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CRRAB I Mike Rose > Diseases for Quiz 5 (final week) > Flashcards

Diseases for Quiz 5 (final week) Flashcards

1
Q

Iron Overload Cardiomyopathy

A

Heriditary of transfusional hemachromatosis

Chronic/years long accumulation of excess iron (hemosiderin) in heart, liver, lungs, endocrine glands, joints, skin

Clinical sequelae: cardiomyopathy/heart failure (usually dilated type +/- conduction disturbance, cirrhosis/hepatoma, endocrine dysfunction (diabetes), arthritis, and skin darkening (bronze diabetes)

Excess hemosiderin induces myocyte toxicity and secondary fibrosis.

Dx: >45% transferrin sat, HFE gene mutation, hepatic iron index (liver biopsy)

Rx: phelbotomy

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2
Q

Cardiomyopathy due to cardiotoxic drugs

A

Especially secondary to anthracycline (doxo and daunorubicin) dose-dependent myocyte toxicity.

May resolve post drug cessation

Can cause dilated or restrictive CM w/ HF

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3
Q

Amyloidosis

A

Usually systemic deposition of amorphous proteinaceous substance.

CONGO RED positive

Clinical: CHF, nephrotic syndrome and peripheral neuropathy.

Typically primary (AL.monoclonal protein/derived –> multiple myeloma)

If transthyretin-derived: may be genetic/heart limited.

Typically restrictive CM w/ low EKG voltage.

Dx: tissue biopsy

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4
Q

Myocarditis

A

Myocardial inflammation associated w/ normal-sized or dilated heart.

Multiple causes:

Infection- viral most common in US (adenovirus/coxsackie B, adeno, parvo) Also lymes in endemic areas.

Chagas in developing world

Immune mediated: RF, post viral infection, SLE polymyositis (libman sacks), heart transplant rejection. a

Unknown cause: Sarcoidosis, giant cell myocarditis

Clincal: Acute HF w/ viral prodrome. May also have chest pain, arrhythmia, sudden death

Common cause of HF in children

Cardiac enzymes may be increased.

Rx: supportive Rx for HF and rate/rhythm control. LVAD if severe. Many will improve gradually w/ or w/o Rx.

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5
Q

Acute pericarditis

A

Most common pericardial disorder

Developed countries: viral –> usually self-limited course

Developing countries: HIV/AIDS and TB

Clincal features: Chest pain (>95%) typically sharp and pleuritic (lessened by sitting/leaning forward)

Pericardia friction rub

EKG w/ new widespread ST elevation

Pericardial effusion present in 60% of cases

Rx: bed rest and anti-inflammatory drugs

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6
Q

Chronic/recurrent pericarditis

A

May require pericardial surgical window (pericardiectomy) or installation of sclerosing agents to obliterate pericardial space.

May result in chronic constrictive pericarditis. –> impaired vent filling caused by thick scar. (sx = HF (often right) / mimic restrictive CM.

Pathology:
-Fibrinous/serofibrinous: serous fluid and fibrinous exudate is most common
-Hemorrhagic (malignancy is first consideration)-
Granulomatous (TB)
-myopericarditis (can have elevated troponin levles

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7
Q

Myxoma

A

Commonest primary heart neoplasm. Most commonly found in atria (left).

Sx: from mechanical blockage of AV valves or embolization

Dx: ECHO

Rx: Surgical excision

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8
Q

Rhabdomyoma

A

Commonest primary cardiac neoplasm in children particularly those w/ tuberous sclerosis.

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9
Q

Eisenmenger’s Syndrome

A

Untreated Large L –> R shunt. Eventual extreme pulmonary HTN which reverses the flow and generally causes death.

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10
Q

Pulmonary stenosis/atresia

A

Isolated or part of TOF

RV hypertrophy or hypoplasia

PDA may be vital to survival until surgical repair (PGE1)

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11
Q

Aortic stenosis/atresia

A

severe disease: LV hypoplasia (hypoplastic left heart syndrome –> incompatible with life)

PDA may be vital to survival until surgical repair (PGE1)

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12
Q

Sudden Cardiac Death

A

85% due to atherosclerotic CAD or diagnosiable cardiomyopathy

5-10% of older adults w/ sudden presumed cardiac death: negative for any compelling gross or microscopic cardiac pathology

Young athletes: hypertrophic cardiomyopathy

Children: myocarditis and coronary artery abnormalities (arise from wrong cusp –> ischemia)

LongQT
Brugada syndrome
Catecholaminergic polymorphic ventricular tachycardia
commotio cordis = ventricular dysrhythmia due to blunt chest trauma
Fright response = vfib due to overwhelming symp discharge

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13
Q

Hypertension ***

A

Affects up to 30% of adult U.S. population.

