Case Presentation
A 3-month old male, the second child of a family with consanguineous marriage, with pancreatic insufficient CF who was born at full term gestation with birth weight of 3.25 kg status post two operations for intestinal obstruction at 3 and 46 days of life. Sweat test were 98 mmol/L and 90 mmol/L. He was admitted because of fever, tachypnea and hypoxia, with oxygen saturation of 78% in room that increased to 97% on 4 L/min of oxygen via oxyhood. He weighed 3.5 kg. Lungs were remarkable for bilateral crackles and presence of bilateral lower limb edema. On admission, WBC was (16.9 x 109 )/L (21% neutrophil, 62% lymphocyte and 10% monocyte), Hb 9.4 g/L, platelet of (412 x 109 )/L. Electrolytes and liver function tests were normal. Chest radiograph and chest CT (Figure 1) showed patchy infiltrates and increased interstitial markings bilaterally. EKG was normal with QT interval of 0.37. G6PD level was normal. He was treated with Meropenem and Vancomycin until the deep throat culture results showed Klebsiella pneumonia that was sensitive to Meropenem. Vancomycin was discontinued. Hydroxychloroquine was started at 3mg/kg/dose, twice a day for 10 days. On the third day of admission, he was started on aerosolized Salbutemol and hypertonic saline (5%) three times a day with improvement in oxygen requirement and his respiratory symptoms. After five days of inhalation therapy, his clinical status improved with resolution of respiratory distress and oxygen requirement. His bilateral lower limb edema was attributed to hypoalbuminemia and treated with nutritional supplementation and appropriate pancreatic enzyme dosing.