considered physiologic at any age
Term
Kernicterus:
Definition
Acute bilirubin encephalopathy
Bilirubin concentration that is toxic varies among infants

a. If total bilirubin > 25 mg/dL: consider acute toxicity

b. The more immature the infant, the greater the risk 75% of infants who develop kernicterus die

2. 80% of survivors have neurologic sequela
Term
phototherapy for hyperbinejdida
Definition
Converts bilirubin into lumirubinis (water soluble)

2. Lumirubinis water soluble and is excreted in urine or bile (no conjugation required)

3. Types available

a. Bili blanket

b. Bili lights: Blue fluorescent lights most effective
Term
drugs that can cause hyperlipedemia9 name 6)
Definition
hemotherapeutic agents

ii. Isoretinoin

iii. Corticosteroids

iv. Anti-retroviral agents

v. Oral contraceptives

vi. Beta blockers
Term
ulcerative colitis
Definition
Primarily affects colon and rectum, inflammation usually in a continuous pattern
Term
crohns dissease
Definition
Affects any part of the GI tract, often discontinuous, patch, and is associated with transmural inflammation
Term
crohns
Definition
4.56/100000;
dicontinuous pattern, cobble stone appearance
Term
crohns other symptoms
Definition
Joints: Arthritis,synovitis, polychondritis

2. Biliary Tract: pericholangitis, cholelithiasis, autoimmune hepatitis

3. Skin: Oral lesions, aphthous ulcers, stomatitis, glossitis

4. Eyes: Conjunctivitis, uveitis, scleritis, neuritis, retinal vascular disease

5. Essentially any organ can be affected
Term
TPMT
Definition
TPMT is an enzyme that converts 6-MP into 6 –methylmercaptopurine, a hepatotoxic metabolite

i. High activity (89% individuals):
Term
crohns disease feeding
Definition
exclusive enteral nurition
Term
pneumonia
Definition
pneumonia is the most common infectious cause of death in children younger than 5 years of age.
Term
pneumoniA risk factors
Definition
Recent history of upper respiratory tract infection

2. Comorbidities: asthma, bronchopulmonary dysplasia, cystic fibrosis, sickle cell disease, congenital heart disease

3. Others: lower socioeconomic status, crowded living environment, exposure to cigarette smoke (first- or second-hand)
Term
treatment for S. pneumoniae
Definition
isolates with a minimum inhibitory concentration (MIC) ≤2 mcg/mL, ampicillin or penicillin G are still preferred.
Term
for CAP strept pneumonia
Definition
S. pneumoniae isolates with an MIC ≥4 mcg/mL, ceftriaxone is preferred.
Term
For children with CAP who develop pulmonary complications, such as an empyema or lung abscess,
Definition
coverage against methicillin-resistant S. aureus should also be considered. Vancomycin or clindamycin (if community resistance rates are low) may be added to empiric therapy for CAP in these situations.
Term
CLABSI
Definition
Central line-associated bloodstream infection
typically categorized as a hospital-acquired infection.
Term
COST OF clabsi
Definition
mean inpatient cost of nearly $60,000 and mean length of stay of 19 days
Term
primary site of CLABSI
Definition
The hub is a primary source for catheters in place ≥ 14 days
Term
Common pediatric pathogens for CLABSI
Definition
include gram-positive bacteria including Staphylococcus aureus, coagulase-negative staphylococci, and Enterococcus species, and gram-negative bacteria including Escherichia coli and Klebsiella species. Candida species are also commonly encountered.
Term
(CRBSI)
Definition
catheter-related bloodstream infection (CRBSI)
Differential time to positivity (DTP) is an alternate method to confirm CRBSI and doesn’t require catheter remove. With DTP method, the difference in time to positivity of cultures obtained from the CVC and either a peripheral sample or sample obtained from a different lumen. The premise behind DTP is that culture obtained from the colonized lumen will grow more quickly than that obtained peripherally or from non-colonized lumen.
Term
Neonatal conjunctivitis (ophthalmia neonatorum)
Definition
acquired during birth and presents within the first month of life.
Chlamydial conjunctivitis accounts for 17 – 40% of neonatal conjunctivitis.

2. Infants born to mothers with active chlamydial infections have a 20 – 50% risk of developing conjunctivitisNontypeable Haemophilus influenzae, Streptococcus pneumoniae, Staphylococcus aureus, Staphylococcus epidermidis, and Klebsiella species are other bacterial causes of neonatal conjunctivitis.

4. Herpes simples virus (HSV) is the most common viral pathogen in neonatal conjunctivitis
Term
Erythromycin for bacterial conjuctivits covers
Definition
Erythromycin has activity against common gram-positive organisms and Chlamydia but has limited activity against potential gram-negative pathogens.
Term
meningoencephalitis caused by what bacteria
Definition
Listeria monocytogenes, Treponemum pallidum, Toxplasmagondii.
Term
California Encephalitis Project (CEP) was created to collect epidemiological data regarding encephalitis.
Definition
Over 1500 cases of encephalitis reported from 1998-2005

a) 16% had an identifiable cause

b) Viral pathogens accounted for 69% of identifiable
Term
Enteroviruses (e.g., Coxsackievirus A or B, enterovirus 69, 70, or 71
Definition
commonly during the spring or summer.
Term
commonly during the spring or summer.
Definition
Enteroviruses (e.g., Coxsackievirus A or B, enterovirus 69, 70, or 71)
Term
virus common in often in fall and winter
Definition
Respiratory viruses (e.g., influenza A or B, human metapneumovir
Term
Typical symptoms of viral encephalitis
Definition
altered mental status, behavioral changes, muscle weakness or paralysis, seizures, and non-epileptiform movements.
Term
Younger childeren sx of encephalitis are
Definition
Lethargy, poor feeding, weak suck reflex, irritability, loss of head control
Term
neonatal HSV encephalitis
Definition
A pregnant woman with primary infection during the 3rd trimester who has not developed antibodies to HSV (i.e., has not seroconverted) by the time she delivers has a 33% chance of passing the infection to her infant.

2. Pregnant women who reactivate during pregnancy only have a 3% chance of transmitting the virus to their infants.eonatal HSV encephalitis
Incubation period is 2 days to 2 weeks.
Term
encephalitis- which ones do you get igM for
Definition
Serum IgM may be collected to evaluate for EBV, West Nile virus, eastern equine virus, La Crosse virus, and other arboviruses.
Term
Treatment of choice for HSV encephalitis:

a) Neonates: WHAT TO TREAT AND HOW LONG
Definition
acyclovir 20 mg/kg/dose IV every 8 hours

i. Duration:

i. Disease limited to SEM: 14 days

ii. Encephalitis or disseminated disease: 21 days

ii. Neonates with HSV encephalitis should have a repeat CSF PCR performed towards the end of therapy. If the PCR is still + for HSV, then antiviral therapy should be continued. The optimal duration of therapy in this situation is unknown.
Term
treatment for encephalitis hsv
Definition
cyclovir 10 mg/kg/dose IV every 8 hours

i. Duration: 14-21 days

c) Up to half of all neonates with HSV infection will have cutaneous recurrences.

i. Suppressive therapy with oral acyclovir for 6 months following initial infection has been shown to improve neurological outcomes.

i. Dose: 300 mg/m2/ dose 3 times per day

ii. Monitor for neutropenia
Term
Epiglottis
Definition
previously was most commonly caused by Haemophilus but post HIB vaccine it's now more commonly group B strept(GAS), strept pneumo, staph aureus and gm negative organisms
Term
ages and other info on epiglottis
Definition
2-7 years and 4:1 more in boys than girls
was 84% hib 1969-1977 compared to no occurrences in 1995-2003
Term
what are three d's for epiglottis?
Definition
drooling dysphagia and distress
Term
GAS pharyngitis vs epiglottis vs croup- what are difference?
Definition
croup will also have cough and stridor
epiglottis will have drooling and stridor
GAS pharyngitis will have stridor and cough
Term
epiglottis prophylaxis for household contacts
Definition
rifampin 20mg/kg once daily or 600mg po qd for (for household members) for 4 days if have unimmunized people or immunocompromised people or child less than 12 months who hasn't received all of HIB vaccine
Term
describe 3 outbreaks due to lack of vaccinations
Definition
1) disney land- measles 2014- 45% were unvaccinated
mumps oubreak 2011-2013 pertussis outbreak 2012
2)
Term
hepatits b vaccine
Definition
inaactivated vacc 3 doses: 1 at birth 1 at 2-3 mos
Term
rotavirus vaccine
Definition
live vaccine 2 or 3 doses series
rotarix(RV1)- 2 and 4 mos of age
rotateq(RV2)-2, 4 and 6 mos
1st dose by <15 weeks and last dose by 8mos of age
Term
diphtheria, tetanus, acellular pertussis
Definition
5 dose series
2, 4, 6 months of age
dose 4 at 18months of age and dose 5 at 4-6 years
Term
contraindications potential for dtap vaccine
Definition
fever>105 within 48 hours
CV collapse within 48 hrs
persistent crying within 3 days
convulsions within 3 days
Term
Tdap
Definition
has a reduced dose of diphtheria and acellular pertussis for people>7

