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
2
Q
Cushing’s syndrome:
Hyper or hypo:
glycemia, kalemia, natremia, tension, blood volume
A
hyperglycemia, hypertension, hypernatremia, hypokalemia, increased blood volume
3
Q
oral signs of Addison’s disease
A
tan skin, diffuse pigmentation on gingiva, tongue, hard palate, and buccal mucosa.
4
Q
will pigmentation of the mouth/skin disappear with therapy in Addison’s disease?
A
skin (cutaneous) will but oral pigmentation wont
5
Q
- primary adrenocortical deficiency is?
- secondary adrenocortical deficiency is?
- tertiary adrenocortical deficiency is?
A
- problem at gland = adrenal gland, usually autoimmune (lymphocytic adrenalitis), can be TB (2nd most common) or metastatic tumor or infections.
6
Q
The adrenocorticotropic hormone test (ACTH test/corticotropin test) measures ____ gland function
A
pituitary
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)
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)
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
10
Q
Addison’s disease is treated by administering
A
cortisol (hydrocortisone)
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.
12
Q
Waterhouse-Friderichsen syndrome is due to what bacteria
A
meningococcemia = associated with meningococcal meningitis
13
Q
the adrenal cortex produces what 3 hormones
A
sex hormones, glucorticoid hormones, and mineralcorticoid hormones
14
Q
- Osteoarthritis (degenerative joint disease) is the most common joint disease. it is characterized by the progressive ___ of __
A
erosion of articular cartilage
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
16
Q
inflammation of osteoarthritis is accompanied by
A
pain swelling and stiffness
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
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
19
Q
- primary/secondary osteoarthritis occurs without known cause and is mostly related to aging
A
primary
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
21
Q
Histiocytosis X (Langerhans cell histiocytosis) = group of diseases.
- Pulmonary Langerhans cell histiocytosis is most often seen in __ and may regress upon
A
- adult smokers, quitting
22
Q
Histiocytosis X (Langerhans cell histiocytosis) = group of diseases.
- Hand Christian Schuller Disease = seen in ___ with the classic triad of?
A
- kids, “BED”
Bone defects
diabetes insipidus
exopthalamos
23
Q
- Diabetes insipidus is an uncommon condition in which the kidneys are unable to prevent the ___
- DI caused by a lack of ADH is called ___diabetes insipidus.
- When DI is caused by a failure of the kidneys to respond to ADH, the condition is called ___diabetes insipidus
A
- excretion of water
- central
- nephrogenic
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
25
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Histiocytosis X (Langerhans cell histiocytosis) = group of diseases.
1. Eosinophilic Granuloma
```
proliferation of Langerhans cells mixed with eosinophils, lymphocytes, plasma cells, neutrophils. most common and most benign form of Histiocytosis X (Langerhans cell histiocytosis)
26
the outcome of Histiocytosis X (Langerhans cell histiocytosis) depends on what 2 things
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
27
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
Bruton disease
28
primary hyperparathyroidism is most often caused by
parthyroid adenoma
29
symptoms of primary hyperparathyroidism
painful bones, renal stones, abdominal groans, psychic moans
30
which condition causes a loss of lamina dura around multiple teeth
hyperparathyroidism
31
in hyperPTism affects on calcium, phosphorous, PTH?
increased Ca, PTH
| decreased phosphorous
32
1. primary hyperPTism is result of ___, __ or __
| 2. secondary is caused by condition that gives rise to
1. adenoma (85-95%), primary hyperplasia (5%), carcinoma (1%)
2. chronic HYPOcalcemia, which in turn leads to compensatory overactivity of the PTH gland.
