PATHOPHYSIOLOGY NR 507WK3TD1

PATHOPHYSIOLOGY NR 507WK3TD1 Week 3: Cardiovascular, Cellular, and Hematologic Disorders - Discussion Part One Loading... Discussion This week's graded topics relate to the following Course Outcomes (COs). 1 2 3 4 5 6 ...

PATHOPHYSIOLOGY NR 507WK3TD1 Week 3: Cardiovascular, Cellular, and Hematologic Disorders - Discussion Part One Loading... Discussion This week's graded topics relate to the following Course Outcomes (COs). 1 2 3 4 5 6 7 Analyze pathophysiologic mechanisms associated with selected disease states. (PO 1) Differentiate the epidemiology, etiology, developmental considerations, pathogenesis, and clinical and laboratory manifestations of specific disease processes. (PO 1) Examine the way in which homeostatic, adaptive, and compensatory physiological mechanisms can be supported and/or altered through specific therapeutic interventions. (PO 1, 7) Distinguish risk factors associated with selected disease states. (PO 1) Describe outcomes of disruptive or alterations in specific physiologic processes. (PO 1) Distinguish risk factors associated with selected disease states. (PO 1) Explore age-specific and developmental alterations in physiologic and disease states. (PO 1, 4) Discussion Part One (graded) A 17-year-old African American from the inner city complains of severe chest and abdominal pain. Upon examination the attending physician performs and EKG, chest x-ray, and an abdominal and chest clinical examination and finds nothing. Assuming, she is drug seeking he sends her home. She comes back to the ER 4 hours later and now you see the patient. She explains that she was running track thispast afternoon at school and that despite being very hot (100 F) she pushed on. Afterwards, she starts feeling extensive pain in her chest and abdomen. She has jaundiced eyes, her blood pressure is 98/50, pulse is 112, T = 99.9 F, R = 28. The pain seems out of proportion to the physical findings. 0 What is your list of differential diagnoses in this case and explain how each of these fits with the case patient as described above. Be sure to list at least four (4) pertinent differential diagnoses. Indicate which of these you would select as the most likely diagnosis and explain why. Now, as she is in the ER she begins to exhibit stroke like features. ? Does this change your differential? How do you treat this patient now? Are they any preventative actions that could have been taken? Responses Rechel DelAntar Differential Diagnoses Hello Professor and Class, Differential Diagnoses A case of a 17 year old African American from the inner city complaining of sever chest and abdominal pain seen in the ER but was released after clinical examinations and tests reveal no abnormalities with the assumption that the patient is drug seeking. She comes back 4 hours after running track in school with symptoms of extensive chest and abdominal pain and jaundice and expressed low-grade fever of 100F earlier. BP 98/50, tachycardic at 112 RR=28. Once n the ER, patients symptoms have progressed to exhibiting stroke like features. Based on the patient’s presentation and symptoms, patient may have: 1. Sickle Cell Disease – The term sickle cell disease (SCD) describes a group of inherited red blood cell disorders. People with SCD have abnormal hemoglobin, called hemoglobin S or sickle hemoglobin, in their red blood cells. In SCD, a person inherits 2 abnormal genes, one from each parent. In all forms, one of the genes causes the production of hemoglobin S. In the case of Sickle cell anemia, a patient has 2 hemoglobin S, hemoglobin SS. Among all the types of SCD, sickle cell anemia is the most common type (National Heart, Lung and Blood Institute, 2015). Normally, red blood cells are flexible and round, moving easily through the blood vessels. In sickle cell disease, red blood cells become rigid and sticky and shaped like sickles/crescent moons. These irregularities causes the cells to get stuck in small blood vessels, which can slow or block blood flow and decrease oxygen supply to parts of the body. Pain is the major symptom when the patient is in “crisis”. Pain develops when sickle-shaped red blood cells block blood flow through tiny blood vessels to your chest, abdomen and joints. Pain is sporadic and can last for hours or weeks. Continued poor oxygen supply to organs may eventually lead to organ damage. Stroke is a common manifestation of the disease because of blood flow obstruction (Mayo Clinic, 2014). This diagnosis fits the patient’s presentation and her experiencing signs of stroke is one of the symptoms of the disease. 2. Hemochromatosis - causes your body to absorb too much iron from the food you eat. Excess iron is stored in your organs, especially your liver, heart and pancreas. Too much iron can lead to life- threatening conditions, such as liver disease, heart problems and diabetes. Symptoms include abdominal pain, weakness, fatigue, heart failure such as chest pain and liver failure (National Institute of Diabetes and Digestive and Kidney Disease, 2014). Although some of these symptoms are similar to the one in the case study, hemochromatosis occurs mostly among Caucasians and the pain in this disease is more chronic and not severe which does not fit the profile of the patient. 3. G6PD – Glucose-6-dehydrogenase Deficiency is a hereditary type of hemolytic anemia in which red blood cells break down when the body is exposed to certain drugs or the stress of infection. It is common between Mediterranean and African origin and is characterized by abnormally low levels of glucose-6-phosphate dehydrogenase, an enzyme involved in the pentose phosphate that is especially important in the red blood cell. G6PD deficiency is the most common human enzyme defect. There is no 5/15/2016 7:54:30 PMspecific treatment, other than avoiding known triggers. Symptoms include jaundice, fever, fatigue, tachycardia and abdominal pain from splenomegaly and hyperbilirubinimia (US National Library of Medicine, 2016). Although some of the symptoms fit the case study, patients with G6PD do not experience chest pain. Also the pain experienced in patients with this disease are not as severe as the one described n the case study. This disease also does not lead to stroke like symptoms. 