1 dose at 11-12 or as soon as possible
Term
Tdap
Definition
administered every ten years or post exposure to wounds that are possible for tetanus
Term
haemophilus B vaccine
Definition
inactivated vaccine
3-4 in series for infants andchildren
ActHIB® (DTaP, IPV, Hib), Menhibrix® (Hib, meningococcal-CY), Pentacel® (Hib):

i. #1 at 2 months

ii. #2 at 4 months

iii. #3 at 6 months

iv. #4 (booster) at 12-15 months
OR 3 doses series is
PedvaxHIB® (Hib) or COMVAX® (Hib and Hep B):

i. #1 at 2 months

ii. #2 at 4 months

iii. #3 (booster at 12-15 months)
Term
Hiberix
Definition
only use as booster a 12m-4 years
Term
pneumococcal vaccine for usual risk patients
Definition
inactivated vaccine; Pneumococcal conjugate (PCV13): 4 dose series for infants and children

a. #1 at 2 months of age

b. #2 at 4 months of age

c. #3 at 6 months of age

d. #4 at 12-15 months of age

3. Pneumococcal polysaccharide (PPSV23)
Term
pneumococcal vaccine for high risk(who is high risk )
Definition
Chronic heart disease

ii. Chronic lung disease (including asthma ONLY if also receiving high-dose oral corticosteroids)

iii. Diabetes

iv. Cerebrospinal fluid leak

v. Cochlear implant

vi. Sickle cell disease

vii. Anatomic or functional asplenia

viii. HIV

ix. Chronic renal failure
x. Nephrotic syndrome
xi. Treatment with radiation or immunosuppressive agents
xii. Solid organ transplantation xiii. Congenital immunodeficiency
Term
poliovirus(IPV)
Definition
4 doses (2, 4, 6-12 and 4-6 years)
Term
MMR
Definition
live vaccine at 12-15 months and 4-6 years
Term
varicella vaccine
Definition
live at 12-15months and 4-6 years
Term
Hepatitis A Vaccine
Definition
inactivated so at 12-23 months and separated 6y a yeaars
Term
HPV vaccine
Definition
1st dose at 12 years, second dose 2-3 months later and third doses
Term
dtap
Definition
administered every ten years
Term
What vaccinations can HIV patients get and what does their CD4 count need to be?
Definition
if their cd4 is greater than 15%;
RV, MMR, and varicella vaccines.
Term
what vaccinations can HSCT patients receive and when
Definition
influenza- 4-6 mos after tx
-hould receive 3 doses of PCV13 starting 3-6 months after transplantation. After PCV13 series is completed, 1 dose of PPSV23 should also be administered
hould receive 3 doses of PCV13 starting 3-6 months after transplantation. After PCV13 series is completed, 1 dose of PPSV23 should also be administered
3 doses of Hib starting 6 months after transplantation; doses should be separated by 1 month.
The MMR vaccine should be administered at least 24 months after transplantation, if the patient is immunocompetent.
Term
what vaccinations can HSCT patients receive and when
Definition
influenza- 4-6 mos after tx
-hould receive 3 doses of PCV13 starting 3-6 months after transplantation. After PCV13 series is completed, 1 dose of PPSV23 should also be administered
hould receive 3 doses of PCV13 starting 3-6 months after transplantation. After PCV13 series is completed, 1 dose of PPSV23 should also be administered
3 doses of Hib starting 6 months after transplantation; doses should be separated by 1 month.
The MMR vaccine should be administered at least 24 months after transplantation, if the patient is immunocompetent.
Term
what is meningitis?
Definition
-inflammation of the meninges, often as a response to an infectious agent.
Term
How does meningitis occur?
Definition
bacterial colonization in nasopharynx, translocation to blood and then across bbb and once there the inflammation response makes bbb more leaky
Term
common bacterial causes for each age group for meningitis
Definition
ge/Population
Causative Organism
<1 month
Group B Streptococcus, gram negative enteric organisms (especially Escherichia coli and Klebsiella species), Listeria monocytogenes
1-3 months
Group B Streptococcus, E. coli, S. pneumoniae, Neisseria meningitidis, Haemophilus influenzae type B
4 months-5 years
S. pneumoniae, N. meningitidis, H.influenzae type B (in unimmunized or under-immunized children)
≥6 years
S. pneumoniae, N. meningitidis
CSF shunts
Coagulase – Staphylococcus, S. aureus, Pseudomonas aeruginosa, Propionibacterium acnes
Term
what are meningitis symptoms?
Definition
lassic signs/symptoms: fever, headache, stiff neck

2. Additional findings: nausea, vomiting, altered mental status, photophobia, abnormal reflexes

3. Infants and young children may present with non-specific signs or symptoms, such as lethargy, decreased tone, poor feeding, and bulging fontanelles.

E. References:
Term
who is at risk for meningitis?
Definition
Medical conditions:

1. Asplenia, sickle cell disease

2. Complement disorders

3. Immunocompromised

4. Cochlear implants, CSF leaks

5. CNS shunts
Term
what dose bacterial meningitis csf look like?
Definition
low glucose, protein 100-500(high), and wbc 1000-5000 with lots of neutrophils and opening pressure is very high 200-500
Term
what does viral meningitis csf look like?
Definition
opening pressure slightly high, normal glucose, sl high protein, lower wBc but still elevated with 50% lymphocytes
Term
what is normal csf like?
Definition
opening pressure 50-80, glucose low, protein <170 for neonates and <50 for older, 0-5 wbcs and mainly monocytes
Term
How long should you treat for bacterial meningitis?
Definition
Duration of therapy:

a. N. meningitidis: 7 days

b. H. influenzae type B: 7 days

c. S. pneumoniae: 10-14 days

d. S. agalactiae (group B Streptococcus): 14-21 days

e. Gram negative enteric organisms: 21 days

i. Neonates should be treated for a minimum of 21 days, and at least 14 days following the first negative CSF culture.

f. L. monocytogenes: ≥21 days
Term
pertussis
Definition
s a highly contagious, respiratory tract infection caused by the organism Bordetella pertussis.

B. It is known commonly as whooping cough, and can be a significant cause of morbidity and mortality, especially in young infants.
Term
Pertussis is a toxin-mediated disease.
Definition
Bacteria attach to respiratory cilia, and release toxins that paralyze the cilia.

2. Toxin production also causes inflammation of the respiratory tract.

3. These factors combined lead to a decreased ability to clear respiratory secretions.
Term
Who is most susceptible to pertussis and why?
Definition
rior to the 1940s, when the first pertussis vaccine became available, more than 200,000 people developed pertussis each year in the United States. Since that time, the incidence of pertussis has dropped by approximately 80%.

B. Adolescents and adults with waning pertussis immunity are often the reservoir for infants and children who become infected with B. pertussis.

C. Infants younger than 12 months of age, and adolescents older than 10 years of age are at highest risk of developing pertussis.
Term
Pertussis infections commonly exhibit three phases: catarrhal, paroxysmal, and convalescent.
Definition
catarrhal- runny nose, sore throat malaise like any uri
paroxysmal= coughing which is worst at night
convalescent- cough gradually improves
Term
what is pertussis treatment?
Definition
. Azithromycin has become the drug of choice for pertussis because it is generally well tolerated, has fewer drug-drug interactions than other macrolide antibiotics, and has the shortest duration of therapy of antimicrobials used for pertussis.

B. Dose:

a. Infants ≤5 months of age: 10 mg/kg/day x5 days

b. Infants/children ≥6 months of age: 10 mg/kg on day 1, then 5 mg/kg/day on days 2-5

c. Adolescents/adults: 500 mg on day 1, then 250 mg/day on days 2-5
Term
what is some complications for pharygitis?
Definition
Complications following GAS pharyngitis include acute rheumatic fever, post-streptococcal glomerulonephritis, and post-streptococcal reactive arthritis. Latency between GAS pharyngitis and onset of rheumatic fever and post-infectious glomerulonephritis have been suggested between 2 – 4 weeks and 10 days, respectively, from initial infection
Term
medulloblastoma- worse outcomes for oncogenes(3 of them)
Definition
most common of brain tumors(20%)Amplification of oncogene OTX2 has been described in association with meduloblastoma and overexpression of oncogenes ERBB2 and MYCC are linked to worse outcome
Term
HOw do you treat medulloblastoma?
Definition
complete resection and radiation and adjuvant chemo
Term
what chemo drugs do you use to treat?
Definition
Lomustine (CCNU) + vincristine + cisplatin
Term
cisplatin toxicity?
Definition
dealyed nausea and vomiting
nephrotoxic- prevent with mannitol and high rate ivfs(wastes phosphate, magnesium bicarb)
ototoxicity long term
Term
what is efs for medulloblastoma
Definition
at 5 years is 80%
Term
What is the nadir?
Definition
nadir is the day number post chemo(day1) that is the lowest point of the wbc after receiving chemo caused by bone marrow suppression
Term
what is neutropenia?
Definition
severe neutropenic ANC < 500(or WBC <0.5)
neutropenia ANC<1000
Term
what does cranial radiation put you at risk for long term?
Definition
-learning disabilities
- endocrine systems which includes thyroid and growth hormone
Term
What are short term toxicities from radiation?
Definition
-n/v(depending on where at- esp cranial and stomach/esophagus/abdomen)
-skin burns(no ointments to radiation areas)
-CNS toxicities-fatigue and sleepiness and lack of energy(kicks in about 6 weeks after- for TBI)
Term
What types of pediatric cancers do we do autologous stem cell transplants
Definition
-cancers treated are medulloblastoma, osteosarcoma, ewings sarcoma, neuroblastoma(solid tumors)- mainly stage 4(usually means multiple sites of metastases)
-use high dose chemo that would normally cause aplastic marrow so we give auto transplant after chemo- usually more than once we do this...
Term
Glioblastoma or Astrocytoma- where do they come from and what areas do they affect.
Definition
Astrocytomas rise from astrocyte cells present in the brain stem and spinal cord
Glioblastoma- from glial cells which surround neurons
VERY poor prognosis
Term
What are alkalating agents?(used for solid tumors alot and very bone suppressing)
Definition
Anthracyclines – daunorubicin, doxorubicin and idarubicin

