Advanced Pathology: Glycoproteins, Cell Membranes, Mitochondria, Chromosomes, and Immunolo, Exams of Pathophysiology

Download Advanced Pathology: Glycoproteins, Cell Membranes, Mitochondria, Chromosomes, and Immunolo and more Exams Pathophysiology in PDF only on Docsity! Advanced Patho Test 1 Glycoproteins - surface markers called antigens, they identify cells as a part of the individuals own tissues Cell membrane - Phospholipid bilayer with proteins and cholesterol Sodium-Potassium pump - The plasma membrane is more soluble to potassium and less soluble to sodium. This makes it easier to sodium to be retained while potassium leaks out of the cells. The pump uses ATP to constantly move these two ions across the plasma membrane. This is called active transport For ever three sodium ions pumped out of the cell two potassium ions are pumped in. In the heart this pump gets calcium out of the internal environment of the heart muscle which relaxes it. * In dysfunction sodium does not adequately leave the cell which draws in water causing cellular swelling Mitochondria - Converts organic nutrients into cell energy in the form of ATP. Anaerobic metabolism - Also called glycolosis, it is done outside the mitochondria. Glucose is used to create energy in this scenario. This produces significantly less cellular energy, only yielding 2 ATP and pyruvic acid. In cellular hypoxia pyruvic acid in converted to lactic acid which is noxious to cells causing muscle pain and biochemical alterations such as acidosis. Aerobic metabolism - Requires oxygen and provides the maximum amount of energy for cellular function (34 ATP). Lysosomes - Contain digestive enzymes such as lipases, proteases, and lysozyme. These enzymes degrade ingested foreign substances and cellular debris. When a cell dies the lysosome releases digestive enzymes to destroy the cell parts in a process called autolysis. When the lysosome is used to destroy foreign matter ingests by macrophage the process is called heterolysis. Proteasomes and peroxisomes - Proteasomes enzymatically degrade polypeptide chains and proteins Peroxisomes break down long chain fatty acids and free radicals. (Malfunction can cause ALS) Disease can occur when these two malfunction. Endoplasmic reticulum - Smooth ER: location for lipid production, which includes corticosteroids, oils, and phospholipids. Rough ER: A tubular network with attached ribosomes that synthesize proteins. Ribosomes - Deranged cellular growth within a specific tissue, often as a result of chronic inflammation or a precancerous condition. These cells will vary in size, shape, and architectural organization compared to healthy cells. Neoplasia - Means new growth and usually refers to disorganized, uncoordinated, uncontrolled proliferative cell growth that is cancerous. Free radical injury - Free radicals are present in cigarette smoke, pesticides, and other toxins. Oxidizing agents with the ability to penetrate the cell's plasma membrane, disrupt internal organelles, and damage the nucleus and its DNA. Free radical generation can overwhelm the mechanism of removal, in which case a form of cell injury known as oxidative stress occurs. A,C, E, and beta-carotene can counteract free radical injury. Aneurysm - Weakened area in an arterial wall ex. hptn can cause rupture of an aneurysm Endothelial injury - ex. hptn exerts a shearing force against the endothelial cell membranes, creating multiple areas of injury on the interior walls of arteries. ex. Hyperglycemia can cause the endothelium to stimulate endothelin a vasoconstrictor that can cause arterial narrowing. Angiotensin II - constant secretion can be a stimulus for arterial narrowing, raising BP and cause worsen heart disease. LDL - Areas of endothelial injury causes inflammation attracting WBC and platelets to the area. This causes diminished vasodilators capacity of the artery and sets up the conditions for the LDL cholesterol deposit and clot formation. During the formation of atherosclerosis nitric oxide is depleted, this inhibits the dilatory capacity of arteries. Gangrene - Occurs when tissues endure prolonged ischemia, infarction, and necrosis. Then they are exposed to bacteria that thrive on the decaying tissue. Gametes - AKA Germ cells, include the sperm and the Ovum Somatic cells - Mutations that arise in the body cells, these mutations are not passed down to off spring and do not cause hereditary diseases. Chromosomes - Forms an "X" and the central point is called a centromere. The upper arm portion of the chromosome is referred to the "P" arm The lower arm portion of the chromosome is referred to as the "Q" arm. There are 23 chromosomes and 22 pairs carry genes related to body traits (somatic cells) The Y chromosomes contain the sex determining region Each chromosomes is in a pair with a corresponding gene known as an allele(inherited from each parent) Karyotype - A picture of 23 pairs of chromosomes in an individual. Can be used to identify someone like fingerprints. Can be assembled from any human cell with a nucleus. Autosomal traits - traits that are not sex linked Genotype - an individual's genetic make-up Phenotype - Genetic traits manifest themselves in an individual. The actual physical or somatic expression of the gene Penetrence - Determined by whether or not the disorder's symptoms are clearly evident. expressivity - Based on the symptoms severity Mitochondrial disorders - During human fertilization the ovum destroys the sperm's mitochondria so all of the mitochondrial DNA comes from the mothers. - infertility - amenorrhea - hptn - aortic valve abnormalities - hypothyroidism - Coarctation of the aorta - visual problems (amblyopia, strabismus, and cataract) Instead of 46 chromosomes people affected by this have a total of 45 chromosomes. Instead of two X chromosomes they only have one. Treatment - estrogen therapy - growth hormone administration PCR - Polymerase Chain reaction - Produces multiple copies of a short segment of DNA and amplifies it for further study. Commonly used for genetic analysis in cancer, forensics, research, and infectious disease detection. Karyotyping - Stains condensed chromosomes of miotic cells with Giemsa dye. It gives an overall picture of an individual's chromosome pairs. Southern Blotting - Molecular genetic testing technique used to detect DNA fragments, which can vary because of various types of mutations. - gene insertions - gene deletions - single nitrogenous base changes - expanded trinucleotide repeats Fluoresence in Situ hybridization - Maps the genetic material in an individuals cells. Does not need miotic cells like karyotyping Can detect numerous chromosomal abnormalities and translocations not detectable by routine karyotyping. Maternal serum screening - Performed on pregnant women's blood to determine if the fetus has an increased risk of having a open neural tube defect, down syndrome, or trisomy 18. Chorionic Villus Sampling - Offered to when a woman is 35 or older at time of delivery because of increased chance of a fetal chromosome problem. It can also be offered if an inherited genetic problem is identified in the family. It is performed between 10-12 weeks of pregnancy under ultrasound. Contraindicated in a pregnant women with vaginal bleeding, uterine fibrosis, retroverted uterus, and those carrying twins. Amniocentesis - Performed on amniotic fluids between 16-18 weeks of pregnancy. Recommended for pregnant women older than 35 years of age. Percutaneous Umbilical Cord blood sampling - Examines blood from the fetal umbilical cord to detect fetal abnormalities. It provides a means of rapid chromosome analysis and is useful when information cannot be obtained by CVA, amniocentesis, or ultrasound. Since the risk is so high it should only be done on pregnancies that are at a high risk for genetic defects. Familial Hypercholesterolemia - Autosomal dominant disorder that causes severe elevations in total cholesterol and LDL. Total cholesterol levels of infants and children with homozygous FH are higher then 600mg/dl In patients with heterozygous FH cholesterol levels are 200-400 mg/dl. Early manifestations: - Xanthomas: deposits of cholesterol under the skin. - Xanthelasma: Specifically occurs around the skin and eye. *Corneal arcos is a light-colored ring that is obvious around the iris of the eye. * AN MI under the age of 55 in a first degree relative is indicative of FH. Familial Adenomatous polyposis - An Autosomal dominant inherited disorder characterized by the early onset of hundreds to thousands of adenomatous polyps throughout the colon. If left untreated individuals develop colon CA by the age of 40. - dissections diagnosis - genetic testing - MRI - CT - echo Cystic fibrosis - Autosomal recessive disease * The CFTR gene locus us 7q31 Pancreatic enzyme insufficiency because of reduced water content that causes mucus plugging of the pancreas's ductules. The respiratory epithelium produces excess amounts of thick mucus that blocks airways and causes a high susceptibility to pulmonary infection for a patient's lifetime Diagnostic tsting - Scren infants for IRT (a pancreatic enzyme) It should be elevated - Sweat test (check for chloride) Not always reliable - CT - abd ultrasound - genetic testing - chest