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Henoch-Schonlein Purpura
Vasculitis - IgA immune complex deposition

Triad: Purpura, arthritis, abdominal pain. (can involve kidneys or GI hemorrhage)

High IgA, ESR, CRP

Most spontaneously resolves
Churg-strauss
Eosinophilic autoimmune vasculitis of medium and small vessels
Largely pulmonary, GI and peripheral nerves. Can also affect heart, skin and kidneys.
Dx: Eosinophils, granulomas, and pANCA. (Wegener's = cANCA)
Waardenberg syndrome
White forelock, deafness, heterochromic or brightly colored eyes
Auto-dominant.  Neural crest disorder
Kawasaki's disease
Systemic necrotizing medium-sized vessel vasculitis
Dx: Fever >= 5 days and 4/5:
1. Bilateral conjunctivitis
2. Oropharyngeal (injection, lip fissures, strawberry tongue)
3. Edema/erythema of hands or feet.
4. Polymorphous rash (mostly truncal)
5. Cervical adenopathy, usually unilateral
Mostly kids <5 years
Mostly blood vessels, skin, mucous membranes and lymph nodes
Can cause coronary artery aneurysms (5%)
Starts w/ high, persistant fever
High-dose aspirin and IVIg
Prinzmetal's angina
Transient coronary vasospasm (usually with static atherosclerosis)
Occur at rest and assoc w/ ventricular dysrhythmias
Hallmark is ST ELEVATION during pain
Dx: Angiography
Tx: Ca channel blockers and nitrates
Dressler's syndrome
Post-MI: fever, malaise, pericarditis, leukocytosis and pleuritis.  Weeks to months after MI
Tx: Aspirin
Mitral stenosis
Almost always due to rheumatic heart disease
Pulm congestion
Can cause pulm HTN->RVF
exertional dyspne, orthopnea, PND, palpitations, chest pain, thromboembolism, hemoptysis
opening snap+low-pitched diastolic rumble.  Loud S1
Dx: CXR (LA big), echo: LA big, thick valve, "fish mouth" shape
Tx: Diuretic for pulm edema, endocard prophyl, warfarin
Balloon or replace valve when severe
Aortic stenosis
Causes: Calcified bicuspid valve (or tricusp in elderly), rheumatic fever
Sx: angina, syncope, heart failure sx
Harsh crescendo-decresdendo systolic mumur, radiates to carotids. S4, parvus et tardus
Dx: CXR, ECG (LVH), ECHO (LVH, thick valve, dilated aortic root). Cardiac cath definitive
Tx: Replace valve in all patients with symptoms
Aortic regurgitation
LV dilation and hypertrophy w/ the extra volume. Eventually, inc left-sided pressures
Causes: Acute-endocarditis, trauma, dissection
Chronic-valvular disease (rheumatic, bicuspid valve, marfan's, ehlers-danlos, ankylosing spond, SLE), aortic root disease (syphilis, osteo imperfecta, dissection, behcet's, reiter's, HTN)
Sx: DOE, PND, orthopnea, palpitations, angina
Widened pulse pressure, diastolic decresendo murmur, 'water-hammer' pulse
Dx: CXR (LVH, dilated aorta), ECG (LVH), ECHO, cardiac cath
Tx: if no Sx-salt restriction, diuretics, vasodilators, digoxin, afterload reduction
Valve replacement if Sx
Endocarditis prophyl before dental and GI procedures
Mitral regurgitation
Causes: acute-endocarditis, papillary muscle rupture (ischemia/infarct). Chronic-rheumatic fever, marfan's, cardiomyopathy
Sx: DOE, PND, orthopnea, palpitations, pulm edema.
Holosystolic murmur at apex, S3 gallop, afib common.
Dx: CXR (big LV, pulm edema), ECHO (MR, big LA+LV)
Tx: reduce afterload w/ vasodilators. also salt reduction, diuretics, digoxin, antiarrhythmics. warfarin if afib.
Repair/replace valve before LV too dmged
Tricuspid regurgitation
Causes: usually RV dilation of any sort, also endocarditis (IVDA), rheumatic heart dz, epstein's anomaly (Li), carcinoid, SLE
Sx: RVF, pulsatile liver, blowing holosystolic murmur.
Dx: Echo
Tx: Treat underlying cause. If severe and no pulm HTN, then surgical correction possible
Mitral valve prolapse
Connective tissue disorders
Sx: most asymptomatic forever. May have palpitations and pain.
Mid or late systolic click, mid-to-late systolic murmur.
Standing and valsalva increase murmur, squatting decreases
Dx: echo
Tx: if systolic murmur, prophylaxis for dental procedures
B blockers for pain sometimes
Rheumatic heart disease
after group A strep. causes valvular abnormalities; mitral stenosis most common
Dx: 2 major, 2 minor.
Major: migratory polyarthritis, erythema marginatum, cardiac disease, chorea, subcutaneous nodules
Minor: fever, ESR, polyarthralgias, hx of rheum fever, long PR, preceding strep infx
Tx: Treat the strep w/ penicillin or erythromycin to prevent. Acute rheum fever tx w/ NSAIDs. (CRP to monitor Tx).  Give prophyl abx before dental/GU if hx of rheum fever
wiskott-aldrich
recurrent infections, eczema, and thrombocytopenia. poor response to capsular antigens and fungal/viral septicemia due to T cell dysfunction
pityriasis rosea
herald patch (annular, scaley, red). salmon-colored, xmas tree distribution, following skin lines.
wilms\' tumor
abdominal pain, mass, HTN. also anemia, hematuria, fever. HTN from renal artery pressure->ischemia. smooth+firm, rarely cross midline 3 years median age assoc w/ hemihypertrophy, aniridia, horseshoe kidney, hypospadia
Poststreprococcal GN
Most common cause of nephritic syndrome (hematuria, proteinuria).
10-14 days after URI or skin GAS infection
Mostly children age 2-6
Sx: hematuria, edema, HTN, low complement, proteinuria.
ASO may be elevated
self-limited with excellent prognosis. Can sometimes turn into rapidly-progressing GN in adults
lumpy bumpy appearance on light microscopy (immune complexes in GBM)
Tx: supportive - anti-HTN, loop diuretics
Goodpasture's syndrome
Classic triad: Proliferative glomerulonephritis, pulmonary hemorrhage, and IgG anti-glomerular basement membrane antibody

