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568 Cards in this Set

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PAGET'S DISEASE PATHOPHYSIOLOGY
EXCESSIVE BONE TURNOVER BY OSTEOCLASTS +/- ASSOCIATED WITH PARAMYXOVIRUS INFECTION
PAGET'S S/S
DEEP BONE PAIN
FREQUENT #
INCREASED CRANIAL DIAMETER (=FRONTAL BOSSING)
DEAFNESS D/T CN VIII COMPRESSION
PAGET'S LAB & XRAY FINDINGS
INCREASED ALP, URNIARY HYDROXPROLINE
NORMAL Ca, PO4
XRAY - MARKEDLY EXPANDED BONY CORTEX - INCREASED DENSITY, THICKENED TRABECULAE, CHARACTERISTIC "JIGSAW" OR "MOSAIC" BONE PATTERN
PAGET'S - Rx
NSAIDS, CALCITONIN, ALENDRONATE
Paget's - complications
#'s, high output cardiac failure, deafness, secondary osteosarcoma
heliotrope rash
violacious periorbital rash in dermatomyositis
Grotton's papules
papules on dorsum of hand over bony prominences in dermatomyositis
polymyositis lab and EMG findings
elevated serum creatine, aldolase, CPK
EMG - fibrillations
complications of polymyositis
myocarditis, cardiac conduction defects, malignancy
Felty's triad
hepatosplenomeagly
TCP
RA
Lab, synovial fluid and Xray findings in RA
rh factor = anti-Fc IgG Ab - elevated in >75% (not specific)
+/- ESR elevated
synovial fluid - turbbid, decreased viscosity, WBC 3000-50000 cells/microL
Xray - juxta-articular dimineralization (early) joint space narrowing and erosions (late)
JRA - most common type?
pauciarticular - <30% develop iridocyclitis
ANA type - most common - asymmetric involvement of large joints with iridocyclitis
RF type - poor Px - concurrent with AS in HLA-B27+ males
Systemic acute febrile JRA
least common
characteristic arthritis with daily high spiking fevers
evanescent salmon coloured rash
+/- hepatosplenomegaly and serositis
Scleroderma S/S
thickening of skin of face +/or distal extremities
CREST
severe form - pulmonary fibrosis, cor pulmonales, ARF, malignant HTN
Anticentromere Ab
specific for CREST
anti-Scl-70 = anti-topoisomerase 1
specific for systemic scleroderma
treatment for scleroderma
glucocorticoids, penicillamine for skin changes, CCB for Raynaud's, ACEI for renal dz/HTN
SLE criteria
DOPAMINE RASH =
Discoid rash
Oral ulcers
Photosensitivity
Arthritis
Malar rash
Immunologic criteria
Neurologic symptoms (lupus cerebritis, seizures)
Elevated ESR
Renal disease
ANA +
Serositis
Hematologic abnormalities - anemia, leukopenia, TCP
Anti-dsDNA, Anti-Sm Ab's
highly specific, not sensitive for SLE
drug induced SLE antibodies
Antihistone Ab
Anti-Ro Ab's
neonatal SLE
Anti-phospholipid Ab's
hypercoagulability, thromboembolic disease, recurrent spontaneous abortions
Rx for SLE
NSAIDs (initial)
Steroids for acute exacerbations
hydroxychloroquine, cyclophosphamide, azithroprine for progressive/refractory cases
SLE pathogenesis, epidemiology
90% female (esp. African American)
Antibody-mediated cellular attack with deposits of antigen-antibody complexes
Drugs inducing SLE
hydralazine
pencillamine
procainamide
Temporal arteritis pathogenesis
subacute granulomatous inflammation of large vessels (includes aorta, ext. carotid - temp. br, vertebral a's)
temporal arteritis S/S
new h/a uni/bilateral with scalp pain
temporal tenderness
jaw claudication
fever
transient/perm monocular blindness
+/- wt loss, myalgia/arthralgia
bx findings in temporal arteritis
thrombosis, media necrosis, lymphocyes, plasma cells, giant cells
HSP path and epi
immune mediated small vessel vasculitis
children 2-11 y/o
HSP S/S
palpable purpura on buttocks, legs
asym. migartory periarticular swelling
abdominal pain
preceding URTI (75%)
Complications of HSP
GIB, intussusception, GN - degree of renal involvment determines Px
Barlow's maneuver
for DDH
pressure on inner aspect of abd thigh, hip add - causes post. dislocation
Ortolan's maneuver
for DDH
thighs abdocuted from midline with anterior pressure on greater trochanter - +ve = soft click (femoral head reduced into acetabulum)
Allis'/Galeazzi's sign
for DDH
knees at unequal heights when baby's hips and knees flexed (dislocated side lower)
Evaluation of DDH
clinical, U/S esp after 10 weeks old (not xray until >4m old)
Monteggia's #
diaphyseal # of prox ulna with subluxation of radial head
Galeazzi's #
diaphyseal # of radius with dislocation of distal r/u joint - d/t direct blow to radius
Rx - ORIF radius, cast in supination to reduce r/u jt
clavicular #
most common #'d long bone in kids
can be associated with brachial plexus injuries
greenstick #
incomplete # - cortex of only one side of bone
Rx - reduction with casting, repeat Xray 7-10days
nursemaid's elbow
radial head subluxation secondary to child being pulled or lifted by the the hand
Rx - gentle supination of elb at 90
Torus #
buckling of cortex of long bone secondary to trauma
- usually distal radius or ulna
Salter-Harris # type I
physis
Salter-Harris # type II
metaphysis and physis
Salter-Harris # type III
epiphysis and physis
Salter-Harris # type IV
epiphysis, metaphysis, physis
Salter-Harris # type V
crush injury of physis
Rx of Salter-harris #'s
Types I, II - conservative
III - V - Sx
Rx of DDH
Splint with Pavlik harness (maintains hip flexed, abd) if <6m old
6-15m old - spica cast
15-24m old - ORIF
DDx of limp in children
"STARTSS HOTT"
Septic joint
Tumor
AVN (LCPD)
RA/JRA
TB
SCA
SCFE
HSP
Osteomyelitis
Trauma
Toxic synovitis
osteosarcoma
2nd most common primary malignancy of bone (MM 1st)
metaphyseal region of distal femur, prox. tibia, prox. humerus - mets to lungs
males, 2nd-3rd decades
osteosarcoma vs Ewing's
Osteo
metaphyseal region distal femur, prox tibia/humerus
Xray - Codman's triangle, "sunburst" pattern
Ewing's
diaphyseal-metaphyseal regions of pelvis, femur, tibia
multilayered "onion-skinning"
Codman's triangle
Osteosarcoma Xray finding = periosteal new bone formation at diaphyseal end of bone
lesion of Broca's area
Broca's aphasia - motor (expressive) - broken speech, normal comprehension
lesion of Wernike's area
sensory (fluent/receptive)aphasia - poor comprehension
lesion to arcuate fasciculus
conduction aphasia - poor repitition with good comprehension, fluid speech
Lesion to amygdala bilaterally
Kluver-Bucy syndrome - hyperorality, hypersexuality, disinhibited behaviours
lesion to fronal lobe
frontal release signs - personality changes
deficits in concentration, orientation, judgement
right parietal lobe lesion
spatial neglect syndrome - agnosia of contralateral side of the world
Lesion to mamillary bodies bilaterally
Wernicke-Korsakoff's encephalopathy - confabulations, anterograde amnesia
CN XII lesion
tongue deviates towards lesion side
CN V lesion
jaw deviates towards
unilateral cerebellum lesion
patient falls towards side of lesion
CN X lesion
uvula deviates AWAY from lesion side
CN XI lesion
head turns towards side of lesion
R optic nerve defect
right hemianopsia
optic chiasm lesion
bitemporal hemianopsia
R optic tract lesion
L homonymous hemianopsia
R Meyer's loop lesion
L upper quadrant anopsia = right temporal lesion
R dorsal optic radiation lesion
L lower quadrant anopsia = R parietal lesion
R visual cortex lesion
L hemianopsia with macular sparing
anterior cerebral artery distribution
medial surface of brain, leg-foot area of motor and sensory cortices
middle cerebral artery distribution
lateral aspect of brain, Broca's and Wernicke's areas
Anterior communicating artery defects
most common circle of Willis aneurysm, +/- visual field defects
Posterior communicating artery defects
CN III palsy
common area of aneurysm
Lateral striae arteries distribution
internal capsule, caudate, putamen, globus pallidus = "arteries of stroke"
stroke of anterior Circle of Willis
general sensory and motor dysfunction, aphasia
Stroke of posterior Circle of Willis
vertigo, ataxia, visual defects, coma
Poliomyelitis/Werdnig-Hoffman disease spinal cord lesions
LMN lesions only = flaccid paralysis
MS spinal cord lesions
mostly white matter of cervical region - random and asym lesions
ALS
combined U/LMN deficits
NO sensory deficits
Anterior spinal artery lesion
spares dorsal columns
Tabes dorsalis
impaired propriocetion
locomotor ataxia
d/t tertiary syphilis
syringomyelia
affects ventral white commissure and ant. horns
Vit B12 neuropathy/Friedreich's ataxia
affects dorsal columns, lateral corticospinal and spinocerebellar tracts
Broca's aphasia
disorder of language production with intact comprehension - pts aware
impaired repitition
Associated with arm and face hemiparesis (right), hemisensory loss, apraxia of oral muscles
d/t L superior MCA stroke
Wernicke's aphasia
disorder of language comprehension
neologisms, paraphasic errors
pts unaware
no hemiparesis, dysarthria
L inf/post MCA embolic stoke
poor Px c/w Broca's
Locked in syndrome S/S, causes
awake and alert, unable to move except eyes and eyelids
central pontine myelinolysis
brain stem CVA
advanced ALS
Persistent vegetative state
normal sleep-wake cycles
most common - diffuse cortical injury
hypoxic-ischemic injury
5 A's of dementia
Aphasia
Amnesia
Agnosia
Apraxia
disturbances in Abstract thought
Causes of dementia
"DEMENTIAS"
neuroDegenerative diseases
Endocrine
Metabolic
Exogenous
Neoplasm
Trauma
Infection
Affective disorders
Stroke/Structural
Peripheral vs central vertigo
Peripheral - intermittent, severe, constant nystagmus, always horizonal, unidirectional, associated with hearing loss, no brain stem S/S
Central - constant, less severe, +/- nystagmus (absent, uni/bidirectional, +/- vertical)Rare hearing loss, often brain stem S/S (ataxia, dysarthria, CN abN, motor dysfunctions)
Most common causes dysequilibrium
BPPV (50%)
Ménière's
Others - hypothyroid, aminoglycoside/furosemide toxicity, stroke, trauma, labyrinthitis, acoustic neuroma
Nylen-Barany Maneuver
= Dix-Hallpike maneuver
pt sitting to supine quickly turning head to side - +ve if reproduces vertigo +/or nystagmus = BPPV
BPPV S/S
transient episodic vertigo <1min
nystagmus triggered by changes in head position (classic - turning in bed/getting up in am)
N/V
Symptoms decrease with repetitive testing
Meniere's disease S/S
(=endolymphatic hydrops)
intermittent peripheral vertigo d/t distention of endolymphatic compartment of inner ear
N/V
ear fullness
hearing loss
tinnitus
episodes resolve hrs-days
Causes of Meniere's
head trauma
syphilis
audiometry findings in Meniere's
low-frequency pure-tone hearing loss
Treatment of Meniere's
low salt diet
acetazolamide
actue attacks - antihistamines, antiemetics, benzo's
surgical decompression if refractory
acute h/a causes
SAH
hemorrhagic CVA
meningitis
seizure
acutely increased ICP
hypertensive encephalopathy
post-LP
ocular dz (glaucoma, iritis)
new migraine
subacute h/a causes
temporal arteritis
intracranial tumor
SDH
pseudotumor cerebri
trigeminal/glossopharyngeal neuralgia
post-herptic neuralgia
HTN
chronic/episodic h/a causes
migraine
cluster h/a
tension h/a
sinusitis
dental dz
neck pain
Migraine h/a S/S
throbbing h/a >2hrs, <24hrs
N/V
photophobia
noise sensitivity
"Classic" - u/l, visual aura (scintillating scotomas or field cuts)
"common" - b/l, periorbital without associated symp
Treatment of migraine h/a
ASA/NSAIDS, triptans
prophylaxis - BB, TCA, CCB, valproic acid
Cluster h/a description
brief, severe, usually u/l periorbital h/a 30min-3hrs
affects same part of head, same time of day (night), same time of year
triggers - EtOH, vasodilators
Cluster h/a S/S
ipsilateral tearing of eye
conjunctival injection
Horner's syndrome
nasal stuffiness
Treatment of cluster h/a
acutely - high flow O2 (100% non-rebreather)
ergots
sumatriptan
intranasal lidocaine
corticosteroids
Prophylaxis of cluster h/a
ergots
CCB
prednisone
lithium
valproic acid
topiramate
SAH causes
ruptured aneurysm (berry)
AVM
trauma (most common)
Berry aneurysms associated with?
