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156 Cards in this Set
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- Back
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Pt with Turner's - 45XO/46XY - what should you be thinking of?
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Higher risk of gonadoblastoma - need gonadectomy
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The only congenital heart disease that does not predispose to endocarditis
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ASD
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Organ most at risk with HUS?
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kidneys
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Infant born to mom with no prenatal care has an infected umbilical cord stump. Gets poor sucking, fatigue, rigidity, spasms. Dx?
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Neonatal tetanus
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Recurrent giardia infections - should make you think of...
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IgA deficiency. IgA inhabits GItract, which is where giardia does its damage.
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Treatment of pinworm infection
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mebendazole
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Main complication after surgical repair of heart after congential heart defect
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pericardial effusion
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Pt has seizures, unilateral cavernous hemangioma (leading to a change in pigmentation), intracranial tram-line calcifications, hemianopia, hemiparesis... dx?
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sturge-weber syndrome
that is a port-wine stain |
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how common is vsd? what is the murmur like
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most common congen heart defect
holosys murmur over L sternal border and diastolic murmur due to increased flow through the mitral |
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symptoms of congenital rubella
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cardiac defects, cataracts, deafness
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symptoms of congenital rubella
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chorioretinitis, hydrocephalus, intracranial calcifications
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little kid has big unilat cervical LAD, fever, malaise after animal exposure - dx?
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tularemia (francisella tularensis)
without animal exposure and acute onset would be due to staph aureus (lymphadenitis once the node is erythematous and tender) |
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what is cutis marmorata
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lace-like pattern on skin in response to stress or cold. can be there in pts with downs or trisomy 18 (Edwards)
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Niacin deficieny - signs/sx
which b vit? |
B3 def
Diarrhea, dermatitis, dementia (and death if severe) - pellagra consider in pt with ibd. May have glossitis |
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thiamine def
which b vit? and dx? |
B1
beriberi or wernicke-korsakoff (vision changes, ataxia, memory loss) |
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riboflavin - which b vit and sx?
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b2
chelosis, glossitis, seborrheic dermatitis, pharyngitis, edema/erythema of mouth |
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pyridoxine - which b vit? sx of deficiency?
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irritability, depression, dermatitis, stomatitis, increased homocysteine --> thrombombolism
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cyanocobalamine - which b vit?
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b12
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good abx to use when staph is a major concern, but still worried abt strep
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dicloxacillin. penicillin and amoxicillin will get inactivated by staph's beta lactamases
cephalexin or clinda are good too. |
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When do you see Burr cells?
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uremia or due to preparation of sample
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21-hydroxylase deficiency - will males or females have abnormal genitals at birth?
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females
so dx is later in males |
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galactose metabolic pathway disorders (2)
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galactose-1-phosphate uridyl transferase deficiency - more severe - shock if untreated. many findings with this
galactokinase deficiency - milder presentation both are AR and have cataracts associated |
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criteria for rheumatic fever
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history of GAS infxn
Joints (migratpry polyarth) O - carditis N - subcut nodules E - erythema marginatum S - sydenham's chorea |
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langerhan's cell histiocytosis - presentation?
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lytic bone lesions, mild hypercalcemia.
think abt it if someone (incl. child) has solitary painful lytic bone lesion. also think of neoplasms in this case.... |
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McCune-Albright syndrome - presentation
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3 Ps - prec puberty, pigmentation, polyostotic fibrous dysplasia of bones
precoc. puberty, cafe au lait spots, bone defects, endocrine abnormalities. cushing-like defects too |
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Peutz-Jeghers syndrome - presentation?
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GIT polyposis and mucocut. pigmentation. May have prec puberty with it
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Von Recklinghausen syndrome - what are skin lesions like?
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small, regular borders, may have axillary/genital freckles
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Sturge-Weber syndrome - presentation?
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MR, seizures, visual changes, port-wine stain over trigem nerve distribution.
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Tx of breast mild jaundice (as opposed to breast feeding failure jaundice)
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temporarily stop breast feeding
cause is likely a substance in breast milk to inactivates UDP glucoronosyl transferase |
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characteristics of an innocent murmur
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<2/6, changes with position, systolic, no other sounds.
