outpatient week 4 Flashcards
pathophys of hereditary angioedema
unchecked activation of complement factors C1/2/4
drugs for acute treatment of hereditary angioedema
Cinryze, Berinert = c1 esterase inhibitors
hereditary angioedema =
AD
low plasma levels of C1 esterase
presentation of hereditary angioedema
- subq tissue
- abd organs
- upper airway
prophylaxis for hereditary angioedema attacks
danazol (androgen)
stimulated c1-inh synthesis
C1 inhibitors also inhibits
factor XII – hageman factor
PTA
kallikrein
hereditary angioedema mos tcommon symptom
nonpitting cutaneous edema
What symptoms distinguish hereditary angioedema from other forms of angioedema?
acute abdominal pain, nausea, vomiting
hereditary angioedema, what tests would point to something else
ESR, eosinophilia
measles genome
-ss RNA paramyxovirus
measles prodrome
4 C’s: conjunctivitis, cough, coryza, koplik’s spots
measles serious complication
immediate - encephalitis
7 years later - subacute sclerosing panencephalitis
koplik’s spots
bright red lesions with white central dot on buccal mucosa
progression of measles infection
koplik spots
1-2 later maculopapular rash starting at head
measles other complication
pneumonia, diarrhea, OM
What metabolic conversion is defective in homocysteine?
homocysteine is not remethylated to methionine
treatment for homocysteinemia
folate, B6, b12
homocysteinemia enzyme deficiencies
cystathionine B-synthase
methyltetrahydrofolate reductase
symptoms of hand-foot-and-mouth disease?
nonspecific, sore throat
vesicles on mucosa, tongue, hands, feets
hand-foot-and-mouth disease complications
CNS dz (aseptic meningitis)
myocarditis
pulm hemorrhage
hand-foot-and-mouth disease duration of infection
1-2 weeks
incubation for hand-foot-and-mouth disease?
4-6d
how does hand-foot-and-mouth disease spread
fecal-oral route
where does slipping occur in Slipped capital femoral epiphysis (SCFE)
cartilage in hypertrophic zone is weak point
3 physcial exam findings in Slipped capital femoral epiphysis (SCFE)
tredelenburg gait
external rotation during passive hip flexion
limited hip internal rotation and abduction
Slipped capital femoral epiphysis (SCFE) is =
slippage of epiphysis relative to metaphysis at level of grwoth plate
common presentation of Slipped capital femoral epiphysis (SCFE)
thigh and groin pain
What additional procedure is performed in patients with hypothyroidism and slipped capital femoral epiphysis?
prophylactic pinning of non-slipped side
SCFE additional symptoms
knee pain
xray in SCFE
medial and anterior displacement of metaphysis relative to epiphysis, which remains in acetabulum
treatment of SCFE
surgical - percut pinning
SCFE more common in
males, AA, obese
hypothyroidism
down syndrome
SCFE bilateral
20%
SCFE compications
avascular necrosis
premature OA
typhlitis =
life threatening NEC that occurs in neutropenic patients
tyhplitis CT finding
diffuse cecal wall thickening
suspect typhlitis when
neutropenic pt with fever and abodminal pain in RLQ
NEC diagnosis
pneumatotis intestinalis (gas within wall of intestines)
primary pathophysiology in neonatal respiratory distress syndrome?
insufficient surfactant levels progressive atelectasis (alveolar collapse)
ABG for NRDS
hypoxemia, hypercapnia, acidosis
NRDS CXR
ground-glass granularity (can be normal after birth)
How does atelectasis lead to hyaline membrane formation in neonatal respiratory distress syndrome?
lack of ventialtion –> hypoxemia, hypercapnia, acidosis –> pulmonary aterial vasoconstriction, R2L shunt through lung, foramen ovale, ductus arterious
decreased perfusion to lun g= ischmic injury
–> decrease surfactant further
proteinaceous effusion enters alveolar spaces
factors that decrease risk of NRDS
prenatal corticosteroid use
maternal hypertension
What causes retinopathy of prematurity in infants with neonatal respiratory distress syndrome?
cycles of hypoxia and hyperoxia induce VEGF
abnormal proliferation of bvs in retina
NRDS supportive measures
CPAP or intubation with mech vent
components of NRDS prevention and treatment
prenatally - corticosteroids
antenatally - surfactant administration, resp support
NRDS risk factors
male sex, maternal diabetes
multiple births or C/S w/o labor
intrauterine asphyxia
What causes persistent patent ductus arteriosus in infants with neonatal respiratory distress syndrome?