PcKD
coarctation of aorta
CN involvment in SAH
CN III palsy with pupil involvement associated with Berry aneurysms
Treatment of SAH
prevent rebleeding - most likely in 1st 48hrs
prevent vasospasm - nimodipine and IV fluids, maintain elevated BP
antiseizure meds - phenytoin
NO NSAIDs
Complications of SAH
rebleeding aneurysm>AVM
extension into brain parenchyma (AVM)
vasospasm (in 1/3 of aneurysmal SAH)
obstructive hydrocephalus
EDH CT findings
lens-shaped CONVEX hyperdensity
SDH CT findings
cresent-shaped, CONCAVE hyperdensity
parenchymal hemorrhage causes
HTN - usually in basal ganglia
tumor
amyloid angiopathy (elderly)
vascular malformations (AVM, cavernous hemangiomas)
Cushing's triad
alterations in BP, HR, and respiratory patterns
Parkinson's tetrad
resting tremor
bradykinesia
rigidity
postural instability
treatment of parkinson's
dopamine agoinsts - brompcriptine, levo/carbadopa
MAO-B inhibitors - selegiline (neuroprotective, decreases levodopa need)
COMT inhibitors - entacapone (increase levodopa availability, may decrease motor fluctuations)
(amandatine, antiAch - limited efficacy)
astrocytomas
Adults
S/S h/a, increased ICP
+/- u/l paralysis CN V-VII, X
slow, protracted course
Rx - resection, XRT
GBM
most common primary
h/a, increased ICP
rapid progression, poor Px (<1yr)
meningiomas
women>men
from dura or arachnoid
increased incidence with age
acoustic neuroma
ipsilateral hearing loss
tinnitus
vertigo
signs of cerebellar dysfunction
Derived from Schwann cells
Medulloblastoma
children
from 4th vent - increased ICP
highly malignant - can seed subarachnoid space
Rx - Sx + chemo + XRT
ependymoma
children
from ependyma of ventricle (4th) or spinal cord
hydrocephalus
Rx - Sx + XRT
Neurocutaneous syndromes
NF 1,2
Tuberous sclerosis
NF 1 mutation
NFT-1 gene on 17q
NF 2 mutation
defective gene chrom. 22
NF1 diagnostic criteria
>=2 of:
1) 6 cafe au lait spots (>5mm kids, 15mm adults)
2) 2 neurofibromas (any type)
3)feckling-axillary/inguinal
4) optic glioma
5) 2 Lisch nodules = pigmented iris hamartomas
6)osseus abnormalities
7) 1st degree relative
NF2 diagnostic criteria
bilateral acoustic neuromas
1st degree relative
unilateral acoustic neuroma
any 2 of:
neurobfibroma
meningioma
glioma
schwannoma
Other features (not criteria)
seizures, skin nodules, cafe-au-lait spots
Tuberous Sclerosis - characterized by:
seizures (begin <1y/o)
mental retardation
skin and eye lesions
Tuberous sclerosis - presentation
infantile spasms
ash-leaf hypopigmented lesions
(trunk and extremities)
Skin lesions in tuberous sclerosis
sebaceous adenomas (small red nodules over nose and cheeks like acne)
shagreen patch (rough papule in L/S region with orange peel consistency)
Ash-leaf hypopigmented lesions (trunk and extremities)
Retinal lesions in tuberous sclerosis
mulberry tumors (arise from nerve head)
phakomas (round, flat, grey lesions near disc)
Tests and findings in Tuberous sclerosis
calcified tubers in periventricular area (rarely transform into malignant asyrocytomas)
Skin lesions with Wood's lamp
ECG for rhabdomyoma (apex of LV in 50% pts)
Renal U/S - renal hamartomas, PcKD
Pulmonary - angiomyolipomas (cause generalized cystic or fibrous changes)
Thiamine deficiency
Wernicke's encephalopathy
Korsakoff's dementia
Wernicke's encephalopathy
triad = encephalopathy, ophthalmoplegia (LR palsy,nystagmus, conjugate gaze palsy), ataxia (polyneuropathy, CB and ventricular dysfunction)
Korsakoff's dementia
Wernicke's (encephalopathy, ophthalmoplegia, ataxia) + amnesia, horizontal nystagmus
Way to elicit Wernicke's encephalopathy
Large dose glucose administration
Cyanocobalamin deficiency
= B12 deficiency
Combined system disease or subacute combined degeneration
Cyanocobalamin deficiency S/S
gradual, progressive onset
symm. P/N
leg stiffness
spasticity
paraplegia
bowel/bladder dysfunction
Dementia
Folate deficiency
irritability
memory loss
personality changes without CSD of B12
Seizure causes in infants
perinatal injury
infection
metabolic
congential
Childhood (2-10y/o) causes of seizures
idiopathic
infection
trauma
fever
Adolescent causes of seizures
idiopathic
trauma
drug withdrawl
AVM
Young adult (18-35y/o) causes of seizures
trauma
alcoholism
brain tumor
Adult (>35y/o) causes of seizures
trauma
stroke
metabolic disorder
alcoholism
brain tumor
Lab finding in epileptic seizure
elevated prolactin
Simple partial seizures
motor, sensory, autonomic, or psychic features without LOC
+/- post-ictal focal neuro
deficit resolves 1-2 days
Can evole into generalized
DDx from acute CVA with MRI
Complex partial seizures
temporal lobe (70-80%)
char by:
impaired LOC
auditory/visual hallucinations
deja vu
automatisms
post-ictal confusion/disorientation, amnesia
Symp can mimic schizophrenia/acute psychosis
Can evolve into generalized
Normal EEG in seizure
Does NOT r/o epileptic sz disorder
First line anti-convulsant in children
phenobarbital
1st line treatment of idiopathic generalized seizures
Valproic acid
Adjuncts - lamotrigine, topiramate
treatment of secondary tonic-clonic seizures
Same as partial seizures
Absence seizures EEG finding
3/sec spike-and-wave discharges (classic)
Absence seizures treatment
ethosuximide - 1st line
Valproic acid, zonisamide
West syndrome
Infantile spasms
Abnormal interictal EEG (v. high amplitude slow waves)
Arrest of psychomotor devt at age of sz onset
West syndrome S/S
begins 3-12m
sz - tonic, b/l, symm in clusters of 5-10 individual spasms - occur when drowsy or on awakening
MR in most pts
West syndrome Rx
ACTH
prednisone
anti-epileptics (clonazepam, valproic acid)
Status epilepticus definition
prolonged (>30min) or repetitive sz without a return to baseline
20% mortality
Status epilepticus causes
anticonvulsant withdrawl/non-comliance
anoxic brain injury
EtOH/sedative withdrawl
drug intoxication
metabolic - hyponatremia
trauma
infections
Treatment of Status epilepticus
ABC's
IV benzo + phenytoin loading dose
If sz continue - intubate + phenobarbital loading dose
glucose, thiamine, naloxone
Risk factors for CVA
non-modifiable - male, age, genetics, race (African-American, Hispanic, Asian)
Modifiable - HTN, DM, obesity, smoking, hypercholesterolemia, carotid stenosis, heavy EtOH, cocaine, IVDU, AFib
CVA most common etiology
athlerosclerosis of extracranial vessels (internal and common carotids, basilar, vertebral)
Underlying causes of lacunar infarcts
athlerosclerosis
HTN
DM
cardiac causes of CVA
Afib (risk increased 5-6x)
emboli - mural thrombi, diseased/prosthetic valves, paradoxic (venous) in R-to-L shunt (ASD, PFO)
MCA stroke S/S
aphasia (dominant hemisphere)
neglect (non-dominant)
contralat. hemiparesis, gaze preference, homonymous hemianopsia
ACA stroke S/S
leg paresis
amnesia
personality changes
foot drop
gait changes
cognitive changes
PCA stroke S/S
homonymous hemianopsia
memory deficits
dyslexia/alexia
Basilar stroke S/S
coma
CN palsies
apnea
visual symp
drop attacks
dysphagia
lacunar stroke S/S
pure motor or sensory
dysrthria-clumsy hand syndrome
ataxic hemiparesis
tPA contraindications
SBP >185 or DBP >110 despite aggressive antihypertensive Rx
prior ICH
CVA/head trauma in past 3m
recent MI
current anticoagulant Rx with INR >1.7
heparin in last 48hrs with increased PTT
platlets <100000
major Sx in last 14d
GI/GU bleed in past 21d
sz present at CVA onset
glucose <50/>400mg/dL
age <18
5 A's of Guillain-Barre
Acute inflammatory demyelinating polyradiculopathy
Ascending paralysis
Autonomic neuropathies
Arrhythmias
Albuminocytologic dissociation
Findings in Guillain-Barre
EMG/nerve conduction studies - diffuse demyelination
CSF - protein >55mg/dL with little/no pleocytosis
Myasthenia gravis antibodies
postsynaptic ACh receptor Ab (85-90%)
antistriational Ab (85% of pts with thymoma)
Labert-Eaton Syndrome antibodies
autoantibodies to presynaptic Ca channels
Myasthenia Gravis assoicated disorders
thymoma
thyrotoxicosis
autoimmune - SLE, RA
Myasthenia Gravis S/S
fatiguable ptosis/diplopia
dysphagia
proximal muscle weakness
symp worsen throughout day
"Myasthenic crisis"
respiratory compromise and aspiration (rare)
Drug induced myasthenic syndrome cause
aminoglycosides
Myasthenia Gravis findings
edrophonium - dramatic improvement (diagnostic)
Abnormal single fiber EMG +/or decremental response to repetitive nerve stim
ACh Ab (85-90%)
Antistriational Ab (85% of pts with thymoma)
Treatment of MG
anticholinesterases - neostigmine, pyridostigmine
immunosuppressants - prednisone
Resection of thymoma - can be curative
ALS pathogenesis
loss of motor neurons in spinal cord, brain stem, and motor cortex
ALS S/S
asymmetric, slowly progressive weakness of arms, legs, CNs
