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.