2. Oxazophosphorines – cyclophosphamide and ifosfamide

3. Platinum analogues – cisplatin and carboplatin

4. Nitrosoureas – lomustine
Term
What are Anthracyclines side effects?– daunorubicin, doxorubicin and idarubicin
What drug to prevent it?
Definition
cardiomyopathy especially with accumulation of lifetime dose
daunorubicin= The incidence of irreversible myocardial toxicity increases as the total cumulative (lifetime) dosages approach 550 mg/m2 in adults, 400 mg/m2 in adults receiving chest radiation, 300 mg/m2 in children >2 years of age, or 10 mg/kg in children <2 years of age.
doxorubicin-The risk of cardiomyopathy increases with cumulative exposure and with concomitant cardiotoxic therapy; the incidence of irreversible myocardial toxicity increases as the total cumulative (lifetime) dosages approach 300 to 500 mg/m2 (with an every-3-week regimen
red-orange urine and radiation recall and secondary malignancies
Zinecard(Dexrazoxane) will help to protect the heart.
Term
What are side effects of methotrexate? and how do we protect the tissues from this toxicity?
Definition
Folate antagonists – methotrexate
mucositis, bone marrow suppression,rash(especially hands and feet peeling), renal toxicity
Term
What drug do we use to rescue the normal cells for patients receiving methotrexate? It's a vitamin
Definition
Leucovorin(Antidote, Methotrexate; Folic Acid Derivative)= only helps healthy cells because it bypasses the metabolic pathway that methotrexate blocks
Term
If you have severe delayed methotrexate excretion and subsequent renal toxicity what drug do we use to neutralize the methotrexate?
Definition
Glucarpidase (carboxypeptidase G2) - single dose . very expensive. mtx level not relevant for 48 hours after dose.
Term
What pediatric cancer is cytarabine most commonly treat and what side effect should you remember most for a f/n patient?
Definition
Term
What side effects do cyclophosphamide and ifosfamide have?
Definition
bladder cystitis- due to acrolein sitting bladder which causes hemorrhagic cystitis-can short or long term occurence(ie months after dosing)- to prevent - hydrate well so that patient empties their bladder every HOUR and MESNA!
Term
HOw does MESNA dose compare to cyclophosphamide dose?
Definition
60-120% of dose given before/during and after at 4 hour intervals- regimens vary alot but not really any right way to do it.
Term
What are procarbazine, darcarbazine and telezolomide used for?
Definition
Temozolomide is the oral equivalent of dacarbazine(transformed to MTIC [(methyl-triazene-1-yl)-imidazole-4-carboxamide] via the cytochrome P450 system
dacarbazine for hodgkins and solid tumors
procarbazine for hodgkins(avoid tyramine)
Term
How does methotrexate work? and how do we rescue?
Definition
Weak organic acid that acts as a “fraudulent substrate”

2. Inhibition of dihydrofolate reductase which deprives cells of the various folate co-enzymes and lead decreased biosynthesis of thymidylic acid and the purines (adenine and guanine), which then leads to decreased synthesis of DNA, RNA, and proteins

3. Inhibition can be reversed by a thousand-fold excess of the natural substrate (dihydrofolate = FH2) by the administration of leucovorin which bypasses the blocked enzyme and replenishes the folate pool
Term
What is the glucarpidase dose? how does it work? Can/should you give leucovorin at the same time?
Definition
Glucarpidase dose is 50 units/kg IV x 1. recombinant bacterial enzyme that metabolized antifolates
When patients have received glucarpidase, methotrexate levels can only reliably be measured using chromatographic methods for 48 hours after administration
Do not give leucovorin within two hours of the dose but you can continue using leucovorin to rescue the tissue in case of leakage from 3rd space places.
Term
How does ARA C work? WHat is the neurotoxicity associated with it?What infection is associated with high dose cytarabine and what abx do you need for AML patients?
Definition
Most active in the S phase but can halt the progression of cells from G1 to S phase

2. Metabolized by deoxycytidine kinase to Ara-CTP (rate limiting step)

3. Ara-CTP is a competitive inhibitor of DNA polymerase after being incorporated into a DNA chain  halts chain elongation
. Signs/symptoms: slurred speech, personality changes, unsteady gait, problems with writing, tremor, somnolence, etc.
realize that fevers are common side effect DURING arac infusion- so if fever while receiving arac for AML(fever myalgia and bone pain) may not need antibiotics
. strept viridans associated infections so use vanco for fever/neutropenia
Term
What are side effects 6-mercaptopurine and 6- thioguanine( Also azathioprine)?
Definition
Liver toxicity and bone marrow suppression and mucositis.
Give in evening on an empty stomach.
risk of relapse was 2.56 times higher when mercaptopurine was given in the morning compared to at night so give at night!!

thiopurine methyltransferase (TPMT) polymorphisms affect toxicity profile (myelosuppression) and drug dosing
Term
What drug interactions do you have to worry about for methotrexate?
Definition
no PPI during administration especially for high dose, no bactrim d/t delayed excretion, and no folic acid in vitamin and no and no nsaids d/t reduced clearance of mtx and no amoxicillin due to reduced clearance of methotrexate.
Term
Why do we use high dose methotrexate?
Definition
High concentration of MTX allow greater amounts of passive diffusion which can potentially overcome drug resistance by decreasing reliance on active transport across the cell membrane a lower doses

b. Increased intracellular concentrations of MTX overcomes drug resistance secondary to increase dihydrofolate reductase

c. May promote increased MTX polyglutamation resulting in more prolonged MTX action

d. Leucovorin competes with MTX for active transport into cells (limiting entry of MTX) and intracellular leucovorin replenishes reduced folate stores and competes with methotrexate
Term
What are side effects of etoposide infusion and what do you do to prevent this?
Definition
DIlution of the infusion and extend the infusion if hyptoension, and/or allergic reactions occur and can add premeds of benadryl, acetaminophen and consider steroids. allergic reaction infusion related due to tween 80 for IV diluent so Etopophos is the water soluble formulation so if people have allergic to etoposide can switch to etopophos.
Term
What other long term side effects do you see?
Definition
liver toxicity and secondary leukemias. and will use oral for prolonging life to keep cancer at bay that isn't responding or stopped responding to conventional chemo(this is palliative chemo).
Term
What cancers do we use etoposide to treat?
Definition
solid tumors and AML
Term
HOw do vincristine and vinblastine work?
Definition
work at microtubules so neurotoxicity is main side effect( peripheral neuropathies and constipation(slows down bowel so need stool regimen). and 3A4 substrate so consider drug interactions.
Term
What cancers is vincristine/vinblastine used for?
Definition
both leukemias (especially ALL) and solid tumors and lyumphomas
Term
What chemotherapy drugs cause SIADH?
Definition
cyclophosphamide and vincristine
Term
What type of tumors are topotecan and irinotecan used for? WHat are main side effects?
Definition
relapsed ALL and AML, ovarian cancer, solid tumors.
Diarrhea that you need loperamide to treat or atropine because it can life threatening d/t severity so should give loperamide at first sign of diarrhea and give scheduled and cefpodoxime or other oral ceph to prevent typhilits and neurotoxicities(neuropathies that can be severe and irreversible)
Term
What are side effects of asaparaginase? How do we give now? what if you have an anaphylaxis?What is it used to treat?
Definition
side effects are hyperglycemia, local reaction if given IM, pancreatitis, clotting disorders(blood clots), hyperammonia, not as much nausea/vomiting but can , don't give on same day as vincristine d/t increased neurotoxicities for both . Treats leukemia especially ALL
Term
WHat are these used for-bleomycin?
Definition
hodgkins lymphoma and germ cell cancers.
concern for pulmonary toxicities.
Term
What cancer is dactinomycin used for? what are side effects?
Definition
for solid tumors like osteosarcoma,ewings and wilms tumor
radiation recall and hepatoxicity
Term
What is hydroxyurea used for? What are toxicities?
Definition
solid tumors, sickle cell anemia, and CML
Side effects are GI, pancreatitis, and anemias.
Term
What is rituximab used for?
Definition
non-hodgkins lymophoma, autoimmune disorders, and lyphoproliferative disease. Can lyse cells quickly so can cause tumor lysis synderome especially in the lymphoma patients.
Term
What are rituximab side effects?
Definition
infusion related which can be life threatening especially for first infusion so slow rate titration, HA and infection related side effects d/t long term suppression of Bcells(months). IT binds to CD 20 antigens.
Term
What is brentuximab used for ? What are toxicities?
Definition
CD 30 specific
to treat hodgkins lymphoma.
pulmonary toxicities, peripheral neuropathies.
Term
What is dintuximab and where does it work and for what cancers? what are the side effects? Used in combo with GMcsf and IL-2
Definition
binds to disialoganglioside GD2, which is highly expressed in neuroblastoma cells so used as immunotherapy in combination IL 2.
Can cause severe pain and neuropathies during the infusion so use Pain meds and PCA. then can cause hypotension, capilllary leak syndrome and neurotoxities like blindness, and electrolyte toxicities and SIADH protocol has a huge decision tree based on these toxicities and what to do including stopping the infusion and never using again if need be.
Term
What is All-trans retinoic acid (ATRA, tretinoin, Vesanoid) used for? what are side effects?
Definition
Acute promyelocytic leukemia (APL). concern for “Retinoic-Acid Syndrome” (occurs in about 25% of patients) which is increased wbc, fever resp problems, hypotension and can use steroids to treat.
Term
What is Isotretinoin (CRA, 13-cis-retinoic acid, Amnesteem®, Claravis®, Sotret®, Myorisan™, Absorica™, Zenatane™)
Definition
for neuroblastoma
side effects are headaches, photosensitivity and liver toxicity. and REMS program d/t teratogenecity
Term
What are tyrosine kinase inhibitors and what are they used for
Definition
Imatinib...and damatinib-inhibits BCR-ABL tyrosine kinase, the genetic aberration associated with Philadelphia chromosome positive for ALL and CML
Term
For Imatinib- dosing and side effects
Definition
Myelosuppression