4. Heart Failure - often referred to as congestive heart failure (CHF), occurs when the heart is unable to pump sufficiently to maintain blood flow to meet the body's needs. There are different types of heart failure, there is right sides or left sided heart failure referring to the side of the heart being affected. There is also systolic or diastolic heart failure. When the condition is severe, blood backs up to the lungs causing elevated pulmonary pressure and congestion leading to right sided heart failure (cor pulmonale). Backward failure of the right ventricle leads to congestion of systemic capillaries. This generates excess fluid accumulation in the body causing congestion in other organs. Symptoms include chest pain, shortness of breath, tachycardia, jaundice and coagulopathy (from impaired liver function from congestive hepatopathy), abdominal distention and pain (McDonagh, T., 2011). Although some of the symptoms are the same as the case study, these symptoms do not occur suddenly. Heart Failure occurs over time and would not have allowed the patient to be able to run track because at this stage of symptomatology, the patient would have been very debilitated that ambulating short distances would render them dyspnic. 5. Leukemia - is a clonal malignant disorder of leukocytes in the blood and blood-forming organs. The common feature of all forms of leukemia is an uncontrolled proliferation of malignant leukocytes, causing an overcrowding of bone marrow and decreased production and function of normal hematopoietic cells. There are different types of leukemia, Acute myeloid leukemia (AML) and acute lymphocytic leukemia (ALL) progress much faster and symptoms may worsen more quickly than with the chronic leukemias (CML and CLL) (Mc,Cance, K.L., et. al., 2013). Symptoms include fever, fatigue, weakness, abdominal pain, easy bruising and petechiae. Although some symptoms match that of the patient in the case study, leukemia does not show symptoms of chest pain or jaundice as well as stroke and does not for the case study. References: Mayo Clinic. (2014). Diseases and Conditions: Sickle Cell Anemia. Retrieved from http://www.mayoclinic.org/diseases-conditions/sickle-cell-anemia/basics/symptoms/con- 20019348 McCance, K.L., Huether, S.E., Brashers, V.L. and Rote, N.S. (2013). Pathophysiology: The biologic basis for disease in adults and children (7 ed.). St. Louis, MO: Mosby. . th McDonagh, T. (2011). Oxford textbook of heart failure. Oxford: Oxford, University Press. National Institute of Diabetes and Digestive and Kidney Disease. (2014). Hemochromatosis. Retrieved from http://www.niddk.nih.gov/health- information/health-topics/liver-disease/hemochromatosis/Pages/facts.aspx. National Heart, Lung and Blood Institute. (2015). What is sickle cell disease? Retrieved from http://www.nhlbi.nih.gov/health/health-topics/topics/sca. US National Library of medicine. (2016). Glucose-6-phosphate dehydrogenase Deficiency. Retrieved from https://ghr.nlm.nih.gov/condition/ glucose-6-phosphate-dehydrogenase-deficiency. Rechel DelAntar reply to Rechel DelAntar RE: Differential Diagnoses The signs and symptoms of sickle cell disease are caused by the sickling of red blood 5/16/2016 10:59:33 PM cells. Painful episodes can occur when sickled red blood cells, which are stiff and inflexible, get stuck in small blood vessels. These episodes deprive tissues and organs of oxygen-rich blood and can lead to organ damage, especially in the lungs, kidneys, spleen, and brain. A particularly serious complication of sickle cell disease is high blood pressure in the blood vessels that supply the lungs (pulmonary hypertension). Pulmonary hypertension occurs in about one-third of adults with sickle cell disease and can lead to heart failure. The rapid breakdown of red blood cells may also cause yellowing of the eyes and skin, which are signs of jaundice. Since sickle cell isinherited, genetic counseling is an important part of managing this disease (National Heart, Lung and Blood Institute, 2015). Health maintenance for patients with sickle cell disease starts with early diagnosis, preferably in the newborn period and includes penicillin prophylaxis, vaccination against pneumococcus bacteria and folic acid supplementation. Treatment of complications often includes antibiotics, pain management, intravenous fluids, blood transfusion and surgery all backed by psychosocial support. Blood Transfusions are often necessary in cases of severe anemia. Currently, Stem cell transplantation is the only cure for SCD patients (Sickle Cell Disease Association of America, 2016). References: National Heart, Kung and Blood Institute. (2015). How is Sickle Cell Disease treated. Retrieved from https://www.nhlbi.nih.gov/health/health-topics/topics/sca/treatment. Sickle Cell Disease Association of America. (2016). About Sickle Cell Disease. Retrieved from http://www.sicklecelldisease.org/index.cfm?page=about-scd. Instructor Brown reply to Rechel DelAntar RE: Differential Diagnoses What part of the Heart failure causes the chest pain? Rechel DelAntar reply to Instructor Brown RE: Differential Diagnoses 5/19/2016 4:04:11 PM Hello Professor and Class, Heart failure (HF), often referred to as congestive heart failure (CHF), occurs when the heart is unable to pump sufficiently to maintain blood flow to meet the body's needs. Heart failure is caused by many conditions that damage the heart muscles which include Coronary Artery Disease, heart attack (MI), Cardiomyopathy and hypertension. In these cases, there is a decrease in blood supply to the myocardium either due to blockage from thrombus or damage to the heart muscles from infections, alcohol abuse or drug abuse and increased work load of the heart due to elevated pressures such as in hypertension. Decrease blood flow to the heart causes decreased oxygenation to the myocardium causing symptoms of chest pain (national Heart, Lung and Blood Institute, 2015). Reference: National Heart, Lung and Blood Institute. (2015). What is Heart Failure? Retrieved from https://www.nhlbi.nih.gov/health/health-topics/topics/hf. 5/17/2016 7:02:48 PM Alice Jeffries Discussion Part One 5/16/2016 3:36:45 AM Dr. Brown and class, The four differential diagnosis are below. While working in Arizona on a cardiac floor, we had a patient who was regularly admitted with sickle cell disease. She was a young