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Uworld Notes

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- Bordatella pertussis: -paroxysmal cough, inspiratory whoop, postussive emesis -lymphocyte predominate leukocytosis -Tx: MACROLIDE -Confirm with bacterial culture or PCR from nasopharyngeal secretions in 1 st month -Serology after 1 month Hereditary angioedema: C1 inhibitor deficiency or dysfunction – leads to elevated levels of edema producing factors C2b and bradykinin Characterized by: Rapid onset of: -noninflammatory edema of face limbs and genitalia -laryngeal edema – can be life threatening -edema of intestines -> colicky abdominal pain -NO evidence of urticaria Neonatal tetanus: -born to unimmunized mother -Frequently following umbilical stump infection -present in first two weeks of life with: poor suckling and fatigue followed by rigidity, spasms, and opisthotonus -High mortality: -first week apnea, second week sepsis Neimann pick: Sphingomyelinase deficiency Auto rec Age 2-6 months Loss of motor milestones Hyptonia Feeding difficulties Cherry red macula Hepatosplenomegaly Areflexia Tay-sachs: B-hexoaminisidase A def Auto rec Loss of motor milestones Hypotonia Fedding difficulties Cherry red macula HYPERreflexia
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Bordatella pertussis:-paroxysmal cough, inspiratory whoop, postussive emesis-lymphocyte predominate leukocytosis-Tx: MACROLIDE-Confirm with bacterial culture or PCR from nasopharyngeal secretions in 1st month-Serology after 1 month

Hereditary angioedema:C1 inhibitor deficiency or dysfunction leads to elevated levels of edema producing factors C2b and bradykinin

Characterized by:Rapid onset of:-noninflammatory edema of face limbs and genitalia-laryngeal edema can be life threatening-edema of intestines -> colicky abdominal pain-NO evidence of urticaria

Neonatal tetanus:-born to unimmunized mother-Frequently following umbilical stump infection-present in first two weeks of life with:poor suckling and fatigue followed by rigidity, spasms, and opisthotonus-High mortality:-first week apnea, second week sepsis

Neimann pick:-

Sphingomyelinase deficiencyAuto recAge 2-6 monthsLoss of motor milestonesHyptoniaFeeding difficultiesCherry red maculaHepatosplenomegalyAreflexiaTay-sachs:B-hexoaminisidase A defAuto recLoss of motor milestonesHypotoniaFedding difficultiesCherry red maculaHYPERreflexia

Down syndrome second trimester quadruple screen:Low MSAFP(maternal serum a fetal protein), low estriol, increased B-hcg, inc inhibin A

PAPP-A: pregnancy assoc plasma protein is a glycoprotein produced by the trophoblast. During FIRST trimester PAPP-A can be measured with B-hcg and US nuchal transluceny with detection rate of 85% for down syndrome -- Not used in second tri

Hypercalcemia:Mainfestations of Ca >12mg/dL:-constipation 2/2 altered intestinal smooth m tone-anorexia-vomiting-weakness-polyuria 2/2 defect in concentrating ability of renal tubules (nephrogenic diabetes insipidus)-confusion/lethargy

Medulloblastoma: 2nd MC infratentorial tumor in children-highly radiosensitive-can met through CSF>90% develop in cerebellar vermis-Posterior vermis syndrome:-truncal dystaxia-unbalanced gait-horizontal nystagmus

Astrocytoma: MC infratentorial tumor in children-develop in Cerebellar hemispheres-Hemispheric syndrome:-arm, leg and gait ataxia with ipsilateral cerebellar signs

GBS screening at 35-37 weeks gestation. Results are valid for ~5weeks.

Anti-D Ig AKA Rh antibody testing: done between 28-32 WGA-half life of anti-D abs is ~6weeks, covers any potential exposure through majority of 3rd trimester

Mixed cryoglobulinemia-seen in 30% of pts with Hepatitis C-arthralgia of miced cryoglobulinemia a/w a chronic vasculitic syndrome characterized by palpable purpura, lymphandenopathy, nephropathy, and neuropathy

TB is a common cause of chronic primary adrenal insufficiency

Primary adrenal insuff:Etiology: Autoimmune, infection (TB, HIV, fungal), hemorrhagic infarction(meningococcemia), metastatic CA(lung)

Presentation:Acute: shock, abd tenderness, unexplained F, n/v, anorexiaHyponatremia, hyperkalemia, hypercalcemia, and eosinophilia

