A 4-month-old infant girl was admitted to the pediatric intensive care unit with acute renal failure and respiratory distress. The parents were worried regarding the salty taste of their daughter’s skin and an offensive stool odour since birth. The patient had a continuous paroxysmal cough leading to shortness of breath and tachypnea. She also had one-week H/o vomiting, lethargy and decreased oral intake.
During the initial examination, the patient was irritable in severe distress, tachypneic, tachycardic with normal blood pressure, and severely dehydrated. She had palpable subcutaneous crepitation over her neck. There was intercostal and subcostal retraction; on auscultation, air entry was decreased with the bilateral presence of crepitation.
A basic workup revealed metabolic alkalosis in capillary blood gas, hypokalemia, hyponatremia, hypochloremia and renal impairment with high urea and creatinine and low urine chloride. As shown in the figure, a chest X-ray showed pneumomediastinum and subcutaneous emphysema. In addition, a CT scan of her neck showed a tracheal tear with pneumomediastinum, indicating cystic fibrosis (CF), as shown in the figure.
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