Term
nonorganic failure to thrive (FTT) |
|
Definition
related to poverty or poor caregiving |
|
|
Term
|
Definition
seen in babies who are lying in a bed too much --> sign of nonorganic failure to thrive |
|
|
Term
how much calories do infants need |
|
Definition
120cal/kg in first year
100/kg after that |
|
|
Term
caloric requirement in FTT inhfants |
|
Definition
|
|
Term
how long should meals last |
|
Definition
|
|
Term
signs of baby with CMV inrauterine infection |
|
Definition
developmental delay IUGR microencephaly cataracts seizures hepatosplenomegally jaundice pupura |
|
|
Term
diff twix cmv and toxo intrauterine infection |
|
Definition
look the same in a neonate
however in CMV babies will show calcified brain densities of CMV in perventricular pattern
it is scattered brain densities in toxo |
|
|
Term
when do kids become "picky" eaters |
|
Definition
|
|
Term
what is renal tubular acidosis |
|
Definition
inherited or acquired defect that affects the kidneys ability to absorb filtered bicarb OR excrete ammonia |
|
|
Term
|
Definition
normal anion gap hyperchloremic metabolic acidosis
net loss of bicarb and gain of H |
|
|
Term
|
Definition
1. distal 2. proximal 3. mixed 4. hypoaldosteronism
"DPMH" "Renal tubes Dip Pretzels in Mixed Horseradish" |
|
|
Term
most common types of RTA in children |
|
Definition
|
|
Term
|
Definition
|
|
Term
Substance abuse vs substance dependence |
|
Definition
Energy is used to acquire drugs in dependance |
|
|
Term
Drug that causes nystagmus and ataxia |
|
Definition
|
|
Term
Drugs that cause pinpoint pupils |
|
Definition
|
|
Term
|
Definition
3500 is average 2700 to 4600g is ok |
|
|
Term
|
Definition
|
|
Term
Cause of clinodactly in Down's syndrome |
|
Definition
|
|
Term
|
Definition
Association of fetal dysmorphic features that includes Vertebral problems Anal atresia Tracheoesophageal fistula OR esophageal atresia Renal problems/radial annomolies Limb problems |
|
|
Term
|
Definition
Grey spots around iris seen in Down syndrome |
|
|
Term
Most common cardiac defects in Down's syndrome |
|
Definition
Endocadial cushion #1 Vsd #2 |
|
|
Term
Gi issues in Down syndrome |
|
Definition
12% have gi atresia of some sort |
|
|
Term
|
Definition
|
|
Term
Down's syndrome hypotonic or hypertonic mm? |
|
Definition
|
|
Term
Genetic cause of Down syndrome |
|
Definition
Non disjunction Failure of chromosomes to separate in meiosis |
|
|
Term
Long term risks of Down syndrome |
|
Definition
Leukemia Early aging + Alzheimer's Cervical spine instability Hypothyroidism Hearing/vision issue |
|
|
Term
|
Definition
Area of missing skin and hair Seen in pataus (13) |
|
|
Term
|
Definition
|
|
Term
Which trisomy has omphaceole |
|
Definition
|
|
Term
What reflex is impaired in Down's syndrome |
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
Unique allergies seen in pataus |
|
Definition
|
|
Term
How does the HIV screening test work |
|
Definition
|
|
Term
When will HIV test be positive |
|
Definition
|
|
Term
Lase neg and false pos in HIV test |
|
Definition
False posi is rare False neg more coming, esp in early infection or in severe aids or sp immunization |
|
|
Term
Confirmation test after posi screen for HIV |
|
Definition
|
|
Term
How do we prevent vertical transmission of HIV in HIV + pregnant woman |
|
Definition
Ziduvine given in 2nd trimester and given to baby for first 6 weeks |
|
|
Term
|
Definition
HIV posi mothers should not breast feed |
|
|
Term
|
Definition
|
|
Term
How long do we test neonates for HIV if born from posi mother |
|
Definition
|
|
Term
When do you start pcp prophy In HIV+ infants |
|
Definition
|
|
Term
Common ade to all HIV meds |
|
Definition
N/v/d Ha Osteopenia Drug rash Neutropenia, anemia Hyperlipidemia and hyperglycemia |
|
|
Term
|
Definition
|
|
Term
Childhood vaccines NOT given to HIV kids |
|
Definition
|
|
Term
|
Definition
Leukocyte adhesion deficiency |
|
|
Term
|
Definition
Slow wound healing Recurrent resp infections Skin infections |
|
|
Term
|
Definition
|
|
Term
|
Definition
T cell deficiency --> B cell dysfunction Thymus problem Bubble boy (very sever, nearly absent immune system) Recurrent infantile infections Different types, most common is adenosine deaminase |
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
Major feature of Digeorge |
|
Definition
|
|
Term
Other features of Digeorge |
|
Definition
Boot shaped heart Small chin, pointy ears Ftt Velocardialfacial abnormalities |
|
|
Term
Electrolyte abnormality in Digeorge |
|
Definition
|
|
Term
Presentation of klinefelters in adolescence |
|
Definition
1. Gynecomastia 2. Long extremities 3. Hypogonadism 4. Developmental delay (spec ed) 5. Immaturity/insecurity |
|
|
Term
|
Definition
|
|
Term
What learning difficulties do klinefelters kids have |
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
Explosive tempers Social issues (jail) Modular acne Large teeth Long asymmetric ears Pectus excavatum |
|
|
Term
|
Definition
|
|
Term
|
Definition
Increased carrying angle of arms seen in turners |
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
Risks of goats milk for infants vs breast milk |
|
Definition
FolTe, b12, iron def
Brucellosis if not pasteurized |
|
|
Term
What is breast. Ilk low in |
|
Definition
|
|
Term
Metabolic disturbance in infants breast feeding from vegan mothers |
|
Definition
|
|
Term
Signs of b12 def in infants. |
|
Definition
Seizure Encephalopathy Stroke |
|
|
Term
|
Definition
Hyperostosis (pain/swelling of long bones) Hepatomegaly Alopecia Increased ICP |
|
|
Term
Vit E def in premature infants |
|
Definition
|
|
Term
|
Definition
Pellagra Diarrhea Dementia Dermatitis |
|
|
Term
|
Definition
|
|
Term
Fontainel fullness in infant |
|
Definition
|
|
Term
|
Definition
|
|
Term
galactosemia caused by _____ in infants |
|
Definition
uridyl transferase deficiency |
|
|
Term
|
Definition
N/V FTT hepatosplenomegally liver, kidney failure jaundice, aminoaciduria, hypogly seizure, lethargy |
|
|
Term
infection in galactosemia |
|
Definition
|
|
Term
longterm issues with galactosemia |
|
Definition
speech difficulty ataxia dysmetria premature ovarian failure cataracts lo bone density MR |
|
|
Term
|
Definition
|
|
Term
inheritance of galactosemia |
|
Definition
|
|
Term
low phenylalanine formulas are for |
|
Definition
|
|
Term
low isoluecine, leucine, valine formulas are for..... |
|
Definition
maple syrup urine disease |
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
use bowel loop to form ducts to allow bile to drain from liver in biliary atresia |
|
|
Term
fractures in pt with biliary atresi |
|
Definition
biliary atresia = no bile in gut = fat malabs = vit ADEK malabs = vit D rickets |
|
|
Term
|
Definition
|
|
Term
bone problem in infants without vitD |
|
Definition
|
|
Term
tx kids with liver gailure and ascites |
|
Definition
|
|
Term
most common form of nunutritional rickets |
|
Definition
familial primary hypophosphatemia |
|
|
Term
who gets familial primary hypophosphatemia |
|
Definition
|
|
Term
pathogenesis of familial primary hypophosphatemia |
|
Definition
kidney issue = low P and low conversion of inactive to active vit D = low Ca |
|
|
Term
labs in familial primary hypophosphatemia, what must it be differentiated from? |
|
Definition
NORMAL PTH (must be differentiated from hyperparathyroid) low D low Ca hi AP hi P in urine |
|
|
Term
signs of calcium deficietnt rickets |
|
Definition
