Term
|
Definition
| initially no change hematocrit, plasma proteins replaced more quickly than RBC |
|
|
Term
|
Definition
| rate or loss exceeds regenerating, usu limited by availibility or IRON |
|
|
Term
|
Definition
destruction of RBC, retention of iron and expansion of marrow,
intravascular- hemoglobinemia, hemoglobiuria, hemoglobiura, hemosiderinura, can lead to acute tubular necrosis
extravascular- more common, phagocy in spleen and liver, can lead to jaundice, bilirubin gallstones and splenomegaly |
|
|
Term
warm autoimmuno hemolytic anemia
fix or not? where are RBCs? |
|
Definition
| IgG > 37 deg C doesnt fix complement, RBC in spleen cause splenomegaly and hemolysis is extravascular |
|
|
Term
warm autoimmuno hemolytic anemia
fix or not? where are RBCs? |
|
Definition
| IgG > 37 deg C doesnt fix complement, RBC in spleen cause splenomegaly and hemolysis is extravascular |
|
|
Term
cold agglutin AHA
fix or not? where are RBCs? |
|
Definition
| IgM < 30C, fixes complement, RBC seq in the liver (Kuppfer cells), extravascular lysis in spleen, anemia, Raynaulds phenom |
|
|
Term
|
Definition
| massive intravascular hemolysis, complement dependent, IgG autoantibodies against P group antigen, occurs after infections |
|
|
Term
|
Definition
|
|
Term
| antimalarial drugs can trigger |
|
Definition
| a hemolytic crisis (primaquine and quinacrine for eg) |
|
|
Term
|
Definition
| irregularly shaped erythrocyte fragment caused by mechanical trauma or an intrinsic abnormality of erythrocytes |
|
|
Term
|
Definition
inherited defect in membrane, mutation spectrin or ankrin cytoskeletal protein
rbc less deformable, more vulnerable to destruction in spleen, anemia, splenomegaly, jaundice |
|
|
Term
|
Definition
autosomal recessive, mut glutamine to valine at amino acid 6 of beta chain, protects again malaria
complications: actue chest syndrome, CNS stroke, aplastic crisis, prone to infection
Consequences of sickling are membrane damage and extravascular hemolytic anemia, microvascular occlusions, Occlusion rate correlates with infection, inflammation, dehydration, and acidosis which trigger sickling, Prenatal diagnosis with restriction fragment length polymorphisms (RFLPs), Radio Hair on end (not as dramatic as thalasemia) |
|
|
Term
| what are precipitating factors for sickle cell anemia? |
|
Definition
cold exposure, decreased fluid, high altitude, overexcretion, increased blood viscosity, infection, blood loss, pain, anxiety
then use gel electrophoresis to test for HBS Ag |
|
|
Term
|
Definition
Beta point mutation, alpha gene deletion
In beta, unpaired alpha globin chains form aggragates that damage RBC precursors and impair eryhtropoeisis, Globin chain aggreagtes precipitate and damage RBC membrane |
|
|
Term
| Glucose-6- phosphodiesterase deficiency |
|
Definition
| G6PD is enzyme in hexose monophosphate shunt that produces GSH (needed for protection form oxidants), can protect against malaria, on X chromosome |
|
|
Term
| what will cause symptoms of G6PD to come out? |
|
Definition
| exposure to oxidative stress- antimalarials, sulfonimides, aspirin infections. |
|
|
Term
|
Definition
| in G6Pd the Hg oxidizes to Met-Hg and denatures/precipitates in cytoplasm. these lea to INTRAVASCULAR hemolysis, "bite" cells produced as splenic macrophages try to pluck them out and dec the size of RBC and make SPHEROcytes |
|
|
Term
| paroxysmal noctural hemoglobinuria |
|
Definition
mutation in PIGA gene causes RBC sensitive to comoplement lysis, acquired membrane defect secondary to mutation that alters myeloid stem cells, deficiency CD 55 and 59 and x-linked
hemolysis in your SLEEP when blood is more acidic.
positive sucrose lysis test, acute leukemia transformation |
|
|
Term
|
Definition
| Parapsites cause hemolytic anemia, hyperplasia of phagocyte system (splenomegaly), cerebral malaria |
|
|
Term
|
Definition
| Megaloblastic, decreased intrinsic factor activity causes b12 malabsorption from genetic defect or chronic atrophic gastritis |
|
|
Term
|
Definition
| same as B12 but no CNS changes. best source of folate from uncooked fruit and vegetable |
|
|
Term
|
Definition
| normochromic, normocytic, defect in multipotent myeloid stem cell, usu with neutropenia and thrombocytopenia |
|
|
Term
|
Definition
| autosomal recessive, inherited form of aplastic anemia, can cause aplastic anemia, renal hypoplasia, absent or hyperplastic thumbs, hyperpigmentation of skin, microcephaly |
|
|
Term
|
Definition
Genetic, Defect in erythroid progenitor, Normochromatic, normocytic
mediastinal mass, marrow shows decrease erythroid precursors, hemorrhage from gingiva |
|
|
Term
|
Definition
describes incomplete expansion of the lungs or the collapse of previously inflated lungs. may compromise oxygenation and predispose to infection.
hypoxia happens.
