Uworld Peds Flashcards
thiamine deficiency causes what?
- wet beriberi (dilated cardiomyopathy)
- dry beriberi (peripheral neuropathy)
- Wernicke-Korsakoff syndrome
B2 deficiency symptoms?
riboflavin
- angular cheilosis
- stomatitis
- glossitis
- normocytic anemia
- seborrheic dermatitis
monosymptomatic enuresis tx
- behavioral modifications
- enuresis alarm
- desmopressin = 1st line
- TCAs = 2nd line (imipramine)
- more common in boys
- bed wetting is normal until 5YO; begin interventions after that age
Friedreich ataxia
- autosomal recessive
- excessive tocopherol transfer protein
- necrosis & degeneration of cardiac muscle fibers –> myocarditis, MI, cardiomyopathy*, T wave inversion
- ataxia
- DB
- scoliosis, hammer toes
immune thrombocytopenia etiology, tx
-post-viral infection Ab to platelets –> destruction in spleen –> thrombocytopenia –> bruising & bleeding
- tx: observation for spontaneous recovery (within 6 months) in kids
- 1st line: IVIg or glucocorticoids (kids that bleed or adults with plt<30K)
gynecomastia
- enlarged benign glandular tissue in male breast
- cause: inc testicular production of estrogen OR peripheral conversion of pro-hormones to estrogen OR androgen deficiency
- 2/3 of pubertal boys
- goes away within 2 years
excessive intake of cow’s milk can lead to deficiency of what?
iron
- -> Fe deficiency anemia
- occurs with greater than 24 ounces/ day of milk
myocarditis viral cause, px, tx
-coxsackievirus B, adenovirus
- viral prodrome (fever, lethargy)
- dilated cardiomyopathy*
- respiratory distress (pulmonary edema)
- hepatomegaly due to RHF/ congestion
-tx: diuretics, inotropes
compartment syndrome cause, px, tx
- tissue pressure > perfusion pressure –> muscle & nerve ischemia
- acute trauma
- px: severe pain, pallor, poikilothermia, paresthesia, pulselessness, paralysis
- tx: emergent fasciotomy (compartment release)
what disease can vitamin A be used in?
-measles
- reduces M&M via immune enhancement
- inc GI and respiratory epithelium to regenerate
Parinaud’s syndrome px, causes
- paralysis of vertical gaze
- eyelid retraction (Collier’s sign)
- pseudo-Argyll Robertson pupil
-causes: pressure in rostral midbrain –pinealoma, germinoma
craniopharyngioma vs pituitary adenoma
- cranio. has CALCIFIED cystic parasellar lesion
- pituitary adenomas are not calcified
- both produce bitemporal hemianopia, endocrine changes
- pituitary adenoma often is a prolactinoma
Turner syndrome pt at risk for?
osteoporosis due to lower estrogen levels
–> inc risk for bone fractures
-tx: estrogen replacement for growth and prevention of fractures
vitamin A deficiency px
- night blindness
- photophobia
- dry scaly skin
- dry conjunctive, cornea
- bitot spots = dry, sliver plaques on bulbar conjunctiva
vesicoureteral reflux
= urinary reflux from bladder into kidney –> hydroureter –> hydronephrosis, blunting of calices, renal insufficiency
-complications: parenchymal scarring, HTN, proteinuria, edema
acute rheumatic fever px, tx
- Joints: migratory arthritis
- <3: pericarditis, friction rub, diffuse ST elevations
- Nodules: subcutaneous
- Erythema marginatum
- Syndeham chorea
- tx: long acting IM benzathine penicillin G
- until adulthood to eradicate bacterial carriage/ to prevent recurrent ARF
heart defect associated with DiGeorge syndrome?
truncus arteriosus
Ebstein’s anomaly
- droopy tricuspid valve into right ventricle –> tricuspid regurgitation & RA enlargement
- tall P waves, RAD
-associated with maternal lithium* use
tricuspid valve atresia
- hypoplastic right ventricle
- underdevelopment of pulmonary valve/ artery
- decreased pulmonary markings
- LAD
- large P waves due to RA enlargement
-need ASD or VSD for survival
lead poisoning tx
-determine venous lead levels:
5-44mcg/dL –> no meds & remeasure in a month
45-69 –> DMSA (dimercaptosuccinic acid)
> 70 –> dimercaprol + EDTA
juvenile angiofibroma px, tx
nasal obstruction + visible mass + frequent epistaxis
- dangerous bc composed of many blood vessels
- benign, boys, nasopharynx
-tx only if angiofibroma is enlarging, obstructing airway, or causing chronic nosebleeds
serum sickness-like reaction time of onset? definition?
