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NBDE PART 1 MICRO/PATH > Micro/Path Diseases > Flashcards

Micro/Path Diseases Flashcards

1
Q

Addison’s disease:
Hyper or hypo:
glycemia, kalemia, natremia, tension, blood volume

A

hypoglycemia, hypotension, hyponatremia, hyperkalemia, decreased blood volume
(remember down everything except potassium) = opposite of cushing’s syndrome

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2
Q

Cushing’s syndrome:
Hyper or hypo:
glycemia, kalemia, natremia, tension, blood volume

A

hyperglycemia, hypertension, hypernatremia, hypokalemia, increased blood volume

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3
Q

oral signs of Addison’s disease

A

tan skin, diffuse pigmentation on gingiva, tongue, hard palate, and buccal mucosa.

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4
Q

will pigmentation of the mouth/skin disappear with therapy in Addison’s disease?

A

skin (cutaneous) will but oral pigmentation wont

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5
Q
  1. primary adrenocortical deficiency is?
  2. secondary adrenocortical deficiency is?
  3. tertiary adrenocortical deficiency is?
A
  1. problem at gland = adrenal gland, usually autoimmune (lymphocytic adrenalitis), can be TB (2nd most common) or metastatic tumor or infections.
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6
Q

The adrenocorticotropic hormone test (ACTH test/corticotropin test) measures ____ gland function

A

pituitary

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7
Q

The pituitary gland produces hormone ACTH which stimulates outer layer of adrenal gland (adrenal cortex). It stimulates the secretion of ___

A

glucocorticoids such as cortisol and has little control over the secretion of aldosterone (the other major hormone of adrenal cortex)

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8
Q

The ACTH test is used to determine if?

A

too much cortisol is being produced (Cushing’s syndrome) or if not enough cortisol is being produced (Addison’s disease)

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9
Q

____ adrenal insufficiency can result from prolonged or improper use of glucocorticoids which are used to treat RA, asthma, and other inflammatory illnesses

A

secondary

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10
Q

Addison’s disease is treated by administering

A

cortisol (hydrocortisone)

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11
Q

Waterhouse-Friderichsen syndrome is a __ insufficiency and __ collapse due to __ of the __

A

adrenal insufficiency and vascular collapse due to hemorrhagic necrosis of the adrenal cortex.

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12
Q

Waterhouse-Friderichsen syndrome is due to what bacteria

A

meningococcemia = associated with meningococcal meningitis

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13
Q

the adrenal cortex produces what 3 hormones

A

sex hormones, glucorticoid hormones, and mineralcorticoid hormones

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14
Q
  1. Osteoarthritis (degenerative joint disease) is the most common joint disease. it is characterized by the progressive ___ of __
A

erosion of articular cartilage

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15
Q

is osteroarthritis inflammatory or intrinsic disease of cartilage

A

more of an intrinsic disease of cartilage = breakdown of cartilage although inflamm cells are present in SMALL numbers

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16
Q

inflammation of osteoarthritis is accompanied by

A

pain swelling and stiffness

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17
Q

osteoarthritis most commonly affects which joints

A

the ones exposed to constant wear and tear = intervertebral joints, phalangeal joints (fingers), the knees and the hips

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18
Q

Osteoarthritis: Osteophytes (bony spurs) formed at ___interphalangeal joints are called Heberden nodes and the ones formed at ___ are called Bouchard nodes

A 
distal = heberden (think d comes first in heberden than in bouchard)
Proximal = Bouchard
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19
Q
  1. primary/secondary osteoarthritis occurs without known cause and is mostly related to aging
A

primary

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20
Q

primary/secondary osteoarthritis is caused by another disease or condition like obesity, repeated trauma or surgery, abnormal joints at birth, gout, DM, hormone disorders

A

secondary

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21
Q

Histiocytosis X (Langerhans cell histiocytosis) = group of diseases.

  1. Pulmonary Langerhans cell histiocytosis is most often seen in __ and may regress upon
A
  1. adult smokers, quitting
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22
Q

Histiocytosis X (Langerhans cell histiocytosis) = group of diseases.