myopathy rachitic rosar pectus deformities tetany |
|
|
Term
teeth issues in D rickets vs C rickets |
|
Definition
vitD rickets = intraglubular dentin issues Ca rickets = enamel issues |
|
|
Term
phosphorous levels in vitD def |
|
Definition
|
|
Term
|
Definition
celiac cystic fibrosis gi tract anomolies |
|
|
Term
radiological findings in familial hypophosphatemia |
|
Definition
coarse trabecular bone widening, fraying, cupping of metaphysis in distal tibia, femur, radius and ulna |
|
|
Term
|
Definition
sun makes previtD in skin converted to 25-D in liver converted to 1,25-D in kidney |
|
|
Term
how do urine amino acid levels change with Ca and phorphorus levels |
|
Definition
they are usually hi in deficiencies of P and Ca |
|
|
Term
what does hepatic disease do to Ca and P |
|
Definition
|
|
Term
what do anticonvulsants do to Ca and P |
|
Definition
|
|
Term
how do AP levels change with Ca and P levels |
|
Definition
elevated in deficiency of Ca and P |
|
|
Term
what anticonvulsants have the greatest effect on Ca and P. how does it work |
|
Definition
phenobarbital and pheyntoin inc CYP450 activity --> lo vit D |
|
|
Term
tx low Ca and P in pts on anticonvulsants |
|
Definition
|
|
Term
renal osteodystrophy pathogenesis |
|
Definition
damaged kidney retains phosphorous this turns on parathyroid hi PTH ==> increased bone turnover
also kidney damage diminishes vitD |
|
|
Term
what are Ca levels in renal osteodystrophy |
|
Definition
|
|
Term
|
Definition
AR reduced activity of 25-D hydroxylase tx with massive vitD doses |
|
|
Term
Fanconis sydrome affects what anatomically |
|
Definition
|
|
Term
what abnormalities are there in fanconis syndrome |
|
Definition
wasting of glu, amino acids, uric acid, phosphate and bicarb
the low phosphate causes low PTH and low Ca |
|
|
Term
clinical features of fanconins in children |
|
Definition
polyuria, polydipsia rickets ftt elevated P, glu, aa etc in urine |
|
|
Term
|
Definition
renal tube acidosis hypokalemia hyperchloremia non-anion gap |
|
|
Term
UE fracture in infants indicative of abuse |
|
Definition
spiral fracture of humerus |
|
|
Term
vit replacement in CF kids |
|
Definition
|
|
Term
Schmid metaphyseal dysplasia |
|
Definition
AD rickets in children short stature, leg bow, waddling gait normal serum Ca, P and AP |
|
|
Term
labs in familiarl hypophosphatemia (ca, p, vit D, AP, amino acid in urine) |
|
Definition
low P low-normal Ca low vit D hi AP hi amino aciduria |
|
|
Term
|
Definition
slow return of skin to normal position after it has been pinched
sign of dehydration |
|
|
Term
pathogenesis of ketoacidosis |
|
Definition
insulin unavailability --> lipid oxidation --> hi ketones and free fatty acids |
|
|
Term
|
Definition
deep rapid inspirations associated with acidosis |
|
|
Term
|
Definition
hypotension, hypothermia, kussmaul resp, tachy |
|
|
Term
|
Definition
|
|
Term
|
Definition
anion gap metabolic acidosis |
|
|
Term
|
Definition
|
|
Term
|
Definition
elevated in reflection of dehydration |
|
|
Term
first step in pt with dka |
|
Definition
|
|
Term
4 things to manage in DKA |
|
Definition
|
|
Term
how do you manage IVF in DKA |
|
Definition
1. give 1-2 L NS bolus 2. check Na (corrected), if less than 135 --> NS if>135 use .5NS 3. check glu q1hr 4. if glu <200 add D5 to IVF |
|
|
Term
how do you manage K in DKA |
|
Definition
1. check it 2. if < 3.3 give KCl piggyback until over 3.3, if >5.3 continue without KCl, if 3.3>5.3 add 20mEq to IVF |
|
|
Term
how do you manage insulin in DKA |
|
Definition
1. give .1 U/kg bolus IV 2. start pump at .1U/kg/hr IV 3. if glu has not fallen by 50 after 1st hr rpt bolus 4. once glu <200, dec pump to .05/kg/hr 5. stop when DKA is over |
|
|
Term
|
Definition
glu <200 fasting anion gap <10 |
|
|
Term
|
Definition
if pH > 7, do nothing if pH 6.9-7 give 50 bicarb if pH less than 6.9 give 100 mmol bicarb |
|
|
Term
|
Definition
|
|
Term
|
Definition
1. birth 2. 1-2mo 3. 6-18mo |
|
|
Term
|
Definition
|
|
Term
|
Definition
2 doses within 1 and 2 yrs |
|
|
Term
# of rotavirus vacc doses |
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
meningeococcal vacc schedul |
|
Definition
dose 1 at 11-12 yo booster at 16yo |
|
|
Term
pneumo vacc type for children |
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
2mo, 4mo, 6mo-2yr, 4-6 yr |
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
1st dose at 1 yr 2nd dose at least 3mo later |
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
start between 9-12 yo give at time =0, 1-2mo, 6mo (3 total doses) |
|
|
Term
|
Definition
|
|
Term
allergy to neomycin or gelatin? |
|
Definition
|
|
Term
at what age can you no longer get hib |
|
Definition
not recommended if over 5yo |
|
|
Term
how should babies sleep to prevent sids |
|
Definition
|
|
Term
when will baby sleep thru the night |
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
when can potty training start |
|
Definition
no earlier than 2 yrs. start when child shows interest |
|
|
Term
when can babies fix and follow human face? |
|
Definition
|
|
Term
when is screening hct done on child |
|
Definition
|
|
Term
what vacc can you give to asymptomatic hiv pts |
|
Definition
|
|
Term
bugs that cause bacterial pneumonia in children up to 5 yo |
|
Definition
#1 viral causes #2 pneumococcus and HiB |
|
|
Term
cause of pneumonia in peds pts over 5 |
|
Definition
mycoplasma or pneumococcus |
|
|
Term
tx of pneumonia in peds pt over 5 |
|
Definition
azithro (macrolides) or cephalosporin like ceftriaxone or cefuroxim |
|
|
Term
pneumonia cause in intubated pts |
|
Definition
|
|
Term
pneumonia in ppl with chronic lung disease |
|
Definition
aspergillosis and pseudomona |
|
|
Term
dermatomal skin rash and pneumonia sx |
|
Definition
|
|
Term
retinitis and pneumonia sx |
|
Definition
|
|
Term
pt with refractory asthma and pneumonia |
|
Definition
|
|
Term
|
Definition
|
|
Term
SW US travel and pneumonia |
|
Definition
|
|
Term
|
Definition
|
|
Term
infected sheep or cattle and pneumonia |
|
Definition
|
|
Term
standard antituberculous therapy while awaiting culture and sensitivities |
|
Definition
isoniazid, rifampin and pyrazinamide |
|
|
Term
drug to add if suspect drug resistant TB |
|
Definition
|
|
Term
|
Definition
optic neuritis and cataracts "ocular toxicity) |
|
|
Term
newborn with pneumonia sx and eye discharge |
|
Definition
|
|
Term
very general rules of age groups and pneumonia cause |
|
Definition
1. newborn GBS 2. toddler RSV 3. adolescent mycoplasm |
|
|
Term
how to confirm organophosphate poisoning |
|
Definition
decreased serum pseudocholinesterase and erythrocyte cholinesterase levels |
|
|
Term
first thing to do in child with suspected organophsophate poisoning |
|
Definition
undress them, they can continue to absorb poison from clothes |
|
|
Term
|
Definition
cardiac=htn, tachycardia, arrythmia mm= fasciculations, weak, tremor resp= diaphrag paralysis |
|
|
Term
|
Definition
GI emesis, diarrhea, no bowel control urinary incontinence bronchorrhea and bronchospasm hypotension bradycardia tearing, drooling, miosis |
|
|
Term
pesticides that cause organophosph poisoning |
|
Definition
parathion malathion diazinon |
|
|
Term
signs and sx of cholinergic excess |
|
Definition
Diarrhea Urination Miosis Bradycardia Broncorrhea Emesis Lacrimation Salivation dumbbells |
|
|
Term
cetnral effects of cholinergics |
|
Definition
|
|
Term
drugs to counteract organophosphates |
|
Definition