and abd x-ray Treatment - pancreatic enzyme supplements - bronchodilators - mucolytics - nebulizer treatments - antibiotics - anti-inflammatory Tay Sachs disease - A lysosomal storagte disease that results from a mutation on chromosome 15. Prevelant is Ashkenazi jews Ganglioside is not broken down so it causes progressive destruction of neurons and brain cells. The cerebellum, basal ganglia, brainstem, spinal cord, and autonomic nervous system are notably affected. Infants born with this disease appear normal up until 6 months. Motor coordination, lethargy, muscle flaccidity, and increasing cognitive impairment become apparent. * Characteristic diagnostic in this disease is a "cherry red spot" seen on the retina on ophthalmology exam. Death occurs by age 3 Niemann-Pick Disease - Results from an accumulation of sphingomyelin because of the deficiency of the lysosomal enzyme sphingomyelinase. Largely affected by Ashkenzaki Jews Type A: Severe deficiency that causes widespread neurological involvement and visceral accumulation of sphingomyelin. Individuals affected die by age 3. Clinical manifestations become apparent by 6 months - protuberant abd - progressive GI problems - fever - generalized lymphadenopathy - progressive motor dysfunction Type B: Same as type A with no CNS involvement. individuals live into adulthood. Diagnosis - biochemical assay - biopsy of liver or bone marrow Gaucher disease - Autosomal recessive disorder Caused by a mutation in the gene that codes for glucocerebrosidase. Most common in Ashkenazi Jews Type 1: CNS, spleen, skeleton, and WBC are affected. May not be apparent until adulthood. Clinical manifestations - splenomegaly - thrombocytopenia - bone fractures * Treatment is geared toward enzyme replacement and bone marrow transplant. Type II: affects neurons and is apparent in infants. Does not specifically affect Jews. - blood transfusions Klinefelter syndrome - The addition of more than one extra X or Y chromosome to a male karyotype which results in variable physical and cognitive abnormalities. manifestations - lack of development of testes - gynecomastia - skeletal and cardiovascular abnormalities Fragile X syndrome - Martin-Bell syndrome is the most common cause of inherited cognitive impairment. It is a disorder of the X chromosome characterized by long sequences of the three nucleotides C,G, and G. Problems include mild-to-moderate autistic like behavior - hand flapping - avoiding eye contact - attention deficit - hyperactivity - decreasing IQ - dpn affect - shyness Males have a long face with a large mandible. large testicles, and large everted ears. hypermobile joints, scoliosis, and high arched palate are also common. Diagnostic - Southern blot - PCR Prader- Willi Syndrome - "Angelman syndrome" caused by a deletion or disruption of genes in the proximal arm of chromosome 15. Common characteristics - obesity - hypotonia - low IQ - short stature - ataxia gait - seizures - behavioral problems - short stature - hypogonadism - hypogonadotropic - small hands and feet * constant hunger Huntington Disease - Adult onset, autosomal dominant inherited disorder that is associated with degeneration of specific neurons in the basal ganglia and cortex. Clinical presentation - movement disorder - cognitive disorder - behavioral disorder * Chorea is the most common movement disorder seen in HD. mild chorea causes dancelike movements or tics. Severe chorea occurs later and can cause uncontrollable flailing of the extremities termed ballism. Eventually becomes like Parkinson movements (muscle rigidity, postural instability, and slowed up movements). diagnosis can be found through genetic testing Solutes - Deliver nutrients and electrolytes to cells carry away waste products from cellular metabolism Diffusion - Higher to lower concentration electrolytes use diffusion Osmosis - Molecules of a solvent to pass through a semipermeable membrane from a less concentrated solution to a more concentrated one. * Albumin cannot pass through the membrane Facilitated transport - The passing of certain molecules through the plasma membrane with assistance from carrier proteins Ex. glucose in transported into the cell by insulin * Natriuresis is the excretion of a large amount of both sodium and water by the kidneys in response to excess ECF volume. ANP - produced by the artria BNP - produced but he ventricles and the brain CNP - produced by endothelial cells of the arteries and ventricular cells of the heart * BNP and ANP promote natriuresis at the glomerulus by increasing the glomerular filtration rate. Hyponatremia - symptoms - headache - lethargy - apathy - confusion - nausea - diarrhea - muscle cramps Severe hyponatremia can cause seizures, coma, and irreversible neurological damage because of brain swelling. hypernatremia - Clinical manifestations - weight gain - pulmonary edema - dehydration - tachycardia - irritability - mental changes Hypokalemia - causes - NPO - alcoholics - bariatric surgery - eating disorder - burns - albuterol - epinephrine clinical manifestations - N/V - cardiac arrhythmias - muscle fatigue and weakness - leg cramps - prolonged PR, prominent U wave and flattened t wave Hyperkalemia - causes - decreased renal function Clinical manifestations - numbness and tingling of extremities - peaked T waves, wide QRS - bradycardia - mental confusion - apathy Treatment - 75 meq of bicarb, 10 units of insulin, and 50% dextrose infusion - Lasix - calcium gluconate or chloride - kayexelate hypocalcemia - * half of the calcium in the body is bound to albumin so low albumin can give the appearance of low calcium (pseudohypocalcemia). the ionized calcium would show more in contrast to the normal body level of calcium. Less then 8.7 Clincial mainfestations - paresthesia around the mouth, hands, feet - muscle spasms of face and extremities - laryngeal spasm - hypotension * Chronic hypocalcemia causes - bone pain and fragility - dpn - dementia - trousseau's and chovstek's sign Treatment - calcium and vit D Clinical manifestations - chovstek's and trousseau's sign - EKG changes similar to hypokalemia hypermagnesmia - levels greater then 2.5mEq/L Cause - renal dysfunction Clincial manifestations - diminished neuromuscular function - hypotension - arrhythmias * levels greater then 10mg can cause cardiac arrest Treatment - IV calcium - dialysis Acid - donates hydrogen ions in a solution Base - A compound that accepts an H in a solution Alkaline - When basic ions predominate in a solution, the solution is alkaline ABG - PH 7.35-7.45 Pco2 35-45 Po2 90-100 HCO3 22-26 Sao2 95%-100% Anion Gap - represents the concentration of the unmeasured anions in the bloodstream . Usually exists in metabolic acidosis It is defined as the quantity of anions not balanced by the cations in the blood stream normal range is 8 to 16mEq.L * Low serum albumin reduces the accuracy of the anion gap calculation A high anion gap would be a value between 16-20mEq/L Acidosis - In acidosis albumin has a less affinity for calcium so the amount of free ionized calcium is increased, making neurons less excitable. hemoglobin has a less affinity to oxygen Alkalosis - Albumin tightly binds to calcium and the amount of free ionized calcium is reduced. The neurons become more excitable Hemoglobin - heme - composed of iron and porphyrin (metabolized into biliverdin which breaks down into bilirubin). carries carbon dioxide away from tissues composed of four polypeptides that carries 4 oxygen molecules Hgb in infants facilitates oxygen transfer through the placenta and is replaced by 6 months Hemoglobin begins to drop all of its oxygen as PO2 falls below 60mm Hg. / high Co2, fever, increased (2,3) dpg, acidosis causes a lower affinity for oxygen to hemoglobin hemoglobin has a greater affinity for carbon monoxide. normal ranges: women 12-16 Men 13-18 iron - Iron is transported by a protein called transferrin (TIBC) to the bone marrow for erythroposiesis. iron is stored in ferritin complexes that are present in all cells, commonly found in the bone marrow, liver and spleen. Medications that trigger hemolytic anemia - cephalosporins, quinidine, penicillins, levodopa, NSAIDS, and methyldopa IgG class hemolysis cam occur at any temp and is called warm agglutinin syndrome IgM class hemolysis can occur at low temps and is called cold agglutinin syndrome Alloimmune hemolysis: occurs when antibodies are formed against antigens on the RBC surface. ex. transfusion reactions and hemolytic disease of the newborn. Blood smear would show misshapened and damaged RBCs - anisocytosis - spherocytosis - poikilocytosis Sickle Cell anemia - triggers for vaso-occlusive crisis - hypoxia - acidosis - pregnancy - infection - cold Clinical manifestations - priapism - retinal ischemia - hand-foot syndrome -stroke thalassemia - The polypeptide chains of the Hgb structure is deficient, leading to reduced Hgb synthesis and decreased RBCs. Heinz bodies are pathognomonic for thalassemia affects African, Mediterranean, and southeast asia Autosomal recessive disease Beta thalassemia major "aka" cooley's disease is the most severe form Clinical manifestations - bone pain form high amount of erythropoiesis - chipmunk appearance of children - splenomegaly - hepatomegaly - iron overload