Chronic: fatigue, wt loss, n/v, abd pain, hyperpigmentationHYPOTENSION, hyponatremia, hyperK, hyperCa, anemia, eosinophilia

All adrenal insufficiencies will have Non-anion gap metabolic acidosis

Dx: Measure ACTH and serum cortisol with high dose ACTH stim testPrimary adrenal insuff: low cortisol, high ACTHSecondary adrenal insuff: low cortisol, low ACTH

HIV tx side effects:Didanosine pancreatitisAbacavir hypersensitivity syndromeAny NRTIs- lactic acidosis, Steven johnsons syndromeNevirapine liver failureIndinavir(protease inhibitor) crystal induced(needle shaped) nephropathy

Crystal induced nephropathy:

Common etiologies:Acyclovir (IV >oral) low urine solubility -> precipitates in tubules -> renal tubular obstruction

SulfonamidesMethotrexateEthylene glycolProtease inhibitors

Elevated creatinine w/in 1-7 days of starting drugUrinalysis can show: hematuria, pyuria, crystal

Give IV fluids with drug to reduce risk of AKI

ADPKD:HTN is common early finding, often present before decline in renal function-Ace inhibitors are preferred HTN tx in ADPKDAlso present with: hematuria, proteinuria, palpable renal masses, or progressive renal insuff. sMay have flank pain due to renal calculi, cyst rupture or upper UTI

ShockEpinephrine- Alpha - 1 agonist to increase vasoconstriction Beta 2 agonist to relax bronchial smooth muscle and decrease vascular permeability

Dopamine can be used as additional vasopressor for persistent hypotensionHigh dose acts on beta 1 and alpha -1Does not help respiratory symptoms due to lack of significant beta 2 activity

Adult smoker with hard, non-tender, submandibular or cervical nodes is highly concerning for head and neck CA.Vast majority of head and neck CA is Squamous Cell CA

Brain death refers to a total loss of brain function and is a legally acceptable definition of death. Family permission is NOT legally required to discontinue life support

Herediatary spherocytosis: -auto dom. Disorder of spectrin that provides scaffolding for RBCs-RBCs are not flexible and get trapped in fenestrations of spleens red pulpfindings: positive family hx, splenomegaly, spherocytosis with increased retic count

Thalassemia minor-heterozygous for B-thalassemia-typically asymptomatic-microcytic target cells on peripheral smear

G6PD def-X linked rec-G6PD required to create NADPH necessary to create glutathione and prevent oxidation of hgb. -Rxn occurs in response to oxidant stress(antimalarial drugs, sulfas) fava beans, infection-bite cells-heinz bodies after crystal violet staining

Succinocholine is a depolarizing neuromusc blocker often used during rapid sequence intubation-rapid onset 45-60 seconds-rapid offset 6-10 minutes-Can cause significant potassium release and life threatening arrhythmias-should NOT be used in pts at high risk for hyperkalemia-crush or burn injury >8hrs old (high risk of rhabdo)-pts with demyelinating syndroms (guillain-barre)-pts with tumor lysis syndrome

Sick euthyroid syndrome AKA low T3 syndrome:Any pt with an acute, severe illness (MI, shock, etc) may have abnormal thyroid func tests.-likely 2/2 caloric deprivation and increase in cytokine levels(IL-1 and IL-6)-Most common hormone pattern:-low total and free T3, 2/2 decreased peripheral conversion of T4->T3-normal T4 and TSH-If non-thyroidal illness persists, T4 and TSH levels also decrease-On recovery, may see transient increase in TSH. Often misinterpreted as subclinical hypothyroid.-Therefore, Thyroid function test should not be performed in pts recovering from major illness

Benzo overdose symptoms:Slurred speech, drowsiness, unsteady gait

Distinguish from:-Opioid OD by lack of respiratory depression and lack of pupillary constriction-Alcohol intoxication by lack of nystagmus

Myasthenia Gravis:Can affect muscles anywhere in the body,most common sx ptosis and double visionDX: EMG and Acettlcholine receptor Ab test CT of the chest to look for thymoma should be done in all newly diagnosed MG pts--Thymectomy is useful in such cases, results may not be seen for 3-4years