3 types- resorption, compression and contraction |
|
|
Term
|
Definition
reversible.
if air highway blocked in form of tumor, foreign body,
air that is in lung cannot go out and it gets resorped into lung and eventually it collapses
(if someone aspirates something move likely to get in right lung since its more vertical) |
|
|
Term
|
Definition
air, knife wound, car accident, blood. you wont have the negative pressure since the lung is too compressible.
eg transudate in CHF. |
|
|
Term
|
Definition
fibrosis of the lung.
now the alveoli doesnt have as much surface area. |
|
|
Term
| obstructive dx in lung vs restrictive |
|
Definition
obs will limit airflow into lung
and restrictive dx reduces the expansion of lung parenchyma eg is lung fibrosis. |
|
|
Term
|
Definition
amt of air u breathe in
FEV1 is what u breathe out in one second
FEV1/FVC norm is 80% |
|
|
Term
| normal FEV1 FVC and ratio values |
|
Definition
|
|
Term
| FEV1 FVC and ratio in OBSTRUCTIVE |
|
Definition
|
|
Term
| FEV1 and FVC and ratio in restrictive |
|
Definition
FEV1 2.8L
FVC 3.1L
FEV1/FVC = 90% |
|
|
Term
|
Definition
obsructive
permant inc in size in airspaces distal to terminal bronchioles
destruction of alveolar walls
weight loss, barrel chest, hyperventilation, normal Hb oxy, pink puffers |
|
|
Term
| how do ppl with empysema die usually? |
|
Definition
| respiratory insufficiency and cor pulmonale |
|
|
Term
|
Definition
| respiratory bronchioles and alveolar ducts involved but distal alveoli spared. upper lbes, middle aged men. 1/2 of smokers, usu have oss of lung parenchyma but NOT FIBROSIS |
|
|
Term
|
Definition
| whole acinus, from respiratory bronchioles to distal alveoli. lower lobes and younger women most affected |
|
|
Term
| balance of normal alveoli need: |
|
Definition
alpha 1 anti trypin made in LIVER, anti protease.
protease produced bc activation of neutrophils but the antiproteases destroy them before they destroy lung elastin fibers in alveolar wall.
anti protease in lung not acting on elastinfiber |
|
|
Term
| genetic dx that can get u emphysema |
|
Definition
| point mutation that wont let anti trypsin out of liver into the blood. just a simple test then can fix but need to get the test. |
|
|
Term
| SOD/GTH in lung get desroyed by |
|
Definition
| free radials from cigarette smoke then once destroyed will get unchecked elastase and emphysema |
|
|
Term
|
Definition
• Persistent and productive cough:
– 3 months a year for 2 consecutive years.
excessive mucuous secretion in the bronchial tree. |
|
|
Term
|
Definition
ratio of gland usu .4 or less. in chron bronch usu 0.5 or more.
mucous gland hypertrophy in chron bron person make it bigger.
goblet cell number INCREASE so its golbet cell hypertrophy.
eventually squamous cell metaplasia bc losing ability to fight infection. |
|
|
Term
|
Definition
| secrete a proteinaceous fluid that lubricates the bronchiolar mucosa. |
|
|
Term
| mucous secreting cells in the treachea |
|
Definition
|
|
Term
|
Definition
| perforate the walls of the alveoli and permit the passage of bacteria and flui between adjacent alveoli |
|
|
Term
|
Definition
| induced by goblet cell metaplasia with mucus plugging of the bronchiolar lumen, inflammation and bronchiolar wall fibrosis |
|
|
Term
|
Definition
|
|
Term
|
Definition
"extrinsic" or "atopic" and are due to IgE and Th2-mediated resonse to environment antigens.
occurs mostly in childhood. |
|
|
Term
|
Definition
non-neoplastic rxn to inhaled dust
silicosis is most prevalent chronic occupational dx in the world. fibrogenic resp by macrophages to produce nodular foci of collagen depostiion |
|
|
Term
|
Definition
| virulent, mostly in adults, no predisposing diseases, alveolar consolidation and its uncommon |
|
|
Term
|
Definition
| less virulent, influenza/bronchitis immunosuppresion, very young, elderly, bronchial inflamm, alveolar consolid and its also VERY common |
|
|
Term
|
Definition
| 1-2 days. first phase of lobar pneumonia. develops due to acute inflamm response in the alveoli of the lung. lobe becomes red/frothy and blood stained fluid canbe squeezed from it. large numbers of causative bacteria can be found. onset is sudden with fever and chills. |
|
|
Term
|
Definition
| 2-4 days, second stages of lobar pneumonia. lobe is solidified (liver-like) by confluent, dense infiltrate of neutrophils, fibrin in alveolar spaces and extravasated RBCs. clinically there is pain on breathing due to pleural exudates and productive cough. |
|
|
Term
|
Definition
| 4-8 days the affected lobe is more solid, with residual fibrin, but WBC and RBC disintegrating and being resorbed. Abs to bacteria appear in the blood and organisms disappear uite quickly. coughing is not so marked and less productive. pain and high fever persist. |
|
|
Term
|
Definition
| with elimination of bacteria the inflammatory process subsides and since there is no tissue destruction the lung returns to norm. enz digestion of exudate, resorbed, ingested by macrophages and coughed up. dx ends in a "crisis" with sudden drop in temp. |
|
|
Term
| main predisposing conditions to bronchopneumonia are |
|
Definition
acute bronchitis
chronic bronchitis and bronchiectasis
cancer
immunesuppression |
|
|
Term
| causes of viral pneumonia |
|
Definition
| influenza, adenovirus, CMV, respratory syncytial virus (children, RSV) |
|
|
Term
| what causes the development of caseating necrosis in the tubercles? |
|
Definition
| the wax D component of the cell wall. cann lead to extensive and progressive destruction of lung parenchyma. thus as it persists can destroy more and moreof lung and eventually kill the patient. |
|
|
Term
| primary pulmonary hypertension |
|
Definition
| idiopathic, heart disease, mitral stenosis |
|
|
Term
| secondary pulmonary hypertension |
|
Definition
| thromboembolism, lung fibrosis, emphysema |
|
|
Term
| what is the most common lung tumor |
|
Definition
95% are derived from bronchial epithelium (carcinomas)
5% others including metastatic |
|
|
Term
| what are the 3 types of non small cell lung cancer? |
|
Definition
| squamous cell, adenocarcinoma, and large cell carcinoma |
|
|
Term
|
Definition
|
|
Term
| bronchioloalveolar cell carcinoma |
|
Definition
subcategory of adenocarcinoma. usu peripheral in location. tumor grows along alveolar walls rather than actually forming glands.
bc of this unusual growth pattern the tumor often presents as a pneumonia-like infiltrate on chest x-ray and may be misdiagnosed! |
|
|
Term
|
Definition
tobacco related. group of neoplasm that lack cytological differentiation and prob rep squamous cell or glandular neoplam that is too undiff to categorize.
