NUR 529 Exam 1 Blueprint Fall 2023 PDF

The University of Alabama Capstone College of Nursing NUR 529 Exam 1 Blueprint Pages below are from Porth’s Pathophysiology: Concepts of Altered States, 10th ed., and the current e-book o...

The University of Alabama Capstone College of Nursing NUR 529 Exam 1 Blueprint Pages below are from Porth’s Pathophysiology: Concepts of Altered States, 10th ed., and the current e-book on Coursepoint. Older versions are not included, this course does not utilize older textbook versions. Format for page numbers below is Porth 10th ed. “hard copy” book/ Course Point Porth E-Book. Example: p. 967/975. If the pages correlate between editions then only one will be listed. Unit 1. Chapter 1. There are 2 questions from this unit. Starts on p. 2 1. Concepts of Health and Disease. Vocabulary: pathogenesis, morphology and histology, clinical manifestations, diagnosis, and clinical course. Pathogenesis: explains how the disease process evolves. Sequence of cellular and tissue events that take place from the time of initial contact with an etiologic agent until the ultimate expression of a disease. Morphology: Refers to the fundamental structure or form of cells or tissues. Morphologic changes are concerned with both the gross anatomic and microscopic changes that are characteristic of a disease. Histology: Deals with the study of the cells and extracellular matrix of body tissues. Histologic sections play an important role in the diagnosis of many types of cancers. Clinical Manifestations: Sometimes, the condition produces manifestations, such as fever, that make it evident that the person is sick. Diagnosis: Designation as to the nature or cause of a health problem. The diagnostic process requires a careful history, physical examination, and diagnostic tests. Clinical Course: Describes the evolution of a disease. A disease can have an acute, subacute, or chronic course. Acute disorder is one that is relatively severe, but self-limiting. Chronic disease implies a continuous, long-term process. Subacute disease is an intermediate or between acute and chronic; not as severe as an acute disease and not as prolonged as a chronic disease. 2. Concepts of Health and Disease: Disease. The second question comes from the same content section. A disease is considered an acute or chronic illness that one aquires or is born with that causes physiologic dysfunction in one or more body systems. Each disease generally has specific signs and symptoms that characterize its pathology and identifiable etiology. Unit 2. Chapters 2- 6. There are 16 questions from this unit. Starts on p. 49  3. Chapter 3. Cellular Adaptation: Hyperplasia-physiologic vs pathologic, examples. Hyperplasia refers to an increase in the number of cells in an organ or tissue. It occurs in tissues with cells that are capable of mitotic division, such as the epidermis, intestinal epithelium, and glandular tissue. There are two common types of physiologic hyperplasia: hormonal and compensatory. Hormonal examples include: breast and uterine enlargement during pregnancy. Compensatory examples include: regeneration of liver after partial hepatectomy. Most forms of nonphysiologic hyperplasia are due to excessive hormonal stimulation or the effects of growth factors on target tissues. Example: excessive estrogen production can cause endometrial hyperplasia and abnormal menstrual bleeding; benign prostatic hyperplasia is related to the action of androgens. 4. Chapter 3. Cellular Adaptation: Metaplasia, examples. Metaplasia represents a reversible change in which one adult cell type (epithelial or mesenchymal) is replaced by another adult cell type. Metaplasia is thought to involve the reprogramming of undifferentiated stem cells that are present in the tissue undergoing the metaplastic changes. Usually occurs in response to chronic irritation and inflammation and allows for substitution of cells that are better able to survive under circumstances in which a more fragile cell type might succumb. Examples: adaptive substitution of stratified squamous epithelial cells for the ciliated columnar epithelial cells in the trachea and large airways of a habitual cigarette smoker. 5. Chapter 3. Cellular Adaptation: Dysplasia, examples. Read about Pap smear. Dysplasia is characterized by deranged cell growth of a specific tissue that results in cells that vary in size, shape, and organization. Minor degrees of dysplasia are associated with chronic irritation or inflammation. Dysplasia is strongly implicated as a precursor of cancer. Examples: In cancers of the respiratory tract and uterine cervix, dysplastic changes have been found adjacent to the foci of cancerous transformation. Pap smear: it has been documented that cancer of the uterine cervix develops in a series of incremental epithelial changes ranging from severe dysplasia to invasive cancer. 6. & 7. Chapter 3. Cell Injury and Death: Mechanisms of Cell Injury: Types of necrosis Necrosis refers to cell death in an organ or tissue that is still part of a living organism. Liquefaction Necrosis: occurs when some of the cells die but their catalytic enzymes are not destroyed. Example: softening of the center of an abscess with discharge of its contents. Coagulation necrosis: acidosis develops and denatures the enzymatic and structural proteins of the cell. Characteristic of hypoxic injury and is seen in infarcted areas. Infarction occurs when an artery supplying an organ or part of the body becomes occluded and no other source of blood supply exists. Caseous necrosis: form of coagulation necrosis in which the dead cells persist indefinitely. Most commonly found in the center of TB granulomas. and Ischemia vs Infarction (2 questions from this content).  