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Acute chest syndrome in children with sickle cell disease
Insights
Acute chest syndrome (ACS) in children with sickle cell disease presents with fever and chest pain. Antibiotics and supportive care are standard treatments, with Mycoplasma pneumoniae identified in some cases.
Area of Science:
- Pediatrics
- Hematology
- Pulmonology
Background:
- Acute chest syndrome (ACS) is a serious complication in sickle cell disease (SCD).
- Understanding the clinical presentation and causative agents of ACS is crucial for timely management.
Purpose of the Study:
- To prospectively investigate the clinical characteristics, radiographic findings, and potential etiologies of acute chest syndrome in pediatric patients with sickle cell disease.
Main Methods:
- Prospective study of 50 pediatric patients with ACS over one year.
- Data collection included demographics, clinical signs (fever, chest pain), chest X-ray findings, and laboratory results (ESR, hemoglobin, mycoplasma pneumoniae titer, blood cultures).
- Treatment involved antibiotics, oxygen therapy, hydration, and blood transfusions.
Main Results:
- The study included 50 patients (54% male) aged 1-12 years. Fever (54%) and chest pain (24%) were common symptoms. Right lung involvement was most frequent on chest X-ray (60%).
- Elevated erythrocyte sedimentation rate and low hemoglobin levels were observed. Mycoplasma pneumoniae titers were positive in 14% of patients; blood cultures were negative.
- Three patients required chest tubes for pleural effusion. All patients received antibiotics, primarily Cefuroxime or Ceftriaxone with Erythromycin, alongside supportive care.
Conclusions:
- Acute chest syndrome in pediatric sickle cell disease patients exhibits diverse clinical and radiographic presentations.
- While Mycoplasma pneumoniae may be a contributing factor in some cases, bacterial infection was not confirmed by blood cultures.
- Comprehensive supportive care, including antibiotics, remains the cornerstone of ACS management in this population.
Abstract:
Acute chest syndrome (ACS) is an acute pulmonic process in patients with sickle cell disease. We prospectively studied 50 patients with ACS admitted to the Pediatric Medical Ward during one year period (Jan. 1993 through Dec. 1993). Twenty eight of them were males and twenty two were females giving a male: female ratio of 1.2:1. The age ranged between one and 12 years. Twelve (24%) of the patients had chest pain on presentation. Twenty seven (54%) patients had significant temperature (> 38 degrees C). The x-ray findings showed that the right lung was involved in 30 patients, the left in 10 patients and both lungs in 10 patients. Three patients had pleural effusion that required chest tube insertion. Laboratory profiles showed that the erythrocyte sedimentation rate ranged between 15 and 90 mm/h, and their hemoglobin ranged between 4.2 gm and 12 gm/dl. Seven (14%) patients had significantly positive mycoplasma pneumoniae titer. None of the blood cultures was positive. All of our patient received antibiotic, usually either Cefuroxime or Ceftriaxone with Erythromycin in addition to other supportive measures such as blood transfusion, oxygen therapy and hydration therapy.