33
in rare instance HYPOparathyroidism is associated with congenital thymic hypoplasia called
DiGeorge's syndrome
34
Osteoporosis, central giant cell granulomas, and metastatic calcifications are all manifestations of
hyperparathyroidism
35
butterfly rash over cheeks and bridge of nose
systemic lupus erythematous = autoimmune CT disease, 80% are women, usually childbearing age. fever, malaise, lymphadenopathy, weight loss, raynauds phenomenon
36
stiffness of joints
Rheumatoid arthritis = chronic systemic inflammatory disorder that may affect many tissues/organs: skin, bloodvessels, heart, lungs muscles, but principally attacks the joints
37
skin rash
dermatomyositis = muscle disease that causes inflammation and a skin rash = inflammatory myopathy. cause unknown
38
systemic vasculitis
polyarteritis nodosa = BV disease unknown cause = necrotizing immune complex inflammation of small and medium sized arteries.
39
widespread CT fibrosis
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
40
systemic vasculitis is associated with what virus
HBV (hepatitis B)
41
most common DEMYELINATING DISORDER
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
42
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.
sclerosis = scar tissue.
43
symptoms of MS
visual and speech disturbances, paresthesias (tingling, prickling, numbness), depression, mood swings
44
In MS, women/men are affected 2x more
women
45
the lesions of MS are caused by an immune response that is directed against the
myelin sheath
46
MS is a white/grey matter disease
white matter (myelin)
47
lesions of MS appear as multiple, well circumscribed glassy, gray tan, irregularly shaped __
plaques
48
treatment for MS
injectable interferon - reduces frequency of relapses of MS
49
in primary amyloidosis the patients have some form of:
a. macrophage dyscrasia (abnormal mixture)
b. erythrocyte dyscrasia
c. hemoglobin dyscrasia
d. plasma cell dyscrasia
plasma cell dyscrasia
50
amyloidosis = generic term to describe various conditions of EXTRACELLULAR misfolded proteins (amyloids) cause tissue damage and compromise organ function. amyloids are made of ___ proteins
insoluble fibrous proteins = scleroprotein. = beta pleated sheats = insoluble and resistant to proteolysis
51
1. primary amyloidosis means it is related to
| 2. secondary means
1. an immune disease
| 2. (reactive) complication of some other chronic inflammatory process
52
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
primary amyloidosis
53
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
secondary amyloidosis
54
Alzheimer's, DM type 2 and Parkinson's are associated with amyloid diseases bc they are all characterized by
deposits of amyloid
55
in DM type 2 the amyloids deposited in islet cells are called
amylin
56
Urticaria (hives) results from antigen-induced release of vasoactive mediators from mast cell granules thru sensitization with specific __ antibodies
IgE
57
IgE independent urticarial may result from substances that in certain individuals directly incite the degranulation of mast cells such as ___
opiates, curare, and certain antibodies
58
another IgE independent urticarial is exposure to chemicals such as __ that suppress prostaglandin synthesis from arachidonic acid
aspirin
59
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
C1
60
__ is the first symptom of hives
| quickly followed by ___
itching ==> wheals
61
___ are smooth slightly elevated areas that are redder or paler than the surrounding skin and usually remain small
wheals
62
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
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
DM both type 1 and 2 common condition is
hyperglycemia = from defect in insulin secretion, insulin action, or most commonly both.
64
classical triad of symptoms for DM
polyuria, polyphagia, polydipsia
65
leading cause of end stage renal disease, adult-onset blindness and non traumatic lower extremity amputations
DM
66
Type __ diabetes is an AUTOIMMUNE DISORDER characterized by pancreatic _ cell destruction and absolute deficiency of insulin
1 = Beta
67
type 2 DM = combination of peripheral resistance to insulin actions and an inadequate secretory response by the pancreatic beat cells called
relative insulin deficiency
68
management of both types of DM can be done by monitory blood glucose (short term) and long term by
Hb1AC levels
69
Typically normal blood sugar should be?
<6%
70
RA = chronic systemic inflammatory disorder that may affect many tissues or organs-skin, BV, heart, lungs, and muscles but principally attacks
joints
71
RA attacks joints producing a NONSUPPARATIVE PROLIFERATIVE and inflammatory ___ that often progresses to damage the articular cartilage and ankylosis (stiffness) of joints
synovitis
72
Cause of RA is __ however genetic predisposition, environment, and autoimmunity have pivotal roles in the development, progression, and chronicity of the disease
unknown.
