Rx: Flash 250 Flashcards

1
Q

SSPE (Subacute Sclerosing Panencephalitis)

A
  • Rare complication of measles (Rubeola) that occurs 7-9 years after the initial infection
  • Initially characterized by personality changes, lethargy, difficulty in school and odd behavior.
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2
Q

Factor V Leiden (AD)

A
  • Most common inherited cause of hypercoagulability.
  • AD
  • Factor Va is resistant to cleavage by protein C.
  • PE, DVT, and high D-dimer
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3
Q
  • Primary or secondary to autoimmune diseases such as SLE.
  • Associated w/ fetal loss, arterial and venous thrombosis.
  • Diagnosed in the presence of a lupus anticoagulant, which produces prolonged PTT that is not correctable by mixing 1:1 with fresh frozen plasma.
  • Increase in coagulation time due to antibodies directed against platelet phospholipids
A

Antiphospholipid Antibody Syndrome (APA)

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4
Q

Heparin-induced thrombocytopenia (HIT)

A
  • Prothrombotic, low-platelet state (platelets being used) that typically occurs 5-14 days after the initiation of heparin therapy.
  • Antibodies are formed against heparin-platelet factor 4 complexes.
  • Activated platelets cause stroke, MI, or DVT.
  • Cessation of all heparin-containing products→ anticoagulation with direct thrombin inhibitors
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5
Q

HLA-DR4

A
  • RA and diabetes mellitus (OM→G I Need DR. 4 my RA)

- RA can also be DR1 or both

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6
Q

Stylopharyngeus muscle: orgin, insertion, action, innervation

A
  • Origin: styloid process
  • Insertion: thyroid cartilage.
  • Action: elevates pharynx and larynx during swallowing and speaking.
  • Innervation: CN IX (glossopharyngeal)
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7
Q

Neurofibromatosis TYPE 1 (AD)

A
  • AD (NF1 gene=17)
  • NF1→ Café-au-lait spots, cutaneous neurofibromas, Lisch nodules in the iris, central nervous system tumors such as optic gliomas and astrocytomas.
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8
Q

Leber Hereditary Optic Neuropathy (LHON)

A
  • Mitochondrial mutation

- Degeneration of optic nerve with rapid central vision loss leading to a central scotoma that is permanent

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9
Q

Von Hippel-Lindau Syndrome

A
  • AD
  • Abnormal blood vessel growth leading to angiomas and hemangioblastomas in the retina, brain and spinal cord.
  • Cystic growths in the kidneys and pancreas
  • Pheochromocytomas (resulting in essential hypertension)
  • Islet cell tumors and clear cell renal carcinoma
  • Untreated retinal hemangiomas can rupture leading to retinal detachment
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10
Q

Von Hippel-Lindau Syndrome INHERITANCE

A
  • AD

- Deletion of VHL gene on the short arm of chromosome 3.

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11
Q

Astrocyte

A
  • GFAP+
  • Potassium metabolism
  • Maintaining blood brain barrier
  • Repair and support cells of central nervous system
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12
Q

Guillain-Barre Histology

A

Histology characterized by: perivenular and endoneurial infiltration with lymphocytes, macrophages and plasma cells.

  • Classically follows a diarrheal illness (campylobacter jejuni)
  • Molecular mimicry
  • Respiratory Acidosis
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13
Q

Gout Extra-articular manifestation

A

Gouty nephropathy is most common extra-articular manifestation of chronic gout. 20% of chronic gout patients will die from renal failure.

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14
Q

-YeLLow when parallel and blue when perpendicular

A

Gout

-Monosodium urate is negatively biofirengent

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15
Q

Hemophilia A (8) or B (factor 9) inheritance

A

-X-linked recessive (MD same)

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16
Q

Toxic Shock Syndrome

A
  • Staph Aureus exotoxin (TSST-1) SUPERANTIGEN
  • Non-specifically cross-links MCH-II molecules to T-lymphocytes
  • 2-20% of T-cells activated
  • Supraphysiological production of cytokines:
    • IL-1, TNF-alpha, IL-6, IL-12, INF-gamma
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17
Q
  • Glycogen storage disorder
  • Glycogen phosphorylase is deficient in muscle
  • Enzyme liberates glucose-1-phosphate from branches of a glycogen molecule
  • Adolescence or early adulthood
  • Muscle cramping, rapid fatigue, and poor endurance during exertion
  • Severe myoglobinuria can be seen
A

