Summary
Skin, iliac crest cartilage and tendon of a patient affected with Larsen's syndrome were subjected to biochemical and ultrastructural investigation. A substantial increase in the ratio of glucosamine to galactosamine was found both in skin and cartilage. Ultrastructural abnormalities of collagen fibres and proteoglycan filaments were also found in Larsen's tissues. The significance of these findings are discussed.
Résumé
Des fragments de peau, de tendon et de cartilage prélevé au niveau de la crête iliaque chez un sujet atteint de syndrome de Larsen ont fait l'objet d'examens chimiques et d'observations en microscopie électronique. Une augmentation notable du rapport glucosamine/galactosamine a été mise en évidence, tant au niveau de la peau que du cartilage. On a également trouvé des anomalies de la structure des fibres collagènes et des filaments de protéoglycans. L'auteur discute la significantion de ces faits.
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Cetta, G., Lenzi, L., Ruggeri, A. et al. Biochemical and structural abnormalities of the connective tissue in Larsen's syndrome. International Orthopaedics 3, 47–53 (1979). https://doi.org/10.1007/BF00266325
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DOI: https://doi.org/10.1007/BF00266325