73
women or men affected more in RA
women 3-5x more
74
Still's disease
RA in young pp
75
__ has disposition to affect Metacarpophalangeal joint (MCP) and proximal interphalangeal joints (PIP)
RA
| symptoms start in hands/feet followed by wrists, ankles, elbow, knees
76
onset of RA is fast/slow
gradual
77
RA pattern of joint involvement varies, but is usually symmetrical/asymmetrical and involves small/large bones
symmetrical, small
78
radiographic hallmarks of RA =
joint effusions,
| juxta-articular osteopenia w/erosions, narrowing of joint space with loss of articular cartilage
79
proliferative inflammation of synovial membranes
RA
80
osteophyte
bony spur = osteoarthritis
81
RA, SLE, polyarteritis nodosa, dermatomyositis, scleroderma are all
collagen diseases
82
___ is the main component of connective tissue, and is the most abundant protein in mammals
Collagen
83
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
1. triple
2. α
3. disulfide
84
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
1. Left
2. Gly-Y-Z
3. proline - hydroxyproline
85
```
COLLAGEN TYPES
Type I:
Type II: .
Type III:
Type IV
```
Type I: BONE | Type II: carTWOlage. | Type III: ArTHREE (Artery). | Type IV: Under the floor
86
Histamine does/does not play a major role in type 3 hypersensitivity rxns
does not
87
nuclear antigens
systemic lupus erythematosus
88
_hypersensitivity rxns are initiated by antigen-activated T lymphocytes (sensitized) including CD4 and CD8
Type IV
89
the tuberculin (Montoux) rxn is what type of hypersensitivity rxn
type IV
90
anti-Sm
antibodies specific to SLE
91
Autoantibodies/antinuclear antibodies
lupus
92
Anti-DNA antibodies
LUPUS
93
patients with lupus produce __ antibodies
abnormal
94
when only the SKIN is involved it is called ___ lupus
discoid
95
when internal organs are involved, it is called __ lupus
SYSTEMIC LUPUS ERYTHEMATOUS
96
Usual cause of death of lupus
renal failure
97
Acricyanosis is aka? often associated with
Raynaud's phenomenon
| lupus
98
butterfly rash in SLE usually worsens with
sunlight
99
6 drugs can induce lupus as a side effect. name them
hydralazine (high bp medicine), quinidine and procainamide (for abnormal heart rhythm), phenytoin (for epilepsy), isoniazid (Nydrazid, Laniazid for TB), penicillamine (used for RA)
100
autoimmune disorder caused by production of antibodies against acetylcholine receptor = results in inhibition of neuromuscular transmission and even paralysis
myasthenia gravis
101
Eaton Lambert syndrome
autoimmune disorder causes weakness
102
autoimmune disorder caused by inadequate RELEASE OF AcH
Eaton Lambert syndrome
103
in phenylketonuria, __ cannot be synthesized in adequate amounts
TYROSINE
104
T/F newborns with phenylketonuria don't have any symptoms
true
105
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 ____
1. recessive, phenylalanine hydroxylase (PAH)
| 2. phenylalanine ==> tyrosine
106
1. pp with PKU will find ___ in the urine
phenylalanine and its degradation products (phenylketones aka phenylpyruvate)
107
is phenylalanine an essential or non essential amino acid
essential
108
____ is a defect in melanin production that results in partial or full absence of pigmentation
albinism
109
Albinism is caused by the absence or defect of ___, a copper-containing enzyme involved in the production of melanin.
tyrosinase
110
Deficiency of the intestinal and kidney transport protein of cysteine causes kidney stones. this disease is called
cystinuria
111
___ 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
Alkaptonuria (black urine disease, black bone disease, or alcaptonuria)
112
___ 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
Maple syrup urine disease (MSUD), also called branched-chain ketoaciduria,