UMN and LMN signs
Diagnostic findings in ALSq
Combination of U and LMN signs in >= 3 extremities
Findings in ALS
EMG/NCS - widespread denervation and fibrillation potentials
treatment in ALS
supportive
aggressive pulmonary toilet
Riluzole - reduces presynaptic glutamate release, may slow progression
Risk factors for carpal tunnel syndrome
repetitive use injury
pregnancy
DM
hypothyroid
acromegaly
RA
obesity
MS possible pathogenesis
T cell mediated autoimmune progression
MS classic triad
scanning speech
intranuclear ophthalmoplegia
nystagmus
Most common presenting complaints in MS
limb weakness
optic neuritis
P/N
diplopia
urinary retention
vertigo
Exacerbating factors in MS
infection
heat
trauma
vigorous activity
(decreasing - pregnancy)
MRI findings in MS
multiple, asymmetric, often periventricular white matter lesions
Corpus callosum lesions ~pathognomonic
Active lesions enhance with gadolinium
CSF findings in MS
mononuclear pleocytosis (>5cells/microL) 25%
elevated IgG - 80%
oligoclonal bands (non-specific) albuminocytologic dissociation
Treatment for MS
Steroids - acute exacerbations
Immunomodulators - ABC
Avonex/Rebif (interferon-β-1a)
Betaseron (interferon-β-1b)
Copaxone (copolymer-1)
Symtomatic Rx for spasticity, pain, fatigue, depression
Closed angle glaucoma epidemiology
elderly
Asians
Causes of closed angle glaucoma
acute closure of narrow anterior chamber angle ==>
pupillary dilatation - prolonged time in dark, stress, meds
ant. uveitis
dislocation of lens
Closed angle glaucoma S/S
extreme pain and blurred vision, ~always u/l
eye hard and red
pupil dilated, non-reactive to light
N/V
increased IOP
Treatment of Closed angle glaucoma
lower intraocular pressure - acetazolamide
pilocarpine once pressure drops
laser iridotomy - curative
open angle glaucoma risk factors
>40 y/o
FHx
African-American
DM
myopia
Open angle glaucoma pathogenesis
intraocular pressure increase d/t diseased trabecular meshwork that obstructs proper drainage of eye ==> gradual pressure increase causing progressive visual loss
Open angle glaucoma S/S
frequent lens changes in pts >35y/o
mild h/a
visual disturbances
impaired adaption to darkness
visual loss begins peripherally
Fundoscopic findings in open angle glaucoma
cupping of optic disc
Treatment of open angle glaucoma
preventative - >40y/o see ophthalmologist q3-5yrs (annual if increased RFs)
topical α-blockers - timolol, betaxolol (decrease aqu. humor producation)
pilocarpine (increases aqu. outflow)
carbonic anhydrase inhibitors if eye drops insufficient control of IOP
laser trabeculoplasty
Macular degeneration risk factors
Caucasian
female
smokers
FHx
Macular degeneration types
atrophic - gradual visual loss
exudative - more rapid and severe visual loss
macular degeneration S/S
painless loss of central vision
macular degeneration fundoscopy findings
pigmentary or hemorrhagic disturbances of macular region
treatment of macular degeneration
limited
laser photocoagulation may delay loss of central vision in exudative type
CRAO S/S
sudden, painlesss, u/l blindness
pupil accomodates
sluggish pupil reaction to direct light
CRAO fundoscopy findings
cherry-red spot on fovea
arteries +/- bloodless appearance
+/- retinal swelling
CRVO S/S
rapid painless vision loss
elderly pts
fundoscopy findings of CRVO
retinal hemorrhages
cotton-wool spots
edema of fundus
sequelae of CRVO
macular degeneration
glaucoma
Treatment of CRAO
thrombolysis of ophthalmic artery within 8hrs of symp onset
IV acetazolamide -decrease IOP - increase drainage of aqu. humor
sequelae of CRAO
untreated - retinal infarction, permanent blindness
Treatment of CRVO
laser photocoagulation - variable results
Estrogen functions (8)
follicle growth
endometrial proliferation
myometrial excitability
hepatic synthesis of transport proteins
feedback inhibition of FSH
LH surge
smooth muscle relaxation
Progesterone functions (5)
endometrial gland secretions
spiral artery development
decreased myometrial excitability
increased body temperature
inhibition of LH, FSH
hCG functions (2)
maintians corpus luteum for T1 by acting like LH
detect pregnancy (in urine at 8 days)
physiologic changes in pregnancy - CVS (4)
increased CO (30-50%), HR (10-15bpm), SV
possible S3, syst. murmurs (Normal) (new diast. murmur never normal!)
decreased SVR
decreased BP T1, normalizes by 40 weeks
cardiomeagly on CXR d/t heart displacement upwards
physiologic changes in pregnancy - cervix (2)
softening and cyanosis ~4wks
bloody show = thick mucus clot
physiologic changes in pregnancy - endocrine (3)
high estrogen => ↑TBG, bound T3, T4, unchanged active unbound hormone
HPL - acts as insulin antagonist to maintain fetal glucose => prolonged post-prandial hyperglycemia, fasting hyperinsulinemia, triglyceridemia, exaggerated starvation ketone response
↑total and free cortisol
physiologic changes in pregnancy - GI (4)
N/V (<=70%) resolves by 14-16wks
↑acid reflux d/t ↓LES tone
constipation d/t ↓bowel motility,↑H2O absorption
↑biliary cholesterol
physiologic changes in pregnancy - hematologic
↑plasma V (50%), RBC mass (30%)=> ↓Hb, hct = 'physiologic anemia'
WBC count ↑s throughout X=10
pregnancy = hypercoagulable state
physiologic changes in pregnancy - pulmonary
↑tidal V (40%)
↓TLC, RV,ERV
RR same
↑minute vent. => ↑Pa/PAO2, ↓Pa/PACO2 = 'dyspnea of pregnancy'
physiologic changes in pregnancy - Renal
kidneys dilate
↑GFR (50%)
↑RPF (30%)
↑estrogen/progesterone => ↑RAAS = ↑aldosterone
physiologic changes in pregnancy - skin
↑estrogen - stigmata like liver disease
↑αMSH, steroids => hyperpigmentation - midline (linea nigra), face (chloasma), perineum
physiologic changes in pregnancy - vagina
thick acidic secretions
Chadwick's sign = violet colouration from ↑blood flow
Nagle's rule = EDD
add 9m + 7d to 1st day of LMP
Ways to determine GA
uterine size
quickening (17-18wks)
fetal heart tones (10wks)
U/S fetal crown-rump length (5-12wks) biparietal diameter (20-30wks)
Labs at initial visit for pregnancy
CBC, UA + culture, Pap, blood type, Rh + Ab screen, rubella Ab titer, HBsAg, syphilis screen, cervical gonoccocal & chlamydia
PPD
glucose teting
HIV
Labs at 15-19wks GA
MSAFP or triple screen (=MSAFP, estiol, β-hCG)
amnio if >35y/o
Labs at 26-28wks GA
glucose loading test
give Rhogam at 28 wks if mom Rh-ve
Labs at 32-36wks GA
cerval chlamydia + gonorrhea in high risk
GBS screen
causes of ↑MSAFP (MoM >2.5)
1. open NTD - anencephaly, SB
2. abdominal wall defects - gastroschisis, omphalocele
3. multiple gestation
4. incorrect dates
5. fetal death
6. placental abnormalities - abruption
causes of ↓MSAFP (MoM<0.5)
need amnio and karyotyping to r/o chromosomal abnormalities
- trisomy 18, 21
pattern of triple screen with Down's
↓AFP, estriol
↑hCG
pattern of triple screen with trisomy 18
↓AFP, estriol, hCG
Compare complications of amnio with CVS
amnio (15-17wks)
fetal maternal hemorrhage 1-2%
fetal loss 0.5%
CVS (10-12wks)
fetal loss 1%
inability to Dx NTD
limb defects if <9wks GA
Indications for percutaneous umbilical blood sampling
Ax/Rx Rh isoimmunization/erythroblastosis fetalis
karyotyping
fetal infection (CMV, toxo, rubella)
genetic dz
fetal acid-base status
Normal labour and deliver stages
first
latent - onset to 3-4cm
active - 4cm to 10cm (1.2cm/h primi, 1.5cm/h
multip)increased with CPD
2nd - complete dilatation to delivery
34d - delivery of infant to placenta
Factors affecting active phase of labour
3P's
power
passenger
pelvis
Normal NST
FHR monitored by doppler correlated with reported mvts by mom
- acceleration ≥15bpm above baseline for ≥15sec twice in 20min
positive contraction stress test
repetitive late decels during ≥3 contractions in 10min
Biophysical profile
"Test the Baby MAN" - give score of 0 or 2 for each
Tone
Breathing
Movement
Amniotic fluid volume
NST
"+ve test" = score 0-2
Early decelerations
begin and end with contraction
caused by cephalic compression (no fetal distress)
Variable decelerations
at any time during contraction
Caused by:
umbilical cord compression (change maternal position)
Late decelerations
begin at peak of contraction and end after contraction has finished
- uteroplacental insufficiency
- fetal hypoxemia
- potentially d/t abruption or hypotension
- if repetitive and severe deliver ASAP
Hyperemesis Gravidarum
intractable N/V after 14-16wks
poor wt gain/wt loss
more common in G1P0, molar pregnancies
labs - ketonemia, ketonuria, hyponatremia, hypokalemic-hypochloremic m.a.
Diagnosis of GDM
fasting serum glucose >126mg/dL
random >200mg/dL
abnormal GTT
1hr (50g) >140gm/dL
3hr (75g)with any 2 of -
fasting ≥95,
1h ≥180
2h ≥155
3h ≥140
Pregestational DM is associated with?