lady as described in the case study. She was often treated like a drug seeker, which was so sad because she would not get treatment until she was in crisis. I believe the case study diagnosis is sickle cell disease. Because the risk of stroke in children with SCD is higher than children without SCD due to the change in shape, stiffening and distorted red blood cells, and the changes in the vascular system that causes narrowing of arteries (Adams, 2014), the signs of stroke would not change my initial diagnosis. The increased velocity of blood going through areas of stenosis increases the risk of stroke by 39% for each 10 cm/sec above the median velocity (Adams, 2014). Blood transfusion can be used to halt an impending stroke in adolescents with SCD (Adams,2014). Testing can be done in advance to determine the velocity of blood flow through vessels, and if indicated, patients may be treated with a protocol including frequent blood transfusions (Adams, 2014). Also important is regular follow up appointments with medical professionals and preventing the crisis in advance by staying hydrated and avoiding causative factors such as certain physical activities that could lead to crisis, extreme temperatures, and stress. Sickle cell disease (SCD): Can emerge during adolescence and more patients are treated in hospitals during the age range of 13 to 18 years old, and primary of African-Caribbean and African descent (Musumadi, Westerdale, and Appleby, 2012). The crisis could have started due to stress, dehydration, or exercising in hot temperatures. Hallmark signs of a crisis include pain, dehydration can lead to tachycardia and increased respirations, and low blood pressure, while the breaking down of the red blood cells during the crisis can lead to elevated bilirubin levels and jaundice. Sickle cell trait: According to the CDC website, people with sickle cell trait do not usually have symptoms of sickle cell disease, however they sometimes do, especially under extreme circumstances including rigorous training, dehydration, or extreme conditions (CDC.gov, n.d.).; I think that sickle cell trait may be the cause because the signs and symptoms match and the patient was probably dehydrated from exercising in extreme heat. Additionally, the complications of sickle trait are less common than cell anemia and may not have been experienced prior to this occurrence. Sickle cell trait would affect same population as sickle cell disease, including patients of African-Caribbean and African descent (Musumadi, 2012). Cholangitis: Infection of the bile duct is uncommon in children and adolescents, but not unheard of. The presenting signs and symptoms include right upper quadrant abdominal pain, fever, and jaundice (Duman, Savas, Recep Aktas, and Akcam, 2014). Cholelithiasis: gallbladder disease is a common reason for jaundice in adults, however gallstones usually take several decades to develop (Sargent, and Clayton, 2011). Ali Adams, R. J. (2014). Prevention of Stroke in Sickle Cell Anemia. Journal Of Law, Medicine & Ethics, 42(2), 135-138. doi:10.1111/jlme.12128 Duman, L., Savas, C., Recep Aktas, A., & Akcam, M. (2014). Choledochoduodenal fistula: An unusual cause of recurrent cholangitis in children. Journal of Indian Association Of Pediatric Surgeons, 19, 172-174 3p. doi:10.4103/0971-9261.136479 Musumadi, L., Westerdale, N., & Appleby, H. (2012). An overview of the effects of sickle cell disease in adolescents. Nursing Standard, 26, 35-40 6p. Sargent, S., & Clayton, M. (2011). Adult jaundice--the pathophysiology, classification and causes. Gastrointestinal Nursing, 9(4), 34-40 7p. What you should know about sickle cell trait. (n.d.). Retrieved from http://www.cdc.gov/ncbddd/sicklecell/documents/SCD%20factsheet_Sickle%20Cell%20Trait.pd Instructor Brown reply to Alice Jeffries 5/17/2016 7:04:57 PMRE: Discussion Part One What is happening in the sickle cell process to cause pain? Alice Jeffries reply to Instructor Brown RE: Discussion Part One Dr. Brown, Healthy hemoglobin cells are flexible and can move easily through vessels, however sickle cells aren’t flexible and can stick to the walls of the vessels causing a blockage that prevents blood flow. Without blood and oxygen in the tissue, a person with sickle cell disease can have sudden pain attacks that can occur without any warning (What is Sickle Cell Disease, n.d.). What Is Sickle Cell Disease? (n.d.). Retrieved from http://www.nhlbi.nih.gov/health/health- topics/topics/sca/ 5/20/2016 8:06:04 PM Lorna Durfee Discussion Part One 5/16/2016 2:06:54 PM Discussion Part One (graded) A 17-year-old African-American from the inner city complains of severe chest and abdominal pain. Upon examination the attending physician performs and EKG, chest x-ray, and an abdominal and chest clinical examination and finds nothing. Assuming she is drug seeker, he sends her home. She comes back to the ER 4 hours later and now you see the patient. She explains that she was running track this past afternoon at school and that despite being very hot (100 F) she pushed on. Afterward, she starts feeling extensive pain in her chest and abdomen. She has jaundiced eyes, her blood pressure is 98/50, pulse is 112, T = 99.9 F, R = 28. The pain seems out of proportion to the physical findings. 0 What is your list of differential diagnoses in this case and explain how each of these fits with the case-patient as described above? Be sure to list at least four (4) pertinent differential diagnoses. Indicate which of these you would select as the most likely diagnosis and explain why. Now, as she is in the ER, she begins to exhibit stroke-like features. Does this change your differential? How do you treat this patient now? Are they any preventative actions that could have been taken? Subjective: A 17-year-old African-American from the inner city complains of severe chest and abdominal pain. She comes back to the ER 4 hours later and now you see the patient. She explains that she was running track this past afternoon at school and that despite being very hot (100 F) she pushed on. Afterward, she starts feeling extensive pain in her chest and abdomen. 