polycythemia
an increased number of circulating red blood cells
secondary - body's response (to lung disease or high elevation)
relative - caused by dehydration
vera - neither of the above
megaloblastic anemia
anemia characterized by many large immature and dysfunctional red blood cells (megaloblasts) in the bone marrow (B-12 deficiency)
dermatomyositis
an idiopathic inflammatory myopathy with focal or extensive degeneration of muscle fibers related to inflammatory lymphocyte and macrophage infiltrates.
4 types of hypersensitivity
I - allergy/immediate (ex: asthma, anaphylaxis)
II - cytotoxic, antibody-dependent (ex: myasthenia gravis, grave's)
III - immune complex (ex: arthritis, lupus)
IV - delayed, antibody-independent (ex: contact dermatitis, MS, Mantoux test)
Which leukemia is the most common malignancy in children?
Acute lymphocytic (lymphoid) leukemia (ALL); Acute myelogenous (myeloid) leukemia (AML) represents approximately 20% of childhood leukemias; chronic forms are seen mostly in adults
Wiskott-Aldrich syndrome
X-linked recessive immune disorder which affects cell signaling and cytoskeletal organization, characterized by recurrent infections, severe thrombocytopenia, and eczema
DiGeorge syndrome
Congenital defect in thymus development---> absence of T-Cells (Bcells and Ig are unaffected)
Can be parathyroid hypoplasia--> low Ca+
cardiac abnormality (tetralogy of ballot), abnormal facies, thymic aplasia, cleft palate, Hypocalcemia and 22q11.2 delet
Bruton X-linked agammaglobulinemia
Failure of B-cell precursors to develop into mature B cells
Caused by mutations in Bruton tyrosine kinase (Btk) on Xq21.22
Underdeveloped germinal centers
NO plamsa cells
melena
abnormally dark tarry feces containing blood (usually from gastrointestinal bleeding)
How does nicotine affect the cardiovascular system?
adversely affects serum lipid levels by increasing LDL;
increases platelet aggregation;
decreases myocardial oxygen supply;
increases the release of endogenous catecholamines that increase myocardial oxygen demand
pulsus paradoxus
Beats have weaker amplitude with inspiration, stronger with expiration. Best determined during B/P measurement; reading decreases (>10 mm Hg) during inspiration and increases with expiration. Common finding in cardiac tamponade and with severe bronchospas
cardiac tamponade
excessive external pressure on the heart resulting from excessive pericardial effusion. This impedes filling of the chambers, leading to systemic venous congestion because the vena cavae cannot empty into other atria. This causes pulsus paradoxus and neck
infective endocarditis
a myocardial infection characterized by heavy bacterial colonization (vegetative growths) of cardiac VALVES
secondary hypertension
increased blood pressure that is caused by another known condition. Most commonly, the hypertension is caused by renal disease.
Which manifestations are typically associated with left-sided congestive heart failure (CHF)?
dyspnea, oliguria, and crackles
adult respiratory distress syndrome (ARDS)
rapid shallow breathing, respiratory alkalosis, refractory hypoxemia, and diffuse alveolar infiltrates
V/Q ratio
V = ventilation, Q = perfusion : ratio that relates volume of air to liters of blood passing through N = 0.8 - 1.0
symptoms indicative of cystic fibrosis
recurrent pulmonary infections, cough with thick tenacious sputum, and steatorrhea
Which type of lung cancer is associated with early metastasis and has the worst prognosis?
small-cell (oat cell) carcinoma
Adenocarcinoma is the most common type and has the largest doubling time (180 days), and thus the slowest metastasis
Which acid-base imbalance would temporarily be relieved by breathing into a paper bag held over the mouth?
Respiratory alkalosis is caused by hyperventilation; breathing into a paper bag is a simple remedy because re-breathing one's own carbon dioxide replenishes carbonic acid
struvite stones
magnesium phosphate stones that form in alkaline urine, commonly found in patients with persistent UTIs
uric acid stones
these occur due to high levels of uric acid/gout. Can occur due to a high protein diet or high alcohol intake. Grow in pH of 5.1-5.9
calcium stones
Most common kidney stones (75-80%). Conditions that cause hypercalcemia (cancer, elevated PTH, high VitD, milk-alkali syndrome, prolonged bed rest) can lead to hypercalciuria and stones.
what bacteria most often cause meningitis?
Neisseria meningitidis, Streptococcus pneumoniae, or, in children Haemophilus influenzae
describe the characteristics of pain impulses transmitted by fine, myelinated A fibers
fast traveling and sharp, localized pain
describe the characteristics of pain impulses transmitted by unmyelinated C fibers
slow transmission and dull (burning or aching) pain
myxedema
advanced hypothyroidism in adults characterized by sluggishness, slow pulse, puffiness in the hands and face, and dry skin
tropical sprue
damage to small bowel villi due to unknown organism resulting in malabsorption - similar to celiac disease except occurs in tropical regions (carribean), arises after infectious diarrhea and responds to antibiotics (secondary vit b12 or folate deficiency
dumping syndrome
When a concentrated solution of high osmolarity is taken in large amounts, water moves rapidly to the stomach and intestines from fluid surrounding the organs and vascular compartement. The pt will have a feeling of NAUSEA, FULLNESS, DIARRHEA; this can ca
list types of diarrhea and their causes
secretory: enterotoxins
osmotic: maldigestion (e.g. lactose intolerance, celiac disease)
exudative: inflammatory bowel diseases or severe infections
motility-related
inflammatory: caused by damage to mucosal lining / brush border
How is Crohn's disease distinct from ulcerative colitis?
the patient with ulcerative colitis develops abscesses, ulcerations, and granulation tissue, whereas the patient with Crohn disease develops the characteristic deep, linear ulcers that become fissures and then fistulas
Obstructive jaundice would result from gallstone blockage of which structure?
Jaundice occurs when bilirubin levels in the blood become abnormally high. When this is due to an obstruction, it must be within the bile flow system, most commonly the bile duct. No other location for a gallstone will lead to this effect.
describe different types of aphasia
1) Anomic aphasia is a fluent aphasia with intact grammar structure and word-finding deficit.