Ischemia: Characterized by impaired oxygen delivery and impaired removal of metabolic end products such as lactic acid. In contrast to pure hypoxia, which depends on the oxygen content of the blood and affects all cells in the body, ischemia commonly affects blood flow through limited numbers of blood vessels and produces local tissue injury. Infarction: occurs when an artery supplying an organ or part of the body becomes occluded an no other source of blood supply exists. As a rule, the shape of the infarction is conical and corresponds to the distribution of the artery and its branches. An artery may be occluded by an embolus, thrombus, disease of arterial wall, or pressure from outside the vessel. 8. Chapter 3. Cell Injury and Death-Intracellular Accumulations: Fatty necrosis 9. Chapter 3. Cell Injury and Death- Intracellular waste buildup. Intracellular accumulations represent the buildup of substances that cells cannot immediately use or eliminate. The substances may accumulate in the cytoplasm or in the nucleus. The accumulation may be abnormal substance that the cell has produced, and in other cases, the cell may be storing exogenous materials or products of pathologic processes occurring elsewhere in the body. Example: accumulation of beta-amyloid fragments, which progress to a skeletal muscle disorder called myositis. 10. Chapter 3. Cell Injury and Death-Pathologic Calcifications-dystrophic. Dystrophic calcifications represent the macroscopic deposition of calcium salts in injured tissue. Often visible to the naked eye as deposits that range from gritty, sand-like grains to firm, hard rock material. Pathogenesis involves the intracellular or extracellular formation of crystalline calcium phosphate. Components of the calcium deposits are derived from the bodies of dead or dying cells as well as from the circulation and interstitial fluid. Commonly seen in atheromatous lesions of advanced atherosclerosis, areas of injury in the aorta and large blood vessels, and damaged heart valves. 11. Chapter 3. Cell Injury and Death-Pathologic Calcifications-metastatic. Metastatic calcification occurs in normal tissues as a result of increased serum calcium levels. Almost any condition that increases the serum calcium level can lead to calcification in inappropriate sites such as lung, renal tubules, and blood vessels. Major causes are hyperparathyroidism, increased metabolism of calcium from bone, cancer with bone lesions, or immobilization 12. Chapter 3. Cell Injury and Death-Injury from physical agents: lead poisoning, clinical manifestations, vulnerable population. Lead poisoning: particularly toxic metal. Flaking paint, lead-contaminated dust and soil, lead contaminated root vegetables, lead water pipes or soldered joints, pottery glazes, newsprint, and toys made in foreign countries. Lead is absorbed through the gastrointestinal tracts or the lungs into the blood. A deficiency of calcium, iron, or zinc increases lead absorption. Clinical manifestations: toxicity of lead is related to its multiple biochemical effects. Has the ability to inactivate enzymes, compete with calcium for incorporation into bone, and interfere with nerve transmission and brain development. Major targets are red blood cells, GI tract, kidneys, nervous system. Anemia is the cardinal sign. Demyelination of cerebral and cerebellar white matter and death of cortical cells. Most serious manifestation is acute encephalopathy. Vulnerable population: In children, most lead is absorbed through the lungs. Although children have the same or a lower intake of lead, the absorption in infants and children is greater; thus, they are more vulnerable to lead toxicity. Lead also crosses the placenta. 13. Chapter 4. Genetic Control of Cell Function: From Genes to Proteins 14. & 15. Chapter 6. Screening, Diagnosis, and Treatment: Diagnostic Methods (2 questions from this content) Screening: represents a secondary prevention measure for the early recognition or cancer in an otherwise asymptomatic population. Screening can be achieved through observation (skin, mouth, external genitalia), palpation (breast, thyroid, rectum and anus, prostate, lymph node), and laboratory tests and procedures (pap smear, colonoscopy, mammography). Diagnostic Methods: tumor markers are antigens expressed on the surface of tumor cells or substances released from normal cells in response to the presence of tumor. Nearly all markers can be elevated in benign conditions and most are not elevated in the early stages of malignancy. Papanicalaou Test is a cytologic method used for detecting cancer cells, it consists of a microscopic examination of a properly prepared slide for the purpose of detecting the presence of abnormal cells. The usefulness of the pap test relies on the fact that cancer cells lack the cohesive properties and intercellular junctions that are characteristic of normal tissue. Tissue biopsy involves the removal of tissue specimen for microscopic study. Obtained in number of ways including: needle biopsy, bronchoscopy, cystoscopy, laproscopic methods. Immunohistochemistry involves the use of antibodies to facilitate the identification of cell products or surface markers. Can be used to determine the site of origin of metastatic tumors. Microarray Technology uses “gene chips” that can simultaneously perform miniature assays to detect and quantify the expression of large numbers of genes. The advantage of microarray technology is the ability to analyze a large number of changes in cancer cells to determine overall patterns of behavior that could not be assessed by conventional means.  