↑risk congenital malformations and maternal/fetal morbidity during labour and delivery
Pregnancy induced HTN definition
HTN with proteinuria <300mg/dL
maternal complications of pregestational DM (8)
DKA or HHNK
pre/eclampsia
CPD
Preterm labour
infection
polyhydramnios
PPH
maternal mortality
fetal complications of pregestational DM (11)
macrosomia
cardiac and renal defects
hypoglycemia from hyperinsulinemia
hypocalcemia
RDS
polycythemia
hyperbilirubinemia
NTD (sacral agenesis)
IUGR
birth injury (sh. dystocia)
perinatal mortality
Preeclampsia
new-onset HTN
proteinuria
non-dependent edema
>20wks
Risk factors for pre/eclampsia
nulliparity
black race
extremes of age
multiple gestation
molar pregnancy
renal dz (SLE, DM1)
FHx
chronic HTN
Explanation for pre/eclampsia
vasospasm causing hemorrhage and organ necrosis
Mild preeclampsia S/S
BP>140/90 2x 6hrs apart
rapid wt gain
nondependent edema
proteinuria 1-2+, >300mg/24h
Severe preeclampsia S/S
BP>160/110 2x 6hrs apart
proteinuria >5g/24h 3-4+
cerebral changes - h/a, somnolence
visual changes - blurred, scotoma
hyperactive reflexes, clonus
oliguria (<500ml/24h)
HELLP
oligohydramnios, IUGR
pulmonary edema, cyanosis
RUQ/epigastric pain
Severe pre-eclampsia Rx
deliver (C/S or induce) ASAP
BP <160/100 - diast 90-100
- IV hydralazine, +/or labetalol
prevent seizures - MgSO4
Mg Toxicity S/S, Rx
Loss of DTRs
respiratory paralysis
coma
Rx - IV Ca gluconate
Complications of preeclampsia
prematurity
fetal distress
stillbirth
placental abruption
sz
DIC
ICH
serous retinal detachment
fetal/maternal death
Complications of eclampsia
ICH
aspiration pneumonia
hypoxic encephalopathy
thromboembolic events
maternal/fetal death
Teratogenic effects of - EtOH
microcephaly
midfacial hypoplasia
MR
IUGR
cardiac defects
Teratogenic effects of - cocaine
bowel atresias
IUGR
microcephaly
Teratogenic effects of - streptomycin
CN VIII damage
ototoxicity
Teratogenic effects of - tetracycline
teeth discolouration
inhibition of bone growth
small limbs
syndactyly
Teratogenic effects of - sulfonamides
kernicterus
Teratogenic effects of - quinolones
cartilage damage
Teratogenic effects of - isotretinoin
heart and great vessel defects
craniofacial dysmorphisms
deafness
Teratogenic effects of - coumadin
stippling of epiphyses
IUGR
nasal hypoplasia
MR
Teratogenic effects of - ACEI
oligohydramnios
fetal renal damage
Teratogenic effects of - lithium
Ebstein's abnormality (abnormal attachments of tricuspid valve leaflets to annulus of TV)
other cardiac dz
Teratogenic effects of - carbamazepine
fingernail hypoplasia
IUGR
microcephaly
NTD
Teratogenic effects of - phenytoin
nail hypoplasia
IUGR
MR
craniofascial dysmorphism
microcephaly
Teratogenic effects of - valproic acid
NTD
cariofacial and skeletal defects
Symmetric IUGR
symm - 20%
all U/S parameters ↓
occurs early
caused by fetal problem
- cytogenetic
- infection
- anomalies
Asymmetric IUGR
80%
↓abdominal circumference, all other U/S parameters N
Late
Placenta mediated
- HTN
- poor nutrition
- maternal smoking
Oligohydramnios
AFI <5 on U/S
40x ↑perinatal mortality without ROM
Causes
- fetal urinary tract abnormalities (renal agenesis, PcKD, GU obstruction)
- chronic uteroplacental insufficiency (SGA fetuses)
- ROM
Complications of oligohydramnios
msk abnormalities
- club foot
- facial distortion
pulmonary hypoplasia
fetal hypoxia d/t cord compression
IUGR
Polyhydramnios
AFI >20 on U/S
usually asymp vs fundal ht >expected
Causes
maternal DM
multiple gestation
isoimmunization
pulmonary abnormalities (cystic lung malformation)
fetal anomalies (duodenal atresia, TEF, anencephaly)
twin-twin transfusion synd
Complications of polyhydramnios
preterm labour
fetal malpresentation
cord prolapse
When to give Rhogam
mom is Rh -ve at 28wks
father is Rh +ve
father's Rh status unknown
postpartum if baby Rh+ve
Complications of Rhesus isoimmunization
hydrops fetalis
fetal hypoxia and acidosis
kernicterus
prematurity
fetal death
hydrops fetalis
when Hb <7g/dL
↓protein and oncotic pressure
edema
jaundice
high output cardiac failure
hydatiform mole - complete
46XX
paternally derived (sperm fertilization of empty ovum)
hydatiform mole - partial
N ovum fertalized by 2 sperm or by haploid sperm that duplicated own chromosome
69XXY
contains fetal tissue
Risk factors for GTD
extremes of age
diet deficiency - folate, β-carotene
blood group - type A♀ by type O♂
Molar pregnancy S/S
uterine bleeding (most common)
hyperemesis gravidarum
pre/eclampsia <24wks (preeclampis in T1 is pathognomonic for hydatiform mole)
uterine size >dates
hyperthyroidims
no FHR
enlarged ovaries with b/l theca-lutein cysts
expulsion of grape-like molar clusters into vagina
findings of complete hydatiform mole
"snow-storm appearance" on U/S
↑↑↑ βhCG (>100000mIU/ml)
no gestational sac/fetus
CXR - +/- lung mets
T3 bleeding - most common causes
placental abruption
placenta previa

Other causes:
bloody show
ruptured vasa previa
early labour
ruptured uterus
marginal placental separation
genial tract lesions/trauma
placenta accreta
Rx of hydatiform mole
D&C - "cluster of grapes" tissue
close f/u βhCG
prevent pregnancy x 1yr
malignant - MTX or dactinomycin
residual uterine dz - hysterectomy
chemo/XRT - mets
Placental abruption
premature separation of normally implanted placenta
1/100
Risk factors for placental abruption
HRN
trauma
tobacco/cocaine
PHx
rapid decompression of overdistended uterus
Symptoms of placental abruption
Painful
dark vaginal bleeding that does not stop spontaneously
uternine hypertonicity
fetal distress
Dx of placental abruption
clinical
U/S 50% sensitivity - look for retroplacental clot
use to r/o previa
Complications of abruption
hemorrhagic shock
DIC (10%)
recurrence - 5-16%
fetal hypoxia
Placenta previa
abnormal placental implantation
total - covers os
marginal - extends to margin of os
low-lying - close to os
1/200
Risk factors for placenta previa
prior C/S
grand multip
advanced maternal age
multiple gestation
PHx
Placenta previa S/S
painless
bright red bleeding, often stops 1-2hrs +/- contractions
first bleed 29-30wks
no fetal distress
Dx placental previa
U/S - sensitivity >95%
Rx placenta previa
No vaginal exams!
tocolytics
Serial U/S
Ax FLM
C/S
Complications of placenta previa
increased risk of accreta
vasa previa
preterm delivery
PROM
IUGR
congenital anomalies
recurrence 4-8%
Primary biliary cirrhosis S/S
pruritis
jaundice
heptosplenomegaly
increased ALP, bilirubin, cholesterol
+ve antimitochondrial Ab
xanthalasma
Chalazion
inflammatory lesion of meibonian tear glands
seen in rosacea, blepharitis
hordeolum
purulent inflammation of eyelid
usually d/t Staph
Serious side-effects of PTU and Rx
agranulocytosis
Stop immediately if develops sore throat or fever
neonatal gonoccocal conjunctivitis
copious purulent eye discharge
swollen eyelids
chemosis
2-3d after birth d/t exposure to genital secretions
neonatal chlamydia conjunctivitis
scant mucoid discharge (not as purulent as gonococcal)
mild hyperemia
5-14d after birth d/t genital tract exposure
risk factors for colonic polyp to become malignant
villous adenoma
sessile adenoma
>2.5cm
Acute bacterial prostatitis organisms
young pts - chlamydia, gonorrhea
elderly - E. coli (most common), G-ve rods
Prostatic manipulations can cause septicemia (contraindicated)
Need urine sample
Empiric anaerobic coverage in neutropenic pts is indicated when...?
Any evidence of:
necrotizing mucositis
peridontal abscess
perirectal abscess/cellulitis
typhilitis (=necrotizing neutropenic colitis)
intra-abd/pelvic abscess
anaerobic bacteremia
Most common organisms in febrile neutropenia
gram negatives esp. P. aeroginosa
Antibiotics of choice in febrile neutropenia (on spec)
cefipime
ceftazidime
Malignant otitis externa S/S, cause
severe ear pain
ear discharge
radiates to TMJ
worse with chewing
worsens despite topical Abx
granulation tissue in EAM
Most common - P. aeroginosa
rare - staph, aspergillus
Preferred Rx for Absence sz
1. ethosuximide
2. valporic acid
3. lamotrigmine, clonazepam
Drug of choice for partial seizures
phenytoin
Splenic sequestration
vaso-occlusion and pooling of RBCs in spleen
marked decrease Hb, increased retics
P/E - rapidly enlarging spleen +/- hypotensive shock
10-15% mortality
50% recurrence
Rx - splenectomy
acute chest syndrome in SCA
chest pain
fever
new infiltrate on CXR
aplastic crisis in SCA
transient arrest of erythropoesis usually d/t infection
parvovirus B19 most common cause
recommendations for pneumococcal vaccine
1. >65y/o
2. chronic dz - CVS, renal, lung, liver
3. immunosuppressed/DM
4. chronic EtOH, institutionalized
Criteria for home O2
1. All COPD pts with PaO2 <55mmHg or SaO2 <88% on r/a
2. cor pulmonale, PHTN, Hct>55% when PaO2 <60mmHg
3. exercise or nocturnal hypoxia
hereditary spherocytosis
AD
chronic extravascular hemolysis (spleen only)
spectrin deficiency (RBC membrane intrinsic defect)
indirect hyperbilirubinemia
increased osmotic fragility
defect in decay accelerating factor
paroxysmal nocturnal hemoglobinuria
Acute GVHD
50% of transplants from matched siblings
S/S
Skin maculopapular rash of palms, soles face +/- generalizes
GI - bloody diarrhea
liver - abnormal LFTs, jaundice
Acute GVHD pathophysiology
recognition of host major and minor HLA antigens by donor T cells leading to cell-mediated response
immunoblastic lymphoma
EBV induced lymphocytosis in BMT
cholesteatoma definition, symptoms
epithelial cyst with desquamated keratin (most commonly infected with P. aeroginosa)
located medial to normal TM
congenital or aquired from OM, eustacian tube defects
present with recurrent infections or progressive conductive hearing loss
cholesteatoma presentation
Most common - drainage and granulation tissue unresponsive to abx
O/E
marginal TM perforation
pus, mucous, granulation
tissue filled ear canal
destroys bone - can extend into cranium
Rx - Sx
Serous otitis media
d/t prolonged blockage of eustacian tube
conductive hearing loss
dull TM, hypomobile
air bubbles seen in middle ear
Chronic otitis media
purulent aural discharge
TM thickened with calcific patches and perforation
no exudation of debris or granulation tissue
Complication - tympanic sclerosis (rare hearing loss)
medical treatment of gallstones
ursodeoxycholic acid
low fat diet
progressive and bilateral loss of central vision with preservation of navigational (peripheral) vision
macular degeneration
Koplik spots
pathognomonic of rubeola (measles)
=bluish white lesions (can appear like table salt crystals) on erythematous buccal mucosa opposite 1st and 2nd upper molars (sometimes conjunctivae and vagina also)
fever, non-productive cough, coryza, non-purulent conjunctivitis, Koplik spots
prodrome of measles (rubeola)
Kawasaki's disease S/S
<10y/o
fever >5days
mucous membrane changes (injected pharynx, strawberry tongue, fissure lips)
extremity changes (edema, erythema, desquamation)
>=1cervical LN >1.5cm
polymorphous rash
scarlet fever S/S
sand-paper like erythematous rash +/- desquamation
strawberry tongue
fever
pharyngitis
exanthema subetum, 6th disease, rubeola - what virus?