  1. Hand Christian Schuller Disease = seen in ___ with the classic triad of?
A
  1. kids, “BED”
    Bone defects
    diabetes insipidus
    exopthalamos
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23
Q
  1. Diabetes insipidus is an uncommon condition in which the kidneys are unable to prevent the ___
  2. DI caused by a lack of ADH is called ___diabetes insipidus.
  3. When DI is caused by a failure of the kidneys to respond to ADH, the condition is called ___diabetes insipidus
A
  1. excretion of water
  2. central
  3. nephrogenic
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24
Q 
Histiocytosis X (Langerhans cell histiocytosis) = group of diseases.  
Letterer-Siwe Disease occurs in?
A

kids under 2 years old.
clinical feature = cutaneous lesions resembling a seborrheic eruption caused by infiltrates of Langerhans cells over the front and back of the trunk and scalp

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25
Q 
Histiocytosis X (Langerhans cell histiocytosis) = group of diseases. 
1. Eosinophilic Granuloma
A

proliferation of Langerhans cells mixed with eosinophils, lymphocytes, plasma cells, neutrophils. most common and most benign form of Histiocytosis X (Langerhans cell histiocytosis)

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26
Q

the outcome of Histiocytosis X (Langerhans cell histiocytosis) depends on what 2 things

A

the age at time of diagnosis and how many organs involved. younger children have higher death rate than older. and major organ problems liver/lung/bone marrow - poor prognosis

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27
Q

X-linked infantile agammaglobulinemia with a defective tyrosine kinase B cell gene which result in a lack of mature B cells. this decreases all 5 immunoglobulin isotypes and increases risk of infection what disease is this

A

Bruton disease

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28
Q

primary hyperparathyroidism is most often caused by

A

parthyroid adenoma

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29
Q

symptoms of primary hyperparathyroidism

A

painful bones, renal stones, abdominal groans, psychic moans

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30
Q

which condition causes a loss of lamina dura around multiple teeth

A

hyperparathyroidism

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31
Q

in hyperPTism affects on calcium, phosphorous, PTH?

A

increased Ca, PTH

decreased phosphorous

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32
Q
  1. primary hyperPTism is result of ___, __ or __

2. secondary is caused by condition that gives rise to

A
  1. adenoma (85-95%), primary hyperplasia (5%), carcinoma (1%)
  2. chronic HYPOcalcemia, which in turn leads to compensatory overactivity of the PTH gland.
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33
Q

in rare instance HYPOparathyroidism is associated with congenital thymic hypoplasia called

A

DiGeorge’s syndrome

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34
Q

Osteoporosis, central giant cell granulomas, and metastatic calcifications are all manifestations of

A

hyperparathyroidism

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35
Q

butterfly rash over cheeks and bridge of nose

A

systemic lupus erythematous = autoimmune CT disease, 80% are women, usually childbearing age. fever, malaise, lymphadenopathy, weight loss, raynauds phenomenon

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36
Q

stiffness of joints

A

Rheumatoid arthritis = chronic systemic inflammatory disorder that may affect many tissues/organs: skin, bloodvessels, heart, lungs muscles, but principally attacks the joints

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37
Q

skin rash

A

dermatomyositis = muscle disease that causes inflammation and a skin rash = inflammatory myopathy. cause unknown

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38
Q

systemic vasculitis

A

polyarteritis nodosa = BV disease unknown cause = necrotizing immune complex inflammation of small and medium sized arteries.

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39
Q

widespread CT fibrosis

A

Scleroderma = widespread CT disease = changes in skin, bv, muscles and internal organs. common in young women, tight-mask like facial skin, raynauds phenomenon, pain, stiffness, fingers and joints swell, gerd, increased chance of Barret’s esophagus

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40
Q

systemic vasculitis is associated with what virus

A

HBV (hepatitis B)

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41
Q

most common DEMYELINATING DISORDER

A

multiple sclerosis = disorder of the CNS (brain and spinal cord) caused by progressive damage to the outer covering of nerve cells (myelin) =decreased nerve function. cause is unknown but may be an autoimmune component to the disease with CD4 and CD8 T cell involvement

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42
Q

multiple sclerosis - inflammation destroys the covering of the nerve cells in the myelin sheath leaving behind multiple areas of scar tissue called ___ on the nerve cells. - results in slowing/blocking of nerve impulses = symptoms of MS.

A

sclerosis = scar tissue.

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43
Q

symptoms of MS

A

visual and speech disturbances, paresthesias (tingling, prickling, numbness), depression, mood swings

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44
Q

In MS, women/men are affected 2x more

A

women

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45
Q

the lesions of MS are caused by an immune response that is directed against the

A

myelin sheath

46
Q

MS is a white/grey matter disease

A

white matter (myelin)

47
Q

lesions of MS appear as multiple, well circumscribed glassy, gray tan, irregularly shaped __

A

plaques

48
Q

treatment for MS

A

injectable interferon - reduces frequency of relapses of MS

49
Q

in primary amyloidosis the patients have some form of:

a. macrophage dyscrasia (abnormal mixture)
b. erythrocyte dyscrasia
c. hemoglobin dyscrasia
d. plasma cell dyscrasia

A

plasma cell dyscrasia

50
Q

amyloidosis = generic term to describe various conditions of EXTRACELLULAR misfolded proteins (amyloids) cause tissue damage and compromise organ function. amyloids are made of ___ proteins

A

insoluble fibrous proteins = scleroprotein. = beta pleated sheats = insoluble and resistant to proteolysis