16. Chapter 1. Page 10. What is evidence-based practice? (definition) Evidence based practice refers to making decisions in health care based on scientific data that has shown a specific way of managing a disease, patient symptoms, and complaints. Evidence-based practice is based on the integration of the individual clinical expertise of the practitioner with the best external clinical evidence from systematic research. 17. Chapter 1. Prenatal care. Page 9. Describe dietary recommendations o prevent neural tube defects. Folic Acid 18. Chapter 1. List examples of secondary prevention. Page 9. Secondary prevention detects disease early when it is still asymptomatic and treatment measures can cure or stop the disease from progressing. Examples include: pap smear for early detection of cervical cancer, asking if a person smokes, blood pressure measurement, cholesterol levels, and colonoscopy. Genetic and Congenital Disorders. Starts on p. 94 19. Chapter 5. Pg. 93. Genetic and Congenital Disorders: Period of vulnerability. The embryo’s development is most easily disturbed during the period when differentiation and development of the organs are taking place. This time interval, which is often referred to as the period of organogenesis, extends from day 15 to day 60 after conception. Environmental influences during the first 2 weeks after fertilization may interfere with implantation and result in abortion or early resorption of the products of conception. 20. Chapter 5. Pg. 94. Genetic and Congenital Disorders: Infectious agents-TORCH. TORCH T- toxoplasmosis O- Other (varicella-zoster, listeriosis, leptospirosis, Epstein-barr virus, TB, syphilis) R – Rubella (German measles) C- Cytomegalovirus H – Herpes Common clinical and pathologic manifestations include growth retardation and abnormalities of the brain (microcephaly, hydrocephalus), eye, ear, liver, hematopoietic system (anemia, thrombocytopenia), lungs (pneumonitis), and heart (myocarditis, congenital heart disorders) 21. Chapter 5. Pg. 96. Genetic and Congenital Disorders: Folic acid deficiency. Most birth defects are related to exposure to a teratogenic agents. Folic acid deficiency has been implicated in the development of neural tube defects (anencephaly, spina bifida, encephalocele). Studies have shown a decrease in neural tube defects when folic acid was taken long term by women of reproductive age. The recommendation is that all women of childbearing age receive 0.4mg of folic acid daily and continue upon becoming pregnant. 22. Chapter 5, page 86, and the Coursepoint Activity-Pathophysiology Module 1.7 Genetic Disorders. X-inked recessive inheritance, who is affected? Sex-linked disorders are almost always associated with the X chromosome and the inheritance is predominantly recessive. Because of the presence of a normal X, female heterozygotes(carriers) rarely experience the effects of a recessive defective gene, whereas all males who receive the gene are typically affected as they only have the mutant copy. 23. Chapter 5, page 84. Marfan’s disorder. Also, in lecture in module. Marfan syndrome is an autosomal dominant disorder of the connective tissue, which gives shape and structure to other tissues in the body and holds them in place. The prevalence is estimated to be 1 per 5000. Marfan syndrome affects several organ systems including the eyes, cardiovascular system, and the skeletal system. The skeletal deformities, which are the most obvious features of the disorder include a long thing body with exceptionally long extremities, long tapered fingers, hyperextensible joints, spinal deformities (kyphosis and scoliosis). Pectus excavatum (depressed sternum) is also sometimes present and may require surgery to repair. The most common eye disorder is bilateral dislocation of the lens because of weakness of the suspensory ligaments. The most life-threatening aspects of the disorder are the cardiovascular defects which include mitral valve prolapse, progressive dilation of the aortic valve ring, and weakness of the aorta and other arteries. There is no cure Unit 3. Chapters 7- 8. There are 3 questions from this unit. 24. Chapter 7. Stress and Adaptation-The Stress Response: Acute vs Chronic Stress & hormonal effects on the body. Acute Stress: reactions are associated with the ANS, the fight or flight response. Manifestations include a pounding headache, a cold, moist skin, and stiff neck. In situations of life threatening trauma, these acute responses may be lifesaving in that they divert blood from less essential to more essential body functions. Chronic stress: The stress response is designed to be an acute self-limited response in which activation of ANS and HPA axis is controlled in a negative feedback manner. Pathophysiologic changes can occur in the stress response system. Function can be altered in several ways: when a component of the system fails, when the neural and hormonal connections among components of the system were dysfunctional, and when the original stimulus for the activation of the system is prolonged or of such magnitude that it overwhelms the ability of the system to respond appropriately. Chronicity and excessive activation of the stress response can result from chronic illnesses as well as contribute to the development of long-term health problems. 