Affecting small muscular arteries –> hyaline arteriolosclerosis (benign HTN) or hyperplastic arteriolosclerosis/fibrinoid (malignant HTN)

95% essential (multifactorial/familial causation)

If untreated 50% mortality from ischemic heart disease or CHF and 33% mortality from stroke

5% have secondary/potentially reversible disease (especially renal disease and adrenal/endocrine disorders)

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14
Q

Secondary HTN ***

A

5% of HTN –> don’t want to miss!

Clues:

  • severe HTN despite multiple antihypertensive agents used
  • Acute BP rise in patient w/ previously stable BP
  • Age
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15
Q

Fibromuscular dysplasia (FMD) ***

A

Narrowing of medium-sized arterial lumens by usually medial fibroplasia of unknown cause esp. affecteing RENAL and CEREBRAL vessels

Young to middle aged women

String of beads angiographic appearance from alternating fibromuscular webs/stenosis and aneurysmal dilations

Found in workup from renovascular HTN or cerebrovascular disease.

Dx: Bruits on physical exam,

Rx: revasculazation surgery/ angioplasty

KEY POINT: consider FMD in any young adult w/ refractory or malignant HTN or stroke.

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16
Q

Cushing’s syndrome

A

AKA hypercortisolism.

Cause of secondary HTN

From Rx, paraneoplastic, etc.

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17
Q

Pheochromocytoma

A

NE secreting tumor

paroxysmal HTN w/ headaches, palpitations, diaphoresis

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18
Q

Primary Aldosteronism

A

Increased mineralocorticoid states:

Lab clues: HYPOKALEMIA (less than 50%); plasma aldoseterone/renin ratio.

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19
Q

Hypertension Encephalopathy

A

Loss of autoregulation of cerebral blood flow:

  • vasodilation and HTN
  • endothelial injury (fibrinoid necrosis)
  • lumen obliteration causing cerebral edema
  • headache, N/V, focal neurological signs, mental status changes, then: STUPOR, COMA, SEIZURES, DEATH
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20
Q

HTN end-organ pathology

A
  • LVH
  • CHF and sudden death
  • Increased risk of CAD
  • Stroke
  • Microvascular aneurysm rupture (hemorrhage in deep white matter, basal ganglia or brainstem = most lethal CNS vascular event
  • Large vessel or lacunar infarct
  • HTN encephalopathy
  • Renal disease (two mechanisms –> lumen stenosis, loss of renal blood flow autoregulation: hyperperfusion –> glomerulosclerosis from malig HTN)
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21
Q

Diabetic Vascular Implications ***

A

Magnifies vascular morbidity

  • increases large vessel atherosclerotic risk
  • has synergistic effects w/ HTN in arteriolar lumen (non-enzymatic glycosylation w/ protein migration

Common cause of non-bypassable PAD

Skin ulceration w/ secondary infx (MRSA) w/ frequent bone involvement (osteomyeltis) and commonly distal leg infarction = gangrene

Diabetic retinopathy, nephropathy, neuropathy

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22
Q

Calciphylaxis

A

Systemic calcification of arterioles w/ intimal fibrosis and lumen obstruction

Typically uremic patients (ESRD)

Causing painful ischemic skin necrosis and secondary ulceration.

Rx: skin debridement - aim to keep Ca x P less than 55 and normalize PTH levles

*almost always dialysis patients

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23
Q

Type A Aortic Dissection

A

Ascending aorta (+/- involvement of arch/descending aorta)

Majority of dissections

Debakey I ( ascending + arch & descending)
Debakey II (Ascending only)

Rx: surgery ASAP

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24
Q

Type B Aortic dissection

A

Descending throracic aorta and/or the arch

Debakey III (Descending/arch only)

Medical management possible

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25
Q

Vasculitis ***

A

Vascular Wall inflammation
-ischemic effects often in multiple organ systems (abdominal pain, HTN or renal insufficiency w/ hematuria, leukocyturia, cutaneous rash -palpable purpura, neurologic/opthalmic sx)

  • often w/ consitutionals sx (fever, malaise, myalgias, arthralgias
  • overall sx often non-specific (ddx infx, coagulopathyies, neoplasms, drug toxicity, atheroembolism, amyloidosis, migraine)
  • mononeuritis mulitples (asymmetric polyneuropathy)

Diseases:

  • ANCA mediated (wegener’s, microscopic polyangiitis, Churg-strauss syndrome)
  • -Giant cell arteritis
  • Takayasu arteritis
  • Kawasaki disease
26
Q

Takayasa arteritis

A

Granulomatous vasculitis affecting large/medium sized arteries especially aortic arch and branch vessels.

