UWorld - All Subjects Flashcards
Your patient presents with irritability, abdominal cramps, anorexia, perioral dermatitis, and neurological signs (hand tremors and globally decreased reflexes). What is the most likely explanation?
inhalant abuse (glue, shoe polish, gasoline, spray paint)
What are the acute symptoms of inhalant use?
neurologic - tremors, disorientation, headaches, slurred speech, hallucinations
gastrointestinal - stomach cramps, nausea
cardiovascular - arrhythmia
respiratory - wheezing, coughing
psychiatric - irritability, mood swings, neurocognitive impairment, grandiosity
What are the symptoms of chronic inhalant use?
weight loss, anorexia, neurocognitive impairment, cerebellar dysfunction, and peripheral neuropathy (decreased reflexes)
What is an adverse effect of treatment with calcineurin inhibitors (tacrolimus, cyclosporine). When are these medications prescribed?
acute calcineurin inhibitor renal toxicity -> HTN and AKI caused by constriction of the afferent and efferent renal arterioles. (This is usually reversible with a decrease in the blood levels of the drugs)
These drugs are prescribed for organ transplant immunosuppression and inhibit lymphocyte-mediated rejection via inhibition of IL-2 production.
What adverse effect is shared by beta-lactam antibiotics as well as NSAIDs? What would you see on diagnostic tests?
both can trigger AKI due to acute interstitial nephritis (AIN). Evidence of inflammation (positive leukocyte esterase and elevated white blood cells with casts) is expected on urine studies.
In what time frame is acute tubular necrosis a concern following renal transplantation? What would you see on urine microscopy?`
most common in the first week following transplantation, usually resulting from preoperative graft ischemia.
You would expect to find muddy brown casts on urine microscopy.
PKU inheritance, deficiency, accumulated product, symptoms, diagnosis
inheritance: autosomal recessive
deficiency: phenylalanine hydroxylase
accumulation: phenylalanine
symptoms: severe intellectual disability, seizures, musty body odor, hypo pigmentation involving skin, hair, eyes, and brain nuclei
diagnosis: newborn screening exam OR quantitative amino acid analysis (if did not receive newborn screening)
Fructose intolerance deficiency, accumulated product, symptoms
deficiency: aldolase B
accumulated product: fructose-1-phosphate
symptoms: vomiting, poor feeding, lethargy; seizures/encephalopathy follow if fructose is not removed from the diet
galactosemia deficiency, symptoms, and time of presentation
deficiency: galactose-1-phosphate uridyl transferase
symptoms: jaundice, hepatomegaly, and failure to thrive after consumption of breast milk or infant formula
time: within the first few days of life
What are the levels of HgA, HgA2, HgF, and HgS in a normal patient?
HgA - 95-98%
HgA2 - ~2/5%
HgF - <1%
HgS - absent
What are the levels of HgA, HgA2, HgF, and HgS in a patient with beta-thalassemia minor?
HgA - decreased
HgA2 - increased
HgF - near normal
HgS - absent
What are the levels of HgA, HgA2, HgF, and HgS in a patient with beta-thalassemia major?
HgA - absent
HgA2 - significantly elevated
HgF - significantly elevated
HgS - absent
What are the levels of HgA, HgA2, HgF, and HgS in a patient with sickle cell trait?
HgA - significantly decreased
HgA2 - near normal
HgF - near normal
HgS - elevated
What are the levels of HgA, HgA2, HgF, and HgS in a patient with sickle cell disease?
HgA - absent
HgA2 - near normal
HgF - significantly elevated
HgS - significantly elevated
When do patients with beta thalassemia major present? What symptoms do they have? What symptoms can develop if this condition remains untreated?
- patients present around 6-12 months of life (due to HbF predominating in the first few months of life)
- present with fatigue and pallor due to microcytic anemia. splenic hemolysis can cause jaundice, dark urine, and splenomegaly.
- if left untreated, patients can develop skeletal abnormalities due to extramedulllary hematopoiesis.
What CXR findings are suggestive of foreign body aspiration?
unilateral lung hyperinflation with mediastinal shift to the unaffected side
BUT a normal radiograph does not rule out FBA because at least 30% of radiographs are normal
What procedure is used to confirm the diagnosis of foreign body aspiration and remove the object?
bronchoscopy
What is a common sequelae of congenital CMV?
sensorineural hearing loss
What are 2 common sequelae of congenital toxoplasmosis?
chorioretinitis and obstructive hydrocephalus
What is a common sequelae of congenital syphilis?
osteoarticular destruction (saber shins, Hutchison teeth, saddle nose)
What are 2 common sequelae of congenital rubella?
cataracts and sensorineural hearing loss (also present with PDA)
What 5 signs/symptoms would you expect in a congenital CMV infection?
- hepatosplenomegaly
- jaundice
- periventricular calcifications
- microcephaly
- thrombocytopenia
What is the treatment for congenital hearing loss?
antiviral therapy (valganciclovir, ganciclovir) - can preserve hearing function and improve neurocognitive development
What is the difference between the brain findings of congenital toxoplasmosis vs congenital CMV?
Toxo - obstructive hydrocephalus as well as parenchymal calcifications
CMV - hydrocephalus ex vacuo (due to parenchymal volume loss) and periventricular calcifications
What are the symptoms of pertussis (including the 2 stages)? What is the treatment and prophylaxis protocol?
symptoms -
first 1-2 weeks (catarrhal stage) - mild cough and rhinorrhea
paroxysmal stage - characteristic whooping cough
treatment - macrolide Abx (azithromycin, clarithromycin)
prophylaxis - macrolide antibiotic is recommended for post exposure prophylaxis for close contacts
What are 3 physical exam findings suggestive of aortic stenosis?
- diminished and delayed carotid pulses (pulses parvus et tardus)
- late-peaking, crescendo-decrescendo systolic murmur
- soft and single S2
(can develop an S4 heart sound due to increased pressure load and concentric left ventricular hypertrophy)
In what patient will an S3 heart sound be heard?
- chronic severe mitral regurgitation
- chronic aortic regurgitation
- heart failure
- occasionally in those with high cardiac output states, such as pregnancy or thyrotoxicosis
In what condition is a loud S1 heart sound typically heard?
mitral stenosis
What is the treatment for herpes zoster?
antiviral therapy: acyclovir, valacyclovir, famciclovir
postherpatic neuralgia: TCAs, pregabalin, gabapentin
etiology, clinical presentation, and management of primary Raynaud phenomenon?
Etiology: no underlying cause
Clinical presentation: usually women < 30 yo, no tissue injury, negative ANA and ESR
Management: avoid aggravating factors, calcium channel blocker for persistent symptoms
Etiology, clinical presentation, and management of secondary Raynaud phenomenon?
Etiology: connective tissue diseases, occlusive vascular conditions, sympathomimetic drugs, vibrating tools, hyperviscosity syndrome, nicotine
Clinical presentation: usually men age >40yo, symptoms of underlying disease, tissue injury or digital ulcers, abnormal nail fold capillary examination
Management: evaluate and treat underlying disorder, calcium channel blocker for persistent symptoms, aspirin for patients at risk of digital ulceration
What is the mechanism and adverse effects (3) of methotrexate?
mechanism: folate antimetabolite adverse effects: 1. hepatotoxicity 2. stomatitis 3. cytopenias
What is the mechanism and adverse effects (2) of leflunomide?
mechanism - pyrimidine synthesis inhibitor
adverse effects -
1. hepatotoxicity
2. cytopenias
What is the mechanism and adverse effect of hydroxychloroquine?
mechanism - TNF and IL-1 suppressor
adverse effect - retinopathy
What is the mechanism and adverse effects (3) of sulfasalazine?
mechanism - TNF and IL-1 suppressor adverse effects - 1. hepatotoxicity 2. stomatitis 3. hemolytic anemia
What are the adverse effects (4) of TNF inhibitors?
- infection
- demyelination
- CHF
- malignancy
What drugs belong to the group TNF inhibitors? (5)
adalimumab, certolizumab, etanercept, golimumab, infliximab
What is the clinical presentation of a lesion in the posterior limb of the internal capsule (lacunar infarct)
- unilateral motor impairment
- NO sensory or cortical deficits
- NO visual field abnormalities
What is the clinical presentation of a middle cerebral artery occlusion?
- C/L somatosensory and motor deficits (face, arm, and leg)
- conjugate eye deviation toward side of infarct
- homonymous hemianopia
- aphasia (dominant hemisphere)
- hemineglect (non dominant hemisphere)
What is the clinical presentation of an anterior cerebral artery occlusion?
- contralateral somatosensory and motor deficit, primarily in lower extremity
- abulia (lack of will or initiative)
- dyspraxia, emotional disturbances, urinary incontinence
What is the clinical presentation of a vertebrobasial system lesion?
- alternate syndromes with contralateral hemiplegia and ipsilateral cranial nerve involvement
- possible ataxia
What are the metabolic causes of peripheral neuropathy?
- diabetes mellitus
- hypothyroidism
- vitamin B12 deficiency
What are the toxic causes of peripheral neuropathy?
- alcohol use
- medications (phenytoin, disulfiram, platinum chemotherapy)
- heavy metals
What are the infectious causes of peripheral neuropathy?
- HIV
2. Lyme disease
What are the hereditary causes of peripheral neuropathy?
- Charcot-Marie-Tooth
2. Porphyria (acute intermittent porphyria)
Early insidious short-term memory loss, language deficits and spatial disorientation, later personality changes - what dementia subtype does this describe?
Alzheimer disease
stepwise decline, early executive dysfunction, cerebral infection and/or deep white matter changes on neuroimaging - what dementia subtype does this describe?
Vascular dementia
Early personality changes, apathy, disinhibition and compulsive behavior - what dementia subtype does this describe? What would you see on imaging?
Frontotemporal demenita
On imaging, you would see frontotemporal atrophy
visual hallucinations, spontaneous Parkinsonism, and fluctuating cognition - what dementia subtype does this describe?
dementia with Lewy bodies
ataxia early in disease, urinary incontinence, dilated ventricles on neuroimaging - what dementia subtype does this describe?
normal pressure hydrocephalus
behavioral changes, rapid progression, myoclonus and/or seizures - what dementia subtype does this describe?
prion disease
What is the epidemiology of Hodgkin lymphoma?
Epidemiology - bimodal peak incidence: age 15-35 and >60; associated with EBV in the immunosuppressed
What are the manifestations of Hodgkin lymphoma?
painless LAD, mediastinal mass, B symptoms (fever, sweating, weight loss), pruritus
How do you diagnose Hodgkin lymphoma?
lymph node biopsy that shows Reed-Sternberg cells on histology
Eye involvement, eye “stuck shut,” discharge, discharge reappearing after wiping, sensation, prodromal symptoms, conjunctival appearance of viral conjunctivitis?
eye involvement - unilateral OR bilateral
eye “stuck shut” - yes
discharge - water, scant, stringy
discharge reappearing after wiping - no
Sensation - burning, gritty
Prodromal symptoms - sometimes
Conjunctival appearance - diffuse injection; follicular or “bumpy”
Eye involvement, eye “stuck shut,” discharge, discharge reappearing after wiping sensation, prodromal symptoms, conjunctival appearance of bacterial conjunctivitis?
Eye involvement - unilateral OR bilateral
Eye “stuck shut” - yes
Discharge - purulent, thick
Discharge reappearing after wiping - yes
Sensation - unremitting discharge
Prodromal symptoms - no
Conjunctival appearance - diffuse injection; nonfollicular
Eye involvement, Eye “stuck shut,” discharge, discharge reappearing after wiping, sensation, prodromal symptoms and conjunctival appearance of allergic conjunctivitis?
Eye involvement - bilateral Eye "stuck shut" - yes Discharge - watery, scant, stringy Discharge reappearing after wiping - no Sensation - itchy Prodromal symptoms - no Conjunctival appearance - diffuse injection, follicular or "bumpy," conjunctival edema (chemosis)
What are red flags when diagnosing conjunctivitis that suggest another etiology?
- decreased visual acuity
- photophobia
- ciliary flush
- foreign body sensation
- corneal opacity/infiltrate
- fixed/distorted pupil
- trouble keeping eye open
- severe headache with nausea
What is the MOA of atomoxetine? What does it treat?
norepinephrine reuptake inhibitor; non stimulant option for ADHD
What is the MOA of guanfacine and clonidine? What are they used to treat?`
alpha-2 adrenergic agonists; non-stimulant options for ADHD (used when there is a poor response to or intolerable side effects from stimulants or coexisting conditions (tic disorders))
When is it appropriate to use atomoxetine over a stimulant medication when treating ADHD?
appropriate for patients with a history of illicit substance use or when there is a strong family preference against stimulant medication
How does methotrexate cause oral ulcers/stomatitis as well as macrocytic anemia?
MOA - inhibition of dihydrofolate reductase, which causes cellular folate depletion
This leads to impaired DNA synthesis, which is most pronounced in rapidly dividing cells. Causes bone marrow suppression -> microcytic anemia
autoimmune disorder in which IgG antibodies form against platelet membrane proteins
immune thrombocytopenia
What is the prognosis for cases of immune thrombocytopenia?
most cases resolve within 3 months; however, some patients continue to have platelets < 100,000 for > 1 year, which is known as chronic ITP
What is the treatment for chronic immune thrombocytopenia?
- examination and blood test for infection (H
IV, Hep C, CMV) and autominne disorders (SLE, thyroid disease)
Then treat as if acute ITP:
- asymptomatic = observation
- combo of glucocorticoids, anti-D immune globulin (if Rh-positive and Coombs-negative) and/or IVIg for bleeding episodes)
What is the treatment for acute immune thrombocytopenia?
observation alone if asymptomatic.
OR (if bleeding and/or platelets < 30,000) combo of glucocorticoids, anti-D immune globulin (if Rh-positive and Coombs-negative) and/or IVIg for bleeding episodes
What is the second ling therapies for immune thrombocytopenia, and when would you use them?
second line therapies = rituximab, thrombopoeitin receptor agonists, or splenectomy
You would consider using these if the patient has bleeding and thrombocytopenia requiring repeated pharmacologic interventions
What are the etiology, clinical findings, and laboratory findings of immune thrombocytopenia?
Etiology: platelet autoantibodies AND preceding viral infection
Clinical findings: petechia, ecchymosis, mucosal bleedings (epistaxis, hematuria)
Lab findings: isolate thrombocytopenia < 100,000, few platelets (size normal to large) on peripheral smear
What is the first step in workup of primary amenorrhea, and what path do you take after?
- evaluate for presence of uterus.
Present -> test FSH
Absent -> Karyotype (46, XY (androgen insensitivity) or 46, XX (mullein agenesis)
What is the next step in workup of a patient with primary amenorrhea, uterus present on exam, and Low FSH?