2) Broca aphasia, also known as verbal motor or EXPRESSIVE aphasia, is a deficit of language output or speech production with intact ability to write and to unde
describe portal hypertension
a result of increased pressure within the hepatic-portal circulation characterized by, among other signs, jaundice, esophageal varices, and periumbilical varices (caput medusae)
atrophy
decreased cell size
(causes: disuse, denervation, ischemia, starvation, interruption of endocrine signals, persistent injury, aging)
hypertrophy
increased cell size (and augmented functional capacity)
response to increased demand
hyperplasia
increased cell number (if cells capable of mitotic division)
(causes: increased demand, hormonal stimulation, persistent injury)
metaplasia
conversion of one cell type to another (fully reversible)
ex: cig smoke irritates mucosa, causing change in cell types
dysplasia
disorderly growth - PREneoplastic
most freq in squamous epithelium, also in intestinal mucosa
examples of hyperplasia induced by trophic hormones
estrogen causing increase in # of endometrial / uterine stromal cells
thyroid or prostate enlargement caused by dysregulation
four types of tissue necrosis
coagulative, liquefactive (abscess/cyst), fat, caseous (tb/lungs)
heart/brain/pancreas/lungs
three types of gangrene
dry, wet, gas (latter two may be rapidly fatal)
causes of tissue hypoxia
ischemia (most common), heart failure, lung disease, RBC disorders
three ways that ischemia leads to cell injury
deprivation of oxygen
accumulation of metabolic wastes
deprivation of nutrients for glycolysis
three components of ischemia-reperfusion injury
calcium overload
formation of free-radicals
subsequent inflammation
deficiency of vitamin A
blindness, vulnerability to infection (measles esp)
deficiency of vitamin D
rickets, osteomalacia
deficiency of vitamin E
spinocerebellar degeneration
deficiency of vitamin K
bleeding disorders
deficiency of vitamin B1
beriberi, Wernicke/Korsakoff syndromes
deficiency of vitamin B2 (riboflavin)
stomatitis, glossitis, dermatitis, cheilosis (lips), corneal vacularization
deficiency of niacin
pellagra (three ds: dementia, dermatitis, diarrhea)
deficiency of vitamin B6 (pyridoxine)
glossitis, dermatitis, cheilosis (lips), peripheral neuropathy
deficiency of vitamin B12
megaloblastic pernicious anemia AND degeneration of posterolateral spinal cord tracts (combined system disease)
deficiency of vitamin C
scurvy
deficiency of folate
megaloblastic anemia, neural tube defects
list ways that bacteria cause cell injuries
secretion of digestive enzymes, production of exotoxins, release of endotoxins (on lysis), and indirect injury due to immune response
list physical/mechanical factors injurious to cells
extremes of temperature, abrupt changes in atmospheric pressure, mechanical deformation, electricity, ionizing radiation
etiology of Tay-Sachs and Gaucher diseases
enzymes needed to metabolize lipids are impaired, resulting in accumulation of lipids in neurologic tissue
mucopolysaccharidoses
genetic diseases in which the enzymatic degradation of intracellular connective tissue complexes is impaired, resulting in cellular accumulation (mental retardation, connective tissue disorders)
hemochromatosis
excessive absorption and storage of dietary iron in body tissues causing dysfunction
aneuploidy
abnormal number of chromosomes (more or less than 46)
monosomy
one less chromosome than usual
polysomy
More than two chromosomes to a set
autosome
any chromosome other than a sex chromosome
general rules for chromosomal abnormalities
autosomal monosomy = non-viable
autosomal polysomy = may be viable, nearly always severely disabled
extra/missing sex chromosomes = more common, less debilitating
Trisomy 21
Down Syndrome: most common chromosomal disorder (1 in 1,000)
S/S: MR, protruding tongue, low-set ears, epicanthal folds, poor muscle tone, short stature, congenital heart deformities, susceptible to respiratory infections and leukemia
Incidence rises with
nondisjunction
error in meiosis in which homologous chromosomes fail to separate
Trisomy 18 and 13
Edwards / Patau Syndromes - much less common than Trisomy 21 and resulting in more severe disabilities (avg life expectancy: weeks)
Cri du Chat
Deletion of short arm of chromosome 5: severe retardation, round face, congenital heart anomalies; characteristic infant cry
Generally thrive better than those with a trisomy disorder
Klinefelter Syndrome
syndrome in males that is characterized by small testes and long legs and enlarged breasts and reduced sperm production and mental retardation (XXY)
Turner Syndrome
Chromosome disorder in females. a x chromosome is missing or part of one x is deleted (monosomy X).