0 Objective: She has jaundiced eyes, her blood pressure is 98/50, pulse is 112, T = 99.9 F, R = 28. The pain seems out of proportion to the physical findings. TESTS: EKG, chest x-ray, and an abdominal and chest clinical examination and finds nothing. Assuming, she is drug seeking he sends her home Assessment: Plan: DIFFERENTIALS#1: Sickle Cell Disease – Brandow and Liem (2011) state that Sickle Cell Disease is the most common inherited blood disorder in the United States. It affects about 89,000 Americans or 1 in 400 African Americans. The disorder is a result of hemoglobin structure that leads to chronic hemolytic anemia and complex chronic disease. The hallmark of this disease is the vasoocllusive painful event. Other symptoms can be fever, acute chest syndrome, priapism and ischemic stroke (Brandow & Liem, 2011, p. 1). Sickle Cell Disease is caused by the inheritance of the sickle beta-globin gene. This disorder is a disorder of hemoglobin structure and leads to a complex disease (Brandow & Liem, 2011, p. 2). Patients will have a chronic hemolytic anemia and can suffer from complications that arise that can be life-threatening. The acute sickling of red cells results in pain or organ dysfunction. These sickling events can cause damage to organs that is irreversible (Brandow & Liem, 2011, p. 2). Sickle cell anemia has an inherited characteristic that leads to the formation of abnormal hemoglobin. In hemoglobin S one amino acid in the pair of beta-globin chains is changed from normal glutamic acid to valine. When the altered hemoglobin becomes deoxygenated, it crystallizes and-and changes it’s the shape of the RBC from disk to Crescent. The cell membrane becomes damaged, and this leads to hemolysis and the cells have a shorter lifespan. Gould & Dyer, 2011, p. 256). Because of the changes in the RBCs, small blood vessels become obstructed, and this results in thrombus formation. There can be infarctions or areas where there is tissue necrosis. There can be severe crises when a patient is dehydrated, or there is an infection. Also, larger blood vessels can also be involved that affect the brains and other organs Gould & Dyer, 2011, p. 257). Hemolysis leads to hyperbilirubinemia, jaundice, and gallstones. #2: Hemolytic Anemia – The National Heart, Lung and Blood Institute (2014) tell us that with this condition the red blood cells are destroyed and removed from the bloodstream before their normal lifespan is over. These cells carry oxygen to the body. Red blood cells are formed in the bone marrow. They live for approximately 120 days and then die. When blood cells die, the marrow normally will replace them. However, in hemolytic anemia the marrow cannot make enough to meet the needs of the body. Anemia is when the blood has lower than normal amount of red blood cells. Hemoglobin is an iron-rich protein that carries oxygen from the lungs to the rest of the body. The anemia can make patients tired and can cause shortness of breath, dizziness, headache and chest pain. With hemolytic anemia, there can be jaundiced as the red blood cells die and release hemoglobin into the bloodstream. The hemoglobin breaks down into bilirubin, and that gives the eyes the yellowish color and skin. There can be a pain in the upper abdomen (The National Heart, Lung and Blood Institute, 2015). #3: Hepatitis A Infection - Lai and Chopra (2016) relate that hepatitis is inflammation of the liver. A virus causes hepatitis A. Hepatitis A is carried in the stool of infected people. A common way to spread the virus is when an infected person does not wash their hands after using the bathroom and touches food or another surface. This virus causes a short-lived illness. It presents as a mild flu-like illness. There is a period from infection to development of this disease, and it is approximately 30 days. The symptoms are feeling run down, fatigue, nausea, and vomiting. There is a lack of appetite and fever. As things progress the urine become dark- colored, stools are light in color and pain under the ribs on the right side of the abdomen (Lai & Chopra, 2016). #4: Hypertrophic Cardiomyopathy - Maron (2015) explains that hypertrophic cardiomyopathy is a genetic disease where the heart muscle become thick and hypertrophied. The heart cells are abnormal and not arranged in an organized pattern. There is fibrosis between cells. It can also affect the mitral valve causing regurgitation back into the left atrium causing higher pressure in the heart. The prevalence is 1 to 500 adults. However, the majority of cases are undiagnosed. The most common symptoms are chest pain, shortness of breath on exertion, tired and fatigued. The cause of this disorder is a mutation in one of the different genes which encode the structure of the heart. Not all the genes responsible have been identified. Symptoms can be present themselves during puberty. The symptoms are dyspnea on exertion, chest pain, fainting, palpitations. There is a lack of stamina and palpitations and lightheadedness (Maron, 2016).DIAGNOSIS: I chose this diagnosis as this patient presents with signs and symptoms of Sickle Cell Disease. She has fever and chest and abdominal pain. She may be dehydrated, and this can set off the sickle cell crisis as can infection. She also has jaundiced eyes. Patients can suffer from complications that arise that can be life-threatening. The acute sickling of red cells results in pain or organ dysfunction. These sickling events can cause damage to organs that is irreversible (Brandow & Liem, 2011, p. 2). She appears to be in crisis. Now, as she is in the ER, she begins to exhibit stroke-like features. ? Does this change your differential? My differential will not change. Larger vessels can be affected, and this could affect the brain. Also, she is now having stroke like symptoms. However, I do still feel that cardiomyopathy could be a problem as she is at the right age for development of this. I would have thought if she had Sickle Cell that it would have been evaluated before now. She has jaundice, and this makes the diagnosis of Sickle Cell fit. How do you treat this patient now? Are they any preventative actions that could have been taken? Field, Vichinsky and DeBaun (2016) provide the guideline for treatment. Prevention of complications of acute complications of Sickle Cell Disease includes health management with a hematologist or expert in the field of Sickle Cell Disease. The prevention could have included a thorough examination. Her immunizations need