Test TSH and prolactin
If both are normal -> functional hypothalamic amenorrhea
What is the next step in workup of a patient with primary amenorrhea, uterus present on exam, and Normal FSH?
Dx = imperforate hymen
What is the next step in workup for a patient with primary amenorrhea, uterus present on exam, and High FSH?
Karyotype -> 46, XX = primary ovarian insufficiency
45, XO -> Turner syndrome
What is the diagnosis of a patient with primary amenorrhea, uterus present on exam, Low FSH, high TSH, and high Prolactin?
hypothyroidism
What is the diagnosis of a patient with primary amenorrhea, uterus present on exam, Low FSH, normal TSH, and high prolactin?
prolactinoma
What is the most common risk factor for orbital cellulitis? What bacteria usually cause this?
sinusitis, particularly of the ethmoid or maxillary sinuses; strep species and staph aureus
What are the 3 possible causes of hyperbilirubinemia when it is mainly unconjugated hyperbilirubinemia?
- Overproduction (hemolysis)
- Reduced uptake (drugs, portosystemic shunt)
- Conjugation defect (Gilbert syndrome)
What are the 6 possible causes of direct (conjugated) hyperbilirubinemia with predominantly elevated AST and ALT?
- viral hepatitis
- autoimmune hepatitis
- toxin/drug-related hepatitis
- hemochromatosis
- ischemic hepatitis
- alcoholic hepatitis
What are the 2 possible causes of direct (conjugated) hyperbilirubinemia with normal AST, ALT, and alkaline phosphatase?
- Dubin-Johnson syndrome
2. Rotor syndrome
What are the 6 possible causes of direct (conjugated) hyperbilirubinemia with predominantly elevated alkaline phosphatase? What is the next step in workup?
- cholestasis of pregnancy
- malignancy (pancreases, ampullary)
- cholangiocarcinoma
- primary biliary cirrhosis
- primary sclerosing cholangitis
- choledocholithiasis
Next step in work-up: abdominal imaging (U/S or CT) and antimitochondrial Ab
What are the 3 criteria of the Light criteria, and what does it mean?
- pleural fluid protein/serum ration >0.5
- Pleural fluid lactate dehydrogenase (LDH)/serum LDH ration >0.6
- Pleural fluid >2/3 of the upper limit of normal (ULN) for serum LDH
If positive, pleural effusion is exudative.
What are the laboratory/imaging findings of interstitial lung disease?
- CXR can show reticular or nodular opacities
- high-resolution chest CT usually shows fibrosis, honeycombing, or traction bronchiectasis
- PFTs: normal or increased FEV1/FVC ratio, decreased total lung capacity, decreased reserve volume
- exertion usually causes significant hypoxemia due to V/Q mismatch
(Will have increased alveolar-arterial gradient)
What is the first step in workup of a patient with suspected acromegaly?
IGF-1 level
If normal -> rules out acromegaly
What is the next step in workup of a patient suspected to have acromegaly and has an elevated IGF-1 level?
oral glucose suppression est
If adequate GH suppression -> rules out acromegaly
What is the next step in workup of a patient suspected to have acromegaly, has elevated IGF-1, and inadequate growth hormone suppression on oral glucose suppression test?
MRI of brain
You suspect your patient has acromegaly. They have an elevated IGF-1, inadequate oral glucose suppression test, and a pituitary mass on MRI. What’s next?
evaluate surgical vs medical management
You suspect your patient has acromegaly. They have an elevated IGF-1, inadequate oral glucose suppression test, and a normal pituitary on MRI. What’s next?
evaluate for extra pituitary cause of acromegaly (ectopic growth hormone and growth hormone-releasing factor secreting tumors)
What is the path, Sx, and Tx of scabies?
Path: Sarcoptes scabiei mite infestation, spread by direct person-to-person contact
Sx: extremely pruritic pathognomonic burrows and small, erythematous papules; rash located on interdigital web spaces, flexor wrists, extensor elbows, axillae, umbilicus and genitalia
Tx: topical 5% permethrin OR oral ivermectin
What is the immediate treatment for DKA?
Normal saline and regular insulin infusion
When do you add glucose into DKA treatment? In what form?
5% dextrose when serum glucose is = 200mg/dL
When do you switch from IV to SQ insulin in a patient with DKA? When do you hold IV insulin/Tx all together? How do you switch from IV to SQ?
hold IV insulin if K+ < 3.3 mEq/L)
Switch to SQ from IV insulin for the following:
- able to eat
- glucose < 200mg/DL, anion gap < 12 mEq/L and serum HCO3 >/= 15 mEq/L
Overlap SQ and IV insulin by 1-2 hours.
When do you add potassium to a patient’s DKA treatment?
add IV K+ if serum K+ <5.3 mEq/L; nearly all patients are K+ depleted
When do you consider adding bicarbonate for patients in DKA?
pH = 6.9
When do you consider adding phosphate for patients in DKA?
phosphate < 1.0 mg/dL, cardiac dysfunction, or respiratory depression
Monitor serum calcium frequently
What is the path, Sx, lab findings, and Tx of Fanconi anemia?
Path: inherited DNA repair defect, bone marrow failure
Sx: short stature, hypo/hyperpigmented macule, abnormal thumbs, genitourinary malformations
Lab findings: pancytopenia, positive chromosomal breakage testing
Tx: hematopoietic stem cell transplant
What is the MOA of phenytoin? What does acute toxicity look like? Severe toxicity?
MOA - inhibits voltage-gated sodium channels
Acute toxicity - signs of cerebellar dysfunction - horizontal nystagmus, ataxia, dysmetria, slurred speech, N/V, and hyperreflexia
Severe toxicity: AMS (lethargy, confusion), coma, paradoxical seizures, and death
What is the MOA of heparin-induced thrombocytopenia type 2? (HIT)
immune system forms IgG autoAb that covers platelets and forms complexes (heparin-PF4-HIT Ab) resulting in thrombocytopenia and arterial and venous thrombus
How does type 2 HIT cause arterial and venous thrombus?
HIT Abs activate platelets, resulting in platelet aggregation and the release of procoagulant factors. The risk of thrombus is as high as 50% in untreated HIT
What is the MOA of heparin?
binds antithrombin, which inactivates factor Xa; this prolongs activated partial thromboplastin time
What symptoms result from vertebrobasilar insufficiency?
dizziness, vertigo, ataxia, and visual disturbances
What is the epidemiology, Sx, Lab findings, and Tx of hereditary spherocytosis?
Epidemiology: usually autosomal dominant, Northern European descent
Sx: hemolytic anemia, jaundice, splenomegaly
Lab findings: increased MCHC, negative Coombs test, Spherocytes on peripheral smear, increased osmotic fragility on acidified glycerol lysis test, abnormal eosin-5-maleimide binding test
Tx - folic acid supplementation, blood transfusion, splenectomy
What are the signs/symptoms, dx, and tx of dermatomyositis?
sx: muscle weakness (proximal, symmetrical, UE = LE), skin findings (Gottron’s papules, heliotrope rash), extra muscular findings (interstitial lung disease, dysphagia, myocarditis)
Dx - increased CPK, aldolase, LDH, anti-RNP, anti-Jo-1, anti-Mi2, diagnostic uncertainty - EMG, skin/muscle biopsy
Tx - high dose glucocorticoids PLUS glucocorticoid-sparing agent, screen for malignancy
What would microscopy show with aspergillus infection? What kind of patients get this and what does it cause?
immunocompromised patients; causes pulmonary disease (upper lobe cavitary disease)
microscopy shows acid-fast negative hyphae
What is present on gram stain with nocardia infection? What is the treatment?
presence of filamentous, gram-positive, weakly acid-fast rods
Tx = TMP/SMX
What is the path, Sx, Lab findings, and Tx of Henoch-Schonlein purpura?
Path: IgA-mediated leukocyctocalstick vasculitis
Sx - palpable purpura, arthritis/arthralgia, abdominal pain, intussusception, renal disease similar to IgA nephropathy
Lab findings: normal platelet count and coagulation studies, normal to increased creatinine, hematuria +/- RBC casts +/- proteinuria
Tx - supportive (hydration and NSAIDs) for most patients, hospitalization and systemic glucocorticoids in patients with severe symptoms
What are the clinical symptoms of SLE?
Constitutional: fever, fatigue, and weight loss
Symmetric, migratory arthritis
Skin: butterfly rash + photosensitivity
Serositis: pleurisy, pericarditis, peritonitis
Thromboembolic events (due to vasculitis + antiphospholipid antibodies)
Neurologic: cognitive dysfunction + seizuress
What are the laboratory findings suspected in SLE?
hemolytic anemia, thrombocytopenia + leukopenia
hypocomplementemia (C3+C4)
Antibodies: ANA (sensitive), Anti-dsDNA + anti-Sm (specific)
Renal involvement: proteinuria + elevated creatinine
How does Behcet disease typically present?
multiple oral and genital ulcers that are recurrent and painful (hematologic and renal abnormalities are not typically seen)
What is Felty syndrome? What are some Sx?
Felty syndrome is a potentially serious complication of RA characterized by neutropenia and splenomegaly. Recurrent bacterial infection is common (due to neutropenia) and may lead to LAD.
HIV dementia path, Sx, Tx
path: patients with long-standing HIV disease and CD4+ count <200. Macrophage-mediated signaling pathways and associated toxicity leads to neuronal dysfunction
Sx - increasing apathy and impaired attention, subcortical dysfunction with slowed movement and difficulty with smooth limb movement
Tx - adherence to antiretroviral therapy may improve HAD symptoms
How does cutaneous blastomycosis manifest? Where is it commonly found?
cutaneous blastomycosis manifests as well-circumscribed verrucous nodules and plaques that progress to micro abscesses
Endemic to the central US
What is another name for multiple system atrophy? What are the 3 main symptoms?
Shy-Drager syndrome
Sx:
1. Parkinsonism
2. Autonomic dysfunction (postural hypotension, abnormal sweating, disturbance of bowel or bladder control, abnormal salivation or lacrimation, impotence, gastroparesis, etc)
3. Widespread neurological signs (cerebellar, pyramidal, or lower motor neuron)
What is treatment for multiple system atrophy/Shy-Drager syndrome?
Treatment is aimed at intravascular volume expansion with fludrocortisone, salt supplementation, alpha-adrenergic agonists, and application of constrictive garments to the lower body
What is another name for familial dysautonomia? How is it inherited? How does it present?
Riley-Day syndrome
Autosomal-recessive seen predominantly in children of Ashkenazi Jewish ancestry
Presents at birth with feeding problems and low muscle tone; it is characterized by gross dysfunction of the autonomic nervous system (no tears) with severe orthostatic hypotension (swings from severe HTN to postural hypotension)
Epidemioplogy, Sx, Dx, and Tx of Leprosy?
Epidemiology: mycobacterium leprae, primarily in developing world, spread by respiratory droplets or armadillos
Sx: macular, anesthetic skin lesions with raised borders; nodular, painful nearby nerves with loss of sensory/motor function
Dx: full thickness biopsy of skin lesion; not culturable
Tx: dapsone + rifampin (add clofazimine if severe)
What type of patients should be treated if their PPD skin test shows induration of >/= 5mm?
- HIV-positive patients
- Recent contacts of known TB case
- Nodular or fibrotic changes on CXR consistent with previously healed TB
- Organ transplant recipients and other immunosuppressed patients
What type of patients should be treated if their PPD skin test shows induration of >/= 10mm?
- Recent immigrants (< 5 years) from TB-endemic areas
- Injection drug users
- Residents and employees of high-risk settings (prisons, nursing homes, hospitals, homeless shelters)
- Mycobacteriology lab personnel
- Higher risk for TB reactivation (diabetes, leukemia, ESRD, chronic malabsorption syndromes)
- Children age <4, or those exposed to adults in high-risk categories
At what PPD induration level do all patients need to be treated regardless of health or risk status?
> /= 15mm
When does neonatal abstinence syndrome present? What are the symptoms?
- presents in the first few days of life
Sx: high-pitched cry, sleeping and feeding difficulties, tremors, seizures, autonomic dysfunction (sweating, sneezing), tachypnea, vomiting, diarrhea
(within 48 hours of birth for heroin withdrawal and 48-72 for methadone)
What is the treatment for neonatal abstinence syndrome?
symptomatic care to calm the infant and help them sleep, providing small, frequent feeds, and keeping the infant in a low-stim environment (Pharmacologic treatment, including morphine, methadone, and buprenorphine, should only be used when supportive treatment alone does not control withdrawal symptoms)
What are the symptoms for prenatal exposure to cocaine? what are the possible long-term effects?
Prenatal exposure can result in jitteriness, excessive sucking, and a hyperactive Moro reflex
Long-term effects on behavior, attention level, and intelligence may be seen
primary adrenal insufficiency combined with fevers, pulmonary airspace disease (daily cough) with LAD and prior residence in Southeast Asia; diagnosis?
miliary TB (autoimmune adrenalitis is the most common etiology of primary adrenal insufficiency/Addison disease but disseminated chronic infection can also cause the disorder)
Sx, associated conditions, and Dx of porphyria cutanea tarda?
Sx: blisters, bullae, scarring, hypopigmentation/hyperpigmentation on sun-exposed skin (back of hands, forearms, face); scarring and calcification similar to scleroderma
Associated conditions: Hepatitis C, HIV, excessive alcohol consumption, estrogen use, smoking
Dx: mildly elevated liver enzymes and iron overload; elevated plasma or urine porphyrin levels
What are the symptoms of mixed cryoglobulinemia, and what is its associated condition?
Sx: palpable purpura, arthralgias, and glomerulonephritis
Associated with hepatitis C due to immune complex deposition in small blood vessels, leading to endothelial injury and end-organ damage
Path, Sx, and lab findings of systemic sclerosis?
Path: progressive tissue fibrosis, vascular dysfunction
Sx: systemic: fatigue, weakness
Skin: telangiectasia, sclerodactyly, digital ulcers, calcinosis cutis
Extremeties: arthraglias, contractors, myalgias
GI: esophageal dysmotility, dysphagia, dyspepsia
Vascular: Raynaud phenomenon
Lab findings: Antinuclear antibody, Anti-topoisomerase I (anti-Scl-70) antibody, Anticentromere antibody
What are the complications of systemic sclerosis?
Lung: interstitial lung disease, pulmonary arterial HTN
Kidney: HTN, scleroderma renal crisis (oliguria, thrombocytopenia, microagniopathic hemolytic anemia)
Heart: myocardial fibrosis, pericarditis, pericardial effusion
What are the symptoms of lithium toxicity?
Neurologic - AMS, seizure, fasciculations, tremor
GI - vomiting, diarrhea
When is hemodialysis considered for treatment of lithium toxicity?
