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Table 1 Chronologic HIES scoring

From: Case report of a young child with disseminated histoplasmosis and review of hyper immunoglobulin e syndrome (HIES)

Age (mo)

Date

9

June, 2008

20

May, 2009

23

Aug, 2009

33

July, 2010

IgE score (IU/ml)

1 (187)

1 (371)

8 (1,011)

8 (1,106)

Skin abscess

0

0

0

2

Pneumonia

2

4

6

8

Lung anomalies

0

0

8

8

Fractures

4

4

4

8

Eczema

2

2

2

2

URIs/year

2

2

2

2

Candidiasis

1

1

1

1

Serious infections

0

0

0

4

Young age correction

7

5

5

3

NIH Score

19

19

36

46

Pneumonia

N/A

N/A

6 × 2.5

8 × 2.5

Pathologic fractures

N/A

N/A

4 × 3.33

8 × 3.33

STAT3 Score

N/A

N/A

28.32

46.64

  1. NIH score: unlikely, < 20; indeterminate, 20-40; suggestive of AD-HIES, > 40
  2. STAT3 score: possible, > 30+IgE ≥ 1,000 IU/ml, probable: > 30+IgE ≥ 1,000 IU/ml + low Th17 and/or positive F.Hx; definitive, > 30+IgE ≥ 1,000 IU/ml + heterozygous STAT3 mutation