short stature, webbed neck, undeveloped ovaries, sterility, amenorrhea, wide chest, congenital heart defects
Multiple X females
relatively common disorder; normal appearance but may experience menstrual abnormalities
Double Y males
relatively common disorder; will generally be taller than average
List some autosomal dominant disorders
familial hypercholesterolemia, polycystic kidney disease, Huntington disease, hereditary spherocytosis, Marfan syndrome, neurofibromatosis, myotonic dystrophy, tuberous sclerosis, familial polyposis coli, Von Willebrand disease, Ehlers-Danlos syndrome, os
Marfan syndrome
Autosomal dominant; fibrillin gene mutation - connective tissue disorder of skeleton, heart, and eyes
Tall with long extremities, fingers and toes, pectus excavatum, hyperextensive joints; cardiac issues; malpositioned or displaced lens
Huntington disease
a degenerative disease of the nervous system caused by a lethal dominant allele that has no obvious phenotypic effect until the individual is 35-45 yrs old (mental deterioration, involuntary movements of arms and legs)
List some autosomal recessive disorders
sickle cell anemia, cystic fibrosis, Tay-Sachs disease, phenylketonuria, mucoplysaccharidoses, galatosemia, homocystinuria, lysosomal storage disease, Wilson disease, hemochromatosis, glycogen storage diseases, thalassemias, congenital adrenal hyperplasia
phenylketonuria
a buildup of phenylalanine caused by an autosomal recessive allele. We screen for this at birth in the US to prevent mental retardation. A special diet is required for the life of the individual
cystic fibrosis
a human genetic disorder caused by a recessive allele for a chloride channel protein in epithelial cells; characterized by an excessive secretion of mucus and consequent vulnerability to infection; fatal if untreated (4% whites are carriers - most common
Patterns of inheritance for sex-linked (X-linked) genetic disorders
affected individuals are almost always male
affected fathers transmit defective gene to all daughters, no sons
unaffected males do not carry the defective gene
carrier female has 1 in 2 chance of producing affected son/ carrier dgt
females affected only r
list some x-linked recessive disorders
Duchenne muscular dystrophy, hemophilia (A & B), chronic granulomatous disease, SCID, Wiskott-Aldrich syndrome, diabetes insipidus, Lesch-Nyhan syndrome, fragile X syndrome
thalassemia
an inherited condition (autosomal recessive) in which abnormal hemoglobin is produced, resulting in anemia
list some examples of multifactorial (polygenic) inheritance
traits: height, weight, intelligence
disorders: hypertension, cancer, diabetes mellitus, cleft lip, and several forms of congenital heart defects
pregnancy categories for medication administration
A - no increased risk shown in any trimester based on human research
B - safe based on animal studies only OR adverse effect in animal studies contradicted by human research showing no increased risks
C - no studies in animals or humans OR adverse effect
chorionic villus sampling
a technique of prenatal diagnosis in which a small sample of the fetal portion of the placenta is removed and analyzed to detect certain genetic and congenital defects in the fetus
TORCH
toxoplasmosis, others (hep B, coxsackievirus B, mumps, poliovirus), rubella, cytomegalovirus, herpes
perinatal infections with these organisms can cause congenital malformations (microcephaly, small eyes, heart defects, hepatomegaly/jaundice, splenomegaly
cachexia
a condition of physical wasting away due to the loss of weight and muscle mass that occurs in patients with diseases such as advanced cancer or AIDS
anaplasia
loss of structural differentiation within a cell or group of cells often with increased capacity for multiplication, as in a malignant tumor
list three retroviruses that may cause cancer
Epstein-Barr: Burkitt lymphoma
HIV: Karposi sarcoma
Human T-lymphocyte virus type I: T-cell leukemia-lymphoma
list four genes linked to cancers
BRCA1 - breast cancer
Rb - retinoblastoma
P53 - suppressor gene defective in half of human cancers
VHL - renal cancer
myc oncogene
allows cells to grow indefinitely in culture but alone cannot induce tumor formation
ras oncogene
allows anchorage independence (proliferation without cell matrix) but alone cannot induce tumor formation
leukocytosis
an abnormal increase in the number of white blood cells in the blood as a result of infection (as in leukemia)
paraneoplastic syndrome
a syndrome or other systemic disturbance associated with but not directly related to a primary tumor or its invasion/metastases. Examples: Fever, weight loss, hypercalcemia, neurologic findings, hematologic syndromes, hypercoagulable state. May be the fir
What are the exceptions to the tumor terminology rules:
suffix -oma = benign
carcinoma/sarcoma = malignant ?
lymphomas, hepatomas, melanomas and leukemias are all malignant
proto-oncogenes
a class of genes (which normally code for components of the cellular growth-activating pathways) whose over-activity or over-expression contributes to cancer (gain-of-function mutations), enhancing cell proliferation and survival.
oncogene
a proto-oncogene in its mutant, overactive or over-expressed form
tumor suppressor genes
a category of genes (which normally inhibit cell proliferation) that may contribute to cancer if lost, inhibited, under-active or under-expressed (loss-of-function mutations)
Rb gene
an example of a tumor-suppressor gene that encodes proteins that are negative regulators or inhibitors of cell division (retinoblastoma)
P53 gene
A tumor-suppressor gene that codes for a specific transcription factor that promotes the synthesis of proteins that inhibit the cell cycle. (more than half of all human tumors lack functional P53)
in normal cells, allows for a "break" so DNA can be repair
ras gene
an oncogene causing anchorage independence in cancer cells
myc gene
an oncogene allowing a cancer cell to grow indefinitely
three steps of carcinogenesis
initiation, promotion, progression
telomerase
catalyzes the lengthening of telomeres in eukaryotic germ cells, thus restoring their original length and compensating for the shortening that occurs during DNA replication (cancer cells use to gain immortality)
name 3 tumor markers
PSA: prostate-specific antigen for prostate cancer
AFP: alpha-fetoprotein for hepatic carcinoma, gonadal tumors
CEA: carcinoembryonic antigen for adenocarcinoma of colon, pancreas, breast, ovary, lung, stomach
angiogenesis
the formation of new blood vessels (necessary for a tumor to enlarge more than about 2 mm in diameter)
tumor grading
histologic characterization of tumor cells (determination of the degree of anaplasia) - 3 or 4 classes of increasing degrees of malignancy
tumor staging
describing the location and pattern of spread of a tumor within a host
TNM staging
tumor, node, metastasis (generally ANY distant metastasis results in a Stage IV determination
Cancer's 7 warning sings
C - change in bowel or bladder habits
A - a sore that does not heal
U - unusual bleeding or discharge
T - thickening or lump
I - indigestion or difficulty swallowing
O - obvious change in wart or mole
N - nagging cough or hoarseness
Cancer's warning signs in children
C - continued unexplained weight loss
H - headaches with vomiting in the morning
I - increased swelling or persistent pain in bones or joints
L - lump or mass in abdomen, neck or elsewhwere
D - development of whitish appearance in pupil of the eye
R - rec
paraneoplastic syndromes
Symptoms that cannot be explained by the local or distant spread of the tumor commonly caused by substances released from the tumor or an immune response triggered by the tumor
list examples of paraneoplastic syndromes
1. hypercalcemia [associated with abnormal production of parathyroid hormone-related protein (PTHrP) by tumor cells
2. Cushing syndrome secondary to excess adrenocorticotropic hormone (ACTH) secretion
3. hyponatremia and water overload secondary to excess
IgA
the class of immunoglobulin having alpha heavy chains; these antibodies are those in dimeric form present in mucosal secretions; present in the blood in monomeric form
List 3 main types of vasoactive chemicals released during the inflammatory process
histamine, prostaglandins, leukotrienes
chemotaxis
movement in response to the presence of a chemical, such as inflammatory mediators attracting neutrophils to an injured site
opsonin
molecules that 'tag' pathogenic microorganisms for destruction by cells of inflammation (neutrophils and macrophages); antibodies and complement are the two main opsonins
passive immunity
the transfer of plasma (sera) containing preformed antibodies against a specific antigen from a protected or immunized person to an unprotected or nonimmunized person
active immunity
the body's immune response (to confer a protected state) as a result of active infection or immunization - requires memory B cells
dendritic cell
monocyte-derived cell type that specializes in capturing and presenting antigens to T cells (strategically located in subcutaneous and submucosal tissues)
structurally and functionally similar to macrophages
some types produce IFN-a & -b (type 1 interfero
describe monocytes and macrophages
account for about 5% of total WBC count
monocytes circulate for 3 days before entering tissues > macrophage
phagocytize foreign substances and help activate T cells
unlike short-lived neutrophils which die while fighting infections, macrophages may live f
what are some of the names of macrophages found in different tissues?