Lacunar strokeMajor risk factors: HTN, DM, hyperlipidemia, smokingPathology: likely 2/2 combination of microatheroma and lipohyalinosis-infarcts are usually of thrombotic origin-2/2 small size, lacunes are often missed on noncontrast CT scans during or shortly after event.-MC site for lacunar infarct is posterior limb of the internal capsule leading to a pure motor stroke affecting the contralateral face, arm and leg equally-other lacunar stroke syndromes: ataxic hemiparesis, clumsy-hand dysarthria, pure sensory stroke and mixed sensory-motor stroke

Creutzfield jakob disease:Prion proteins cause rapid neuronal loss and death within 1yr-should be suspected in pts 50-70yrs old with rapidly progressing dementia and myoclonus-other sx: insomnia, apathy, behavioral changes, impaired vision

Dx:Spongiform changes on postmortem brain biopsy Genetic testing for PNRP protein geneBi or tri phasic sharp wave complexes on EEGElevated 14-3-3 proteins on CSF samples

Key features of ds:-long incubation period-characteristic spongiform changes-lack of inflammatory response(no change seen on cbc)

Prinzmetal angina(variant angina) : chest pain 2/2 coronary vasospasm-risk factor: smoking-pts often lack CV risk factors-episodes characteristically occur at night 12A-8A and can be assoc with transient ST elevations-Negative cardiac enzyme panel-Tx: CCB diltiazem-Avoid aspirin and B-blockers bc they can promote vasoconstriction

Lewy body dementia:Lewy bodies = eosinophilic intracytoplasmic inclusion made of alpha synuclein protein

Two of three essential for Dx:-fluctuating cognition with pronounced variations in attention and alertness-Recurrent visual hallucinations that are typically well-formed and detailed-spontaneous motor features of parkinsonism

Tx of psych symptoms: acetylcholinesterase inhibitors -> Rivastigmine

Key to distinction between LBD and parkinsons is the early appearance of dementia in LBD and of motor symptoms in parkinsons

Chronic pancreatitis is assoc/w increased risk of pancreatic CA

Abd US preferred intial test if jaundiced can detect biliary tract dilation and obstruction

Abd CT w/ contrast preferred initial test without jaundice CT is more sensitive for pancreatic CA, delineating tumor extent and staging tumor

Normal CA 19-9 values do NOT r/o pancreatic CA-used as a marker of longitudinal ds in known pancreatic CA pts

Hypoxia in PNA occurs 2/2 alveolar and interstitial inflammation which causes areas of V/Q mismatch

-Manifests as increase in alveolararterial gradient

Giardiasis:Food borne, water borne, or p2pTrophozoites ahere to mucosal surfaces by adhesive disks, producing malabsorption-generally chronic duration of sx-Tx metronidazole

Glucagonoma: rare pancreatic tumor-mild DM or hyperglycemia with necrotic migratory erythemaNME: erythematous plaques or papules that coalesce to form large painful inflammatory blister and or crustingwith central clearing.-typically on face, perineum, extremities-diarrhea and wt loss-anemia: normocytic normochromic likely 2/2 anemia of chronic ds or glucagons direct effect on erythopoesis-DX: Glucagon levels > 500

Stepwise approach to Tx of Ascites:1. Sodium and water restriction (2L/day)2. Spironolactone3. Loop diuretic when max dose of spironolactone not enougha. Not more than 1 L/ day of diuresis4. Frequent abd paracentesis (2-4 L/day)a. Watch for hepatorenal syndrome

Psoriatic arthritis:-5-30% of pts with psoriasis-Classically involves DIP joints-Morning stiffness, deformity, dactylitis(sausage fingers)-nail involvement ie onycholysis-well demarcated red plaques with silver scaleTx: NSAIDS, methotrexate, anti-TNF agents

Dermatomyositis: -can present with Gottrons papules:violaceous plaques, slightly scaly, over the MCP joints

CLL:-MC leukemia in adults-B-cell leukemia-smudge cells on pathology: leukocytes that have undergone partial breakdown during prep of stained smear bc of greater fragility-Thrombocytopenia is a poor prognostic favor Stage IV-characteristic findings:-lymphadenopathy, splenomegaly, anemia, thrombocytopenia-Avg life span 8-10 years

TCA overdose:CNS, cardiac, anticholinergic findings-TCAs inhibit fast sodium channels in his-purkinje system, decreasing conduction velocity, increasing duration of repolarization and prolonging refractory periods-> hypotension, QRS widening, ventricular arrhythmias-Tx: Sodium bicarb to reduce cardiac toxicity (pH goal 7.5)-increased pH decreases drug avidity for Na channels-increased extracellular sodium increases electrochem gradient across cardiac cells and affects ability of TCAs to bind fast sodium channels