Important complication
HHV6 - high fever in infants for 3-4days
Most common cause infantile seizures
rubella S/S
mild fever
malaise
post. cervical lymphadenopathy
patchy erythema of throat and palate
3 day rash - fine, pink, maculopapular, rapidly migrates from face to trunk
Iron toxicity S/S, Cx, Rx
corrosive to GI tract
hemorrhagic gastroenteritis
acidosis
hypotension
Cx - intestinal perforation
Rx - defuroximine IV
EDTA
lead toxicity
CHD risk factor equivalents
DM
symptomatic CAD
AAA
peripheral arterial disease
multiple risk factors present that confer 10yrs risk of CHD >20%
LDL in CHD or equivalent
goal LDL <100
lifestyle change at LDL = 100
statins if LDL>130
LDL with 2/more risk factor
goal LDL <130
lifestyle change at LDL = 130
statins if LDL>160
LDL with 0-1 risk factor
goal LDL <160
lifestyle changes at/>160
statins at LDL >190
SLE features
systemic - fever, fatigue, wt loss
mucocutaneous symp - discoid rash, malar rash, alopecia, oral/nasopharyngeal ulcers, photosensitivity
serositis - pericarditis, pleuritis
renal dz
mesangial
focal proliferative
diffuse proliferative - "wire-loop" pattern from immune-complex deposition and GBM thickening (progressive irreversible)
membranous
Neurologic - psychosis, depression, seizures, neuropathy
Non-erosive arthritis
Hematologic
hemolytic anemia, leuko/lymphopenia, TCP
Acute renal crisis
Cx of systemic sclerosis
malignant HTN - hypertensive encephalopathy, retinopathy, seizures, LV failure
SLE - most common cause of death
chronic renal failure
effect modification
effect of the main exposure on outcome is modified by effect of another variable
e.g. risk of estrogens in dvt of thromboembolic dz is modified by smoking
Indications for C/S
Maternal
prior classical
active genital herpes
cervical ca
Maternal/Fetal
CPD (most common cause)
placenta previa/abruption
failed operative vaginal delivery
Fetal
Malposition
distress
cord compression
erythroblastosis fetalis
Post-Partum Hemorrhage causes
uterine atony (most common -90%)
genital tract trauma
retained placental tissue
Risk factors for uterine atony
uterine overdistension - multiple gestation, macrosomia, polyhydramnios
exhausted myometrium (rapid/prolonged labour, oxytocin stim)
Conditions interfering with contractions (anaesthesia, myomas, MgSO4)
Dx of uterine atony
palpation of soft, enlarged, "boggy" uterus
Rx of uterine atony
bimanual uterine massage
Oxytocin infusion
methylergonovine (if no HTN)
prostin (PGF2α) if no asthma
Risk factors for genital tract trauma
precipitous labour
operative vaginal delivery
large baby
inadequate episiotomy repair
Risk factors for retained placental tissue
placenta accreta/increta/percreta
placental previa
uterine leiomyomas
preterm delivery
previous C/S, D&C
Dx of retained placental tissue
manual and visual inspection of placenta and uterine cavity for missing cotyledons
+/- U/S of uterus
Rx of retained placental tissue
manual removal and curettage with suctioning
Post-partum infection
"7W's"
Womb (most common)
endometritis
Wind
atelectasis, pneumonia
Walk
DVT, PE
Water
UTI
Wound
episiotomy, incision
Weaning
mastitis, breast engorgement/abscess
Wonder drugs
drug fever
Also:
pelvic abscess
septic pelvic thrombophlebitis (consider these if no improvement with 3drugs in 48hrs)
Definition of and risk factors for post-partum infection
≥38°C for ≥2d of 1st 10 post-partum days (not including 1st 24h)
RF
C/S especially emergent
PROM
prolonged labour
multiple exams
intrauterine manipulations
Management of post-partum infections
pelic exam to r/o hematoma, lochial block
U/A & culture
blood cultures
admit, broad spect Abx - clinda + gent
Sheehan's syndrome
pituitary ischemia and necrosis → ant pituitary insufficiency secondary to massive obstetric hemorrhage and shock
primary cause of ant pituitary insufficiency in adult ♀
Sheehan's syndrome S/S
failure to lactate - most common (d/t ↓prolactin)
weakness
lethargy
cold insensitivity
genital atrophy
menstrual disorders
Lactation physiology
↑estrogen/progesteron during pregnancy → breast hypertrophy and inhibit prolactin
↓↓hormones with delivery releases prolactin
suckling releases prolactin and oxytocin → myoepithelial cell contraction and milk ejection (=let down reflex)
Colostrum
protein, fat, secretory IgA → passive immunity and protects against enteric bacteria
Breast milk
within 1 week
+++ leukocytes → active immunity
protein, fat, lactose, water
Benefits of breast feeding
active and passive immunity to baby
decreased incidence allergies
mother-child bonding
maternal wt loss
Contraindications to breast feeding
HIV
active hepatitis
medications - tetracycline, chloramphenicol, warfarin
Mastitis
cellulitis of periglandular tissue caused by nipple trauma + introduction of S/ aureus from infants nostrils
~2-4wks post-partum
u/l >> b/l
focal breast tenderness
erythema, edema, warmth
+/- purulent nipple d/c
+ve breast milk culture
↑WBC
fever
Rx - continue BF, po abx (penicillin, dicloxacillin, erythromycin)
I&D for abscess
teratogenic effects of phenytoin
digital hypoplasia
facial abnormalities
sulfonamides should be avoided in the last few weeks of pregnancy because...?
They competitively inhibit binding of bilirubin to albumin increasing the risk of neonatal hyperbiliruminemia
Nitrofurantoin should be avoided in late pregnancy because...?
risk of hemolysis d/t deficiency of erythrocyte phosphate dehydrogenase in newborn.
menorrhagia
>80ml blood loss/cycle or prolonged bleeding (>8d)
Caused by
leiomyomas
adenomyosis
endometrial hyperplasia/polyps
1° bleeding disorders
pregnancy Cx
metrorrhagia
bleeding between periods
caused by
endometrial polyps
endometrial/cervical ca
pregnancy Cx
exogenous estrogen
menometrorrhagia
heavy bleeding during and b/t periods
caused by
endometrial polyps
endometrial/cervical ca
pregnancy Cx
exogenous estrogen
polymenorrhea
frequent menstruation (<21d cycle)
Caused by:
anovulation
oligomenorrhea
infrequent menstruation (>35d cycle)
Caused by:
pregnancy (most common)
HPGA disruption
systemic dz
hypomenorrhea
unusually low menstrual volume and duration, regular frequency
Caused by:
hypogonadotropic hypogonadism (common in athletes/anorexics)
OCP's
Asherman's syndrome (=uterin scarring d/t Sx or infection)
outlet obstruction
Dysfunctional uterine bleeding
dx of exclusion = abnormal menstrual bleeding when no pathologic cause found
Cause - anovulation (90%)
post-menopausal bleeding
uterine bleeding >1yr after menopause
Cause:
vaginal atrophy
exogenous hormones
cancer
w/u for apparent ovulatory disorder
transvaginal U/S
sonohysterogram
D&C (gold standard)
w/u for apparent anovulatory disorder
βhCG, CBC, coags
endocrine - FSH, LH, TSH, prolactin
endometrial bx in all women with post-menopausal or chronic anovulatory bleeding
Rx for anovulatory DUB
OCPs or cyclic progestin therapy (=medroxyprogesterone 10mg/d x 10d/m)
If profuse
- high dose IV estrogen
- D&C
- endometrial ablation or hysterectomy (last resort)
Rx of ovulatory DUB
NSAIDs +/- OCP
primary amenorrhea
absence of menses and lack of secondary sexual characteristics by age 14
or
absence of menses by age 16 with/without secondary sexual characteristics
Secondary amenorrhea
absence of menses for 3 cycles or for 6 months with prior normal menses
Causes of primary amenorrhea
anatomic
Mullerian anomalies
vaginal agenesis
imperforate hymen
testicular feminization
Ovarine/uterine dysfunction
Ovarian failure
gonadal dysgenesis (turner's)
steroidogenic enzyme defects
constitutional devt delay
Central Regulatory disorders
hypothalamic dysfunction (Kallman's syndrome, anorexia, excess exercise, wt loss, stress, tumor, infection)
1° pituitary dysfunction (rare)
Causes of secondary amenorrhea
Anatomic
Asherman's syndrome
cervical stenosis (scarring of os)
Ovarian/uterine dysfunction
ovarian failure
pregnancy
PCOS
Central regulatory disorders
hypothalamic dysfunction (anorexia, exercise, wt loss, stress)
pituitary dysfunction (Sheehan's, panhypopituitism)
hyperprolactinemia
Evaluation of secondary amenorrhea
βhCG
serum prolactin
TSH
as indicated
- progestin challenge (if serum prolactin normal)
- serum FSH, estradiol
- CT/MRI
Elevated prolactin effects
inhibits FSH, LH release
positive progestin challenge test
= withdrawl bleeding after 5d of progesterone
means estrogen is present, outflow tract patent
Therefore anovulatory problem
Causes of anovulation
hypothalamic dysfunction
PCOS
cushing's syndrome
ovarian/adrenal tumor
estrogen-progesteron challenge
+ve = bleeding
suggests functional uterus without adequate estrogen stimulation
Causes - problem with follicular or HPA
-ve = asherman's syndrome
Fibrocystic change
cystic change, nodularity, stomal proliferation, epithelial hyperplasia
Cause
exaggerated stromal response to hormones and growth factors
Seen in ≤50% of postmenopausal women
Fibrocystic change S/S
cyclic (often premenstrual)
b/l breast pain
tenderness, swelling
Dx and Rx of fibrocystic change
FNA
↓caffeine, nicotine
Vit. E supplements
hormonal therapy - danazol, progestins, tamoxifen
diuretics for premenstrual mastalgia
increased breast cancer risk with fibrocystic change if...?
