Name all the commonly tested X-linked recessive diseases.

Lesch-Nyhan WISHes DUCHy Tim BURTON would stop making G-rated FABles, and make a movie about at a CHRONIC HEME-loving OCTopus HUNTER.

Lesch-Nyhan, Wiskot-Aldrich, Duchenne's, Burton's agamma., G6PD def., Fabry's, Chronic Granulomatous disease, Hemophelia A and B, Octular Albinism, Hunter's

Name the 4 Trinucleotyde Repeat Syndromes.

What are the repeats they're associated with?

What is there inheritance pattern?

MyoTonic dystrophy (AD) - CTG

Freidrich's Ataxia (AR) - GAA

FraGile X-syndrome (X-linked) - CGG

Huntington's Chorea (AD) - CAG

Are deep tendon reflexes preserved or lost/diminished or increased in Myasthenia Gravis?

What medications exacerbate myesthenia gravis?

-Preserved

-Meds that exacerbate MG:

  -antibiotics: aminoglycosides and tetracyclines

  -beta-blockers

  -antiarrhythmics: quinidine, procainamide, and lidocaine

Familial hemiplegic migraine

Uncontrolled HTN

CAD

Prinzmetal angina

Pregnancy

Ischemic Stroke

Basilar Migraine

What's the best initial step in managing a suspected stroke or TIA?

CT of head w/o contrast b/c if intracranial hemorrhage is present, this drastically changes management (t-PA is contraindicated)

Aspririn is the best initial treatment, and if aspirin is not tolerated you can use dipyramidol

How does the sympathetic and parasympathetic NS innervate the eye (who do these nn fibers get to the pupil)?

What innervates the touch sensation of the cornea?

SNS - supracervical ganglia -> common carotid -> internal carotid -> V1 -> long ciliary nerve

PNS - Edinger Westfall nucleus -> CN III

V1 provides touch sensation to cornea

Prolonged QRS suggests a _____arrhythmia

Prolonged QT suggests a _____arrhythmia

QRS --> bradyarrhythmia

QT --> tachyarrhythmia

TMP-SMX to prophy against Pneumocystis and Toxoplamosis

You can also give gancyclovir or valcyclovir to prophy against CMV

How do you differentiate a case control study from a cross sectional study?

What types of odds ratios are you able to derive from these studies?

A cross sectional study is examining at a specific point in time who does and doesn't have disease while at the same time looking for factors thay might be associated with the disease. This type of study gives you prevelence of disease, and prevalence odds ratio (used to compare prevalence of disease btwn diff populations)

A case control study is looking back in time and comparing those this a disease to those without with respect to the presence/absence of a risk factor of interest. This type of study establishes causality, and gives you exposure odds ratio (i.e. the odds of you having had a risk factor given your disease status).

What are the main Sx of TCA overdose?

What is the MCC of TCA overdose-related death?

How does NaHCO3 have cardioprotective effects when a person has tricyclic anti-depressant overdose?

Sx - anticholinergic (hyperthermia, dilated pupils, intestinal ileus), Na channel blockade (prolonged QRS), alpha-1 blockade (hypotension), inhibition of NE and 5-HT reuptake (insomnia, anxiety, seizures), anti-histamine (sedation)

MCC of death - TCA induced hypotension

1) The sodium load helps in alleviates the depressant effect on fast-action sodium channels

2) Alkylinization of serum decreases drug avidity for sodium channels

What is the treatment for:

1) (initially treating) comedonal acne

2) mild-moderate acne

3) moderate-severe acne

4) nodulocystic/scarring acne

What must you avoid in pts with acne?

1) Topical retinoids

2) Topical erythromycin or clinda -> suppress P. acnes

3) Systemic AbX (tetracyclin, minocyclin, doxycycline, erythromycin, TMP-SMX)

4) Oral retinoids

Avoid acne-promoting agents such as make-up, creams, oils, steroids, androgens

What are the clinical features and fundoscopic exam findings of optic neuritis in pts with MS?

What other eye pathology may you see in MS pts?

-Monocular vision loss

-Painful eye mov't

-Central visual field defects

-Decreased pupillary light reaction

-Fundoscopy is normal

Remember, these pts may also have internuclear ophthalmoplegia due to destruction of the MLF (which connects the nucleus of VI to the contralateral nucleus to III) -> one eye stares at you when you try to have them look L or R

A 49 y/o female p/w progressive exertional dyspnea. She also complains of swelling and pain in fingers over past 6 months. Exam shows prominent JVP, lungs are CTAB, skin is thickened and discolored over fingertips and interphalangeal joints, and mild pitting ankle edema.

Dx?

Pathogenesis?

What antibody might be present in this pt?

Dx - systemic sclerosis, which affects every organ system:

  -skin begins to thicken at hand/feet w/ associated edema

  -Raynaud phenomenon and calcinosis cutis are also common. Can cause "mask facies"

  -Pulm HTN results in RVF -> JVD and LE edema

  -Esophageal and gastric dysmotility -> GERD

  -Renal disease -> HTN (treat with ACI-I)

-Pt may have anti-topoisomerase I Abs (aka anti-SLC 70 abs)

What is the best initial screening test for suspected adrenal insufficiency (Addisons)?

What's the best initial screening test for suspected Cushings?

(Qid 2174)

Cosyntropin (ACTH analogue) stimulation test

-A rise in cortisol levels above 20 mcg/dL 30-60 mins after administration of cosyntropin r/o Addison's disease

Cushings:

  -Inital screening test is overnight (low dose) dexamethasone suppression test

        -Give 1 mg dexameth, and measure serum cortisol at 8 am. If <5 -> Cushings can be excluded. If >5, you give high dose Dexameth to determine cause of Cushings

When is it recommended for adults to receive:

1) Td vaccine

2) Intramuscular or Intranasal influenza vaccine

3) Pneumococcal polysaccharide vaccine

1) Td should be given Q 10 yrs after age 18, and a single TdAP booster is rec'd for adults of all ages in place of Td

2) Intramuscular influenza vaccine for pregnant women, and pts with comorbidities. Intranasal (live-attenuated) given to non-pregnant, healthy pts under 50

3) Pneumoccocal for adults 65 yrs +, unless pt has chronic disease, including CV, pulm, hepatic, renal, metabolic (DM), or pts who are immunosuppressed -> then you give it to them regardless of age

   -No booster req'd if given > 65 y/o; if given <65 y/o then give booster 5 years later when pt is > 65 y/o

What is the pathophysiology behind neuroleptic malignant hyperthermia?

How does it present?

What's the treatment?

Pathophys - sympathoadrenal hyperactivity. Use of anti-psychotics in genetically predisposed ppl can cause increased Ca2+ release from SR -> increased myocyte contractility

Sx - confusion, fever, mm rigidity, diaphoresis, CK of 50,000

Tx - dantrolene (mm relaxant that works on the RyR receptor) is main treatment, followed by bromocriptine

What 4 pathogenic factors contribute to the development of hepatic encephalopathy?

What are the precipitating factors for hepatic encephalopathy?

1) Accumulation of NH3 in blood

2) Production of false neurotransmitters

3) Increased sensitivity of CNS to inhibitoy NTs like GABA

4) Zinc deficiency

Precipitating factors:

1) High protein diet

2) Alkylosis

3) Diuretic therapy

4) Extensive gastro intestinal bleeding

5) Narcotics, hypnotics, sedatives

6) Medication containing NH4 or amino compounds

7) High volume paracentesis

8) Hepatic or systemic infection

9) Portocaval shunts

How do the following present on fundoscopic exam?

1) Ocular toxoplasmosis

2) Herpes keratitis

3) CMV retinitis

4) HIV retinopathy

1) White, fluffy lesion surrounded by retinal edema and vitritis. Its a severe necrotizing retinochoroiditis and encephalitis

2) Minute clear vesicles in the corneal epithelium. Dendritic ulcer is MC presentation. P/W pain, photophobia, and decreased vision

3) Yellow-white patches of retinal opacification and hemorrhages. Pts may be asymptomatic

4) Benign, cotton-wool spots in retina which remit spontaneously

1) Pre-renal azotemia

2) Upper GI bleeding - hemoglobin is broken down by GI bacteria -> increased GIT abs. of urea

3) Corticosteroid use -> increased mm breakdown leads to increased protein delivery to liver -> increased urea cycle -> increase urea

What are the 3 different reasons why a person with DMI might have hyperglycemia in the morning?

How can you differentiate them?

1) Waning of circulating insulin in morning - 3 am glucose will be nml

2) Somogyi effect (release of catecholamines and glucagon in response to hypoglycemia) - 3 am glucose will be low

3) Dawn effect (decreased insulin sensitivity in morning due to GH release) - 3 am glucose will be high

What are the 4 MCC of priapism (including 2 drugs)?

What commonly used drugs can cause impotence?

1) Trazadone and prazocin (alpha-1 blocker)

2) SS anemia and leukemia - usually seen in kids

3) Perineal or genital trauma - results from laceration of caverous artery

4) Neurogenic lesions - spinal cord injury or cauda equina compression

Paget's Disease of Bone (osteitis deformans)

MCly involved bones are skull, clavicles, long bones, pelvis

A 62 y/o man comes in b/c of malaise, confusion, dyspnea, and productive cough. He's been recovering from a URI for the past week. He reports coughing up copious yellow blood streaked sputum. T 104, BP 150/90, HR 110. CXR shows infiltrates in lung midfields bilaterally as well as multiple thin-walled cavities.

Dx?

Dx - saphylococcal, S. pneumo, or H. flu super-infection of lung resulting in secondary pneumatoceles

-The hint was this pt's viral URI Sx. The pt then acutely worsened, suggesting post-viral URI necrotizing pulmonary bronchopneumonia

-Its likely NOT bronchiectasis b/c this would produce chronic bronchopneumonia.

Pt comes in having been stick with a needle after drawing blood from a pt who is Hep B positive. Pt had a Hep B vaccination 1 yr ago and was found to have HBsAb-positive serum.

NSIM?

NSIM - reassurance, his Abs will take care of any possible exposure to Hep B

-You only give HBIG and Hep B Vaccine to pts whi unknown immunity or unvaccinated pts

Name 3 key diagnostic ECG features of RV hypertorphy.

Of LV hypertrophy (3 as well).

RVH

  -R axis deviation (down in I, up in II)

  -dominant R wave in V1

  -disurpted R wave progression

LVH

  -L axis deviation (up in I, down in II)

  -S wave of V1 + R wave of V6 > 35 mm

  -R wave of aVL > 11 mm

-ST elevation in "inferior leads" (II, III, aVF)

-Possibly bradycardia due to ischemia/infarction of SA node

Name 3 key diagnostic ECG features of RBBB.

LBBB (2).

RBBB

  -widened QRS

  -R' in V1

  -Broad, slurred S waves in lateral precoridal leads (V5-V6)

LBBB

  -Broad, slurred R waves in lateral precordial leads (V5-V6)

  -S waves in R-precordial leads (V1-V3)

-Diffuse ST segment elevation

-PR segment depression (why???)

Name 2 key diagnostic ECG features of hyperkalemia.

Hypokalemia (4)

Hyper

  -Prolonged QRS (hyperkalemia causes an overall membrane depolarization that inactivates many sodium channels)

  -Peaked T waves (hyperkalemia directly increases K channel activity and speed)

Hypo -prolonged PR interval, ST depression, T wave inversion, prominent U waves

-Totally inverted P, QRS, T complexes in limb leads

-Normal precordial leads

-Shortened PR interval (nml is 0.12-0.2)

-Wide QRS and the delta wave

What population and how often should the following vaccinations be given?

1) Td

2) Influenza

3) Pneumoccocal

1) One Td should be substituted with TDAP, then all subsequent Td it should be given every 10 years

2) Flu vaccine should be given intramuscularly to all adults, and in healthy, non-pregnant pts the intranasal one can be given

3) Pneumococcal vaccine should be given all healthy adults once 65 yrs old, and ppl under 65 who have chronic medical cond'ns: lung, CV, liver, renal, metabolic diseases, asplenia, immunosuppression (these pts may need booster 5 years later)

Indinavir (HIV protease inhibitor)

Acyclovir

Sulfonamide Therapy

What 3 pathogens can result in Guillan Barre?