microglial cells (brain)
alveolar macrophages (lungs)
Kupffer cells (liver)
mesangial phagocytes (kidney)
synovial A cells (joints)
monocytes (blood)
Langerhans cells (skin)
Natural Killer cell
a type of lymphocyte lacking T-cell and B-cell markers, produced in and released from bone marrow and found mainly in the spleen and circulation
what are the primary lymphoid organs?
bone marrow and thymus gland (where lymphocytes develop)
T-cells mature in the thymus, B-cells in the bone marrow
list the secondary lymphoid organs
tonsils and adenoids, spleen, lymph nodes, Peyer patches (in intestinal walls and respiratory/urogenital tracts) and appendix
(T and B lymphocytes migrate to these locations once matured)
list & describe the main types of leukocytes (white blood cells)
Developed from lymphoid stem cells:
- B cell and T cell lymphocytes
- Plasma cells (mature B cells in lymph nodes; secrete antibodies)
- Natural Killer (NK) cells
Developed from myeloid stem cells:
- monocytes, which mature into macrophages
- granulocytes
which components of the immune system are innate and which are specific?
innate (no previous exposure required): NK cells and phagocytic cells such as neutrophils and macrophages
specific (respond more effectively after previous exposure): B and T cell lymphocytes
which secondary lymphoid organs have no afferent (incoming) lymphatic vessels
tonsils and spleen
(they do have efferent drainage to allow migration of activated lymphocytes to other lymphoid organs)
cytokines
hormonal signaling molecules, produced in the bone marrow and by various other cells, which stimulate stem cell growth, proliferation and differentiation into particular cell types
shift to the left
an increased ratio of bands (immature) to mature neutrophils, commonly seen with acute bacterial infections
describe neutrophils
account for 60-80% of total WBC count
half-life of 4-10 hours in circulation
early responders to acute bacterial infection
phagocytes which engulf and degrade microorganisms
produce chemical mediators which help to destroy microorganisms
describe eosinophils
account for 1-6% of total WBC count
mature in bone marrow in 3-6 days, circulate for about 30 minutes
half-life of 12 days in tissue
particularly associated with allergic reactions and intestinal parasites
release inflammatory chemicals
describe basophils
account for 0-2% of total WBC count
circulate in the vascular system (whereas mast cells are found in connective tissue, especially around blood vessels, under mucosa)
life span of days (whereas mast cells live weeks or months)
have IgE receptors for IgE
list some of the surface receptors on macrophages
Fc receptors - bind to part of antibody called constant fragment
C3b receptors - bind to complement component C3b
Mannose receptors - recognition of common microbial structures
selectins/integrins - bind to capillary walls & extracellular matrix
lymphocytes
NK, B and T cells account for 20-30% of the total WBC count
Exposure to antigens stimulates B cells to mature into antibody-secreting plasma cells as well as memory cells
two types of T cells
T helper cells (CD4 proteins): secrete cytokines to activate other cells
Cytotoxic T cells (CD8 proteins): destroys foreign cells, mutants, and virally infected cells
CRP
C-reactive protein is an acute phase protein released by the liver in response to macrophage-dervied cytokines - it binds to bacterial cell membranes, acting as an opsonin to facilitate phagocytosis
CRP activity is a nonspecific indicator of inflammation
ESR
erythrocyte sedimentation rate ("sed. rate") is a nonspecific but useful indicator of inflammation -- acute phase proteins released by the liver cause an increase in serum fibrinogen, which coats RBCs, reducing their charge so that they aggregate more rea
4 types of exudate
serous - watery, low protein, mild inflammation (blister)
fibrinous - sticky, thick, healing requires removal
purulent - pus, severe inflammation, neutrophils + protein + debris
hemorrhagic - contains many RBCs, most severe inflammation
5 ways antibodies function to enhance the localization and removal of antigens from the body
precipitation & agglutination
neutralization of bacterial toxins
opsonization
complement activation
five classes of antibodies (immunoglobulins)
IgG - monomer, 75-80%, 3-wk half-life, equally intravasc/interstitial, two of these molecules needed to activate complement
IgM - pentamer, 10%, 10-day half-life, predominantly intravascular, first antibody produced post-exposure, major antibody on b-cell
4 types of hypersensitivity
I - atopic, anaphylactic (immediate) > IgE, mast cells > 15-30 min (ex: asthma, rhinitis, atopic eczema, bee-sting reaction)
II - cytotoxic, cytolytic (tissue specific) > IgM or IgG, NKs/complement > 15-30 min (ex: ABO tfx, newborn hemolysis, myasthenia g
primary immunodeficiency disorders
SCID: functional B and T cells lacking; sepsis, opportunistic infections
Wiskott-Aldrich: eczema, thrombocytopenic purpura,
DiGeorge: T-cell agenesis related to lack of thymus function
chronic mucocutaneous candidiasis: abnormal T cells re: Candida
select
anemia
hematocrit: 25-30% or hemoglobin: 8-10 g/dl
