Table 2.

Common Features of HIES patients by mutation region.

Feature	DNA region mutations N=35	SH2 region mutations N=30	P value
HIES Score median (IQR+)	73 (60, 82)	79 (65, 86)	0.193$
IgE Median (IQR)	11,500 (7240, 21800)	10,100 (5602, 17640)	0.330$
Highest absolute eosinophil count median (IQR)	936 (810, 1420)	845 (524, 971)	0.154$
>3 pneumonias	23(66%)	18(60%)	0.965^
Pneumatoceles or bronchiectasis	24 (69%)	23 (77%)	0.582*
>4 boils	27 (77%)	20 (67%)	0.479^
Candidiasis
None	4 (11.8%)	6 (20.7%)	0.57^
Oral/vaginal	13 (38.2%)	9 (31.0%)
Fingernail	17 (50.0%)	14 (48.3%)
Newborn Rash	29 (83%)	22(76%)	0.544*
Moderate-Severe Eczema	34 (97%)	27 (90%)	0.302^
>1 retained primary teeth	27(77%)	19 (63%)	0.750^
>10 degree scoliosis	18 (51%)	21(70%)	0.282^
>2 fractures	13 (37%)	15 (54%)	0.112^
Hyperextensibility	23 (66%)	20(77%)	0.404*
Facial Changes	28 (80%)	25 (86%)	0.741*
Hyperintensities on brain MRI	16 (62%)	15 (56%)	0.974^
Arnold Chiari Malformations	13 (42%)	9 (35%)	0.598*
Coronary Artery abnormalities	12(63%)	8(50%)	0.506*

^*P-values determined by Fisher’s exact test.

^$P-values determined by Wilcoxon test.

^{^}P-values determined by Cochran-Armitage Trend test

⁺IQR = Interquartile Range

Percentages are based on the number of non-missing values