Feb 23, 2024 NURS 6501 Knowledge Check Concepts of Pediatrics
NURS 6501 Knowledge Check Concepts of Pediatrics
NURS 6501 Knowledge Check Concepts of Pediatrics
Scenario 3: Hemophilia
8-month infant is brought into the office due to a swollen right knee and excessive bruising. The parents have noticed bruising about a month ago but thought the bruising was due to the attempts to crawl. They became concerned when the baby woke up with a swollen knee. Infant up to date on all immunizations, has not had any medical problems since birth and has met all developmental milestones.
FH: negative for any history of bleeding disorders or other major genetic diseases.
PE: within normal limits except for obvious bruising on the extremities and right knee. Knee is swollen but no warmth appreciated. Range of motion of knee limited due to the swelling.
DIAGNOSIS: hemophilia A.
Question
1. What is the pathophysiology of Hemophilia
Your Answer:
Hemophilia is a genetic disorder that affects the blood’s ability to clot properly, leading to prolonged bleeding and difficulty in forming stable blood clots. The disorder is caused by a deficiency or dysfunction of specific clotting factors in the blood. There are different types of hemophilia, and in this scenario, the diagnosis is hemophilia A, which is characterized by a deficiency of clotting factor VIII.
Pathophysiology of Hemophilia A
Normal Blood Clotting Cascade: In a normal blood clotting process, there is a complex cascade of reactions that involves a series of clotting factors, which are proteins produced by the liver. These factors work together in a stepwise manner to form a stable blood clot, preventing excessive bleeding when there is an injury or damage to blood vessels.
Clotting Factor VIII Deficiency: In hemophilia A, there is a deficiency or dysfunction of clotting factor VIII. Factor VIII is essential for the activation of another clotting factor, factor X, which is a crucial step in the clotting cascade. Without sufficient factor VIII, the clotting cascade is disrupted, leading to impaired blood clot formation.
Prolonged Bleeding: Due to the deficiency of factor VIII, individuals with hemophilia A experience prolonged bleeding after injuries, surgeries, or even minor trauma. Small blood vessels are unable to form stable clots, leading to excessive bleeding and bruising. Bleeding can occur both externally and internally into joints, muscles, and other tissues.
Spontaneous Bleeding: In some cases, bleeding can occur spontaneously without apparent cause, as seen in the scenario where the infant woke up with a swollen knee. This is especially common in more severe cases of hemophilia.
Joint and Muscle Bleeding: Repeated bleeding episodes, especially into joints and muscles, can lead to chronic inflammation and damage. The repeated bleeding causes irritation and inflammation in these areas, leading to pain, swelling, and limited joint movement.
Treatment: Hemophilia A is managed by replacing the deficient clotting factor VIII. This can be done through intravenous infusions of clotting factor concentrates. Prophylactic treatment may be prescribed to prevent bleeding episodes. Additionally, patients and their families are often educated about managing bleeding risks and preventing complications.
An 11-year-old boy is brought to the clinic by his parents who states that the boy has not been eating and listless. The mother also notes that he has been easily bruising without trauma as he says he is too tired to go out and play. He says his bones hurt sometimes. Mother states the child has had intermittent fevers that respond to acetaminophen.Maternal history negative for pre, intra, or post-partum problems.PMH: Negative. Easily reached developmental milestones.PE: reveals a thin, very pale child who has bruises on his arms and legs in no particular pattern.LABS: CBC revealed Hemoglobin of 6.9/dl, hematocrit of 19%, and platelet count of 80,000/mm3. The CMP demonstrated a blood urea nitrogen (BUN) of 34m g/dl and creatinine of 2.9 mg/dl.DIAGNOSIS: acute leukemia and renal failure and immediately refers the patient to the Emergency Room where a pediatric hematologist has been consulted and is waiting for the boy and his parents.CONFIRMED DX: acute lymphoblastic leukemia (ALL) was made after extensive testing.