pallor, fatigue, malaise, SOB, decreased activity tolerance
thrombocytopenia
platelet count: < 20,000
petechiae, easy bruising, bleeding gums, occult hematuria, retinal hemorrhages, spontaneous intracranial bleeding (fatal)
neutropenia
neutrophil count < 500
infection
myleoproliferative diseases
Chronic Myeloid Leukemia (CML): ^ granulocytes, splenomegaly (fatigue, weight loss, sweats, bleeding, abd discomfort); primarily adults, insidious onset, poor response to chemotherapy, Philadelphia chromosome; 15% of all leukemia cases
Polycythemia Vera (
Acute Myeloid Leukemia (AML)
adult onset primarily (median age 64); usually a malignancy of granulocytes; bone marrow aspirate must have > 20% blasts; many subtypes; acute onset; responds fairly well to treatment; worse prognosis than ALL; bone pain, anemia, thrombocytopenia, infecti
Types of lymphoid neoplasms
Leukemias (blood/marrow):
- Chronic Lymphoid Leukemia (CLL): 95% associated with mature B cell; initially asymptomatic, then fatigue, weight loss, anorexia, infection, enlarged painless lymph nodes; primarily affects adults
- Acute Lymphoblastic Leukemia
Hairy Cell Leukemia
A rare (2%0 lymphoid neoplasm of the elderly. Mature B-cell tumor. Cells = filamentous, hairlike projections. Splenomegaly. HTLV-II
Multiple Myeloma
Malignant tumor of bone marrow (or lymph nodes, liver, spleen, kidney); overproduction of immunoglobulins and destruction of bone tissue. Occurs only in adults, usually 40+ (median age: 65)
disease affects kidneys, immune, circulatory systems
verrucae
warts - papillomaviruses (HPV) - stratum corneum irregularly thickened
herpes simplex virus
type 1: above the waist (most common on lips/face/mouth) 10-14 d
type 2: genital area NO CURE
postherpetic neuralgia
complication of herpes zoster (shingles) in people over 50 - can cause blindness
three genera of fungi which commonly infect human skin
Microsporum, Trichophyton, Epidermophyton
can cause tinea infection - AKA dermatophytes
list common tinea/dermatophyte infections and their locations
capitis - scalp
barbae - beard
faciei - face
corporis - trunk
manus - hand
cruris - groin
pedis - foot
(all previous preceded by "tinea")
onychomycosis - nails
Candida albicans manifests itself how?
newborns: thrush (oral candidiasis)
infants/bedridden: intertrigo
immune impaired: mucocutaneous candidiasis (systemic)
impetigo
acute, contagious - caused by staphylococci or streptococci
vesicles, pustules, yellowish crusts
treat with 2% mupirocin (Bactroban) or 1% retapamulin (Altabax)
syphillis
Treponema pallidium can be cured in the primary and secondary stages using penicillin (contagious during both stages)
three stages of syphillis
Primary - single chancre on genitalia
Secondary - disseminated rash, not clearly distinguishable
Tertiary - damages CV and CNS
leprosy
Mycobacterium leprae - low rate of infectivity, responsive to sulfone drugs (dapsone)
compare two types of lupus erythematosus (LE)
Chronic Discoid LE: thickened patches with enlarged follicles & elevated border, seen on scalp, ears, chest / can cause atrophy, scarring, pigment changes / chronic course with gradual progression and slow healing
Systemic LE: diffuse, puffy lesions, also
seborrheic dermatitis
areas of high oil gland concentation (inflammatory condition)
cradle cap, dandruff - not curable but may be controlled
psoriasis
papules / plaques with silvery scale - cause unknown (multifactorial inherited inflammatory conditon)
lichen planus
any of several eruptive skin diseases characterized by hard thick lesions grouped together and resembling lichens growing on rocks
wrists, ankles, trunk (& sometimes nails) - can spread with scratching
pityriasis rosea
rash, unknown origin - young adults - spring/fall - viral? - macule or papule surrounded by erythema, spreads with central clearing - trunk or neck (+ maybe extremities, face, scalp) - pruritis (mile to severe)
pemphigus
group of disorders - bullous eruptions (blisters) - autoimmune?
vulgaris, vegetans, foliaceus, erythematosus
rhus dermatitis
allergic contact dermatitis - poison ivy/oak/sumac
pruritus
an intense itching sensation
vasculitis
inflammation of a blood vessel
what are three effects of sympathetic activation on the heart
increases heart rate (chronotropic effect)
increases speed of conduction (dromotropic effect)
induces heart muscle to contract more forcefully (inotropic effect)
pernicious anemia
lack of intrinsic factor > no B12 absorbed > disrupted DNA synthesis in bone marrow blast cells > megaloblastic dysplasia (autoimmune?)
neurologic lesions/degeneration also seen
ACYANOTIC congenital heart anomalies
atrial or ventricular septal defects, patent ductus ateriosus, coarctation of the aorta, pulmonary/aortic stenosis or atresia
these disorders result in left-to-right shunting of blood
CYANOTIC congenital heart anomalies
tetralogy of Fallot, transposition of great arteries, truncus ateriosus and tricuspid atresia
these disorders result in right-to-left shunting of blood
cardiac tamponade
external compression of heart chambers such that filling is impaired - can happen with sudden or large pericardial effusion
types of hemophilia
A - deficiency in Factor VIII - 85% - X-linked recessive or spont. mut.
