Table 4. Clinical data of adrenal insufficiency.

Cases		1	2	3	4	5	6	7
Disease		XL-agammaglobulinemia	Blau syndrome	XL-CGD	CTLA-4 haploinsufficiency	CVID (NFkB2)	LRBA def.	FHL3 (UNC13D)
IUIS category		3	7	5	4	3	4	4
Sex		Male	Male	Male	Female	Female	Male	Female
Current age		12 yr 2 mon	4 yr 5 mon	12 yr	37 yr 6 mon	11 yr 7 mon	20 yr 10 mon	21 yr 1 mon
Age at HCT							16 yr 4 mon
Diagnosis age of IEI		3 yr 5 mon	1 yr 1 mon	5 yr 7 mon	24 yr 3 mon	4 yr 8 mon	10 yr 8 mon	15 yr
Onset age of adrenal insufficiency		6 yr 3 mon	3 yr 8 mon	10 yr 3 mon	36 yr	6 yr 11 mon	15 yr 11 mon	15 yr
Laboratory data
	ACTH, pg/mL	29.4	107.6	12.4	9.3	10	ND	8.5
	Cortisol basal/peak,a µg/dL	4.7/17.9	8.7/13.3	1.2/3.9	4.2/12.5	0.9/12	0.4/2.1	1.4/5.3
Duration of glucocorticoid use		Untreated	Pd 2.5 yr	Pd 2.7 yr, HCS is ongoing	Pd 8.8 yr	Pd 1 yr, HCS is ongoing	Pd 11 mon	Pd 4 mon

XL = X-linked, CGD = chronic granulomatous disease, CTLA-4 = cytotoxic T-lymphocyte-associated protein 4, CVID = common variable immunodeficiency, LRBA = lipopolysaccharide-responsive and beige-like anchor protein, def. = deficiency, FHL3 = familial hemophagocytic lymphohistiocytosis type 3, IUIS = the International Union of Immunological Societies, HCT = hematopoietic cell transplantation, IEI = inborn errors of immunity, ACTH = adrenocorticotropic hormone, Pd = prednisolone, HCS = hydrocortisone.

^aACTH stimulation test.