Table III.

Descriptions of mortalities among the SCID study (n = 12)

Age at diagnosis (days)	Family history	Absolute lymphocyte count (/mm3)	Molecular defect	Infections	Referred for HSCT	Received HSCT or enzyme replacement	Cause of death
96	Yes	50	ADA	None	Yes		Acute respiratory distress syndrome (presumed infectious)
116	Yes	300		Pneumocystis jiroveci	No	No	Encephalitis (presumed infectious)
191	No	6200	γc-chain	Pneumocystis jiroveci	Yes	Yes	GVHD/PTLD
				Staphylococcus aureus bacteremia
124		1540		None			Neurological abnormality
30	Yes	500	ADA	Rhodotorula mucilaginosa	Yes	No	Lymphoma
60		680		Influenza A	No	No	H1N1 Influenza A
110	Yes	7900		Escherichia coli bacteremia	Yes	Yes	Respiratory failure
				Pseudomonas bacteremia
				Adenovirus
30	Yes	1020	γc-chain	CMV	No	No	Disseminated CMV, acute neurological event
60	No	200		None	Yes	No	Myeloproliferative syndrome
116		4500		CMV	No	No	CMV pneumonitis, multi-organ failure
120	Yes	1100	MHC Class II	RSV, Adenovirus, CMV	Yes	Not a candidate because of co-morbidities	Disseminated CMV, respiratory failure
		288	ADA		Yes	Yes	GVHD/PTLD

ADA adenosine deaminase deficiency; GVHD graft-versus-host disease; PTLD post-transplant lymphoproliferative disorder; CMV cytomegalovirus; MHC major histocompatibility complex; RSV respiratory syncytial virus