What is the Tx for GB?

-HSV, Mycoplasma, H. flu

-Tx: IVIG, and if progression continues -> plasmapheresis

What is a chalazion? Tx?

What is a hordeolum? Tx?

Chalazion - chronic, sterile, granulomatous inflammatory lesion of the meibomian glands (produce an oily substance for eye that prevents tear spillage onto the cheek)

  -Tx - incision and curettage; if persistent, get a histopathologic exam to r/o malignancy

Hordeolum - purulent infection of one of the glands of the eye lid, and is caused by staph

   -Tx - application of warm packs; if not resolved in 48 hrs -> anti-staph AbX

A 30 y/o woman comes in b/c of a painful rash on her R-hand and tender swelling of her R-axilla. Exam shows multiple pruritic, erythematous viscicles and papules over R-hand. She says she keeps 2 cats at home.

Dx?

Tx?

Dx - Cat scratch disease due to B. henselae

Tx - 5 day course of oral azithromycin

1) Cardiac arrhythmias, specifically prolonged QT interval (also seen in hypokalemia and hypomagnesemia)

2) Rickets/Osteomalacia

3) Increased neuromuscular irritability due to hypocalcemia

  -numbness/tingling - circumoral, fingers, toes

  -tetany - hyperactive DTRs, Chvostek sign, Trousseau's sign

  -grand mal seizures

4) Basal ganglia calcifications

45 y/o female p/w constipation, abd pain, urinary frequency, and constant thirst. She is obese ant has been attempting fad diets for 6 months. She sys she's been taking numerous OTC vitamins and minerals. PMHx positive for AF for 4 years, wfor which she takes diltiazem. UA is nml.

Dx?

Vitamin D overdose -> increased absorption of Ca  from gut and decreased excretion of calcium from kindey -> hypercalcemia

-Four main Sx: constipation, abd pain, plolyuria, polydipsia

40 y/o male comes in b/c of severe R-sided HA, n/v, and eye pain. He was fixing a light bulb when the pain suddenly came on. He now says he sees "halos around lights." Exam hows R-pupil mid-dilated and non-reactive to light, and is hard to palpation.

Dx? 

What test can you peform regularly in pts to prevent this?

Tx (5 types)?

Dx - closed angle glaucoma

Tonometry measures IOP, should be performed regularly in pts w/ or at risk for glaucoma

Tx:

  -Manitol (IV) - first line Tx, osmotic diuretic

  -Acetezolamide - CA inhibitor, reduces production of AqH

  -Beta-blockers (timolol) - decreases production of AqH

  -Cholinergic Agonists (pilocarpine) - open the canals of Schlemm -> allow drainage of AqH

  -Prostaglandins (latanoprost = PGF2) - increases AqH outflow

Qid 3251, SU 443

What is the drug of choice for treatment of dermatitis herpetiformis?

When does it take effect?

-Dapsone

-It takes effect w/in hours of beginning drug

According to the USPSTF, when should women begin getting the following, and how often?

1) Mammogram

2) Pap Smear

3) Lipid panel

4) Colonoscopy

Qid 4231

1) Mammogram - Q 2 yrs in women 50-74

2) Pap smear - starting at 21 (regardless of sexual activity) Q 2 yrs, after age 30 its Q 3 yrs (if negative Pap smears); women beyond 65 who have 3 consecutive Pap smears no longer require screening

3) Lipid panel - start at 35 for men and 45 for women, Q 5 yrs (no more than that)

4) Colonoscopy - start at 50, Q 10 yrs

  -If first degree relative has Hx of colon cancer -> start 10 yrs earlier

A 34 y/o immigrant from Mexico comes in b/c of severe SOB, cough, and hemoptysis. CXR shows R upper lobe fibrosis and her sputum is positive for acid-fast bacilli.

Dx?

Tx?

What SE must she be monitored for?

Mechanism for SE?

Dx - active pulmonary Tuberculosis

Tx - RIPE: Rifapin (RNA polymerase inhibitor), INH (inhibits mycolic acid synth), Pyrizinamide (inhibits FA synthase; works on intracellular MTB), Ethambutol (inhibits carbohydrate polymeration -> MTB can synth cell wall)

-Pt must be monitored for:

  -Optic neuropathy - ethambutol causes red-green color blindness

  -Neuropathy - INH, b/c of its chemical similarity to B6, can reduce NT synthesis

  -Hepatitis - INH is metabolized by liver to an ammonium containing compound which is hepatotoxic. Toxicity depends on rate of metabolism, and can range from severe hepatitis, to mild subcliical hepatic injury (mild elevations in aminotransferases; <100). If only mild elevation in LFTs -> continue Tx; if SS of hepatitis present -> discontinue INH

  -Red Urine - Rifampin turns urine Red/Orange

45 y/o female comes in b/c of several months of numbness and ocassional nocturnal pain in R palm. PE shows flattening of the thenar eminence.

Dx?

NISM?

What 7 conditions predispose to this?

Dx - carpel tunnel syndrome

NSIM - wrist splinting, if this doesn't work try corticosteroid injection

7 conditions that predispose to this include:

  -obesity

  -DM

  -pregnancy

  -hypothyroidism

  -RA

  -CT disorders

  -aromatase inhibitor (anastrozol) use

For HIV positive patients, when do you begin prophylaxis against the following, and what drug is used?

1) Pneumocystis carinii

2) Myocobacterium avium intracellularae

3) CMV

4) Toxoplasmosis

5) Histoplasmosis
Qid 2268

1) PCP - CD4 <200 or pt has Hx of oropharygneal candidiasis -> TMP/SMX

2) MAC - CD4 <50 ->  azythromycin (Tx is clarithomycin + ethambutol)

3) CMV - CD4 <50 -> gancyclovir (foscarnet if resistant)

  -recall CMV MCly causes retinitis, but can also cause esophagitis and encephalitis

4) Toxo - CD4<100 -> TMP-SMX

5) Histoplasmosis - CD4 <100, and if pt lives in endemic area -> intraconazole

For HIV positive pts, what 3 vaccinations should you make sure they get, and how often?

Why vaccines can they NOT get?

Make sure they get:

-Pneumococcal polysaccharide vaccine Q 5-6yrs

-Influenza vaccine (IM) yearly

-Hep B vaccine (if not already immune)

You CANNOT give HIV positive pts live vaccines:

-Small Pox        -Sabin polio

-Yellow Fever     -MMR

-Chicken Pox

What are common acute rxns a/w the following HAART drugs:

1) Protease Inhibitors (esp Ritonavir & Indinavir)

2) NRTIs (esp Didanosine and Abacavir)

3) NNRTIs (esp Nevirapine)

Which ones can cause pancreatitis?

1) Protease-Is

  -Cushing-like syndrome

  -Ritonovir - pancreatitis

  -Indinavir - crystal-induced nephropathy (ppt of drug in urine -> obstruction of urine flow)

2) NRTIs

  -Lactic acidosis

  -Didanosine - pancreatitis

  -Abacavir - hypersensitivity syndrome

3) NNRTIs

  -Steven-Johnson syndrome

  -Neviripine - liver failure (Never drink EtOH)

Pancreatitis = Ritonaivir, Didanosine, Zalcitabine (did zal have pancreatitis, right?)

Name the autoimmune disease a/w with the following auto antibody:

1) centromere                7) Microsomal

2) DNA topoisomerase I  8) Jo

3) Mitochondria              9) Helicase (Mi-2)

4) Transglutaminase       10) RNP

5) Smooth Muscle  11) Glutamate decarboxylase

6) Desmoglein

1) CREST

2) Scleroderma (topoisom = Scl-70)

3) Autoimmune Hepatiti

4) Celiacs (also endomyceal, gliadin)

5) PSC

6) Pemphigus vulgaris

7) Hashimotos

8 & 9) Poly/Dermato-myositis

10) Mixed CT disease

11) DMI

What are the 5 MCC of pancreatitis?

What is the best imaging technique for initially ID etiology of pancreatitis if gallstones are suspected?

What is the role of CT in evaluating pancreatitis?

5 MCC:

-EtOH (clear #1)    -Hyper-TG

-Gallstones (#2)     -Hyper-Ca

-Meds

-Best initial imaging if Gallstones suspected is RUQ Abd U/S b/c it has higher sensitivity (rule out) than CT 

-CT is used to confirm Dx, and assess severity and complications of pancreatitis

What are the 3 main steps (in order) in lowering potassium levels in someone with severe hyperkalemia?

Qid 3654, SU 306

1) Give IV calcium gluconate - Ca stabilizes resting membrane potential of myocardium (decreases excitability)

  -Use caution in giving Ca to pts on digoxin b/c hyper-Ca can predispose to digoxin toxicity)

2) Shift K+ intracellularly by giving:

  -Glucose + Insulin (most rapid)

  -Sodium Bicarbonate - alkylosis causes H/K shift (K goes into cell, H comes out)

  -beta2-agonist (albuterol)

3) Removal of K+ from body

  -Kayexalate - GI K+ exchage resin, absorbs K+ in colon, preventing K+ reabsorption

  -Hemodyalisis - most rapid and effective

  -Diuretics (furosemide)

50 y/o female comes in b/c of lethargy, confusion, anorexia, n/v. Last year she was Dx'd with RA, and is currently taking indomethacine and methotrexate for it. Vitals and exam is wnl. Labs show: Na 122, K 3.7, HCO3 22, Glucose 90, BUN 9, Uric acid low. Serum osm 265 (nml 275-295), urine osm 500.

Dx?               What's the classic volume & natremic

Tx?                status of these pts?

Causes of this condition?

Dx - SIADH, classically they are hypotonic hyponatremic with euvolemia

Tx - d/c NSIAD as it may be cause of SIADH

Causes:

  -Neoplasms        -Ventilator use

  -CNS disorders   -Post-op state/Pain

-Meds (vincristine, SSRIs, oxytocin, morphine, cyclophosphamide, carbamazepine, NSAIDs)

  -Pulm disorders

When does the USPTF recommend you begin screening for colon cancer?

What if the pt has a first degree relative with Hx of colon cancer?

What are the 5 different ways you can screen for colon cancer?

-Begin screening pts with avg risk at age 50

-Begin screening pts with first degree relative affected with colon cancer at age 40

-5 different ways to screen for colon cancer:

  -fecal occult blood testing (FOBT)

  -Flexible sigmoidoscopy

  -a combination of the two listed above

  -colonoscopy

  -double contrast barium enema

36 y/o female comes in b/c of malaise, high-grade fever, and chills. She has pain in multiple joints. She is an IV drug abuser and prostitute. Exam shows multiple pustules on extensor surfaces of forearms, joints show no redness/swelling, but R-ankle is tender. Blood cultures are negative.

Dx?

Qid 2230

Dx - disseminated gonococcemia

-Dissemination is most likely to occur during menstrual periods

-Blood Cx are commonly negative due to specific growth requirements of the strain of gonoccoci that disseminates

-Acute HIV syndrome presents with fever, malaise, LN, pharyngitis, myalgias, arthralgias, and cutaneous manifestations like those of viral exanthems (morbiliform macules), NOT pustules!

22 y/o female presents with fever, HA, n/v, myalgias. She has no significant PMHx aside from menorrhagia and is sexually active with 1 partner. Exam shows redness and desquamation over trunk and extremities. Blood and urine Cx negative. Pregnancy test negative. She says shes highly absorbent tampons, and has had one in place for 2 weeks because of heavy bleeding.

Most likely Dx?

Dx - Toxic Shock Syndrome

-caused by staph aureus TSST toxin ("scalded skin sydnrome")

How do you distinguish between CMV retinitis and HSV/VZV retinitis in AIDs pts?

CMV - painless loss of vision; fundoscopy shows fluffy or granular lesions located near the retinal vessels and associated hemorrhages

HSV/VZV - initial Sx are keratitis and conjunctivitis with eye pain, f/b rapidly progressive bilateral necrotizing retinitis (aka "acute retinal necrosis syndrome") and vision loss. Fundoscopy shows widespread pale, peripheral lesions and central necrosis of retina

70 y/o male comes in b/c of cough. CXR shows no infiltrates except for RU lobe lung lesion found incidentally. Chest CT w/ IV contrast shows 1x2 cm round lesion in RUL. He is admitted, and on day 3 develops renal failure. He has Hx of DMII, HTN, and CAD.