|
|
Term
|
Definition
antagonizes muscarinic receptor |
|
|
Term
when was asbestos used to build shit |
|
Definition
|
|
Term
what happens if a child eats the mercury in a thermometer |
|
Definition
|
|
Term
sx of large amount of mercury poisoning |
|
Definition
gi if ingested if vapor=gi sx, fever, chill, HA, visual change, chest pain, pneumonitis, Pulmonary edema |
|
|
Term
ataxia, dysarthria and paresthesis = toxicity from? |
|
Definition
seen in methyl mercury intox (from fish) |
|
|
Term
gingivostomatitis, tremor and neuropsych issues = toxicty from ? |
|
Definition
chronic inorganic mercury intox (like from a work place or home) |
|
|
Term
|
Definition
nausea, vomiting, abdo pain, diarrhea (hemorrhagic) thrid spacing of fluids --> hypovolemic shock arrythmias |
|
|
Term
leading nonpharm ingestion fatality from toxic substance |
|
Definition
|
|
Term
toddler with ear pain and fever |
|
Definition
|
|
Term
what does tympanic membrane look like in acute otitis media |
|
Definition
red, opaque, poor movement, bulging |
|
|
Term
|
Definition
surgical procedure with TM incision and placement of pressure equalization tubes to ventilate the middle ear and prevent fluid accumulation |
|
|
Term
|
Definition
fluid behind TM but no signs and sx of acute otitis media |
|
|
Term
|
Definition
surgery to drain pus from middle ear. cut TM, need specialist to preform |
|
|
Term
most common bacterial causes of acute otitis media |
|
Definition
|
|
Term
systemic sx of acute otitis media |
|
Definition
malaise, fever, diarrhea, HA, N/V |
|
|
Term
abx for acute ottitis media |
|
Definition
|
|
Term
how long should a pt take amoxicillin for acute otitis |
|
Definition
|
|
Term
what if amoxicillin fails to treat middle ear infection |
|
Definition
switch to amox-clav or a cephalosporin or consider draiage |
|
|
Term
how long can fluid stay behind the ear after infection |
|
Definition
|
|
Term
rare/serious complications of acute otitis media |
|
Definition
mastoiditis temporal bone osteomyelitis facial nerve paralysis epidural/subdural absess, meningitis, other brain infections |
|
|
Term
otitis media + signs of increased ICP |
|
Definition
|
|
Term
|
Definition
red ear can be swimmers ear painful external canal |
|
|
Term
|
Definition
topical polymixin insertion of wick to help drain |
|
|
Term
|
Definition
|
|
Term
pt with otitis media + bluging behind the ear |
|
Definition
|
|
Term
|
Definition
|
|
Term
very young (babies) with otitis media tx> |
|
Definition
admit to hospital and evaluate for sepsis in neonates it is easy for otitis media to turn to sepsis fast |
|
|
Term
initial test for gonno and chlamydia? |
|
Definition
nuclaic acid amplification test (NAAT) |
|
|
Term
F/U test if NAAT + for gonnorhea in a child |
|
Definition
culture or do a second NAAT that targets a different part of the genome |
|
|
Term
|
Definition
|
|
Term
do you always have to culture/rpt test if first NAAT is + for gonno or chlamydia? |
|
Definition
only in children. because a + test --> sexual abuse |
|
|
Term
how long after a sexual assault can you still get dna from the perpatrator from the victims clothes (rape kit) |
|
Definition
|
|
Term
anal conylomatata in children under 3 yo |
|
Definition
HPV condylomata 2/2 contact during birth |
|
|
Term
itching anus + wt loss + abdo pain |
|
Definition
|
|
Term
|
Definition
|
|
Term
loss of stellate pattern of anuse, low anal tone and fissures around anus |
|
Definition
suspect repeat penetrating injury |
|
|
Term
tx narcotic related respiratory and mental status depression |
|
Definition
|
|
Term
how do you administer APGAR score? |
|
Definition
do it at 1 minute and 5 minutes of life |
|
|
Term
what is measured in APGAR |
|
Definition
appearance (color) pulse rate grimace (response to stimuli) activity (motor) respirations |
|
|
Term
what are the scores you can get for appearance in APGAR |
|
Definition
0 = blue/pale 1 = body pink and extremities blu 2 = pretty pink baby |
|
|
Term
what are the score you can get for pulse in APGAR |
|
Definition
0 = absent 1 = <100 2 = >100 |
|
|
Term
what are the scores you can get for grimace in APGAR |
|
Definition
0 = no response to stimuli 1 = grimace only 2 = cough/sneeze |
|
|
Term
what is the stimuli you give a baby to measure grimace? |
|
Definition
stick something up their nose (catheter, suction bulb) |
|
|
Term
what are the scores you can get for activity in APGAR |
|
Definition
0 = limp 1 = some flexion of extremities 2 = flexed active motion |
|
|
Term
what are the scores you can get for respiration in APGAR |
|
Definition
0 = no effort 1 =slow irrigular breathing 2 = good crying |
|
|
Term
infant with scaphoid abdomen, heart sounds on right side and respiratory distress |
|
Definition
|
|
Term
how do you manage an infant with scaphoid abdomen, heart sounds on right side and respiratory distress? |
|
Definition
must do endotracheal tube? |
|
|
Term
why are infants with diaphragmatic hernia in respiratory distress? |
|
Definition
if they had the hernia in utero, they lungs dont have room to develop properly and they get pulmonary hypoplasia |
|
|
Term
why cant you bag and mask an infant with a diaphragmatic hernia? |
|
Definition
it will cause accumulation of bowel gas and because the bowels are in the thorax this will cause further compression of the lungs |
|
|
Term
management of a newborn with bradycardia and otherwise uncomplicated pregnancy |
|
Definition
1. give 100% O2 2. if HR still less than 60, do chest compressions for 30 seconds 3. if HR still less than 60, give epinephrine (or other drug) |
|
|
Term
how is infant breathing different when crying and when quiet |
|
Definition
babies are obligate nose breathers. when crying they can breath thru mouth, but when quiet they go thru nose. |
|
|
Term
baby who is well ventilated ONLY when crying. baby goes in respiratory distress when not crying. |
|
Definition
|
|
Term
|
Definition
when back of the nasal passage is blocked due to congenital abnormality |
|
|
Term
management of choanal atresia |
|
Definition
endotracheal intubation to bypass teh nasal airway obstruction followed by surgical repair |
|
|
Term
how can you identify choanal atresia |
|
Definition
try to pass a feeding tube thru the nostril
see if mist is coming out of nostrils by holding up a cold metal under the nose. |
|
|
Term
definition of cerebral palsy (CP) |
|
Definition
disorder of movement or posture from the CNS
must be CNS issue, this differentiates it from neuropathies or myopathies |
|
|
Term
what are the most common comorbidities with CP |
|
Definition
1/3 of CP pts also have seizures 2/3 of CP pts are also mentally retarded |
|
|
Term
how must the CNS insult occur in order to qualify as CP |
|
Definition
must be a 1 time insult must present in 1st yr of life must be static (non progressing although the symptoms may progress like skeletal deformities 2/2 the mm weakness, the insult in the cns itself is not degenerative) |
|
|
Term
|
Definition
periventricula leukomalacia atrophy or small ifnarctions |
|
|
Term
|
Definition
if you hold a baby upright with feet touching the floor they step forward. |
|
|
Term
when should stepping response go away? |
|
Definition
|
|
Term
what if the stepping response persists |
|
Definition
|
|
Term
|
Definition
stepping response after 3 mo Moro reflex after 6mo asymmetrical tonic neck reflex after 6 mo |
|
|
Term
|
Definition
babies reach out arms when they lose support |