Question
1. Explain what ALL is?
Selected Answer: Acute lymphocytic leukemia (ALL) is also called acute lymphoblastic leukemia. Acute means that leukemia can progress quickly and, if not treated, would probably be fatal within a few months. Lymphocytic means it develops from immature forms of lymphocytes, a type of white blood cell. ALL is a malignant, clonal disease of the bone marrow in which early lymphoid precursors proliferate and replace the normal hematopoietic cells of the marrow. In most cases, the leukemia cells invade the blood quickly. They can also sometimes spread to other parts of the body, including the lymph nodes, liver, spleen, central nervous system, and testicles (in males). Acute lymphocytic leukemia is the most common type of cancer in children, and treatments result in a good chance of a cure. Acute lymphocytic leukemia can also occur in adults, though the chance of a cure is greatly reduced. Signs and symptoms of acute lymphocytic leukemia may include: bleeding from the gums, bone pain, fever, frequent infections, frequent or severe nosebleeds, lumps caused by swollen lymph nodes in and around the neck, armpits, abdomen or groin, pale skin, shortness of breath, weakness, fatigue or a general decrease in energy.
Correct Answer:
Acute lymphoblastic leukemia (ALL) is a malignant (clonal) disease of the bone marrow in which
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NURS 6501 Knowledge Check Concepts of Pediatrics
early lymphoid precursors proliferate and replace the normal hematopoietic cells of the marrow. ALL is the most common type of cancer and leukemia in children in the United States. The malignant cells of acute lymphoblastic leukemia (ALL) are lymphoid precursor cells (ie, lymphoblasts) that are arrested in an early stage of development. This arrest is caused by an abnormal expression of genes, often as a result of chromosomal translocations or abnormalities of chromosome number. These aberrant lymphoblasts proliferate, reducing the number of the normal marrow elements that produce other blood cell lines (red blood cells, platelets, and neutrophils). Consequently, anemia, thrombocytopenia, and neutropenia occur, although typically to a lesser degree than is seen in acute myeloid leukemia. Lymphoblasts can also infiltrate outside the marrow, particularly in the liver, spleen, and lymph nodes, resulting in enlargement of the latter organs.Response Feedback: [None Given]
In this exercise, you will complete a 10- to 20-essay type question Knowledge Check to gauge your understanding of this module’s content.
Possible topics covered in this Knowledge Check include:
Growth and development
Normal growth patterns
Scoliosis (ortho)
Kawasaki
Alterations in children
Congenital (heart syndrome)
PDAs
Sudden Infant Death Syndrome (SIDS)
Asthma
Lead poisoning and effects on neurological functioning
Sickle cell
Hemophilia
Photo Credit: laflor / E+ / Getty Images
(Note: It is strongly recommended that you take the Knowledge Check at least 48 hours before taking the Final Exam.)
Complete the Knowledge Check By Day 5 of Week 11
To complete this Knowledge Check:
Module 8 Knowledge Check
Final Exam
This 101-question exam is a test of your knowledge in preparation for your certification exam. No outside resources, including books, notes, websites, or any other type of resource, are to be used to complete this exam. You are expected to comply with Walden University’s Code of Conduct.
This exam will be on topics covered in Weeks 7, 8, 9, 10, and 11. Prior to starting the exam, you should review all of your materials. This exam is timed with a limit of 2 hours for completion. When time is up, your exam will automatically submit.
(Note: It is strongly recommended that you take the Knowledge Check at least 48 hours before taking the Final Exam.)
Photo Credit: Getty Images
By Day 7 of Week 11
Complete and submit your Final Exam.
To complete your exam:
Final Exam
What’s Coming Up?
Congratulations! After you have finished all of the assignments for this week, you have completed the course. Please submit your Course Evaluation by the end of the week.
Week 11: Concepts of Pediatrics
Pediatric disorders can present unique challenges to patients, families, and healthcare providers. Disorders in these areas are complicated by the fact that young patients can have difficulties communicating symptoms. Furthermore, the manner in which disease and disorders manifest in children may be unique.
APRNs working to support these patients and their loved ones must demonstrate not only support and compassion, but expertise to communicate and guide understanding of diagnoses and treatment plans. This includes an understanding of disease and disorders at the pediatric level.
This week, you examine pathophysiology in pediatrics. You apply key terms, concepts, and principles in this area to demonstrate an understanding of the impact they have on altered physiology in children.
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Learning Objectives
Students will:
Analyze concepts and principles of pathophysiology across the lifespan
Learning Resources
Required Readings (click to expand/reduce)
McCance, K. L. & Huether, S. E. (2019). Pathophysiology: The biologic basis for disease in adults and children (8th ed.). St. Louis, MO: Mosby/Elsevier.