Dx?                        Pts at risk?

Pathogenesis?         Tx?

Dx - contrast induced nephropathy

Pathogenesis - contrast causes renal vasoconstriction and tubular injury 

Pts at risk - pts with Hx of DMII (+/- chronic renal insufficiency) are at increased risk

Tx - CIN is avoided by giving adequate IV hydration w/ isotonic sodium bicarb, and administration of N-acetylcysteine

  -This could have been avoided by using a non-ionic contrast agent

How does vertebral compression fracture present in an elderly woman?

How about Lumbargo (Lumbosacral strain)?

VCF - acute onset of severe back pain, with no history of trauma. On exam they may have spinal (NOT paravertebral) tenderness. Pts who are on corticosteroids are at increased risk!!!

Lumbargo - less dramatic onset, related to physical strain, usually have paravertebral mm tenderness rather than spinal tenderness

What is the pathophysiology behind Bartter and Gitelman syndrome?

How do you distinguish it from Conn's syndrome or a Renin secreting tumor?

Bartter and Gitelman syndromes are - deficiency in sodium and chloride reabsorption in the thick ascending limb and distal convoluted tubules (respectively).

-Both classically present with polyuria, polydypsia, and growth/mental retardation

-Pts will have elevated plasma renin & aldosterone levels (to compensate for Na and H2O loss)

-Pts will have hypokalemia, metabolic alkylosis, and urine chloride will be markely elevated (>40mEq/L)

-Unlike Conn's syndrome and pts with Renin secreting tumors, Bartter's pts are normotensive and have normal serum sodium

NPH - ataxia and incontinence are early signs of disease, and dementia occurs later

Alzheimers - memory loss, language difficulties, and apraxia are early signs of disease, whereas incontinence occurs later

What are the charateristic findings of:

-Pick's Disease

-Lewey Body Dementia

-Multi-infarct Dementia

Which one of these is sensitive to neuroleptic medication?

-Pick's -characteristically have personality changes (euphoria, disinhibition, apathy), compuslive behaviors (peculiar eating habits), hyperorality, and impaired memory. Visual and spatial functions are intact.

-LBD - initially visual hallucinations predominate. Pts also have fluctuating cognative impairment, and features of Parkinsonism not responsive to DA agents

   -Sensitive to neuroleptics (e.g. taking haloperidol for agitation can cause worsening of mm rigidity)

-MID - sudden deterioration in congative, motor, and sensory perception. RFs include old age, male, black, smoking, HTN, DMII, vasculitis

Define erysipelas. Organism responsible?

Where/how does it typically present?

RFs?

Tx?

-Its a cellulitis confined to dermis and lymphatics, caused by streptococcus pyogenes NOT staph

-Classically presents as well-demarcated, fiery red, painful lesion, on LE and face. High fever and chills may be present

-RFs - lymphatic obstruction (mastectomy), local trauma or abscess, fungal infections, DM, EtOHism

-Tx - uncomplicated cases iwh IM or oral PCN or erythromycin, complicated Tx like cellulitis (IV oxicillin or cephalosporin)

Define suppurative hidradenitis.

Where and now does it present?

Its a chronic follicular occlusive disease MC affecting interiginous skin (axilla, groin, inframammary region)

-Presents as painful inflammatory nodules and draining sinus tracts that can last weeks or longer

What are the skin findings of Rosacea?

What are common precipitating factors?

What's the Tx for Rosacea?

Skin findings - erythema, pustules, papules, telangiectasias on face. Unlike acne, there are no comedones

Worsens with alcohol use, hot beverages, extreme temperature change (including drinking/eating hot drinks/foods), and emotional stress

Tx - topical metronidazol for induction, and systemic AbX (tetracycline) for maintenance

27 y/o male with no PMHx presents with fever, chills, and malaise for 3 days. He noticed a painless ulcer on his penis 3 weeks ago which never resloved. Exam shows tender inguinal LAN on the right.

Dx, and how do you Dx?

Tx?

What complications might arise if left untreated?

Dx: Lymphogranuloma venereum (C. trachomatis)

To Dx: serologic tests (complement fixation, immunofluorescence)

Tx: PO doxycycline

Complications:

  -proctocolitis

  -perianal fissures

  -rectal stricture

  -obstructed lymphatics -> elephantitis of genitals

25 y/o is brought in b/c of confusion, hallucinations, and generalized tonic-clonic seizures. PMHx significant for illicit drug use. Temp is 104. Neurologic exam shows upgoing plantar reflexes bilaterally. CSF analysis shows low glucose, high protein, high WBCs w/ mosty lymphocytes, gram stain negative.

Dx?

NSIM?

Dx - HSV encephalitis; commonly p/w:

  -bizzare behavior

  -hallucinations

  -impaired mental status

  -aburpt onset of fever

NSIM - IV acyclovir whenever suspicion of SHV encephaltitis

Confirm Dx with PCR of CSF

What is the initial imaging for any pt with jaundice?

What if this is not conclusive?

When is Percutaneous Transhepatic cholangiogram indicated?

-Abd U/S

-If non-conclusive -> abd CT (effective in detecting bile and pancreatic duct dilation, mass within pancreas)

-If both U/S and CT non-conclusive -> ERCP

PTC indicated for pts with previously ID'd bilary tract dilation but are NOT candidates for ERCP. PTC also allows for therapeutic drainage of infected bile (cholangitis pts), extraction of biliary tract stones, dilation of benign biliary strictures, and stent placement across malignant strictures

How does opioid intoxication present with respect to:

-Resp. Rate

-Pupil Size

-Heart Rate

-BP

-Bowel Sounds

-Mental Status

-RR - decreased (is best predictor of intox, and is frequent cause of mortality)

-Pupil size - meiosis (constricted)

-HR - bradycardia

-BP - hypotension (due to histamine release)

-Bowel sounds - decreased (opioids cause constipation)

-Mental Status - depressed

-Hypokalemia 2/2 transcellular shift (H+ moves out of cells, K+ moves into cells) -> mm weaknes

-Hypocalemia - less H+ in serum allows for more negative charges to be available on albumin -> albumin binds MORE Ca2+ -> less FREE Ca2+ --> tetany and parasthesias

Metabolic alkylosis 2/2 increased Na/K exchange from the principle cells of collecting duct -> increased lumenal K+ available for K/H exchange by the intercalated cells -> increased H+ excretion

Hypokalemia (see above mechanism) + increased K/H transcellular shift throughout the body in response to metabolic alkylosis

Mild hypernatremia due to:

  -increased ANP release in reponse to increased BP -> vasodilation of renal efferent arteriols -> increased RBF and GFR -> increased sodium excretion

Hypocalcemia - decreased plasma H+ -> increased negative charges on albumin -> increased binding of albumin to Ca2+ -> less FREE Ca2+

Note: Generalized Edema is NOT seen in hyperaldosteronism!

It depends on the pts hemodynamic status and whether they respond to fluids

  -If pt is hemodynamically stable -> CT abd

  -If pt is hemodynamically unstable assess response to IV fluids

      -If responsive to fluids -> CT abd

      -If unresponsive to fluids -> Ex Lap!

58 y/o male with widely disseminated small cell carcinoma of lung is found to have hypotension, decreased serum cortisol, and profound electrolyte disturbances.

Dx?

What would you expect his Na, K, and acid/base status to be?

Dx - Addison's Disease 2/2 metastasis to adrenal gland (adrenal is MC organ metastisized to by lung cancer). The tip off is decreased cortisol

-This pt would have:

  -Hyponatremia

  -Hyperkalemia

  -Metabolic Acidosis

A 58 y/o male with recently diagnosed small cell carcinoma of lung is brought in b/c of metal status abn. CT of head is nml.

Dx?

A 58 y/o male comes in b/c of profound muscle weakness and numbness around his lips. Vitals are BP 180/100, HR 80, T 98. On exam he as a positive Chovtec sign.

What's most likely Dx?

Dx - Conn's syndrome

-Increased aldosterone results in:

  -Increased sodium/water reabsorption -> HTN

  -Increased potassium excretion -> hypokalemia -> mm weakness

  -Increased H+ excretion -> metabolic alkylosis -> increased capacity for Ca2+ to bind albumin -> decreased free Ca2+ -> functional hypocalcemia -> perioral parasthesias, tetany, positive Chovtec and Treusseau sign

A 15 y/o girl who was recently treated for Lyme's disease presents with sunburn appearing rash shoulders, face, and forearms. Her mother says she makes sure to put suncreen on her daughter when going outside.

What's the most likely cause?

What's the best treatment for a pt with Sphincter of Oddie Dysfunction?

ERCP sphincterotomy

Note: Calcium Channel Blockers and Nitrates are NOT efficacious

Name 4 infections that can result in primary adrenal insufficiency.

-CMV

-Fungal infections

-TB

-N. meningitis (Friedrichson Waterhouse Syndrome)

Recall, adrenal insufficiency can also result from autoimmune destruction (esp if pt has Hx of autoimmune diseseases) and neoplastic infiltration (MC from primary lung and breast cancer)

67 y/o male comes in b/c of malaise, fatigue, anorexia, n/v. Pt was recently Dx'd with pneumonia and is on AbX. PMHx is significant for HTN, HL, and DVTs, for which he takes HCTZ, simvastatin, and coumadin. Clinical chemistry shows hyponatremia, hyperkalemia, low HCO3, low glucose.

What's most likely Dx?

Pathophs?

Dx - adrenal hemorrhage -> Addison's

Pathophys:

  -acute stress (pneumonia) can cause increase in ACTH levels -> increases adrenal blood flow. In pts who are chronically anticoagulated (as in this pt), this increased blood flow can reslt in adrenal hemorrhage -> adrenal necrosis

Qid 4305

29 y/o male presents with malaise, fatigue, n/v, and anorexia. Labs show hyponatremia, hyerkalemia, and metabolic acidosis. Serum cortisol is noted to be low. This has never happened before. Pt has no personal or family Hx of autoimmune disorders, including Addison's disease. CT abd shows bilateral enlargement of adrenal glands w/o calcifications.

What may be the Dx?

Adrenoleukodystrophy

-This is due to build up of long chain fatty acids w/in adrenal glands, and is a congenital disorder. Females serve as carriers, but do not manifest the disease.

-Typically affects young males

Pt comes in saying the took a bottle full of acetaminophen.

NSIM?

How long do you wait to administer N-acetylcystine?

NSIM - administer activated charcol and obtain acetaminophen levels at 4 hours after ingestion (b/c recent data shows no matter how much they took, the acetaminophen levels in the blood will be detectable starting 4 hrs post-ingestion, so getting blood levels before this time is useless!). If acetaminophen levels are high enough, then you can give N-acetylcystine

-N-acetylcystine should be given within 8 hrs post-ingestion

When a pt is on a ventilator, what settings effect pO2?

pCO2?

What FiO2 do you initially set the ventilator?

What do you do if pt pO2 is too high? Low?

Qid 3433

-pO2 is influenced by FiO2 and PEEP level

-pCO2 is influenced by minute ventilation, which is a function of respiratory rate and tidal volume

-Ventilator is usually initially set at 80% (0.8), pending results of the first blood gas analysis; the vent can then be adjusted accordingly

-If pt's pO2 is too high -> turn down FiO2 to non-toxic levels (<60)

        -Recall, nml FiO2 at sea level is 21% (0.21)

-If pt's pO2 is too low -> either turn up FiO2, or increase PEEP

A neurological cond'n caused by inflammation of carotids and vagus. The pain is sharp and localized to carotid artery distribution in neck

-MRI is sometimes req'd

45 y/o male is brought in b/c of leg weakness and lower back pain for past several days. He has DMII and HTN. Vitals: temp 101, HR 100, BP 150/100. He smells of urine. Exam shows tenderness over palpation to L2-L5. Neuro exam shows decreased sensation to temp and touch.

Dx?

NSIM?

Tx?