|
|
Term
asymettrical tonic neck reflex |
|
Definition
when the face is turned to one side, the limbs on that side are extended and the limbs opposite the face are flexed |
|
|
Term
|
Definition
involvement of single lateral side |
|
|
Term
|
Definition
four-limb involvement with greater impairment of lower extremities |
|
|
Term
|
Definition
four-limb involvemtn with significant impairment of all extremities although the upper limbs may be less impaired than lower limbs |
|
|
Term
when do most children walk? |
|
Definition
|
|
Term
asthma, rectal prolapse and digital clubbing |
|
Definition
|
|
Term
|
Definition
recurrent sinusitis diarrhea/failure to thrive in children nasal polyps mouth breathing |
|
|
Term
why diarrhea/failure to thrive in CF |
|
Definition
pancreatic exocrine deficiency |
|
|
Term
what is the respiratory issue most CF pts have |
|
Definition
|
|
Term
bugs that cause pneumonia in CF pts |
|
Definition
|
|
Term
what is unique about the strains of psudomonas found in CF pneumonia |
|
Definition
heavy, slime producing mucoid varients |
|
|
Term
|
Definition
lung colonizer seen in CF pts, assocaited with rapid clinical deterioration |
|
|
Term
do airways react to bronchodialators in CF? |
|
Definition
about 50% of CF pts have some airway reactivity tho inhalers etc have unpredictable responses |
|
|
Term
complications of advanced CF |
|
Definition
pneumothorax hemoptysis cor pulmonale |
|
|
Term
|
Definition
|
|
Term
what is the longterm effect of CF stool? |
|
Definition
can increase risk for obstruction, volvulus and intussception |
|
|
Term
|
Definition
fatty liver, large liver, or biliary cirrhosis, gallbladder disease |
|
|
Term
|
Definition
|
|
Term
causes of false + sweat chloride |
|
Definition
anorexia hypothyroid nephrogenic DI |
|
|
Term
what chromosome is effected in CF |
|
Definition
|
|
Term
what is the most common mutation in CF |
|
Definition
amino acid 508, single phenylalanine deletion |
|
|
Term
how does the blood test for CF work |
|
Definition
detects a pancreatic enzyme seen in newborns with CF, called immunoreactive trypsinogen |
|
|
Term
management of infants with positive blood test for CF |
|
Definition
follow up with sweat chloride testing |
|
|
Term
what vit def are seen in CF |
|
Definition
|
|
Term
metabolic abnormality in CF |
|
Definition
hyponatremic hypochloremic alkalosis |
|
|
Term
|
Definition
must be careful about dehydration |
|
|
Term
|
Definition
ileus in a newborn that begins in utero |
|
|
Term
what is the likely cause of meconium ileus |
|
Definition
deficiency of proteolytic enzymes associated with CF |
|
|
Term
what type of infants get necrotizing enterocolitis |
|
Definition
|
|
Term
bulging frontal fontanelle |
|
Definition
indicates increased intracranial pressure |
|
|
Term
how does vit A relate to a bulging frontal fontanelle |
|
Definition
disorders of vitA metabolism, including vitA toxicity or excess can cause pseudotumor cerebri --> bulging anterior fontanelle |
|
|
Term
clinical manifestations of ALL in children |
|
Definition
joint pain leg pain low grade fever petechiae hepatosplenomegally |
|
|
Term
most common childhood cancer |
|
Definition
|
|
Term
what is more common ALL or AML |
|
Definition
|
|
Term
peak age of incidence of ALL |
|
Definition
|
|
Term
|
Definition
most leukemias come with easy bleeding as a sign/sx as well as easy bruising |
|
|
Term
major features of idiopathic thrombocytopenic purpura |
|
Definition
bleeding, bruising low platelets |
|
|
Term
|
Definition
NO, unlike ALL, ITP does not present with and leukocyte or RBC abnormalities |
|
|
Term
major features of aplastic anemia |
|
Definition
|
|
Term
what do lymphocytes look like in mononucleosis |
|
Definition
they actually look the same as leukemic lymphoblasts |
|
|
Term
|
Definition
hi leukocytes that resemble a leukemia |
|
|
Term
what causes a leukamoid reaction |
|
Definition
sepsis pertussis acute hemolysis granulomatous disease vasculitis |
|
|
Term
what is most commonly the misdiagnosis of a pt with ALL |
|
Definition
juvenile rheumatoid arthritis, they can get hepatosplenomegally |
|
|
Term
end-all diagnostic test for ALL |
|
Definition
|
|
Term
what is the typical leukocyte count for kids with ALL |
|
Definition
|
|
Term
can you dx leukemia with a peripheral blood smear? |
|
Definition
no, sometimes you wont see the leukemic blasts in peripheral smear, though if you do its a good indication to investigate further. bm biopsy is the best test. |
|
|
Term
most common site for bone marrow aspiration |
|
Definition
|
|
Term
normal marrow concentration of blasts |
|
Definition
|
|
Term
normal marrow concentration of blasts in ALL |
|
Definition
must have a minimum of 25% to dx ALL |
|
|
Term
who has a better prognosis in ALL, boys or girls |
|
Definition
|
|
Term
what age group has the worst ALL prognosis |
|
Definition
those less than 1 yr andn those over 10 |
|
|
Term
what leukocyte counts have a worse prognosis in ALL? |
|
Definition
pts with HIGHER leukocyte counts (over 50K) have a WORSE prognosis |
|
|
Term
where else do you have to look for leukocytes in ALL (besides bone marrow and peripheral smear? |
|
Definition
also check CSF abnormal leukocytes in CSF = worse |
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
what is combination chemotherapy? |
|
Definition
step-wise chemo 1. Induction 2. Consolidation 3. Prophylactic CNS tx 4. Maintenance |
|
|
Term
|
Definition
prednisone vincristine asparaginase --this causes remission in about 4 wks in most kids with uncomplicated ALL |
|
|
Term
|
Definition
neuropsych issues seizures leukenceophalopathy |
|
|
Term
|
Definition
growth hormone deficiency |
|
|
Term
ade of cylcophosphamide in tx ALL of children |
|
Definition
|
|
Term
|
Definition
DS increases risk for ALL also kids who have siblings with DS are at higher risk for ALL |
|
|
Term
chemotherapy and immunizations |
|
Definition
children CANNOT have any immunizations while on chemotherapy |
|
|
Term
best initial screening to test if trying to differentiate twix ALL and juvenille rheumatoid arthritis |
|
Definition
CBC with dx and platelets |
|
|
Term
|
Definition
|
|
Term
step-wise management of blue/gray baby born with a heart beat but not breathing spontaneously |
|
Definition
1. try O2 and physical stimulation to spark spontaneous breathing 2. back and mask 3. endotracheal intubation |
|
|
Term
what do babies born to uncontrolled diabetics look like? |
|
Definition
they are large they are usually blue/grey because they are often born in respiratory distress |
|
|
Term
what are the insulin and glucose levels in babies born to uncontrolled diabetic mothers? |
|
Definition
hyperinsulinemia hypoglycemia |
|
|
Term
how do you manage a newborn with hypoglycemia? |
|
Definition
1. check glucose 2. if 25-40 feed the baby 3. if less than 25 give IV glucose |
|
|
Term
besides hi insulin and lo glucose, what other blood abnormalities are found in infants born to diabetic mothers |
|
Definition
hypocalcemia hyperbilirubinemia polycythemia |
|
|
Term
glucose levels that dx gestational diabetes (GDM) |
|
Definition
|
|
Term
|
Definition
|
|
Term
at what hct is polycythemia treated in infant |
|
Definition
|
|
Term
how is polycythemia treated in infant |
|
Definition
partial exchange transfusion |
|
|
Term
caudal regression syndrome |
|
Definition