Lithium levels >2.5 (Patient with levels > 4 and creatinine >2.0 should generally be prescribed dialysis)
What drugs affect the excretion rate of lithium?
NSAIDs, ACE inhibitors, angiotensin receptor blockers, and dehydration by any cause. (lithium is really excreted)
How does systemic juvenile idiopathic arthritis present?
- Arthritis in >/= 1 joint for >/= 6 weeks (commonly affected joints = hips, knees, and hands)
- Fever for >/= 2 weeks (quotidian pattern in which temp spikes once daily, often in the evening). A pink rash often accompanies the fever.
Hepatosplenomegaly and LAD are also common.
What lab findings are common to find in juvenile idiopathic arthritis?
- leukocytosis
- elevated inflammatory markers
- thrombocytosis
- hyperferritenemia
- anemia of chronic inflammation and iron deficiency
- Rheumatoid factor and antinuclear antibodies are typically negative
What receptors does PCP act on and in what part of the brain? What NT are affected?
- N-methyl-D-aspartate receptors, particularly in the hippocampus and limbic system (causing excitatory and psychotic effects)
- sigma receptor complex (causing psychotic and anticholinergic effects)
- Dopamine, norepinephrine and serotonin receptors (causing adrenergic and dopaminergic effects)
Path and Sx of DiGeorge syndrome
Path: chromosome 22q11.2 deletion -> defective development of pharyngeal pouches
Sx: CATCH:
C: conotruncal cardiac defects (ToF, truncus arteriosus, interrupted aortic arch)
A: abnormal facies
T: thyme hypoplasia/aplasia (T-cell deficiency)
C: craniofacial deformities (cleft palate)
H: hypocalcemia/hypoparathyroidism
What type of acid base disturbance does acute salicylate toxicity cause?
- Leads to respiratory alkalosis by stimulating the respiratory center in the medulla and causing tachypnea
- Then causes an anion gap metabolic acidosis by uncoupling of oxidative phosphorylation in the mitochondria leading to anaerobic metabolism (with resultant low HCO3- from acid buildup)
What will ABG show in salicylate toxicity?
- Low PaCO2 - due to primary respiratory alkalosis (and respiratory compensation for metabolic acidosis)
- Low HCO3- - due to primary metabolic acidosis (and metabolic compensation for respiratory alkalosis)
- Near normal pH - as the 2 primary acid-base disturbances shift the pH in opposite directions
Sx, Dx, Tx and prognosis of transient synovitis
Sx/patient: age 3-8 with preceding viral illness, afebrile, hip pain, limp, able to bear weight
Dx: normal to mildly elevated WBC count, ESR, CRP, small effusions on ultrasound
Tx: conservative, NSAIDs
Prognosis: full recovery in 1-4 weeks, recurrence uncommon
Most common organisms causing osteomyelitis in healthy children? Empiric Abx therapy?
Organism: staph aureus
Abx: low likelihood of MRSA -> Nafcillin/oxacillin OR cefazolin
High likelihood of MRSA -> clindamycin OR vancomycin
Most common organisms causing osteomyelitis in children with sickle cell disease? Empiric Abx therapy?
Organisms: salmonella spp., staph aureus
Abx: Low likelihood of MRSA -> nafcillin/oxacillin OR cefazolin
High likelihood of MRSA -> clindamycin OR vancomycin (same as healthy children)
PLUS third-generation cephalosporin (ceftriaxone, cefotaxime)
What is the MOA of hydroxyurea? When is it indicated? What is the main side effect?
MOA - increase fetal hemoglobin
Indicated - in patients with frequent pain crises, history of acute chest syndrome, and/or severe symptomatic anemia
Side effect: myelosuppression (neutropenia, anemia, thrombocytopenia)
What positioning relieves pain from spinal stenosis? What position makes it worse?
Lumbar flexion (walking uphill, leaning on cane, walking while leaning forward, leaning on shopping cart) relieves the pain from spinal stenosis; Extension of the lumbar spine further narrows the spinal canal and worsens the symptoms
How is spinal stenosis diagnosed? What is the treatment?
Dx - MRI of spine
Tx - conservative with physical therapy and exercise but can require surgical intervention
How does lumbar disk herniation typically present? What position makes the pain worse?
typically causes acute back pain with unilateral radiation down the sciatic nerve to the foot (sciatica); usually follows an inciting event
Lumbar flexion makes the pain worse
How does theophylline toxicity present?
CNS stimulation (HA, insomnia, seizures), GI disturbances (N/V), and cardiac toxicity (arrhythmia)
What concurrent illnesses and drugs can lead to theophylline toxicity? By what pathway is theophylline metabolized?
cirrhosis, cholestasis, respiratory infections with fever, drugs - cimetidine, ciprofloxacin, erythromycin, calrithromycin, verapamil
Theophylline is metabolized by the cytochrome oxidase system in the liver
What are the cardiac, pulmonary, and endocrine side effects of amiodarone?
Cardiac - sinus bradycardia, heart lock; risk of proarrhythmias: QT prolongation & risk of tornado de pointes
Pulmonary - chronic interstitial pneumonitis (cough, fever, dyspnea, pulmonary infiltrates)
Endocrine: hypothyroidism, hyperthyroidism
What are the GI/hepatic, ocular, derm, and neurologic side effects of amiodarone?
GI/hepatic: elevated transaminases, hepatitis
Ocular: corneal microdeposits, optic neuropathy
Derm: blue-gray skin discoloration
Neurologic: peripheral neuropathy
What organism causes erysipelas and how does it manifest?
Organism: strep pyogenes
Sx: superficial dermis & lymphatics; raised, sharply demarcated edges; rapid spread and onset; fever early in course
What organisms cause non purulent cellulitis and how does it manifest?
Organisms: S pyogenes, MSSA
Sx: deep dermis + subcutaneous fat; flat edges with poor demarcation; indolent (over days); localized (fever later in course)
What organisms cause purulent cellulitis and how does it present?
Organisms: MSSA, MRSA
Sx: purulent drainage; folliculitis: infected hair follicle; furuncles: folliculitis -> dermis -> abscess; carbuncle: multiple furuncles
What tumor type causes hypercalcemia of malignancy via PTHrP? What is the mechanism? What lab values would you see?
Tumor type: squamous cell; renal + bladder; breast + ovarian
Mechanism: PTH mimic
Labs: decreased PTH, increased PTHrP
What tumor type causes hypercalcemia of malignancy via bone metastasis? What is the mechanism? What lab values would you see?
Tumor type: breast, multiple myeloma
Mechanism: increased osteolysis
Labs: decreased PTH and PTHrP, decreased Vitamin D
What tumor type causes hypercalcemia of malignancy via 1,25-dihnydroxyvitamin D? What is the mechanism? What lab values would you see?
Tumor type: lymphoma
Mechanism: increased calcium absorption
Labs: decreased PTH, increased Vitamin D
What analgesics are associated with drug-induced pancreatitis?
acetaminophen, NSAIDs, mesalamine, sulfasalazine, opiates
What antibiotics are associated with drug-induced pancreatitis?
isoniazid, tetracyclines, metronidazole, TMP/SMX
What anti epileptics are associated with drug-induced pancreatitis?
valproic acid, carbamazepine
What antihypertensives are associated with drug-induced pancreatitis?
thiazides, furosemide, enalapril, losartan
What antivirals are associated with drug-induced pancreatitis?
lamivudine, didanosine
What immunosuppressants are associated with drug-induced pancreatitis?
azathioprine, mercaptopurine, corticosteroids
fever, back pain, and focal spinal tenderness suggest what diagnosis? What is the next steps in workup?
vertebral osteomyelitis; next get blood cultures, ESR/CRP, and plain spinal X-rays
What do you expect to see on ESR/CRP lab tests and X-rays for vertebral osteomyelitis? What is the next steps in workup?
elevated ESR/CRP but normal X-rays; MRI -> CT-guided needle aspiration/biopsy
Path, associated conditions, Sx, and Tx of Wernicke encephalopathy
Path: thiamine deficiency
Associated conditions: chronic alcoholism, malnutrition, hyperemesis gravidarum
Sx: encephalopathy, oculomotor dysfunction (horizontal nystagmus, bilateral abducens palsy), postural & gait ataxia
Tx: IV thiamine followed by glucose infusion
How does autoimmune hepatitis present?
Sx:
1. Asymptomatic -> identified by abnormal LFTs
2. Symptomatic -> fatigue, anorexia, nausea, jaundice, can progress to fulminant liver failure +/or cirrhosis
Often associated with other autoimmune disorders
What laboratory findings would you expect in a case of autoimmune hepatitis?
- hepatocellular pattern (increased AST + ALT)
- hypergammaglobulinemia
- elevated autoantibodies (anti-smooth muscle, anti-liver/kidney microsomal type 1m antinuclear)
What would you find on histology of autoimmune hepatitis? What is the treatment?
histology: portal + periportal lymphoplasmacytic infiltration
Tx: prednisone +/- azathioprine
What are risk factors for hepatorenal syndrome?
advanced cirrhosis with portal hypertension and edema
What are precipitating factors for hepatorenal syndrome?
- reduced renal perfusion
- GI bleed, vomiting, sepsis, excessive diuretic use, spontaneous bacterial peritonitis
- reduced glomerular pressure + GFR (NSAID use, which constricts afferent arterioles)
How is hepatorenal syndrome diagnosed?
- renal hypoperfusion
- FeNa < 1% (or urine Na < 10 mEq/L)
- Absence of tubular injury
- No RBC, protein, or granular cast sin urine
- No improvement in renal function with fluids
How is hepatorenal syndrome treated?
- address precipitating factors (eg hypovolemia, anemia, infection)
- Splanchnic vasoconstrictors (midodrine, octreotide, norepinephrine)
- Liver transplantation
Sx, Dx, and Tx of disseminated gonococcal infection
Sx: purulent mono arthritis OR triad of tenosynovitis, dermatitis, migratory polyarthralgia
Dx: Detection of Neisseria gonorrhoeae in urine, cervical, or urethral sample; culture of blood, synovial fluid (less sensitive)
Tx: 3rd gen cephalosporin IV AND oral azithromycin
How would you describe the rash of Lyme disease?
annular macule (erythema migrans)
breath sounds, tactile fremitus, percussion, and mediastinal shift with lung consolidation (lobar pneumonia)?
breath sounds: increased
tactile remits: increased
percussion: dullness
mediastinal shift: none
breath sounds, tactile fremitus, percussion, and mediastinal shift with pleural effusion?
breath sounds: decreased or absent
tactile fremitus: decreased
percussion: dullness
mediastinal shift: away from effusion (if large)
breath sounds, tactile fremitus, percussion, and mediastinal shift with pneumothorax?
breath sounds: decreased or absent
tactile fremitus: decreased
percussion: hyperresonance
mediastinal shift: away from tension pneumothorax
breath sounds, tactile fremitus, percussion, and mediastinal shift with atelectasis (mucus plugging)?
breath sounds: decreased or absent
tactile fremitus: decreased
percussion: dullness
mediastinal shift: toward atelectasis (if large)
What drug would you expect to cause sympathetic nervous system stimulation? What symptoms would you see?
cocaine; tachycardia, pupil dilation, diaphoresis, and tremors
What psych symptoms do you expect to see with cocaine use disorder?
anxiety, irritability, mood wings, panic attacks, grandiosity, impaired judgment, and psychotic symptoms (paranoia, hallucinations)
CVP (right-sided preload), PCWP (left-sided preload), cardiac index (LV output), SVR (after load), and SvO2 in hypovolemic shock?
CVP: decreased PCWP: decreased cardiac index: decreased SVR: increased SvO2: decreased
CVP (right-sided preload), PCWP (left-sided preload), cardiac index (LV output), SVR (after load), and SvO2 in cardiogenic shock?
CVP: increased PCWP: increased cardiac index: decreased SVR: increased SvO2: decreased
CVP (right-sided preload), PCWP (left-sided preload), cardiac index (LV output), SVR (after load), and SvO2 in obstructive shock?
CVP: increased PCWP: decreased cardiac index: decreased SVR: increased SvO2: decreased
CVP (right-sided preload), PCWP (left-sided preload), cardiac index (LV output), SVR (after load), and SvO2 in septic shock?
CVP: decreased PCWP: decreased cardiac index: increased SVR: decreased SvO2: increased
What tumor do those with myasthenia graves commonly have?
thymoma
Risk factors, Sx, Tx, and complications of cryptorchidism?
Risk factors: prematurity, small for gestational age, low birth weight, genetic disorders
Sx: empty, hypo plastic, poorly rugged scrotum or hemiscrotum, +/- inguinal fullness
Tx: orchiopexy before age 1 year
Complications: inguinal hernia, testicular torsion, sub fertility, testicular cancer
Path, cause, Sx, and lab findings of aplastic anemia
Path: bone marrow failure due to hematopoietic stem cell deficiency (CD34+)
Causes: autoimmune, infections (parvo, EBV), drugs, radiation/toxins
Sx: fatigue, weakness, bleeding and bruising, recurrent infections
Labs: pancytopenia; biopsy: hypo cellular bone marrow with fat and stroll cells
What drugs can cause aplastic anemia?
carbamazepine, chloramphenicol, sulfonamides
What ECG findings would you see during an episode of syncope in someone with vasovagal syncope?
bradycardia with sinus arrest
Path, Sx, Dx, and Tx of Sturge-Weber syndrome?
Path: mutation in GNAQ gene
Sx: port-wine stain (trigeminal nerve distribution), leptomeningeal capillary-venous malformation, seizures +/- hemiparesis, intellectual disability, visual field defects, glaucoma
Dx: MRI of the brain with contrast
Tx: laser therapy, anti epileptic drugs, intraocular pressure reduction
Sx of tuberous sclerosis?
seizures, intellectual disability, hypopigmented ash-leaf spots, angiofibromas (molar erythematous papule), and shagreen patches (flesh-colored plaque on the back)
What is the difference between Stevens-Johnson syndrome and toxic epidermal necrolysis?
They are a spectrum of the same disease:
< 10% of body surface area = Stevens-Johnson syndrome
10-30% = SJS/TEN overlap
>30% = TEN
What are the clinical Sx of Stevens-Johnson syndrome/toxic epidermal necrolysis?
- 4-8 days after exposure to trigger (2 days after repeat exposure)
- acute influenza-like prodrome
- rapid-onset erythematous macule, vesicles, bullae
- necrosis and sloughing of epidermis
- mucosal involvement
What are common triggers of SJS/TEN?
Drugs 1. Allopurinol 2. Antibiotics (sulfonamides) 3. Anticonvulsants (carbamazepine, lamotrigine, phenytoin) 4. NSAIDs 5. Sulfasalazine Other 1. Mycoplasma pneumoniae 2. Vaccination 3. Graft-vs-host disease
What is the difference between vaccine strain and wild type varicella?
