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+
Pt with asthma gets sick –> hyperinflated lungs, accessory muslces for respriation
management?
bronchospasm (acute asthma crisis)
reversible obstruction of bronchi 2/2 hyperreactivity and airway inflammation
O2 + SABA + corticosteroids
OSA risk factors
BMI >
Age >
neck circumference >
echo?
loud S2 in second left intercostal space
BMI > 35
AGE > 50
NECK > 40 cm
increased pulmonary artery pressure
pulmonic valve closure – pulmonary HTN loud S2
acute abdominal pain in setting of recent MI, bloody diarrhea
ischemic colitis
bloody diarrhea, microangiopathic hemolyic anemia, thrombocytopenia, renal failure
E coloi 0157:H7 (HUS)
pt with previous stillborn greater than 32 weeks is now pregnant
next step in management
weekly non-stress testing in the third trimester starting at 32 weeks
also serial ultrasaonography throughout gestation and screened for medical conditions
mucopurulent discharge with pruritis
’’ ^^^ “ + erythematous friable cervix and cervical motion tenderness
vaginitis
cervicitis
both can be caused by neisseria gonorrhea and chlamydia trachomatis
complications: PID or tubo ovarian or intra-abdominal abscess
cervical motion tenderness, purulent cervical discharge, uterine and adnexal tenderness –> tubo ovarian or intra-abdominal abscess
PID – can cause tubo-ovarian or intra-abdominal abscess
d/t chlaymdia or gonorrhea
dx: NAAT
chlamydia: obligate intracellular organism
tx: azithromycin or doxycycline for chlamydia
ceftriaxone for gonorrhea
fluctuant swollen cyst - caused by obstruciton of gland outflow
if erythema and tenderness to palpation:
bartholin gland cyst
bartholin gland abscess
pt with hx of pheochromocytoma, now has medullary thyroid carcinoma
what does medullary thryoid carcinoma produce ?
MEND 2A/B
medullary thryoid carcinoma arises from parafollicular C cells and produces Calcitonin
sxms: hypo-calcemia
dx: thryoid US and bx
tx: thyroidectomy and chemoradiation
pt with ulcerative colitis – severe bloody diarrhea, colonoscopy superficial ulcers, biopsy: intracellular inclusion bodies
what next?
CMV HHV-5
colitis, retinitis, esophagitis, encephalitis, pneumonia
give **ganciclovir – inhibits viral DNA polymerase (but doesnt need to phophyrlate thymidine kinase like the other -lovir’s do)
crohns manifestations
enterocutaenous fistulas, UVEITIS,
intestinal obstruction from Strictures
bowel perforatio, intra-abdominal abscess, intra-abdominal fistulas
Dx: imaging Bx
fluoroscopic small bowel follow through: narrowing of intestinal lumen usually at terminal ileum, with stricutres or fistulas
tx: NSAIDS - ANTI INFLAMMATORY FIRST
immunomodulators
glucocorticoids
pancreatic adenocarcinoma: painless obstructive jaundice
pt also has abdominal aneurysm: should they repair
no, pancreatric adenocarcinoma is poor prognosis with death within one year
walking barefoot on beach, or had oysters, shelfish, dock - gets cut
get erythema and hemorrhagic bullae (crepitus) over leg or gets diarreha
then septicemia
what next?
vibrio vulnificus gram negative
worse in pt with LIVER problems: alcoholic cirrhosis, hemochromatosis, liver transplant; or in DM or CKD
wound infection, diarrhea, sepsis
can start as mild cellulitis and turn into necrotizing fascitis with feer, pain, crepitus, hemorrhagic bullae
Dx: wound or blood cultures
Tx: IV abx: minocycline or doxycycline + cefoTax or cefTri
then undergo surgical debridement (in order to get rid of infected tissue, vs hyperbaric oxygen therapy for necrotizing soft tissue like in clostridium)
sepsis, hypovolemia, medication or substance-induced hypotension, cardiogenic shock (MI) ANY SORt of decreased perfusion leads to:
ATN
sepsis
pigmented granular, muddy brown casts
renal tubular epithelial cells – bound in a TAMM-HORSFALL (uromodulin) mucoprotein matrix
BUN:Cr < 20
NO casts but eosinophils in urine
AIN
antibiotics, NSAIDS, PPI, infections or autoimmune disorders: sarcoidosis or SLE
primary: painless chancre
secondary: fever, diffuse maculopapular rash with palms and soles, condylomata lata
tertiary: tabes dosralis, aoritis, gummas
syphillis
tx: PCN
or sensitize if allergic
ooorrr doxycycline
white-gray flat topped lesions
vs
verrucuous papules
condyloma lata – syphillis
condyloma aCUMinata verruous
pt with SLE OR Sjorgen and pregnant, fetus at risk for?
complet A-V block
antibodies: anti-Ro and anti-La cross placenta
what month baby has stranger anxiety
6 mo
peaks from 7-10mo
fever, anemia, thrombocytopenia, neuologic (AMS, confusion), renal failure (Cr elevated), Increased indirect bilirubin and jaundice
Thrombotic Thrombocytopenic Purpura TTP
inhibition: ADAMTS13: metalloprotease that degrades vWF
tx: PLASMAPHARESIS
leads to microangiopathic hemolytic anemia
renal failure from microthrombi (vWF decreased so no platelet adhesion, clumps of RBC’s floating around)
11 year old boy with 3 yr hx of increasing muscle weakness/atrophy of both shoulders and pelvic girdles + areflexia
Juvenile Spinal Muscular Dystrophy
AR mutation in SMN genes at chromosome 5q13: degeneration of motor neurons and proximal muscle weakness, resulting in hypotonia and areflexia
able to walk on their own and then slowly lose function
type 1: hoffman baby dies 2: dubowitz never able to walk 3: welander^^
pterygium
benign growth of conjunctival tissue
d/t UV radiation
tx: topical lubrication and REDUCTION OF UV light exposure – wear sunglasses
OR surgical excision
if recurrent: surgery + antimetabolite mitomycin C
Tinea Capitis
d/t Microporum Gypseum
Trichophyton Tonsurance, Epidermophyton Floccosum, Microsporum Canis
tx: oral antifungal: Griseofulvin or Terbinafine (BOTH hepatoxicity) check LFTs
older man with bloody urine, flank pain, weight loss or fever
with vague wrist pain, xray: lytic lesion of radius with pathologic fracture
Renal Cell Carcinoma
older, male smokers – may show polycythemia or hypercalcemia
paraneoplastic syndrome: Erythropoietin or Parathyroid hormone related peptide
Dx: CT
spreads hematogenously: lung brain bone metastatic diseas