(variants are giant cell carcinoma, clearcell carcinoma and spindle cell carcinoma) |
|
|
Term
| polycystic ovary syndrome |
|
Definition
| common cause dysmenorrhea and infertility |
|
|
Term
| squamous cell carcinoma of the lung |
|
Definition
| can cause SIADH bc releases inappropriate ADH. also can produce PTH |
|
|
Term
| primary hyperparathyroidism |
|
Definition
| will have high Ca and high PTH, unrelated. |
|
|
Term
| what is the most common cause of secondary hyperparathyroidism |
|
Definition
|
|
Term
| afferent arterioles blood go to |
|
Definition
| capillary bed of glomeruli |
|
|
Term
|
Definition
get blood from capillies
and vascularize medulla |
|
|
Term
| what makes up the slit diaphagm |
|
Definition
| visceral epithelial cell which form the barrier. |
|
|
Term
| juxtaglomerular cell do and are made of |
|
Definition
they sense GFR and secrete renin
made of macula densa and lacis cell (like mesangial, bounded by mac dense, aff arteriole and glomerulus) |
|
|
Term
|
Definition
| gross hematuria, mild has mod proteinuria, and hypertension |
|
|
Term
|
Definition
| VERY HIGH proteinuruia (>3.5mg/dl), hypoalbuminemia, sev edema, hyperlipidemia and lipiduria |
|
|
Term
|
Definition
most common glomerular dx world wide
berger
dx
microscopic or gross hematuria
trap IgA in mesangium and activation of alt complement activation
onset after upper resp inf or fastro int inf
genetic and env presdisposition |
|
|
Term
| can cysts be acquired from dialysis? |
|
Definition
|
|
Term
| clear cell carcinoma usu have mutation in |
|
Definition
| VHL gene locus, 85% have spon mutation in tumor |
|
|
Term
| secondary hyperthyroidism |
|
Definition
most common caus eis renal failure, dec serum ca2_ stim pth gland
-bone changes
-brown tumors, intraosseous masses of osteoclasts, giant cells, hemorrhagic depbris
-may cause nephrolithiasis |
|
|
Term
|
Definition
| obstructive but NOT chronic since it is reversible |
|
|
Term
| il8 and tnf in the lung injure |
|
Definition
type 1 alveolar cells in IPF and chronic restrictive dx.
the pulm macrophages cause these problems. they neutrophils |
|
|
Term
| auto dom polycystic kiney dx (ADPKD) |
|
Definition
multiple expanding cysts
-destry kidney parenchyma
-2 genes pkd1 and pkd2)
-onset as adult
-enlarged kidneys
clear turbid cysts
-cysts an induce ischemia from increasing pressure
FLANK PAIN
-berry aneurysms |
|
|
Term
| auto recess polycystic kidney dx |
|
Definition
in children
-genetic, PKHD1
-small cysts gives kidney sponge like appearance
-infantile onset has poor prog
-survive infancy- get liver cirrhosis |
|
|
Term
|
Definition
assoc with dialysis
-multiple small cysts
-norm in size, sponge like appearance
-likely due to obstruction of tubules by interstitial fibrosis or oxalate crystals |
|
|
Term
|
Definition
see inc levels of ca oxalate, ca phosphate, mg ammonium phos in the urine
-inc uric acid levels (gout,lekemia)
-alt urine pH
alt amino acid transport
-lacks inhibitors of mineral precipitation (mucopolysacc, disphosphonates, nephrocalcin) |
|
|
Term
| 3 kinds of renal carcinoma tumors |
|
Definition
| clear cell carcinoma, papillary carcinoma and chromophobe renal carcinoma |
|
|
Term
| renal cell carcinoma is usu |
|
Definition
adenocarinoma from tubular epithelim
-old males
-VHL syndrome usu develop RCC
usu large at disovery 3-15cm
-yellow, gray,white
well def margins but small processes project
-may fungate into pelvis, ureters or renal vein |
|
|
Term
| acute nephritic syndrome is assoc with |
|
Definition
post-streptoccocal nephritis
-hematuria, azotemia, variable proteinuria, oliguria, edema and hypertension |
|
|
Term
| rapidly progressing glomerulonephritis is assoc with |
|
Definition
idiopathic, goodpastures
acute nephritis, proteinuria, and acute renal failure |
|
|
Term
| chronic renal failure is assoc with |
|
Definition
IgA neuropathy
azotemia-->uremia prgressing for yrs |
|
|
Term
| asymptomatic hematuria and proteinuria is assoc with |
|
Definition
chronic glomerulonephritis
-glomerular hematuria and subnephritic proteinuria |
|
|
Term
|
Definition
MCD and membranous glomerulopathy
>3.5gm proteinuria, hypoalbuminemia, hyperlipiemia and lipiduria |
|
|
Term
| what is increased in the serum in post strep nephritis? |
|
Definition
cryoglobulins
(also dec C3 in complement components and inc anti-strep antibodies) |
|
|
Term
| rapidly progressing glomerulonephritis types |
|
Definition
type 1- idiopathic, good pasture (anti-GBM)
type 2- idiopathic and post-infectious
type 3- idiopathic and wegener's granulomatosis (pauci immuno and antineutrophil Abs) |
|
|
Term
type 1 RPGN has what IF patter?