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edema of turners syndrome - pitting or nonpitting?
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nonpitting - due to dysgenesis of lymphatic network
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GBS meningitis - can it occur in a baby of 2 weeks old?
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yes! there is a late-onset variety
early is sepsis, late is meningitis |
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MCCs of neonatal meningitis (incl 2 week olds)
1-3 |
1.) GBS 2.) E coli 3.) Listeria
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Child ingested poison - steps of management?
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1.) airway
2.) endoscopy to assess extent of damage |
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which children are more sick - measles or rubella?
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measles
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roseola infantum/HHV 6 - do they have physical exam signs at the same time as their fever?
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no
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transient tachypnea of the newborn - findings
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clear lungs with prominent vascular markings, fluid lines in fissures, overaeration, flat diaphragm.
probably won't see hypoxemia or acidosis or hypercapnia |
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female infant <3 months has vaginal bleeding - is that ok?
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yeah - may still be maternal hormone withdrawal
suspect abuse if it is odored or discolored |
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neonate has conjuncitivitis - topical erythro or oral?
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oral bc if it is chlamydial, you want to prevent chlamydia pneumonia
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is silver nitrate ophthalmic drop effective against chlamydia?
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no
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difference btwn slipped cap femoral epiphysis and legg-calve-perthes disease
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SCFE - adolescent obese kids LCP- younger children - idiopathic infarction of femoral head
both can result in avascular necrosis of femoral head |
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recurrent hemarthrosis (e.g. in hemophiliac) can lead to...
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iron deposition and synovial thickening with fibrosis of joints
due to the iron and cytokines in the blood having a proliferative effect on the joint |
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MCC of subarachnoid hemorrhage in children
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AVM rupture into subarachnoid space
the avm may also cause seizures and migraine-like HAs |
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RSV causing bronchiolitis may increase risk of...
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asthma later in life
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things that can cause a continuous murmur in a child
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PDA
coaractation of aorta due to collaterals developing btwn hyper and hypoperfused vessels! |
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MCC polycythemia in term infants
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delayed clamping of umb cord so excess transfer of placental blood
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Manifestations of polycythemia in a newborn
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resp distress, poor feeding, neurologic manifestations
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what vitamin can help with measles
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A
does it by enhancing immunity and increasing resp and GI epithelium growth |
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medulloblastoma - where does it develop?
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cerebellar vermis - so they have issues with gait, trunk dystaxia, nystagmus, etc.
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patients with congenital heart dz and neuro issues - what is going on
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brain abscess - more likely with R to L shunts
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Dx of PKU (2)
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blood phenylalanine levels (quant) or Guthrie urine test (qualitative)
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Tumor that has concentric whorls and calcified psammoma bodies
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meningioma
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defect in lesch-nyhan syndrome and what is elevated?
what can these boys get? |
hypoxanthine-guanine phosphoribosyl transferase
uric acid levels are increased gout at an early age |
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TB meningitis - csf findings
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slightly increased cell count with increased lymphocytes
very high protein, low glucose. subacute clinical picture |
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normal amt that hemoglobin will drop once baby is born
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30%. more than that is pathologic
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vesicoureteral reflux can lead to...
how to dx it |
utis-->renal scarring-->renal failure or HTN in a child
voiding cystourethrogram or radionuclide cystogram |
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pt is obese, MR, almond shaped eyes, narrow bifrontal diam of head, short, microphallus, cryptorchidism - dx?
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prader-willi syndrome
this is the one where phenotypic expression depends on whether mom or dad gave it to you |
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pt has hypoglycemia, macroglossia, visceromegaly, characteristic earlobe crease - dx?
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Beckwith-Wiedemann syndrome
easy to confuse this with maternal DM or hypothy (will have umb hernia, not omphalocele) |
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immune mediated thrombocytopenia - when to tx?