McArdle Disease

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18
Q
  • Weakness, weight loss, and a hyperviscosity syndrome
  • B-cell neoplasm with overproduction of IgM leading to…
  • Blood vessel damage, impaired cranial blood flow (headaches) and ocular blood flow (visual disturbances).
  • A monoclonal “M spike” is seen in the gamma region of serum protein electrophoresis
A

Waldenstrom Macroglobulinemia

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19
Q

L5-S1 Herniation

A
  • Radiculopathy of S1 nerve root
  • Decreased sensation of posterior lateral leg and lateral foot
  • Weakness in toe and plantar flexion and foot inversion
  • Ankle jerk test
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20
Q
  • Complication of allogeneic blood or marrow transplantation
  • Mediated by donor lymphocytes reacting with MHC antigens on recipient cells that are recognized as foreign.
  • Occurs weeks after transplant
A

Acute Graft-Versus-Host Disease (GVHD)

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21
Q

Carcinoembryonic antigen (CEA)

A

-Embryonic protein elevated and can be used as tumor maker in 70% of colorectal and pancreatic cancers. As well as in gastric and breast cancers.

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22
Q

Adenocarcinoma in lungs markers?

A

TTF-1

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23
Q

Squamous Cell Carcinoma markers?

A

p63 and CK 5/6

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24
Q

Hairy Cell Leukemia

A
  • TRAP+ (found in B-lymphocyte neoplasms like HCL)
  • Vague symptoms: Fatigue and easy bruising
  • Anemia and thrombocytopenia
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25
Q
  • Present with focal weakness
  • Tumor marker bombesin
  • N-MYC
A

Neuroblastoma

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26
Q

Ortner Syndrome

A
  • Enlarged LEFT atrium compressing the LEFT recurrent laryngeal nerve
  • Hoarseness
  • LEFT recurrent loops under aortic arch
  • Palatoglossus muscle receives innervation from vagus nerve (and others) affected
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27
Q

What is the action of the gluteus maximus?

A

-Extends and laterally rotates the thigh and aids in standing from the siting position.

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28
Q

What innervates the gluteus maximus?

A
  • Inferior gluteal nerve (commonly injured in posterior hip dislocation)
  • Damage prevents person from walking up steps or jumping
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29
Q

What are the other electrolyte abnormalities of NMS?

A

Hyperkalemia, hyperuricemia, hyperphosphatemia and Hypocalcaemia (PUcK)

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30
Q

-Aggregates of alpha-synuclein

A

DLB (Dementia with Lewy Bodies)

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31
Q

TTP (thrombotic thrombocytopenia purpura)

*FAT-RN

A

F-fever, A- microangiopathic hemolytic anemia (schistocytes) T- Thrombocytopenia (leading to bruising and petechiae) R- Renal failure N- Neurological disturbances

  • Ab against ADAMS13 protease (vWF metalloprotease)
  • Large multimers of vWF are not cleaved and accumulate → formation of platelet micoaggregates→ occlude small vessels throughout the body (multiple punctate red lesions inside the mouth)
  • Tx: plasma exchange to remove antibody
32
Q
  • Most common serum monoclonal antibody (M-protein) is IgG (55%) followed by IgA monoclonal (25%)
  • Hypercalcemia, proteinuria, anemia
A
  • Multiple myeloma (plasma cell malignancy)

- Lytic bone lesions = hypercalcemia in the setting of normal alkaline phosphatase levels

33
Q

General signs of Prolactinoma due to mass effect?

A

Bitemporal hemianopsia and chronic HA

34
Q

Woman signs of prolactinoma due to increased prolactin secretion

A

Women: amenorrhea, galactorrhea, infertility, diminished libido

35
Q

Male signs of prolactinoma due to increased prolactin secretion

A

decreased libido, impotence, gynecomastia

36
Q

-“Fried egg” appearance with perinuclear halos and “chicken wire” capillary patterning.

A

Oligodendroglioma

37
Q

Oligodendroglioma

A
  • Benign tumors derived from oligodendrocytes

- (myelinate CNS) *Note one oligodendrocytes myelinates multiple CNS neurons (up to 30).