Vaccine strain = <10 lesions, maculopapular and/or vesicular, mildly contagious
Wild type = > 100 lesions, vesicular in successive crops, highly contagious
Both have an incubation period of 1-3 weeks
When do you administer charcoal in acetaminophen intoxication?
single dose >/= 7.5g (pediatric >/= 150mg/kg) and = 4 hours since ingestion
What do you do after administering charcoal during acetaminophen intoxication?
check acetaminophen levels
If acetaminophen levels are above the Rumack-Matthew nomogram line, what is the treatment? What other scenarios would cause you to administer the treatment?
If levels above treatment line in nomogram, >10 micrograms/mL if timing of ingestion is unclear, or any evidence of liver injury:
administer N-acetylcysteine and monitor for liver injury
What are the two congenital causes of primary male hypogonadism?
- Klinefelter syndrome
2. Varicocele
What are the 5 acquired causes of primary male hypogonadism?
- radiation
- infection (mumps)
- trauma
- medications (alkylating agents, glucocorticoids)
- chronic disease
What are the findings expected in primary male hypogonadism?
decreased energy/libido, decreased body hair, gynecomastia, increased LH/FSH, decreased testosterone/sperm count
What is the 1 congenital cause of secondary male hypogonadism?
Kallman syndrome
What are the 2 gonadotropin suppression causes of secondary male hypogonadism?
- hyperprolactinemia
2. glucocorticoids/opiates
What are the 4 gonadotroph cell damage causes of secondary male hypogonadism?
- benign/malignant tumors
- pituitary apoplexy
- infiltration (hemochromatosis),
- systemic disease
What findings are expected with secondary male hypogonadism?
decreased energy/libido, decreased body hair, gynecomastia less likely than primary, decreased or normal LH/FSH, decreased testosterone/sperm count (but not as significantly decreased as primary)
What 3 labs/imaging should be ordered for work-up of secondary male hypogonadism?
prolactin, transferrin, +/- MRI
What type of effusions are rheumatoid effusions? What else is significant about them?
exudative effusions with very low (<50) glucose, and very high LDH ( >700), may also have low pH
What are the 4 probable causes of effusions with very low glucose levels?
RA, empyema, malignancy, TB
What are the clinical findings of HSV encephalitis?
fever, HA, seizure, AMS, +/- focal neurologic findings (hemiparesis, cranial nerve palsies, ataxia)
What are the diagnostic findings of HSV encephalitis (CSF and MRI)
CSF: increased WBCs, increased RBCs, increased protein, normal glucose, HSV DNA on PCR
Brain MRI: temporal lobe hemorrhage/edema
What is the treatment for HSV encephalitis?
IV acyclovir
How does hypothyroidism cause abnormal uterine bleeding?
low thyroxine levels feed back to the hypothalamus and increase the secretion of TRH, simulating the antihero pituitary and increasing TSH and prolactin levels -> hyperprolactinemia inhibits the hypothalamus and anterior pituitary, causing decreased FSH and LH levels -> an ovulation and abnormal uterine bleeding
What are the FSH, TSH, and prolactin levels of women going through menopause?
elevated FSH, normal TSH and prolactin
What is the cause of swelling in babies with Turner syndrome? What is a complication?
congenital lymphedema (lymphatic network dysgenesis) -> accumulation of protein-rich interstitial fluid in the hands, feet, and neck
Severe obstruction of lymphatic vessels can result in cystic hygorma of the neck and fetal hydrops
What is age related sicca syndrome? (ARSS) What is the treatment?
exocrine output from lacrimal and salivary glands declines with age, associated with atrophy, fibrosis, and ductal dilation of the glands; treatment is mostly artificial tears or other ocular lubricants
Level of block, ECG findings, QRS complex, Exercise/atropine effect, vagal maneuvers effect, and risk of complete heart block for mobitz type 1?
level of block: usually AV node
ECG findings: progressive prolonged PR interval leads to a nonconductor P wave (“group beating”)
QRS: narrow
Exercise or atropine: improves type 1 AV block
Vagal maneuvers: worsens type 1 AV block
Risk of complete heart block: low risk
Level of block, ECG findings, QRS complex, Exercise or atropine effect, vagal maneuvers effect, and risk of complete heart block for mobitz type 2?
Level of block: below the level of AV node (bundle of His)
ECG findings: PR interval remains constant with intermittent nonconductor P waves
QRS: narrow or wide
Exercise or atropine: worsens type 2 AV block
Vagal maneuvers: paradoxically improves type 2 AV block
Risk of complete heart block: higher risk, indication for pacemaker
gait unsteadiness, shock sensation in the spine, atrophy/weakness in UE, and increased tone/reflexes in the lower extremities?
spinal cord compression (myelopathy) in the cervical spine (most common in older adults who have cervical spondylosis with spinal canal narrowing due to formation of osteophytes in the lateral vertebral bodies and ossification of the posterior longitudinal ligament)
What is the Lhermitte sign and what 2 conditions can it present in?
Lhermitte sign = electric shock-=like sensation down the spine; presents in spinal cord compression and spinal cord inflammation from MS
periodic blindness when standing up or stooping forward, morning headaches, enlarged blind spots on visual filed testing?
papilledema due to increased ICP
Amaurosis fugax
painless, transient monocular blindness that lasts a few seconds; vascular (embolus to ophthalmic artery) in origin
Path, Pt, Dx, and Tx of X-linged agammaglobulinemia
Path: tyrosine kinase defect that prevents development of mature B cells
Pt: recurrent sinopulmonary and GI infections after age 6 months; absence of lymphoid tissue examination
Dx: decreased Ig and B cells; normal T cell concentration; no response to vaccinations
Tx: Ig replacement therapy, prophylactic Abx if severe
What does adenosine deaminase deficiency cause? Pt?
causes impaired T cell development -> severe combined immunodeficiency (SCID); Pt with severe, recurrent viral, fungal, and bacterial infections and failure to thrive
What are patients with complement deficiencies at risk of?
disseminated bacterial infections, particularly with encapsulated bacteria (Strep pneumo, neisseria)
What disorder is caused by impaired oxidative burst? Pt?
chronic granulomatous disease; Its with recurrent skin and pulmonary infections with catalase-positive organisms (Staph aureus, serratia)
What are the risk factors for stress ulcerations? Where do they commonly form?
shock, sepsis, coagulopathy, mechanical ventilation, traumatic spinal cord/brain injury, burn, and high-dose corticosteroids; commonly form in proximal stomach and duodenum
What is the cause and pt of anterior cord syndrome?
cause: anterior spinal artery injury
Pt:
1. B/L hemiparesis (lateral corticospinal tracts; at the level of the cord injury and below)
2. Diminished B/L pain and temp (lateral spinothalamaic tract; 1-2 levels below the cord injury because LST decussates 1-2 levels before the corresponding level)
3. Intact B/L proprioception, vibratory sensation, and light touch (Dorsal columns; blood is supplied from the posterior spinal arteries)
Path, Pt, and Tx of herpangina (hand, foot, mouth)
Path: coxsackie A virus
Pt: 1-7 yo, late summer/early fall; fever, pharyngitis, gray vesicles/ulcers on oropharynx; tx = supportive
Path, Pt, and Tx of herpetic gingivostomatitis
Path: HSV-1
Pt: 6 months - 5 yo; no seasonality; fever, pharyngitis, erythematous gingiva, clusters of vesicles on anterior oral mucosa/lips
Tx: oral acyclovir
Path, Pt, Dx, and Tx of Wilson Dz
Path: autosomal recessive mutation of ATP7B
Pt: hepatic: acute liver failure, chronic hepatitis, cirrhosis
neurologic: Parkinsonism, gait disturbance, dysarthria
psychiatric: depression, personality changes, psychosis
Dx: decreased ceruloplasmin, increased urinary copper excretion, kayser-fleischer rings on slit-lamp, increased copper content on liver biopsy
Tx: chelators (D-penicillamine, Tridentine), Zinc (interferes with copper absorption)
What is the treatment for an ischemic stroke with presentation within 3-4.5 hours of symptoms onset?
intravenous alteplase (tPA)
What is the treatment for an ischemic stroke with no prior anti-lately therapy? (>4.5 hours after Sx onset)
aspirin
What is the treatment of an ischemic stroke in a patient already on aspirin therapy (>4.5 hours after Sx onset)?
aspirin + dipyridamole OR clopidogrel
What is the treatment of an ischemic stroke with evidence of atrial fibrillation (>4.5 hours after Sx onset)?
long term anticoagulation (warfarin, dabigatran, rivaroxaban)
What is the treatment of an ischemic stroke with larger anterior circulation artery occlusion within 24 hours of symptom onset?
mechanical thrombectomy (regardless if patient received alteplase), then aspirin
What is the treatment of an ischemic stroke with intracranial large-artery atherosclerosis?
aspirin + clopidogrel for 90 days, then aspirin
What are the inclusion criteria for thrombolytics in stroke?
- ischemic stroke with measurable neurodeficits
2. symptom onset <3-4.5 hours before treatment initiation
What are the strict exclusion criteria for thrombolytics in stroke?
- hemorrhage or multi lobar in fact involving >33% of cerebral hemisphere on CT scan
- stroke/head trauma in the past 3 months
- history of intracranial hemorrhage, neoplasm, or vascular malformation
- recent intracranial/spinal surgery
- active bleeding or arterial puncture in the last 7 days at non compressible site
- blood pressure >185/110 mmHg
- platelets < 100,000 or glucose <50mg/dL
- Anticoagulant use with INR >1.7, PT >15 sec, or increased active PTT
What are the relative exclusion criteria for thrombolytic use in stroke?
- minor or rapidly improving neurodeficits
- major surgery/trauma in past 14 days
- MI in the past 3 months
- GU or GI bleeding in the past 21 days
- seizure at stroke onset
- pregnancy
What are clinical clues to neck pain due to a strain?
- antecedent history of neck injury
2. pain/stiffness with neck movement
What are clinical clue to neck pain due to facet osteoarthritis?
- older individuals
- pain/stiffness worse with movement
- relieved with rest
What are clinical clues to neck pain due to radiculopathy?
- pain radiates to shoulder/arm
- dermatomal sensory/motor/reflex findings
- positive spurling test
What are clinical clues to neck pain due to spondylitic myelopathy?
- LE weakness, gait/bowel/bladder dysfunction
2. Lhermitte sign
What are clinical clues to neck pain due to spondyloarthropathy?
- young men
- HLA-B27
- Relieved with exercise
- prolonged morning stiffness
What are clinical clues to neck pain due to spinal metastasis?
- constant pain
- worse at night
- not responsive to position changes
What are clinical clues to neck pain due to vertebral osteomyelitis?
- focal tenderness
- fevers and night sweats
- IVDU, immune compromise, or recent infection
What are the 3 main side effects of clozapine?
- neutropenia/agranulocytosis
- seizures
- myocarditis
What maternal factors can lead to small for gestational age infants?
preeclampsia, malnutrition, placental insufficiency, multiparty, drug use
What fetal factors can lead to SGA infants?
genetic factors, chromosomal abnormalities, congenital infections, or inborn errors of metabolism
What complications are SGA infants at risk of?
hypoxia, perinatal asphyxia, meconium aspiration, hypothermia, hypoglycemia, polycythemia
Path, Pt, Dx, and Tx of malaria?
Path: plasmodium falciparum, P viva, P ovale, or P malariae parasites via bite of Anopheles mosquito
Pt: periodic febrile paroxysms, nonspecific malaise, HA, N/V, abdominal pain, diarrhea, myalgia, pallor, jaundice, petechiae, hepatosplenomegaly
Dx: thin and thick peripheral blood smears
Tx: antimalarial drugs
Name 5 antimalarial drugs
- Atovaquone-proguanil
- doxycycline
- mefloquine
- chloroquine
- hydroxychloroquine
What are complications of malaria for both children and adults?
children: seizure, coma, hypoglycemia, metabolic acidosis
adults: jaundice, acute renal failure, acute pulmonary edema
What is the management of exertional heat stroke?
- rapid cooling: ice water immersion preferred; can consider: high-flow cool water dousing, ice/wet towel rotation, evaporative cooling
- fluid resuscitation
- electrolyte correction
- management of end-organ complications
- no role for antipyretic therapy
What 2 conditions are associated with acanthosis nigricans?
- insulin resistance
2. GI malignancy
What 3 conditions are associated with multiple skin tags?
- insulin resistance
- pregnancy
- Crohn disease (perianal)
What condition is associated with porphyria cutanea tarda, and cutaneous leukocytoclastic vasculitis (palpable purpura) secondary to cryoglobulinemia?
hepatitis C
What condition is associated with dermatitis herpetiformis?
celiac disease
What condition is associated with sudden-onset, severe psoriasis, recurrent herpes zoster, and disseminated molluscum contagiosum?
HIV
What 2 conditions are associated with severe seborrheic dermatitis?
- HIV infection
2. Parkinson disease
What condition is associated with explosive onset multiple, itchy seborrheic keratoses?
GI malignancy
What condition is associated with pyoderma gangrenosum?
inflammatory bowel disease
What should be administered in cases or cardiac toxicity (hypotension, QRS prolongation, or ventricular arrhythmia), why?
sodium bicarbonate; improves systolic BP, narrows the QRS complex, and decreases the incidence of ventricular arrhythmia
Path, Pt, Dx, and Tx of carbon monoxide poisoning?