how to treat type 1? |
|
Definition
|
|
Term
type 2 RPGN has what kind of IF?
how to treat type 2? |
|
Definition
lumpy bumpy
steroids and cytotoxic agents |
|
|
Term
| acute pyelonephritis assoc with |
|
Definition
UTI
focal abscesses, neutrophil infil and pus in tubules |
|
|
Term
|
Definition
dilation of renal pelvis and calyces, atrophy due to obstruction of urine flow
tub defects first than glomerular defects
leads to renal failure and uremia |
|
|
Term
| diabetes is the main cause for |
|
Definition
| end stage renal dx, , adult-onset blindness, and nontraumatic extremity amputations in the US |
|
|
Term
| diagnosis of diabtes mell requires these glc levels: |
|
Definition
>125mg/dL fasting
or
random >200mg/dL |
|
|
Term
| secondary diabetes mellitus can be caused by |
|
Definition
destruction of pancreatic tissue (eg chronic pancreatitis, surgery and cystic fibrois)
drug induced interfere eg corticosteroids, some diuretics
endocrinopathis that interfere with insulin effect eg acromegaly, cushing syndrome
CMV
genetics like down syndrome |
|
|
Term
| MHC II HLA genes assoc with type 1 DM |
|
Definition
|
|
Term
| hyperosmolar nonketotic coma |
|
Definition
elderly type 2 DM who cannot maintain water intake
-sev dehydration
-50% mortality |
|
|
Term
| early sign of DM kidney injury is |
|
Definition
|
|
Term
| leading cause of death in diabetics |
|
Definition
| DM nephropathy following MI |
|
|
Term
| symmetric peripheral neuropathy |
|
Definition
seen in diabetes
-tingling and numbess of extremitis |
|
|
Term
|
Definition
seen in diabetics
-low BP, bowel and bladder dysfunction and impotence |
|
|
Term
| anterior pituitary gland blood supply from |
|
Definition
| superior hypophyseal artery and hypothalamus via a portal venous system |
|
|
Term
| blood supply for posterior pit gland |
|
Definition
| inferior hypophyseal artery |
|
|
Term
| which hormones are acidophils? |
|
Definition
|
|
Term
| which hormones are basophils? |
|
Definition
|
|
Term
|
Definition
destruction of pit from trauma, infarction, infection, granulomatous dxx, iatrogenic or neoplastc causes. lead
leads to weakness and hair loss |
|
|
Term
|
Definition
panhypopituitarism due to peripartum shock-induced nec of the pit gland
amenorrhea, no milk |
|
|
Term
|
Definition
| atrophic pituitary gives empty appearance to sella on imaging, rarely causes clin pit hypofunction |
|
|
Term
| anterior pit hyperfunctions are usu due to |
|
Definition
|
|
Term
| amenorrhea-galactorrhea syndrome |
|
Definition
| due to prolactinoma, often asymptomatic in males |
|
|
Term
|
Definition
| due to GH adenoma before growth plates in the long bones ossify, greater than 7 ft tall |
|
|
Term
|
Definition
| due to GH adenoma after epiphyses ossify. prognathism, separation of teeth, enlarged hands and feet, DM, arthritis |
|
|
Term
|
Definition
| ACTH-producing adenoma giving rise to cushing's syndrome |
|
|
Term
| posterior pituitary hypofunction and hyperfunction |
|
Definition
diabetes insipidus is hypo
syndrome of inapp ADH secretion is hyper |
|
|
Term
|
Definition
nephrogenic DI has inc ADH and the renal tubules are just insens to ADH
in central something causes loss of ADH secretion |
|
|
Term
|
Definition
hyponetremia leading to cerebral edema with confusion, coma and death
cause usu ectopic ADH production from small cell cancer of lung
tx by restricting water, diuresis and tx cause |
|
|
Term
| how are where are T3 and T4 stored? |
|
Definition
| both stored as thyroglobulin in the thyroid follicles |
|
|
Term
| ectopic sites for thyroid |
|
Definition
| lingual, thyroglossal duct, retrosternal, ovary |
|
|
Term
| best marker for hypothyroidism |
|
Definition
|
|
Term
|
Definition
| enlarged epithelial cells with abundant eosinophilic granular cytoplasm as a result of altered mitochondria.[2] They generally stain pink and are prominently found in histological sections of thyroid glands affected with Hashimoto's. |
|
|
Term
| familial graves dx assoc with what genes |
|
Definition
|
|
Term
|
Definition
diffuse symmetric goiter involutes to form colloid nodules and multinodular goiter
diff from hasimotos thyroiditis where enlargement is due to inflammation and theres autoantibodies |
|
|
Term
| primary cause for hypoparathyroidism and hyperparathyroidism |
|
Definition
surgery
hyper- parathyroid adenoma, parathyroid hyperplasia |
|
|
Term
| common cause for secondary hyperparathyroidism |
|
Definition
| chronic renal failure will cause inc prod of PTH cause theres low serum Ca |
|
|
Term
| tertiary hyperparathyroidism |
|
Definition
| rare- dev of autonomous PTH production in pt with secondary |
|
|
Term
| drugs that can cause hypercalcemia |
|
Definition
| thiazide diuretics, lithium, vit a+d toxicity, milk-alkali syndrome, estrogens |
|
|
Term
| waterhouse-friderichsen syndrome |
|
Definition
adrenal hemorrhage with fulminany septicemia due to meisseria meningitidis with shock, fever and petechial-purpuric rash. high mortality
cause acute insufficiency of the adrenal cortex |
|
|
Term
|
Definition
primary insufficiency of adrenal cortex.