cellular atypia on FNA
Fibroadenoma
benign, slow growing tumor with epithelial and stromal components
most common lesion in <30y/o
Dx and Rx of fibroadenoma
round, firm, discrete, nontender, mobile, solitary mass
Rx - Sx excision
recurrence common
phyllodes tumor
rapidly growing, often large type of fibroadenoma
rarely malignant = cystosarcoma phyllodes
Risk factors for breast ca
female
older
FHx (1°)
PHx
fibrocystic change with cellular atypia
nulliparity
early menarche
late menopause
1st full term pregnancy >35y/o
Breast cancer S/S
hard, irregular, immobile, painless breast lump
+/- nipple discharge
skin changes, axillary adenopathy suggest advanced disease
breast cancer common site and metastases
upper outer quadrant
LN, bones, brain, lung, liver
Evaluation of breast cancer
palpable mass
mammogram with microcalcifications, hyperdense regions
U/S
FNA if cystic - if cyst doesn't resolve, bloody aspirate or recurs → excise
Bx - direct needle core, stereotactic, open surgical
ER/PR receptors/herzneu amplification
Inflammatory breast cancer
highly aggressive, rapid growth
invades lymphatics
causes skin inflammation
Paget's disease of breast
DCIS of nipple
itching, burning
nipple erosion (can be mistaken for infection)
associated with focus of invasive carcinoma
bilateral breast cancer
more common in young women and lobular type
Rx of breast LCIS
↑risk invasive in both breasts therefore → close f/u or b/l mastectomy (in high risk)
Rx of breast DCIS
depends on size
small tumors - local excision + close f/u
larger tumors - wide local excision + XRT or simple mastectomy (no node dissection necessary)
Invasive breast cancer
ductal more common
staging - size, nodes, mets → bone scan, CBC, Ca, CXR
Localized dz → lumpectomy + axillary node dissection + XRT or modified radical mastectomy
chemo for all premenopausal ♀ with +ve nodes
chemo in postmenopausal ♀ with +ve nodes and -ve hormone receptors
Rx for breast cancer with +ve estrogen receptors
hormone therapy (tamoxifen)
mets/recurrence with chemo
Rx option for herzneu amplification and metastatic dz
Herceptin
primary dysmenorrhea
no obvious organic cause
occurs (usually <20y/o)
may be d/t ++ PGs, leukotrienes, or ψ factors
Rx for primary dysmenorrhea
NSAIDs
OCP
dysmenorrhea
marked pain with periods that requires medication and prevents normal activity
Secondary dysmenorrhea
associated with specific pelvic pathology:
endometriosis (most common)
adenomyosis (=endometrial glands and stoma in myometrium)
myomas
pelvic congestion
PID
ovarian cysts
cervical stenosis
pelvic adhesions
Endometriosis
endometrial glands and stroma outside the uterus
common sites:
ovaries, cul de sac, uterosacral ligaments
proposed etiologies
endometrial cell implantation via retrograde menstruation
vascular and lymphatic dissemination of endometrial cells
metaplasia in peritoneal cavity
typically in ♀ of reproductive age
Risk factors of endometriosis
FHx
nulliparity
infertility
endometriosis S/S
premenstrual pain that resolves at onset of menses
dyschezia
chronic pelvic pain
dysparenuria
abnormal bleeding
infertility
tender nodularity along uterosacral ligament
fixed, retroverted uterus
tender, fixed adnexal masses
Evaluation of endometriosis
definitive dx by direct visualization laroscopy/laparotomy
rust-coloured to dark brown "powder burns" or raised blue "raspberry lesions"
in severe dz implants surrounded by extensive adhesions
ovarian endometriomas ("chocolate cysts" = cystic collections of thick, dark, old blood)
pain severity ≠ extent of dz
Rx of endometriosis
medical - OCP, progestin (to suppress ovulation/menses)
- danazol/GnRH agonists (to suppress estrogen production)
Sx
laporoscopic ablation
TAH-BSO with lysis of adhesions for severe, recurrent dz
Ectopic pregnancy locations and risk factors
locations
ampulla of oviduct 95%
RF
Hx of PID (most common)
prior ectopic
tubal/pelvic Sx
DES exposure in utero
IUD
incidence ~1/100
Ectopic pregnancy S/S
classic triad
1. amenorrhea
2. light vaginal bleeding
3. lower abd/pelvic pain
tender palpable pelvic/adnexal mass
ruptured ectopic S/S
sudden, sharp abd pain with
- orthostatic hypotension
- ↑HR
- generalized abd & adnexal tenderness with rebound
- shoulder pain
- shock
DDx of ectopic pregnancy
spontaneous abortion
ruptured corpus luteum cyst
PID
adnexal torsion
appendicitis
pyelo
pancreatitis
diverticulitis
regional ileitis
UC
Evaluation of ectopic pregnancy
βhCG quantity(levels <expected) and doubling time (>expected)
serum progestrone <<< normal (<15ng/ml) - nonspecific
U/S
-transabdominal if βhCG ≥5000mIU/ml
- transvaginal if ≥1500
↑βhCG without intrauterine pregnancy on U/S highly suspicious
definitive dx - lap or U/S visualization
Rx of ectopic
follow closely with serial βhCG & U/S
- expectant for asymp with ↓ing βhCG, fallopian tube pregnancy, no U/S evidency of intra-abd bleeding & <3.5cm ectopic mass
- MTX for early stable, unruptured ectopics
- stabilize & Sx with salpingostomy/ectomy or salpingo-oophorectomy
- Rhogam as appropriate
Vaginitis S/S
increased vaginal d/c
vulvovaginal pruritis
+/- vulvar edema/erythema
Evaluation of vaginitis
vaginal pH with nitrazine paper
micro exam with wet prep & KOH
gram stain
chlamydia Ag tests
r/o UTI with U/A, cultures
Bacterial vaginosis frequency, S/S, Rx
relative frequency 50%
main symp = stale odour
mild vulvar irritation
homogenous, greyish-white d/c
with fishy/stale odour
vaginal pH >4.5
clue cells on saline smear
+ve whiff test with KOH prep
Rx - po metronidazole
trichomonas vaginosis frequency, S/S, Rx
25% (may coexist with bact)
main symp = d/c
strawberry petechiae in upper vagina/cervix
profuse, malodourous, yellow-greenish frothy d/c
pH >4.5
motile trichomonas on saline smear
-ve KOH prep
Rx po metronidazole
treat partner (STD)
check for other STDs
Candidal vaginosis frequency, S/S, Rx
25%
main symp = pruritis
erythematous, excoriated vulva/vagina
thick, white, cottage-cheese d/c
normal pH (3.5-4.5)
-ve saline smear
pseudohyphae on KOH prep
Rx topical antifungals (miconazole) or po fluconazole
Cervicitis S/S
N. gonorrhea, C. trachomatis
yellowish-green, mucopurulent d/c
coinfection common
- 50% pts with gono have chlamydia
CMT in absence of other S/S of PID
PID
includes endometritis, salpingitis, oophoritis, myometritis, peritonitis
ususally bacterial
PID Risk factors
young age at 1st intercourse
multiple partners
unprotected sex
frequent intercourse
mucopurulent cervicitis
prior PID
IUD use
PID S/S
1-3d hx lower abd pain +/- fever, N/V
hx recent menses
purulent cervical/vaginal d/c
u or b/l lower abd, uterine, adnexal and CMT
DDx PID
ectopic
endometriosis
ovarian tumors
hemorrhagic cysts
adnexal torsion
UTI/pyelo
appendicitis/diverticulitis/
regional ileitis/UC
Evaluation of PID
minimal dx criteria =
lower abd, adnexal & CMT
supportive
fever >38
↑ESR, CRP
WBC >10000
+ve cervical swab (gono/chl)
pelvic abscess on U/S
Definitive dx = lap with pus in peritoneal cavity
Rx of PID
inpt IV abx = cefoxitin or cefotetan
+ doxycycline or clindamycin + gentamicin until asymp
then doxycycline po x14d
Outpt abx =
ceftriaxone
+ doxy or ofloxacin
+ clinda or metro x14d
Hospitalize for:
pelvic/tubo-ovarian abscess
peritonitis
non-compliance
N/V preventing po meds
high fever/WBCs
no improvement after 48-72h
teenage/nulliparous
Cx of PID
TOA - suspect with severe pain, high fever, N/V, adnexal mass
Ectopic pregnancy
infertility
Fitz-hugh-curtis syndrome (5-10% of PID)
Toxic Shock syndrome S/S, Rx
pre-formed TSST-1 toxin sudden onset fever, diffuse sunburn-like rash, diarrhea, V, pharyngitis, h/a +/or myalgias
can rapidly progress to hypotensive shock
desquamation palms/soles in 1-2wks
menses/tampon use associated in 50%
Rx - antistaph abx (nafcillin, oxacillin) +/-steroids
vulvar cancer
4th most common gyne ca
after menopause (60s)
squamous cell (90%)
RF DM, obesity, HTN, vulvar dystrophies, HPV 16/18
vulvar pruritis (most common)
erythematous/ulcerated vulvar lesion +/or mass
Rx wide local excision + LN dissection +/- XRT
Cervical ca
3rd most common gyne ca
RF - early intercourse, multiple partners, immunocompromised, tobacco, STDs, HPV 16/18/31
cervical ca S/S
meno +/or metrohhagia
post-coital bleeding
pelvic pain
vaginal d/c
cervical d/c +/or ulceration
pelvic mass
fistulas
Indications for colposcopy and endocervical curettage
dysplasia
squamous intraepithelial neoplasia
2 consecutive ASCUS
Rx of cervical ca
CIN I = LSIL - most regress spontaneously - Pap + colposcopy q3m
CIN II/III = HSIL - cryosx, laser, or LEEP
Cold knife conization - only for lesions that can't be fully visualized, discrepancy b/t high gr. cytology and bx, adenocarcinoma in situ, +ve ECC, or microinvasive SCC
Invasive ca
early - radical hysterectomy + LN dissection
all stages - XRT, chemo
uterine leiomyoma
most common benign gyne lesion
more common in blacks, >35y/o
hormonally responsive
increase size with pregnancy
regress after menopause
0.1-0.5% transformation to leiomyosarcoma
fibroids S/S
asymp
abnormal uterine bleeding
anemia
pelvic pressure
dysmenorrhea
urinary frequency
pain
infertility (uncommon)
firm, nontender, irregularly inlarged "lumpy-bumpy" uterus
Evaluation/Rx of fibroids
U/S to confirm dx
expectant
hysterectomy/myomectomy
medical - medroxyprogestrone
danazol
GnRH agonists (shrink tumors but growth resumes when meds stopped) can use in perimenopausal
If grows after menopause CANNOT BE FIBROID