Path: smoke inhalation, defective heating systems, gas motors operating in poorly ventilated areas
Pt: mild-mod: HA, confusion, malaise, dizziness, nausea
severe: seizure, syncope, coma, MI, arrhythmias
Dx: ABG: carboxyhemoglobin level, ECG +/- cardiac enzymes
Tx: high-flow 100% O2, intubation/hyperbaric O2 if severe
What brain findings can be expected in cases of schizophrenia?
loss of cortical tissue volume with ventricular enlargement + decreased volume of the hippocampus and amygdala
What brain findings can be expected in cases of obsessive-compulsive disorder?
structural abnormalities in the orbitofrontal cortex and basal ganglia
Minimal change disease Path, Pt, Dx, and Tx
Path: T cell-mediated injury to podocytes: most common cause of nephrotic syndrome in children, median age 2-3 yo
Pt: edema, fatigue
Dx: proteinuria, renal biopsy without microscopic changes
Tx: corticosteroids
aspirin-exacerbated respiratory disease Path and pt
Path: pseudo allergic reaction to NSAIDs, occur in patients with comorbid asthma,a, chronic rhino sinusitis with nasal polyposis, or chronic urticaria
Pt: asthmatic symptoms (cough, wheezing, chest tightness), nasal and ocular symptoms (nasal congestion, rhinorrhea, or periorbital edema), and facial flushing within 30 minute to 3 hours after NSAID ingestion)
Vitamin K deficiency risk factors
- inadequate dietary intake
- disorder of fat malabsorption (cystic fibrosis, biliary atresia)
- disorders of intestinal inflammation (celiac disease, inflammatory bowel disease
- decreased production by bacterial flora (frequent Abx use)
Which cofactors is vitamin K necessary for? What lab value will be abnormal?
factors II, VII, IX, and X (2, 7, 9, 10, Vitamin K will help you win) and protein C and S; PT will be prolonged (aPTT will only be prolonged in severe vitamin K deficiency
Pt, ECG, and Tx of sick sinus syndrome
Pt: elderly patients, bradycardia (fatigue, dyspnea, dizziness, syncope), bradycardia-tachycardia syndrome (atrial arrhythmias (a fib), palpitations)
ECG: sinus bradycardia, sinus pauses (delayed P waves), senatorial nodal exit block (dropped P waves)
Tx: pacemaker, +/- rate-control medication (if tachyarrhythmias)
Cryoglobulinemia Type I disease associations, Pt, and complement levels
Dz associations: lymphoproliferative or hematologic (ex: multiple myeloma)
Pt: asymptomatic, hyper viscosity (blurry vision), thrombosis (Raynaud phenomenon), skin (livedo reticularis, purpura)
Complement levels: normal
Cryoglobulinemia Mixed (types II & III) disease associations, Pt, and complement levels
Dz associations: chronic HCV, HIV, SLE
Pt: systemic: fatigue, arthralgias; renal: glomerulonephritis, HTN; pulmonary: dyspnea, pleurisy; skin: palpable purpura, leukoclasitc vasculitis
What disease has anticardiolipin antibodies?
antiphospholipid antibody syndrome
Bell palsy Path, Pt, Tx; how do you tel the difference between Bell palsy and stroke?
Path: peripheral neuropathy involving cranial nerve VII
Pt: acute-onset unilateral facial drooping involving the upper and lower face; may be preceded by a prodrome of auricular pain or dysacusis (distortion of sound); may hav decreased tearing, hyperacusis, and a reduced sense of taste
Tx: oral glucocorticoids
You can tell the difference between Bell palsy and stroke bc Bell palsy involves the upper and lower face while stroke typically spares the upper face.
Presentation and risk factors of chronic venous insufficiency
Pt: leg discomfort, leg pain, leg swelling that is worse in the evening or following prolonged standing; improves with walking or leg elevation; pitting edema, medial malleolus ulcer
Risk factors: advancing age, obesity, family history, pregnancy, sedentary lifestyle, previous LE trauma, and previous LE venous thrombosis
Tuberous sclerosis Path, Pt, Screening
Path: mutation in TSC1 or TSC2; autosomal dominant
Pt: derm: ash-leaf spots, angiofibromas of the malar region, shagreen patches
neuro: CNS lesions (subependymal tumors), epilepsy (infantile spasms), intellectual disability, autism & behavior disorders
CV: rhabdomyomas
Renal: angiomyolipomas
Screening:
Tumor screening: regular skin and eye examinations, serial MRI of the brain and kidney, baseline echo + serial ECG
Baseline EEG
neuropsychology screening
Neurofibromatosis type 1 Path, Pt, f/u
Path: autosomal dominant neurocutaneous disorder
Pt: seizures, intellectual disability, hyper pigmented cafe au lait macules, axillary freckling, and neurofibromas
f/u: optic nerve glioma
Neurofibromatosis type 2 Path, Pt, f/u
Path: autosomal dominant disorder
PT: hearing loss and tinnitus in young adults; vestibular schwannomas
f/u: intracranial meningiomas and cutaneous plaques can occur
What causes mitral valve prolapse? What does MVP cause? What will you find on physical exam when it causes this?
MVP is caused by myoxmatous degeneration of the mitral valve leaflets and chordae; presents with amid-systolic click followed by a mid-to-late systolic murmur
MVP is the most common cause of mitral regurgitation. MR presents with displaced apical impulse, holosystolic murmur, and third heart sound
What are the 3 pulmonary causes of hemoptysis?
- bronchitis
- lung cancer
- bronchiectasis
What are the 2 cardiac causes of hemoptysis?
- mitral stenosis
2. acute pulmonary edema
Multifocal atrial tachycardia causes (3), Pt, Tx
Path: 1. exacerbation of pulmonary disease (COPD)
2. Electrolyte disturbance (hypokalemia)
3. catecholamine surge (sepsis)
Pt: typically asymptomatic; rapid, irregular pulse; ECG> >/=3 p-wave forms + atrial rate >100/min
Tx: correct underlying disturbance; AV nodal blockade (verapamil) if persistent
In severe asthma exacerbation, what indicates the need for endotracheal intubation?
- PaCO2 elevated or normal (suggests inability to meet increased respiratory demands and impending respiratory failure)
- markedly decreased breath sounds
- absent wheezing
- decreased mental status
- marked hypoxia with cyanosis
Chronic granulomatous disease Path, Pt, Dx
Path: majority X-linked recessive Pt: recurrent pulmonary + cutaneous infections with catalase-positive pathogens (S aureus, Serratia, Burkholderia, Aspergillus) Dx: neutrophil function testing: 1. dihydrorhodamine 123 test 2. nitro blue tetrazolium test
How does erythema toxicum neonatorum present? What’s the treatment?
Pt: asymptomatic; scattered, erythematous papule and pustules
Tx: none
How does neonatal HSV present? What is the treatment?
3 Types:
1. vesicular clusters on the skin, eyes, + mucous membranes
2. CNS infection
3. Fulminant, disseminated, multiorgan disease
Tx: acyclovir
How does staphylococcal scalded skin syndrome present? What is the treatment?
Pt: fever, irritability; diffuse erythema -> flaccid, flexural blistering; positive Nikolsky sign
Tx: oxacillin, nafcillin, or vancomycin
Granulomatosis with polyangiitis Pt, Dx, and management
Pt:
Upper resp: sinusitis/otitis, saddle-nose deformity
Lower resp: lung nodules/cavitation
Renal: rapidly progressive glomerulonephritis
Skin: livedo reticularis, non healing ulcers
Dx: ANCA: PR3 (~70%), MPO (~20%)
Biopsy:
Skin (leukocytoclastic vasculitis), kidney (pauci-immune glomerulonephritis), lung (granulomatous vasculitis)
Management: corticosteroids + immunomodulators (MTX, cyclophosphamide)
What would expect on polysomnogram of someone with major depressive disorder?
decreased REM sleep latency, decreased slow-wave sleep, and increased REM sleep duration and density
Pt, Dx, and Tx of viral myocarditis
Pt: young adults (<55 yo), viral prodrome (fever, malaise, myalgias), heart failure (dyspnea, orthopnea, edema), chest pain, sudden cardiac death
Dx: ECG: nonspecific Echo: 4-chamber dilation cardiac MRI: late enhancement of the epicardium biopsy: lymphocytic infiltration, viral DNA or RNA
Tx: medication (diuretics, ACE inhibitor, beta blocker), temporary ventricular assist device if needed, heart transplant if no recovery
What are the symptoms of Kawasaki disease?
Fever for >/= 5 days, cervical lymph node > 1.5 cm, rash, bilateral nonexudative conjunctivitis, mucositis, swelling and/or erythema of palms/soles
Epidemiology, Dx criteria, Tx, and complications of Kawasaki disease
Epidemiology: 90% age <5; increased incidence in East Asian ethnicity
Dx criteria: Fever >/= 5 days plus >/= 4 of the following findings:
- Conjunctivitis: bilateral, nonexudative
- Mucositis: injected/fissured lips or pharynx, “strawberry tongue”
- Cervical LAD: >/= 1 lymph node > 1/5cm in diameter
- Rash: erythematous, polymorphous, generalized; perineal erythema and desquamation; morbilliform (trunk, extremities)
- Erythema + edema of hands/feet
Tx: aspirin + IVIg
Complications: coronary artery aneurysms, myocardial infarction and ischemia
What are the historical clues and key findings of pediatric acute hemiplegia caused by ischemic stroke
Historical clues:
- prothrombotic disorder (ATIII deficiency)
- cardiac disease (PFO)
- vasculopathy (SCD)
Key findings:
- focal infarct on brain imaging
What are the historical clues and key findings for pediatric acute hemiplegia caused by intracranial hemorrhage?
Historical clues:
- recent trauma
- bleeding disorder (hemophilia)
- signs of increased ICP (vomiting, bradycardia)
Key findings:
- hemorrhage on brain imaging
What are the historical clues and key findings for pediatric acute hemiplegia caused by seizure (Todd paralysis)?
Historical clues:
- symptoms preceded by limb jerking or loss of consciousness
- presence of poetical confusion
Key findings:
- symptoms self-resolve
What are the historical clues and key findings for pediatric acute hemiplegia caused by hemiplegic migraine?
Historical clues:
- onset in adolescence
- positive family history
- history of headache with aura
Key findings:
- symptoms self-resolve
Clinical features and Dx of Felty syndrome
Clinical features:
- RA:
- severe erosive joint disease and deformity
- rheumatoid nodules
- vasculitis (mononeuritis multiplex, necrotizing skin lesions) - Neutropenia (ANC < 2000)
- Splenomegaly
Dx: 1. Anti-CCP + RF are positive in >90% of patients
- Markedly elevated ESR, often >85
- Peripheral smear + bone marrow biopsy to rule out other causes of neutropenia
What are the 2 bugs that cause foodborne illness via ingeste3d enterotoxin? What symptoms should clue you in to these causes?
Bugs: staph aureus and Bacillus cereus
Symptoms: quick onset (1-6 hours), vomiting predominant
What are the 3 bugs that cause foodborne illness via enterotoxin made in the intestine? What symptoms should clue you in to these causes?
Bugs: Clostridium perfringens, ETEC/STEC, Vibrio cholerae
Symptoms: delayed onset (>1 day), watery/bloody diarrhea
What are the 3 bugs that cause foodborne illness via bacterial epithelial invasion? What symptoms should clue you in to these causes?
Bugs: Campylobacter jejuni, nontyphoidal salmonella, Listeria monocytogenes
Symptoms: variable onset, watery/bloody diarrhea, fever, systemic illness (listeria)
Substernal chest pain; radiation to arm, shoulder or jaw; precipitated by exertion; relieved by rest or nitroglycerin - what type of chest pain is this?
coronary artery disease
Sharp/stabbing chest pain; worse with inspiration - what cause of chest pain should you think of?
pulmonary/pleuritis (pleurisy, pneumonia, pericarditis, PE)
pericarditis - worse when lying flat
PE, pneumo - respiratory distress, hypoxia
Sudden, severe “tearing” chest pain; radiates to back; elderly men; HTN and risk factors for atherosclerosis - what type of chest pain should you think of?
aortic (dissection, intramural hematoma)
Nonexertional chest pain; relieved by antacids; upper abdominal and substernal; associated with regurgitation, nausea, dysphagia; nocturnal pain - what type of chest pain should you think of?
GI/esophageal
persistent and/or prolonged chest pain; worse with movement or change in position; often follows repetitive activity - what type of chest pain should you think of?
chest wall/MSK
Types and causes of prosthetic valve dysfunction?
- transvalvualr regurgitation (cusp degeneration)
- Paravalvular leak (annular degeneration, infective endocarditis)
- valvular obstruction/stenosis (valve thrombus)
What are the clinical manifestations of prosthetic valve dysfunction?
- new murmur (regurgitant or stenotic)
- Macroangiopathic hemolytic anemia
- Heart failure symptoms, thromboembolism
Paravalvular leak causes
more commonly occurs with mechanical valves
results from dehiscence of the valve from the aortic or mitral annulus, often due to annular degeneration or underlying infective endocarditits
Transvalvular regurgitation causes
more commonly affects bioprosthetic valves
can result from cusp degeneration or occasionally valvular thrombus that impairs valve closure
fever, morning headache and vomiting, and focal neurologic deficit should make you think of what?
brain abscess
Pt, Dx, and Tx of primary hyperaldosteronism
Pt - HTN, metabolic alkalosis, hypokalemia, mild hypernatremia; no significant peripheral edema due to aldosterone escape
Dx -
- elevated plasma aldosterone, low plasma renin
- Plasma aldosterone to plasma renin activity ratio > 20 suggests diagnosis
- Adrenal suppression testing after oral saline load confirms diagnosis
- Abdominal imaging (CT) and adrenal venous sampling to distinguish between unilateral adrenal adenoma and bilateral adrenal hyperplasia
Tx - 1. Unilateral adrenal adenoma - surgery (preferred); aldosterone antagonists (spironolactone, eplerenone) for poor surgical candidates or patients refusing surgery
2. Bilateral adrenal hyperplasia - aldosterone antagonists
What are the connective tissue diseases that can cause pericarditis?
RA, lupus
What is a distinguishing feature of uremic pericarditis and what is the treatment?
serum BUN > 60 mg/dL; tx = dialysis
What are the 2 most common causes of osteomyelitis in neonates?
E. coli and Strep agalactiae
When would you think of Pseudomonas as a cause of osteomyelitis?
osteomyelitis of the foot after a puncture wound of the plantar surface; IV drug abuses are also at risk of pseudomonas osteomyelitis
What type of osteomyelitis are patients with sickle cell anemia most at risk for?
salmonella (encapsulated)
When would you think of osteomyelitis caused by Staph epidermidis?
patients who have indwelling vascular (hemodialysis) catheters
How is a cohort study different than a case control study?
cohort studies focus on a group of people whereas case control studies focus on outcomes to start.
cohort studies can be prospective or retrospective whereas case control is retrospective (positive for disease, negative for disease -> look back at risk factor exposure)
What is epidermolysis bullosa?
group of inherited disorders characterized by epithelial fragility (bullae, erosions, ulcers) triggered by minor trauma. These conditions are caused by mutations of proteins involved in intrapeidermal and dermoepidermal adhesion complexes int he basement membrane zone
look for friction-induced blisters at the palms and soles, thickening of the skin of the feet, and oral blisters with bottle-feeding as an infant
Dx - biopsy of a fresh blister for immunofluorescence microscopy
What lab tests should be ordered when evaluating for HTN? (renal, endocrine, cardiac, other)
Renal: 1. serum electrolytes (Na, K, Ca) 2. serum Cr 3. Urinalysis 4. Urine albumin/creatinine ratio (optional) Endocrine: 1. Fasting glucose or HgA1c 2. Lipid profile 3. TSH Cardiac: 1. ECG 2. Echo (optional) Other: 1. CBC 2. Uric acid (optional)
What are the Rome IV diagnostic criteria?
Diagnostic criteria for irritable bowel syndrome.