common causes are autoimmno adrenalitis and TC
have weakness, anorexia, nausea, vomiting, weight loss, diarrhea, hypotension, and hyperpigmentation
(no inc in skin pig if secondary insuff) |
|
|
Term
| hyperaldosteronism (conn's syndrome) |
|
Definition
excessive aldosterone causes sodium retention and potassium excretion, leading to hypertension and hypokalemia
primary is caused by aldosterone-producing adrenal corical adenoma and sometimes by cortical hyperplasia and is curable cause of hypertension with low serum renin
secondary die to increased levels of plasma renin as with CHF and dec renal perfusion |
|
|
Term
| 21-hydroxylase deficiency causes |
|
Definition
95% of cases of adrenogenital syndromes that have defects in enz of corticosteroid synthesis which leads to dec cortisol synthesis.
causes precocious virilism in males and masculinization/pseudohermaphroditism in females. |
|
|
Term
|
Definition
tumor of adrenal medullary chromaffin cells produces catecholamies. can be lethal.
10% tumor- fam, bilat, extra-adren (paragangliomas) and 10% malignant
sus hypertension, paroxysmal hypertensive crisis
10-20% have MEN 2 or 3
-asymp or diagnosed at autopsy |
|
|
Term
|
Definition
most common extracranial tumor in kids, rare in adults
usu in adrenal glands and symp ganglia
diag when find mass in young pt
urine catecholamines and metabs usu high
prognosis better w younger pts and local dx |
|
|
Term
|
Definition
MEN 2a
hyperplasia in parathyroid, pheochromocytoma in adrenal and meduallary carcinoma of thyroid |
|
|
Term
|
Definition
| adenomas in pituitary, hyperplasia and adenomas in parathyroid, hyperplasia and tumors in islets, and corticalhyperplasia in adrenal |
|
|
Term
|
Definition
| pheochromocytoma in adrenal, medullary carcinoma of thyroid and extra endocrine there is marfanoid habitus and mucocutaneous ganglioneuromas |
|
|
Term
| increaed hemolysis causes |
|
Definition
| hemosiderinyria, jaundice, hemoglobinemia, methemalbuminemia and hemoglobinuria |
|
|
Term
| what happens in the marrow when theres increased hemolysis? |
|
Definition
| increased number of normoblasts, with pressure atrophy of inner cortical bone and new bone formation on the outside, esp in rib and facial bones |
|
|
Term
| chronicity of increased hemolysis will cause |
|
Definition
| gallstones, hemosiderosis and extramedullary hematopoiesis |
|
|
Term
|
Definition
mutation of glutamine to valine at amino acid 6 of the beta chain
8% of african americans are carriers - protect again malaria
deoxy-hemoglobin S crystallizes leading to hemolysis (anemia) and obstruction of small vessels (vaso-occlusive crisis).
ini the sickle cells is reversible but after membrane damages occurs theres scarring which makes it irreversible
the spleen is initially enlarged then focal scarring with ca and hemosiderin deposits get functional autosplenectomy predisposes to infections.
prenatal diagnosis with RFLP |
|
|
Term
|
Definition
mutation of glutamine to valine at amino acid 6 of the beta chain
8% of african americans are carriers - protect again malaria
deoxy-hemoglobin S crystallizes leading to hemolysis (anemia) and obstruction of small vessels (vaso-occlusive crisis).
ini the sickle cells is reversible but after membrane damages occurs theres scarring which makes it irreversible
the spleen is initially enlarged then focal scarring with ca and hemosiderin deposits get functional autosplenectomy predisposes to infections.
prenatal diagnosis with RFLP |
|
|
Term
|
Definition
normochromic, normocytic anemia, usu w neutropenia and thrombocytopenia
marrow is hypocellular, replaced by fat and fibrous stroma, 50% cases idiopathic
other causes: fanconis anemia (auto recess other assoc findings: renal hyperplasia, hypoplastic thumbs or radii, hyperpigmentation of skin, microcephaly), whole body irridiation, dose-related or idiosyncratic drug reactions, postinfectious
can also see in tumors metastatic to the marrow (myelophthisic anemia), diffuse liver dx, chronic renal failure and endocrine disorders |
|
|
Term
|
Definition
most common nutritional disorder in the world
hypochromic, microcytic anemia
inc enzymatic activity in bone marrow and sideroblasts and stainable iron absent
iron depleted from enzymes leads to oxidation of membrane proteins and decreased plasticity of RBCs -->pokilocytosis |
|
|
Term
|
Definition
in iron-deficiency anemia
microcytic hypochromic anemia, atrophic glossitis and esophageal webs. |
|
|
Term
|
Definition
megaloblastic anemia
b12 malabsorption from genetic defect or chronic atrophic gastritis with loss of paritel cells, resulting in dec intrinic factor activity. |
|
|
Term
| 3 autoantibodies assoc with pernicious anemia |
|
Definition
Ab that blocks binding of intrinsic factor to B12
Ab that binds to IF and IF-b12 complex
Ab that binds to parietal cell brush border |
|
|
Term
| where do you see myelin degeneration in pernicious anemia? |
|
Definition
| dorsal and lateral tracts of spinal cord |
|
|
Term
| folate deficincy is the same as pernicious anemia except you dont see the |
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Definition
|
|
Term
where do u find schizocytes (helmet cells, burr cells)?
