Rx: Flash 250 Flashcards
(77 cards)
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.
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
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)
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
5
Q
HLA-DR4
A
- RA and diabetes mellitus (OM→G I Need DR. 4 my RA)
- RA can also be DR1 or both
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)
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.
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
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
10
Q
Von Hippel-Lindau Syndrome INHERITANCE
A
- AD
- Deletion of VHL gene on the short arm of chromosome 3.
11
Q
Astrocyte
A
- GFAP+
- Potassium metabolism
- Maintaining blood brain barrier
- Repair and support cells of central nervous system
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
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.
14
Q
-YeLLow when parallel and blue when perpendicular
A
Gout
-Monosodium urate is negatively biofirengent
15
Q
Hemophilia A (8) or B (factor 9) inheritance
A
-X-linked recessive (MD same)
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
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
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
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
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)
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.
22
Q
Adenocarcinoma in lungs markers?
A
TTF-1
23
Q
Squamous Cell Carcinoma markers?
A
p63 and CK 5/6
24
Q
Hairy Cell Leukemia
A
- TRAP+ (found in B-lymphocyte neoplasms like HCL)
- Vague symptoms: Fatigue and easy bruising
- Anemia and thrombocytopenia
25
- Present with focal weakness
- Tumor marker bombesin
- N-MYC
Neuroblastoma
26
Ortner Syndrome
- 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
27
What is the action of the gluteus maximus?
-Extends and laterally rotates the thigh and aids in standing from the siting position.
28
What innervates the gluteus maximus?
- Inferior gluteal nerve (commonly injured in posterior hip dislocation)
- Damage prevents person from walking up steps or jumping
29
What are the other electrolyte abnormalities of NMS?
Hyperkalemia, hyperuricemia, hyperphosphatemia and Hypocalcaemia (PUcK)
30
-Aggregates of alpha-synuclein
DLB (Dementia with Lewy Bodies)
31
TTP (thrombotic thrombocytopenia purpura)
| *FAT-RN
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
- Most common serum monoclonal antibody (M-protein) is IgG (55%) followed by IgA monoclonal (25%)
- Hypercalcemia, proteinuria, anemia
- Multiple myeloma (plasma cell malignancy)
| - Lytic bone lesions = hypercalcemia in the setting of normal alkaline phosphatase levels
33
General signs of Prolactinoma due to mass effect?
Bitemporal hemianopsia and chronic HA
34
Woman signs of prolactinoma due to increased prolactin secretion
Women: amenorrhea, galactorrhea, infertility, diminished libido
35
Male signs of prolactinoma due to increased prolactin secretion
decreased libido, impotence, gynecomastia
36
-“Fried egg” appearance with perinuclear halos and “chicken wire” capillary patterning.
Oligodendroglioma
37
Oligodendroglioma
- Benign tumors derived from oligodendrocytes
| - (myelinate CNS) *Note one oligodendrocytes myelinates multiple CNS neurons (up to 30).
38
- 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
Aflatoxin
39
- 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
Turcot Syndrome (AD)
40
Turcot Syndrome (AD) forms
1) APC gene: polyposis and medulloblastoma
| 2) hMLH1 DNA mismatch repair gene: polyposis and glioblastoma multiforme
41
-MOST COMMON tumor associated with electrolyte abnormality.
- 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
- 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
Reed-Sternberg Cells
43
- Cannot rotate arm laterally (pitching injury)
| - Innervated by suprascapular nerve
Infraspinatus tear
44
Splenectomy
- 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
What is the most common nonhematopoietic bone tumor?
Osteosarcoma
46
Osteoporosis Labs
-DOES NOT produce gross metabolic abnormalities
47
Osteosarcoma
- Metaphyseal region of long bones
- Sunburst pattern
- Rb gene (also associated with retinoblastoma)
- Males less than 20 years of age
48
- Pro-inflammatory cytokine
- Induce acute-phase protein synthesis
- Produced by T-lymphocytes
IL-6
49
- Metaphyseal region of long bones
- Sunburst pattern
- Rb gene (also associated with retinoblastoma)
- Males less than 20 years of age
Osteosarcoma
50
- 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.
IL-10
51
- T-lymphocyte growth factor
| - Produced by Th cells
IL-2
52
IL-5
- Stimulates B-cell growth and immunoglobulin secretion (IgE)
- IgA class switching
- Produced by Th2 cells and mast cells
53
Richter’s Transformation
SLL and CLL→ to large B cell lymphoma
54
IL-9
- Produced by T-lymphocytes
| - Plays a role in mast cell activation
55
- Bilateral acoustic neuromas
- Mutation of a tumor suppressor gene merlin located on Chromosome 22
- Optic gliomas and juvenile cataracts (2 eyes 2 ears)
Neurofibromatosis Type 2
56
Syringomyelia
- Usually C8-T1
- Enlargement of central canal of the spinal cord
- Chiari Type I malformation (Downward herniation of cerebellar tonsils into foramen magnum)
57
Syringomyelia what is damaged?
-Crossing fibers of spinothalamic tract are damaged (loss of pain and temperature) with preserved dorsal column function (intact position and vibratory sense)
58
- 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)
Churg-Strauss syndrome
59
Schizophrenia:
| Dysfunction in mesolimbic pathway
positive symptoms
60
Schizophrenia:
| Dysfunction in mesocortical
Negative symptoms (SOCO is gross)
61
- 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).
Lambert-Eaton
62
Macular sparing
- 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
- 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
HSV-1 Encephalitis
Note:
- Mildly elevated levels of proteins and RBCs
- Temporal lobe
64
Smudge Cells
Chronic Lymphocytic Leukemia (CLL)
65
Chronic Lymphocytic Leukemia (CLL)
- 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
- 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
MS
67
Lab values of neuroleptic malignant syndrome (PUcK)
- Increased Creatine phosphokinase
- Leukocytosis and thrombocytosis
- Hypocalcemia
- Metabolic acidosis
- Elevated liver function tests
- Blood and urine myoglobin and hyperuricemia
68
- 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
Schizotypal personality disorder
69
Multiple Sclerosis
- 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
Internuclear Opthalmoplegia
- 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
HLA-DR5
pernicious anemia and Hashimoto thyroiditis
72
SLE, MS, and Goodpasture syndrome
HLA-DR2
73
- 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
Bernard-Soulier (Big Suckers)
74
- 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
vWD laboratory findings
75
DiGeorge Syndrome
| -CATCH-22
- 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
Central Pontine Myelinolysis
- 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
“locked-in syndrome”
- Only oculomotor and trochlear nerves are uninjured
- Blinking and up-gaze = only communication
- Lesion in base of pons (BASILAR ARTERY)