Recurrent abdominal pain/discomfort >/= 1 day/week for the past 3 months + >/= 2 of the following:
- related to defecation (improves or worsens)
- change in stool frequency
- change in stool form
What are alarm features of IBS that indicate the need for further testing?
- Older age of onset (>/= 50)
- GI bleeding
- Nocturnal diarrhea
- Worsening pain
- Unintended weight loss
- Iron deficiency anemia
- Elevated C-reactive protein
- Positive fecal lactoferrin or calprotectin
- Family history of early colon cancer or IBD
Diagnostic criteria, workup, and treatment of obesity hypoventilation syndrome
Diagnostic criteria:
- Obesity with BMI >/= 30 kg/m
- Awake daytime hypercapnia (PaCO2 > 45 Hg)
- No alternate cause of hypoventilation
Workup:
- ABG on room air (hypercapnia, normal A-a gradient)
- No intrinsic pulmonary disease on CXR
- Restrictive pattern on PFTs
- Normal TSH
- Polysomnography
Treatment:
- Nocturnal positive-pressure ventilation as first line therapy
- Weight loss (bariatric surgery in select cases)
- Avoidance of sedative medications
- Respiratory stimulants (acetazolamide) as last resort
What is the definition of status epileptics and what is the complication that can arise?
status epilepticus = seizure lasting > 5 minutes or a cluster of seizures with the patient not recovering a normal mental status in between
cortical laminar necrosis is the hallmark of prolonged seizures and can lead to persistent neurologic deficits and recurrent seizures (MRI of brain will show cortical hyperintensity)
Etiology, risk factors, presentation, and complications of popliteal (Baker) cyst
Etiology - extrusion of fluid from knee joint space into semimembranosus/gastrocnemius bursa
Risk factors: trauma (meniscal tear), underlying joint disease (OA, RA)
Presentation: asymptomatic bulge behind knee that diminishes with flexion; OR posterior knee pain, swelling, stiffness
Complications:
- venous compression (leg/ankle swelling)
- Dissection into calf (erythema, edema, positive Homan sing)
- Cyst rupture (acute calf pain, warmth, erythema, ecchymosis)
Hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu syndrome) inheritance and presentation
autosomal dominant
diffuse telangiectasia (ruby-colored papule that blanch with pressure), recurrent epistaxis, and widespread arteriovenous malformations (AVMs)
How can pulmonary arteriovenous malformations (such as in Osler-Weber-Rendu syndrome) present?
AVMs in the lungs can shunt blood from the high tot the left side of the heart, causing chronic hypoxemia, digital clubbing, and a reactive polycythemia. Pulmonary AVMs can also present as massive, sometimes fatal, hemoptysis
What are 5 risk factors for atraumatic splenic rupture?
- hematologic malignancy (leukemia, lymphoma)
- Infection (CMV, EBV, malaria)
- Inflammatory disease (SLE, pancreatitis)
- Splenic congestion (cirrhosis, pregnancy)
- Medications (anticoagulation, G-CSF)
Pt, Dx, and Tx of atraumatic splenic rupture?
Pt - diffuse or LUQ abdominal pain, peritonitis; referred left shoulder pain (Kehr sign); hemodynamic instability
Dx - acute anemia; intraperitoneal free fluid on imaging
Tx - catheter-based angioembolization (stable patients); emergency splenectomy (unstable patients)
What is the most likely causative bug of secondary bacterial pneumonia in younger adults? What is the presentation and tx?
community-associated methicillin-resistant staph aureus (CA-MRSA)
Pt - severe, necrotizing pneumonia that is rapidly progressive and often fatal; high fever, productive cough with hemoptysis, leukopenia, and multilobar cavitary infiltrates
Tx - ICU admission, broad-spectrum empiric Abx, including either vancomycin or linezolid
What are the 3 treatment options for preventing relapse in alcohol use disorder? MOA?
- Acamprosate - glutamate modulator at metabotropic glutamate receptor 5; first line treatment that can be used safely in patients with liver disease
- Naltrexone - mu-opioid receptor antagonist; has been associated with hepatotoxicity so C/I with liver disease or those taking opioid medication
- Disulfiram - second line therapy in highly motivated patients
What are the overlapping features of Marfan syndrome and homocystinuria?
- pectus deformity
- tall stature (increased arm:height ratio, decreased upper:lower segment ratio)
- Arachnodactyly
- Joint hyperlaxity
- Skin hyperelasticity
- Scoliosis
What features regarding Marfan syndrome distinguish it from homocystinuria?
- autosomal dominant
- normal intellect
- aortic root dilation
- upward lens dislocation
What features regarding homocystinuria distinguish it from Marfan syndrome?
- autosomal recessive
- intellectual disability
- thrombosis
- downward lens dislocation
- megaloblastic anemia
- fair complexion
Epidemiology, Pt, Dx, and Tx of craniopharyngioma
Epidemiology: benign, slow growing; derived from remnants of Rathke pouch; bimodal age distribution (5-14 and 50-75)
Pt:
- optic chiasm compression -> bitemporal hemianopsia
- pituitary stalk compression -> endocrinopathies:
a. growth failure in children (decreased TSH or decreased GH)
b. pubertal delay in children or sexual dysfunction in adults (decreased LH + FSH)
c. diabetes insipidus (decreased ADH)
Dx: MRI/CT scan: calcified and/or cystic suprasellar mas
Tx: surgical resection +/- radiation therapy
time course, involved coronary artery, Pt, and echo findings of right ventricular failure following MI
time: acute
involved artery: RCA
pt: hypotension, clear lungs, Kussmaul sign
echo: hypokinetic RV
time course, involved coronary artery, pt, and echo findings of papillary muscle rupture following MI
time: acute or within 3-5 days
artery: RCA
pt: severe pulmonary edema; new holosystolic murmur
echo: severe mitral regurgitation with flail leaflet
time, course, involved artery, pt, and echo findings for inter ventricular septal rupture following MI
time: acute or within 3-5 days
artery: LAD (apical septal) or RCA (basal septal)
pt: chest pain, new holosystolic murmur, biventricular failure, shock
echo: left-to-right ventricular shunt; increased O2 level from RA to RV
timing, artery, pt, and echo findings of free wall rupture following MI
time: within 5 days (50%) to 2 weeks
artery: LAD
pt: chest pain, shock, distant heart sounds
echo: pericardial effusion with tamponade
timing, artery, pt, and echo findings of left ventricular aneurysm following MI
time: up to several months
artery: LAD
pt: subacute heart failure, stable angina
echo: thin and dyskinetic myocardial wall
How does C. difficile infect?
pathology is mediated by the release of exotoxins (toxin A and toxin B)
How do loud noises cause hearing loss? What kind?
loud noises may induce sensorineural hearing loss (SNHL) via both mechanical damage and metabolic overload of cochlear hair cells; affected individuals experience bilateral, high-frequency SNHL
What are 5 risk factors for splenic abscess?
- infection (infective endocarditis) with hematogenous spread
- Hemoglobinopathy (sickle cell disease)
- Immunosuppression (HIV)
- IV drug use
- Trauma
What is the presentation, dx and treatment of splenic abscess? What bugs commonly cause this?
pt:
1. classic triad of fever, leukocytosis, and LUQ pain
2. Left-sided pleuritic chest pain with left pleural effusion commonly seen
3. possible splenomegaly
dx: abdominal CT
tx: combo of broad-spectrum Abx and splenectomy; possible percutaneous drainage in poor surgical candidates
bugs: staph, strep, and salmonella
mitral valve prolapse, fever with systemic manifestations (malaise, weight loss), and splenic abscess - this should all make you think of what underlying diagnosis?
endocarditis
presentation and serology of systemic sclerosis?
pt:
1. Systemic: fatigue, weakness
2. Skin: telangiectasia, sclerodactylyl, digital ulcers, alcinosis cutis
3. Extremities: arthralgias, contractures, myalgias
4. GI: esophageal dysmotility, dysphagia, dyspepsia
5. Vascular: Raynaud phenomenon
serology:
1. antinuclear Ab
2. Anti-topoisomerase I (atni-Scl-70) Ab
3. Anticentromere Ab
What are the complications of systemic sclerosis?
Lung: interstitial lung disease, pulmonary arterial HTN
Kidney: HTN, scleroderma renal crisis (oliguria, thrombocytopenia, MAHA)
Heart: myocardial fibrosis, pericarditis, pericardial effusion
A woman with shiny, thickened skin with multiple telangiectasias likely has what?
systemic sclerosis
What are the risk factors for HIV-associated neurocognitive disorders?
- long-standing HIV disease
- Age >/= 5-0
- CD4 count < 200
What might you see on MRI imaging of HIV-associated neurocognitive disorder?
diffuse brain atrophy, ventricular enlargement and diffuse white matter changes
How can you tell the difference between PML and HAND (HIV-associated neurocognitive disorder on imaging? what about clinically?
HAND has diffuse white matter lesions whereas PML has well-delineated, asymmetric white matter lesions. PML will also show focal cortical involvement, including motor deficits (focal arm/leg weakness or hemiparesis), ataxia, and vision abnormalities
What is a complication of unrepaired VSDs?
Unrepaired, large VSDs can result in permanent pulmonary HTN and Eisenmenger syndrome (cyanosis from right-to-left shunting).
What is the path, pt, dx, and tx of tinea versicolor?
path: Malassezia globosa
pt: hypo pigmented, hyper pigmented, or mildly erythematous lesions (face in children, turn and UE in adolescents + adults), +/- fine scale, +/- pruritus
dx: KOH prep shows hyphae and yeast cells in a “spaghetti + meatballs” pattern
tx: topical ketoconzaole, terbinafine, or selenium sulfide
What are risk factors for acute opioid intoxication?
- substance abuse
- chronic opioid use
- hospitalized patients (esp post-op)
- hepatic or renal insufficiency
What are clinical findings of acute opioid intoxication?
- somnolence, AMS
- pinpoint pupils (mitosis)
- shallow breathing + decreased RR
- bradycardia, hypothermia, decreased bowel sounds
- respiratory acidosis on ABG
How does cancer pain management differ based on mild vs. moderate vs. severe pain?
mild: nonopioids (acetaminophen, NSAIDs)
moderate: weak opioids +/- nonopioids (codeine, hydrocodone, tramadol)
severe: strong short-acting opioids (morphine, hydromorphone) -> calculate total daily dose and convert to long-acting formulation (fentanyl patch, oxycodone) PLUS short-acting opioids for breakthrough pain
What are the common organisms causing bacterial meningitis in those age 2-50? What empiric Abx should be used?
organisms: Streptococcus pneumonia, Neisseria meningitidis
Abx: vancomycin + 3rd-gen cephalosporin
What are the common organisms causing bacterial meningitis in those over age 50? What empiric Abx should be used?
S. pneumoniae, N. meningitidis, Listeria
Abx: vancomycin + ampicillin + 3rd-gen cephalosporin
What are the common organisms causing bacterial meningitis in the immunocompromised? What empiric Abx should be used?
S. pneumoniae, N. meningitidis, Listeria, gram negative rods
Abx: vancomycin + ampicillin + cefepime
What are the common organisms causing bacterial meningitis in those with neurosurgery/penetrating skull trauma? What empiric Abx should be used?
gram negative rods, MRSA, coagulase-negative staph
Abx: vancomycin + cefepime
What will iron, TIBC, and ferritin levels be for iron-deficiency anemia?
- depressed serum iron level
- increased TIBC
- decreased serum ferritin level
What will iron and ferritin levels be in thalassemias?
normal to high serum iron and ferritin levels
What will TIBC and ferritin levels be for anemia of chronic disease?
- below normal TIBC
2. normal or increased serum ferritin level
What will iron and ferritin levels be in sideroblastic anemia?
normal to high serum iron and ferritin levels
What is the most common cause of stridor in infants? How does it present?
laryngomalacia; presents with inspiratory stridor that worsens in supine position in an infant who is otherwise feeding and growing well ƒ
With is the micro, pt, dx, and management of epiglottitis?
Micro: Hib
Pt: distressed (tripod position, sniffing position, stridor), dysphagia, dysphonia, drooling, high fever
Dx: X-ray: “thumb sign” (enlarged epiglottis)
Management: intubation + Abx (ceftriaxone and vancomycin)
What are the characteristics of a simple renal cyst?
- thin, smooth, regular wall
- unilocular
- no septae
- homogenous content
- absence of contrast enhancement on CT/MRI
- usually asymptomatic
- no follow-up needed
What are the characteristics of a malignant cystic mass in the kidney?
- thick, irregular wall
- multilocular
- multiple septal, occasionally thick and calcified
- heterogenous content (both solid + cystic)
- presence of contrast enhancement on CT/MRI
- May cause pain, hematuria, or HTN
- Requires follow-up imaging + urological evaluation for malignancy
What clues indicate vasovagal syncope?
- trigger (eg. emotional stress, prolonged standing)
2. prodrome (eg. nausea, sweating, warmth)
What clues indicate syncope due to carotid hypersensitivity?
tactile stimulus of carotid sinus while standing
What clues indicate syncope due to autonomic dysfunction?
- orthostasis
2. history of predisposing disease (eg. DM, Parkinson)
What clues indicate syncope due to hypovolemia?
- orthostasis
2. history consistent with volume loss
What clues indicate syncope due to LV outflow obstruction?
- syncope with exertion
2. systolic murmur
What clues indicate syncope due to ventricular arrhythmia?
- no warning symptoms
2. history of cardiomyopathy or ischemic HD
What clues indicate syncope due to conduction impairment?
- preceding fatigue or lightheadedness
2. ECG abnormalities (eg. sinus pauses, dropped QRS)
What signs indicate esophagitis caused by Candida albicans in HIV patients? What will biopsy show?
Signs: oral thrush usually present; white plaques throughout esophagus
Biopsy: pseudohyphae
What signs indicate esophagitis caused by HSV in HIV patients? What will biopsy show?
Signs: orolabial lesions usually present; vesicular or ulcerative round/ovoid lesions (“volcano like”)
Biopsy: multinucleate giant cells
What signs indicate esophagitis caused by CMV in HIV patients? What will biopsy show?
Signs: large, linear ulcers in distal esophagus
Biopsy: intranuclear/intracytoplasmic inclusions
What are the etiologies of SIADH?
- CNS disturbance (stroke, hemorrhage, trauma)
- Medications (carbamazepine, SSRIs, NSAIDs)
- Lung disease (pneumonia)
- Ectopic ADH secretion (small cell lung cancer)
- Pain and or nausea
What are the clinical features of SIADH? What is the treatment?
- mild/moderate hyponatremia: nausea, forgetfulness
- severe hyponatremia: seizures, coma
- Euvolemia (eg. moist mucous membranes, no edema, no JVD)
Tx: fluid restriction +/- salt tablets; hypertonic (3%) saline for severe hyponatremia
What are the lab findings of SIADH?