rare congenital malformation found in GDM babies characterized by hypoplasia of the sacrum and lower extremities |
|
|
Term
when are women screen for GDM |
|
Definition
|
|
Term
why are babies born from GDM so big? |
|
Definition
growth hormone effects of insulin |
|
|
Term
where is the weight distributed in macrosomia |
|
Definition
|
|
Term
why elevated bilirubin in babies from GDM |
|
Definition
because of the polycythemia because of the o2 insufficiency because the babies are too big |
|
|
Term
signs of hypocalcemia in babies of GDM |
|
Definition
irritability decreased cardiac contractility |
|
|
Term
what other congenital defects are possible in babies of GDM (besides caudal regression syndrome) |
|
Definition
neural tube defects congenital heart disease |
|
|
Term
|
Definition
insulin dependant AND vascular disease |
|
|
Term
what is at greatest risk of thrombosing in babies with polycythemia |
|
Definition
|
|
Term
|
Definition
|
|
Term
common heart abnormality in babies of GDM |
|
Definition
septal hypertrophy subaortic stenosis cardiomyopathy |
|
|
Term
|
Definition
insulin requiring but without vascular disease |
|
|
Term
|
Definition
|
|
Term
|
Definition
insulin dependant with no vasc disease |
|
|
Term
baby with respiratory distress and born to GDM who still has bradycardia after ET intubation? |
|
Definition
suspect cardiac abnormality (from hypocalcemia) |
|
|
Term
baby born to GDM that is managed initally but then presents with new abdo mass? |
|
Definition
suspect hydronephrosis from renal vein thrombosis |
|
|
Term
surfactant and babies from GDM |
|
Definition
they will have surfactant deficiency and RDS at less premature ages than other babies |
|
|
Term
head circumference in babies born to GDM |
|
Definition
|
|
Term
baby that is SMALL for gestational age and has a mother with GDM |
|
Definition
suspect placental insufficiency |
|
|
Term
small left colon syndrome |
|
Definition
seen in babies from GDM, can result in obstruction that is seen as decreased meconium passage |
|
|
Term
does breast feeding increase or decrease risk for hyperbilirubinemia |
|
Definition
|
|
Term
which is neurotoxic conjugated or unconjugated bilirubin? |
|
Definition
|
|
Term
|
Definition
increased rbc destruction due to transplacental maternal ab passage active against the infants RBC antigens |
|
|
Term
what parts of brain are more susceptible to kernicterus |
|
Definition
|
|
Term
signs of kernicterus in infant |
|
Definition
loss of moro reflex lethargy poor feeding |
|
|
Term
what is direct bilirubin attached to |
|
Definition
|
|
Term
what is high, direct or indirect bilirubin, in physiological hyperbilirubinemia of a newborn |
|
Definition
|
|
Term
what are signs of non-physiological jaundice |
|
Definition
1. appearance in the first 24-36 hrs 2. rise over 5mg/dl/24hrs 3. total over 12mg/dl 4. persists after 10-14days |
|
|
Term
what infection causes non physiological jaundice |
|
Definition
rubella and toxoplasmosis |
|
|
Term
work up of hyperbilirubinemia in newborn |
|
Definition
if suspect physiological just monitor bili levels
if more suspicious or persistant do CBC, coombs, lft, peripheral smear |
|
|
Term
tx unconjugated bilirubinemia |
|
Definition
|
|
Term
when do you start phototherapy |
|
Definition
if bili is 16-18mg/dl in day 1-3 of life after day 3 only do if bili is 20+ |
|
|
Term
what if phototherapy does not work |
|
Definition
|
|
Term
what is deficient in fcrigler najjar |
|
Definition
|
|
Term
what drug given to mother reduces neonatal jaundice (tho it is not used therapeutically) |
|
Definition
barbituates induce glucuronyl transferase --> decreased risk of jaundice |
|
|
Term
how do sepsis and acidosis effect bilirubinemia? |
|
Definition
they increase the risk of kernicterus from hyperbilirubinemia by weakening BBB |
|
|
Term
coombs test in gilberts syndome? |
|
Definition
|
|
Term
why do babies get physiological jaundice? |
|
Definition
they have physiological reduction in UDGT |
|
|
Term
faint wheezes in an asthmatic |
|
Definition
indicates that the asthma exacerbation is more severe and less air is passing thru the airways |
|
|
Term
cardiovascular findings in asthmatic |
|
Definition
|
|
Term
|
Definition
blood pressure that varies more widely with respiratoration than normal. bp variance greater than 10mm Hg between inspiration and experiation |
|
|
Term
other things that cause pulsus paradoxus |
|
Definition
other obstructive airway disease pericardial tamponade constrictive pericarditis |
|
|
Term
|
Definition
kids who get RSV bronchiolitis develop asthma |
|
|
Term
acute v late phase of asthma |
|
Definition
acute: immediate IgE response --> vasodialation and leaky cappillaries, sm mm constriction, mucus secretion
late: infiltration of inflammatory cells into the airway parenchyma --> causes the chronic inflammation seen in asthma |
|
|
Term
|
Definition
|
|
Term
do you use corticosteroids for acute or chronic asthma tx |
|
Definition
|
|
Term
when does the late phase reaction occur |
|
Definition
2-4 hours after initial wheezing episode |
|
|
Term
sudden onset respiratory symptoms fever in a baby who was previously healthy |
|
Definition
|
|
Term
|
Definition
hyperinflated lungs atelectasis |
|
|
Term
|
Definition
|
|
Term
What labs do you want to test in a pt suspicious for GH deficiency |
|
Definition
|
|
Term
What does the growth curve look like in a pt with GH def |
|
Definition
they "fal off " the curve |
|
|
Term
are the bodies of children with GH deficiency proportional to head size?` |
|
Definition
|
|
Term
|
Definition
GH injections until full size is reached |
|
|
Term
|
Definition
how old the bones appear compared to the chronological age
provide an extimate of the rematining growth pot'l of the bones |
|
|
Term
consititutional growth delay (CGD)? |
|
Definition
when a healthy child grows slower than expected
seen in kids who had a parent who was also a "late bloomer" |
|
|
Term
will the bone age be >,< or = the chronolocial age in a kid with CGD |
|
Definition
|
|
Term
|
Definition
age at which the child's measured height is in the 50%ile |
|
|
Term
growth RATE in CGD vs GH deficiency |
|
Definition
in CGD the rate is normal
in GH def the rate slows |
|
|
Term
how does the bone age compare to the chronological age in a kid with familial short stature |
|
Definition
|
|
Term
|
Definition
usually in school aged kids |
|
|
Term
what do you always want to check in girls who "fall off" growth curve? |
|
Definition
|
|
Term
other endocrine cause of short stature |
|
Definition
|
|
Term
early onset sepsis syndrom |
|
Definition
sepsis in the first 6d of life |
|
|
Term
prophy for GBS sepsis in neonate |
|
Definition
give IV penicillin or ampicillin during labor |
|
|
Term
|
Definition
respiratory distress tachypnea bradycardia hypotn |
|
|
Term
most common first sign of neonate sepsis |
|
Definition
|
|
Term
pallor and delayed cappillary refill in neonate? |
|
Definition
indicates overwhelming shock |
|
|
Term
platelets in neonate with sepsis |
|
Definition
|
|
Term
other non GBS causes of early onset neonate sepsis |
|
Definition
|
|
Term
respiratory signs of sepsis |
|
Definition
grunting refuse to feed apnea cyanosis tachypnea |
|
|
Term
risk factors for GBS neonate sepsis |
|
Definition
premie mother under 20 yo ROM over 18 hrs from delivery intrapartum fever |
|
|
Term
when do you screen for GBS |
|
Definition
|
|
Term
tx early onset sepsis in neonate? |
|
Definition