1-2 weeks
hypersensitivity reaction –> fever, urticaria, rash, arthralgias, lymphadenopathy
-resolves with withdrawal of offending agent
Bordatella pertussis infection
- whooping cough
- px: severe, paroxysmal cough, inspiratory whoop, posttussive emesis
- dx: pertussis PCR or lymphocytic leukocytosis
- tx: macrolides
- prevention: DTaP & TdaP
congenital rubella px
- sensorineural hearing loss
- PDA
- cataracts
- glaucoma
DiGeorge syndrome px
- Conotruncal cardiac defect = truncus arteriosus
- Abnormal facies
- Thymic aplasia/hypoplasia –> T-cell lymphopenia
- Cleft palate
- HypoCa <–parathyroid gland hypoplasia
empiric antibiotic tx =
-ceftriaxone + vancomycin
ceftriaxone –l Strep pneumoniae & Neisseria meningitidis
vancomycin –l resistant strep pneumo strains
breastfeeding contraindications?
- active abuse of street drugs or alcohol
- active untx TB
- maternal HIV
- herpetic breast lesions
- varicella infections <5 days earlier OR 2 days after delivery
- chemo, ongoing radiation, certain meds
nursemaid’s elbow
= subluxation of radial head
- cause: child pulled, lifted, swung by arm
- tx: closed reduction –apply pressure on radial head & hyperpronate forearm
neonatal sepsis px
-temp instability
(febrile in term infants; hypothermic in preterm infants)
-poor feeding
-irritability & lethargy
-resp distress, vomiting, seizures, jaundice
prostaglandin E1 vs indomethacin
keeps PDA open vs closes it
- PGE = vasodilator
- indomethacin = NSAID & PGE-inhibitor
Down syndrome associations?
- complete AV canal
- VSD, ASD
- duodenal atresia
- Hirschsprung disease
- Alzheimer’s disease
- ALL
- hypothyroidism
- DB type 1
- atlantoaxial instability
- hypotonicity
- facial dysmorphisms
Hirschsprung disease site of obstruction?
level of rectosigmoid junction
MCC of congenital hypothyroidism
thyroid dysgenesis
aplasia, hypoplasia, ectopic gland
leukocoria
= white reflex
-retinoblastoma until proven otherwise –> refer to ophthalmologist
retinoblastoma
- highly malignant tumor
- MCC of childhood intra-ocular tumor
- death from liver and brain mets
- px: leukocoria
PANDAS
=pediatric autoimmune neuropsychiatric disorders
- acute onset of OCD after recent group A streptococcal infection
- tx: high dose SSRI, psychotherapy
classic vs late-onset congenital adrenal hyperplasia
- classic = presents in neonates, adrenal insufficiency, ambiguous genitalia
- late onset = late childhood, androgen excess (premature adrenarche/pubarche, cystic acne, accelerated linear growth, advanced bone age)
central vs peripheral precocious puberty
gonadotropic-dependent vs -independent precious puberty
- differentiate with GnRH stimulation (should inc in -dependent form)
- inc bone age in -dependent form
most common pathogen in children with CF?
Staph aureus
especially in the setting of concurrent influenza infection
racemic epinephrine effects in respiratory distress?
-alpha and beta andrenergic effects:
- alpha –> dec bronchial secretions & mucosal edema
- beta –> inc smooth muscle relaxation
upper GI series
- tracks the movement of barium through upper GI via X-ray
- specific test to dx volvulus/ malrotation
tinea corporis
= ringworm = superficial fungal infection
- px: erythematous, scaly, pruritic rash with central clearing
- tx: terbinafine
pts with pyloric stenosis px with? at risk for?