endometrial ca RF
most common gyne ca
strongly associated with high levels unopposed estrogen -
HRT
chronic anovulation
early menarche
late menopause
ovarian granulosa cell tumors
obesity
tamoxifen
DM, HTN, nulliparity, FHx
endometrial ca S/S
postmenopausal bleeding
menorrhagia
metrorrhagia
lower abd pain and cramping
uterus fixed and immobile if spread to adnexa/peritoneum
Signs of mets
hepatosplenomegaly
general lymphadenopathy
abdominal masses
evaluation and Rx of endometrial ca
Pap smear (not sensitive)
U/S to r/o fibroids, polyps, endometrial hyperplasia
ECC and EMB - glandular cell hyper/anaplasia with invasion of stroma, myometrium or bvs
Surgical staging
+/- adjuvant XRT
hormone Rx - high dose progestins for stage I
Chemo (doxorubicin + cisplatin) for advanced/recurrent dz
ovarian ca RF
leading cause gyne ca deaths
most common in post-menopausal and prepubescent
RF
FHx breast, ovarian ca
chronic uninterrupted ovulation
protective - OCPs
Catagorization of ovarian ca
epithelial cell (most common)
- >20y/o
germ cell eg dysgerminoma
sex cord-stromal eg functional tumors
ovarian ca S/S
asymp until late
abdominal pain
bloating
pelvic pressure
urinary frequency
early satiety
constipation
vaginal bleeding
systemic symp
palpable solid, fixed, nodular pelvic mass
ascites
pleural effusions
Evaluation and Rx of ovarian ca
pelvic U/S +/- CT/MRI
CA-125, AFP, LDH, hCG used to monitor progression (not screening)
Surgical staging includes:
TAH-BSO, omentectomy, tumor debulking
XRT for dysgerminomas
Post-Sx chemo (carboplatin + paclitaxel)for epithelial cell (high recurrence, poor px)
prevention of ovarian ca
strong fhx (≥2 1° relatives)
annual screening with CA-125 + transvaginal U/S
consider prophylactic oophorectomy after childbearing
OCPs may help decrease risk
Causes of hirsuitism and virilization
PCOS
congenital adrenal hyperplasia
cushing's syndrome
sertoli-leydig cell ovarian tumors
luteoma of pregnancy
adrenal tumors
PCOS
oligomenorrhea with S/S of increased circulating androgens
characterized by:
b/l polycystic ovaries
chronic anovulation
infertility
affects 15-30y/o
PCOS S/S
obesity
amenorrhea
hirsuitism
infertility
symptoms d/t excess LH and androgen overproduction
associated with DM, insulin resistance
increased risk endometrial ca
Evaluation and Rx of PCOS
serum FSH:LH >3
↑serum androstenedione, DHEA
U/s - enlarged ovaries with +++ large subcapsular cysts
Rx
wt loss
infertility - clomiphene citrate or metformin
OCPs for hirsuitism, amenorrhea, acne
female infertility most common causes
endometriosis > PID
Threatened abortion
minimal bleeding
+/- abdominal pain
no POC expelled
closed os
normal U/S
Rx
avoid heavy activity, pelvic and bedrest
inevitable abortion
profuse bleeding
severe cramping
open os
Rx
emergent D&C
incomplete abortion
some POC expelled
open os
retained fetal tissue on U/S
Rx
emergent D&C
missed abortion
no uterine bleeding
no POC expelled
closed os
no FHR
retained fetal tissue on U/S
Rx
evacuate uterus
D&C
complete abortion
minimal bleeding/cramping
all POC expelled
closed os
empty uterus on U/S
septic abortion
fever/chills
peritoneal signs
often hx recent TA (maternal mortality 10-50%)
↓BP, T
oliguria
resp distress if in shock
↑WBC
Rx
evacuate uterus
D&C
IV Abx
intrauterine fetal demise
mother may report absence of fetal mvts
uterus SGA
no FHR/mvts on U/S
Rx
induce labour and evacuate uterus to avoid D&C
Stress incontinence S/S
small amts of urine loss with exertion/straining
no symp supine/asleep
+/- cystocele/urethrocele
stress incontinence mechanism
mech - change in urethrovesical angle causes intra-abd pressure increases to be transmitted to bladder>urethra
stress incontinence RF
pelvic relaxation
chronically increased intra-abd pressure
weakened urethral closing mechanisms (eg estrogen deficiency, meds)
stress incontinence dx and rx
dx
normal U/A, culture, cystometrogram
demonstrable leakage w stress
"+ve Q-tip test" (angle of cotton swab shows >30° change when pt strains)
Rx
kegels
estrogen
pessaries +/or Sx
urge incontinence S/S
unpredictable large V urine loss
day and night urgency/frequency
exam often normal
urge incontinence mechanism
involuntary, uninhibited detrusor muscle contractions
urge incontinence risk factors
UTIs
bladder stones
bladder ca
neurologic dz
DM
Dx and Rx of urge incontinence
cystometrogram reveals involuntary bladder contractions associated with leakage, normal residual volume and sensation
Rx
anticholingerics (oxybutynin)
βadrenergics (metaproterenol)
behaviour modification
+/or sx denervation
Down syndrome
most common chrom disorder and cause of congenital MR
MR
flat facial profile
prominent epicanthal folds
simian crease
duodenal atresia
congenital hrt dz (most common septum-primum ASD d/t endocardial cusion defects)
alzeiher's >35y/o
↑risk ALL
Edward's syndrome
trisomy 18 (1/8000)
severe MR
rocker-bottom feet
low set ears
micrognathia
congenital hrt dz
clenched hands
prominent occiput
death by 1y/o
Patau's syndrome
trisomy 13 1/6000
severe MR
microphthalmia
microcephaly
cleft lip/palate
abnormal forebrain structures
polydactyly
congenital hrt dz
death by 1y/o
Klinefelter's syndrome
XXY 1/850
testicular atrophy
eunuchoid body shape
tall, long extremities
gynecomastia
female hair distribution
one of most common causes of hypogonadism in males
Turner's syndrome
XO 1/3000
short stature
webbed neck
ovarian dysgenesis
coarctation of aorta
most common cause primary amenorrhea
Double Y males
XYY 1/1000
phenotypically normal
very tall
severe acne
antisocial behaviour in 1-2%
phenylketonuria
phenylalanine normally converted to tyrosine
in PKU tyrosine becomes essential
↓phenylalanine hydroxlase or tetrahydrobiopterin cofactor
findings
MR
fair skin
eczema
musty body odour
Fabry's disease
deficient α-galactosidase A → +++ ceramide trihexoside
findings
renal failure
X-linked recessive
Krabbe's disease
AR
absent galactosylceramide β-galactosidase → +++ galactocerebroside
findings
optic atrophy
spasticity
early death
Gaucher's disease
AR
↓β-glucocerebrosidase → glucocerebroside +++ in brain, liver, spleen, and bone marrow (gaucher cells with "crinkled paper" enlarged cytoplasm)
Type I - most common
normal life span
Niemann-Pick disease
AR
↓sphingomyelinase → +++ sphingomyelin and cholesterol in reticuoloendothelial and parenchymal cells
die by 3y/o
Tay-Sachs
AR
absence of hexosaminidase A → +++ ganglioside
death by 3y/o
cherry-red spot on macula
1/30 Askanasi Jews (1/300 others)
Metachromatic leukodystrophy
AR
↓ arylsuflatase A → +++ sulfatide in brain, kidney, liver, peripheral nerves
Hurler's syndrome
AR
↓α-L-iduronidase → corneal clouding, MR
Hunter's syndrome
X-linked recessive
↓iduronate sulfatase
mild form of Hurler's with no corneal clouding and mild MR
X-linked lysosomal storage diseases (2)
Fabry's
Hunter's
AR lysosomal storage diseases (6)
Niemann-Picks
Krabbe's
Gaucher's
Hurler's
Tay-Sachs
metachromic leukodystrophy
Fragile X syndrome
X linked recessive
affects methylation and expression of FMR1 gene
2nd most common cause genetic MR
CGG repeats
macro-orchidism
long face with large jaw
large everted ears
autism
Left to right shunts
"3d's"
ASD
VSD
PDA
Congenital heart disease classification and intrauterine risk factors
presence or absence of cyanosis
RF
maternal EtOH, drugs
exogenous hormones (OCPs)
lithium
congenital infection
ASD S/S
presents late childhood/early adulthood
large defects can → CHF → cyanosis
easy fatiguability
frequent resp infections
FTT
RV heave
wide and fixed S2
SEM at ULSB (from ↑flow across pulmonary valve)
ASD evaluation
Echo with colour flow doppler (dx)
flow b/t atria
paradoxic ventricular wall motion
dilated RV
ECG - R axis deviation
CXR - cardiomegaly, increased pulm. vascular markings
ASD Rx
small defects - Ø
Abx prophylaxis before dental procedures for ostium primum defects
Sx closure in infants with CHF, >2:1 pulm:systemic blood flow ratio
Early correction prevnts Cx
- arrhythmias, RV dysfunction, Eisenmenger's syndrome
Eisenmenger's syndrome
L→R shunt → pulmonary vascular hyperplasia, irreversible PHTN and shunt reversal
VSD S/S
most common comgenital heart defect
frequent resp infections
FTT
CHF
pansystolic murmur at LLSB
loud pulmonic S2
Severe defects - systolic thrill, cardiomegaly, crackles
VSD evaluation and Rx
echo dx
ECG - L/RVH
Rx
CHF with diuretics, ionotropes
small usually Ø
large - early Sx to prevent Cx - Eisenmenger's syndrome
endocarditis and septic emboli prophylaxis (amoxicillin) before dental/pulmonary procedures
PDA risk factors and S/S
RF
high altitude
maternal T1 rubella
more common in premature & ♀
S/S
slowed growth
recurrent LRTIs
L/E clubbing
symp of CHF
wide pulse pressure
continuous "machinery M" at 2nd LICS
loud S2
bounding peripheral pulses
Evaluation and Rx of PDA
colour flow doppler showing blood flow from aorta to PA = Dx
echo - LA, LV enlargement
ECG +/- LVH
CXR +/- cardiomegaly
Rx
indomethacin unless needed for survival
if fails or if >6-8m old - Sx closure
Coarctation of aorta RF and S/S
♂>♀
Turner's syndrome is RF
25% have bicuspid AV
S/S
asymp HTN in childhood
SOBOE
syncope
claudication
epistaxis
h/a
SBP > in U/E, R arm
femoral pulses weak/delayed
late systolic M in L axilla
forceful apical impulse
L/E wasting if advanced
Evaluation of coarctation of aorta
cardiac cath = Dx
ECG - LVH
echo + colour flow doppler
CXR
- "reverse 3 sign" (d/t pre and post-dilatation of coarct segment)
