Table 2

- Patient’s summarized time line.

Relevant past medical history and interventions
An 8-year-old girl with CF (delF508/delF508, pancreatic insufficiency, and chronic P. aeruginosa colonization) and FMF (M694V/M694V)
DatesSummaries from initial and follow-up visitsDiagnostic testingInterventions
March 1, 2021Start to have fever, cough, and increased sputum. Hospitalization and initiation of treatment for acute pulmonary exacerbation of cystic fibrosis.Infiltration on chest radiography. P. aeruginosa growth in sputum culture.IV piperacillin-tazobactam and IV amikacin treatment were initiated.
March 13, 2021New onset of fever, hypoxia, and deterioration in general condition one day after dental intervention.COVID-19 PCR test negative. S.epidermidis isolated in blood culture.IV teicoplanin and IV meropenem were initiated.
March 14, 2021Patient diagnosed with MAS.Anemia hyperferritinemia. Hypofibrinogenemia. Elevated transaminases.IV pulse methylprednisolone 30 mg/kg/day for 3 consecutive days was initiated.
March 29, 2021Clinical improvement was observed. Patient was discharged after 2 weeks.Laboratory tests returned to reference ranges.Corticosteroid therapy was gradually tapered over a period of 3 months.
  • CF: cystic fibrosis, FMF: familial Mediterranean fever, IV: intravenous, COVID-19: coronavirus disease 2019, MAS: macrophage activation syndrome, PCR: polymerase chain reaction, P. aeruginosa: Pseudomonas aeruginosa, S.epidermidis: Staphylococcus epidermidis