Dx - epidural abscess - enclosed infections in the epidural space. Characterized by back pain, f/c, and leukocytosis. Abscess can restrict blood flow to spinal cord as it enlarges

NSIM - MRI of spine

Tx - immediate surgical debridement

Which two statins are the most potent at lowering LDL?

Which two have highest rates of myopathy?

Which two have the lowest rates of myopathy?

-Most potent at lowering LDL: rosuvastatin and atorvastatin (RAke down LDL)

-Most myotoxic: simvastatin and lovastatin (SLow muscles down)

-Least myotoxic: pravastatin and fluvastatin (PH/Faster muscles)

A 50 y/o male immigrant comes in with hemoptysis, night sweats, and fever. CXR shows upper lobe nodule. Labs show hypercalcemia.

Dx?

Source of hypercalcemia?

Dx - pulmonary TB

-Hypercalcemia is 2/2 granuloma formation causing overproduction of calcitriol (active Vit D)

Recall: Macrophages in the granuloma increase their production of 1-alpha hydroxylase enzyme, so more 1,25-hydroxy-VitD is made

32 y/o male comes in b/c of f/c and sob for past 3 days. He has cp on deep inspiration. He's HIV positive, and his CD4 one month ago was 378. He's an IV drug abuser. Temp is 102. Exam shows scattered crackle's in both lungs. CT chest shows multiple nodular lesions w/ small cavities scattered from apex to base of both lungs.

What organism is culprit?

Staph aureus

-Pt likely had tricuspid endocarditis 2/2 staph aureus introduction via IV drug abuse, with septic emboli to lung

Recall, pts with Hx of IV drug abuse, skin infections, and pts with infected medical devises (prosthetic valves) are more likely to have staph bacteremia

TB on the other hand would present with a more chronic course and CT chest would not show lower lobe involvement

37 y/o male with Hx of small cell carcinoma of lung presents with back pain. Labs show hypercalcemia, nml PTH levels, and CXR shows lytic bone lesions on thoracic spine.

What's factor is being released from tumor cells that causes these lesions?

Cytokines

-Tumors that metastisize and cause osteolytic lesions do so by production of cytokines such as IL-1 and TNF.

-The most frequent tumors to do this are breast and lung cancer

Recall: tumors that produce PTHrP are: squamous cell carcinoma of esophagus and lung, and Renal Cell Carcinoma

When a pt get's bitten by a dog causing puncture of skin, what are the guidelines for post-exposure rabies prophylaxis?

Qid 3002

In any dog bite, an attempt to capture dog is made:

1) IF dog not captured, assume it's rabid, and give pt post-exposure prophy

2) If dog captured and doesn't show signs of rabies, it's kept for obstervation for dev't of rabies for 10 days. If dog develops features -> post-exposure prophy. Dog's Dx is confirmed by fluorescent anti-body exam of brain

3) Post-exposure prophy should be started immediatley for expsosures invovleing head and neck

Post-exposure prophy = active AND passive immunization for rabies

68 y/o male w/ DMII and CAD undergoes total knee replacement. On post-op day 5 he complains of new onest abd pain. Over past 12 hrs, he voided 200 mL. Review of chart shows infrequent recording of fluid input/output over past 4 days. Vitals are nml. Labs show hyponatremia, and BUN 40, creat 4.2.

Dx?      Pathophy?

NSIM?

Dx - acute kidney injury

Pathophys - bladder distension under general anesthesia or use of epidural anesthesia can lead ot inefficient detrusor contraction and acute urinary retention. As males over 50 have high incidence of BPH, this is likely exacerbating the detrusor inactivity.

NSIM - place a foley catheter to restore nml UOP and resolve/prevent hydronephrosis, tubular atrophy, and renal injury. If this doesn't resolve oliguria, alternative AKI etiologies (intrinsic or prerenal) must be considered

Ipratropium (cholinergic antagonist)

Albuterol nebulizer

Systemic steroids

60 y/o male with Hx of HTN and DMII comes in with fatigue, urinary frequency, and blurred vision. He had a URI 1 week ago. BP is 160/90, and HR 90. His mucus membranes are dry. Urine is positive for glucose but not ketones.

Dx?              Tx?

Pathophys?

Qid 4516, SU 189

Dx - nonketotic hyperosmolar syndrome

Pathophys - physiologic stressors (like infection) -> increased catecholamines and cortisol -> increased gluconeognesis and glycogenolysis and worsening of insuling resistance -> massive hyperglycemia -> osmotic diuresis -> dehydration

 -The pt has diuresis -> hyperosmolar serum

 -Blurred vision is caused by myopic increase in lens thickness and intraoccular hypotension 2/2 hyperosmolarity

Tx - IV fluid replacement

What two weird things will you see in a pt with Legionella pneumonia?

Tx?

SU 348

GI symptoms (abd pain, loose stools, etc)

Hyponatremia

Tx: quinolone or macrolyde

What conditions can you see pulsus paradoxus in?

Qid 4771

Cardiac diseases - tampenade, pericardial effusion

Pulmonary diseases - tension pneumo, severe asthma

  -In asthma, elevated intrathoracic pressures during inhalation are thought to exacerbate compression of LV -> pulsus paradoxus

-PaO2 55 mmHg, OR

-O2 sat <88%, OR

-PaO2 55-59 mmHg + polycythemia (Hct >55%) or evidence of cor pulmonale

The peak airway pressure (max pressure during respiratory cycle) is equivalent to what?

Elastic pressure (aka plateau pressure) is equivalent to what?

-Peak airway pressure = relative pressure + elastic pressure + PEEP

  -Think of it this way: Relative pressure is the pressure w/in airways, elastic pressure is the intrinsic collapsing pressure the lung exerts, so if the ventilator set to match the sum of ONLY those two pressures -> inward pressure would match outward pressure -> no airflow would occur. The addition of PEEP forces causes air to flow into the lung, and at the point where the PEEP is greatest, the elastic recoil of lung is greatest, as is the pressure w/in the airways

-Elastic pressure (pressure at the end of inspiratory hold maneuver) = lung elastance x volume of gas delivered. Thus, as compliance (inverse of elastance) decreases, elastic pressure increases

Why do you not get pulmonary edema in during a state of low albumin?

Qid 4591

Because:

1) Alveolar capillaries are more permeable to albumin at baseline than most other capillary systems -> decrease albumin gradient

2) Lymphatic drainage is greater in lung than most other parts of body (especially skeletal mm)

Qid 4591

According to the CDC, what vaccines are required for travel to North Africa?

How about Sub-Saharan Africa?

How about equitorial South America?

How about Asia?

Qid 4080

-North Africa - Hep A, Hep B, Typhoid, Polio booster

-Sub-Saharan Africa - Yellow fever, Meningiococcal

-South America - Yellow fever

-Asia - Meningoccocal

Note:

  -Hep A is MC vaccine-preventable infectious disease

  -Rabies vaccinatino is not req'd for travel to any country

22 y/o male comes in b/c of R-eye pain, photophobia, blurred vision, tearing, and redness for 4 days. He's had 2 prior episodes before in past 5 years, both occuring when he had the flu. He said he says he went to the beach the past few days with friends and got lots of sun. Exam shows corneal vescicles.

Dx?  Tx?

Dx - Herpese Simplex keratitis

  -Typically precipitated by excessive sun exposure, outdoor occupation, fever, or immunodeficiency

  -Exam may how corneal vescicles and dendritic ulcers

Tx - antiviral therapy (oral or topical)

Note: Herpes zoster ophthalmicus (VZV infection) usually occurs in elderly, and p/w fever, malaise, w/ periorbital burning, itching. Exam may how vescicular rash in V1 distribution

High dose acyclovir within 72 hrs after eruption of rash

Recall, HZO p/w:

  -itching, burning, rash over periorbital region (ophthalmic brach of CN V)

  -fever, malaise, generalized painful LN

How do you differentiate Herpes simples keratitis from Herpes zoster opthalmicus?

Qid 2854

HSK

  -p/w pain, photophobia, blurred vision, tearing, and redness

  -precipitated by excessive sun exposure, fever, immunodeficiency

HZO

  -p/w fever malaise & periorbital burning/itching

  -usually presents in elderly

  -vescucular rash may be present in distribution opthalmic division of CN V

BOTH may p/w dendriform corneal ulcers!!!

22 y/o p/w with eye pain and redness. She wears contacts. Exam shows corneal haziness w/ central ulcer and adjacent stromal abscess. Dx?

33 y/o agricultural worker comes in b/c of eye pain and redness. He said some debris blew across his eye 2 days ago, which is when this all startedExam of cornea shows multiple stromal abscesses. Dx?

Young contact wearer may have had a corneal abrasion  -> bacterial keratitis

Agricultural worker may have suffered a corneal injury -> Fungal keratitis

Shy Drager (aka multiple system atrophy) is characterised by:

  -Parkinsonism

  -Autonomic dysfunction (postural hypotension, abn sweating, disturbance of bowel/bladder control, abn salivation/lacrimation, impotence, gastroparesis)

  -Widespread neurological signs (cerebellar, pyramidal, LMN)

Riley Day (familial dysautonomia) is characterized by:

  -gross dysfunction of the ANS w/ severe orthostatic hypotension

Shy Drager should be suspected in any pt with Parkinsonism who experiences orthostatic hypotension, impotence, incontience, or other autonomic Sx.

A 55 y/o male comes in b/c of numerous falls. Exam shows masked facies, bradykinsia, resting tremor, dry mouth/skin. He also complains of erectile dysfunction, urinary incontinence, and dizziness when standing up.

Dx?

Qid 4130

45 y/o woman comes in b/c of reddness over her face. Exam shows telangiectasias, pustules, and papules. She says this usually happens when she goes out in hot sunny weather.

Dx?

Tx?

Qid 2776, SU 396

Dx - Rosacea

Tx - topical metronidazol BID for several months, then systemic AbX (tetracycline) for maintenance therapy. Telangiectasias require laser surgery

This typically effects fiar skinned, light haired/eyed individuals.

What is the standard treatment for acute pancreatitis?

What if pt has acute necrotizing pancreatitis?

What's the only way to distinuish between steiral and infected necrotizing pancreatitis

-Analgesics (meperidine and fentanyl favored over morphine)

-IV fluids to maintain intravascular volume

-NPO +/- NG suction

Note: These three things are done for pts with acute cholecystitis as well!

Acute necrotizing pancreatitis:

  -enteral feeding

  -decontamination of gut w/ non-absorbable AbX

  -Systemic AbX

-CT guided percutaneous aspiration of pancreatic inflammation w/ Gram stain/culture of aspirate distinguishes sterile from infected nec. pancreatitis

What type of arthritis p/w "sausage digits", nail involvement, predominantly DIP involvement, morning siffness, well-demarcted red plaques w/ silvery scaling on dorsum of each hand?

Tx?

Psoriatic arthritis

Tx - NSAIDs, anti-TNF agents, and methotrexate

Note: systemic corticosteroids are relatively contraindicated!

19 y/o male is brought in by his parents b/c of paranoia. Vitals are BP 180/96, HR 120. He appears euphoric, and eye exam shows conjunctival injection.

Dx?

What 3 suggestions might you give a pt to avoid straining his/her back when lifting objects?

What mov't(s) should he/she avoid?

1) Exercise of supporting mm (including abd mmm)

2) Appropriate sleeping posture (avoid sleeping on stomach)

3) Keep back straight while lifting object

Avoid twisting and bending

What's the treatment for overflow incontinence?

Stress incontinence?

Urge incontinence?

Intermittent catheterization, cholinergic agents (bethanachol), alpha-blockers (terazocin, doxazocin)

Stress intontinence (in order of what to do first) -Kegel exercises, estrogen repalcement therapy, use of a Pessary, surgery (urethropexy)

Urge incontinence

-antocholinergic agents (oxybutylin), TCAs (imipramine), bladder training (behavioral therapy),

40 y/o female comes in b/c of f/c, and pain oner neck. Exam of neck shows erythema and induration. Serosanguinous fluid is draining from a small defect in skin near center of lesion. There is no crepitus. Histologic analysis shows gram positive branching bacteria.

Dx?

Tx?