B - deficiency in Factor IX (aka Christmas Disease)
terminology for blood clots
general term: thrombus
inflamed vein caused by a clot: thrombophlebitis
embolus: blood clot that has moved & become stuck in a new spot
emblous in the heart: myocardial infarction
embolus in the lungs: pulmonary embolism
embolus in the brain: stroke
ideal cholesterol, LDL, HDL
Total cholesterol should be < 200
LDL should be < 160
HDL should be > 40
aterial pulse pressure
difference between systolic and diastolic pressures
pulse pressure
determined by stroke volume, speed of ejection and aterial distensibility
function of the alpha-1 receptors
innervation of the arterioles
another name for antidiuretic hormone
vasopressin
normal calcium concentration in blood
9-11 mg/dl or
4.5-5.5 mEq/L
normal magnesium concentration in blood
1.5 - 2.5
normal phosphate concentration in blood
2.5 - 4.5 (higher in children, neonates)
normal potassium concentration in blood
3.5 - 5.0 (higher in neonates)
normal sodium concentration in blood
135 - 145 (higher in neonates)
normal range of blood pH
7.35 - 7.45
Lower - acidic / higher - alkaline
normal range of PaCO2
35 - 45
Lower - alkalosis / higher - acidosis
normal range of HCO3-
22-26
lower - acidosis / higher - alkalosis
acute respiratory failure - blood gas values?
PaO2 - < 60 mmHg (hypoxemia)
PaCO2 - > 50 mmHg (hypercapnia)
pH - < 7.3 (acidosis)
list some causes of acute bronchitis
viral: influenza A & B, parainfluenza, respiratory syncytial, coronavirus, rhinovirus, Coxsackie, adenovirus
bacterial: streptococcus pneumoniae, haemophilus influenzae, mycoplasma, moraxella, chlamydia pneumoniae
other: heat, smoke inhalation, chemicals,
describe chronic bronchitis
type B COPD - "blue bloater" - ^ bronchial mucus & cough > 3 months each year for 2+ years - persistent, irreversible airway obstr. - SOB on exertion - edema, hypervolemia - hx of smoking - low PaO2 and high PaCO2 - persistent hypoxemia >> polycythemia -
describe emphysema
type A COPD - "pink puffer" - destructive changes of alveolar walls - caused by smoking, air pollution, certain occupations - may follow bacterial lung infection - exertional dyspnea - accessory muscles, pursed-lip breathing - cough minimal or absent - di
bronchiectasis
recurrent inflam of bronchial walls, chronic cough, aneurysm-like dilatations of the bronchiloes (creating pockets of infection/pus) - 50% of cases associated with cystic fibrosis
treated with antibiotics and removal of secretions
bronchiolitis
widespread bronchiloar inflammation (assoc with smoking and various infectious agents) - mucosal swelling, ^ mucus production, bronchial muscle constriction - narrowing airway - wheezing, dyspnea
treated with bronchodilating agents, mgmt of underlying cau
cystic fibrosis
autosomal recessive disorder of exocrine glands / mucus cells - secretions are excessively thick - airway obstruction, atelectasis, air trapping - assoc sx related to pancreas dysfx - most common genetic disorder in the U.S.
treatment focuses on removing
epiglottitis
inflammation of the epiglottis caused by Hib - rapid onset of fever, pain, difficulty swallowing, drooling - MEDICAL EMERGENCY
croup
viral infection of subglottic area (6 mos. to 3 yrs.) - cough, stridor following upper resp infection
treated with humidification, oxygenation, inhaled epinephrine
diffuse interstitial pulmonary fibrosis
restrictive disorder - thickening of alveolar interstitium - immune-mediated disorder following lung injury - immune cell infilration, fibrin deposition >> stiff, noncompliant lungs - decreased tidal volume, increased respiratory rate - treated with corti
sarcoidosis
a chronic disease of unknown cause marked by the formation of nodules in the lungs (granulomas - fibrotic, associated with histiocytes) and liver and lymph glands and salivary glands
sx: progressive dyspnea, fever, enlarged lymph nodes, inflammation
reduc
hypersensitivity pneumonitis
group of inflammatory lung disorders associated with inhalation of organic particles - antigen-antibody complexes deposited in the lung, leading to inflammation and granuloma production
sx: general inflammation (fever, chills, malaise), dyspnea, dry cough
Acute (Adult) Respiratory Distress Syndrome (ARDS)
damage to alveolar-capillary membrane - decline in PaO2 does not respond to supplemental oxygen therapy - assoc with trauma, sepsis or shock - mortality rate of 50% - noncardiogenic pulmonary edema (leaky capillaries) - atelectasis (lack of surfactant) -
Infant Respiratory Distress Syndrome (IRDS)
aka hyaline membrane disease - similar to (adult) ARDS - premature neonates (60% of those born at < 30 wks - primary cause is lack of surfactant - nasal flaring, expiratory grunt, thoracic retractions, rapid shallow respiration - severe hypoxemia and acid
list neuromuscular diseases that can affect respiration
poliomyelitis, amyotrophic lateral sclerosis (ALS), muscular dystrophies, Guillain-Barre Syndrome, myasthenia gravis
primary differentiating feature between bacterial and viral pneumonia?
bacterial - productive cough (also possible inflammatory exudate)
viral - NON-productive cough (no exudative fluids)
Severe Acute Respiratory Syndrome (SARS)
acute respiratory infection caused by a coronavirus - severe pneumonia with mortality rate of 10%
hormones released by the kidney to adjust fluid excretion
aldosterone induces kidneys to conserve saline (salt + water) - urine becomes concentrated as kidneys retain more water
ANP and BNP promote saline excretion
extracellular fluid volume (ECV) deficit
aka saline deficit - result of loss of sodium-containing fluids (emesis) - reduced volume of vascular & interstitial fluids - normal serum Na - weight loss, poor turgor, oliguria, postural hypotension
other causes: diarrhea, gastric suction, fistula drian
extracellular fluid volume (ECV) excess
aka saline excess - kidneys retaining sodium & H2O - increased volume of vascular & interstitial fluids - normal serum Na - weight gain, peripheral edema, distended neck veins, dyspnea
causes: excessive IV infusion of isotonic solutions or renal retention
hyponatremia
aka water excess - excessive ADH secretion or hypotonic fluid intake - low serum Na, body fluids abnormally dilute - cell swelling causes confusion, lethargy, seizure, coma
other causes: medical infusion of hypotonic solutions (D5W by IV, irrigation, tap
hypernatremia
aka water deficit - inadequate water intake or excessive excretion/loss - high serum Na, body fluids too concentrated - cell shriveling causes confusion, lethargy, seizure, coma (same sx as hyponatremia)
causes: tube feeding, IV infusion of hypertonic sol
clinical dehydration
ECV deficit (sodium-containing fluids lost) + hypernatremia (water loss) = loss of hypotonic fluids such as in gastroenteritis - sx of both!
