Examkin

Soru

Zorluk: OrtaHematology and Oncology

An 18-year-old male with homozygous sickle cell disease is brought to the emergency department due to a two-day history of progressively worsening chest pain, productive cough, and subjective fevers. Initial vital signs show a temperature of 38.8°C (101.8°F), blood pressure of 118/76 mm Hg, heart rate of 112/min, and respiratory rate of 26/min. Pulse oximetry reveals an oxygen saturation of 87% on room air. Physical examination is notable for scleral icterus and decreased breath sounds with crackles over the right lower lung fields. Laboratory evaluation demonstrates a hemoglobin of 7.2 g/dL, leukocyte count of 16,500/mm³, and reticulocyte count of 12%. A chest radiograph reveals a new consolidation in the right lower lobe.

What is the most appropriate chronological sequence for the management of this patient's condition?

  1. 1Administer supplemental oxygen and parenteral opioid analgesics
  2. 2Initiate empiric intravenous antibiotic therapy
  3. 3Perform an exchange red blood cell transfusion
  4. 4Prescribe daily oral hydroxyurea

Cevap

The correct chronological sequence is to first administer supplemental oxygen and analgesia, followed by empiric intravenous antibiotics, then perform an exchange red blood cell transfusion if clinical deterioration occurs, and finally prescribe daily oral hydroxyurea for long-term prevention after recovery.
The management of acute chest syndrome (ACS) follows a stepwise approach based on acuity. The absolute first priority is stabilization with supplemental oxygen to reverse hypoxia-induced sickling, accompanied by rapid pain control. Once the patient is stabilized, empiric antibiotics (e.g., ceftriaxone and azithromycin) are given because underlying pulmonary infections are the most common trigger for ACS and cannot be reliably distinguished from infarction. If the patient's respiratory status continues to decline or if they present with severe disease (e.g., needing mechanical ventilation), red blood cell exchange transfusion is indicated to rapidly replace HbS with normal hemoglobin. Finally, after the patient has recovered from the acute episode, long-term preventive therapy with hydroxyurea is initiated in the outpatient setting to increase fetal hemoglobin and reduce the frequency of future crises.

Adım Adım Çözüm

1
Analyze the clinical presentation.
The patient has sickle cell disease and presents with fever, chest pain, hypoxia, and a new pulmonary infiltrate, which is diagnostic for acute chest syndrome (ACS).
Establishing the diagnosis of ACS determines the specific treatment pathway.
2
Determine the immediate resuscitative priorities.
Hypoxia and pain promote further red blood cell sickling. Providing supplemental oxygen and parenteral analgesics is the first necessary intervention.
Reversing the acute triggers of sickling prevents the microvascular occlusion from worsening.
3
Identify the underlying trigger and initial medical therapy.
Infection is a leading cause of ACS. Empiric antibiotics covering typical and atypical organisms must be started promptly after initial stabilization.
Pneumonia often triggers or is indistinguishable from ACS, making early antimicrobial therapy the standard of care.
4
Determine the intervention for potential clinical deterioration.
If respiratory status worsens despite initial measures, an exchange transfusion is necessary to acutely lower the hemoglobin S concentration.
Rapid removal of sickled cells restores adequate microvascular perfusion and oxygenation in refractory or severe cases.
5
Establish a long-term preventive plan.
Hydroxyurea therapy is planned for the outpatient setting once the patient recovers from the acute crisis.
Hydroxyurea increases fetal hemoglobin (HbF) and is indicated to prevent future episodes, but it has no role in acute management.

Anahtar Kavram

The management of acute chest syndrome in sickle cell disease begins with immediate supportive care (oxygen, pain control, fluids), followed by empiric antibiotics for potential infectious triggers, exchange transfusion for severe or refractory cases, and finally, long-term prevention with hydroxyurea.
Tahmini Süre:1m 30s
Bu soruyu puanla

Konular

Hematology and Oncology

Bu konudan daha fazla soru