b. Fluid retention/edema

c. Diarrhea

d. Rash

e. Fatigue

f. Rarer - heart failure, hepatotoxicity

4. Comments and clinical pearls

a. Give in one or two daily doses with food
Term
Dosing for Dasatinib?What extra dose it do?
Definition
SRC kinase inhibitor too. QTC prolonger! Myelosuppression

b. Headache

c. Pleural/pericardial effusions

d. Pulmonary arterial hypertension

e. QT prolongation

f. QT prolongation

g. Give in one daily dose with or without food
Term
Which tumor is common for neurofibromatosis?
Definition
Up to 20% of children with neurofibromatosis will have a CNS tumor, typically tumors affecting the optic nerve and visual pathway. Half of all optic nerve tumors occur in children with neurofibromatosis. While these children may also have other types of CNS tumors, they are typically low-grade gliomas and usually not malignant.
Term
What are sypmtoms of medulloblastoma?
Definition
The cerebellum is involved in a number of functions including motor movement, coordination, language comprehension and concentration. Facial muscles are the first to be affected, leading to vision issues, and uncontrollable spasms in the lips, eyelids, and cheeks. Impaired coordination of the legs, motor movements in the arms and fingers, as well as mental confusion, speech problems and behavioral outbursts may occur as other areas of the brain are affected.n/v, headaches truncal instability, papilledema, clumsiness and attention problems(declining academic performance)
Term
WHich child has poorer prognosis for epnedymoma?
Definition
Younger age confers a generally poorer prognosis, as does an incomplete resection of the tumor. While anaplastic histology is a poor predictor of survival, a complete resection generally improves prognosis. Tumors of the cranium generally fare worse than those of the spinal cord.
Term
What is fanconi's syndrome?What drug can cause it(especially in cumulative doses)
Definition
Fanconi syndrome is a disorder of the kidney tubes in which certain substances normally absorbed into the bloodstream by the kidneys are released into the urine instead.ifosfamide
Term
When should the uroprotectant mesna be utilized in pediatric patients?
Definition
Only for cyclophosphamide doses ≥ 1 gm/m2 and for all ifosfamide doses
Term
Which drug is associated with VOD?
Dactinomycin
Dacarbazine
Procarbazine
Lomustine
Definition
dactinomycin
Term
Which tumor cell contains rosettes?
Definition
his rosette (seen in – you guessed it – ependymoma) consists of tumor cells surrounding an empty lumen. It is thought that these structures represent attempts by the tumor cells to recreate little ventricles with ependymal lining. One thing to note: although these guys are characteristic of ependymoma, they’re not seen in every case. In fact, it’s fairly uncommon to find them at all (they’re only present in a small percentage of well-differentiated
Term
A 6-year old female has slowly progressive cranial neuropathy, motor weakness and speech and swallowing disturbances. She is diagnosed with DIPG. What role does chemotherapy play in her treatment?

It is first line treatment
It is used in conjunction with radiation
It is investigational
It is not recommended
Solution:
Definition
The standard for treatment of DIPG is radiation therapy, except in younger children, where chemotherapy may be used to try to delay or avoid the use of radiation, due to adverse long-term effects. No chemotherapeutic regimen has demonstrated long-term survival for these patients, even when used with radiation, high-dose marrow-ablative chemotherapy with autologous stem cell rescue. However, there are still investigational regimens under study.
Term
WHo should you NOT irradiate for tumors?
Definition
Patients less than 3 years old.
Term
What is primary initial treatment for medulloblastoma
Definition
decrease ICP with vp shunt
Term
What are main bleeding sites for hemophilia?
Definition
There are six major sites of serious bleeding which could potentially threaten life, limb or function and they are: intracranial, spinal cord, throat, intra-abdominal, limb compartments, ocular. These require immediate assessment and intervention due to bleeding within a closed space, potential compression of vital organs, or potential loss of life, limb or function. While gum bleeding may be serious, especially after tooth extraction/oral surgery, the gums do not meet the above requirements.
Term
. JC is a 12-year old male with severe hemophilia A, who was hit in the mouth with a baseball and has mucocutaneous bleeding from his mouth and gums. His weight is 46kg. His current FVIII level is < 1%. Calculate his on-demand dose of FVIII.
Definition
Since his current level is < 1% and our goal is FVIII of 50%, we would use the FVIII calculation of “Units of FVIII required = weight (kg) x desired level (%) x 0.5”, therefore, 46kg x 50(%) x 0.5 = 1150 IU. Answer B does not use the 0.5 factor for FVIII. Answer C is the result of the use of the FIX calculation for children under 15 years of age, and Answer D uses a desired level of 100%.
Term
What types of cells does neuroblastoma, ganglioneuroblastoma and glanglionereuroma come from?
Definition
nlike neuroblastic tumors, which are derived from neuroblasts. neuroblastoma is most likely to occur before the age of 5 years. There is a slight male predominance with the disease and no significant racial or ethnic disparities have been noted in its incidence after the first year of life. Although CNS tumors are the most common solid tumors to occur in children, neuroblastoma is the most common extracranial solid tumor to affect peds but brain tumor is second most common tumor after leukemia.
Term
For COG what are considered part of the diagnosis testing for neuroblastoma? What other test can be included?
Definition
hromosome 11 aberrations are not considered within the risk stratification schema that the COG uses for patients with neuroblastoma unlike MYCN oncogene status, DNA index and tumor histology. In the revised risk group stratification developed by the INRG, chromosome 11 aberrations along with grade of tumor differentiation are used to stratify patients.
Term
What is the treatment dosing of the isotretionin for neutroblastoma?(brand namesbsorica; Amnesteem [DSC]; Claravis; Myorisan; Zenatane)
Definition
160 mg/m2/day orally BID for 14 consecutive days followed by 14 consecutive days of rest. This regimen is typically given for 6 cycles following autologous HSCT in patients with high-risk neuroblastoma
Term
Opsomyoclonus-ataxia syndrome occurs in about 2-3% of patients with neuroblastoma. Which of the following therapies has shown encouraging results in the management of this syndrome to date?
Definition
rituximab
Term
Why do Hemophilia A and B occur (are genetically inherited X-linked recessive bleeding disorders resulting)
Definition
from decreased or absent circulating levels of functional factor VIII (FVIII)=A or factor IX (FIX)=B, respectively.
Term
which is more common hemophilia A or B?
Definition
A is more common at 80% of the disorders
Term
What chromosome is the gene for hemophillia on ? What are the common abnormalities?
Definition
X chromosome. FOr A(factor 8) = intron 22 inversion. for B missense mutations are more common
Term
severity of hemophillia depends on percentage of circulating factor. what is normal percentage?
Definition
100percent is normal. mild is5-40% so then there is prolonged bleeding for severe trauma or surgery. for mod 1-5%spontaneous bleeding is rare but with trauma there could be bleeding and then 1% activity= spontaneous bleeds are probable(70% A's have severe form and 50% of Bs
Term
in hemophillia what happens to the PTT
Definition
it is significantly prolonged
Term
for Hemophillia A , what does desmopressin do
Definition
Releases bound FVIII into the systemic circulation, elevating the concentration available for coagulation.

2. Useful for home treatment of minor bleeds

3. Tachyphlaxis may occur with frequently repeated doses.

4. Monitor for hyponatremia/water intoxication: may require restriction of fluids.

5. Not recommended for children less than 2 years of age
Term
what does aminocaproic acid do
Definition
Aminocaproic acid

1. For treatment of excessive bleeding with minor injury/procedures

2. Loading dose should be used
Term
what is tranexamic acid work for ?
Definition
use only for active bleeds that are for minor dental workk or surgery
Term
WHat is best treatment for hemophillias?
Definition
he basis of treatment for hemophilia is replacement of the deficient factor.

1. Current practice uses recombinant DNA technology to produce pure factor concentrates.

2. Use of factor has dramatically increased life expectancy and quality of life

3. Dose is based on the bleeding site and patient’s weight.

1. Desired percent correction of factor activity will vary.
Term
HOw do you calculate at F8 dose?
Definition
1 IU/kg will raise serum FVIII activity by 2%

2. Units of FVIII required = weight (kg) x desired level (%) x 0.5
Term
HOw is factor 1Xdosed?
Definition
FIX

1. Binds to sites in endothelium, therefore more factor is required to achieve the same rise in activity as FVIII.

2. 2 IU/kg will raise serum FIX activity by 2%

3. Units of FIX required = weight (kg) x desired level (%) x 1

a. If using recombinant FIX (rFIX) a dose adjustment of 1.2 for adults and 1.4 for children is required.
Term
HOw often is factor 8 given(hemophillia A)?
Definition
based on half life so VIII: half-life of 3-6 hours during initial equilibration into extravascular spaces, then becomes closer to 12 hours. Dose initially at every 8 hours until good hemostasis, then extend to every 12 hours, then every 24 hours as the patient recover
Term
HOw often is factor IX dosed?
Definition
FIX: half-life 18-24 hours, so once daily dosing is typical unless surgical or severe trauma where twice daily dosing may be required.
Term
Why is continous infusion Factor 8 used?
Definition
Continuous infusions of FVIII are commonly used to avoid peaks/troughs of activity as well as reduce the overall factor consumption in moderate and severe bleeds. Infusion rates of 2-3 IU/kg/hr may maintain activity level around 50-60%.
Term
What can cause serious complications in the treatment of hemophillia?
Definition
formation of antibodies against FVIII and FIX. Antibodies will neutralize the procoagulant activity of the factor which leads to treatment failure
Term
When and who is highest risk for developing inhibitors in hemophillia
Definition
2. Highest risk of development of inhibitors occurs within the first 50 exposures to FVIII, with risk decreasing after 200 treatment days.