Causes lumenal stenosis w/ downstream ischemic effects (weak arm pulses/claudication/raynaud’s, neuro/ocular sx, abdominal pain, HTN/renal failure, leg claudiction)

Prefers adolescent/young adult women

AKA aortic arch syndrome or pulseless disease

Chronic/relapsing w/ risk of stroke, CHF, MI, renal failure, aneurysm rupture

Rx: glucocorticoids (+/-MTX)

ddx: fibromuscular dysplasia

27
Q

Giant Cell Arteritis ***

A

Aka temporal arteritis

Most common vasculitis in the elderly in U.S. (esp. Scandanavians)

Affects medium and large arteries especially the extracranial carotid system

Feared complication = VISION LOSS from ischemic optic neuropathy

Dx: age > 50 years, localized headache of new onset, tenderness/decreased pulse over temporal artery, ESR > 50 (often >100) –> temporal artery Bx

Other sx: jaw claudication, aortic arch involvement, systemic sx, POLYMYALGIA RHEUMATICA (40-50% of GCA patients), aortic aneurysm

Rx: Corticosteroids (initiate before Bx in suspected cases w/ ocular sx!

28
Q

Polyartertis Nodosa

A

Very uncommon

Segmentally affecting medium to small arteries

Neutrophilic vasculitis w/ aneurysm formation

Systemic vascultiis chiefly affecting: kidney, heart, GI, liver (30% of cases associated w/ Hep. B)

Young adults favored

Sx:

  • malaise, myalgias, fever, weight loss
  • typically HTN
  • abdominal pain/GI bleeding
  • skin rash, neuropathy, renal failure, testicular pain

Untreated: fatal in most cases (bowel infarction/perf or HTN cardiovascular disease)

Dx: biopsy of involved organs, arteriography, no serological dx

Rx: corticosteroids/cyclophosphamide (cytoxan)

29
Q

Kawasaki Disease ***

A

Mucocutaneous lymph node syndrome

Acute febrile illness of children (asians 3% death rate)

Leading cause of acquired heart disease in children

Clinical dx: bilateral conjunctival injection, oral mucous membrane changes, palmar/plantar erythema, polymorphous skin rash, cervical lymphadenopathy

Rx: IVIG + aspirin

30
Q

Leukocytoclastic (cutaneous) vasculitis ***

A

Neutrophilic small vessel vasculitis

MOST COMMON vasculitis in clinical practice

Palapable purpura: classically involving lower extremeties +/- mroe extensive skin eruption

(palpable bumps = vasculitis; smooth = petichiae)

70% of cases: two categories

  • transient , benign –> hypersensitivity rxn to a drug or anigen +/- serum sickness
  • skin manifestation of systemic vasculitis: caused by either INFECTION, or any of the vascultis syndromes except takayasu, GCA, or Kawasaki

23%infx, 20% drug, 12% CTD, 4%malignancy, 4% primary systemic vascultitis, 30% idiopathic

31
Q

Henoch-Schonlein Purpura

A

cutaneous/leukocytoclastic vasculitis with IgA deposition in patients

32
Q

Cryoglobulinemic Vasculitis ***

A

Cryglobulins = measureable cold-precipitated immunoglobulins.

predominantly monoclonal protein –> waldenstrom’s or mult. myeloma

Predominantly polyclonal immunoglobulin type –> chronic/ai inflammatory diseases (SLE, Sjogren’s) and viral infx (hep C)

Hepatitis C = most common cause of cryoglubulinemia

Cutaneous vasculitis present in nearly all patients

Associated sx: arthralgias, myalgias, weakness, neuropathy, and 10-30% w/ glomerulonephritis = poor prognosis

33
Q

Anti-neutrophil cytoplasmic antibody (ANCA) ***

A

Wegener’s granulomatosis or Microscopic polyangitis

Common cause of pulmonary renal syndrome: pulmonary infiltrates/nodules (often w/ hemoptysis) and glomerulonephritis - w/ hematuria/proteinuria

ddx: Wegener’s, microscopic polyangiitis, goodpasture’s syndrome, and SLE

34
Q

Wegener’s Granulomatosis ***

A

Systemic largely small vessel vasculitis associated w/ tissue necrosis and focal giant cell/granuloma formation