- "rib notching" (d/t collateral circulation thru intercostal a's
Rx of coarctation
Sx correction or balloon angioplasty
continue abx prophylaxis after Rx
Right to Left shunts
"5T's"
Tetrology
Transposition
Truncus arteriosis
Tricuspid atresia
Total anomalous pulmonary venous return
Transposition of the Great Vessels
pulm and syst circulation are in parallel
- aorta connected to RV
- PA connected to LV
incompatible with life unless PDA or septal defect
RF
Apert's syndrome
Down's
Cri-du-chat
trisomies 13, 18
Transposition of GV S/S
critical illness and cyanosis typically immediately after birth
tachpnea
progressive resp failure
Evaluation and Rx of Transposition of GV
eval
Echo
CXR - "egg-shaped silhouette" (narrow heart base and absence of main PA segment)
Rx
keep PDA open with PGE1
Ballon atrial septostomy
Sx - arterial or atrial switch
Tetrology of Fallot
"PROVe"
Pulmonary stenosis
(=RV outflow obstruction)
RVH
Overriding aorta
VSD
Tetrology of Fallot S/S
most common cyanotic hrt dz of childhood (transposition is most common of infancy)
early cyanosis from R-to-L shunting
cyanosis, dyspnea, fatiguability
squat for relief during hypoxic episodes (=tet spells)
hypoxemia - FTT, MS changes
SEM at LSB (d/t RV outflow obstruction)
RV lift
single S2
+/- signs of CHF
RF and Evaluation of tetrology of fallot
Down's
cri-du-chat
trisomies 13, 18
Eval
echo and cath
CXR - "boot-shaped heart" with ↓pulm vascular markings
ECG - RAD, RVH
Tetrology of Fallot Rx
PGE1 to maintain PDA
cyanotic spells - O2, propranolol, knee-chest position, fluids, morphine
temporary palliation with artificial shunt (balloon atrial septostomy) before Sx
HBV vaccination
birth, 2m, 6m
DTaP vaccination
2,4,6, 15-18m, 4-6yrs
Hib vaccination
2,4,6,12-15m
IPV vaccination
2,4,6m,4-6yrs
PPV vaccination
2,4,6,12-15m
MMR vaccination
12-15m, 4-6yrs
varicella vaccination
12-15m
HAV vaccination
2yrs in certain regions and high risk groups
influenza vaccination
>6m with asthma, CF, DM, HIV, BPD, SCA, chronic heart dz
Contraindications and precautions for vaccinations
current mod-severe illness
severe allergy to vaccine component or prior dose
encephalopathy within 7d of prior pertussis vaccination
recent admin of Ab-containing blood products for live injected vaccines
Live vaccines in immunocompromised and pregnancy (Exception HIV without immune compromise can have MMR and varicella)
Precautions
progressive neuro disorders
prior rxn to pertussis
Not contraindications
mild illness +/or low gr fever
current abx
prematurity
Milestones at 2m
GM - lifts head/chest prone
FM - tracks past midline
L - alert to sounds, coos
Social/Cog - recognizes parent, social smile
milestones at 4-5m
GM - rolls f-to-b(4m), b-to-f(5m)
FM - grasps rattle
L orients to voice, ah-goo
S/C - enjoys looking around, laughs
milestones at 6m
GM - sits unassisted
FM - transfers objects, raking grasp
L - babbles
S/C - stranger anxiety
milestones at 9-10m
GM - crawls, pulls to stand
FM - 3-finger pincer grasp
L - mama/dada (non-specific)
S/C - waves bye-bye, pat-a-cake
milestones at 12m
GM - cruises (11m), walks alone
FM - 2finger pincer grasp
L - mama/dada (specific)
S/C - imitates actions
milestones at 15m
GM - walks bwds
FM - uses cup
L - 4-6 words
S/C - temper tantrums
milestones at 18m
GM - runs, kicks ball
FM - builds tower 2-4 blocks
L - names common objects
S/C - copies parent in tasks
milestones at 2yrs
GM - walks up/down steps with help, jumps
FM - builds tower of 6 blocks
L - 2 word phrases
S/C - follows 2-step commands, removes clothes
milestones at 3yrs
GM - rides tricycle, climbs stairs alternating (3-4yrs)
FM - copies circle, uses utensils
L - 3 word sentences
S/C - brushes teeth with help, washes/dries hands
milestones at 4yrs
GM - hops
FM - copies cross
L - counts to 10
S/C - cooperative play
Intussusception
one portion of bowel telescopes into adjacent segment usually prox to ileocecal valve
most common cause of bowel obstruction <2yrs
Intussusception Risk factors
Meckel's
intestinal lymphoma (>6y/o)
HSP
parasites
polyps
adenovirus
rotavirus
celiac disease
CF
Intussusception S/S
abrupt onset colicky abd pain
drawing up of legs and V
young infants - pallor and sweating
palpable "sausage-shaped" RUQ mass
advanced signs - "red, currant jelly stools" lethargy, fever
Intussusception eval and Rx
AXR, U/S
air contrast barium enema - dx, often curative
Sx reduction/resection if necessary
Pyloric stenosis
hypertrophy of pyloric sphincter
first born males
1/500 births
pyloric stenosis S/S
nonbilious emesis progresses to projectile emesis after every feeding from 2wks - 4m old
palpable olive-shaped, mobile, nontender epigastic mass
visible peristaltic waves
Pyloric stenosis DDx
DDx - duodenal atresia ("double-bubble on Xray)
malrotation
volvulus
meconium ileus
GERD
gastroenteritis
pylorospasm
overfeeding
Pyloric stenosis eval and Rx
Abd U/S - hypertrophic pylorus - dx
barium studies - "string sign" (narrow pyloric channel) or pyloric beak
Rx acid/base/volume first then Sx - pyloromyotomy
B-cell deficiencies
most common immunodeficiencies (50%)
present after 6m with recurrent sinopulmonary, GI and GU infections with encapsulated organisms (H. flu, S. pneumo, N. menigitides)
T-cell deficiencies
present earlier than B (1-3m old)
opportunistic and low grade fungal, viral, and intracellular bacterial infections
+/- secondary B-cell deficiencies
phagocyte deficiencies
characterized by mucous membrane infections, abscesses, and poor wound healing
catalase +ve (staph aureus) and GN enterics common
Delayed umbilical cord separation may be early sign
Complement deficiencies
characterized by recurrent bacterial infections with encapsulated organisms
Congenital immunodeficiencies
present with:
chronic/recurrent infections
unusual or opportunistic organisms
incomplete Rx response
FTT
Kawasaki disease
"CRASH and burn"
Conjunctivitis
Rash
Adenopathy
Strawberry tongue
Hands and feet (red, swollen, flaky skin)
AND burn
= fever >40°C for ≥5d
Kawasaki disease
multisystem acute vasculitis affecting young kids (80% <5y/o) esp. Asian
Acute phase:
Dx = fever + ≥4 of:
fever >40°C for ≥5d
b/l nonexudative painless conjunctivitis
polymorphous rash (primarily truncal)
cervical lymphadenopathy (often u/l, ≥1 LN ≥1.5cm)
Diffuse mucous membrane erythema (strawberry tongue)
erythema of palms/soles
indurative edema of hands and feet, desquamation of fingertips (late)
Subacute phase:
thrombocytosis
↑ESR
Rx of Kawasaki's
Acute phase
- high dose ASA
- IVIG (to prevent aneurysms)
Untreated pts at risk for coronary artery aneurysms during convalescene
NO steroids - ↑risk aneurysm formation
B cell deficiencies include...?
X-linked (Bruton's) agammaglobulinemia
common variable immunodeficiency
IgA deficiency (most common of all immunodeficiencies)
X-linked (Bruton's) immunodeficiency
profound B-cell deficiency in Boys only
can present <6m old
at risk for life-threatening Pseudomonas infections
Dx - quantitative Ig levels and specific Ab responses
Rx - prophylactic abx, IVIG
Common variable immunodeficiency
Ig levels drop in 2nd-3rd decade of life
↑risk lymphoma, autoimmune dz
IgA deficiency
most common
usually asyp
recurrent infections
T-cell deficiencies include...?
Thymic aplasia (DiGeorge)
Ataxia-telangectasia
DiGeorge syndrome
"CATCH-22"
Congenital heart disease
Abnormal facies
Thymic aplasia
Cleft palate
Hypocalcemia
22q deletion
Presents with tetany in 1st days of life
Dx
- absolute lymphocyte count
- mitogen stim response
- delayed hypersensitivity skin testing
Rx
thymus or BMT
IVIG for Ab deficiency
Ataxia-telangectasia
DNA repair defect
Oculocutaneous telangectasias and progressive CB ataxia
Rx
BMT
IVIG for Ab deficiency
No Rx to limit progression
Combined immunodeficiencies includes...?
SCID
Wiskott-Aldrich syndrome
SCID
severe lack of B and T cells
frequent, severe bacterial infections
chronic candidiasis
opportunistic organisms
Rx
BMT or stem cell transplant
IVIG for Ab deficiency
PCP prophylaxis until BMT
Wiskott-Aldrich syndrome
X-linked
less severe B & T cell dysfunction
eczema
↑IgE, IgA
↓IgM
TCP
Rx
supportive
IVIG and aggressive abx for infection
rarely survive to adulthood
Phagocytic immunodeficiencies include...?
CGD
Chediak-Higashi syndrome
CGD
Xlinked or autosomal recessive
deficient superoxide production by PMNs, macrophages
Chronic pulm, GI, GU infections
osteomyelitis
hepatitis
+/- anemia, lymphadenopathy, hypergammaglobulinemia
Dx
Nitroblue tetrazolium test=Dx
absolute neutrophil count
adehsion, chemotaxic, phagocytic, bactericidal assays
Rx
TMP-SMX qd
γ-interferon reduces incidence of serious infections
Chediak-Higashi syndrome
AR
defect in neutrophil chemotaxis
Oculocutaneous albinism
neuropathy
neutropenia
Dx
absolute neutrophil count
adhesion, chemotactic, phagocytic, and bactericidal assays
Rx
TMP-SMX qd
Complement deficiencies includes...?
C1 esterase deficiency (hereditary angioneurotic edema)
Terminal complement deficiency (C5-9)
C1 esterase deficiency
= hereditary angioneurotic edema
AD
recurrent episodes of angioedema lasting 2-72hrs - provoked by trauma/stress
can result in life-threatening a/w edema
Dx
total hemolytic complement (CH50)
Rx
danazol qd
purified C1 esterase and FFP prior to Sx
Terminal complement deficiency
susceptible to recurrent meningococcal & gonococcal infections
SLE, glomerulonephritis (rare)
Dx
total hemolytic complement (CH50)
Rx
menigococcal vaccine
Abx