25. Chapter 8. Compartmental Distribution of Body Fluids: Edema assessment and treatment. Edema can be defined as palpable swelling produced by expansion of the interstitial fluid volume. Assessment: daily weight, visual assessment, measurement of the affected part, application of finger pressure to assess for pitting edema. Treatment: directed toward maintaining life when the swelling involves vital structures, correcting or controlling the cause, and preventing tissue injury. Edema of the lower extremities may respond to simple measures such as elevating the feet. Diuretic therapy is commonly used for an increase in ECF volume. Albumin may be administered to raise the plasma colloidal osmotic pressure when the cause of the edema is hypoalbuminemia. Elastic support stockings and sleeves increase interstitial fluid pressure and resistance to outward movement of fluid from the capillary into the tissue spaces. 26. Chapter 8. Mechanisms of Acid-Base: Function of the lungs and kidneys in acid-base regulation. Respiratory: Control of extracellular co2 by the lungs. Only comes into play when the chemical buffers do not minimize H+ changes. Only about 50 to 75% effective as a buffer system, occurring within minutes and is maximal within 12 to 24 hours. In acting rapidly, it prevents large changes in pH from occurring while waiting for the much more slowly reacting kidneys to respond. Kidneys: Third line of defense in acid-base disturbances and play three major roles. First is through excretion of H+ from fixed acids that result from protein and lipid metabolism. Second is accomplished through the reabsorption of HCO3 that is filtered through the glomerulus, so it’s not lost in the urine. The third is the production of new HCO3 that is released back into the blood. Unit 4. Chapters 10- 12. There are 10 questions from this unit. Begins on p. 263. 27. Chapter 10. Infectious Diseases. Diagnosis and Treatment of Infectious Diseases: Detection and Diagnosis of infectious diseases. The diagnosis of an infectious disease requires 2 criteria: the recovery of a probable pathogen or some evidence of its presence from the infected sites of a diseased host and accurate documentation of clinical signs and symptoms compatible with an infectious process. Diagnosis types: Culture: refers to the growth of a microorganism outside of the body, usually on or in artificial growth media such as agar plates or broth. In the case of bacterial pathogen, identification is based on microscopic appearance and gram stain reaction, shape, texture, and color (morphology) of the colonies and by a panel of biochemical reactions that fingerprint salient biochemical characteristics of the organism. Fungi and mycoplasmas are cultured in much the same way as bacteria but with more reliance on microscopic and colonial morphology for identification. Some fungi can take weeks to grow and identify through culture. In the setting of acute infection, it is important that cultures are obtained prior to antibiotic administration. The causative organism cannot be identified by culture in up to 33% of people presenting with sepsis. Serology: indirect means of identifying infectious agents by measuring serum antibodies in the diseased host. A tentative diagnosis can be made if the antibody level, also called the antibody titer, against a specific pathogen rises during the acute phase of the disease and falls during convalescence. Serologic identification of an infectious agent is not as accurate as a culture, but may be a useful adjunct. IgM specific antibodies generally rise and fall during the acute phase of the disease, whereas the synthesis of IgG increases during the acute phase and remains elevated until or beyond resolution. IgM antibodies do not cross the placenta, so if IgM is elevated in a neonate it did not come from mother, it originated in child. Antigen detection incorporates features of culture and serology but reduces to a fraction the time required for diagnosis. This method relies on purified antibodies to detect antigens of infectious agents in specimens obtained from the diseased host. DNA and RNA sequencing: DNA probe hybridization – small fragements of DNA are cut from the genome of a specific pathogen and labeled with compounds that allow detection. Polymerase chain reaction (PCR) – this method incorporates two unique reagents: a specific pair of oligonucleotides called primers and heat-stable DNA polymerase. 28. Chapter 10. Infectious Diseases. Diagnosis and Treatment of Infectious Diseases: General intervention methods used in treatment of infectious diseases. Antimicrobial Agents: can be categorized roughly according to mechanism of anti-infective activity, chemical structure, and target pathogen. 1. Antibacterial agents: antibiotics. Most antibiotics are actually produced by other microorganisms, primarily bacteria and fungi, as by-products of metabolism. Effective only against other prokaryotic organisms. Bactericidal if it causes irreversible and lethal damage  to the bacterial pathogen and bacteriostatic if its inhibitory effects on bacterial growth are reversed when the agent is eliminated. 2. Antiviral agents: Viral replication requires the use of eukaryotic host cell enzymes, and the drugs the effectively interrupt viral replication are likely to interfere with host cell production as well. Protease inhibitors are solely for the treatment of HIV, they inhibit an HIV-specific enzyme that is necessary for late maturation events in the virus life cycle. 3. Antifungal agents: The target site of the two most important families of antifungal agents is the cytoplasmic membranes of yeasts or molds. 