Clinical and microbiological findings in patients with impaired response to IFN-γ
| Patient . | Sex . | Age at onset . | Current age . | Consanguinity . | BCG infection . | Other organ involvement . | FTT . | Diarrhea . | Other organism . | Treatment . | Outcome . |
|---|---|---|---|---|---|---|---|---|---|---|---|
| F27_P27 | F | 2.4 m | - | Yes | BCGosis | Liver, spleen | - | - | CMV | Solonex, rifampicin, ofloxacin, ethambutol, Valgan 50 mg | Dead |
| F28_P28 | F | 2 m | - | Yes | BCGosis | Liver, spleen | - | - | - | ATT | Dead |
| F29_P29 | M | 1 m | 10 y | Yes | BCGosis | Lymph node, bone, skin, meninges, lungs, liver, spleen | - | Yes | - | Prednisolone, ATT | Alive |
| F30_P30 | M | 6 m | - | Yes | BCGosis | Liver, spleen, skin | - | - | - | ATT | Dead |
| F31_P31 | F | 1 m | - | Yes | BCGosis | Liver, spleen, lungs, skin | Yes | - | Enterobacter spp., C. guilliermondii | ATT | Dead |
| F32_P32 | M | 1 m | - | No | No | Abdomen | - | - | - | - | Dead |
| F33_P33 | F | 2 m | 10.5 y | No | BCGosis | Liver, spleen, bone | - | - | CMV, P. aeruginosa | ATT | Alive |
| F34_P34 | F | 1 m | 11 y | No | BCGosis | Bone | - | - | - | ATT | Alive |
| F35_P35 | F | 3 m | 6 y | No | BCGosis | Liver, spleen | - | - | - | ATT | Alive |
| F36_P36 | F | 3.5 y | 17 y | No | BCGosis | Skin, bone, liver, spleen | - | - | M. abscessus, Staphylococcus aureus, M. contagiosum | IFN-α, ATT, antibiotics | Alive |
| F37_P37a | M | 6 m | - | Yes | Not vaccinated | Lungs, abdomen, liver, spleen | - | - | CMV | Valganciclovir, ATT | Died 2 years later with unrelated cause |
| F37_P37b | M | 9 m | 14 y | Yes | Not vaccinated | Lungs | - | - | CMV, Neisseria spp. | Valganciclovir, ATT, antibiotics | Transplanted, alive |
| F38_P38 | M | 3 m | - | Yes | BCGosis | Lungs | - | Yes | - | ATT | Lost to follow-up |
| F39_P39 | M | 2 m | - | Yes | BCGosis | Lungs, abdomen, liver, spleen | - | - | - | ATT | Dead |
| F40_P40 | M | 2 m | 6 y | Yes | BCGosis | Liver, spleen | - | - | - | ATT | Alive |
| F41_P41 | M | 3 m | 2 y | Yes | BCGosis | Liver, spleen | - | - | HSV | ATT, clarithromycin, acyclovir | Alive |
| F42_P42a | F | 2 m | - | Yes | BCG adenitis | Lungs | Yes | - | - | Rifampicin, ethambutol, isoniazid, Septran | Dead |
| F42_P42b | F | 3 m | - | Yes | BCG adenitis | - | - | - | - | ATT | Dead |
| F43_P43 | M | 2.4 m | - | Yes | BCG adenitis | - | - | - | Acinetobacter spp., Klebsiella spp., CMV | ATT, Taxim, valganciclovir | Dead |
| Patient . | Sex . | Age at onset . | Current age . | Consanguinity . | BCG infection . | Other organ involvement . | FTT . | Diarrhea . | Other organism . | Treatment . | Outcome . |
|---|---|---|---|---|---|---|---|---|---|---|---|
| F27_P27 | F | 2.4 m | - | Yes | BCGosis | Liver, spleen | - | - | CMV | Solonex, rifampicin, ofloxacin, ethambutol, Valgan 50 mg | Dead |
| F28_P28 | F | 2 m | - | Yes | BCGosis | Liver, spleen | - | - | - | ATT | Dead |
| F29_P29 | M | 1 m | 10 y | Yes | BCGosis | Lymph node, bone, skin, meninges, lungs, liver, spleen | - | Yes | - | Prednisolone, ATT | Alive |
| F30_P30 | M | 6 m | - | Yes | BCGosis | Liver, spleen, skin | - | - | - | ATT | Dead |
| F31_P31 | F | 1 m | - | Yes | BCGosis | Liver, spleen, lungs, skin | Yes | - | Enterobacter spp., C. guilliermondii | ATT | Dead |
| F32_P32 | M | 1 m | - | No | No | Abdomen | - | - | - | - | Dead |
| F33_P33 | F | 2 m | 10.5 y | No | BCGosis | Liver, spleen, bone | - | - | CMV, P. aeruginosa | ATT | Alive |
| F34_P34 | F | 1 m | 11 y | No | BCGosis | Bone | - | - | - | ATT | Alive |
| F35_P35 | F | 3 m | 6 y | No | BCGosis | Liver, spleen | - | - | - | ATT | Alive |
| F36_P36 | F | 3.5 y | 17 y | No | BCGosis | Skin, bone, liver, spleen | - | - | M. abscessus, Staphylococcus aureus, M. contagiosum | IFN-α, ATT, antibiotics | Alive |
| F37_P37a | M | 6 m | - | Yes | Not vaccinated | Lungs, abdomen, liver, spleen | - | - | CMV | Valganciclovir, ATT | Died 2 years later with unrelated cause |
| F37_P37b | M | 9 m | 14 y | Yes | Not vaccinated | Lungs | - | - | CMV, Neisseria spp. | Valganciclovir, ATT, antibiotics | Transplanted, alive |
| F38_P38 | M | 3 m | - | Yes | BCGosis | Lungs | - | Yes | - | ATT | Lost to follow-up |
| F39_P39 | M | 2 m | - | Yes | BCGosis | Lungs, abdomen, liver, spleen | - | - | - | ATT | Dead |
| F40_P40 | M | 2 m | 6 y | Yes | BCGosis | Liver, spleen | - | - | - | ATT | Alive |
| F41_P41 | M | 3 m | 2 y | Yes | BCGosis | Liver, spleen | - | - | HSV | ATT, clarithromycin, acyclovir | Alive |
| F42_P42a | F | 2 m | - | Yes | BCG adenitis | Lungs | Yes | - | - | Rifampicin, ethambutol, isoniazid, Septran | Dead |
| F42_P42b | F | 3 m | - | Yes | BCG adenitis | - | - | - | - | ATT | Dead |
| F43_P43 | M | 2.4 m | - | Yes | BCG adenitis | - | - | - | Acinetobacter spp., Klebsiella spp., CMV | ATT, Taxim, valganciclovir | Dead |
F, female; FTT, failure to thrive; M, male; m, months; y, years.