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if platelets are <30k with steroids.
if over that - don't worry it will resolve on its own. |
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tx of absence seizures
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ethosuximide or valproic acid
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drug of choice for partial seizures
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phenytoin
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which brain tumors are common in kids
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infratentorial more common than supra. but benign astrocytomas are most common in both categories.
if supra, can occur in cerebral hemispheres second most common is medulloblastoma (cerebellar vermis) |
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ekg findings of WPW syndrome
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shorted pr interval, delta wave, wide qrs
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tx of septic joint in a child
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SURGICAL DRAINAGE!!!!!
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Diamond-Blackfan syndrome/congenital hypoplastic anemia
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macrocytic, low retics, congenital anomalies (shield chest, webbed neck, triphalangeal thumbs etc.)
only affects red cells |
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fanconis anemia
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AR inheritance.
progressive pancytopenia and macrocytosis will also have cafe au lait spots, microcephaly, short, horseshoe kidneys, absent thumbs |
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Gaucher's disease - what is deficient, who is at risk, pathological sign
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acid beta-glucosidase
ashkenazi jews fatigue, bruising, bone pain. crinkled cells on pathology on xray you will also see erlenmyer flask deformity of distal femur |
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you can have blue sclera with...
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marfans or osteogenesis imperfectica
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Riboflavin deficiency - Which vit and sx?
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B2
edematous mucus membranes, cheilitis, stomatitis, glossitis, photophobia |
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Niacin deficiency - which vit and sx?
who can you see it in? |
BPellagra - Vit B3
red rash, red tongue, diarrhea, vomiting. seen in alcoholics and those on isoniazid and carcinoid syndrome/hartnup dz |
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Thiamine def - which vit and sx?
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B1
wet and dry berberi and wernicke-korsakoff syndrome |
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how to confirm dx of laryngomalacia
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direct laryngoscopy - will see epiglottis rolled in from side to side
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eroding abscess in oral cavity, lungs or GIT that has yellow "sulfur" granules - bug?
tx? |
actinomyces israelii
penicillin |
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child has congenital lesion causing him to pass small amts of urine and midline mass in lower abd - dx?
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posterior urethral valves
dx with voiding cystourethrogram |
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treatment for lyme dz
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DEPENDS ON AGE!!!
<9 - amox >9 - doxy and if stage 3 (disseminated) ceftriaxone or pen G |
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tx of hemolytic uremic syndrome
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supportive - plasmapheresis, dialysis if necc, steroids
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neonatal abstinence syndrome - presentation
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irritability, high pitched cry, tremors, sneezing, sz, diarrhea.
due to heroin/methadone |
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prenatal exp to phenytoin causes...
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fetal hydantoin syndrome - nail hypoplasia, MR, dysmorphic facies
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fetal exp to valproic acid causes...
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NT defects, cardiac anomalies, abn facies
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pt with sickle cell has painful joint that is not erythematous or tender to palpation - dx?
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osteonecrosis/avascular necrosis
typically in humeral and femoral heads |
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GI and cardiac anomalies assoc with Down's syndrome
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Hirschsprungs dz
esoph atresia pyloric stenosis malrot of bowel endocardial cushion defect VSD PDA |
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spondylolisthesis
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devo disorder - forward slip of vertebrae - you can palpate a "step-off"
there will be neuro dysfunction with this |
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normal for a kid to occasionally masterbate?
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yes
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baby had shoulder dystocia and now that side has no moro reflex - dx and tx?
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clavicle fracture. don't need to tx
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how to differentiate septic arthritis vs. transient synovitis (4)
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septic (need 3/4)
wbc>12k temp > 39 esr > 40 won't bear weight. if transient - just need to do nsaids and rest. usually follows a URI or mild trauma |
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glycogen storage diseases
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1 - glucose-6-phosphatase deficiency/von gierke's
2 - acid maltase def/Pompe's 3 - glycogen debranching enzyme def 4 - branching enzyme deficiency/amylopectinosis |
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congenital problems in an infant of a diabetic mother
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caudal regression syndrome
transpos of great vessels duod atresia and small left colon anencephaly and NT defects |
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pt is tall, thin, hyperlaxity of skin and joints, and had a stroke - dx?
tx? |
homocytinuria (deficiency in cystathionine synthase)
high doses of bit b6 and restric of methionine with supplem of cysteine |
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lens dislocated uo - dx?
down - dx? |
up-marfans
down - homocystinuria |
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when do you see sail sign on cxr and what is it?