Classic triad of involvement:

  1. )ENT disease (95%)- nasal/sinus sx- sinusitis, mucosal ulceration, etc.
  2. ) Pulmonary disease (90%) - pulmonary nodules/infiltrates +/- cough, dyspnea, hemoptysis
  3. ) Kidney disease (80%) - often rapidly progressive glomerulonephritis w/ threat of ESRD

90% c-ANCA positive

Dx: tissue biopsy

Rx: corticosteroids + cyclophosphmide

35
Q

Microscopic Polyangiitis ***

A

Likely a part of a disease spectrum w/ wegener’s

Systemic vasculitis primarily affecting kidneys (80%)

Typically acute presentation w/ renal failure

Dx: ANCA positive in 75%, renal Bx

Rx: corticosteroids + cyclophosphamide

36
Q

Churg-Strauss Syndrome

A

AKA allergic angiitis and granulomatosis

Rare systemic vasculitis associated w/ SEVERE ASTHMA.

50% ANCA positive

Rx: glucocorticoids

37
Q

Misc. Small Vessel Vasculitis ***

A

Connective tissue disease associated (SLE, RA, myositis, relapsing polychondritis, Sjogren’s)

Drug induced (may be ANCA positive)

Infx: esp. rickettsial disease)

38
Q

Thromboangiitis Obliterans (Buerger’s Disease)

A

Distal extremity arteritis in heavy cigarette smokers (usually males may progress to ulceration, gangrene, amputation

Rx: Smoking cessation

39
Q

Varicose veins ***

A

Superficial veins of upper and lower legs

15% of adult males; 30% of adult females

Venous valve failure due to:

  • leg dependency for prolonged periods
  • obesity
  • pregnancy
  • familial tendency

Stasis dermatitis = congestion, edema, thrombosis, pain, chronic ulceration: rare risk for embolism

40
Q

Hemorrhoids

A

anorectal venous plexus engorgement

Can be complicated by: inflammation, thrombosis/ulceration

Associated w/ constipation/straining and pregnancy

41
Q

Esophageal varices ***

A

Potentially fatal venous engorgement:

Secondary to portal HTN (cirrhosis, portal vein thrombosis, hepatic vein thrombosis(Budd-Chiari syndrome)).

Increased portal venous pressure: opens porto-systemic shunts causing esophago-gastric varices

Risk of massive/fatal GI bleeding

42
Q

Deep Vein Thrombosis (DVT)

A

Ileofemoral >90% of DVT cases

First clinical sx may be fatal PE

+/- unilaterally swollen/tender leg

Virchow’s triad: Associated w/ variable combinations of impeded venous circulation (stasis) vs. hypercoag vs. endothelial injury/dysfunciton

Risk factors: prolonged immobilization/post-op, CHF, obesity, pregnancy, injured endothelium (inflammation/sepsis), systemic hypercoaguability (DIC, factor V leiden)

Dx: venous US w/ doppler, chest CT angio if PE suspected.

Well’s score = risk stratification

Non PE complications: post thrombotic/thrombophlebetic syndrome or visceral infarction (esp. bowel and brain)

Many people w/ DVT have both acquired and congenital risk factors for hypercoaguability.

43
Q

Disseminated Intravascular Coagulation (DIC)

A

Acute DIC w/ systemic microvascular thromboses

Chronic DIC w/ usually localized DVTs

Multiple causes: sepsis, cancer (esp. lung/pancreas), neoplastic hemic disease (AML/M3 and chronic myeloproliferative syndromes), AI diseases, antiphospholipid antibodies

Trousseau’s sign = recurrent thrombosis despite ongoing anti-coag –> CANCER

44
Q

Congential Hypercoaguability

A

Factor V leiden, prothrombin gene mutation, deficiencies in factors C, S, or antithrombin III

Suspect if unprovoked thrombis occurs in patient

45
Q

Post thrombotic/thrombophelbitic syndrome

A

Post DVT

  • Chronic venous insufficiency (from valve destruciton
  • Often severe stasis dermatitis sequelae
  • usually due to inadequate course of anti-coag w/ inadequate clot lysis
46
Q

Hemangiomas

A

Commonest vascular neoplasm. Benign

  • Dominantly perfer the skin (cherry/purple papules)
  • May involve any visceral organ: but favors liver, spleen, soft tissue

Pyogenic granuloma - rapidly growing/friable capillary hemangioma in response to trauma

47
Q

Lymphangioma

A

Uncommon w/ largest examples most frequent in neck/axilla of young children (cystic hygroma)

48
Q

Glomus tumor

A

Glomus cell proliferation associated w/ vascular channels: Painful small tumors of distal digits.

