Exam 2 Diseases Flashcards
1
Q
What is pulmonary edema? where does it start then proceeds to
A
Pulmonary edema: fluid accumulation in lungs
starts in interstitial tissues then proceeds to fill up distal air spaces
2
Q
Causes of pulmonary edema
A
INC intravascular pressure (CHF)
hypoproteinemia
vascular damage from infections, autoimmune disease
3
Q
what are the problems with pulmonary edema
A
inhibits normal O2 exchange
predisposes pt to infection
4
Q
what is the main cause of a pulmonary thromboemboli?
Describe the problems that arise from a small, large, and very large emboli
A
main cause: deep veins of legs or pelvic veins
small emboli: may cause minimal damage
large emboli: causes hemorrhage or infarction
very large emboli lodge at the bifurcation of pulmonary arteries can cause sudden death
5
Q
what are the predisposing factors to Thrombo emboli
A
chronic illness, prolonged bed rest (immobility), hypercoagulable state (factor V leidin) and deep vein thromboses (DVTS)
6
Q
what are obstructive pulmonary diseases
A
group of diseases that results in airflow limitation or obstruction
7
Q
4 disorders of obstructive pulmonary diseases
A
emphysema, chronic bronchitis, bronchiectasis, and asthma
8
Q
what is COPD
A
emphysema and chronic bronchitis combined
9
Q
what does emphysema result in
A
alveolar wall destruction and overinflation, alveolar parenchymal destruction,
10
Q
what does chronic bronchitis result in
A
productive cough and airway inflammation
11
Q
what does asthma result in?
is asthma a reversible or irreversible obstruction
A
asthma= bronchial hyperresponsiveness triggered by allergens, infections, etc Asthma= reversible obstruction
12
Q
what is emphysema?
major cause?
genetic causes?
A
emphysema is a permanent enlargement of the distal small air spaces due to destruction of alveolar septal walls
major cause- smoking
genetic causes: TGF B1 polymorphisms and a1 antitrypsin deficiency
13
Q
clinical aspects of emphysema
path:
A
Emphysema: dyspnea, cough, prolonged exhalation= pink puffers
path: imbalance between protease and ant protease enzymes
14
Q
What is centriacinar emphysema
A
centriacinar: involves the central portion of the acini usually affects upper lobes most often related to smoking
15
Q
what is panacean emphysema:?
A
paracinar: involves entire acini unit from respiratory bronchioles to terminal alveoli usually affects lower lobes and seen in pts with a1 AT deficiency
16
Q
What says a pt has chronic bronchitis
A
pt has cough + sputum production in 3 consecutive months in 2 consecutive years
17
Q
what may pts w chronic bronchitis have
A
may have hypoxemia and cyanosis aka BLUE Bloaters
18
Q
Chronic bronchitis
pathogenesis:
pathology:
A
pathogenesis: chronic irritation (smoking) and infections
path: INC mucus gland layer, chronic inflammation, fibrosis, and narrowing of airways. Edema, seromucous gland hypertrophy and excessive secretions
19
Q
Chronic bronchitis and emphysema predisposing factor smoking: what does smoking cause in lungs?
A
smoking! causes mucus gland hypertrophy, INC smooth m tone, inhibits cilia, inhibits phagocytosis, induces squamous metaplasia
20
Q
Chronic bronchitis and emphysema predisposing factors
A
smoking, atmosphere pollutants, infections, genetic factors= CF and a1 AT deficiency
21
Q
what is bronchiectasis
A
bronchiectasis is chronic infection w permanent major airway dilation secondary to obstruction infection or both
22
Q
clinical aspects of bronchiectasis:
complications of bronchiectasis
A
clinical: severe cough, bloody mucoid expectoration, dyspnea
complications: abscess, pneumonia, bronchopleural fistula and empyema
23
Q
predisposing factors to bronchiectasis
A
obstructive tumors, foreign bodies, cystic fibrosis (mucus plugs), other COPD, CF: supportive or necrotizing pneumonia
24
Q
pathology of bronchiectasis
A
dilated distal bronchi and bronchioles, chronic infection w inflammation and variable purulence
25
what is asthma?
