Hurler-Scheie syndrome (MPS I H-S): In a less severe subclass of MPS I, known as Hurler-Scheie syndrome (also called MPS I H-S), onset of symptoms is usually between the ages of three and eight.
MPS I H-S (Hurler-Scheie syndrome)
MPS I S (Scheie syndrome)
Symptoms ...
There are three variants, differing widely in their severity, with Hurler syndrome being the most severe, Scheie syndrome the mildest and Hurler-Scheie syndrome giving an intermediate phenotype.
At least three forms of Hurler-Scheie syndrome with similar features have been proposed. These are: Hurler syndrome, Hurler-Scheie syndrome, and Scheie syndrome in decreasing order of clinical severity.
This disorder was once divided into three separate syndromes, Hurler syndrome (MPS I-H), Hurler-Scheie syndrome (MPS I-H/S), and Scheie syndrome (MPS I-S), listed from most to least severe.
Scheie Syndrome (MPS IS)
Hurler-Scheie Syndrome (MPS IH/S)
Hurler Syndrome (MPS IH) ...
There are mild and severe forms of the syndrome, the mild form is called Scheie Syndrome, the severe form is called Hurler syndrome, and children with an intermediate form have Hurler-Scheie syndrome.
There is a disease of intermediate severity due to the presence of one Hurler mutation and one Scheie mutation. The disease is termed Hurler-Scheie syndrome.
Mucopolysaccharidosis type I Hurler-Scheie syndrome ... joint stiffness, joint problems, stiff joints
Mucopolysaccharidosis type I Scheie syndrome ... joint stiffness, joint problems
Multicentric osteolysis -- nodulosis -- arthropathy ...
 See also: Hurler syndrome, Scheie syndrome, Deficiency, Symptom, Mucopolysaccharides
  
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