Clinical sx: fever, myalgia, rapidly progressive renal failure, hemoptysis, cough, dyspnea.

Lung disease first by days-weeks.

Can follow flu or kidney surgery

Dx: anti-GBM antibodies, kidney biopsy

Tx: steroids and immunosuppressants
Acute interstitial nephritis
Inflammation of interstitium (not glomerulus or tubules)
Usually allergic reaction. (penicillins, NSAIDs, diuretics, anticoagulants, phenytoin, sulfas)
Can be due to infection, esp in kids. Strep spp and legionella
Collagen vascular diseases like sarcoid too
AIN causes ARF, and classically also fash, fever, eosinophilia. Maybe pyuria and hematuria
Dx: Increased BUN and Cr, Eosinophils in urine.
Chronic interstitial nephritis
Slowly progressive form of interstitial nephritis. NO signs or sx of hypersensitivity. (unlike AIN)
Causes: prolonged UT obstruction, reflux nephropathy, NSAIDs, heavy metals, arteriolar nephrosclerosis w/ HTN.
Renal papillary necrosis
Mostly assoc w/ analgesics, DM, sickle cell, UT obstruction, UTI, alcoholism, transplant rejection.
Dx by excretory urogram
Sloughed papillae can cause ureteral obstruction
Renal tubular acidosis
Disorder of renal tubules that leads to a non-anion gap hyperchloremic metabolic acidosis.  Glomerular function is normal.
Acidemia and urine alkalosis.
Type 1 = distal = inability to excrete H+ into urine.
Hypokalemic, hyperchloremic, non-anion gap metabolic acidosis. Lose Na, Ca, K, SO4, PO4 into urine.  70% have renal stones (alkaline urine leads to more Ca and PO4 secretion into it).
Causes: congenital, MM, nephrotoxicity, autoimmune, analgesics.
Tx: Sodium bicarb. (also prevents stones). Phosphate salts to promote excretion of titratable acid

Type 2 = proximal = inability to reabsorb HCO3-
Same Hypokalemic, hyperchloremic, non-anion gap metabolic acidosis.
Lose Na and K in urine.
Causes: Fanconi's syndrome, cystinosis, wilson's, lead tox, MM, nephrotic syndrome, amyloidosis.
NO stones (unlike Type 1)
Tx: Underlying cause. (Can't give bicarb; that's what is being dumped). Sodium restriction increases Na resorbtion, which pulls HCO3- with it.