Dx - Actinomyces israelii

  -causes infection of cervicofacial, thoracic, or abd region causing draining fluid contining sulfur granules (appears yellow)

Tx - high dose PCN for 6-12 weeks

Note: Surgical debridement is req'd for complicated cases; however debridement alone w/o AbX is rarely successful

50 y/o female comes in b/c of fever and neck pain. Exam of neck shows erethyma and induration, and a draining sinus tract. Gram stain is negative, but acid fast stain shows bacilli.

Dx?

CHF - commonly have bibasilar crackles, sometimes have wheezing (cardiac asthma) and decreased breath sounds at bases (2/2 pleural effusions from CHF). ABG will show hyoxia, hypocapnea, and respiratory alkylosis

COPD - commonly have widespread bilateral wheezes, and ABG will show hypoxia and respiratory acidosis

1) Fluid overload not responsive to medical Tx

2) Hyper-K not responsive to medical Tx

3) Uremic pericarditis

4) Refractory metabolic acidosis

Relative:

1) GFR <10 mL/min (<15 mL/min in DM pts)

2) Creatinine >8 mg/dL (>6 in DM pts)

3) Severe uremic Sx (seizure, coma)

  Note: Decision to start HD is not based soley on BUN and creat

Contraindications:

1) Debilitating chronic disease

2) Severe irreversible dementia

Lower lobes

Panacinar

Recall in AAT, pt may hve Hx of neonatal hepatitis. AAT can progress in adulthood to cirrhosis and liver failure (2/2 buildup of non-functional AAT w/in hepatocytes)

Chronic smoking results in emphysematous change to upper lobes of lung and results in centriacinar emphysema

Describe the common findings in:

Neurofibromatosis I

Neurofibromatosis II

NFI: cafe au lait spots, neurofibromas, CNS tumors (gliomas, meningiomas), Lisch nodules, macrocephaly

NFII: cafe au lait spots, bilateral acoustic neuromas, multiple meningiomas, cataracts

   -NF II pts are much less likely to have neurofibromas

A 72 y/o Caucasian female complains of frequent HA, fatigue, and WL. Her shoulder mm feel stiff in the morning. A tender cord is palpated in R-temporal area. ESR is 80.

Dx?

What are 2 serious complications of this disease?

Dx - Temporal arteritis

2 Serious complications include:

  -Blindness - for this reason, pt should immediately be placed on high dose steroids

  -Aortic aneurysms - for this reason, pts should be followed w/ serial CXRs

What 3 vaccines are recommended for all HIV-infected pts?

What vaccine should be given to men who have sex with men?

What vaccines are under the same guidlines as all other adults?

HIV pts - influenza, Hep B, and S. pneumoniae

MSM - Hep A

Vaccines that fall under the same guidelines for both nml and HIV-infected adults include:

  -Meningococcal

  -HPV

  -H. flu type B

  -tetanus + diphtheria (Td) booster Q 10 yrs

A 40 y/o ale comes in b/c of blurred vision in his R eye. Exam shows conjunctival injection, and fundoscopic exam shows papillary edema. He says he had a penetrating injury to the L eye a few weeks ago, which led to vision loss in that eye.

Dx?

Pathophysiology?

Dx - Sympathetic ophthalmia (aka spared eye injury)

Pathophys: Some antigens contained w/in eye are protected from immunologic recognition by natural barriers. Breaking these barriers (such as in penetrating eye injury) -> uncovering of "hidden" Ags. An immune response against these Ags can involve autoAbs as well as cell-mediated Rxn

This can present with anterior uveitis, panuveitis, papillary edema, blindness

An 80 y/o comes in b/c of signs and symptoms of BPH. He has HTN, for which he take metoprolol. DRE shows smooth, firm enlarged prostate w/o induration or asymmetry. UA shos no proteinuria or hematuria. Creatinine is 2.1 mg/dL, and one year ago it was 1.2 mg/dL.

NSIM?

What's best choice for long term management?

NSIM - abdominal U/S to assess for hydronephrosis

  -If hydronephrosis is present -> place a Foley to improve urinary obstruction

-Surgical intervention (transurethral resection of prostate) is a good option for improving pt's urinary obstruction over the long term

What is the definition of chronic hepatitis?

What's the best means of evaluating a pt's hepatic injury if the have acute hepatitis?

Chronic hepatitis?

Chronic hepatitis is hepatic injury for at least 6 months

  -As far as infectious hepatitis, persistant viral serology for at least 6 months -> chronic

Acute -LFTs and viral serology

Chronic - liver biopsy

1) Hypocalcemia - b/c of decreased release of PTH and bone resistance ot PTH when Mg is low

2) Hypokalemia - b/c Mg is important cofactor for K+ uptake and maintenenace of intracellular K+ levels

A 22 y/o w/ DMI is brought in b/c of n/v, and altered mental status. She is found to be in DKA and is treated appropriately. Several days after discharge, she returns w/ f/c, and severe sinus pain. Exam shows black eschar of frontal and maxillary sinuses. She is again treated appropriately. On day 2 of admission, she shows marked fatigue, pallor, and pain/burning near site of IV line.

Dx?

Pathophysiology of latest findings?

Course: This pt had DKA c/b mucormycosis, which was treated with amphotericine/flucytosine induction.

Dx - amphotericin B toxicity

Pathophys:

  -anemia - due to destruction of EPO secreting cells in peritubular capillaries of kidney

  -IV phlebitis - unkown etiology, but is a SE of ampho

  -Nephrotoxicity b/c ampho causes:

     1) Renal vasoconstriction -> decr GFR

     2) Direct toxicity to tubular cells resulting in

           -Type 1 (distal) RTA, and hypokalemia/hypomagnesemia (ALWAYS follow pt's K+ and Mg+ when administering amphotericin)

           -Acute tubular necrosis

78 y/o male is brought in b/c of sudden onset hemiparesis and aphagia 45 mins ago. CT w/o contrast shows no acute bleed, and tPA therapy is given. Blood pressure measurement on admission was 200/120, and it's now 225/120.

NSIM?

At what BP level is Tx indicated?

Qid 2288

This is HTN a/w acute period of stroke.

NSIM - calcium channel blockers (nicardipine) and ACE inhibitors.

  -IV nitroglycerine and nitroprusside are NOT indicated!

Tx is only indicated when systolic >220, diastolic >130

What visual disturbances do pts with retinal detachment typically complain of?

What might you find on opthalmoscopic exam?

What is retinal detachment caused by?

Tx?

Pts complain of photopsia (flashes of light) and floaters (spots in visual field). Most classic is a "curtain coming down over my eyes" (this is also seen in amurosis fugaue)

Ophthalmoscopic exam may show retinal tears and/or grey, elevated retina.

Retinal breaks are caused by myopia or trauma -> fluid seeps in and separates retinal layers

Tx: Laser therapy and cryotherapy; these create permanent adhesions btwn neurosensory retina, retinal pigment epithelium, and choroid

What visual disturbance do pts with choroidal rupture complain of?

What might you see on opthalmoscopic exam?

What is choroidal rupture usually caused by?

Pts may complain of central visual defect/black spot (scotoma, also seen in optic neuritis) and/or blurred vision.

Exam may show retinal edema, hemorrhagic detachment of macula, subretinal hemorrhage, and crescent-shaped streak concentric to optic nerve

Caused by blunt ocular trauma

What visual disturbances do pts with central retinal artery occlusion complain of?

What might you see on ophthalmoscopic exam?

Pts complain of sudden painless loss of vision in one eye.

Exam may show pallor of the optic disk, cherry red fovea (b/c fovea get's its blood supply from choroidal vessels, which are not occluded), and boxcar segmentation of blood in retinal vv

What visual disturbances do pts with proliferative diabetic retinopathy complain of?

What might you see on ophthalmoscopic exam?

What retinal abnormality are these pts at risk of developing?

Pts are initially asymptomatic, and then later complain of decreased visual acuity.

Exam may show neovascularization (classic finding) 2/2 chronic microvascular insufficiency, vitrous hemorrhage, and macular edema. These changes may lead to retinal detachement.

Remember, retinal involvement in DM begins with non-proliferative retinopathy, where BM glycosylation -> increased permeability and BV weakness -> microaneurysms and macular edema and plexiform exudates. This then over time progresses to proliferative retinopathy as described above. See Robbins pg 186 for more info

What visual disturbances do pts with exudative degeneration typically complain of?

What might you see on ophthalmoscopic exam?

What retinal abn are these pts at risk of developing?

Pts complain of painless, progressive, blurring of central vision, which can be acute or insidious. It occurs bilaterally. Testing may reveal central scotoma (also seen in choroidal rupture, and optic neuritis)

Exam may show growth of abn vessels in retinal space

Can lead to retinal detachment

1) Mucocutaneous signs (each counts as one)

  -Butterfly rash

  -Photosensitivity

  -Oral or nasopharyngeal ulcers

  -Discoid rash

2) Arthritis (like RA it effects MCP and PIP, but unlike RA it is non-deforming)

3) Pericarditis, pleuritis

4) Hematologic disease - hemolytic anemia w/ reticulocytosis, leukopenia, lymphopenia, thrombocytopenia

5) Renal disease - proteinuria > 0.5 g/day, casts

6) CNS - seizures, psychosis

7) Immunoligic manifestations - positive LE prep, false positive test for syphilis, anti-dsDNA, anti-Sm Ab

8) ANAs

40 y/o female p/w for her first visit rash on shoulders and back, and scales on hands. She claims to have had these findings for several years. She says she has difficulty climbing stairs b/c "her legs get tired." She was Dx'd with lung cancer 3 years ago, and is now in remission. Labs are signficiant for elevated serum CK.

Dx?

45 y/o African American female p/w malaise, fever, WL, cough, diffuse joint tenderness, and tender red nodules on both shins. Exam shows uveitis. CXR shows bilateral hilar adenopathy and parencymal infiltrates. ECG results suggest conduction block.

Dx?    

What peculiar lab findings might you see?

Tx?

Dx - Sarcoidosis

Labs - may see hypercalcemia (giant cells w/in the granulomas over-expressing 1-alpha-hydroxylase), elevated ACE levels

Tx - systemic steroids, if this fails -> methotrexate

It's related to insulin resistance:

-Increased fat accumulation (in hepatocytes and peripherally) over years contributes to insulin resistance -> increased rate of lypolysis and elevated levels of insulin.

-Intrahepatic FA oxidation -> oxidative stress and release of proinflammatory cytokines (TNF-apha) -> liver inflamation, fibrosis, cirrhosis

For influenza pneumonia, fill in the following:

1) Common present signs/Sx

2) CBC findings

3) UA findings

4) CXR findings

5) Fastest way to confirm Dx

6) Tx

1) f/c, malaise, cough, corzya

2) Mild leukopenia

3) Proteinuria

4) May be nml or may show intestitial or alveolar pattern

5) Confirm Dx by nasal swab for Influenza Ags

6) Tx: Neuraminidase inhibitors (oseltamivir and zanamivir), less effective are rimantidine/amantidine (block viral uncoating)

  -Tx MUST BE STARTED W/IN 48 HRS to be effective

According to USPSTF, what patient population should you routinely screen for AAA?

How do you screen for AAA?

-Anemia

-Metabolic abn, particularly hyperphosphatemia, and increased PTH levels

-Increased homocysteine levels - 2/2 impaired metabolism and decreased removal

-Accelerated atherogenesis - 2/2 enhanced oxidative stress 2/2 uremia and bio-incompatable renal replacement therapies

-Increased calcium intake (given to HD pts to correct for hyperphosphatemia) -> enhances coronary artery calcification

-Inhibition of NO - common finding in dialysis pts, can cause vasoconstriction and HTN

What's the differential Dx (8) for confusion?

What's the empirical Tx for confusion?

DDx: intoxication, infection, vitamin deficiency, endocrine disturbance (hypoglycemia), post-ctal state, hypertensive encephalopthy, or CNS hypoxia

Empirical Tx: administration of thiamine for Wernicke's encephalopathy, dextrose for hypoglycemia, supplemental O2 for hypoxia, and naloxone for opiate OD

A 22 y/o female is brought in by her mother sho says she found her in the bathroom very drowsy and confused. Her HR 50, BP 80/40. Exam shows diplopia, ataxia, and slurred speach. Her mother says she also found a bottle of her anti-anxiety pills next to her.