to be current. She could have been given fluids to prevent dehydration. Her symptoms of abdominal pain and chest pain should have been given further consideration as well as jaundice. There needs to be genetic testing. The use of folate could be considered. The patient needs to start a hydroxyurea treatment to reduce the sickle cell process. Also, she would need pain medication for the vaso-occlusive events and antibiotics for infection (Field, Vichinsky, & DeBaun, 2016). Furthermore, she needs to rehydrate with fluids and further assessment regarding the stroke symptoms. References Brandow, A. M., & Liem, R. (2011). Sickle cell disease in the emergency department: Atypical complications and management. Clinical Pediatric Emergency Medicine, 12(3), 202-212. Retrieved from http://doi.org/10.1016/j.cpem.2011.07.003 Field, J. J., Vichinsky, E. P., & DeBaun, M. R. (2016). Overview of the management and prognosis of sickle cell disease. Retrieved May 16, 2016, from http://www.uptodate.com/contents/overview-of-the-management-and-prognosis-of-sickle- cell-disease Gould, B. E., & Dyer, R. (2011). Blood and lymphatic disorders. In Pathophysiology for the health professions (4th ed., pp. 256,257). St. Louis, MO: Saunders/Elsevier. Lai, M., & Chopra, S. (2016). In T.W. Post (Ed.) UpToDate. Hepatitis A. Retrieved from http://www.uptodate.com/contents/hepatitis-a-beyond-the-basicsMaron, M. S. (2016). In T.W. Post (Ed.) UpToDate. Hypertrophic cardiomyopathy. Retrieved from http://www.uptodate.com/contents/hypertrophic-cardiomyopathy-beyond-the-basics The National Heart, Lung and Blood Institute. (2015). What Is Hemolytic Anemia? Retrieved from https://www.nhlbi.nih.gov/health/health-topics/topics/ha 5/17/2016 7:06:53 PM Instructor Brown reply to Lorna Durfee RE: Discussion Part One What is going on with Hepatitis A Infection to cause Flu like symptoms? Lorna Durfee reply to Instructor Brown RE: Discussion Part One 5/18/2016 2:31:42 PM What is going on with Hepatitis A Infection to cause flu-like symptoms? Dr. Brown: Rutherford (2015) states that the cause of Hepatitis A is an RNA virus that is single stranded called the picornavirus. Hepatitis A spreads by fecal-oral contact and can occur in areas where there is poor hygiene. In undeveloped countries, we see Hepatitis A spreads and is waterborne and can be food-borne. There have been cases from eating raw shellfish. The virus spread through fecal shedding, and this happens before symptoms develop, and this stops after the symptoms have already begun (Rutherford, 2015). Feng et al. (2014) tell us that unlike other picornaviruses, hepatitis A virus (HAV) is cloaked in the host membranes when released from cells. The membranes provide protection from the neutralizing antibodies and facilitate the spread of the infection into the liver (Feng et al., 2014, p. 169). Plasmacytoid dendritic cells (pDCs) play a critical role in host antiviral immunity. Their presence in the blood is usually low. However they migrate to sites of infection and when they are activated will produce large quantities of IFN-a (interferon). These pDCs also secrete pro-inflammatory cytokines, maturation markers, antigens, and this contributes to CD4 T-cell and B- cell development. These cells bridge innate and adaptive antiviral immunity. PDCs senses viruses via endosomal TLR7 and TLR9 and they can sense viral nucleic acids in the cytosol (Feng et al., 2014, p. 169). Hepatitis A virus (HAV) causes moderate to severe acute inflammatory liver injury (Feng et al., 2014, p. 169). Murray, Rosenthal, and Pfaller (2016) explain that the goal for immune response is to eliminate the virus and the host cells that harbor or replicate the virus. For viruses, interferon and cytokines start the response. The inflammatory response does not activate until T cells become activated (Murray, Rosenthal, & Pfaller, 2016, p. 84). When a patient feels flu-like symptoms, it is the immune system that comes into action. There is an increase in body temperature with fever. The body does this to limit replication of the virus. As stated before, the release of cytokines and interferon from the infected cells trigger local and systemic response. Feeling sick with fever is a response the body takes when infected with Hepatitis A to launch an immune response to stop the infection (Murray, Rosenthal, & Pfaller, 2016, p. 84). ReferencesFeng, Z., Li, Y., McKnight, K. L., Hensley, L., Lanford, R. E., Walker, C. M., & Lemon, S. M. (2014). Human pDCs preferentially sense enveloped hepatitis A virions. Journal of Clinical Investigation, 125(1), 169-176. doi:10.1172/jci77527 Murray, P. R., Rosenthal, K. S., & Pfaller, M. A. (2016). Medical Microbiology (8th ed.), (p.84). Philadelphia, PA: Elsevier. Rutherford, A. E. (2015). Hepatitis A, Acute. In Merck manual online. Retrieved from https://www.merckmanuals.com/professional/hepatic-and-biliary- disorders/hepatitis/hepatitis-a,-acute Brooke Lobianco The Case of Jane Doe Patient: Jane Doe – 17-year-old female Chief Complaint: Extensive chest and abdominal pain Medical History Medications -N/A -N/ A Allergies - N/A 5/16/2016 7:14:25 PM Surgical History - N/A Social History - N/A Vitals: B/P- 98/50 HR- 112 RR- 28 Temp- 99.9 F Physical Exam: Patient is tachypenic, tachycardic and hypotensive with jaundiced sclera. 1. Sickle Cell Disease with Associated Crisis Sickle cell crisis fits the presentation based on Jane’s symptoms of extensive chest and abdominal pain, increased effort of breathing and jaundiced sclera. The fact that the patient is African American also supports the diagnosis of sickle cell disease as the sickle cell trait occurs among about 1:12 blacks (McCance, Huether, Brashers & Rote, 2013). Jane was also just out running which can lead to dehydration thus increasing her risk for inducing a sickle cell crisis. Sickle cell crisis may not fit the presentation based on the patient’s advanced age as sickle cell disease is normally diagnosed earlier in life. Sickle cell disease (SCD) is a group of inherited disorders characterized by the presence of an abnormal form of hemoglobin within the erythrocytes. Patients who have SCD inherit two abnormal hemoglobin genes, one from each parent. In all forms of SCD, at least one of the two abnormal genes causes a person’s body to make hemoglobin S. When a person