Musculoskeletal, Hematologic, and Oncologic Conditions

  In this unit, you cover various musculoskeletal, hematologic, and oncologic conditions in children and management plans. In your initial discussion, please select a topic and include the following: Pathophysiology Epidemiology Physical exam findings Differential diagnoses and rationale Management plan to include diagnostic testing, medications if applicable, follow-up plans, and referrals if needed Topics may include: Microcytic anemia Hemolytic anemia Sickle cell anemia and trait Hereditary spherocytosis Idiopathic thrombocytopenic purpura Hemophilias Leukemia Non-Hodgkin lymphoma Hodgkin lymphoma Brachial plexus injury Clavicle fracture Costochondritis Scoliosis Developmental dysplasia of the hip Legg-Calve-Perthes disease Slipped capital femoral epiphysis Genu varum, genu valgum Osgood-Schlatter disease Subluxation of the radial head

Sample Solution

     

Topic: Sickle Cell Anemia and Trait

Pathophysiology:

Sickle cell anemia is a genetic disorder characterized by an abnormality in the hemoglobin molecule (HbS). Instead of normal round red blood cells (RBCs), HbS causes RBCs to become sickle-shaped. These sickle cells are fragile, inflexible, and prone to clumping together. This clumping blocks blood flow, leading to tissue ischemia, pain crises, and organ damage.

Full Answer Section

   
  • Iron Deficiency Anemia: Similar symptoms of pallor and fatigue, but iron studies will differentiate between the two conditions.
  • Thalassemia: Another genetic blood disorder presenting with anemia, but specific blood tests can distinguish between sickle cell and thalassemia.
  • Vitamin Deficiencies: Deficiencies in vitamin B12 or folate can cause anemia, but history and blood tests will help differentiate.

Management Plan:

  • Hydration: Increased fluid intake to prevent sickling.
  • Pain Management: Managing pain during acute crises with medications like opioids and nonsteroidal anti-inflammatory drugs (NSAIDs).
  • Hydroxyurea: This medication reduces the formation of sickle cells and may decrease the frequency of crises.
  • Blood Transfusions: Used in severe cases of anemia or to prevent complications like stroke.
  • Folic Acid Supplementation: Essential for red blood cell production and reduces the risk of birth defects in pregnant women with sickle cell trait.
  • Pneumococcal Vaccination: Recommended to prevent serious infections.
  • Bone Marrow Transplant: A potentially curative option but carries significant risks.

Diagnostic Testing:

  • Complete Blood Count (CBC): Low hemoglobin and hematocrit (percentage of red blood cells in blood) are indicative of anemia.
  • Peripheral Blood Smear: Shows characteristic sickled red blood cells.
  • Hemoglobin Electrophoresis: Confirms the presence of abnormal hemoglobin S.
  • Newborn Screening: Most states screen newborns for sickle cell disease.

Follow-up Plans:

  • Regular checkups with a hematologist to monitor for complications and treatment response.
  • Annual vaccinations.
  • Pre-conception counseling for women with sickle cell trait.

Referrals:

  • Hematologist: A specialist in blood disorders for diagnosis and ongoing management.
  • Pain specialist: For management of severe pain crises.
  • Genetic counselor: For pre-conception counseling and family planning discussions.
 

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