GASTROINTESTINAL (GI) ADENOVIRUS TREATED WITH ORAL RIBAVIRIN IN SEVERE COMBINED IMMUNODEFICIENCY (SCID). 53

Abstract

JH presented at 5 months of age with FTT, and respiratory distress due to parainfluenza virus type III (PFIII). Our evaluation led to a diagnosis of SCID. Respiratory symptoms improved with long-term nebulized Ribavirin and supportive care, but GI tract adenovirus (AV) resulted in continuing management problems (cramps, diarrhea, FTT). For definitive therapy JH received a maternal-donor, T-cell-depleted bone marrow stem cell transplant(no pre-conditioning chemotherapy). T lymphocyte function reconstituted during the subsequent 3-4 months, with elimination of the PFIII. GVHD affecting the bone marrow developed, with ensuing life-threatening pancytopenia. Worsening GI symptoms and increase in stool AV were noted with anti-GVHD therapy(corticosteroids and cyclosporin A). We hypothesized that exacerbation of the AV infection was in part due to the immunosupressive treatment and, additionally, that the immune response generated against the AV was resulting in simultaneous activation of maternal T lymphocytes capable of causing and/or worsening the GVHD. Cognizance that systemic AV infections can be catastrophic in immunocompromised patients prompted us to escalate therapy. Recent studies indicating successful and less toxic usage of oral Ribavirin for the treatment of various viruses led us to consider this over parenteral therapy. Low dose therapy, 20 mg/kg/day was initiated, with equivocal results. One week later, dosing of 200 mg/kg/day resulted in immediate clinical improvement (reduction in vomiting, loose stools, abdominal cramping, and improved feeding). Stool AV was quantified by enumerating viral particles via electron microscopy. Post-treatment viral load was decreased to 20% of that of immediate pre-treatment levels. To our knowledge, this is the first report of oral Ribavirin therapy for GI tract AV infection. While long-term effects of Ribavirin remain unknown, clinical improvement in JH demonstrated that oral Ribavirin can be of benefit as anti-viral therapy in immunodeficient patients.