Table 1.
Clinical and microbiological characteristics of the children with inborn errors of immunity and cytomegalovirus infection (n = 25)
| n (%) | |
|---|---|
| Clinical featuresa: | |
| • Respiratory symptoms/hypoxemia | 18 (72) |
| • Fever | 16 (64) |
| • Diarrhea | 11 (44) |
| • Hepatitis | 8 (32) |
| • Neurological symptoms | 8 (32) |
| • Retinitis | 7 (28) |
| • Asymptomatic (only viremia) | 2 (8) |
| Highest viral load prior to HSCT (copies/mL, median, and IQR) |
325 000 (5 400–2 100 000) |
| Coinfection by other virusesb | 10 (40) |
| Other coinfectionsc | 10 (40) |
| Oxygen therapy | 16 (64) |
| Invasive mechanical ventilation due to CMV infection | 10 (40) |
| PICU admission due to CMV infection | 11 (44) |
| Length of PICU stay, days; median (IQR) | 15 (8.5–29) |
| Antiviral therapy | |
| • Ganciclovir/valganciclovir | 25 (100) |
| • Foscarnet | 8 (32) |
| • Cidofovir | 3 (12) |
| • Leflunomide | 2 (8) |
| • Anti-CMV hyperimmune globulin | 4 (16) |
| • Adoptive therapy with CMV specific T-cells prior to HSCTd | 2 (8) |
| Number of drugs prescribed for CMV treatment (sequentially and/or in combination) | |
| • 1 | 15 (60) |
| • 2 | 7 (28) |
| • 3 or more | 3 (12) |
| Treatment duration, days; median (IQR) | |
| • Ganciclovir/valganciclovir | 36 (20.8–90) |
| • Foscarnet | 29.5 (19.8–50.8) |
| Treatment toxicitye: | 4 (16) |
| • Hematologic | 3 (12) |
| • Hepatic | 1 (4) |
| • Renal | 1 (4) |
BAL bronchoalveolar lavage, CMV cytomegalovirus, CSF cerebrospinal fluid, HSCT hematopoietic stem cell transplantation, PICU pediatric intensive care unit, IQR interquartile range
aCMV was isolated from blood and BAL; blood and CSF; blood, BAL, and CSF; and blood and urine in one patient each
bViruses: Epstein-Barr virus (3 cases), respiratory syncytial virus (3), adenovirus (2), norovirus (2), enterovirus (1), parainfluenza (1), parvovirus B19 (1). One patient was infected by enterovirus, norovirus, and respiratory syncytial virus and another by Epstein-Barr virus, norovirus, and adenovirus
cOther coinfections: Pneumocystis jirovecii (6), Pseudomonas aeruginosa (2), Candida spp. (2), Campylobacter jejuni (1), Cryptosporidium parvum (1), Aspergillus spp. (1), Serratia marcescens (1), Stenotrophomonas maltophilia. One patient was infected by Candida and C. jejuni, another by C. parvum, P. aeruginosa, and Aspergillus spp., and other by P. jirovecii, Candida spp., and S. marcescens
dThis therapy failed to control CMV load in these two patients, dying both of them before receiving HSCT
eOne patient experienced both hematological and renal toxicity. He received ganciclovir, foscarnet, cidofovir, CMV-specific hyperimmune globulin, and CMV-specific T cells