| what does asthma lead to
asthma: INC irritability and prominence of smooth m in bronchi and bronchioles
asthma leads to marked reversible episodes of contraction and airway constriction
26
what are the initiating factors of asthma
| what percentage of adults? children? and where is it
allergies, infections, exercise, drugs, emotions
27
Clinical symptoms of asthma
asthma: wheezing, long exhalation, hyperinflation of lungs
28
Two types of asthma and describe each type
Atopic asthma: allergic, extrinsic, type 1 hypersensitivity (IgE), environmental antigen and family history is common
Non atopic= intrinsic, may be initiated by viruses, air pollutants
29
What can atopic and non atopic asthma both be triggered by
emotional stress, exercise, cold temps
30
Asthma pathology
Asthma - INC mucus glands, smooth m hypertrophy w inflammation w eosinophils and type 2 helper T cells
31
Pathogenesis of Asthma:
Antigen binds to surface of IgE on mast cells releasing a large number of mediators including histamine and leukotrienes
32
Treatment of asthma
attack may subside spontaneously if not: inhalation bronchodilators for immediate relief (albuterol) and controller medications (corticosteroids)
33
What is a leading cause of death and often complicates other chronic debilitating diseases? w
what can it be caused by
Pneuomia: can be caused by any organism: bacterial, viral, fungal, parasites
34
Predisposing factors to bacterial pneumonia
loss of cough reflex, injury to cilia, DEC phagocytosis, pulmonary edema, immunocompromised condition
35
Clinical aspects of Bacterial pneumonia
Clinically bacterial pneumonia leads to cough dyspnea, fever, chills, and sputum production
36
what is bronchopneumonia? what organism?
| occurs in who?
Bacterial!
| bronchopneumonia: patchy process, begins around the small bronchi common in very young and old
37
what is lobar pneumonia? what organism
| occurs in who?
bacterial! occurs in healthy adults
| lobar pneumonia: involves an entire lobe= 90% Streptococcus pneumonia
38
patchy areas of consolidation is what
bronchopneumonia
39
describe the stages of inflammation in lobar pneumonia
1. congestion.
2. red hepatization
3. gray hepatization
4. resolution
40
Complications of pneumonia
emphyema, abscess, pericarditis, bacteremia
41
What is seen with mycoplasma pneumonia and viruses
Atypical Interstitial Pneumonia
42
What is clinically seen with atypical interstitial pneumonia
highly variable, mild fever to headache, dry cough, myalgia to life threatening
43
Pathology of Atypical (interstitial) Pneumonia
interstitial inflammation, mononuclear cells, congestion, hyaline membranes (diffuse alveolar damage)
44
What is Acute Respiratory Distress?
| what causes ARDS
ARDS is a rapidly developing serious condition with some histologic features as interstitial pneumonia
ARDS causes by shock, infections, trauma, Drug OD, irritants, etc
45
What does ARDS cause
ARDS= injury to endothelium and alveolar epithelium and INC endothelial permeability = leaky!
46
what is the mortality rate of ARDS
30%!!
47
What are the clinical symptoms of a pulmonary abscess?
| whats the course of a PA?
Pulmonary abscess clinically: cough, fever, purulent sputum
| course: scar. cavitation, progressive enlargement
48
what are the predisposing factors to a pulmonary abscess
PA disposing factors = bronchiectasis, aspiration, septic emboli, airway obstruction, dental sepsis
49
What is caused by Mycobacterium Tuberculosis? How much of the world is infected by this?
M Tuberculosis = TUBERCULOSIS
| infects 1/3 of the world!
50
what is the most common cause of death from an infectious disease? how many deaths per year?