causes of edema at capillary level
increased capillary hydrostatic pressure
increased insterstitial fluid osmotic pressure
blockage of lymphatic drainage
decreased capillary osmotic pressure
compare contrast hydrostatic/osmotic pressure at the capillary level
cap hydrostatic (outward push of fluids against inner cap walls)
interstitial osmotic (inward pull force caused by interstitial particles)
cap osmotic (inward pulling force caused by vascular particles)
interstitial hydrostatic (outward push of fluids aga
how do water and electrolytes move in & out of cells
water - osmosis
electrolytes - transport by membrane channels & carriers
some causes of hypokalemia (low potassium)
anorexia, alkalosis, potassium-wasting diuretics, corticosteroid therapy, black licorice, diarrhea, emesis
some causes of hyperkalemia (high potassium)
large transfusion of stored blood, acidosis, potassium-sparing diuretics, oliguria, adrenal insufficiency, renin deficiency
some causes of hypocalcemia
chronic kidney disease (deficiency of activated vita D), steatorrhea, pancreatitis, chronic diarrhea, hypoparathyroidism, high phosphates (from intake or tumor lysis syndrome), hypomagnesemia, alkalosis, large transfusion of citrated blood, rapid infusion
some causes of hypercalcemia
vita D overdose (shark cartillage), hyperparathyroidism, immobilization, Paget disease, bone tumors, multiple myeloma, leukemia, thiazide diuretics
some causes of hypomagnesemia
chronic alcoholism, malnutrition, ileal resection, chronic diarrhea, steatorrhea, pancreatitis, elevated free fatty acids, diabetic ketoacidosis, hyperaldosteronism, emesis, gastric suction
some causes of hypermagnesemia
ingestion/aspiration of seawater, medications (laxatives, antacids), oliguric renal failure, adrenal insufficiency
some causes of hypophosphatemia
chronic alcoholism, chronic diarrhea, excessive/long-tern use of some antacids, refeeding after starvation (e.g. anorexia), TPN, respiratory alkalosis, insulin, epinephrine, alcohol wd, diuretic phase after extensive burns, diabetic ketoacidosis, diuretic
some causes of hyperphosphatemia
excessive use of certain enemas/laxatives, crushing injury, rhabdomyolysis, chronic kidney disease, adrenal insufficiency
describe manifestation of potassium imbalances
hypokalemia >> hyperpolarization, smooth muscles LESS reactive to stimuli (leading to hypoactive bowels, hypotension)
hyperkalemia >> hypopolarization, smooth muscles MORE reactive to stimuli (leading to intestinal cramping, diarrhea)
both cause skeletal
describe manifestations of calcium imbalances
hypocalcemia >> decreases threshold potential of nerve/muscle cells, leading to hyperexcitability (twitching, tetany), Trousseau sign, Chvostek sign
hypercalcemia >> increases threshold potential of nerve/muscle cells, leading to neuromuscular depression
describe manifestations of magnesium imbalances
similar to calcium imbalances - Mg inhibits ACh at neuromuscular junctions, thus:
hypomagnesemia >> hyperexcitability (hyperreflexia, twitching)
hypermagnesemia >> depresses neuromuscular excitability (hyporeflexia and flaccid paralysis)
describe manifestations of phosphate imbalances
hypophosphatemia >> cellular ATP deficit >> general depression of all physiologic functions (cardiac, CNS, respiratory, etc.)
hyperphosphatemia >> hypocalcemia (neuromuschular excitability) OR precipitation of calcium phosphate into soft tissues (stiff, a
Trousseau sign
carpal spasm after occlusion of aterial blood flow to the hand for approximately 3 minutes (indicated increased neuromuscular irrability from ANY cause, including hypocalcemia)
Chvostek sign
spasm of muscles in the cheek and corner of the mouth produced by tapping the facial nerve in front of the ear (indicated increased neuromuscular irrability from ANY cause, including hypocalcemia)
why must hypomagnesemia be treated prior to hypokalemia?