which kind of anemia assoc with? |
|
Definition
conditions due to mechanical destruction of rbc's eg:
-prosthetic cardiac ball valves
-advanced atherosclerosis
-small vessel thrombi- DIC
-hemolytic uremic syndrome
-malignant hypertension
-thrombocytic thrombocytopenic purpura
-ulcerative colitis
assoc with microangiopathic anemia (mechanical hemolysis) |
|
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Term
|
Definition
caused by defect in either alpha or beta chain. b thalassemias usu caused by point mutations and alpha caused by gene deletions.
anemia bc less hemoglobin produced |
|
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Term
|
Definition
glutathione (GSH) prevents injury by exogenous oxidants
g6pd is enz in hexose monophosphate shunt that produces GSH
hemoglobin oxidizes to methhemoglobin and precipitates in the cytoplasm, clumps (heinz bodies) attach to cell membrane and increase osmotic fragility- the heinz bodies are removed in spleen dec size of RBCs and tend to form spherocytes |
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Term
|
Definition
mutant enz has shorter life than RBC hemolytic crisis affects older cells.
present in 6-10% african americans |
|
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Term
|
Definition
| enzymatic has lower activity throughout life- hemolytic crisis can be diastrous |
|
|
Term
| what can trigger hemolytic crisis? |
|
Definition
| antimalarials (primaquine and quinacrine |
|
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Term
|
Definition
does not fix complement
polyclonal IgG, active at 37 deg celsius.
primary or secondary to lymphoma. neoplasm, drugs, SLE
RBC sequestered in SPLEEN leading to splenomegaly
hemolysis is EXTRAvascular, usu in spleen |
|
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Term
|
Definition
fixes complement
monoclonal IgM, agglutinate in cooler prts of body
acute when Ab titers rise following mycoplasma pneumonia or mononucleosis or chronic (lymphoma)
RBCs seq in the LIVER
massive INTRAvascular hemolysis, complement dependent which follows from exposure to cold
if complement not suff to make MAC attack then theres extravascular hemolysis in the spleen
also IgG autoantibodies again P-blood group antigens, bind complement at low temperatures
occurs after infections (mycoplasma, measles, mumps) |
|
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Term
|
Definition
most common leukemia in adults >60
->15klymphocytes with 10%blasts
4015k early low count CLL, subleukemic CLL
usu B cell with weak sig, rare cases of T cell
generalized lymadenopathy, splenomegaly, hepatomegaly
-homogenous population of small mature lymphocytes |
|
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Term
|
Definition
most common leukemia in 15-59yrs
-azurophilic granules are modified primary lysosomes
-meyeloperoxidase stain distinguys myeloblasts from lymphoblasts
-esterases (NCE) specific for myeloids, ANE specific specific for monocytes
-AUER RODS
-retinoic acid can induce differentiation to various degrees
-presence of philly chrom is bad sign- only is 3% |
|
|
Term
| chronic myelocytic leukemia |
|
Definition
assoc with philly chrom t(9:22) forming a fusion BCR/ABL
if NO phil chrom then worse prognosis.
>100k wbcs
-huge spleens
-mostly neutrophils w scattered myelocytes and promyelocytes, inc # of basophils typical
-dec alkaline phosphate activity
may dev myelofibrosis late in course
-usu blast crisis and death |
|
|
Term
| secondary lymphoid organs and tissue |
|
Definition
| waldeyer's ring, bronchus- associated lymphoid tissue, lymph nodes, spleen, lamina propria, peyers patches, urogenital lymphoid tissue |
|
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Term
|
Definition
most common form of lymphoma
-resembles normal germinal center B cells.
-often painless lymphadenopath
-peripheral lymphoid neoplasms (mature)
classified according to predom pop of cells
grade 1- small cleaved cell
grade 2-mixed
grade 3- large
recent therapy using anti CD20 Ab success in some
t(14:18) causes bcl2 overamplication which prevents apop |
|
|
Term
| diffuse large B-cell lymphoma |
|
Definition
usu 60yrsold
5% of childhood lymphomas
cells large in size (4x small lymphocytes) w diffuse growth pattern
express CD20 usu
-bcl2 positive in 30%, 20% have translocation in bcl-6 on chrom 3
complete remission is 60-80% of pts and 50% remain dx free for several yrs
bcl-6 seems to have better prognosis |
|
|
Term
|
Definition
african (endemic)- latently inf w EBV
sporadic (non endemic)
hIV assoc 25% are EBV +
tl of c-myc common |
|
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Term
|
Definition
CRAB (ca, renal failure, anemia, bone lesions)
presence fo monoclonal plasma cell proliferation involving bone marrow
-prolif of plasma cells dep on cytokines esp IL6
-HHV8 etiology is controversial
-most freq karyotypic abnormal is deletion of 13q and rearrange 13q
BJP cause ren fail, M protein, high IgA or G
surv usu only 3 yrs |
|
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Term
|
Definition
monoclonal gammopathy of undetermined signif
-no MM. 20$ will develop plasma cell dyscrasia in 10-15yrs |
|
|
Term
| lymphoplasmacytic lymphoma (Waldenstrom macrogloulinemia) |
|
Definition
monoclonal IgM elecation causing hyperviscosity syndrome
visual impairment, neurologic symptoms, bleeding, cryoglobulinanemia
bone marrow shows inc plasmacytic cells |
|
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Term
|
Definition
3% of NHL in US
-tumor cells resemble lymphocytes ion mantle zone of norm germinal centers
-usu tl of t(11;14) causes overexp of cyclin D1 |
|
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Term
|
Definition
|
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Term
|
Definition
arise from lymphoid tiss in mucosa-assoc lymphoid tissues eg stomach, intestine and SALIVARY GLAND
-low grade MALToma in stomach comm assoc with H pylori and tx target against organism cures 2/3 of patients |
|
|
Term
| peripheral T cell lymphoma (PTCL) and natural killer cell neoplasms |
|
Definition
15% NHL in US but more common in asia
-no speciic histo,
-mixed pop of small and large cells or uniform prolif or diffuse large cells
-but generally appear to have increased vascularity
-use immmunophenotypic and genotypic analysis to diagnose |
|
|
Term
| adult t-cell leukemia lymphoma |
|
Definition
very rare in US
-assoc with HTLV-1
-floret-like cells in periphral blood
-skin lesions, hepatosplenomeagly, lymphocytosis and hyperalcemia |
|
|
Term
| mycosis fungoides/Sezry syndrome |
|
Definition
dx involving skin
-cutaneous T-cell lymphoid neoplasm
-mycosis fungoides term used for tumor involving only skin.