38
Q
  • Can cause liver cancer by intercalating into host DNA
  • Mycotoxin produced by Aspergillus Flavus growing on peanuts and grains
  • Regulated in USA so most likely seen in Asia and Africa
A

Aflatoxin

39
Q
  • AD
  • Characterized by: Colorectal tumors (GI bleeding) and Brain Tumors (Seizures)
  • Two dominate forms:
    1) APC gene: polyposis and medulloblastoma
    2) hMLH1 DNA mismatch repair gene: polyposis and glioblastoma multiforme
A

Turcot Syndrome (AD)

40
Q

Turcot Syndrome (AD) forms

A

1) APC gene: polyposis and medulloblastoma

2) hMLH1 DNA mismatch repair gene: polyposis and glioblastoma multiforme

41
Q

-MOST COMMON tumor associated with electrolyte abnormality.

A
  • SCLC can secrete many different hormones including ADH (SIADH)→Hyponatremia→ seizures and lower threshold for seizures
  • Others paraneoplastic syndromes associated with SCLC are Cushing’s and Lambert Eaton.
42
Q
  • Bi-lobed, owl-eyed nuclei that stain positive for CD15 and CD30
  • Seen in Hodgkin lymphoma…EXCEPT lymphocyte-predominant Hodgkin lymphoma which features variant Reed-Sternberg Cells
  • B-cells associated with Hodgkin lymphoma may express T-cell marker interleukin-21
A

Reed-Sternberg Cells

43
Q
  • Cannot rotate arm laterally (pitching injury)

- Innervated by suprascapular nerve

A

Infraspinatus tear

44
Q

Splenectomy

A
  • Blunt trauma most commonly causes splenic injury. If removed:
  • Highly susceptible to encapsulated organisms: Neisseria meningitides, Strep pneumonia, and H.flu* (vaccinate for these three first two weeks after Splenectomy).
  • Also susceptible to Klebsiella
  • Howell Jolly bodies
  • Target cells
45
Q

What is the most common nonhematopoietic bone tumor?

A

Osteosarcoma

46
Q

Osteoporosis Labs

A

-DOES NOT produce gross metabolic abnormalities

47
Q

Osteosarcoma

A
  • Metaphyseal region of long bones
  • Sunburst pattern
  • Rb gene (also associated with retinoblastoma)
  • Males less than 20 years of age
48
Q
  • Pro-inflammatory cytokine
  • Induce acute-phase protein synthesis
  • Produced by T-lymphocytes
A

IL-6

49
Q
  • Metaphyseal region of long bones
  • Sunburst pattern
  • Rb gene (also associated with retinoblastoma)
  • Males less than 20 years of age
A

Osteosarcoma

50
Q
  • Anti-inflammatory cytokine
  • Produced by monocytes and regulatory T-cells
  • Inhibits activated T-cell responses.
  • Similar function to TGF-beta in many disease processes, as both molecules inhibit inflammation and promote resolution and healing.
A

IL-10

51
Q
  • T-lymphocyte growth factor

- Produced by Th cells

A

IL-2

52
Q

IL-5

A
  • Stimulates B-cell growth and immunoglobulin secretion (IgE)
  • IgA class switching
  • Produced by Th2 cells and mast cells
53
Q

Richter’s Transformation

A

SLL and CLL→ to large B cell lymphoma

54
Q

IL-9

A
  • Produced by T-lymphocytes

- Plays a role in mast cell activation

55
Q
  • Bilateral acoustic neuromas
  • Mutation of a tumor suppressor gene merlin located on Chromosome 22
  • Optic gliomas and juvenile cataracts (2 eyes 2 ears)
A

Neurofibromatosis Type 2

56
Q

Syringomyelia

A
  • Usually C8-T1
  • Enlargement of central canal of the spinal cord
  • Chiari Type I malformation (Downward herniation of cerebellar tonsils into foramen magnum)
57
Q

Syringomyelia what is damaged?