- hyponatremia
- serum osmolality <275 (hypotonic)
- urine osmolality > 100
- urine sodium > 40
Genetics, Sx, and prognosis of friedreich ataxia?
Genetics: autosomal recessive; loss of function, trinucleotide repeat (GAA) in frataxin gene
Sx: neurologic deficits (cerebellar ataxia, dysarthria, loss of vibration and/or position sense, absent DTRs), hypertrophic cardiomyopathy, skeletal deformities (scoliosis), diabetes mellitus
Prognosis: mean survival age 30-40; mortality due to cardiac dysfunction (arrhythmia, CHF)
Etiology, Pt, Labs, and Tx of chronic pancreatitis?
Etiology: alcohol use, CF, ductal obstruction (malignancy, stones), autoimmune
Pt: chronic epigastric pain with intermittent pain-free intervals; malabsorption: steatorrhea and weight loss; diabetes mellitus
Labs: amylase/lipase can be normal + nondiagnostic; CT scan or MRCP can show calcifications, dilated ducts, and enlarged pancreas
Tx: pain management, alcohol + smoking cessation, frequent, small meals, pancreatic enzyme supplements
rapidly progressive dementia, myoclonus, and sharp, triphasic, synchronous discharges on EEG?
Creutzfeldt-Jakob disease
What are causes of PTH-dependent hypercalcemia? What do you expect on labs?
Causes: primary (or tertiary) hyperparathyroidism, familial hypocalciuric hypercalcemia, lithium
Labs: hypercalcemia + high-normal or elevated PTH
What are the causes of PTH-independent hypercalcemia? What do you expect for PTH level? What should you do next in work-up?
Causes: malignancy, Vit D toxicity, granulomatous diseases, drug-induced (thiazides), milk-alkali syndrome, thyrotoxicosis, Vit A toxicity, immobilization
Labs: suppressed PTH
W/u: measure PTHrP, 25-hydroxyvitamin D + 1,25-dihydroxyvitamin D
Path, Pt, Management, and complications of mastoiditis?
Path: infection of the mastoid air cells, complications of acute otitis media, most commonly due to Strep pneumoniae
Pt: fever + otalgia; inflammation of mastoid; protrusion of the auricle; opacification of mastoid air cell son CT scan or MRI
Mangament: IV Abe, drainage of purulent material (tympanovstomy, mastoidectomy)
Complications: extra cranial extension ( subperiosteal abscess, facial nerve palsy, hearing loss, labyrinthitis); intracranial extension (brain abscess, meningitis)
child with microcephaly, small palpebral fissures, mid face hypoplasia, smooth philtrum, and thin vermillion border - what dx?
fetal alcohol syndrome
What might you find on brain imaging of a patient with fetal alcohol syndrome?
a decrease in the volume of the frontal lobe
What are the Sx of acute dystonia? Pharmacotherapy?
Sx: sudden, sustained contraction of the neck, mouth, tongue, + eye muscles
Tx: benztropine, diphenhydramine
What are the Sx of akathisia? Pharmacotherapy?
Sx: subjective restlessness, inability to sit still
Tx: beta blocker (propranolol), benzodiazepine (lorazepam), benztropine
What are the Sx of tardive dyskinesia? Pharmacotherapy?
Sx: gradual onset after prolonged therapy (> 6 months): dyskinesia of the mouth, face, trunk + extremities
Tx: valbenazine, deutrabenazine
What are the Sx, Tx, and prevention of neonatal tetanus?
Sx: difficult feeding, truisms; spasms + hypertonicity: clenched hands, dorsiflexed feet, opisthotonus
Tx: supportive care; Abx + tetanus immune globulin
Prevention: immunization; hygienic delivery + cord care
history, Pt, and labs of methemoglobinemia?
Hx: exposure to oxidizing substances (dapsone, nitrites, local/topical anesthetic)
Pt: cyanosis; pulse ox sat ~85%; dark chocolate-colored blood
Labs: saturation gap (>5% difference between oxygen saturation on pulse ox and ABG), normal PaO2
What is the antidote for methemoglobinemia? MOA? What is the next alternative?
methylene blue; acts as an electron acceptor for NADPH and is reduced to leucomethylene blue, which in turn reduces methemoglobin to hemoglobin
Alt: high-dose vitamin C can be used with methylene blue is unavailable or contraindicated (G6PD def)
What is the treatment for lead poisoning?
dimercaprol
What are the respiratory tract features of primary ciliary dyskinesia? What are the extra pulmonary features?
Resp tract features: chronic sinopulmonary infections, nasal polyps, bronchiectasis, digital clubbing
Extra pulmonary features: situs inversus (50% cases), infertility due to immotile spermatozoa, normal growth
What are the respiratory tract features of cystic fibrosis? What are the extra pulmonary features?
Resp tract features: chronic sinopulmonary infections, nasal polyps, bronchiectasis, digital clubbing
Extra pulmonary features: pancreatic insufficiency, infertility due to absent vas deferens (azoospermia), failure to thrive
What is eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)?
systemic disorder characterized by chronic rhinosinusitis, including nasal polyps, asthma, and prominent eosinophilia.
What age is considered early secondary sexual development in boys vs girls? What is the next step in workup?
girls <8 and boys <9; evaluate bone age
What is the next step in workup in a child with early secondary sexual development with advanced bone age?
Check basal LH -> low basal LH -> do GnRH stimulation test
High basal LH -> central precocious puberty
What is the diagnosis based on possible outcomes of the GnRH stimulation test in the case of a child with early secondary sexual development?
Low LH -> peripheral precocious puberty
High LH -> central precocious puberty
What other characteristics are looked at to determine the diagnosis of a child with early secondary sexual development and normal bone age?
isolated breast development -> premature thelarche
isolated pubic hair development -> premature adrenarche
What is the first line treatment for chronic stable angina? What effect does it have on the heart?
beta blockers -> decrease myocardial contractility and HR
What is the second line treatment of chronic stable angina? What effect does it have on the heart?
nondihydropyridine CCBs -> decrease myocardial contractility and HR
What is added to beta blocker when needed for the treatment of chronic stable angina? What effect does it have on the heart?
dihydropyridine CCBs -> coronary artery vasodilation -> decrease afterload by systemic vasodilation
When are nitrates added for the treatment of chronic stable angina? What effect do they have on the heart?
long-acting, added for persistent angina; decrease preload by dilation of capacitance veins
What is used for alternative therapy for refractive angina? What effect does it have on the heart?
ranolazine -> decrease myocardial calcium influx
Manifestations of CVID? Dx? Tx?
Manifestations:
- recurrent respiratory (pneumonia, sinusitis, otitis) + GI (Salmonella, Campylobacter) infections
- Autoimmune disease (RA, thyroid disease)
- Chronic lung disease (bronchiectasis)
- GI disorders (chronic diarrhea, IBD-like conditions)
Dx: significantly decreased IgG, decreased IgA/IgM; no response to vaccination
Tx: immunoglobulin replacement therapy
positive HBsAg, HBeAg, + IgM anti-HBc - what dx?
early phase acute HBV
Positive IgM anti-HBc - what dx?
window phase of acute HBV
Positive IgG anti-HBc, anti-HBs, and anti-HBe - dx?
recovery phase of acute HBV
positive HBsAg + IgG Anti-HBc - dx?
chronic HBV carrier
positive HBsAg, HBeAg (+/-), IgM anti-HBc, IgG anti-HBc - dx?
acute flare of chronic HBV
positive Anti-HBs - dx?
vaccinated against HBV
positive Anti-HBs and IgG anti-HBc?
immune due to natural HBV infection
epidemiology of optic neuritis?
primarily in young women; associated with MS; immune-mediated demyelination
Pt, Dx, and Tx of optic neuritis?
Pt: 1. acute, peaks at 2 weeks
- monocular vision loss
- eye pain with movement
- “washed-out” color vision
- afferent pupillary defect
Dx: MRI of the orbits + brain
Tx: IV corticosteroids
What is CVP (right-sided preload), PCWP (left-sided preload), cardiac index (LV output), SVR (after load), and SvO2 of hypovolemic shock?
CVP (right-sided preload)- decreased PCWP (left-sided preload) - decreased cardiac index (LV output) - decreased SVR (after load) - increased SvO2 - decreased
What is CVP (right-sided preload), PCWP (left-sided preload), and cardiac index (LV output), SVR (after load), and SvO2 of cariogenic shock?
CVP (right-sided preload) - increased PCWP (left-sided preload) - increased cardiac index (LV output) - decreased SVR - increased SvO2 - decreased
What is CVP (right-sided preload), PCWP (left-sided preload), and cardiac index (LV output), SVR (after load), and SvO2 of obstructive shock?
CVP (right-sided preload) - increased PCWP (left-sided preload) - decreased Cardiac index (LV output) - decreased SVR - increased SvO2 - decreased
What is CVP (right-sided preload), PCWP (left-sided preload), and cardiac index (LV output), SVR (after load), and SvO2 of distributive shock?
CVP (right-sided preload) - decreased PCWP (left-sided preload) - decreased cardiac index (LV output) - increased SVR - decreased SvO2 - increased
conjunctival injection, tarsal inflammation, and pale follicles in a child - dx? path? dx? tx? complications?
trachoma
path: caused by chlamydia trachomatis serotypes A, B, and C (leading cause of blindness worldwide)
pt: often concomitant nasopharyngeal infection (rhinorrhea, pharyngitis)
dx: clinical via examination of the tarsal conjunctivae; bug visible by Giemsa stain
tx: azithromycin; eye surgery to preserve vision in case of trichiasis
comp: repeated or chronic infection leads to scarring of the eyelids and inversion of the eyelashes (trichiasis); over time, the lashes rub on the eye and cause ulcerations and blindness
What lab findings would you expect in causes of intravascular hemolysis?
(ex: paroxysmal nocturnal hemoglobinuria, DIC); Hgb released binds to haptoglobin, and the hemoglobin-haptoglobin complex is cleared by the liver. Thus, decreased serum haptoglobin, elevated indirect bilirubin levels (from heme breakdown), and raised serum LDH (released from RBCs)
What lab findings would you expect in cases of extravascular hemolysis?
RBCs predominantly destroyed by phagocytes in the RES system (LNs, spleen); slightly low to normal haptoglobin, slightly elevated LDH, and elevated indirect bilirubin
(ex: Ab-mediated RBC destruction (autoimmune hemolytic anemia) or intrinsic RBC enzyme (G6Pd) or membrane (hereditary spherocytosis) defects
What is the difference between autoimmune hemolytic anemia and hereditary spherocytosis?
AIHA - peripheral smear shows spherocytes without central pallor; NEGATIVE family history; POSITIVE Coombs test
hereditary spherocytosis - same peripheral smear; STRONG family history; NEGATIVE Coombs test
What are the risk factors, Pt, and lab findings in an adult with lead poisoning?
Risk factors: occupational exposure (lead paint, batteries, ammunition, construction)
Pt: GI (abdominal pain, constipation, anorexia), neurologic (cognitive deficits, peripheral neuropathy), hematologic (anemia)
Labs: anemia, elevated venous lead level, elevated serum zinc protoporphyrin level, basophilic stippling on peripheral smear, hyperuricemia (due to impaired purine metabolism)
Etiology, Pt, Labs, and tx of primary adrenal insufficiency?
Etiology: autoimmune adrenalitis (most common), infection (TB), metastatic infiltration
Pt: fatigue, weakness, anorexia/weight loss; nausea, vomiting, abdominal pain; salt craving, postural hypotension; hyperpigmentation; acute adrenal crisis: confusion, hypotension/shock
Labs: hyponatremia, hyperkalemia, eosinophilia; low morning cortisol, high ACTH
Tx: glucocorticoids, mineralocorticoids
What is the most common cause of otitis externa? 2nd most common? What is the treatment?
Most common: pseudomonas aeruginosa
Second most common: Staph aureus
Treatment: topical antibiotic (fluoroquinolone) +/- topical glucocorticoid
What are 8 common causes of macrocytic anemia?
- folate deficiency
- Vit B12 deficiency
- Myelodysplastic syndromes
- Acute myeloid leukemias
- Drug-induced (hydroxyurea, zidovudine, chemotherapy agents)
- liver disease
- alcohol abuse
- hypothyroidism
In the case of traumatic head/neck injury, when is cervical spine imaging necessary? What is the preferred imaging modality?
CS imaging is necessary if any of the following is present:
- neurologic deficit
- spinal tenderness
- altered mental status
- intoxication
- distracting injury
CT scan is the preferred screening test to evaluate for CS injury
Does acidosis or alkalosis cause hyperkalemia?
acidosis
Do beta-2 adrenergic agents cause hyperkalemia or hypokalemia?
hypokalemia (stimulate the Na-K-ATPase pump and the Na-K-2 Cl cotransporter); also stimulate the release of insulin, which further promotes intracellular K shift
What is the etiology, pleural fluid analysis, pleural fluid gram stain and culture, and treatment of uncomplicated parapneumonic effusions?
Etiology: sterile exudate in pleural space
Pleural fluid analysis: pH >/= 7.2, glucose >/= 60, WBC = 50,000
Pleural fluid gram stain + culture = negative
Tx = Abx
What is the etiology, pleural fluid analysis, pleural fluid gram stain and culture, and treatment of complicated parapneumonic effusions?
Etiology: bacterial invasion of pleural space
Pleural fluid analysis: pH <7.2, glucose <60, WBC > 50,000
Pleural fluid gram stain + culture: negative (usually false neg due to low bacterial count)
treatment: Abx + drainage
HTN, elevated Cr, proteinuria, hematuria, and RBC casts along with fatigue, cytopenias, and hypocmplementemia - dx?
SLE
Causes, pt, and evaluation of hydrocephalus in children
causes: impaired CSF circulation, impaired CSF absorption, excessive CSF production
pt: 1. macrocephaly +/or rapidly enlarging head circumference
2. prominent scalp veins
3. full anterior fontanelle
4. behavioral changes +/or developmental delay
5. signs of increased ICP (HA, vomiting, papilledema, HTN, bradycardia)
evaluation: neuroimaging (U/S if open fontanelles, MRI if not)
Pt, clinical course, and treatment of vitiligo?
Pt: depigmented macules on sacral areas + extensor surfaces; face commonly affected; lesions may be symmetrical, dermatomal, or unilateral
course: 1. most cases progress gradually
2. regimentation is spontaneous in 10-20% of cases
3. increased incidence of other autoimmune disorders (lupus, thyroid, pernicious anemia, Addison dz)
treatment: limited disease: topical corticosteroids
extensive/unresponsive disease: oral corticosteroids, topical calcineurin inhibitors, PUVA (psoralen + UVA light)
blunt trauma to abdomen in a hemodynamically unstable patient -> + peritonitis -> next step?
laparotomy
blunt trauma to abdomen in a hemodynamically unstable patient -> negative peritonitis -> next step?