3. Risk factors for development of inhibitors include genetic factors, family history of inhibitors and African heritage. Inhibitors may also develop after intensive factor exposure or immunological challenge.
Term
how is inhibitor levels measured
Definition
Inhibitors are measured by the Bethesda assay: one Bethesda unit (BU) is the amount of inhibitor needed to inactivate 50% of FVIII or FIX in pooled normal plasma.

2. Low-responding: may still respond to factor replacement therapy at higher doses or more frequent dosing intervals. They have a peak titer of less than 5 BU/mL.
Term
Systemic OnsetJIA
Definition
Arthritis in 1 or more joints, with or preceded by fever of at least 2 weeks duration, documented as daily for at least 3 days and with 1 or more of the following:

Rash (evanescent)
Lymphadenopathy
Hepatomegaly or splenomegaly
Serositis
Onset: childhood
1-17%
Term
Oligoarticular JIA (joints= number of I's)
Definition
Arthritis affecting 1-4 joints during the first 6 months of disease (usually lower extremities) 27-56%
Affects females more often than males (3:1)

Persistent – affects no more than 4 joints throughout disease course
Extended – affects more than 4 joints after the first 6 months of disease
Onset: early childhood; peak 2-4 years
Term
Polyarthritis (RF-negative)
Definition
Arthritis affecting 5 or more joints in the first 6 months of disease; RF test = negative

Onset: biphasic peaks at 2-4 years and 6-12 years 11-28%
Term
Polyarthritis (RF-positive)= adult almost like!
Definition
Arthritis affecting 5 or more joints in the first 6 months of disease; RF test = positive on at least 3 occasions that are 3 months apart

Onset: late childhood or adolescence
2-7%
Term
Dactylitis
Definition
Dactylitis or sausage digit is inflammation of an entire digit (a finger or toe), and can be painful.
Term
Psoriatic
Definition
Arthritis and psoriasis; or arthritis and 2 or more of the following

Dactylitis
Nail pitting or onycholysis
Psoriasis in a first-degree relative 2-11%
Term
Enthesitis-related
Definition
Enthesitis is inflammation of the entheses, the sites where tendons or ligaments insert into the bone. It is also called enthesopathy, or any pathologic condition involving the entheses.
Term
Enthesitis-related
Definition
Arthritis and enthesitis, or arthritis or enthesitis with at least 2 of the following:

Sacroiliac joint tenderness or lumbosacral pain
Presence of HLA-B27 antigen
Onset of arthritis in male > 6 years old
Acute anterior uveitis Uveitis is a form of eye inflammation. It affects the middle layer of tissue in the eye wall (uvea)
First degree relative with HLA-B27 associated disease
Term
undifferentiated JIA
Definition
Undifferentiated

Arthritis that fulfills criteria for no category or 2 or more categories 11-21%
Term
JIA
Definition
Juvenile idiopathic arthritis (JIA) is defined by the International League of Associations for Rheumatology (ILAR) as arthritis of unknown etiology that begins before the sixteenth birthday and persists for at least 6 weeks with other known conditions exclude
Term
WHat are poor prognosis for arthritis JIA
Definition
Features of poor prognosis (must satisfy 1)
 Arthritis of the hip (23---25) or cervical spine
 Arthritis of the ankle (25---27) or wrist (26,28) AND marked (29) or prolonged (23,25,26,29,30) inflammatory marker elevation
 Radiographic damage (erosions or joint space narrowing by radiograph) (31)
Term
WHat is the definition of NSAID
Definition
nonsteroidal antiinflammatory drugs used commonly in clinical practice in the US and includes selective cyclooxygenase 2 inhibitors but not aspirin.
Term
what are calcineurin inhibitors?
Definition
cyclosporine and tacrolimus.
Term
what are the tumor necrosis factors?
Definition
umor necrosis factor α (TNFα) inhibitors refer to adalimumab, etanercept, and infliximab.
Term
are Glucocorticoid joint injections recommended for JIA
Definition
he use of glucocorticoid joint injections for active arthritis was recommended, regardless of concurrent therapy (no DMARD, nonbiologic DMARD, or biologic DMARD) or JIA treatment group (level C)
Term
What drug should be used for the glucocorticoid joint injections?
Definition
Glucocorticoid joint injections should be performed with triamcinolone hexacetonide, owing to its demonstrated superior efficacy
Term
HOw long do the glucocorticoid injections for JIA work
Definition
Intraarticular glucocorticoid injections are expected to result in clinical improvement of arthritis for at least 4 months
Term
Can methotrexate be used with tumor necrosis factors?
Definition
Continuing methotrexate when initiating a TNFα inhibitor (etanercept or adalimumab) was recommended for patients who had a partial previous clinical response to methotrexate
Term
for h/o 4 or fewer joints for JIA
Definition
Treatment recommendations for patients with a history of arthritis of 4 or fewer joints. These recommendations are intended for patients with juvenile idiopathic arthritis (JIA) who have only developed active arthritis in 4 or fewer joints in total throughout the history of their disease course and are based upon duration of current therapy, disease activity, and features of poor prognosis. If criteria for escalation of therapy are not met, then continue current therapy along with adjunct nonsteroidal antiinflammatory drugs (NSAIDs) or glucocorticoid joint injections, as needed. Recommendations for reduction of therapy are not addressed. See Table 1 for definitions of disease activity and features of poor prognosis. * = sulfasalazine may be an appropriate treatment for patients with the enthesitis-related arthritis category of JIA (see text for details); MTX = methotrexate; TNFα = tumor necrosis factor α.
Term
How long can steroid monotherapy be continued?
Definition
Continuation of NSAID monotherapy (without additional therapy) for longer than 2 months was inappropriate for patients with active arthritis, irrespective of poor prognostic features.
Term
When should MTX be initiated
Definition
nitiation of methotrexate was recommended as initial treatment (without prior therapy) for patients with high disease activity and features of poor prognosis
Term
When do you add sulfasalazine and for who
Definition
nitiation of sulfasalazine was recommended following glucocorticoid joint injection or an adequate trial of NSAIDs for patients with the enthesitis-related arthritis category of JIA with moderate or high disease activity, irrespective of features of poor prognosis (level B) (84). Initiation of sulfasalazine was uncertain for patients who are not diagnosed with the enthesitis-related arthritis category of JIA.
Term
What conditions do you start tumor necrosis factors in
Definition
nitiation of a TNFα inhibitor was recommended for patients who have received glucocorticoid joint injections and 3 months of methotrexate at the maximum tolerated typical dose and have moderate or high disease activity and features of poor prognosis (level C) (10,15). Initiation of a TNFα inhibitor was also recommended for patients who have received glucocorticoid joint injections and 6 months of methotrexate and have high disease activity without features of poor prognosis (level C) (10,15).

Additionally, initiation of a TNFα inhibitor was recommended for patients specifically with the enthesitis-related arthritis category of JIA who have received glucocorticoid joint injections and an adequate trial of sulfasalazine (without prior methotrexate) and have moderate or high disease activity, irrespective of prognostic features (level C) (11,85).
Term
When do you use abatecept
Definition
nitiation of abatacept was uncertain prior to initiation of a TNFα inhibitor.
Term
When do you use Hydroxychloroquine for JIA
Definition
Initiation of hydroxychloroquine monotherapy (with or without concurrent NSAIDs) was inappropriate for patients with active arthritis (level C
Term
With 5 or more joints what is that treatment scenario
Definition
reatment recommendations for patients with a history of arthritis of 5 or more joints. These recommendations are intended for patients with juvenile idiopathic arthritis who have developed active arthritis in 5 or more joints in total throughout the history of their disease and are based upon duration of current therapy, disease activity, and features of poor prognosis. If criteria for escalation of therapy are not met, then continue current therapy along with adjunct nonsteroidal antiinflammatory drugs (NSAIDs) or glucocorticoid joint injections, as needed. Recommendations for reduction of therapy are not addressed. See Table 2 for definitions of disease activity and features of poor prognosis. * = leflunomide may be an appropriate treatment alternative (see text for details); MTX = methotrexate; TNFα = tumor necrosis factor α.

Images in this article
Term
What is recommended initial treatment for 5 or more joints?
Definition
Methotrexate
Initiation of methotrexate was recommended as initial treatment for patients with high disease activity, irrespective of poor prognostic factors, and for patients with moderate disease activity and features of poor prognosis (level B) (40,77---80). Following approximately 1 month of NSAIDs, initiation of methotrexate was recommended for patients with low disease activity and features of poor prognosis (level B) (40,77---80). Following approximately 1 to 2 months of NSAIDs, initiation of methotrexate was recommended for patients with moderate disease activity without features of poor prognosis (level B) (40,77-
Term
What is the next to do after methotrexate
Definition
The TFP generally favored the use of methotrexate over leflunomide, owing to greater personal and collective experience with methotrexate. However, initiation of leflunomide was recommended as one treatment approach as initial treatment for patients with high disease activity and features of poor prognosis (level B) (77). Following a brief trial of NSAIDs, initiation of leflunomide was recommended as one treatment approach for patients with high disease activity without features of poor prognosis and for patients with moderate disease activity with features of poor prognosis (level B) (77).