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before 2 years.
thymic shadow |
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how to dx HIV in an infant
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serologic testing doesn't work (maternal abs)
need to do pcr, p24 antigen or viral culture |
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Osgood-Schlatter/traction apophysitis - presentation
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pain in knee with sports. often bilateral. edema and tenderness over tibial tubercle with hetertopic bone formation (firm mass felt)
pain when extending knee against resistance. |
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patellofemoral stress syndrome vs patellar tendonitis
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stress syndrome is in runners. pain when going down stairs.
tendonitis - repetitive kicking or jumping. |
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cxr of meconium aspiration
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hyperinflation with flattened diaphragm bc the meconium acts like a valve
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why is maternal DM a risk factor for RDS?
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fetal insulin antagonizes cortisol in maturing the lungs
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pathologic change seen in minimal change disease
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on electron microscopy you see effacement of foot processes of podocytes - tx with steroids
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MCC nephrotic syndrome in adults
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membranous glomerulonephritis
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specific symptoms that suggest congenital rubella vs. toxo or cmv -
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deafness, cardiac anomalies, purple "blueberry muffin" spots
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triad of congenital rubella
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sensorineural deafness, cardiac malformations, cataracts
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which lysosomal storage diseases have cherry red macula
how to differentiate? |
neimann pick dz and tay sachs
differentiate by niemann pick having HSMegaly and cervical LAD |
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boy has unilat gynecomastia - he is 14 - dx?
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benign pubertal gynecomastia
only need to work up if substantial pain |
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neuroblastoma - does it cross midline?
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yes - usually
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pretern neonate has increased gastric residual volume - dx?
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necrotizing enterocolitis
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neonate has a foot in adduction that over corrects with active and passive manipulation - dx? tx?
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mild metatarsus adductus - reassurance
if it did not correct, need to do orthosis or corrective shoes if completely rigid - manage with serial cast |
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at what age is bedwetting no longer normal?
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5
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MOA of using hydroxyurea in pts with sickle cell dz
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increases fetal hemoglobin levels
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Myotonic muscular dystrophy - presentation and inheritance
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wasting of all muscles but a normal birth. emaciated thin face. slow relaxation of muscles (can't release hand after handshake)
aut dom |
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normal pattern for growth and height in a newnate
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weight - doubles by 5 months, triple by 1 yr
height - up 50% in first yr, doubles by 4 years, tripples by 13 |
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RDW values in iron def anemia, thalessemia and chronic dz
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iron def - highly variable (>20%)
thal or chronic dz - less variable |
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pt with turners - will they have MR?
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no
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pathophys of iron pois and tx?
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corrodes gi mucosa (n/v, hematemesis) and accum in mitochon (metab acidosis). hypotension as well.
radio-opaque treatment is ipecac or IV deferoxamine |
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encepphalopathy and cerebral edema - seen with iron or lead pois?
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lead
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erythema toxicum
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benign condition in neonates - eosinophils around skin lesions (pustules and vesicles)
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difference in pathophys of clostridium botulism in adults and kids
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adults - preformed toxin
kids - organism is in the food and then produces toxin in the GIT |
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when to operate on umbilical hernia in child
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if persists to age 3-4, over 2cm in diam, or symptomatic
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adverse rxns to DTaP vaccine are due to which component?
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pertussis
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newborn doesn't pass stool, has bilious vomiting, and ground glass appearance on abd xrays - dx?
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meconium ileus from CF
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most common congen heart lesion in edward's disease
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VSd
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homogentisic acid is in the urine - dx?
