Home (Scheie syndrome)
Home  
 
 
Home » Disease » Scheie syndrome


 

Scheie syndrome

Disease Scarlet feverSchilder Disease

Scheie syndrome
From Healthscout's partner site on acid reflux, AcidRefluxConnection.com ...

 


Scheie syndrome
Alternate Names : Mucopolysaccharidosis type I S, MPS I S
Definition ...

Mucopolysaccharidosis (lysosomal disease) in which there is a defect in _ L iduronidase. Fibroblasts from Scheie syndrome patients do not cross correct fibroblasts from Hurler syndrome, although the two conditions are clinically distinct.

Hurler and Scheie Syndromes (MPS IH, IS, IH/S)
Hurler and Scheie Syndromes (MPS IH, IS, IH/S) ...

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.

Scheie Syndrome (MPS IS)
Hurler-Scheie Syndrome (MPS IH/S)
Hurler Syndrome (MPS IH) ...

Scheie syndrome patients have stiff joints, clouding of the cornea, aortic regurgitation (reflux through the aortic valve in the heart), and survival to a late age with little if any impairment of intellect.

MPS I H-S (Hurler-Scheie syndrome)
MPS I S (Scheie syndrome)
Symptoms ...

Scheie syndrome
Scheuermann's disease
Schistosomiasis
Schizoaffective disorder
Schizoid personality disorder
Schizophrenia
Schizophrenia - disorganized type
Schizophrenia - paranoid type
Schizotypal personality disorder ...

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.

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 ...

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.

MPS I H (Hurler syndrome)
MPS II, Hunter syndrome
MPS IV (Morquio syndrome)
MPS I S (Scheie syndrome) ...

For example, children with a form of mucopolysaccharidosis called Hurler syndrome often die before age 10 from obstructive airway disease, respiratory infections, or cardiac complications. A child with the type known as Scheie syndrome can live into ...

There are 2 clinical subtypes of disease due to deficiency of alpha-L-iduronidase: Hurler syndrome and Scheie syndrome.

See also: Hurler syndrome, Deficiency, Mucopolysaccharides, Symptom, Hunter syndrome

Disease Scarlet feverSchilder Disease

 
 rssRSS