Clopidogrel: anti-platelet, acts by antagonizing ADP-appropriate alternative for those who cannot tolerate aspirin-Clop + aspirin better than apirin alone

Following pts post unstable angina/NSTEMI:-clopidogrel for at least 12m + ASA indefinitely

Percutaneous coronary interventions (PCI):-Clop + ASA better than ASA alone for first 30 days, helps prevent subacute stent thrombosis-Drug-eluting stents require longer duration bc epithelialization occurs slowly-30 days for bare metal stents-up to 1 yr drug eluting stents

Polycythemia vera:Clonal myeloproliferative dz of the pluripotent hematopoietic stem cell.Characteristic features:-increased RBC mass-mild granulocytosis-elevated platelets-low EPO

May be present:-HTN as a result of expanded blood volume-increased incidence of peptic ulcer histamine release from basophils-gouty arthritis increased cell turnover

Multiple myeloma:Monoclonal proliferation of plasma cells>3g/dL M protein on SPEP>10% plasma cells in bone marrow

CRAB:Calcium (hypercalcemia)Renal impairmentAnemiaBones (bone pain, lytic lesions, fractures)

Increased risk of infx due to a total decrease in functional antibodies and leukopenia secondary to bone marrow crowding with malignant plasma cells

-----MGUS monoclonal gammopathy of undetermined significance< 3g/dL M protein on SPEP decreased stroke vol -> reduced SBP

Pulsus paradoxus also seen in severe asthma and COPD: drop in intrathoracic pressure is greatly exaggerated. Negative pressure leads to pooling of blood in pulmonary vasculature decreasing LV preload

HIV+ pt:Only live vaccines that can be administered ar MMR and varicella if:-CD4 count > 200-no history of AIDS defining illness

Acute glaucoma:First line tx: IV mannitolAlso can administer:Acetazolamide carbonic anhydrase inhibitor, decreases aqueous humor productionPilocarpine opens canals of schlemm, allowing drainage of aqueous humorTimolol B-blocker, decreases aqueous humor production

Avoid mydriatic agents such as atropine

Glucocorticoid induced myopathy:-One of most common drug induced myopathies-typically weeks to months after starting tx-PAINLESS proximal muscle weakness (LE before UE) manifests as difficulty in ADL such as getting out of chair, climbing stairs, brushing hair,-No muscle inflammation, normal ESR, normal CK-likely 2/2 increased muslcle catabolism and decreased anabolism as a direct effect of the steroids-improves with discontinuation of steroid therapy

Polymyalgia Rheumatic:-seen in up to 50% of pts with temporal(giant cell) arteritis-aching and morning stiffness-Pain and decreased range of motion in shoulders, neck and hip girdle-normal muscle strength-ESR > 40-Normal CK-Symptoms improve rapidly with glucocorticoids

Metabolic effects of HCTZ:-Decreased glucose tolerance -> hyperglycemia-increased LDL-increased triglycerides-hyponatremia-hypokalemia-hypercalcemia

Dacrocystitis: infx of lacrimal sac-occurs in infants and pts >40yo-sudden onset of pain and redness in medial canthal region-fever, prostration, elevated leukocyte count-may express purulent fluid from lacrimal duct-S. aureus and GAS common organisms

Episcleritis: infx of episcrleral tissue-Acute onset of mild to mod discomfort, photophobia and watery discharge

Hordeolum: abscess on upper or lower eyelid-MC cause S. aureus-Red, tender swelling over the eyelid

Chalazion: chronic granulomatous infx of meibomian gland-Hard, painless nodule-presents as lid discomfort

Rabies:Pts exposed to high risk wild animals raccoons, bats, skunks should receive post exposure prophylaxis if animal is unavailable for testing.-if animal can be tested, pt should start PEP but may discontinue if animal tests negative

Subarachnoid hemorrhage:Most commonly 2/2 ruptured berry aneurysmSevere HA at onsetMeningeal irritation (neck stiffness)Focal deficits uncommon