- projectile vomiting
- olive shaped abdominal mass
- poor weight gain
- dehydration
-risk: hypochloremic metabolic alkalosis
orbital cellulitis
= infection of orbital soft tissue posterior to orbital septum
- px: pain with eye movement, proptosis, ophthalmoplegia, diplopia
- bacterial sinusitis MCC –due to proximity of sinuses to orbital space & valveless orbital venous system
contraindications to rotavirus vaccine
- anaphylaxis to vaccine ingredients
- hx of intussusception
- hx of uncorrected congenital malformation (Meckel’s)
- SCID
respiratory distress syndrome cause, px in infants
-cause: surfactant deficiency; often premature neonates
- tachypnea; RR>60
- grunting; to inc end-expiratory pressure
- nasal flaring; to dec nasal airway resistance
- retractions; to pull in the compliant chest wall
- hypoxia & cyanosis; from atelectasis
-diabetic mothers: insulin blocks maturation of sphingomyelin
transient tachypnea of the newborn cause
inadequate alveolar fluid clearance at birth –> mild pulmonary edema
-usually resolves by DOL 2
hemolytic uremic syndrome cause, px
-cause: E coli toxin release, Shiga toxin
- uremia + thrombocytopenia + hemolytic anemia
- jaundice, bloody diarrhea, renal insufficiency
neonatal polycythemia
- hct > 65%
- px: ruddy/plethoric
- hypoglycemia & hypoCa due to inc cellular uptake
hyperIgM syndrome
-X-linked defect of CD40 ligand (present on T cells, which binds to CD40 on B cells)
- recurrent sinopulmonary infections (acute otitis media, PNA, sinusitis)
- inc viral infections
- inc risk of opportunistic infections (PCP)
-tx: antibiotics prophylaxis & interval IVIg administrations
meconium ileus
- neonatal bilious emesis
- common in CF pts
- microcolon on imaging
- dx: xray, enema
- tx: hyperosmolar enema to break up/dissolve obstruction; surgery
QT prolongation risk for? tx? avoid what?
- syncope
- ventricular arrhythmias
- sudden cardiac death
- tx: beta blocker + pacemaker
- avoid: electrolyte derangement, vigorous exercise, meds that lengthen QT
growing pains
- bilateral, lower extremity pain
- no systemic sx
- worse at night*
- children 2-12 YO
- tx: reassurance, massage, NSAIDs
positive “target sign” on ultrasound
- diagnostic of intussusception
- illustrates bowel telescoped into larger lumen
DMD vs BMD
- Duchenne starts at an earlier age (3-5 YO) vs BMD at 5-15 YO
- DMD has intellectual disability
- DMD often wheelchair bound by adolescence due inc weakness
- both have cardiomyopathy –> cause of death (earlier age of death in DMD)
trachoma cause, px, tx, complications
- Chlamydia trachomatis serotypes A-C
- px: follicular conjunctivitis + pannus neovascularization
- tx: topical tetracycline or oral azithromycin
- scarring of cornea –> blindness
“floppy baby” syndrome causes
- Werdnig-Hoffman syndrome = autosomal recessive degeneration of anterior horn cells & cranial nerve motor nuclei
- infant botulism (from honey or canned food)
congenital hypothyroidism px
- normal at birth*
- apathy
- weakness
- hypotonia
- large tongue
- sluggish movement
- abdominal bloating
- umbilical hernia
Fe deficiency anemia vs thalassemia
-both produce microcytic anemia
- thalassemia microcytosis is more severe than Fe-def.
- thalassemia: tear drop cells, target cells on peripheral smear
acquired aplastic anemia causes, px?
- injury to bone marrow
- secondary to radiation, drugs, insecticides, toxins, infections (parvovirus B19)
-px: normocytic/ macrocytic anemia + leukopenia + reticulocytopenia + thrombocytopenia
transient proteinuria
- MCC of isolated proteinuria in children
- repeat urine dipstick on 2 other occasions to rule out persistent proteinuria
septic arthritis tx
emergent surgical drainage + IV antibiotics
-often preceded by mild infection (cellulitis)
infantile colic
- excessive crying in an otherwise healthy infant
- more than 3 hours/day, more than 3d/week, more than 3 weeks/month
- resolves spontaneously by 4 months
-no recognized tx; often inconsolable
breastfeeding failure jaundice
-lactation failure –> dec bili elimination & inc enterohepatic circulation
- px: suboptimal breastfeeding, dehydration
- during 1st week of life
breast milk jaundice
- high levels of beta-glucoronidase in breast milk –> deconjugated intestinal bill –> inc enterohepatic circulation
- starts during 1st week, peaks during 2nd
hand foot syndrome
- vaso-occlusion in sickle cell anemia
- -> dactylitis
- due to vascular necrosis of metacarpals & metatarsals
what do you give to an un-immunized pt who had VZV contact?