IV AMG + penicillin or ampicillin |
|
|
Term
tx late onset sepsis in neonate? |
|
Definition
vanco or 3rd gen cephalosporin |
|
|
Term
newborn with puffy eyelids red conjuctivae, purulent eye discharge in 3rd day of life? |
|
Definition
|
|
Term
time frame of gonno opthalmia vs chlamydial conjuctivae? |
|
Definition
gonno in day 2-5 after birth chlam day 5014 |
|
|
Term
tx chlamdyia conjuctivitis? |
|
Definition
|
|
Term
risk of oral erythromycin in newborn |
|
Definition
hypertrophic pyloric stenosis |
|
|
Term
managment of a baby born to a well-managed HIV+ mother (mom was on antivirals during pregnancy to keep viral load low) |
|
Definition
still give baby ziduvine after brith |
|
|
Term
transient tachypnea after birth |
|
Definition
result of incomplete evacuation of fetal lung fluid in a healthy term infant |
|
|
Term
management of transient tachypnea in newborn |
|
Definition
|
|
Term
how do you know a baby with tachypnea has transient tachypnea vs another problem? |
|
Definition
they will be tachpneic but still have a good sucking in of air with each breath |
|
|
Term
sick neonate with diffuse rash that has monocytosis and eventually dies? |
|
Definition
|
|
Term
age that SIDS is most likely |
|
Definition
1mo-5mo risk upto 10yr of life |
|
|
Term
sleep position that INCREASES risk for SIDS |
|
Definition
|
|
Term
|
Definition
pulmo htn --> R2L blood shunt |
|
|
Term
causes of eisenmenger syndrome |
|
Definition
VSD atrioventricular canal lesion PDA |
|
|
Term
causes of wide pulse pressure |
|
Definition
fever hyperthyroid anemia AVF PDA |
|
|
Term
|
Definition
pulmo congestion systemic hypoperfusion |
|
|
Term
are acyanotic heart defects usually LtoR or RtoL |
|
Definition
|
|
Term
how can a person switch from an acyanotic heart defect to becoming cyanotic? |
|
Definition
initially blood is shunted to lungs, then once pulmo congestion causes pulmo HTN the flow switches |
|
|
Term
most common heart lesion in kids |
|
Definition
|
|
Term
what part of septum do most VSDs occur |
|
Definition
|
|
Term
|
Definition
harsh holosystolic left sternal border |
|
|
Term
what if a VSD does not sound very harsh? |
|
Definition
this is actually a bad sign, means there is a big hole |
|
|
Term
signs of severe VSD in newborn |
|
Definition
dyspnea poor feeding sweating when feeding ftt |
|
|
Term
|
Definition
after first few weeks of life, even into months, present late as the pulmo pressure decreases |
|
|
Term
|
Definition
cardiomegally and pulmo vasc congestion |
|
|
Term
direction of shunt in VSD |
|
Definition
|
|
Term
|
Definition
yes, biventricular. both sides hypertrophy |
|
|
Term
what happens to small vsds? |
|
Definition
close spontaneously 6-12 months |
|
|
Term
|
Definition
diuretics (furosimide or hydrochlorothiazide) |
|
|
Term
|
Definition
|
|
Term
after how long will a baby with a vsd start to develop pulmonary vascular resistance |
|
Definition
|
|
Term
when does eisenmenger usually happen? |
|
Definition
|
|
Term
what are the major acyanotic heart defects? |
|
Definition
VSD ASD PDA atrioventricular septal defects |
|
|
Term
function of ductus arteriosus |
|
Definition
shunts blood from pulmonary arteries to aorta to bypass lungs |
|
|
Term
what causes normal closure of ductus arteriosus |
|
Definition
|
|
Term
when does ductus normally close |
|
Definition
always at least by 2 days |
|
|
Term
what does PDA sound like? |
|
Definition
systolic or continous machine like murmer |
|
|
Term
when do you NOT close a PDA |
|
Definition
if they also have an interuption in aortic flow. like coarc of aorta |
|
|
Term
|
Definition
most are asymptomatic large ones --> mild growth failure, exercise intolerance |
|
|
Term
|
Definition
|
|
Term
murmur of ASD diastolic or systolic? |
|
Definition
|
|
Term
|
Definition
left upper and midsternal border |
|
|
Term
|
Definition
enlarged RA, RV and pulmo artery |
|
|
Term
what hypertrophies in ASD |
|
Definition
|
|
Term
|
Definition
usually nothing in children in adults may lead to pulmo HTN |
|
|
Term
management of atrioventricular septal defect |
|
Definition
|
|
Term
signs of untreated atrioventricular septal defect |
|
Definition
ftt heart failure recurrent pulmo infections |
|
|
Term
most common downs syndrome heart defect |
|
Definition
arioventricular canal defect |
|
|
Term
|
Definition
|
|
Term
cyanotic heart lesions make the skin look _________ |
|
Definition
|
|
Term
what besides severe coarc of aorta requires a pda for life? |
|
Definition
transposition of the great vessels |
|
|
Term
what direction is the shunt in transposition of great vessles (TGV) |
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
prostaglandins to keep PDA
then atrial septostomy |
|
|
Term
|
Definition
creating an atrial septum |
|
|
Term
is pulmonary valve stenosis cyanotic or acyanotic? |
|
Definition
|
|
Term
diseases with pulmonary stenosis |
|
Definition
glycogen storage disease noonans syndrome |
|
|
Term
boot shaped heart and dec pulmo vasc |
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
supracardiac shadow caused by the anomalous pulmo veins endering the innominate vein and persistant left vena cava |
|
|
Term
child assuming a squatting position after a bout of agitation or stress |
|
Definition
|
|
Term
tricuspid valve abnormalities are cyanotic or acyanotic |
|
Definition
|
|
Term
|
Definition
|
|
Term
does ebstein anomoly require pda |
|
Definition
|
|
Term
major features of pulmonic stenosis heart murmur |
|
Definition
systolic murmur mitral area to back loud in axilla |
|
|
Term
heart defects in tetralogy |
|
Definition
overriding aorta pulmonary stenosis rvh vsd |
|
|
Term
body aches that wax and wane with fever for a long time (20 days) |
|
Definition
juvenille rheumatoid arthritis (JRA) |
|
|
Term
|
Definition
rash spiking fevers that come and go joint swelling LAD organomegallly |
|
|
Term
child with exercise induced cyanosis and a systolic murmur |
|
Definition
|
|
Term
|
Definition
leukocytosis thrombocytosis anemia |
|
|
Term
|
Definition
|
|
Term
ddx for fever of unknown origin in a child |
|
Definition
infectious hematologic rheumatologic |
|
|
Term
what type of fever seen in JRA |
|
Definition
|
|
Term
arthritis characteristics |
|
Definition
1. swelling/effusion and 2+ of following -limited ROM -tenderness/pain on motion -increased heat in 1+ joint |
|
|
Term
|
Definition
pauciarticular = 1-4 joints effected polyarticular = 5+ joints |
|
|
Term
more severe systemic signs of JRA |
|
Definition
pericarditis pleural effusion hepatitis encephalopathy |
|
|
Term
|
Definition
irritation of the iris seen in the early onset pauciarticular type of JRA |
|
|
Term
|
Definition
|
|
Term
|
Definition
seen in late onset pauciarticular type of JRA |
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Term
who gets early v late onset pauciarticular type of JRA |
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Definition
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Term
sore throat postauricular and occipatal LAD joint swelling |
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Definition
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Term
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Definition
salmon colored and diffuse |
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Term
who gets slipped capital femoral epiphysis |
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Definition
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Term