4. Antiparasitic agents: Treatment of parastitic illness is based on exploiting essential components of the parasites metabolism or cellular anatomy that are not shared by the host. Any relatedness between the target site and the cells of the host increases the likelihood for toxic reactions in the host. Immunotherapy: Involves supplementing or stimulating the hosts immune response so that the spread of a pathogen is limited or reversed. IVIG and cytokines are examples. Surgical Intervention: surgical removal of infected tissues, organs, or limbs. Usually only needed in present day when the infective agent is resistant to all treatments. May be used to hasten the recovery process by providing access to an infected site by antimicrobial agents (drainage of abscess), cleaning the site (debridement), or removing infected organs or tissue (appendectomy). Surgery might be the only means of a complete cure such as endocarditis. 29. Chapter 11. Innate and Adaptive Immunity: Epithelial Barriers. The intact skin is by far the most formidable physical barrier available to infection because of its design. It is comprised of closely packed cells that are organized in multiple layers that are continuously shed. In addition, a protective layer of protein, known as keratin, covers the skin. Sheets of tightly packed epithelial cells line and protect the gastrointestinal, respiratory, and urogenital tracts and physically prevent microorganisms from entering the body. These cells destroy the invading organisms by secreting antimicrobial enzymes, proteins, and peptides. When pathogens are able to breach the epithelial defenses, the innate immune response is initiated by the bodys leukocytes, which recognize common surface receptors present on the invading microorganisms. 30. Chapter 11. Innate and Adaptive Immunity. See p. 261: Infectious disease terminology. Toxins produced by bacteria. Host – any organism capable of supporting the nutritional and physical growth requirements of another Infection – presence and multiplication within a host of another living organism, with subsequent injury to the host Colonization – act of establishing a presence Microflora – Internal and external exposed surfaces of the human body are normally and harmlessly inhabited by a multitude of bacteria Mutualism – interaction in which both the microorganism and the host derive benefits from the interaction  Parasitic relationship – only the infecting organism benefits from the relationship and the host either gains nothing or sustains injury from the interaction. Infectious disease – if the host sustains injury or pathologic damage from a microorganism Virulence – disease producing potential Pathogens – microorganisms capable of causing disease Prokaryotes – unicellular organisms, bacteria, lack organized nucleus Plasmids – smaller extrachromosomal pieces of circular DNA that bacteria often harbor (sometimes contain genetic material that increased the virulence or antibiotic resistance) Cytoplasm – contains reproductive and metabolic machinery of the cell Cytoplasmic membrane – flexible lipid membrane 31. Chapter 11. Adaptive Immunity. Passive Immunity. Adaptive immunity: acquired when the host mounts an immune response to an antigen either through the process of vaccination or from environmental exposure. It is called active immunity because it requires the host’s own immune system to develop an immunologic response including the development of memory. Usually long lasting but requires a few days to weeks after a first exposure to sufficiently develop an appropriate immunologic response that culminates in the destruction of the presenting antigen. Passive immunity: Immunity transferred from another source. Most common form of passive immunity is that conferred from mother to fetus. IgG antibodies are transferred to the fetus via the placenta and also after birth in breast milk and colostrum so infants have some degree of protection from infection for 3 to 6 months. Produces only short-term protection that lasts weeks to months. 32. Chapter 11. Pg. 292. Innate and Adaptive Immunity: Immunoglobulins. Primary & secondary immune response. Antibodies are protein molecules are also known as immunoglobulins. Igs are classified into 5 different categories based upon their role in the humoral defense mechanism. The Ig is compromised of four-polypeptide chains with at least 2 identical antigen-binding sites. 33. Chapter 9: Page 317. Describe the emergency care of a client with anaphylaxis. Anaphylaxis is a castastropic, systemic, life-threatening IgE-mediated hypersensitivity reaction associated with the widespread release of histamine into the systemic circulation that produces massive vasodilation, hypotension, arterial hypoxia, and airway edema. Clinical manifestations occur along a continuum in severity and can be graded on a scale of I to IV. Grade I reactions are usually confined to the cutaneous and mucosal tissues manifesting as erythema and urticaria, with or without angioedema. Grade II reactions progress to include moderate multisystem signs such as hypotension, tachycardia, dyspnea, and GI disturbances. Grade III reactions become life threatening because of the development of bronchospasm, cardiac dysrhythmias, and cardiac collapse. Once a hypersensitivity reaction reaches grade IV, cardiac arrest has occurred and management is purely resuscitative in nature. Preventing exposure to potential triggers that cause anaphylaxis is essential. The initial management should focus on withdrawal of the offending allergen, maintenance of a patent airway, establishment of appropriate intravenous access, volume resuscitation, and administration of epinephrine. 