FAST
blunt trauma to abdomen in a hemodynamically unstable patient -> + free fluid on FAST -> next step?
laparotomy
blunt trauma to abdomen in a hemodynamically unstable patient -> negative free fluid on FAST -> next step?
consider CTAP (CT scan of abdomen + pelvis) or DPL (diagnostic peritoneal lavage); evaluate for other sources of hemorrhage
blunt trauma to abdomen in a hemodynamically stable patient -> + peritonitis -> next step?
laparotomy
blunt trauma to abdomen in a hemodynamically stable patient -> negative peritonitis -> next step?
FAST
blunt trauma to abdomen in a hemodynamically stable patient -> free fluid on FAST -> next step?
CTAP
blunt trauma to abdomen in a hemodynamically stable patient -> negative free fluid on FAST -> next step?
consider CTAP or serial abdominal examinations based on suspicion for intraabdominal injury
When is radioactive iodine not recommended as a treatment for Graves disease?
patients with mild disease probably only need PTU/methimazole; not recommended with opthalmopathy because it can worsen it (can give glucocorticoids to help prevent this though)
REM sleep behavior disorder path + pt
path - degeneration of the brainstem nuclei responsible for inhibiting spinal motor neurons during normal REM sleep -> incomplete or absent muscle atonia; can be a sign of subsequent Parkinson disease or dementia with Lewy bodies
pt - dream enactment, can be awoken easily, often do not recall movement during sleep but recall dreams; more common in older adult men
Possible etiologies of constrictive pericarditis
- idiopathic or viral pericarditis
- cardiac surgery or radiation therapy
- TB pericarditis (in endemic areas)
Pt and Dx findings of constrictive pericarditis
Pt:
- fatigue + DOE
- Peripheral edema + ascites
- increased JVP
- Pericardial knock may be heard
- Pulsus paradoxus
- Kussmaul’s sign
Dx findings:
- ECG may be nonspecific or show atrial fibrillation or low-voltage QRS
- Imaging shows pericardial thickening and calcification
- JVP tracing shows prominent x + y descents
What is Kussmaul’s sign? When might you see it?
lack of the typical inspiratory decline in central venous pressure; constrictive pericarditis
Frequency, age of onset, clinical features, and sex ratio of systemic juvenile idiopathic arthritis?
Frequency: 10% age of onset: age < 18 Clinical features: 1. arthritis in >/= 1 joint for >/= 6 weeks 2. quotidian fever for >/= 2 weeks 3. evanescent rash 4. hepatosplenomegaly 5. LAD Sex ratio: F=M
Frequency, age of onset, clinical features, and sex ratio of polyarticular juvenile idiopathic arthritis?
Frequency: 40% age of onset: age 2-5 OR 10-14 clinical features: 1. arthritis in >/5 joints 2. may be complicated by uveitis sex ration: F >M
Frequency, age of onset, clinical features, and sex ratio of oligoarticular juvenile idiopathic arthritis?
frequency: 50% age of onset: 2-4 clinical features: 1. arthritis in <5 joints 2. may be complicated by uveitis sex ratio: F > M
Pt + Dx of chronic lymphocytic leukemia
Pt: 1. LAD (cervical, supraclavicular, axillary) 2. HSM 3. mild thrombocytopenia + anemia 4. often asymptomatic Dx: 1. severe lymphocytosis + smudge cells 2. flow cytometry 4. LN + bone marrow biopsy not generally needed
Prognostic factors and complications of chronic lymphocytic leukemia
Prognostic:
- median survival 10 years
- worse prognosis with:
- multiple chain LAD
- HSM
- anemia + thrombocytopenia
Complications:
- infection
- autoimmune hemolytic anemia
- secondary malignancies (Richter transformation)
How does hairy cell leukemia usually present?
splenomegaly and pancytopenia
What does CML look like on peripheral smear?
massive leukocytosis with a variety of neutrophil precursor cells (eg promyelocytes, myelocytes)
What are the risk factors for pancreatic adenocarcinoma?
- smoking
- hereditary pancreatitis
- nonhereditary chronic pancreatitis
- obesity + lack of physical activity
What is the clinical pt and lab findings of pancreatic adenocarcinoma?
Pt:
1. systemic symptoms (eg weight loss, anorexia),
2. abdominal/back pain
3. jaundice
4. recent-onset atypical diabetes mellitus
5. unexplained migratory superficial thrombophlebitis
6. hepatomegaly + ascites with metastasis
Lab findings:
1. cholestasis (increased all phos + direct bilirubin)
2. increased CAA 19-9
3. abdominal ultrasound (if jaundiced) or CT scan (if no jaundice)
What are high-risk features of pediatric TBI in those age < 2
- AMS (fussy behavior)
- LOC
- severe mechanism of injury (fall > 3 feet, high impact, MVC)
- confrontal scalp hematoma
- palpable skull fracture
What are high-risk features of pediatric TBI in a child age >/= 2-18
- AMS (somnolence, agitation)
- LOC
- severe mechanism of injury (fall > 5 feet, high impact, MVC)
- vomiting, severe headache
- basilar skull fracture signs (CSF, rhinorrhea)
What is chemoprophylaxis protocol for close contacts of those with meningococcal meningitis?
rifampin, ciprofloxacin, or ceftriaxone
In what situations might you expect infective endocarditis caused by staph aureus?
- prosthetic valves
- intravascular catheters
- implanted devices
- IV drug users
In what situations might you expect infective endocarditis caused by viridians streptococci?
- gingival manipulation
2. respiratory tract incision or biopsy
In what situations might you expect infective endocarditis caused by staph epidermidis?
- prosthetic valves
- intravascular catheters
- implanted devices
In what situations might you expect infective endocarditis caused by enterococci?
nosocomial urinary tract infections
In what situations might you expect infection endocarditis caused by strep gallolyticus (formerly strep bovis)
- colon carcinoma
2. inflammatory bowel disease
In what situations might you expect infective endocarditis caused by fungi? (eg candida)
- immunocompromised host
- intravascular catheters
- prolonged Abx therapy
optic glioma, lisch nodules, axillary + inguinal freckling, pseudoarthrosis, scoliosis, cafe au lait spots - dx?
neurofibromatosis 1
history of recent URI, fatigue, dyspnea, elevated JVP, cardiomegaly on imaging, audible S3, bibasilar rales on PE, pulmonary vascular congestion on imaging studies - dx?
viral myocarditis
fixed splitting of the second heart sound should make you think of what?
atrial septal defect
opening snap is heard in patients with what?
mitral stenosis
Etiology, Pt, Dx, and Tx of urethritis in men
Etiology:
1. Neisseria gonorrhoeae
2. Chlamydia trachomatis
3. Mycoplasma genitalium
4. Trichomonas (rare)
Pt: dysuria, discharge, urgency, increased voiding frequency
Dx: urinalysis, gram stain + culture, NAAT
Tx: azithromycin OR doxycycline PLUS ceftriaxone if gonococcus suspected or not ruled out
Etiology of prerenal acute kidney injury
Decreased renal perfusion:
- true volume depletion
- decreased EABV (effective arterial blood volume) (eg heart failure, cirrhosis)
- displacement of intravascular fluid (sepsis, pancreatitis)
- renal artery stenosis
- afferent arteriole vasoconstriction (NSAIDs)
Clinical features and Tx of prerenal acute kidney injury
- increase in serum Cr (50% from baseline)
- decreased urine output
- BUN/Cr ratio > 20:1
- fractional excretion of sodium < 1%
- unremarkable (“bland”) urine sediment
Tx: restoration of renal perfusion
What are risk factors for sternal dehiscence? How do you fix it?
- obesity
- macromastia (large breasts)
- sequelae of COPD (barrel chest deformity, chronic cough)
- smoking
- diabetes
Tx - surgical emergency involving surgical exploration, debridement, and sternal fixation to prevent cardiac damage
What is a porcelain gallbladder? What disorder do you see this in? What are these patients at an increased risk of? Tx?
porcelain gallbladder = calcium-laden gallbladder wall with bluish color and brittle consistency
associated with chronic cholecystitis
at increased risk of gallbladder adenocarcinoma
cholecystectomy is considered for patients with porcelain gallbladder
Epidemiology, pt, and dx of renal cell carcinoma
Epidemiology: age > 50, increased risk with smoking, obesity, and chemical exposure
Pt:
1. hematuria (microscopic or gross +/- clots)
2. abdominal/flank mass
3. flank pain
4. left-sided varicocele that does not decompress
5. paraneoplastic syndrome (EPO production or increased calcium)
Dx: abdominal CT, partial/complete nephrectomy for histology
etiology and pt of urethral stricture
etiology: male > female, urethral trauma (catheterization) urethritis, radiotherapy
Pt: weak or spraying strep, incomplete emptying, irritative voiding (dysuria, frequency)
dx, tx, and complications of urethral stricture
dx: postvoid residual, uroflowmetry, urethrography, cystourethroscopy
tx: dilation, urethroplasty
Complications: acute urine retention, recurrent urinary tract infection, bladder stones
Path, Pt, Labs, and Tx of classic congenital adrenal hyperplasia?
path: autosomal recessive, 21-hydroxylase deficiency
Pt:
1. ambiguous genitalia in girls
2. salt-wasting syndrome (affects most girls and boys -> hypotension, dehydration + vomiting)
Labs: decreased sodium, increased potassium, decreased glucose, increased 17-hydroxyprogesterone
Tx: 1. glucocorticoids + mineralocorticoids
2. high salt diet
3. genital reconstructive surgery for girls
4. psychosocial support
How is the presentation of CAH different in female vs male infants?
excess androgen leads to virilization in female (46, XX) infants; male (46, XY) infants are phenotypically normal or hypervirilized (enlarged phallus)
corneal edema/cloudiness; fixed, mid-dilated pupil; conjunctival redness - dx?
acute glaucoma
Pt and Tx of neuroleptic malignant syndrome?
Pt: fever, confusion, muscle rigidity (generalized), autonomic instability (abnormal vital signs, sweating)
Tx:
1. stop antipsychotics or restart dopamine agents
2. supportive care (hydration, cooling), ICU
3. dantrolene or bromocriptine if refractory
Symptoms, endoscopic findings, biopsy, and complications of ulcerative colitis
Symptoms: bloody diarrhea, WL, fever Endoscopic findings: 1. erythema, friable mucosa 2. pseudopolyps 3. involvement of rectosigmoid 4. continuous colonic involvement (no skip lesions) Biopsy: mucosal + submucosal inflammation, crypt abscesses Complications: 1. toxic megacolon 2. primary sclerosing cholangitis 3. colorectal cancer 4. erythemanodosum, pyoderma gangrenosum 5. spondyloarthritis
description, associated signs, and causes of cerebellar gait disorder
description: staggering, wide-based associated signs: dysdiadochokinesia, dysmetria, nystagmus, Romberg sign causes: 1. cerebellar degeneration 2. stroke 3. drug/alcohol intoxication 4. vitamin B12 deficiency
description, associated signs, and causes of gait apraxia (frontal gait)
description: magnetic (freezing): start and turn hesitation
associated signs: dementia, incontinence, frontal lobe signs
causes:
1. frontal lobe degeneration
2. normal pressure hydrocephalus
description, associated signs, and causes of parkinsonian gait
description: short steps, shuffling
associated signs: bradykinesia, resting tremor, postural instability, decreased arm swing
causes: Parkinson disease
description, associated signs, and causes of steppage gait
description: foot drop - excessive hip and knee flexion hile walking, slapping quality, falls
associated signs: distal sensory loss + weakness
cause: motor neuropathy
description, associated signs, and causes of vestibular gait
description: unsteady, falling to one side
associated signs: normal sensation, reflexes + motor strength; nausea, vertigo
causes: acute labyrinthitis, Meniere disease
bone pain, headaches, unilateral hearing loss, femoral bowing - dx? cause?
paget disease of bone; osteoclast dysfunction leads to bone breakdown and a compensatory increase in bone formation
Epidemiology, Pt, Dx, and Tx of progressive multifocal leukoencephalopathy (PML)
Epidemiology: JC virus reactivation, severe immunosuppression (untreated AIDS)
Pt: slowly progressive, confusion, paresis, ataxia, seizure
Dx:
1. MRI of the brain: asymmetric white matter lesions; no enhancement/edema
2. Lumbar puncture: CSF PCR positive for JC virus
3. brain biopsy (rarely needed)
Tx: often fatal; if HIV positive -> retroviral therapy
Definition, Pt, and Tx of nonalcoholic fatty liver disease
Definition:
1. hepatic steatosis on imaging or biopsy
2. exclusion of significant alcohol use
3. exclusion of other causes of fatty liver
Pt: mostly asymptomatic, metabolic syndrome, +/- steatoheaptitis (AST/ALT ration <1), hyper echoic texture on u/s
Tx: diet + exercise, consider bariatric surgery if BMI >/= 35
Pulmonary manifestations of sarcoidosis?
hilar LAD + interstitial infiltrates
cutaneous manifestations of sarcoidosis?
papules, nodules + plaques; ereythema nodosum
ophthalmologic manifestations of sarcoidosis?
anterior + posterior uveitis; keratoconjunctivitis sicca
neurologic manifestations of sarcoidosis?
facial nerve palsy, central diabetes insipidus, hypogonadotropic hypogonadism
cardiovascular manifestations of sarcoidosis?
AV block, dilated or restrictive cardiomyopathy
GI manifestations of sarcoidosis?
HSM, asymptomatic LFT abnormalities
Etiology, Pt, Dx, and Tx of pulmonic valve stenosis?
Etiology: congenital or squired (rarely, carcinoid)
Pt: severe: right-sided heart failure in childhood
mild: symptoms (dyspnea) in early adulthood
crescendo-decrescendo murmur (increased on inspiration)
systolic ejection click + widened split of S2
Dx: echo
Tx: percutaneous balloon valvulotomy (preferred), surgical repair in some cases
Etiology of lithium toxicity
Acute toxicity: intentional overdose
chronic toxicity:
decreased renal perfusion (decreased lithium clearance):
1. dehydration
2. thiazide diuretics, NSAIDs, ACE inhibitors)
Pt and Tx of lithium toxicity
Acute toxicity:
1. GI: N/V/D
2. late neurologic sequelae
Chronic toxicity (neurologic):
1. lethargy, confusion, agitation
2. ataxia, tremor/fasciculations, seizure
Tx: IV hydration, hemodialysis (severe cases)
Dx criteria for diagnosis of major depressive disorder
- > /= 2 weeks
- > /= 5 of 9 symptoms: depressed mood + SIGECAPS
- significant functional impairment
- no lifetime history of mania
