Abstract
Clinical symptoms of a male infant are described and compared with cases now classified as the De Barsy syndrome, a distinct disorder related to cutis laxa. Morphologically, clastic fibres in skin were frayed and reduced in number and density. The collagen fibril network was normal. Biochemical studies on collagen metabolism in a skin specimen and in cultured skin fibroblasts showed a normal amino acid content and a normal electrophoretic pattern of collagen constituents. The chemotactic migration of cultured fibroblasts was diminished when compared with fibroblasts from donors of different age groups. Immunological investigations revealed an impaired granulocyte function.
Original language | English |
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Pages (from-to) | 428-434 |
Number of pages | 7 |
Journal | European Journal of Pediatrics |
Volume | 145 |
Issue number | 5 |
DOIs | |
State | Published - Oct 1986 |
Externally published | Yes |
Keywords
- Chemotactic migration
- Collagen metabolism
- De Barsy syndrome
- Elastin
- Granulocyte function