Hereditary spherocytosis - Cause by a lack of membrane proteins in RBCs. Autosomal dominant disorder Some have no symptoms complications - aplastic anemia crisis - hemolytic anemia - cholelithiasis - cholecystitis treatment - blood transfusions - splenectomy - folic acid treatments Blood transfusion reactions - nonhemolytic febrile reactions and extravascular hemolysis are common in patients who have developed antibodies from prior transfusions. occurs between 1-6 hours after transfusions (fever, malaise, chills). They at thought to stem from the formation of cytokines during the storage of blood. Anaphylactic reactions are most often observed in those with IgA deficiency. symptoms occur in fewer then 10ml (dyspnea, flushing, uticaria, abd pain, hypotension, facial swelling, bronchospasm, edema of tongue) Vit B12 deficiency - Causes - gastric bypass - age - alcoholism - Lack of intrinsic factor (carrier protein of vit B12 in stomach) - proton pump inhibitors - gastric atrophy - autoimmune - h. pylori - celiacs - crohns at risk - COPD - high altitudes - severe heart or lung disease Symptoms - splenic enlargement - orthopnea HgB does not usually go higher then 17 or 18g/dl Treatment - can be reversed when the reason was polycythemia started can be fixed. Acute inflammation reaction - - Vascular permeability - Cellular chemotaxis - systemic response vascular permeability - histamine and bradykinin enable the blood vessels to dilate and become more permeable Cytokines - inflammatory mediators released by WBCs - tumor necrosis factor - interleukins * They modulate the inflammatory process amplifying or deactivating the process. C-reactive protein - * prevents WBC adhesion to endothelium - An elevated CRP indicates active inflammation ESR - A measure of the rate at which RBCs precipitate out of the plasma; it is influenced by fibrinogen levels in the blood. Leukemoid reaction - high elevation in WBC count to 50,000 which can occur in leukemia WBC - normal range 4,100-10,900 neutrophils - first responders, rush to the site in the first 24-48 hours. life span on 10hr-days lymphocytes eosinophils - mainly released during allergic reactions and parasitic infection monocytes - respond after the fist initial 24-48 hours. Matures into macrophages and the primary function if phagocytosis. synthesizes and secretes cytokines basophils * neutrophils, basophils, and eosinophils are referred to as granulocytes. Shift to the left - A high number of bands present. This indicates an increase in newly formed neutrophils. Granuloma - An area where macrophages have aggregated and are transformed into epithelia-like or epithelioid cells. Cell cycle - G0- Cells are resting and not undergoing mitotic division G1- Cells into the cell cycle during this state where they prep for mitosis G2- Cells continue to undergo necessary activities before mitosis S- Cells undergo chromosomal duplication in prep for mitotic division. M- Cell completes mitosis and divides to regenerate itself. Labile cells - Cells that continusouly proceed through the cell cycle. They continue to divide and replicate throughout life. ex. skin, GI cells, GU cells, oral mucosal cells, cancer cells Stable cells - Cells that are in a resting stage until stimulated when they enter the cell cycle. ex. hepatocytes and bone cells Permanent cells - Cannot regenerate and do not enter the cell cycle ex. neurons and cardiac cells Keloid Formation - - night sweats - bone pain (sternum, tibia, femur) - possible CNS involvement Chronic lymphocytic leukemia - A B-cell lymphocyte malignancy risk factors - agent orange used during the Vietnam war B-CLL lymphocytes resemble mature B cells and release low levels of IG or none at all. they contain proto-oncogene bcl 2 which suppresses apoptosis. B-CLL that produces abnormal IG on the surface is called CD38. Patients with greater then 20% of these cells have an unfavorable prognosis * patients with B-CLL cells that are negative for ZAP-70 have a good prognosis then those who don't. S/S - WBC greater then 20,000 - Flow cytometry Treatment - Monocolonal antibodies are effective against B-CLL cells with specific markers like CD38 or ZAP-70 Acute Myelogenous Leukemia - Proliferation of unidentified myeloid cells in the bone marrow. Chronic Myelogenous Leukemia - Clinical Course - Chronic phase: neutrophils begin to lose their differentiations. 10% blast cells - Accelerated Phase: Neutrophils are more undifferentiated and unable to functions. 