pulmonary vasoconstriction due to hypoxia
increased pulmonary vascular resistance
R2L shunting
3 complications of NRDS treatment
persistent PDA
bronchopulmonary dysplasia
retinopathy of prematurity
NRDS drugs
artificial surfactant
amp/gent till blood cultures return
cause of BPD in NRDS
barotrauma from mechanical ventialtion
test that indicates fetal lung maturity
lecithin to sphingomyelin ration of more than 2:1
prevention of NRDS
steroids to mothers delivering at less than 34 weeks
craniopharyngoma triad
increased ICP (HA, vomit)
bitemporal hemianospia
calcified lesion of sella
Niemann Pick Vs Tac Sachs
niemann - HSM
Niemann Pick enzyme
sphingomyelinase deficiecny
Tay Sachs enzyme
beta hexoaminidase A deficiency
nursemaid’s elbow
subluxation of radial head
nursemaid’s elbow typical presentation
arm either fully extended by their side or
slightly flexed with a pronated forearm
nursemaid’s elbow mechanism of injury
axial traction on pronated forearm with extended elbow
nursemaid elbow treatment
manual reduction - hyperpronation or supination of forearm followed by flexion of the elbow from 0 to 90 degrees while holding pressure over radial head
Legg-Calve-Perthes disease xray findings
medial joint space widening
small femoral head
subcondral fracture
Legg-Calve-Perthes disease
avascular necrosis of proximal femoral epiphysis
Legg-Calve-Perthes disease physicla exam findings
hip stiffness with loss of internal rotation and abduction
gait disturbance
limb length discrepancy
nonoperative treatment for Legg-Calve-Perthes disease
observation, restricted activity, physical therapy
Legg-Calve-Perthes disease treatment
containment of hip within acetabulum by bracing or surgical means
presentation of Legg-Calve-Perthes disease
gradual painless limp followed by intermittent knee, hip, groin or thigh pain
what causes Legg-Calve-Perthes disease
disruption in vascular supply with subsequent revascularization
demogrpahic affected by Legg-Calve-Perthes disease
4-8 years, male
Legg-Calve-Perthes disease risk factors
family hx, low birth weight
second hand smoke
asian, inuit, central european
ADHD
operative treatment for Legg-Calve-Perthes disease
acetabular reconstruction
children over 8
Legg-Calve-Perthes disease complication
osteoarthritis
progressive avascular necrosis
early arthroplasty in cases of permanent dysplasia
What cardiac abnormality is commonly associated with postductal aortic coarctation?
bicuspid aortic valve
What ECG findings are associated with aortic coarctation in mild and severe disease?
-normal in mild or early disease.
-Late or severe disease = pure left ventricular hypertrophy,
or RVH associated with ST and T wave changes in the left precordial leads and some conduction delays.
What medical therapies are employed in the management of aortic coarctation?
- Alprostadil (PG E1) infusion to keep PDA open,
- dopamine or dobutamine to improve contractility in those with heart failure, and
- supportive care for associated symptoms.
What is the typical presentation of infants with preductal coarctation when the ductus arteriosus closes
differential cyanosis, with well perfused upper extremities and cyanotic lower extremities.
What classic CXR findings are associated with aortic coarctation?
rib notching
indentation at the site of coarctation and dilation pre and post coarctation, creating a “3” sign at the base of the aorta.
What surgical procedures are available for the management of aortic coarctation?
surgical correction and balloon angioplasty
What clinical findings are associated with post-ductal aortic coarctation?
- Hypertension in upper extremities → patients may complain of a long history of headaches refractory to pharmacologic therapy
- Weak pulse in lower extremities → patients may complain of a long history of claudication (pain in the legs aggravated by exercise due to vascular insufficiency)
- Intense interscapular systolic murmur
onset of DMD
2-6
DMD complications
progressive cardiac issues
scoliosis
flexion contractions
death by 20 - resp issues
retinoblastoma differential
persistent fetal ocular vasculature
congenital cataracters
severe retinopathy of prematurity
pathophysiology of bullous impetigo
exfoliative toxin A targets desmoglein-1
bullous impetigo
S aureus
minority is GAS
bullous impetigo age range
2-5
staph vs strep bullous impetigo
staph: erythematous vesicles
strep: erythematous pustules
bullous impetigo apperance
clear yellow fluid in bullae
thin brown crust when reuptured
bullous impetigo risk factors
poverty, crowding, poor hygeine, scabies infection
what can follow impetigo
PSGN and rhematic fever
testing for bullous impetigo?
gram stain
treatment of bullous impetigo
washing all areas
eryhtomycin cephalosporins or topical antibiotics
What conditions can result in bacterial penetration and development of nonbullous impetigo?
scratching, dermatophytosis
viral infections (varicella, herpes)
inscet bites
immunosuppresion
causes of nonbullous impetigo
S. aureus in US
Strept pyo outside US
indications for bacterial culture with nonbullous impetigo
suscipion of mRSA
outbreak occuring
PSGN present
antibiotics for less severe nonbullous impetigo
mupirocin
retapmulin
clindamycin
(alll topical)
antibiotics for more severe nonbullous impetigo
systemic
dicloxacillin, cephalexin
vanc
clinda
What physical exam finding is pathognomonic for Eisenmenger’s syndrome with an associated unrepaired patent ductus arteriosus?