Chapter 14: Cancer in Children, including Summary Review
Chapter 20: Alterations of Neurologic Function in Children (stop at Childhood tumors); Summary Review
Chapter 34: Alterations of Cardiovascular Function in Children (stop at Defects decreasing pulmonary blood flow); Summary Review
Chapter 37: Alterations of Pulmonary Function in Children (stop at Congenital malformations); Summary Review
Chapter 40: Alterations of Renal and Urinary Tract Function in Children, including Summary Review
Chapter 43: Alterations of Digestive Function in Children, including Summary Review
Chapter 46: Alterations of Musculoskeletal Function in Children (stop at Avascular diseases); (start at Cerebral palsy) (musculoskeletal tumors in children); Summary Review
Chapter 48: Alterations of the Integument in Children, including Summary Review
Chapter 50: Shock, Multiple Organ Dysfunction Syndrome, and Burns in Children, including Summary Review
U.S. National Library of Medicine. (2019). Normal growth and development. Retrieved from https://medlineplus.gov/ency/article/002456.htm
Document: NURS 6501 Final Exam Review (PDF document)
Note: Use this document to help you as you review for your Final Exam in Week 11.
Module 8 Overview with Dr. Tara Harris
Dr. Tara Harris reviews the structure of Module 8 as well as the expectations for the module. Consider how you will manage your time as you review your media and Learning Resources throughout the module to prepare for your Knowledge Check and your Final Exam. (3m)
Pediatrics – Week 11 (14m)
Wyatt, K. (2018, February 4). Pediatrics – Growth and development milestones review [Video file]. Retrieved from https://www.youtube.com/watch?v=ZG60nC3RJwc
Note: The approximate length of the media program is 34 minutes.
Online Media from Pathophysiology: The Biologic Basis for Disease in Adults and Children
In addition to this week’s media, it is highly recommended that you access and view the resources included with the course text, Pathophysiology: The Biologic Basis for Disease in Adults and Children. Focus on the videos and animations in Chapter 20, 34, 37, 40, 43, and 46 that relate to alterations in hematological function in children. Refer to the Learning Resources in Week 1 for registration instructions. If you have already registered, you may access the resources at https://evolve.elsevier.com/
Question 5
4 out of 4 points
Scenario 3: Hemophilia
8-month infant is brought into the office due to a swollen right knee and excessive bruising. The parents have noticed bruising about a month ago but thought the bruising was due to the attempts to crawl. They became concerned when the baby woke up with a swollen knee. Infant up to date on all immunizations, has not had any medical problems since birth and has met all developmental milestones.
FH: negative for any history of bleeding disorders or other major genetic diseases.
PE: within normal limits except for obvious bruising on the extremities and right knee. Knee is swollen but no warmth appreciated. Range of motion of knee limited due to the swelling.
DIAGNOSIS: hemophilia A.
Question
1. What is the pathophysiology of Hemophilia
Selected Answer:
Hemophilia is the most prevalent severe hereditary bleeding disorder and is characterized by the inability to form thrombi in response to injury, resulting in continuous bleeding. Both hemophilia A and B result from mutations in the F8 gene and F9 gene. Changes or mutations of the genes result in deficiency or dysfunction of clotting factors VIII and IX, respectively. Specifically, “inversions in introns 1 and 22 of the factor VIII gene are the most frequently observed mutations and account for most severe cases of hemophilia A” . Another type of mutation that may result in hemophilia is a point mutation. In this instance, a single nucleotide in the DNA is added, deleted, or changed. When these alterations take place, the amino acid chain is typically destroyed. Otherwise, the protein chain can disrupt protein function, inhibit intracellular processing, or result in protein clearance.
Correct Answer:
Hemophilia A is caused by an inherited or acquired genetic mutation that results in dysfunction or deficiency of factor VIII, or by an acquired inhibitor that binds © 2020 Walden University 5 factor VIII. Of genetic cases, up to approximately one third are the result of de novo mutations not present in the mother’s X chromosome. Inadequate factor VIII results in the insufficient generation of thrombin by the FIXa and FVIIIa complex by means of the intrinsic pathway of the coagulation cascade. This mechanism, in combination with the effect of the tissue-factor pathway inhibitor, creates an extraordinary tendency for impaired clotting in response to trauma and, especially in persons with severe hemophilia, with spontaneous bleeding.
