Pediatric Knowledge Questions

Acute Lymphoblastic Leukemia

This is a malignancy of the hematological system, mainly affecting the white blood cells, though the red blood cells and platelets are also affected. It affects both the B and T lymphocytes and its hallmark is the replacement of the bone marrow and other lymphoid organs with immature lymphocytes and their progenitors. There is uncontrolled proliferation of abnormal, immature cells, which leads to their overwhelming numbers in the bone marrow. Even though this condition affects both adults and children, 80% of the cases occur in children.

Pediatric Knowledge Questions

A study by Puckett and Chan (2022) found that acute lymphoblastic leukemia accounts for 2% of lymphoid neoplasms in the United States of America. In addition, the condition affects more males than females and is more prevalent in Caucasians as compared to African- Americans. The signs and symptoms include easy bruising, fatigue, recurrent infections, lymphadenopathy, hepatomegaly, and splenomegaly.

Furthermore, B symptoms like weight loss, night sweats, and fever also occur. These presenting features are related to thrombocytopenia, anemia, and neutropenia as a result of the replacement of normal bone marrow elements with malignant cells.

Why Acute Renal Failure Occur In Some Patients With Acute Lymphoblastic Leukemia

Goyal et al. (2022) define acute renal failure as a sudden reduction in the function of the kidney, which is denoted by the increase in creatinine level and decreased production of urine. Most of the time, this change is reversible with immediate intervention. Acute lymphoblastic leukemia can cause kidney injury through several mechanisms. To begin with, it can cause acute tubular necrosis due to tumor lysis syndrome or lysozyme-induced necrosis of the tubules.

In addition, there is usually poor intake, early satiety, and anorexia in these patients. This causes volume depletion resulting in pre-renal kidney injury. Additionally, there is also infiltration of the kidney by the malignant cells causing direct injury though this is seen in rare cases (Rose et al., 2019). Furthermore, there can be glomerular injury as a result of lysozymes passed in the urine. This can also lead to injury of the kidneys.

Pathophysiology of Acute Sickle Cell Crisis

Sickle cell disease is one of the congenital hemolytic disorders that cause a lot of morbidity in patients. In this condition, there is the production of abnormal Hemoglobin S which is not as elastic and deformable as normal Hemoglobin A. This causes the red cells to have a sickle shape and are rigid, therefore unable to pass through the tiny capillaries.

During stressful conditions such as dehydration, hypoxia, overexertion, or infection, there is hemolysis and occlusion of the blood vessels by the sickled red cells. This leads to stimulation of the nociceptive nerve fibers (Sundd et al., 2019). The patients, therefore, present with severe excruciating pain that is the hallmark of acute sickle cell crisis. Apart from pain, obstruction of the microvasculature also leads to the development of ischemia, necrosis, edema, and damage to various organs of the body.

Genetic Basis For Sickle Cell Disease

This is one of the most common congenital hemolytic diseases. Its inheritance is autosomal recessive, meaning that two people who are carriers can give birth to a child with the disease. In this condition, there is the substitution of valine for glutamic acid in the hemoglobin–beta gene located in chromosome 11 (Sedrak & Kondamudi, 2022).

This will lead to the production of defective hemoglobin S, which is rigid and vulnerable to hemolysis. The hemoglobin causes polymerization and a change in the shape and ability of the red cells to deform. The cells are therefore trapped in the capillaries and microvasculature.

Pathophysiology of Hemophilia

Hemophilia is a hereditary hemorrhagic disorder characterized by excessive and prolonged bleeding in affected individuals. In the process of clot formation, there are two pathways involved, the intrinsic and the extrinsic pathways (Mehta & Reddivari, 2022). These factors involve the activation of various clotting factors that eventually leads to the formation of a clot to stop bleeding.

Factors VIII and IX are important in the stimulation of the intrinsic pathway. In this condition, there is a deficiency of the two factors leading to failure of activation of the intrinsic pathway. This is what causes the prolonged bleeding. A deficiency of factor VIII is termed hemophilia A, while that of factor IX is termed hemophilia B.

Conclusion

Hematological conditions in children usually have a lot of morbidity and mortality and most of them have a genetic component. Early identification and diagnosis can help in better management. Genetic counseling and testing are therefore important for couples before they conceive.

References