-neoplastic lymphocytes CD4+ tend to infiltrate epidermis (epidermotropism)
*if have lymphoma cells (leumic cells) dx referred to as SEZRY syndrome |
|
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Term
|
Definition
most common malig in young adult and has a bimodal age involvement
histo- RS cells, and reactive lymphocytes histiocys and granulocytes make a minor fraction 1-5% of total tumor mass. |
|
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Term
|
Definition
found in cervical esophagus
-composed of a variety of glandular types of mucosa
-no bio sinig |
|
|
Term
| etiologies of esoophagitis |
|
Definition
| reflux, pill (eg Fe), allergy, crohns?, viral, trauma, radiation, pemphigus, pemphigoid, vasculitis, fungal, GVH |
|
|
Term
|
Definition
| assoc with reflux allergy, dysphagia, endoscopy, |
|
|
Term
|
Definition
| difficulty swallowing solid not liquids |
|
|
Term
| is the risk for cancer more in short or long segemtn |
|
Definition
|
|
Term
| is SCC assoc with reflux? |
|
Definition
NO
asso with cigs and ROH.
in mid esoph
dec relative to adenocarcinoma |
|
|
Term
| neuroendocrine cells in the fastric cardia? |
|
Definition
| seratonin cells- role in control of lower esoph sphincter |
|
|
Term
| 3 functions of small bowel |
|
Definition
absorption of nutrients
motility
brrier/immune system |
|
|
Term
| which area of colon is most sensitive to ischemic injury? |
|
Definition
mucosal compartment is most sensitive
the ulcerations can be repaired by reepithelialization |
|
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Term
|
Definition
last part of the large bowel just before the rectum (the sigmoid shaped sigmoid colon) twists on its self.
It is by far the most common type of volvulus, accounting for 75 to 90 % of all volvulus. |
|
|
Term
|
Definition
anywhere in the GI tract
-transmural inflammation (fistulae, strictures)
-extra intestinal manisfestations
-carcinoma risk increased in inflammed area
-microscopically u see granulomas, fibrosis, cryptitis and knife ulceration |
|
|
Term
|
Definition
colon only- foreshortening
-distal to proximal
-mucousal centered
-risk of carcinoma and primary sclerosing cholangitis
-microscopic u see: crypt distortion, muscularis mucosa thichening, cryptits/expan of lam pro |
|
|
Term
| main principle in crypt abscess |
|
Definition
breach in epithelial interity
(infection, ischemia, BD, trauma) |
|
|
Term
|
Definition
malabsorbtion/diarrhea/iron def anemia
-microscopic of small bowel biopsy
villous atrophy, crypt hyperplasia, intraepithelial lymphocytosis
anti-gliadin antibodies
responsed to gluten free diet |
|
|
Term
| are colonic diverticuli usu true divertici? |
|
Definition
| NO - do not have all layers of cell wall |
|
|
Term
| most common tumor of colon and small bowel |
|
Definition
|
|
Term
| familial adenomatous polyposis |
|
Definition
germ line mutation in APC gene
-knudson hit hypothesis
-inc risk of small bowel adenocarcinomas
-gastric fundic gland polyps, some get dysplasia but NO inc rik of gastric cancer
-extra-intestinal manifestations: desmoid, tumors, osteomas, CNS (turcots syndrome) |
|
|
Term
| can low grade dysplasia go to invasion without going thru high grade dysplasia? |
|
Definition
|
|
Term
|
Definition
not dysplastic
-was mucous retention polyp
-SMAD4 mutation
-single JP polyp has no malig potential |
|
|
Term
|
Definition
multiple juvenile polyps
-def inc risk of cancer perhaps 10%
VERY RARE
-auto dom with variable penetrance, SMAD4 |
|
|
Term
| inflammatory and filiform polyps |
|
Definition
not dysplastic
-no cysts as in a JP
-results of repair from inflammation (eg ul colitis and crohn's), may occur sporadically |
|
|
Term
|
Definition
auto dom variably penetrant
-STK11/LKB1 serine/threonine kinase
-mucocutaneous melanin ppigmentation
-hamatomatous polyps may occur in entire GI tract
-inc risk of cancer
-RARE |
|
|
Term
|
Definition
| DNA mismatch repair proteins that are usu messed up in HNPCC |
|
|
Term
|
Definition
10-15% all colon cancers
-germ line mutation in DNA mismatch repair genes
-auto dom |
|
|
Term
| MSI (microsatallite instability) |
|
Definition
| 10% of sporadic colon cancers will have MSI with NO germ line mutation in MLH1 or MSH2 |
|
|
Term
|
Definition
epithelial tumors derived from endocrine cells in the mucosa
-bio behavior dependent upon site: appendix good, small bowel NOT so much
-definite malig potential
-may present as polyps |
|
|
Term
| pseudomyxomatous peritonei |
|
Definition
if the mucin has CELLS- bad prognosis
if no cells- good prognosis |
|
|
Term
| pancreatic ductal adenocarcinoma |
|
Definition
most common malignant pancreatic tumor
-most occur in pancreatic head
-painless jaundice
-whipple operative procedure
-bad prognosis |
|
|
Term
| mucinous cystic neoplasms |
|
Definition
multicystic complexity
-dysplastic, mucinous, epithelial lining