Response Feedback:
[None Given]
Scenario 1: Acute Lymphoblastic Leukemia (ALL)
Question 1: Explain what ALL is.
ALL is a type of blood and bone marrow cancer characterized by the production of too many lymphocytes by the bone marrow. The characteristic feature of ALL is chromosomal abnormalities and genetic changes involved in the differentiation and proliferation of lymphoid precursor cells (Malard & Mohty, 2020). The pathogenesis of ALL entails abnormal proliferation and differentiation of a clonal population of lymphoid cells. It is classified into L1, L2, and L3. L1 is the most common form found in children and has the best prognosis (Malard & Mohty, 2020). L2 is the most frequent ALL found in adults. L3 is the rarest form of ALL. Common symptoms include pallor, fatigue, weakness, fever, weight loss, abnormal bleeding and bruising, and lymphadenopathy.
References
Malard, F., & Mohty, M. (2020). Acute lymphoblastic leukemia. Lancet (London, England), 395(10230), 1146–1162. https://doi.org/10.1016/S0140-6736(19)33018-1
Question 2: Why does ARF occur in some patients with ALL?
Acute kidney injury and acute renal failure (ARF) are documented complications of ALL. Kidney infiltration is prevalent in hematologic malignancies like ALL and occurs in 60-90% of patients. Rose et al. (2019) explain that acute kidney injury is seen in some patients with ALL and is caused by leukemic infiltration. Leukemic infiltrates are caused by uric acid nephropathy that results in renal enlargement and ARF. ARF in patients with ALL is considered to be caused by acute tubular compression and microvasculature disruption, causing acute tubular necrosis (Prada Rico et al., 2020). The symptoms linked with kidney infiltration secondary to ALL include flank pain, hematuria, and frothy urine.
References
Prada Rico, M., Rodríguez-Cuellar, C. I., Arteaga Aya, L. N., Nuñez Chates, C. L., Garces Sterling, S. P., Pierotty, M., González Chaparro, L. E., & Gastelbondo Amaya, R. (2020). Renal involvement at diagnosis of pediatric acute lymphoblastic leukemia. Pediatric reports, 12(1), 8382. https://doi.org/10.4081/pr.2020.8382
Rose, A., Slone, S., & Padron, E. (2019). Relapsed Acute Lymphoblastic Leukemia Presenting as Acute Renal Failure. Case reports in nephrology, 2019, 7913027. https://doi.org/10.1155/2019/7913027
Question 3: Explain the pathophysiology of acute SCD crisis. Why is pain the predominate feature of acute crises?
SCD crisis is characterized by a throbbing, sharp pain with a sudden onset. The common pain sites are joints, the lower back, and the extremities. Darbari et al. (2020) explain that the SCD crisis is a multifactorial process involving the occlusion of small blood vessels by sickled red blood cells and adherent blood cells. It also involves thrombosis, large-vessel intimal hyperplasia, and embolization of bone marrow fat, contributing to hypoxia, ischemia, and tissue inflammation and damage. The combination of hypoxia, ischemic tissue damage, and inflammation makes SCD crisis pain distinctive. Accumulation of sickled red blood cells and other adherent cells causes vaso-occlusion in smaller vessels without an inflammatory trigger (Jang et al., 2021). Furthermore, ischemia-reperfusion injury caused by microvascular occlusions leads to chronic inflammation through increased production of oxidants and increased leukocyte adhesion, which further causes SCD crisis and tissue damage.
References
Darbari, D. S., Sheehan, V. A., & Ballas, S. K. (2020). The vaso‐occlusive pain crisis in sickle cell disease: definition, pathophysiology, and management. European Journal of Hematology, 105(3), 237–246. https://doi.org/10.1111/ejh.13430
Jang, T., Poplawska, M., Cimpeanu, E., Mo, G., Dutta, D., & Lim, S. H. (2021). Vaso-occlusive crisis in sickle cell disease: a vicious cycle of secondary events. Journal of Translational Medicine, 19(1), 1–11. https://doi.org/10.1186/s12967-021-03074-z
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