what makes pain better or worse in pericardidits |
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Definition
better when leaning forward worse when taking deep breaths or coughing |
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Term
EKG of pericarditis in kid |
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Definition
ST elevation low voltage qrs complexes |
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Term
systemic onset disease JRA |
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Definition
type of JRA with more systemic signs (rash, organomegally, LAD) |
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Term
what do hypothyroidism and trisomy 21 have in common |
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Definition
both cause macrocytic anemia |
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Term
what part of GI tract absorbs b12 |
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Definition
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Term
juvenile pernicious anemia |
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Definition
child cannot secrete intrinsic factor |
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Term
besides malabs or malnutrition what else can cause b12 def |
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Definition
diphyllobothrium lactum (tapeworm) |
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Term
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Definition
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Term
unpastuerized goats milk risk |
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Definition
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Term
signs of juvenile pernicious anemia |
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Definition
weakness glossitis anemia and fatigue |
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Term
what part of gi makes GIF |
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Definition
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Term
where does diphyllobothrium come from |
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Definition
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Term
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Definition
alternate name for lead poisoning |
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Term
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Definition
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Term
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Definition
anorexia hyperirritability/emotional lability decreased playing in kids speech regression abdo pain and bone pain vomiting/constip |
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Term
signs of lead encephalopathy |
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Definition
persistant vomiting ataxia ams coma sz |
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Term
long term complic of lead |
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Definition
cognitive deficits aggressive behaviour |
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Term
where is lead stored in the body |
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Definition
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Term
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Definition
CaEDTA chelation DMSA aka BAL |
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Term
at what level do you consider chelation |
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Definition
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Term
what do you do if pt has level 10-14 of lead |
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Definition
educate close monitor (recheck in 3mo) |
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Term
at what lead level do you start environmental investigation |
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Definition
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Term
when do you do long bone xr in lead poisoning |
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Definition
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Term
infants exposed to mercury show what signs |
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Definition
microcephaly, LBW, sz developmental delay hearing loss |
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Term
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Definition
ataxia blurry vision tremors smell and taste issues dementia |
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Term
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Definition
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Term
chronic arsenic exposure signs |
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Definition
skin lesions peripheral neuropathy encephalopathy |
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Term
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Definition
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Term
signs of orellanine poison |
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Definition
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Term
signs of ethanol toxicity in children |
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Definition
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Term
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Definition
hypertension dialated pupils hyperthermia |
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Term
lab studies in post-strep glomerulonephritis |
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Definition
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|
Term
what will c3 and c4 look like in post strep glomerulonephritis |
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Definition
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|
Term
other signs of post strep gn |
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Definition
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Term
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Definition
IgA nephropathy recurrent painless hematuria preceded by URI |
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Term
Henoch Schonlein purpura major features |
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Definition
nephritis palpable purpura 4-5 yo Purpura, Abdominal Pain, Arthralgias and Hematuria |
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Term
what else has nephritis with low c3 |
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Definition
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Term
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Definition
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Term
do abx decrease the risk of post strep gn? |
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Definition
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Term
interval twix GBS throat and post strep gn? for impetigo and post strep gn? |
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Definition
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Term
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Definition
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Term
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Definition
supportive maintain fluid balance with restriction/supplementation |
