Dentigerous cysts are benign odontogenic cysts that are associated with the crowns of permanent teeth. They are usually single in occurrence and located in the mandible. Multiple cysts are reported in patients with conditions such as mucopolysaccharidosis and basal cell nevus syndrome. We present the radiologic findings of bilateral impacted maxillary cuspids with dentigerous cysts displacing t...

We investigated measuring serum alpha-L-iduronidase (EC 3.2.1.76) by a sensitive fluorometric assay in 28 members of a canine family with mucopolysaccharidosis I. If assayed on the day of collection, serum was an acceptable specimen for identifying affected, carrier, and normal dogs. The overall correlation (r) between iduronidase activity in serum and mononuclear leukocytes was 0.966. However,...

Mucopolysaccharidosis type VI or Maroteaux Lamy syndrome is an autosomal recessive lysosomal storage disorder resulting from a deficiency of arylsulfatase B, the clinical features include short stature, hepatosplenomegaly, dysostosis multiplex, stiff joints, corneal clouding, cardiac abnormalities, and facial dysmorphism, with intelligence usually normal. We present evidence of the possible exi...

UNLABELLED Mucopolysaccharidosis III is a rare genetic disease characterized by progressive cognitive decline and severe hyperactivity that does not respond to stimulants. Somatic features are relatively mild. Patients are often initially misdiagnosed as having idiopathic developmental delay, attention deficit/hyperactivity disorder and/or autism spectrum disorders, putting them at risk for unn...

the typical findings of short stature, enlarged head, broad nose, thickened lips, and macroglossia. He had deficient iduronate 2-sulfatase enzyme activity in white cells. He had been receiving weekly i.v. enzymatic replacement therapy A 62-year-old man with chronic atrial fibrillation was admitted to hospital with dyspnea on effort. He had a medical history of mucopolysaccharidosis type II (MPS...

Introduction One of the most important manifestations of mucopolysaccharidosis (MPS) type I, II and VI is a progressive disease of the osteoarticular system. The evaluation of the disease advancement is difficult due to the complexity of symptoms. The characteristic features are progressive limitation of joint mobility and joint pain. These symptoms affect the quality of patient life. A uniform...

The case is reported of a young woman with the Maroteaux-Lamy syndrome (mucopolysaccharidosis type VI) who presented with rapidly progressive dyspnoea due to mitral stenosis. Mitral valve replacement was performed and the appearance of the valve was typical of mucopolysaccharide infiltration. Dyspnoea in patients with the Maroteaux-Lamy syndrome may be due primarily to cardiac valve involvement...