Multiple dentigerous cysts as a rare presentation of maroteaux– Lamy syndrome

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Prenatal diagnosis of Maroteaux-Lamy syndrome.

Maroteaux-Lamy syndrome exhibits deficient activity of the enzyme arylsulfatase-B in cultured skin fibroblasts. Prenatal diagnosis was successfully attempted in two pregnancies of a consanguineous Chaldean couple whose first child is affected with Maroteaux-Lamy syndrome. In both instances, deficient arylsulfatase-B activity was observed in amniotic fluid cell cultures, and the diagnosis was co...

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Sheehan’s syndrome presenting as psychosis: a rare clinical presentation

 Sheehan’s syndrome (SS) refers to the occurrence of varying degree of hypopituitarism after parturition (1). It is a rare cause of hypopituitarism in developed countries owing to advances in obstetric care and its frequency is decreasing worldwide. However, it is still frequent in underdeveloped and developing countries. Sheehan’s syndrome is often diagnosed late as it evolves slowly (2,3). Re...

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Multiple Hypercementosis: Report of a Rare Presentation

Abstract  Hypercementosis is identified by an excessive, non-neoplastic deposition of radicular cementum and is mostly presented as a solitary lesion or in rare cases as a multiple type. It usually occurs in the premolar and molar region of mandible with no sex predilection. In this paper, we reported a 57-year-old female patient with multiple radiopaque lesions affecting right maxillary second...

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Valvular heart disease in four patients with Maroteaux-Lamy syndrome.

BACKGROUND Maroteaux-Lamy syndrome is a lysosomal storage disease of mucopolysaccharide metabolism (MPS type VI) that may involve the mitral and aortic valves. Affected patients have other skeletal and oropharyngeal malformations that complicate anesthetic and surgical management. METHODS AND RESULTS The present report describes the clinical, echocardiographic, and pathological findings in fo...

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Deep anterior lamellar keratoplasty for Maroteaux-Lamy syndrome.

PURPOSE To present a case of Maroteaux-Lamy syndrome (MLS), which underwent deep anterior lamellar keratoplasty (DALK) for visual rehabilitation. METHODS A 15-year-old girl with MLS was admitted with severe corneal opacity. We performed DALK on her left eye for visual rehabilitation. Big-bubble technique was used to perform lamellar separation. RESULTS Corneal graft was clear 24 months afte...

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ژورنال

عنوان ژورنال: Annals of Maxillofacial Surgery

سال: 2017

ISSN: 2231-0746

DOI: 10.4103/ams.ams_13_17