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Term
risks of IgA nephropathy? |
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Definition
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|
Term
benign familial hematuria |
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Definition
AD persistent/intermittent hematuria without preogression to CKD |
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|
Term
biopsy of benign familial hematuria |
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Definition
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|
Term
immune complex deposition with IgA in mesangium? |
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Definition
IgA Nephropathy or henoch schonlein purpura |
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|
Term
endothelial cell swelling with fibrin deposition |
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Definition
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Term
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Definition
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Term
kidney issues and oral ulcers? |
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Definition
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|
Term
|
Definition
wilms tumor gonadal dysgenesis nephropathy |
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Term
first tests to do in precocious puberty |
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Definition
FSH LH bone age radiograph |
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|
Term
what makes you more suspicious for central vs non-central precocious puberty? |
|
Definition
if under 6yo, more likely to be central
if male central more likely |
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|
Term
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Definition
no signs of puberty in girls by age 13 |
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|
Term
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Definition
2ndary sex characteristics in girls before 8 and boys before 9 |
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|
Term
precocious pseudopuberty and causes |
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Definition
gonadotropin INDEPENDANT cause of puberty
exogenous hormone or adrenal/ovarian tumors usually the cause |
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Term
|
Definition
early active adrenal androgens (hi pubic/axillary hair and vertical growth) |
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Term
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Definition
early breast development without the pubic/axiallary hair |
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|
Term
cause of true precocious puberty |
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Definition
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|
Term
bone growth in girls with central precocious puberty |
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Definition
if under 6yo early maturtion of bone loss of ultimate height portential
if over 6yo they have slowly maturing bones with preserved height potl |
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|
Term
most significant family history in precocious puberty |
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Definition
any hx of congenital adrenal hyperplasi |
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|
Term
scrotum in prepubertal and pubertal boy |
|
Definition
prepubertal = thick and non vascular scrotum
pubertal= thin and vascular |
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|
Term
test LH in precocious puberty |
|
Definition
use immunometric assay. With this test LH should be undectable in normal prepubescent children |
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|
Term
tx cetnral precocious puerty *moa) |
|
Definition
GnRH agonist to desensitize pituitary |
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|
Term
|
Definition
1 = flat 2 = buds 3 = buds + areola grow 4 = nipple and areola form separate mound from breast 5 = areola rejoins berast contour, end |
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|
Term
|
Definition
1 = none 2 = straight and fine 3 = coarser darker hair 4 = adult but limited area 5 = full |
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|
Term
when do tanner stages 2 start |
|
Definition
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|
Term
at what tanner stages does penis grow in length v width |
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Definition
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Term
virilization in child with PREPUBERTAL testes |
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Definition
think congenital adrenal hyperplasia and check 17alpha deoxyhydrogenase |
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|
Term
most common cause of fullterm newborn with ambiguous genital |
|
Definition
congenital adrenal hyperplasia |
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|
Term
inheritance of congenital adrenal hyperplasia |
|
Definition
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|
Term
|
Definition
discrepancy between gonad morphology and external genitalia |
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|
Term
female pseudohermaphroditism |
|
Definition
XX with ambigous gennitalia |
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|
Term
most common cause of female pseudohermaphroditism |
|
Definition
congenital adrenal hyperplasia |
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|
Term
male pseudohermaphroditism |
|
Definition
XY with ambiguous genitalia |
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|
Term
|
Definition
xx or xy with opposite internal sex organs (ovary and testes) |
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|
Term
|
Definition
1. usually a mixed 46 XY/45XO 2. testis with sertoli and leydig cells but no germinal elements 3. maybe a streak ovary on one side 4. labioscrotal fusion |
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|
Term
hyperpigmentation of labioscrotal folds |
|
Definition
more common infants with congenital adrenal hyperplasia |
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|
Term
most important factor in determining and infants sex assignment |
|
Definition
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|
Term
what cells used to find karyotype |
|
Definition
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|
Term
circumcision and hypospadias |
|
Definition
dont do it foreskin will be used for reconstruction |
|
|
Term
hypotonia, small penis, cryptoorchidism |
|
Definition
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|
Term
salt-losing CAH is deficiency of what |
|
Definition
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|
Term
features of salt losing CAH |
|
Definition
FTT gi obstructive signs and vomit cardiovascular collapse |
|
|
Term
testicular feminization results from |
|
Definition
-decreased androgen binding to target or androgen insensitivity - |
|
|
Term
testicular feminization karyotype |
|
Definition
|
|
Term
appearance of testicular feminization |
|
Definition
phenotypical females short or atretic vagina |
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|
Term
later signs of prader willi |
|
Definition
|
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