Complications:Rebleeding first 24 hrsVasospasm 3-10 days after events. -major cause of morbidity and mortality-occurs in up to 30% of SAH pts-prophylaxis: Nimodipine-CT angiography to dx vasospasmHydrocephalus/inc ICPSeizuresHyponatremia 2/2 SIADH

Lumbar spinal stenosis: degenerative condition, spinal canal is narrowed resulting in compression of one or more spinal roots-back pain may radiate to buttocks and thighs- worse during walking and extension-lumbar flexion alleviates symptoms-numbness and paresthesias may occur-Dx confirmed with MRI

--Iliac artery stenosis:Claudication in buttocks and thighs-pain worse with activity, relieved with rest-pain not affected by flexion or extension--

Lumbar disk herniation:-Acute onset back pain with or without radiation down one leg-lumbar flexion and sitting make pain worse

Sporotrichosis:Sporothrix schenkii is a dimorphic fungus-Initial lesion is reddish nodule that later ulcerates-Fungus spreads from site of inoculation via lymphatis, forming subcutaneous nodules and ulces-adenopathy and systemic signs of infx are usually ABSENT

Tx: oral potassium iodide

Serum calcium decreases by 0.8mg/dL for every 1g/dL decrease in serum albumin-ionized plasma Ca (physiologically active form)is hormonally regulated and remains stable

-Increased extracellular pH (alkalosis) causes an increase in the affinity of serum albumin to calcium

.Subdural hematomas:Slow venous bleedElderly and alcoholics falls/blunt traumaMC Sx: HA and gradual loss of consciousnessCT: white crescent +/- midline shift

Pseudotumor cerebri:Young female, HA (days-weeks), +/- n/v, normal neuro imaging, elevated CSFNeurologic signs generally absent except papilledema, visual defects and sometimes CN VI palsy-May have Hx of provoking agents: glucocorticoids, Vit A, OCPs-Pathology: impaired absorption of CSF by arachnoid villi

Tx: -wt reduction-Acetazolamide if wt reduction fails-If continues to worsen, shunting or optic nerve sheath fenestration to prevent blindness, the most significant complication

Renal Fibromuscular dysplasia:Tx: percutaneous angioplasty with stent placement*****High yield

Macrocytic anemia in an alcoholic likely 2/2 folate deficiency-alcohol abuse causes folate deficiency by impairing its enterohepatic cycle and inhibiting its absorption

Tuberculosis induced adrenal insufficiency is the most common cause of adrenal insufficiency worldwide presents with hypotension, fatigue, weight loss, nausea/vomiting, abdominal pain, hypoglycemia, hyperkalemia, and fever

It is recommended that patients' TSH levels be re-evaluated 4-6 weeks after initiation of levothyroxine

Aortic stenosis:-Exertional symptoms chest pain, dyspnea, dizziness, syncope-Delayed and diminished carotid pulse pulsus parvus et tardus-Single and soft S2-audible S4Harsh ejection crescendo-descendo systolic murmur in 2nd intercostal space with radiation to carotis

Scleroderma:

May see esophageal dysmotilityCharacteristic features of scleroderma dysmotility:-absence of peristaltic waves in lower 2/3rds of esophagus-significant decrease in lower esophageal sphincter tone

MVP is MCC of chronic mitral regurg in developed countries-MVP occurs due to myxomatous degeneration of mitral valve leaflets and chordae

Digitalis toxicity:Causes increased ectopy and increased vagal tone-Atrial tachycardia with AV block occurs from combination of these effects and is relatively specific for dig toxicity

MCC brain metastases: Lung > breast > unknown primary > melanoma > colon

Multiple brain metastases: lung CA, malignant melanoma

Solitary brain metastases: Breast, colon, renal cell CA

Complications after MI:Reinfarction: hours 2 daysVentricular septal rupture: hours 1 weekFree wall rupture: hours 2 weeksPost infarction angina: hours 1 monthPapillary muscle rupture: 2 days 1 monthPericarditis: 1 day 3 monthsLeft ventricular aneurysm: 5 days 3 monthsHallmark of LV aneurysm: persistent ST-segment elevation after a recent MI and deep Q waves in the same leadsProgressive LV enlargement and remodeling can also lead to mitral annular dilation with mitral regurg

HIV;TMP-SMZ used as prophylaxis to prevent PCP and toxoAzithromycin prophylaxis against MAC*****High Yield***MC site of ulnar nerve entrapment is the elbow where the ulnar n. lies at the medial epicondylar groove