has two hemoglobin S genes, Hemoglobin SS, the disease is called sickle cell anemia. This is the most common and often most severe kind of SCD (McCance et al., 2013). Red blood cells that contain normal hemoglobin are disc shaped (like a doughnut without a hole). This shape allows the cells to be flexible so that they can move through large and small blood vessels to deliver oxygen. Sickle hemoglobin is not like normal hemoglobin. It can form stiff rods within the red cell, changing it into a crescent, or sickle shape. Sickle-shaped cells are not flexible and can stick to vessel walls, causing a blockage that slows or stops the flow of blood. When this happens, oxygen can’t reach nearby tissues (Vasaikar et al., 2015). Clinical manifestations of a sickling may include pallor, fatigue, jaundice and irritability (McCance et al., 2013). Extensive sickling can precipitate four types of crises: vaso-occlusive, aplastic, sequestration, and hyperhemolytic. Based on the patient’s presentation it is reasonable to believe that she is currently in a vaso-occlusive sickle cell crisis. Vaso- occlusive crisis may develop spontaneously and is extremely painful (McCance et al., 2013). The patient is tachypenic and tachycardic which may indicate acute chest syndrome. Acute chest syndrome is the second-most common complication and it accounts for about 25% of deaths in patients with SCD (Vasaikar et al., 2015). Sickled RBCs attach to the endothelium of the injured, underventilated, and inflamed lung and, as they fail to be reoxygenated, will eventually undergo additional inflammation and lung infarction (McCance et al., 2013).2. Cholelithiasis With Associated Cholangitis Cholelithiasis with acute cholangitis fits the presentation based on the patient’s symptoms of abdominal pain, jaundice and mildly septic state. Typically patients who have cholelithiasis (gallstones) are asymptomatic. Gallstones are commonly two types: cholesterol and pigmented. Cholesterol gallstones form in bile that is supersaturated with cholesterol produced by the liver. It is unknown why the hepatocytes secrete bile that is supersaturated with cholesterol. Pigmented stones are either black or brown in nature. Black stones are associated with hyperbilirubinbilia and hemolytic diseases such as sickle cell. Brown stones are associated with bacterial infection of the bile ducts (McCance et al., 2013). In some cases, gallstones can cause biliary obstruction leading to inflammation and infection in the bile ducts. Infection is presumably acquired from bacteria ascending from the biliary junction with the duodenum (McCance et al., 2013). Typical clinical presentation of cholangitis includes right upper quadrant abdominal pain, fever and jaundice (Abraham, Rivero, Erlikh, Griffith, & Kondamudi, 2014). Although Jane is not clearly septic, which is normally seen with cholangitis, she does present with elevated temperature, mild hypotension and tachycardia, and tachypnea. Jane’s abdominal pain presents as diffuse with associated chest pain, which is not necessarily seen with cholangitis. 3. Autoimmune Hepatitis A likely differential diagnosis for Jane, is that of autoimmune hepatitis. Autoimmune hepatitis is a chronic disease in which the body’s immune system attacks the normal cells of the liver, causing inflammation and liver damage, which could result in cirrhosis of the liver and liver failure (NIH, 2014). Cirrhosis occurs as scar tissue replaces healthy liver tissue, blocking the normal flow of blood through the liver (NIH, 2014). Those who have pre- existing autoimmune disorders and a genetic predisposition are at greater risk (NIH, 2014). This etiology is most common in female age 20-40 years of age, making up 70% of reported cases (Goroll & Mulley, 2014). While some patients are asymptomatic, acute forms may present with jaundice of the skin and eyes, fever, amenorrhea, anorexia, and abdominal pain over the liver area (Goroll & Mulley, 2014). Diagnosis is confirmed by blood tests and liver biopsy, with an expected rise in blood gamma globulin, aminotransferase, and heightened titers of antinuclear antibody and antibody to smooth muscle (Goroll & Mulley, 2014). Treatment is typically a combination of immunosuppressants and steroids (NIH, 2014). Prednisone is a steroid utilized for inflammation reduction and immunosuppression, while immunosuppressants such as azathioprine are used to prevent the immune system from attacking itself (NIH, 2014). It is important to note, however, that progression of this condition will likely require a liver transplant (NIH, 2014). Jane’s symptoms immolate this condition, as she does present with abdominal pain, and jaundice. What makes this diagnosis questionable is Jane’s lack of reported anorexia, nausea, amenorrhea, and fever. This diagnosis also does not explain Jane’s chest pain. However, Jane’s temperature is 99.9 degrees Fahrenheit, which can be interpreted as the high end of normal. 4. Acute Appendicitis With Jane’s clinical presentation of abdominal pain, a differential diagnosis of acute appendicitis is one to consider. Acute appendicitis is due to mucosal ischemia of the appendix, which results from ongoing mucous secretion distal to an obstruction of the lumen of the appendix (Memon, Khatoon, Memon, & Junejo, 2015). The collection of mucous within the lumen leads to vein compression, which results in increased luminal pressure and subsequent thrombosis and engorgement of the appendiceal venules (Memon et al., 2015). 