34. Chapter 11: Page 279. Innate immunity barriers. Describe innate barriers that help protect humans against pulmonary infections. Unit 5: Disorders of Neural Function. Chapters 13-16, 18. There are 12 questions from this unit. 35. Chapter 13. Metabolic Requirements of Nervous Tissue. Consequences of unmet demand. Nervous tissue has a high rate of metabolism. The brain receives 15% to 20% of the total resting cardiac output and consumes 20% of its oxygen. Despite its substantial energy requirements, the brain cannot store oxygen or effectively engage in anaerobic metabolism. An interruption in the blood or oxygen supply to the brain rapidly leads to clinically observable signs and symptoms. Interruption also leads to the accumulation of metabolic by-products that are toxic to neural tissue. 36. Chapter 14. Somatosensory Function, Pain, Headache, and Temperature Regulation: Types of pain- compare definitions, begins on p. 402-403. Acute pain: elicited by injury to body tissues and activation of nociceptive stimuli at the site of local tissue damage. Generally short duration and tends to resolve when the underlying pathologic process has resolved. Chronic pain: Pain that persists longer than might be reasonably expected after an inciting event. Continues for years. Sustained by factors that are both pathologically and physically remote from the originating cause. Cutaneous and Deep Somatic Pain: cutaneous pain arises from superficial structures. Deep somatic pain originates in deep body structures (muscles, tendons, joints, blood vessels). It is more diffuse than cutaneous pain. Visceral Pain: Origin in the visceral organs and is one of the most common pains produced by disease. The important difference between surface pain and visceral pain is the type of damage that causes pain. Strong contractions, distention, or ischemia affecting the walls of the viscera can cause severe pain. Referred Pain: pain that is perceived at a site different from its point of origin but innervated by the same spinal segment. Example: STEMI pain is referred to left arm, neck, and chest. 37. Chapter 15. Disorders of the motor unit/Disorders of the Neuromuscular Junction: Myasthenia gravis, neuromuscular junction pathophysiology.p. 443. Myasthenia Gravis is a disorder of transmission at the neuromuscular junction because of antibody-mediated attack on the nicotinic AChR or muscle-specific tyrosine kinase that affects communication between the motor neuron and the innervated muscle. Can occur at any age but peak incidence is young adulthood. The exact mechanism is unclear but it is thought to be caused by sensitized helper T cells and an antibody-directed attack on the acetylcholine receptor in the neuromuscular junction. The antibody attack leads to a shedding of the acetylcholine receptor-rich terminal portions of the folds in the end plate of the muscle fiber, fewer receptors, and a widened synaptic space that impairs signal transmission. The antibodies do not directly block the binding. The neuromuscular junction serves as a synapse between a motor neuron and a skeletal muscle fiber. The transmission of impulses at the neuromuscular junction is mediated by the release of the neurotransmitter acetylcholine from the axon terminals. Acetylcholine binds to the specific receptors in the end-plate region of the muscle fiber surface to cause muscle contraction. 38. Chapter 15. Disorders of Motor Function: Multiple Sclerosis. Clinical manifestations and course. Multiple sclerosis is characterized by inflammation and destruction of mostly the white matter of the CNS myelin of the brain, spinal cord, and optic nerve. The peripheral nervous system is spared, and there is usually no evidence of an associated systemic disease. The lesions of MS consist of hard, sharp-edged, demyelinated patches that are visible throughout the white matter as well as sometimes the gray matter of the CNS. Areas commonly affected by MS are the optic nerve, corticobulbar tracts (speech and swallowing), corticospinal tracts (muscle strength), cerebellar tracts (gait and coordination), spinocerebellar tracts (balance), medial longitudinal fasciculus (conjugate gaze function of the extraocular eye muscles), and posterior cell columns of the spinal cord (position and vibratory sensation). Usually presents with an acute or subacute episode of parathesias, optic neuritis, diplopia, or specific types of gaze paralysis. Other common symptoms are abnormal gait, bladder and sexual dysfunction, vertigo, nystagmus, fatigue, and speech disturbance. These symptoms usually last for days to weeks and then completely or partially resolve. Four categories of the disease: Relapsing-remitting, secondary progressive, primary progressive, or progressive relapsing. 39. Chapter 15. Disorders of the cerebellum and basal ganglia: Parkinson’s Disease Degenerative disorder of basal ganglia function that results in variable combinations of tremor, rigidity, akinesia/bradykinesia, and postural changes. Characterized by progressive destruction of the nigrostriatal pathway with subsequent reduction in striatal concentrations of dopamine. Mean onset is 57 years old. Because the basal ganglia also influence the autonomic nervous system, people with Parkinson disease often have excessive sweating, sebaceous gland secretion, and salivation. Cognitive dysfunction may also be present. Treatment must be highly individualized. No treatment will prevent the disease progression, only symptom management. Botulinum toxin injections may be used in the treatment of dystonias such as eyelid spasm and limb dystonias. Antiparkinson drugs act by increasing the functional ability of the underactive dopaminergic system or by reducing the excessive influence of excitatory cholinergic neurons. 