Carcinoid tumors are neuroendocrine tumors that cause flushing, secretory diarrhea, bronchospasm and cardiac valvular abnormalitiesCarcinoid cells cause increased production of serotonin from tryptophan (required for niacin synthesis), resulting in niacin deficieny pellagra: dermatitis, diarrhea, dementia

Hereditary nonpolyposis colorectal cancer (HNPCC), or Lynch syndrome

colorectal cancer, ovarian, gastric, biliary tract, brain, prostate, skin, and especially endometrial cancers are all associated with this autosomal dominant genetic defect.

family history of multiple cancers. Colonoscopy every 1-2 years starting between the age of 20-25. ---Gardner's syndrome: adenomatous polyps - plus osteomas, lipomas, fibromas, cysts, and dental abnormalities. ---Peutz-Jeghers syndrome: multiple pigmented spots most often found on the lips, oral mucosa, and GI mucosa. Hamartomas are found on colonoscopy. ---Familial adenomatous polyposis: hundreds to thousands of adenomatous polyps. ---Turcot's syndrome: familial adenomatous polyposis and CNS malignancy.

Primary biliary cirrhosis presents with fatigue and pruritis, primarily affecting women. Expected findings include jaundice, hypercholesterolemia, elevated alkaline phosphatase and IgM levels. The hallmark finding associated with this disease is antimitochondrial antibodies. Treatment is supportive while UDCA may be used to slow the progression of liver disease.

Dopamine agonists (pramipexole, ropinirole, levodopa/carbidopa, and bromocriptine) are the first line treatment for restless leg syndrome. Patients should also be placed on iron supplementation.

AML: patients over 30, bone marrow biopsy reacts with myeloperoxidase and sudan black, associated with auer rods. Anemia, thrombocytopenia, and leukopenia (decrease in functioning white cells even though total white count may be high due to proliferation of immature white cells) associated with AML results in increased infections, bleeding, fever, and lymphadenopathy. Leukocyte count may also be very high.

Epididymitis1st line Tx: ceftriaxone plus doxycycline.

MCC in patients under 35 is Chlamydia. Epididymitis in older men usually results from E. Coli.

Patients will present with fever/chills, an erythematous and tender scrotum, and complain of frequency, urgency or dysuria. Uretheral discharge may also be present. Cremasteric reflex is tested to rule out testicular torsion

A. IgA Nephropathy: Follows viral illness, IgA and C3 on staining. Normal C3B. PSGN: Occurs at least 1 week after strep throat or skin infection. High ASO. Low C3. Lumpy Bumpy pattern of deposition on microscopyC. Wegeners: Damages both lungs and kidneys. C-ANCA is positive. Involves upper airway: sinusitis, otitis media, nasal ulceration.D. Goodpastures: Damages both lungs and kidneys. Linear deposit of anti glomerular basement membrane antibodies. Does not affect upper airway.E. MCD: fusion of podocyte foot processes. Asymptomatic child becomes puffy and edematous.F. Alports: Deafness and hematuriaG. Membranous nephropathy: Adult with proteinuria and a thickened glomerular basement membrane. Spike and dome pattern.H. Diabetic nephropathy: Kimmelstiel-Wilson nodulesI. FSGS: Associated with IV drug abuse and HIV. Sclerosis on biopsyJ. Lupus nephritis: Multiple types so biopsy is essential to determine treatmentK. Amyloidosis: Apple green birefringence with Congo red stainingL. Membranoproliferative nephropathy: tram track appearance of the basement membrane

Ascending cholangitis-Charcot's triad: RUQ pain, fever, and jaundice. More serious cases present with Reynold's pentad: Charcot's triad plus hypotension and altered mental status

CREST syndrome: Calcinosis, Raynaud's phenomenon, Esophageal dysmotility, Sclerodactyly, and Telangiectasias.-Anti-centromere antibodies

C-ANCA (cytoplasmic-staining antineutrophil cytoplasmic antibodies) are antibodies against proteinase-3 and are highly specific for Wegener's granulomatosis

Autoantibodies against myeloperoxidase is the most common target of p-ANCA (perinuclear-staining antineutrophil cytoplasmic antibodies) found in many disease processes making its specificity relatively low.