- immunocompetent: varicella vaccine
- immunocompromised: varicella immunoglobulin (VZIG)
-acyclovir for those with active varicella infections
hydroxyurea effects, SE
- inc fetal hemoglobin in circulation
- -> reduces vaso-occlusive events
-SE: bone marrow suppression (leukopenia, anemia, thrombocytopenia)
Kawasaki disease tx
- aspirin – antithrombotic to prevent coronary aneurysms (despite risk of Reye syndrome)
- IVIg – dec inflammation
Kawasaki disease px
- fever >5 days
- bilateral nonexudative conjunctivitis
- cervical lymphadenopathy
- rash
- swelling of feet/hands
- mucositis, strawberry tongue
- coronary aneurysm
IgA deficiency px
- recurrent sinus, pulmonary, GI infections (mucous membranes)
- anaphylaxis to transfusions that contain IgA (may have Ab against IgA)
-inc susceptibility to Giardia –> diarrhea
Tetralogy of Fallot murmur, px, changes with squatting?
harsh, systolic ejection murmur over the left upper sternal border
- due to pulmonary stenosis
- single S2
-“Tet spells” from exertion/ agitation
-cyanosis due RV outflow obstruction & R–>L shunting
(pre-, post-, intra-valvular stenosis; or atresia)
- squatting –> inc afterload –> dec R–>L shunting across VSD –> improved cyanosis & inc systolic murmur
- inhaled O2 stimulates pulmonary vasodilation & systemic vasoconstriction
ventricular septal defect
- holosystolic murmur at left lower sternal border –flow thru VSD
- apical diastolic rumble –inc flow across the mitral valve
ASD murmur
wide and fixed splitting of S2
-due to inc flow across the pulmonic valve
RTA
1 – defective H+ secretion
2 – dec bicarp reabsorption in PCT
4 –defective Na/K exchange; hyperK, hyperCl acidosis
-px as failure to thrive
sickle cell pts are at risk for infection by which organisms?
- Strep pneumo (bacteremia)
- H. influenzae type B
- Neisseria meningitides
- Salmonella (osteomyelitis)
-give prophylactic penicillin till age 5
most common congenital heart disease?
ventricular septal defect
Fanconi anemia
- congenital aplastic anemia –> bleeding, fatigue
- short, microcephaly, hypogonadism, hypo/hyperpigmented areas, strabismus
- tx: hematopoietic stem cell transplantation
- chromosomal breaks; autosomal recessive
milk-protein allergy
- only infants
- non-IgE immune response to dairy/soy –> rectal/colonic inflammation –> painless bloody stools
- reflux, vomiting, anemia, eczema
- tx: mom avoids dairy/soy or give formula
- often resolves by 1YO
if an infant px with hemihyperplasia what do you screen for? how?
hepatiblastoma & Wilms tumor
-via alpha-fetoprotein & ultrasound
tx for non-bullous vs bullous impetigo
-topical mupirocin
(staph or strep pyogenes)
-oral cephalexin, dicloxacillin, clindamycin
(stap)
most common primary bone tumor in kids?
osteosarcoma
osteosarcoma
- metaphyses of long bones
- tender soft tissue mass
- “sunburst” pattern, periosteal reaction
- inc alk phos & LDH
Ewing sarcoma vs osteoid osteoma
- osteolytic lesion + periosteal reaction –> reactive bone layers (“onion skin” appearance)
- sclerotic, cortical* lesion + central lucency + pain worse at night
rickets clinical findings
- genu varum (bowing of tibia and femur)
- delayed closure of anterial fontanelle
- enlarged costochondral joints (“rachitic rosary”)
- craniotabes = thinning of skull (“ping pong ball”)
SCID
-recurrent sinus, pulmonary, opportunistic, viral infections; recurrent oral candidiasis, persistent diarrhea
- absent lymph nodes, tonsils
- lymphopenia
- absent thymic shadow
Bruton’s agammaglobulinemia
- recurrent pyogenic infections (strep pneumo, H flu)
- dec IgG, IgA, IgM, IgE
- dec B cells
- young boys
-common variable immunodeficiency has a similar px in older pts
Wiskott-Aldrich syndrome
- boy
- eczema
- thrombocytopenia
- recurrent infection with encapsulated germs
- low IgM; high IgA, IgE
Reye syndrome
- ASA use in children
- hepatic failure + encephalopathy
- microvesicular steatosis
- cerebral edema
- inc ammonia, ALT, AST
- vomiting, change in mental status, agitation
tx’s for what?