40. Chapter 16. Mechanisms of Brain Injury. Primary and Secondary Brain Injuries. Primary brain injuries: Damage is caused by impact. Include focal (contusion, laceration, hemorrhage) or diffuse (concussion, diffuse axonal injury). Secondary brain injuries: damage results from the subsequent brain swelling, infection, or cerebral hypoxia. Most common cause is ischemia. Hematomas. Subdural, acute vs chronic subdural hematomas.  Hematomas: Result from vascular injury and bleeding. Epidural Hematoma: usually caused by head injury in which the temporal area of the skull is fractured. Develops between the inner table of the bones of the skull and the dura. Usually results from a tear in an artery associated with a skull fracture. More common in younger people because the dura is less firmly attached to the skull surface than in an older person. Traumatic Intracerebral Hematomas: single or multiple. Occur in any lobe of the brain but are most common in the frontal or temporal lobes, related to the bony prominences on the inner skull surface. Subdural: Develops in the area between the dura and the arachnoid (subdural space) and usually is the result of a tear in the small bridging veins that connect veins on the surface of the cortex to dural sinuses. These veins are snapped in head injury when the brain moves suddenly in relation to the skull. Acute: Acute subdural hematomas progress rapidly and have a high mortality rate because of the severe secondary injuries related to edema and increased ICP. Chronic: Symptoms of chronic subdural hematoma develop weeks after a head injury, so much later that the person may not remember having a head injury. More common in alcoholics and older adults because brain atrophy causes the brain to shrink away from the dura and stretch fragile bridging veins. 41. Chapter 16. Mechanisms of Brain Injury. Stroke. Ischemic vs hemorrhagic. Ischemic: Caused by cerebrovascular obstruction by thrombosis or emboli. Five main mechanisms of stroke subtypes and their frequency: 20% large artery thrombosis (atherosclerotic disease), 25% small penetrating artery thrombosis disease (lacunar stroke), 20% cardiogenic embolism, 30% cryptogenic stroke (undetermined cause), and 5% other. Hemorrhagic: Most frequently fatal stroke results from the spontaneous rupture of a cerebral blood vessel. Common predisposing factors are advancing age and hypertension. Vomiting commonly occurs at onset, and headache occurs after. 42. Chapter 16. Disorders of Brain Function/Cerebrovascular Disease/Stroke. Clinical manifestations. Specific manifestations are determined by the cerebral artery that is affected, by the area of brain tissue that is supplied by that vessel, and by the adequacy of the collateral circulation. Always sudden in onset and focal and usually are one sided. Most common symptoms are a facial droop, arm weakness, and slurred speech. Other frequent symptoms are unilateral numbness, vision loss in 1 eye or to 1 side, language disturbance, and sudden unexplained imbalance or ataxia. 43. Chapter 18. Neurocognitive Disorders: Alzheimer’s Disease. Diagnosis and treatment. See chap 18 ppt and page 534. A diagnosis of alzheimers can only be confirmed by microscopic examination of tissue obtained from a cerebral biopsy or at autopsy. The diagnosis is based on clinical findings. Neuroimaging and metabolic screening are important steps in excluding other conditions when diagnosing. There is no curative treatment for AD. Medications may slow the progression and improve depression, agitation, or sleep disorders. Cholinesterase inhibitors have been shown to be effective in slowing the progression of the disease by potentiating the action of available acetylcholine and inhibiting acetylcholinesterase. Antipsychotics are not approved for using in treating agitation. Two major goals of care are maintaining socialization and providing support for caregivers. 44. Chapter 15. Disorders of the motor unit/Polyneuropathies: Guillain-Barre Syndrome. Cause, clinical manifestations, and treatment. Guillain-Barre syndrome is an acute immune-mediated polyneuropathy. The syndrome defines a clinical entity that is characterized by rapidly progressive ascending symmetrical limb weakness and loss of tendon reflexes. Most common cause of acute, flaccid nontraumatic paralysis. Manifested by infiltration of mononuclear cells around the capillaries of the peripheral neurons, edema of the endoneurial compartment, and demyelination of ventral spinal roots. Cause probably has an immune component. Most people report an acute, influenza-like illness before the onset of symptoms. Clinical manifestations include progressive ascending muscle weakness of the limbs, producing a flaccid paralysis. Parasthesia and numbness often accompany the loss of motor function. Paralysis will progress to involve the respiratory muscles. Treatment includes support of vital functions and prevention of complications such as skin breakdown and thrombophlebitis. Treatment is most effective if initiated early in the course of the disease. Plasmapheresis and high-dose intravenous immunoglobulin therapy and generally the mainstay of treatment. 