70-80% of diagnosis is based upon the clinical signs and symptoms associated with the condition. These symptoms consist of colicky peri- umbicular pain that radiates and eventually localizes near the right iliac fossa (Memon et al., 2015). Additional symptoms include nausea, vomiting, local peritonitis, and muscle guarding (Memon et al., 2015). Although Jane does have abdominal pain, the diagnosis of acute appendicitis would not explain her chest pain or her jaundice. Additionally, this condition is more common in males, age 13 years-20 years (Goroll & Mulley, 2014), a description that Jane does not fit. ***Jane’s demonstration of stroke symptoms does not change my primary differential, as sickle cell disease predisposes victims to heightened chances of infarction. The main focus turns to preserving her brain tissue. In stroke incidences, the medical team would collaborate to determine if a patient is a candidate for receiving tissue plasminogen activator (tPA) to dissolve the blood clot causing the stroke. With Jane’s primary differential diagnosis being sickle cell disease with associated crisis, her situation is unique and is not covered by an abundance of research.In a particular event, a 45 y/o African-Caribbean female with sickle cell disease underwent an elective angiogram for evaluation of a pre-existing un-ruptured intra-cavernous aneurysm (Ganesalingam, Redwood, & Jenkins, 2013). During the procedure, this patient suffered a right MCA stroke and was given tPA within 60 minutes of symptom onset (Ganesalingam et al., 2013). She subsequently demonstrated clinical and hematological evidence of sickle cell crisis, leading to a necessary manual exchange transfusion within several hours of thrombolysis (Ganesalingam et al., 2013). The benefits of this particular patient receiving tPA were unclear, as there is extremely limited literature pertaining to this topic. With Jane being so young and otherwise seemingly healthy, tPA should be administered. Unfortunately, Jane’s diagnosis comes equipped with non-modifiable risk factors, mainly her African American descent. However, Jane was running in the extreme heat. A preventative measure includes maintaining hydration and adequate oxygenation. Perhaps when running, Jane needs to take more frequent breaks. References Abraham, S., Rivero, H. G., Erlikh, I. V., Griffith, L. F., & Kondamudi, V. K. (2014). Surgical and nonsurgical management of gallstones. American Family Physician, 89(10), 795-802 8p. Autoimmune Hepatitis. (2014). Accessed: http://www.nih.gov. Ganesalingam, J., Redwood, R., & Jenkins, I.H. (2013). Thrombolysis of an acute stroke presentation with an incidental unruptured aneurysm. JRSM Cardiovascular Disease, 2(1), 1-4. Goroll, A.H. & Mulley, A.G. (2014). Evaluation of nausea and vomiting. In A.H. Goroll & A.G. Mulley (Eds.), Primary Care Medicine: Office Evaluation and Management of the Adult Patient (7 ed.) (pp. 492). Philadelphia, PA: Wolters Kluwer. th Goroll, A.H., & Mulley, A.G. (2014). Management of hepatitis. In A.H Goroll & A.G. Mulley (Eds.), Primary Care Medicine: Office Evaluation and Management of the Adult Patient (7 ed.) (pp. 564). Philadelphia, PA: Wolters Kluwer. th McCance, K. L., Huether, S. E., Brashers, V. L., & Rote, N. S. (2013). Pathophysiology: The biologic basis for disease in adults and children (7th ed.). St. Louis, MO: Mosby. Memon, A.S., Khaton, S., Memon, R.A., & Junejo, A. (2015). Acute Appendicitis. Professional Medical Journal, 22 (9), 1212-1216. Vasaikar, N. B., Sonawane, S., Patil, S., Borse, L. B., Borse, S. L., & Pawar, S. P. (2015). A review on sickle cell anemia. Pharma Science Monitor, 6(2), 118-132. Lanre Abawonse Discussion Part One 5/16/2016 9:46:29 PM Sickle cell crisis. This can mean a lifetime of pain when it occurs (Dorman, 2005). The sickle cell crisis is caused by sickled hemoglobin. Hemoglobin S, in the absence of oxygen, polymerizes, forming rods that develop the sickle or crescent shape of the red blood cell (Yontz, & Waller, 2011). The autosomal recessive disorder results in abnormalities of the globin genes of the hemoglobin molecule of the red blood cells. Dorman (2005) noted that when a trigger, such as dehydration, infection, high altitude, strenuous exercise, or cold weather causes the RBCs to release oxygen, HbS forms long polymers, changing thenormally round and pliable RBCs into stiff cells with a crescent, or sickle, shape. This disorder is common among African Americans who have sickle cell disease (Farhat, & Junsanto-Bahri, 2016). These risk factors and symptoms are consistent with the patient’s presenting symptoms, making this a likely diagnosis. Hemolytic anemia(HA) It is known that HAs are a group of disorders characterized by premature red blood cell (RBC)destruction (less than 120 days) that exceeds the compensatory capacity of the bone marrow to increase RBC production and keep up with the loss (Ruiz & Cervantes, 2015). This occurs when there are too few red blood cells in the body as a result of premature destruction of red blood cells. Destruction occurs more quickly than the bone marrow can produce new cells (Green, 2015). In addition to the classic symptoms of anemia (paleness, fatigue, dyspnea, palpitations), findings can include familial or personal history of jaundice. Decreased oxygen to tissues can cause “fatigue, dyspnea, syncope, angina, compensatory tachycardia, and organ dysfunction” (McCance, Huether, Brashers, & Rote, 2013, p. 1000). Based on the patient’s symptoms, there is a possibility that the patient has inherited an intrinsic defect that is seen in a deficiency of glucose 6-phosphate dehydrogenase (G6PD) that is triggered by an external factor (Ruiz & Cervantes, 2015). Cholelithiasis is an inflammation of the gallbladder wall; it may be either acute or chronic. Hung, et al., (2011) noted that cholelithiasis is one of the most common disorders among patients presenting to emergency rooms with abdominal discomfort–e.g., epigastric pain, nausea, vomiting, loss of appetite. One of the classic symptom is the intense pain in the right upper quadrant that may radiate to the right scapula, which is known as biliary colic. This patient is acutely ill and if obstruction is present, the patient may complain of fever, clay-colored stool, tea colored urine, jaundice, nausea and vomiting (Gore, 2013). Cholelithiasis is a possible diagnosis due to patient’s present condition of abdominal pain, fever and jaundice. Heat exhaustion. Heat has various effects on the human body, including several well-recognized acute heat illness syndromes, exacerbations of chronic lung and cardiovascular disease, and multiple other syndromes associated with dehydration (Hess, Saha, and Luber, 2014). Heat exhaustion can lead to acute heat illness resulting primarily from diminished heat dissipation capacity, classically from environmental exposure, although exertion may be a factor. During the period of exercise, the body’s heat production is over generated leading to sweatproduction. As the body tries to compensate for the loss of water, physical symptoms that include fatigue, weakness, dizziness, nausea, vomiting, headache, tachycardia, hypotension, increased