differential cyanosis (more pronounced in feet than hands)
What ratio is used as a measurement of the degree of shunting across a patent ductus arteriosus?
pulmonic to systemic flow ratio
How does the typical treatment of a patent ductus arteriosus differ between adults and children?
infants - ibuprofen, indomethacin
adults - interventional closure
What is the classic presentation of a patent ductus arteriosus that presents in later childhood?
exercise intolerance
murmur
wide systemic pulse pressure
displaced apical impulse
What is the classic heart sound associated with a patent ductus arteriosus?
continuous machine like murmur
contraindications to prostaglandin inhibitors
necessary for survival
contraindicated - intraventricular hemorrhage
Legg - Calve - Perthes RISK FACTORS
family history, low birth wt, smoking
asian/inuit
HSP tetrad
palpable purpura
polyarticular arthitis
abd pain
renal failure
nonbullous impetigo bacterial penetration with
scratching, insect bites
dermatophytosis
viral (varicella, herpes)
immunosuppresion
aortic coarc clinical findings
HTN, headache
weak pulses in lowe rlegs/claudication
interscapular systolic murmur
lung abnormalities in BPD
fewer and larger alveoli
BPD causes
disruption of lung development
BPD risk factors
premature
mechanical venitlation
oxygen toxicity
infection - post or antenatal
BPD pulmonary function abnormalities
decreased tidal volume
increased airway resistance
increasing V/P mismatch
BPD physical exam findings
tachypnea
pulmonary edema
audible rales
effect of larger alveoli in BPD
decreased gas transfer area
BPD criteria
infant that requires suppl oxygen for at least 28 d postnatal
BPD management
weaning patient from ventiatlion, CPAP, supplemental oxygen
BPD complication
pulmonary hypertension
clinical course of BPD
improvement between 2-4 months
turners - ovary issue
streak ovaries
triple bubble sign
jejunal atresia
grey vesicles on posterior pharynx
herpangina
treatment for strabismus
patch normal eye
indications for renal ultrasound
less than 24 months, febrile UTI
indications for voiding cystourethrogram
less than 1 month or 2 years with recurrent UTIs or organism other than E coli
biliary cyst triad
jaundice, abd pain, palpable pass
biliary cyst treatment
surgial
pink stain in neonatal diapers
uric acid crystals
regain birth weight by
10-14 d
percent of weight babies lose
7%
delayed umbilical cord falling off
leukocyte adhesion def
rotavirus contraindications
hx of intussceiption
SCID
hx of uncorrected GI malformation
stridor that improves iwth neck extension
vascular ring
features of turner’ ssyndrome
webbed neck, wide spaced nipples
horseshoe kidney
lymphedema of hands and feet
aortic coaractaton, bicuspid aortic valve
turner’s syndrome - short stature caused by
absence of SHOX gene
Turner syndrome amanagement
hormone replacemtn
monitor for autoimmune hypothyroidism
surgical correction of cardiovascular abnormalities
resection of intrabdominal gonads to prevent maligancy
turner’s skeletal abnormalities
madelung deformity
high arched palate
narrow maxilla
small mandible
turner’s - ob complicatiion
ovarian dysgenesis
incapable of having children
pathophysiology of LAD type 1
failure to express integrin CD18
LAD =
neutrophils unable to leave circulation and enter tissues
LAD most common infectious organisms
staphylococcal
enteric GN
fungal - candida
types of infections with LAD
URT, skin
pathophysiology of LAD type 2
abnormalities in E-selectin
absent siaylated Lewis X
LAD type 1 clinical findings
omphalitis
perirectal or labial cellulitis
LAD type 2 clinical findings
short stature
abnormal facies
cognitive impairment
LAD, CBC
leukocytosis in absence of infection
how to distinguish LAD type 1 vs 2
flow cytometry for integrins
Bombay blood group for type 2
LAD management
prophylatic antibiotics
type 1 - BMT
type 2 - fucose supplementation
persistent urachus
complete failure of urachal duct to close
fistula between bladder and umbilicus, urine will drain from ubilicus
Erb’s palsy
C5-6
upper arm to be adducted and internally rotated with forearm extended
cystic hygroma
lymphatic malformation
painless soft mass superior to clavicle
diastasis recti
separation of left and right side of rectus abdominis
absence of the radius associated with
TAR syndrome
fanconic anemia
holt-oram syndrome
single umbilical vessel
think of other anomalies
branchial cleft cysts
anterior margin of sternocleidomastoid
C8-T1 injury
klumpke’s plasy
isolated hand paralysis, horner’s syndrome
meonicum plug
obstruction of left colon and rectum due to dense, dry meconium
can be a manifestation of CF
small cord can indicate
poor nutritional status or intrauterine compromise
absent pectoralis muscle
Poland syndrome
narrow chest cavity
Jeune syndrome
where are accessory nipples found
along milk line (axilla to pubis)