-def malignant potential
-prognosis dictated by presence or absence of invasion |
|
|
Term
| solid cystic pseudopapillary tumor |
|
Definition
-female predominant
-reproductive ae
-little to no malignant potential
-epithelial and endocrine differentiation
- |
|
|
Term
| serous microcystic adenoma |
|
Definition
| benign tumor, rare, VHL, female, 66yrs, body and tail, epithelial tumor |
|
|
Term
|
Definition
exocrine tumor, rare, oler adults, def MALIG POT, etiology unkown,
**do NOT has KRAS and p53 mutations |
|
|
Term
|
Definition
removes heme and aids in cholesterol homeostasis
helps in lipid absorbtion from the small bowel |
|
|
Term
|
Definition
RNA virus
not incorp into genome
85% of ppl infected go on to chronic hepatitis
cirrhosis may follow
Ab are relatively ineffective |
|
|
Term
|
Definition
DNA virus
chronic hep may occur
cirrhosis may then follow
Abs effective
hepatitis, ground glass hepatocytes |
|
|
Term
|
Definition
RNa virus
fecal, oral spread
ACUTE HEP ONLY
Abs effective
hepatitic, lobular disarray, cholestasis, rarely may lead to fulminant necrosis |
|
|
Term
|
Definition
lymphocytes directed to kill hepatocytes
criteria complex
Rx with steroids
may present w acute or chronic picture
may lead to cirrhosis
flares
female |
|
|
Term
|
Definition
metab product is toxic
acute injury only
zone 3 damage due to P450 metab
sosage effect |
|
|
Term
| steatosis and steatohepatisis |
|
Definition
fat in hepatocytes
zone three vs zone one
mechanisms
assoc conditions/etiologies
implications |
|
|
Term
iron injury to liver:
cu:
alpha-1 anti-trypsin: |
|
Definition
hemochromatosis
wilson's dx
biliary cirrhosis |
|
|
Term
|
Definition
| genetic dx of copper metab, may present in a wide variety of ways (chronic, steatohepatitis, acute liver failure and necrosis, psychiatric manifestations) |
|
|
Term
|
Definition
antiprotease important in lung
predominant production in liver
point mutations may lead to problems in secretion from hepatocyte
neonatal jaundice
may lead to cirrhosis with a biliary pattern |
|
|
Term
|
Definition
neonatal dx
ini bilirubin is normal
progressive inc in bili
quickly lead to cirrhosis
surgical tx outscome variable (Kasai) |
|
|
Term
| what happens when gallstones are lodged in the common duct? |
|
Definition
|
|
Term
| what are extrahepatic syndromes? |
|
Definition
| biliary atresia, primary sclerosing cholangitis, and strictiure/stones |
|
|
Term
| what are intra hepatic syndromes? |
|
Definition
| primary biliary cirrhosis, GVHD, chronic rejection, idiopathi adulthood ductopenia, sarcoid/drugs/others |
|
|
Term
| primary biliary cirrhosis |
|
Definition
female
-+AMA
-inflammatory destruction of intrahepatic ducts
-pathogenesis "autoimmune"
-small bile duct disease
"puritis
"LFT pattern |
|
|
Term
| primary sclerosing cholangitis |
|
Definition
large duct dx
assoc w ulcerative colitis
ERCP diagnosis: "beads on a string" |
|
|
Term
| nuc in ameloblastomas and scwnomas are |
|
Definition
|
|
Term
| most common histo appearance of ameloblastoma |
|
Definition
|
|
Term
| plexiform variant of ameloblastoma |
|
Definition
long interconnecting cords and islandsof ameloblastic epithelium
shows epithelium proliferating in a 'cord like fashion', hence the name 'plexiform'. There are layers of cells in between the proliferating epithelium with a well-formed desmosomal junctions, simulating spindle cell layers. |
|
|
Term
| acanthomatous variant of amelobastoma |
|
Definition
| squamous metaplasia of stellate metaplasia |
|
|
Term
| conventional ameloblastomas are treated by |
|
Definition
surgery
with unicystic can be less agressive |
|
|
Term
|
Definition
| females, ant, cross midle, non neoplastic, |
|
|
Term
|
Definition
benign mand>max
multiple ocular lucencies
honeycomb
young adults |
|
|
Term
| compound vs complex odontoma |
|
Definition
compound has multiple mini but clearly recogniz teeth
complex is irreg mass of enamel dentin and cementum
excise and recurrence is rare |
|
|
Term
|
Definition
tooth like strxr
well def,mix RL, RO cons cutterage tx |
|
|
Term
|
Definition
true mixed tumor w neoplastic epi and mesen cells
children
MMR area and over 50% assoc with crown of unerupt teeth
well def or uni or multi RL |
|
|
Term
|
Definition
| can be ery aggressive and high tendency to recur. most req radical surgery w subsequent radiataion tx. its a cancer so pot for metasis. |
|
|
Term
|
Definition
| circular calcification usu found in CEOT |
|
|
Term
|
Definition
glnd like 2nd decade of life f>m snowflake like calcification -round duct like tubular strxr duct-like space is PATHOGNOM
It should be differentially diagnosed from a dentigerous cyst and the main difference is that the radiolucency in case of AOT extends apically beyond the cementoenamel junction. |
|
|
Term
| usu acute renal rfailure due to |
|
Definition
|
|