thirst, and profuse sweating can occur. However, there is no clear explanation for a relationship of jaundice with possible heat exhaustion. Now, as she is in the ER she begins to exhibit stroke like features. ? Does this change your differential? There are numerous problems that can develop with a patient in sickle cell crisis. I have personally witnessed stroke like symptom during the crisis time as well as bed wetting and impaired mentation. With the new presenting stroke like symptoms, it will not change the sickle cell diagnosis. The crisis may be preceded by a stressor such as dehydration which was evidenced with the exercising when the symptom developed. High altitude can also trigger the crisis, and patient can get irritable, have severe pain, fever, and hyperhemolytic crisis. Complications can include stroke, bacterial infection, sequestration of the spleen, avascular necrosis, leg ulcers, end-organ damage, renal disease, hypertension, acute chest syndrome, and priapism (Yontz & Waller, 2011). How do you treat this patient now? Are they any preventative actions that could have been taken? Sickle cell crisis can interrupt life adaptability. Therefore, the patient should be informed to avoid the causes of crisis such as dehydration, infection, hypoxia, high altitudes, vigorous exercise and stress. Genetic testing can help identify mutant genes and other treatments for this patient should include non-opiod analgesics, analgesia (morphine sulfate, meperidine, and ketorolac), hydration and oxygen if needed (Yontz & Waller, 2011). Reference Dorman, K. (2005). Sickle cell crisis! Managing the pain. Rn, 68(12), 33-2p 6p. Farhat, R. & Junsanto-Bahri, T. (2016). Anemia, sickle cell. In F. J. Domino (Ed.), The 5-minute clinical consult 2016 (24th ed., pp. 54-55). Philadelphia: Wolters Kluwer Health/Lippincott Williams & Wilkins. Green, K. O. (2015). Hemolytic anemia. Salem Press Encyclopedia Of Health, Hess, J. J., Saha, S., & Luber, G. (2014). Summertime Acute Heat Illness in U.S. Emergency Departments from 2006 through 2010: Analysis of a Nationally Representative Sample. Environmental Health Perspectives, 122(11), 1209-1215 7p. doi:10.1289/ehp.1306796Hung, S., Liao, K., Lai, S., Li, C., & Chen, W. (2011). Risk factors associated with symptomatic cholelithiasis in Taiwan: a population-based study. BMC Gastroenterology, 11111. doi:10.1186/1471-230X-11-111 Ruiz, E. F., & Cervantes, M. A. (2015). Diagnostic approach to hemolytic anemias in the adult. Revista Brasileira De Hematologia E Hemoterapia, 37(6), 423-425. doi:10.1016/j.bjhh.2015.08.008 Yontz, C. J., & Waller, K. V. (2011). Homozygous sickle cell anemia and secondary complications: a case study. Clinical Laboratory Science, 24(2), 78-84 7p. Liberty Neoh Discussion Part One 5/17/2016 12:28:02 AM Dr. Brown and Class, Based from the symptoms the patients were experiencing, I would say that the patient has a sickle cell crisis. Sickle cell crisis is responsible for the high rate of morbidity and mortality in Sickle Cell Disease (SCD). It is a major hematological genetic disorder where oxygenated Hemoglobin A (HbA) and Hemoglobin S (HbS) has the same solubility, but on deoxygenation, HbS is about 100 times less soluble. The deoxygenated Hemoglobin S polymerizes by clumping to form long strands, which stretches the membrane of the red blood cell. This defect causes polymerization of hemoglobin molecules and erythrocyte sickling under hypoxic conditions leading to sickle cell crisis caused by sickled red blood cells which obstruct capillaries and restrict blood flow to an organ, resulting in ischemia, pain, and organ damage (Antwi-Boasiako et al, 2015). There has been increasing evidence that asthma predisposes to certain complications of sickle cell disease including acute painful crises, acute chest syndrome, pulmonary hypertension and stroke. Acute pain in the right upper quadrant is common in sickle cell patients. Pain that is different from previous pain experiences, more importantly, when accompanied by more jaundice than usual and nausea and vomiting, then further hepatobiliary workup is needed. Increasing nausea and vomiting with food points to the gallbladder. Colic pains point to the gallbladder. Right upper quadrant fullness with dull pains points to the liver. General jaundice may be associated to gallbladder and liver disease (Ballas et al, 2012).Dehydration can occur due to uninterrupted strenuous exercise in a hot environment and can alter the body's homoeostatic mechanisms. (Hoxha et al, 2015). The authors also stated, “The volume of consumed fluids should be greater than the volume of sweat lost in order to make provision for the ongoing obligatory urine losses.” There are factors in team sports, that can affect the internal mechanism that regulate fluid intake. Namely, loss of fluid, fluid availability, the ability to intake the liquid, palatability liquid, gastrointestinal comfort, awareness of the hypohydration problems, fear of weight gain, and fear of urge to urinate (Hoxha et al, 2015). Although the patient in our case study reported having severe abdominal pain, the other symptoms did not quite match this diagnosis. Heat exhaustion was the reason for a substantial number of emergency room visits in the United States each year and the frequency of these presentations is correlated with extremely high heat weather events. Predictors of adverse outcomes include chronic disease burden, especially chronic hematological conditions, ages 18 years or younger or 65 years or older, male gender, living in low-income or rural areas, and residing in the West, South, or Midwest. Also the data reinforce current evidence about the impact of illness due to extreme weather events and that these findings may help community education and relief efforts to mitigate the adverse effects of climate change on public health (Thien Mac, V. 2015). Patients who are hospitalized due to heat exhaustion suffered mostly with fluid and electrolytes imbalance. The patient in our case study does not seem to have this health issue. Pulmonary embolism (PE) is an obstructive disease of the pulmonary arterial system occurring in different stages and locations. It is commonly caused by the embolization of thrombus originating from the deep veins