Bullous pemphigoid affects patients over 65; blisters spread throughout the skin but avoids mucous membranes; biopsy will reveal a linear deposit of IgG.-Negative nikolsky--pemphigus vulgaris also causes blistering but more commonly in 30 to 60 year olds. It involves the mucous membranes and biopsy will show IgG antibodies against desmoglein between the epidermal cells. +Nikolsky sign

Renal tubular acidosis presents with a non-anion gap hyperchloremic metabolic acidosis

RTA Type I: distal tubular defect The deficiency is in H+ secretion leading to urinary pH over 6.0. hypokalemia. Increased excretion of calcium and phosphate 2/2 alkaline urine -> inc risk nephrolithiasis -70% have stones Common causes of RTA Type I are Sjogren's, SLE, and other autoimmune disorders. Tx: sodium bicarbonate.

RTA Type II is caused by a defect in proximal tubule's ability to reabsorb bicarbonate. Tx: alkali replacementLow bicarb, high chloride, and low potassium are classic findings of RTA II but may be seen with other RTAs. RTA IV can be ruled out because it results in hyperkalemia. So to differentiate between RTA I and II, look at urine pH. In RTA I, there is an inability to acidify urine beyond pH of 5.5- most frequently occurs in children as part of Fanconis syndrome (abnormal excretion of glucose, amino acids, citrate, and phosphate into the urine, as well as vitamin D deficiency and hypokalemia). It is also associated with multiple myeloma and carbonic anydrase inhibitor use.

Renal Tubular Acidosis (RTA) type IV: -caused by HYPOaldosteronism. -present with hyponatremia, hyperchloremia and hyperkalemia and an acidic urine. -commonly seen in diabetes and interstitial nephritis resulting in a hyporeninemic state. -only RTA that presents with hyperkalemia

A good algorithm to follow if you get RTA questions:Step one: Determine it is RTA (hyperchloremic acidosis with a normal anion gap and near normal GFR in the absence of diarrhea = RTA)

Step two: Look at the potassium level, if it is higher than the normal range (3.5-4.5) then it is automatically RTA-IV

Step three: Look at urine pH. If it is lower than 5.5 it is automatically RTA-II. If it is higher than 5.5 go to step 4.

Step four: Look at the bicarb level. If the bicarb level is near normal it is RTA-I. If the bicarb level is markedly decreased it is RTA-II.

Step five: confirm diagnosis as follows: -RTA-I: administer ammonium chloride (an acid). If urine pH does not drop below 5.5 as serum pH decreases, you have your diagnosis.

-RTA-II: administer bicarb. If the urine pH continually rises as bicarb is given, you have your diagnosis.

-RTA-IV: salt restrictive diet. If urine sodium is persistently high, while serum sodium begins to decrease, you have your diagnosis.

three most common causes of small bowel obstruction in by ABC. Adhesions(previous abd surgx), bulges (hernia), cancer.

Hepatic encephalopathy:elevated arterial ammonia level since the liver is responsible for clearing ammonia. Tx: avoiding sedatives, and initiating lactulose which promotes excretion of ammonia.

(inferior petrosal sinus sampling) is used to confirm the pituitary as the source of ACTH production when there is no detectable adenoma on imaging and ectopic sources have been ruled out.

McCune-Albright syndrome presents with short stature, caf au lait spots, precocious puberty, bone lesions (polyostotic fibrous dysplasia), and associated endocrinopathies

Thiamine deficiency:Wet beriberi-dilated cardiomyopathy dilated LV with decreased LVEF-tachycardia-peripheral edema-DOE

Hyperthyroidism during pregnancy: -MC 2/2 Grave's diseaseTx:1st trimester: PTU2nd and 3rd Tri: Methimazole

Retinal artery occlusion is acute, painless, monocular loss of vision due to an embolus. It presents with a pale retina with a cherry-red macula (which gets continued blood supply from ciliary artery).

Churg-Strauss:allergic rhinitis, asthma, blood eosinophilia, and positive p-ANCA

Lambert-Eaton Myasthenic Syndrome: autoantibodies against PREsynaptic calcium channels causing muscle weakness most prevalent in patients with small cell lung carcinoma. Repeated nerve stimulation improves symptoms.

ALL peaks between 3-5 years of age. Bone invasion causes pancytopenia resulting in anemia, bone pain, infections, and signs of low platelets. Bone marrow biopsy: increased blasts of lymphoid lineage.