Dx?

Tx?

Dx - benzodiazapine overdose

Tx - flumazenil (GABA-R antagonist)

How do the following differ from each other as far as presentation, and progression of Sx throughout the day:

-Compression Fracture

-Ligamentous Sprain

-Lumbar Osteoarthritis

-Ankylosing Spondylitis

Compression fracture - focal vertebral pain, pain increases in intensity upon straining and coughing, pain occurs with sleeping

Ligamentous Sprain - Pain is perispinal (NOT directly over vertebrae), worse with movement, but pt feels best in morning

Lumbar OA - pain worsens as day progresses and is relieved with rest

Ankylosing Spondylitis - progressive limitation of back motion. Occurs in young men (<40 yrs). Typically worse in morning and improves throughout day

27 y/o female p/w decreased sensation and weakness of 4th and 5th digits for 3 days. She has no past medical Hx. She does not smoke or drink, is sexually active with one partner but uses protection. She says she woks  as a desk clerk and uses a computer for about 8 hrs a day.

What's most likely Dx?

Pathophys?

Dx - Ulnar Nerve Syndrome

-Pathophys - due to ulnar nerve entrapment at medial epicondyle 2/2 prolonged leaning while typing

What's the best treatment for the following complications of DM?

-Neuropathy

-Gastroparesis

-Foot ulcer

-Retinopathy

-Nephropathy

-Neuropathy - NSAIDs, tricyclic antidepressants, and gabapentin

-Gastroparesis - promotility agents like metoclopromide; also, low fat diet, and exercise

-Foot ulcer - prevention! Have them get regular foot care, regular podiatry visits

-Retinopathy - referal to ophtho for possible photocoagulation

-Nephropathy - ACE-I slows progression of microalbuminuria

When does the neuropathic pain of diabetes characteristically occur and when is it worse?

As the disease progresses, what happens to the pain?

It characterisitcally occurs at rest and is worse at night.

As the disease progresses, the pain subsides and finally disappears.

A 50 y/o male p/w pain and swelling of inner R-eye for 2 days. Exam shows tenderness, edema, and redness over medial canthus. Pressure over medial canthus expresses purulent material. Visual acuity is nml.

Dx?

Dacrocystitis

Usually caused by strep pygenes or staph

Responds to systemic AbX therapy

50 y/o male p/w acute onset mild-moderate L-eye discomfort, photophobia, and watery discharge Exam shows diffuse L-bulbar conjunctival injection.

Dx?

54 y/o male p/w pain and swelling of R-upper eyelid. Exam shows erethym, and tender fluctuant mass of R-upper eyelid, but no involvement of R-eyeball, and no decrease in visual acuity.

Dx?

A 49 y/o female p/w painless lower eyelid nodule and mild eyelid discomfort.

Dx?

What is the difference btwn angioedema and urticaria (hives) as far as:

-part of skin/subQ involved

-presenting Signs/Sx

Angioedem is characterized by localized edema of deep subQ -> non-pitting puffy skin with firm swelling that is more tender and burning or pruritic (b/c there are fewer mast cells/sensory nerve endings in deeper tissues)

Urticaria is characterized by pruritic, blanchging swelling of the dermis 2/2 mast cell release of histamine (causes local increase vascular permeability).

  -Urticaria is sort of analogous to eresypelas, which is infection of the dermis, whereas cellulitis is infection of the deep subQ

Live viruses are contraindicated in HIV pts. What is the exception to this rule, and what state must the pt be to receive that vaccination?

MMR vaccine is exception to this generaliatoin b/c measles is life-threating in HIV pts

MMR vaccine can be administered to HIV pts w/ CD4 counts > 200 micro-L and without Hx or evidence of AIDs defining illness

In fact, even if HIV pt has had MMR given to them as a child, they should receive a booster due to their increased susceptibility to measles

Mixed venous oxygen content!

-In vasodilitory shock, since the peripheral resistance arteriols are all dilated -> resistance goes down -> so blood rushes through microcirculation so fast that tissue doesn't have enough time to exchange O2 -> high mixed venous O2

-In hypovolemic and cardiogenic shock, cardiac output is low -> tissue has much more time for gas exchange in microcirculation -> low mixed venous O2

Measure PCWP via Swan Gans Catheter

-Cardiogenic shock - low cardia output results in back up of blood into LA and pulmonary venous system -> high PCWP

-Hypovolemic shock - low PCWP

What test can you do to distinguish between ARDs vs Left Heart Failure?

Measure PCWP with a Swan Gans Catheter

-L-Heart Failure - low cardiac output results in back up of blood into LA and pulmonary venous system -> high PCWP

-ARDs - increased vessel permeability -> leakage of fluid and proteins from vascular space into interstitial space of lung -> decreased blood volume in pulmonary vasculature -> low PCWP

Seborrheic dermatitis is associated with what two unrelated diseases?

What is Seborrheic dermatitis?

What is it thought to be caused by?

Tx?

-Parkinsons disease and HIV

SD - waxy scaling w/ underlying erythema on the scalp, central face, presternal region, interscapular areas, umbilicus, and body folds.

  -Malassezia fur fur (aka Pityrosporum ovale) may play a role in pathogenesis of SD

  -Topical anti-fungal agents are effective in Tx this cond'n

Pale or pink macules that don't tan or as hyperpigmented macules.

Caused by fungal infection of skin (Malassezia globosa)

How does pityriasis rosea present?

What pathogen is it a/w?

Oval shaped erythematous and mildly scaling patches arranged in a "Christmas tree" pattern on trunk. Eruption begins w/ larger lesion known as herald patch

A/w HHV-7

22 y/o female p/w erythematous papules, pustules, and telangiectasias of her face. She says these lesions worsen when she drinks hot beverages. Surrounding skin is thickened and greasy.

Dx?

What can you tell her to avoid?

Tx?

Dx - rosacea

Avoid EtOH, hot beverages, extremes of temperature, and reduce emotional stressors

Tx - Topical metronidazol (gel form) for induction effective and pplied BID

  -Systemic AbX (tetracycline) used for maintenance

What lipid abnormality is an indication for thyoid function testing?

What's the mechanism for this abn?

What medication do you need to be cautious about giving and why?

Hyperlipidemia, specifically hypercholesterolemia b/c pts with hypothyroidism have decreased LDL-R -> decreased LDL clearance

Need to be cautious when giving statins to these pts b/c hypothyroid pts are more susceptible to statin myopathy

What is the firstline management of non-ruptured esophageal varices?

Ruptured?

Non-ruptured - beta-blockers; sometimes nitrates are added on

Ruptured - endoscopic sclerotherapy

Note: Octreotide is used in the management of hepatorenal syndrome, not esophageal varices

What is the medical management for hepatorenal syndrome?

What is the definitive treatment?

Octreotide, and sometimes midodrine (alpha-1 agonist)

Definitive Tx is liver transplant

What's the pathophys behind hepatorenal syndrome?

What precipitates it?

What might you see on renal biopsy?

What are the clinical features?

-Pathophys - renal hypoperfusion 2/2 renal vasoconstriction of renal vessels

-Commonly precipitated infection and diuretics

-Renal biopsy will show normal kidney morophology b/c this is a functional renal failure

-Clinical features - azotemia, oliguria, hyponaturemia but low urine sodium (why???), hypotension

Which anti-psychotic medication might cause hypothermia and why?

Qid 3139

Fluphenazine (more potent than haloperidol)

  -Can cause hypothermia by inhibiting body's shivering mechanism and/or inhibiting autonomic thermoregulation

  -For this reason, pts taking antipsychotics shoould be advised to avoid prolonged exposure to cold temperatures

What visual disturbance migth a pt with central retinal vein occlusion complain of?

What might you see on exam?

RFs?

Acute or subacute loss of vision. It's less acute than central retinal artery occlusion.

Fundoscopic exam may show "blood and thunder" appearance, including optic disk swelling, retinal hemorrhages, dilated vv, and cotton wool spots

RFs: coagulopathy, hyperviscosity, chronic glaucoma, atherosclerotic RFs

Tx: no Tx is particularly effective

A 40 y/o male comes in b/c of a tingling sensation of feet. He has had lower back pain for 1 week since lifting somthing. He had a URI 1 month ago that lasted 4 days. He has a Hx of IV drug abuse. Vitals are nml and stable. Muscle strength is reduced in LE but preserved in UE. LE sensation is decreased. He has hyper-reflexia of LE and positive Babinski.

Dx?

NSIM?

Dx - spinal cord compressoin

  -typicall presents with isolated, symetric LE Sx, loss of sensation, and UMN signs

  -since he has bilateral Sx and it's present only in LE, lesion is most likely located in spinal cord

NSIM - MRI of spine

Note: Guillain-Barre Syndrome does not present with UMN signs (Babinski, hyperreflexia) and rarely p/w sensory findings

Thiazides - increase LDL, total cholesterol, and TG (VLDL) levels

Beta-blockers (propranolo) - increases TGs (VLDL) and lowers HDL levels

Metformin

  -Due to it's a/w lactic acidosis, renal failure can cause worsening of acidosis

  -This is why you MUST check a pt's renal function before and during treatment with metformin

NSAIDs - block PGE2 -> decreased vasodilation of afferent glomerular arterioles -> decreased GFR

ACE-I - block Angiotensin II -> decreased vasoconstriction of efferent glomerular arterioles -> decreased GFR

Cyclosporine - mechanism unknown

Oral or IV bisphosphonates

What is the diagnostic criteria for fibromyalgia?

What should you advise the pt?

Tx?

-Diagnostic criteria

  -Widespread musculoskeletal pain for at least 3 months in absence of joint swelling or mm weakness

  -Pain in at least 11 of 18 possible tender points

     -These points include: occiput, neck, shoulders, ribs, elbows, upper quadrant of buttocks, and inner aspect of knees

-Advise pt to remain active and productive

-Tx: SSRIs and TCAs have shown some effect and may be beneficial. Avoid narcotics

51 y/o caucasian female p/w low back pain radiating to buttocks. She complains of persistent mm pain in arms and shoulders that worsens w/ exercise. Exam shows no joint swelling or decreased mm strength. Palpation to outer upper quadrant of buttocks and medial knees elicits pain. ESR is 12 mm/hr

Dx?

Does injection of lidocaine into the shoulder decrease the pain of rotator cuff tear?

Rotator cuff tendonitis?

Adhesive capsulitis?

RC tear - no

Adhesive capsulitis (frozen shoulder) - no

RC tendinitis (resulting in RC impingement) - yes!

In a rotator cuff tear, there is:

  1) Weakness of shoulder

  2) Sx do not improve with lidocaine injection

Both p/w pain upon reaching and lifting arm overhead

Unilateral numbness, weakness, and swelling 22 compression of subclavian vessels and lower trung of brachial plexus

Pt may have weakened radial pulse and reproduction of Sx w/ specific amr mov'ts

Parasthesias and pain of neck and arm

Weakness may affect shoulder, elbow, or wrist, depending on cervical root(s) involved

Mov't of neck exacerbates Sx

What is the most relyable indicator or opioid intoxication?

What is the goal in management of such a pt?

-Respiratory depression is most relyable indicator

-Goal is to increase respiratory rate to at least 12/min by giving naloxone

Note: Pupil constriction (meiosis) is not a relyable indicator of opioid intox, as pupils can be nml or even enlarged (especially if pt OD'd on meperidine or propoxyphene).

What is the empirical Tx for agitation?

What condition is this Tx contraindicated in?

Qid 4622

Epirical Tx - haloperidol; benzodiazapines can also be used but in non-elderly pts (b/c they metabolize them slower)

Haloperidol is contraindicated in Lewey Body Dementia (beware of pts with visual hallucinations!)

Name 4 first-generation anti-histamines and 3 second/third generationg anti-histamines.

What is the difference in SE profile btwn the two?