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alkaptonuria (urine is black too)
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lab findings in classic phenylketonuria
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phenylalanine levels > 20
normal to low-normal tyrosine levels increased urinary phenylalanine metabolites (phenyllpyruvic and o-hydroxyphenylacetic acids) normal tetrahydrobiopterin levels |
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Klumpke paralysis - which nerves affected and manifestations
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cervical 7, 8, and first thoracic nerve.
paralysis of that hand and ipsilat horners |
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Erb-Duchenne palsy - which nerves and manifestations
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5th and 6th CERVICAL nerves
absent moro reflex, intact grasp of affected arm, waiters tip |
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findings seen in SGA infants
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hypoxia --> polycythemia
hypogly, hypocalc (less transfer across placenta), hypothermia |
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hsp pathophys and manifestations
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iga vasculitis of small vessels
rash, arthralgias, abd pain, renal disease |
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tx of local impetigo
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topical mupirocin or oral erythromycin
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when is jaundice abnormal?
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in first 36 hours of life.
conjugated. rising > 5/24h persists past day 14 |
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associated things with tricuspid atresia
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LVH/left axis deviation
asd and vsd so blood can flow around (necessary for survival) |
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screening lab for renal tubular acidosis
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low bicarb and high chloride levels
the issue is that they can't reabs bicarb or excrete H+ normal anion gap |
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Freidrich's ataxia - presentation and cause of death
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ataxia, dysarthria
scoliosis, hammer toes hypertrophic cardiomyopathy they die of cardiac of resp complicatyions. this is the most common type of spinocerebellar ataxia |
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first step to do once you see a gastroschisis
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sterile wrapping of the exposed bowel
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is omphalocele or gastroschisis associated with other abnormalities?
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omphalocele - do a feta chromosomal analysis if you see one
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should you do c section in pt with omphalocele or gastroschisis?
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only if you would otherwise for the mom/baby
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causes of floppy baby syndrome
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botulism or werdnig-hoffman syndrome (AR d/o involving degen of ant horn cells and CN motor nuclei)
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in an adult, what can you use botulinum toxin to treat?
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spastic torticollis
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periph smear sign of asplenia
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howell-jolly bodies
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when do you see heinz bodies?
helmet cells? |
heinz - g6pd and thalassemia (denatured hgb)
helmet - DIC, HUS, TTP (traumatic) |
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MCC of congenital hypothy
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thyroid agenesis
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causes of floppy baby syndrome
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botulism or werdnig-hoffman syndrome (AR d/o involving degen of ant horn cells and CN motor nuclei)
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in an adult, what can you use botulinum toxin to treat?
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spastic torticollis
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periph smear sign of asplenia
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howell-jolly bodies
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when do you see heinz bodies?
helmet cells? |
heinz - g6pd and thalassemia (denatured hgb)
helmet - DIC, HUS, TTP (traumatic) |
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MCC of congenital hypothy (in USA)
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thyroid agenesis
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abx to tx CF pt who has PNA
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pseudomonas coverage
do ceftazadime + gentamycin |
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why would a premature kid hav ea communicating hydrocephalus?
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SAH - common in premature kids
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when to immunize child exposed to varicella zoster?
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within first 3-5 days. don't bother after that.
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APGAR scoring
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appearance - body blue, extrem blue, all pink
HR - 0, <100, >100 nasal stim - no resp, grimace, cough tone - limp, some flexion, active flexion resp - none, slow and irreg, normal |
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what is more worrisome - premature adrenarche (axillary hair), thelarche, or pubarche (pubic hair)?
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pubarche - often assoc with cns d/o
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child has damage to palate - worry abt what strucutre?
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ICA (dissection or thrombosis)
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first thing to do with suspicion of congenital diaphragmatic hernia
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place orogastric tube and connect it to suction to stop further lumg compression.
also intubate |
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another name for measles
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rubeola
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MCC infantile febrile seizures
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sixth dz/exanthema subitum/roseola
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when to use rigid vs flexible bronchoscopy
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rigid won't help you get a foreign body
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what is the gram stain of the following organisms:
strep pneum staph neisseria listeria and bacillus psuedomonas, haemophilus, klebsiella, legionella |
g+ diplo
g+ cocci in clusters g - cocci gprs gnrs |
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most common heart disorder in downs - what issue can it cause
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endocardial cushion defect - can cause pulm htn and loud p2
must be surgically corrected |
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wen does baby start babbling, sits unsupported, and starts raking?
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6 months
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when can kid walk up stairs without help
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2 years
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