- sodium bicarbonate
- deferoxamine
- EDTA
- calcium gluconate
- succimer
- N-acetylcysteine
- TCA or ASA overdose
- Fe poisoning
- mod-severe lead poisoning
- cardio-protective in hyperK
- mild-mod lead poisoning
- acetaminophen toxicity
most common cancer in children?
acute lymphoblastic leukemia
sweat test for CF
- quantitative policarpine iontophoresis
- chloride >60
tx for TCA overdose? sx of TCA overdose?
- sodium bicarbonate
- seizure, hypotension, prolonged QRS
- cholinergic agonist used to tx anticholinergic effects of TCA (dry oral mucosa, dilated pupils)
- may need benzodiazepines to tx the seizure
neonatal jaundice with conjugated hyperbilirubinemia is caused by?
neonatal cholestasis
- from impaired hepatic excretion of bill due to:
- extrahepatic obstruction
- liver cell injury
-conjugated bili >2mg/dL
eczema herpeticum
HSV + atopic dermatitis
= numerous vesicles over the area of atopic dermatitis
-tx: acyclovir
enterobiasis
-helminthic infection by pinworm, Enterobius vermicularis
- dx: “scotch tape test”
- px: n/v, abdominal pain, vulvovaginitis
- tx: mebendazole, albendazole; pyrantel pamoate
conduct disorder
antisocial personality disorder
oppositional defiant disorder
ADHD
- 18YO or younger –> disruptive behavior; aggression to others & animals; property damage, theft, violations of social rules
- 18YO or older with conduct disorder
- late childhood/adolescence; negative hostile, defiant
- dx before 7YO; impulsivity, inattention, hyperactivity; can become conduct disorder
when should you administer vaccines in preterm infants?
- immediately, i.e. by chronologic age (NOT gestational age)
- unless infant weights <2kg
most common complication of sickle cell trait?
painless microscopic or gross hematuria
clubfoot tx
nonsurgical first:
- stretching, manipulation, serial plaster casts, malleable splints, taping
- surgery if above doesn’t work
-must tx immediately*
developmental dysplasia of the hip
=dislocation of femoral head from acetabulum
- must be dx before 6 months
- infants screened until 1YO
- dx: leg maneuvers, asymmetric leg-length or skin folds, ultrasonography
- tx: hip harness
acute cervical adenitis in children cause, tx
- staph or strep
- tx: clindamycin
MCC of viral meningitis
non-polio enteroviruses
- echovirus
- coxsackieviruses
myotonic muscular dystrophy
- adolescent/ adult-onset muscular dystrophy
- autosomal dominant
- myotonia, facial weakness, foot drop, dysphagia
- cardiac conduction anomalies
- cataracts
- testicular atrophy
- baldness
clotting defect vs platelet aggregation defect px
- hemophilias: hemarthroses, hematomas
- vWD: easy or prolonged mucosal bleeding, ecchymoses, petechiae
aplastic anemia vs aplastic crisis
- pancytopenia
- transient arrest of erythropoiesis secondary to parvovirus B19 infection
leukocyte adhesion deficiency type 1 px
-leukocytosis (neutrophil predominance) + absence of neutrophils in inflamed/ infected tissue
- recurrent bacterial infections
- delayed umbilical cord separation*
- necrotic periodontal infection
McCune-Albright syndrome px
- Precocious puberty
- Pigmentation (cafe au lair spots)
- Polyostotic fibrous dysplasia = bone defects
-associated with Cushing’s disease
B cell vs T cell deficiency
- bacterial infections
- viral & fungal infections
neonatal abstinence syndrome px
- irritability
- high pitched cry
- poor sleeping
- tremors
- seizures
- sweating
- sneezing
- tachypnea
- poor feeding
- vomiting
- diarrhea
- heroin withdrawal within 48 hours
- methadone withdrawal within 48-72 hours
benign vs pathologic murmurs
- grade II/IV, dec with standing
- grade III/IV, inc with standing, abnormal S2