1st generation - diphenhydramine, chlorpheniramine, doxepin, hydroxyzine

2nd generation - loratidine, desloratadine, fexofenidine

2nd/3rd generation drugs do not block cholinergic, alpha-1 adrenergic, or 5-HT receptors. Also they are more selective of peripheral H1 receptor as opposed to CNS ones -> less sedation effect

A 22 y/o male comes in b/c of itching and redness of his R-arm after bee sting. He perscribed doxepine and told to return if the swelling worsens. The next day, he returns b/c of mouth dryness, and dysuria.

Dx?

22 y/o female comes in with mild acne. She is perscribed topical retinods, and told to discontinue overuse of make-up. She returns 5 weeks later b/c acne has worsened. Exam shows mild-moderate facial acne.

NSIM?

NSIM - reassurance

  -It takes about 6 weeks to notice effects of meds, and in meantime, skin may get worse before it gets better

What test can you do to screen for cystinuria if you suspect it?

What might you see on UA?

Cyanide nitroprusside test - look up how this works

May see hexagonal crystals on UA

What is the main disease modifying drug used in rheumatoid arthritis?

What SE must you look out for?

What are 2 alternative drugs?

Methotrexate

  -SE include: bone marrow suppressiong (anemia, thrombocytompenia, leukopenia), pulmonary fibrosis, hepatotoxicity, oral ulcers (stomatitis), mild alopecia

2 alternatives include: hydroxychloroquine (SE is visual loss, so do eye exam Q 6 months), Sulfasalazine

What's the test of choice for diagnosing ankylosing spondylitis?

What is the gender ratio for AS?

Test of choice is pelvic X-ray

  -If it shows fusion of sacroiliac joint, that's diagnostic of AS!!!

  -If pelvic X-ray is negative or equivocal, you can do an MRI of spine as it's highly sensitive and specific for sacroilitis

Gender ratio for AS is 2:1, male:female

1. Blood glucose level > 250 mg/dL

2. pH <7.3

3. Serum bicarb <15-20 mmol/L

4. Detection of plasma ketones

IV nml saline!!!

  -1 L nml saline for first hr, 1 L nml saline for hrs 2-3

  -switch to D5-half nml saline once Osm gets to 150 mg/dL

What is the NSIM for a pt with suspected melanoma?

NSIM - excisional biopsy w/ narrow margins b/c wide margins is inappropriate if the lesion turns out to be benign

AFTER the Dx of melanoma has been made, wide excision (of 1-3 cm) is NSIM

What visual disturbances to pts with macular degeneration present with?

What is this a result of?

Central vision loss, but peripheral and navigational vision are always spared

It's a result of atrophy of outer retina, retinal pigment epithelium, Bruch's membrane and choriocapillaries

What are 5 complications of nephrotic syndrome that directly relates to loss of protein in urine?

Why do these occur?

-Hypercoagulability - loss of anti-thrombin III

-Iron-resistant microcytic hypochromic anemia - transferrin loss

-Osteomalacia/Rickets - loss of VitD binding protein

-Hypothyroidism - loss of thyroxine binding globulin

-Increased susceptibility to infection - loss of low molecular weight complement

What will you see in a joint aspirate in a pt with pseudogout?

Gout?

Pseudogout - rhomboid shaped positively birefringent crystals (calcium pyrophosphate)

Gout - needle shaped negatively birefringent crystals (monosodium urate)

46 y/o female with Hx of RA comes in complaining of R-knee swelling and tenderness for 3 days. She says pain began shortly after R-knee replacement surgery. Exam shows swelling, warmth, and decreased range of motion. Temp 101, BP 120/80, HR 90. CBC shows leukocytosis w/ left shift. Synovial analysis shows positively birefringent crystals and negative gram stain.
Dx?

Tx?

Dx - pseudogout

Tx - symptomatic management with NSAIDs

Do both of the following:

1) Administer atropine (competes with organophosphates at muscurinic-R) -> reverses Sx

2) Remove all clothing and wash pt's body b/c it may be contaminated with pesticides and you need to prevent transcutaneous absorption

CT abdomen

If both CT and U/S are negative, then you do ERCP (gold standard)

What's the Tx for early syphilis?

What if pt is allergic to that medication?

What's the Tx for neurosyphilis?

Qid 3256

Early syphilis - single IM dose of benzathine penicillin G

  -For pts allergic to penicillin, 14 day course of tetracycline is used

Neurosyphilis - IV aqueous crystalline penicillin

What 4 types of neuropathy can be seen in pts with longstanding DM?

Of these, which is most common?

Distal symmetrical sensorimotor polyneuropathy

Proximal neuropathy

Mononeuropathy

Autonomic neuroapthy

MC is distal sensorimotor polyneuropathy - pts classically p/w "stocking glove" pattern of sensory loss

Pure small fiber neuropathy is characterized by what?

Pure large fiber neuropathy is characterized by what?

Small - pain, allodynia, parasthesias

  -there is not appreciable sensory loss, and ankle jerk is preserved

  -think about it this way: you're losing the sensory neurons and interneurons that inhibit/modulate pain

Large - numbness, loss of pressure, propioception, and vibratory senses; ankle jerk is lost

  -less pain

What are the 6 stages (0-5) of DM foot ulcers?

What is the management for each?

0 - high risk foot w/o ulcer

1 - ulcer contained to skin (full thickness skin)

2 - ulcer penetrates to ligaments or mm

3 - ulcer deep enough to cause osteomyelitis, cellulitis, or abscess formation (think infectous!)

4 - localized gangrene

5 - extensive gangrene involving whole foot

Tx:

0 - continue to observe

1-2 - proper wound care and debridement

3 - admit, debride, culture deep parts, IV AbX (infectious is often polymicrobial so make sure ot cover anaerobic and gram (-))

4-5 - surgical consult for amputation

What are the 2 criteria for Dx of "brain death?"

Does a brain dead pt:

1) have increased HR when given atropine?

2) have DTRs?

3) spontaneously breath at high pCO2?

Qid 4159

Criteria:

1) Loss of cortical and brain stem functions

2) Proving irreversibility of brain activity loss (sufficient observation length)

Brain dead pts:

1) do not have increased HR with atropine b/c vagal control of heart is lost, so HR becomes invariant

2) DTRs are intact b/c spinal cord may still be functioning

3) no spontaneous breathing with hypercarbia (pCO2 >60) b/c loss of lower brainstem centers

How does hemochromatosis manifest with respect to involvement of the following organs:

-liver                  -thyroid

-pancreas           -gonads

-heart

-joints

-skin

liver - cirrhosis, very high risk of HCC

pancreas - DM

heart - restrictive cardiomypathy, conduction block

joints - arthritis

skin - bronze appears 2/2 hemosiderin deposition

thyroid - hypothyroidism

gonads - hypogonadotropic hypogonadism (decreased libido, small testes) 2/2 deposition in pituitary and hypothalamus

In what pt population/situation should you suspect myocarditis?

How do you Dx it?

Tx?

ANY young patient who p/w Sx of heart failure (PND, cardiomegally, hepatomegally, LE edema, pleural effusions, 3rd heart sound)

Dx by echocardiogram - you'll see dilated ventricles w/ diffuse hypokinesis, reflecting dilated cardiomyopathy

  -If echocardiogram is equivocal - myocardial biopsy (gold standard)

Tx - supportive, involving management of CHF Sx

How does rubella present?

What's the Tx?

-Fever, malaise, and tender suboccipital LN
(after 2-3 wks)

-Rash that decends from head to toe

Tx: acetaminophen for Sx relief

How does rocky mountain spotted fever present?

Tx?

-Red macular rash that begins over wrists and ankles and spreads centrally

-Influenza-like prodrome occurs

Tx: doxycyline everyone except pregnant women, and chloramphenicol for pregnant women

1) chemical or physical agents (trichloracetic acid, 5-FU, podophilin [antimitotic agent])

2) Immune therapy (imiquimod, IFN-alpha)

3) Surgery (cryosurgery, excisional procedures, laser Tx)

Hyperkalemia 2/2 RBCs losing K+ to surrounding solution

Hypocalcemia 2/2 citrate chelation

What's the difference between Heparin Induced Thrombocytopenia 1 and 2?

What's the difference in management?

1 - occurs <48hrs after administration, and is caused by platelet aggregation. No Tx is necessary

2 - occurs 3-12 days after administration, and is caused by antibody-mediated injury to platelet. Tx is IMMEDIATE discontinuation of heparin, and administration of direct thrombin inhibitor (lepiruden, argatroban)

Note: HIT is a hypercoagulable state 2/2 antibody mediated activation of platelets -> release of procoagulant mediators

22 y/o male p/w pain and swelling of his R-foot. He is a construction worker, and said he stepped on a nail at work a few days ago. Temp is 101. Exam shows red, swollen, warm R-heel. Xray is suggestive of osteomyelitis.

Organism causing osteomyelitis?

NSIM?

Pseudomonas aeruginosa - hint was Hx of nail puncture wound through his shoe (pseudomonas lives in moist environments like inside shoe)

NSIM - oral or parenteral quinolone and aggressive surgical debridement

How does essential tremor present?

What are 2 treatment modalities for essential tremor?

Intention tremor of upper extremity that is markedly decreased by alcohol use

  -disoriented handwriting often present

  -Other Sx of Parkinsonism are absent (bradykinesia, rigidity, shuffling gait)

Tx: beta-blockers, and anticonvulsants (commonly topiramate and primidone [anticonvulsant that converts to phenylethlmalonamide and phenobarbitol])

62 y/o male is brought in by his son due to progressive cognitive decline over the past year. Exam shows ophthalmoplegia, bradykinesia and limb rigidity, however no tremor.

Dx?

Dx - progressive supranuclear palsy (PSP)

  -Caused by deneration of brainstem (ophthalmoplegia, basal ganglia (rigidity, bradykinesia), and cerebellum

  -Like Parkinsons, it p/w rigidity and bradykinesia

  -Unlike Parkinsons, p/w ophthalmoplegia, but no tremor

In adults with meningitis, what comes first lumbar puncture or AbX?

Qid 3633

SU pg 356

Abx are given first followed by LP and CT

Children, on the other hand, should first have an LP and then a AbX (LP is easy to do in kids even when agitated)

-Restrictive lung disease

-Cauda equina syndrome

-Spinal fracture w/ spinal cord injury

-Osteoporosis

-Sponylodiscitis

-Rapid rewarming with warm water (40-44 C)

      -This results in less tissue damage than slow rewarming.

-Rapid rewarming with dry heat is not effectiveb/c it takes longer and results in tissue damage if temp is too high. Dry heat can also cause thermal injury b/c decreased sensitivity of frost bitten extremity

  -There should be NO ATTEMPT TO DEBRIDE frost bitten area initially. Only after complete rewarming and re-evaluation of extremity may this be done. True demarcation of where margins of healthy tissue is may take weeks

What is the goal BP for a pt with DM?

What's the firstline drug for BP control in these pts?

Goal BP - 130/80

Firstline drug - ACE-I

What is the AbX of choice for both human and dog bites?

What is the AbX of choice for Legionnaire's disease?

Human and dog bite - amoxicillin-clavulanate

Legionarre's disease - erythromycin

Disconinue heparin products (including LMWH)

  -Its also recommended to continue anti-coagulation therapy despite thrombocytopenia b/c these pts are in a hypercoagulable state -> use direct thrombin inhibitor (lepiruden or argatroban)

IV fluids (b/c RV infarcts required high preload to maintain BP) and discontinue nitrates and diuretics (b/c they decreased preload)

-Nitrates are contraindicated in RV infarction, AO stensois, and recent phosphodiesterase use

What is the empirical Tx of infective endocarditis in pts w/ suspected IV drug abuse?

What about non-IV drug abusers?

IV drug abusers - vancomycin (since MRSA is such a common cause)

Non-IV drug abusers - ampicillin-sulbactam (since MRSA is less common a cause)

II - p/w more bullae and blisters (think of pemphigus and pemphigoid)

III - erethematous and maculopapular rash

57 y/o female with Hx of HTN p/w continuous loss of urine for the past several months. She denies dysuria or hematuria, and had a UTI once 17 years ago. She had surgery for removal of invasive cervical cancer 2 yrs ago. BP 108/82, HR 80. Exam is unremarkable.

Dx?

How do you Dx?

