A 5-year-old boy is brought to the emergency department with severe leg pain that started 6 hours earlier. His mother says that their heating system broke last night so she "tried to keep him warm with lots of blankets." The patient was given ibuprofen and oxycodone at home without relief of pain. He played outside with his brother yesterday but did not experience any trauma or injury. He has not had fever, cough, vomiting, or rash. Medical history includes mild persistent asthma and sickle cell disease, and he has been hospitalized once for a vaso-occlusive pain episode. Temperature is 37.2 C (99 F), blood pressure is 100/62 mm Hg, pulse is 150/min, and respirations are 20/min. Physical examination shows a young child lying in bed in significant discomfort, crying and holding his left leg. Mucous membranes are moist and capillary refill is

Question

A 5-year-old boy is brought to the emergency department with severe leg pain that started 6 hours earlier.  His mother says that their heating system broke last night so she "tried to keep him warm with lots of blankets."  The patient was given ibuprofen and oxycodone at home without relief of pain.  He played outside with his brother yesterday but did not experience any trauma or injury.  He has not had fever, cough, vomiting, or rash.  Medical history includes mild persistent asthma and sickle cell disease, and he has been hospitalized once for a vaso-occlusive pain episode.  Temperature is 37.2 C (99 F), blood pressure is 100/62 mm Hg, pulse is 150/min, and respirations are 20/min.  Physical examination shows a young child lying in bed in significant discomfort, crying and holding his left leg.  Mucous membranes are moist and capillary refill is <2 seconds.  The lungs are clear to auscultation, and the abdomen is soft, nontender, and nondistended without organomegaly.  The left anterior calf is markedly tender to palpation but does not have erythema, warmth, or swelling.  A peripheral intravenous line is inserted, and a complete blood count is drawn. The child is admitted to the hospital and experiences improvement of his leg pain overnight.  The next morning, however, he develops chest pain and difficulty breathing.  Temperature is 38.9 C (102 F), blood pressure is 94/62 mm Hg, pulse is 124/min, and respirations are 28/min.  The patient's pulse oximetry shows 95% on room air.  Examination reveals diminished left-sided breath sounds with mild subcostal retractions.  Hemoglobin is stable from admission at 9.5 g/dL, and a blood culture is sent.  A chest x-ray is obtained.  Which of the following is the best next step in management of this patient?

Quizplus · Accepted Answer

Explanation: 11ec2b69_bf80_759f_8f63_b1a11092e05d_MD0005_00 Acute chest syndrome (ACS) is a potentially life-threatening pulmonary complication of sickle cell disease (SCD). The diagnosis of ACS requires the presence of a new pulmonary infiltrate on chest x-ray and >1 of the following: Fever >38.5 C (101.3 F), hypoxemia, chest pain, or respiratory distress. This patient meets diagnostic criteria for ACS based on x-ray findings, fever, chest pain, and difficulty breathing. new pulmonary infiltrate on chest x-ray Causes of ACS in children include infection, asthma exacerbation, and pulmonary infarction. Because infection is the most common cause, empiric broad-spectrum antibiotics should be started promptly. First-line therapy is a third-generation cephalosporin (eg, ceftriaxone, cefotaxime) plus a macrolide (eg, azithromycin) to cover Streptococcus pneumoniae and Mycoplasma pneumoniae, respectively. Additional management includes pain control to prevent splinting and subsequent hypoventilation and atelectasis. Judicious intravenous fluids should also be administered to prevent dehydration and further sickling. (Choice A) Blood transfusion in ACS is indicated for an oxygen saturation <92%, significant anemia, or worsening symptoms despite initial treatment. Although transfusions are often required in ACS, they are avoided in SCD unless necessary, given the risk of alloimmunization. (Choice C) Continuous positive airway pressure is a form of noninvasive ventilation that can be helpful in patients with an increasing oxygen requirement or significant respiratory distress. It is not indicated in this patient who is on room air and has only mildly increased work of breathing. (Choice D) Pulmonary embolism is a complication of ACS that can be diagnosed with CT angiography of the chest, and is more common in adults. This patient also does not have severe respiratory distress or hypoxemia. (Choice E) A tension pneumothorax presents with dyspnea, hypotension, diminished breath sounds on the affected side, and tracheal deviation toward the unaffected side. Needle thoracostomy is indicated for decompression of a tension pneumothorax but has no role in the treatment of ACS. Educational objective: Acute chest syndrome in sickle cell disease is defined by the presence of a new pulmonary infiltrate on chest x-ray and fever, hypoxemia, chest pain, tachypnea, or increased work of breathing. Initial treatment includes a third-generation cephalosporin (eg, ceftriaxone) and macrolide (eg, azithromycin), pain control, and intravenous fluids. References: Managing acute complications of sickle cell disease in pediatric patients. (http://www.ncbi.nlm.nih.gov/pubmed/27775898)

A 5-Year-Old Boy Is Brought to the Emergency Department with Severe