Type 4 = low aldosterone.
Results in HYPERkalemia (unlike others)
Common w/ interstitial renal disease and diabetes.
Decreased Na absorption and decreased H+ and K+ secretion
Hartnup syndrome
Auto recess loss of amino acid transporter (uncharged AAs).  Causes nicotinamide deficiency.
Sx similar to pellagra: dermatitis, diarrhea, ataxia, psych disturbances.
Give nicotinamide if sx
Fanconi's syndrome
Defective proximal tubule transport of: glucose, AA, Na, K, PO4, uric acid, and HCO3-. Lose all of these in urine.
Tx w/ PO4, K, alkali and salt supplementation

minimal change disease
nephrotic presentation. most common in kids. assoc w lymphoma no histo on light; fusion of foot processes on electron responds to steroids. excellent prognosis
focal segmental glomerulosclerosis
name = some parts of some glomeruli affected. usually nephrotic presentation. often have HTN and hematuria too. primary: nephrotic secondary: kidney failure and proteinuria. hiv, drrugs, familial, etc Tx: diuretics, ACEI, immunosuppressives. fair to poor prognosis
membranous glomerulonephritis
immune complexes in basement membrane - \"spike and dome\" appearance. glomerular capillary walls thickened. 85% primary. secondary: hep C/B, drugs (gold, captopril, penicillamine), neoplasm, lupus. nephrotic presentation. tx immunosuppression. fair to good prognosis. 40% remission
IgA nephropathy - berger\'s
asymptomatic hematuria/mild proteinuria. most common cause of glomerular hematuria. mesangial IgA and C3 deposition common pres: hematuria day or 2 after URI. good prognosis.
alport\'s syndrome
glomerulonephritis, hearing loss. can have lens bulging. almost always have hematuria. can also have pyuria, proteinuria, high-freq hearing loss. x linked or auto recessive no tx
membranoproliferative glomerulonephritis
secondary glomerular disease. usually due to hep C. could also be hep B, syphilis, lupus. assoc w/ cryoglogulinemia deposits in GBM and mesangium (membranous only in GBM). deposits activate complement and cause dmg. tram-tracking of GBM and mesangial thickening
Aortic stenosis
Murmur:
  • Harsh late-peaking crescendo-decrescendo systolic murmur
  • Heard best- left 2nd ICS
  • Radiation to the carotids.
Possible associated findings:
  • Abnormal carotid pulse
  • Diminished and delayed ("pulsus parvus and tardus")
  • Sustained Apical impulse
  • Calcified aortic valve on CXR
Mitral Regurgitation
Murmur:
  • Blowing holosystolic murmur
  • Heard best at the apex
  • Radiation to the axilla and inferior edge of left scapula.
Possible associated findings:
  • S2: wide physiologic splitting
  • S3
Aortic insufficiency
Murmur:
  • Soft blowing early diastolic decrescendo murmur
  • Heard best at the left 2nd ICS without radiation
  • May also hear systolic flow murmur and diastolic rumble (Austin Flint)
Possible associated findings:
  • Dilated apical impulse
  • Abnormal and collapsing arterial pulses
Tricuspid regurgitation
Murmur:
  • Soft holosystolic murmur
  • Heard best at the LLSB without radiation
  • Intensity increases with inspiration or pressure over liver
Possible associated findings:
  • Elevated neck veins
  • Systolic regurgitant neck vein
  • Systolic retraction of apical pulse
  • Edema, Ascites or both
Pulmonic Insufficiency
Murmur:
  • High frequency early diastolic decrescendo murmur
  • Heard best at 2nd-3rd ICS
  • Increases with inspiration
Associated findings:
  • Abnormal S2 splitting
  • Sustained pulmonary hypertension
Pulmonary stenosis
Murmur:
  • Harsh crescendo-decrescendo systolic murmur
  • Heard best sternal border bat 2nd or 3rd intercostal spaces
  • Increases with inspiration
Associated findings:
  • Ejection sounds heard at sternal edge, 2nd or 3rd intercostal space
  • Wide physiological splitting of S2
  • Prominent A wave of the jugular venous pulse
Mitral stenosis
Murmur:
  • Low frequency rumbling mid-diastolic murmur, with presystolic component possible
  • Heard best at apex
  • Accentuated in left lateral decubitus position
Associated findings:
  • Apical impulse absent or small