Tuberculosis= 3 million deaths per year
51
What has been the trend of TB in the US?
| What causes an INC in TB infection?
US: mid 80s TB cases slowly rose but have been declining since late 90s
HIV, overcrowding, poor living conditions and immigrants INC risk of TB infection
52
Describe the bio of Mycobacterium Tuberculosis.
| whats the classic result of TB
M TB= bacillus rod, aerobe, non motile, slow growing w a waxy coat that resists acid destaining = acid fast bacillus = AFB
result of TB= caveating granulomatous inflammation
53
TB pathogenesis: what is at the site of an early TB infection, how is it acquired
acquired by inhalation
Ghon lesion= site of early infection
Ghon complex - lung lesion + hilar lymph nodes
54
what is Cavitary TB
Cavitary TB is TB at the apex of the lungs that results in significant scarring, may seed the large airways, lymph nodes, or blood and lead to an effusion (direct extension to the pleura)
55
What does reactivation of TB lead to?
| What is TB called that widely disseminates and what might happen with this TB
Reactivation of TB induces Type 4 hypersensitivity and tissue necrosis
widely disseminated TB = miliary TB can lead to multi organ involvement
56
What does Miliary TB result from? what is the name for what causes Miliary TB?
Miliary TB results from spread via lymphatics or blood
| caused by Millet seeds
57
What can cause Granulomatous lung disease
TB, fungal infections like histoplasmosis, and sarcoidosis
58
What is the leading cause of cancer deaths? what are the risk factors for this cancer
LUNG cancer= leading cancer killer!
| risk factors= smoking, asbestos, radon, nickel, chromates, pollutants, lung scar
59
Clinical symptoms of Lung Cancer? What may lung cancer tumors do?
Clinical of lung cancer: cough, weight loss, chest pain, hemoptysis
Lung cancers may produce hormones like ADH, ACTH, PTH, etc
60
what is the 5 year survival for lung cancer?
| if localized?
all types = 16%
| if localized = 45%
61
What are Pneumoconioses?
| what size particle inhalation is the most dangerous and why
Pneumoconioses are a group of lung disorders caused by inhalation of dusts/ particles
particle size, shape, concentration all important
particles 1-5 micrometers most dangerous bc they lead to fibrosis scarring
62
what is the most prevalent form of occupational disease?
Silicosis
63
Autosomal dominant disorders: expression can be identified in all affected persons but to differing extents, what is this called
variable expressivity
64
What is a AD disease that is relatively common and has highly variable expressivity but shows a nearly 100% penetrance (everyone shows it who has it)
Neurofibromatosis
65
Age of onset is often early in life with these disorders, complete penetrance is common and many of the disorders conditions are enzyme defects leading to metabolic dysfunction
Autosomal Recessive Disorders: heterzygous pts typically have reduced but adequate levels of normal functional enzyme
66
what sex chromosomes carries all sex linked disorders?
| what does the other chromosome have?
X chromosome = all sex linked disorders!
| Y chromosome has SRY gene and 1 trait for hairy ears. Y = no diseases
67
What does unfavorable lyonization do to a female
results in X linked disease expression in a heterozygous female
68
what is a AD disorder that is a mutation of the FBN1 gene resulting in abnormal fibrillin?
what is its prevalence
Marfan syndrome= abnormal elastic production due to FBN1 mutation
occurs 1/5000 people= Abe Lincoln
69
What does Marfan syndrome result in?
tall thin body, abnormally long legs, arachnodactyly, dislocation of eyes, aortic aneurysm and dissection leading to heart failure and aortic rupture
70
what is a disorder w dominant and recessive forms that results in problem with collagen synthesis
Ehlers Danlos Syndrome
71
What are two diseases caused by mutations in genes encoding receptor proteins or channels? name the protein or channel for both
Familial Hypercholesterolemia: LDL receptor
| Cystic Fibrosis: chloride channel protein
72
what is a common AD inherited disorder that results from mutation of the gene that encodes the LDL receptor?
what is its frequency?
what does it result in?
