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Table 2 An overview of results during the different stages of the consensus procedure

From: Capturing phenotypic heterogeneity in MPS I: results of an international consensus procedure

 

'results, 1st written round' Considered to be of major importance by the majority and redundant by none of the experts

'results, 2nd written round' Resulting from statistical comparisons of the median expert scores

'results, consensus meeting' Final consensus list

Cognitive decline

x

  

Age at onset of symptoms

x

x

x

Developmental delay

x

x

x

Hydrocephalus

x

  

Kyphosis

x

x

x

Obstructive sleep apnea syndrome

x

 

E

Joint stiffness/arthropathy/contractures

x

 

x

Large head/frontal bossing

 

x

x

Early diagnosis

 

x

 

Dysostosis multiplex

 

x

E

Coarse facial features

 

x

 

Cardiomyopathy

  

x

Growth retardation

  

E

  1. E: excluded from the final severity scale after working group discussions