TNFα inhibitors
Initiation of a TNFα inhibitor was recommended for patien
Term
When do you use tumor necrosis factors
Definition
nitiation of a TNFα inhibitor was recommended for patients who have received methotrexate or leflunomide for 3 months at the maximum tolerated typical dose and have moderate or high disease activity, irrespective of poor prognostic features (level B) (10,15). Initiation of a TNFα inhibitor was also recommended for patients who have received methotrexate or leflunomide for 6 months and have low disease activity, irrespective of poor prognostic features (level B)
Term
WHat is kawasaki's disease?
Definition
- self limited vasculitis
-most acquired heart disease in kids
-Coronary artery aneurysms develop in 3-5% of treated patients and up to 25 % of untreated patients.
Term
Kawasaki's disease - how is it thought that it occurs?
Definition
bacterial superantigen toxin
triggered by microorganisms and subsequent immunologic response
Term
What is the heritage basis for KD?
Definition
japanese 32.5% african american 16.9 hispanics 11.1 caucasian 9.1 more male than female and more in winter and spring 76% before age 5
Term
KD can be amongs twins and family members- true or false
Definition
true
Term
what are sypmtoms of KD for diagnosis?
Definition
ever present for ≥ 5 days plus ≥ 4 of the following principal findings:

1. Rash (polymorphous)

2. Bilateral conjunctivitis (non-exudative)

3. Oropharyngeal changes (strawberry tongue, erythematous or cracked lips, diffuse erythema of oropharyngeal mucosa)

4. Cervical lymphadenopathy (usually unilateral, ≥ 1.5 cm)

5. Extremity changes (acute: erythema of palms and soles, edema of hands and feet; subacute: periungual desquamation)

b. Fever present for ≥ 5 days, < 4 principal findings, and coronary artery disease detected by 2D echocardiography or coronary angiography
Term
awhat is the prmiary way to diagnose CF?
Definition
sweat chloride test result >60 on two occasions.
Term
What is tobra nebulizers approved for?
Definition
maintenance for 28 day cycles for suppression of pseudomonas mucoid in cf
Term
What is Ivacaftor for?
Definition
It's called a potentiater because it increases the chance that th CFTR channges open it will improve secretions within two weeksPotentiates epithelial cell chloride ion transport of defective (G551D mutant) cell-surface CFTR protein thereby improving the regulation of salt and water absorption and secretion in various tissues (eg, lung, gastrointestinal tract)
Term
Which patient can you not use DPI's in
Definition
Patient has a severe milk allergy and DPI formulations can lead to severe allergic reaction as the formulation has milk protein
Term
What symptoms are most suggestive of croup?
Definition
barking cough, inspiratory stridor, and hoarseness is most suggestive of croup. Tachypnea, hypoxia, and retractions can be present with croup, but this child has a normal respiratory rate, normal oxygen saturation, and is not in respiratory distress. He has a low-grade fever which can be found in croup, but symptoms of upper airway inflammation are usually present, such as cough or stridor. Also common is a prodrome consistent with a viral upper respiratory tract infection, such as nasal congestion (as in this child), rhinorrhea, and pharyngitis.
Term
what is the normal budesonide dose?
Definition
NIH Asthma Guidelines (NAEPP 2007): Administer once daily or in divided doses twice daily

Children ≤4 years:

"Low" dose: 0.25 to 0.5 mg/day

"Medium" dose: >0.5 to 1 mg/day

"High" dose: >1 mg/day

Children 5 to 11 years:

"Low" dose: 0.5 mg/day

"Medium" dose: 1 mg/day

"High" dose: 2 mg/day
Term
what is the dosing steps for fluticasone inhaler?
Definition
Asthma Guidelines: National Asthma Education and Prevention Program guidelines (NAEPP 2007): HFA inhaler (refers to Flovent HFA 44 mcg, 110 mcg, and 220 mcg strength): Note: Administer in divided doses twice daily:

"Low" dose:

Infants and Children ≤4 years: 176 mcg/day

Children 5 to 11 years: 88 to 176 mcg/day

Children ≥12 years and Adolescents: 88 to 264 mcg/day

"Medium" dose:

Infants and Children ≤11 years: >176 to 352 mcg/day

Children ≥12 years and Adolescents: >264 to 440 mcg/day

"High" dose:

Infants and Children ≤11 years: >352 mcg/day

Children ≥12 years and Adolescents: >440 mcg/day
Term
What is the dosing steps for ciclesonide?
Definition
Asthma guidelines: Global Initiative for Asthma guidelines (GINA 2015): Note: Other expert guideline recommendations suggest that doses >200 mcg/day may provide minimal additional benefit while increasing risks for adverse events; add-on therapy should be considered prior to dose increases >200 mcg/day (Lougheed 2010).

Children ≤5 years: "Low" dose: 160 mcg/day

Children 6 to 11 years:

"Low" dose: 80 mcg/day

"Medium" dose: >80 to 160 mcg/day

"High" dose: >160 mcg/day

Children ≥12 years and Adolescents:

"Low" dose: 80 to 160 mcg/day

"Medium" dose: >160 to 320 mcg/day

"High" dose: >320 mcg/day
Term
what are steps for mometasone?
Definition
NIH Asthma Guidelines (NAEPP, 2007) (give in divided doses): Note: 220 mcg inhaler delivers 200 mcg mometasone furoate per actuation; NAEPP uses doses based on delivery, while manufacturer recommended doses are based on inhaler amount; Children ≥12 years and Adults:

"Low" dose: 200 mcg/day (200 mcg/puff: 1 puff/day)

"Medium" dose: 400 mcg/day (200 mcg/puff: 2 puffs/day)

"High" dose: >400 mcg/day (200 mcg/puff: >2 puffs/day)
Term
What are primary symptoms of bronchiolitis?
Definition
The other components (tachypnea, wheezing, and cough) are central features of bronchiolitis and lower respiratory tract viral infections in children younger than 2 years. Bronchoconstriction is primarily found in asthma exacerbations which can be triggered by viral infections. Bronchiolitis can occur in children with pre-existing asthma, leading to bronchoconstriction in hyperreactive airways, but bronchiolitis does not commonly cause bronchoconstriction in children without asthma.
Term
HWat are the most common viral infections in patients <12 months of age?
Definition
RSV is the most common cause of bronchiolitis in children younger than 12 months. Immunity from RSV infection is not permanent and reinfections are common throughout life. Rhinovirus is the second most common cause of bronchiolitis in infants while influenza is less common. Atypical bacteria rarely cause bronchiolitis in infants and are usually found only in coinfections with viruses.
Term
what is the general cutoff for supplemental oxygen per AAP?
Definition
Data are lacking to support the use of a specific SpO2 cutoff value. The American Academy of Pediatrics practice guideline suggests SpO2 <90 percent as the threshold to start supplemental oxygen
Term
What type of pulmonary therapy is used for severe bronchiolitis to try to prevent intubation?
Definition
Heated humidified high-flow nasal cannula (HFNC) therapy and/or continuous positive airway pressure (CPAP) are used to reduce the work of breathing, improve gas exchange, and avoid the need for endotracheal intubation in children with bronchiolitis who are at risk for progression to respiratory failure [38
Term
what are risks for severe or complicated bronchiolitis/
Definition
Prematurity (gestational age <37 weeks)
●Age less than 12 weeks
●Chronic pulmonary disease, particularly bronchopulmonary dysplasia (also known as chronic lung disease)
●Congenital and anatomic defects of the airways
●Congenital heart disease
●Immunodeficiency
●Neurologic disease
Environmental and other risk factors, such as passive smoking, crowded household, daycare attendance, concurrent birth siblings, older siblings, and high altitude (>2500 meters) can also contribute to more severe disease
Infants with Down syndrome have several risk factors for severe bronchiolitis: congenital heart disease, anatomic abnormalities of the respiratory tract, muscle dystonia, and immune dysfunction. While prematurity can be a risk factor, this child was not born at < 29 weeks gestation which is associated with high risk of severe disease. Family history of asthma in a parent places the child at risk for asthma or viral-induced wheezing but not bronchiolitis. Breastfeeding for at least 6 months reduces the risk for respiratory infections and bronchiolitis in previously healthy infants, but formula feeding is not a strong risk factor for disease severity
Term
ONce an infant on synagis has an rsv infection should they continue their synagis for the rest of the season?
Definition
no
Term
what are the guidelines for rsv prophy?
Definition
ontinuation of monthly palivizumab is not recommended after a child is hospitalized for RSV infection because the likelihood of a second RSV hospitalization in the same season is highly unlikely, even if the child remains in a high-risk population during the RSV season. In the United States, RSV outbreaks begin in November/December, peak in January/February, and end by late March/April, depending on geographical location. Palivizumab prophylaxis is given monthly (to a maximum of 5 doses/season) beginning in October or November and continuing through February or March. Although passive smoke exposure is a risk factor for bronchiolitis, it is not a high-risk category that would prompt palivizumab use without the presence of other criteria. Even though this child remains at high-risk for bronchiolitis (gestational age 28 weeks and postnatal age less than 12 months) and is exposed to passive smoke, he is unlikely to require hospitalization from RSV again this season; therefore, palivizumab prophylaxis should be discontinued.
Term
In a 9month old with acute OM and UR symptoms, what vaccines has he gotten so he won't have that type of infection and what is a common bug?
Definition
The pneumococcal and Hib vaccination series are likely to have started in this patient. Recent literature suggests a trend toward non-typeable Haemophilus influenzae as most likely bacterial cause in AOM. Klebsiella species may be expected in patients with otitis externa, but would not be expected in AOM. Viral processes often precede AOM, and these can lead to ET inflammation and dysfunction. However, the causative role of viruses, including RSV, in AOM is unclear.
Term
What is most predictive of AOM?
Definition
013 AAP guidelines for the diagnosis and management of AOM describe impaired TM mobility as most predictive for AOM with a 95% sensitivity and 85% specificity. Cloudiness and bulging TM are highly predictive for AOM, but with lower combined sensitivity and specificity than impaired mobility. Children pulling/tugging at ear has low sensitivity and specificity.
Term
IN the 2006 meta analysis for AOM who benefited the most from abx?
Definition
2013 AAP and American Academy of Family Physicians (AAFP) guideline recommends immediate treatment for children <6 months, children with severe signs or symptoms (defined by moderate or severe ear pain, ear pain for ≥48 hours, or temperature ≥39°C [102.2°F]) and bilateral AOM in children <24 months of age [3]. The 2013 AAP/AAFP guideline recommends either immediate treatment or observation (with pain control) for children between 6 and 24 months with unilateral nonsevere AOM and for children ≥24 months with unilateral or bilateral nonsevere AOM. However, given the additional analysis now available showing a high rate of treatment failure among children <24 months with unilateral nonsevere AOM [20], we suggest that such children be treated with antimicrobial therapy.