Familial Hypercholesterolemia
1 in 500 people
results in impaired metabolism and INC LDL cholesterol in plasma
73
What an Familial Hypercholesterolemia cause (2)?
Describe the difference in LDL levels between heterozygotes and homozygote
what drug is used for TX
multiple xanthomas of the skin and premature atherosclerosis
hetero= 2-3x INC LDL
homo- 5X INC LDL, often die by MI by age of 20
lomitapid
74
what are 3 diseases caused by mutations in enzyme proteins
Phenylketonuria, Galactosemia, and Lysosomal storage disease
75
What is an AR disorder that leads to hyperphenylalaninemia and PKU eventually causing mental retardation by 6 months of age?
what do affected pts lack?
prevalence
Phenylketonuria
1:10,000 caucasians
Lack of phenylalanine hydroxylase
76
what do storage diseases lead to?
| What is a frequent involvement of storage diseases that leads to what?
Storage diseases lead to accumulation of large, insoluble molecules (sphingolipids, mucopolysaccharides) in macrophages which leads to hepatosplenomegaly
frequent involvement w CNS leading to neuronal damage, retardation and early death
77
name the 4 lysosomal storage diseases
Tay sachs
Niemman Pick
Gaucher
Mucopolysaccharidoses
78
Mucopolysaccharide storage disease is inherited how>
due to what?
affected Pts have what
AR! Mucopolysaccharide storage disease
Due to lack of enzyme to degrade mucopolysaccharides
affected pts have coarse facial features, clouding of cornea, joint stiffness and mental retardation
79
what is AR, caused by deficiency of Alpha L iduronidase w a life expectancy of 6-10 years?
how is it treated
HURLER DISEASE = deficient alpha L iduronidase
| tx: bone marrow transplant or enzyme for 300 k per year
80
what is an X linked disorder with a deficiency of L iduronate sulfatase
Hunter syndrome- no corneal clouding and a milder clinical course otherwise similar to Hurler syndrome
81
What can down syndrome cause
Mental retardation, epicanthic folds, flat facial profile, cardiac malformations, INC susceptibility to infection. LARGE TONGUE, INC risk for acute leukemia
82
what is Klinefelter syndrome:
male hypogonadism when there are at least 2 X chromosomes and 1 or more & chromosome - most are 47 XXY
83
what is turner syndrome
partial or complete absence of one of the X chromosomes = 45X
84
what does Klinefelter syndrome result in
Phenotypically = male
INC length of lower limb, reduced body hair, gynecomastia, hypogonadism, usually male sterility
Dentally: INC freq of TAURODONTISM
85
Turner syndrome results in what?
short stature, webbing of neck, low posterior hairline, shield like chest, high arched palate, congenital cardio malformations, failure to develop secondary sex characteristics, primary amenorrhea
86
What is a distinctive cell of Hodgkin lymphoma?
| What is hodgkins tx with
Hodgkins= reed sternberg cell
| tx with chemo and radiotherapy is highly curable
87
what is specific to Hodgkin lymphoma about its distribution amongst the population
Hodgkin Lymphoma is bimodal age distribution 20-30 y/os and greater than 50 y/os
88
these two things characterize Hodgkins lymphoma
painless lymphadenopathy and splenomegaly
89
describe the reed sternberg cell
| what percentage of lymph node cells are RS cells? whats the origin of RS cells? what is often present in RS cell?
reed sternberg cell is a large cell with mirror image nuclei and prominent nuclei, compromises about 2% of cells in involved lymph node, Germinal center B lymphocyte origin and Epstein Barr Virus is present is 70% of RS cells
90
describe low stage Hodgkins and tx
| describe high stage hodgkins and tx
low stage: localized= chemo and radiation
| high stage: wide spread disease w distant or bone marrow involvement= chemo
91
with hodgkins what are B signs/ symptoms? what are they associated with
B signs/ symptoms= fever, night sweats, unexplained weight loss
B signs= worse prognosis, most often with stage 3 and 4 disease
92
what is tx of Hodgkins associated with?