A

-Crossing fibers of spinothalamic tract are damaged (loss of pain and temperature) with preserved dorsal column function (intact position and vibratory sense)

58
Q
  • Evidenced by tingling, numbness, pain, and purpura at the extremities is vasculitis associated with eosinophilia, asthma, and …
  • Granulomatous (and necrotizing) vasculitis affecting small vessels
  • MPO-ANCA (p-ANCA)
A

Churg-Strauss syndrome

59
Q

Schizophrenia:

Dysfunction in mesolimbic pathway

A

positive symptoms

60
Q

Schizophrenia:

Dysfunction in mesocortical

A

Negative symptoms (SOCO is gross)

61
Q
  • Autoimmune disease with Ab’s against presynaptic voltage gated calcium channels
  • Symptoms tend to improve with repetitive nerve and muscle stimulation (opposite of myasthenia gravis in which symptoms worsen with stimulation).
A

Lambert-Eaton

62
Q

Macular sparing

A
  • Occurs with occipital lobe lesions that spare the occipital pole (extreme posterior of lobe)
  • PCA supplies occipital lobe and infarcts that spare occipital pole → spare central vision (macula fibers).
63
Q
  • Symptoms range from amnesia and behavioral changes (ex: hypomania) to Kluver-Bucy syndrome (characterized by loss of anger or fear responses and hypersexuality)
  • Can lead to coma and death in later stages
A

HSV-1 Encephalitis

Note:

  • Mildly elevated levels of proteins and RBCs
  • Temporal lobe
64
Q

Smudge Cells

A

Chronic Lymphocytic Leukemia (CLL)

65
Q

Chronic Lymphocytic Leukemia (CLL)

A
  • Clonal B-lymphocytes arrested in the B-cell differentiation pathway, intermediate between pre-B and mature B cells
  • Presents in elderly individual with hepatoslenomegaly and LAD
66
Q
  • symptoms often become worse after an increase in body temperature (ex: hot shower or fever)
  • Most common among white women ages 20-30
  • Other symptoms: hemisensory, hemiparesis, optic neuritis, internuclear opthalmoplegia and/or bladder and bowel incontinence
A

MS

67
Q

Lab values of neuroleptic malignant syndrome (PUcK)

A
  • Increased Creatine phosphokinase
  • Leukocytosis and thrombocytosis
  • Hypocalcemia
  • Metabolic acidosis
  • Elevated liver function tests
  • Blood and urine myoglobin and hyperuricemia
68
Q
  • Life long pattern of peculiar or eccentric behavior
  • Odd speech and thinking
  • Magical beliefs
  • Social isolation
  • Mild paranoia
  • Social Anxiety
  • Unusual perceptual experiences
  • Inappropriate or constricted affect
A

Schizotypal personality disorder

69
Q

Multiple Sclerosis

A
  • Type IV hypersensitivity disease
  • BETA-INTERFERON FOR TREATMENT
  • Separation of lesions in time and space on T2 flair MRI
  • Inflammatory and demyelinating process that produces periventricular plaques characterized by oligodendrocytes loss and reactive gliosis
  • Skin rash a less known symptom
70
Q

Internuclear Opthalmoplegia

A
  • If right eye affected person will “see double” when looking to left
  • If right eye: when look to left right eye cannot adduct and left eye will abduct however with nystagmus.
71
Q

HLA-DR5

A

pernicious anemia and Hashimoto thyroiditis

72
Q

SLE, MS, and Goodpasture syndrome

A

HLA-DR2

73
Q
  • Inherited defect in platelet adhesion due to decreased surface expression of glycoprotein Ib
  • Increased bleeding time
  • Low platelet count (due to absence of GPIba and the filamin A-binding site)
  • Normal PT and PTT
  • Peripheral blood smear = GIANT PLATELETS
A

Bernard-Soulier (Big Suckers)

74
Q
  • Most common inherited bleeding disorder (AD)
  • Increased bleeding time
  • Increased PTT
  • vWD is a ligand for platelet adhesion to a damaged vessel wall and the plasma carrier for factor VIII
  • Can be acquired also via malignancy, autoimmunity, or drug therapy
A

vWD laboratory findings

75
Q

DiGeorge Syndrome

-CATCH-22

A
  • Cleft palate
  • Abnormal facies
  • Thymic aplasia (T-lymphocyte deficiency)
  • Cardiac defects (Truncus Arteriosus)
  • Hypocalcemia
  • 22q11 chromosomal deletion
  • Thymus is derived from third branchial (pharyngeal) pouch
76
Q

Central Pontine Myelinolysis

A
  • Affects primarily corticospinal and corticobulbar tracts
  • Rapid over correction of hyponatremia
  • Corticobulbar damage: dysphagia, dysarthria, or paralysis of the head and neck musculature
  • Can lead to “locked-in syndrome”
77
Q

“locked-in syndrome”

A
  • Only oculomotor and trochlear nerves are uninjured
  • Blinking and up-gaze = only communication
  • Lesion in base of pons (BASILAR ARTERY)