Table 1.

Clinical findings of the THES2 patient

Age	3 mo	5 mo*	6 mo	7 mo
Immunoglobulins (mg/dL)
IgA	37	359	1120	921
IgE	210	106	58	ND
IgG	88	1670	1020	823
IgM	66	24	5.3	11.2
Functional assay
Mitogens (PHA, ConA, PWM)	Normal
NK Function	Normal
Clinical phenotypes
Intractable diarrhea in infancy
Gastrointestinal and hepatic pathologies
Woolly hair appearance
ANCA-associated vasculitis
Positive p-ANCA and serine protease 3
Pauci-immune necrotizing and crescentic glomerulonephritis

^*Patient started steroids and IgG replacement therapy at 5 months of age. ND, not done.