Adverse events and outcomes
Syndrome and patient ID no. . | Infectious adverse events . | Immune adverse events . | Other adverse events . | Status . |
---|---|---|---|---|
Typical complete | ||||
DiGeorge syndrome | ||||
DIG103 | Central line infections with Enterococcus faecalis, day + 4, with Candida parapsilosis, day + 48, and Staphylococcus epidermitis, day + 53; fever and coagulopathy for 3 weeks starting day + 89 (cultures negative) | None | Feeding intolerance, days + 89 to + 114, with diarrhea, coagulopathy, and fever; clot around central line, day + 67, associated with elevated platelets (701 000/mm3), treated with aspirin until patient had bleeding via rectum | At home on tube feedings 2.1 years after thymus transplantation |
DIG105 | Stool positive for C difficile toxin, days - 5, + 13, + 56, + 77; stool positive for enterovirus, day + 13; wound infection from suture removal, day + 39; fever, day + 161—treated 2 days in hospital (culture negative) | Thrombocytopenia (to 30 000/mm3) with elevated antiplatelet antibodies, day + 19, that responded to IVIG; ANC dropped to 315/mm3 but responded to discontinuation of trimethoprim/sulfamethoxazole | Poor oral intake | At home on oral feedings 1.5 years after transplantation |
Atypical complete | ||||
DiGeorge syndrome patients | ||||
DIG102 | Adenovirus in stool, days + 8, + 28, + 53, + 98, + 99, + 119; central line infections, days + 340, + 432, + 580 | Nephrotic syndrome, days + 261 to + 416; urticaria started day + 286, resolved; mediastinal lymphadenopathy with biopsy day + 913 showing normal nodes | Hypoxemia at night, day + 603 from VCFS, resolved without therapy; sucrase deficiency (feeding intolerance) since birth | At home on sacrosidase in oral feedings 2.7 years after transplantation |
DIG104 | Respiratory syncytial virus (RSV) in lungs, day - 6 to death at day + 137; enterovirus in stool, days + 1 to + 27; urine with yeast, days + 33, + 42; blood positive for S epidermidis, days + 56, + 76, + 94; C difficile toxin in stool, days + 115, + 127; respiratory arrest, day + 120 from RSV; intubated, day + 130 for RSV | Rash and lymphadenopathy, day - 6 to death | Infantile spasms, day + 104 to death; bladder retention, day + 16 to death, secondary to fatty filum of spinal cord; developmental delay from birth to death; decubitus ulcer, day + 115 | Death from RSV on day + 137 after transplantation |
DIG106 | Blood with S epidermidis, days - 8, 0, + 93; urine with enterococcus, days + 7, + 23, + 142; stool positive for C difficile toxin, days + 36, + 182; rhinovirus in tracheal aspirate, days + 126, + 133, + 142, + 147, then negative after treatment | Absolute eosinophil count to 10 804/mm3 with rash on day + 39, treated with steroids | Swallow study showed aspiration, so patient was placed on all tube feedings; pulmonary hypertension, moderate; severe developmental delay | At home on tube feedings 1.5 years after transplantation |
DIG107 | Stool positive for adenovirus, day - 10; stool positive for rotavirus, days - 7, + 30, + 41, + 56, + 80, + 89; urine positive for Klebsiella pneumoniae, day - 7 | None | Frank aspiration on swallowing, study day - 9; deafness documented day + 40; diarrhea secondary to rotavirus until 6 months after transplantation | At home on tube feedings 1.4 years after transplantation |
Syndrome and patient ID no. . | Infectious adverse events . | Immune adverse events . | Other adverse events . | Status . |
---|---|---|---|---|
Typical complete | ||||
DiGeorge syndrome | ||||
DIG103 | Central line infections with Enterococcus faecalis, day + 4, with Candida parapsilosis, day + 48, and Staphylococcus epidermitis, day + 53; fever and coagulopathy for 3 weeks starting day + 89 (cultures negative) | None | Feeding intolerance, days + 89 to + 114, with diarrhea, coagulopathy, and fever; clot around central line, day + 67, associated with elevated platelets (701 000/mm3), treated with aspirin until patient had bleeding via rectum | At home on tube feedings 2.1 years after thymus transplantation |
DIG105 | Stool positive for C difficile toxin, days - 5, + 13, + 56, + 77; stool positive for enterovirus, day + 13; wound infection from suture removal, day + 39; fever, day + 161—treated 2 days in hospital (culture negative) | Thrombocytopenia (to 30 000/mm3) with elevated antiplatelet antibodies, day + 19, that responded to IVIG; ANC dropped to 315/mm3 but responded to discontinuation of trimethoprim/sulfamethoxazole | Poor oral intake | At home on oral feedings 1.5 years after transplantation |
Atypical complete | ||||
DiGeorge syndrome patients | ||||
DIG102 | Adenovirus in stool, days + 8, + 28, + 53, + 98, + 99, + 119; central line infections, days + 340, + 432, + 580 | Nephrotic syndrome, days + 261 to + 416; urticaria started day + 286, resolved; mediastinal lymphadenopathy with biopsy day + 913 showing normal nodes | Hypoxemia at night, day + 603 from VCFS, resolved without therapy; sucrase deficiency (feeding intolerance) since birth | At home on sacrosidase in oral feedings 2.7 years after transplantation |
DIG104 | Respiratory syncytial virus (RSV) in lungs, day - 6 to death at day + 137; enterovirus in stool, days + 1 to + 27; urine with yeast, days + 33, + 42; blood positive for S epidermidis, days + 56, + 76, + 94; C difficile toxin in stool, days + 115, + 127; respiratory arrest, day + 120 from RSV; intubated, day + 130 for RSV | Rash and lymphadenopathy, day - 6 to death | Infantile spasms, day + 104 to death; bladder retention, day + 16 to death, secondary to fatty filum of spinal cord; developmental delay from birth to death; decubitus ulcer, day + 115 | Death from RSV on day + 137 after transplantation |
DIG106 | Blood with S epidermidis, days - 8, 0, + 93; urine with enterococcus, days + 7, + 23, + 142; stool positive for C difficile toxin, days + 36, + 182; rhinovirus in tracheal aspirate, days + 126, + 133, + 142, + 147, then negative after treatment | Absolute eosinophil count to 10 804/mm3 with rash on day + 39, treated with steroids | Swallow study showed aspiration, so patient was placed on all tube feedings; pulmonary hypertension, moderate; severe developmental delay | At home on tube feedings 1.5 years after transplantation |
DIG107 | Stool positive for adenovirus, day - 10; stool positive for rotavirus, days - 7, + 30, + 41, + 56, + 80, + 89; urine positive for Klebsiella pneumoniae, day - 7 | None | Frank aspiration on swallowing, study day - 9; deafness documented day + 40; diarrhea secondary to rotavirus until 6 months after transplantation | At home on tube feedings 1.4 years after transplantation |