10-20% blast cells - Blast crisis phase: Myeloid blast cells do not differentiate at all. 20% blast cells Symptoms: - bone pain - hepatomegaly - resp distress (due to pulm accumulation of WBC) - Night sweats - WBC as high as 150,000 at diagnosis Non-Hodgkin's Lymphoma - Etiology - hep C - EBV - H. Pylori Treatment - chemo/radiation Hodgkin Lymphoma - Malignancy of B lymphocytes * Diagnosed by the presence of Reed-Sternberg cells in the lymphoid tissue. Multiple myeloma - A hematologic neoplasm that arises in B lymphocytes, this results in abnormal plasma cells and then abnormal Igs. It is incurable. Three types - Monoclonal gammopathy of undetermined significance (MUGS): benign - Smoldering MM: requires 2-3 month observation - Active MM: results from critical chromosomal translocations in the DNA of affected plasma cells. Translocations of chromosome 4 and 14 or loss of the short arm of chromosome 17 have a poor prognosis as well as The deletion of chromosome 13 is associated with a poor prognosis. Translocations at chromosome 11 and 14 have favorable prognosis. The abnormal Ig and fragments are referred to as M-proteins. MGUS - - Fewer then 10% plasma cells in the bone marrow - the absence of lytic bone lesions, anemia, hypercalcemia, and renal insufficiency. - The presence of a serum monoclonal protein whether IgA, IgG, or IgM at a concentration of 3g/dl or lower. * requires yearly observation Active MM - bone destruction and prevents bone formation renal failure neuro complications serum hypercalcemia back pain Antiphospholipid syndrome - possibly because of autoimmune women have a history of repeated miscarriages thrombosis can be precipitated by trauma, surgical procedures, oral contraceptives, and abrupt with drawl of anticoagulants. PT - normal ranges 10-14 seconds aPTT - normal range 25-40 seconds D-Dimer - measures fibrin degradation products If elevated it indicates that recent clotting activity may be caused by acute or chronic condition, such as PE, DIC, or thromboembolism heparin - activates anti-thrombin the body's natural clot dissolving substance. antidote is protamine sulfate Fondaparinux (arixtra) - factor Xa inhibition sub q injection given once daily prophylaxis thrombosis in general surgical and orthopedic patients Dabigatran (Pradaxa) - direct thrombin factor inhibitor given orally prevents strokes in patients with a-fib that is not caused by valve disorder need no monitoring it is not easily reversed and the effects lasts 24 hours Rivaroxaban (Xarelto) - oral factor Xa inhibitor peeks 4 hours after a dose it is recommended for novalvular a-fib once a day dosing that can last up to 24 hours a day. cannot be easily reversed Warfarin (coumadin) - frequent monitoring of PT and INR peaks 90min after dosing contraindicated in pregnancy reversed by vitamin K drug induced thrombocytopenia - taken drug for 1 week or intermittently for a long period of time. then presents with petechae or pupura symptoms develop within 1-2 days after first exposure to the drug platelet counts decrease to 20,000 or lower Systemic symptoms - lightheadedness - chills - N/V - fever severe systems - bleeding from GI tract - epistaxis - bleeding from gums symptoms usually resolve 2 days after taking causative medication. platelet count returns in a week H.I.P - antibody development to heparin - replacement therapy for missing factor - desmopressin acetate (mild form of HA) Essential Thrombocytosis - Disorder of megakaryocyte proliferation that increases the number of circulating platelets. * complication could be AML Clinical presentation - neuro symptoms (headache, paresthesias of finger and toes) - TIA - IUGR - ischemia of placenta - spontaneous abortions - pulm hptn Diagnosis - bone marrow biopsy - elevated uric acid Treatment - plateletpharesis - lifestyle modifications (weight loss, smoke cessitation) Von Willibrand disease - vWF deficiency causes decreased platelet adhesion and reduced levels of factor VIII, which results in defective clot formation Gene mutation on chromosome 12 clinical presentation - excessive menstrual loss - many are unaware until surgical or dental procedure end up with prolonged bleeding Diagnosis - apTT (usually elevated) - low levels of VIII - vWF antigen in bloodstream Treatment - Desmopressin (stimulates endothelial cells to release vWF and plasminogen activator) - factor VIII products that contain vWF Hemolytic-uremic syndrome - Two forms - Shiga-toxin producing HUS - Non-shiga-toxin producing HUS toxins cause the lysis of RBCs symptoms - gastroenteritis - fever - bloody diarrhea - abd pain - low urine output diagnostic test - UA(reveals proteinuria and hematuria) - BUN/Creatiine is high - stool culture (e-coli 0157:H7) Treatment - maintenance fluid - prophylactic phenytoin * Treatment of