51
Q
  1. primary amyloidosis means it is related to

2. secondary means

A
  1. an immune disease

2. (reactive) complication of some other chronic inflammatory process

52
Q

abnormal production of Ig by malignant plasma cells (ex multiple myeloma) is called? builds up in spleen liver kidneys heart lungs, skin, TONGUE, thyroid, intestines ,BV

A

primary amyloidosis

53
Q

amyloidosis due to complication of another disease such as TB, RA, Familial Mediterranean fever. amyloid builds up in spleen liver kidney adrenals lymph nodes. BUT HEART IS RARELY INVOLVED

A

secondary amyloidosis

54
Q

Alzheimer’s, DM type 2 and Parkinson’s are associated with amyloid diseases bc they are all characterized by

A

deposits of amyloid

55
Q

in DM type 2 the amyloids deposited in islet cells are called

A

amylin

56
Q

Urticaria (hives) results from antigen-induced release of vasoactive mediators from mast cell granules thru sensitization with specific __ antibodies

A

IgE

57
Q

IgE independent urticarial may result from substances that in certain individuals directly incite the degranulation of mast cells such as ___

A

opiates, curare, and certain antibodies

58
Q

another IgE independent urticarial is exposure to chemicals such as __ that suppress prostaglandin synthesis from arachidonic acid

A

aspirin

59
Q

complement mediated urticarial is seen in hereditary angioneurotic edema caused by an inherited deficiency in __ inhibitor that results in uncontrolled activation of the early components of the system

A

C1

60
Q

__ is the first symptom of hives

quickly followed by ___

A

itching ==> wheals

61
Q

___ are smooth slightly elevated areas that are redder or paler than the surrounding skin and usually remain small

A

wheals

62
Q

Angioedema is closely related to urticarial and is characterized by ___ edema of both the dermis and subcutaneous fat. It may involve part or all of the hands, feet, eyelids, lips, or genitals and even the lining of the mouth throat and airways making breathing difficult

A

deeper
Angioedema (Quincke’s edema) is the rapid swelling (edema) of the dermis, subcutaneous tissue, mucosa and submucosal tissues. It is very similar to urticaria, but urticaria, commonly known as hives, occurs in the upper dermis

63
Q

DM both type 1 and 2 common condition is

A

hyperglycemia = from defect in insulin secretion, insulin action, or most commonly both.

64
Q

classical triad of symptoms for DM

A

polyuria, polyphagia, polydipsia

65
Q

leading cause of end stage renal disease, adult-onset blindness and non traumatic lower extremity amputations

A

DM

66
Q

Type __ diabetes is an AUTOIMMUNE DISORDER characterized by pancreatic _ cell destruction and absolute deficiency of insulin

A

1 = Beta

67
Q

type 2 DM = combination of peripheral resistance to insulin actions and an inadequate secretory response by the pancreatic beat cells called

A

relative insulin deficiency

68
Q

management of both types of DM can be done by monitory blood glucose (short term) and long term by

A

Hb1AC levels

69
Q

Typically normal blood sugar should be?

A

<6%

70
Q

RA = chronic systemic inflammatory disorder that may affect many tissues or organs-skin, BV, heart, lungs, and muscles but principally attacks

A

joints

71
Q

RA attacks joints producing a NONSUPPARATIVE PROLIFERATIVE and inflammatory ___ that often progresses to damage the articular cartilage and ankylosis (stiffness) of joints

A

synovitis

72
Q

Cause of RA is __ however genetic predisposition, environment, and autoimmunity have pivotal roles in the development, progression, and chronicity of the disease

A

unknown.

73
Q

women or men affected more in RA

A

women 3-5x more

74
Q

Still’s disease

A

RA in young pp

75
Q

__ has disposition to affect Metacarpophalangeal joint (MCP) and proximal interphalangeal joints (PIP)

A

RA

symptoms start in hands/feet followed by wrists, ankles, elbow, knees

76
Q

onset of RA is fast/slow

A

gradual

77
Q

RA pattern of joint involvement varies, but is usually symmetrical/asymmetrical and involves small/large bones

A

symmetrical, small

78
Q

radiographic hallmarks of RA =

A

joint effusions,

juxta-articular osteopenia w/erosions, narrowing of joint space with loss of articular cartilage

