Week 5 Discussions Answers
· Describe childhood acute lymphocytic leukemia (ALL) and G6PD deficiency and the incidence, pathophysiology, clinical manifestations, evaluation, and treatment of each.
Accounting for most childhood cancer, ALL or acute lymphoblastic leukemia is of greater prevalence in industrialized countries. It is more prominent in Hispanic and black males. There are 5 different types of acute lymphocytic leukemias. T-cell ALL, B-cell ALL, pre-B cell ALL, unclassified ALL, and Common ALL. There is no clear cause for this type of cancer, most research is focused on genetic predisposition, viral infections, and environmental factors. As with other cancers, exposure to pesticides, radiation, and magnetic fields can increase the likelihood of developing acute lymphoblastic leukemia. Clinical manifestations for ALL are associated with bone marrow failure. Signs are usually sudden and include pallor, fatigue, bleeding, and fever. Diagnostically, children will have a hemoglobin less than 7g/dL, platelets less than 100,000/mm, total white blood cell count greater than 100,000/ mm. One common manifestation that usually leads to the discovery of acute lymphocytic leukemia is the report of bone or joint pain. Evaluation is done through a bone marrow biopsy; through this they will discover a blast cell which is the marker for acute lymphoblastic leukemia. In healthy children, blast cells are present in less than 5% bone marrow. Treatment includes a combination of chemotherapy and radiation. With acute lymphoblastic leukemia, given the variety of risk groups, different drug therapy treatments have been placed. Treatment programs are designed to induce remission, prevent CNS decline, maintain remission, and maintenance in general.
Glucose-6-Phosphate Dehydrogenase Deficiency is an x linked recessive disorder that is mostly present in males. This enzyme allows erythrocytes to maintain metabolic processes even if you take certain blood thinning medications. Usually, this disorder is asymptomatic unless infection, fever, or acidosis takes place. Hemolysis can take place when ingesting substances with oxidant properties like aspirin. When there is a deficiency of G6PD, hemoglobin and plasma membranes become weak, inhibiting other enzymes from activating properly. Clinical manifestations include hemolytic anemia after a severe infection or ingestion of oxidative drugs. Signs include pallor, dark urine, back pain, shock, and cardiovascular collapse. When G6DP is suspected, laboratory tests need to be done as soon as a crisis episode is over because it is when young erythrocytes are more evident. Treatment is done once hemolysis happens. Treatment includes blood transfusions iron therapy.
Huether, K.M. S. ([Insert Year of Publication]). Pathophysiology: The Biologic Basis for Disease in Adults and Children (7th ed.). Elsevier Health Sciences (US). https://online.vitalsource.com/books/9780323088541
Richardson SR, O’Malley GF. Glucose 6 Phosphate Dehydrogenase Deficiency. [Updated 2022 Sep 26]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK470315/
· Describe the pathophysiology of acute rheumatic fever and rheumatic heart disease, and discuss the pathophysiology, clinical manifestations, evaluation (include the revised Jones Criteria), and treatment of rheumatic fever.*** 1010, 1171
Acute rheumatic fever is a systemic inflammatory disease caused by group A beta-hemolytic streptococci, however, the body’s natural immune response to the infectious agent is delayed (McCance & Huether, 2014). If left untreated, rheumatic fever can cause scarring and deformity of the cardiac structures and weakening of the valves between the chambers of the heart leading to rheumatic heart disease.
Pathophysiology- Acute rheumatic fever can only begin as a pharyngeal infection (sore throat) with group A beta-hemolytic streptococci (strep throat). In Acute rheumatic fever the immune system attacks healthy tissue in the heart, joints, skin, and CNS. It usually affects children 5-15 years of age (McCance & Huether, 2014).
Clinical manifestations of acute rheumatic fever can include fever, painful, tender, red, hot, swollen joints with migration to another join, chest pain, fatigue, flat or slightly raised, painless rash with a ragged edge, small painless bumps beneath the skin, heart murmur, jerky uncontrolled body movement in the hands, feet, and face, and outburst of unusual behavior such as crying or inappropriate laughing ( Rheumatic fever 2022).
Evaluation- The revised Jones criteria is a benchmark in diagnosing acute rheumatic fever. Essential elements of the criteria are evidence of a streptococcal infection. The diagnostic tool looks at low to moderate-high-risk populations and evidence of carditis, and arthritis for major criteria. Minor criteria include carditis, arthralgia, fever, and markers of inflammation such peak ESR greater than or equal to 60mm and/or CRP greater than or equal to 3.0 mg/dL.
Treatment- If the rheumatic fever is treated with antibiotic therapy in a timely manner, the infection will not progress to rheumatic heart disease. According to McCance & Huether, (2014) antibiotic therapy initiated within nine days from the onset of infection will usually stop the progression to rheumatic heart disease. Treatment can ease pain, reduce damage from inflammation, and prevent a recurrence.