hypomagnesemia can cause hypokalemia by increasing urinary excretion of potassium
list the body's chief buffers and where they can be found
bicarbonate - extracellular
phosphate - intracellular and urine
hemoglobin - RBCs
protein - intracellular, blood
what is the ratio of bicarbonate ions to carbonic acid necessary to keep blood pH in normal range
20 bicarbonate ions to 1 carbonic acid
what is the only acid that the kidneys cannot excrete
carbonic acid - this is excreted solely by the lungs
list endocrine hormones in the kidney & their functions
aldosterone & angiotensin II (AII) stimulate reabsorbtion of Na & H2O
atrial natriuretic peptide (ANP) & urodilatin inhibit reabsorption
ADH increases permability of collecting tubule to water, resulting in increased reabsorption and reduced blood osmolal
list diuretics and their mechanisms of action
osmotics increase solute load in tubule
ACE inhibitors block production of AII and aldosterone
loop diuretics block Na-K-Cl pumps in the ascending Loop of Henle
thiazides block Na reabsorption in the distal tubule
aldosterone inhibitors block the action o
specific gravity of normal urine
1.003 to 1.03
It is also normally acidic
pyelonephritis
infection of renal pelvis and interstitium
predisposing factors: vesicouretal reflux, preganancy, neurogenic bladder, instrumentation, urinary obstruction, female sexual trauma
chronic: manifested as small atrophic kidneys (scarring, blunting)
nephrotic syndrome
increased glomerular permeability to proteins, resulting in:
proteinuria, hypoalbumenemia, generalized edema (decreased colloid osmotic pressure), hyperlipidemia, hypercoagulability (liver activity stimulated by hypoalbumenemia)
azotemia
accumulation in the blood of nitrogen-bearing waste products (urea) that are usually excreted in the urine
characteristic findings in renal failure
increased phosphorous, decreased calcium, increase in creatinine
causes of incontinence
urge - involuntary detrusor muscle contractions
stress - increased intra-abdominal pressure + pelvic muscle laxity
overflow - urinary retention + overdistended bladder (secondary to obstruction or detrusor inactivity or sphincteric malfunction)
pyuria
presence of white blood cells in the urine
dysuria
painful or difficult urination
condylomata acuminata
genital warts, caused by various strains of papillomavirus
HPV infection is a risk factor for cervical cancer
corpus luteum
site of ruptured follice (after ovulation) - releases estrogen, progesterone
metrorrhagia
bleeding between menstrual periods
menorrhagia
increase in the amount or duration of menstrual bleeding
polymenorrhea
increased frequency of menstruation
oligomenorrhea
infrequent menstruation
cystocele
hernia in which the urinary bladder protrudes through the wall of the vagina
leiomyomas
benign tumor of smooth muscle (usually in the uterus or digestive tract) - mostly commonly seen in reproductive years
endometriosis
the presence of endometrium elsewhere than in the lining of the uterus
list some benign neoplasms of the breast
adenomas, fibroadenomas, papillomas,
what two types of sexually transmitted infections result in the formation of ulcerative lesions but do not progress to systemic involvement?
chancroid (soft chancre) and granuloma inguinale
what STDs can progress to systemic involvement?
syphillis, cytomegalovirus, hepatitis B
treponema pallidum
anaerobic spirochete that causes syphillis
list enetric organisms that can be transmitted through anal intercourse
giardia, campylobacter, shigella
what STDs can lead to pelvic inflammatory disease?
PID is usually the result of salpingitis caused by gonorrhea or chlamydia
what is required if HSV type 2 herpes lesions are active at the time of delivery?
caesarean section - in newborns, HSV 2 may result in severe damage to organ systems (high mortality rate)
two types of bone
cancellous/trabecular - spongy, lighter - thin plates called trabeculae
compact/cortical - dense, covers cancellous bone- concentric layers
stages of fracture healing
1 - hematoma (days 1-3)
2- fibrocartilage formation (weeks 1-2)
3 - callus formation (weeks 2-6)
4 - ossification (3 weeks to 6 months)
classification of joints
1. synarthroses - nonsynovial joints
a. - fibrous (sutures in skull, gomphosis in teeth, syndesmosis as in fibula-tibia joint)
b. - cartilaginous (symphysis, synchondrosis)
2. diarthroses - synovial joints (designed for mobility)
synotosis
ossified joint after complete growth
examples: skull sutures, first sternocostal joint
two kinds of biaxial joints
saddle (sellar) - as in thumb
condyloid - as in metacarpophalangeal joints in hand
(movement in two planes, i.e flexion/extension and abduction/add.)
two kinds of uniaxial joints
hinge/ginglymus - as in finger, elbow or knee (flexion/extension)
pivot - as in 1st/2nd vertebrae (rotation) or elbow
two kinds of triaxial joints
carpal joints (wrists), ball-and-socket joints (hips, shoulders)
motion three planes
contrast ligaments and tendons
ligaments attach bone to bone and guide joint motion while providing mechanical stability
tendons attach muscle to bone and assist in the generation of movement
tendons have greater collagen content than ligaments
neither are contractile structures (like
most common sites for bone metastases
vertebral bodies, pelvis, proximal ends of the femur and humerus, and the ribs
malignant bone tumors
ostrosarcoma - 10-30 y.o. or 60-70 y.o., found in active epiphyseal growth areas (distal femur, proximal tibia/fibula/humerus), flat bones
leads to fractures, early metastasis to lungs, compromised joint fx
chondrosarcoma - slow development, pain not prom
benign primary bone tumors
osteochondroma - hereditary, 30 or younger, usually on metaphyses of long bones, asymptomatic bony projections capped with cartilage
chondroma/enchondroma - 30-40 y.o., cartilage-forming tumor, medullary cavity or subperiosteal layers, sm bones of hands/f
types of muscular dystrophy
Duchenne - most common, males only, X-linked (death by age 20)
Becker - inherited, slower muscle degeration
fascioscapulhumeral - rare, autosomal dominant, shoulder/face
All - muscle tissue replaced by fat & fibrous connective tissue, calf hypertrophy com
nociception
physiologic mechanisms involved in pain
4 stages: transduction, transmission, perception, modulation
encopresis
involuntary defecation not attributable to physical defects or illness
pheochromocytoma
a vascular tumor of the adrenal gland
secretes catecholamines, causing headache, tachycardia, sweating
Wilson disease
copper accumulation - liver, neurological
degrees of burns
1st - no blisters
2nd - fluid-filled blisters (partial-thickness) or flat blisters (full-thickness)
3rd - subcutaneous blisters
desquamation
loss of bits of outer skin by peeling or shedding or coming off in scales
stratum corneum
outermost layer of the epidermis
hallmark for diagnosis of TB
Ghon (Ranke) complex - a pulmonary calcified lesion
Mallory-Weiss
esophageal tear
Graves
exopthalmia
Hashimoto
autoimmune thyroditis - enlarged thyroid
Cushing
truncal obesity, moon face, buffalo hump, HTN
Addison
hyperpigmentation, dehydration, orthostatic hypotension
hepatitis - transmission routes
A - fecal-oral route (contaminated food)
B - blood or sex or perinatal
C - blood (IV drugs, transfusions before 1990) this type is the type most likely to cause chronic liver disease
D - blood
E - fecal-oral
thalassemia
alpha - mostly in Asians - usualy silent carriers or mild anemia
beta - mostly in Mediterraneans - can be major, minor or intermedia
major = homozygous, minor = heterozygous