NBME 10 Flashcards
puberty in girls
thelarche: breast development (increased produciton of estrogen and growth of mammary tissue, asymmetry is normal)
pubarche: pubic hair
growth spurt
menarche: menses (estradiol stimulation of the endometrial lining rather than ovulation and can be accompanied by prolonged vaginal spotting)
follow up in 6 months*
prolong bleeding after circumcision, eczema, thrombocytopenia (low platelets), and recurrent infections of oral thrush, otitis media, pneumococcal bacteremia
increased risk for?
Wiskott-Aldrich Syndrome
WAS gene on X chromosome – codes for actin cytoskeleton rearrangment that occurs during interaction between T lymphocytes, antigen-precenting cells, B lymphocytes
–> impaired innate and adaptive immune system
increased risk: autoimmune disease + malignancy: Leukemia*
Encapuslated bacteria + opportunistic pathogens
recurrent viral, bacterial, and fungal infections starting at birth, failure to thrive, and severe lymphopenia with impaired cellular and humoral immunity
SCID (x linked) and Adenosine deaminase deficiency (AR)
mutation in Interluekin-2 Receptor gamma chain
progressive ataxia, telangiectasias, abnormal eye movemnts, decreased immunoglobulin concentrations (leading to recurrent sinopulmonary infections)
Ataxia telangiectasia (AR) dna repair defect
somatic mutations accumulate –> lead to protein dysfunction and increased risk: Hodgkin Lymphoma
eczema and cutaneous cold abscesses
Hyper-IgE syndrome : defect in the JAK-STAT signaling pathway –> impaired Th17 cel differentiation
thymic aplasia and hypocalcemia (looks like SCID with risk of viral and fungal infections), with cardiac and craniofacial defects
DiGeorge syndrome: 22q11 deletion that leads to failure of 3rd and 4th branchial pouches to develop
recurrent BACTERIAL infection and low/absent immunoglobulin concentrations
X-linked agammaglobulinemia : mutatition in BTK gene encodes for tyrosine kinase: needed for B lymphocyte development/proliferation
next step when starting anti-TNF alpha (infliximab)
Interferon-gamma release assay (IGRA) or PPD to test for latent TB
TNF-alpha needed to fight off granuloma formation
if positive –> treat for LATENT TB with 9 months of isonizaid
sickle cell: vaso-occlusive crisis management
IV fluids and pain control
children: dactylitis, acute chest syndrome; adults diffuse pain
4 units of blood transfusion + IV crystalloids/colloids, now has dypsnea, pulmonary edema, JVD, widened pulse pressure, HTN
Transfusion-Associated Cardiac Overload TACO – fluid overload 2/2 large volume blood transfusions
STOP blood transfusion, minimize IV fluids, then give loop diuretic, O2 or positive pressure ventilation
pt thinks she has small worms underneath skin, has tried digging them out, no hx of depression/ ah. no illicit drug use
delusional parasitosis (delusional disorder)
NMDA receptor antagonist, nystagmus, agitation
next step?
complications to look out for?
PCP intoxicaiton - INHIBITS reuptake of dopamine, norepi, and serotonin
stimulates alpha-adrenergic receptors
give benzo’s for agitation
then labs for rhabdomyolysis, hyperthermia, HTN
intubate if comatose
HSV ENCEPHALITIS
how does it spread?
dx?
tx?
HSV-1 from oropharynx to CNS via trigeminal nerve
or
reactivated latent HSV-1 infection
dx: need lumbar puncture: run PCR to detect HSV1
neuroimaging: unilateral or bilateral temporal lobe
tx: IV acyclovir
STROKE workup next best step
4.5 hours
if less than –> Alteplase thrombolytic tx
if more than –> give aspirin then BP control, statins, and DVT prevention
unilateral facial paralysis with loss of right labial fold and unable to wrinkle forehead
next step?
bell palsy - likely viral-mediated inflammation, or immune reaction 2/2 to herpes or herpes zoster, or lyme disease
facial nerve – decreased taste 2/3 anterior tongue, lacrimation
dx: no further testing
tx: glucorcorticoids, or artificial tears to prevent xerophthalmia or corneal abrasion
AMS + Ophthalmoplegia (+nystagmus) + ataxia s/p gastric bypass
vitamin B-1 thiamine deficiency
can result in damage/atrophy of mamillary bodies manifests as Wernicke encephalopathy
child with unilateral cervical adenopathy with purple hue over mass, excisional biopsy showed multiple granulomas
lymphadenitis by mycobacterium avium - intracellulare M. avium common in immunocompromised children (or normal)
r/o bartonella cat scratch or Francisella tularemia
pt with diabetes + BACK PAIN + fever, malaise, fatigue
next step?
MRI of spine to confirm vertebral osteomyelitis (likely hematogenous spread from diabetic ulcer)
also do blood cultures and bone biopsy
22F with swelling on front of right ankle
next best step?
Osteosarcoma
next best step: MRI of right tibia
MCC bone cancer in under 30yo usually in LONG bones (distal femur, proximal tibia, distal tibia)
familial cases (retinoblastoma or p53 gene), body dysplasia (osteitis deformans paget disease), fibrous dysplasia nad radiation
xray: destruciton of both medullary and cortical bone as well as sunburst periosteal reaction
MRI to evaluate local invasion and staging
tx: surgical resection + chemo
muscle weakness + cramps, abdominal ileus, parasthesia, hyporeflexia
with laxative abuse
hypo-Kalemia
can also cause long QT syndrome or torsades depointe (polymorphic ventricular tachycardia)
xray: no pulmonary edema
S4 heard, no peripheral edema
what is contraindicated
ST elevation: AVF and I & II
means Right sided MI
especially with symptoms of no L sided MI (pulm edema, peripheral edema)
DO NOT GIVE nitroglycerin or opiods (they are preload-lowering meds) used to treat angina
you want to give IV fluids to maintain CO, then send to coronary reperfusion with PCI or with fibrinolytics
Diabetic nephropathy
nonenzymatic glycosylation of GBM(thickening) and efferent arterioles(mesangial expansion)
eosinophilic nodular glomerulosclerosis (kimmelstiel-wilson nodules)
initial increase in GFR with microalbuminuria
causes HTN
nonhealing ulcer medial aspect of leg
bronze discoloration
woody induration
venous insufficiency
retrograde blood flow
venous valves
hemosiderin deposition from extravasated RBCs
chronic edema
pt with HIV CD4+ 650
PPD skin test: 6mm
next step?
PPD skin test > 5mm with HIV –> treatment with LATENT TB now – 9 mo isoniazid
no active symtpoms pulmonary or dissiminated infection
if they are under 200 CD4+, then can have a false negative, retest when above 200 CD4+