49
Q

Bacillary angiomatosis

A

Vascular proliferation associated w/ Bartonella infection (i.e. cat scratch species in immunosuppressed patients (esp. HIV)

Often mis dx w/ Kaposi’s sarcoma

50
Q

Kaposi’s sarcoma

A

Malignant vascular proliferations w/ skin +/- visceral involvemtn.

Associated w/ HHV-8

3 populations:

  1. ) HIV/AIDS
  2. ) Transplant
  3. ) Eastern European Men
51
Q

Angiosarcoma

A

Aggressive malignant vascular neoplasia. Reddish/purple lesions in skin w/ hematogenous metastases.

Patients w/ chronic lymphedema: incresaed risk of angiosacroma (i.e. post mastectomy)

Axillary dissection patients = Stewart-Treves Syndrome

52
Q

Aortic Stenosis

A

Early to mid-systolic

Heard best over the base and tends to radiate to the carotids.

Crescendo-decrescendo

Late stages may have decreased systolic pressure and slow carotid upstroke

Etiology - degenerative calcific, rheumatic, bicuspid (40s and 50s)

Pathology - pressure gradient over 50 mm or valve area represents critical obstruction

Sx: Angina, Syncope, DOE

Rx: Valve replacement

53
Q

Mitral Insufficiency (Regurgitation)

A

Holocystolic

Heard best at the apex and radiates to the axilla

Etiology: Acute MI w/ papillary muscle dysfunciton (supplied by RCA), rheumatic heart disease, congential, endocarditis, calcification w/ age.

Sx: Acute: pulmonary edema, SOB Chronic: severe fatigue, a fib (secondary to atrial enlargement)

Rx: treat CHF, Afib. Surgery for patients w/ severe MR w/ limitations

54
Q

Mitral Stenosis

A

Diastolic murmur heard best at apex w/ patient in left lateral recumbent position.

Low pitch rumble

Opening snap precedes murmur

Etiology - most a rheumatic (can be silent until excacerbated by pregnancy or other stresses)

Sx: DOE, cough, hemoptysis, arterial embolism

Rx: antibiotic prophylaxis, CHF, AFIB, Valve replacement

55
Q

Aortic Insufficiency

A

High ptiched diastolic murmur heard best at the base.

Best heard w/ patient sitting.

Increased PP–> secondary to regurge and increased SV –> bounding pulses –> quincke’s pulse (nail bed)

Etiology: 2/3 rheumatic, endocarditis, trauma (airbag)

Pathology –> increased SV –> deterioation of LV function –> sx

Sx: Asymptomatic for year. Uncomfortable awareness of heartbeat when lying down. Exertional dyspnea first sx followed by orthopnea and PND. Angina. CHF.

Rx: Difficult to time

56
Q

Mitral Valve Prolapse (click-murmur syndrome

A

Mid or late systolic click

May be followed by a a high pitched late systolic murmur, heard best at apex

Etiology: Very common. Congenital or genetic.

Females between 14-30.

Pathophys: myxomatous degeneration of posterior leaflet.

Usually benign, but may trigger arrhythmias

Rx: reassurance

57
Q

Tricuspid stenosis

A

Rare

Associated w/ mitral stensois

Giant A wave in the neck

Diastolic at LLSB

58
Q

Tricuspid regurge

A

Functional from RV dilation. Sign of right sided HF.

Holocystolic murmur at LLSB

Prominent v waves of neck.

59
Q

Pulmonic stenosis

A

Usually preceded by ejection click.

Midsytolic murmur at left 2nd and 3rd interspaces.

Benign in young people.

60
Q

Innocent murmurs

A

Always systolic ejection in nature

Occure w/o evidence of phsyiologic abnormalities

Grade I or II

No thrills or radiation

End well before S2

Found in 30-50% of children

61
Q

Leriche syndrome (aortoiliac obstructive disease)

A

fatigue of both lower extremities
erectile dysfunction
pallor and global atrophy of both lower extremities

CRRAB I Mike Rose (13 decks)

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