NURS 6501 Knowledge Check Pediatrics

NURS 6501 Knowledge Check Pediatrics

NURS 6501 Knowledge Check Pediatrics

Question 1

4 out of 4 points

	Scenario 1: Acute Lymphoblastic Leukemia (ALL) 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 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]

·  Question 2

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	Scenario 1: Acute Lymphoblastic Leukemia (ALL) 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.     Why does ARF occur in some patients with ALL?
	Selected Answer: Renal injury in ALL is common and can occur through many different mechanisms, including prerenal acute kidney injury, acute tubular necrosis, reno-vascular disease, obstruction, glomerulonephritis, and parenchymal infiltration of tumor cells. Although renal involvement is not uncommon in ALL, renal failure is rarely a presenting symptom in ALL and is thought to be a poor prognosis indicator. Leukemic may lead to significant impairment of renal function if it is bilateral and diffuse, particularly involving the cortical region. Acute renal failure in patients with acute leukemia is usually a consequence of a chemotherapeutic regimen, leading to tumor lysis syndrome—the tumor lysis syndrome results in acute uric acid and calcium phosphate nephropathy. The most common form of kidney injury in leukemia is related to prerenal AKI in the setting of volume depletion. When infiltration is suspected, kidney biopsy is typically recommended as the extent of infiltration can give prognostic information regarding the malignancy as the rate of infiltration parallels the stage and grade of the disease  Correct Answer: Renal failure as a result of hyperuricemia can be associated with ALL, particularly at diagnosis or during active treatment. Uric levels rise as an end product of purine metabolism from cellular destruction. Because the major excretory pathway is through the kidneys, urates can precipitate Response Feedback: [None Given]

·  Question 3

4 out of 4 points

	Scenario 2: Sickle Cell Disease (SCD) A 15-year-old male with known sickle cell disease (SCD) present to the ER in sickle cell crisis. The patient is crying with pain and states this is the third acute episode he has had in the last 10-months. Both parents are present and appear very anxious and teary eyed. A diagnosis of acute sickle cell crisis was made.  Question 1.     Explain the pathophysiology of acute SCD crisis. Why is pain the predominate feature of acute crises?
	Selected Answer: The term “sickle cell crisis” describes several acute conditions such as the vaso- occlusive crisis (acute painful crisis), aplastic crisis, splenic sequestration crisis, hyperhemolytic crisis, hepatic crisis, dactylitis, and acute chest syndrome. Sickle cell disease is an autosomal recessive disorder of a gene mutation. On chromosome 11, nucleotide mutation substitutes glutamic acid for valine at position six on the beta-globin subunit. A sickle cell crisis occurs when sickle-shaped red blood cells clump together and block small blood vessels that carry blood to certain organs, muscles, and bones. This causes mild to severe pain. The pain can last from hours to days. “Painful event” and “painful crisis” are other terms used to describe these episodes. Some people with sickle cell disease have many painful events, while others have few or none. The pain can happen in any part of the body. Nevertheless, the most common areas include the: bones of the spine, arms, and legs; chest; and abdomen. Inflammatory mediators such as plasma cytokines lead to a pro-inflammatory state, causing further complications of vaso-occlusion. It is postulated that the intestinal microbiome may be a potential trigger for the vaso-occlusive crisis. While some triggers (cold temperature, dehydration, low humidity, stress) for pain are identifiable, most episodes do not have an identifiable cause. Correct Answer: Approximately half the individuals with homozygous HbS disease experience vaso-occlusive crises. The frequency of crises is extremely variable. Some individuals have as many as 6 or more episodes annually, whereas others may have episodes only at great intervals or none at all. Each individual typically has a consistent pattern for crisis frequency. Triggers of vaso-occlusive crisis include the following: • Hypoxemia: May be due to acute chest syndrome or respiratory complications • Dehydration: Acidosis results in a shift of the oxygen dissociation curve • Changes in body temperature (eg, an increase due to fever or a decrease due to environmental temperature change) Many individuals with HbSS experience chronic low-level pain, mainly in bones and joints. Intermittent vaso-occlusive crises may be superimposed, or chronic low-level pain may be the only expression of the disease. © 2020 Walden University 3 Sickle RBCs express higher than normal amounts of adhesion molecules and are sticky. During inflammatory reactions, leukocyte release of mediators increases the expression of adhesion molecules on endothelial cells. These reactions further promote sickled erythrocytes to be come arrested during movement through the microvascular. The sluggish and stagnant red cells within the inflamed vascular vessels result in extended exposure to low oxygen tension, sickling, and vascular obstruction. Lysed sickle erythrocytes release hemoglobin and free hemoglobin can bind and inactivate nitic oxide (NO), which is a powerful vasodilator and inhibitor of platelet aggregation. Decreased blood pH reduces hemoglobin affinity for oxygen leading to an increasing fraction of deoxygenated HbS at any oxygen tension and predisposition to sickling. As less oxygen is taken up by hemoglobin in the lungs, the PO2 drops promoting additional sickling. The intense pain of an acute crisis is due to lack of oxygen to major organs and bones. The lack of oxygen leads to ischemia and organ death. Response Feedback: [None Given]

·  Question 4

4 out of 4 points

	Scenario 2: Sickle Cell Disease (SCD) A 15-year-old male with known sickle cell disease (SCD) present to the ER in sickle cell crisis. The patient is crying with pain and states this is the third acute episode he has had in the last 10-months. Both parents are present and appear very anxious and teary eyed. A diagnosis of acute sickle cell crisis was made.  Question 1.     Discuss the genetic basis for SCD.
	Selected Answer: Sickle Cell Disease is inherited and involves an autosomal recessive pattern which means both copies of the gene in each cell have mutations. The parents of an individual with an autosomal recessive condition carry one copy of the mutated gene, but they typically do not show signs and symptoms. Hemoglobin (Hb), a gene that determines how red blood cells are made and how they work, is inherited from both parents. Each birth parent provides one hemoglobin (Hb) gene. Normal hemoglobin is called HbA. HbS is a change to the normal Hb gene. An abnormal HbS gene causes red blood cells to become sickle-shaped, limiting the amount of oxygen the red blood cells can carry through the body. Correct Answer: SCD denotes all genotypes containing at least one sickle gene, in which HbS makes up at least half the hemoglobin present. Major sickle genotypes described so far include the following: • HbSS disease or sickle cell anemia (the most common form) – Homozygote for the S globin with usually a severe or moderately severe phenotype and with the shortest survival • HbS/b-0 thalassemia – Double heterozygote for HbS and b-0 thalassemia; clinically indistinguishable from sickle cell anemia (SCA) • HbS/b+ thalassemia – Mild-to-moderate severity with variability in different ethnicities • HbSC disease – Double heterozygote for HbS and HbC characterized by moderate clinical severity • HbS/hereditary persistence of fetal Hb (S/HPHP) – Very mild or asymptomatic phenotype Sickle cell trait or the carrier state is the heterozygous form characterized by the presence of around 40% HbS, absence of anemia, inability to concentrate urine (isosthenuria), and hematuria. Under conditions leading to hypoxia, it may become a © 2020 Walden University 4 pathologic risk factor. Sickle cell disease produces illness, while sickle cell trait usually does not. People who inherit two genes for sickle hemoglobin (one from each parent) have sickle cell disease. With a few exceptions, a child can inherit sickle cell disease only if both parents have one gene for sickle cell hemoglobin Response Feedback: [None Given]

·  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]