Vesico-vaginal fistula

  -Pts with Hx of pelvic surgery or irradiation are at risk

Dx is made with intravenous pyelogram

  -Will see dye leaking from fistula

45 y/o male is brought in b/c his son found him on bathroom floor next to an unknown bottle. He is in severe pain, and his tongue is white. He is salivating heavily and has difficulty swallowing.

Dx?

NSIM?

Dx - caustic substance ingestion

NSIM - after maintaining an airway, do an upper GI endoscopy w/in first 24 hrs to assess extent of injury

Recall, NG lavage is doen for pts who have ingested poisons or OD'd on drugs such as EtOH. However, it's not used in caustic substance ingestion b/c it can ppt GI hemorrhage!

1) Chronic arthralgias (often w/ a false pasitive Rh factor or ANA)

2) Cryoglobulinemia

3) Porphyria cuteanea tarda

4) Membranoproliferative Glomerulonephritis

5) B-Cell lymphomas

6) Plasmacytomas

7) Autoimmune diseases (Sjogrens, thyroiditis)

8) Lichen planus

Describe pathologic changes of hypertensive kidney disease.

Describe pathologic changes of diabetic kidney disease.

HTN:

  -Nephrosclerosis - hypertrophy and intimial medial fibrosis of renal arterioles (efferent and afferent)

  -Glomerulosclerosis - progressive loss of glomerular capillary surface area w/ glomerular and peritubular fibrosis

  -Kidneys decrease in size

DM

  -Characterized by: increased ECM, BM thickening, mesangial expansion, and fibrosis

  -First year of DM - glomerular hyperperfusion and renal hypertrophy with increased GFR

  -First 5 yrs of DM - glomerular BM thickening, hypertrophy, and mesangial volume expansion w/ GFR returning to nml

  -5-10 yrs of DM - microalbuminuria, which later progresses to overt nephropathy

What will you see on microscopy of pts with Whipple's Disease?

What Sx do pts with Whipple's Disease p/w?

How do you Dx?

Microscopy - PAS positive macrophages in lamina propria

  -Recall: You also see PAS macrophages in mycobacterium avium infections of GI, and PAS positive globules in pts with Alpha-1-antitrypsin deficiency

Sx include: chronic malabsorptive diarrhea (steatorrhea, flatulance, and abd distension), protein-losing enteropathy, WL, non-deforming arthritis, LN, fever

Dx by SI biopsy and viral PCR

What are the criteria for Dx of monoclonal gammopathy of undetermined significance (MGUS)?

What tests should you order for Dx?

It's essentially the absence of findings a/w MM, including absence of:

  -anemia - CBC

  -lytic bone lesions - Bone X Ray

  -hypercalcemia -Chem 7

  -renal insufficiency - Chem 7

Nasal smear for eosinophils

  -Predominnace of eosinophils indicates allergic rhinitis

  -This test however has a low specificity - other causes of nasal eosinophilia include: nasal polyposis (including aspirin sensitivity), non-allergic rhinitis w/ eosinophilia)

Note: A common cause of non-allergic rhinitis is vasomotor rhinitis (chronis use of diphenhydramine) and is characterized by absence of eosinophelia

What key part of the History may help in distinguishing between cardiac vs. vasovagal syncope?

Cardiac will have sudden onset of syncope w/o warning signs

  -pt may have Hx of structural heart disease (e.g. MI), frequent ectopic beats, use of diuretics (causes electrolyte imbalance)

Vasovagal is typically preceded by presyncopal dizziness, weakness, and nausea

Antigen in urine or serum

  -Fungal blood cultures are sensitive, but take way too long (up to 6 weeks)

  -Another clue that it's Histo is presence of palatal ulcers!

Recall: Urine is the most productive fluid for culturing CMV!

What is the Tx for:

Blastomyocosis

Histoplasmosis

Coccidiodomycosis

Sporotrichosis

Blasto - PO intraconazole; Ampho for meningitis

Histo - PO intraconazole; Ampho if severe/fungemia

Coccidiodo - PO fluconazole; Ampho if severe/fungemia

Sporo - PO potassium iodide or intraconazole; Ampho for severe/fungemia

How does Lumbar Spinal Stenosis present?

How is Dx confirmed?

Tx?

p/w lower back pain and pain in legs that is worsened with back extension (walking) and relieved with back flexation (sitting, lying) -> this is termed neurogenic claudication and is easily differentiated from PVD by nml peripheral pulses and nml ankle-brachial index

  -flexation widens spinal canal

  -extension narrows spinal canal

Dx confirmed by MRI of spine

Tx: May be conservative or surgical (laminectomy)

For mechanically ventilated pts, what do you do if:

pO2 is low

pO2 is high

pCO2 is low

pCO2 is high

Low pO2 - turn up PEEP

High pO2 - turn down FiO2

Low pCO2 - turn down venilation rate or tidal vol

High pCO2 - turn up ventilation rate or tidal vol

What is the screening test of choice for acromegally?

What is the confirmatory test of choice for acromegally?

Screening test - IGF-1 (somatomedin C) levels

Confirmatory test - Oral glucose supression test

imaging

1) Worsening HTN - 20-50% of pts will experience an increase of 10 mmHg in diastolic BP. More common with IV administration as opposed to SubQ

  -2/2 increase in viscosity of blood -> increased resistance (recall R = [8*viscosity*length]/pi*r²

2) Headaches

3) Flu-like Syndrome - responsive to anti-inflammatory drugs; less common with SubQ administration

4) Red cell aplasia

45 y/o HIV positive man is brought in b/c of seizures. He has no Hx of seizures. T 98, BP 130/88, HR 90. He is not compliant with his medications, and his CD4 count is 60. CT head shows a single weakly enhancing lesion in brain.

Most likely Dx?

Tx?

Dx - primary CNS lymphoma (Toxo a little less likely given its characteristics on CT and fact that pts is afebrile)

Tx - Brain irradiation

What drug is used for prophylaxis agaist toxoplasmosis?

What drug is used to treat toxoplasmosis?

Prophy - TMP-SMX

Tx - sulfadiazine and pyrimethamine

What are Light's Criteria for classifying an effusion as an exudate?

Effusion must have at least one of the following:

-Pleural fluid protein/serum protein ratio >0.5

-Pleural fluid LDH/serum LDH ratio >0.6

-Pleural fluid LDH > 2/3 of upper limit of nml for serum LDH

1) Determine if transudate or exudate using Light's Criteria. Exudate needs 1 of the following:

  -Fluid protein/Serum protein >0.5

  -Fluid LDH/Serum LDH >0.6

  -Fluid LDH > 2/3 upper limit nml of serumLDH

2) Deterimine if uncomplicated or complicated. Complicated have: positive gram stain, positive culture, pH<7.2, glucose <60 mg/dL

  -If complicated -> requires chest tube placement as well as AbX b/c it's unlikely to resolve spontaneously w/ AbX alone!!!

All pts with systolic dysfunction should be given what drug even if asymptomatic?

What is the initial Tx of symptomatic heart failure?

ACE-I

initial Tx of choice - ACE-I + diuretic

What two things might you find in disseminated Blastomycosis?

Where is the highest infection rate?

Tx?

Ulcerated skin lesions and lytic bone lesions

  -Recall, Coccidiodomycosis also p/w bone lesions, and a maculopapular rash

Great Lakes, Mississippi and Ohio rr basins; highest infection rate is in Wisconsin

Tx: PO intraconazole; Ampho for meningitis

What is presbyopia?

How does it present?

Tx?

Age-related disorder that results in loss of elasticity of lens -> prohibits accommodation of lens, which is req'd in order to focus on near objects

Presents with poor near-sided vision--pts will classically hold reading material far away to read

Tx - reading glasses

Presence of papilledema on ophthalmoscop

  -Renal failure (elevated creatinine and oliguria) can develop rapidly w/o Tx but is not req'd for Dx

Recall, the pathologic change responsible for end-organ damage in m-HTN is fibrinoid necrosisi of small arterioles

What is the Tx of choice for Nocardiosis?

What is the Tx of choice for Actinomyces infection?

Nocardiosis - TMP-SMX

  -p/w Sx of TB: WL, fever, night sweats

  -p/w cough productive of purulent sputum

  -CXR shows alveolar infilatrates and nodules, often with cavitation

Actinomyces - Penicillin G

Recall the SNAP mneumonic:

Sulfa for Nocardia, Actinomyces treated with Penicillin

Pain syndrome characterised by tenderness of costochondral or costosternal junctions

  -pain is sharp, focal, lasts for hours, and worsens with inspiration and mov't

  -tenderness is reproducible to palpation

Tartrate-Resistant Acid Phosphatase (TRAP)

CD11c

What fundoscopic findings are seen in pts with vitreous hemorrhage?

How do these pts present?

Vitreous hemorrhage - fundus is hard to visualize and/or retina has dark red glow to it

P/W sudden vision loss and onset of floaters

For pts with Bell's Palsy, what physical exam finding excludes central facial paralysis and why?

Qid 2673

1) Anticoagulation is contraindicated in that pt (recent surgery, hemorrhagic stroke, bleeding diathesis, active bleeding)

2) Hx of adverse RXN to anticoagulation (HIT)

3) Pts who develop DVT/PE despite sufficient anticoagulation

What is a SE of acyclovir?

How can you prevent this from happening?

Renal tubular obstruction

  -Acyclovir is poorly soluble in urine and easily precipitates in renal tubules -> tubular obstruction -> renal failure

  -Crystallineuria w/ renal tubular obstruction usually occurs during administration of large parenteral doses of acyclovir

Prvent this with adequate hydration

This is also seen with indinavir (HIV protease inhibitor)

Hydroxycholorquine

  -This drug can cause retinopathy and corneal damage so eye exams every 6 months are indicated for these pts

What is happening in Sick Euthyroid Syndrome?

What will the pt's thyroid function tests look like as the disease progresses?

Does giving thyroid hormone help?

Sick Euthyroid Syndrome is when the body is trying to conserve energy during times of illness by shutting down metabolism, and it does this by decreasing thyroid function (since thyroid hormone drives metabolism).

  -Early on, T₄ -> T₃ conversion is shut down, so pt will have nml TSH and T₄, but low T₃

  -Later on TSH drops, causing a decrease in both T4 and T3

Giving these pts thyroid hormone does not help, and infact can increase the likelihood of mortality in these pts

For every 1.0 unit below normal a pt's albumin is, how much do you add to the pt's Ca²⁺?

A 45 y/o male comes in w/ fatigue, constipation, muscle weakness, and a 15 lb weight gain over the past 2 months. Vitals are T 98, BP 116/92, HR 62. Labs show low T3 and T4. The pt is perscribed levothyroxine and discharged home. He returns 2 weeks later saying all his Sx have worsened since taking the levothyroxine.

NSIM?

Most likely Dx?

NSIM - measure serum cortisol

Most Likely Dx - adrenal insufficiency 2/2 malignancy of thyrotropin cells -> decreased TSH; damage to ACTH producing cells -> decreased ACTH

What does the presence of following casts indic indicate?

-Muddy brown granular casts

-RBC casts

-WBC casts

-Fatty casts

-Broad and waxy casts

-Muddy brown granular casts - indicates presence of renal tubular epithelial cells

     -Non-specific finding in ATN

-RBC casts - glomerulonephritis or vasculitis

-WBC casts - interstitial nephritis and pyelo

-Fatty casts - nephrotic syndrome

-Broad waxy casts - broad casts arise from tubules that are enlarged (2/2 compensatory hypertrophy) in response to decreased renal mass

Pt p/w slurred speach, unsteady gait, drowsiness and mild respiratory depression. Pt's pupils are nml and reactive. Pt does not drink alcohol.

What drug has pt most likely OD on?

Benzodiazapine

  -Unlikely opioid overdose by the fact that there's only mild respiratory depression and the pupils are nml

  -Not as likely to be phenytoin as there's no nystagmus

Pt comes in with horizontal nystagmus, cerebellar ataxia, malar rash on face, and confusion. Pt also has labs showing megalobastic anemia.

What drug did this pt most likely OD on?

Phenytoin

  -May also see: gingival hyperplasia, hirsutism, induction of cytochrome P450, generalized lymphadenopathy