Hodgkins tx puts pt at low risk of development of secondary tx related acute leukemia
93
what is NON hodgkin lymphoma, percent that are of B cell origin? when does incidence INC?
Non hodgkin lymphoma is neoplastic lymphocytes originating in lymph nodes or extranodal lymphoid tissue. 85% are of B Cell origin (rest T cell), Incidence rises steadily after age 40
94
Clinical aspects of NON hodgkin lymphoma
painless lymph node enlargement, systemic symptoms in 30% of pts, immune abnormalities, splenomegaly, may involve GI tract, bones, CNS
95
Non Hodgkins lymphoma what is better: nodular or diffuse, small or large
Nodular better than diffuse
| Small cell better than large
96
with Non Hodgkins lymphoma what is an exception to the normal prognosis rule
with NHL you use grading (sub typing) instead of staging which is normally used in prognosis
97
describe the differences between acute and chronic leukemias
acute: rapid onset, survival may be months, mostly circulating blasts, INC WBC and INC blasts in bone marrow
chronic: slow indolent onset, survival years/ decades, mostly mature forms INC WBC, and normal blasts in bone marrow
98
what is the most frequent cancer in those 15 and younger with INC WBC and thrombocytopenia. What may these pts have?
what pts have good prognosis? what pts have bad prognosis?
Acute Lymphoblastic Leukemia, pts may have lymphadenopathy and splenomegaly
children have good prognosis
adults have bad prognosis
99
what leukemia is more common in adults with INC WBC count often accompanied by anemia and thrombocytopenia? what is it a clonal proliferation of
Acute Myelogenous Leukemia= clonal proliferation of primitive myeloid cell
100
what is present in the cytoplasm of acute myelogenus leukemia?
tx of it? what percent of patients have remission but many relapse
Acute Myelogenous Leukemia= myeloperoxidase present in the cytoplasm . tx w chemo, 70% of pts have remission but many relapse
101
what is the most common adult leukemia that expresses surface IgM with kappa light chain restriction? what doe this leukemia expression? who does this favor?
Chronic lymphocytic leukemia- clonal proliferation of Mature b lymphocytes identical to small lymphocytic lymphoma.
High expression of BCL2
favors males. adults over 60
102
what does chronic lymphocytic leukemia present with? what develops as it progresses. what does its tumor cells do and cause?
chronic lymphocytic leukemia presents with splenomegaly and lymphadenopathy,
leads to anemia and thrombocytopenia
Its tumor cells suppress normal B cells leading to HYPOgammaglobulinemia
103
What is a stem cell disorder of clonal proliferation of immature granulocytes?
Leads to INC In what?
Presents with what?
Chronic myelogenous leukemia- profil of granulocytes may have thrombocytosis and anemia and splenomegaly
104
chronic myelogenous leukemia is a cytogentic abnormality of what?
mutation of what genes?
chemo targets what?
Chronic myelogenous leukemia= philadelphia chromosome
fusion of BCR and ABL genes
chemo targets blockage of bcr-abl tyrosine kinase
105
what is a clonal proliferation of monoclonal plasma cells leading to monoclonal heavy and light chain production with IgG being most common
Multiple Myeloma- disease of late middle age to elderly
106
what are the lab/ clinical findings of multiple myeloma
```
lytic bones lesions, hypercalcemia
bone marrow infiltration by plasma cells
INC serum monoclonal protein
Bence Jones proteinuria
Renal failure
infections
```
107
Blood and marrow findings of multiple myeloma
circulating plasma cells in blood are uncommon.
RBC show stacking (ROULEAUX formation)
marrow plasma cell infiltrates in single cells and sheets