non-stx-HUS requires plasma exchange within the first 24 hours of the patients illness and then continued once or twice for at least 2 days after complete recovery. Aspirin as well to inhibit platelet aggregation. DIC - Active formation of fibrin clots with simultaneous depletion of coagulation factors and platelets. Also a suppression of fibrinolysis. The patient unpredictably forms clots and undergoes random periods of bleeding. Clinical conditions that cause DIC - malignant disease - shock - massive trauma - burns - hypoxia - acidosis - fetal death Diagnosis - hemolytic anemia Short-term immunity but immediate. Toxoids - vaccines produced against toxin-producing bacteria. has no disease-producing effects anergy panel - a test of immunocompetence antibody titers - confirms immunity against an antigen by measuring IgM and IgGs. Type I immediate hypersensitivity - Also called an allergy or atopic disorder, it occurs in individuals previously exposed to an antigen. ex. Allergic rhinitis , systemic anaphylaxis Systemic anaphylaxis: hypotension, laryngeal edema, bronchospasm, angioedema, tongue swelling, uticaria. treatment: - antihistamines - glucocorticoids - epinephrine Type II Cytoxic hypersensitivity - ex. blood transfusion recation Type III immune complex hypersensitivity - antigen combines with IG within circulation and these complexes are then deposited in tissues. Type IV delayed hypersensitivity - Initiated by T lymphocytes that have had previous exposure to an antigen. ex. poison ivy, transplant rejection, TB test Systemic Lupus Erthematosus - characterized by remissions and exacerbations with fever, skin rash, joint inflammation, and damage to the kidney. * EBV antibodies increase the risk of SLE Exacerbation can be caused by - excess estrogen - UVA light formation of autoantibodies particularly antinuclear antibodies (anti-dsDNA and anti-Sm) clinical presentation - butterfly rash ont he bridge of the nose - Raynauds phenomenon - splenic enlargement - nephrotic syndrome - pleuritis - pleural effusions - mood disorders Diagnosis - anti-dsDNA and anti-Sm levels - high ANA levels - ESR elevated - CRP elevated Treatment - NSAIDS - antimalarial drugs - methotrexate - immunosuppressive drugs - corticosteroids Rheumatoid arthritis - The immune system attacks it own synovial tissue starting an inflammatory process that results in destruction on cartilage, bone, tendons, and ligaments. B cells secrete an autobody commonly referred to as RF which can be found in the synovial fluid continual inflammation causes hypertrophy Clinical presentation - Symmetrical, tender, swollen joints (fingers, wrists, knees, hips, and feet) - stiff joints in the morning or after period of prolonged rest Diagnosis - Swan neck deformity - Bakers cyst - boutonniere - elevated serum RF, ESR, and CRP - telangiectasia syndrome Clinical manifestations - shiny, smooth, and stretched skin - facial masklike appearance - dysphagia - malabsorption - malignant hptn - abd pain - intestinal obstruction diagnosis - elevation of ANA - CT of lungs (ground glass of honeycomb fibrosis) - esophageal manometry treatment - NSAIDS - corticosteroids - immunosuppressants Polyarteritis Nodosa - Necrotizing inflammation of blood vessels. Vascular lesions resemble those found in type III hypersensitivity reactions Usually involves renal and visceral blood supply. Diagnosis - presence of anti-neutrophil cytoplasmic antibodies. clinical amifestation - hptn - fever - abd pain - arthralgia - peripheral neuritis treatment - immunosuppressants - corticosteroids Sever combined immunodeficiency - syndrome that have both anti-body mediated and cell-mediated immune responses malfunction of T and B cell infants present with many different kinds of infection during the early weeks of life and are diagnosed by age 3. Live vaccines should not be given Treatment - bone marrow transplant without treatment child dies with first 2 years of life IgA deficiency - Those older then 6 months who have recurrent upper and lower resp tract infections with encapsulated bacteria should be evaluated for IgA deficiency Normal levels of IgG and IgM and indetectable levels of IgA clinical manifestations - chronic nasal discharge - dyspnea - cough Chronic Mucocutaneous Candidiasis - Inability of T lymphocyte to respond to challenges of the fungus candida albicans Diagnosis - presence of yeast cells and pseudohyphae Hypogammaglobulinemia - decreased number of Igs related to a defect in B cell development and maturation. can be genetic or acquired acquired etiology - immunosuppressants - renal loss of Igs - b-cell related malignancies - severe burns