The board loves to test using side effects of one drug to treat another condition so be aware of the following:

Beta Blockers: Used for essential tremor, thyrotoxicosis, migraines, and some arrhythmiasThiazide diuretics: Used for osteoporosis or kidney stones-Reduced Ca+ excretion in the urineCalcium channel blockers: Used for Raynauds, esophageal spasm, and some arrhythmiasAlpha-1 antagonists: Used for benign prostatic hyperplasia (BPH)-SE: Orthostatic HypoTN

The pheochromocytoma 10% rules are as follows: 10% recurrence rate after surgery10% are familial10% are bilateral10% are extra-adrenal10% are malignant10% occur in children

(left main coronary artery branches into the left circumflex and left anterior descending arteries.

Occlusion of the left main is associated with ST-T elevation in aVR.

LAD supplies the anterior left ventricle and septum. ECG changes are seen in V1-6 (septal leads are V1-4).

LCX supplies the posterolateral left ventricle and the anterolateral papillary muscle.

marginal branch of the left coronary artery) supplies the left margin of the heart toward the apex.

Empyema:-complication of pneumonia-may have: pus, pH TUBERCULOSIS

Bordatella pertussis:1st line tx: macrolide

The classic triad of fat embolism: respiratory changes (dyspnea, tachypnea, cough, hypoxia), neurological abnormalities (mental status change), and petechial rash.

Patients who present with benign paroxysmal positional vertigo (BPPV) are best treated with the Epley maneuver to reposition the canalith. The vertigo experienced is typically episodic, lasting about 30 seconds and brought on by changes in head position. The diagnosis can be confirmed by performing the Dix-Hallpike maneuver when a nystagmus is elicited.

Menieres disease: Triad: vertigo, tinnitus, hearing loss. In contrast to BPPV, the vertigo lasts much longer, hours to days. The acute vertigo is managed with benzodiazepines (diazepam) or vestibulosuppressants. Diuretic therapy and a low salt diet may help prevent reoccurrences.Antihistamines, anticholinergics, a low sodium diet, and diuretics may lower endolymphatic pressure by reducing the amount of endolymphatic fluid to reduce the disease process

Labyrinthitis: Look for someone with a viral infection who about a week later develops vertigo, nausea/vomiting, hearing loss, and nystagmus. Treatment is with steroids to reduce inflammation.

Remember: aminoglycosides are ototoxic ----

LP purple, polygonal, pruritic, papules. Wickham striae are fine white reticulations seen on the surface of the lesions and in the oral cavity and are very specific for the disease.

Half of patients with skin lesions will also have oral striae. Upon observation of the initial lesions, the disease will spread symmetrically over one to four weeks

histological characteristics of LP are 1) damaged basal epidermal keratinocytes and 2) a linear arrangement of lymphocytic infiltrate in the papillary dermis at the interface with the epidermis

Seborrheic keratosis:Benign skin tumor-stuck on appearance-not related to sun-light exposre- sudden appearance of multiple SKs associated with internal malignancy (the sign of Leser-Trelat).

Renal vein thrombosis:-can be complication of nephrotic syndrome-antithrombin III is lost in the urine and leads to increased risk of venous and arterial thrombosis-Presents with sudden onset:-abd pain-fever-hematuria

-Can occur in any form of nephrotic syn.-Most common with Membranous glomerulonephritis

Lupus anticoagulant:-antiphospholipid antibody-leads to prolonged PTT-Pt is HYPERCOAGUABLE

Common foci of A-fib:Cardiac tissue surrounding pulm veins-different type of myocytes which are more susceptible to aberannt rhythms

SLE Serositis: pleurisy, pericarditis, peritonitisANAAnti-ds & anti-smProteinuria and elevated CrSymmetric, migratory arthritis

Amenorrhea 2/2 Morbid obesity:-amenorrhea is 2/2 anovulation-FSH and LH wnl-ovaries produce estrogen but progesterone is not being produced at the normal post ovulation levels. Therefore progesterone withdrawal menses at the end of the cycle does not occur Premature ovarian failure:Women less than 40yoFSH > 40LH >25

Cryoprecipitate:Insoluble products from FFP: fibrinogen, vWF, F VIII, F XIIIUsed for replacement therapy for pts with fibrinogen, vWF, or FVIII deficiency

FFP:: contains all clotting factors and plasma proteins from one unit of blood.-indicated for active bleeding with severe coagulopathy:-liver disease-DIC-supratherapeutic INR

Platelets transfusion indicated for:Platelet


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