Guidelines from many other countri
Term
what is most predictive of AOM in children?
Definition
013 AAP guidelines for the diagnosis and management of AOM describe impaired TM mobility as most predictive for AOM with a 95% sensitivity and 85% specificity. Cloudiness and bulging TM are highly predictive for AOM, but with lower combined sensitivity and specificity than impaired mobility. Children pulling/tugging at ear has low sensitivity and specificity.
Term
what patients should receive close observation and wait to start abx if AOM?
Definition
Observation with close follow-up is recommended by the 2013 AAP guidelines for children 6 – 23 months unilateral AOM without severe symptoms. Amoxicillin would not be inappropriate, however observation does not increase complications and results in similar parent satisfaction scores compared with antibiotics. Observation has been suggested to potentially decrease antibiotic use, which would limit children to potential antibiotic adverse effects. Amoxicillin/clavulanate would be inappropriate if this child were prescribed antibiotics because the child has no history of recent antibiotic use. Similarly, cefdinir would be inappropriate since this child has no known allergies.
Term
what are most common organisms for CLABSI in children?
Definition
Coagulase-negative staphylococcus and S. aureus are most common bacterial causes of CLABSI in children. Group A beta-hemolytic streptococci would not be expected to be associated with CLABSI. This patient does not have risk factors for fungal CLABSI, including but not limited to prolonged ICU stay, TPN receipt, or previous fungal CLABSI without catheter removal. Based on what is known of the patient’s clinical course, P. aeruginosa, while possible, would not be a higher probability than Coagulase-negative staphylococcus.
Term
When can you start treatment for kawasakis disease and why?
Definition
Ivig 2gm/kg but wait till day 5 because it's been found that the fever remains persistent and recrudescence that requires treatment
Term
Why a single high dose Ivig for is instead of multiple lowere doses?
Definition
Because the single high dose showed lower incidence of CAA
Term
What does the high dose aspirin do?
Definition
High dose for 48-72 hours after Afeb but this is controversial and not universal then low dose for 6-8 weeks until repeat echo. No studies have shown a reduction inCAA
Term
What are the symptoms for KD
Definition
CRASH and BURN conjunctivitis rash adenoids they strawberry tongue hands and feet burn for fever
Term
What monoclonal antibody is used to treat kawasakis disease?
Definition
Abciximab binds to platelet glycoproteins IIB/iiiCa receptors to prevent platelet aggregation. It should be used in the acute phase of a CAA is already formed
Term
What are long term consequences of CAA?
Definition
Stenosis thrombosis and vessel ruoture
Term
Who is a most risk for coronary artery abnormalities?
Definition
If there is evidence of changes in the coronary artery on initial presentation
Term
What is a z score?
Definition
Is how far away the number is from the mean of a normal distribution based on standard deviations.
Term
What happens when there is treatment failure to Ivig for KD
Definition
Continued fever past 48 hours after Ivig dose or recrudescence fever which is a temp of 99.5 or less for more than 24 hours after Ivig followed by a temperature of 100.4
Term
What does prednisone help with the treatment of KD?
Definition
Addition to Ivig substantially reduced coronary artery anomalies
Term
Acyanotic lesions
Definition
Maintain normal or excessive blood flow to the pulmonary vasculature
Term
Cyanotic lesions
Definition
Maintains normal flow to tissues but may limit blood flow to lungs
Term
What type of side effects are there when a coarctation of the aorta is closed?
Definition
Rebound hypertension when end to enastomis occurs because manipulation of the aorta causes sympathetic nervous system stimulation and stimulates the renin aldosterone system- it doesn't usually respond to Ace inhibitors or beta blockers
Term
When does muscular constriction occur of the ductus arteriosis?
Definition
96 hours after birth
Term
What are risk factors for pre-eclampsia?
Definition
Pre-eclampsia in a first degree relative (2-5 fold risk increase)

B. Age ≥ 35 years

C. History of chronic hypertension, kidney disease, diabetes, and/or obesity

D. Twin or molar pregnancy

E. Previous pre-eclampsia

F. Fetal congenital abnormality

G. Reside at high altitude
Term
WHat is gestational hypertension?
Definition
hypertension that develops after 20 weeks gestation
ystolic BP ≥ 140 mmHg or diastolic BP ≥ 90 mmHg AND

b. 24 hour proteinuria ≥ 0.3 g/24 hr or ≥ 1 on dipstick from 2 urine samples collected 4-6 hours apart or at least within 7 days
Term
WHat are the sypmtoms of severe eclampsia?
Definition
Systolic BP ≥ 160 mmHg or diastolic BP ≥ 110 mmHg at 2 different times at least 6 hours apart

b. 24 hour proteinuria ≥ 5 g/24 hr or ≥ 3 g protein from 2 urine samples collected 4 hours apart

c. Urine output < 500 mL in 24 hours

d. Cerebral or visual symptoms

e. Pulmonary edema or cyanosis

f. Epigastric or RUQ pain

g. Impaired liver function

h. Thrombocytopenia

i. Fetal growth restriction
Term
What is HELPP syndrome?
Definition
HELLP syndrome (hemolysis, elevated liver enzymes, low platelets) has different criteria but in general includes:

a. Platelets < 100,000 – 150,000

b. AST or ALT > 40 IU/L (an alternative criteria is AST > 70 IU/L)

c. LDH > 600 IU/L alone or with hemolysis on peripheral smear and TBili > 1.2 mg/dL
Term
What is eclampsia?
Definition
Eclampsia: development of convulsions and/or unexplained coma during pregnancy or postpartum in patients with signs and symptoms of pre-eclampsia (usually symptoms align with those for severe pre-eclampsia)
Term
What is the only treatment for eclampsia?
Definition
-only treatment is delivery for the fetus
Step one is maternal stabilization

