EC Pulmonology and Respiratory Medicine

Background: Cystic Fibrosis (CF) has been described before in Saudi Arabia and all Arab countries. The presentation is usually recurrent chest infection, failure to thrive and electrolyte imbalance. Sepsis is a rare presentation in such group of patients due to normal immunological status.

Objective: To describe the first case report of CF infant presenting as recurrent Staphylococcus hominis and Methicillin Resistant Staph aureus (MRSA) sepsis with recurrent chest infection despite normal immunological workup.

Method: A case report and literature review of an infant presenting with recurrent septicemia and negative immunological work up with positive CFTR gene.

Results: A 7 months old infant presented to the local hospital with recurrent fever, diarrhea, failure to thrive with Z score for weight/Age of (-8.2) and recurrent pneumonia monthly since the age of 2 months. He was referred to our center to rule-out immunodeficiency. He developed hypoxic respiratory failure requiring Intubation for 3 weeks with endovascular infection with polymicrobial central line associated blood stream infection (CLABSI) and persistent positive cultures of MRSA septicemia. He developed tension pneumothorax on top of parenchymal lung disease that required chest tube drainage for 10 days. His Broncho alveolar lavage grew: Klebsiella pneumoniae, Stenotrophomonas maltophilia and Candida parapsilosis in addition to Influenza B virus and RSV. His sepsis improved with the appropriate antimicrobial treatment and weight improving to Z score of -5.9 after one month of nutritional support and CF management. He was discharged in a good clinical condition. His immunological workup was normal but the whole genome study confirmed the diagnosis of CF with homozygous c.1375_1383del. His weight Z score at 2 years was (-1.02).

Several case reports in the literature describe CF presenting as sepsis in pediatric patients. One report describes three infants who initially presented with pneumonia and septic shock which led to their CF diagnosis. Such presentation in uncommon. 5 publications reported similar presentation with total of 8 infants.

Conclusion: CF could present with recurrent sepsis. Proper antimicrobial treatment and detailed history could prevent prolonged morbidity and mortality of this group of patients.

 Keywords: Cystic Fibrosis Septicemia; Staphylococcus hominis; CF MRSA Sepsis

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