Pretransplant ribavirin and interferon-α therapy for rhinovirus interstitial pneumonia in a RAG1-deficient infant.

Severe combined immunodeficiency (SCID) is one of the most serious inborn errors of immunity leading to a fatal infection in early infancy. Allogeneic hematopoietic cell transplantation (HCT) or elective gene therapy prior to infection or live-attenuated vaccination is the current standard of curative treatment. Even in the era of newborn screening for SCID, pretransplant control of severe infection is challenging for SCID. Multiple pathogens are often isolated from immunocompromised patients, and limited information is available regarding antiviral strategies to facilitate curative HCT. We herein present a case of successfully controlled pretransplant pneumonia after ribavirin and interferon-α therapy in an infant with RAG1-deficiency. A four-month-old infant presented with severe interstitial pneumonia due to a co-infection of rhinovirus and Pneumocystis jirovecii. The tentative diagnosis of SCID prompted to start antibiotics and trimethoprim-sulfamethoxazole on ventilatory support. Because of the progressive respiratory failure four days after treatment, ribavirin and then pegylated interferon-α were started. He showed a drastic response to the treatment that led to a curative HCT 32 days after admission. This patient received the genetic diagnosis of RAG1-deficiency. Currently, he is an active 3-year-old boy with normal growth and development. The review of literature indicated that rhinovirus had a comparable or rather greater impact on the mortality of pediatric patients than respiratory syncytial virus. Considered the turn-around time to the genetic diagnosis of SCID, prompt ribavirin plus interferon-α therapy may help to control severe rhinovirus pneumonia and led to the early curative HCT for the affected infants.

Actinotignum schaalii Can Be an Uropathogen of "Culture-Negative" Febrile Urinary Tract Infections in Children with Urinary Tract Abnormalities.

Accurate diagnosis and treatment of febrile urinary tract infections (UTI) during childhood are important for the prevention of renal parenchymal damage and functional loss, and detection of underlying diseases related to chronic kidney disease (CKD). Actinotignum schaalii (A. schaalii)-related febrile UTI in children is rare, and its incidence and risk factors remain unclear. A 3-year-old boy with a history of UTI presented with fever and vomiting. Although the culture of his urine specimen in air was negative, A. schaalii was observed in a 5% carbon dioxide (CO2) culture condition, as well as an anaerobic one. A diagnosis of febrile UTI was made, and he recovered with antibiotic therapy. He was found to have CKD associated with vesicoureteral reflux (VUR) after further investigations. A. schaalii is one of the causative agents of febrile UTI in children with urinary tract abnormalities. Although the culture in the air could show negative results, urine culture in 5% CO2 and anaerobic conditions is useful for diagnosis. Our case is the youngest and the first known case of A. schaalii-related febrile UTI associated with VUR in children.