b. Immediate delivery if onset at 36-37 weeks or greater

c. Delivery at 24-36 weeks depends on severity of pre-eclampsia

d. Assess risk/benefit for delivery at less than 24 weeks

e. Administer antenatal corticosteroids if indicated
Term
what is goal bp for preclampsia?
What are three choices of treatment?
Definition
. Maternal BP goal should be 140-150 SBP/90-100 DBP
hydralazine, labetalol, and nifedipine.
Term
what dose of magnesium can be used for eclampsia?
Definition
4-6 g IV over 15-20 minutes, then 1-2 g/h; titrate to desired Mg level
Recurrent seizure may be treated with an additional 2 g magnesium sulfate over 3-5 min
Term
Why is blood typing done on the first visit?
Definition
Rho(D) immune globulin at 28 weeks for all nonsensitized Rh-negative women and within 72 hours after delivery of Rh-positive infant
Term
what is PROM?
Definition
spontaneous rupture of membranes before 37 weeks gestation.
Term
What are the SLE cateogries?
Definition
malar rash, discoid rash, photosensitivity, oral ulcers, arthritis, serositis, renal disorder(high protein or casts in urine), h/o pleuretic pain, neurologic disorder, hematologic disorder(low counts with hemolytic anemia. antiphospholipid antibodies.
Term
what are the 4 nucleotide bases that make up DNA?
Definition
adenine, cytosine, thymine and guanine
Term
What are codons?
Definition
Nucleotide triplets which specify and amino acid and which are used to translate encoded information into a functional protein
Term
what is a polymorphisms?
Definition
variants that are relatively common, occurring in greater than 1% of the population, and can involve sequence variations (insertions or deletions) other than a single nucleotide change
Term
what is a mutation?
Definition
a rare gene variant that are associated with genetic diseases
Term
what are SNP's?
Definition
Single nucleotide polymorphisms (SNPs) involve a single nucleotide change in the DNA sequence and are the most common type of polymorphisms
Term
What are haplotypes?
Definition
Several SNPs on the same chromosome may be inherited together in groups or blocks known as haplotypes.
Term
What does phenotype refer to?
Definition
the patient's drug response
Term
What is pharmacogenetics?
Definition
Pharmacogenetics focuses on variation in drug response that occurs because of large, single-gene (monogenic) effects (e.g., inherited differences in drug-metabolizing enzyme capacity. This approach is inherently limited given that drug response is most often polygenic or determined by the combined influence of multiple genes and/or gene networks.
Term
What are pharmacogenomics?
Definition
Pharmacogenomics, in contrast, seeks to determine how variations in multiple genes or the entire genome contribute to variation in drug response.
Term
What are candidate gene studies?
Definition
1. Candidate gene studies evaluate whether a polymorphism occurs more frequently in individuals with a certain drug response phenotype.requier prior knowledge of the function of the gene candidate and the drugs mwchanism of action
Term
What are TAG SNP studies and what makes them easier to perform?
Definition
Tag SNP studies are an alternative to the candidate gene studies that utilizes tag SNPs that are selected because they are in linkage disequilibrium with other SNPs in the chromosomal region of interest. This decreases the number of genotypes that have to be determined and enhances the ability to quickly scan the entire genome
Term
Genome wide distribution studies- what are they? are they low or high cost?
Definition
Genome-wide association studies (GWAS) evaluate many SNPs (100,000 to 1 million) to determine whether certain SNPs occur more frequently in individuals with a specific drug response phenotype
2. GWAS do not require prior knowledge of gene function of drug mechanism of action and can therefore be used to investigate gene-response associations for drugs that are still under development or for SNPs whose functional consequences are unknown
3. Limitations of GWAS include technical complexity, high cost, need for large sample sizes and a high risk of spurious or false positive associations
Term
what are examples of gene response associations relevant to peds?
Definition
Thiopurine Methyltransferase and 6-Mercaptopurine, cysteinyl Leukotriene and leukotriene repsonse modifiers, Maternal CYP2D6 Genotype and Opioid Exposure in Breastfeeding Infants
Term
What is the TPMT variant and how does it affect patients taking 6mp?
Definition
TPMT is a phase two metabolizing enzyme. hree (*2, *3A and *3C) are common and collectively account for more than 95% of inherited variation in TPMT activity.
Term
What does homozygous mean?
Definition
they have two of the same gene (one from mom and one from dad
Term
What happens if a person is homozygous for reduced tpmt actvitiy?
Definition
PT allelic variants, three (*2, *3A and *3C) are common and collectively account for more than 95% of inherited variation in TPMT activity.
5. Children with a homozygous variant TMPT
Term
what happens if a person has a SNP in the leukotriene C4 synthase gene promoter?
Definition
A SNP in the leukotriene C4 synthase gene promoter leads to increased leukotriene production and inflammation. Individuals with a genotype conferring increased leukotriene C4 synthase activity may respond better to leukotriene C4 receptor antagonists.
Term
what happens if you have an allelic variation in the promoter region of ALOX5?
Definition
Allelic variation in the promoter region of the arachidonate 5-lipoxygenase (ALOX5) gene is also associated with response to leukotriene synthesis inhibitors.
Term
Explain codeine metabolism? Which cyp is it metabolized by?
Definition
Codeine is converted to its pharmacologically active metabolite, morphine, by CYP2D6. Morphine, in turn, is converted to morphine 3-glucuronide (inactive) and morphine 6-glucuronide (active) by the phase II enzyme, uridyl glucuronoslytransferase (UGT) 2B7.
Term
there are two components to codeine metabolism that are affected by pharmacogenetics.
Definition
Individuals homozygous for the UGT2B7*2 allele show higher morphine-6-glucuronide/morphine ratios.

imilarly, individuals with multiple copies of the CY2D6 gene (known as gene duplication) have an ultra-rapid metabolizing phenotype and are more susceptible to toxicity due to enhanced conversion of codeine to morphine.
Term
What does desmopressin do for hemophilia A?
Definition
Releases bound FVIII into the systemic circulation, elevating the concentration available for coagulation.- good for minor bleeds at home- watch out for hyponatremia
Term
How do you calculate factor 8 dosing?
Definition
IU/kg will raise serum FVIII activity by 2%

2. Units of FVIII required = weight (kg) x desired level (%) x 0.5
Term
what is dose of factor IX for hemophilia?
Definition
2 IU/kg will raise serum FIX activity by 2%

3. Units of FIX required = weight (kg) x desired level (%) x 1

a. If using recombinant FIX (rFIX) a dose adjustment of 1.2 for adults and 1.4 for children is required.
Term
What is ITP?
Definition
ITP is an autoimmune disorder of isolated low circulating platelets (< 100,000/microL) caused by destruction of antibody-sensitized platelets in the reticuloendothelial system. White blood cell count and hemoglobin are normal.

B. It was previously known as idiopathic thrombocytopenic purpura or immune thrombocytopenic purpura.
Term
How does ITp occur?
Definition
A misdirected humoral antibody response targeted against platelets results in a shortened life-span of circulating platelets.

1. Normal platelets last 8-10 days, whereas the circulating platelets in a child with ITP live only a few hours; once they are coated with antibody, they are rapidly cleared from circulation.

2. The initial insult is suspected to be viral.

1. Epitopes on the surface of the virus may be similar to epitopes on the surface of platelets and produce a B-lymphocyte response with IgG antibody production that binds to platelets in cross-reactions, which results in platelet removal from circulation.

2. Over time, the virus is eliminated, the titer of the antibody gradually decreases, secondary to the half-life of IgG of about 3 weeks, and the platelets recover.
Term
what is treatment for ITP?
Definition
1) watchful Grade 1: minor bleeding, few petechiae, no mucosal bleeding

Observation

Grade 2: mild bleeding, many petechiae, no mucosal bleeding

Observation or treatment in selected children

Grade 3: Moderate bleeding, overt mucosal bleeding, disruption of lifestyle

Treatment

Grade 4: Mucosal bleeding or suspected internal hemorrhage

Treatment
Term
what are B cell leukemia positive for?
Definition
B-cell ALL often positive for:

i. CD10 (referred to a “CALLA” – common ALL antigen)

ii. CD19

iii. CD20 (sometimes)

iv. L3 ALL will express CD10 + CD19, CD20, CD22, CD25 and surface immunoglobulin
Term
what are favorable ALL genetics?
Definition
t(12;21)ETV6/RUNX1

1. Historically referred to as TEL/AML1

2. Present in 20-25% of patients

ii. Hyperdiploidy

1. 54-58 chromosomes present in cells

2. Prognosis is highly favorable in the presence of trisomy 4 and 10

b. Unfavorable
Term
what are unfavorable cytogenetics of ALL?
Definition
Philadelphia chromosome

1. t(9;22) BCR/ABL translocation

2. Affects 3-5% of children (more often in adolescents) whereas ~25% of adults are affected

ii. MLL gene rearrangements

1. Seen in infants and associated with t(4;11)

2. Can also manifest at 11q23 rearrangement

iii. Intrachromosomal amplification of chromosome 21 (iAMP21)

iv. Ploidy

1. Hypodiploid (< 45 chromosomes)

2. Extreme hyperdiploid (> 58 chromosomes)
Term
what are more common variances for AML
Definition
21% - Core binding factor AML (CBF AML) 21%

a. Collectively refers to chromosomal aberrations of t(8;21) and Inv(16)/t(16;16)

b. More prevalent in teenagers

2. 11% - t(15;17) 11%

3. 23% - MLL gene rearrangements

a. More prevalent in infants and younger children

4. 25% - Other karyotypic alterations

5. 20% - Normal karyotype
Term
what are common mutations for AML
Definition
FLT3 mutations

a. FMS-like tyrosine kinase 3 (FLT3) is most commonly mutated gene seen in pediatric AML (seen in ~15% of patients)

i. ITD refers to internal tandem duplication

b. Associated with a poor response to induction chemotherapy and higher relapse rate

2. NPM1 mutations

a. Nucleophosmin (NPM1) encodes for a molecular chaperone that moves between the nucleus and cytoplasm

b. Associated with a decreased risk for relapse and improved survival

3. CEBPA mutations

a. The CEBPA gene encodes for a transcription factor that regulates granulocyte proliferation and terminal differentiation

b. Tend to occur in patient with normal cytogenetics

c. Associated with a decreased risk for relapse and improved survival

5. Pathological features and classification
Term
what is TAPVR
Definition
total anamolous pulmonary venous return
Term
What is normal MAP in an infant?
Definition
it's their gestational age. so if 26 weeks MAP would be 26. if the are 40 weeks then MAP is 40,
Term
what is an inadequate statistical power?
Definition
its a failure to find a difference between treatments when a difference actually exists and often results are from an inadequate sample size.
Term
What three enzymes does HIV need to replicate once inside the cell?
Definition
Once inside, the HIV-1 virus requires three enzymes, reverse transcriptase, integrase and protease to complete its life cycle
Term
what are the six classes of HIV drugs?
Definition
Nucleoside Reverse Transcriptase inhibitors (NRTI)
non-nucleoside Reverse Transcriptase inhibitors (NNRTI)
Integrase Inhibitors
Protease Inhibitors (PI)
Chemokine receptor antagonists
Fusion inhibitors (FI)
Term
what cd4 counts in HIV indicate need for treatment?
Definition
Ages 1 to <3: CD4 count <1000 cells/mm3
b. Ages 3 to <5: CD4 count <750 cells/mm3
c. Ages ≥ 5: CD4 counts < 500 cells/mm3
Term
what does cART entail for HIV?
Definition
cART therapy is a regimen containing at least three drugs from at least two classes of antiretroviral drugs. This is made up of a 2 drug backbone consisting of NRTI’s and then in pediatrics either a NNRTI or a PI. In more advanced HIV pediatric patients, the addition of an integrase inhibitor, chemokine receptor atagonist or fusion inhibitor might be considered for more resistant HIV strains.
Term
whats the most common bacteria that can cause impetigo?
Definition
staph aureus the community acquired MRSA, B-hemolytic strept like strept pyogenes(grp A Strept)
Term
what exclusively causes bullous impetigo?
Definition
staph aureus which releases staph exfolliative toxins which cause skin sloughing
Term
what is treatment for imeptigo?
Definition
topical = mupirocin and retapamulin for 5 days total
oral= usual is penicillin but can use 1st generation ceph and for cases of MRSA clinda or bactrim
pcn allergies= macrolides
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