45. Chapter 18. Neurocognitive Disorders: Schizophrenia-neurophysiology of symptoms. Page 530. The complete set of pathogenic mechanisms underlying schizophrenia is not clear. Research suggest that changes in the dysregulation of the dopamine and serotonergic system and other neurotransmitter changes such as decreased glutamate activity through dysfunction of its N-methyl-D- aspartate receptor play a role. Many of the symptoms of impaired cognition seen in schizophrenia are through to be tied to deficits in GABA. Neuroimaging suggests several functional abnormalities occur which include loss of cortical gray matter, abnormal cortical thinning, reduced numbers of synaptic structures on neurons, reduced dendritic spine density of pyramidal neurons in the prefrontal cortex, and arrested migration of hippocampal neurons. 46. Chapter 18. Neurocognitive Disorders: Mood disorders-neurophysiology of symptoms. Page 531. Epidemiologic and neurobiologic studies suggest interactions between biologic and psychosocial factors over time explain the risk of developing MDD. Multiple possible biologic theories have been identified in the development of depressive disorder including inflammation, HPA axis hyperactivity, low levels of neurotrophic growth factor, and low levels of vitamin D. Growing evidence supports some forms of depression may be linked to ongoing inflammation in the body. Neuroimaging indicates smaller hippocampal volumes and hippocampal hypertrophy in people who have depression and have experienced early abuse. Neurologic disorders of the limbic system and basal ganglia are also involved in the development of mood disorders. Dopamine plays a role in depression. Unit 6. Disorders of Special Sensory Function. Chapters 19, 20. There are 4 questions from this unit.  47. Disorders of Eye Movement. Begins on p. 577. Strabismus vs amblyopia: differences in presentation. Strabismus (squint) refers to any abnormality of eye coordination or alignment that results in loss of binocular vision. When images from the same spots in the visual space do not fall on corresponding points of the two retinas, diplopia (double vision) occurs. May be divided into two forms: nonparalytic forms (concomitant) where there is no primary muscle impairement, and paralytic (nonconcomitant) in which there is weakness or paralysis of one or more of the extraocular muscles. Called intermitted, or periodic, when there are periods in which the eyes are parallel. Amblyopia: Sometimes called lazy eye. Decrease in visual acuity resulting from abnormal visual development in infancy or early childhood. Vision loss ranges from mild (worse than 20/25) to severe (legal blindness, 20/200 or worse). 48. Disorders of the Conjunctiva. Begins on p. 550. Compare the clinical manifestation of allergic, viral, and bacterial conjunctivitis. How can tell one from the other diagnostically? Allergic: Usually characterized by itching. Bilateral tearing, itching, and redness of the eye. Treatment includes allergen avoidance, use of cold compresses, eye washes with tear substitute, and oral antihistamines. Viral: Common cause is adenovirus. Symptoms include generalized conjunctival hyperemia, copious tearing, and minimal exudate. Usually associated with upper respiratory tract infection. Usually resolves over time with use of cool compresses and artificial tears. Topical antivirals are not recommended. Bacterial: Burning, tearing, mucopurulent or purulent discharge. Normally begins in one eye and within 24 to 48 hours it speards to the unaffected eye. Drainage may be green, white, or yellow. Treatment includes antibiotic drops or ointments. N. gonorrhoeae is the most serious form of conjunctivitis than can lead to corneal ulceration/perforation and permanent blindness if not treated; symptoms include conjunctival redness, chemosis (swelling around the cornea), lid swelling, and tender/swollen periauricular lymph nodes. 59. Disorders of Hearing and Vestibular Function: Otitis Media-Clinical Manifestations Acute onset of otalgia (pulling of the ears in an infant) that may interfere with sleep and/or activity, fever (>39), irritability, otorrhea, hearing loss, evidence of middle ear inflammation, middle ear effusion (decreases mobility of the tympanic membrane). Children older than 3 may have rhinorrhea, vomiting, and diarrhea. Ear pain usually increases as the effusion accumulates behind the tympanic membrane. Perforation of the tympanic membrane may occur acutely, allowing purulent material from the eustachian tube to drain into the external auditory canal.  Otitis media with effusion symptoms: Complaint of intermittent ear pain, sensation of fullness in ear, complaint of hearing loss, dizziness, decreased tympanic membrane mobility, visible air-fluid level with or without bubble. 50. Disorders of Hearing and Vestibular Function: Otitis Media-Risk Factors. Page 589. May occur in any age group, but is more frequently diagnosed in children between the ages of 3 months and 3 years. Smoking in the house is a significant risk factor. Other risk factors include prematurity, daycare attendance, having an unimmunized status, bottle-feeding, feeding in the supine position, being overweight or obese, a family history of otitis media, being male, and sharing a bedroom. More frequent in children with craniofacial anomalies or congenital syndromes associated with craniofacial abnormalities. Strucutural immaturity contributes to the increased risk because the eustachian tube is shorter and more horizontal, and it can spread more easily through the eustachian canal of infants who spend most of their day supine. To reduce the risk of contracting otitis media: routine childhood vaccinations against pneumococci and flu, elimination of household smoking, exclusive breast-feeding until 6 months of age, avoiding of feeding while lying down and propping bottles, use of xylitol, and selecting daycare facilities with small staff-to-child ratio.