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Table 2 NIH Scoring System of HIES with Clinical and Laboratory Tests

From: Idiopathic non-cirrhotic portal hypertension in a patient with Talaromyces marneffei infection: a case report

Clinical Findings

Points

0

1

2

3

4

5

6

7

8

10

Highest serum – IgE level (IU / ml)

<200

200–500

  

501–1000

   

1001–2000

>2000

Skin abscesses

None

 

1–2

 

3–4

   

>4

 

Pneumonia (episodes over lifetime)

None

 

1

 

2

 

3

 

>3

 

Parenchymal lung anomalies

Absent

     

Bronchiectasis

 

Pneumatocele

 

Retained primary teeth

None

1

2

 

3

   

>3

 

Scoliosis, maximum curvature

<10°

 

10–14°

 

15–20°

   

>20°

 

Fractures with minor trauma

None

   

1–2

   

>2

 

Highest eosinophil count (cell/μl)

<700

  

700–800

  

>800

   

Characteristic face

Absent

 

Mildly present

  

Present

    

Midline anomaly

Absent

    

Present

    

Newborn rash

Absent

   

Present

     

Eczema (worst stage)

Absent

Mild

Moderate

 

Severe

     

Upper respiratory infections per year

1–2

3

4–6

 

>6

     

Candidiasis

None

Oral

Fingernails

 

Systemic

     

Other serious infections

None

   

Severe

     

Fatal infection

Absent

   

Present

     

Hyperextensibility

Absent

   

Present

     

Lymphoma

Absent

   

Present

     

Increased nasal width

<1 SD

1–2 SD

 

>2 SD

      

High palate

Absent

 

Present

       

Young – age correction

>5 yaers

  

2–5 years

 

1–2 years

 

≤ 1 years

  
  1. At ≥ 15 points, the subject is likely to carry an HIES genotype; at 10–14 points, the presence of an HIES genotype is indeterminate; and at <10 points, the subject is unlikely to have an HIES genotype