79
Q

proliferative inflammation of synovial membranes

A

RA

80
Q

osteophyte

A

bony spur = osteoarthritis

81
Q

RA, SLE, polyarteritis nodosa, dermatomyositis, scleroderma are all

A

collagen diseases

82
Q

___ is the main component of connective tissue, and is the most abundant protein in mammals

A

Collagen

83
Q
  1. Collagen is composed of a __ helix
  2. The triple-helical domain structure of collagens consists of three distinct __-chains and earns collagen the name “tropocollagen”
  3. Helix formation is aided by ___bonds between N- and C-terminal propeptides
A
  1. triple
  2. α
  3. disulfide
84
Q
  1. Each of collagen’s 3 chains contain a characteristic Left/Right handed amino acid sequence of polyproline, often termed as polyproline type II helix.
  2. The proper folding of each of these chains requires a glycine residue to be present in every third position in the polypeptide chain. For example, each α-chain is composed of multiple triplet sequences of of __ in which Y and Z can be any amino acid.
  3. Y is commonly found as ___ and Z is usually present as ___. The presence of hydroxyproline in the Y position is also thought to contribute to the stability of the helical form
A
  1. Left
  2. Gly-Y-Z
  3. proline - hydroxyproline
85
Q 
COLLAGEN TYPES
 Type I: 
Type II: . 
Type III: 
Type IV
A

Type I: BONE | Type II: carTWOlage. | Type III: ArTHREE (Artery). | Type IV: Under the floor

86
Q

Histamine does/does not play a major role in type 3 hypersensitivity rxns

A

does not

87
Q

nuclear antigens

A

systemic lupus erythematosus

88
Q

_hypersensitivity rxns are initiated by antigen-activated T lymphocytes (sensitized) including CD4 and CD8

A

Type IV

89
Q

the tuberculin (Montoux) rxn is what type of hypersensitivity rxn

A

type IV

90
Q

anti-Sm

A

antibodies specific to SLE

91
Q

Autoantibodies/antinuclear antibodies

A

lupus

92
Q

Anti-DNA antibodies

A

LUPUS

93
Q

patients with lupus produce __ antibodies

A

abnormal

94
Q

when only the SKIN is involved it is called ___ lupus

A

discoid

95
Q

when internal organs are involved, it is called __ lupus

A

SYSTEMIC LUPUS ERYTHEMATOUS

96
Q

Usual cause of death of lupus

A

renal failure

97
Q

Acricyanosis is aka? often associated with

A

Raynaud’s phenomenon

lupus

98
Q

butterfly rash in SLE usually worsens with

A

sunlight

99
Q

6 drugs can induce lupus as a side effect. name them

A

hydralazine (high bp medicine), quinidine and procainamide (for abnormal heart rhythm), phenytoin (for epilepsy), isoniazid (Nydrazid, Laniazid for TB), penicillamine (used for RA)

100
Q

autoimmune disorder caused by production of antibodies against acetylcholine receptor = results in inhibition of neuromuscular transmission and even paralysis

A

myasthenia gravis

101
Q

Eaton Lambert syndrome

A

autoimmune disorder causes weakness

102
Q

autoimmune disorder caused by inadequate RELEASE OF AcH

A

Eaton Lambert syndrome

103
Q

in phenylketonuria, __ cannot be synthesized in adequate amounts

A

TYROSINE

104
Q

T/F newborns with phenylketonuria don’t have any symptoms

A

true

105
Q
  1. Phenylketonuria (PKU) is an autosomal ___metabolic genetic disorder characterized by mutations in the gene for the hepatic enzyme ____, rendering it nonfunctional
  2. This enzyme is necessary to metabolize the amino acid ___ to the amino acid ____
A
  1. recessive, phenylalanine hydroxylase (PAH)

2. phenylalanine ==> tyrosine

106
Q
  1. pp with PKU will find ___ in the urine
A

phenylalanine and its degradation products (phenylketones aka phenylpyruvate)

107
Q

is phenylalanine an essential or non essential amino acid

A

essential

108
Q

____ is a defect in melanin production that results in partial or full absence of pigmentation

A

albinism

109
Q

Albinism is caused by the absence or defect of ___, a copper-containing enzyme involved in the production of melanin.

A

tyrosinase

110
Q

Deficiency of the intestinal and kidney transport protein of cysteine causes kidney stones. this disease is called

A

cystinuria

111
Q

___ is a rare inherited genetic disorder of phenylalanine and tyrosine metabolism. This is an autosomal recessive condition that is due to a defect in the enzyme homogentisate 1,2-dioxygenase

A

Alkaptonuria (black urine disease, black bone disease, or alcaptonuria)

112
Q

___ is an autosomal recessive metabolic disorder affecting branched-chain amino acids. It is one type of organic acidemia. The condition gets its name from the distinctive sweet odor of affected infants’ urine. caused by a deficiency of the branched-chain alpha-keto acid dehydrogenase complex (BCKDC), leading to a buildup of the branched-chain amino acids (leucine, isoleucine, and valine) and their toxic by-products (ketoacids) in the blood and urine

A

Maple syrup urine disease (MSUD), also called branched-chain ketoaciduria,

NBDE PART 1 MICRO/PATH (20 decks)

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