ﺑﺎﺯﮔﺸﺖ ﺑﻪ ﺻﻔﺤﻪ ﻗﺒﻠﯽ
خرید پکیج
تعداد آیتم قابل مشاهده باقیمانده : 3 مورد
نسخه الکترونیک
medimedia.ir

Scoring system with clinical and laboratory tests for individuals in kindreds with HIES

Scoring system with clinical and laboratory tests for individuals in kindreds with HIES
Clinical findings Points*
0 1 2 3 4 5 6 7 8 10
Highest serum-IgE level (international units/mL) <200 200 to 500     501 to 1000       1001 to 2000 >2000
Skin abscesses None   1 to 2   3 to 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 to 14°   15 to 20°       >20°  
Fractures with minor trauma None       1 to 2       >2  
Highest eosinophil count (cells/microL)Δ <700     700 to 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 to 2 3 4 to 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 to 2 SD   >2 SD            
High palate Absent   Present              
Young-age correction >5 years     2 to 5 years   1 to 2 years   ≤1 year    
HIES: hyperimmunoglobulin E syndrome; IgE: immunoglobulin E; SD: standard deviation.
* The entry in the furthest right column is assigned the maximum points allowed for each finding.
¶ Normal <130 international units/mL.
Δ 700/microL = 1 SD, 800/microL = 2 SD above the mean value for normal individuals.
◊ For example, cleft palate, cleft tongue, hemivertebrae, other vertebral anomaly, etc.[1]
§ Compared with age- and sex-matched controls.[2]
References:
  1. Grimbacher B, Holland SM, Gallin JI, et al. Hyper IgE syndrome with recurrent infections: An autosomal dominant multisystem disorder. New Engl J Med 1999; 340:692.
  2. Farkas LG. Anthropometry of the Head and Face in Medicine, 2nd ed, Raven Press, New York, 1994.
Reproduced from: Grimbacher B, Schäffer AA, Holland SM, et al. Genetic linkage of hyper-IgE syndrome to chromosome 4. Am J Hum Genet 1999; 65:735. Table used with the permission of Elsevier Inc. All rights reserved.
Graphic 109912 Version 1.0

آیا می خواهید مدیلیب را به صفحه اصلی خود اضافه کنید؟