The Esthetic management of pediatric patient with a hereditary disease (Schwachman-Diamond syndrome)
김가영, 이권희, 김민수,
소속 상세정보
김가영 ( Kim Kaa-Yeong ) - Pocheon CHA Medeical collage hospital Dept of Pedodontics
이권희 ( Lee Kwan-Hee ) - Pocheon CHA Medeical collage hospital Dept of Pedodontics
김민수 ( Kim Min-Soo ) - Pocheon CHA Medeical collage hospital Dept of Pedodontics
KMID : 0613420040130020007
Abstract
The Schwachman-Diamond syndrome is an autosomal recessive syndrome(1/20,000 births), consisting of pancreatic insufficiency, neutopenia, which may be intermittent, neutrophil chemotaxis defects, metaphyseal dysostosis, failure to thrive and short stature. Patients present in infancy with poor growth and grease, foul-smelling stools that are characteristic of malabsorption. These children can be readily differentiated from those with cystic fibrosis by their normal
sweat chloride levels, lack of the cystic fibrosis gene, and characteristic metaphyseal lesions. Pathologically, the pancreatic acini are replaced by fat with little fibrosis. The neutropenia may be cyclic. Recurrent pyogenic infections otitis media, pneumonia, dermatitis(fig 1), sepsis are common and a frequent cause of death. In dental examination, these patients had a poor oral hygine and moderate generalized marginal gingivitis, also show delayed primary tooth exfoliation and oral development. This report illustrates a case that pancreatic agenesis 6 yeas-old boy with various esthetic
dental problems has been served the esthetic dental restoration of 6 years.
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