  • Irregular pulse ( atrial fibrillation)
  • Loud S1
  • Elevated neck veins with exaggerated A wave
Hypertrophic cardiomyopathy
Murmur:
  • Harsh quality midsystolic murmur
  • Heard best LSB
  • Increases with decreased venous return
Possible associated findings:
  • Sustained apical beat to palpation
  • S4 (50% of the time)
DMII meds
Sulfonyl ureas: glipizide, glyburide, glimepiride. Secretagogues.
Meglitinides: repaglinide, nateglinide. Similar to sulfonyls.
Thiazolidinediones ('glitazones'): increase insulin sensitivity. can cause hepatotox
Also give: insulin, statins (LDL<100), ACIE (BP<130/80), ASA
Osteoporosis
BMD <2.5STDs; thin, postmenopausal women; Fx: hip, vetebral, radial;  assoc w/ smoking, caffiene, EtOH, dec estrogen, steroids
Dx: DEXA
Tx: Ca + Vit D; Bisphosphonates ("-dronates"); SERMs (raloxifene); intranasal calcitonin; Estrogen replacement short term; PTH in very high risk
Paget's disease
Inc bone turnover, both resorption and formation -> mosaic pattern; >40yrs; assoc w/ hyperPTH
Hx: asymp or aching bone pain, headaches, Fx, nerve entrapment (hearing loss)
Dx: Hx, radionuclide bone scan (most sens), inc serum alk phos
Tx: Most asympt.  Can give disphosphonates, calcitonin. NSAIDs for pain.
Complications: Pathologic Fx, cardiac, osteosarcoma
Hyperparathyroidism
80% of primary due to single adenoma.  Secondary: kidney disease. Tertiary: when secondary progresses to dysregulation and hyperCa++
Hx: stones (nephrolith), bones (pain, Fx), groans (abd pain, N/V, PUD, pancreatitis), psychiatric overtones (fatigue, depress, anxiety). Assoc w/ renal insuff and gout.
Dx: hyperCa++, hypoPO4, hypercalciuria. Radionuclide if need to localize.
Tx: if Sx, parathyroidectomy. Acute hyperCa: fluids, bisphosphonate, calcitonin.  Secondary: oral phosphate binders and dec dietary PO4.
Gastritis
Acute: often NSAIDs, EtOH, H pylori, severe illness
Chronic:
Type A: 10%. Autoantibodies to parietal cells in fundus. Pernicious anemia. assoc w/ other autoimmune. inc risk of gastric adenocarcinoma
Type B: 90%.  antrum. Either NSAID or H pylori. inc risk of PUD and gastric cancer
H pylori
Dx: urease breath test, serum IgG (exposure, not active infx), stool antigen, or endoscopic biopsy.
Triple therapy: amoxicillin, clarithromycin, omeprazole
Gastric cancer
Intestinal type: metaplasia of gastric mucosal cells. risks - nitrites, salt, few vegetables, H pylori, chronic gastritis
Diffuse type: poorly differentiated; not assoc w/ H pylori or gastritis.  Signet ring cells seen on path
Hx/PE: indigestion, loss of appetite. Then abd pain, weight loss, upper GI bleeding
(Gastric adenocarcinoma that mets to ovary = "Krukenberg tumor")
Ulcers
After a meal, pain from Gastric ulcer is Greater, while Duodenal pain Decreases
Zollinger-Ellison
Gastrin-secreting tumor in duodenum or pancreas. 20% assoc w/ MEN I (pancreas, pit, parathy).
Dx: inc fasting serum gastric, or inc gastric after a secretin stim test.  Octreotide scan to localize.
Tx: PPIs and resection
Acute and chronic diarrhea causes
Acute
Pre-formed toxins: S aureus, Bacillus cereus
Noninvasive bacteria: enterotoxigenic E coli, Vibrio cholerae, C. difficile
Invasive bacteria: enteroinvasive E coli, Salmonella, Shigella, Campylobacter, Yersinia
Parasites: Giardia, Entamoeba histolytica
Opportunistic: Cryptosporidium, Microsporidium, CMV
CHRONIC
increased secretion: carcinoid, VIPoma
Malabsorption/osmotic: Bacterial overgrowth, pancreatic insufficiency, mucosal abnormalities, lactose intolerance
IBD
inc motility: IBS
Camplyobacter diarrhea
G- spiral, microaerophilic, flagella.
most common etiology of infectious diarrhea. Contaminated food or water. Young children and young adults. 7-10 days.
Fecal RBCs and WBCs. Often bloody
Tx: Erythromycin
C difficile diarrhea
G+ anaerobic, spore-forming rods. 
After recent tx w/ antiobiotics (CLINDA, penicillins, cephalosporins).  Watch for toxic megacolon
Fever, abd pain, fecal RBCs and WBCs. 
Dx: C diff toxin in stool. Sigmoidoscopy shows pseudomembranes
Tx: PO metronidazole or vanco
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